Neurologist (brain, nervous system)
37 years of experience

Accepting new patients
Central LA
Children's Hospital of Los Angeles
4650 W Sunset Blvd
Los Angeles, CA 90027
323-669-2471
Locations and availability (2)

Education ?

Medical School Score Rankings
University of California at San Francisco (1973)
  • Currently 4 of 4 apples
Top 25%

Awards & Distinctions ?

Awards  
One of America's Leading Experts on:
Seizures
Castle Connolly America's Top Doctors® (2002 - 2008, 2010 - 2014)
Castle Connolly's Top Doctors™ (2012 - 2013)
Appointments
University of Southern California School of Medicine
Professor of Clinical Neurology
Associations
American Board of Pediatrics
American Board of Psychiatry and Neurology
Epilepsy Foundation

Affiliations ?

Dr. Mitchell is affiliated with 4 hospitals.

Hospital Affilations

Score

Rankings

  • Children's Hospital of Los Angeles *
    4650 W Sunset Blvd, Los Angeles, CA 90027
    • Currently 1 of 4 crosses
  • LAC-USC Medical Staff
  • University Childrens Medical Group
  • UCLA Medical Center
  • * This information was reported to Vitals by the doctor or doctor's office.

    Publications & Research

    Dr. Mitchell has contributed to 92 publications.
    Title Possible Mitochondrial Dysfunction and Its Association with Antiretroviral Therapy Use in Children Perinatally Infected with Hiv.
    Date July 2010
    Journal The Journal of Infectious Diseases
    Excerpt

    Mitochondrial dysfunction has been associated with both human immunodeficiency virus (HIV) infection and exposure to antiretroviral therapy. Mitochondrial dysfunction has not been widely studied in HIV-infected children. We estimated the incidence of clinically defined mitochondrial dysfunction among children with perinatal HIV infection.

    Title Neurodevelopmental and Epilepsy Outcomes in a North American Cohort of Patients with Infantile Spasms.
    Date June 2010
    Journal Journal of Child Neurology
    Excerpt

    Studies have suggested disparate variables affecting long-term outcomes in patients with infantile spasms. Using a retrospective chart review, the authors identified 109 patients who had follow-up data for at least 1 year since the onset of spasms. Patient and treatment variables were recorded, in addition to neurodevelopmental and seizure outcomes. Etiology was strongly associated with motor and cognitive status but not with long-term seizure control. Lag time to initiation of treatment was not predictive of any outcome, nor for need to use a second agent to resolve spasms, even when controlling for etiology. However, patients who responded to the first medication achieved superior seizure and cognitive outcomes. The delayed impact of individual medications could not be analyzed because many patients received multiple agents. While etiology and response to first medication predict better outcomes, the majority of patients with infantile spasms continue to have epilepsy with long-term motor and cognitive disabilities.

    Title Long-term Follow-up of Neuroblastoma-associated Opsoclonus-myoclonus-ataxia Syndrome.
    Date March 2010
    Journal Neuropediatrics
    Excerpt

    The aim of this study is to describe the long-term neurological, neuropsychological and neuroradiological sequelae and to determine prognostic factors for neurological outcome in children with neuroblastoma-associated opsoclonus-myoclonus-ataxia (OMA) syndrome.

    Title Impact of Medications Prescribed for Treatment of Attention-deficit Hyperactivity Disorder on Physical Growth in Children and Adolescents with Hiv.
    Date January 2010
    Journal Journal of Developmental and Behavioral Pediatrics : Jdbp
    Excerpt

    To examine the relationships between physical growth and medications prescribed for symptoms of attention-deficit hyperactivity disorder in children with HIV.

    Title Cortical Dysplasia with Prominent Rosenthal Fiber Formation in a Case of Intractable Pediatric Epilepsy.
    Date August 2009
    Journal Human Pathology
    Excerpt

    We report a case of a 5-year-old boy with intractable epilepsy who underwent therapeutic corticectomy. Histopathologic findings within the resection specimen included severe cortical dysplasia associated with abundant subpial and intraparenchymal Rosenthal fibers in a large right frontal lesion that merged into the basal ganglia. Rosenthal fiber proliferation may represent a reactive process, are frequent in pilocytic astrocytomas, and are a defining feature of Alexander disease. There was no evidence of neoplasm or leukodystrophy in this case. Genetic analysis of the specimen showed a few previously reported polymorphisms but no mutation in the GFAP gene. This case is unique among several hundred cortical resection specimens that we have studied, including numerous cases of severe cortical dysplasia.

    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 Insights on Chronic-relapsing Opsoclonus-myoclonus from a Pilot Study of Mycophenolate Mofetil.
    Date May 2009
    Journal Journal of Child Neurology
    Excerpt

    Opsoclonus-myoclonus syndrome is characterized by abnormal lymphocyte trafficking into brain. The authors hypothesized that mycophenolate mofetil, a lymphocyte proliferation inhibitor, might be therapeutic. The cerebrospinal fluid and blood immunophenotypes of 15 children with predominantly chronic-relapsing opsoclonus-myoclonus syndrome were compared before and after treatment by flow cytometry. Mycophenolate mofetil reduced the cerebrospinal fluid expansion of HLA-DR+ activated T cells (-40%); the frequency of other T-cell or natural killer cell subsets remained unchanged, but cerebrospinal fluid B cells increased significantly. Adrenocorticotropic hormone dose was lowered by 64% over an average of 1.5 years, yet 73% eventually relapsed despite therapeutic drug levels. Prior treatment with rituximab prevented relapse-associated increase in cerebrospinal fluid B cells, without hindering mycophenolate mofetil-induced reduction in T-cell activation. These data demonstrate resistant immunologic problems in chronic-relapsing opsoclonus-myoclonus syndrome. Mycophenolate mofetil did not prevent relapse. The novel effect of mycophenolate mofetil on chronically activated T cells may contribute to its efficacy in T-cell mediated neurological disorders.

    Title Reviews of Infant Botulism at Childrens Hospital Los Angeles.
    Date October 2008
    Journal Journal of Child Neurology
    Title Infant Botulism: 20 Years' Experience at a Single Institution.
    Date March 2008
    Journal Journal of Child Neurology
    Excerpt

    Admissions from January 1, 1985, to December 31, 2005, with the diagnosis of infant botulism were reviewed to describe the clinical presentation, course, outcome, and changes related to the availability of botulism immune globulin treatment. Botulism diagnoses were confirmed by the finding of toxin or Clostridium botulinum organisms in stool samples (type A, 14; type B, 25; type not noted, 5). Twenty-four patients were admitted from 1985-1994 and 20 from 1995-2004. Infants in the two decades were similar in age, demographics, and presenting features. Ventilator support was needed in 13 of 24 (54%) in 1985-1994 and in 15 of 20 (75%) in 1995-2005; 43 required nasogastric feeding. Seventeen patients were treated with botulism immune globulin. Length of stay was shorter in infants treated with botulism immune globulin (13.5 vs 23 days, P = .009), with a trend toward reduced need for nasogastric feeding and in shorter duration of tracheal intubation. All patients recovered fully. Even with the availability of botulism immune globulin, meticulous supportive care remained essential for recovery.

    Title Major Adverse Events Associated with Treatment of Infantile Spasms.
    Date March 2008
    Journal Journal of Child Neurology
    Excerpt

    Few studies have focused on tolerability and adverse events associated with natural adrenocorticotropic hormone injections for treatment of infantile spasms. Using a retrospective chart review of 130 patients, the authors compare major adverse events, weight and blood pressure changes, and unplanned medication changes associated with adrenocorticotropic hormone (ACTH) injections versus other antiepileptic drugs. Children treated with adrenocorticotropic hormone injections experienced significant short-term weight gain and blood pressure elevations, which were readily reversible with weaning off the drug. Twenty-three percent of patients treated with adrenocorticotropic hormone (14 of 60) and 17% of patients treated with other antiepileptic drugs (11 of 65) experienced a major adverse event during treatment. Few patients overall required a change in medication due to intolerable side effects. Despite early changes in weight and blood pressure, short courses of high-dose natural adrenocorticotropic hormone are generally well tolerated with no increased major adverse events when compared to antiepileptic drugs in the treatment of infantile spasms.

    Title Iatrogenic Botulism in a Child with Spastic Quadriparesis.
    Date December 2007
    Journal Journal of Child Neurology
    Excerpt

    Botulinum toxins are potent neurotoxins used in a variety of neurological disorders. Few pediatric reports have been published to date regarding the potential hazards of therapeutic use of botulinum toxins. We describe the case of a 10-year-old boy who developed systemic weakness following treatment of spasticity with botulinum toxin type B. The patient developed iatrogenic botulism with ptosis, facial diplegia, neck flexor and extensor weakness, and profound hypopharyngeal laxity with respiratory compromise from which he eventually recovered. Clinicians should be mindful of the risk for systemic botulism when using local injections of the neurotoxin.

    Title Pharmacokinetics of Levetiracetam in Infants and Young Children with Epilepsy.
    Date July 2007
    Journal Epilepsia
    Excerpt

    PURPOSE: To assess the single-dose pharmacokinetics of levetiracetam and its major metabolite ucb L057 in infants and young children with epilepsy. METHODS: Eligible patients with a stable regimen of antiepileptic medications received a single oral dose of levetiracetam 20 mg/kg administered as a 10% oral solution followed by a 24-hour pharmacokinetic evaluation. RESULTS: Thirteen subjects (age 2.3-46.2 months) enrolled and received levetiracetam; 12 provided evaluable pharmacokinetic data. Levetiracetam was rapidly absorbed and reached peak plasma concentration (t(max)) 1.4 +/- 0.9 hours after dosing. The mean half-life (t(1/2)) of levetiracetam was 5.3 +/- 1.3 hours, and the apparent clearance was 1.46 +/- 0.42 mL/min/kg. Graphical differences were observed among three age subgroups (1 to <6 months, 6 to <24 months, and 24 to <48 months); however, statistical analysis was limited due to each subgroup's small sample size. No significant gender differences were detected. Treatment-emergent adverse events were seen in three patients (23.1%) but were not considered to be related to levetiracetam. CONCLUSIONS: The mean t(1/2) of levetiracetam was shorter and its apparent clearance was more rapid for infants and young children than that previously reported for adults. When determining dosage, age-dependent drug clearance should be considered; these findings suggest that a larger dose of levetiracetam (corrected for body weight) needs to be considered for infants and young children with epilepsy than that given to adults with epilepsy. A single dose of levetiracetam was well tolerated in this study population.

    Title Mood and Behavioral Dysfunction with Opsoclonus-myoclonus Ataxia.
    Date September 2006
    Journal The Journal of Neuropsychiatry and Clinical Neurosciences
    Excerpt

    Opsoclonus-myoclonus ataxia syndrome is a paraneoplastic syndrome of cerebellar damage associated with neuroblastoma. The authors assessed psychiatric symptoms of opsoclonus-myoclonus ataxia syndrome in 17 children, who were 16 months to 12(1/2) years of age. Psychiatric symptoms examined included disruptive behavior, affective dysregulation, irritability, impulsivity, cognitive impairment, and poor attention.

