bluuebunny/crossref_metadata_2025 huggingface dataset:
Ischemic Stroke in the Young: Evaluation and Age Comparison of Patients Six Months to Thirty-nine Years
Authors: Kerr Lynne M. , Anderson Deborah M. , Thompson Joel A. , Lyver Susan M. , Call Gregory K.
Published: December 2008

Ischemic stroke in the young is uncommon, but we currently evaluate at least one young stroke patient at our institutions each week. We undertook this chart review of strokes in patients between the ages of 6 months and 39 years to review all conditions associated with, and thus possibly contributory to, the stroke. We also compare younger and older age groups to observe if age-dependent factors exist. Of 100 total ischemic strokes, 22 were in persons 6 months to 18 years and 78 were in persons 19 to 39 years. Seventy-five percent of strokes were associated with a condition known or postulated to increase stroke risk. Some of these conditions are well accepted as causes for stroke, such as some forms of heart disease, whereas others are only postulated, such as the hypercoagulable states. Taken as a whole, associated conditions were approximately equally divided between infectious/inflammatory, structural, and presumed hypercoagulable conditions. Strokes in the first two decades of life were more commonly associated with infectious/inflammatory conditions, whereas strokes in the next two decades more commonly had structural or presumed hypercoagulable associated conditions. Since many strokes remain unexplained, it would be valuable to determine the significance, if any, of conditions less well known as risk factors for stroke. (J Child Neurol 1993;8:266-270).
Tests for Juvenile Myasthenia Gravis: Comparative Diagnostic Yield and Prediction of Outcome
Authors: Afifi Adel K. , Bell William E.
Published: December 2008

The clinical picture, family history, laboratory data, treatment modalities, and outcome in 27 juvenile myasthenics seen over a 25-year period are presented. Onset was after 10 years of age in the majority of patients. Half presented with ocular signs, the other half with generalized-onset myasthenia. Half of those with ocular-onset myasthenia progressed to generalized myasthenia. The female-to-male ratio was 3.5:1. Myasthenia gravis was reported in the mother of one patient. Ptosis was the most common presenting sign. It was unilateral at onset in 33% of patients and remained unilateral in 11%. Pharmacologic tests (neostigmine methylsulfate and edrophonium chloride) were positive in 92% of patients. Serology was positive in 63%, whereas repetitive nerve stimulation was positive in 33% when distal nerves were stimulated and in 66% when proximal and distal nerves were stimulated. Seropositivity tended to increase with generalization of the myasthenic process. No statistically significant difference in seropositivity was noted between males and females. Anti-striated muscle antibodies were detected in two patients, neither of whom had thymoma. The yield of repetitive stimulation increased with generalization of the myasthenic process and when proximal nerves were stimulated. No statistically significant difference was noted in the decremental response between seropositive and seronegative patients. The majority of ocular myasthenics were treated with pyridostigmine bromide monotherapy. With generalization of the myasthenic process, other modes of medical and surgical therapies were needed. All patients given corticosteroids ultimately underwent thymectomy. The mean age at presentation and the duration of symptoms at presentation were longer in thymectomized patients than in those without thymectomy. In thymectomized patients, those with thymic hyperplasia were all seropositive. A higher percentage of patients with normal thymus were in remission without medications than those with hyperplastic thymus. The English language literature on juvenile myasthenia is reviewed and compared with our data. The small number of patients and tests in this study preclude their use to demonstrate statistical significance or test statistical hypotheses. (J Child Neurol 1993;8:403-411).
Topical Review: Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, and Strokelike Episodes (MELAS): Current Concepts
Authors: Hirano Michio , Pavlakis Steven G.
Published: December 2008

Mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes (MELAS) syndrome is one of many mitochondrially inherited multisystem diseases. The features of 110 reported mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes patients are reviewed to define the clinical spectrum of this disease. The clinical disorder, in addition to emerging concepts of genetic etiology, is promoting our understanding of mitochondrial functions. New knowledge may lead to more rational therapies. Finally, the recent revolution in the study of mitochondrial diseases may further our understanding of other degenerative disorders and even aging. (J Child Neurol 1994;9:04-13).
Elevated Biopterin and Homovanillic Acid Levels in Cerebrospinal Fluid From Children With Aseptic Meningitis
Authors: Komori Hironori , Matsuishi Toyojiro , Yamada Shigeto , Ichikawa Kotaro , Amamoto Masano , Yamashita Fumio
Published: December 2008

To examine biopterin fractions and biogenic amine metabolites in cerebrospinal fluid in aseptic meningitis, the concentrations of homovanillic acid, 5-hydroxyindoleacetic acid, and the total, the oxidized form, and the reduced form of biopterin were determined in cerebrospinal fluid specimens from 15 children with aseptic meningitis in the acute phase, 15 children with aseptic meningitis in the recovery phase, and six other children as controls. The concentration of each substance was significantly higher in the acute phase than in the recovery phase. Homovanillic acid in the acute phase was significantly increased compared to that in the control group. The concentrations of the total, the oxidized form, and the reduced form of biopterin, and 5-hydroxyindoleacetic acid were higher in the acute phase than those in the controls; however, the differences were not significant. The concentration of each substance in the recovery phase was not significantly different from that in the controls. There was no difference in the 5-hydroxyindoleacetic acid/homovanillic acid ratio or in the reduced form/total biopterin ratio among the patients in acute and recovery phases and the controls. These results suggested that levels of biopterin and biogenic amine metabolites in cerebrospinal fluid are increased in the acute phase of aseptic meningitis and return to normal during the recovery phase. This is the first report of increased concentrations of biopterin fractions and biogenic amine metabolites in aseptic meningitis. (J Child Neurol 1994;9:22-25).
Abnormal Carbohydrate Metabolism in Cerebrospinal Fluid in Rett Syndrome
Authors: Matsuishi Toyojiro , Urabe Fujiko , Percy Alan K. , Komori Hironori , Yamashita Yushiro , Schultz Rebecca S. , Ohtani Yasuyo , Kuriya Norikazu , Kato Hirohisa
Published: December 2008

