bluuebunny/crossref_metadata_2025 huggingface dataset:
Executive pay and the search for legitimacy: An investigation into how UK Remuneration Committees use corporate performance comparisons in long-term incentive pay decisions
Authors: Ogden Stuart , Watson Robert
Published: June 2008

This article draws on case studies from five of the UK privatized water plcs in order to examine how Remuneration Committees operationalized and justified their executive long-term incentive pay (LTIP) schemes. The article uses these as `revelatory' examples to illuminate how problematic decisions about executive pay can be. The water plc Remuneration Committees, concerned about potential stakeholder criticism, devoted considerable time and resources to ensure that their choice of LTIP comparators and performance metrics would be regarded as legitimate. The difficulties they encountered in pursuing this illustrate the underlying indeterminacy and incoherence of normative guidelines for implementing good governance in these substantive areas of executive remuneration. The article analyses this in terms of institutional incompleteness and argues that the resulting uncertainty creates theoretical as well as practical issues for resolving debates about what constitutes good governance.
Simulation Review
Authors: Parkis Michael , Shea Julia
Published: June 2008

PSYCHE-PATHS, $2.98, KMS Industries, Inc., Scientific Games Division, Ann Arbor, Michigan. 1968. Available through book and game sellers. During the week from October 16 to October 23, 1970, Julia Shea's sixth-grade class at Wylie Middle School in Dexter, Michigan, played Psyche-paths. The week prior, 4 of these students had been among the 27 from Wylie who had competed in an Equations tournament in nearby Ann Arbor, and they had won. Mrs. Shea's students are sophisticated game players. All of them have access to chess, Equations, Probe, On-Sets, Eurocard, Nim, Perquacky, and Clue, as well as games they create whenever their regular assignments are finished. They play for fun and they play to learn, and we wanted their reaction to Psyche-paths.
Simulation and Games Forum
Authors: Dukes Richard L. , Kidder Steven S. , Siegel Gilbert B. , Stadsklev Ronald
Published: June 2008

The purpose of the "Simulation and Games Forum" is to provide a medium for informal and informative discussion about games and simulation. The forum may contain comments on papers previously published; initiate a topic for discussion, unrelated to previous publications in SIMULATION AND GAMES; or give a prospectus of research projects, prior to their initiation, so that researchers with similar interests may exchange ideas and possibly arrange to share data. This section is intended to generate debate, to lay out issues on which readers might differ, and to foster communication among readers. Submissions will be published in the issue following receipt. They should be limited to 700 words (approximately 21/2 pages, typed double-space) and sent in letter form addressed to the Editors.
Simulation and Games Forum
Authors: Woodman William F.
Published: June 2008

The purpose of the "Simulation and Games Forum" is to provide a medium for informal and informative discussion about games and simulation. The forum may contain comments on papers previously published; initiate a topic for discussion, unrelated to previous publications in SIMULATION AND GAMES; or give a prospectus of research projects, prior to their initiation, so that researchers with similar interests may exchange ideas and possibly arrange to share data. This section is intended to generate debate, to lay out issues on which readers might differ, and to foster communication among readers. Submissions will be published in the issue following receipt. They should be limited to 700 words (approximately 2Y2 pages, typed double-space) and sent in letter form addressed to the Editors.
Single‐Dose Pharmacokinetics, Pharmacodynamics, Tolerability, and Safety of the Soluble Guanylate Cyclase Stimulator BAY 63‐2521: An Ascending‐Dose Study in Healthy Male Volunteers
Authors: Frey Reiner , Mück Wolfgang , Unger Sigrun , Artmeier‐Brandt Ulrike , Weimann Gerrit , Wensing Georg
Published: June 2008

The aim of the study was to assess the safety, tolerability, pharmacokinetics, and pharmacodynamics of BAY 63–2521, a new drug in development for pulmonary hypertension. Fifty‐eight healthy male volunteers received a single oral dose of BAY 63–2521 (0.25–5 mg) or placebo. No serious adverse events were reported; there were no life‐threatening events. Heart rate over 1 minute, an indicator of the effect of a vasodilating agent on the cardiovascular system in healthy subjects, was increased dose dependently versus placebo at BAY 63–2521 doses of 1 to 5 mg (P < .01). Mean arterial and diastolic pressures were decreased versus placebo at doses of 1 mg (P < .05) and 5 mg (P < .01). Systolic pressure was not significantly affected. BAY 63–2521 was readily absorbed and exhibited dose‐proportional pharmacokinetics. The pharmacodynamic and pharmacokinetic properties of BAY 63–2521 suggest that it can offer a unique mode of action in the treatment of pulmonary hypertension.
Adverse effects of prolactin in rodents and humans: breast and prostate cancer
Authors: Harvey Philip W. , Everett David J. , Springall Christopher J.
Published: June 2008

Drugs and chemicals shown to induce mammary carcinogenesis in the rat/rodent via prolactin excess have traditionally been argued to pose little or no risk to humans in a regulatory toxicology context. The basis for this assumption is reviewed and placed into context with new evidence in humans that prolactin may be a tumour promoter in the breast and prostate. This evidence includes epidemiology, patient studies involving endocrine evaluation and molecular biology in human cells. It is concluded that hyperprolactinaemia is associated with an increase in breast cancer risk in both post and premenopausal women, that rat carcinogenicity studies are predictive of the human response, and that in a regulatory toxicology context prolactin-induced mammary tumours from nongenotoxic drugs and chemicals are an adverse effect that should not be ignored. More evidence is required concerning prostate cancer risk but molecular biology indicates that prolactin also induces prostate cell proliferation and inhibits apoptosis, which are similar to the responses observed in breast cancer cells.
Pituitary volume in psychosis: the first review of the evidence
Authors: Pariante Carmine M.
Published: June 2008