    Title Double-blind Placebo-controlled Trial of Adjunctive Levetiracetam in Pediatric Partial Seizures.
    Date July 2006
    Journal Neurology
    Excerpt

    OBJECTIVE: To evaluate the efficacy and tolerability of levetiracetam (LEV) as adjunctive therapy in children (4 to 16 years) with treatment-resistant partial-onset seizures. METHODS: This multicenter, randomized, placebo-controlled trial consisted of an 8-week baseline period followed by a 14-week double-blind treatment period. During the treatment period, patients received either placebo or LEV add-on therapy and were up-titrated to a target dose of 60 mg/kg/day. RESULTS: One hundred ninety-eight patients (intent-to-treat population) provided evaluable data. The reduction in partial-onset seizure frequency per week for LEV adjunctive therapy over placebo adjunctive therapy was significant (26.8%; p = 0.0002; 95% CI 14.0% to 37.6%). A 50% or greater reduction of partial seizure frequency per week was attained in 44.6% of the LEV group (45/101 patients), compared with 19.6% (19/97 patients) receiving placebo (p = 0.0002). Seven (6.9%) LEV-treated patients were seizure-free during the entire double-blind treatment period, compared with one (1.0%) placebo-treated patient. One or more adverse events were reported by 88.1% of LEV-treated patients and 91.8% of placebo patients. The most common treatment-emergent adverse events were somnolence, accidental injury, vomiting, anorexia, hostility, nervousness, rhinitis, cough, and pharyngitis. A similar number of patients in each group required a dose reduction or withdrew from the study as a result of an adverse event. CONCLUSION: Levetiracetam adjunctive therapy administered at 60 mg/kg/day is efficacious and well tolerated in children with treatment-resistant partial seizures.

    Title Longitudinal Neurodevelopmental Evaluation of Children with Opsoclonus-ataxia.
    Date December 2005
    Journal Pediatrics
    Excerpt

    OBJECTIVE: We previously reported on children with opsoclonus-ataxia and found pervasive neurodevelopmental deficits, years after onset, without a clear relationship to treatment modality or timing of treatment. A significant negative correlation of functional status with age at testing raised a question of whether opsoclonus-ataxia is a progressive encephalopathy. We attempted to answer this question with serial testing. In addition, we examined the relationship between clinical course and developmental outcome. METHODS: Thirteen of 17 children with opsoclonus-ataxia, all with neuroblastoma, who were previously reported were reevaluated a second time 2 to 4 years after the initial assessment. One subject who lived out of state was partially reevaluated and is included. Five new subjects (2 with neuroblastoma and 3 without) were also enrolled. Each was evaluated twice at a minimum interval of 1 year between sessions. Intercurrent medical course was recorded, emphasizing medication and relapse history. Cognitive, adaptive behavior, academic, speech and language, and motor abilities were assessed. RESULTS: For the group as a whole, overall standardized, age-adjusted cognitive scores improved. Generally, younger subjects' cognitive and adaptive behavior scores improved more than older subjects. Although all subjects had gains in speech, language, and motor function, some progressed at a slow pace, and in some instances, standard scores dropped. There was a striking influence of clinical course. Although initial presentation was severe and all subjects required high doses of corticosteroids or corticotropin, 5 had a monophasic course and were able to be weaned from treatment without relapses. Fourteen had multiple relapses over the years, generally with reduction of medication or intercurrent illnesses. Of the 5 children with monophasic course, 4 are currently functioning in the average range with a full-scale IQ of > or =90 and age-appropriate academic and adaptive skills. CONCLUSIONS: The results continue to raise concern that opsoclonus-ataxia is sometimes a progressive encephalopathy. A minority of children with opsoclonus-ataxia have a monophasic course. Despite initial severity of symptoms, these children may have a more benign prognosis. For the majority of children with opsoclonus-ataxia, the course includes multiple relapses and requires prolonged treatment. Developmental sequelae are significant in these children with chronic course.

    Title Catastrophic Presentation of Infant Botulism May Obscure or Delay Diagnosis.
    Date December 2005
    Journal Pediatrics
    Excerpt

    Three infants with infant botulism are presented to illustrate how atypical, early, and severe features may obscure or delay diagnosis. Two boys aged 6 weeks and 20 days, respectively, presented with rapid deterioration after brief periods of poor feeding, one with an apparent life-threatening event at home and the other with a full cardiopulmonary arrest. Initial abnormal laboratory findings of coagulopathy suggested sepsis in the first infant. In the second infant, severe acidosis and hypoglycemia suggested an underlying metabolic disorder. A third infant, aged 1 month, was hospitalized originally with an admitting diagnosis of "pharyngitis" resulting from his inability to take adequate feedings. He received intravenous fluids and antibiotics. One week later he suffered a respiratory arrest. Laboratory findings of severe hyponatremia and acidosis at the time of his arrest suggested a metabolic etiology. Even retrospectively, none of these infants had the typical initial complaint of constipation, and none were noted to have ptosis or facial weakness before catastrophic collapse. However, in each case, the parent had initially brought the child to the physician for "poor feeding" or "poor suck," which was not recognized by medical personnel as a result of bulbar weakness. Ultimately, all 3 infants were found to have infant botulism. All 3 had received antibiotics before catastrophic collapse, possibly contributing to the rapidity of the deterioration. Each recovered, although the delay in diagnosis made them ineligible for treatment with botulism immunoglobulin.

    Title Opsoclonus Myoclonus Syndrome in Neuroblastoma a Report from a Workshop on the Dancing Eyes Syndrome at the Advances in Neuroblastoma Meeting in Genoa, Italy, 2004.
    Date November 2005
    Journal Cancer Letters
    Excerpt

    Opsoclonus-myoclonus syndrome (OMS) is a rare neurologic syndrome that, in children, associates with neuroblastoma in more than half of the cases. The etiology of this condition is thought to be immune mediated, but, though immunosuppressive therapies may ameliorate the acute symptoms, no effective treatment to prevent the common neuropsychologic sequelae has been established. This paper summarizes the results obtained at the 2004 Advances in Neuroblastoma Research meeting, providing status of the art information on immune pathogenesis, clinical features, acute and chronic neurologic manifestations, current and novel therapeutic approaches. It is emphasized that, due to the rarity of OMS in general and neuroblastoma-associated OMS in particular, international collaborations are needed to better define the pathogenesis and therapy of this disease, propose common evaluation criteria and identify new treatment modalities.

    Title Mitochondrial Disorders: a Potentially Under-recognized Etiology of Infantile Spasms.
    Date January 2003
    Journal Journal of Child Neurology
    Excerpt

    Infantile spasms represent an age-dependent response of the immature brain to a wide variety of insults. An unselected group of children with infantile spasms were reviewed to determine etiology; a metabolic work-up was undertaken if the etiology was unclear from history and examination (cryptogenic). Of the 56 infants, 34 had a recognizable etiology (symptomatic), 1 had normal development (idiopathic), and 21 had cryptogenic infantile spasms. Among the latter, results of plasma lactate and pyruvate or urine organic acids were available in 17. In 2 infants (monozygotic twins), mitochondrial DNA testing revealed the relatively common A3243G mitochondrial mutation. In these twins and 11 of the remaining 15, body fluid metabolite testing suggested possible defective energy metabolism. Our twins and previous reports suggest that mitochondrial disorders should be considered in the differential diagnosis of infantile spasms. Among our cases remaining cryptogenic, signs of abnormal energy metabolism were prevalent, suggesting that metabolic derangements may be common causes or secondary consequences of infantile spasms.

    Title Vigabatrin for Infantile Spasms.
    Date January 2003
    Journal Pediatric Neurology
    Excerpt

    We reviewed 20 infants receiving vigabatrin for infantile spasms. Patients were not enrolled in a formal study. All families obtained the medication abroad. Age at initiation of vigabatrin ranged from 1 to 48 months; nine infants had received prior treatment with various antiepileptic medications. Patients were begun on the lowest practical dose of 125-250 mg/day, with gradual daily increments to a target of 100 mg/kg/day, but maintained at the lowest effective dosage. Video electroencephalogram was obtained to document resolution of spasms and hypsarrhythmia. Of 20 infants, 12 responded with cessation of spasms and resolution of hypsarrhythmia, at doses of 25-135 mg/kg/day (median = 58 mg/kg/day). Partial responses were observed in six patients, whereas two had no response at 111 and 125 mg/kg/day. Additional new seizure types developed in three infants after initial response to vigabatrin. Increasing the vigabatrin did not have any clinical benefit. Vigabatrin is an effective, well-tolerated treatment for infantile spasms. The response is dose-independent, suggesting that starting at a low dose and gradually increasing, rather than beginning with an arbitrary 100 mg/kg/day dose is advantageous.

    Title Status Epilepticus and Acute Serial Seizures in Children.
    Date October 2002
    Journal Journal of Child Neurology
    Excerpt

    Status epilepticus is defined as a seizure that persists for a sufficient length of time or is repeated frequently enough to produce a fixed and enduring epileptic condition of 30 minutes or longer. Status epilepticus is a life-threatening condition that often occurs in children. The degree of mortality and neurologic morbidity, as well as the risk for recurrence, is highly dependent on the etiology and duration of the seizures. Although much has been written about pediatric status epilepticus, many issues remain unresolved. A better understanding of the different types of seizures and their etiologies may help in the prevention and treatment of status epilepticus. The vast extent of status epilepticus in both children and adults mandates that new options for prevention and treatment be given a close scrutiny and high priority. This article will review the most current information on convulsive and nonconvulsive status epilepticus, including the potential for neurologic damage, changes in magnetic resonance imaging after status epilepticus, risk for recurrence, and current treatment options available for treating status epilepticus in children.

    Title Opsoclonus-ataxia Caused by Childhood Neuroblastoma: Developmental and Neurologic Sequelae.
    Date February 2002
    Journal Pediatrics
    Excerpt

    OBJECTIVE: Opsoclonus-ataxia, also called "dancing eye syndrome," is a serious neurologic condition that is often a paraneoplastic manifestation of occult neuroblastoma in early childhood. Despite resection of tumor and immunosuppressive therapy, outcome generally includes significant developmental and behavioral sequelae. There is controversy about how treatment alters outcome. The goals of this study were to understand the ongoing neurologic and developmental deficits of children who are treated for opsoclonus-ataxia with associated neuroblastoma; to relate treatment history to outcome; and to quantify objectively the acute changes in motor function, speech, mood, and behavior related to intravenous immunoglobulin (IVIg) treatment. METHODS: Patients were children with opsoclonus-ataxia caused by neuroblastoma, regardless of interval since diagnosis. Records were reviewed, and children underwent comprehensive evaluations, including neurologic examination and tests of cognitive and adaptive function, speech and language, and fine and gross motor abilities. Psychiatric interview and questionnaires were used to assess current and previous behavior. In 6 children, a videotaped standardized examination of eye movements was performed. Additional examinations were performed immediately before and 2 to 3 days after treatment with IVIg in 5 children. RESULTS: Seventeen children, ages 1.75 to 12.62 years, were examined. All had a stage I or II neuroblastoma resected 3 months to 11 years previously. None received any other treatment for the tumor. All but 1 had received at least 1 year of either oral corticosteroids or corticotropin (ACTH); 12 had received 1 or more courses of IVIg, 2 g/kg. Three had received other immunosuppressive treatment, including cyclophosphamide. Cognitive development and adaptive behavior were delayed or abnormal in nearly all children. Expressive language was more impaired than receptive language. Speech was impaired, including both intelligibility and overall output. Fine and gross motor abilities were impaired. Increased age was strikingly associated with lower scores in all areas. Behavioral problems early in the course included severe irritability and inconsolability in all; later, oppositional behavior and sleep disorders were reported. Opsoclonus abated in all, but abnormalities in pursuit eye movements were found in all 6 children cooperative with standardized examination. Outcome did not differ in children who were treated with ACTH versus oral steroids. Three children who had received cyclophosphamide fared poorly. Immediate versus delayed treatment was not associated with better outcome. IVIg improved both gross and fine motor and speech function acutely, but we could not confirm long-term benefit of IVIg. Total number of courses of IVIg was not associated with outcome. CONCLUSIONS: Opsoclonus-ataxia caused by neuroblastoma causes substantial developmental sequelae that are not adequately prevented by current treatment. The increased deficits in older children raise concern that this represents a progressive encephalopathy rather than a time-limited single insult. Although the study is cross-sectional and neither randomized nor blinded, we were unable to confirm a purported advantage of either ACTH over corticosteroids or of cyclophosphamide. A randomized study is needed but is difficult for this rare condition.

