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Surgical Specialist, Neurological Surgeon
24 years of experience
Accepting new patients
Video profile

Credentials

Education ?

Medical School
Universiteit Van Amsterdam (1988)
Foreign school

Awards & Distinctions ?

Awards  
One of America's Leading Experts on:
Carotid Artery Diseases (CAD)
Intracranial Aneurysm
Castle Connolly's Top Doctors™ (2012 - 2013)
Top 10 Doctor - Neighborhood (2014)
Mid-City West
Neurological Surgeon
Associations
American Board of Neurological Surgery
American Association of Neurological Surgeons

Affiliations ?

Dr. Schievink is affiliated with 2 hospitals.

Hospital Affiliations

Score

Rankings

  • Cedars-Sinai Medical Center
    8700 Beverly Blvd, West Hollywood, CA 90048
    •  
    Top 25%
  • John Muir Medical Center-Concord Campus
  • Publications & Research

    Dr. Schievink has contributed to 139 publications.
    Title Chronic Cerebellar Hemorrhage in Spontaneous Intracranial Hypotension: Association with Ventral Spinal Cerebrospinal Fluid Leaks: Clinical Article.
    Date December 2011
    Journal Journal of Neurosurgery. Spine
    Excerpt

    Spontaneous intracranial hypotension is an important cause of new-onset daily persistent headache. Cerebellar hemorrhage has been identified as a possible feature of spontaneous intracranial hypotension. The authors reviewed the MR imaging studies from a group of patients with spontaneous intracranial hypotension to assess the presence of cerebellar hemorrhage.

    Title Frequency of Intracranial Aneurysms in Patients with Spontaneous Intracranial Hypotension.
    Date September 2011
    Journal Journal of Neurosurgery
    Excerpt

    Spontaneous intracranial hypotension (SIH) is a significant cause of new-onset daily persistent headache. A generalized connective tissue disorder also involving the intracranial arteries has been suspected in the population with SIH. Therefore, the authors reviewed angiographic studies for the presence of intracranial aneurysms in a group of patients with SIH.

    Title Hemodialysis Headache and Presyrinx in Spontaneous Intracranial Hypotension.
    Date December 2010
    Journal Neurology
    Title Screening for Intracranial Aneurysms in Patients with Bicuspid Aortic Valve.
    Date May 2010
    Journal Neurology
    Excerpt

    Bicuspid aortic valve (BAV) is a common congenital heart defect affecting half to 2% of the population. A generalized connective tissue disorder also involving the intracranial arteries has been suspected in this patient population. We therefore screened a group of patients with BAV for the presence of intracranial aneurysms.

    Title Spontaneous Spinal Cerebrospinal Fluid Leaks As the Cause of Subdural Hematomas in Elderly Patients on Anticoagulation.
    Date February 2010
    Journal Journal of Neurosurgery
    Excerpt

    Subdural hematoma is a relatively common complication of long-term anticoagulation, particularly in the elderly. The combination of anticoagulation and cerebral cortical atrophy is believed to be sufficient to explain the subdural bleeding. The authors report a series of elderly patients who were on a regimen of anticoagulation and developed chronic subdural hematomas (SDHs) due to a spontaneous spinal CSF leak. They reviewed the medical records and imaging studies of a consecutive group of patients with spontaneous intracranial hypotension who were evaluated at Cedars-Sinai Medical Center. Among 141 patients with spontaneous spinal CSF leaks and spontaneous intracranial hypotension, 3 (2%) were taking anticoagulants at the time of onset of symptoms. The mean age of the 3 patients (1 woman and 2 men) was 74 years (range 68-86 years). All 3 patients had chronic SDHs measuring between 12 and 23 mm in maximal diameter. The SDHs resolved after treatment of the underlying spontaneous spinal CSF leak, and there was no need for hematoma evacuation. Epidural blood patches were used in 2 patients, and percutaneous placement of a fibrin sealant was used in 1 patient. The presence of an underlying spontaneous spinal CSF leak should be considered in patients with chronic SDHs, even among the elderly taking anticoagulants.

    Title Spontaneous Spinal Cerebrospinal Fluid Leak As a Cause of Coma After Craniotomy for Clipping of an Unruptured Intracranial Aneurysm.
    Date April 2009
    Journal Journal of Neurosurgery
    Excerpt

    Spontaneous spinal CSF leaks are best known as a cause of orthostatic headache, but may also be the cause of coma. The authors encountered a unique case of a spontaneous spinal CSF leak causing coma 2 days after craniotomy for clipping of an unruptured aneurysm. This 44-year-old woman with autosomal dominant polycystic kidney disease underwent an uneventful craniotomy for an incidental anterior choroidal artery aneurysm. No intraoperative spinal CSF drainage was used. Two days after surgery the patient became comatose with a left oculomotor nerve palsy. Computed tomography scanning revealed a right extraceberal hematoma and loss of gray-white matter differentiation. The hematoma was evacuated and a diagnosis of hemodialysis disequilibrium syndrome was made. Continuous hemodialysis and hyperosmolar therapy were instituted without any improvement. The CT scans were then reinterpreted as showing sagging of the brain, and the patient was placed in the Trendelenburg position which resulted in prompt improvement in her level of consciousness. A CT myelogram demonstrated an upper thoracic CSF leak that eventually required surgical correction. The patient made a complete neurological recovery. Neurological deterioration after craniotomy may be caused by brain sagging caused by a spontaneous spinal CSF leak, similar to intracranial hypotension due to intraoperative lumbar CSF drainage.

    Title Cerebral Venous Thrombosis in Spontaneous Intracranial Hypotension.
    Date February 2009
    Journal Headache
    Excerpt

    The occurrence of cerebral venous thrombosis has been reported among patients with spontaneous intracranial hypotension, but a causal relationship has not been clearly established. We reviewed our experience with spontaneous intracranial hypotension and cerebral venous thrombosis and we reviewed the relevant literature to evaluate the relationship between these 2 entities.

    Title Absence of Tgfbr2 Mutations in Patients with Spontaneous Spinal Csf Leaks and Intracranial Hypotension.
    Date July 2008
    Journal The Journal of Headache and Pain
    Excerpt

    A heritable connective-tissue-disorder often is suspected in patients with spontaneous spinal CSF leaks and intracranial hypotension, but the nature of the disorder remains unknown in most patients. The aim of this study was to assess the gene encoding TGF-beta receptor-2 (TGFBR2) as a candidate gene for spinal CSF leaks. We searched the TGFBR2 gene for mutations in eight patients with spontaneous spinal CSF leaks who also had other features associated with TGFBR2 mutations, i.e., skeletal features of Marfan syndrome, arterial tortuosity, and(or) thoracic aortic aneurysm. The mean age of these 7 women and 1 man was 38 years (range 14-60 years). We detected no TGFBR2 mutations and conclude that TGFBR2 mutations are not a major factor in spontaneous spinal CSF leaks.

    Title Diagnostic Criteria for Spontaneous Spinal Csf Leaks and Intracranial Hypotension.
    Date June 2008
    Journal Ajnr. American Journal of Neuroradiology
    Excerpt

    BACKGROUND AND PURPOSE: Comprehensive diagnostic criteria encompassing the varied clinical and radiographic manifestations of spontaneous intracranial hypotension are not available. Therefore, we propose a new set of diagnostic criteria. MATERIALS AND METHODS: The diagnostic criteria are based on results of brain and spine imaging, clinical manifestations, results of lumbar puncture, and response to epidural blood patching. The diagnostic criteria include criterion A, the demonstration of extrathecal CSF on spinal imaging. If criterion A is not met, criterion B, which is cranial MR imaging findings of spontaneous intracranial hypotension, follows, with at least one of the following: 1) low opening pressure, 2) spinal meningeal diverticulum, or 3) improvement of symptoms after epidural blood patch. If criteria A and B are not met, there is criterion C, the presence of all of the following or at least 2 of the following if typical orthostatic headaches are present: 1) low opening pressure, 2) spinal meningeal diverticulum, and 3) improvement of symptoms after epidural blood patch. These criteria were applied to a group of 107 consecutive patients evaluated for spontaneous spinal CSF leaks and intracranial hypotension. RESULTS: The diagnosis was confirmed in 94 patients, with use of criterion A in 78 patients, criterion B in 11 patients, and criterion C in 5 patients. CONCLUSIONS: A new diagnostic scheme is presented reflecting the wide spectrum of clinical and radiographic manifestations of spontaneous spinal CSF leaks and intracranial hypotension.

    Title Frequency of Spontaneous Intracranial Hypotension in the Emergency Department.
    Date June 2008
    Journal The Journal of Headache and Pain
    Excerpt

    Spontaneous intracranial hypotension is considered a rare disorder. We conducted a study on the frequency of spontaneous intracranial hypotension in the emergency department (ED). We identified patients with spontaneous intracranial hypotension evaluated in the ED of a large urban hospital between 1 January 2003 and 31 December 2006. For comparison, we also identified all patients with spontaneous subarachnoid haemorrhage (SAH). Eleven patients with previously undiagnosed spontaneous intracranial hypotension were evaluated in the ED during the four-year time period. All patients presented with positional headaches and the duration of symptoms varied from one day to three months. None of the patients were correctly diagnosed with spontaneous intracranial hypotension in the ED. During the same time period, 23 patients with aneurysmal SAH were evaluated. Spontaneous intracranial hypotension is more common than previously appreciated and the diagnosis in the ED remains problematic.

    Title Anaphylactic Reactions to Fibrin Sealant Injection for Spontaneous Spinal Csf Leaks.
    Date April 2008
    Journal Neurology
    Title Upright Mri in Spontaneous Spinal Cerebrospinal Fluid Leaks and Intracranial Hypotension.
    Date November 2007
    Journal Headache
    Excerpt

    Orthostatic headaches are the hallmark of spontaneous intracranial hypotension, but MRIs are traditionally obtained in the supine position. We investigated the utility of upright MRI of the brain in 6 patients with spontaneous intracranial hypotension. No discernable differences were noted between the supine and upright images.

    Title Precipitating Factors of Spontaneous Spinal Csf Leaks and Intracranial Hypotension.
    Date September 2007
    Journal Neurology
    Title Reversal of Coma with an Injection of Glue.
    Date May 2007
    Journal Lancet
    Title Reversible Cerebral Vasoconstriction in Spontaneous Intracranial Hypotension.
    Date April 2007
    Journal Headache
    Excerpt

    Myelography showed an opening pressure of 0 cm H2O and multiple thoracic meningeal diverticula in a 52-year-old woman suffering from orthostatic headaches of instantaneous onset. MR-angiography showed severe segmental arterial stenosis of the anterior and posterior circulation, which resolved over a 4-day period following an epidural blood patch. Spontaneous intracranial hypotension should be considered in the differential diagnosis of reversible cerebral vasoconstriction.

    Title Epidemiology of Cervical Artery Dissection.
    Date April 2007
    Journal Frontiers of Neurology and Neuroscience
    Excerpt

    The diagnosis of cervical artery dissection has become routine. In young patients, spontaneous CAD is the cause of up to one-fourth of strokes. The incidence of spontaneous CAD is approximately 5 per 100,000 per year and is highest in autumn. The long-term recurrence rate is only approximately 1% per year, but it is higher in patients with familial arterial disease.

    Title Spontaneous Spinal Cerebrospinal Fluid Leaks: a Review.
    Date August 2006
    Journal Neurosurgical Focus
    Excerpt

    Spontaneous intracranial hypotension has become a well-recognized clinical entity, but it remains an uncommonly, and probably underdiagnosed, cause of headache; its estimated prevalence is only one in 50,000 individuals. The clinical spectrum of spontaneous intracranial hypotension is quite variable and includes headache, neck stiffness, cranial nerve dysfunction, radicular arm pain, and symptoms of diencephalic or hindbrain herniation. Leakage of the spinal cerebrospinal fluid (CSF) is the most common cause of spontaneous intracranial hypotension. A combination of an underlying weakness of the spinal meninges and a more or less trivial traumatic event is often found to cause this event in these patients. Typical magnetic resonance imaging findings include diffuse pachymeningeal enhancement, subdural fluid collections, and downward displacement of the brain, sometimes mimicking a Chiari I malformation. Opening pressure is often, but not always, low, and examination of CSF may reveal pleocytosis, an elevated protein count, and xanthochromia. The use of myelography computerized tomography scanning is the most reliable method for the accurate localization of the CSF leak. Most CSF leaks are found at the cervicothoracic junction or in the thoracic spine. The initial treatment of choice is a lumbar epidural blood patch, regardless of the location of the CSF leak. If the epidural blood patch fails, the blood patch procedure can be repeated at the lumbar level, or a blood patch can be directed at the exact site of the leak. Surgical repair of the CSF leak is safe and generally successful, although a distinct structural cause of the leak often is not found.

    Title Quadriplegia and Cerebellar Hemorrhage in Spontaneous Intracranial Hypotension.
    Date July 2006
    Journal Neurology
    Title Spontaneous Spinal Cerebrospinal Fluid Leaks and Intracranial Hypotension.
    Date May 2006
    Journal Jama : the Journal of the American Medical Association
    Excerpt

    CONTEXT: Spontaneous intracranial hypotension is caused by spontaneous spinal cerebrospinal fluid (CSF) leaks and is known for causing orthostatic headaches. It is an important cause of new headaches in young and middle-aged individuals, but initial misdiagnosis is common. OBJECTIVE: To summarize existing evidence regarding the epidemiology, pathophysiology, diagnosis, and management of spontaneous spinal CSF leaks and intracranial hypotension. EVIDENCE ACQUISITION: MEDLINE (1966-2005) and OLDMEDLINE (1950-1965) were searched using the terms intracranial hypotension, CSF leak, low pressure headache, and CSF hypovolemia. Reference lists of these articles and ongoing investigations in this area were used as well. EVIDENCE SYNTHESIS: Spontaneous intracranial hypotension is caused by single or multiple spinal CSF leaks. The incidence has been estimated at 5 per 100,000 per year, with a peak around age 40 years. Women are affected more commonly than men. Mechanical factors combine with an underlying connective tissue disorder to cause the CSF leaks. An orthostatic headache is the prototypical manifestation but other headache patterns occur as well, and associated symptoms are common. Typical magnetic resonance imaging findings include subdural fluid collections, enhancement of the pachymeninges, engorgement of venous structures, pituitary hyperemia, and sagging of the brain (mnemonic: SEEPS). Myelography is the study of choice to identify the spinal CSF leak. Treatments include bed rest, epidural blood patching, percutaneous placement of fibrin sealant, and surgical CSF leak repair, but outcomes have been poorly studied and no management strategies have been studied in properly controlled randomized trials. CONCLUSIONS: Spontaneous intracranial hypotension is not rare but it remains underdiagnosed. The spectrum of clinical and radiographic manifestations is varied, with diagnosis largely based on clinical suspicion, cranial magnetic resonance imaging, and myelography. Numerous treatment options are available, but much remains to be learned about this disorder.

    Title Pseudo-subarachnoid Hemorrhage: a Ct-finding in Spontaneous Intracranial Hypotension.
    Date February 2006
    Journal Neurology
    Excerpt

    Increased attenuation in the basilar cisterns or along the tentorium cerebelli resembling subarachnoid hemorrhage (SAH) may be found on CT in the absence of blood (pseudo-SAH). The authors found pseudo-SAH on CT in four of 40 patients with spontaneous intracranial hypotension. All four patients had brain sagging with obliteration of the cisterns and pachymeningeal enhancement along the tentorium cerebelli. Spontaneous intracranial hypotension should be included in the differential diagnosis of pseudo-SAH.

    Title Cranial Mri Predicts Outcome of Spontaneous Intracranial Hypotension.
    Date February 2006
    Journal Neurology
    Excerpt

    The outcome of spontaneous intracranial hypotension has been unpredictable. The results of initial MRI were correlated to outcome of treatment in 33 patients with spontaneous intracranial hypotension. A good outcome was obtained in 25 (97%) of 26 patients with an abnormal MRI vs only 1 (14%) of 7 patients with a normal MRI (p = 0.00004). These findings show that normal initial MRI is predictive of poor outcome in spontaneous intracranial hypotension.

    Title Spectrum of Subdural Fluid Collections in Spontaneous Intracranial Hypotension.
    Date November 2005
    Journal Journal of Neurosurgery
    Excerpt

    OBJECT: Spontaneous intracranial hypotension is a noteworthy but commonly misdiagnosed cause of new daily persistent headaches. Subdural fluid collections are frequent radiographic findings, but they can be interpreted as primary rather than secondary pathological entities, and uncertainties exist regarding their optimal management. The authors therefore reviewed their experience with subdural fluid collections in 40 consecutive patients with spontaneous spinal cerebrospinal fluid (CSF) leaks and intracranial hypotension. METHODS: The mean age of the 26 female and 14 male patients was 43 years (range 13-72 years). Subdural fluid collections were present in 20 patients (50%); 12 of these patients (60%) had subdural hygromas alone, and eight (40%) had subacute to chronic subdural hematomas (SDHs) associated with significant mass effect. The subdural hygromas resolved within several days to weeks following treatment of the underlying CSF leak. Three patients with SDHs underwent evacuation of the hematoma prior to the establishment of the diagnosis of spontaneous intracranial hypotension, but the SDHs did not resolve until the underlying spinal CSF leak was treated. In the remaining five patients, the CSF leak was treated primarily and the SDHs resolved over a 1- to 3-month period without the need for evacuation. CONCLUSIONS: Subdural fluid collections are common in spontaneous intracranial hypotension, varying in appearance from thin subdural hygromas to large SDHs associated with significant mass effect. These collections can be safely managed by directing treatment at the underlying CSF leak without the need for hematoma evacuation.

