bluuebunny/crossref_metadata_2025 huggingface dataset:
The Role of Models in Secondary Science Instruction
Authors: Woodward John
Published: December 2008

This article reviews common curricular alternatives for addressing the needs of secondary students with learning disabilities in science. For the most part, special education strategies for modifying science materials reflect generic techniques. Support for these methods is based on the argument that they place relatively few demands on teachers. Moreover, these methods readily apply to the commercial texts used in general and special education classrooms, however, the increasing complexity of secondary materials, as well as the call for a richer understanding of science. Make techniques like direct instruction, mnemonics, and graphic organizers less tenable. A curriculum revision method that emphasizes models and context-rich problem solving is proposed as a contemporary alternative.
Instructional Materials for Diverse Learners
Authors: Tyree Rhonda Beach , FIORE THOMAS A. , COOK REBECCA A.
Published: December 2008

In today's diverse classrooms, teachers need and students deserve educational tools that serve a wide range of learners. Fortunately, making instructional materials more accommodating does not have to mean sacrificing the needs of one group of students for those of another. Many features that are good for diverse learners are good for all learners. Text characteristics that have been shown to enhance learning are proposed to improve the audience appropriateness, text organization, and use of organizational aids in instructional materials. Reform of the design, selection, and use of materials will require a concerted effort by publishers, policymakers, and practitioners.
New Perspectives on Teaching From Successful Adults with Learning Disabilities
Authors: REIFF HENRY B. , GINSBERG RICK , GERBER PAUL J.
Published: December 2008

The experiences of adults with learning disabilities who have found success in employment may provide new perspectives about teaching students with learning disabilities. A qualitative research project with 71 successful adults with learning disabilities indicates that their individual achievements share a number of commonalities. Moreover, these commonalities represent skills and processes that can be taught and learned. This article presents a model of vocational success developed from interviews with the 71 subjects. The authors discuss methods so that practitioners may use the model to help students with learning disabilities inculcate behaviors that will facilitate successful adult adjustment. Instructional approaches that teach these skills and processes will embrace a success rather than deficit model, a focus on strengths rather than a preoccupation with weaknesses.
The Use of a Daily Calendar to Increase Responsibilities Fulfilled by Secondary Students with Special Needs
Authors: FLORES DEBORAH MAE , SCHLOSS PATRICK J. , ALPER SANDRA
Published: December 2008

This paper describes the use of a personal calendar to increase responsibilities fulfilled by secondary students with special needs. A multiple baseline, across-skills design demonstrated that students with disabilities can be taught to carry and use a personal calendar. Using a calendar was associated with an increase in the extent to which personal responsibilities were accomplished. Finally, follow-up data indicated that calendar usage and responsibility completion was maintained beyond initial training.
State Policies Affecting School-Linked Integrated Services
Authors: SULLIVAN CANDACE J. , SUGARMAN JULE M.
Published: December 2008

This article describes current federal, state, and local initiatives affecting school-linked integrated services and notes significant changes in federal policies and programs that will influence future developments. after delineating barriers to services integration efforts, outlining state policy trends, and addressing issues of finance, we describe some promising state approaches and consider implications of recent federal actions devolving much responsibility for federal programs to states and localities. the article is intended to be a useful guide for educators seeking to understand how state and local policies and programs related to school-linked services integration efforts affect the ability of school and communities to operate effective, inclusive education programs.
Consultation as a Technology and the Politics of School Reform
Authors: Fuchs Douglas , Fuchs Lynn S.
Published: December 2008

Special education and school psychology researchers have demonstrated that consultation can be effective. Yet, practitioner surveys suggest it is used infrequently. What accounts for this research-to-practice gap? We argue that consultation is effective and unused for the same reason: It is an educational technology. That is, whereas its developers have perfected a methodical and replicable problem-solving process that draws on an armamentarium of validated interventions, this very model and related methods are viewed as out of step with the zeitgeist of school reform. We explore this unfortunate irony and propose several modest recommendations.
Attitudes and Attributes of General Education Teachers Identified as Effective Inclusionists
Authors: Olson Myrna R. , Chalmers Lynne , Hoover John H.
Published: December 2008

School principals and special education teachers identified general education teachers who were the most skilled at including students with disabilities in their classrooms. After 10 individuals identified by both principals and special educators were interviewed, seven themes emerged. These teachers (a) described their own personalities as tolerant, reflective, and flexible; (b) accepted responsibility for all students; (c) described a positive working relationship with special educators; (d) reported adjusting expectations for integrated students; (e) indicated that their primary inclusionary attitude was showing interpersonal warmth and acceptance in their interactions with students; (f) felt that there was insufficient time available for collaboration; and (g) expressed reservations about fully including all students. Results are discussed in terms of teacher preparation, administrative practices, implications for increased inclusion, and suggestions for further research.
An Exploratory Study of Mainstreamed Seventh Graders' Perceptions of an Inclusive Approach to Instruction
Authors: Summey Heidi K. , Strahan David B.
Published: December 2008

