bluuebunny/crossref_metadata_2025 huggingface dataset:
Nitrazepam for Refractory Infantile Spasms and the Lennox-Gastaut Syndrome
Authors: Chamberlain Marc C.
Published: December 2008

Infantile spasms and the Lennox-Gastaut syndrome are considered to be age-specific pediatric epileptic syndromes and together constitute a significant percentage of medically resistant seizures in childhood. Twenty children, ages 4 to 28 months (median, 12 months), with medically refractory infantile spasms or the Lennox-Gastaut syndrome, were treated with the investigational benzodiazepine nitrazepam in an open-label study. Daily dosage of nitrazepam ranged from 0.5 to 3.5 mg/kg, with a median dosage of 1.5 mg/kg, divided into two doses per day. Side effects included pooling of oral secretions (12 children) and sedation (six children); however, no serious side effects were seen. Responses to nitrazepam were as follows: five complete responses (cessation of all seizures), seven partial responses (greater than 50% reduction of seizures), and eight with no response. Median duration of response was 9 months (range, 4 to 16 months) in children with infantile spasms and 14 months (range, 8 to 26 months) in children with the Lennox-Gastaut syndrome. Nitrazepam is an effective anticonvulsant in this small cohort of children with medically refractory infantile spasms and the Lennox-Gastaut syndrome, resulting in a 25% response rate and only modest side effects. (J Child Neurol 1996;11:31-34).
Combination Therapy of Infantile Spasms With High-Dose Pyridoxal Phosphate and Low-Dose Corticotropin
Authors: Takuma Yuichi , Sela Tohru
Published: December 2008

A new combination therapy, high-dose pyridoxal phosphate (40 to 50 mg/kg daily) and low-dose corticotropin (0.01 mg [0.4 IU]/kg daily), was tried in 28 children with infantile spasms. Monotherapy with pyridoxal phosphate provided excellent seizure control in three (11%) of the 28 subjects. Corticotropin was subsequently added to the regimen of the remaining 25 patients. At 1 month after discontinuing corticotropin, 21 (84%) of the 25 patients experienced no seizures, and 22 (88%) of the 25 showed improvement in their electroencephalographic findings. The mean interval until achievement of seizure control was 4.1 days after the initiation of corticotropin. The outcome in the 21 patients has been followed for a mean period of 34.9 months (range, 2 to 81 months). Of these 21 patients, six (29%) have had relapses of infantile spasms, and 10 (48%) have experienced normal development. Transient increases in liver enzymes occurred in 14 (50%) of the 28 patients, but none of the patients developed more serious side effects. The investigators conclude that combination therapy with high-dose pyridoxal phosphate and low-dose corticotropin is a promising new therapy. (J Child Neurol 1996;11:35-40).
Increased ALZ-50 Immunoreactivity in Sudden Infant Death Syndrome
Authors: Sparks D. Larry , Davis Daron G. , Mellert Bigelow Tuesday , Rasheed Karim , Landers Teresa M. , Liu Huiachen , Coyne Carolyn M. , Hunsaker John C.
Published: December 2008

Neuronal expression of the ALZ-50 epitope was investigated in hippocampus and medulla from infants dying of sudden infant death syndrome or known causes (controls). Hippocampal studies include data from 31 infants dying of known causes between 32 weeks' gestation and 16 months postpartum and 46 infants who died of sudden infant death syndrome. The medulla at the level of the mid olivary protuberance was investigated in 22 infants with sudden infant death syndrome and 11 controls matched for age and postmortem interval. Medullary sections were also examined using immunohistochemical methods to demonstrate reactivity to glial fibrillary acidic protein antibody. The density of ALZ-50-immunodecorated neurons in control hippocampus rises from the level observable in utero to a maximum between 1 and 4 months of age and declines thereafter. The density of ALZ-50-immunoreactive neurons in hippocampus is significantly increased in infants with sudden infant death syndrome at all ages. Significant regionally specific increases in the number of ALZ-50-immunoreactive neurons, and glial fibrillary acidic protein-reactive cells were found in sudden infant death syndrome medulla; coincidental increases were observed in only the solitary nucleus. Neurons exhibiting the ALZ-50 epitope may reflect apoptotic neuron death of normal development, and increased numbers of immunoreactive neurons may suggest enhanced neurodegeneration in sudden infant death syndrome. (J Child Neurol 1996; 11: 101-107).
Familial Dystonia and Choreoathetosis in Three Generations Associated With Bilateral Striatal Necrosis
Authors: Craver R.D. , Duncan M.C. , Nelson J.S.
Published: December 2008

Nine cases of dystonia and choreoathetosis (six females and three males) have developed in three generations of a single family. There has been one death. Neuropathologic examination disclosed bilateral striatal necrosis. In this family, the neurologic disorder has evolved gradually or in association with a febrile illness. There has been no neurologic recovery. The disease is worse in females, has been transmitted only through females, and shows incomplete penetrance and anticipation. The maternal inheritance pattern suggests either an autosomal dominant trait also affecting male reproductive ability or a defect involving the mitochondrial genome. (J Child Neurol 1996;11:185-188).
Low Levels of Nerve Growth Factor in Cerebrospinal Fluid of Children With Rett Syndrome
Authors: Lappalainen Raija , Lindholm Dan , Riikonen Raili
Published: December 2008