    Title Filum Ependymoma Mimicking Spontaneous Intracranial Hypotension.
    Date September 2005
    Journal Headache
    Excerpt

    A 34-year-old man with a 2-week history of orthostatic headaches and a "dry tap" at lumbar puncture was found to have a lumbar intradural mass on magnetic resonance imaging (MRI) examination. A myxopapillary ependymoma was resected and the patient's headache completely resolved. The combination of spontaneous orthostatic headaches and a "dry tap" at the time of lumbar puncture does not always indicate the presence of a spontaneous cerebrospinal fluid (CSF) leak and intracranial hypotension.

    Title Frequency of Incidental Intracranial Aneurysms in Neurofibromatosis Type 1.
    Date May 2005
    Journal American Journal of Medical Genetics. Part A
    Excerpt

    Neurofibromatosis type 1 (NF1) is often mentioned among the heritable connective tissue disorders associated with intracranial aneurysms, but the association has not been firmly established. We therefore reviewed a contemporary series of hospitalized patients with NF1, many of whom underwent brain magnetic resonance imaging (MRI). We identified patients with NF1 who were hospitalized at Cedars-Sinai Medical Center, Los Angeles, California, between January 1, 1997 and December 31, 2001 through the hospital's centralized medical records system using DRG codes. The mean age of the 39 patients was 30.4 years, and 22 patients had undergone MRI of the brain for the evaluation of symptoms due to the presence of central or peripheral nervous system tumors. Incidental intracranial aneurysms were detected in 2 (5%) of the 39 patients. Limiting the patient population to the 22 patients who had undergone MRI examination, the detection rate was 9%. This was significantly (P < 0.005) higher than the aneurysm detection rate in a control population (0/526 [0%]) of patients hospitalized for primary or secondary brain tumors, all of whom had undergone MRI examination. Our study suggests that patients with NF1 are at an increased risk of developing intracranial aneurysms.

    Title Racial Disparities in Subarachnoid Hemorrhage Mortality: Los Angeles County, California, 1985-1998.
    Date November 2004
    Journal Neuroepidemiology
    Excerpt

    We examined the racial distribution of subarachnoid hemorrhage (SAH) mortality in a unique multiracial community. Mortality rates for SAH among the residents of Los Angeles County were calculated from death certificate data (1985-1998). Residential postal zones were classified into three strata as a measure of socioeconomic status. The number of SAH deaths was 2,897. The age-adjusted SAH mortality rate was 1.9 in whites, 2.7 in Hispanics, 3.0 in Asians and 3.7 in blacks. In those younger than 70 years of age, the SAH mortality rate among blacks was 2.2 times that of whites and 1.8 times that of Hispanics and Asians. The SAH mortality rate declines after age 70 in blacks. The SAH mortality rate was higher in women than in men in all races and it was highest in elderly Asian women (23.5 per 100,000). An inverse relationship was observed between income and SAH mortality rates in all racial groups except whites.

    Title Treatment of Spontaneous Intracranial Hypotension with Percutaneous Placement of a Fibrin Sealant. Report of Four Cases.
    Date June 2004
    Journal Journal of Neurosurgery
    Excerpt

    Spontaneous intracranial hypotension due to a cerebrospinal fluid (CSF) leak in the spine is an important cause of new, daily persistent headaches. Most patients respond well to conservative treatments including epidural blood patching. Limited options for effective treatment are available for patients in whom these treatments fail. The authors treated four patients (mean age 38 years; range 26-43 years) with percutaneous placement of a fibrin sealant. All these patients presented with intractable positional headaches. The CSF leak was located in the lower cervical spine in three patients and in the lower thoracic spine in one patient. Four to 20 milliliters of fibrin sealant was injected at the site of the CSF leak. Two of the four patients became asymptomatic within days of the procedure and thus avoided surgery. There were no complications of this procedure. Percutaneous placement of a fibrin sealant is a safe, minimally invasive treatment for spontaneous spinal CSF leaks and should be considered in patients in whom conservative treatment has failed.

    Title False Localizing Sign of C1-2 Cerebrospinal Fluid Leak in Spontaneous Intracranial Hypotension.
    Date April 2004
    Journal Journal of Neurosurgery
    Excerpt

    OBJECT: Spontaneous intracranial hypotension due to a spinal cerebrospinal fluid (CSF) leak is an important cause of new daily persistent headaches. Spinal neuroimaging is important in the treatment of these patients, particularly when direct repair of the CSF leak is contemplated. Retrospinal C1-2 fluid collections may be noted on spinal imaging and these are generally believed to correspond to the site of the CSF leak. The authors undertook a study to determine the significance of these C1-2 fluid collections. METHODS: The patient population consisted of a consecutive group of 25 patients (18 female and seven male) who were evaluated for surgical repair of a spontaneous spinal CSF leak. The mean age of the 18 patients was 38 years (range 13-72 years). All patients underwent computerized tomography myelography. Three patients (12%) had extensive retrospinal C1-2 fluid collections; the mean age of this woman and these two men was 41 years (range 39-43 years). The actual site of the CSF leak was located at the lower cervical spine in these patients and did not correspond to the site of the retrospinal C1-2 fluid collection. CONCLUSIONS: A retrospinal fluid collection at the C1-2 level does not necessarily indicate the site of the CSF leak in patients with spontaneous intracranial hypotension. This is an important consideration in the treatment of these patients because therapy may be inadvertently directed at this site.

    Title Connective Tissue Disorders with Spontaneous Spinal Cerebrospinal Fluid Leaks and Intracranial Hypotension: a Prospective Study.
    Date March 2004
    Journal Neurosurgery
    Excerpt

    OBJECTIVE: Intracranial hypotension attributable to a spontaneous spinal cerebrospinal fluid (CSF) leak is an increasingly recognized cause of postural headaches. The cause of these leaks is poorly understood, but it is likely multifactorial and may involve a primary connective tissue disorder. We undertook a study to estimate the contribution of systemic connective tissue disorders to the development of spontaneous spinal CSF leaks. METHODS: We examined a group of 18 consecutive patients with spontaneous spinal CSF leaks for features of a connective tissue disorder. RESULTS: The mean age of the 15 female patients and 3 male patients was 38 years (range, 22-55 yr). Seven patients (38%) demonstrated stigmata of a systemic connective tissue disorder, and three distinct types of disorders could be identified, as follows. 1) The association of spontaneous spinal CSF leaks and minor skeletal features of Marfan syndrome was noted for three patients. 2) Ehlers-Danlos syndrome Type II was noted for two patients. 3) Joint hypermobility associated with marked attenuation of the dorsal muscular fascia, precluding proper wound closure, was noted for two patients. In addition, isolated small-joint hypermobility was observed for five patients (28%). Slit-lamp ocular examinations, echocardiographic evaluations, histopathological examinations of skin biopsy specimens, and renal scanning did not reveal any other features of a systemic connective tissue disorder. CONCLUSION: Findings suggesting connective tissue disorders are common among patients with spontaneous spinal CSF leaks, and manifestations may be subtle. A variety of disorders can be identified, probably reflecting genetic heterogeneity. Problems with wound healing may occur as a result of the systemic nature of the underlying connective tissue disorder.

    Title Recurrent Spontaneous Spinal Cerebrospinal Fluid Leak Associated with "nude Nerve Root" Syndrome: Case Report.
    Date February 2004
    Journal Neurosurgery
    Excerpt

    OBJECTIVE AND IMPORTANCE: Spontaneous spinal cerebrospinal fluid (CSF) leaks have been noted occasionally at multiple sites in the same patient, but recurrent spontaneous spinal CSF leaks have not been documented. We describe a patient with a recurrent CSF leak who was found at surgery to have an absence of the entire nerve root sleeve at multiple thoracic levels. CLINICAL PRESENTATION: A 29-year-old woman bodybuilder noted an excruciating orthostatic headache associated with nausea. The neurological examination was unremarkable, and a magnetic resonance imaging examination showed the typical changes of intracranial hypotension. Computed tomographic myelography showed an extensive bilateral lower cervical CSF leak. INTERVENTION: The patient underwent bilateral lower cervical nerve root explorations, and several small dural holes were found. The CSF leaks were repaired, but 3 months later, computed tomographic myelography showed a new CSF leak in the midthoracic area. A thoracic laminectomy was performed, and several nerve roots were found to be completely devoid of dura. After the CSF leaks were repaired, there was significant improvement in her headaches. CONCLUSION: A recurrent spontaneous spinal CSF leak may occur in patients with intracranial hypotension at a site previously documented not to be associated with a CSF leak. Absent nerve root sleeves may be found in patients with spontaneous spinal CSF leaks ("nude nerve root" syndrome), and these patients may be at increased risk of developing a recurrent CSF leak.

    Title Misdiagnosis of Spontaneous Intracranial Hypotension.
    Date January 2004
    Journal Archives of Neurology
    Excerpt

    BACKGROUND: Spontaneous intracranial hypotension is an important cause of "new daily persistent headaches" but is not a well-recognized entity. The misdiagnosis of spontaneous intracranial hypotension can have serious consequences. METHODS: The clinical course in 18 consecutive patients with spontaneous intracranial hypotension who were evaluated for definitive surgical treatment of the underlying spontaneous spinal cerebrospinal fluid leak from January 1, 2001, through June 30, 2002, was investigated by correspondence with the patients and physicians. RESULTS: Seventeen patients (94%) initially received an incorrect diagnosis, and the diagnostic delay ranged from 4 days to 13 years (median, 5 weeks; mean, 13 months). Migraine, meningitis, and psychogenic disorder were the most commonly entertained diagnoses. Diagnostic or therapeutic procedures for disorders that mimicked spontaneous intracranial hypotension included cerebral arteriography in 2 patients, craniotomies for Chiari malformation in 2 patients, craniotomy for evacuation of subdural hematomas in 1 patient, and brain biopsy in 1 patient. CONCLUSIONS: Patients with spontaneous intracranial hypotension are commonly misdiagnosed, causing a significant delay in the initiation of effective treatments and exposing patients to the risks associated with treatment for disorders that mimic intracranial hypotension. Increasing the awareness of this spontaneous type of intracranial hypotension is required to decrease the high rate of misdiagnosis.

    Title Recurrent Spontaneous Spinal Cerebrospinal Fluid Leaks and Intracranial Hypotension: a Prospective Study.
    Date December 2003
    Journal Journal of Neurosurgery
    Excerpt

    OBJECT: Intracranial hypotension due to a spontaneous spinal cerebrospinal fluid (CSF) leak is an increasingly recognized cause of postural headaches, but reliable follow-up data are lacking. The authors undertook a study to determine the risk of a recurrent spontaneous spinal CSF leak. METHODS: The patient population consisted of a consecutive group of 18 patients who had been evaluated for consideration of surgical repair of a spontaneous spinal CSF leak. The mean age of the 15 women and three men was 38 years (range 22-55 years). The mean duration of follow up was 36 months (range 6-132 months). The total follow-up time was 654 months. A recurrent spinal CSF leak was defined on the basis of computerized tomography myelography evidence of a CSF leak in a previously visualized but unaffected spinal location. Five patients (28%) developed a recurrent spinal CSF leak; the mean age of these four women and one man was 36 years. A recurrent CSF leak developed in five (38%) of 13 patients who had undergone surgical CSF leak repair, compared with none (0%) of five patients who had been treated non-surgically (p = 0.249). The recurrent leak occurred between 10 and 77 months after the initial CSF leak, but within 2 or 3 months of successful surgical repair of the leak in all patients. CONCLUSIONS: Recurrent spontaneous spinal CSF leaks are not rare, and the recent successful repair of such a leak at another site may be an important risk factor.

    Title Development of an Intracranial Ependymoma at the Site of a Pre-existing Cavernous Malformation.
    Date August 2003
    Journal Surgical Neurology
    Excerpt

    BACKGROUND: The ability of vascular anomalies to induce neoplastic transformation in normal brain parenchyma has been suggested but not demonstrated. We present a novel case in which a patient with a pre-existing cavernous malformation developed an adjacent ependymoma. CASE DESCRIPTION: A 72-year-old man developed an anaplastic ependymoma at the site of a pre-existing cavernous malformation. This is the first documented instance of an ependymoma developing at the site of an existing cavernous malformation. The colocalization of both lesions and the low incidence of supratentorial ependymomas in this age group makes it unlikely that their coexistence represents a random event. Immunohistochemistry demonstrated vascular endothelial growth factor (VEGF) production by the cavernous malformation and robust VEGF receptor expression by the ependymoma. CONCLUSIONS: Based on these findings, we suggest that production of VEGF by vascular malformations may play a role in the neoplastic transformation of adjacent tissue.

    Title A Syndrome of Spontaneous Cerebral and Cervical Artery Dissections with Angiolipomatosis. Report of Two Cases.
    Date June 2003
    Journal Journal of Neurosurgery
    Excerpt

    A primary or systemic arteriopathy is frequently suspected in patients with spontaneous cerebral or cervical artery dissections. The authors report on two patients with such dissections accompanied by angiolipomatosis, a previously unreported association, and propose a common developmental defect in these patients. A 50-year-old man with subcutaneous angiolipomatosis developed painful monocular blindness. Angiography studies revealed a spontaneous extracranial internal carotid artery (ICA) dissection and an ipsilateral fusiform intracranial ICA aneurysm. The ICA dissection was treated with aspirin, and after 6 months a craniotomy was performed. The aneurysm was found to be fusiform; it involved the entire supraclinoid portion of the ICA, and was wrapped with cotton. A 49-year-old man with a congenitally bicuspid aortic valve and subcutaneous angiolipomatosis developed posterior neck pain. Magnetic resonance imaging and angiography demonstrated a fusiform distal vertebral artery aneurysm. A craniotomy was performed and the aneurysm was found to incorporate the posterior inferior cerebellar artery as well as a perforating artery: the lesion was wrapped cotton. The tunica media of the arteries of the head and neck as well as the aortic valvular cusps are derived from neural crest cells, and angiolipomatosis has been associated with tumors of neural crest derivation. These associations indicate that a neural crest disorder may be the underlying abnormality in these patients.

    Title Intracranial Aneurysm Surgery in Ehlers-danlos Syndrome Type Iv.
    Date October 2002
    Journal Neurosurgery
    Excerpt

    OBJECTIVE: Ehlers-Danlos syndrome Type IV is a heritable connective tissue disorder with frequent neurovascular manifestations, such as intracranial aneurysms. Patients with this syndrome have notoriously fragile blood vessels, and the reported mortality rate for any type of vascular surgical procedure is 40%. This syndrome is rare, however, and the complication rate of aneurysm surgery may have been overestimated. METHODS: We reviewed our experience with aneurysm surgery in a group of patients with Ehlers-Danlos syndrome Type IV. RESULTS: The patient population consisted of three women and one man with a mean age of 44 years (age range, 20-57 yr). One patient, who had a ruptured anterior circulation aneurysm, died as a direct result of surgery because of marked vascular fragility. Three patients underwent successful surgery, consisting of a craniotomy and clipping of a ruptured anterior circulation aneurysm in two patients and a craniotomy and clip ligation of the parent artery in one patient with a ruptured dissecting vertebral artery aneurysm. Intraoperatively, mild vascular or connective tissue fragility was commonly observed. Postoperative complications (e.g., spontaneous pneumothorax and vertebral artery dissection) also were common but did not result in permanent morbidity. CONCLUSION: The risk of neurovascular surgery is high in patients with Ehlers-Danlos syndrome Type IV and intra- and postoperative complications are common. However, most patients tolerate the operation without permanent morbidity.

    Title Spontaneous Spinal Cerebrospinal Fluid Leaks and Minor Skeletal Features of Marfan Syndrome: a Microfibrillopathy.
    Date March 2002
    Journal Journal of Neurosurgery
    Excerpt

    OBJECT: Spontaneous spinal cerebrospinal fluid (CSF) leaks are increasingly recognized as a cause of postural headaches. The authors examined a group of patients suffering from spontaneous spinal CSF leaks who also had minor skeletal features of Marfan syndrome for abnormalities of fibrillin-containing microfibrils. METHODS: Patients with spontaneous CSF leaks were evaluated for the clinical characteristics of connective tissue disorders. Skin biopsies were obtained in three patients with skeletal manifestations that constitute part of the Marfan syndrome phenotype. Cultured fibroblasts were studied for fibrillin-1 synthesis and incorporation into the extracellular matrix (ECM) by performing quantitative metabolic labeling and immunohistochemical analysis. Among 20 consecutive patients found to have spinal CSF leaks, four (20%) exhibited minor skeletal features of Marfan syndrome, but lacked any ocular or cardiovascular abnormalities. The mean age of these patients (30 years) was lower than that of the 16 patients without skeletal abnormalities (44 years; p = 0.01). Abnormalities in fibrillin-1 metabolism and immunostaining were detected in all three patients with the skeletal abnormalities who underwent examination, but not in a control patient without these skeletal manifestations. CONCLUSIONS: Twenty percent of patients who experience spontaneous spinal CSF leaks have minor skeletal features of Marfan syndrome. The authors demonstrated abnormalities in fibrillin-1 protein deposition in all patients examined, but only one person was found to have a fibrillin-1 abnormality typically found in classic Marfan syndrome. The results indicate that there is a heterogeneous involvement of other components of ECM microfibrils at the basis of this cerebrospinal manifestation. In addition, the authors identified a connective-tissue etiological factor in a group of disorders not previously classified as such.

