Neurologist (brain, nervous system)
19 years of experience
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Accepting new patients
Central Oklahoma City
1200 N Phillips Ave
Suite 7900
Oklahoma City, OK 73104
405-271-2006
Locations and availability (2)

Education ?

Medical School
Monash University (1991)
Foreign school

Awards & Distinctions ?

Associations
American Board of Psychiatry and Neurology

Affiliations ?

Dr. Ng is affiliated with 2 hospitals.

Hospital Affilations

Score

Rankings

  • St Joseph's Hospital
    350 W Thomas Rd, Phoenix, AZ 85013
    • Currently 4 of 4 crosses
    Top 25%
  • St Josephs Hospital and Med Center
  • Publications & Research

    Dr. Ng has contributed to 81 publications.
    Title Eeg and Video-eeg Seizure Monitoring Has Limited Utility in Patients with Hypothalamic Hamartoma and Epilepsy.
    Date August 2011
    Journal Epilepsia
    Excerpt

    Hypothalamic hamartomas (HHs) are a malformation of the ventral hypothalamus and tuber cinereum, associated with gelastic seizures and epilepsy. We sought to determine the spectrum of electroencephalography (EEG) abnormalities in a large cohort of HH patients.

    Title Long-term Outcome After Emergency Resection of Hypothalamic Hamartomas for Status Gelasticus.
    Date April 2011
    Journal Neurosurgical Focus
    Excerpt

    Gelastic seizures are epileptic events characterized by bouts of laughter. They are rare and mostly associated with hypothalamic hamartomas (HHs). Status gelasticus, a rare form of status epilepticus, is defined as a prolonged cluster of gelastic seizures (> 20-30 minutes) without necessarily involving loss of awareness between seizures. Emergency resection of the hamartoma is highly effective in these situations and should be considered as early as possible. The authors retrospectively reviewed their surgical cases to document the success, complications, and long-term follow-up after emergency resection of HHs for status gelasticus.

    Title Syndrome of Alternating Hypernatremia and Hyponatremia After Hypothalamic Hamartoma Surgery.
    Date April 2011
    Journal Neurosurgical Focus
    Excerpt

    In this paper, the authors' goal was to describe the occurrence of alternating hypernatremia and hyponatremia in pediatric patients who underwent resection of hypothalamic hamartomas (HHs) for epilepsy. Hypernatremia in patients after pituitary or hypothalamic surgery can be caused by diabetes insipidus (DI), whereas hyponatremia can occur due to a syndrome of inappropriate antidiuretic hormone, cerebral salt wasting, or excessive administration of desmopressin (DDAVP). The triphasic response after surgery in the pituitary region can also explain variations in sodium parameters in such cases.

    Title Repeat Surgery for Hypothalamic Hamartoma in Refractory Epilepsy.
    Date April 2011
    Journal Neurosurgical Focus
    Excerpt

    Hypothalamic hamartomas (HHs) often cause pharmacoresistant epilepsy, incapacitating behavioral abnormalities, and cognitive decline. Surgical intervention offers the patient the best opportunity of seizure resolution, which occurs in approximately 50%–60% of patients, and improvement in both cognitive and behavioral difficulties. For those in whom the initial operation has failed, further medical treatment options remain quite limited, whereas, in some cases, a second surgery may improve seizure outcome. The authors retrospectively reviewed their surgical cases to document the success rate and complications of reoperations in patients with HHs.

    Title Gamma Knife Surgery for Hypothalamic Hamartomas and Epilepsy: Patient Selection and Outcomes.
    Date January 2011
    Journal Journal of Neurosurgery
    Excerpt

    The authors present outcomes obtained in patients who underwent Gamma Knife surgery (GKS) at 1 institution as part of a multimodal treatment of refractory epilepsy caused by hypothalamic hamartomas (HHs).

    Title Unexpected Upper Airway Obstruction Due to Disseminated Human Papilloma Virus Infection Involving the Pharynx in a Parturient.
    Date October 2010
    Journal Acta Anaesthesiologica Taiwanica : Official Journal of the Taiwan Society of Anesthesiologists
    Excerpt

    Human papilloma virus has been implicated as the cause of juvenile-onset recurrent respiratory papillomatosis in first-born children. Thus, cesarean section is strongly recommended by obstetricians to avoid direct contact with papilloma in an infected birth canal. We report a parturient with silent hypopharyngeal papilloma, which was associated with severe clinical problems at the induction of general anesthesia. The anesthesiologist considered general anesthesia for this case because of disseminated skin warts and secondary pus across the patient's body. Although the patient's breathing was smooth before starting general anesthesia, it was difficult to maintain positive pressure ventilation despite administration of a muscle relaxant. High air-way resistance without chest wall motion was noted, despite the insertion of an oral airway. Therefore, direct laryngoscopy was immediately performed and an obstructing mass was found. This mass acted as a check valve during positive ventilation. Our report should remind clinicians that human papilloma virus infection, although benign, can be disastrous in certain circumstances, as in our case where it resulted in airway obstruction and distal spread during cesarean section. Its presence necessitated preoperative laryngoscopic evaluation and aggressive treatment.

    Title Intravenous Levetiracetam in Children with Seizures: a Prospective Safety Study.
    Date August 2010
    Journal Journal of Child Neurology
    Excerpt

    In 2006, intravenous levetiracetam received US Food and Drug Administration (FDA) approval for adjunctive treatment of partial onset seizures in adults with epilepsy, 16 years or older. We have established the safety, tolerability, and dosage of intravenous levetiracetam in children. This prospective study included 30 children (6 months to <15 years of age). Patients were administered a single dose of intravenous levetiracetam (50 mg/kg, maximal dose 2500 mg) over 15 minutes. A blood level of levetiracetam was performed 10 minutes after the infusion. The treated children's average age was 6.3 years (range 0.5-14.8 years). The mean levetiracetam level was 83.3 microg/mL (range 47-128 microg/mL). There were no serious adverse reactions. Minor reactions included sleepiness, fatigue, and restlessness. An apparent decrease in seizure frequency across all seizure types was noted. The dose of 50 mg/kg was well tolerated by the patients and is a safe, appropriate loading dose.

    Title A Case of Symptomatic, Lesional Epilepsy (hypothalamic Hamartoma) Masquerading As a Generalized Epilepsy with Lennox-gastaut Syndrome.
    Date July 2010
    Journal Seminars in Pediatric Neurology
    Title Editorial Comment.
    Date July 2010
    Journal Seminars in Pediatric Neurology
    Title Dramatic First Words Spoken in 2 Children After Vagus Nerve Stimulation.
    Date July 2010
    Journal Seminars in Pediatric Neurology
    Excerpt

    Vagus nerve stimulation has become an accepted adjunctive treatment for refractory epilepsy with more recent FDA approval in the treatment of depression. Many "positive" effects have been noted in patients with epilepsy namely increased alertness, improved cognition, behavior and mood. These changes appear to be independent of seizure reduction and antiepileptic drug decrease. We present two children (aged 8 and 9 years) who were non-verbal and spoke their first words shortly after vagus nerve stimulators were implanted. The mechanism is unclear although vagus nerve stimulation has been clearly shown to induce neuronal, chemical and perfusion changes in both subcortical and cortical regions of the brain. There is likely a combined effect on primary speech areas as well subcortical and mamillothalamic tracts, and possibly even stimulation and changes at the local vocal cord level contributing to this phenomenon. Our observation has important implications in encephalopathic patients both with and without epilepsy.

    Title Central Precocious Puberty Due to Hypothalamic Hamartomas Correlates with Anatomic Features but Not with Expression of Gnrh, Tgfalpha, or Kiss1.
    Date July 2010
    Journal Hormone Research in Pædiatrics
    Excerpt

    Hypothalamic hamartomas are the most common identifiable cause of central precocious puberty (CPP). Hamartoma characteristics proposed to be associated with CPP include specific anatomic features and expression of molecules such as gonadotropin-releasing hormone (GnRH), transforming growth factor alpha (TGFalpha), and GRM1A, which encodes the type 1 metabotropic glutamate receptor alpha isoform. We sought to determine whether hamartomas that cause CPP could be distinguished by anatomic features, expression of these molecules, or expression of KISS1, whose products signal through the receptor GPR54 to stimulate GnRH release.

    Title Reversible Lamotrigine-induced Neurobehavioral Disturbances in Children with Epilepsy.
    Date March 2010
    Journal Journal of Child Neurology
    Excerpt

    A retrospective review was performed in patients who developed neurobehavioral adverse reactions to lamotrigine. Data were obtained from interviews, examinations, and routine medical records. There were 7 male and 2 female patients with epilepsy with a mean age of 5 years. All 9 patients became hyperactive and agitated over a broad range of lamotrigine dosing (0.7-14.0 mg/kg per d). Five patients developed self-injurious and violent behaviors. Two patients developed severe insomnia. The most affected patient was a 6-year-old boy whose mood and affect became extremely volatile. He also experienced threatening visual and auditory hallucinations and insomnia. All 9 patients had dramatic improvement and/or resolution of the adverse neurobehavioral effects following discontinuation or reduction of lamotrigine. Reversible, severe neurobehavioral disturbances associated with lamotrigine therapy have not been reported in the literature. While idiosyncratic and uncommon, this is a potentially significant, clinical side effect. Further studies are necessary to clarify the population at risk.