We conducted a case study of 11 seventhgrade students with mild disabilities who were included in a general language arts classroom. We interviewed students, administered individual reading inventories, and observed lessons. Based on this information, we generated student profiles that provided a framework for planning a unit on the novel the outsiders. During the teaching of the novel, we served as participant observers. At the end of the semester, we administered a reading assessment and conducted follow-up interviews. Results indicated that all 11 students were more engaged in classroom activities during the unit, and 8 of the 11 demonstrated more consistent reading strategies. Results underscore the complexity of inclusion and suggest that some of these students may need more intensive, one-on-one intervention.
Classroom Teachers' Instructional Interactions with Students Who Are Exceptional, At Risk, and Typically Achieving
Authors: Jordan Anne , Lindsay Linda , Stanovich Paula J.
Published: December 2008

The conversational interactions of 9 teachers and their third-grade students were recorded during individual seatwork time in academic lessons. teachers' views about their responsibilities in working with students who are exceptional or at risk of academic failure were quite divergent and were related to their instructional discourse strategies. teachers who saw themselves as instrumental in effective inclusion engaged in more academic compared to nonacademic interactions. this group also exhibited greater use of techniques to extend students' thinking, compared to those teachers who held contrasting views. they also interacted more with their students who are exceptional and at risk than with their typically achieving students, and at higher levels of cognitive extension than did the other teachers, who seldom interacted with the students who were in the exceptional and at-risk group. the results shed light on how teachers differ in adapting instruction for students in inclusive classrooms, and how instruction might be differentially delivered as a function of teachers' views about inclusion.
Effects of Acceptability on Teachers' Implementation of Curriculum-Based Measurement and Student Achievement in Mathematics Computation
Authors: Allinder Rose M. , Oats Robert G.
Published: December 2008

We investigated the hypothesis that treatment acceptability influences teachers' use of a formative evaluation system (curriculum-based measurement) and, relatedly, the amount of gain effected in math for their students. Twenty-one special education teachers implemented curriculum-based measurement for 4 months. On the basis of their responses to a questionnaire assessing treatment acceptability of curriculum-based measurement, teachers were divided into groups (high- and low-acceptability). We compared the two groups of teachers on (a) five measures of implementation and (b) amount of growth evidenced by their students in math. Results indicated that high- and low-acceptability teachers differed on two of five implementation measures, and that there was a significant difference in the rate of growth effected in their students in math. Implications of results relative to the use of formative evaluation and measurement of treatment acceptability are discussed.
Can Computers Teach Problem-Solving Strategies to Students with Mild Mental Retardation?
Authors: Mastropieri Margo A. , Scruggs Thomas E. , Shiah Rwey-Lin
Published: December 2008

In this investigation, students with mild mental retardation were provided with an animated tutorial computer program to learn mathematical problem solving. after training, results indicated that all students obtained significant gains from pretests to posttests; further, all students reported positive attitudes toward computers after training. transfer of computer-assisted problem solving to paper-and-pencil problem solving was less consistent. anecdotal observations indicated that students appeared to rely on asking the adult trainer for assistance during initial tutorial sessions, but demonstrated more independence at the concluding sessions. implications for future research and practice are discussed on the basis of these preliminary findings.
Deconstructing Disability: A Philosophy for Inclusion
Authors: Danforth Scot , Rhodes William C.
Published: December 2008

This article offers derrida's deconstruction as a philosophy and practical strategy that challenges the assumed, factual nature of "disability" as a construct explaining human differences. The appeal of deconstruction lies in the contradictory philosophy currently articulated by the inclusion movement, a philosophy that simultaneously supports the disability construct as objective reality while calling for students "with disabilities" to be placed in educational settings designed for students considered nondisabled. This article proposes deconstruction as one coherent philosophical orientation for inclusion, an approach that critiques the political and moral hierarchy of ability and disability. A deconstructionist critique of disability is explained and demonstrated. Practical suggestions for the utilization of deconstruction by special educators are outlined.
The Effect of Parent Participation in Strategies to Improve the Homework Performance of Students Who Are At Risk
Authors: Callahan Kevin , Rademacher Joyce A. , Hildreth Bertina L.
Published: December 2008

The effect of teaching parents of at-risk students to facilitate a home-based self-management program to improve homework performance and academic achievement was Investigated. The parents of 26 sixth- and seventh-grade students from two middle school programs for at-risk youth received training and implemented home-based self-management and reinforcement strategies. Results indicated that overall levels of homework completion and homework quality increased significantly for those students whose parents consistently implemented the 10-week homework program. Significant increases in mathematics achievement also occurred. These results suggest that the practice of homework may be an important element of academic programming for students at risk and students with disabilities and that parents may play a primary role in the homework process.
Visions of the Special Class
Authors: Safford Philip L. , Safford Elizabeth J.
Published: December 2008

