bluuebunny/crossref_metadata_2025 huggingface dataset:
An Exploration of the Use of Curriculum-Based Measurement By Elementary Special Educators
Authors: Swain Kristine D. , Allinder Rose M.
Published: December 2008

This study examined special educators' use of curriculum-based measurement as progress monitoring. Teacher characteristics were examined to determine if there was a relationship between certain teacher characteristics and utilizing curriculum-based measurement. A questionnaire was completed by 191 special educators in the Midwest. Results showed that 45% (n = 86) of the respondents utilized curriculum-based measurement for at least one purpose (monitoring ongoing progress, part of the prereferral process, evaluating programs, or identifying students with disabilities) and 55% (n = 105) did not use curriculum-based measurement. Curriculum-based measurement for progress monitoring in reading is utilized significantly more often than curriculum-based measurement in math or spelling. Teachers also responded that they had an interest in attending a workshop and reading information on curriculum-based measurement. Suggestions for practice and future research are explained.
The Phoneme Perception Task (PPT): A Measure of Phoneme Perception in Orally Read Sentences
Authors: Jerger Margaret
Published: December 2008

Analysis of scores on the Phoneme Perception Task (PPT), a new measure of perception of phonemes in sentences, revealed an overall 75% accuracy of classification of students' group membership (56 students who never received special education services in grades 2 through 5 (non LD group) and 40 students of corresponding age, attending a school for students with learning disabilities (LD group). Concurrent validity was determined using the SCAN: A Screening Test for Auditory Processing Disorders (r =.39) (Keith 1986) and the Test of Written Language-2 (TOWL-2) Spontaneous Writing Quotient (Hammill & Larsen, 1988) ( r =.48). Interjudge reliability (.97) indicated strong observer agreement. Internal consistency (alpha = .74) was considered to be reduced by specific items that need revision prior to further study.
District-Wide Strategy To Monitor the Placement and Performance of Students From Ethnically Diverse Populations: a Case Study
Authors: Hall Tracey E.
Published: December 2008

Disproportionate representation of students from ethnically diverse populations in special education has been a concern at every level from the judicial system and federal legislation to the parents of identified children. Yet, over-referral itself may not be the problem. Rather, the failure may be for students of varying ethnicities to make improvements once identified and receiving services in special education. This study presents a formative evaluation method to monitor student progress in a school district using curriculum-based measurement (CBM). Student performance was measured and graphed throughout the school year and individual results overlaid on district CBM normative data using each student's peer group. The resulting graphic displays clearly demonstrate student success or failure in these curriculum areas, therefore, from two perspectives: norm and individual-referenced. Implications for practice are discussed, particularly by noting the need to focus on progress monitoring rather than placement rate as the basis for judging disproportionate representation of ethnically diverse populations in special education.
Research Digest: Assessing Biobehavioral States in Individuals With Profound Disabilities: An Alternative To Traditional Approaches
Authors: Richards Stephen B.
Published: December 2008

The purpose of this article is to provide a summary of seminal research and recent articles directly related to the assessment of biobehavioral states in individuals with profound disabilities, to discuss the implications of the research findings included, and to present some recommendations for further investigations.
Pharmacotherapy of Spasticity: Oral Medications and Intrathecal Baclofen
Authors: Krach Linda E.
Published: December 2008

Spasticity is a common problem in children with neurologic impairment, particularly in those with cerebral palsy. Clinicians commonly make use of oral medications to attempt to reduce spasticity and increase function. Little has been published in the literature concerning the use of these medications in children and their effects on both muscle tone and function. This article is a review of the sites of action, side effects, and efficacy of benzodiazepines, baclofen, dantrolene sodium, alpha2-adrenergic agonists, and gabapentin. A brief review of intrathecal baclofen is included as well. (J Child Neurol 2001;16:31-36).
Kindergarten Readiness Skills in Children With Sickle Cell Disease: Evidence of Early Neurocognitive Damage?
Authors: Grant Steen R. , Hu X. Joan , Elliott Vanessa E. , Miles Mark A. , Jones Shalita , Wang Winfred C.
Published: December 2008

Young children with sickle cell disease are at risk of brain damage, including stroke. We tested the hypothesis that such patients are also at risk of cognitive impairment. We characterized the cognitive ability of kindergarten children to minimize the effect of disease-related school absence. The Memphis City Schools use the Developing Skills Checklist, a teacher-administered test given in the classroom, to assess kindergarten-appropriate skills. Data were obtained for 34 patients, who were matched to controls by gender, race, date of birth, school, and approximate income. Two controls were selected for each patient, and paired t-tests were used to compare patient's scores to composite control scores. Patients scored lower than controls in auditory discrimination (P < .O1), and there was a trend (P < .10) toward lower patient scores in language. Deficits cannot be attributed to school absence and may predict academic problems for patients with sickle cell disease. (J Child Neurol 2002;17:111-116).
Significance of Microcephaly Among Children With Developmental Disabilities
Authors: Watemberg Nathan , Silver Sarah , Harel Shaul , Lerman-Sagie Tally
Published: December 2008