    Title The Value of Specifying Brand-name Antiepileptic Drugs.
    Date December 2009
    Journal Archives of Neurology
    Title Stimulus-induced Diffuse Voltage Attenuation (sidva): a Previously Undescribed Eeg Pattern in a Critically Ill Child.
    Date August 2009
    Journal Journal of Child Neurology
    Excerpt

    We report the case of a 15-month-old critically ill child with stimulus-induced diffuse voltage attenuation, a previously unreported electroencephalogram pattern. No clinical evidence of seizure activity was associated with these EEG changes. The patient went on to have a full recovery. This case suggests that critically ill children have distinct stimulus induced electrographic patterns from those seen in adults. Additional research is needed to define the significance of these patterns.

    Title Clobazam in the Treatment of Lennox-gastaut Syndrome.
    Date June 2009
    Journal Epilepsia
    Excerpt

    This randomized, double-blind, dose-ranging study evaluated safety and efficacy of clobazam (CLB) as adjunctive therapy for drop seizures in patients with Lennox-Gastaut syndrome (LGS).

    Title Correct Definition and Reference of the Term "status Gelasticus".
    Date January 2009
    Journal Journal of Child Neurology
    Title Prosthetic Mitral Leaflet Malfunction Due to Inappropriate Size of Implant.
    Date October 2008
    Journal Acta Anaesthesiologica Taiwanica : Official Journal of the Taiwan Society of Anesthesiologists
    Excerpt

    We report a case of unexpected malfunction of prosthetic mitral leaflets, discovered by transesophageal echocardiography (TEE) immediately after implantation, due to selection of an inappropriate size of prosthesis; the problem was amended immediately by surgical revision. The subject was a 48-year-old man admitted for mitral valve replacement because of severe mitral stenosis. He had undergone mitral commissurotomy 21 years previously. Preoperative echo findings showed severe mitral stenosis with regurgitation and a huge thrombus in the left atrial chamber. During operation, the surgeon also discovered severe calcification over the mitral valve with rheumatic changes in both leaflets and the chordae. Because the posterior leaflet had totally fused with the annulus, resection could not be performed. A 33-mm Edwards-Carpentier porcine xenograft was then installed and sutured onto the approximate annular position. The posterior chordae were also not resected, and the atriotomy was closed. Once the aortic cross-clamp was removed and the patient was re-warmed, the left atrium distended rapidly. No ventricular wall motion was seen. Immediate TEE evaluation demonstrated that the prosthetic valve was dysfunctional and the leaflets were immobile. Total cardiopulmonary bypass was then resumed. A smaller 29-mm valve was reimplanted in the same anatomic position. The 33-mm tissue valve was then re-examined and checked for any structural abnormality or functional inadequacy. The prosthetic valve stent was intact and the leaflets were mobile upon gross inspection. The patient was successfully weaned from cardiopulmonary bypass with high dose inotropic agents and intra-aortic balloon pump support. In conclusion, TEE can provide us with immediate, accurate and detailed information to evaluate an unexpected circumstance and disclose an operative misadventure during or after cardiac surgery.

    Title Intellectual Functioning in Presurgical Patients with Hypothalamic Hamartoma and Refractory Epilepsy.
    Date September 2008
    Journal Epilepsy & Behavior : E&b
    Excerpt

    OBJECTIVE: The goal of the work described here was to examine the relationship between intellectual test performance in patients with hypothalamic hamartoma (HH) with refractory epilepsy and their seizure histories, as well as the size and neuroradiographic anatomical features of the HH. It was predicted that the level of estimated intelligence and the pattern of intellectual test performance would significantly correlate with the size of the HH and neuroanatomical features. METHOD: In this cross-sectional design study, 49 patients with HH between the ages of 5 and 55 years were classified by age at time of examination, as well as pattern of performance on the Wechsler intelligence scales. All patients were included in data analysis irrespective of their ability to participate in psychometric testing. Patients with a prior history of neurosurgical treatment were excluded. RESULTS: For those patients functionally capable of participating in cognitive testing (n=42), a summary index score, which estimated level of intellectual function (composed of the Vocabulary, Block Design, and Coding subtests of the Wechsler intelligence scales), was significantly correlated only with number of antiepileptic drugs (AEDs) the patient was taking at the time of evaluation (r=-0.66, n=38, P=0.05). In contrast, a categorization method addressing the pattern of intellectual test performance (including those patients who were not functionally capable of participating in cognitive testing, n=49) was significantly correlated with number of AEDs (r=+0.35, n=48, P=0.01), size of HH (r=+0.38, n=49, P=0.01), presence of precocious puberty (PP: r=+0.41, n=49, P=0.01), and anatomical classification of HH (r=+0.39, n=49, P=0.01). CONCLUSIONS: The findings confirm the wide range of cognitive functioning in the population of patients with HH and refractory epilepsy, and suggest that multiple variables are correlated with intellectual test performance in patients with HH with refractory epilepsy. Although the present cross-sectional design study does not answer the question of whether or not epilepsy severity produces lower intelligence in this patient population, number of AEDs and neuroanatomical features of the HH lesion are identified as being significantly related to cognitive performance in this patient sample.

    Title Clarification of the Term "status Gelasticus" and Treatment and Prognosis of Gelastic Seizures.
    Date July 2008
    Journal Pediatric Neurology
    Title The Hitchhiker's Guide to the Child Neurologist's Genetic Evaluation of Epilepsy.
    Date May 2008
    Journal Seminars in Pediatric Neurology
    Excerpt

    Over the past several decades, familial aggregation studies as well as twin studies have supported a genetic component to seizures. The recent advent of the genome project has served as a catalyst in the search for elucidating the hereditary influences of various epilepsies. Overlapping seizure features may lead to ambiguity when attempting to isolate a single phenotype. Conversely, the phenomenon of genetic heterogeneity implies that multiple genetic mutations may give rise to a similar phenotype. Despite valiant attempts at strictly defining epilepsy phenotype and mode of penetrance, one must also consider the role of environment in gene expression. Genetics (testing) in epilepsy is no longer limited to the idiopathic epilepsies but may have an equally significant role in the symptomatic epilepsies. This article guides the reader through the genetics of epilepsy via discussion of the phenotypic description of known genetic childhood epilepsy syndromes, illustration of the associated gene mutations identified thus far, and the implications of genetic testing in clinical practice.

    Title Endoscopic Resection of Hypothalamic Hamartomas for Refractory Symptomatic Epilepsy.
    Date May 2008
    Journal Neurology
    Excerpt

    BACKGROUND: Hypothalamic hamartomas (HHs), rare developmental abnormalities of the inferior hypothalamus, often cause refractory, symptomatic, mixed epilepsy, including gelastic seizures. We present 37 patients with HH who underwent transcortical transventricular endoscopic resection. METHODS: Between October 2003 and April 2005, 42 consecutive patients with refractory epilepsy who underwent endoscopic resection of HH were studied prospectively. The endoscope was held by an articulated pneumatic arm and tracked with a frameless stereotactic neuronavigation system. Data collection and follow-up were performed by personal interview. Five patients were excluded. The remaining 37 patients (22 males, 15 females; median age 11.8 years; range 8 months to 55 years) had frequent and usually multiple types of seizures. RESULTS: Postoperative MRI confirmed 100% resection of the HH from the hypothalamus in 12 patients. At last follow-up (median 21 months; range 13-28 months), 18 (48.6%) patients were seizure free. Seizures were reduced more than 90% in 26 patients (70.3%) and by 50% to 90% in 8 patients (21.6%). Overall, the mean postoperative stay was shorter in the endoscopic patients compared with our previously reported patients who underwent transcallosal resection (mean 4.1 days vs 7.7 days, respectively; p = 0.0006). The main complications were permanent short-term memory loss in 3 patients and small thalamic infarcts in 11 patients (asymptomatic in 9). CONCLUSIONS: Endoscopic resection of hypothalamic hamartoma (HH) is a safe and effective treatment for seizures. Its efficacy seems to be comparable to that of transcallosal resection of HH, but postoperative recovery time is significantly shorter.

    Title The Role of Neurosurgery in Status Epilepticus.
    Date December 2007
    Journal Neurocritical Care
    Excerpt

    INTRODUCTION: Status epilepticus remains a life-threatening condition that afflicts both adults and children which although occurs in patients with epilepsy, often presents as new-onset seizure activity also. Refractory status epilepticus poses a management challenge for neurological and neurosurgical teams. CASE REPORT AND METHODS: Subdural grid electrodes were used to record cortical discharges and guide tumor resection involving eloquent cortex and multiple subpial transections in a 48-year-old man with left hemiparesis in status epilepticus. He had been refractory to multiple medical therapies in persistent epilepsia partialis continua for a prolonged period. As an alternative to higher-dose suppressive medical therapy, the patient elected to proceed with subdural grid mapping after seizure semiology ("negative" scalp electroencephalogram) localized the seizure focus to the right hemisphere, motor cortex. Following tumor removal, multiple subpial transections were subsequently performed over large areas of the motor and sensory strips and successfully resolved the status epilepticus. RESULTS: The patient made an excellent recovery, became seizure free, had improved left-sided strength and was discharged home shortly after. CONCLUSION: This case illustrates a potentially life-saving technique for the treatment of refractory status epilepticus. Multiple subpial transections and other neurosurgical intervention should be considered for patients with status epilepticus. When localization with surface electrodes is poor, especially in eloquent cortex, subdural grid recording can be used to direct focal resection and/or multiple subpial transections to minimize neurological deficits. A review and summary of previously published neurosurgery cases for status epilepticus is discussed.