A role for neurotrophic factors has been postulated in some human neurodegenerative disorders such as Alzheimer's and Parkinson's diseases. The known developmental effects of these substances suggested that, in some neurologic diseases affecting children, neurotrophic factors might be inadequate. Using a sensitive, two-site enzyme-linked immunoassay, we examined the content of nerve growth factor in the cerebrospinal fluid of 11 children with Rett syndrome and of 24 control patients with various neurologic diagnoses or suffering from other diseases. Nerve growth factor levels were significantly lower in the cerebrospinal fluid of the patients with Rett syndrome than in control patients. The lower level of cerebrospinal fluid nerve growth factor in Rett syndrome suggests that lack of nerve growth factor may be involved in the pathogenesis of this disease or reflect the underlying brain damage present. (J Child Neurol 1996;11:296-300).
Electroencephalographic Evaluation of Children Without Neuropsychiatric Disturbances but With Poor School Performance
Authors: Micheloyannis Sifis , Tzenaki Maria , Bamboukas Michael , Giachnakis Manolis , Paritsis Nikos , Prokopakis Manolis , Molinari Luciano
Published: December 2008

The estimation of brain function using neurophysiologic methods is of importance because, besides neuropsychiatric diseases, a variety of systemic diseases can influence brain function. Several types of brain dysfunction, resulting from many kinds of influences on the central nervous system, can cause poor school performance. Besides these dysfunctions of organic origin, there may be others that do not have an organic basis but may reflect the adverse effects of the environment. Often, the differential diagnosis between them is difficult. We performed a neurologic examination and an evaluation using the Illinois Test of Psycholinguistic Abilities and questionnaires (Eysenck and Rutter) to assess the personality of 22 poor school performers aged 10 to 12 years and 22 controls with good school performance of similar age. In order to assess the possibility of using quantitative electroencephalographic methods to evaluate nonorganic poor school performers, we estimated the power spectrum and coherence of the electroencephalographic signals at rest and their changes during motor tasks and auditory stimulation. Our results indicate that there were no signs of organicity in the group of poor school performers. (J Child Neurol 1996;11:309-312).
Child Neurology: Past, Present, and Future
Authors: Fishman Marvin A.
Published: December 2008

The emergence of child neurology as an independent discipline was enhanced by pediatricians developing an organ system approach to the study of illnesses of children and indirectly by their interest in normal psychomotor development, a function of maturation of the nervous system. Technological innovations, advances in neuroimaging, and the development of therapeutic agents directly benefited patients with neurologic disorders and enhanced the role of the neurologist. The American Board of Psychiatry and Neurology was founded in 1934. It formally recognized child neurology as a special area of expertise in 1959 when Sidney Carter was appointed as a director and the clinical examination was changed to include an hour devoted specifically to child neurology. In 1969, special certificates in child neurology were issued. Thirty candidates, or more, have successfully passed the examination each year since then. As of Spring 1995, there had been 1007 certificates issued to child neurologists. In 1991 and 1992, alternate pathways to training in child neurology were developed. These substituted a year of internal medicine or a year of basic neuroscience research for 1 year of pediatrics. Relatively few child neurologists have chosen these alternate pathways. The performance of child neurologists, on Board examinations has generally been comparable to that of adult neurologists with the latter holding a slight edge. In 1990, a study of first-time takers of the certification examination was begun. In child neurology, 54% of the cohort passed both parts I and II on their first attempts, and as of July 1995, 81% of the original cohort was certified. In adult neurology, the comparable rates are 56% and 84%. Accreditation of child neurology training programs began in 1987. As of November 1995, there were 75 accredited child neurology training programs, which offered slightly over 200 total positions. Changes in health care delivery are currently taking place and have placed greater emphasis on primary care physicians. Proposals have been put forth that, if enacted, will reduce the current number of medical students and also reduce the total number of residents being trained. Reductions as great as 40% of current resident number have been proposed. Child neurology will need to reassess our current work force and training needs and re-evaluate our role in the training of primary care physicians. There appears to be a paradox. Explosions of information in both the basic and clinical neurosciences have directly benefited our patients, but yet our continued growth as a subspecialty is being challenged. (J Child Neurol 1996;11:331-335).
Correlation of Medical and Neurosurgical Events With Neuropsychological Status in Children at Diagnosis of Astrocytoma: Utilization of a Neurological Severity Score
Authors: Ater Joann L. , Moore Bartlett D. , Francis David J. , Castillo Rosario , Slopis John , Copeland Donna R.
Published: December 2008