    Title Haploinsufficiency for One Col3a1 Allele of Type Iii Procollagen Results in a Phenotype Similar to the Vascular Form of Ehlers-danlos Syndrome, Ehlers-danlos Syndrome Type Iv.
    Date December 2001
    Journal American Journal of Human Genetics
    Excerpt

    Mutations in the COL3A1 gene that encodes the chains of type III procollagen result in the vascular form of Ehlers-Danlos syndrome (EDS), EDS type IV, if they alter the sequence in the triple-helical domain. Although other fibrillar collagen-gene mutations that lead to allele instability or failure to incorporate proalpha-chains into trimers-and that thus reduce the amount of mature molecules produced-result in clinically apparent phenotypes, no such mutations have been identified in COL3A1. Furthermore, mice heterozygous for Col3a1 "null" alleles have no identified phenotype. We have now found three frameshift mutations (1832delAA, 413delC, and 555delT) that lead to premature termination codons (PTCs) in exons 27, 6, and 9, respectively, and to allele-product instability. The mRNA from each mutant allele was transcribed efficiently but rapidly degraded, presumably by the mechanisms of nonsense-mediated decay. In a fourth patient, we identified a point mutation, in the final exon, that resulted in a PTC (4294C-->T [Arg1432Ter]). In this last instance, the mRNA was stable but led to synthesis of a truncated protein that was not incorporated into mature type III procollagen molecules. In all probands, the presenting feature was vascular aneurysm or rupture. Thus, in contrast to mutations in genes that encode the dominant protein of a tissue (e.g., COL1A1 and COL2A1), in which "null" mutations result in phenotypes milder than those caused by mutations that alter protein sequence, the phenotypes produced by these mutations in COL3A1 overlap with those of the vascular form of EDS. This suggests that the major effect of many of these dominant mutations in the "minor" collagen genes may be expressed through protein deficiency rather than through incorporation of structurally altered molecules into fibrils.

    Title Spontaneous Retroclival Hematoma Presenting As a Thunderclap Headache. Case Report.
    Date October 2001
    Journal Journal of Neurosurgery
    Excerpt

    An excruciating headache of instantaneous onset, or thunderclap headache, may be caused by a variety of serious disorders, including aneurysmal subarachnoid hemorrhage, cerebral venous sinus thrombosis, pituitary apoplexy, and carotid artery or vertebral artery dissection. The authors describe a patient with this type of headache who was found to have a spontaneous retroclival hematoma. A 49-year-old woman experienced an instantaneous excruciating headache. Results of computerized tomography (CT) scans of the head were normal, but on examination of the cerebrospinal fluid xanthochromia was found. Magnetic resonance (MR) imaging of the cervical spine revealed a retroclival hematoma. Three cerebral angiographic studies did not reveal the source of the hemorrhage and a repeated MR image demonstrated resolution of the hematoma. The patient made an uneventful recovery. Spontaneous retroclival hematoma is an exceedingly rare type of intracranial hemorrhage and may be associated with normal findings on CT scans. Spontaneous retroclival hematoma should be included in the differential diagnosis of thunderclap headache.

    Title Spontaneous Intracranial Hypotension Mimicking Aneurysmal Subarachnoid Hemorrhage.
    Date August 2001
    Journal Neurosurgery
    Excerpt

    OBJECTIVE: An excruciating headache of instantaneous onset is known as a thunderclap headache. A subarachnoid hemorrhage is the prototypical cause, but other serious disorders may also present with a thunderclap headache, including cerebral venous sinus thrombosis, carotid artery dissection, and pituitary apoplexy. We report a group of patients with thunderclap headaches as the initial manifestation of spontaneous intracranial hypotension caused by a spinal cerebrospinal fluid leak. METHODS: Among 28 patients with spontaneous intracranial hypotension due to a documented spinal cerebrospinal fluid leak, four (14%) initially experienced an excruciating headaches of instantaneous onset. RESULTS: The mean age of the four patients (two men and two women) was 35 years (range, 24-45 yr). Nuchal rigidity was present in the three patients who sought early medical attention, and they underwent emergency computed tomographic scanning, lumbar puncture, and cerebral angiography to rule out an aneurysmal subarachnoid hemorrhage. The delay between the onset of headache and diagnosis of intracranial hypotension ranged from 4 days to 5 weeks. A fourth patient did not seek medical attention until 1 month after the ictus. CONCLUSION: Spontaneous intracranial hypotension should be included in the differential diagnosis of thunderclap headache, even when meningismus is present.

    Title Spontaneous Dissection of the Carotid and Vertebral Arteries.
    Date March 2001
    Journal The New England Journal of Medicine
    Title Quadrigeminal Variant of Perimensencephalic Nonaneurysmal Subarchnoid Hemorrhage.
    Date February 2001
    Journal Neurosurgery
    Title The Treatment of Spontaneous Carotid and Vertebral Artery Dissections.
    Date January 2001
    Journal Current Opinion in Cardiology
    Excerpt

    Spontaneous dissections of the carotid and vertebral arteries in the neck are a common cause of stroke in young and middle-aged people. Moreover, they are increasingly recognized as the cause of a wide variety of other, more subtle, neurologic signs and symptoms. The cause of these arterial dissections largely remains unexplained but probably involves a combination of genetic and environmental factors. Magnetic resonance imaging has largely surpassed angiography as the imaging study of choice. The treatment of carotid and vertebral artery dissections is based on rather incomplete evidence. Anticoagulation with heparin followed by warfarin remains the treatment of choice in most major medical centers and is supported by the demonstration of emboli as the most common cause of stroke in these patients. The burgeoning interest in endovascular techniques has resulted in many patients being treated for carotid and vertebral artery dissections with percutaneous angioplasty and stent deployment. Although the treatment of dissections is generally well tolerated and the radiographic results are impressive, most dissections heal spontaneously and the associated aneurysms never rupture and rarely cause delayed ischemic symptoms. Surgical treatment of dissections, consisting of an in situ interposition graft or extracranial-intracranial bypass, is indicated only for those patients with persistent symptoms refractory to maximal medical therapy who are not candidates for endovascular treatment.

    Title Origin of Pretruncal Nonaneurysmal Subarachnoid Hemorrhage: Ruptured Vein, Perforating Artery, or Intramural Hematoma?
    Date November 2000
    Journal Mayo Clinic Proceedings. Mayo Clinic
    Excerpt

    Pretruncal (perimesencephalic) nonaneurysmal subarachnoid hemorrhage (SAH) is a benign variant of SAH. Although angiography fails to show a source of the hemorrhage, mild basilar artery narrowing may be observed. The cause of pretruncal nonaneurysmal SAH has not been established. Recent imaging studies have demonstrated that the center of this type of SAH is not around the mesencephalon but is in the prepontine or interpeduncular cistern with the hemorrhage closely associated with the basilar artery. We review the possible sources of hemorrhage in these cisterns and hypothesize that pretruncal nonaneurysmal SAH is caused by a primary intramural hematoma of the basilar artery. Such an intramural hematoma would explain bleeding under low pressure, the location of the hemorrhage anterior to the brainstem, and the typical findings of hemorrhage adjacent to the basilar artery lumen on magnetic resonance imaging and mild basilar artery narrowing on angiography. Although an intramural hematoma of the basilar artery would be easily identified at surgical exploration, such surgeries have never included the extensive base-of-the-skull approaches that are necessary to visualize the artery in the prepontine cistern.

    Title Superficial Temporal Artery to Middle Cerebral Artery Bypass and External Carotid Reconstruction for Carotid Restenosis After Angioplasty and Stent Placement.
    Date November 2000
    Journal Mayo Clinic Proceedings. Mayo Clinic
    Excerpt

    The recent proliferation of endovascular treatment of carotid atherosclerotic disease will increase the number of patients who require treatment for recurrent carotid stenosis after angioplasty and stent placement. The optimal management of these patients has not yet been defined. We describe a 66-year-old woman who required 2 surgical procedures for recurrent in-stent carotid stenosis. She experienced numerous transient ischemic attacks 5 months after left extracranial internal carotid artery angioplasty and stenting for asymptomatic stenosis. Angiography showed high-grade in-stent restenosis, left intracranial carotid artery stenosis, and poor collateral flow to the left middle cerebral artery circulation. The patient underwent a superficial temporal artery to middle cerebral artery bypass, and the transient ischemic attacks resolved. Five months later, angiography showed progressive stenosis of the external carotid artery at the site of the stent. The patient underwent successful external carotid reconstruction with an on-lay patch. Extracranial-intracranial bypass grafting may be used successfully in the treatment of recurrent extracranial carotid artery stenosis after angioplasty and stent placement. Also, external carotid artery reconstruction at the site of an internal carotid artery stent can be performed safely.

    Title Diffuse Vasospasm After Pretruncal Nonaneurysmal Subarachnoid Hemorrhage.
    Date April 2000
    Journal Ajnr. American Journal of Neuroradiology
    Excerpt

    Pretruncal (perimesencephalic) nonaneurysmal hemorrhage is a benign form of subarachnoid hemorrhage (SAH). Angiographic changes of vasospasm are uncommon in patients with this type of hemorrhage, and if vasospasm is present, it is mild and focal. We report two patients with pretruncal nonaneurysmal SAH who developed severe and diffuse vasospasm, expanding the clinical spectrum of this type of SAH. The first patient was a 40-year-old woman who suffered pretruncal nonaneurysmal SAH. Angiography performed on the seventh day post hemorrhage showed diffuse and severe vasospasm affecting both the anterior and the posterior circulation. The patient was treated with hypervolemia, and she remained asymptomatic. Follow-up angiography showed resolution of the vasospasm. The second patient was a 67-year-old woman who suffered pretruncal nonaneurysmal SAH. The results of the initial angiography were normal. Repeat angiography on the ninth day post hemorrhage showed severe vasospasm in the anterior circulation and moderate vasospasm in the posterior circulation. Nine hours later, the patient developed transient dysphasia, and she was treated with hypervolemia. Three days later, a transcranial Doppler examination showed normalization of blood velocities. The presence of diffuse and severe vasospasm does not exclude a diagnosis of pretruncal nonaneurysmal SAH.

    Title Intracranial Hypotension Without Meningeal Enhancement on Magnetic Resonance Imaging. Case Report.
    Date March 2000
    Journal Journal of Neurosurgery
    Excerpt

    Meningeal enhancement on magnetic resonance (MR) imaging is considered the hallmark radiological feature of intracranial hypotension. The authors report on a patient who exhibited progressively symptomatic intracranial hypotension due to a lumbar cerebrospinal fluid (CSF) leak, but in whom MR imaging demonstrated no pachymeningeal enhancement. This 24-year-old man presented with a 6-week history of progressive orthostatic headaches that were associated with photo- and phonophobia. Four weeks before the onset of the headaches, the patient had undergone a lumbar laminectomy. Brain MR images revealed subdural fluid collections and brain sagging; however, meningeal enhancement was not present. Myelography demonstrated a CSF leak at the site of the laminectomy. At surgery, a large dural tear was repaired. The patient recovered well from the surgery, with complete resolution of his headaches. The absence of meningeal enhancement on MR imaging does not exclude a diagnosis of symptomatic intracranial hypotension.

    Title Chronic Subdural Hematoma in Autosomal Dominant Polycystic Kidney Disease.
    Date January 2000
    Journal American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation
    Excerpt

    Autosomal dominant polycystic kidney disease (ADPKD) has been associated with an increased incidence of aneurysmal subarachnoid hemorrhage and intracerebral hematomas. We describe five patients with chronic subdural hematomas, a previously unrecognized complication of ADPKD. In four of the five cases, no trigger was apparent. Clinical presentation was subtle, with mild hemiparesis, headache, or both in four patients and transient neurological deficits mimicking transient ischemic attacks in one patient. In three of the five patients, a retrocerebellar arachnoid cyst was found, suggesting a plausible causal relation between the intracranial arachnoid cysts and the subdural hematomas. In one patient, subdural hematoma was in close proximity to the frontally located arachnoid cyst.

    Title Marfan Syndrome and Intracranial Aneurysms.
    Date December 1999
    Journal Stroke; a Journal of Cerebral Circulation
    Title Cerebral Hemorrhage in Recipients of Renal Transplantation.
    Date November 1999
    Journal Mayo Clinic Proceedings. Mayo Clinic
    Excerpt

    OBJECTIVE: The causes and circumstances of intracerebral hematoma after kidney transplantation have been poorly studied. No data are available on its impact on outcome after a successful renal grafting. PATIENTS AND METHODS: We used the Mayo Clinic medical diagnostic index to identify the patients with a diagnosis of intracerebral hematoma among the 1573 patients who received a renal transplant at the Mayo Clinic between 1966 and 1998. RESULTS: Ten intracranial hematomas occurred in 9 patients and were the cause of death in 6 (1%) of the 530 patients known to have died. The interval from renal transplantation to intracranial hematoma ranged from 12 to 114 months (average, 57 months). All patients with intracranial hemorrhage had poorly controlled hypertension. Intracranial hematoma was more frequently and significantly associated with autosomal dominant polycystic kidney disease (4/146 [2.7%]; P<.01) and with diabetes mellitus (3/410 [0.7%]; P<.01) than with other underlying causes of renal failure (2/1017 [0.2%]). CONCLUSION: In this preliminary study, the risk of cerebral hemorrhage may have increased 10-fold in patients with autosomal dominant polycystic kidney disease and 4-fold in patients with diabetes mellitus, when compared with the population of patients having other causes of renal failure. Most cerebral hemorrhages were catastrophic and fatal but appeared to be responsible for only 1% of the deaths after renal transplantation.

    Title Intraoperative Spinal Angiography.
    Date July 1999
    Journal Journal of Neurosurgery
    Excerpt

    OBJECT: The use of intraoperative angiography of the spine has become available to neurosurgeons as an adjunct in the management of complex spinal vascular malformations. These vascular malformations are rare, and the use of intraoperative angiography of the spine has not been well described. The authors report their recent experience with the use of this diagnostic modality. METHODS: Between 1995 and 1997, nine consecutive patients with type II or Type IV spinal arteriovenous malformations (AVMs) underwent intraoperative spinal angiography. The cervical spine was involved in three patients, the thoracic spine in five, and the thoracolumbar junction in one. In three patients, intraoperative spinal angiography revealed an unexpected finding (residual filling of the AVM). The results obtained using postoperative spinal angiography in all patients showed complete agreement with the intraoperative studies. No complications arose from obtaining the intraoperative spinal angiograms. CONCLUSIONS: Intraoperative spinal angiography is technically feasible, can be performed safely, and has adequate resolution. It detects unexpected residual AVM in one-third of cases.

    Title Early Anticoagulation in Patients with Prosthetic Heart Valves and Intracerebral Hematoma.
    Date April 1999
    Journal Neurology
    Title Brain Protection for Cerebral Aneurysm Surgery.
    Date February 1999
    Journal Neurosurgery Clinics of North America
    Excerpt

    The modern management of patients undergoing surgery for intracranial aneurysm often includes temporary periods of cerebral ischemia to minimize the risks final dissection and clipping or to allow vascular reconstruction. This article reviews the concepts of ischemic thresholds and examines the effects of the physiologic variables that may affect outcome from an ischemic episode. The protective agents presently available and the agents in development that may limit the extent of ischemic injury are reviewed extensively as well.

    Title Rapid Development of Giant Fusiform Cerebral Aneurysms in Angiographically Normal Vessels.
    Date February 1999
    Journal Neurology
    Title Long-term Graft Patency Rates and Clinical Outcomes After Revascularization for Symptomatic Traumatic Internal Carotid Artery Dissection.
    Date January 1999
    Journal Neurosurgery
    Excerpt

    INTRODUCTION: Surgical management of traumatic internal carotid artery (ICA) dissection remains controversial. Therefore, the delayed outcomes and graft patency rates of patients who underwent bypass procedures for symptomatic traumatic ICA dissection were studied. METHODS: Between September 1989 and August 1996, 13 patients (9 male and 4 female patients; mean age, 30.6 yr) underwent 16 revascularization procedures for symptomatic traumatic ICA dissection. The duration of clinical follow-up averaged 47.3 months (range, 12-94 mo) from the date of diagnosis. The duration of radiographic follow-up (catheter or magnetic resonance angiography, duplex Doppler ultrasonography) averaged 24 months (range, 12-60 mo). RESULTS: ICA dissection was caused by blunt (n = 11) or penetrating trauma (n = 2). Associated angiographic abnormalities included seven ipsilateral ICA occlusions, six dissecting aneurysms, two carotid-cavernous fistulae, and six contralateral traumatic ICA dissections. Patients requiring early revascularization (n = 6) underwent bypass procedures an average of 19.2 days after their injuries. Medically managed patients who developed ischemia later were revascularized a mean of 7.8 months after injury. The mean Glasgow Coma Scale score at the time of presentation was 10 (range, scores of 6-15), and the mean Glasgow Coma Scale score before revascularization was 14 (range, scores of 9-15). There were 14 saphenous vein ICA bypasses (8 cervical-to-petrous, 3 cervical-to-middle cerebral artery, 3 petrous-to-supraclinoid) and 2 superficial temporal artery-to-middle cerebral artery bypasses. There was one early postoperative graft occlusion, which responded to surgical thrombectomy. One patient with multiple other traumatic injuries died as a result of a pulmonary embolus 12 months after revascularization. All remaining patients had Glasgow Outcome Scale scores of 5, with patent bypass grafts confirmed during follow-up. CONCLUSION: Revascularization for persistently symptomatic traumatic ICA dissection eliminated ischemia and was associated with excellent long-term outcomes and graft patency rates.