    Title Anterior Motor Strip Displacement in a Boy with Right Frontal Gray Matter Heterotopia Undergoing Epilepsy Surgery.
    Date December 2007
    Journal Epilepsy & Behavior : E&b
    Excerpt

    The case of a 14-year-old boy with behavioral problems and medically refractory epilepsy related to symptomatic gray matter heterotopia in the right frontal lobe is reported. The patient underwent subdural cortical grid placement for seizure localization and functional mapping prior to lesionectomy. On the basis of the patient's MRI scan, it was predicted that the motor cortex was located adjacent to the abnormality, well anterior to its expected location. This prediction was confirmed by stimulation mapping. The patient underwent successful resection of the lesion under the guidance of the cortical map. Postoperatively, his seizures and behavior improved markedly. This case highlights how eloquent cortex can rarely be drawn toward a region of gray matter heterotopia. In addition, he is one of the few reported cases of focal cortical dysplasia resection with resultant improved behavior. These observations of unusual cortical representations have implications in epilepsy surgery planning.

    Title Gelastic Seizures Misdiagnosed As Gastroesophageal Reflux Disease.
    Date November 2007
    Journal Clinical Pediatrics
    Excerpt

    Gastroesophageal reflux disease can have variable manifestations including regurgitation, irritability, arching, choking, and apnea. The disorder is also frequently mistaken for seizures (Sandifer syndrome). We report 6 patients in whom the opposite phenomenon occurred: their seizures were mistaken for gastroesophageal reflux disease. Six of 77 patients (6.8%) with gelastic seizures and epilepsy symptomatic of hypothalamic hamartomas were noted to be misdiagnosed with gastroesophageal reflux disease in infancy. As is typical in these patients, gelastic seizures were not diagnosed until months, or often years, later. Delayed diagnosis of hypothalamic hamartomas can lead to a potentially deleterious syndrome involving refractory epilepsy, developmental problems, and precocious puberty. Gelastic seizures should be considered among the conditions that can mimic reflux symptoms.

    Title Chromosomal Abnormality at 6p25.1-25.3 Identifies a Susceptibility Locus for Hypothalamic Hamartoma Associated with Epilepsy.
    Date August 2007
    Journal Epilepsy Research
    Excerpt

    The pathogenesis of hypothalamic hamartoma (HH) associated with epilepsy is unknown. We have identified an individual with HH and refractory epilepsy exhibiting subtle dysmorphic features. High-resolution karyotype identified a duplication of the terminal end of 6p (6p25.1-25.3), confirmed by fluorescent in situ-hybridization (FISH). Copy number analysis with high-density (250K) single nucleotide polymorphism (SNP) genotyping microarrays characterized the abnormality as a series of amplified regions between 1.4 Mb and 10.2 Mb, with a small tandem deletion from 8.8 Mb to 9.7 Mb. There are 38 RefSeq genes within the duplicated regions, and no known coding sequences within the deletion. This unique patient helps identify 6p25.1-25.3 as a possible susceptibility locus for sporadic HH.

    Title Endoscopic Resection of Hypothalamic Hamartoma for Refractory Epilepsy: Preliminary Report.
    Date August 2007
    Journal Seminars in Pediatric Neurology
    Excerpt

    The intrahypothalamic subtype of hypothalamic hamartoma (HH) is usually associated with refractory epilepsy, cognitive impairment, and organic behavioral disturbance. It is often a devastating disorder for both patient and family. The gelastic (laughing) seizure is the hallmark seizure type. However, multiple other seizure types can often develop during the course of the disease, and are typically refractory to antiepilepsy drugs (AEDs). Previously it was uncertain if HH tissue was responsible for seizure genesis or whether resection of the HH would result in improvement of the seizures. Recently both of these questions have been answered in the affirmative. Surgical resection using a transcallosal, interforniceal approach has recently been shown to be efficacious and generally safe for the treatment of the refractory seizures. However, even more recently, we have been performing the majority of HH surgical resection by using an endoscopic technique with a transventricular approach. This article presents the details of the operative technique and discusses preliminary outcome data, particularly with comparison to the transcallosal technique. Using an endoscope holder with micromanipulator facilitates endoscopic resection. Linking the endoscope to a system of frameless stereotaxis is essential for successful resection. Forty-four patients age 8 months to 44 years have undergone endoscopic resection. The ideal candidate for endoscopic removal has a hamartoma completely or nearly completely involving one wall of the third ventricle and is 1 cm or less in greatest diameter. Because it is essential to be able to visualize the lesion within the third ventricle for resection, there must be at least 6 mm of space between the top of the lesion and the roof of the third ventricle. Patients with intractable epilepsy caused by HH can be rendered seizure free or show marked improvement in seizure frequency by surgical removal, surgical disconnection, or radiosurgical ablation of the lesion. Which of these options should be recommended for an individual patient is not yet clear. One of the options involves resection or disconnection of the HH with a transventricular endoscopic approach. In selected patients, endoscopic resection of HH is effective in the treatment of intractable epilepsy, with lower complication rates and shorter hospital stays than transcallosal resection.

    Title Intravenous Leiomyomatosis with Extension to the Heart: Echocardiographic Features: a Case Report.
    Date August 2007
    Journal Angiology
    Excerpt

    A 40-year-old female was admitted to the hospital for an operation with a presumptive diagnosis of right atrial myxoma. She had had a hysterectomy for leiomyoma 8 months earlier and has since experienced progressive dyspnea on exertion, pedal edema, and two syncope episodes in the past 2 months. Cardiac murmur was detected and two-dimensional echocardiography was arranged. A mobile right atrial mass was discovered, which was thought to be a myxoma. The patient subsequently received open heart surgery. Histologic examination of the resected mass confirmed intravenous leiomyomatosis. Magnetic resonance imaging performed on the eighth postoperative day revealed an intravascular mass from the right common iliac vein to the upper abdominal inferior vena cava. The patient underwent a second operation one and half months after the first operation, with resection of the tumor mass in the inferior vena cava and resection of multiple uterine myoma. She recovered well. Repeat magnetic resonance imaging 6 months following the second operation showed that the inferior vena cava and right atrium were clear of tumor.

    Title Clobazam.
    Date April 2007
    Journal Neurotherapeutics : the Journal of the American Society for Experimental Neurotherapeutics
    Excerpt

    Catastrophic childhood epilepsies such as infantile spasms (IS), progressive myoclonic epilepsy, and Lennox-Gastaut syndrome (LGS) are rare but debilitating and frequently persist into adulthood. Early, targeted use of medications that have demonstrated efficacy in the management of LGS or its associated epilepsies may simplify the patient's treatment regimen and reduce the incidence of adverse events. Key to the overall benefit to the patient is to maximize seizure control while minimizing adverse effects, especially behavioral and cognitive problems. Clobazam has demonstrated clinical benefit and has been administered safely in more than 50 European studies in which data were reported on greater than 3000 pediatric and adult patients with epilepsy, 300 of whom were diagnosed with LGS; therefore, its use is now being investigated in the U.S. This review will explore the use of clobazam in the treatment of epilepsy, particularly with regard to its potential benefit in LGS. Though not currently approved for use in the U.S., a program is underway to gain Food and Drug Administration approval for the treatment of pediatric and adult patients with refractory epilepsy, specifically in LGS. A phase 2 study will be completed in late 2006 to investigate the safety and efficacy of clobazam as adjunctive therapy in 68 pediatric and adult patients with LGS.

    Title Hypothalamic Hamartoma and Infantile Spasms.
    Date March 2007
    Journal Epilepsia
    Excerpt

    PURPOSE: A small number of patients with hypothalamic hamartoma (HH) present with infantile spasms (IS) as an initial or early seizure type. We describe the clinical features of IS associated with HH. METHODS: Our series of patients with HH and epilepsy was reviewed to identify cases with a history of IS. The clinical features and neuroradiological findings in this study group were compared to a control group of patients with HH and refractory epilepsy, but without a history of IS. RESULTS: We identified six patients with HH and a history of IS in this series (n = 122, 4.9%). Five of the six are male. Four of the six patients (67%) developed IS as their first seizure type. The mean age for onset of IS was 6.2 months (range 4-9 months). Results of electroencephalographic (EEG) study at the time of IS diagnosis showed hypsarrhythmia in two (33%). Five patients were treated with adrenocorticotropic hormone (ACTH), and four of the five (80%) responded with control of IS. However, these patients developed other seizure types, and were ultimately refractory to medical management. Aside from the IS, no significant differences in clinical and imaging features were determined between the study group and the control group. CONCLUSIONS: HH should be included in the differential diagnosis for infants presenting with IS. These patients may have hypsarrhythmia on initial EEG, and may respond to ACTH treatment with improvement of IS. However, all became refractory with other seizure types, more commonly seen in HH patients. Focal pathologies associated with IS may be subcortical, as well as cortical, in nature. We have not identified any predictive features for the occurrence of IS in the HH population.