We analyzed lactate, pyruvate, and citric acid cycle intermediates in cerebrospinal fluid by high-performance liquid chromatography in Rett syndrome patients (n = 27; mean age, 5.7 ± 3.4 years) and age-matched female controls (n = 12; mean age, 7.0 ± 3.3 years). The lactate, pyruvate, α-ketoglutarate, and malate were significantly elevated in Rett syndrome compared to the controls. The lactate/pyruvate ratio was not different. On the other hand, cerebrospinal fluid citrate, cisaconitate, succinate, fumarate, and oxaloacetate were not significantly different in Rett syndrome patients than in the controls. We also evaluated the correlation between these acids and clinical symptoms and signs, including clinical stage, seizures, medications (anticonvulsants or naltrexone), developmental quotient, self-abuse, and hyperventilation or apnea or both. The concentrations of all these acids did not differ significantly with clinical stage. Lactate elevation significantly correlated with apnea. Lactate and pyruvate elevation significantly correlated with hyperventilation or with both breathing abnormalities. Our observations in this sample of patients with Rett syndrome led us to speculate that patients with the Rett syndrome may have defective carbohydrate metabolism. Elevated mitochondrial reduced nicotinamide-adenine dinucleotide-linked substrates suggest that reduced nicotinamide-adenine dinucleotide-ubiquinone oxidoreductase may be deficient in the brain in Rett syndrome patients. (J Child Neurol 1994;9:26-30).
Cerebral White-Matter Changes Suggesting Leukodystrophy in Ataxia Telangiectasia
Authors: Chung E.O. , Bodensteiner J.B. , Noorani P.A. , Schochet S.S.
Published: December 2008

Ataxia telangiectasia is an autosomal recessive disorder characterized by progressive cerebellar ataxia, recurrent sinopulmonary infections, oculocutaneous telangiectasia, selective immunoglobulin deficiency, and defective cellular immunity. We report a 4-year-old girl with ataxia telangiectasia whose initial magnetic resonance imaging (MRI) scan at 17 months of age showed leukoencephalopathy compatible with a leukodystrophy, a neuroimaging feature of ataxia telangiectasia that has not been described. Ataxia telangiectasia was not suspected until the child developed more typical clinical features. Diffuse white-matter high signal intensity on T2-weighted MRI scans may occur in the early stages of ataxia telangiectasia. This disease should be considered in the differential diagnosis of any child with a history and MRI findings suggestive of one of the leukodystrophies. The nonneurologic manifestations of ataxia telangiectasia may be of help diagnostically in this clinical setting. (J Child Neurol 1994;9:31-35).
Valproate-Associated Acute Pancreatitis in a Child With Neuronal Ceroid Lipofuscinosis
Authors: Talwar Dinesh
Published: December 2008

An 11-year-old girl with juvenile neuronal ceroid lipofuscinosis developed acute pancreatitis secondary to valproic acid treatment, which resolved after discontinuation of the medication and conservative treatment. To my knowledge, this is the first reported case of neuronal ceroid lipofuscinosis with valproate-associated pancreatitis. Children with neuronal ceroid lipofuscinosis may be susceptible to developing this complication. (J Child Neurol 1994;9:36-37).
Magnetic Resonance Imaging of Postmortem Infant Brains
Authors: Lamont Phillipa , Sachinwalla Toos , Pamphlett Roger
Published: December 2008

In order to obtain postmortem magnetic resonance images of infant brains, the formalin-fixed brains of 37 infants, ranging in age from 17 days to 17 months, were studied. Factors that improved scan resolution included formalin fixation, not washing the formalin from the brain, imaging the brain with a thin film of water over it, and the use of an extremity coil. The scan resolution was of sufficient quality to direct the histopathologist to regions requiring microscopic examination and to map the progress of myelination. (J Child Neurol 1994;9:59-62).
Topical Review: Putative Neurotransmitter Abnormalities in Infantile Spasms: Cerebrospinal Fluid Neurochemistry and Drug Effects
Authors: Pranzatelli Michael R.
Published: December 2008

The neuropharmacologic basis of infantile spasms and the mechanism by which adrenocorticotropic hormone (ACTH) exerts its therapeutic effects are unknown. This is a critical review of cerebrospinal fluid neurotransmitters or their metabolites in infantile spasms before and during treatment with ACTH, and of clinical drug trials with drugs acting on neurotransmission. Cerebrospinal fluid studies have shown lower γ-aminobutyric acid (GABA), ACTH, and 5-hydroxyindoleacetic acid concentrations in patients with infantile spasms compared to controls, elevated lysine and glutamate, variable or no differences in homovanillic acid, 3-methoxy-4-hydroxyphenylglycol, norepinephrine, corticotropin-releasing hormone, and β-endorphin. Chronic treatment with ACTH in infantile spasms reduces cerebrospinal fluid GABA, β-endorphin, and somatostatin, increases norepinephrine and tyrosine, and has variable or no effect on homovanillic acid, 3-methoxy-4-hydroxyphenylglycol, 5-hydroxyindoleacetic acid, histamine, and tryptophan. Small therapeutic trials with drugs that act through different neurotransmitters such as methysergide, α-methylparatyrosine, various benzodiazepine agonists, and vigabatrin lend some support to a role for GABA and monoamines in infantile spasms. These data, though promising, provide only a hint of potential neurotransmitter disturbances, and more basic and clinical data are needed. ( J Child Neurol 1994;9:119-129).
Childhood Pseudotumor Cerebri: Clinical and Intracranial Pressure Response to Acetazolamide and Furosemide Treatment in a Case Series
Authors: Schoeman Johan F.
Published: December 2008