    Title Mr Imaging in Pretruncal Nonaneurysmal Subarachnoid Hemorrhage: is It Worthwhile?
    Date January 1999
    Journal Stroke; a Journal of Cerebral Circulation
    Excerpt

    BACKGROUND and PURPOSE: The cause of pretruncal (perimesencephalic) nonaneurysmal subarachnoid hemorrhage is not known. MRI of the brain or spine is often performed to exclude any other vascular abnormalities. Its diagnostic value is not known. METHODS: We used MR imaging of the brain with routine sequences, gadolinium enhancement, and additional thin T1-weighted axial sections following a triple dose of contrast. RESULTS: We performed MR imaging of the brain in 18 patients with a pretruncal nonaneurysmal subarachnoid hemorrhage. The focal nature of the subarachnoid hemorrhage exclusively in front of the brain stem was confirmed in 14 patients studied within 7 days of the ictus. No vascular abnormalities were found in 17 cases, including 14 patients with gadolinium enhancement. An incidental capillary telangiectasia was found in 1 patient. Fluid-attenuated inverse recovery MR additionally documented blood in the sulci due to cerebrospinal fluid recirculation of blood. Five patients underwent MR imaging of the spine, and no arteriovenous malformations were found. CONCLUSIONS: MR imaging did not reveal a source of pretruncal subarachnoid hemorrhage. The cost of MR imaging probably outweighs the benefit in the evaluation of this variant of subarachnoid hemorrhage.

    Title Nonpositional Headache Caused by Spontaneous Intracranial Hypotension.
    Date December 1998
    Journal Neurology
    Title Surgical Treatment of Superficial Siderosis Associated with a Spinal Arteriovenous Malformation. Case Report.
    Date December 1998
    Journal Journal of Neurosurgery
    Excerpt

    In many patients with superficial siderosis of the central nervous system (CNS) no source of bleeding can be established, despite extensive examinations. The authors report a patient with superficial siderosis and a spinal arteriovenous malformation (AVM) that was not visible on magnetic resonance (MR) imaging or myelography but was identified on angiographic studies. This 71-year-old man presented with a 2-year history of progressive gait difficulties and hearing loss. Examination showed ataxia, hearing loss, and quadriparesis. On MR imaging superficial siderosis of the brain and spinal cord as seen; however, MR imaging of the CNS, as well as cerebral angiography and myelography studies, did not reveal the source of hemorrhage. Spinal angiography revealed a small slow-flow pial AVM at the C-5 level originating from the anterior spinal artery. A C-5 corpectomy was performed and the AVM was obliterated. The patient did well and reported no further progression of his symptoms during 3 months of follow up. Spinal angiography is indicated to complete the evaluation of patients with superficial siderosis, even if results of spinal MR imaging and myelography studies are normal. Obliteration of spinal AVMs may successfully prevent the progression of superficial siderosis.

    Title Screening for Intracranial Aneurysms in Patients with Isolated Polycystic Liver Disease.
    Date December 1998
    Journal Journal of Neurosurgery
    Excerpt

    OBJECT: Isolated polycystic liver disease, that is, polycystic liver disease without kidney cysts, is an entity distinct from polycystic kidney disease. It is not known whether patients with isolated polycystic liver disease are at an increased risk for developing intracranial aneurysms, similar to patients with polycystic kidney disease. The authors screened individuals for intracranial aneurysms in a family in which isolated polycystic liver disease occurred to study the relationship between these two disorders. METHODS: Six siblings requested screening for intracranial aneurysms. Their father had died of a middle cerebral artery aneurysm. Isolated polycystic liver disease was found at autopsy. Their paternal aunt had died of a basilar artery aneurysm, but no autopsy had been performed in that case. Screening with magnetic resonance (MR) angiography and subsequent conventional angiography showed a 5-mm posterior communicating artery aneurysm in one sibling in whom abdominal ultrasound examination yielded normal findings and a posterior communicating artery infundibulum in another sibling in whom an ultrasound examination detected isolated polycystic liver disease. Screening did not detect aneurysms or polycystic liver disease in the other siblings. Thus, of the two patients with isolated polycystic liver disease in this family, one had a ruptured aneurysm and the other had an infundibulum. CONCLUSIONS: Findings in this family suggest an association between isolated polycystic liver disease and intracranial aneurysms. However, because of the delay in onset of the appearance of liver cysts in individuals who carry the disease gene, abdominal ultrasonography is not a useful method to exclude those family members at risk for aneurysm development.

    Title Fibromuscular Dysplasia of the Internal Carotid Artery Associated with Alpha1-antitrypsin Deficiency.
    Date November 1998
    Journal Neurosurgery
    Excerpt

    OBJECTIVE: A deficiency of alpha1-antitrypsin has been implicated in the development of various disorders affecting medium-sized arteries, including intracranial aneurysms, cervicocephalic arterial dissections, and fibromuscular dysplasia (FMD). We performed alpha1-antitrypsin phenotyping in three consecutive patients who underwent bypass surgery for FMD of the extracranial internal carotid artery to test the hypothesis that alpha1-antitrypsin deficiency is a genetic risk factor for the development of FMD. METHODS: The study population consisted of three women (aged 37, 49, and 53 years, respectively) who had bilateral internal carotid artery stenosis caused by FMD. The indications for surgery included ocular or cerebral ischemic symptoms in two patients and progressive stenosis in one patient. The diagnosis of FMD was confirmed by histological examination of the resected segment of artery. The alpha1-antitrypsin phenotype was determined by isoelectric focusing in polyacrylamide gels. RESULTS: Two of the three patients had a heterozygous alpha1-antitrypsin deficiency (PiMZ phenotype). Pathological examination of the resected arterial segment showed typical medial FMD with focal intimal fibroplasia in both patients with the PiMZ phenotype. CONCLUSION: These findings suggest that a heterozygous alpha1-antitrypsin deficiency may be a genetic risk factor for the development of FMD of the internal carotid artery.

    Title Genetics and Aneurysm Formation.
    Date October 1998
    Journal Neurosurgery Clinics of North America
    Excerpt

    This article reviews the current understanding of genetic factors involved in the development of intracranial aneurysms. Of the large number of heritable connective tissue disorders that have been associated with intracranial aneurysms, three are discussed in detail: autosomal dominant polycystic kidney disease, Ehlers-Danlos syndrome type IV, and alpha 1-antitrypsin deficiency. The author reviews the familial occurrence of intracranial aneurysms, which is found in about one-fifth of patients with aneurysm subarachnoid hemorrhage. Guidelines are provided for the evaluation of patients with familial intracranial aneurysms and screening of their relatives.

    Title Rupture of a Previously Documented Small Asymptomatic Intracranial Aneurysm in a Patient with Autosomal Dominant Polycystic Kidney Disease. Case Report.
    Date September 1998
    Journal Journal of Neurosurgery
    Excerpt

    Intracranial aneurysms are common extrarenal manifestations of autosomal dominant polycystic kidney disease (ADPKD). Although their natural history is not completely understood, small asymptomatic intracranial aneurysms in patients with ADPKD often are not treated but are followed with serial magnetic resonance (MR) angiography. The authors report the unique case of a patient with ADPKD who bled from a previously documented asymptomatic 3-mm intracranial aneurysm. This 42-year-old man with ADPKD suffered a subarachnoid hemorrhage (SAH) from a 7-mm left pericallosal artery aneurysm. This aneurysm was clipped and the patient made an excellent recovery. An irregular asymptomatic 3-mm right middle cerebral artery (MCA) aneurysm had also been demonstrated on angiography. While the patient was considering elective surgery for the MCA aneurysm, he suffered a hemorrhage from this lesion 10 weeks after the initial SAH. The aneurysm was clipped and the patient made a satisfactory recovery (he was moderately disabled). In this report the authors indicate that small asymptomatic intracranial aneurysms are not always innocuous in patients with ADPKD, and they suggest that treatment should be strongly considered for these lesions in this group of patients when there is a history of SAH or the aneurysm is irregular in appearance. Because MR angiography studies may not adequately define the configuration of small aneurysms and irregularity may easily be missed, conventional angiography is recommended for patients with ADPKD who are found to have an intracranial aneurysm on screening with MR angiography.

    Title Cervical Bone Spur Presenting with Spontaneous Intracranial Hypotension. Case Report.
    Date September 1998
    Journal Journal of Neurosurgery
    Excerpt

    Spontaneous intracranial hypotension due to a spinal cerebrospinal fluid (CSF) leak is a rare but increasingly recognized cause of postural headaches. The exact cause of these CSF leaks often remains unknown. The authors treated a 32-year-old man with a unique cause of spontaneous intracranial hypotension. He suffered an excruciating headache that was exacerbated by his being in an upright position. The results of four-vessel cerebral angiography were negative; however, magnetic resonance (MR) imaging of the brain revealed pachymeningeal enhancement and hindbrain herniation. A presumptive diagnosis of spontaneous intracranial hypotension was made. Myelography revealed extrathecal contrast material ventral to the cervical spinal cord as well as an unusual midline bone spur at C5-6. The patient's symptoms did not resolve with the application of epidural blood patches, and he subsequently underwent an anterior approach to the C5-6 spur. After discectomy, a slender bone spur that had pierced the thecal sac was found. After its removal, the dural rent was closed using two interrupted prolene sutures. The patient was discharged home 2 days later. On follow up his symptoms had resolved, and on MR imaging the pachymeningeal enhancement had resolved and the cerebellar herniation had improved slightly.

    Title Pretruncal Nonaneurysmal Subarachnoid Hemorrhage.
    Date August 1998
    Journal Mayo Clinic Proceedings. Mayo Clinic
    Excerpt

    OBJECTIVE: To review the diagnostic evaluation and the clinical course of patients with pretruncal nonaneurysmal subarachnoid hemorrhage. MATERIAL AND METHODS: The study population consisted of a consecutive series of patients with a pretruncal nonaneurysmal subarachnoid hemorrhage encountered at Mayo Clinic Rochester during a 6-year interval. We reviewed the clinical manifestations, the neuroimaging characteristics, and the appropriate management. RESULTS: The 15 male and 9 female patients with a pretruncal nonaneurysmal subarachnoid hemorrhage ranged from 3 to 72 years of age (median, 45). Of the 24 patients, 18 had a sudden explosive headache at the time of initial assessment. Ventricular shunting for acute hydrocephalus was indicated in one patient. Neuroimaging studies demonstrated that the center of the hemorrhage was prepontine, and it extended into the interpeduncular or premedullary cisterns. In two patients, a small focal hemorrhage was not noted on an admission computed tomographic scan but was identified on repeated study. A second four-vessel cerebral angiogram, obtained in most patients, showed normal findings in four patients who had had cerebral vasospasm on the first study. In one patient, moderate cerebral vasospasm was found on the second angiogram. No patient had rebleeding. One patient had transient dysphasia associated with cerebral vasospasm after cerebral angiography. Two patients had a family history of aneurysmal subarachnoid hemorrhage. CONCLUSION: The excellent outcome in patients with pretruncal nonaneurysmal subarachnoid hemorrhage is in distinct contrast to the overall somber outcome associated with aneurysmal subarachnoid hemorrhage. A ruptured aneurysm of the posterior circulation may mimic a pretruncal nonaneurysmal subarachnoid hemorrhage and should be excluded on the basis of a technically satisfactory cerebral angiogram.

    Title Alpha-1-antitrypsin Deficiency in Spontaneous Intracranial Arterial Dissections.
    Date August 1998
    Journal Cerebrovascular Diseases (basel, Switzerland)
    Excerpt

    alpha 1-Antitrypsin deficiency has been associated with a variety of vascular disorders including arterial aneurysms, spontaneous extracranial arterial dissections, and arterial fibromuscular dysplasia. We determined the distribution of alpha 1-antitrypsin phenotypes in patients with intracranial arterial dissections, a rare cause of subarachnoid hemorrhage. The study population consisted of 4 consecutive patients with subarachnoid hemorrhage due to spontaneous intracranial arterial dissections. The vertebral artery was involved in 3 patients and the posterior inferior cerebellar artery in 1 patient. Three of these 4 patients were found to have a heterozygous alpha 1-antitrypsin deficiency (PiMZ or PiMS phenotypes). These data support previous studies suggesting that patients with alpha 1-antitrypsin deficiency may be at an increased risk of developing spontaneous arterial dissections.

    Title Seasonal Pattern of Spontaneous Cervical Artery Dissection.
    Date July 1998
    Journal Journal of Neurosurgery
    Excerpt

    OBJECT: The etiology of spontaneous cervical artery dissection is poorly understood; however, it may involve genetic and environmental factors. The purpose of this study was to determine whether seasonality of spontaneous cervical artery dissection exists. METHODS: The seasonal pattern of spontaneous cervical artery dissection was analyzed in a group of 200 consecutive patients (104 females and 96 males with a mean age of 44.9 years) who were evaluated using the Rayleigh test during the period from 1970 to 1990. The majority of patients resided in the midwestern section of the United States, where large seasonal fluctuations in climate occur. A circannual periodicity was found in the frequency of spontaneous cervical artery dissections with a peak occurring in October (p < 0.02). The seasonal variation was substantial, with approximately 58% more patients suffering a cervical artery dissection during autumn than during other seasons. CONCLUSIONS: A seasonal pattern of spontaneous cervical artery dissection exists with a peak occurring in October. The cause of the seasonality remains to be explained; however, weather- or infectious disease-related factors may provide etiological leads.

    Title The Dilemma of Discontinuation of Anticoagulation Therapy for Patients with Intracranial Hemorrhage and Mechanical Heart Valves.
    Date June 1998
    Journal Neurosurgery
    Excerpt

    BACKGROUND: Anticoagulant-related hemorrhage occurs with an incidence of approximately 1%/patient-year in mechanical heart valve recipients. Intracranial hemorrhage poses a difficult clinical choice; continuing anticoagulation therapy may enlarge the volume of the hemorrhage, early reinstitution of anticoagulation therapy may predispose patients to recurrence, and reversal of anticoagulation therapy may place patients at risk for systemic embolization involving the brain. The risk of embolization may also be greater for patients with atrial fibrillation, cage-ball valves in the mitral position, and reduced ventricular function. This dilemma exists because of a lack of data for a large series of patients. METHODS: We reviewed the medical records and neuroimaging studies for a consecutive group of patients admitted with intracranial hemorrhage and mechanical heart valves. We reviewed neurological presenting data, cardiac risk factors for systemic embolization (atrial fibrillation, enlarged atrial chambers, reduced ventricular function, and the type and location of the metallic valve), and hospital management. RESULTS: We studied 39 patients with intracranial hemorrhage and mechanical heart valves (median age, 69 yr). Four patients had experienced previous transient ischemic attacks or minor strokes. The time from valve replacement to intracranial hemorrhage ranged from 2 months to 19 years (median, 6 yr). The type of intracranial hemorrhage was acute subdural hematoma (n = 20), lobar hematoma (n = 10), subarachnoid hemorrhage (n = 4), cerebellar hematoma (n = 3), or basal ganglionic hematoma (n = 2). Thirteen patients died within 2 days of admission. All 26 surviving patients received fresh frozen plasma and vitamin K. Fifteen patients underwent evacuation of acute subdural hematoma, and in one patient an anterior communicating aneurysm was clipped. The duration of discontinuation of anticoagulation therapy varied from 2 days to 3 months (median, 8 d). None of the patients developed transient ischemic attacks, ischemic strokes, valve thrombosis, or systemic embolization. No recurrence of intracranial hemorrhaging was observed during hospitalization and reinstitution of anticoagulation or antiplatelet agent administration. CONCLUSION: Temporary interruption of anticoagulation therapy seems safe for patients with intracranial hemorrhage and mechanical heart valves but without previous evidence of systemic embolization. For most patients, discontinuation for 1 to 2 weeks should be sufficient to observe the evolution of a parenchymal hematoma, to clip or coil a ruptured aneurysm, or to evacuate an acute subdural hematoma.

    Title Heritable Connective Tissue Disorders in Cervical Artery Dissections: a Prospective Study.
    Date May 1998
    Journal Neurology
    Excerpt

    We prospectively evaluated 15 consecutive patients with spontaneous cervical artery dissections. Three patients (20%) had a heritable connective tissue disorder, each with a unique phenotype. None of these patients met the criteria of any of the named syndromes, and collagen and fibrillin analyses were normal. Heritable connective tissue disorders are common among patients with spontaneous cervical artery dissections, but, despite intensive investigations, the type of disorder usually cannot be identified. The underlying arteriopathy in cervical artery dissections is likely to be heterogeneous.

    Title Traumatic Internal Carotid Artery Dissections Caused by Blunt Softball Injuries.
    Date April 1998
    Journal The American Journal of Emergency Medicine
    Excerpt

    This report describes recently treated patients with carotid artery dissection caused by blunt softball injuries, as well as the results of a study of carotid artery trauma in a community. Data obtained through the medical records linkage system used for epidemiologic studies in Olmsted County, MN were used to identify all cases of traumatic internal carotid artery dissection diagnosed from 1987 through 1994. Four patients with traumatic internal carotid artery dissections were identified during the 8-year period under study. In two patients (50%) the carotid dissection was a result of the direct impact of a softball. A 39-year-old-man, who developed transient cerebral ischemic symptoms, and a 35-year-old woman, who developed a painful Horner's syndrome, were struck by a softball on the anterolateral aspect of the neck. Both patients had a low carotid bifurcation. These data suggest that internal carotid artery dissections may be underrecognized sequelae of direct softball injuries to the anterolateral neck. A low carotid bifurcation may be a risk factor for such injuries.