    Title Mitochondrial Dna Depletion, Near-fatal Metabolic Acidosis, and Liver Failure in an Hiv-infected Child Treated with Combination Antiretroviral Therapy.
    Date July 2001
    Journal The Journal of Pediatrics
    Excerpt

    A child with controlled human immunodeficiency virus infection presented with neurologic deterioration, lactic acidosis, and organic aciduria. Muscle biopsy revealed abnormal mitochondrial (mt) morphology, reduced mt enzyme activities, and mtDNA depletion. After adjustment of antiretroviral therapy to a regimen free of nucleoside analogs, marked improvement was seen in clinical status and mt abnormalities.

    Title Adherence to Treatment in Children with Epilepsy: Who Follows "doctor's Orders"?
    Date February 2001
    Journal Epilepsia
    Excerpt

    PURPOSE: The goal of the present study was to examine sociocultural, medical, family environment, and individual cognitive factors that predict adherence to treatment in children with epilepsy. METHODS: The study subjects (4-13 years old) were enrolled in a longitudinal seizure study at the first visit to the seizure clinic, attended at least 6 months, and had at least two appointments. Baseline predictors, which were obtained by interview, chart review, and psychometric testing, included sociocultural and family environment, seizure and previous treatment history, child behavior, cognitive functioning (IQ), and family stress. Four latent factors tapping these indicators of risk (acculturative risk, seizure severity, behavior problems, family environment) and two measured variables (IQ and life events) were hypothesized. Outcomes were visit adherence (proportion of scheduled appointments kept, plus proportion without unscheduled contacts), medication report (proportion of visits at which parent report of medication agreed with records), and medication levels (proportion of serum anticonvulsant levels within expected range for dosage). Two-step analytic procedure included confirmatory factor analysis to validate the hypothetical structure of the baseline risk indicators, followed by structural equation modeling to examine longitudinal relations between baseline risk and subsequent adherence outcomes. RESULTS: Significant prospective relationships included acculturative risk associated positively with visit adherence and medication levels, behavior problems associated negatively with visit adherence and medication levels, family environment associated negatively with medication report, life events associated positively with medication levels and visit adherence, and cognitive functioning (IQ) associated positively with medication levels. Seizure severity was not associated significantly with any adherence outcome. There also were no significant within-time associations between adherence outcomes. CONCLUSIONS: Contrary to clinical expectations, families at higher acculturative risk and with higher life events reported greater adherence. Seizure severity did not influence adherence. The three adherence measures were statistically independent of each other.

    Title Neurocysticercosis and Acquired Cerebral Toxoplasmosis in Children.
    Date February 2000
    Journal Seminars in Pediatric Neurology
    Excerpt

    Neurocysticercosis, prevalent wherever pigs are raised in the presence of poor sanitation, is the most common identifiable cause of new-onset epilepsy throughout the developing world. As immigration patterns have changed, children with neurocysticercosis are seen throughout the United States. Acute cysticercosis, the most common manifestation in children, reflects the host response to the dying parasite. Children typically present with seizures and have an excellent prognosis. Neuroimaging demonstrates a single ring or nodular enhancing lesion surrounded by edema. Short-term anticonvulsant therapy is indicated, but treatment with antiparasitic agents is not required. Other forms, such as active cysts (intact organism), intraventricular or subarachnoid racemous cysticercosis, and cysticercal meningoencephalitis, are less common manifestations of parasitic infection. Toxoplasmosis, caused by the parasite Toxoplasma gondii, can be acquired by ingestion of infected undercooked meat or from oocytes shed in cat feces. Acquired cerebral toxoplasmosis, due to primary or reactivated infections, rarely occurs in immunocompetent children. In children who are immunodeficient as the result of AIDS, chemotherapy, tissue transplantation, or congenital immunodeficiency, toxoplasmosis may be difficult to distinguish from cerebral lymphoma. A variety of techniques, including neuroimaging, Thallium-201 SPECT, polymerase chain reaction analysis of CSF, and special histological methods, may be used to diagnose acquired toxoplasmosis. Antiparasitic therapy, using pyrimethamine and sulfadiazine, and serial neuroimaging often enable clinicians to differentiate toxoplasmosis from other central nervous system lesions. Toxoplasmosis may respond to other antimicrobials, including macrolide antibiotics, dapsone, clinidamycin, and atovaquone. Suppressive treatment is generally required for life in immunodeficient patients. Immunodeficient children with acquired toxoplasmosis have high rates of mortality and neurological sequelae.

    Title Infantile Spasms: Hypothesis-driven Therapy and Pilot Human Infant Experiments Using Corticotropin-releasing Hormone Receptor Antagonists.
    Date February 2000
    Journal Developmental Neuroscience
    Excerpt

    BACKGROUND AND RATIONALE: Infantile spasms (IS) are an age-specific seizure disorder occurring in 1:2,000 infants and associated with mental retardation in approximately 90% of affected individuals. The costs of IS in terms of loss of lifetime productivity and emotional and financial burdens on families are enormous. It is generally agreed that the seizures associated with IS respond poorly to most conventional anticonvulsants. In addition, in the majority of patients, a treatment course with high-dose corticotropin (ACTH) arrests the seizures completely within days, often without recurrence on discontinuation of the hormone. However, the severe side effects of ACTH require development of better treatments for IS. Based on the rapid, all-or-none and irreversible effects of ACTH and on the established physiological actions of this hormone, it was hypothesized that ACTH eliminated IS via an established neuroendocrine feedback mechanism involving suppression of the age-specific endogenous convulsant neuropeptide corticotropin-releasing hormone (CRH). Indeed, IS typically occur in the setting of injury or insult that activate the CNS stress system, of which CRH is a major component. CRH levels may be elevated in the IS brain, and the neuropeptide is known to cause seizures in infant rats, as well as neuronal death in brain regions involved in learning and memory. If 'excess' CRH is involved in the pathogenesis of IS, then blocking CRH receptors should eliminate both seizures and the excitotoxicity of CRH-receptor-rich neurons subserving learning and memory. PATIENTS AND METHODS: With FDA approval, alpha-helical CRH, a competitive antagonist of the peptide, was given as a phase I trial to 6 infants with IS who have either failed conventional treatment or who have suffered a recurrence. The study was performed at the Clinical Research Center of the Childrens Hospital, Los Angeles. The effects of alpha-helical CRH on autonomic parameters (blood pressure, pulse, temperature, respiration) were determined. In addition, immediate and short-term effects on ACTH and cortisol and on electrolytes and glucose were examined. The potential efficacy of alpha-helical CRH for IS was studied, using clinical diaries and video EEG. RESULTS: alpha-Helical CRH, a peptide, did not alter autonomic or biochemical parameters. Blocking peripheral CRH receptors was evident from a transient reduction in plasma ACTH and cortisol. No evidence for the compound's penetration of the blood-brain barrier was found, since no central effects on arousal, activity or seizures and EEG patterns were observed. In addition, a striking resistance of the patients' plasma ACTH to the second infusion of alpha-helical CRH was noted. CONCLUSIONS: Peptide analogs of CRH do not cross the blood-brain barrier, and their effects on peripheral stress hormones are transient and benign. Nonpeptide compouds that reach CNS receptors are required to test the hypothesis that blocking CRH receptors may ameliorate IS and its cognitive consequences. Copyright Copyright 1999 S. Karger AG, Basel

    Title Cognitive and Behavioral Effects of Carbamazepine in Children: Data from Benign Rolandic Epilepsy.
    Date January 2000
    Journal Journal of Child Neurology
    Excerpt

    The effects of antiepileptic drugs on cognition are difficult to delineate, yet of critical importance for children with epilepsy. We investigated the cognitive and behavioral effects of carbamazepine in children with benign rolandic epilepsy. Ten subjects with benign rolandic epilepsy were evaluated with and without carbamazepine treatment. Fourteen unmedicated subjects with migraine headache evaluated twice served as a control group. Subjects were 6 to 12 years of age, fluent in English, and not mentally retarded. We found that children with benign rolandic epilepsy were quicker on a visual-search task and recalled stories better when not treated than when treated with carbamazepine. After correction for multiple comparisons only the memory finding remained significant. Higher carbamazepine serum level was associated with slower performance on the same visual-search task. This latter finding did not meet multiple comparison criteria. Numerous significant practice effects were found within the control group. Comparisons with reliable change indices identified two subjects with benign rolandic epilepsy with particularly poor scores while receiving carbamazepine. These findings suggest some effects on memory from carbamazepine; however, they do not support meaningful dosage-related effects, within the recommended range. Significant practice effects confirmed the need to control for such effects when evaluating treatments. Finally, identification of two subjects who performed more poorly while on carbamazepine suggests that some children might experience particular difficulties while receiving this medication and highlights the need to investigate individual subject responses to treatment.

    Title Glucose Transporter Type 1 Deficiency: a Study of Two Cases with Video-eeg.
    Date January 2000
    Journal European Journal of Pediatrics
    Excerpt

    Glucose transporter type 1 (GLUT1) deficiency is an inborn error of glucose transport. Clinical manifestations are presumed secondary to reduced glucose transport across the blood brain barrier, and include seizures, abnormal tone, developmental delay and hypoglycorrhachia. A high index of suspicion is important as GLUT1 deficiency is a potentially treatable cause of mental retardation. We studied two affected children by continuous video-EEG in order to better understand the cause of the clinical manifestations and improvement on a ketogenic diet. The EEG was characterized by generalized paroxysmal 2-2.5 Hz spike-wave discharges, although normal EEGs were also obtained. Atypical absence seizures were the most prominent clinical seizure. Epileptiform activity and clinical seizures occurred in both children while acutely ketotic and non-ketotic, but were markedly more frequent in one child when non-ketotic. Discharges were not associated with a reduction in substrate for brain metabolism in the blood at that time.Conclusion Atypical absence seizures are common in glucose transporter type 1 deficiency and should alert the clinician to the possibility of this treatable disorder when present in a young child with developmental delay. Our data suggest that the therapeutic mechanism of the ketogenic diet in this disorder is more complicated than simply delivering ketones as an alternative substrate for brain metabolism.

    Title Prevalence and Incidence of Intracranial Haemorrhage in a Population of Children with Haemophilia. The Hemophilia Growth and Development Study.
    Date December 1999
    Journal Haemophilia : the Official Journal of the World Federation of Hemophilia
    Excerpt

    The prevalence of intracranial haemorrhage (ICH) in our population of haemophiliacs was 12%. The incidence of ICH was approximately 2% per year. At entry, 7% (21/309) had clinical histories of ICH without MRI evidence of old haemorrhage, indicating that either the haemorrhages had completely resolved, that routine MRI sequences are not particularly sensitive for the detection of old blood products, or a combination of both of these factors. One half (4/8) of the ICHs documented by entry MRI were clinically silent, and three of the 11 incident cases documented by MRI were clinically silent. HIV infection did not increase the risk of ICH.