To assess the clinical impact of microcephaly among children with developmental disabilities, we reviewed the charts of 1393 consecutive patients from birth to 5 years of age referred to our child development center. Comparisons were made between normal and low IQ microcephalic patients and between children with cerebral palsy with and without small head circumference. Microcephaly was detected in 15.4% of patients. Although mental retardation was more common among microcephalic children (P < .001), almost half had normal intelligence. Prematurity (P < .001), perinatal asphyxia (P < .001), small for gestational age (P < .001), respiratory distress syndrome (P < .001), and brain hemorrhage (P < .001) were associated with microcephaly. Hypotonia (P < .001) and spasticity (P < .001) were the most common neurologic findings. Cerebral palsy (P < .001), growth retardation (P < .001), epilepsy (P < .001), and strabismus (P < .001) were the main associated diagnoses found. Mental retardation was significantly more common among microcephalic patients with cerebral palsy than among normocephalic ones (P < .0004). Microcephaly is common among children evaluated for developmental disabilities. Many of these patients have normal or borderline IQ. Of several perinatal conditions associated with later microcephaly, respiratory distress syndrome and intraventricular hemorrhage show the strongest correlation. Mental retardation is not a risk factor for other neurologic complications in microcephalic children. However, in children with cerebral palsy, microcephaly is a risk factor for mental retardation. (J Child Neurol 2002;17:117-122).
Review Article: The Management of Children With Spinal Dysraphism
Authors: Liptak Gregory S. , Bloss Jill W. , Briskin Henra , Campbell Jeanette E. , Hebert Elizabeth B. , Revell Gail M.
Published: December 2008

Improvements in technology have dramatically increased the survival of children with spinal dysraphism. Because this complex condition affects multiple organ systems as well as the psychosocial functioning of the child and family, these children require care from a host of specialists in order to achieve optimum functioning. This article reviews the pathophysiology and discusses the current management of the medical and psychosocial effects of spinal dysraphism. It also briefly discusses strategies for intervention, long-term outcomes, and some controversies regarding care and screening. ( J Child Neurol 1988;3:3-20).
Review Article: Corn Oil Ketogenic Diet for Children With Intractable Seizures
Authors: Woody Robert C. , Brodie Marilou , Hampton Debra K. , Fiser Robert H.
Published: December 2008

The ketogenic diet is a well-established, but relatively infrequently used, therapy for intractable seizures in children. Currently, the standard ketogenic diet is formulated using medium chain triglyceride (MCT) oil as its major caloric source. Cost, availability, and gastrointestinal side effects limit its use in some cases. We report the successful substitution of corn oil for MCT oil in six children treated with the ketogenic diet for intractable seizures. In each case, the corn oil ketogenic diet was effective, well-tolerated by the patient and parents, and free of clinical toxicity. Corn oil also has the major advantages of being much less expensive and more available than MCT oil. Its use in the ketogenic diet appears to be effective, safe, and practical.( J Child Neurol 1988;3:21-24)...
Psychologic and Neurologic Function Following Treatment for Childhood Temporal Lobe Astrocytoma
Authors: Mulhern Raymond K. , Kovnar Edward H. , Kun Larry E. , Crisco J. Jeffrey , Williams J. Michael
Published: December 2008

Seven school-aged children treated for temporal lobe astrocytomas with surgical resection and irradiation were prospectively tested to evaluate their intellectual, academic, personality, and neurologic status after therapy. At their most recent follow-up examination, neuropsychologic functioning was adequate in only two patients. The other five children manifested either intellectual deterioration, learning disability, mental retardation, or psychopathology. These deficits were associated with poor postoperative performance status, inadequate seizure control, tumor recurrence, and younger age at diagnosis. No pattern of intellectual, academic, or personality dysfunction emerged in association with left- versus right-hemisphere tumors. ( J Child Neurol 1988;3:47-52).
Colpocephaly: Frequency and Associated Findings
Authors: Noorani P.A. , Bodensteiner J.B. , Barnes P.D.
Published: December 2008

Colpocephaly is an abnormal enlargement of the occipital horn of the lateral ventricle, also described as persistance of the fetal configuration of the lateral ventricles. Since it was first described, colpocephaly has been found in association with a number of abnormalities of the brain. In order to determine the frequency of this ventricular configuration at our hospital, we reviewed 3,411 computed tomographic (CT) scans done over a 26-month period. Fourteen cases were identified in which the CT scan had the appearance of colpocephaly. These CT scans were performed during the evaluation of a number of different clinical problems. Agenesis of the corpus callosum was the most frequenly associated malformation. The types of malformations commonly associated with colpocephaly suggest that colpocephaly is merely a marker of disordered brain formation and that insults occurring anytime between one and four months of gestation may result in this anomaly. (J Child Neurol 1988;3:100-104) .
The Evolution of Ischemic Cerebral Infarction in Infancy: A Sonographic Evaluation
Authors: Fischer Asma Q. , Anderson Joseph C. , Shuman Robert M.
Published: December 2008

Cranial sonography provides a noninvasive, portable method for imaging the infant brain. This study describes the time-dependent, sonographic findings of infantile cerebral infarction, as well as computed tomographic (CT) scan and neuropathologic confirmation. Three hundred ninety-five infants under 18 months of age were sonogrammed over a period of 18 months. Three infants were diagnosed by cranial sonography and confirmed by CT scan and/or autopsy to have acute ischemic cerebral infarcts. The cases were followed with serial cranial sonograms for up to 18 months of age. The acute sonographic findings included a hyperechoic zone around the infarcted tissue. The subacute infarct had a checkerboard pattern, while the chronic infarcts were anechoic. (J Child Neurol 1988;3:105-109).
Review Article: Imaging of the Pediatric Spine and Its Contents
Authors: Sarwar Mohammad
Published: December 2008