The purpose of this study was to investigate the efficacy of combined therapy with acetazolamide and furosemide in normalizing intracranial pressure in children with pseudotumor cerebri. The role of repeated lumbar cerebrospinal fluid pressure monitoring in evaluating the response to therapy is also demonstrated. Continuous 1-hour lumbar cerebrospinal fluid pressure monitoring was done in eight children with pseudotumor cerebri on admission and at weekly intervals until the baseline pressure had normalized. (One child had two episodes of pseudotumor cerebri). All patients were treated with oral acetazolamide and furosemide until papilledema had cleared. Raised intracranial pressure was present on admission in all nine episodes of pseudotumor cerebri. Six children had an increased baseline cerebrospinal fluid pressure, whereas raised intracranial pressure was diagnosed in three children on account of an abnormal pulse wave and/or pressure waves. The mean baseline pressure was significantly lower after the 1st week of treatment than on admission ( P = .007) and normalized in all patients within 6 weeks of start of therapy. All children had a rapid clinical response. Combined therapy with acetazolamide and furosemide is an effective first-line method of treating raised intracranial pressure in children with pseudotumor cerebri. The good correlation found between the clinical response and normalization of baseline cerebrospinal fluid pressure suggests that clinical monitoring of treatment is adequate in most children with this condition. ( J Child Neurol 1994;9:130-134).
Focal Cerebral Metabolic Abnormality in a Patient With Continuous Spike Waves During Slow-Wave Sleep
Authors: Park Yong D. , Hoffman John M. , Radtke Rodney A. , DeLong G. Robert
Published: December 2008

We report an 11-year-old boy with continuous spike-wave discharges during sleep accompanied by partial motor and atypical absence seizures, psychomotor regression, and severe behavior problems. During wakefulness, epileptiform discharges occurred over the right parietal region, suggesting that the continuous spike-wave discharges during sleep were a manifestation of secondary bilateral synchrony. Bilateral suppression of the spike-and-wave activity was observed after right-sided intracarotid amobarbital injection, further supporting the impression of secondary bilateral synchrony. The right superior temporoparietal increase in metabolic activity during continuous spike-wave discharges and noncontinuous spike-wave discharges was seen on [18F]fluorodeoxyglucose positron emission tomography and supports a right temporoparietal focus in our case. The presence of a focal abnormality suggests that surgical therapy may be effective. ( J Child Neurol 1994;9:139-143).
Idiopathic Intracranial Hypertension in Children: The Iowa Experience
Authors: Babikian Paul , Corbett James , Bell William
Published: December 2008

Idiopathic intracranial hypertension is an uncommon condition in children and is seen only about once a year in a large referral hospital. Sex incidence is equal, and obesity is a feature in about one third of patients. This article reviews the 30-year experience in an institution where idiopathic intracranial hypertension in adults has been extensively studied. Symptomatic causes of intracranial hypertension and both medical and surgical therapeutic options are reviewed. ( J Child Neurol 1994;9:144-149).
Dystrophin and DNA Diagnosis in a Large Pediatric Muscle Clinic
Authors: Richards Sue , Iannaccone Susan T.
Published: December 2008

Eighty-seven unrelated patients from a large muscle clinic setting were analyzed by DNA for deletions in the dystrophin gene for diagnosis of Duchenne/Becker muscular dystrophy. The clinical phenotype of the patient population included 72% Duchenne, 13% Becker, and 15% outlier patients. Dystrophin gene deletions were detected in 61% of these patients, and disease phenotype was predicted by DNA with an accuracy of 95%. While DNA did not confirm diagnosis in all patients, dystrophin analysis of muscle biopsies, when available, predicted a disease phenotype. In the 66 patients in which muscle biopsies were available for analysis, the results of the dystrophin analysis agreed with actual clinical phenotype with 86% accuracy. Less agreement between dystrophin and clinical phenotype predictions were found in the Becker patient population. We suggest that, in at least 61% of Duchenne/Becker patients, DNA analysis provides a rapid and accurate diagnosis. DNA is less invasive and less expensive than biopsy and may allow family risk assessment. Therefore, DNA analysis may become the first recommended laboratory procedure for Duchenne/Becker diagnosis, and muscle biopsy with dystrophin analysis may become necessary only for those patients with undetectable gene mutations. ( J Child Neurol 1994;9:162-166).
Maternal-Fetal HLA Sharing and Risk of Newborn Encephalopathy and Seizures: A Pilot Study
Authors: Cowan Linda D. , Hudson Leslie , Bobele Gary , Chancellor Isabel , Baker Judy
Published: December 2008