    Title An Open-label Study of Repeated Use of Diazepam Rectal Gel (diastat) for Episodes of Acute Breakthrough Seizures and Clusters: Safety, Efficacy, and Tolerance. North American Diastat Group.
    Date November 1999
    Journal Epilepsia
    Excerpt

    PURPOSE: To assess safety of diazepam rectal gel (DZPRG) for control of acute seizures in epilepsy patients and to evaluate tolerance with repeated use of DZPRG at intervals of > or =5 days. METHODS: Subjects were persons with epilepsy, age 2 years or older, with seizure clusters or prolonged seizures. Onset of a treatable episode was defined; caregivers were trained to administer DZPRG and to monitor respiration, seizures, and adverse effects (AEs). DZPRG was dispensed in a single-use, prefilled syringe; dosage was determined by age and weight. Maximal use was > or =5-day intervals, < or =5 times/month. After use, caregivers returned data booklets and syringe. Caregivers and physicians completed global ratings yearly. RESULTS: In 149 subjects treated, 77% of 1,578 administrations resulted in seizure freedom for the next 12 h. One hundred twenty-five received two or more treatments (two to 78; median, 8), 0.03-4.3/month (median, 0.4). To evaluate tolerance, subjects with two or more episodes were divided into low (two to seven episodes) and high use (eight to 78 episodes treated). There was no difference in proportion seizure free 12 h after the first administration versus last administration, for either infrequent or frequent administration. Sedation occurred in 17%, attributed to DZPRG in 9%. No respiratory depression was attributable to DZPRG. Three subjects withdrew because of AEs attributable to (agitation) or possibly attributable to DZPRG (chest pain, rash). Five subjects withdrew because of AEs unrelated to DZPRG. Caregiver and physician global ratings were highly positive at both 12 and 24 months. CONCLUSIONS: DZPRG is safe and effective in children and adults with epilepsy with breakthrough seizures. Neither tolerance nor significant medication-related AEs were seen with repeated DZPRG administration at intervals > or =5 days.

    Title Rectal Diazepam Gel for Treatment of Acute Repetitive Seizures. The North American Diastat Study Group.
    Date June 1999
    Journal Pediatric Neurology
    Excerpt

    The purpose of these investigations was to determine from combined data the response to rectal diazepam (DZP) gel (Diastat [Athena Neurosciences, South San Francisco, CA]) in home treatment of children with episodes of acute repetitive seizures (ARS). A subset of patients aged 2-17 years were selected from two prospective placebo-controlled studies of children and adults. In both studies a prospective, double-blind, placebo-controlled design was used. The treatment groups (68 DZP; 65 placebo) did not differ significantly in age, race, seizure type or etiology, or in the median number of ARS episodes per month before study entry. DZP-treated children demonstrated a significant reduction in median seizure frequency compared with the placebo group (0.00 vs 0.25 seizures per hour, P = 0.001). Significantly more DZP-treated children remained seizure free during the observation period (40 vs 20, P = 0.001). Somnolence was the only adverse effect present significantly more often in the DZP-treated children (25.0% vs 7.7%, P = 0.0095). There were no instances of serious respiratory depression. Rectal DZP was demonstrated to be an effective and safe treatment to abort an episode of ARS in a child and, additionally, lessened the likelihood of seizure recurrence within the next 12 hours.

    Title Hemophilia Morbidity, Cognitive Functioning, and Academic Achievement.
    Date January 1999
    Journal The Journal of Pediatrics
    Excerpt

    Data from the Hemophilia Growth and Development Study (HGDS) were used to evaluate the association between hemophilia morbidity, measured by abnormalities in coordination and gait (CG), and intellectual ability and academic achievement. The CG abnormalities observed in the HGDS participants (n = 333) were primarily due to hemophilia-related morbidity. Although HGDS participants performed within the average range for age on measures of intellectual ability, there were meaningful differences between CG outcomes at baseline and throughout the 4 years of study. Participants without CG abnormalities consistently achieved higher scores than those with CG abnormalities on Reading, Spelling, and Arithmetic subtests of the Wide Range Achievement Test-Revised. Our findings suggest that lowered achievement is related to the functional severity of hemophilia.

    Title Myelopathy Resulting from Invasive Aspergillosis.
    Date December 1998
    Journal Pediatric Neurology
    Excerpt

    Aspergillus, a ubiquitous mold, may cause invasive and fatal disease in immunosuppressed patients. Myelopathy is an uncommon presentation of invasive aspergillosis. This report describes three children admitted to the hospital between 1988 and 1995 who developed myelopathy as the first evidence of invasive aspergillosis. All had advanced leukemia and were profoundly immunosuppressed because of chemotherapy and broad-spectrum antibiotics. Weakness and pain presented first; then, sensation to pain and temperature was lost 2 to 6 days later, followed by complete myelopathy. Multiple brain lesions were seen on magnetic resonance imaging in one patient. Despite antifungal therapy, aspergillosis proved fatal within 1 month of onset of myelopathy in all patients. Physicians caring for immunocompromised children should be aware of myelopathy as a presentation of invasive aspergillosis.

    Title Treating Repetitive Seizures with a Rectal Diazepam Formulation: a Randomized Study. The North American Diastat Study Group.
    Date December 1998
    Journal Neurology
    Excerpt

    OBJECTIVE: To evaluate the effectiveness and safety of a single-dose treatment for acute repetitive seizure (ARS) episodes (e.g., clusters) administered in a nonmedical setting by caregivers. BACKGROUND: Patients with epilepsy may experience ARS episodes despite optimal anticonvulsant treatment. Such episodes require rapid treatment as medical emergencies. Typically, the patient is treated in an emergency medical setting with i.v. medication by trained medical personnel. METHODS: The authors undertook a multicenter, randomized, parallel, double-blind study of a single administration of Diastat (diazepam rectal gel) for treating episodes of ARS. ARS episodes and treatment criteria were defined for each patient at the start of the study. Caregivers were taught to determine ARS episode onset, administer a predetermined dose of study medication, monitor outcome, count respirations, and record seizures and adverse events. RESULTS: A total of 29 centers enrolled 158 patients, of whom 114 patients had a treated ARS episode (Diastat, n = 56; placebo, n = 58). Diastat treatment reduced median seizure frequency (p = 0.029). More Diastat patients were seizure free post-treatment (Diastat, 55%; placebo, 34%; p = 0.031). Kaplan-Meier analysis of the time to the next seizure favored Diastat treatment (p < 0.007). The most common adverse event was somnolence. CONCLUSION: Administration of a single rectal dose of Diastat was significantly more effective than placebo in reducing the number of seizures following an episode of ARS. Caregivers could administer treatment safely and effectively in a nonmedical setting.

    Title A Randomized Study of Combined Zidovudine-lamivudine Versus Didanosine Monotherapy in Children with Symptomatic Therapy-naive Hiv-1 Infection. The Pediatric Aids Clinical Trials Group Protocol 300 Study Team.
    Date November 1998
    Journal The Journal of Pediatrics
    Excerpt

    OBJECTIVE: The Pediatric AIDS Clinical Trials Group (PACTG) Protocol 300 assessed the clinical efficacy and safety of combination zidovudine/lamivudine (ZDV/3TC) compared with either didanosine (ddI) alone or combination ZDV/ddI. STUDY DESIGN: Children with symptomatic human immunodeficiency virus (HIV) infection, 6 weeks through 15 years of age, were stratified according to age and randomly assigned to receive ddI, ZDV/3TC, or ZDV/ddI. The primary endpoint was time to first progression of HIV disease or death. Enrollment in the ZDV/ddI arm stopped after 11 months on the basis of results of PACTG Protocol 152, but blinded follow-up continued. RESULTS: For the 471 children who could be evaluated, the median age was 2.7 years, median CD4 cell count was 699 cells/mm3, and median log10 HIV RNA was 5.1/mL. Median follow-up was 9.4 months. Patients receiving ZDV/3TC had a lower risk of HIV disease progression or death than those receiving ddI alone (15 vs 38 failures, P = .0006) and a lower risk of death (3 vs 15 deaths, P = .0039). Weight and height growth rates, CD4+ cell counts, and RNA concentrations showed results favoring ZDV/3TC. For patients concurrently randomized to all 3 treatment arms, both ZDV/3TC and ZDV/ddI recipients had lower risk of HIV disease progression than those who received ddI alone (P = .0026 and P = .0045). CONCLUSIONS: Combination therapy with either ZDV/3TC or ZDV/ddI was superior, as determined by clinical and laboratory measures, to monotherapy with ddI.

    Title Hemophilia Growth and Development Study: Relationships Between Neuropsychological, Neurological, and Mri Findings at Baseline.
    Date May 1998
    Journal Journal of Pediatric Psychology
    Excerpt

    OBJECTIVE: To determine the effects of human immunodeficiency virus (HIV) infection on children's development by identifying neurological and environmental variables associated with neuropsychological measures of cognitive development in HIV-seronegative (HIV-) and HIV-seropositive (HIV+)children and adolescents with hemophilia. METHODS: Participants (N = 298; 60% HIV+) were males ages 7-19 years enrolled in the Hemophilia Growth and Development Study (HGDS). Least squares modeling was used to determine whether there was a difference at baseline in mean neuropsychological test scores by HIV status, age, and neurological baseline findings, adjusting for selected environmental and medical history variables. RESULTS: The participants were within age expectations for general intelligence. Variables associated with lowered neuropsychological performance included academic problems, coordination and/or gait abnormalities, parents' education, and previous head trauma. CONCLUSIONS: Hemophilia-related morbidity has a subtle adverse influence on cognitive performance. HIV infection was not associated with neuropsychological dysfunction in this group even when MRI abnormalities were present.

    Title A Randomized Comparative Trial of Stavudine (d4t) Versus Zidovudine (zdv, Azt) in Children with Human Immunodeficiency Virus Infection. Aids Clinical Trials Group 240 Team.
    Date February 1998
    Journal Pediatrics
    Excerpt

    OBJECTIVES: To compare the safety and tolerance of stavudine (d4T) versus zidovudine (ZDV, AZT) in symptomatic human immunodeficiency virus-infected children 3 months to 6 years of age. METHODS: In an initially double-blind trial, 212 evaluable human immunodeficiency virus-infected children who had received no more than 6 weeks of previous antiretroviral therapy were randomized to receive either d4T (1 mg/kg orally every 12 hours, maximum 40 mg orally every 12 hours) or zidovudine (180 mg/m2 orally every 6 hours, maximum 200 mg orally every 6 hours). The study was unblinded after a median follow-up period of 6.3 months; median follow-up at study closure was 17.3 months. Tolerance, safety, disease progression, and immunologic responses were evaluated. RESULTS: The patient population was young (median age, 1.2 years; range, 0.3 to 6.4 years), with a median baseline CD4+ lymphocyte count of 965 cells/microL (range, 18 to 4238 cells/microL). Neutropenia < 400/microL occurred significantly more commonly among zidovudine recipients (1-year event rates of 20% both up to the time of unblinding and throughout the entire study) than among children receiving d4T (1-year event rates of 5% up to the time of unblinding and 6% throughout the entire study). In exploratory activity analyses using all data collected until study closure, children treated with d4T showed consistently greater positive changes from baseline in weight-for-age-and-gender z scores. As expected in this population of young children, median absolute CD4+ lymphocyte counts decreased in both treatment groups. Smaller changes from baseline were noted among d4T recipients. CONCLUSIONS: In children between the ages of 3 months and 6 years, d4T and zidovudine are largely comparable in terms of safety and tolerance. Neutropenia occurs significantly less commonly among children treated with d4T. There was evidence that weight gain and absolute CD4+ lymphocyte counts were better maintained in children receiving d4T.