The pituitary gland regulates hypothalamic-pituitary-adrenal (HPA) axis activity by secreting adrenocorticotropic hormone (ACTH), and HPA axis abnormalities have been described in psychosis. Moreover, the pituitary gland secretes prolactin, and some antipsychotics increase the secretion of this hormone. Therefore, it is possible that psychosis is associated with an abnormal volume of the pituitary, as a consequence of a dysfunction in either or both these hormonal systems. The present review of the studies conducted so far clearly indicates that the pituitary is a dynamic organ, which changes differently at different stages of the psychotic disorder, in response to both the disorder itself and the treatment with antipsychotics. Specifically, the pituitary is larger in the months immediately preceding or following the psychosis onset, independently from antipsychotic treatments. However, following this initial enlargement, the pituitary tends to become smaller, as suggested by studies in patients with psychosis of at least two years of duration. On top of these dynamic changes that are linked to the course of the disorder, antipsychotics, and especially antipsychotics inducing hyperprolactinaemia, exert additional enlarging effects on pituitary volume. We suggest that the increased pituitary volume associated with the development of psychosis is due to activation of the hormonal stress response and, specifically, to an increase in the size and number of corticotroph cells producing ACTH, while the increased pituitary volume induced by antipsychotics is linked to the stimulating effects of these drugs on lactotroph cells producing prolactin. Future studies should address these issues that are relevant in improving the care of patients with psychosis.
The Political Economy of International Economic Comparisons
Authors: Roy Donald
Published: June 2008

Of the proportion which the product of any region bears to the people, an estimate is commonly made according to the pecuniary price of the necessities of life, which is never certain, because it supposes what is far from the truth, that the value of money is always the same, and so measures an unknown quantity by an uncertain standard. It is competent enough when the markets of the same country, at different times, and those times not too distant, are to be compared; but of very little use of the purpose of making one nation acquainted with the state of another.
A New Psychometric Instrument Assessing Vulnerability to Risk of Suicide and Self-Harm Behaviour in Offenders
Authors: Perry Amanda E. , Olason Daniel T.
Published: June 2008

This study aimed to develop a new psychometric instrument to assess vulnerability to risk of suicide and nonfatal self-harm behaviour in young adult male and female offenders. In total three studies were conducted to assess the psychometric properties of the new instrument using both exploratory and confirmatory factor analysis in different samples. Participants in all three studies included a total of 1,166 young adult offenders across six Her Majesty's Prisons. The new instrument, Suicide Concerns for Offenders in Prison Environment (SCOPE), contained 28 items scoring on two subscales. The factorial structure of the new instrument initially obtained with exploratory factor analysis was subsequently confirmed in a new sample. The internal consistency of the two subscales were acceptable but the test—retest reliability coefficients were moderate. Concurrent validation with the Beck Hopelessness Scale was acceptable and SCOPE showed the ability to discriminate between those at risk and those with no known history of attempted suicide and nonfatal self-harm behaviour ( p < 0.01).
Anticonvulsant Blood Levels: Historical Review With a Pediatric Focus
Authors: Snodgrass S.R. , Parks Bruce R.
Published: June 2008

Epilepsy is heterogeneous and its treatment is often complicated by variable drug responses. Buchtal et al reported a close correlation between serum phenytoin levels, electroencephalographic fmdings, and clinical status in 1960. They suggested that physicians adjust dosage to attain a "therapeutic level." The concept was enthusiastically received. "Therapeutic serum levels" were proposed for most anticonvulsant drugs, and by 1975, most authorities believed that pharmacokinetic factors explained individual differences in drug response. However, Froscher found that measuring levels did not improve patient outcome. More recently, Schumacher's double-blind study found no correlation between phenytoin levels and seizure control or adverse effects. Pharmacodynamic variables (differences in drug responsiveness) are more important than pharmacokinetic factors for many drugs, especially receptor-active drugs. Pharmacokinetic variables were studied first, and led to a simplistic model. They are less significant than pharmacodynamic factors in the case of warfarin anticoagulation. Anticonvulsant levels can reveal noncompliance and pharmacokinetic differences. They say nothing about pharmacodynamics. Reports of "subtherapeutic levels" imply a need to increase dosage, but this is not supported by outcome data. We still lack evidence that specific drug levels are a valid intermediate target 40 years after Buchtal's paper. Responses to some anticonvulsants could depend primarily on pharmacokinetic factors, while pharmacodynamic factors could be supreme for others. (J Child Neurol 2000;15:734-746).
Continuous Neostigmine Infusion in Post-Thymectomy Juvenile Myasthenic Crisis
Authors: Briassoulis George , Hatzis Tassos , Liakopoulou Theodota , Youroukos Sotiris
Published: June 2008

A 10-year-old boy with myasthenia gravis had severe post-thymectomy myasthenic crisis necessitating reintroduction of mechanical ventilation on the 5th day after thymectomy. He did not respond to therapy with oral pyridostigmine, corticosteroids, or high-dose intravenous immunoglobulin. Finally, in addition to the usual supportive care, he was treated successfully with continuous intravenous infusion of neostigmine. Continuous infusion of neostigmine was used for the first time in post-thymectomy myasthenic crisis in a child to the best of our knowledge. ( J Child Neurol 2000; 15:747-749).
Moyamoya Syndrome After Radiation Therapy for Optic Pathway Glioma: Case Report
Authors: Serdaroglu Ayşe , Şimşek Filiz , Gücüyener Kivilcim , Oguz Aynur , Karadeniz Ceyda , Balibey Muzaffer
Published: June 2008