    Title Surgical Treatment of Spontaneous Spinal Cerebrospinal Fluid Leaks.
    Date February 1998
    Journal Journal of Neurosurgery
    Excerpt

    OBJECT: Spontaneous spinal cerebrospinal fluid (CSF) leaks are an increasingly recognized cause of intracranial hypotension and may require neurosurgical intervention. In the present report the authors review their experience with the surgical management of spontaneous spinal CSF leaks. METHODS: Between 1992 and 1997, 10 patients with spontaneous spinal CSF leaks and intracranial hypotension were treated surgically. The mean age of the seven women and three men was 42.3 years (range 22-61 years). Preoperative imaging showed a single meningeal diverticulum in two patients, a complex of diverticula in one patient, and a focal CSF leak alone in seven patients. Surgical exploration in these seven patients demonstrated meningeal diverticula in one patient; no clear source of CSF leakage could be identified in the remaining six patients. Treatment consisted of ligation of the diverticula or packing of the epidural space with muscle or Gelfoam. Multiple simultaneous spinal CSF leaks were identified in three patients. CONCLUSIONS: All patients experienced complete relief of their headaches postoperatively. There has been no recurrence of symptoms in any of the patients during a mean follow-up period of 19 months (range 3-58 months; 16 person-years of cumulative follow up). Complications consisted of transient intracranial hypertension in one patient and leg numbness in another patient. Although the disease is often self-limiting, surgical treatment has an important role in the management of spontaneous spinal CSF leaks. Surgery is effective in eliminating the headaches and the morbidity is generally low. Surgical exploration for a focal CSF leak, as demonstrated on radiographic studies, usually does not reveal a clear source of the leak. Some patients may have multiple simultaneous CSF leaks.

    Title Pretruncal Subarachnoid Hemorrhage: an Anatomically Correct Description of the Perimesencephalic Subarachnoid Hemorrhage.
    Date January 1998
    Journal Stroke; a Journal of Cerebral Circulation
    Title Familial Intracranial Aneurysms: an Autopsy Study.
    Date January 1998
    Journal Neurosurgery
    Excerpt

    OBJECTIVE: Familial intracranial aneurysms are more common than has been appreciated, but systematic autopsy studies of affected individuals have not been reported. We reviewed the autopsy findings of a group of patients with familial aneurysms to elucidate the nature of the putative underlying arteriopathy. METHODS: Using a computerized diagnostic index, we identified all patients with intracranial aneurysms in whom postmortem examination had been performed at the Mayo Clinic between January 1, 1992, and December 31, 1994. The medical records, radiographic studies, and autopsy findings of these patients were reviewed. RESULTS: Among the 28 patients with intracranial aneurysms, 3 (11%) had one or more first-degree relatives with documented intracranial aneurysms. The mean age of the three patients (two women and one man) was 54 years. Microscopic examination of the vascular system revealed medial changes, consisting of degeneration of elastic fibers and increased ground substance, in the systemic arteries of 2 of the 3 patients with familial aneurysms but in none of the 25 patients with sporadic aneurysms. These nonspecific medial changes involved both common and extracranial internal carotid arteries in one patient and the entire aorta as well as intracranial and common carotid arteries in the other patient. CONCLUSION: These observations suggest that an underlying arteriopathy in patients with familial intracranial aneurysms involves the tunica media and commonly may affect systemic (extracranial) arteries.

    Title Aortic Dissection Decades Following Internal Carotid Artery Dissection--report of Two Cases.
    Date December 1997
    Journal Angiology
    Excerpt

    Recurrent dissections involving carotid, vertebral, or renal arteries have been described in patients with spontaneous cervical artery dissections, with a maximal interval between dissections of fourteen years. The authors describe 2 patients in whom aortic dissections developed twenty-five and forty years, respectively, following carotid artery dissections. These 2 patients constituted 8% of the total number of patients from Rochester, Minnesota, who were diagnosed with aortic dissection between 1987 and 1992. The first patient, a forty-five-year-old woman, presented in 1948 with right neck pain and headache, associated with several episodes of transient numbness of the right face and numbness and clumsiness of the left upper and lower extremities. Examination showed right miosis. Angiography showed a stenosis of the extracranial right internal carotid artery beginning several centimeters from the bifurcation. She died at age eighty-five from an aortic dissection. The second patient, a thirty-eight-year-old man, noted left orbital and frontotemporal headaches and drooping of the left eyelid in 1962. Examination showed left oculosympathetic palsy. Angiography showed stenosis and an aneurysm in the midportion of the extracranial left internal carotid artery. He died at age sixty-three from an aortic dissection. These cases suggest that following a carotid artery dissection the risk of a recurrent arterial dissection may remain elevated for a prolonged period of time and the recurrent dissection may involve the aorta.

    Title Intracranial Aneurysms in Marfan's Syndrome: an Autopsy Study.
    Date November 1997
    Journal Neurosurgery
    Excerpt

    OBJECTIVE: Marfan's syndrome is a heritable connective tissue disorder that has been associated with intracranial aneurysms. However, the prevalence of intracranial aneurysms in Marfan's syndrome is unknown and pathological studies of affected vessels have not been reported. We therefore examined the neuropathological findings in a group of patients with Marfan's syndrome. METHODS: We identified all patients with Marfan's syndrome in whom postmortem examination had been performed at the Mayo Clinic between 1969 and 1993. RESULTS: Autopsy included examination of the brain in seven patients with Marfan's syndrome (five men and two women with a mean age of 28 yr). Each of two patients had one or more intracranial aneurysms. The first patient, a 32-year-old man who died as a result of aortic dissection, was observed to have an incidental saccular supraclinoid carotid artery aneurysm (7 mm). Microscopic examination of the remainder of the cerebral arteries revealed duplication and fragmentation of the internal elastic lamina. The second patient, a 20-year-old man who died as a result of a subarachnoid hemorrhage, had ruptured saccular supraclinoid carotid artery (3 mm) and anterior cerebral artery (20 mm) aneurysms as well as unruptured fusiform middle cerebral artery (18 mm) and posterior cerebral artery (13 mm) aneurysms. Microscopic examination of the cerebral arteries revealed widespread changes consisting of intimal proliferation, medial degeneration, and fragmentation of the internal elastic lamina. CONCLUSION: These findings confirm an association between Marfan's syndrome and intracranial aneurysms. Microscopic involvement of cerebral arteries in Marfan's syndrome may be variable, even among those with intracranial aneurysms.

    Title Neurological Picture. Coma Nails.
    Date November 1997
    Journal Journal of Neurology, Neurosurgery, and Psychiatry
    Title Intracranial Arterial Dolichoectasia in Autosomal Dominant Polycystic Kidney Disease.
    Date October 1997
    Journal Journal of the American Society of Nephrology : Jasn
    Excerpt

    Autosomal dominant polycystic kidney disease (ADPKD) is a systemic disorder with a variety of cardiovascular manifestations. This study presents a group of patients with ADPKD who had intracranial arterial dolichoectasia. One hundred seventy-eight ADPKD patients were screened with magnetic resonance angiography, 40 ADPKD patients had conventional angiography, and 98 ADPKD patients underwent a brain autopsy. For comparison, 360 patients without ADPKD who had magnetic resonance angiography and conventional angiography or brain autopsy were also studied. The prevalence of asymptomatic intracranial arterial dolichoectasia was 2.2% (4 of 178), 2.5% (1 of 40), and 2.0% (2 of 98) in the three ADPKD groups, respectively. None of the patients without ADPKD had intracranial arterial dolichoectasia. In addition to the seven patients with asymptomatic disease, two ADPKD patients with vertebrobasilar dolichoectasia had posterior circulation ischemic symptoms. The mean age of the nine patients (five men and four women) was 56.6 yr (range, 41 to 67 yr). The posterior circulation was involved in five patients, the anterior circulation was involved in two patients, and both were involved in two patients. Arterial dissection was believed to have caused middle cerebral artery dolichoectasia in one patient, and intracranial arterial dissections were strongly suspected in two other patients. Six of the nine patients with intracranial arterial dolichoectasia had additional vascular manifestations of ADPKD. Some patients with ADPKD are at an increased risk of developing intracranial arterial dolichoectasia and dissections. Recognizing this association is important because (1) it may be a cause of stroke; (2) it may mimic a saccular aneurysm on radiographic studies; and (3) it suggests that the arteriopathy of ADPKD may be more generalized than previously believed.

    Title Natriuretic Peptide System and Endothelin in Aneurysmal Subarachnoid Hemorrhage.
    Date October 1997
    Journal Journal of Neurosurgery
    Excerpt

    The natriuretic peptide system consists of atrial natriuretic peptide (ANP), brain natriuretic peptide (BNP), and C-type natriuretic peptide (CNP). The system is implicated in the control of body fluid homeostasis, causes natriuresis and diuresis (ANP and BNP), and regulates vascular tone (CNP). A reciprocal relationship between ANP and endothelin (ET) has been suggested, and earlier studies have documented a possible role of ET in cerebral vasospasm after aneurysmal subarachnoid hemorrhage (SAH). The authors studied plasma ANP, BNP, CNP, and ET for 6 consecutive days in 13 patients with SAH by using radioimmunoassay. The median admission values for ANP were 31.5 pg/ml (range 16.8-323 pg/ml [normal 15 +/- 7 pg/ml]); for BNP, 45.3 pg/ml (range 2.2-80.2 pg/ml [normal 12 +/- 9 pg/ml]); for CNP, 7.7 pg/ml (range < 2-20 pg/ml [normal 5.2 +/- 3 pg/ml]); and for ET, 11 pg/ml (range 6.5-25.1 pg/ml [normal 7.2 +/- 4 pg/ml]). Additional increases (defined as > 100% increase on two consecutive measurements) were noted in ANP (11 patients), BNP (10 patients), and CNP (three patients), and resulted in a negative fluid balance in 10 of the 13 patients. The CNP increased in three of four patients with cerebral vasospasm and in one of nine patients without cerebral vasospasm (Fisher's exact test, p = 0.2). No major fluctuations in plasma ET were noted. In seven patients, the plasma ET level did not increase beyond 10 pg/ml during the days of measurement. In six patients, only an occasional sample showed an increase to a maximum of 25 pg/ml. Changes in BNP, ANP, and CNP were independent of each other. The authors conclude that both plasma ANP and BNP increase after SAH and often result in a negative fluid balance. Plasma ANP and BNP seem differentially regulated in the presence of SAH but not by the level of the plasma ET. The possible role of CNP as a regulatory response to cerebral vasospasm needs further exploration.

    Title Perimesencephalic Nonaneurysmal Subarachnoid Hemorrhage: First Hint of a Cause?
    Date September 1997
    Journal Neurology
    Title A Surgeon with a Nasty Taste in His Mouth.
    Date August 1997
    Journal Lancet
    Title Genetics of Intracranial Aneurysms.
    Date June 1997
    Journal Neurosurgery
    Excerpt

    The etiology and pathogenesis of intracranial aneurysms are clearly multifactorial, with genetic factors playing an increasingly recognized role. Intracranial aneurysms have been associated with numerous heritable connective tissue disorders, which account for at least 5% of cases. Of these disorders, the most important are Ehlers-Danlos syndrome Type IV, Marfan's syndrome, neurofibromatosis Type 1, and autosomal dominant polycystic kidney disease; the association with intracranial aneurysms, however, has been firmly established only for polycystic kidney disease. Familial intracranial aneurysms are not rare but account for 7 to 20% of patients with aneurysmal subarachnoid hemorrhage and are generally not associated with any of the known heritable connective tissue disorders. First-degree relatives of patients with aneurysmal subarachnoid hemorrhage are at an approximately fourfold increased risk of suffering ruptured intracranial aneurysms, compared to the general population. Various possible modes of inheritance have been identified in families with intracranial aneurysms, suggesting genetic heterogeneity. Although the benefits have never been quantified, screening for asymptomatic intracranial aneurysms should be considered in families with two or more affected members. The yield of such a screening program may approximate 10%. Although it is unlikely that there is a single gene with major effect, much effort is currently being directed at locating intracranial aneurysm genes.

    Title Spinal Meningeal Diverticula in Autosomal Dominant Polycystic Kidney Disease.
    Date May 1997
    Journal Lancet
    Title Follow-up of Intracranial Aneurysms in Autosomal Dominant Polycystic Kidney Disease by Magnetic Resonance Angiography.
    Date February 1997
    Journal Journal of the American Society of Nephrology : Jasn
    Excerpt

    The purpose of this study was to assess the value of magnetic resonance angiography (MRA) in the follow-up of patients with autosomal dominant polycystic kidney disease (ADPKD) and saccular intracranial aneurysms (ICA), the risk of MRA-defined growth of asymptomatic incidental ICA, and the rate of development of MRA-defined de novo ICA in these patients. Between 1989 and 1995, 15 asymptomatic incidental ICA measuring 1.5 to 6.5 mm in diameter, three symptomatic aneurysms, and one asymptomatic concurrent aneurysm were detected by MRA in this study in 18 patients from 15 families. Four-vessel cerebral angiography in the three patients with symptomatic ICA and autopsy in one patient with an asymptomatic incidental ICA did not reveal additional aneurysms undetected by MRA. Thirty MRA studies were obtained in 10 of the 15 patients with incidental ICA during a cumulative clinical follow-up of 500 months (mean, 33.3; range, 0 to 65 months). The cumulative interval between the initial and the last MRA was 306 months (mean, 30.6; range, 14 to 51 months). No change in aneurysmal size or development of de novo aneurysms was detected. Eight MRA studies were obtained in the three patients with symptomatic ICA during a cumulative clinical follow-up of 130 months (mean, 43.3; range, 23 to 64 months). The cumulative interval between the first and the last MRA was 95 months (mean, 31.7; range, 15 to 49 months). Development of de novo aneurysms was not detected. These results indicate that MRA is an appropriate technique to follow small asymptomatic incidental ICA in patients with ADPKD and that the risk for rapid growth of these aneurysms is low. Although the results of this study should be viewed as preliminary, they do not suggest a higher rate of development of de novo aneurysms or a higher frequency of multiple aneurysms in patients with ADPKD and ICA as compared with patients with sporadic ICA in the general population.

    Title Intracranial Aneurysms and Cervicocephalic Arterial Dissections Associated with Congenital Heart Disease.
    Date February 1997
    Journal Neurosurgery
    Excerpt

    OBJECTIVE: The number of patients with congenital heart disease who survive to adolescence and adulthood continues to increase. We review our experience with noninfectious intracranial aneurysms and cervicocephalic arterial dissections in patients with congenital heart disease, expanding the clinical spectrum of the cerebrovascular abnormalities that may be encountered in this group of patients. METHODS: All patients with congenital heart disease and intracranial aneurysms of cervicocephalic arterial dissections, who were evaluated at the Mayo Clinic between 1969 and 1992, were identified. RESULTS: Congenital heart disease was diagnosed in 3 (8%) of 36 children with intracranial aneurysms, in 5 (0.3%) of 1994 adults with intracranial aneurysms, in 1 (4%) of 25 children with cervicocephalic arterial dissections, and in 5 (2%) of 250 adults with cervicocephalic arterial dissections. The mean age of the 14 patients was 32 years (range, 13-58 yr). The congenital heart disorders consisted of complex cardiac anomalies in three patients (truncus arteriosus, transposition of the great arteries, and tricuspid atresia in one patient each), pulmonic valve or arterial stenosis in two patients, aortic coarctation in four patients, and bicuspid aortic valve in five patients. Only one patient had an intracranial aneurysm and coarctation of the aorta. CONCLUSION: Individuals with a variety of congenital heart disorders may be at an increased risk of intracranial aneurysm development and cervicocephalic arterial dissection, particularly in adolescence. The muscular arteries of the head and neck are derived from neural crest cells and the neural crest is also of major importance in early cardiac development, suggesting that an abnormality of the neural crest may be the common pathogenetic factor explaining this association.

    Title Fibromuscular Dysplasia of the Internal Carotid Artery: a Clinicopathological Study.
    Date January 1997
    Journal Clinical Neuropathology
    Excerpt

    Although angiographic changes of fibromuscular dysplasia (FMD) of the carotid artery are regularly noted, histologic confirmation is rare. We therefore reviewed all patients with a histologic diagnosis of FMD affecting the internal carotid artery who were seen at our institution over a 25-year-period. FMD of the internal carotid artery was a very rare autopsy finding and was detected in only 4 of 20,244 consecutive autopsies (0.02%). In an additional 6 patients FMD of the internal carotid artery was identified in a surgical specimen. All patients were female and their mean age was 53 years. Nine patients had the medial form of FMD and 1 the intimal form. By immunohistochemistry the lesions consist of proliferations of smooth muscle with a minor fibroblastic component. FMD was rarely limited to 1 carotid artery and multivessel involvement was present in 9 patients. In reference to this experience epidemiologic, pathologic, and etiologic features of FMD of the carotid arteries are discussed.

    Title Intracranial Aneurysms.
    Date January 1997
    Journal The New England Journal of Medicine
    Title Progressive Intracranial Aneurysmal Disease in a Child with Progressive Hemifacial Atrophy (parry-romberg Disease): Case Report.
    Date October 1996
    Journal Neurosurgery
    Excerpt

    Intracranial aneurysms are uncommon in children, and their presence often leads to suspicion of a systemic connective tissue disorder. We describe the case of a young male patient with progressive hemifacial atrophy (Parry-Romberg disease) and multiple intracranial aneurysms, a previously undescribed association, and propose that a neural crest defect may be the underlying abnormality in this patient. At age 5 years, the patient was treated for a giant aneurysm of the left cavernous carotid artery with carotid ligation in the neck and a superficial temporal artery-middle cerebral artery bypass. At age 12 years, the patient was similarly treated for a giant aneurysm of the right cavernous carotid artery, which had progressed from a previously noted minute dilatation at age 5 years, with carotid ligation and a superficial temporal artery-middle cerebral artery bypass. At age 21 years, the patient was endovascularly treated for a de novo saccular aneurysm of the left posterior cerebral artery at the P1-P2 junction and a fusiform aneurysm of the distal left posterior cerebral artery. Various studies have suggested that the facial dermis, the subcutaneous tissues, and the skeleton, as well as the tunica media of the cervicocephalic arteries, all arise from neural crest cells, and a disorder of neural crest migration might explain the constellation of findings in this patient.