    Title Longitudinal Neurological Follow-up of a Group of Hiv-seropositive and Hiv-seronegative Hemophiliacs: Results from the Hemophilia Growth and Development Study.
    Date November 1997
    Journal Pediatrics
    Excerpt

    BACKGROUND: Boys and young men with hemophilia treated with factor infusions before 1985 had a substantial risk of acquiring the human immunodeficiency virus (HIV) and the acquired immunodeficiency syndrome. This study was designed to assess the effects of HIV and hemophilia per se on neurological function in a large cohort of subjects with hemophilia, and to investigate the relationships between neurological disease and death during follow-up. METHODS: Three hundred thirty-three boys and young men (207 HIV seropositive and 126 HIV seronegative) were evaluated longitudinally in a multicenter, multidisciplinary study. Neurological history and examination were conducted at baseline and annually for 4 years. The relationship between neurological variables, HIV serostatus, CD4+ cell counts, and vital status at the conclusion of the study was examined using logistic regression models. RESULTS: The risks of nonhemophilia-associated muscle atrophy, behavior change, and gait disturbance increased with time in immune compromised HIV-seropositive subjects compared with HIV seronegative or immunologically stable HIV-seropositive subjects. The risk of behavior change in immune compromised HIV-seropositive hemophiliacs, for example, rose to 60% by year 4 versus 10% to 17% for the other study groups. Forty-five subjects (13.5%), all of whom were HIV seropositive, died by year 4. Subjects who died had had increased risks of hyperreflexia, nonhemophilia-associated muscle atrophy, and behavior change. CONCLUSIONS: These results indicate that immune compromised, HIV-seropositive hemophiliacs have high rates of neurological abnormalities over time and that neurological abnormalities were common among subjects who later died. By contrast, immunologically stable HIV-seropositive subjects did not differ from the HIV-seronegative participants. Hemophilia per se was associated with progressive abnormalities of gait, coordination, and motor function.

    Title Cavus Deformity of the Foot Secondary to a Neuromuscular Choristoma (hamartoma) of the Sciatic Nerve. A Case Report.
    Date October 1997
    Journal The Journal of Bone and Joint Surgery. American Volume
    Title Pediatric Neurocysticercosis in North America.
    Date June 1997
    Journal European Neurology
    Title Inhibition of Pituitary-adrenal Secretion by a Corticotropin Releasing Hormone Antagonist in Humans.
    Date April 1997
    Journal Molecular Psychiatry
    Excerpt

    Corticotropin releasing hormone (CRH) is the primary modulator of ACTH release from the pituitary, and a neuromodulator in limbic and autonomic brain regions. Dysfunction of CRH-mediated neurotransmission is emerging as a critical mechanism in several disorders. Therefore, modulation of CRH availability at receptor sites is a potentially powerful therapeutic tool. Inhibitory analogues of CRH have been tested in rodents and primates, but their safety and hormonal effects in humans are unknown. We administered a CRH-antagonist, alpha-helical-CRH-(9-41) to six individuals. Each received two intravenous infusions: 50 micrograms kg-1 on day 1, and 100 micrograms kg-1 on the following morning. These doses block both endocrine and central effects of CRH in experimental animals. ACTH, cortisol, electrolytes, glucose and autonomic parameters were monitored in comparison with control values. Infusion of CRH antagonist did not alter heart rate, blood pressure, temperature or plasma electrolytes and glucose. Pre-infusion plasma ACTH levels averaged 26.8 +/- 6.7 pg ml-1 on day 1, and 29.0 +/- 5.8 pg ml-1 on day 2. Post-infusion values were 11.8 +/- 2 and 11.5 +/- 2.4 pg ml-1, significantly lower than pre-infusion levels. Plasma cortisol levels, which averaged 21.4 +/- 4 micrograms dl-1 on the first morning and 22.9 +/- 4.2 on the second, also decreased significantly after CRH antagonist infusions (to 14.0 +/- 2.9 micrograms dl-1 on day 1, and 13.9 +/- 3.0 micrograms dl-1 on day 2). Hormonal changes were transient, and circadian rhythm was not affected. Though not measured formally, euphoria, anxiety or somnolence were not observed. In conclusion, CRH antagonist administration to adults reduces hormonal secretion by pituitary corticotrophs, with resulting decrease in plasma ACTH and cortisol.

    Title Usefulness of [18f]fluorodeoxyglucose Positron Emission Tomography in Pediatric Epilepsy Surgery.
    Date September 1996
    Journal Pediatric Neurology
    Excerpt

    We sought to analyze our experience with pediatric epilepsy surgery patients to determine the place of [18F]fluorodeoxyglucose (FDG) positron emission tomography (FDG-PET) in the preoperative evaluation of such children relative to chronic invasive intracranial monitoring. Fifty-six children who received an interictal FDG-PET as part of a phase 1 epilepsy surgery evaluation were compared with 44 children who did not have this study in a retrospective analysis of 100 patients accrued over a 4-year period. There was no significant difference between the two groups of children in terms of age or follow-up or was there a significant difference between the FDG-PET group and the no-FDG-PET group in regard to the numbers of children who had surgery, the type of procedure done, whether chronic invasive intracranial monitoring was performed, or outcome. The hypometabolic area demonstrated on interictal FDG-PET was concordant with that of the epileptogenic zone as mapped out with ictal recordings from subdural electrodes in 2 of 13 patients in whom a complete data set was available for comparison. In the other 11 children there was either poor agreement between interictal FDG-PET and ictal electrocorticographic data or the interictal FDG-PET was normal in the face of an epileptogenic focus which was successfully mapped by invasive electrophysiologic techniques and excised. We conclude that one cannot exclude a child with intractable partial seizures from surgical consideration because the interictal FDG-PET is normal; nor is there sufficient correlation between the interictal hypometabolic area on FDG-PET and the epileptogenic zone in terms of anatomic location and size to justify forgoing chronic invasive intracranial monitoring in children with intractable partial seizures being evaluated for epilepsy surgery unless there is absolute concordance between all neuroimaging, clinical, and video-electroencephalographic data.

    Title Status Epilepticus and Acute Repetitive Seizures in Children, Adolescents, and Young Adults: Etiology, Outcome, and Treatment.
    Date July 1996
    Journal Epilepsia
    Excerpt

    Status epilepticus (SE) is one of the most common neurologic emergencies in children, adolescents, and young adults. SE may be due to acute neurologic conditions such as meningitis, encephalitis, or stroke, complicated febrile seizures, intractable epilepsy, degenerative diseases, intoxication, or may be the first manifestation of epilepsy. Initial treatment of convulsive SE is usually with an intravenous benzodiazepine (BZD) [lorazepam (LZP) or diazepam (DZP)], phenobarbital (PB), or phenytoin (PHT). LZP is less likely to cause respiratory depression than DZP and is therefore preferred. Sequelae and risk for recurrence of SE are primarily related to the underlying cause. Refractory SE (RSE) is most often symptomatic of an acute neurologic condition or neurodegenerative disease. Treatment for RSE is difficult, usually requiring intensive support of vital functions. Reported treatments for RSE include very high dose PB, continuous infusions of pentobarbital or BZDs (DZP, midazolam), lidocaine, inhalation anesthesia, and propofol. Outcome is related to underlying cause. Nonconvulsive SE may present as confusion or may mimic psychiatric illness. Response to BZDs is usually rapid but may not be sustained. Rapid initiation of oral or rectal valproate may be useful. Epilepsia partialis continua (EPC) is almost always due to an acute or chronic destructive lesion. Surgical treatment may be the only effective modality in some children with EPC. Acute treatment of breakthrough seizures and clusters of seizures at home with rectal BZDs (usually DZP, 0.2-0.5 mg/kg) may prevent progression to SE in some children and adolescents and reduce the need for visits to emergency facilities.

    Title High-dose Corticotropin (acth) Versus Prednisone for Infantile Spasms: a Prospective, Randomized, Blinded Study.
    Date May 1996
    Journal Pediatrics
    Excerpt

    OBJECTIVE: To compare the efficacy of corticotropin (ACTH) (150 U/m2/day) and prednosone (2 mg/kg/day) given for 2 weeks, in suppressing clinical spasms and hypsarrhythmic electroencephalogram (EEG) in infantile spasms (IS). AACTH and prednisone are standard treatments for IS. ACTH at high doses causes severe dose- and duration-dependent side effects, but may be superior to prednisone, based on retrospective or uncontrolled studies. Blinded prospecive studies have shown equal efficacy of prednisone and low-dose ACTH, and low versus high-dose ACTH. DESIGN: A prospective, randomized, single-blinded study. SUBJECTS AND METHODS: Patient population consisted of consecutive infants fulfilling entry criteria, including the presence of clinical spasms, hypsarrhythmia (or variants) during a full sleep cycle video-EEG, and no prior steroid/ACTH treatment. Response required both cessation of spasms and elimination of hypsarrhythmia by the end of the 2-week treatment period, as determined by an investigator "blinded" to treatment. Treatment of responders was tapered off over 12 days; those failing one hormone were crossed-over to the other. RESULTS: OF 34 eligible infants, 29 were enrolled. Median age of patients was 6 months. Twenty-two infants were "symptomatic" with known or suspected cause, and seven were cryptogenic (two normal). Of 15 infants randomized to ACTH, 13 responded by EEG and clinical criteria (86.6%); Seizures stopped in an additional infant, but EEG remained hypsarrhythmic (considered a failure). Four of the 14 patients given prednisone responded (28.6%,, with complete clinical-EEG correlation), significantly less than with ACTH, (chi2 test). CONCLUSIONS: Using a prospective, randomized approach, a 2-week course of high-dose ACTH is superior to 2 weeks of prednsone for treatment of IS, as assessed by both clinical and EEG criteria.

    Title Cerebrospinal Fluid Corticotropin and Cortisol Are Reduced in Infantile Spasms.
    Date February 1996
    Journal Pediatric Neurology
    Excerpt

    Infantile spasms respond to ACTH, and levels of the hormone in cerebrospinal fluid of untreated infants with this disorder were found to be lower than in age-matched controls. In this study we analyzed cerebrospinal fluid cortisol and ACTH using improved immunoassays in a larger cohort of infants with infantile spasms. Analysis of 20 patients and 15 age-matched controls revealed significantly lower levels of both ACTH and cortisol in the cerebrospinal fluid. These data, combined with the efficacy of ACTH and glucocorticoids for infantile spasms, support an involvement of the brain-adrenal-axis in this disorder.

    Title Clinical Utility of Video-eeg Monitoring.
    Date August 1995
    Journal Pediatric Neurology
    Excerpt

    This study investigated the efficiency of simultaneous video-electroencephalography (EEG) monitoring in documenting paroxysmal events, the value in clinical diagnosis, and the effect on treatment. In this retrospective review, 230 children underwent this procedure between January, 1990 and December, 1992. The data demonstrated that video-EEG monitoring can be used as a daytime procedure with a high success rate (80%) in detecting and differentiating the nature of recurrent paroxysmal behaviors that have occurred on a daily basis. Video-EEG monitoring has a high diagnostic rate in differentiating seizure versus nonseizure events (70%), in classifying seizure types (88%), and in evaluating the candidacy for epilepsy surgery (64%). Video-EEG diagnosis resulted in an alteration of clinical management in 45% of patients. Continuous video-EEG monitoring is an efficient and valuable procedure in the diagnosis and management of paroxysmal disorders in children.

    Title Long-term Prognosis for Children with Epilepsy.
    Date August 1995
    Journal Current Problems in Pediatrics
    Excerpt

    In discussions of prognosis that are based primarily on epidemiologic data, it is important to emphasize two issues: first, statistical rates of recurrence or remission apply to groups, not individuals. Whereas important and useful information is obtained from examining the epidemiology of epilepsy, it is the individual child who is the concern of the physician, family, and community. That individual child may do well despite indicators that suggest a risk of poor outcome, or may do poorly, experiencing recurrent seizures or adverse effects of treatment, despite apparent "low risk." It is unimportant to a parent that "90% of children with this type of epilepsy do well" if his or her child is doing poorly. All treatment decisions, including the decision to withdraw anticonvulsants after prolonged seizure-free periods, must be made individually. An adolescent or young adult, seizure-free on treatment, may be unwilling to risk even a 10% to 25% risk of recurrence if it means giving up driving privileges or risking a seizure in school or on the job. In addition, although it is appropriate to reassure most parents of children with newly-diagnosed epilepsy that seizures do not cause cognitive or behavioral deficits, problems do occur frequently and must be addressed. Comprehensive treatment programs for children with epilepsy must include evaluation and treatment of coexisting learning, cognitive, and behavior problems, as well as family education to prevent excessive parental anxiety and overprotection.