We present a 4-year-old girl with neurofibromatosis-1 who developed moyamoya syndrome characterized by bilateral stenosis or occlusion of the distal internal carotid arteries and their branches, leading to the development of an abnormal vascular network. In light of a literature review, the postradiation vasculopathy of the moyamoya type and its relationship with neurofibromatosis-1 are discussed. ( J Child Neurol 2000;15:765-767) .
Myasthenia Gravis and Associated Autoimmune Diseases in Children
Authors: Tsao Chang-Yong , Mendell Jerry R. , Lo Warren D. , Luquette Mark , Rennebohm Robert
Published: June 2008

Myasthenia gravis has been associated with other autoimmune disorders. We report two children with myasthenia gravis and another autoimmune disease: an 18-month-old boy with ocular myasthenia gravis and Hashimoto's disease and a 14-year-old girl presenting with autoimmune polymyositis, then generalized myasthenia gravis 2 years later. The rare combinations of myasthenia gravis and Hashimoto's disease or polymyositis in children are discussed, and we also briefly review myasthenia gravis and other associated autoimmune diseases in children. ( J Child Neurol 2000;15:767-769).
Review Article: Holoprosencephaly and Related Midline Cerebral Anomalies: A Review
Authors: Leech Richard W. , Shuman Robert M.
Published: June 2008

We propose a simple pathogenetic mechanism that reduces a bewildering variety of central nervous system malformations to a manageable group sharing defects of midline prosencephalic growth. It is neither new nor innovative, but attempts to summarize many pathologic entities within a concept that accounts for known embryologic events and the sequence and timing of those events. We propose midline prosencephalic dysgenesis as a category of malformations including aprosencephaly, holoprosencephaly, septo-optic dysplasia, and agenesis of the corpus callosum. (J Child Neurol 1986; 1:3-18)
Review Article: Nerve Conduction Studies in Infants and Children
Authors: Miller Robert G. , Kuntz Nancy L.
Published: June 2008

The electrophysiologic evaluation of peripheral nerves may provide critically important information, both with respect to diagnosis and prognosis, in the child with a suspected neuromuscular disorder. However, special attention to various technical considerations is necessary to avoid misleading results. Utilizing these techniques, both hereditary and acquired neuropathies may be identified and characterized. The latter has become especially important in view of recent advances in the treatment of acquired demyelinating neuropathies. (J Child Neurol 1986;1:19-26)
Suppressed Pituitary ACTH Response After ACTH Treatment of Infantile Spasms
Authors: Ross Diana L.
Published: June 2008

Suppression of an adrenocorticotropic hormone (ACTH) response to insulin hypoglycemia has been reported in ACTH-treated adults. There are no guidelines for withdrawal of ACTH treatment in children. After observing suppressed morning cortisol in several children, insulin tolerance tests were performed in five children within 48 hours after tapered withdrawal of ACTH treatment for myoclonic seizures. ACTH response, as determined by cortisol and β-endorphin radioimmunoassay, was adequate in four of the children. One child showed low basal levels and minimal elevation during hypoglycemia for both β-endorphin (0 to 3 pg/ml) and cortisol (3.6 to 4.4 μg/dL) on initial testing, but normal responses six weeks later. Measurement of β-endorphin response supported a central basis for suppression in the child, who had had an adrenal hemorrhage during gram-negative sepsis while on ACTH. ACTH release is transiently suppressed in some children after exogenous ACTH treatment. Tapered withdrawal and stress coverage is recommended. (J Child Neurol 1986:1;34-37).
Juvenile Acute Intermittent Porphyria With Hypercholesterolemia and Epilepsy: A Case Report and Review of the Literature
Authors: Kaplan Peter W. , Lewis Darrell V.
Published: June 2008

A case of acute intermittent porphyria in a 10-year-old boy with seizures and hypercholesterolemia is presented. The problems of management when seizures and porphyria coincide and discussion of hypercholesterolemia are included. A comprehensive review of the world literature reveals that prepubertal patients with acute intermittent porphyria are predominantly male and show an increased incidence of seizures when compared to older age groups. The principal clinical features in all age groups include abdominal pain, vomiting, fever, and tachycardia in addition to mental changes, limb paresis, and hyporeflexia. (J Child Neurol 1986;1:38-45)
Development of Paranasal and Mastoid Sinuses: A Computed Tomographic Pilot Study
Authors: O'Tuama L.A. , Swanson M.S.
Published: June 2008

Paranasal sinus and mastoid disease in children is an important potential source of intracranial sepsis. Cranial computed tomographic (CT) scans are a primary imaging modality for assessment of the paranasal sinuses and mastoids. Radiographic assessment of paranasal sinus development has largely been confined to plain radiographs and polytomographic examination. We report a pilot, quantitative analysis of 30 CT scans obtained from patients without known ear, nose, or throat disease, and aged from 3 weeks to 13.8 years. Ethmoid, sphenoid, and mastoid sinus development was assessed by a "pneumatization index" (PI) derived from each CT scan. At all ages, the rank order of sinus development was ethmoid > mastoid > sphenoid. Individual developmental rates showed considerable variation. In the newborn, mastoid and sphenoid sinus development was minimal, whereas ethmoid sinuses showed substantial aeration. The data suggest that, for children older than 5 years of age, an ethmoid sinus PI ≤ 0.714 is highly suspicious of paranasal sinus disease. (J Child Neurol 1986; 1:46-49)
Chronic Renal Failure Presenting as Proximal Muscle Weakness in a Child
Authors: Berretta Jeanne S. , Holbrook C. Tate , Haller Jerome S.
Published: June 2008