    Title The Histopathology of Hypothalamic Hamartomas: Study of 57 Cases.
    Date March 2007
    Journal Journal of Neuropathology and Experimental Neurology
    Excerpt

    Hypothalamic hamartomas (HHs) are rare developmental tumors that cause seizures or pituitary axis dysfunction, usually beginning in childhood. We analyzed HH tissue from 57 patients whose tumors were resected through recently developed transcallosal interforniceal and transventricular endoscopic surgical approaches. All cases were composed of abnormally distributed but cytologically normal neurons and glia, including fibrillary astrocytes and oligodendrocytes. Neuronal elements predominated in most cases, but a relative increase in astrocytic elements was seen with increasing age. All had various sized nodular foci of neurons as well as areas of diffusely distributed neurons with interspersed glial cells. Smaller neurons predominated, and most cases had only a few interspersed large ganglion cells. Immunohistochemistry demonstrated extensive production of synapse-associated proteins. Immunohistochemistry for phosphorylated and nonphosphorylated neurofilament and alpha-internexin demonstrated staining patterns consistent with mature neurons. In contrast to cortical dysplasia, atypical large ganglion-like balloon cells were almost never seen. In summary, although their number and distribution vary, mature smaller neurons were the most prominent and most consistent histologic feature of HH. Nodules of these small neurons were a universal feature of the microarchitecture of HH lesions associated with epilepsy. Characterization of these neurons may aid in understanding the mechanism of seizure development in HH.

    Title Neurosurgical Treatment of Status Epilepticus.
    Date March 2007
    Journal Journal of Neurosurgery
    Excerpt

    OBJECT: The purpose of this study was to report the cases of five patients with status epilepticus who were treated successfully with neurosurgery. METHODS: Five children presented with refractory status epilepticus, including complex partial status epilepticus, epilepsia partialis continua and "status gelasticus." Multiple medical therapies had failed to control the seizures, and focal resection was performed. Seizures were fully controlled in four patients, and in one patient seizure frequency was reduced by more than 90%. CONCLUSIONS: Refractory status epilepticus is associated with a high mortality rate and by definition does not respond well to medical treatment, even optimal medical treatment. Neurosurgical treatment should be considered as another therapeutic option once medical management has failed, and neurosurgery should not necessarily be a last resort.

    Title Endoscopic Surgery for Hypothalamic Hamartomas Causing Medically Refractory Gelastic Epilepsy.
    Date January 2007
    Journal Child's Nervous System : Chns : Official Journal of the International Society for Pediatric Neurosurgery
    Excerpt

    INTRODUCTION: There is increasing evidence that removal or disconnection of hypothalamic hamartomas can lead to seizure control and improvement in behavior. The purpose of this study is to report our experiences with endoscopic removal of these hamartomas. METHODS: This is a prospective review of the early outcome of 44 patients undergoing endoscopic resection utilizing frameless stereotaxis and a micromanipulator. RESULTS: All patients had normal-sized ventricles. Complete removal was possible in 14 patients, 13 of whom were seizure free at follow-up. Four patients suffered complications that have persisted for more than 3 months: One had significant hemiparesis and three had persistent difficulties with short-term memory. Postoperatively, no patient suffered a permanent hormonal deficiency. In one patient who had undergone a previous attempt at transcallosal resection, the procedure was abandoned because of the lack of internal anatomic landmarks. In a second patient, insufficient mass could be removed due to the size of the lesion and the distorted anatomy. Consequently, 1 week later, the patient underwent a transcallosal approach. CONCLUSION: In appropriately selected patients, sessile hypothalamic hamartomas can be removed completely or they can be completely disconnected from the hypothalamus with an acceptable risk. At least a year after surgery is needed to evaluate the efficacy of the procedure in terms of control of seizures and improvements in behavior and emotional ability.

    Title Childhood Mesial Temporal Sclerosis.
    Date November 2006
    Journal Journal of Child Neurology
    Excerpt

    The prevalence and clinical characteristics of mesial temporal sclerosis have not been well studied in children. All brain magnetic resonance imaging (MRI) reports of children less than 14 years of age were reviewed from two tertiary institutions. A 52-month period from one institution and a 37-month period from the other were reviewed. All reports of definite or possible mesial temporal sclerosis were noted. These patients' MRIs were then reviewed to confirm the MRI diagnostic criteria of mesial temporal sclerosis. The charts of the patients who satisfied these criteria were reviewed in detail. Three thousand one hundred brain MRI reports were reviewed. Twenty-six reports of mesial temporal sclerosis were found. Twenty-four of the 26 films satisfied the criteria of mesial temporal sclerosis by MRI after the films were reviewed. The prevalence among all pediatric brain MRI studies was 0.77%. All patients had presented with seizures, that is, there were no "incidental" findings of mesial temporal sclerosis. Four patients had a history of febrile seizures. Mesial temporal sclerosis is an uncommon finding in children, but when it occurs, it is always associated with epilepsy. Asymptomatic mesial temporal sclerosis or mesial temporal sclerosis not associated with a seizure disorder did not occur in our series. Febrile seizures can occur in association with mesial temporal sclerosis presenting in childhood.

    Title Transcallosal Resection of Hypothalamic Hamartoma for Intractable Epilepsy.
    Date August 2006
    Journal Epilepsia
    Excerpt

    PURPOSE: To present the results of transcallosal surgical resection of hypothalamic hamartoma (HH) in 26 patients with refractory epilepsy in a prospective outcome study. METHODS: Patients with refractory epilepsy symptomatic to HH were referred for surgical resection of their HH (mean age, 10.0 years; range, 2.1-24.2 years). A transcallosal, interforniceal approach was used to remove and/or disconnect the hamartoma. Volumetry was obtained on pre- and postoperative brain MRI scans to determine percentage of resection. Outcome assessment included determination of postoperative seizure frequencies in comparison to baseline and the incidence of postoperative complications. Postoperative changes in cognitive and behavioral functioning, in comparison to baseline, were elicited by parental report. RESULTS: The average postoperative follow-up interval was 20.3 months (range, 13-28 months). Fourteen (54%) patients were completely seizure free, and nine (35%) had at least a 90% improvement in total seizure frequency. Parents reported postoperative improvement in behavior in 23 (88%) patients and in cognition in 17 (65%) patients. Transient postoperative memory disturbance was seen in 15 (58%) patients, but persisted in only two (8%). Two (8%) patients had persisting endocrine disturbance requiring hormone replacement therapy (diabetes insipidus and hypothyroidism in one each). With univariate analysis, the likelihood of a seizure-free outcome correlated with younger age, shorter lifetime duration of epilepsy, smaller preoperative HH volume, and 100% HH resection. CONCLUSIONS: Refractory epilepsy associated with HH can be safely and effectively treated with surgical resection by a transcallosal, interforniceal approach. Short-term memory deficits appear to be transient for most patients, and family perception of the impact of surgery on cognitive and behavioral domains is favorable. Complete resection yields the best result.

    Title Coining of a New Term, "status Gelasticus".
    Date May 2006
    Journal Epilepsia
    Title Labetalol Pretreatment Reduces Blood Pressure Instability During Surgical Resection of Pheochromocytoma.
    Date April 2006
    Journal Journal of the Formosan Medical Association = Taiwan Yi Zhi
    Excerpt

    To evaluate the effect of pretreatment with the mixed alpha- and beta-adrenergic blocker, labetalol, on blood pressure instability during surgical resection of pheochromocytoma.

    Title Surgical Strategies for Approaching Hypothalamic Hamartomas Causing Gelastic Seizures in the Pediatric Population: Transventricular Compared with Skull Base Approaches.
    Date November 2005
    Journal Journal of Neurosurgery
    Excerpt

    OBJECT: The authors provide evidence that direct resection of hypothalamic hamartomas (HHs) can improve associated gelastic and nongelastic seizures. METHODS: Ten children younger than 17 years of age underwent resection of HHs (nine sessile and one pedunculated) that were causing refractory epilepsy. Lesions were approached from above transventricularly through a transcallosal anterior interforniceal approach in six cases, endoscopically through the foramen of Monro in one, and from below with a frontotemporal craniotomy including an orbitozygomatic osteotomy in three. Medical charts were reviewed retrospectively, and follow-up data were obtained through office records and phone calls. Follow-up periods ranged between 12 and 84 months (mean 16.8 months). All patients in whom the approach was from above had sessile HHs. Five were free from seizures at follow up and two had a reduction in seizures of at least 95%. The transventricular route allowed excellent exposure and visualization of the local structures during resection. Among the three patients in whom the approach was from below, one became free of seizure after two procedures and one had a 75% reduction in epilepsy; the latter two had sessile HHs. The exposure was inadequate, and critical tissue borders were not readily apparent. Although the HH was adequately exposed and resected, the epilepsy persisted in the third patient, who had a pedunculated lesion. The overall rate of major permanent hypothalamic complications appeared to be slightly lower for the orbitozygomatic osteotomy group. CONCLUSIONS: Sessile lesions are best approached from above. Approaches from below adequately expose pedunculated hamartomas. The likelihood of curing seizures seems to be higher when lesions are approached from above rather than from below.