A pilot case-control study was done to collect data on whether susceptibility to newborn encephalopathy and neonatal seizures is influenced by the degree of maternal-fetal sharing of HLA antigens. Cases included 13 infants with moderate or severe newborn encephalopathy and seven infants with neonatal seizures but no other signs of encephalopathy. Controls were neurologically normal infants matched to cases by date of birth, sex, race, and payment status. Infants and their mothers were typed for HLA-A, -B, -DR, and -DQ antigens. The observed frequency of sharing of maternal antigens was greater than expected (ie, 0.5) for cases compared to controls at the HLA-B, -DR, and -DQ loci but not for HLA-A. The risk of neurologic problems in the neonatal period was increased 6.3 times when there was more than one match at the HLA-DR or -DQ locus. Placental abnormalities were noted at delivery only among cases, and the mean placental weight in cases was 598 g versus 695 g in controls. Further studies with sample sizes sufficiently large to statistically test this hypothesis are needed. (J Child Neurol 1994;9:173-177).
Immunoglobulins in the Treatment of Guillain- Barré Syndrome in Early Childhood
Authors: Al-Qudah Abdelkarim A.
Published: December 2008

This is a prospective study on the use of immunoglobulins in the treatment of Guillain-Barré syndrome in four children, three of whom were younger than 3 years of age. All of them were unable to walk and were still deteriorating when the treatment was started. Three patients started recovering within 24 hours, and all the patients were fully mobile within 6 weeks after receiving first dose of immunoglobulins. This report shows the efficacy of intravenous immunoglobulins in the treatment of young children presenting with acute Guillain-Barré syndrome. (J Child Neurol 1994;9:178-180).
Cocaine Metabolites in the Neonate: Potential for Toxicity
Authors: Konkol Richard J. , Murphey Laine J. , Ferriero Donna M. , Dempsey Delia A. , Olsen George D.
Published: December 2008

Recent reports indicate that cocaine metabolites have biologic activity and could be toxic. To explore this possibility, two studies were initiated. The first study aimed to define the distribution of cocaine species by quantifying levels of cocaine and its metabolites norcocaine, benzoylecgonine, and benzoylnorecgonine in newborn cord blood and meconium. The second study sought to determine whether they produced a clinical effect. Compared to cord blood, meconium had a greater number of metabolites and a higher concentration of cocaine and cocaine metabolites, including the previously undetectable norcocaine and benzoylnorecgonine derivatives. Benzoylecgonine was the most common species found in both sources and was usually lower in concentration in blood. An inverse relation existed between meconium benzoylecgonine levels and the serum catabolic enzyme pseudocholinesterase, implying genetic variability in cocaine metabolism. To determine whether cocaine and/or its metabolites could be linked to a distinct clinical state, a second study focusing on newborn behavior was performed with an independent large cohort of cocaine-exposed infants. Neonates with increased signs of "neuroexcitation" had benzoylecgonine and no cocaine in urine, whereas lethargic neonates had detectable urinary cocaine. These findings support the hypothesis that cocaine metabolites, especially benzoylecgonine, may play a role in altering newborn behavior and produce a clinical syndrome distinct from that related to the parent compound. (J Child Neurol 1994;9:242-248).
Cardiac and Central Nervous System Vasculitis in a Child With Dermatomyositis
Authors: Jimenez Cita , Rowe Peter C. , Keene Daniel
Published: December 2008

Cerebral vasculitis and clinically important myocardial inflammation are rare in juvenile dermatomyositis. We report a previously healthy 6-year-old girl with dermatomyositis who died after a fulminating clinical deterioration. Postmortem examination of the heart revealed characteristic endothelial tubuloreticular aggregates and evidence of capillary necrosis and secondary thrombosis, associated with extensive hemorrhagic myocardial necrosis. Endothelial necrosis was also evident in the cerebrocortical capillaries. (J Child Neurol 1994;9:297-300).
Use of a Calcium Channel Blocker (Nicardipine HCl) in the Treatment of Childhood Moyamoya Disease
Authors: Hosain Syed A. , Hughes John T. , Forem Sandra L. , Wisoff Jeffrey , Fish Irving
Published: December 2008

Moyamoya disease is a cerebrovascular disease characterized radiologically by progressive narrowing and occlusion of the arteries contributing to the circle of Willis and its branches. There is formation of an exuberant collateral network of blood vessels at the base of the brain, which is thought to arise in response to chronic ischemia. Clinically, the course is variable, with patients having repeated transient ischemic attacks, strokes, migraine, and seizures. Effective treatment is not available. The etiology and pathophysiology of moyamoya disease are largely unknown. Two patients with arteriographically proven moyamoya disease were identified. Both patients were symptomatic before age 5 years. Despite successful encephaloduroarteriosynangiosis revascularization procedures, they continued to experience an inexorable downhill course. A calcium channel blocker (nicardipine HCl) was introduced in order to prevent further symptoms. After the introduction of nicardipine, no further strokes occurred in either patient. There were no further episodes of transient ischemic attacks, seizures, or headache in one patient and decreased frequency in the other. In patients with moyamoya disease, nicardipine may have a beneficial effect on cerebral hemodynamics and may prevent ischemic sequelae by optimizing existing collateral circulation. (J Child Neurol 1994;9:378-380).
Long-Term MRI Changes in Brain After Pediatric Open Heart Surgery
Authors: Miller Geoffrey , Mamourian Alexander C. , Tesman Johanna R. , Baylen Barry G. , Myers John L.
Published: December 2008