A 13-year old boy presented with a three-year history of slowly progressive proximal muscle weakness, particularly involving the lower extremities. Chronic renal failure was uncovered in the course of his evaluation. Urologic investigation showed small and poorly functioning kidneys with a BUN of 118 mg/dL and a creatinine of 10.7 mg/dL. There were no anomalies of the proximal or distal collecting systems or history suggestive of recurrent urinary tract infection. The neurologic examination revealed proximal muscle weakness primarily of the lower extremities and especially of the proximal musculature of the pelvic girdle. Nerve conduction studies were normal. The electromyogram (EMG) showed high-voltage polyphasic potentials consistent with neurogenic muscle disease. A biopsy of the right quadriceps muscle demonstrated type II muscle fiber atrophy with histochemical staining. The patient's clinical findings, EMG studies, and muscle biopsy were not specific for either neurogenic or myopathic disease. Following a period of home peritoneal dialysis and renal transplantation, there was significant clinical improvement of the muscle weakness. (J Child Neurol 1986;1:50-52)
Computed Tomography Demonstration of Cerebral Calcification in Postasphyxial Encephalopathy
Authors: Kanarek Keith S. , Gieron Maria A.
Published: June 2008

Postasphyxial, diffuse cerebral calcification was demonstrated by computed tomographic (CT) brain scans in two infants. Calcification was seen in the white matter in both patients and in the basal ganglia in one. The neurologic outcome was poor in both infants. ( J Child Neurol 1986;1:56-60)
Review Article: Topical Review of the WISC-R for Pediatric Neuroclinicians
Authors: Kaufman Alan S. , Long Sandra W. , O'Neal Marcia R.
Published: June 2008

This article will familiarize neuroclinicians with the WISC-R and with the results of countless research studies on this popular test of the intelligence of children and adolescents. In addition to a description of the WISC-R and its subtests, and a discussion of the WISC-R from a historical context, the article reviews research in the following areas: (1) relationship of the WISC-R to Wechsler's preschool and adult scales; (2) interpretation of verbal-performance IQ differences; (3) clinical implications of scatter; (4) relationship of an IQ score to present and future functioning; and (5) comparison of IQs on the WISC-R with scores on other tests for children and adolescents. (J Child Neurol 1986;1:89-98.)
Intracranial Arterial Aneurysms in Childhood: More Recent Considerations
Authors: Zee Chi-Shing , Segall Hervey D. , McComb J. Gordon , Stanley Philip , Little F. Miles , Ahmadi Jamshid , Bird C. Roger , Feldman Rochelle
Published: June 2008

Nineteen children with congenital, mycotic, traumatic and tumoral arterial aneurysms were studied neuroradiologically. The important role of computed tomography (CT) was shown in two traumatic aneurysm cases where bleeding was clinically inapparent. Among the unique cases described was a boy with a pituitary tumor in whom an aneurysm was discovered incidentally; coexistence of these lesions in childhood has not been documented previously. Nor has a tumoral aneurysm been mentioned (the case reported had a surrounding primary anaplastic sarcoma). Rare cases included a cavernous carotid mycotic aneurysm and infants with hemorrhage from congenital distal middle cerebral artery aneurysms. Marked cellular responses within the aneurysm walls, believed to be a reaction to hemorrhage, were noted in both infants. (J Child Neurol 1986; 1:99-114)
Megalencephaly Due to Impaired Cerebral Venous Return in a Sturge-Weber Variant Syndrome
Authors: Fishman Marvin A. , Baram Tallie Z.
Published: June 2008

An infant with a Sturge-Weber variant syndrome developed progressive megalencephaly and eventual hydrocephalus, which required shunting. Cerebral angiography revealed absence of the deep cerebral venous system and the development of abnormal drainage channels via the periorbital veins. It is postulated that the abnormal enlargement of the brain was due to the impaired venous return. Resistance of the brain to continued expansion may have caused an increase in hydrostatic pressure and the development of hydrocephalus. ( J Child Neurol 1986; 1:115-118)
An Outline of Continuum Modeling of Brain Tissue Mechanics
Authors: Karni Zvi , Ivan Leslie P. , Bear Jacob
Published: June 2008

Modern, very sensitive, and noninvasive devices (strain-gauges, accelerometers, etc) detect deformations and motions in the order of a few microns. The observation that the rise in intracranial pressure produces measurable suture strain suggests that noninvasive determination of mechanical parameters in the intracranial cavity, such as density, velocity, pressure, energy, and their time rates, is clearly possible. Based on the above premise, and utilizing the concept of representative elementary volume (REV), this paper presents the conceptual part of a theory that defines the mechanical behavior of the brain and skull under various conditions. The systematic review of brain tissue mechanics leads to a continuum model of a mixed stress-displacement boundary value type, which defines the dynamics of the visco-elastic brain tissue in mathematical terms. (J Child Neurol 1986; 1:119-125).
Globoid Cell Leukodystrophy: Cranial Computed Tomography and Evoked Potentials
Authors: Darras Basil T. , Kwan Eddie S. , Gilmore Herbert E. , Ehrenberg Bruce L. , Rabe Edward F.
Published: June 2008