ABSTRACT

Although the advent of public school special day classes for children with disabilities at the end of the 19th century and the beginning of the 20th marked a major shift in the service model, apparently in the direction of educational and societal inclusion, such classes had different meanings for different students. They reflected at least three different “visions,” which can be understood by the metaphors cluster, clinic, and clearing-house, though the last was not sustained for the students for whom it was intended, nor can it be today. The story of each may offer guidance for special educators today and in the future.
A National Survey of State Initiatives on Alternative Education
Authors: Katsiyannis Antonis , Williams Brenda
Published: December 2008

Concerns over violence, vandalism, disruption, substance abuse, school failure, and dropout rates in the schools have resulted in an increased emphasis on alternative education. The purpose of this study was to examine the availability and nature of state legislative and policy mandates regarding alternative education. Among respondents, there was variability in state policy/legislative mandates and funding mechanisms, acceleration in alternative education activity, a commitment to providing technical assistance, limited state compliance-monitoring and program evaluation, and an emphasis on interagency collaboration and partnerships with the private sector. Implications for practice and further research are provided.
Dyslexia Marker Variables
Authors: Spagna Michael E.
Published: December 2008

Despite warnings that the field of learning disabilities (LD) must address the issue of population heterogeneity, the LD research community still lacks operational definitions of specific learning disabilities as well as a systematic approach for reporting sample characteristics. Recently, however, a definition of dyslexia has been proposed that might signal a significant advance. This article builds on this definition of dyslexia by: (a) reintroducing the concept of marker variables, (b) proposing a strategy for developing an updated marker variable system, (c) presenting a preliminary working set of dyslexia marker variables, and (d) calling for the eventual adoption of this or similar marker variable systems to facilitate future research efforts.
Scientific Basis of Spasticity: Insights from a Laboratory Model
Authors: Thompson Floyd J. , Parmer Ron , Reier Paul J. , Wang David C. , Bose Prodip
Published: December 2008

A variety of central nervous system injuries, diseases, and developmental deficits can lead to motor disorders that present complex mixtures of symptoms. Those that have a fundamental similarity characterized by the appearance of exaggerated velocity-dependent resistance to the lengthening of skeletal muscles are called spasticity. Reports based on clinical observations of motor disorders have and continue to provide the essential database of information regarding the range and distribution of unifying and discordant features of spasticity. Laboratory investigations employing animal models of motor disorders following experimental lesions of the central nervous system have reproduced some of the neurophysiologic changes that accompany injury of the central nervous system in humans. Those experimental lesions produced by spinal cord contusion/compression reproduce many of the histopathologic features displayed in traumatic injury of the human spinal cord as well. Studies using this model have revealed not only changes in reflex threshold and amplitude but also alterations in fundamental rate-modulation processes that regulate reflex excitability during repetitive stimulation. This report characterizes insights obtained from a laboratory investigation in search of fundamental mechanisms that contribute to the development of spasticity and provides a vantage point for understanding therapeutic strategies for treatment of spasticity. (J Child Neurol 2001;16:2-9).
Clinical Considerations in Cerebral Palsy and Spasticity
Authors: Brunstrom Janice E.
Published: December 2008

The ultimate goal for management of patients with cerebral palsy is to help them grow up to become as independent as possible, learn to make their own choices in life, and pursue their own dreams. Optimal mobility is crucial to achieving independence and is also necessary for better health and quality of life in these patients. This article discusses the treatment of spasticity in cerebral palsy, addresses tone management issues in relationship to mobility and physical fitness, and introduces the reader to a comprehensive approach to the management of patients with cerebral palsy. (J Child Neurol 2001;16:10-15).
Physical Management of Spasticity
Authors: Helsel Patty , McGee Jennifer , Graveline Chantal
Published: December 2008

Physical management of people with spasticity is a complex task requiring the collaborative work of a multidisciplinary team. This article reviews some of the most commonly used treatment techniques and theories that lay the framework for the physical management of spasticity. Most crucial to the management of the person with spasticity is the skillful application of the techniques and theories discussed. Given the complexity in the standardized evaluation of children with spasticity, it is recommended that existing outcome measures or standardized scales be applied wherever possible as an adjunct tool to the clinician's assessment and interventions. One must recognize the benefits of such scales and their limitations. Therefore, it is recommended that further development of standardized assessments be supported by funding agencies to develop both quantitative and qualitative measurement tools that will address the particular needs of the person with spasticity. Review of the literature supports the many interventions that are listed; however, further research is recommended by the consensus group. (J Child Neurol 2001;16:24-30).
Clinical Utility of Botulinum Toxin in the Treatment of Cerebral Palsy: Comprehensive Review
Authors: Edgar Terence S.
Published: December 2008