We performed magnetic resonance imaging (MRI) on the brain and neurologic examinations on 23 children after open heart surgery for congenital heart disease. Twenty children also had psychometric assessments. Examinations were performed at a mean age of 66 months (range, 26 to 180 months). Age at operation was less than 1 month in 43% and more than 6 months in 45%. Abnormal scans were found in 17 (74%) and showed diffuse findings consistent with hypoxic-ischemic encephalopathy, with or without areas of cortical infarction; focal cortical infarction alone; and (in one patient) callosal agenesis and abnormal neuronal migration. Normal IQ and neurologic examinations were found in all six of those who had a normal MRI, and five of six children with changes consistent with focal cortical infarction without diffuse change had a normal neurologic examination. Cerebral palsy and mental retardation was common in the group with diffuse abnormality (in eight of nine children), and this was more likely to occur in those who underwent prolonged (> 45 minutes) hypothermic circulatory arrest and operation during early infancy (P = .004). Focal cortical findings without diffuse changes were more likely in those who underwent open heart surgery without hypothermic circulatory arrest and were older than 6 months at operation, and these children were less likely to have frank neurodevelopmental sequelae. Thus, in our population, focal cortical lesions were common after open heart surgery, and, in addition, diffuse brain abnormality on MRI plus neurologic sequelae were common after prolonged hypothermic circulatory arrest. (J Child Neurol 1994;9:390-397).
Atelencephalic Aprosencephaly
Authors: Harris Cheryl P. , Townsend Jeannette J. , Norman Margaret G. , White Valerie A. , Viskochil David H. , Pysher Theodore J. , Klatt Edward C.
Published: December 2008

Absence of the telencephalon and diencephalon characterizes the syndrome of aprosencephaly, while in atelencephaly, only the telencephalon is absent. Atelencephalic aprosencephaly is characterized by the presence of at least a rudimentary diencephalon. Embryologically, aprosencephaly is thought to occur after the optic vesicles form but before the cerebral vesicles appear. The syndrome is quite rare, with only 10 cases previously reported. We describe two fetuses with atelencephalic aprosencephaly. A 25-week estimated gestational age fetus was born to first-cousin parents and had a prenatal ultrasonographic diagnosis of anencephaly. The second, a 19-week estimated gestational age fetus, was thought to have semilobar holoprosencephaly by prenatal ultrasound. At autopsy, neuropathologic examination in both cases showed virtual absence of the cerebral hemispheres with an incomplete diencephalon. Microscopic examination in one case revealed disorganized neuropil with a proliferative vasculopathy. The optic globes were completely formed and attached to hypoplastic optic nerves, but retinal dysplasia was apparent histologically in both cases, and bilateral colobomata were present in one case. The findings in these cases demonstrate a spectrum of congenital variations that lie between the syndromes of atelencephaly and aprosencephaly, underscoring the complexity of the congenital anomalies. ( J Child Neurol 1994;9:412-416).
The Treatment of Infantile Spasms by Child Neurologists
Authors: Bobele Gary B. , Bodensteiner John B.
Published: December 2008

Infantile spasms is a refractory seizure disorder for which a number of different treatment regimens are available. No information is available on which treatment regimens are most widely used and which would be of practical importance in designing clinical trials to determine efficacy of new treatments. We proceeded to gather data on the most commonly employed methods of treating infantile spasms. A survey was mailed in 1991 to all junior, active, and emeritus members of the Child Neurology Society asking details about the treatment of infantile spasms. Telephone follow-up on a random sample of nonresponders was made. The total response rate was 58.3%. Most respondents who treat infantile spasms use corticotropin (ACTH) as their drug of first choice (88%). The most frequently used dosage was 40 IU per day, and the most frequent duration of treatment was 1 to 2 months. The most frequently reported side effects of ACTH or oral corticosteroid treatment were cushingism, behavior changes or irritability, hypertension, topical infections, and systemic infections. Of those not using ACTH, valproic acid was the next most commonly employed agent, followed by oral corticosteroids. The results were similar for all respondents regardless of age, sex, type of practice, number of cases of infantile spasms seen, location (United States or abroad) or whether the survey was completed by mail or telephone. These data suggest that there is a relative uniformity among child neurologists in the management of infantile spasms despite the publication of many alternative treatment strategies. (J Child Neurol 1994;9:432-435).
Topical Review: Parasitic Infections of the Central Nervous System in Children. Part I: Congenital Infections and Meningoencephalitis
Authors: Lowichik Amy , Siegel Jane D.
Published: December 2008

This review article presents the epidemiology, pathogenesis, clinical presentation, laboratory and radiologic findings, and treatment of parasitic infections of the central nervous system in children. Some obscure parasitic infections are included. To assist the clinician faced with a specific case, infections are categorized first by predominant clinical manifestations : congenital disease, meningoencephalitis, focal lesions, and disseminated multisystem disease. Within the clinical categories, parasites are grouped according to the geographic area where most human infections occur. Congenital infections and those that present most frequently as meningoencephalitis are discussed in this part of the review. ( J Child Neurol 1995; 10:4-17).
Acute Basal Ganglia Infarction in Propionic Acidemia
Authors: Haas Richard H. , Marsden Deborah L. , Capistrano-Estrada Sylvia , Hamilton Ronald , Grafe Marjorie R. , Wong Wade , Nyhan William L.
Published: December 2008