We are at the crossroads of imaging of the diseases of the spine and its contents. There are different competing imaging modalities, each with its own merits and demerits. This review paper is designed to identify the strengths and weaknesses of the various available imaging modalities and to recommend one or a combination of imaging modalities that are suited for assessing a particular disease process. At present, magnetic resonance imaging has proved its extreme usefulness in depicting diseases of the spinal cord and its coverings. As more innovations and sophistications occur in magnetic resonance imaging, it is hoped that it will play a pivotal role in the investigation of abnormalities of the spinal cord and perhaps even of the spine itself. ( J Child Neurol 1990;5:3-18).
Cavernous Angioma Presenting as Atypical Facial and Head Pain
Authors: Epstein Mark A. , Berman Peter H. , Schut Luis
Published: December 2008

Recurrent headache is a common pediatric problem. As the differential diagnosis of headache is extensive, physicians rely on the mode of presentation to focus any investigation. A report of an adolescent in whom atypical facial and head pain caused by a preexisting cerebellar cavernous angioma is presented. Facial pain and headache resolved following excision of the tumor. ( J Child Neurol 1990;5:27-30).
Isolated Lissencephaly: Report of Four Patients From Two Unrelated Families
Authors: Pavone Lorenzo , Gullotta Filippo , Incorpora Gemma , Grasso Sebastiano , Dobyns William B.
Published: December 2008

Lissencephaly is a brain malformation manifested by a smooth cerebral surface and caused by incomplete neuronal migration. Clinical sequellae include minor craniofacial changes (bitemporal hollowing, small jaw), severe mental retardation, and other neurological abnormalities. Patients with classical or type I lissencephaly and its sequellae but no other significant anomalies are classified as having isolated lissencephaly sequence.

Possible causes of isolated lissencephaly sequence include ischemia or viral infection during the time of neuronal migration, microdeletion within the Miller-Dieker syndrome critical region in chromosome band 17p13.3, and Mendelian inheritance. The last is based on a report of a single family with three affected children in 1933. We report four patients with isolated lissencephaly sequence from two unrelated families who provide further support for autosomal (or possibly X-linked) recessive inheritance. In the first family, three brothers were affected. In the second, the parents are first cousins. ( J Child Neurol 1990;5:52-50).
Bromide Therapy for Pediatric Seizure Disorder Intractable to Other Antiepileptic Drugs
Authors: Woody Robert C.
Published: December 2008

Triple bromide elixir was used as an adjunctive antiepileptic drug in 11 children whose seizure disorders were intractable to other antiepileptic therapy. The patients' ages ranged from 2 to 17 years. The seizure disorders treated included photosensitive epilepsy (one case), acquired epileptic aphasia (one case), Lennox-Gastaut syndrome (three cases), and symptomatic localization-related epilepsies (six cases). Two patients' seizures completely stopped with bromide therapy. Four patients had a significant and sustained improvement on bromide therapy, while three more had a transient improvement. In these six patients with complete or significant control, the mean therapeutic dose was 33 mg bromide/kg daily, and the mean therapeutic serum concentration was 14.1 mmol/L (range, 4 to 30.5 mmol/L). The combination of bromide with valproate appeared to be particularly effective in these patients. Toxicity was minimal, and in only one patient was the medication stopped, because of anorexia and weight loss. Given the low cost, long half-life, and minimal toxicity when serum bromide concentrations are followed, bromide therapy should be considered as adjunctive antiepileptic drug therapy for patients whose seizures are intractable to other drugs. ( J Child Neurol 1990;5:65-67).
Cushing's Ulcer as a Consequence of Adrenocorticotropic Hormone Administration for Infantile Spasms
Authors: Glauser Tracy A. , Rogers Mark
Published: December 2008

Adrenocorticotropic hormone (ACTH) is the treatment of choice for infantile spasms despite its multiple side effects. This case represents a previously unreported side effect, peptic ulceration, occurring during ACTH treatment of infantile spasms. Two pathophysiologic mechanisms are proposed and examined. A direct effect of ACTH on the duodenum is considered but deemed unlikely. Alternatively, the combination of the patient's communicating hydrocephalus and ACTH's central effects is proposed to have led to increased intracranial pressure and a Cushing's ulcer. The need for pretreatment neuroimaging and H2-receptor antagonist use during ACTH therapy in all cases, along with gastrin level measurements in selected cases, is recommended. ( J Child Neurol 1990;5:111-113).
Cytochrome c Oxidase Deficiency in Muscle With Dicarboxylic Aciduria and Renal Tubular Acidosis
Authors: Pintos-Morell Guillem , Haas Richard , Prodanos Christina , DiMauro Salvatore , Nyhan William L.
Published: December 2008

A patient with deficient activity of cytochrome c oxidase in muscle presented at 1 year of age with extreme failure to thrive. He was found to have dicarboxylic aciduria, renal tubular acidosis, and deficiency of carnitine. Treatment with sodium bicarbonate, riboflavin, and carnitine led to considerable improvement in growth and a significant reduction in the dicarboxylic aciduria. (J Child Neurol 1990;5:147-152).
Self-Injurious Behavior Associated With Clonidine Withdrawal in a Child With Tourette's Disorder
Authors: Dillon James E.
Published: December 2008