    Title Giant Intracranial Aneurysm and Fibromuscular Dysplasia in an Adolescent with Alpha 1-antitrypsin Deficiency.
    Date October 1996
    Journal Journal of Neurosurgery
    Excerpt

    Recent studies have suggested that a deficiency of alpha 1-antitrypsin may be a genetic risk factor for the development of intracranial aneurysms and arterial fibromuscular dysplasia. The authors report a 16-year-old girl with a history of lung disease who suffered a cerebral hemorrhage due to the rupture of a giant intracranial aneurysm arising from the middle cerebral artery. This fusiform aneurysm was associated with fibromuscular dysplasia of the intimal type. She was found to have an unusual alpha 1-antitrypsin deficiency (PiMP phenotype). This case provides further evidence of an underlying arteriopathy in alpha 1-antitrypsin deficiency.

    Title Traumatic Occlusion of One Limb of an Intracranial Arterial Fenestration: an Uncommon Cause of Stroke.
    Date September 1996
    Journal Neurology
    Excerpt

    Three weeks after an automobile accident, a 35-year-old man experienced left throat and neck pain, numbness of the left face and tongue, dysphagia, left arm pain and weakness, and left miosis. At age 27, he had suffered an aneurysmal subarachnoid hemorrhage. Angiography at that time had also demonstrated a fenestration of the left intracranial vertebral artery. At the time of the second presentation, angiography showed that one of the limbs of the fenestration had become occluded. Although the vast majority of intracranial arterial fenestrations are asymptomatic, occlusion of one of the limbs of a fenestration may be the cause of stroke.

    Title Roller-coaster Headache Due to Spinal Cerebrospinal Fluid Leak.
    Date July 1996
    Journal Lancet
    Title Alpha-1-antitrypsin Phenotypes Among Patients with Intracranial Aneurysms.
    Date June 1996
    Journal Journal of Neurosurgery
    Excerpt

    A deficiency of alpha 1-antitrypsin has been implicated in the development of arterial aneurysms, including intracranial aneurysms. The authors determined the prevalence of alpha 1-antitrypsin deficiency of different phenotypes in 100 consecutive patients with intracranial aneurysms and compared the distribution of alpha 1-antitrypsin phenotypes to that in the general population (904 people). The study population consisted of 44 men and 56 women with a mean age of 52 years (range 15-81 years). The heterozygous alpha 1-antitrypsin deficiency states (PiMS and PiMZ) were more common in patients (16%) than in the general population (7%), providing an odds ratio of 2.56 (95% confidence interval (CI) 1.32-4.75; p = 0.005). In addition, one patient (1%) was homozygous for the deficient allele (PiZZ) compared to an expected number of 0.015, providing an odds ratio of 67.0 (95% CI 2.0-363.3; p = 0.015). These findings lead the authors to suggest that the heterozygous and homozygous alpha 1-antitrypsin deficiency states are genetic risk factors for the development of intracranial aneurysms.

    Title Cranial Nerve Palsy in Spontaneous Dissection of the Extracranial Internal Carotid Artery.
    Date June 1996
    Journal Neurology
    Excerpt

    Cranial nerve palsy was present in 23 of 190 consecutive adult patients (12%) with spontaneous dissection of the extracranial internal carotid artery. Ten patients (5.2%) had a syndrome of lower cranial nerve palsies (with invariable involvement of cranial nerve XII with or without additional involvement of cranial nerves XI, X, and IX), seven (3.7%) had palsy of cranial nerve V, and five (2.6%) had a syndrome of ocular motor palsies. Palsy of cranial nerve VIII and ischemic optic neuropathy occurred in one patient each. Three patients had dysgeusia without other cranial nerve involvement, presumably due to involvement of the chorda tympani nerve. Headache or face pain (often unilateral) was present in 83% of patients. Other associated manifestations were cerebral ischemic symptoms, bruits, or oculosympathetic palsy. In one patient, cranial nerve palsy was the only manifestation of internal carotid artery dissection, and in another patient, the disease presented only as a palsy of cranial nerve XII and oculosympathetic palsy. In six patients, a syndrome of hemicrania and ipsilateral cranial nerve palsy was the sole manifestation of internal carotid artery dissection. Cranial nerve palsy is not rare in internal carotid artery dissection. Compression or stretching of the nerve by the expanded artery may explain some but not all of the palsies. An alternative mechanism is likely interruption of the nutrient vessels supplying the nerve.

    Title Spontaneous Intracranial Hypotension.
    Date June 1996
    Journal Journal of Neurosurgery
    Title Recurrent Spontaneous Arterial Dissections: Risk in Familial Versus Nonfamilial Disease.
    Date June 1996
    Journal Stroke; a Journal of Cerebral Circulation
    Excerpt

    BACKGROUND AND PURPOSE: Among patients with spontaneous cervical artery dissections, the risk of recurrent arterial dissection is relatively low at 1% per year, but this risk may be higher for patients with a family history of arterial dissections. We compared the risk of a recurrent arterial dissection in patients with familial versus non familial disease. METHODS: Long-term follow-up was established in 200 patients (104 women and 96 men with a mean age of 44.9 years) with spontaneous cervical artery dissections evaluated at a single institution between 1970 and 1990. RESULTS: Among the 200 patients, 10 (5%) were identified who had a family history of spontaneous arterial dissections. In a multivariate analysis, family history was the only significant variable associated with the risk of recurrent dissection (X2=15.51; P=.0001). A recurrent arterial dissection was identified in 5 (50%) of the 10 patients with familial disease compared with 11 (5.8%) of the 190 patients with nonfamilial disease, with an estimated relative risk of 6.3 (95% confidence interval, 2.2 to 18.3; P=.0007). CONCLUSIONS: Among patients with spontaneous cervical artery dissections, a family history of arterial dissection is an important risk factor for the development of a recurrent arterial dissection.

    Title Spontaneous Spinal Cerebrospinal Fluid Leaks and Intracranial Hypotension.
    Date June 1996
    Journal Journal of Neurosurgery
    Excerpt

    Spinal cerebrospinal fluid (CSF) leaks are often implicated as the cause of the syndrome of spontaneous intracranial hypotension, but they have rarely been demonstrated radiographically or surgically. The authors reviewed their experience with documented cases of spinal CSF leaks of spontaneous onset in 11 patients including their surgical observations in four of the patients. The mean age of the six women and five men included in the study was 38 years (range 22-51 years). All patients presented with a postural headache; however, most had additional symptoms, including nausea, emesis, sixth cranial-nerve paresis, or local back pain at the level of the CSF leak. All patients underwent indium-111 radionucleotide cisternography or computerized tomographic (CT) myelography. The location of the spontaneous CSF leak was in the cervical spine in two patients, the cervicothoracic junction in three patients, the thoracic spine in five patients, and the lumbar spine in one patient. The false negative rate for radionucleotide cisternography was high (30%). Subdural fluid collections, meningeal enhancement, and downward displacement of the cerebellum, resembling a Chiari I malformation, were commonly found on cranial imaging studies. In most patients, the symptoms resolved in response to supportive measures or an epidural blood patch. Leaking meningeal diverticula were found to be the cause of the CSF leak in four patients who underwent surgery. In three patients these diverticula could be ligated with good result but in one patient an extensive complex of meningeal diverticula was found to be inoperable. Two patients had an unusual body habitus and joint hypermobility, and two other patients had suffered a spontaneous retinal detachment at a young age. In conclusion, spontaneous spinal CSF leaks are uncommon, but they are increasingly recognized as a cause of spontaneous intracranial hypotension. Most spinal CSF leaks are located at the cervicothoracic junction or in the thoracic spine, and they may be associated with meningeal diverticula. The radiographic study of choice is CT myelography. The disease is usually self-limiting, but in selected cases our experience with surgical ligation of leaking meningeal diverticula has been satisfactory. An underlying connective tissue disorder may be present in some patients with a spontaneous spinal CSF leak.

    Title The Prognosis of Familial Versus Nonfamilial Aneurysmal Subarachnoid Hemorrhage.
    Date March 1996
    Journal Stroke; a Journal of Cerebral Circulation
    Title Screening for Intracranial Aneurysms. Paper is Ambiguous About Number with Proven Aneurysm.
    Date January 1996
    Journal Bmj (clinical Research Ed.)
    Title Intracranial Cysts in Autosomal Dominant Polycystic Kidney Disease.
    Date January 1996
    Journal Journal of Neurosurgery
    Excerpt

    Autosomal dominant polycystic kidney disease (ADPKD) is a systemic disorder well known for its association with intracranial aneurysms. A series of patients with ADPKD who were screened for the presence of an intracranial aneurysm were reviewed and found to include an unexpectedly high number with intracranial arachnoid cysts. Among 247 patients with ADPKD who underwent magnetic resonance imaging (180 cases) or high-resolution contrast-enhanced computerized tomography (67 cases), there were 151 women and 96 men with a mean age of 44 years. Intracranial arachnoid cysts were found in 20 patients (8.1%) with ADPKD compared to two (0.8%) in a control group without ADPKD matched for age, sex, and method of imaging (p < 0.0001). Multiple intracranial arachnoid cysts were found in two patients. Polycystic liver disease was present in 17 (85.0%) of the 20 patients with intracranial arachnoid cysts compared to 119 (52.4%) of the 227 patients without (p < 0.004). Pineal cysts were found in two patients (0.8%) and choroid plexus cysts were found in three patients (1.2%) but this was not different from the control population. None of the intracranial cysts was symptomatic and none was treated surgically. Intracranial arachnoid cysts are a relatively frequent incidental finding in patients with ADPKD, providing further support for the systemic nature of this disease. In the authors' experience with approximately 1500 patients with ADPKD, no complication has been encountered from an intracranial arachnoid cyst, suggesting that asymptomatic intracranial arachnoid cysts in patients with ADPKD require no treatment.

    Title Familial Aorto-cervicocephalic Arterial Dissections and Congenitally Bicuspid Aortic Valve.
    Date November 1995
    Journal Stroke; a Journal of Cerebral Circulation
    Excerpt

    BACKGROUND: A primary arteriopathy is often implicated in the etiology of spontaneous cervicocephalic arterial dissections, but its exact nature usually remains unknown. We describe the familial occurrence of spontaneous arterial dissections and congenitally bicuspid aortic valve (BAV) and propose a common developmental defect in these families. SUMMARY OF REPORT: In the first family, a 63-year-old man suffered an extracranial internal carotid artery (ICA) dissection, and his 43-year-old cousin with BAV suffered an intracranial vertebral artery (VA) dissection. Two other family members had pathologically proven BAV. In the second family, a 31-year-old woman suffered bilateral extracranial ICA and VA dissections. Her father, at age 46, suffered an aortic dissection associated with cystic medial necrosis and BAV. Her paternal uncle died from an aortic dissection at age 59. In the third family, a 39-year-old woman suffered extracranial ICA and VA dissections, and her brother died at age 48 from an aortic dissection associated with a BAV. CONCLUSIONS: The familial occurrence of spontaneous arterial dissections and BAV suggests a common developmental defect. The aortic valvular cusps and the arterial media of the aortic arch and its branches are derived from neural crest cells, suggesting that a neural crest defect may be the underlying abnormality in these families.

    Title Familial Aneurysmal Subarachnoid Hemorrhage: a Community-based Study.
    Date October 1995
    Journal Journal of Neurosurgery
    Excerpt

    The familial occurrence of intracranial aneurysms has been well described. However, intracranial aneurysms are not rare and the great majority of reported families consist of only two affected members. Therefore, the familial aggregation of intracranial aneurysms could be fortuitous. The authors investigated the familial occurrence of aneurysmal subarachnoid hemorrhage (SAH) in their community to determine whether family members of patients with a ruptured aneurysm are at an increased risk of developing an SAH. All 81 patients from Rochester, Minnesota, who suffered an SAH between 1970 and 1989 from a proven aneurysmal rupture were identified, and they or their families were contacted and a family history was obtained. The number of expected SAHs among first-degree relatives was calculated using previously established age- and sex-specific incidence rates in the community of Rochester. Of the 81 index patients, 76 had complete follow up for family history. Fifteen (20%) of these 76 patients had a first- or second-degree relative with aneurysmal SAH. The number of observed first-degree relatives with aneurysmal SAH was 11, compared to an expected number of 2.66, giving a relative risk of 4.14 (95% confidence interval 2.06-7.40; p < 0.001). In the authors' community, aneurysmal SAH was familial in one of five patients, and this familial aggregation was not fortuitous. The increase in familial risk of aneurysmal SAH is approximately fourfold among first-degree relatives.

    Title Spontaneous Carotid-jugular Fistula and Carotid Dissection in a Patient with Multiple Intracranial Arachnoid Cysts and Hemifacial Atrophy: a Generalized Connective Tissue Disorder? Case Report.
    Date October 1995
    Journal Journal of Neurosurgery
    Excerpt

    The authors report a case of a 45-year-old woman with pulsatile tinnitus who was found to have an unusual spontaneous fistula between the petrous internal carotid artery and internal jugular vein. The fistula resolved spontaneously, possibly related to daily manual compression of the ipsilateral common carotid artery. The patient also had a contralateral carotid artery dissection, multiple intracranial arachnoid cysts, and hemifacial atrophy. Her medical history was significant for easy bruisibility, abnormal scarring, and mitral valve prolapse. This association appears unique and may represent a previously underscribed generalized connective tissue disorder.

    Title Headache and Neck Pain in Spontaneous Internal Carotid and Vertebral Artery Dissections.
    Date September 1995
    Journal Neurology
    Excerpt

    We studied the characteristics of headaches in 161 consecutive symptomatic patients with spontaneous dissections of the internal carotid artery (n = 135) or the vertebral artery (n = 26). For patients with internal carotid artery dissection (ICAD), the mean age was 47 years and for those with vertebral artery dissection (VAD), 40.7 years. A history of migraine was present in 18% of the ICAD group and in 23% of the VAD group. Headache was reported by 68% of the patients with ICAD and by 69% of those with VAD, and, when present, it was the initial manifestation in 47% of those with ICAD and in 33% of those with VAD. Ten percent of patients with ICAD had eye, facial, or ear pain without headache. The median interval from onset of headache to development of other neurologic manifestations was 4 days for the ICAD group and 14.5 hours for the VAD group. For all dissections, headaches typically were ipsilateral to the side of dissection. In the ICAD group, headaches were limited to the anterior head in 60% of patients and were steady in 73% and pulsating in 25%. In the VAD group, headaches were distributed posteriorly in 83% of patients and were steady in 56% and pulsating in 44%. Neck pain was present in 26% of patients with ICAD (anterolateral) and in 46% of those with VAD (posterior). The median duration of the headache in patients with VAD and ICAD was 72 hours, but headaches became prolonged, persisting for months to years, in four patients with ICAD.

    Title Sudden Death from Aneurysmal Subarachnoid Hemorrhage.
    Date June 1995
    Journal Neurology
    Excerpt

    Some patients with aneurysmal subarachnoid hemorrhage (SAH) never reach the hospital alive ("sudden death") and, although their numbers are significant, they are not included in most studies of SAH. To clarify the clinical profile of sudden death from aneurysmal SAH, we reviewed the epidemiology and clinicopathologic features of patients with aneurysmal SAH who never reached medical attention. Using the medical record linkage system employed for epidemiologic studies for the population of Rochester, Minnesota, we identified all patients who were diagnosed with aneurysmal SAH between 1960 and 1989. There were 80 women and 33 men with a mean age of 55 years. Of these 113 patients, 13 (12%) died without reaching medical attention. The proportion of those with sudden death remained stable during the study period. In comparing patients with sudden death with those who reached medical attention, the only significant variable was the frequency of posterior circulation aneurysms that was found in 38%, compared with 14% in those who received medical attention (p = 0.042). At autopsy, intraventricular hemorrhage was present in 12 patients (92%) with sudden death, and intracerebral hemorrhage was present in two (15%). Twelve patients (92%) had acute pulmonary edema. In our community, the frequency of sudden death from aneurysmal SAH has not changed during the last three decades in spite of the advances in medical care. The typical clinical profile of sudden death in SAH includes intraventricular hemorrhage, pulmonary edema, and a ruptured posterior circulation aneurysm. Intracerebral hemorrhage is uncommonly associated with sudden death from aneurysmal SAH.

    Title The Poor Prognosis of Ruptured Intracranial Aneurysms of the Posterior Circulation.
    Date May 1995
    Journal Journal of Neurosurgery
    Excerpt

    The first 48 hours after aneurysmal subarachnoid hemorrhage are critical in determining final outcome. However, most patients who die during this initial period are not included in hospital-based studies. We investigated the occurrence of subarachnoid hemorrhage in a population-based study to evaluate possible predictors of poor outcome. All patients diagnosed with aneurysmal subarachnoid hemorrhage between 1955 and 1984 were selected for analysis of mortality in the first 30 days using the medical record-linkage system employed for epidemiological studies in Rochester, Minnesota. One hundred and thirty-six patients were identified. The mean age of these 99 women and 37 men was 55 years. Rates for survival to 48 hours were 32% for the 19 patients with posterior circulation aneurysms, 77% for the 87 patients with anterior circulation aneurysms, and 70% for the 30 patients with a presumed aneurysm (p < 0.0001). Rates for survival to 30 days were 11%, 57%, and 53%, respectively, in these three patient groups (p < 0.0001). Clinical grade on admission to the hospital, the main variable predictive of death within 48 hours, was significantly worse in patients with posterior circulation aneurysms than in others (p < 0.0001). The prognosis of ruptured posterior circulation aneurysms is poor. The high early mortality explains why posterior circulation aneurysms are uncommon in most clinical series of patients with subarachnoid hemorrhage. The management of incidentally discovered intact posterior circulation aneurysms may be influenced by these findings.