Neuropsychological studies of children who have brain tumors have yielded diverse results with respect to identifying factors that contribute to poor intellectual outcome. The purpose of this study was to evaluate the relationship between pre- and perioperative events, tumor-related factors, and the neuropsychological status of children diagnosed with astrocytoma. Events that could potentially be detrimental to neuropsychological outcome were quantified utilizing a new "neurological severity score." The Neurological Severity Score was developed as a research tool to test our hypothesis that ultimate intellectual outcome is a result of cumulative, interactive insults on the central nervous system. This study constitutes a first step in examining the predictive value of the Neurological Severity Score by evaluating its correlation with baseline neuropsychological status. Fifty-nine children who had astrocytoma (36 supratentorial and 23 infratentorial) received complete neurological and neuropsychological evaluations within 3 months of diagnosis. Each child's neurological history and examination results were scored by an independent observer using the Neurological Severity Score. Neuroimages obtained at diagnosis and at the time of neuropsychological testing were evaluated as well. For the group as a whole, memory, attention, and motor abilities were significantly below age-appropriate norms, whereas intelligence, language, and academic skills were preserved. Patterns of deficits were identified and related to tumor site. There were no significant differences in mean neuropsychological domain scores between groups based on gender, pre- versus post-operative status, ethnicity, tumor grade, or abnormalities on magnetic resonance imaging (MRI). The Neurological Severity Score was significantly inversely correlated with visual-spatial skills, memory, attention, performance IQ, and global IQ. In conclusion, among all the medical and neurological factors present at diagnosis, the neurological severity score had the highest correlation with neuropsychological scores. This instrument has promise as a research tool in investigations of the psychological effects of cancer and its treatment on children. (J Child Neurol 1996;11:462-469).
Carbamazepine Trial for Lesch-Nyhan Self-Mutilation
Authors: Roach E.S. , Delgado Mauricio , Anderson Louise , Iannaccone Susan T. , Bums Dennis K.
Published: December 2008

Anticonvulsants may reduce the self-mutilation of acquired sensory neuropathy, and one report described sensory neuropathy in an older patient with Lesch-Nyhan syndrome. We performed nerve and muscle biopsies on four patients with Lesch-Nyhan syndrome and initiated an uncontrolled pilot trial to see if carbamazepine would reduce the self-mutilation in these patients. All of the boys had clinical features typical of Lesch-Nyhan syndrome, and the diagnosis was confirmed in each by enzyme analysis. No specific abnormalities were identified in either nerve or muscle. Nevertheless, self-mutilation and the need for constant restraint diminished in all four patients, though in one the effect was only transient. Two patients had increased self-mutilation when carbamazepine was stopped, then improved a second time when treatment was restarted. Sensory neuropathy was not confirmed, so any effect of carbamazepine is likely to be on the central nervous system. (J Child Neurol 1996;11:476-478).
Protein C and Protein S Activity in Sickle Cell Disease and Stroke
Authors: Tam David A.
Published: December 2008

Stroke is a significant complication of sickle cell anemia in the pediatric population. The pathophysiology of stroke in sickle cell anemia remains unclear. Protein C and protein S activities were measured in children with sickle cell anemia and stroke, and compared to those with sickle cell anemia who were neurologically normal. Results showed significantly decreased levels of both protein C and protein S activities in children with sickle cell anemia who have had a stroke. This pilot study suggests that a possible coagulopathic state in children with sickle cell anemia may be associated with an increased risk for cerebrovascular disease. Further research in this area is indicated. (J Child Neurol 1997;12:19-21).
An Instrument To Measure Independent Walking: Are There Differences Between Preterm and Fullterm Infants?
Authors: de Groot Laila , de Groot Cees J. , Hopkins Brian
Published: December 2008

In clinical practice walking independently has always been considered a major milestone in development. Nevertheless, little attention has been paid to the quality of movement expressed in the first attempts at walking free. Even when children achieve walking within a normal time range, some of them show features that are deviant. Early walking is difficult to judge, but at the same time may provide a sensitive means for detecting possible developmental impairments. The main aim of this paper is to provide a standardized clinical instrument for the qualitative assessment of early walking in a structured free field situation and to compare preterm and fullterm infants. All subjects were assessed 14 days after being able to walk 5 meters independently. The study group consisted of 52 children, of whom 33 were born prematurely (further distinguished in terms of being small- or appropriate-for-gestational age), and 19 were born fullterm. Judgments of walking performance were made in terms of optimal, near-optimal, near-poor, or poor. After correction for age, the preterm group was still later in the onset of walking, but more importantly, showed a qualitatively different pattern of locomotion. Those who were the youngest and small-for-gestational age were overrepresented in the near-poor and poor categories of walking. (J Child Neurol 1997;12:37-41).
Vigabatrin in Partial Seizures in Children
Authors: Nabbout Rima C. , Chiron Catherine , Mumford John , Dumas Caroline , Dulac Olivier
Published: December 2008

Patients with partial seizures aged 1 week to 19 years (n = 175) were included in several prospective vigabatrin studies at the hospital Saint Vincent de Paul. A decrease in seizure frequency of over 50% was achieved in 70% of patients, with 30% becoming seizure free, and only 6% experiencing an increase. Tuberous sclerosis gave the best response (85%), tumors the lowest (45%), the usual figure for other causes being 70%. Patients with malformations were the most likely to experience seizure increase. Regarding topography of focus, the lowest rate of seizure-free patients was in rolandic foci. Early treatment produced better results. Infants experienced the highest rate of response, but also of relapse, the latter occurring mainly between 6 months and 1 year. The incidence of loss of efficacy depended on etiology and topography of focus, tumors and frontal foci having the highest risk. Occurrence of new seizures was more frequent in young patients with tuberous sclerosis, the third experiencing status epilepticus. Vigabatrin monotherapy could only be achieved in 33% of seizure-free patients, reduction of comedication being a risk of relapse and of further intractability. (J Child Neurol 1997;12:172-177).
Inthrathecal IgA Synthesis in X-Linked Cerebral Adrenoleukodystrophy
Authors: Korenke G. Christoph , Reiber Hansotto , Hunneman Donald H. , Hanefeld Folker
Published: December 2008