    Title The Hypothalamic Hamartoma: a Model of Subcortical Epileptogenesis and Encephalopathy.
    Date October 2005
    Journal Seminars in Pediatric Neurology
    Excerpt

    Although uncommon, the hypothalamic hamartoma (HH) is often associated with a devastating clinical syndrome, which may include refractory epilepsy, progressive cognitive decline, and deterioration in behavioral and psychiatric functioning. Contrary to conventional thinking which attributed seizure origin to cortical structures, the hamartoma itself has now been firmly established as the site of intrinsic epileptogenesis for the gelastic seizures (i.e., characterized by unusual mirth) peculiar to this disorder. It also appears that the HH contributes to a process of secondary epileptogenesis, with eventual cortical seizure onset of multiple types in some patients. Anticonvulsant medications are known to be poorly effective in this disorder. Treatment, including some innovative approaches to surgical resection, is now targeted directly at the HH itself, with impressive results. Younger patients, in particular, may avoid the deteriorating course described earlier. Access to tissue from larger numbers of patients at single or collaborating centers specializing in HH surgery will allow for research into the fundamental mechanisms producing this little understood disorder. Refractory epilepsy associated with HH is the premier human model for subcortical epilepsy and an excellent model for secondary epileptogenesis and epileptic encephalopathy.

    Title Successful Resection of a Hypothalamic Hamartoma and a Rathke Cleft Cyst. Case Report.
    Date October 2005
    Journal Journal of Neurosurgery
    Excerpt

    The authors report the case of a 12-year-old girl with Pallister-Hall syndrome, long-standing refractory, symptomatic epilepsy, mental retardation, and panhypopituitarism in whom two rare, deep midline lesions were detected. She underwent successful transsphenoidal resection of the Rathke cleft cyst and transcallosal resection of the hypothalamic hamartoma within a 4-day period without complications. Neuropathological studies confirmed the neuroimaging diagnoses for the two lesions. The patient has been seizure free for 6 months postoperatively.

    Title Neurologic Complications Associated with Respiratory Syncytial Virus.
    Date July 2005
    Journal Pediatric Neurology
    Excerpt

    Encephalopathy has been demonstrated to be associated with respiratory syncytial virus bronchiolitis. In this study, the data on all patients less than 14 years of age hospitalized with respiratory syncytial virus bronchiolitis over the past 4 years was reviewed. Patients who had concomitant diagnoses consistent with neurologic disease underwent detailed chart review. There were 964 patients (age 0.1 to 13.6 years) with a diagnosis of respiratory syncytial virus bronchiolitis. Thirty-six of these patients had concurrent neurologic diagnoses. Twenty-four patients were excluded because of preexistent neurologic disorders, probable simple febrile seizures, or a history of epilepsy. Twelve respiratory syncytial virus-positive patients had definite neurologic complications without another recognized cause. Seven of these patients had seizures (predominantly generalized tonic-clonic and one with status epilepticus), three had generalized encephalopathy (marked hypotonia and decreased responsiveness) of whom two also developed esotropia. Two patients developed isolated esotropia. There was an incidence of neurologic complications of 1.2% (0.7% seizures) in a total of 964 patients with respiratory syncytial virus bronchiolitis. This percentage does not include presumed simple febrile seizures or exacerbations of preexisting seizure disorder (further 1.3%). Neurologic complications occur with respiratory syncytial virus bronchiolitis, and physicians and other caregivers should be aware of this entity as well as the favorable prognosis.

    Title The Extensor Plantar Response in Neonates is Not the Same As the Babinski Sign.
    Date May 2005
    Journal Pediatric Neurology
    Title Emergency Transcallosal Resection of Hypothalamic Hamartoma for "status Gelasticus".
    Date April 2005
    Journal Epilepsia
    Excerpt

    Hypothalamic hamartomas are rare developmental malformations of the inferior hypothalamus. Typically they result in symptomatic, refractory mixed seizure disorder with trademark gelastic or laughing seizures. We present a 30-month-old boy with a hypothalamic hamartoma and a nearly 2-month history of near-continuous gelastic seizures, which we have labeled "status gelasticus." The seizures were refractory to multiple antiepileptic drugs (AEDs), and emergency transcallosal resection of the hypothalamic hamartoma was performed with resultant near-immediate cessation of the seizures. At 12-month follow-up, the patient was averaging one brief gelastic seizure every 2 weeks, with a longest seizure-free period of 2 months.

    Title Agenesis of the Corpus Callosum is Associated with Feeding Difficulties.
    Date October 2004
    Journal Journal of Child Neurology
    Excerpt

    Our objective was to characterize the common occurrence of feeding and swallowing disorders noticed by our speech therapy department among patients with agenesis of the corpus callosum. All patients with suspected or presumed agenesis of the corpus callosum undergoing therapy for feeding and/or swallowing disorders, including oral and/or pharyngeal dysphagia and oral-sensory disorder, were identified. Their brain magnetic resonance imaging (MRI) studies and charts were reviewed in detail. Seven patients with striking oral-motor and oral-sensory disorder met the criteria for agenesis of the corpus callosum. Particular difficulties noted include oral-sensory defensiveness in five of these seven patients, oral dysphagia/weakness in all seven patients, and pharyngeal dysphagia with risk of aspiration in three of these seven patients. The oral-motor weakness observed in all of the patients affected the strength and coordination necessary for functional oral-phase swallowing. We conclude that feeding and swallowing disorders are significant in children with agenesis of the corpus callosum. Although the pathophysiologic mechanism is not known, recognition of this phenomenon is important because early detection and intervention can facilitate functional feeding and swallowing patterns earlier in these patients, therefore avoiding the adverse and more severe disorders that can arise when dysphagias and/or oral-sensory disorder persist over extended periods of time.

    Title High Frequency Jet Ventilation in Exploration of Tracheostomy--a Case Report.
    Date September 2004
    Journal Acta Anaesthesiologica Taiwanica : Official Journal of the Taiwan Society of Anesthesiologists
    Excerpt

    Exploration of tracheostomy is not uncommon, however, in case a patient with a facial reconstruction flap, trouble may arise because of difficulty in intubation. We hereby report a patient who had a fresh facial reconstruction flap after radical resection of buccal carcinoma, sustained bleeding around the tracheostomy and was scheduled for exploration of tracheostomy. Fiberoptic oral intubation was failed because of profuse secretion and edematous oral mucosa; high frequency jet ventilation (HFJV) was thus applied and made possible with a suction catheter put through the tracheostomy tube. Exploration was performed following withdrawal of the tracheostomy tube. The tracheostomy tube was reinserted under the guidance of the in-place suction tube after uneventful exploration. The probable concomitant complications and contraindications of using HFJV are discussed here. With thorough preparation and careful monitoring, and under the supervision of experienced physicians, transtracheal HFJV can substitute fiberoptic intubation for ventilation in case of exploration of tracheostomy.

    Title Treatment of Refractory Status Epilepticus with Hemispherectomy.
    Date September 2004
    Journal Epilepsia
    Excerpt

    A 7-year-old boy with left hemiparesis secondary to right hemispheric cortical dysplasia was admitted to the hospital with increasing numbers of seizures. Magnetic resonance imaging showed a small dysplastic right hemisphere with abnormally thickened gyri and an apparently normal left hemisphere. Previous video-electroencephalogram (EEG) monitoring showed bilateral independent spikes and generalized slow spike-and-wave episodes on EEG and [18F]fluorodeoxyglucose (FDG) positron emission tomography scan demonstrated scattered areas of regional hypometabolism bilaterally; therefore hemispherectomy was not undertaken at that time. During this hospital stay, nonconvulsive status epilepticus developed and was refractory to multiple medical therapies including pentobarbital (PTB) coma. Burst-suppression pattern during PTB coma appeared to be generalized spike and wave, but when EEG was reviewed with increased time resolution spikes suggested a right hemisphere origin. The patient underwent bilateral intracarotid amobarbital spike-suppression test that showed only minimal suppression of epileptiform discharges with injection of the left carotid, but complete suppression of spike activity after right-sided carotid injection. A right hemispherectomy was performed with complete cessation of status epilepticus. Postoperative EEG showed no epileptiform discharges. Patient follow-up was limited to 12 months after surgery. The patient had regained the ability to walk unaided and was seizure free with a single antiepileptic medication. This case illustrates a potentially life-saving procedure for refractory status epilepticus and several techniques including a spike-suppression test to aid in prediction of cessation of seizures after hemispherectomy.