The evoked potentials and cranial computed tomographic (CT) scan findings in a case of early infantile globoid cell leukodystrophy are presented. The brain stem auditory evoked responses (BAERs) and the flash visual evoked potentials (VEPs) were abnormal. Repeated cranial CT scans showed multiple areas of increased attenuation and progressive cerebral atrophy. These evoked potential and CT scan patterns occurring concomitantly in an infant are suggestive of globoid cell leukodystrophy. (J Child Neurol 1986;1:126-130)
Neuronal Ceroid Lipofuscinosis With Hypergonadotropic Hypogonadism
Authors: Nass Ruth , Petito Carol , Stoner Elizabeth , New Maria
Published: June 2008

A case of adolescent-onset neuronal ceroid lipofuscinosis presenting with chorea and evidencing pyramidal and cerebellar dysfunction as well as hypergonadotropic hypogonadism is reported. In this patient, primary ovarian failure may be due to accumulation of ceroid in the ovaries. ( J Child Neurol 1986; 1:142-144)
Clinical Predictors of Computed Tomography in Epileptic Children
Authors: Koprowski Christopher , Clancy Robert
Published: June 2008

The purpose of this study was to determine which clinical characteristics correlated with abnormal computed tomographic (CT) scans in epileptic children. Thirty variables were examined. Of these, four variables (presence of inherited or congenital disease, focal motor findings, developmental delay, and early onset of seizures) correlated with CT scan outcome. Pardal seizures per se were not highly associated with abnormal CT scans. A crude decision rule based on these results correctly classified the CT scan result in 94% of the patients who were clinically predicted not to have a parenchymal abnormality. ( J Child Neurol 1986; 145-148)
Review Article: Optic Nerve Hypoplasia: A Review
Authors: Ouvrier Robert , Billson Frank
Published: June 2008

Optic nerve hypoplasia is a developmental anomaly of the retina and optic nerves in which there is a reduction in the number of ganglion cells in the retina and of their centripetal fibers projecting through the optic nerve to the lateral geniculate body. The condition may be unilateral or bilateral and is frequently misdiagnosed as optic atrophy. In about 25% of cases, bilateral optic nerve hypoplasia is associated with a variety of cerebral malformations of which the commonest single disturbance is absence of the septum pellucidum (septo-optic dysplasia). Cerebral malformations and their endocrine accompaniments are also seen, though less frequently, in unilateral hypoplasia. The endocrine disturbances that may accompany optic nerve hypoplasia include growth hormone deficiency, adrenal insufficiency, hypothyroidism, and disturbances of antidiuretic hormone production. Precocious puberty and hypogonadism have also been observed. The prognosis of optic nerve hypoplasia depends upon the severity of the changes in the optic nerves and especially the degree of associated cerebral malformation. The finding of optic nerve hypoplasia should lead to thorough ophthalmologic, neurologic, and endocrinologic evaluation of the patient. ( J Child Neurol 1986;1:181-188)
Immunocytochemical Binding to Neurons of Serum from Spinocerebellar Degeneration Patients
Authors: Dhib-Jalbut Suhayl , Archer Gerald E. , Liwnicz Boleslaw H.
Published: June 2008

Antineural antibodies have been described in sera of patients with neurodegenerative disorders. We looked for the presence of those antibodies in the sera of patients with spinocerebellar degeneration. Serum IgG from four patients with familial spinocerebellar degeneration showed strong binding to cerebral cortical neurons, Purkinje cells, and dorsal root ganglia of normal human tissue sections stained with the peroxidase antiperoxidase (PAP) method at serum dilution of 1:500. No binding to neuroglia cells or cells of the granular layer of the cerebellum was seen. Sera from four immediate, asymptomatic relatives (son or sibling) showed only moderate binding to Purkinje cells and to dorsal root ganglia, but not to cortical neurons. Sera from seven patients with neurological diseases other than spinocerebellar degeneration and from five healthy subjects showed no binding to neural elements. The findings may be of value in the diagnosis and screening of patients suspected of having spinocerebellar degeneration; however, the significance of thse antineural antibodies in the pathogenesis of spinocerebellar degeneration is uncertain and awaits further studies. ( J Child Neurol 1986;1:198-205).
Continuous Infantile Spasms as a Form of Status Epilepticus
Authors: Coulter David L.
Published: June 2008

An infant with congenital cytomegalovirus infection first developed seizures at six weeks of age. At 3½ months of age, he developed continuous infantile spasms that lasted for more than an hour. This episode of status epilepticus was terminated by intravenous lorazepam and paraldehyde, and seizures were subsequently controlled for seven months by adrenocorticotropic hormone (ACTH), valproic acid, and phenobarbital. This case demonstrates that continuous infantile spasms may occur as a unique form of status epilepticus in young infants. ( J Child Neurol 1986;1:215-217).
Facioscapulohumeral Dystrophy Associated with Mental Retardation, Hearing Loss, and Tortuosity of Retinal Arterioles
Authors: Matsuzaka Tetsuo , Sakuragawa Norio , Terasawa Kenjiro , Kuwabara Hideaki
Published: June 2008