Cerebrospinal fluid data of 25 patients suffering from various phenotypes of X-linked adrenoleukodystrophy have been evaluated neurochemically including cerebrospinal fluid/serum quotient diagrams. Intrathecal IgA production in 13 of 14 patients with cerebral adrenoleukodystrophy was the most sensitive parameter in cerebrospinal fluid and was not seen in any of the neurologically asymptomatic patients or in the patients with adrenomyeloneuropathy. A blood-cerebrospinal fluid barrier dysfunction was found in 9 of these 14 patients. Additional intrathecal IgG or IgM synthesis was observed in 3 patients each. In 1 patient with lumbar punctures before and after onset of neurologic symptoms intrathecal IgA synthesis was seen only after the appearance of neurologic symptoms. Repetition of lumbar punctures in 5 neurologically symptomatic patients with cerebral adrenoleukodystrophy revealed a similar pattern of intrathecal IgA synthesis with a tendency to decreasing IgA concentration. The pathophysiologic aspects of intrathecal IgA synthesis are discussed in relation to other demyelinating and inflammatory neurologic diseases. (J Child Neurol 1997;12:314-320).
Macrocerebellum: Neuroimaging and Clinical Features of a Newly Recognized Condition
Authors: Bodensteiner John B. , Schaefer G. Bradley , Keller Gina M. , Thompson James N. , Bowen Mary K.
Published: December 2008

Other than hamartomatous enlargement of the cerebellum as in Lhermitte-Duclos syndrome, diffuse enlargement of the cerebellum is not clearly described. We report four patients (ages 9 months to 2 years) with diffusely enlarged cerebelli as identified by measurement of the cerebellum and comparison to age appropriate normal values. The cerebellar measurements were determined in absolute numbers and expressed as ratios of cerebellum to whole brain and supratentorial brain. The clinical features of these four children (3 boys, 1 girl) consistently include global developmental delay, tone abnormalities, preserved reflexes, delayed or abnormal maturation of the visual system (oculomotor apraxia), and deficient or delayed myelination of cerebral white matter. The etiology of the macrocerebellum is unknown but we propose that the cerebellum is responding to the elaboration of growth factors intended to augment the slow development of cerebral structures. Regardless of the etiology, the finding of a macrocerebellum appears to allow the clinician to predict the clinical features of the patient and probably represents a marker for disturbed cerebral development. (J Child Neurol 1997;12:365-368).
Topical Review: Glutamate in Neurologic Diseases
Authors: Bittigau Petra , Ikonomidou Chrysanthy
Published: December 2008

Excitotoxicity has been implicated as a mechanism of neuronal death in acute and chronic neurologic diseases. Cerebral ischemia, head and spinal cord injury, and prolonged seizure activity are associated with excessive release of glutamate into the extracellular space and subsequent neurotoxicity. Accumulating evidence suggests that impairment of intracellular energy metabolism increases neuronal vulnerability to glutamate which, even when present at physiologic concentrations, can damage neurons. This mechanism of slow excitotoxicity may be involved in neuronal death in chronic neurodegenerative diseases such as the mitochondrial encephalomyopathies, Huntington's disease, spinocerebellar degeneration syndromes, and motor neuron diseases. If so, glutamate antagonists in combination with agents that selectively inhibit the multiple steps downstream of the excitotoxic cascade or help improve intracellular energy metabolism may slow the neurodegenerative process and offer a therapeutic approach to treat these disorders. (J Child Neurol 1997;12:471-485).
Immunocytochemical Development of Transferrin and Ferritin Immunoreactivity in the Human Pons and Cerebellum
Authors: Ozawa Hiroshi , Takashima Sachio
Published: December 2008

The distribution and development of transferrin-positive cells in the pons and cerebellum of human fetuses to adults were examined immunohistochemically, compared with those of ferritin-positive cells. Transferrin was present in oligodendrocytes, astrocytes, and neurons. Transferrin-positive neurons appeared at 18 weeks of gestation in Purkinje cells and the pontine reticular formation. In the pontine nuclei, transferrin-positive neurons appeared at 22 weeks of gestation. On the other hand, transferrin-positive glia also appeared at 18 weeks of gestation in the reticular formation, and at 24 weeks of gestation in the cerebellar white matter and pontine nuclei. Transferrin-positive glia and cells appeared earlier in the reticular formation of the pons than ferritin, but the order of its appearance was similar to that of ferritin and myelination. Because iron is involved in the syntheses and functions of dopamine, serotonin, and γ-aminobutyric acid (GABA), transferrin may be carried for various iron uses from an early fetal stage. (J Child Neurol 1998;13:59-63).
Nova Scotia Niemann-Pick Disease (Type D): Clinical Study of 20 Cases
Authors: Jan Mohammed M. S. , Camfield Peter R.
Published: December 2008