    Title Seasons, Snow, and Subarachnoid Hemorrhage: Lack of Association in Rochester, Minnesota.
    Date May 1995
    Journal Journal of Neurosurgery
    Title Brief Report: a Familial Syndrome of Arterial Dissections with Lentiginosis.
    Date March 1995
    Journal The New England Journal of Medicine
    Title Surgical Treatment of Extracranial Internal Carotid Artery Dissecting Aneurysms.
    Date February 1995
    Journal Neurosurgery
    Excerpt

    Aneurysms of the extracranial internal carotid artery (ICA) are uncommon. A significant proportion of such aneurysms are now recognized to be caused by arterial dissection. In some patients, surgical treatment may become necessary. The surgical treatment of 22 patients with spontaneous or traumatic dissecting aneurysms arising from the extracranial ICAs is reviewed. The mean age of the 7 women and 15 men was 39 years. The aneurysm arose from the proximal third of the extracranial ICA in 1 patient, from the middle third in 1 patient, and from the distal third in 20 patients. Five patients underwent cervical carotid ligation; in 13 patients, the aneurysms were resected, and the ICAs were reconstructed, and 4 patients underwent cervical-to-intracranial ICA bypasses. There were 2 postoperative strokes (9%). Facial and lower cranial nerve palsies were commonly seen after high cervical exposure, but these cranial nerve palsies were transient. There were no long-term neurological sequelae during a mean follow-up of 6.2 years. In our relatively limited experience, extracranial ICA dissecting aneurysms can be treated with acceptable morbidity using a variety of techniques. However, the indications for surgical intervention in these aneurysms remain limited.

    Title Coexistence of Fibromuscular Dysplasia and Cystic Medial Necrosis in a Patient with Marfan's Syndrome and Bilateral Carotid Artery Dissections.
    Date December 1994
    Journal Stroke; a Journal of Cerebral Circulation
    Excerpt

    BACKGROUND: A primary arteriopathy is suspected in most patients with spontaneous dissections of the carotid artery, although the nature of this arteriopathy usually remains elusive. Angiographic changes of fibromuscular dysplasia (FMD), however, are found in 10% to 20% of patients with carotid dissections. CASE DESCRIPTION: A 26-year-old woman with Marfan's syndrome presented with bilateral amaurosis fugax after surgical repair of an aortic dissection. Angiography revealed a dissection extending from the ascending aorta into the right internal carotid artery and, separate from the aortic dissection, a dissection of the left internal carotid artery. After surgery, microscopic examination of the right carotid artery revealed a medial dissection but no evidence of an underlying arteriopathy, while the left internal carotid artery displayed the typical features of FMD. Eighteen months later the patient died after resection of a large thoracoabdominal aortic dissecting aneurysm. Microscopic examination of the aorta revealed moderately extensive cystic medial necrosis. CONCLUSIONS: Carotid dissections associated with Marfan's syndrome may be the result of an extension of an aortic dissection or occur isolated from the aorta, even in the same patient. The occurrence of FMD in a patient with Marfan's syndrome in conjunction with previous reports of FMD in a variety of connective tissue disorders suggests that FMD, like cystic medial necrosis, may be a nonspecific disease entity.

    Title Arterial Fibromuscular Dysplasia Associated with Severe Alpha 1-antitrypsin Deficiency.
    Date December 1994
    Journal Mayo Clinic Proceedings. Mayo Clinic
    Excerpt

    OBJECTIVE: To elucidate the putative arteriopathy associated with alpha 1-antitrypsin (alpha 1-AT) deficiency. DESIGN: We retrospectively studied the frequency of occurrence of fibromuscular dysplasia (FMD) in patients with alpha 1-AT deficiency in whom a postmortem examination had been done during a 10-year period at the Mayo Clinic. MATERIAL AND METHODS: The medical records of all patients in whom an autopsy was done at the Mayo Clinic between 1983 and 1992 were reviewed to identify all those with a diagnosis of alpha 1-AT deficiency or FMD. RESULTS: Arterial FMD was found in 2 of 6 patients with alpha 1-AT deficiency (33.3%; 95% confidence interval, 4.3 to 77.7%) in comparison with 23 of 6,690 patients without alpha 1-AT deficiency (0.3%; 95% confidence interval, 0.2 to 0.5%). In both patients with alpha 1-AT deficiency and FMD, the arterial media was thickened and composed of irregular arrays of muscular and connective tissue fibers in a background of mucoid ground substance. CONCLUSION: These findings provide further evidence for an underlying arteriopathy in patients with alpha 1-AT deficiency and suggest that FMD may be a non-specific disorder.

    Title Spontaneous Dissections of Cervicocephalic Arteries in Childhood and Adolescence.
    Date October 1994
    Journal Neurology
    Excerpt

    Among 263 consecutive patients with spontaneous cervicocephalic arterial dissections evaluated at the Mayo Clinic, 18 (6.8%) were 18 years of age or younger (mean age, 12 years). The dissection involved the cervical arteries in 11 patients and the intracranial arteries in seven. Extracranially, the internal carotid artery was involved in eight patients, the vertebral artery in two, and both arteries in one. Intracranially, only the anterior circulation was affected. All the patients had cerebral or retinal ischemic symptoms, usually preceded by headache. Death occurred in a 13-year-old boy with intracranial arterial dissection and coarctation of the aorta. For the 17 remaining patients, the follow-up period ranged from 1 to 21 years. A complete or good clinical recovery occurred in 10 of the 11 patients with cervical arterial dissection but in only four of the seven with dissection of intracranial arteries. Recurrent arterial dissection occurred in two patients with cervical arterial dissections and in one patients with intracranial arterial dissection.

    Title On the Inheritance of Intracranial Aneurysms.
    Date October 1994
    Journal Stroke; a Journal of Cerebral Circulation
    Excerpt

    BACKGROUND AND PURPOSE: The familial occurrence of intracranial aneurysms suggests the presence of a genetically determined underlying arteriopathy. The pattern of inheritance in these families usually is not known. METHODS: A family with seven members with intracranial aneurysms is described and, from the literature before 1994, a total of 238 families with 560 affected members (56% female and 44% male) with intracranial aneurysms not associated with a known heritable disease are reviewed. A segregation analysis was performed on 73 of these families. RESULTS: Two members were affected in the great majority of families (79%); five or more members were reported in only eight families (3%). The most common affected kinship was among siblings. Angiographic screening in 12 families detected an intracranial aneurysm in 29% of 51 asymptomatic relatives. Segregation analysis revealed several patterns of inheritance that were consistent with the compiled pedigrees, but no single mendelian model was the overall best fitting, suggesting that genetic heterogeneity may be important. Twenty-two percent of siblings of male probands had an intracranial aneurysm compared with 9% of siblings of female probands (P = .003). CONCLUSIONS: Genetic heterogeneity may be important in the genetics of intracranial aneurysms. In families with intracranial aneurysms, siblings of an affected male proband may be at a higher risk of developing an aneurysm than siblings of an affected female proband. Screening for intracranial aneurysms in asymptomatic relatives should be considered in families with two or more affected members. In most families, the nature of the underlying arteriopathy remains obscure.

    Title Perimesencephalic Subarachnoid Hemorrhage. Additional Perspectives from Four Cases.
    Date July 1994
    Journal Stroke; a Journal of Cerebral Circulation
    Excerpt

    BACKGROUND: Nonaneurysmal perimesencephalic hemorrhage, a distinct form of subarachnoid hemorrhage, is a recently described variant of intracranial hemorrhage. We describe two patients who presented with unusual features of this type of subarachnoid hemorrhage and also two patients who had a perimesencephalic pattern of hemorrhage due to a ruptured posterior circulation aneurysm. CASE DESCRIPTIONS: The first patient, a 41-year-old woman with perimesencephalic hemorrhage, underwent an exploratory craniotomy because angiography had suggested an anomaly of the basilar tip. No source of hemorrhage could be identified at the time of surgery. The second patient was a 3-year-old boy who presented with opisthotonos and who was found to have a perimesencephalic hemorrhage. Angiography revealed no source for the hemorrhage. The third patient, a 54-year-old man, had a perimesencephalic pattern of subarachnoid hemorrhage from a vertebrobasilar junction aneurysm associated with a fenestration that was missed on the initial angiographic study. The fourth patient, a 43-year-old man, suffered a perimesencephalic pattern of subarachnoid hemorrhage from a small posterior cerebral artery aneurysm, which had not been recognized on two angiograms. CONCLUSIONS: These patients elaborate on the clinical spectrum of subarachnoid hemorrhage with a perimesencephalic pattern. First, a negative exploratory craniotomy suggests that the source of nonaneurysmal perimesencephalic hemorrhage may not be arterial. Second, nonaneurysmal perimesencephalic hemorrhage may also occur in children. Finally, the index of suspicion for a posterior circulation aneurysm should remain high in patients who present with a perimesencephalic pattern of subarachnoid hemorrhage, and these aneurysms may rise from unusual locations.

    Title Neurovascular Manifestations of Heritable Connective Tissue Disorders. A Review.
    Date May 1994
    Journal Stroke; a Journal of Cerebral Circulation
    Excerpt

    BACKGROUND: Heritable disorders of connective tissue are recognized in a small minority of patients with neurovascular diseases. In this report, we review the neurovascular manifestations of four heritable connective tissue disorders: Ehlers-Danlos syndrome, Marfan's syndrome, osteogenesis imperfecta, and pseudoxanthoma elasticum, as well as two other systemic disorders with potential vascular manifestations: neurofibromatosis and polycystic kidney disease. SUMMARY OF REVIEW: Typical neurovascular complications of Ehlers-Danlos syndrome are carotid-cavernous fistulae, intracranial aneurysms, and cervical artery dissections. Arterial dissections and intracranial aneurysms cause the majority of neurovascular symptoms in Marfan's syndrome. Neurovascular disease is uncommon in osteogenesis imperfecta, although carotid-cavernous fistulae and vertebral artery dissections have been reported. Neurovascular disease in pseudoxanthoma elasticum is characterized by intracranial aneurysms and cerebral ischemia caused by premature arterial occlusive disease. Intracranial occlusive arterial disease is the most common neurovascular manifestation of neurofibromatosis, followed by cervical arteriovenous fistulae and aneurysms and intracranial aneurysms. Intracranial aneurysms are the hallmark of polycystic kidney disease. CONCLUSIONS: Recognition of an underlying generalized connective tissue disorder may be of considerable importance, although marked phenotypic heterogeneity often complicates the diagnosis of these disorders. Conversely, the association of certain neurovascular anomalies with generalized connective tissue disorders and recognition of their basic molecular defect may offer clues to the etiology and pathogenesis of these neurovascular diseases in general.

    Title Surgical Treatment of Spontaneous Intracranial Hypotension Associated with a Spinal Arachnoid Diverticulum. Case Report.
    Date May 1994
    Journal Journal of Neurosurgery
    Excerpt

    The authors present the case of a 22-year-old woman who developed spontaneous intracranial hypotension as a result of a cerebrospinal fluid leak from a thoracic extradural arachnoid diverticulum. The patient was successfully treated by ligation of the diverticulum. The entity of spontaneous intracranial hypotension and its relevance to neurosurgery are discussed.

    Title Penetration of the Skull Base by Dissecting Keratocyst.
    Date March 1994
    Journal Journal of Cranio-maxillo-facial Surgery : Official Publication of the European Association for Cranio-maxillo-facial Surgery
    Excerpt

    The extensive destructive potential of the keratocyst has been well recognized but penetration of the keratocyst into the skull base is rare. 3 cases showing such aggressive behavior and rare location were seen and treated; 2 are reported in this paper. Both cases illustrate the importance of early radical treatment once the aggressive nature of keratocyst is recognized.

    Title Alpha 1-antitrypsin Deficiency in Intracranial Aneurysms and Cervical Artery Dissection.
    Date March 1994
    Journal Lancet
    Excerpt

    The pathogenesis of ruptured intracranial aneurysms and cervical artery dissections is poorly understood but may be similar in these two disorders. We report four patients with alpha 1-antitrypsin deficiency who developed a ruptured intracranial aneurysm or spontaneous dissection of the cervical internal carotid artery. Three patients were heterozygous for the deficient allele (PiMZ or PiGZ) and one was homozygous (PiZZ). A deficiency of alpha 1-antitrypsin or one of the other protease inhibitors could result in degradation of the arterial wall through an imbalance between proteolytic enzymes and their inhibitors, thereby predisposing the arterial wall to dissection or aneurysm formation.

    Title Recurrent Spontaneous Cervical-artery Dissection.
    Date February 1994
    Journal The New England Journal of Medicine
    Excerpt

    BACKGROUND. Spontaneous dissection of the internal carotid and vertebral arteries is increasingly recognized as a cause of ischemic stroke in young people. An underlying arteriopathy is often suspected in the pathogenesis of such dissection, but the frequency of recurrent dissection is unknown. METHODS. We describe the long-term follow-up of 200 consecutive patients (104 women and 96 men) with spontaneous cervical-artery dissections evaluated at the Mayo Clinic between 1970 and 1990. All diagnoses were confirmed by angiography. RESULTS. The mean age of the patients was 44.9 years (range, 16 to 76). Internal carotid arteries were affected in 150 patients, vertebral arteries in 37, and both in 13. Multivessel dissections were present in 28 percent of the patients. The mean follow-up was 7.4 years. Recurrent dissection occurred only in arteries not previously involved by dissection. A recurrent arterial dissection developed in 16 patients (8 percent)--within a month after the initial dissection in 4 patients (2 percent) and between 1.4 and 8.6 years later in 12 patients (a rate of 1 percent per year). The cumulative rate of recurrent dissection among patients followed for 10 years was 11.9 percent. Younger patients had a greater risk of recurrent dissection. CONCLUSIONS. Although dissections in multiple cervical vessels are common at presentation, after the first month the risk of recurrent dissection is only about 1 percent per year.

    Title Laparoscopic Placement of Ventriculoperitoneal Shunts: Preliminary Report.
    Date December 1993
    Journal Mayo Clinic Proceedings. Mayo Clinic
    Excerpt

    We used a laparoscopic technique for the percutaneous placement of the peritoneal end of cerebrospinal fluid shunts in adult patients with obstructive or normal-pressure hydrocephalus. Concurrent with the initial cranial part of the procedure, pneumoperitoneum is established in a routine fashion, and a video-laparoscope and grasping forceps are inserted into the peritoneal cavity. With use of a pacemaker introducer kit, the peritoneal catheter is placed percutaneously under direct laparoscopic vision through a small upper abdominal incision into the peritoneal cavity. At the completion of the procedure, the patency of the assembled shunt system can be verified by observing free flow of cerebrospinal fluid from the catheter tip as the valve is being pumped. We found that this technique is particularly useful in technically challenging cases--for example, those involving obese patients and those who have undergone multiple abdominal operations. No complications associated with the technique were encountered.

    Title Internal Carotid Artery Dissection in a Community. Rochester, Minnesota, 1987-1992.
    Date November 1993
    Journal Stroke; a Journal of Cerebral Circulation
    Excerpt

    BACKGROUND AND PURPOSE: Cervical internal carotid artery dissections are diagnosed with an increasing frequency, but reliable epidemiologic data are not available. The aim of this study was to determine the incidence rate of spontaneous cervical internal carotid artery dissection in a defined population. METHODS: Using the medical record linkage system used for epidemiologic studies in Rochester, Minn, all patients diagnosed with spontaneous cervical ICA dissection for 1987 through 1992 were identified. RESULTS: A total of 10 patients with spontaneous cervical internal carotid artery dissection (6 women and 4 men; mean age, 44 years) were identified. For the period 1987 through 1992, the average annual incidence rate for all ages was 2.6 per 100,000 (95% confidence intervals, 0.9 to 4.2). CONCLUSIONS: This study, for the first time, provides incidence rates for spontaneous cervical internal carotid artery dissections. No diagnoses were made before 1987, probably reflecting an increased awareness of the disorder among physicians.

    Title Ocular Motor Nerve Palsies in Spontaneous Dissections of the Cervical Internal Carotid Artery.
    Date November 1993
    Journal Neurology
    Excerpt

    Cranial nerve palsies affecting ocular motor function in patients with spontaneous cervical internal carotid artery dissections are rare. Among 155 patients with spontaneous dissections of the cervical internal carotid artery, four (2.6%) had transient third, fourth, or sixth cranial nerve palsy. The third nerve was involved in two patients, the fourth nerve in one, and the sixth nerve in one. Three patients had ipsilateral headache or facial pain, one had bilateral headaches, and three had oculosympathetic palsy. None had any associated cerebral or retinal ischemic symptoms. Cervical internal carotid artery dissection should be included in the differential diagnosis of palsies of the third, fourth, or sixth cranial nerve, especially when associated with ipsilateral headache or facial pain. Interruption of the nutrient arteries supplying these cranial nerves could explain their involvement by internal carotid artery dissection.