    Title Reliability of Plantar Response Requires Consistency of the Applied Stimulus.
    Date June 2004
    Journal Journal of Paediatrics and Child Health
    Title Magnetic Resonance Imaging Detection of Mesial Temporal Sclerosis in Children.
    Date April 2004
    Journal Pediatric Neurology
    Excerpt

    The objective of this study was to investigate the prevalence and clinical characteristics of mesial temporal sclerosis as diagnosed by brain magnetic resonance imaging in children. A total of 390 consecutive brain magnetic resonance imaging studies in children were reviewed for evidence of mesial temporal sclerosis. Subsequently, the magnetic resonance imaging scans and charts of patients with mesial temporal sclerosis were reviewed and their clinical details were evaluated. The magnetic resonance imaging studies had been performed for multiple indications, including seizures, headache, and developmental problems. In children, the prevalence of mesial temporal sclerosis among all brain magnetic resonance imaging studies was 3.1% (12 of 390 studies) and 12.1% (12 of 99 studies) among all brain magnetic resonance imaging studies performed for seizures. These children all presented with a history of seizure disorder, often had other medical problems, and histopathology (when available) nearly always (5 of 6 patients) confirmed their magnetic resonance imaging diagnosis of mesial temporal sclerosis. The prevalence of mesial temporal sclerosis is low among all pediatric patients who had magnetic resonance imaging brain studies. All our mesial temporal sclerosis patients had clinical seizures; i.e., it was never an "incidental finding". Children with mesial temporal sclerosis often had comorbid conditions, and the diagnosis of mesial temporal sclerosis made by magnetic resonance imaging was accurate when compared with the available histopathology.

    Title Failure to Provide Adequate One-lung Ventilation with a Conventional Endotracheal Tube Using a Transbronchial Approach: a Case Report.
    Date November 2003
    Journal Canadian Journal of Anaesthesia = Journal Canadien D'anesthésie
    Excerpt

    To report a case where failure to provide adequate one-lung ventilation during transbronchial intubation resulted in a potentially fatal mishap.

    Title Idiopathic Intracranial Hypertension in the Pediatric Population.
    Date August 2003
    Journal Journal of Child Neurology
    Title Vascular Injury During a Lumbar Laminectomy.
    Date June 2003
    Journal Chang Gung Medical Journal
    Excerpt

    A lumbar laminectomy is a common and routine operation. Damage to abdominal vascular structures during surgery is a relatively infrequent complication; however, when it does occur, it is sudden and life-threatening. We herein report on 2 cases of abdominal vascular injury which occurred during lumbar microdiscectomies. The first case was a 34-year-old man. A bloody surgical field was noted 45 min into the operation along with an increase in heart rate and a decrease in blood pressure. After fluid resuscitation and an ephedrine injection, his vital signs stabilized. The patient was then sent to the surgical intensive care unit for observation. An emergent abdominal computer tomography scan revealed right retroperitoneal hematoma, and an urgent exploratory laparotomy was performed to check for bleeding and to remove the hematoma. The second case was a 61-year-old woman with recurrent disc herniation. The operation was proceeding smoothly for 90 min, when a large amount of fresh blood suddenly gushed out. Her blood pressure immediately dropped to that of a state of shock. The patient was turned back to a supine position, and an emergent laparotomy was done to repair the injured vessels. Both patients had uneventful recoveries.

    Title Successful Neurosurgical Treatment of Childhood Complex Partial Status Epilepticus with Focal Resection.
    Date June 2003
    Journal Epilepsia
    Excerpt

    The treatment of complex partial status epilepticus continues to be controversial, especially with regard to the intensity of the treatment. Medical therapy and drug-induced coma are sometimes required. Rarely this may not be effective. A healthy 4-year old girl was first seen in complex partial status epilepticus. She had a 1-year history of cryptogenic partial-onset seizures. Detailed magnetic resonance imaging (MRI) studies were normal. Her course was refractory to multiple medical therapies and multiple subpial transection (MST). An urgent epilepsy surgery evaluation resulted in a focal cortical resection being performed over the right mesial parietal region with resultant seizure freedom and no significant neurologic deficit 2 years later. This patient illustrates the need to consider occult focal cortical dysplasia as a cause of nonconvulsive status epilepticus (NCSE) in children, and if it is not responsive to medical management, the utility of performing an urgent epilepsy surgery evaluation.

    Title Anesthetic Management of a Parturient Undergoing Cesarean Section with a Tracheal Tumor and Hemoptysis.
    Date April 2003
    Journal Chang Gung Medical Journal
    Excerpt

    Anesthetic management of a parturient with respiratory failure associated with hemoptysis, dyspnea, and orthopnea is difficult. An anesthesiologist should realize that the patient's major problem is not solved during the surgery. This circumstance is similar to a patient with associated cardiac disease scheduled for non-cardiac surgery. General anesthesia with endotracheal intubation can provide safe oxygenation for both the parturient and the fetus, but with possible unexpected massive hemoptysis and tumor seeding. Prolonged intubation may delay the patient's pulmonary treatment course. Laryngeal mask anesthesia can provide an airway, but must not be secured due to the risk of aspiration. The need of high doses of inhalation drugs may hinder uterine contractions. The addition of a muscle relaxant will change the patient's respiratory patterns and physiology. Regional anesthesia alone might not be tolerated. A decrease in cough strength, as well as dyspnea, orthopnea, and hyperventilation may be harmful to both the parturient and the fetus. However, we successfully managed this case using epidural anesthesia combined with assisted mask ventilation instead of spontaneous breathing usually provided by a simple mask in almost all American Society of Anesthesiology (ASA) class I-II parturients during cesarean section. The anesthetic level was maintained at T8 with 18 ml of 2% Xylocaine mixed with 2 ml of 7% sodium bicarbonate with 1:200,000 epinephrine epidurally and with the patient in a supine position with the head up at 30 degrees to prevent cephalic spreading and to ensure better pulmonary ventilation.

    Title Levetiracetam in Refractory Pediatric Epilepsy.
    Date February 2003
    Journal Journal of Child Neurology
    Excerpt

    Levetiracetam, one of the newer-generation antiepilepsy drugs, is not currently approved for use in children. Given its favorable efficacy, pharmacokinetic, and, particularly, safety profile in adults, we felt that it may be a useful antiepilepsy drug for children with refractory epilepsy. We treated 39 patients (mean age 8.6 years) with open-label levetiracetam for up to 9 months. Seizure frequency, drug dosages, adverse events, and neurologic examinations were documented at baseline and routine follow-up visits. Levetiracetam, as add-on therapy, was effective in reducing seizure frequency in a variety of seizure types but was most effective for partial-onset seizures. Fourteen patients were discontinued for lack of efficacy or adverse events. Ten patients reported improvements in cognition or behavior. Levetiracetam was generally effective and well tolerated in this open-label study. Its apparent positive effects on cognition in some patients are encouraging. Large, well-controlled studies are needed to fully define levetiracetam's potential in children with refractory epilepsy.

    Title Red Man's Syndrome Following Administration of Vancomycin in a Patient Under Spinal Anesthesia--a Case Report.
    Date December 2002
    Journal Acta Anaesthesiologica Sinica
    Excerpt

    Prophylactic antibiotics are frequently administered during anesthesia to reduce the incidence of infection. The most common organisms revealed in wound infections are staphylococci. Vancomycin is the antibiotic of choice for resistant staphylococcal infections and bacterial endocarditis in patient allergic to penicillin. We had a case of tibial osteomyelitis, while undergoing removal of implants under spinal anesthesia developed hypotensions, bradycardia, consciousness change and skin erythematous macular rash after 0.1% vancomycin slow infusion for 10 min. After appropriate management, the patient recovered well and was discharged on the following day. Our report is intended to alert our colleagues that vancomycin can cause hypotension secondary to histamine release, direct myocardial depression and direct peripheral vasodilation. Even cardiac arrest had been reported in the literatures.

    Title Lumbar Spinal Stenosis Causing Congenital Clubfoot.
    Date September 2002
    Journal Journal of Child Neurology
    Excerpt

    Congenital lumbar spinal stenosis is believed to rarely cause neurologic symptoms during childhood. We present a 16-year-old boy with bilateral congenital clubfeet surgically corrected by tendo Achillis releases at 2 years of age who presented with progressive, bilateral footdrop. Magnetic resonance imaging of his lumbosacral spine showed severe spinal stenosis at the L3-5 vertebrae. Congenital lumbar spinal stenosis is probably an under-recognized cause of lower extremity neurologic abnormalities, including clubfoot deformity. Magnetic resonance imaging has made this eminently treatable disorder easier to recognize.

    Title Sphenoid Sinusitis Masquerading As Migraine Headaches in Children.
    Date April 2002
    Journal Journal of Child Neurology
    Excerpt

    The sphenoid sinus is often referred to as the "neglected sinus." Isolated sphenoid sinusitis is a rare disease with potentially devastating complications. It occurs at an incidence of about 2.7% of all sinus infections. Although headache is the most common presenting symptom, there is no typical headache pattern. Three cases of children with isolated sphenoid sinusitis presenting with acute, subacute, and chronic headache symptoms are presented. The sensory innervation of the sphenoid sinus is derived from the ophthalmic and maxillary branches of the trigeminal nerve, which may explain the pathophysiology of the headache, similar to the trigeminovascular pain theory of migraine. There are few reports on sphenoid sinusitis and headache; however, modern neuroimaging has made this probably under-recognized disorder easier to diagnose and treat. Although the diagnosis can be difficult to differentiate from migraine headache, early and appropriate treatment usually results in an excellent outcome without morbidity.