Patients with Niemann-Pick type D have been traced to a single Acadian ancester in Nova Scotia. The objective of this study was to describe the clinical course. A cohort of children with Niemann-Pick type D was identified by chart review. Some children were seen and a telephone interview with the remaining parents was conducted. Twenty children with Niemann-Pick type D were identified. The female to male ratio was 2:1. Five children had severe neonatal jaundice. Early milestones were normal in the majority. Neurologic symptoms generally developed between 5 and 10 years of age with a mean age of 7.2 years at diagnosis. Seizures developed in all between 4.5 and 16 years of age (mean, 10.5 yr), and were followed by significant physical and mental deterioration. The age at death ranged between 11 and 22.5 years (mean, 14.8 yr). In 61%, bronchopneumonia was the cause of death. There is significant variability in the presentation and clinical course of Niemann-Pick type D. (J Child Neurol 1998;13:75-78).
Quantitative Analysis and Classification of Gait Patterns in Cerebral Palsy Using a Three-Dimensional Motion Analyzer
Authors: O'Byrne John M. , Jenkinson A. , O'Brien T.M.
Published: December 2008

Gait disorders in cerebral palsy can be accurately analyzed using the CODA-3 system presenting quantitative data representing movement of the hip, knee, and ankle in the sagittal plane. We describe a technique that classifies abnormal gait automatically on the basis of sagittal kinematic data. Fifty-five hemiplegic and 91 diplegic patients were analyzed using an opto-electronic scanner (CODA-3). The sagittal kinematics of the affected limb in hemiplegics correlated with those of both affected limbs in diplegics. We introduce the concept of the "plegic limb." Sagittal kinematics of 237 affected limbs were studied using cluster statistical analysis. Eight clear groups emerged. The predominant clinical features, typical of each group, were identified and described (eg, stiff leg gait, genu recurvatum, or crouch gait). We propose this classification system as a new technique to use gait analysis data to automatically classify abnormal movements of the lower limb in cerebral palsy. (J Child Neurol 1998;13:101-108).
Lamotrigine Treatment in Childhood Drug Resistant Epilepsy
Authors: Buoni Sabrina , Grosso Salvatore , Fois Alberto
Published: December 2008

The clinical response to lamotrigine has been evaluated in a group of 63 drug resistant epileptic children in an open add-on trial. A negative response was observed in 30 subjects. In 11 patients, after an initial improvement lasting a mean period of 8 months, seizures recurred with the same frequency present before the beginning of the treatment. Twenty-two patients responded to lamotrigine treatment. Period of observation in the responsive group ranged from 1 year to 3 years. Response was complete in 16 patients and in the other 6 a 50% to 90% decrease of seizures was obtained. A complete normalization of the electroencephalogram (EEG) was registered in 10 cases after a mean period of 5 months of therapy. Side effects were mild and limited to 12 patients. Improvement was obtained in social and academic performance. Writing ability improved in one case. Lamotrigine can be considered a useful drug in the therapeutic armamentarium for childhood drug resistant epilepsy. (J Child Neurol 1998;13:163-167).
Gender Study of Neuropsychological and Neuromotor Function in Children With Tourette Syndrome With and Without Attention-Deficit Hyperactivity Disorder
Authors: Schuerholz Linda J. , Singer Harvey S. , Denckla Martha Bridge
Published: December 2008

Neuropsychological and neuromotor functions were compared between boys and girls with Tourette syndrome (TS only), attention-deficit hyperactivity disorder (ADHD only), Tourette syndrome with ADHD (TS+ADHD), and a comparison group, in an age (mean = 10 years) and IQ (Wechsler Full-Scale mean = 111) matched sample ( n = 116). There were no timed-task neuromotor differences among the groups. Analyses of variance revealed a group X gender interaction for Letter Word Fluency and the Rapid Automatized Naming test. Girls with ADHD only were faster than boys on both tasks. When data for girls only were analyzed, girls with Tourette syndrome with ADHD had the greatest variability of reaction time on the Test of Variables of Attention, and were slowest on Letter Word Fluency. Girls with TS only were slower than girls in the other three groups on Letter Word Fluency. Poor Letter Word Fluency is explained as a linguistic executive dysfunction involving speed and efficiency of memory search in this bright group of girls with Tourette syndrome, not otherwise at risk for linguistic difficulties. (J Child Neurol 1998;13:277-282).
Rolandic Type Cerebral Palsy in Children as a Pattern of Hypoxic-Ischemic Injury in the Full-Term Neonate
Authors: Maller Alfreda I. , Hankins Linda L. , Yeakley Joel W. , Butler Ian J.
Published: December 2008

Magnetic resonance images (MRIS) of the brains of 11 patients aged from 1 week to 12 years with a distinctive type of cerebral palsy were selected based on distribution of cerebral lesions, which were restricted to bilateral perirolandic cortical and subcortical regions, including frequent symmetric involvement of basal ganglia and ventrolateral nucleus of thalami. Retrospectively, the perinatal history and clinical features were reviewed to correlate clinical data with this distinctive pattern of brain injury. Clinically affected neonates had an encephalopathy associated with a severe perinatal asphyxial event. Older children with cerebral palsy survived a similar perinatal course and demonstrated spastic quadriparesis with bulbar or pseudobulbar involvement, lack of verbal speech and variable delays in cognitive development. The distribution of hypoxic-ischemic lesions involving bilateral perirolandic regions, basal ganglia, and thalami, appears to correlate with increased metabolic areas of primary myelination in full-term neonates, but not with arterial border zones nor a single cerebral artery distribution. Myelination is a critical process in maturing brain associated with marked increase in tissue respiration and thus greater susceptibility to oxygen deprivation. It is believed that the extent of hypoxic-ischemic brain injury is determined principally by brain maturity and regional metabolic rates at time of insult and this correlates with active myelination in full-term neonates. This study confirms previous data from neuropathologic literature and recent reports of neuroimaging studies of asphyxiated neonates. In addition, retrospective analysis of the clinical data enables recognition of a type of cerebral palsy that might be the hallmark of hypoxic-ischemic injury in term neonates. (J Child Neurol 1998;13:313-321).
Event-Related Potentials (P300) in Primary Headache in Childhood and Adolescence
Authors: Evers Stefan , Bauer Birgit , Grotemeyer Karl-H. , Kurlemann Gerd , Husstedt Ingo-W.
Published: December 2008