A rare case of facioscapulohumeral muscular dystrophy with an early onset and progression is described. The patient had mental retardation, sensorineural hearing loss, and marked tortuosity of the retinal arterioles. On reviewing previous reports of related cases, our case and those of Small shared similar clinical manifestations, although the retinal findings in our patient were different from the typical Coats' disease of Small's patients. However, we postulate that these cases represent a specific nosological entity, although genetic transmission has yet to be proven. ( J Child Neurol 1986;1:218-223).
Midline Telencephalic Dysgenesis: Report of Three Cases
Authors: Leech Richard W. , Shuman Robert M.
Published: June 2008

A series of three complex cerebral malformations is presented. The series was characterized by incomplete development of the commissural and chiasmatic plate of the developing forebrain. Associated anomalies included hypoplasia of the hippocampus, heterotopias, and nonfusion of the cerebellum. Mild facial anomalies were present, as were somatic anomalies of the heart and kidneys. Emphasis is placed on the order and classification of similar cerebral anomalies. ( J Child Neurol 1986;1:224-232)
Pelizaeus-Merzbacher Disease: Clinical and Nosological Study
Authors: Boulloche J. , Aicardi J.
Published: June 2008

Pelizaeus-Merzbacher disease can be diagnosed on genetic and clinical criteria. These include: (1) involvement of several males in a lineage in a manner consistent with X-linked recessive inheritance; (2) early nystagmoid movements; (3) precocious psychomotor deterioration; (4) progressive pyramidal, dystonic, and cerebellar signs. We present seven cases from three families and review 148 cases in 19 families from the literature. Laryngeal stridor present in two of our patients may be a presenting feature. Neurophysiological investigations may be helpful in the diagnosis. ( J Child Neurol 1986;1:233-239)
Cerebro-Oculo-Hepato-Renal Syndrome (Arima's Syndrome): A Distinct Clinocopathological Entity
Authors: Matsuzaka Tetsuo , Sakuragawa Norio , Nakayama Hiroshi , Sugai Kenji , Kohno Yoshiyasu , Arima Masataka
Published: June 2008

Three children with Leber's congenital amaurosis, agenesis of the cerebellar vermis, and infantile polycystic kidneys are described. The common clinical findings of three unrelated patients (two boys and one girl) included severe visual impairment from early infancy, profound psychomotor retardation, hypotonia, nystagmus, characteristic facial appearance with blepharoptosis, and progressive chronic renal insufficiency. The two boys died of uremia at ages 13 and 12 years. The common pathological findings in these two patients consisted of minor disproportions of cerebral lobes, almost total aplasia of the cerebellar vermis, micropolygyria of the dentate nuclei, malformations of the brain stem (including pachygyria of the inferior olivary nuclei and partial absence and anomalous position of the pyramidal tracts), and infantile polycystic kidneys; there was fatty liver in one case and hepatic fibrosis in the other. The clinicopathological findings of our two patients were entirely compatible with those of patients previously reported by Arima and other Japanese authors. Therefore, these patients seem to comprise a distinct clinicopathological entity, cerebro-oculo-hepato-renal syndrome ( Arima's syndrome), different from other syndromes with retinal, cerebellar, and renal abnormalities. ( J Child Neurol 1986;1:338-346)
A Randomized, Double-Blind, Crossover Study of Phenobarbital and Mephobarbital
Authors: Young Richard S. K. , Alger Pamela M. , Bauer Laurence , Lauderbaugh Debra
Published: June 2008

Some pediatric neurologists maintain that mephobarbital (Mebaral) causes fewer behavioral side effects than phenobarbital. Because this hypothesis has not been previously tested, we conducted a prospective, double-blind, randomized, crossover study of these two anticonvulsants. Both drugs were equally effective in reducing the frequency of seizure, although serum phenobarbital levels were significantly higher when the patients were taking phenobarbital compared to mephobarbital. As measured by the Abbott Parent Questionnaire, there was no significant deterioration of behavior with either phenobarbital or mephobarbital, regardless of which drug was administered first. J Child Neurol 1986;1:361-363)
Do Learning Handicaps and Headache Cluster?
Authors: Leviton Alan
Published: June 2008

Children who are brought to medical attention because of frequently recurring or disabling headaches appear to have an increased prevalence of learning handicaps. Some of this increase might reflect Berkson's principle—the selection bias of an overrepresentation of patients with two disorders. Consideration is given to five models that show a relationship between learning handicap and headache. Two of the models are dismissed as too simple. Studies are needed to determine whether learning handicap and headache are indeed associated, and, if so, how. Clinicians are advised not to wait until these studies have been completed before implementing therapeutic programs that might prove helpful to those children with both headache and learning handicap. ( J Child Neurol 1986;1:372-377)
Decreased Creatine Kinase Activity in Cultured Duchenne Dystrophic Muscle Cells
Authors: Iannaccone Susan T. , Nagy Bela , Samaha Frederick J.
Published: June 2008

Muscle cells were cultured from six patients with Duchenne muscular dystrophy and nine normal subjects. Protein and myosin content and pyruvate kinase (PK) activity were similar in normal and Duchenne muscular dystrophy cultures. Creatine kinase (CK) activity was lower in Duchenne muscular dystrophy cultures and the isoenzyme distribution indicated MB-CK was significantly lower, while BB-CK was significantly higher in later Duchenne muscular dystrophy cultures. This abnormal isoenzyme pattern suggested aberrant or impaired maturation of Duchenne muscular dystrophy myotubes in vitro. ( J Child Neurol 1987; 2:17-21).
Low Erythrocyte Zinc Content in Acquired Aphasia With Convulsive Disorder (Landau-Kleffner Syndrome)
Authors: Lerman-Sagie Tally , Statter Marian , Lerman Pinchas
Published: June 2008