    Title Psychosocial, Behavioral, and Medical Outcomes in Children with Epilepsy: a Developmental Risk Factor Model Using Longitudinal Data.
    Date October 1994
    Journal Pediatrics
    Excerpt

    OBJECTIVE. We studied factors predicting the risk of adverse long-term psychosocial, behavioral, and medical outcomes in children with epilepsy. METHODS. Children (N = 157, 4.5 to 13 years) were enrolled in a prospective longitudinal study when first seen. Potential subjects were excluded if they were moderately or severely mentally retarded, had motor or sensory handicaps interfering with testing, or did not speak either English or Spanish. MEASURES. To develop risk predictors, we collected information regarding the child's medical and seizure history, cognitive functioning, and behavior problems, and family functioning. Children and their families were followed for a minimum of 18 months, then underwent reassessment of medical status, parent's attitudes toward epilepsy, and the child's behavioral and cognitive functioning. Data were analyzed by confirmatory factor analysis to develop baseline factors (Sociocultural Risk, Seizure Risk, and Behavior Problems) and outcome factors (Medical/Seizure Problems, Parent's Negative Attitudes Toward Epilepsy, and Behavior Problems), followed by structural equation modeling to determine across-time causal effects. Eighty-eight subjects completed all baseline and outcome measures. RESULTS. Among significant across-time effects, Medical Outcome was predicted by Seizure Risk. An increased number of stressful life events predicted better Medical Outcome. Low acculturation increased Parent's Negative Attitudes and was associated with increased Behavior Problems at baseline. Behavior Problems were stable across time. It is interesting that IQ did not affect any of the outcomes, although its effect may have been mediated through other baseline measures. CONCLUSIONS. Seizure history was the best predictor of ongoing medical difficulties, whereas the most important causes of ongoing parental anxiety and negative attitudes toward epilepsy were sociocultural. Variation in medical or attitudinal outcomes was not influenced by either the child's IQ or reported behavioral problems. These findings suggest that to alter attitudes toward epilepsy, programs should be tailored to the sociocultural background of the family. Studies of quality of life of children with epilepsy should include appropriate sociocultural measures.

    Title Pentobarbital for Status.
    Date May 1994
    Journal Neurology
    Title Effects of Human Immunodeficiency Virus and Immune Status on Magnetic Resonance Imaging of the Brain in Hemophilic Subjects: Results from the Hemophilia Growth and Development Study.
    Date May 1993
    Journal Pediatrics
    Excerpt

    To determine the effects of hemophilia and human immunodeficiency virus (HIV) infection on the nervous system, the authors examined the relationship of brain magnetic resonance imaging (MRI) findings to immunologic function and neurologic examination findings. Baseline examinations included physical and neurologic examination, immunologic and virologic testing, and MRI of the brain. On neurologic examination, muscle atrophy was considered to be related to hemophilia if adjacent joints had arthropathy due to bleeding. Muscle atrophy was considered non-hemophilia-related if unrelated to arthropathy or if muscle atrophy was diffuse. Subjects were boys aged 6 to 19 years, enrolled in a multicenter study of the effects of hemophilia and HIV infection on growth and development, all with congenital coagulopathies requiring factor infusions. Three hundred ten subjects had complete data including neurologic examination, T-cell subsets, HIV antibodies, and MRI. Subjects with HIV infection whose CD4+ counts were < 200/microL were compared with subjects with HIV infection and CD4+ counts > or = 200/microL and with HIV-negative subjects, all of whom had CD4+ counts > 200/microL. MRI studies were normal in 230. Abnormal MRI studies were more frequent in HIV-positive subjects with CD4+ counts < 200 (29.4% abnormal compared with 17% in HIV-positive subjects with CD4+ counts > or = 200 and 15.3% in HIV-negative subjects). Diffuse atrophy accounted for most of the excess abnormalities in HIV-positive subjects with CD4+ counts < 200 (77.3% of abnormal scans). Diffuse atrophy on MRI was associated with decreased muscle bulk on neurologic examination, but not with abnormal tendon reflexes.(ABSTRACT TRUNCATED AT 250 WORDS)

    Title Effects of Antiepileptic Drugs on Reaction Time, Attention, and Impulsivity in Children.
    Date January 1993
    Journal Pediatrics
    Excerpt

    Simple, choice, and complex reaction times, attention (variability of responses and omission errors), and impulsivity (commission and wrong-hand errors on choice and complex reaction time) were repeatedly measured in 111 epileptic children, aged 5 to 13 years, tested a total of 232 times. Antiepileptic drugs (AEDs) were started, stopped, and adjusted throughout the study period, for a variety of clinical indications, and AED serum levels were monitored. The relationship of performance to AED serum level was examined. Overall the nonspecific effect of AEDs was minimal: higher total serum levels of AEDs correlated with more impulsive errors on complex reaction time testing only. In contrast, in 54 children receiving carbamazepine monotherapy, we found a dose-related beneficial effect upon reaction time, with higher serum levels associated with faster responses and fewer omission errors, particularly on complex reaction time. Phenobarbital caused minimal dose-related effects: only variability and impulsive errors increased with increasing serum levels, and only on one segment of the test (73 subjects).

    Title Brain Abnormalities in Male Children and Adolescents with Hemophilia: Detection with Mr Imaging. The Hemophilia Growth and Development Study Group.
    Date November 1992
    Journal Radiology
    Excerpt

    Cranial magnetic resonance (MR) imaging was performed in 124 male patients (aged 7-19 years), from 14 institutions, in whom a diagnosis of moderate to severe hemophilia was made. Blood tests in all subjects were negative for human immunodeficiency virus. Findings in MR studies were abnormal in 25 (20.2%) subjects. Six lesions in five subjects were classified as congenital. The most commonly identified congenital lesion was a posterior fossa collection of cerebrospinal fluid (five cases). Twenty-two subjects had acquired lesions that were probably related to the hemophilia or its treatment. The most commonly acquired lesions were single- or multifocal areas of high signal intensity within the white matter on T2-weighted images noted in 14 (11.3%) subjects. Two subjects had large focal areas of brain atrophy, and six had some degree of diffuse cerebral cortical atrophy. Three subjects (2.4%) had hemorrhagic lesions. To the authors' knowledge, the unexpected finding of small, focal, nonhemorrhagic white matter lesions has not previously been reported.

    Title Brain-adrenal Axis Hormones Are Altered in the Csf of Infants with Massive Infantile Spasms.
    Date July 1992
    Journal Neurology
    Excerpt

    Massive infantile spasms (MIS), a seizure disorder unique to infants, is considered an age-dependent response of the immature brain to various insults and stressors. The seizures improve with ACTH and glucocorticoids, both major components of the brain-adrenal axis. We hypothesized that CNS levels of these hormones are abnormal in infants with MIS and studied CSF from 14 infants with MIS and 13 age-matched controls by analysis for corticotropin-releasing hormone (CRH), ACTH, cortisol, and interleukin-1-beta. ACTH levels in CSF of patients were significantly lower than those of controls, but differences in cortisol levels between patients and controls were not statistically significant. CRH levels in both groups were similar and fluctuated diurnally. These results indicate an alteration of specific CNS components of the brain-adrenal axis in MIS.

    Title Reaction Time, Attention, and Impulsivity in Epilepsy.
    Date May 1992
    Journal Pediatric Neurology
    Excerpt

    Reaction time, attention, and impulsivity were studied in 112 children with epilepsy (4.5-13 years) using a computerized test. We measured simple reaction time (response with each hand separately to a single stimulus), forced choice reaction time (two stimuli presented in random order, one designated for each hand), and choice reaction time with distraction (two response stimuli, one for each hand, with two additional distracting stimuli randomly inserted). We also measured variability of speed of response and errors of omission and commission. Controls were unaffected children of similar age, ethnic, and socioeconomic backgrounds. Children with epilepsy were significantly slower, more variable, and made more omission errors than control children, even when analysis was limited to epileptic patients with IQ greater than 90, but they did not make more commission (i.e., impulsive) errors. Reaction times were related to IQ, but in general were not related to seizure severity, duration of seizure disorder, or duration of medication use. Untreated patients (N = 13) did not differ from those with antiepileptic drug levels in the therapeutic range on the day of testing (N = 52), but differed significantly from normal patients. Epileptic patients demonstrated significant slowing of reaction time and inattention, but not significant impulsivity, compared to normal children; however, these deficits do not appear to be related specifically to seizure history or treatment.

    Title Prognostic Significance of Acute Epilepsia Partialis Continua.
    Date August 1991
    Journal Pediatric Neurology
    Excerpt

    We present 3 patients in whom epilepsia partialis continua was the presenting sign of an acute, rapidly evolving and catastrophic neurologic illness. Initial seizures were partial simple (i.e., eye deviation in one, finger twitching in one) which progressed to multifocal partial seizures. The course of the epilepsia partialis continua was 36-41 days. Prognosis was uniformly poor (i.e., death in 2, vegetative state in 1); therefore, epilepsia partialis continua in the context of an acute neurologic illness may herald a grim outcome.

    Title Stroke As a Late Sequela of Cranial Irradiation for Childhood Brain Tumors.
    Date July 1991
    Journal Journal of Child Neurology
    Excerpt

    Cerebrovascular disease involving large and medium-size vessels is thought to be an uncommon sequela of treatment of childhood brain tumors. We reviewed 11 children who developed cerebrovascular disease manifested by strokes or transient ischemic attacks 6 months to 4 years after treatment of brain tumors, while their tumors were in remission. All had received radiation therapy, and seven had received chemotherapy. One child died of acute bilateral cerebral infarctions due to carotid occlusion on one side and marked stenosis on the other 2 years after receiving radiation therapy for an incompletely resected craniopharyngioma. Pathologically, there was marked subendothelial fibrosis of the vessels of the circle of Willis, with inflammatory changes surrounding some of the vessels. In addition to the widely recognized small-vessel damage caused by radiation and chemotherapy in children (mineralizing microangiopathy), damage to medium and large intracranial vessels may result in late sequelae, manifested by stroke or transient ischemic attacks.

    Title Academic Underachievement in Children with Epilepsy.
    Date April 1991
    Journal Journal of Child Neurology
    Excerpt

    Academic achievement was studied in 78 children with epilepsy, ages 5 to 13 years, to determine how seizures, treatment of seizures, and sociocultural factors influence academic achievement. Cognitive abilities were assessed with either the McCarthy Scales of Children's Abilities or the Wechsler Intelligence Scale for Children--Revised. Achievement was measured with the Peabody Individual Achievement Tests. Achievement scores were corrected for cognitive ability (IQ), and underachievement was defined as achievement score 1/2 standard deviation or more below IQ. Information regarding seizure history (severity, duration) and treatment with anticonvulsant drugs was obtained. In addition, the family was extensively interviewed regarding the child's environment, behavior, and demographic background. The Home Observation for Measurement of the Environment (HOME) scale was completed on a home visit. Underachievement was frequent, ranging from 16% (Reading Recognition) to 50% (General Knowledge), but there was no relationship between severity or duration of seizure disorder or total exposure to anticonvulsant medications and achievement. Major determinants of achievement included subscales of the HOME scale, age (older children more likely to be underachieving), and parental education. An equal proportion of newly diagnosed and/or untreated subjects were underachieving compared to those with longstanding epilepsy and anticonvulsant drug treatment.