The physical properties, mechanism of action, and clinical evidence supporting the use of botulinum toxin in the management of spasticity in cerebral palsy are discussed. Assessment methods, patient selection criteria, and methodology for preparation and administration of botulinum toxin are discussed in detail and a treatment algorithm based on the cumulative experience of the author is provided. Botulinum toxin type A is well tolerated, safe, and effective in the treatment of patients with spastic cerebral palsy. Appropriate patient selection is imperative. Treatment goals need to be well defined and tailored to the individual patient's needs. Growth and development is a continuous and evolving process, necessitating the constant reassessment of the patient and modification of future treatment goals. The ultimate success of management in cerebral palsy is dependent on the development of a comprehensive spasticity team with complementing skills who, together, can significantly improve the quality of life of these patients. (J Child Neurol 2001;16:37-46).
Open-Label Study of Botulinum Toxin for Upper Limb Spasticity in Cerebral Palsy
Authors: Wong Virginia , Ng Annie , Sit Patsy
Published: December 2008

The objective of this study was to assess the efficacy of botulinum toxin for upper limb spasticity in cerebral palsy. An open-label study was conducted in 11 children with cerebral palsy. Post-botulinum toxin assessment was conducted in weeks 1, 4, and 16, with averaged scores being analyzed. The Clinical Global Impression Scale of the mothers showed marked, moderate, and mild improvement in five, four, and two cases, respectively. The Modified Ashworth Spasticity Scale score showed a statistically significant decrease in the mean spasticity score (P < .003). Other tests were performed depending on the cognition of the child. Increase in joint motion occurred in all five children assessed using web space (P = .043). For the Jebson Hand Function Test, improvement occurred in all five cases assessed (P < .03). Four of five (80%) children could perform tasks that they failed before they were given botulinum toxin. Botulinum toxin is useful in decreasing spasticity and improving the upper limb function of young children with cerebral palsy with normal cognition. Motivated families should be selected with a specific target of using botulinum toxin as an adjunct in a habilitation program. (J Child Neurol 2002;17:138-142).
Hyperekplexia in a Girl With Posterior Fossa Malformations
Authors: Goraya Jatinder S. , Shah Durlabh , Poddar Banani
Published: December 2008

Hyperekplexia is a rare clinical syndrome of pathologic startle response to unexpected stimuli such as sound or touch. The majority of cases are familial. Sporadic cases of hyperekplexia have also been reported, mostly in association with brainstem pathology. We describe a girl with sporadic hyperekplexia secondary to previously unreported cerebellar pathology. Her symptomatology was predominantly unilateral, being ipsilateral to the cerebellar involvement. This type of presentation, which may be termed hemihyperekplexia, has not been described before. Response to clonazepam was good but showed diurnal variations. The case is being reported for its unusual features. (J Child Neurol 2002;17: 147-149).
Successful Treatment of Severe Infantile Hyperekplexia With Low-Dose Clobazam
Authors: Stewart Wendy A. , Wood Ellen P. , Gordon Kevin E. , Camfield Peter R.
Published: December 2008

We report two cases of severe infantile hyperekplexia successfully treated with low-dose clobazam. The first case presented at 6 weeks of age with multiple episodes consisting of difficulty diapering because of stiffness and loud inspiratory noises followed by breath-holding in inspiration. She was diagnosed with hyperekplexia and started on clonazepam 0.05 mg daily. This was discontinued because of excessive sleepiness. The second case presented at 3 weeks of age with episodes of crying that would change in pitch and then abruptly stop, followed by leg and arm extension and stiffening. On occasion, there was cyanosis, and she received mouth-to-mouth resuscitation. She was diagnosed with hyperekplexia at 9 months of age. Both infants were treated with clobazam (0.25 and 0.3 mg/kg/day respectively), resulting in resolution of symptoms with no side effects. During treatment, both had minimal startle response to various stimuli and have now been successfully weaned from clobazam. Low-dose clobazam is effective in the treatment of hyperekplexia and is well tolerated in infants. (J Child Neurol 2002;17:154-156).
Review Article: Anterior Cervical Epidural Abscess With Pneumococcus in an Infant
Authors: Marks Warren A. , Bodensteiner John B.
Published: December 2008

Spinal epidural abscess is a rare infection in childhood. We report the first documented case of pneumococcal epidural abscess in an infant and review the literature regarding this entity. In children, the signs and symptoms of spinal epidural abscess may not be as helpful as those in older patients. Furthermore, the offending organism may not be the usual Staphylococcus seen in adults. Infants may recover neurologic function even after prolonged cord compression; however, a high index of suspicion is needed to make the diagnosis in a timely fashion. ( J Child Neurol 1987;2:25-29).
Review Article: Update on Bacterial Meningitis
Authors: Kaplan Sheldon L. , Fishman Marvin A.
Published: December 2008