There is strong evidence for a loss of habituation during cognitive processing in migraine as measured by P300 and contingent negative variation in adults. Event-related potentials evoked by an oddball paradigm have not yet been studied in children and adolescents suffering from different primary headache types. We recorded visually evoked event-related potentials (two consecutive trials, 200 stimuli each) in 48 children and adolescents suffering from migraine without or with aura, from episodic tension-type headache, and from ergotamine-induced headache and analyzed the latencies, amplitudes, and reaction times. No statistically significant differences were noted between all headache types and healthy controls analyzing the averaged parameters for the whole measurement. However, a highly significant loss of cortical habituation as measured by P300 amplitude and latency could be observed in migraine without and with aura by analyzing the first and the second trial of measurement separately. This phenomenon increased with age and could not be seen in healthy controls, or patients with tension-type headache or ergotamine-induced headache. Our data suggest a specific cognitive processing in migraine even in children and adolescents. Measurement of the habituation effect in P300 latency and amplitude provides a specific method to differentiate between primary headache types in childhood and adolescence. (J Child Neurol 1998;13:322-326).
Axonal Neuropathy and Predominance of Type II Myofibers in Infantile Spinal Muscular Atrophy
Authors: Omran Heymut , Ketelsen Uwe-Peter , Heinen Florian , Sauer Manfred , Rudnik-Schönebom Sabine , Wirth Brunhilde , Zerres Klaus , Kratzer Wilfried , Korinthenberg Rudolf
Published: December 2008

Two affected siblings with infantile spinal muscular atrophy (SMA I) presented with generalized muscular hypotonia, which progressed to early death. Quadriceps muscle biopsy did not show the typical neurogenic pattern of spinal muscular atrophy. The histochemical fiber type determination revealed a predominance of type II fibers without type I hypertrophy, an unprecedented finding in spinal muscular atrophy. Sural nerve biopsy exhibited findings typical for axonal neuropathy. In one patient, electrical stimulation of peripheral nerves showed an inexcitability of motor and sensory nerves. Genetic studies revealed homozygous deletions of the telomeric survival motor neuron (SMN) gene and the neuronal apoptosis inhibitory protein (NAIP) gene in the affected children. This is the second case report of molecular genetically proven spinal muscular atrophy associated with axonal neuropathy. We conclude atypical findings on muscle biopsy and evidence of axonal neuropathy are compatible with the diagnosis of infantile spinal muscular atrophy. (J Child Neurol 1998;13:327-331).
Cyclosporin A Acute Encephalopathy and Seizure Syndrome in Childhood: Clinical Features and Risk of Seizure Recurrence
Authors: Gleeson Joseph G. , duPlessis Adre J. , Barnes Patrick D. , Riviello James J.
Published: December 2008

Cyclosporin A is associated with an acute encephalopathy including seizures and alterations in mental status, herein referred to as cyclosporin A acute encephalopathy and seizure syndrome. The clinical history, electroencephalogram (EEG), and neuroimaging findings in 19 children with cyclosporin A acute encephalopathy and seizure syndrome over a 10-year period were reviewed in order to delineate clinical characteristics, imaging features, and to determine the risk of seizure recurrence in this population. All 19 had motor seizures associated with other features of cortical and subcortical dysfunction. The acute mean cyclosporin A level was 342 μg/L, but was within the "therapeutic" range in five cases. Brain imaging by computed tomography (CT) or magnetic resonance imaging (MRI) in the acute or subacute phase revealed lesions characteristic of cyclosporin A toxicity in 14 cases. Acute EEG abnormalities were present in all and included epileptiform discharges or focal slowing. Patients were followed for a median of 49 months (1-9 years). Follow-up imaging (n = 10) showed lesion resolution or improvement in the majority while EEG (n = 10) had normalized in only three. Seizures recurred in six patients and only in those with persistent EEG or imaging abnormalities. No patient had a second episode of cyclosporin A associated neurotoxicity or seizure. It appears that a significant risk of seizure recurrence exists following cyclosporin A acute encephalopathy and seizure syndrome and primarily in those children with persistent EEG or imaging abnormalities. (J Child Neurol 1998; 13:336-344).
Congenital Muscular Dystrophy in Jordanian Children
Authors: Al-Qudah Abdelkarim A. , Tarawneh Musleh
Published: December 2008