    Title Reaction Time, Impulsivity, and Attention in Hyperactive Children and Controls: a Video Game Technique.
    Date October 1990
    Journal Journal of Child Neurology
    Excerpt

    Maturation of sustained attention was studied in a group of 52 hyperactive elementary school children and 152 controls using a microcomputer-based test formatted to resemble a video game. In nonhyperactive children, both simple and complex reaction time decreased with age, as did variability of response time. Omission errors were extremely infrequent on simple reaction time and decreased with age on the more complex tasks. Commission errors had an inconsistent relationship with age. Hyperactive children were slower, more variable, and made more errors on all segments of the game than did controls. Both motor speed and calculated mental speed were slower in hyperactive children, with greater discrepancy for responses directed to the nondominant hand, suggesting that a selective right hemisphere deficit may be present in hyperactives. A summary score (number of individual game scores above the 95th percentile) of 4 or more detected 60% of hyperactive subjects with a false positive rate of 5%. Agreement with the Matching Familiar Figures Test was 75% in the hyperactive group.

    Title Opsoclonus-ataxia Due to Childhood Neural Crest Tumors: a Chronic Neurologic Syndrome.
    Date July 1990
    Journal Journal of Child Neurology
    Excerpt

    Five children with subacute or acute onset of cerebellar ataxia and opsoclonus are described. Two had cerebrospinal fluid pleocytosis at the onset of ataxia and were initially thought to have acute parainfectious cerebellar ataxia of childhood. All were found to have tumors of neural crest origin (two neuroblastomas, three ganglioneuroblastomas). Tumors were small and only found by computed tomographic techniques. Urinary catecholamine metabolites were elevated in only two of the patients. Four of the five failed to improve neurologically with resection of the tumor. All four have had a steroid-sensitive chronic ataxic syndrome that worsens with acute nonspecific illnesses and has resulted in long-term deficits, particularly in speech and gross motor function. This is a metabolic encephalopathy associated with permanent residual neurologic deficits but without visible lesions on neuroimaging studies. We stress the frequency of cerebrospinal fluid pleocytosis in patients with tumor-associated opsoclonus and the clinical difficulty in separating tumor-associated cases from those due to other causes [corrected].

    Title Intraparenchymal Cerebral Cysticercosis in Children: a Benign Prognosis.
    Date June 1989
    Journal Pediatric Neurology
    Excerpt

    This paper reports 26 consecutive cases of cerebral cysticercosis in children, 21 presenting with intraparenchymal mass lesions, two with encephalitic disease, and three with intraventricular (racemous) cysticercosis. The intraparenchymal and encephalitic forms of the disease were benign. Regression of the lesions occurred within four months of diagnosis in all children treated conservatively with antiepileptic drugs, but no antiparasitic drugs. Major morbidity was limited to those patients who were subjected to operative intervention. Intraventricular disease was most malignant; all three patients manifested acute, severely increased intracranial pressure, all required immediate surgical decompression, and one patient died. The apparent overall benign course of intraparenchymal cerebral cysticercosis in children appears not to warrant antiparasitic drug therapy.

    Title Phenobarbital for Status.
    Date May 1989
    Journal Neurology
    Title Very-high-dose Phenobarbital for Refractory Status Epilepticus in Children.
    Date August 1988
    Journal Neurology
    Excerpt

    Status epilepticus refractory to initial anticonvulsant therapy is a serious condition with a high morbidity and mortality. We present 50 cases with refractory status epilepticus (RSE) treated with very-high-dose phenobarbital (VHDPB) without reference to a predetermined maximum level or dose. Maximum serum levels ranged from 70 to 344 micrograms/ml (median, 114 micrograms/ml). VHDPB controlled seizures in all cases where no limits were imposed upon maximum dose (47/50). We found no maximum dose beyond which further doses are likely to be ineffective. Forty patients were intubated prior to VHDPB, but recovered respiratory drive and could be removed from the ventilator despite very high serum levels. This is explained by acute drug tolerance. Hypotension was unusual, related to the highest levels, and easily controlled. VHDPB has many relative advantages over other therapies presently used for RSE.

    Title Encephalitis Lethargica-like Illness in a Girl with Mycoplasma Infection.
    Date August 1988
    Journal Neurology
    Excerpt

    We describe a patient with mycoplasma infection and clinical manifestations of encephalitis lethargica. The patient was brought to our attention after acute neurologic deterioration followed by a prolonged sleep-like state and the emergence of extrapyramidal features. MRI of the brain disclosed a striking pattern of subcortical involvement by the inflammatory process, corroborating the clinical picture.

    Title Intraparenchymal Cerebral Cysticercosis in Children: Diagnosis and Treatment.
    Date July 1988
    Journal Pediatrics
    Excerpt

    Cerebral cysticercosis is being diagnosed with increasing frequency in children in the United States. A total of 52 patients with intraparenchymal cerebral cysticercosis, 21 months to 20 years of age, were observed for 2 to 72 months. Actively inflamed acute lesions, with or without coexisting chronic calcified lesions, were seen in 42 patients. Solitary lesions were seen in 39 patients, multiple lesions in 13. Chronic inactive calcified lesions were seen in ten children. A total of 51 patients had seizures that were either partial or partial with secondary generalization. Patients were treated with anticonvulsants. Praziquantel was not used. All active lesions resolved completely or with punctate calcification in 2 to 9 months, according to radiographic findings. Seizures were easily controlled. Patients were gradually weaned from anticonvulsants and in 60% no seizures recurred. Prognosis was generally good. Characteristics of the CT scans are detailed.

    Title Intraventricular Alpha 2b Interferon for Sspe.
    Date December 1987
    Journal Neurology
    Title Human Immunodeficiency Virus-associated Kaposi's Sarcoma in a Pediatric Renal Transplant Recipient.
    Date October 1987
    Journal Nephron
    Excerpt

    An 11-year-old boy developed Kaposi's sarcoma and progressive T lymphocyte deficiency 5 years after cadaveric kidney transplantation for end-stage renal disease. He had received 17 individual red blood cell transfusions prior to and during transplantation in 1980. Human immunodeficiency virus (HIV) was cultured from blood in cerebrospinal fluid and HIV antibodies were detected with enzyme immunoassay and immunoblot techniques. The recipient of the donor's other kidney was well and HIV antibody-negative. The patient was treated with etoposide with excellent although transient regression of tumor. Allograft function has remained stable despite minimal immunosuppressive therapy and the need for high-dose anticonvulsant therapy. This case represents the first pediatric patient with acquired immune deficiency syndrome (AIDS) and Kaposi's sarcoma following kidney transplantation.

    Title Lorazepam in Childhood Status Epilepticus and Serial Seizures: Effectiveness and Tachyphylaxis.
    Date March 1987
    Journal Neurology
    Excerpt

    We report our experience with 300 consecutive parenteral doses of lorazepam (LOR) for status epilepticus (SE) or serial seizures in 77 children and young adults. The median dose for SE in children less than 12 years old was 0.10 mg/kg. LOR stopped the SE in 79% and diminished the intensity of SE in an additional 4%. Prior acute or chronic anticonvulsant use (excepting chronic benzodiazepines) did not alter effectiveness or increase side effects. Duration of freedom from seizures following acute therapy was independent of LOR dosage. In patients requiring sequential doses, LOR becomes progressively less effective. Side effects were few and, when present, always associated with a single or first dose in a series. LOR is a safe and effective acute anticonvulsant agent for in-hospital control of SE in the pediatric age group. Tachyphylaxis of anticonvulsant action occurs when serial doses are used.

    Title Carbamazepine Versus Phenobarbital for Partial Onset Seizures in Children.
    Date February 1987
    Journal Epilepsia
    Excerpt

    Thirty-nine children were treated with either phenobarbital (PB) or carbamazepine (CBZ) for newly diagnosed partial onset seizures. Drug selection was randomized in 33 subjects. Parents and the psychologist evaluating the child were blind to drug identity. Psychometric and behavioral evaluations were done at intake and at 6- and 12-month follow-ups. There were no significant differences between drugs in effect on behavior or cognitive function. CBZ caused more systemic problems. There was a trend toward better seizure control with CBZ, but this was not statistically significant. Although individual children in each group had changes in behavior or cognitive status, neither group changed significantly, in either acute or chronic follow-up.

    Title Routine Versus Selective Exploration of Penetrating Neck Injuries: a Randomized Prospective Study.
    Date January 1985
    Journal The Journal of Trauma
    Excerpt

    In an effort to settle the controversy regarding the optimal management of penetrating trauma to the neck, a randomized prospective study was conducted in which 160 patients with penetrating neck injuries admitted to Kings County Hospital between 1977 and 1982 were placed, by protocol, into two groups. Group A patients were explored routinely for all injuries to the neck violating the platysma muscle. Group B patients were managed selectively with operation based on clinical or radiographic evidence of major vascular, visceral, or airway injury. Data were collected retrospectively. Length of hospital stay, morbidity, and mortality were compared between groups A and B, as well as between patients explored or not, and no statistical difference was noted. Since there is no clear advantage to either routine or selective exploration in the management of penetrating neck wounds, we conclude that surgeons should base their treatment on their own experience, house staff and nursing support, and radiologic and operating room availability.

    Title Interventional Radiology in the Management of Hepatic Trauma.
    Date May 1984
    Journal The Journal of Trauma
    Excerpt

    Interventional radiology (catheter placement under radiologic guidance) is a safe and effective technique in the management of hemorrhage and infection after hepatic trauma. Twenty procedures in 17 patients were reviewed. All patients with hemorrhage, vascular lesions, and intra-abdominal fluid collections were successfully treated without mortality or substantial morbidity. These techniques are recommended in complicated liver trauma.

    Title The Relative Roles of Neurological Examination, Functional Abilities, and Computed Tomography in the Definition of Treatment Failure in Patients with Anaplastic Gliomas.
    Date November 1983
    Journal Surgical Neurology
    Excerpt

    Forty-one patients with anaplastic gliomas undergoing postoperative treatment and evaluation underwent bimonthly evaluation of the following indices of treatment failure: neurological examination; Karnofsky functional rating; and computed tomography (CT) brain scanning. Treatment failure was declared when neurological examination or Karnofsky rating showed increased impairment or when CT scan revealed an increase in tumor sizes. Most often, all three indices simultaneously indicated treatment failure. In only 6 of 41 cases the CT scan alone was the first indication of treatment failure. During the first 6 months of follow-up, tumor enlargement on CT scan as a sole index of treatment failure occurred in only 3 of 26 cases that showed evidence of treatment failure during that time. For patients with glioblastoma, about 6% of treatment failures within 6 months are predicted to be missed by Karnofsky rating plus neurological examination, whereas CT scan alone is predicted to miss about 30%. It would seem reasonable to rely on the neurological examination and Karnofsky rating for follow-up during the first 6 months after surgery, without routine serial CT scanning during that time.

    Title Abdominal Epilepsy. Cyclic Vomiting As the Major Symptom of Simple Partial Seizures.
    Date April 1983
    Journal Archives of Neurology
    Title Interventional Radiology in Trauma Victims: Analysis of 51 Consecutive Patients.
    Date July 1982
    Journal The Journal of Trauma
    Excerpt

    Injured patients whose treatment included the use of radiologic catheter techniques were reviewed to determine the clinical utility of radiologic intervention. In the past 4 years, 51 patients have undergone 53 radiologic procedures, predominantly angiographic hemostasis. Thirty-eight of 45 sites of hemorrhage from the neck, abdomen, pelvis, and extremities were successfully controlled. Nine of 11 abscesses were adequately drained and definitively treated by percutaneous methods. Twenty-eight of the 31 patients with shock or with life-threatening hemorrhage or sepsis, treated by the radiologist, were salvaged. Based on our results, we believe that a radiologist, knowledgeable about trauma and expert in catheter techniques, plays a vital role in improving patient survival and should be integrated into the trauma team approach.