    Title Respiratory Syncytial Virus.
    Date October 2001
    Journal The New England Journal of Medicine
    Title Encephalopathy Associated with Respiratory Syncytial Virus Bronchiolitis.
    Date July 2001
    Journal Journal of Child Neurology
    Excerpt

    Respiratory syncytial virus is an extremely common cause of childhood respiratory infections resulting in significant morbidity and mortality. Although apnea is a well-known complication in young infants with respiratory syncytial virus bronchiolitis, the encephalopathy associated with this infection is not well recognized. Our study reveals an incidence of encephalopathy of 1.8% in a total of 487 patients with respiratory syncytial virus bronchiolitis studied over a period of almost 4 years. Seizures were the presenting complication. Based on our study of a cohort of children with respiratory syncytial virus bronchiolitis, we believe that neurologic complications, although relatively uncommon, represent a significant component of this common childhood illness. Furthermore, respiratory syncytial virus has been shown to release several mediators that could directly or indirectly be neurotoxic and induce an encephalopathy associated with the respiratory illness.

    Title Visual-evoked Potentials in the Assessment of Optic Gliomas.
    Date May 2001
    Journal Pediatric Neurology
    Excerpt

    The most effective method for the screening and monitoring of optic pathway gliomas in children with neurofibromatosis type 1 remains a dilemma. Children less than 6 years of age are the group at highest risk for the development of optic pathway gliomas. Although an annual ophthalmologic examination currently is recommended as the most efficient method to screen for optic pathway gliomas, this method is often unreliable and inaccurate in young children. Magnetic resonance imaging remains the most sensitive test; however, cost and the need for sedation or general anaesthetic preclude its use as a routine screening test. We previously have recommended visual-evoked potentials (VEPs) as a possible alternative or aid in the assessment of optic pathway gliomas in this group of patients. The aims of this study were to ascertain the sensitivity of VEPs in detecting optic pathway gliomas and to determine whether VEPs are useful in monitoring the progress of optic pathway gliomas. This study provides further evidence of the sensitivity of VEPs in detecting optic pathway gliomas. In addition, VEPs may provide additional useful information concerning the progression of optic pathway gliomas once they have been detected.

    Title Clinical Rhabdomyolysis.
    Date September 2000
    Journal Journal of Paediatrics and Child Health
    Excerpt

    Although rhabdomyolysis is an uncommon disorder, especially in children, it can present as a severe, life-threatening event. Paediatricians need to be aware of this important, probably underdiagnosed disorder in order to implement appropriate early treatment. In this report, seven children with rhabdomyolysis of both forms, endogenous and exogenous, are presented. Despite comprehensive 'up-to-date' investigations being performed on paediatric patients with endogenous (often recurrent) rhabdomyolysis, the majority of these patients' underlying disorders will remain undiagnosed. Overall, these patients usually have a very good prognosis even if repeated, severe life-threatening episodes occur. It is recommended that a regimen of early therapy with fluids and sodium bicarbonate be instituted in all patients with rhabdomyolysis.

    Title Local Supplementation of Ketoprofen Reduces the Incidence of Low Back Pain After Lumbar Epidural Anesthesia.
    Date December 1999
    Journal Changgeng Yi Xue Za Zhi / Changgeng Ji Nian Yi Yuan = Chang Gung Medical Journal / Chang Gung Memorial Hospital
    Excerpt

    Backache is a common postoperative complaint after lumbar epidural anesthesia. Our study was aimed to compare the effect of the local addition of ketoprofen on the incidence of postepidural backache after nonobstetric surgery.

    Title Drug Therapy in Juvenile Dermatomyositis: Follow-up Study.
    Date June 1998
    Journal Journal of Child Neurology
    Excerpt

    A series of 33 patients with juvenile dermatomyositis was reviewed in terms of their prognosis in relation to their drug therapy. This retrospective study was intended to help clarify the use of various therapies in this rare, heterogeneous disease from our hospital's experience in the last 24 years. The results confirmed that oral corticosteroids should remain the undisputed first line of treatment. For more refractory, chronic patients, the results suggest that azathioprine should be the favored drug of first choice (in addition to corticosteroids). There may be a role for cyclosporine as a "rescue" treatment, but this needs to be further defined.

    Title Anesthetic Management of a Parturient Implanted with a Permanent Pacemaker--a Case Report.
    Date September 1997
    Journal Acta Anaesthesiologica Sinica
    Excerpt

    At the present time, a patient with a pacemaker who undergoes an anesthesia for a surgical procedure is very common, but a parturient with a permanent pacemaker scheduled for elective cesarean section (c/s) is very rare. Complete heart block in pregnancy is not a common event and it may be congenital or acquired, particularly secondary to cardiac surgery. In normal women, the heart rate is physiologically increased commensurate with need, whereas in a parturient with installment of an implanted fixed rate pacemaker of VVI mode her heart rate cannot accelerate to cope with increased physiological demand because of the fixed pacing rate. For cesarean section, because of destabilized cardiovascular adaptation, an anesthesiologist must focus on detecting the early signs of compromised cardiac output in order to avoid maternal as well as fetal distress. He may run considerable risk to administer general or regional anesthesia to this kind of parturient. We present a case report in managing the parturient installed a with non-rate response type pacemaker undergoing C/S under epidural anesthesia.

    Title Neonatal Status in Cesarean Section Under Epidural Anesthesia with Supplementary Oxygen.
    Date April 1994
    Journal Ma Zui Xue Za Zhi = Anaesthesiologica Sinica
    Excerpt

    Simple O2 mask has been used in patient under regional anesthesia for increasing the FiO2, especially in the aged and the pregnant. The relationships between maternal FiO2 and umbilical arterial (UA) and venous (UV) PO2, PCO2, pH, and neonatal Apgar score were studied in 45 patients receiving Cesarean section under epidural anesthesia. 2% xylocaine 18-20 ml with adrenaline 1:200,000 was used to attain the sensory level of T-4. Patients were allocated randomly into three groups. Group I, acting as a control group, breath only room air during the course of anesthesia. Group II was breathing through a simple face mask with an oxygen inflow of 6L/min. Group III was breathing oxygen with a flow rate of 10L/min through a simple face mask. UA and UV blood samples were collected separately at the time of delivery for blood gas analysis. The 1-min, and 5-min Apgar scores were recorded also. Mean values of the UA blood samples for the 3 groups (Gp. I, Gp. II and Gp. III respectively) were: PO2--16.50 mmHg, 20.20 mmHg and 19.50 mmHg; PCO2--49.20 mmHg, 48.10 mmHg and 50.3 mmHg; pH--7.31, 7.30 and 7.30. Mean values of the UV blood samples for the 3 groups (Gp. I, Gp. II and Gp. III respectively) were: PO2--28.6 mmHg, 36.9 mmHg and 36.5 mmHg; PCO2--38.20 mmHg, 38.80 mmHg and 40.40 mmHg; pH--7.36, 7.36 and 7.34. There was a significant increase in UA and UV PO2 when using a simple O2 mask (p < 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)

    Title [the Anesthetic Management of Q-t Prolonged Syndrome].
    Date July 1992
    Journal Ma Zui Xue Za Zhi = Anaesthesiologica Sinica
    Title The Evaluation of Subarachnoid Administration of Fentanyl for Surgery and Postoperative Analgesia in Patients Undergoing Cesarean Section.
    Date July 1991
    Journal Ma Zui Xue Za Zhi = Anaesthesiologica Sinica
    Excerpt

    The use of spinal opioids for postoperative analgesia has gained popularity in recent years. In this study, subarachnoid fentanyl 20 micrograms was evaluated to determine its efficacy for postoperative analgesia, its possible side effects and its effects on the newborn. Sixty ASA class I or II at-term parturients undergoing elective cesarean section were randomly divided into two groups. In one group fentanyl 20 micrograms (0.4 ml) with 0.5% heavy marcaine 2.0 ml was given intrathecally and in the other group only 0.5% heavy marcaine 2.0 ml with CSF 0.4 ml was given intrathecally. The average time for patients in the fentanyl group to demand the first dose of narcotic for pain was 6.8 +/- 3.2 h and in the control group it was 3.9 +/- 1.1 h. The incidences of postoperative nausea and vomiting were higher in the fentanyl group than in the control group. Pruritus was only found in the fentanyl group and amounted to 50%. Early or late respiratory depression was not found in the fentanyl group. During operation, all patients were wakeful and alert. Neonatal condition as determined by 1-min and 5-min Apgar score was satisfactory and showed no significant difference in both groups. Examination on neurobehavior and reflexes done at the baby room showed no abnormality in both groups.