A 7-year-old boy with Tourette's disorder, atypical pervasive developmental disorder, borderline mental retardation, and a history of self-injurious behavior was treated for 21 months with clonidine transdermal patches at doses ranging from 0.1 to 0.5 mg weekly. When withdrawn from clonidine over 4 weeks to assess the need for continued therapy, the patient developed multiple self-destructive behaviors involving the theme of suffocation. The importance of careful clinical monitoring of the behavior of patients undergoing withdrawal from prolonged treatment with high doses of clonidine is emphasized. ( J Child Neurol 1990;5:308-310).
The Effect of Gestational Age at Birth on Somatosensory -evoked Potentials Performed at Term
Authors: Majnemer Annette , Rosenblatt Bernard , Willis Diana , Lavallee Johanne
Published: December 2008

Multimodality-evoked potentials are widely used in newborns to assess the maturation and integrity of the sensory pathways. Reliable normative data are needed to maximize the utility of this technique as a diagnostic and research tool. Several electrophysiologic studies on the maturational changes of the auditory brain-stem response have demonstrated that latency measurements decrease as a function of increasing conceptional age. However, maturational studies of the somatosensory-evoked potential, particularly in low-risk premature infants, are limited. The existing evoked potential literature in healthy newborns proposes that maturation of the central nervous system occurs at a predictable rate, irrespective of a given gestational age at birth. Behavioral studies of premature infants suggest that neurologic development may be altered by early extrauterine exposure. The purpose of this study was to determine whether brain-stem auditory- or somatosensory-evoked potential conduction times were comparable in premature and full-term infants matched for conceptional age. The results of this study suggest that myelination is determined by conceptional age, independent of premature birth. ( J Child Neurol 1990;5:329-335).
The Pattern of Neural Tube Defects Created by Secondary Reopening of the Neural Tube
Authors: Campbell L. Rebecca , Sohal G.S.
Published: December 2008

The usual location of human neural tube defects at the rostral or caudal end of the primary neural tube suggests they are caused by failure of closure of the neural tube. In this study, neural tube defects were created by surgical reopening of the neural tube of 3-day-old duck embryos in one of three sites: the forebrain, cervicothoracic region, or thoracolumbar region. It was determined that of the 31 survivors with forebrain incisions, 39% had exencephaly; of the 42 survivors with thoracolumbar incisions, 31% had myeloschisis; but of the 37 with a cervicothoracic incision, only 14% had exposed spinal cord. This indicates that a rostral-caudal distribution of neural tube defects can be seen in a model created by secondary reopening of the neural tube. ( J Child Neurol 1990;5:336-340).
Lissencephaly-Pachygyria Associated With Congenital Cytomegalovirus Infection
Authors: Hayward Jean C. , Titelbaum David S. , Clancy Robert R. , Zimmerman Robert A.
Published: December 2008

We report the presence of major cerebral migrational defects in five severely, multiply handicapped children with congenital cytomegalovirus (CMV) infection. These patients had both computed tomographic (CT) scan and magnetic resonance imaging (MRI) evidence of marked migrational central nervous system defects consistent anatomically with the spectrum of lissencephaly-pachygyria, a disorder commonly idiopathic or associated with chromosomal abnormalities or with unknown early gestational insults. Neuroradiologic features included broad, flat gyri, shallow sulci, incomplete opercularization, ventriculomegaly, periventricular calcifications, and white-matter hypodensity on CT scans or increased signal intensity on long-TR MRI scans. Evidence for congenital CMV infection included prenatal onset of microcephaly, periventricular calcifications, neonatal jaundice, hepatomegaly, elevated CMV-specific immunoglobulin M, or viral isolation from urine. Previous reports of the neurologic sequelae of CMV have emphasized varying degrees of psychomotor retardation, cerebral palsy and epilepsy due to polymicrogyria, periventricular calcification, microcephaly, or rarely, hydrocephalus. Our patients appear to represent extremely severe examples of the effects of CMV on neurologic growth, maturation, and development. Recognition of these severe migrational abnormalities was improved by use of MRI, a technique that affords superior definition of the nature and extent of gyral and white-matter abnormalities. We suggest that these abnormalities may be more common than has previously been recognized. ( J Child Neurol 1991;6:109-114).
Infant-Onset Progressive Myoclonus Epilepsy
Authors: Harbord Michael G. , Hwang Paul A. , Robinson Brian H. , Becker Laurence E. , Hunjan Amrita , Murphy E. Gordon
Published: December 2008

We report the clinical, electroencephalographic, neurophysiologic, and neuroimaging findings in eight children with infant-onset progressive myoclonus epilepsy, all of whom had muscle biopsies performed as part of the diagnostic evaluation. Each child had myoclonic seizures, generalized tonic-clonic seizures, and neurologic regression or marked developmental delay. Four children died before 3 years of age. Electroencephalograms in seven children showed an abnormally slow background with bilateral multifocal paroxysmal discharges but no burst suppression pattern or photoparoxysmal response. Muscle biopsy specimens were submitted for histopathology and respiratory-chain enzyme studies. Nonspecific abnormalities on light microscopy or electron microscopy were found in seven samples, including increased subsarcolemmal deposits of mitochondria or morphologic mitochondrial changes, but no ragged-red fibers were seen. Respiratory-chain enzyme studies were performed on five samples and in three children (all of whom had a history of elevated lactate in serum or cerebrospinal fluid), there were low levels of rotenone-sensitive reduced nicotinamide adenine dinucleotide (NADH) cytochrome c reductase characteristic of a defect in the complex I part of the respiratory-chain pathway. This study has shown that infant-onset progressive myoclonus epilepsy can be distinguished from other myoclonic epilepsy syndromes of infancy by clinical and electrographic features. Furthermore, respiratory-chain enzyme defects are a relatively common cause of infant-onset progressive myoclonus epilepsy. The absence of ragged-red fibers on muscle histopathology does not preclude a mitochondrial enzyme abnormality. (J Child Neurol 1991;6:134-142).
HLA Patterns in Children With Parainflammatory Leukoencephalomyelitis
Authors: Marks Warren A. , Shuman Robert M. , Bodensteiner John B.
Published: December 2008