An 8-year-old girl with propionic acidemia had acute and rapidly fatal symmetric necrosis of the caudate, globus pallidus, and putamen. Clinical presentation was with acute aphasia, generalized hypotonia, and muscle weakness. There was no evidence of metabolic decompensation, and analysis of the organic acids of the urine indicated good metabolic control. Organic acids in the cerebrospinal fluid were unremarkable. These observations indicate that the pathophysiology of "metabolic stroke" is more complicated than previously thought. (J Child Neurol 1995;10:18-22).
Does Iron Deficiency Raise the Seizure Threshold?
Authors: Kobrinsky Nathan L. , Yager Jerome Y. , Cheang Mary S. , Yatscoff Randall W. , Tenenbein Milton
Published: December 2008

To determine the effect of iron status on the seizure threshold, measures of iron sufficiency were prospectively evaluated in 51 children presenting to a pediatric emergency department with a febrile illness with (26) or without (25) an associated febrile seizure. A higher proportion of children from the febrile seizure group had a family history of mental retardation (5/26 versus 0/25, P = .02) or of previous febrile seizures (10/26 versus 2/23, P = .01). The two groups were otherwise comparable for age, sex, race, family history of afebrile seizures, temperature at presentation, white blood cell count, differential, and vitamin and antibiotic use. Patients with febrile seizures were less frequently iron deficient as defined by a free erythrocyte protoporphyrin level above 0.80 ng/L (2/23 versus 10/25, P < .01), hemoglobin concentration less than 110 g/L (1/26 versus 6/25, P < .03), hematocrit less than 0.30 L/L (0/22 versus 4/25, P < .02), mean corpuscular hemoglobin less than 20 pg (0/25 versus 3/24, P < .04), mean corpuscular volume less than 65 fL (0/26 versus 4/24, P < .02), and platelet count higher than 550 x 109/L (0/26 versus 3/25, P < .04). This association was even stronger when adjusted for differences in family history. None of the patients in the febrile seizure group was being treated for iron deficiency at presentation, whereas three of 25 controls used an iron supplement (P < .04). Iron deficiency may protect against the development of febrile seizures. (J Child Neurol 1995;10:105-109).
Replacing Carbamazepine Slow-Release Tablets With Carbamazepine Suppositories: A Pharmacokinetic and Clinical Study in Children With Epilepsy
Authors: Arvidsson Jan , Nilsson Heimo L. , Sandstedt Per , Steinwall Gunilla , Tonnby Bernt , Flesch Gerard
Published: December 2008

A suppository for rectal administration of carbamazepine has been developed for situations in which it is unsuitable to use the oral route of administration. In an open, controlled, within-patient study, the pharmacokinetics, clinical efficacy, and tolerability of carbamazepine slow-release tablets were compared with those of carbamazepine suppositories in children with epilepsy. The pharmacokinetic part of the study comprised 22 children, and an additional nine children were included in the clinical part of the study. Treatment with slow-release tablets was replaced for 7 days with carbamazepine suppositories in bioequivalent dosage. Clinical factors such as the rate of seizures and the local tolerability were studied, and an overall assessment of efficacy was made. In the pharmacokinetic part, 24-hour plasma concentration curves for carbamazepine and carbamazepine-10,11-epoxide were recorded. The plasma concentration profiles (minimum, maximum, and mean concentrations, fluctuation index, and area under the curve) for carbamazepine and the other metabolites did not show any significant differences between oral and rectal administration when the suppository dose was increased by 25% compared to the tablets. No increase in seizure frequency was detected, and the overall assessment was very good to good in 25 of the 29 epileptic children. Increased flatulence during treatment with suppositories was noted in two children, one had anal irritation, and one had nausea/vomiting. Treatment with carbamazepine slow-release tablets in children with epilepsy can be replaced by carbamazepine suppositories in 25% higher dosage, with good clinical effect and appropriate pharmacokinetic values, when it is unsuitable to use the common oral route of administration. (J Child Neurol 1995; 10: 114-117).
Topical Review: Parasitic Infections of the Central Nervous System in Children. Part III: Space-Occupying Lesions
Authors: Lowichik Amy , Ruff Andrea J.
Published: December 2008

In the last part of this three-part review of parasitic infections of the central nervous system in children, we consider parasites which due to their size, distribution, or the nature of the host response, tend to cause focal lesions in the brain and spinal cord and therefore present as space-occupying lesions which occasionally mimic malignant tumors. As in Parts I and II, infections are grouped according to their predominant geographic area. Such infections include cysticercosis, one of the more common and important infections of the central nervous system. (J Child Neurol 1995; 10:177-190).
Glioneurocytoma: Tumor With Glial and Neuronal Differentiation
Authors: Min Kyung-Whan , Cashman Robert E. , Brumback Roger A.
Published: December 2008