    Title Retrograde Venous Bullet Embolism.
    Date October 1981
    Journal The Journal of Trauma
    Excerpt

    Bullets may embolize against normal blood flow into distal veins because of gravity or the Valsalva maneuver. A case is described in which a bullet was fluoroscopically repositioned from the popliteal vein into the area of a lacerated iliac vein and trapped with a balloon catheter obviating popliteal exploration.

    Title Radiologic Management of Traumatic Hepatic Artery-portal Vein Arteriovenous Fistulae.
    Date September 1981
    Journal The Journal of Trauma
    Excerpt

    Traumatic hepatic artery portal vein arteriovenous fistulae (HPF) are uncommon but potentially life-threatening distortions of hepatic circulation. They are curable causes of gastrointestinal bleeding and portal hypertension. HPF may result from lacerations of adjacent arterial and venous walls, pseudoaneurysms, or liver erosion causing a shunt into the portal vein. Symptoms are GI bleeding, or crampy abdominal pain and diarrhea, or in delayed HPF, signs of portal hypertension. Angiography can make the diagnosis and may be therapeutic, as in one reported case, in which successful transcatheter embolization obviated the need for surgical treatment.

    Title Good Performance Rewarded in Cash.
    Date September 1980
    Journal Hospitals
    Title Failure-to-thrive: a Study in a Primary Care Setting. Epidemiology and Follow-up.
    Date June 1980
    Journal Pediatrics
    Excerpt

    A cohort of 312 children followed in three rural primary care centers was studied. All cases of failure-to-thrive were identified (30) and compared to the remainder of the group. With the use of data from medical records, children with history of failure-to-thrive were found to be significantly lighter and shorter than the remainder of the population in each of the first five years of life. More cases had family problems. No differences were found in demographic characteristics or chronic illnesses. In the second phase of the study, cases and an equal-sized control group, from the same social setting, matched for age, sex, mother's marital status, and family problems, were examined at 3 to 6 years of age to assess outcome. This included physical examination, the McCarthy Scale of Children's Abilities, a behavior questionnaire, and an interview to assess life events. Cases were lighter but not shorter than controls. No instances of occult organic disease causing failure-to-thrive were identified. No behavioral or developmental deficits could be attributed to the failure-to-thrive.

    Title Pesticide Residue Concentrations in Soils of Five United States Cities, 1971--urban Soils Monitoring Program.
    Date December 1979
    Journal Pesticides Monitoring Journal
    Excerpt

    Soil samples from five metropolitan areas including Baltimore, Maryland; Gadsden, Alabama; Hartford, Connecticut; Macon, Georgia; and Newport News, Virginia were analyzed for elemental arsenic, organochlorine pesticides, and polychlorinated biphenyls (PCBs). A representative number of samples were analyzed for organophosphorus pesticides, but none was detected. All areas exhibited heavy soil concentrations of organochlorine pesticides including sigma DDT, aldrin, dieldrin, photodieldrin, chlordane, heptachlor, heptachlor epoxide, endrin, endrin ketone, and endosulfan sulfate. PCBs were detected in three of the five metropolitan areas. Within the metropolitan areas, samples from the urban, or core city, locations generally had higher pesticide concentrations than did samples from suburban locations. Finally, pesticide residue concentrations were generally higher in soils of metropolitan areas than in nearby agricultural soils.

    Title Mandatory Open Reduction: Its Role in Displaced Ankle Fractures.
    Date October 1979
    Journal The Journal of Trauma
    Excerpt

    "Satisfactory reduction" is insufficient in discussing ankle fractures; only perfect anatomic reduction will suffice. Fractures extending into joint surfaces carry a higher incidence of disability than those involving the metaphysis and/or diaphysis. The end result is further adversely affected when such fractures occur in weight-bearing joints with resulting disability from persistent pain and stiffness. It is our belief that anatomic reduction of displaced ankle fractures, especially the restoration of fibular length, is almost impossible by closed reduction. Closed reduction may require frequent manipulation and plaster changes as the swelling subsides, and the fragments become displaced. This encourages ankle and subtalar stiffness. For these reasons mandatory open reduction and rigid internal fixation of these fractures are recommended. A review of 300 nonconsecutive cases of open and closed displaced ankle fractures treated by open reduction had an overall infection rate of 1% and an incidence of degenerative arthritis of 3%.

    Title Pesticide Residue Levels in Soils and Crops from 37 States, 1972--national Soils Monitoring Program (iv).
    Date September 1979
    Journal Pesticides Monitoring Journal
    Excerpt

    Residue data from the 1972 (FY--73) National Soils Monitoring Program are summarized. Composite samples of agricultural soil and mature crops were collected from 1,483 of the 1,533 selected 4-hectare sites in 37 states. Analyses were performed for organochlorine and organophosphorus compounds, trifluralin and polychlorinated biphenyls (PCBs); analysis for atrazine was performed only when pesticide application data indicated current-year use. Organochlorine pesticides were detected in 45 percent of the soil samples. The most frequently detected compound was dieldrin, found in 27 percent of all soil samples. Other compounds detected, in order of frequency, included DDT, aldrin, chlordane, and heptachlor epoxide, found, respectively, in 21, 9, 8, and 7 percent of all soil samples. Crop samples were collected from 727 sites. All were analyzed for organochlorines; analyses were performed for organophosphates and atrazine only when pesticide application data indicated current-year use. For all crops, 40 percent of the samples contained detectable levels of organochlorines and 10 percent contained detectable levels of organophosphates. Atrazine was not detected.

    Title Pesticide Residue Levels in Soils and Crops, 1971--national Soils Monitoring Program (iii).
    Date April 1979
    Journal Pesticides Monitoring Journal
    Excerpt

    Data from the 1971 National Soils Monitoring Program are summarized. Composite samples of soil and mature crops were scheduled for collection from 1,533 4-hectare sites in 37 states. Analyses were performed on 1,486 soil samples for organochlorines, organophosphates, PCBs, and elemental arsenic; samples were analyzed for atrazine only when pesticide application data indicated current-year use. Organochlorine pesticides were detected in 45 percent of the soil samples in the following order of frequency: dieldrin, sigmaDDT, aldrin, chlordane, and heptachlor epoxide. Most pesticide levels ranged from 0.01 to 0.25 ppm. Crop samples were collected from 729 sites, and all were analyzed for organochlorines. Crop samples were analyzed for organophosphates and atrazine only when pesticide application data indicated current-year use. Organochlorines were detected in 42 percent of the crop samples analyzed, organophosphates in 13 percent, and atrazine in 1 percent.

    Title Icbm - a Solution to the Problem of Informed Consent.
    Date March 1978
    Journal Journal of Clinical Engineering
    Title Pesticide Levels in Hay and Soils from Nine States, 1971.
    Date February 1977
    Journal Pesticides Monitoring Journal
    Excerpt

    In 1971 hay and soil samples were collected in 9 States to determine the incidence and levels of pesticide residues in hayfields. Residues were detected in 8 percent of the soil samples and 29 percent of the hay samples. DDT and its metabolites, DDE and TDE, were contained in 2 soil samples and 21 hay samples. Heptachlor epoxide and chlordane were detected in 1 soil sample, dieldrin in 5 soil samples, and diazinon in 4 hay samples.

    Title Organochlorine Pesticide Residues in Sugarbeet Pulps and Molasses from 16 States, 1971.
    Date February 1977
    Journal Pesticides Monitoring Journal
    Excerpt

    Sugarbeet pulp and molasses from 57 processing plants in 16 States were sampled for pesticide residues. No molasses samples contained detectable pesticide residues, but about 15 percent of the pulp samples contained low levels of dieldrin, toxaphene, or DDT and its degradation products. Sugarbeet pulp, when used as animal feed, can be a source of pesticidal contamination of human food.

    Title The Computer As an Unbiased Medical Investigator. Experience on an Active Surgical Service.
    Date October 1976
    Journal American Journal of Surgery
    Excerpt

    An adaptable on-line computer system for entry, retrieval, and analysis of medical discharge summaries has been developed and applied in the Trauma Service of a busy city hospital. Each summary occupied 5 to 10 minutes of the physician's time and compared favorably in cost to the standard dictated summary. While the average dictated summary contained 15 +/- 7 relevant facts, the machine-generated summaries were found to have 53 +/- 20 relevant facts. The summaries are well organized, easily comprehensible, and a duplicate copy can be obtained at any time by using the patient's name or number for identification. To date, 495 discharge summaries have been entered and reside in the memory of the computer. Statistical analysis from the data base is done by the computer, thus eliminating human prejudice. It provides facility for rapid and accurate retrospective studies as well as pattern-of-care and individual case review.

    Title Recurrence of Group B Streptococcal Infection.
    Date September 1976
    Journal The Journal of Pediatrics
    Excerpt

    It is clear that the group B streptococcus has become a major pathogen of young infants within the comparatively recent past. Further it is clear that, as with other endemic and epidemic pathogens, increasing clinical and laboratory experience brings to light variations not initially evident. In addition, therapy presumably effective in initial cases may not continue to be so. The following papers by coincidence were received over a relatively brief period of time. Hence, for emphasis of some of the problems related to this organism, they are presented as a group.

    Title Pesticide Residue Levels in Soils and Crops, Fy-70--national Soils Monitoring Program (ii).
    Date April 1975
    Journal Pesticides Monitoring Journal
    Title Pesticide Residues in Onions and Soil--1969.
    Date July 1974
    Journal Pesticides Monitoring Journal
    Title Pesticides in Soil: Organochlorine Pesticide Residues in Soils and Crops of the Corn Belt Region, United States--1970.
    Date October 1973
    Journal Pesticides Monitoring Journal
    Title An Improved Gas Chromatographic Method for the Analysis of 2,4-d Free Acid in Soil.
    Date March 1971
    Journal Journal of Agricultural and Food Chemistry
    Title Social Outcome of Childhood Epilepsy: Associations and Mechanisms
    Date
    Journal Seminars in Pediatric Neurology
    Excerpt

    Children with epilepsy may have serious social and academic problems. It appears simplistic to ascribe these problems to the seizures, medication, or accompanying cognitive deficits. Medications and seizures appear to play a minor role. Accompanying cognitive deficits may have a major effect on behavior and academic function. However, it is likely that the dominant influences for most children with epilepsy are from family, socioeconomic, and cultural factors.

    Title Does One More Medication Help? Effect of Adding Another Anticonvulsant in Childhood Epilepsy.
    Date
    Journal Journal of Child Neurology
    Excerpt

    Objectives: To study adding an anticonvulsant in children with uncontrolled epilepsy on ≥1 appropriate anticonvulsants. Methods: Chart review, patients with intractable epilepsy in a neurology clinic July 1, 2004 to December 31, 2007. Inclusion: Children on ≥1 stable anticonvulsant who had a second, third, or fourth anticonvulsant added. Exclusions: Noncompliance, subtherapeutic doses, and/or serum anticonvulsant levels, inappropriate anticonvulsant for seizure type, inadequate documentation, infantile spasms, or significant dosage changes in the baseline anticonvulsant(s) over the follow-up period. Patients were followed until further therapeutic changes occurred or September 30, 2008, whichever came first. Outcome: ≥50% decrease in seizure frequency. Results: Charts reviewed: 1886. Patients who met criteria: 84. Time to assessment: 4 weeks to 42 months (median = 7 months). ≥50% reduction in seizure frequency: 35 of 52 patients with second agent added; 5 of 30 patients with third agent added (P = .0001). Conclusions: Worthwhile seizure reduction is reasonably likely with the addition of a second anticonvulsant, but much less likely with the addition of third anticonvulsant.

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