    Title Saccular Intracranial Aneurysms in Autosomal Dominant Polycystic Kidney Disease.
    Date November 1992
    Journal Journal of the American Society of Nephrology : Jasn
    Excerpt

    The literature on the association of intracranial aneurysms in autosomal dominant polycystic kidney disease (ADPKD) consists mainly of case reports and small series of patients. To provide a more-detailed description of this association and its frequency, the records of all ADPKD patients with saccular intracranial aneurysms, all ADPKD autopsy cases including brain examination, and sex- and age-matched autopsy cases without ADPKD seen at the Mayo Clinic between 1950 and 1989 and of all Rochester residents with a diagnosis of subarachnoid hemorrhage or ADPKD between 1945 and 1984 were reviewed. The presentation of the 41 patients (22 men and 19 women; mean age, 46.4 yr) with this association was subarachnoid hemorrhage in 33, transient ischemic attacks in 2, incidental angiographic or autopsy finding in 5, and discovery during angiographic screening in 1. Thirty-one, seven, and three patients harbored one, two, and three aneurysms, respectively, arising from the middle cerebral artery (N = 23), anterior communicating artery (N = 16), internal carotid artery (N = 11), and vertebral or basilar artery (N = 4). A family history of intracranial aneurysm, subarachnoid hemorrhage, or intracranial hemorrhage at an early age was present in 22% of the patients. Small aneurysms (less than 5 mm) were less likely to have ruptured or caused symptoms (P less than 0.04). There was a trend for hypertension to be associated with the severity of the subarachnoid hemorrhage. Aneurysmal rupture occurred before age 50 in 64% of patients. Of the 89 ADPKD autopsy cases with brain examination, 22.5% had intracranial aneurysms.(ABSTRACT TRUNCATED AT 250 WORDS)

    Title Familial Intracranial Aneurysms. A Review.
    Date July 1992
    Journal Stroke; a Journal of Cerebral Circulation
    Excerpt

    BACKGROUND: A familial occurrence of intracranial aneurysms is defined by the presence of such aneurysms in two or more first to third-degree family members. Families with two affected members may represent accidental aggregation. Other families show a frequency compatible with an autosomal dominant mode of inheritance. A genetic basis is also suggested by the younger average age of familial cases with a ruptured intracranial aneurysm (42.3 years versus an age range of 50-54 years for nonfamilial cases), occurrence at the same site or a mirror site in sibling pairs, occurrence in identical twins, and the association of intracranial aneurysms with genetically transmitted disorders. SUMMARY OF REVIEW: No reliable data are available about the occurrence of familial intracranial aneurysms among all patients with ruptured aneurysms; a frequency of 6.7% has been reported from a retrospective study, but a large part of the "familial" occurrence can be explained by fortuitous aggregation. The pathogenesis of familial intracranial aneurysms is not fully explained; a (partial) deficiency of type III collagen has been reported in sporadic, but not in familial, cases. Clinical decision analysis shows how the risk of harboring an intracranial aneurysm and the age of the patient are the main determinants for elective screening; lifetime risk of rupture (and therefore age) and surgical risks are the determinants for neurosurgical treatment. CONCLUSIONS: Surgical treatment is recommended for patients aged less than 70 years with a moderate or low surgical risk, and screening (preferably by intra-arterial digital subtraction angiography) is recommended only for relatives aged 35-65 years. Magnetic resonance angiography may develop into a useful alternative for screening, but the risks of diagnostic procedures play only a minor role in the decision analysis.

    Title Rupture of Previously Documented Small Asymptomatic Saccular Intracranial Aneurysms. Report of Three Cases.
    Date June 1992
    Journal Journal of Neurosurgery
    Excerpt

    In a recent study from the Mayo Clinic on the natural history of intact saccular intracranial aneurysms, none of the aneurysms smaller than 10 mm in diameter ruptured. It was concluded that these aneurysms carry a negligible risk for future hemorrhage and that surgery for their repair could not be recommended. These findings and recommendations have been the subject of much controversy. The authors report three patients with previously documented asymptomatic intact saccular intracranial aneurysms smaller than 5 mm in diameter that subsequently ruptured. In Case 1, a 70-year-old man bled from a 4-mm middle cerebral artery aneurysm that had been discovered incidentally 2 1/2 years previously during evaluation of cerebral ischemic symptoms. A 10-mm internal carotid artery aneurysm and a contralateral 4-mm middle cerebral artery aneurysm had not ruptured. Case 2 was that of a 66-year-old woman who bled from a 4-mm pericallosal aneurysm that had been present 9 1/2 years previously when she suffered subarachnoid hemorrhage (SAH) from a 7 x 9-mm posterior inferior cerebellar artery aneurysm. Although the pericallosal aneurysm had not enlarged in the intervening years, a daughter aneurysm had developed. The third patient was a 45-year-old woman who bled from a 4- to 5-mm posterior inferior cerebellar artery aneurysm that had measured approximately 2 mm on an angiogram obtained 4 years previously; at that time she had suffered SAH due to rupture of a 5 x 12-mm posterior communicating artery aneurysm. These cases show that small asymptomatic intact saccular intracranial aneurysms are not innocuous and that careful consideration must be given to their surgical repair and long-term follow-up study.

    Title Spontaneous Dissection of the Cervical Internal Carotid Artery. Presentation with Lower Cranial Nerve Palsies.
    Date May 1992
    Journal Archives of Otolaryngology--head & Neck Surgery
    Excerpt

    Spontaneous dissections of the internal carotid arteries are uncommon but are not rare. They constitute a fairly common cause of ischemic stroke in young patients (young in terms of the age at which strokes generally occur). The common presenting manifestations are (1) unilateral headaches followed after a period of delay by focal cerebral ischemic symptoms or (2) unilateral headaches and ipsilateral incomplete Horner's syndrome. These may or may not be associated with subjective or objective bruits. In rare instances, spontaneous dissections of the internal carotid arteries may present as lower cranial nerve palsies and cause dysphonia, dysarthria, dysphagia, and numbness of the throat. Affected patients may initially present to the otolaryngologist or be referred to one. This article describes eight patients with spontaneous dissections of the internal carotid arteries and lower cranial nerve palsies, and the pertinent literature is reviewed.

    Title Familial Association of Intracranial Aneurysms and Cervical Artery Dissections.
    Date January 1992
    Journal Stroke; a Journal of Cerebral Circulation
    Excerpt

    BACKGROUND AND PURPOSE: The familial occurrence of intracranial aneurysms and cervical artery dissections has been described in different families and supports the hypothesis that a primary arteriopathy may play a role in the pathogenesis of these disorders. Although the basis for this arteriopathy is generally not believed to be similar among cases of intracranial aneurysms and cervical artery dissections, several similarities exist in the epidemiology of these disorders and a common underlying arterial abnormality may be suspected. SUMMARY OF REPORTS: The medical records of all 175 patients with spontaneous dissections of the cervical arteries who were seen at the Mayo Clinic between 1970 and 1989 were reviewed to identify families in which intracranial aneurysms and cervical dissections coexisted. Three families were identified in which intracranial aneurysms and cervical artery dissections were observed among siblings. These families are described in detail. CONCLUSIONS: The familial occurrence of intracranial aneurysms and cervical artery dissections within the same families provides support to the importance of a common underlying arteriopathy in the pathogenesis of both these disorders. The underlying vascular defect may, at least in some cases, be inherited.

    Title Angiographic Frequency of Saccular Intracranial Aneurysms in Patients with Spontaneous Cervical Artery Dissection.
    Date January 1992
    Journal Journal of Neurosurgery
    Excerpt

    The pathogenesis of intracranial aneurysms and spontaneous cervical artery dissection is incompletely understood but a primary arteriopathy, possibly similar in both disorders, may be of importance. To investigate the frequency of intracranial aneurysms in patients with spontaneous cervical artery dissection, the angiograms of 164 patients who were diagnosed at the Mayo Clinic as having spontaneous extracranial carotid or vertebral artery dissection were reviewed. Thirteen intracranial aneurysms were detected in nine (5.5%) of the 164 patients: eight (8.8%) of the 91 female patients and one (1.4%) of the 73 male patients. The frequency of intracranial aneurysms in these patients was significantly higher (p less than 0.01) than that observed in a recent angiographic study from the same institution, estimating the frequency of intracranial aneurysms in the general population (1.1%). The significance of these findings is discussed.

    Title Cervical Vertebral Artery Aneurysms and Arteriovenous Fistulae in Neurofibromatosis Type 1: Case Reports.
    Date January 1992
    Journal Neurosurgery
    Excerpt

    Neurofibromatosis is of particular interest to neurosurgeons because of the various central and peripheral nervous system tumors and, more rarely, cervicocerebral arterial lesions associated with the disease. In the present paper, we report two patients with neurofibromatosis Type 1 (von Recklinghausen's disease) who had anomalies of the extracranial vertebral arteries. A large extracranial vertebral artery aneurysm was incidentally discovered in the first patient, a 43-year-old woman, after rupture of a subclavian artery aneurysm. The second patient, a 28-year-old woman, had an enlarging neck mass and was found to have an extensive extracranial vertebral artery arteriovenous fistula. The vascular lesions associated with neurofibromatosis Type 1 in general and those arising from the extracranial vertebral artery in particular are reviewed.

    Title Screening for Unruptured Familial Intracranial Aneurysms: Subarachnoid Hemorrhage 2 Years After Angiography Negative for Aneurysms.
    Date November 1991
    Journal Neurosurgery
    Excerpt

    The screening of asymptomatic individuals in families with intracranial aneurysms has been advocated to detect unruptured aneurysms before a major hemorrhage occurs. We report a 39-year-old male member of a large Dutch family, with a documented history of intracranial aneurysms, who suffered a subarachnoid hemorrhage 2 years after cerebral digital subtraction angiography using intravenously administered contrast medium showed no abnormalities. Conventional arteriography demonstrated three intracranial aneurysms measuring 3 x 3 mm. Potential alternative screening procedures are discussed.

    Title Spontaneous Carotid-cavernous Fistulae in Ehlers-danlos Syndrome Type Iv. Case Report.
    Date June 1991
    Journal Journal of Neurosurgery
    Excerpt

    Spontaneous bilateral carotid-cavernous fistulae and cervical artery dissection is reported in a 20-year-old woman with Ehlers-Danlos syndrome Type IV. The clinical features of 16 previously published cases of spontaneous carotid-cavernous fistulae associated with Ehlers-Danlos syndrome Type IV are reviewed, for a total of 17 cases. The mean age of the 14 women and three men was 31.6 years. Only direct fistulae were encountered. Diagnostic neuroangiography carried morbidity and mortality rates of 36% and 12%, respectively; neuroradiological treatment resulted in death in one of six patients. The possible value of desmopressin in the management of these patients is discussed. In view of the risks of arterial puncture and surgery, the authors emphasize the importance of early recognition of Ehlers-Danlos syndrome.

    Title Management of Intraventricular Haemorrhage Secondary to Ruptured Arteriovenous Malformation in a Child with Von Willebrand's Disease.
    Date May 1991
    Journal Journal of Neurology, Neurosurgery, and Psychiatry
    Title [dissection of Cervical Arteries As a Cause of Cerebral Ischemia or Cranial Nerve Dysfunction]
    Date November 1990
    Journal Nederlands Tijdschrift Voor Geneeskunde
    Excerpt

    Nine patients with dissections of the cervical arteries are presented. Dissections cause approximately three per cent of non-haemorrhagic stroke and are usually observed in young and middle-aged patients. Dissections very often give rise to head or neck pain. Carotid artery dissection may lead to lower cranial nerve dysfunction and an incomplete Horner's syndrome in case of subadventitial dissection, and to cerebral ischaemia in case of subintimal spread. Vertebral artery dissection may cause brain stem ischaemia (subintimal dissection) or in rare cases a subarachnoid haemorrhage (subadventitial spread). The history frequently reveals a (trivial) traumatic event. Diagnosis is usually established by angiography or MRI. The prognosis is good and recurrences are rare. Treatment with anticoagulants or acetylsalicylic acid seems recommendable, though scientifically unproven.

    Title Cerebrovascular Disease in Ehlers-danlos Syndrome Type Iv.
    Date May 1990
    Journal Stroke; a Journal of Cerebral Circulation
    Excerpt

    We describe two patients with cerebrovascular complications of Ehlers-Danlos syndrome type IV. A 16-year-old girl with spontaneous internal carotid artery dissection and a 46-year-old woman with aneurysmal subarachnoid hemorrhage and multiple aortic dissections were both deficient in collagen type III, analyzed in cultured skin fibroblasts. To our knowledge, spontaneous carotid artery dissection associated with collagen type III deficiency has not been reported previously. Early clinical recognition of this syndrome is of great importance in view of the hazards of angiography and surgery. Collagen type III deficiency plays a role in the pathogenesis of intracranial saccular aneurysms and may also be involved in the pathogenesis of carotid cavernous fistulas and dissections of the cervical arteries.

    Title Familial Cerebral Aneurysms and Type Iii Collagen Deficiency.
    Date February 1990
    Journal Journal of Neurosurgery
    Title Circumstances Surrounding Aneurysmal Subarachnoid Hemorrhage.
    Date October 1989
    Journal Surgical Neurology
    Excerpt

    The circumstances surrounding aneurysmal subarachnoid hemorrhage were investigated in a group of 500 consecutive patients admitted to a neurosurgical center. Subarachnoid hemorrhage occurred during stressful events in 42.8% of the patients, during nonstrenuous activities in 34.4%, and during rest or sleep in 11.8%. The activities or events preceding subarachnoid hemorrhage were not known in the remaining 11.0%. Men were more likely to have suffered their hemorrhage during stressful events than women (54.1% versus 36.6%; p less than 0.00025). Only 30.1% of aneurysms arising from the internal carotid artery ruptured during stressful events compared with 48.1% of aneurysms at other locations (p less than 0.005). Physiological responses to the various activities and events are discussed as they may relate to mechanisms underlying aneurysmal subarachnoid hemorrhage. Two important factors in the precipitation of aneurysmal rupture are increased arterial blood pressure and decreased cerebrospinal fluid pressure around the aneurysm. These factors result in a high transmural pressure and concomitant wall stress. Physical exertion, activities involving the Valsalva maneuver, and emotional strain are associated with an increase in blood pressure and frequently preceded subarachnoid hemorrhage. Fluctuations in cerebrospinal fluid pressure around the aneurysm mainly occur during Valsalva's maneuver and postural changes.

    Title Angiographic Abnormalities Mimicking Fibromuscular Dysplasia in a Patient with Ehlers-danlos Syndrome, Type Iv.
    Date October 1989
    Journal Neurosurgery
    Title Does the Artery of Adamkiewicz Exist in the Albino Rat?
    Date September 1989
    Journal Journal of Anatomy
    Excerpt

    The arterial supply of the spinal cord of the rat in the thoracolumbar region was studied in 26 Wistar albino rats. Contrary to the situation in man, and in variance with earlier reports for the rat, we did not observe a single large artery at the thoracolumbar level supplying a large part of the spinal cord. The anterior spinal artery at this level seemed to be adequately supplied by three to five principal arteries. A considerable variation in the distribution and size of these feeding arteries was observed. It is concluded that the equivalent of the artery of Adamkiewicz is not invariably present in the rat and it would appear that the vascular anatomy of the rat spinal cord is not as similar to that of man as has previously been reported.

    Title Intracranial Aneurysm and Hla-dr2.
    Date September 1988
    Journal Journal of Neurology, Neurosurgery, and Psychiatry
    Title Referral Pattern of Patients with Aneurysmal Subarachnoid Hemorrhage.
    Date May 1988
    Journal Surgical Neurology
    Excerpt

    The referral pattern of 334 patients admitted to a neurosurgical clinic with aneurysmal subarachnoid hemorrhage (SAH) was analyzed. Forty-nine percent of the patients were admitted after the day following the SAH. Failure of patients to seek prompt medical care was a cause of delay in 29 patients and of physician diagnostic errors in 95 patients. Common misdiagnoses included migraine, mental exhaustion, sinusitis, and influenza. A delay at the referring hospital was observed in 97 patients. Early intervention is important for the optimal management of patients with SAH. Educating the public, medical students, and physicians about the signs and symptoms of SAH and the importance of prompt therapy is likely to improve overall outcome after aneurysmal rupture.

    Title Effect of Low Molecular Weight Dextran on Plasma Viscosity.
    Date February 1988
    Journal Surgical Neurology
    Title Relationship Between Blood Viscosity and Cerebral Ischemia After Surgical Treatment of Ruptured Intracranial Aneurysms.
    Date June 1987
    Journal Surgical Neurology
    Excerpt

    To determine the role of blood viscosity after surgical treatment of ruptured intracranial aneurysms, the relationship between blood viscosity and clinical condition was examined in 17 patients. A total of 213 blood samples were analyzed. An inverse correlation was found between blood viscosity and level of consciousness; in addition, blood viscosity was higher when focal neurologic deficit was observed. Hematocrit was similarly related to clinical condition, although the correlations observed were less strong. Postoperative plasma viscosity was higher in patients with focal neurologic deficit. Regular blood viscosity measurements are of value in patients at risk for developing cerebral ischemia.

    Title Cerebrovascular Involvement in Ehlers-danlos Syndrome.
    Date
    Journal Current Treatment Options in Cardiovascular Medicine
    Excerpt

    Ehlers-Danlos syndrome type IV is one of the most prominent heritable disorders of connective tissues associated with neurovascular disease. It is an uncommon disorder characterized by thin translucent skin, distinctive facial features, excessive bruising, and rupture of blood vessels or viscera. The typical neurovascular complications of this syndrome are carotid cavernous fistulas, intracranial aneurysms, and cervical artery dissections. Because of the inordinate fragility of the blood vessels in patients with this syndrome, conservative treatment is always indicated. However, in select cases in which the person or family history indicates a more benign form of the disease, treatment that includes surgical or endovascular treatment of asymptomatic lesions may be indicated.

    Title Diagnostic Criteria for Headache Due to Spontaneous Intracranial Hypotension: a Perspective.
    Date
    Journal Headache
    Excerpt

    The clinical and radiographic manifestations of spontaneous intracranial hypotension are highly variable and many patients do not satisfy the 2004 International Classification of Headache Disorders criteria. We developed new diagnostic criteria for spontaneous intracranial hypotension based on cases we have seen reflecting the variable manifestations of the disorder. These criteria provide a basis for change when the classification criteria are next revised. The diagnostic criteria consist of A, orthostatic headache; B, the presence of at least one of the following: low opening pressure (≤ 60 mm H(2) O), sustained improvement of symptoms after epidural blood patching, demonstration of an active spinal cerebrospinal fluid leak, cranial magnetic resonance imaging changes of intracranial hypotension (eg, brain sagging or pachymeningeal enhancement); C, no recent history of dural puncture; and D, not attributable to another disorder.

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