We report two cases of low-grade glioma in which multiple cellular components, including cells with dense-core granules consistent with "neurocytes," were identified on electron microscopic studies. The first patient was an apparently normal boy until the onset of seizures at age 10 months. Initially, the seizures improved with phenobarbital treatment, but good seizure control was never achieved. Computed tomographic scan at age 23 months showed a calcified, nonenhancing left parietal mass. This tumor was composed of sheets of cells with clear cytoplasm and round to oval nuclei. Mucinous intercellular material stained positively with periodic acid-Schiff, mucicarmine, and alcian blue stains. Foci of calcification were evident. The second patient was a 13-year-old boy with a left parasagittal parieto-occipital mass who presented with a 4-month history of seizures and declining school performance. The tumor was composed of sheets of astrocytes with dark, hyperchromatic, pleomorphic nuclei in a fibrillary and microcystic background. The tumor contained the pleomorphism seen in the adult variant of pilocytic astrocytoma, as well as the microcystic component seen in the juvenile variety. Ultrastructurally in both cases, there were occasional tumor cells having round to oval nuclei with moderate amounts of cytoplasm containing 150- to 250-nm-diameter dense-core granules. These cells were admixed with the majority of tumor cells, which in case 1 had the ultrastructural features of astrocytes and oligodendrocytes and in case 2 had features of protoplasmic or pilocytic astrocytes. Our cases bear superficial resemblance to dysembryoplastic neuroepithelial tumors; however, dysplastic ganglion cells are an essential component for that diagnosis, and there were no dysplastic ganglion cells in either of our cases. Cells containing dense-core granules (neurocytes) were found in both our cases by electron microscopy and appear to be a part of a neoplastic process. The significance of neurocytes in low-grade gliomas is not known. Cerebral tumors consisting of neuroblasts/neurocytes in toto or in part have been increasingly recognized in recent years, and our cases add to the spectrum of such neoplasms. (J Child Neurol 1995;10:219-226).
Topical Review: Brain Abscess in Children: Microbiology and Management
Authors: Brook Itzhak
Published: December 2008

Brain abscess is a serious, life-threatening infection. The infection can originate from contiguous sites of existing infections, such as chronic otitis media, dental infection, mastoiditis, or sinusitis, where anaerobic bacteria predominate. The infection can also occur in children with cyanotic congenital heart disease, in whom the predominant organisms are viridans, microaerophilic, or anaerobic streptococci, or after head trauma, in which case Staphylococcus aureus, viridans cocci, and Streptococcus pneumoniae are the most prevalent isolates. Enterobacteriaceae, Pseudomonas aeruginosa, yeast, fungi, and mycobacteria are prevalent in the immunocompromised. Radioisotope brain scans, computed tomography, and magnetic resonance imaging are important tools that enable accurate diagnosis of the infection. Proper selection of antimicrobial with good intracranial penetration is essential in the management of intracranial infection. Delay in surgical drainage can be associated with high mortality or morbidity. However, brain abscess, especially in the early phase of cerebritis, may respond to antimicrobial therapy without surgical drainage. (J Child Neurol 1995;10:283-288).
Baclofen in the Treatment of Polymyoclonus and Ataxia in a Patient With Homocystinuria
Authors: Awaad Yasser , Sansaricq Claude , Moroney Joan , Fish Irving , Kyriakakos Anastassios , Snyderman Selma E.
Published: December 2008

A patient with homocystinuria due to cystathionine β-synthase deficiency developed severe progressive polymyoclonus and ataxia. To our knowledge, this is the first time polymyoclonus and ataxia have been reported in association with homocystinuria. Although cerebrovascular thrombosis is usually thought to be responsible for neurologic dysfunction in homocystinuric patients, no infarctions were demonstrated on magnetic resonance imaging scans in our case. We have previously reported that baclofen dramatically improved the polymyoclonus and ataxia in a patient with Unverricht-Lundborg disease. Baclofen given to our patient reversed the polymyoclonus and the ataxia as well. This suggests that patients with polymyoclonus and ataxia, no matter what the etiology, may benefit from the use of baclofen. (J Child Neurol 1995; 10:294-296).
The Determination of Sensory Deficits in Children With Hemiplegic Cerebral Palsy
Authors: Cooper Jasmine , Majnemer Annette , Rosenblatt Bernard , Birnbaum Rena
Published: December 2008

Therapeutic intervention for children with cerebral palsy begins with accurate appraisal of abilities and disabilities. Currently, treatment focuses on the identified motor deficits, with any underlying sensory deficits often being overlooked. Sensory input is an essential component of motor function and motor control. Therefore, the objective of this study was to determine the presence and extent of sensory deficits in school-aged hemiplegic children using a formal clinical sensory battery, as well as somatosensory evoked potentials. A cross-sectional comparative study was performed comparing sensory function in hemiplegic children and healthy controls. Nine hemiplegic children and 41 healthy controls between 4 and 19 years of age were assessed. Hemiplegic children were included if they had a minimum level of receptive language function of 30 to 33 months and expressive language ability of 24 to 27 months, and no severe limitation of joint range of the hand. Significant bilateral sensory deficits (88.8%) were ascertained in hemiplegic children (P < .05), when compared to the performance of the healthy controls. Stereognosis and proprioception were the chief modalities affected bilaterally. The extent of sensory loss did not mirror the severity of motor deficit. Conversely, findings on somatosensory evoked potentials were closely related to motor function. Thus, a clinical sensory evaluation should be an integral part of the assessment of children with cerebral palsy. The likelihood of sensory impairment in one or more modalities on the hemiplegic or nonhemiplegic side is underappreciated and needs to be identified by rehabilitation specialists to maximize the functional potential of these children. (J Child Neurol 1995; 10:300-309).
Serial CT Scanning in Childhood Tuberculous Meningitis: Prognostic Features in 198 Cases
Authors: Schoeman Johan F. , Van Zyl Lana E. , Laubscher Jacoba A. , Donald Peter R.
Published: December 2008