This is a consecutive study on 28 patients who have been diagnosed as having congenital muscular dystrophy at Jordan University Hospital in the period from January 1990 to February 1997. Of 75 patients diagnosed as having muscle disease, 55 (73.3%) had muscular dystrophy. Of 55 muscular dystrophy patients, 28 (50.9%) had congenital muscular dystrophy, 11 (20%) had Duchenne muscular dystrophy, 9 (16.4%) had Becker muscular dystrophy, 4 (7.3%) had myotonic dystrophy, 2 (3.6%) had limb-girdle dystrophy, and 1 (1.8%) patient had facioscapulohumeral dystrophy. Age of onset of symptoms of congenital muscular dystrophy (hypotonia and weakness) was documented antenatally or in the first few months in the majority (92.9%) of patients. Parental consanguinity was documented in 21 (75%) of congenital muscular dystrophy cases, and family history of possible similar cases in 15 (53.6%). Congenital muscular dystrophy patients with normal cognitive milestones (n = 16; 57.1%) were slightly more common than patients with cognitive delay. In contrast to previous reports, congenital muscular dystrophy is probably more common in communities with high rates of parental consanguinity than other dystrophies. Our study adds significant support to the most recent literature on this finding. (J Child Neurol 1998;13:383-386).
Topical Review: Applying Classifications of Sleep Disorders to Children With Neurologic Conditions
Authors: Scher Mark S.
Published: December 2008

Information concerning sleep ontogeny and sleep disorders in children is required by many pediatric specialists. Pediatric neurologists, for instance, frequently are called upon to assist in the evaluation of children with undiagnosed symptoms and signs during sleep, as well as to care for children and adolescents with specific neurologic diseases who also experience sleep disturbances. This review discusses pediatric sleep disturbances with specific reference to sleep in children with neurologic conditions. (J Child Neurol 1998;13:525-536).
Periodic Limb Movement Disorder and Restless Legs Syndrome in Children With Attention-deficit Hyperactivity Disorder
Authors: Picchietti Daniel L. , England Sandra J. , Walters Arthur S. , Willis Kevin , Verrico Tracy
Published: December 2008

Sleep disruption can lead to symptoms of attention-deficit hyperactivity disorder (ADHD) in children. Since periodic limb movement disorder and/or restless legs syndrome can cause sleep disruption, we assessed whether these two specific sleep disorders are likely to occur in children with ADHD. We asked a series of 69 consecutive parents of children with ADHD questions about the symptoms of periodic limb movement disorder. Based on a positive response to these periodic limb movement disorder queries, 27 children underwent all-night polysomnography. Eighteen children (aged 2 to 15 years) of the 27 (26% of the 69 children with ADHD) had 5 or more periodic leg movements in sleep per hour of sleep and had complaints of sleep disruption, thus fulfilling the criteria for periodic limb movement disorder. A comparably age- and sex-matched group of children referred to a sleep laboratory for sleep complaints but without ADHD showed only a 5% prevalence (2 of 38 subjects) of periodic leg movements in sleep ( P=.017). Eight of the 18 children with ADHD and periodic limb movement disorder and one of the two control patients with periodic limb movement disorder had both a personal and parental history of restless legs syndrome symptomatology This study further documents the occurrence of periodic limb movement disorder and restless legs syndrome in children and is the first large-scale study establishing a possible comorbidity between ADHD and periodic limb movement disorder. We propose that the sleep disruption associated with periodic limb movement disorder and restless legs syndrome and the motor restlessness of restless legs syndrome while awake could contribute to the inattention and hyperactivity seen in a subgroup of ADHD-diagnosed children. ( J Child Neurol 1998;13:588-594).
Central Nervous System Structure and Function in Sturge-Weber Syndrome: Evidence of Neurologic and Radiologic Progression
Authors: Maria Bernard L. , Neufeld Jennifer A. , Rosainz Louis C. , Drane Walter E. , Quisling Ronald G. , Ben-David Kfir , Hamed Latif M.
Published: December 2008

Sturge-Weber syndrome is characterized by the presence of a port-wine nevus, epilepsy, stroke-like episodes, headache, and developmental delay. We studied 20 cases to test the hypothesis that decreased cerebral blood flow alters neurologic function by affecting cellular glucose metabolism. Group A consisted of 10 patients with a mean age of 1.75 years and early seizure onset (6.8 months), whereas group B was composed of older patients (mean age, 15.3 years) with later onset of seizures (3.7 years). Neurologic disease was more severe in group A, but group B had more widespread structural brain defects — shown on computed tomographic scans and magnetic resonance imaging — and metabolic brain defects — shown on hexamethylpropyleneamine oxime and [18F] fluorodeoxyglucose single photon emission computed tomographic scans. Six group A cases had hypoperfusion at baseline and five of nine had worsening of perfusion and glucose metabolism 1 year later. A total of 119 stroke-like episodes occurred in six group A cases and eight group B cases; there were 65% fewer strokes in children treated with aspirin. The data suggest that progressive hypoperfusion and glucose hypometabolism are associated with neurologic deterioration in Sturge-Weber syndrome. Longitudinal studies are needed to better define the natural history of disease and to evaluate the safety and efficacy of aspirin therapy. ( J Child Neurol 1998;13:606-618).
Tuberous Sclerosis Complex and Neonatal Seizures
Authors: Miller Steven P. , Tasch Ted , Sylvain Michel , Farmer Jean-Pierre , O'Gorman Augustin M. , Shevell Michael I.
Published: December 2008