Abnormal zinc metabolism in a 6-year-old male patient with Landau-Kleffner syndrome (acquired aphasia and convulsive disorder) is the subject of our report. We describe a significant decrease of erythrocyte and plasma zinc levels in the patient as compared with normal. Red blood cell zinc content is normally 38.5 ± 2.7 μg/g hemoglobin v 16.5 μg/g hemoglobin found in the patient. Plasma zinc level is 94.6 ± 10.4 μg/dL in normal in comparison with 50.4 μg/dL in the patient. Normal oral zinc tolerance test performed on the patient precludes altered zinc absorption or increased urinary loss. The possible connection between abnormal zinc metabolism and the pathophysiology of Landau-Kleffner syndrome is discussed. ( J Child Neurol 1987;2:28-30).
Pyridoxine Dependency Seizures
Authors: Pettit Ross E.
Published: June 2008

Pyridoxine dependency is a rare cause of neonatal seizures. Newborns with this disorder are often hyperirritable and fail to respond to the usual anticonvulsants. The diagnosis is established by cessation of seizures after the administration of parenteral pyridoxine. Reported is a case of pyridoxine dependency that illustrates several problems in management. The amount of pyridoxine required to control seizures is variable and may exceed 100 mg per day. The electroencephalogram (EEG) may not change significantly during the initiation of therapy. During intercurrent illnesses, parenteral pyridoxine may need to be given. Additional pyridoxine may be needed even when the EEG is normal. Treatment should continue indefinitely. ( J Child Neurol 1987;2;38-40).
Risks of Neuroleptic Drugs in Children
Authors: Silverstein Faye S. , Johnston Michael V.
Published: June 2008

We surveyed members of the Child Neurology Society to examine their attitudes about use of neuroleptic drugs in children and their experiences with neuroleptic-induced movement disorders. Sixty percent of the membership responded to our questionnaire. Of these responders, over 99% agreed that there were clinical indications for neuroleptics in children, but their criteria varied. Only a minority of the responders routinely incorporated drug holidays in the therapeutic plans of children treated with neuroleptics. The majority had not had clinical experience with patients who developed chronic movement disorders associated with neuroleptic treatment, but 35% (140/410) had encountered patients whose symptoms they considered typical of tardive dyskinesia. However, some of these patients did not meet strict criteria for diagnosis of this movement disorder. The results suggest that neuroleptic-induced movement disorders occur with significant frequency in children and that more detailed prospective studies of the risks and benefits of these drugs in children are warranted. ( J Child Neurol 1987;2:41-43).
Factors Modifying the Prognosis of Wilson's Disease in Childhood
Authors: Kudo Hideaki , Arima Masataka
Published: June 2008

The prognosis of Wilson's disease was investigated in 96 patients, in whom the disease had presented before 15 years of age and had begun between 1965 and 1983 (when D-penicillamine was widely available in Japan). In the activities of daily living, the prognosis was poor in those patients presenting with neurological symptoms. Interruption of D-penicillamine treatment was seen in one third of the patients, and it worsened the prognosis. Toxic side effects were seen in about half of the patients, being more frequent in the patients with initial neurological symptoms. A disappointing 17% of patients with slight or no side effects discontinued the drug. Death occurred in eight patients of whom seven had had initial hepatic symptoms. Not only early diagnosis and treatment before the appearance of hepatic failure or neurological symptoms, but also treatment throughout life without interruption is important for improving the prognosis of Wilson's disease. ( J Child Neurol 1987;2:57-62).
Review Article: The Inherited Neurodegenerative Disorders of Childhood: Clinical Assessment
Authors: Percy Alan K.
Published: June 2008

This review represents an examination of four groups of neurodegenerative diseases, namely the sphingolipidoses, the adrenoleukodystrophy complex, the neuronal ceroid lipofuscinoses, and the sialidoses/sialuria complex. Using a combination of clinical assessment, neuroradiologic appearance, and clinical neurophysiology, one may develop strategies that lead to specific chemical or biochemical determinations for specific diagnoses. In general, disorders of white matter may be distinguished from disorders of gray matter by their appearance on computed tomography and by abnormalities of nerve conduction velocities and auditory brain stem responses. In contrast, disorders of gray matter may be distinguished from disorders of white matter by their appearance on computed tomography and utilization of electroretinography, visual evoked responses, and, to a lesser extent, EEG findings. Where necessary, skin, conjunctival, or nerve biopsy may prove to be useful adjuncts to the diagnosis. Presently, treatment depends on prevention, although aggressive efforts are under way to establish corrective therapy by enzyme replacement. To this end, the use of already existing animal models may prove to be helpful. (j Child Neurol 1987;2:82-97).
EEG Monitoring During Paroxysmal Hyperpnea of Tetralogy of Fallot: An Epileptic or Hypoxic Phenomenon?
Authors: Daniels Stephen R. , Bates Stephen R. , Kaplan Samuel
Published: June 2008

We describe a neurologically compromised infant with tetralogy of Fallot who was having multiple paroxysmal episodes of hypotonia, eye rolling, stiffening, and loss of consciousness. Simultaneous electroencephalography with video monitoring was used to determine if these episodes were a primary epileptic phenomenon or the result of hypoxic central nervous system involvement from paroxysms of hyperpnea and cyanosis. The findings would suggest that the paroxysmal episodes were not primarily epileptic. The electroencephalographic findings during the spell were similar to those found with hypoxia due to other causes. This may have physiological and therapeutic significance and be useful in following other infants with congenital heart disease who have similar episodes, especially when the infants have significant risk factors for seizures. (J Child Neurol 1987;2:98-100).
Juvenile Dermatomyositis Induced by Toxoplasmosis
Authors: Schröter Heide M. , Sarnat Harvey B. , Matheson David S. , Seland T. Peter
Published: June 2008