Serial cranial computed tomographic (CT) scanning and intracranial pressure monitoring were performed on 198 children with stage II and III tuberculous meningitis. The aims of the study were to document the course of tuberculous hydrocephalus during medical and surgical treatment, as well as the prognostic significance of parenchymal changes in the brain as demonstrated by CT. Lumbar cerebrospinal fluid pressure was monitored continuously for a 1-hour period in all patients on admission and at weekly intervals in patients with communicating hydrocephalus for the 1st month of treat ment. Cranial CT scanning was done on admission and repeated in survivors after 1 month and again after 6 months of antituberculous therapy. The raised intracranial pressure of 112 children with communicating hydrocephalus, as demonstrated by air-encephalography, was treated medically (with daily acetazolamide and furosemide) for 1 month. Thirty-one children with noncommunicating hydrocephalus were referred for immediate ventriculoperitoneal shunting. No significant difference was found in the eventual ventricular size or clinical outcome between the two treatment groups. Lumbar cerebrospinal fluid pressure changes in the children with communicating hydrocephalus closely followed changes in the degree of hydrocephalus during the course of treatment.The main cause of permanent neurologic disability was basal ganglia infarction, which occurred unilaterally in 21% and bilaterally in 10% of patients on admission and developed in a further 22% of children during treatment. A prominent subarachnoid space, which was seen on the CT scan of 36% of patients after the 1st month of treatment and which reverted to normal, probably relates to the poor nutritional state of these patients on admission. Serial CT scanning was found to be valuable in defining the respective roles of raised intracranial pressure and parenchymal disease in the outcome of tuberculous meningitis. (J Child Neurol 1995; 10:320-329).
Relapse of Herpes Simplex Encephalitis
Authors: Barthez-Carpentier Marie-Anne , Rozenberg Flore , Dussaix Elisabeth , Lebon Pierre , Goudeau Alain , Billard Catherine , Tardieu Marc
Published: December 2008

We report five children who had recurrent central nervous system signs after conventional acyclovir therapy for herpes simplex encephalitis. Secondary exacerbation was characterized clinically by severe ballismic movement disorder in all five children, associated with fever, impairment of consciousness, and seizures. Biologic analysis in all children and magnetic resonance imaging and neuropathology studies of the brain in three cases were compatible with inflammatory reaction. In contrast, all viral cultures remained negative, herpes simplex virus antigen in one child and DNA tested by polymerase chain reaction in four children were undetectable in the first samples of cerebrospinal fluid during the relapse, suggesting a postinfectious, immune-mediated mechanism of relapse in these patients. (J Child Neurol 1995;10:363-367).
Location of Epileptic Foci on Interictal and Immediate Postictal Single Photon Emission Tomography in Children With Localization-Related Epilepsy
Authors: Otsubo Hiroshi , Hwang Paul A. , Gilday David L. , Hoffman Harold J.
Published: December 2008

Several neuroimaging techniques that supplement electrophysiologic methods of evaluating pediatric patients with localization-related epilepsies before surgery assess both structural and functional abnormalities. For example, single photon emission computed tomography (SPECT) with technetium-99m-hexamethylpropyleneamine oxime 99mTc-HMPAO) has been used to demonstrate abnormal cerebral perfusion. States of cerebral perfusion during the interictal and immediate postictal periods have been reported to correlate with epileptiform foci identified by electroencephalogram (EEG). Between January 1987 and March 1993, we studied 55 pediatric patients with intractable seizures with prolonged video EEG telemetry in the epilepsy monitoring unit, followed by computed tomography, magnetic resonance imaging, and SPECT, before surgery to determine whether SPECT studies with 99mTc-HMPAO improved the accuracy of locating the epileptic focus. Interictal SPECT was performed on all patients, and immediate postictal SPECT (within 10 minutes after seizure ended) on 17 patients monitored in the epilepsy monitoring unit. In 15 (88%) of the 17, the combination of interictal and postictal SPECT studies yielded results corresponding to the EEG abnormality, a result significantly better than that obtained from interictal studies alone: 21 (55%) of 38 (χ2 = 5.647, P = .0175). SPECT scans showed localized abnormal perfusion in the ipsilateral temporal lobe in all six patients with mesial temporal sclerosis, but precise results were not obtained in cases of dual pathology and neuronal migration disorders. Depiction of cerebral perfusion by interictal and immediate postictal SPECT studies can lead to greater accuracy in the localization of epileptic foci. (J Child NeuroL 1995;10:375-381).
MRI Evaluation of the Brain in Infantile Neuronal Ceroid-Lipofuscinosis. Part 1: Postmortem MRI With Histopathologic Correlation
Authors: Vanhanen Sanna-Leena , Raininko Raili , Santavuori Pirkko , Autti Taina , Haltia Matti
Published: December 2008

The purpose of this study was to correlate postmortem magnetic resonance imaging (MRI) with histopathologic findings in brains of a series of autopsied patients with infantile neuronal ceroid-lipofuscinosis, a recessively inherited progressive encephalopathy. Eight formalin-fixed brains (age range at death, 7 to 13 years) were examined with MRI. One patient had also undergone brain MRI 2 years before death. Histopathologic analyses were made from standard areas selected on the basis of the MRI scans. Postmortem MRI findings did not differ significantly from the findings in the patient who was also examined during life. Typical findings were extreme cerebral atrophy and hypointensity of the gray-matter structures in relation to the white matter on T2-weighted images, a pattern the reverse of normal. Characteristic histologic findings were almost complete loss of cortical neurons and secondary loss of axons and myelin sheaths in the white matter. The drastically altered relative intensities of the gray- and white-matter structures on the MRI scans reflected replacement of the neurons with hypertrophic astrocytes and/or macrophages filled with storage material. (J Child Neurol 1995;10:438-443).