    Title Ilioinguinal Nerve Block with 0.375% Marcaine for Postoperative Pain Relief in Cesarean Section.
    Date March 1991
    Journal Ma Zui Xue Za Zhi = Anaesthesiologica Sinica
    Excerpt

    Bilateral ilioinguinal nerve block with local anesthetic drugs to treat postoperative pain in lower segment cesarean section (Pfannestiel incision) under general anesthesia had show profound effective by P. Bunting and I. McConachie. We used the same method but with lower concentration 0.375% marcaine 10 ml to each side in 12 patients. We compare the pain score and the requirement for pethidine intramuscular injection to 12 patients as control group. Pain score were less in the block patients within 8h after surgery, and total amount of pethidine given was 700 mg. In control group, the pain score were higher at the first 8 h than nerve block group during the study (p less than 0.05), and the total amount of pethidine requirement was 1250 mg. There were no observed adverse effect during the study.

    Title [ilioinguinal Nerve Blockade with or Without Epinephrine for Analgesia After Caesarean Section].
    Date March 1991
    Journal Ma Zui Xue Za Zhi = Anaesthesiologica Sinica
    Excerpt

    The analgesic effect of bilateral ilioinguinal nerve blockade with or without epinephrine after caesarean section with Pfannenstiel incision was investigated in 36 ASA class 1-2 parturients. They were randomly classified into 3 groups of 12 each. Before the patients were extubated from standard general anesthesia, bilateral ilioinguinal nerve blockade was performed. Group A patients were the control group. Group B patients received 0.375% plain marcaine 10 mL to each side. Group C patients received 0.375% plain marcaine with 1:200000 epinephrine 10 mL to each side. The pain scores and requirement for post operative analgesia of group B and C were compared with the control group A. The pain scores of group B and C were decreased 30% and 37% respectively during the first eight hours after operation. However, group C patients had lower pain scores during the first day after operation. There was an increased time from anesthesia to the first injection of pethidine in group B and C when compared with group A (4.57 +/- 2.94, 4.38 +/- 2.72 and 1.8 +/- 0.9 hr, respectively). However, no significant difference between group B and C. The total pethidine requirement were decreased in group B and C although the mean pethidine dose was not statistically significant. The results suggest that bilateral ilioinguinal nerve blockade improve the quality of postoperative analgesia. The adding epinephrine can prolong the duration of nerve blockade.

    Title Establishment and Characterization of a New Human Cell Line Derived from Ovarian Clear Cell Carcinoma.
    Date July 1990
    Journal Gynecologic Oncology
    Excerpt

    A new cell line, designated OCC1, was established from the ascitic fluid of a patient with a clear cell carcinoma of the ovary. The cell line grew well without interruption for over 12 months and over 80 passages. The doubling times of OCC1 were 36 and 38 hr at the 10th and 40th passages, respectively. Chromosomal analysis of the cell line showed hypertriploidy with modal number around 70-77. Several structural chromosomal abnormalities were consistently found. Electron microscopy revealed that OCC1 produced a basement membrane-like structure in vitro. Histological evaluation of xenografts from OCC1 in the 33th passage implanted and grown in nude (athymic) mice revealed a morphology identical to that of the original tumor.

    Title [anesthesia for Hepatoma Resection].
    Date July 1990
    Journal Ma Zui Xue Za Zhi = Anaesthesiologica Sinica
    Excerpt

    Hepatocellular carcinoma is one of the leading cancers in Taiwan and is responsible for 20% of cancer deaths. Since long-term survival of hepatocellular carcinoma patients cannot be expected with any treatment other than surgery, the therapeutic value of hepatic resection has become more important than ever before. In Keelung Chang Gung Memorial Hospital, twenty-one patients with hepatoma received hepatic resection during the period of August 1985 to July 1989 were reviewed. These patients were induced for anesthesia with thiopental, succinylcholine and fentanyl; maintenance of anesthesia with isoflurane, N2O and O2. Four of them have abnormal coagulopathy preoperatively. Common intraoperative problems were metabolic acidosis and hypotension. Estimated blood loss showed great variety among these patients. Eighteen patients needed respiratory support and intensive care postoperatively. One patient was noted to have pulmonary edema. Otherwise, the overall procedure was smooth and satisfactory. In addition, there is no significantly difference in liver function test after a month of hepatoma resection. All the patients survived except one who died within two months after surgery.

    Title Pole Bias Scanner Circuit for Quadrupole Mass Spectrometers of Early Design.
    Date August 1985
    Journal Analytical Chemistry
    Title Clomid Induced Combined Extrauterine and Intrauterine Pregnancy--a Case Report.
    Date November 1983
    Journal The Medical Journal of Malaysia
    Title Adrenergic Neuron Blocking Action of Dehydroemetine.
    Date January 1971
    Journal The Journal of Pharmacy and Pharmacology
    Title Assay of Acetylcholine Using Toad Rectus Abdominis Muscle in the Presence of Hemicholinium-like Substances.
    Date January 1970
    Journal The Journal of Pharmacy and Pharmacology
    Title Noradrenaline in Infusion Solutions.
    Date October 1969
    Journal Singapore Medical Journal
    Title Further Observations on the Behaviour of Noradrenaline in Physiological Saline Infusions.
    Date August 1969
    Journal The Medical Journal of Malaya
    Title Interobserver Agreement in the Interpretation of Eeg Patterns in Critically Ill Adults.
    Date
    Journal Journal of Clinical Neurophysiology : Official Publication of the American Electroencephalographic Society
    Excerpt

    The significance of rhythmic and periodic EEG patterns in critically ill patients is unclear. A universal terminology is needed to facilitate study of these patterns, and consistent observer agreement should be demonstrated in its use. The authors evaluated inter- and intraobserver agreement using the standardized terminology (Hirsch et al., J Clin Neurophysiol 2005;22:128-135) recently proposed by the American Clinical Neurophysiology Society. Trained electroencephalographers viewed a series of 10-second EEG samples from critically ill adults (phase I), a set of >/=20-minute EEGs from the same patient cohort (phase II), and then reevaluated the first sample set (phase III). The readers used the proposed terminology to "score" each EEG. For each possible term, interobserver agreement (phases I and II) and intraobserver agreement (phase III) were calculated using pairwise kappa (kappa) values. Moderate agreement beyond chance was seen for the presence/absence of rhythmic or periodic patterns and for localization of these patterns. Agreement for other terms was slight to fair. Inter- and intraobserver agreement were consistently lower for optional terms than mandatory terms. Even when standardized terminology is used, the description of rhythmic and periodic EEG patterns varies significantly. Further refinement of the proposed terminology is required to improve inter- and intraobserver agreement.

    Title Who Coined the Term "status Gelasticus"? We Did.
    Date
    Journal Neuropsychiatric Disease and Treatment
    Title Dissolving Oral Clonazepam Wafers in the Acute Treatment of Prolonged Seizures.
    Date
    Journal Journal of Child Neurology
    Excerpt

    Klonopin (clonazepam; Genentech Inc, South San Francisco, California) oral wafers are benzodiazepines with anticonvulsive and anxiolytic properties. Our institution has been prescribing clonazepam wafers for acute treatment of prolonged seizures for years. Patients' size determined dosing at 0.25, 0.5, 1, or 2 mg wafers. We proceeded to obtain evidence for efficacy. Hospital Institutional Review Board approval was obtained for anonymous patient survey. All children who had been prescribed clonazepam wafers over a 6-year period at our institution were mailed detailed questionnaires. Three hundred eighty-one questionnaires were mailed with 88 replies but only 56 with meaningful data. Average age was 12.1 years. There were 31 males. Efficacy was defined as stopping seizure within 10 minutes, >50% of the time. Thirty-eight of the 56 (68%) patients met this criterion. From these 38 patients, 19 (50%) had seizures stop within 1 minute. Overall results were comparable to Diastat (rectal diazepam; Valeant Pharmaceuticals International, Aliso Viejo, California). Clonazepam wafers are an effective acute therapy for prolonged seizures.

    Title Orbitozygomatic Resection for Hypothalamic Hamartoma and Epilepsy: Patient Selection and Outcome.
    Date
    Journal Child's Nervous System : Chns : Official Journal of the International Society for Pediatric Neurosurgery
    Excerpt

    This study aims to examine the outcomes of ten patients after orbitozygomatic (OZ) pterional surgery in cases of refractory epilepsy caused by hypothalamic hamartomas (HH).

    Title Surgical Resection of Hypothalamic Hamartomas for Severe Behavioral Symptoms.
    Date
    Journal Epilepsy & Behavior : E&b
    Excerpt

    Hypothalamic hamartomas (HHs) are associated with treatment-resistant epilepsy. Many patients also experience severe and sometimes disabling psychiatric problems. The most common behavioral symptoms consist of paroxysms of uncontrolled anger related to poor frustration tolerance. These can include violence, resulting in disrupted family or school relationships, and legal consequences including incarceration. In a large cohort of patients undergoing surgical resection of HHs for refractory epilepsy, 88% of families described an improvement in overall behavioral functioning [1]. Here, we describe four patients (three males, mean age=11.9 years) who underwent surgical resection of HHs largely for behavioral indications. Three patients had relatively well controlled seizures, and one had no history of epilepsy. All patients had striking improvement in their psychiatric comorbidity. HH resection can result in significant improvement in behavioral functioning, even in patients with relatively infrequent seizures. Further investigation under approved human research protocols is warranted.


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