We have evaluated the human leukocyte antigen (HLA) phenotype of six children with parainflammatory leukoencephalomyelitis (PIL). Patients with PIL demonstrate an increased prevalence of the HLA-A1, -A28, -B44, -DR6, and -DR7 antigens. These HLA associations are different from those reported in other inflammatory demyelinating diseases, including multiple sclerosis, optic neuritis, Guillain-Barré syndrome, and chronic relapsing inflammatory polyneuropathy. The HLA constitution of the patient appears to be one important host factor in determining the nature of the immune response to an encephalitogenic challenge. (J Child Neurol 1991;6:150-154).
Rett Syndrome and Mitochondrial Enzyme Deficiencies
Authors: Coker Steven B. , Melnyk Andrew R.
Published: December 2008

The etiology of Rett syndrome is unknown. Structural mitochondrial abnormalities have been described in muscle in patients with Rett syndrome. We report three children with Rett syndrome and normal muscle mitochondrial structure on light and electron microscopy. However, all had abnormalities in mitochondrial respiratory chain enzymes. (J Child Neurol 1991;6:164-166).
Multiple Sulfatase Deficiency With Early Severe Retinal Degeneration
Authors: Harbord Michael , Buncic J. Raymond , Chuang S.A. , Skomorowski M.A. , Clarke Joe T.R.
Published: December 2008

We report an unusual case of multiple sulfatase deficiency in which neurodegeneration was accompanied by early, severe visual impairment associated with prominent pigmentary retinopathy, suggesting a diagnosis of neuronal ceroid-lipofuscinosis. The levels of arylsulfatases A, B, and C, heparan N-sulfatase, N-acetylgalactosamine-6-sulfate sulfatase, and iduronate-2-sulfate sulfatase were all markedly decreased in cultured skin fibroblasts. Screening tests for mucopolysacchariduria were consistently negative; however, thin-layer chromatographic analysis of isolated urinary glycosaminoglycans showed increased amounts of heparan sulfate. ( J Child Neurol 1991;6:229-235).
Cerebral Blood Flow and Oxygen Metabolism in Rett Syndrome
Authors: Yoshikawa Hideto , Fueki Noboru , Suzuki Hisaharu , Sakuragawa Norio , Masaaki Iio
Published: December 2008

Positron emission tomography was performed on six patients with Rett syndrome to investigate cerebral blood flow and oxygen metabolism, and the results were compared with the concurrent clinical status of the patients. The cerebral metabolic rate of oxygen (CMRO 2) was low in five patients, and oxygen extraction fraction was low in four patients; both had a tendency to decline with advancing age. Although the cause is unknown, it is suggested that impaired oxidative metabolism exists in Rett syndrome. An analysis of the distribution among brain regions showed that the ratios of values for the frontal cortex to those for the temporal cortex for both cerebral blood flow and CMRO2 were lower than those for the controls, which may indicate the loss of hyperfrontality in Rett syndrome. (J Child Neurol 1991;6:237-242).
Simultaneous Infantile Spasms and Partial Seizures
Authors: Donat Jane F. , Wright Francis S.
Published: December 2008

We report 11 infants with infantile spasms who had partial seizures that occurred concurrently with the infantile spasms. We studied this phenomenon with time-locked video electroencephalography. The partial seizures began before the infantile spasms and continued after one or more infantile spasms in seven patients. An infantile spasm occurred at the beginning of the partial seizure in five patients. (One patient had both seizure types simultaneously.) The clinical descriptions of these combinations of seizures were confusing, since they did not match known seizure types. The genesis of these seizures may involve an interaction between processes of focal cortical epileptogenesis (partial seizures) and brainstem synchronization (infantile spasms) ( J Child Neurol 1991;6:246-250).
A Japanese Family With Two Types of Muscular Dystrophy: DNA Analysis and the Dystrophin Test
Authors: Matsumura Kiichiro , Toda Tatsushi , Hasegawa Takeshi , Kamei Masaki , Imoto Natsuki , Shimizu Teruo
Published: December 2008