Tuberous sclerosis complex is a multisystemic neurocutaneous disorder, manifesting variably during infancy and childhood that remains poorly described in neonates. When described in this population, tuberous sclerosis complex is most commonly associated with cardiac rhabdomyomas and brain tumors, but is rarely mentioned as an etiology for neonatal seizures. We report two children with tuberous sclerosis presenting for neonatal seizures. Neuroimaging findings of neonatal tuberous sclerosis complex are discussed. Given the absence of many of the traditional stigmata of tuberous sclerosis complex in the neonate, seizures should be considered an important presenting feature of this disorder in this particular age group. ( J Child Neurol 1998;13:619-623).
Special Populations and Tech-Prep: A National Study of State Policies and Practices
Authors: Lombard Richard C. , Hazelkorn Michael N. , Miller Robert J.
Published: December 2008

The late 1990s and the early 21 st century will be a time of monumental change in the nature of employment in the United States as the numbers of unskilled workers decline and the need for a more technologically advanced laborforce increases. To address the need for highly skilled labor, including special populations, the tech-prep curriculum was conceived as an intervention. The Carl D. Perkins Vocational and Applied Technology Education Act of 1990 mandates that special populations have equal access to recruitment, enrollment, and placement activities as well as the full range of vocational programs. A questionnaire was developed and mailed to State Departments of Education to determine if provisions were being made to ensure full inclusion of special populations into tech-prep programs. Thirty-six states responded. Results and implications are discussed.
The Federal Legislative Context and Goals of the State Systems Change Initiative on Transition for Youth with Disabilities
Authors: Johnson David R. , Halloran William H.
Published: December 2008

This article introduces and describes the policy context and current status of the State Systems Change Initiative on Transition for Youth with Disabilities conducted by the U.S. Department of Education, Office of Special Education and Rehabilitative Services (OSERS). This Initiative has been ongoing since 1991 and has provided 45 states and the District of Columbia the opportunity to engage in statewide systems change activities focused on the improvement of school-to-work transition services for youth with disabilities and their families. As authorized under section 626(e) of the Individuals with Disabilities Education Act (IDEA), states have been eligible to compete for one-time, 5-year grants that have been undertaken as cooperative efforts between state education and vocational rehabilitation (or other) agencies.
Parental Participation in Transition Systems Change
Authors: Guy Barbara , Goldberg Marge , Mcdonald Shauna , Flom Ross A.
Published: December 2008

This article describes the results of a national survey that investigated parent participation in transition systems change activities. The purpose of the article is to illustrate the multiple ways in which parents have become major participants in supporting state-level systems change activities on transition for youth with disabilities. The most frequently reported activities in which parents were involved included serving as members on transition planning boards and identifying transition information and training needed by parents and families. The findings indicate that subcontracts between transition systems change project and parent organizations increased the types of systems change activities in which parents were involved.
Transition Services for Youth and Young Adults with Emotional Disturbance: Description and Initial Results of Project RENEW
Authors: Cheney Douglas , Hagner David , Malloy Joanne , Cormier Gail M. , Bernstein Steve
Published: December 2008

The transition from school to adulthood poses a major challenge for youth with emotional disturbance. This article reports the initial results of Project RENEW, a three-year demonstration project funded by the Rehabilitation Services Administration, that provides comprehensive coordination of transition services for youth and young adults with emotional disturbance's social/emotional development, community adjustment, education, and employment. The project uses seven major components: (a) personal futures planning, (b) interagency coordination, (c) naturally supported employment, (d) flexible high school programming, (e) social skill building, (f) mentors, and (g) flexible funding to support an individual's successful transition. Results after the first 18 months of RENEW for 16 youth and young adults (ages 16-22) indicated that 15 were successfully employed, 11 had graduated from high school or its equivalent (adult basic education diploma or GED), 3 were working on their high school diplomas, and most were satisfied with their home, work and school situations.
Regular and Special Educators' Perceptions of Transition Competencies
Authors: Wolfe Pamela S. , Boone Rosalie S. , Blanchett Wanda J.
Published: December 2008

We conducted a survey to examine the (a) perceived importance of 30 transition-related teacher competencies as rated by regular educators and (b) nature of their preparation for tranition programming for students with disabilities. A total of 39 ompleted surveys (72%) were returned from regular educators in ennsylvania. We compared these results with similar data collected from another group of special educators to ascertain the degree of correspondence in transition competency ratings and training between regular and special educators. Results indicated that while the majority of educators had received some inservice training on transition, there were a number of competency areas in which the majority of regular educators received no training. Regular and special educators differentially rated the importance of six teacher competency areas. We discuss the meaning of these results for practice and service delivery.
School-to-Work and Technical Preparation: Teacher Attitudes and Practices Regarding the Inclusion of Students with Disabilities
Authors: Lombard Richard C. , Miller Robert J. , Hazelkorn Michael N.
Published: December 2008

This article describes the results of a national survey of attitudes and practices of secondary school-to-work and tech prep teachers regarding the inclusion of students with disabilities. The purpose of the study was to examine: (a) teacher attitudes toward inclusion; (b) practices and procedures being utilized to promote inclusion; (c) in-service training received by teachers regarding inclusionary practices; and (d) post-school outcomes perceived by teachers to be attainable for students with disabilities who participate in school-to-work and tech prep programs. One hundred and sixty-nine educators representing 45 states participated in this study. The results of this study indicate that secondary school-to-work and tech prep teachers do not feel prepared to meet the needs of students with disabilities who are enrolled in their courses. These teachers also report receiving little to no in-service training regarding inclusive practices, and the vast majority report that they have never participated in the development of Individual Education Plans (IEPs) for students with disabilities.