Recently, advances in identifying the etiologic agent, improving antibiotic therapy, and understanding the pathogenesis of complications of bacterial meningitis have been made. The acute and long-term sequelae and their courses have been documented. Acridine orange staining of the cerebrospinal fluid may identify bacteria in children with partially treated meningitis when gram-staining is not helpful. Monoclonal antibodies for meningococcus group B antigen have been developed and may prove useful for testing cerebrospinal fluid. Several newer cephalosporins have been shown to have excellent in vitro activity against the bacteria commonly associated with meningitis. They are indicated in the treatment of infants between 4 and 8 weeks of age, children in septic shock, children with liver disease, and children with infection with gram-negative enteric agents or bacteria resistant to ampicillin and chloramphenicol. Vasculitis and cerebral infarction may result in some of the complications, such as seizures and hemiparesis, noted in children, and their consequences can be documented by various neuroimaging procedures. The prognosis for ataxia is good, while that for sensorineural deafness is poor. The majority of children will have neither intellectual deficits nor difficulty with academic achievement. An effective vaccine against Haemophilus influenzae type b has been developed and is recommended for children between 18 and 60 months of age. ( J Child Neurol 1988;3:82-93).
Moyamoya Disease in an Infant With Autonomic Dysfunction: Angiographic and MRI Findings
Authors: Welch William C. , McBride Margaret , Kido Daniel K. , Nelson Curtis N.
Published: December 2008

An infant with persistent Harlequinism went on to develop a hemiparesis secondary to Moyamoya disease at 21h months of age. The sympathetic nervous system is proposed to be an etiologic factor in the pathophysiology of Moyamoya disease as well as Harlequinism. This is one of the youngest patients reported in the English literature with Moyamoya disease and the only report of the coexistence of Moyamoya disease and atypical Harlequinism. Magnetic resonance imaging led to the diagnosis, which was confirmed by cerebral angiography. (J Child Neurol 1988;3:110-113).
Peripheral Neuropathy in Four Cases of Group A Xeroderma Pigmentosum
Authors: Tachi Nobutada , Sasaki Kimio , Kusano Takashi , Wakai Shuji , Nagaoka Masato , Annaka Shunpei , Minami Ryoji , Imamura Shigetaka
Published: December 2008

We describe the clinical features and findings of biopsied sural nerves of 4 cases of xeroderma pigmentosum. Nine genetic forms of xeroderma pigmentosum have been reported by complementation studies. These four cases were diagnosed as Group A xeroderma pigmentosum by complementation studies using cultured skin fibroblasts. All cases had delayed mental and motor development in areas such as head control over 4 months of age and walking without support over 18 months of age. Three cases had the gradual onset of a gait disturbance between 6 and 9 years of age. Motor conduction velocity and sensory conduction velocity of the ulnar nerve were slightly delayed. The sural nerve of the slightly impaired patient showed a normal density of myelinated fibers, but a selective reduction of the large myelinated fibers with zebra-body-like structures in Schwann cell cytoplasm. The population density of all nerve fibers was severely diminished in the severely impaired cases. Ultrastructural observation disclosed many denervated Schwann cells and pockets of collagen isolated by loops of denervated Schwann cell cytoplasm. These findings suggest that the degenerative process in peripheral nerves of xeroderma pigmentosum is axonal. Peripheral neuropathy in Group A xeroderma pigmentosum resembles that of patients with ataxia telangiectasia who are known to have a defect in the repair mechanisms of their DNA in cultured skin fibroblasts. (J Child Neurol 1988;3:114-119).
Congenital Blindness, Porencephaly, and Neonatal Thrombocytopenia: A Report of Four Cases
Authors: Manson James , Speed Isobel , Abbott Kimball , Crompton John
Published: December 2008

Four unrelated infants with neonatal thrombocytopenia associated with congenital blindness and porencephaly have been seen over an 18-year period. The association of congenital blindness with neonatal thrombocytopenia has not previously been reported. All children had clinical purpura in the neonatal period; in three cases, thrombocytopenia was confirmed, while in one case, the diagnosis of thrombocytopenia was presumptive; in two cases, there was evidence of circulating maternal serum platelet isoantibodies. Extensive investigation for intrauterine infection was negative in the three cases with confirmed thrombocytopenia. The thrombocytopenia resolved spontaneously after the neonatal period. It is postulated that the porencephalies were the consequence of prenatal cerebrovascular episodes. The etiology of the optic atrophy is unclear. Serial cranial ultrasound investigation is recommended for all neonates with thrombocytopenia, even if neurologically asymptomatic in the neonatal period, and serial prenatal cranial ultrasound investigation is recommended for infants of mothers with a history of having previously had infants with neonatal isoimmune thrombocytopenia. ( J Child Neurol 1988;3:120-124) .
Fibrous Dysplasia in a Patient With Tuberous Sclerosis
Authors: Breningstall Galen N. , Faerber Eric N. , Kolanu Raghuram
Published: December 2008

Though osseous abnormalities are associated with tuberous sclerosis, involvement of the skull base and orbit has not been reported. We describe a patient with superior displacement of the orbit and proptosis that were due to sphenoid hyperplasia and sclerosis characteristic of fibrous dysplasia. A pelvic osteosclerotic lesion suggested polyostotic involvement. The bony abnormalities implied neither concurrent neurofibromatosis nor McCune-Albright syndrome in this patient with tuberous sclerosis. (J Child Neurol 1988;3:131-134).
Measurement of Spontaneous Rotational Movement (Circling) in Normal Children
Authors: Gospe Sidney M. , Mora Bernardo J. , Glick Stanley D.
Published: December 2008