A unique Japanese family with both Fukuyama type congenital muscular dystrophy (FCMD) and Duchenne muscular dystrophy (DMD) is described. Four boys, all from the sixth generation of the same family, were afflicted with severe neuromuscular diseases beginning in early life, three of them presenting the typical phenotype of FCMD and one, that of DMD. Although DNA analysis by Southern blotting with complementary DNAs representing the whole of the dystrophin coding sequence detected neither gross deletions nor duplications, immunohistochemistry and Western blotting of the biopsied skeletal muscle with an antidystrophin monoclonal antibody (dystrophin test) showed that the ~400-kd dystrophin was expressed normally at the sarcoplasmic membrane of the FCMD phenotype patient but was completely absent in the DMD phenotype patient. From these results, it was presumed that two different childhood muscular dystrophies, FCMD and DMD, coexisted in this family. This unique case illustrates the efficacy of the dystrophin test in the differential diagnosis of the two diseases even when conventional means of diagnosis do not give definite answers and DNA analysis of the dystrophin gene is not informative. ( J Child Neurol 1991;6:251-256).
Anencephaly in the United States, 1968-1987: The Declining Incidence Among White Infants
Authors: Snyder Robert D. , Fakadej Anna F. , Riggs Jack E.
Published: December 2008

The ethical issues concerning the use of fetal tissue as a source for organ transplantation has focused interest on anencephaly. For reasons that are not entirely clear, the incidence of anencephaly has been declining. As anencephaly is easily recognized and invariably fatal, mortality figures provide an excellent reflection of incidence. In the United States, between 1968 and 1987, infant mortality rates per 100,000 live births due to anencephaly declined from 22.4 to 12.1 (46.0%) for male infants and from 32.7 to 16.6 (49.2%) for female infants. However, when separated by race, the mortality rate declined for white male infants from 25.0 to 13.3 (46.8%) but only decreased from 9.0 to 7.7 (14.4%) for nonwhite male infants. The mortality rate declined for white female infants from 36.7 to 17.6 (52.0%) and actually increased slightly from 12.8 to 13.2 (3.1%) for nonwhite female infants. Thus, the declining incidence of anencephaly reflects a declining incidence of affected white infants but not of affected nonwhite infants. (J Child Neurol 1991;6:304-305).
Learning Disabilities in Children With Congenital Adrenal Hyperplasia
Authors: Nass Ruth , Baker Susan
Published: December 2008

Early exposure to androgens may be a cause of the higher incidence of learning disabilities among males. To test this hypothesis, we determined the frequency of learning disabilities, as measured by extent of Wechsler Verbal-Performance IQ discrepancy, among female patients with congenital adrenal hyperplasia, who are exposed in utero to excess androgens for their sex. A significantly larger Verbal-Performance IQ discrepancy (10.1 vs 7.7) was found for the 18 female patients than for 27 unaffected female siblings (P = .045, one-tailed). The Verbal-Performance IQ discrepancy of the females with congenital adrenal hyperplasia is in the male range (10.4 to 13.4), similar to males with congenital adrenal hyperplasia (n = 20) and unaffected male siblings (n = 31). In addition to the sex effect, there is an effect of the type of congenital adrenal hyperplasia on the frequency of learning disabilities. Simple virilizers are more likely to be learning disabled than salt-wasters ( P = .04, one-tailed). However, salt-waster patients have a lower IQ (104 vs 117) than simple virilizer patients ( P = .005, one-tailed). Presumably, this is because some salt-waster patients suffer brain injury from episodes of hypotension and hyponatremia. The diffuse insult to the central nervous system may overide the androgen effect and result in a general cognitive impairment. Because of this confounding effect on IQ in the salt-waster form of congenital adrenal hyperplasia, the simple virilizer female versus unaffected female siblings represents the best test of the hypothesis. Simple virilizer females have a significantly larger, male range, Verbal-Performance IQ discrepancy (13.5 vs 7.4) than unaffected female siblings ( P = .03, one-tailed). Early androgen exposure may, as reported in the "normal" male, increase the incidence of learning disabilities in females with congenital adrenal hyperplasia by causing abnormalities of neuronal migration and/or connectivity. (J Child Neurol 1991;6:306-312).
Muscle Biopsy and the Clinical Course of Infantile Spinal Muscular Atrophy
Authors: Zalneraitis Edwin L. , Halperin John J. , Grunnet Margaret L. , Russman Barry S. , Peress Nancy
Published: December 2008

Eight infants with severe early infantile spinal muscular atrophy diagnosed by clinical presentation and muscle biopsy were studied. The extent of alterations in muscle histology, histochemistry, and ultrastructure did not reflect the relative severity of the clinical presentation or the course of the illness. In seven biopsies, ultrastructural studies demonstrated empty sleeves of basal lamina projecting from the surface of small myofibers. We conclude that severe infantile spinal muscular atrophy often results in myofiber atrophy similar to that found in other motor neuron diseases, and it is not solely a hypotrophic process. Muscle biopsy findings are important because they help to establish the diagnosis, but they do not help predict the severity of disease among infants with this condition. (J Child Neurol 1991;6:324-328).
Selective Type II Muscle Fiber Hypertrophy in Severe Infantile Spinal Muscular Atrophy
Authors: Kingma Douglas W. , Feeback Daniel L. , Marks Warren A. , Bobele Gary B. , Leech Richard W. , Brumback Roger A.
Published: December 2008

The diagnostic muscle biopsy finding in severe infantile spinal muscular atrophy (Werdnig-Hoffmann disease, SMA type 1) is considered to be large-group atrophy with isolated clusters of hypertrophic type I myofibers. We present a unique case of severe infantile spinal muscular atrophy with selective hypertrophy of type II myofibers. A male infant presented at age 2 months with breathing difficulties and by age 4 months was hypotonic and weak. Electromyography revealed denervation in all extremity muscles, and nerve conduction velocities were normal but with small compound muscle action potentials. Quadriceps muscle biopsy revealed many hypertrophied type II myofibers (myofibers with a mean least diameter of 25.4 μm). In contrast, the largest type I myofibers were 20 μm in least diameter (mean diameter, 14.9 μm), and there was a normal-size population of type II fibers (mean diameter, 15.7 μm). In addition, sheets of atrophic type I and type II fibers averaged 2.0 μm in least diameter. Sural nerve biopsy was normal. Breathing difficulties progressed, with death ensuing at age 5½ months. Autopsy revealed atrophy of ventral spinal roots with normal dorsal roots. There was loss of anterior horn cells, while remnant neurons were reduced in size. No other pathologic changes were identified. This case indicates that in severe infantile spinal muscular atrophy, relative sparing of the motor units with type II myofibers may occur. (J Child Neurol 1991;6:329-334).
Topical Review Article: The Age-Dependent Epileptic Encephalopathies
Authors: Donat Jane F.
Published: December 2008

The most severe epilepsies that affect neonates, infants, and children include Ohtahara, West, and Lennox-Gastaut syndromes. These three syndromes display considerable similarities and transitional features in their clinical symptoms, seizure phenomena, and electroencephalographic abnormalities. This review examines the similarities and differences between these three syndromes and the other severe epilepsies of infancy and childhood, and discusses the hypothesis that the three disorders form a continuum of epileptic encephalopathies that have a predictable age-related evolution. ( J Child Neurol 1992;7:7-21).
Preliminary Evidence Suggesting Delayed Development in the Hypoglossal and Vagal Nuclei of SIDS Infants: A Necropsy Study
Authors: Konrat Georgina , Halliday Glenda , Sullivan Collin , Harper Clive
Published: December 2008

Little neuropathology has been documented in sudden infant death syndrome (SIDS) infants. Two hypotheses predict abnormalities in the hypoglossal nucleus and dorsal motor nucleus of the vagus: first, that upper airways are obstructed as a result of abnormal innervation (principally the hypoglossal nerve), and second, that they are obstructed as a result of abnormal cardiorespiratory control (the vagus nerve). A quantitative morphometric analysis was carried out to test these hypotheses in SIDS infants and controls (who died in accidents). The following nuclei dimensions were analyzed; length, volume, density, and estimated total cell number. In addition, cell size was analyzed. There were no differences in the anatomical distribution, site, or number of neurons between the groups. The most significant difference between the SIDS and control infants was the neuronal size: control infants had significantly larger neurons. In many other variables, there were trends suggesting a difference between the groups: the volume occupied by the neuronal populations was smaller in the SIDS infants, and therefore the neuronal density was increased. These values suggest differences in the development of these nuclei between SIDS and control infants. ( J Child Neurol 1992;7:44-49).
Maternal Toxemia Is Associated With Reduced Incidence of Germinal Matrix Hemorrhage in Premature Babies
Authors: Kuban Karl C.K. , Leviton Alan , Pagano Marcello , Fenton Terence , Strassfeld Ruth , Wolff Mildred
Published: December 2008

To evaluate prenatal and perinatal risk factors for development of germinal matrix hemorrhage-intraventricular hemorrhage (GMH-IVH), we conducted a prospective epidemiologic study of 449 babies whose birth weight was less than 1501 grams. This study permitted us to test our previously generated hypothesis that babies born to mothers with preeclampsia were at substantially reduced risk of developing GMH-IVH. Seventy-two (16%) of the babies in this population developed GMH-IVH. One (2.5%) of the 40 mothers with a diagnosis of preeclampsia and 71 (17.4%) of 409 mothers without preeclampsia gave birth to babies who developed GMH-IVH. GMH-IVH was seen in 6/107 (5.6%) of babies born to women with hypertension including 4/69 (5.8%) of babies born to women with pregnancy-induced hypertension, compared to 66/352 (18.8%) of babies born to mothers who did not have hypertension. Only 7.3% (8/108) of babies born to women who had proteinuria had GMH-IVH, compared to 18.3% (64/350) of babies whose mothers did not have proteinuria. GMH-IVH was seen in 5/89 (5.6%) of babies whose mothers had both hypertension and proteinuria, whereas 63/332 (19%) of babies born to mothers who lacked both factors, developed GMH-IVH. In stepwise logistic regression analysis, these significant findings were not explained by the presence of labor, postnatal acidemia, need for intubation, antenatal administration of steroids, birth weight, or gestational age. In addition, we found that maternal receipt of magnesium sulfate was associated with diminished risk of GMH-IVH even in those babies born to mothers who apparently did not have preeclampsia. We postulate that the association of preeclampsia and GVH-IVH may be related to prostaglandin effects. Reduced maternal, placental, and umbilical prostaglandin I2 (PGI2) values are characteristic of women with preeclampsia. Thus, babies born to mothers with preeclampsia may have a physiologic state similar to those having received indomethacin, a cyclooxygenase blocker that causes diminished PGI2 levels and an effective GMH-IVH prophylactic agent. We conclude that early third-trimester maternal preeclampsia/toxemia is associated with reduced risk of GMH-IVH in the preterm newborn. ( J Child Neurol 1992;7:70-76).
Developmental Manual Dyspraxia: A Lesson in Mind and Brain
Authors: Deuel Ruthmary K. , Doar Bertha P.
Published: December 2008