An asymmetry of basal ganglia dopaminergic function has been demonstrated in rats and related to both spontaneous and drug-induced rotation. An electronic device that measures the same kind of rotational movements in humans has been developed, and we have utilized this "rotometer" to study spontaneous rotational movement in prepubertal children. There was no significant difference between boys and girls in their average rate of rotation; however, left hemisphere-dominant boys were stronger rotators than left hemisphere-dominant girls. Both boys and girls made significantly more full turns to the left than to the right. These findings did not vary with age. Our observations are strikingly different from those obtained in previous studies of normal adults, in which women were stronger rotators than men, left hemisphere-dominant women turned to the left, and left hemisphere-dominant men rotated to the right. This study suggests that maturational changes in rotational behavior must occur, perhaps progressing to the adult pattern during puberty. The rotometer used in this study may provide useful information regarding the status of the basal ganglia in children with specific neurobehavioral conditions such as attention deficit disorder and Tourette's syndrome. ( J Child Neurol 1990;5:31-34).
Neuroradiological and Pathological Studies on Neonatal Aneurysmal Dilation of the Vein of Galen
Authors: Yamashita Yushiro , Nakamura Yasuhiro , Okudera Toshio , Nishiyori Atsuchi , Mikashima Takatoshi , Yamaga Hiroshi , Matsuishi Toyojiro , Kato Hirohisa
Published: December 2008

Neuroradiologic and neuropathologic findings on neonatal aneurysmal dilation of the vein of Galen are reported. Doppler echocardiography revealed a diastolic reversed Doppler signal, which suggested the presence of a cerebral arteriovenous fistula. Cerebral ultrasonography with intravenous injection of contrast medium was useful in the diagnosis. Digital subtraction cerebral angiography clearly demonstrated both the feeding and draining vessels and the aneurysmal dilation of the vein of Galen, although it failed to detect the abnormal vascular channels. Pathologic and histopathologic findings demonstrated intimal thickening of the abnormal vascular channels, which could produce higher vascular resistance and result in the detection failure with digital subtraction angiography. ( J Child Neurol 1990;5:45-48).
Extensive Wormian Bones in a Patient With the Hallermann-Streiff Syndrome
Authors: Gay Charles T. , Bodensteiner John B. , Barnes Patrick D.
Published: December 2008

A case is reported of a 5-month-old girl with Hallerman-Streiff syndrome who was evaluated for possible premature closure of the cranial sutures. Skull radiographs revealed numerous Wormian bones along sutures in the parietal skull bilaterally. Hallermann-Streiff syndrome is added to the list of diseases in which extensive Wormian bones can appear. ( J Child Neurol 1990;5:50-51).
Reduced Regional Cerebral Metabolic Rate for Glucose at the Terminal Stage in a Case of Late Infantile Neuronal Ceroid Lipofuscinosis
Authors: Fueki Noboru , Iinuma Kazuie , Kojima Akira , Yanai Kazuhiko , Haginoya Kazuhiro , Tada Keiya , Ido Tatsuo , Ito Masatoshi
Published: December 2008

Regional cerebral metabolic rate for glucose was determined for six different areas of the gray matter in an 8-year-old girl with late infantile neuronal ceroid lipofuscinosis. In all regions, the rates were almost half of the control values. The regional cerebral metabolic rate for glucose was relatively preserved in the striatal region and severely reduced in the frontal cortex. (J Child Neurol 1990;5:98-100).
Morphometric Analysis of the Human Infant Brain: Effects of Intraventricular Hemorrhage and Periventricular Leukomalacia
Authors: de la Monte Suzanne M. , Hsu F. Ida , Hedley-Whyte E. Tessa , Kupsky William
Published: December 2008

Absolute and relative growth of cerebral structures was assessed morphometrically in 34 neurologically normal live-born infants surviving up to 1 year of age, 20 infants with fatal intraventricular hemorrhage, and 4 with periventricular leukomalacia. In the normal brain, cerebral cortex grew 25% faster than white matter throughout infancy. Thus, to achieve the normal adult size and 1:1 ratio of cortex to white matter, white matter must continue to grow for several years after cessation of cortical growth. Brains with intraventricular hemorrhage or periventricular leukomalacia manifested accelerated growth of gray-matter structures compared with controls. In addition, brains with periventricular leukomalacia had markedly depressed rates of white-matter growth compared with controls of similar age, whereas brains with intraventricular hemorrhage had disproportionate hydrocephalus. The findings suggest that (1) gray-matter growth is accelerated in premature infants with intraventricular hemorrhage or periventricular leukomalacia, perhaps reflecting perinatal stress; (2) periventricular leukomalacia is associated with impaired growth of white matter during a critical period; and (3) sequential quantitative assessment of ventricular size following intraventricular hemorrhage may assist in early recognition of progressive posthemorrhagic hydrocephalus. ( J Child Neurol 1989;4:101-110).
Quantitative Morphometric Analysis of Brain Growth Using Magnetic Resonance Imaging
Authors: Schaefer G. Bradley , Thompson James N. , Bodensteiner John B. , Hamza Mosha , Tucker Robert R. , Marks Warren , Gay Charles , Wilson Don
Published: December 2008