For decades, pediatricians assiduously documented "soft neurologic signs" in children referred for school learning difficulties, although pediatric neurologists, including Charles F. Barlow, pointed out the dissociation between most neuropsychological abnormalities and the motor findings in question. Later, large epidemiologic studies confirmed the independence of soft signs from other neuropsychological defects. We used a standardized dyspraxia battery to study 164 schoolchildren 5 to 12 years old. We found that in the group as a whole there was a positive correlation between motor performance in the dyspraxia battery and IQ on the Wechsler Intelligence Scale for Children-revised (Full-Scale, Verbal, and Performance). In terms of dyspraxia subscales, we found that Performance IQ and Full-Scale IQ correlated with imitation of nonsense gestures and use of actual objects, whereas Verbal IQ correlated positively only with pantomime on command. In contrast, for the group of 24 subjects whose scores on the battery designated them as dyspraxic, there was no correlation between dyspraxia scores and IQ. Together with the existence of specific, dramatically different, types of dyspraxia among the 24 dyspraxic subjects, present findings uphold the earlier neurologic opinion that "motor soft signs" are not evidence for fixed "brain damage" or even for other types of motor dysfunction, and that dissociation of different specific cognitive dysfunctions is the rule within individual patients. ( J Child Neurol 1992;7:99-103).
Circadian Rhythm in Patients With Hydranencephaly
Authors: Hashimoto Toshiaki , Fukuda Kuniaki , Endo Shoichi , Miyazaki Masahito , Murakawa Kazuyoshi , Tayama Masanobu , Kuroda Yasuhiro
Published: December 2008

Circadian rhythm and sleep were studied in three hydranencephalic infants who were diagnosed on the basis of computed tomographic and/or magnetic resonance imaging scans and electrophysiologic findings. In all three cases, although the active sleep cycle was preserved, quiet sleep decreased and indeterminate sleep increased. The sleep-circadian rhythm was disturbed in all three cases. The hormone secretion rhythm was studied in two cases (cases 1 and 3). In both cases, cortisol secretion showed two or three peaks during the day. In one case (case 3), growth hormone secretion did not show sleep enhancement. Prolactin secretion showed an increase during sleep in both cases. The circadian rhythm of body temperature appeared at 6 months of age and disappeared after 1 year of age in case 1. Case 2 did not show a circadian rhythm of body temperature, but case 3 did at 2 years 6 months of age. However, it was thought that the circadian rhythm of body temperature in case 3 was a false one due to severe opisthotonus. Thus, it is suggested that the development of the circadian rhythm may require the rostral brain structure more than the midbrain and that there may be multiple oscillators in humans ( J Child Neurol 1992;7:188-194).
Acute Transverse Myelitis in a 15-month-old Girl: Report of a Case With MRI Findings
Authors: Yamamoto Katsuya , Nakagawa Hiroshi , Kato Seiichi , Abe Jun-ichiro , Inoue Shigeo , Shibuya Hidenori
Published: December 2008

A 15-month-old girl demonstrated progressive weakness in all limbs. Magnetic resonance imaging (MRI) on admission revealed (1) diffuse and symmetric cervical cord swelling, (2) diffuse decrease and increase in signal intensity within the affected cord on T1- and T2-weighted images, respectively, (3) preserved anatomic structure of gray and white matter of the cord, and (4) lack of gadolinium-diethylenetriamine pentaacetic acid (Gd-DTPA) enhancing effect. She showed rapid clinical improvement in response to steroid therapy, and repeat MRI showed marked reduction in the degree of cord swelling and abnormal signal intensity. Based on the above clinical and MRI findings, the diagnosis of acute transverse myelitis was made. Although various pathologic conditions also could produce similar cord swelling and abnormal signal intensity on MRI, the third and fourth findings mentioned above suggested that the lesion was inflammatory rather than neoplastic. Our case indicates that MRI may be informative in differentiating acute transverse myelitis from other intramedullary disorders. ( J Child Neurol 1992;7:208-212).
Follow-up Study of Muscle Function in Children of Mothers With Myasthenia Gravis During Pregnancy
Authors: Ahlsten Gunnar , Lefvert Ann-Kari , Osterman Per-Olof , Stalberg Erik , Säfwenberg Jan
Published: December 2008

Most infants whose mothers have myasthenia gravis are healthy at birth, but 10% to 15% have a transient neonatal form of myasthenia gravis. In this study, the muscular function and neuromuscular transmission were examined in 31 children, aged 3 months to 31 years (median, 10 years), of 15 myasthenic mothers. Eleven of these children had had the neonatal form of myasthenia gravis. The children were examined clinically and with neurophysiologic methods. Blood samples were taken for HLA typing, creatine kinase levels, and myoglobin and acetylcholine receptor antibody studies. Twenty-nine of the 31 children had no signs of neuromuscular disease. Two children (who had had neonatal myasthenia gravis) had a moderate stationary myopathy, probably unrelated to the myasthenia gravis of their mother. Creatine kinase levels were normal for all subjects. Acetylcholine receptor antibody levels were similar to those of a control population. The HLA type B8 antigen was not significantly more prevalent in the children who had had neonatal myasthenia gravis than in the healthy children. Neonatal myasthenia gravis in a previous sibling was the only factor in the material that predicted the occurrence of myasthenic symptoms in the neonatal period. (J Child Neurol 1992;7:264-269).