Using a simple image analysis system, we have established normative data obtained from magnetic resonance imaging (MRI) scans for the relative size and growth of the major structural domains of the brain. Midsagittal scans of the head were analyzed in 95 subjects aged 0 to 20 years. Only scans with no demonstrable structural abnormalities of the brain were utilized. Age-related changes in the relative growth of specific structures were calculated by dividing patients into 5-year age increments. No age-related changes were found in the size of the posterior fossa, supratentorial intracranial space, or cisterna magna relative to the total intracranial vault space. A significant (P < .01) increase in the size of the corpus callosum relative to that of the supratentorial structures and relative to the total intracranial vault space was noted to occur between the 6- to 10-year-old and 11- to 15-year-old age groups. This may reflect an increase in myelination of the corpus callosum that occurs during this period. A small reduction in the ratio of the posterior fossa to the supratentorial space was found between the 0- to 5-year-old and the 6- to 10-year-old age groups, but the biological significance of this difference is uncertain. It appears from these data that quantitative analysis of MRI scans can be used to draw objective conclusions about the relative sizes and growth of the major brain structures. The development of normative data for these structures and the simplicity of the methodology should have many clinical applications in the assessment of aberrant brain development. ( J Child Neurol 1990;5:127-130).
Outcome in Children With Symptomatic Congenital Cytomegalovirus Infection
Authors: Bale James F. , Blackman James A. , Sato Yutaka
Published: December 2008

To determine factors that are associated with adverse developmental outcome after congenital cytomegalovirus infection, we reviewed the clinical, laboratory, and radiographic findings in 18 children with symptomatic congenital cytomegalovirus infections. When children with adverse outcomes (intelligence or developmental quotients of 50 or less, n = 10) were compared with children with mild sequelae (intelligence or developmental quotients of 70 or higher, n = 8), we found no relationship between developmental outcome and neonatal clinical features (birth weight, jaundice, hepatomegaly, splenomegaly, or petechiae). With the possible exception of intracranial calcifications, no single clinical or radiographic feature was associated with a specific developmental outcome. However, children who had postnatal microcephaly, postnatal seizures, and an abnormal central nervous system imaging study were more likely to have severe developmental sequelae. ( J Child Neurol 1989;4:131-136).
Review Article: Progressive Neuronal Degeneration of Childhood With Liver Disease (Alpers-Huttenlocher Syndrome): A Personal Review
Authors: Harding Brian N.
Published: December 2008

Thirty-two autopsied cases of progressive neuronal degeneration of childhood with liver disease are reviewed. The typical clinical course is intractable seizures and liver failure following a period of developmental delay and failure to thrive in early infancy, but some children first present with seizures. Characteristic changes on the electroencephalogram, loss of visual-evoked potentials, occipital atrophy on computed tomographic scan, and particular changes on liver biopsy may assist diagnosis. Most patients succumb in less than 3 years, but some have a protracted survival into their teens, and very rarely they may present in early adulthood. Liver pathology comprises fatty change, hepatocyte loss, bile duct proliferation, fibrosis, and often cirrhosis. Gradual progression can be followed in sequential biopsies. Macroscopically, the cerebral cortex is variably involved, but usually there is patchy thinning and discoloration, with a striking predilection for the striate cortex. Microscopic changes include spongiosis, neuronal loss, and astrocytosis, which progresses down through the cortical layers. All areas may be affected but the calcarine cortex is usually most affected. Etiology is still obscure, though mitochondrial and slow viral disorders have been postulated. (J Child Neurol 1990;5:273-287).
'Benign' Familial Neonatal Convulsions
Authors: Webb Randall , Bobele Gary
Published: December 2008

Nine family members over five generations are described in whom convulsions occurred in the first week of life. Routine studies, including cranial computed tomography and electroencephalography, failed to reveal an etiology in the proband, nor was a specific etiology ever found in other affected family members. All affected individuals developed normally. Three had recurrent seizures outside the first year of life. This condition is of particular importance to those who care for infants with seizures. An appropriate family history should obviate the need for unnecessary diagnostic tests as well as prolonged anticonvulsant therapy, but counseling regarding the increased risk of subsequent seizures should be provided to affected individuals. ( J Child Neurol 1990;5:295-298).
Shunt-Related Brain Abscess and Ascending Shunt Infection
Authors: Gower David J. , Horton Donald , Pollay Michael
Published: December 2008

We report the fifth case of shunt-related brain abscess. Two prior cases have been associated with colonic perforation and infection with gram-negative enteric organisms. These cases strongly suggest that ascending shunt infection is possible despite bulk cerebrospinal fluid flow in the opposite direction and add to our understanding of shunt infections. ( J Child Neurol 1990;5:318-320).
Friedreich's Ataxia in 13 Children: Presentation and Evolution With Neurophysiologic, Electrocardiographic, and Echocardiographic Features
Authors: Salih Mustafa A.M. , Ahlsten Gunnar , Stalberg Erik , Schmidt Roland , Sunnegardh Jan , Michaelsson Magnus , Gamstorp Ingrid
Published: December 2008

Thirteen children with Friedreich's ataxia were reviewed. The clinical presentation and evolution of the disease was compared to that observed in large series—based mainly on adult patients—and the few studies in children. The mean age of onset (5.3 ± 2.7 years) was lower than that reported in the former studies. Progressive unremitting ataxia of all four limbs was the earliest and most consistent finding, whereas dysarthria and loss of joint or vibration sense occurred with less frequency than that reported in adult series. The tendon jerks were absent or reduced in the lower limbs in almost all children. The universal absence of lower limb reflexes was shown to be too rigid to be obligatory for the diagnosis of early cases of Friedreich's ataxia. Electrophysiologic investigations revealed typical findings, ie, normal or low-normal motor conduction velocities and absent sensory responses. Electromyography showed more features of denervation in the lower limbs than in the upper limbs. Cardiac symptoms and signs were minimal, whereas electrocardiographic abnormalities occurred in 92% of patients, presenting mostly as significant T-wave changes. Concentric symmetric thickening of both the interventricular and left ventricular posterior walls was the major echocardiographic finding. ( J Child Neurol 1990;5:321-326).
Acquired Aphasia With Convulsive Disorder: A Pervasive Developmental Disorder Variant
Authors: Nass Ruth , Petrucha Dorothy
Published: December 2008

A 5½-year-old boy with language delay and behavioral problems was evaluated. His symptoms were consistent with pervasive developmental disorder, and electroencephalography showed frequent generalized spike and polyspike activity. After therapeutic levels of anticonvulsant medication were achieved, improvement was noted in communication skills and behavior, as well as on the electroencephalogram. Although the response of language dysfunction to these drugs has been generally disappointing in previously reported cases of communication disorders associated with electroencephalographic abnormalities, a trial of anticonvulsant medication is probably warranted. ( J Child Neurol 1990;5:327-328).
Juvenile Leigh's Encephalomyelopathy With Peripheral Neuropathy, Myopathy, and Cardiomyopathy
Authors: Grunnet Margaret L. , Zalneraitis Edwin L. , Russman Barry S. , Barwick Morven C.
Published: December 2008

A child with typical histopathologic changes of Leigh's subacute necrotizing encephalomyelopathy presented with a chronic demyelinating neuropathy. During her 11-year course, she developed an unusual myopathy and cardiomyopathy in addition to many of the previously described manifestations of Leigh's disease. Despite an extensive evaluation, the biochemical basis of her condition was never identified. This case demonstrates another unique constellation of clinical alterations associated with subacute necrotizing encephalomyelopathy, and that chronic demyelinating neuropathy can be an important initial presentation of the disease. (J Child Neurol 1991;6:159-163).
Topical Review Article: Organic Acidurias: A Review. Part 1
Authors: Ozand Pinar T. , Gascon Generoso G.
Published: December 2008

Organic acidemias are disorders of intermediary metabolism that lead to accumulation of organic acids in biologic fluids, disturb acid-base balance, and derange intracellular biochemical pathways. Their clinical presentation reflects the resultant systemic disease and progressive encephalopathy. While in some organic acidemias, disturbed acid-base metabolism is the predominant presenting feature, in others it is less prominent or even absent. The etiologies of the more than 50 different phenotypes include impaired metabolism of branched-chain amino acids, vitamins, glucose, lipids, glutathione, and γ-aminobutyric acid and defects of oxidative phosphorylation. Most organic acidemias present with neurologic manifestations, which include acutely or subacutely progressive encephalopathy that involves different parts of the nervous system. The age of presentation and the associated systemic, hematologic, and immune findings provide additional guidelines for differential diagnosis. We summarize major organic acidemias, while emphasizing their usual and unusual neurologic presentations. ( J Child Neurol 1991;6:196-219).
Neuroblastoma: A Neurochemical Approach
Authors: Schor Nina Felice
Published: December 2008

Neuroblastoma is among the most common malignancies of childhood. Despite greatly improved therapy for some pediatric tumors, the prognosis for children with metastatic neuroblastoma has not changed significantly in the past 10 years. With conventional chemotherapy, radiation therapy, and surgery, children with metastatic neuroblastoma have a 20% long-term survival rate. We describe here approaches to neuroblastoma that target its neuronal characteristics. On the one hand, the neurotransmitter receptors on the surface of the neuroblastoma cells and, on the other hand, specific isozymes that distinguish neuroblastoma cells from their normal counterparts are the focus of these experimental therapies. In the former case, specificity for tumor cells is effected by (1) selective protection of normal neuronal elements from toxicity, or (2) selective potentiation of toxicity for neural tumor cells. It is hoped that these strategies will be generalizable to other neural crest-derived tumors. (J Child Neurol 1991;6:220-228).