A 10-year-old girl from southern Alberta, Canada, who had close contact with cats, developed typical features of dermatomyositis. The diagnosis was confirmed by muscle biopsy. A toxoplasmosis titer was 1:16,384 by indirect fluorescent antibody technique, and the IgM response to toxoplasma was positive. Only minimal improvement followed prednisone and azathioprine administration, but she rapidly improved after 4 weeks of treatment for toxoplasmosis with pyrimethamine and sulfadiazine. A year after the onset of dermatomyositis, she showed no weakness or cutaneous lesions, and a repeat muscle biopsy no longer showed inflammation, perifascicular atrophy, or regeneration of myofibers. She remains asymptomatic more than 2 years after discontinuation of all medications. Investigation for immune deficiency disease 1 year after therapy revealed that lymphocytic response to T-cell and B-cell mitogens was normal, as were immunoglobulin and complement levels. She had mild impairment of natural killer cell activity and a positive antinuclear factor. Her rapid improvement on specific therapy and lack of significant long-term immune deficiency is consistent with acute toxoplasmosis infection in an immunologically competent child. (J Child Neurol 1987;2:101-104).
CT Scan and MRI Findings in a Child With Schizophrenia
Authors: Woody Robert C. , Bolyard Keith , Eisenhauer Gail , Altschuler Lisa
Published: June 2008

Morphologic brain changes have long been recognized in some adult schizophrenic patients. The ready availability of computed tomographic (CT) scans and the recent advent of magnetic resonance imaging (MRI) now permit noninvasive neuroimaging of schizophrenic patients. We describe a prepubertal schizophrenic child with neuroradiographic findings similar to those found in adult schizophrenic patients: ex vacuo enlargement of the lateral ventricles, dilatation of the third ventricle, and cerebellar abnormalities. This patient appears to be the first schizophrenic child documented to have these morphologic changes of the brain and suggests the need for further neuroradiographic studies to define the frequency and significance of structural changes that may occur in childhood schizophrenia. ( J Child Neurol 1987;2:105-110).
Psychiatric Symptomatology in Language-Impaired Childrén: A Comparison
Authors: Cantwell Dennis P. , Baker Lorian
Published: June 2008

This paper reports a detailed comparison of two groups of children from the same psychiatric clinic, one with a delay in language development and an age- and sex-matched control group with no such delay. The language-delay group was highly likely to have either a diagnosis of mental retardation or infantile autism. Very few psychiatric symptoms occurred more frequently in the language-delay group than in the comparison group, and those that did were characteristic of retarded or autistic children. The language-delay group was more likely to have other developmental abnormalities, hearing impairment, and evidence of central nervous system dysfunction. (J Child Neurol 1987;2:128-133).
Pitfalls in Diagnosing Brain Death in Infancy
Authors: Toffol Gilbert J. , Lansky Lester L. , Hughes John R. , Blend Michael J. , Pavel D.G. , Kecskes Susan A. , Ortega Romulo E. , Tan Walter S.
Published: June 2008

A 3-year-old child with phenotypic trisomy 18 syndrome survived 26 days after a cardiopulmonary arrest, secondary to an acute viral illness. The child was deeply comatose. No barbiturates, other sedatives, or aminoglycoside antibiotics had been recently administered. The child was normothermic with adequate cardiovascular function. Brain stem function was absent, as assessed by testing of brain stem reflexes. Serial cerebral radionuclide angiograms (CRAG) documented intact cerebral blood flow while electrocerebral silence (ECS) was present on two consecutive EEG recordings within 24 hours. Preservation of intracranial circulation was confirmed by rapid rotational computed tomographic (CT) scans. Cranial CT scans also revealed communicating hydrocephalus, and bilateral basal ganglia hemorrhages. This unusual case illustrates discordance between apparent irreversible loss of cortical function as indicated by electrocerebral silence with preserved cerebral blood flow. The implications of these apparent paradoxical events will be discussed in the context of defining brain death in children. ( J Child Neurol 1987;2:134-138).
Adrenoleukodystrophy: From Bedside to Molecular Biology
Authors: Moser Hugo W.
Published: June 2008

Although X-linked adrenoleukodystrophy was first described in 1923, the key advances in understanding the disorder followed discoveries beginning in 1973 that it was characterized by the tissue accumulation of very long chain fatty acids, particularly hexacosanoic acid (C26:0). Very long chain fatty acid assays in plasma, red cells, fibroblasts, or amniocytes permit prenatal and postnatal diagnosis and carrier detection. The phenotype of X-linked adrenoleukodystrophy is varied and ranges from the severe and fatal childhood form, to persons who remain asymptomatic in adult life. The underlying biochemical defect is the impaired capacity to degrade very long chain fatty acids, a reaction which normally takes place in the peroxisome. The locus of the adrenoleukodystrophy gene has been mapped to the terminal (Q28) segment of the long arm of the X-chromosome, in close proximity to the loci of Hemophilia A and red-green color blindness. A DNA probe (St14) for this portion of the X-chromosome is of aid for carrier detection. A new dietary regimen, which combines restricted very long chain fatty acid intake with the administration of a glycerol trioleate oil, is capable of reducing plasma very long chain fatty acid levels, and may offer neurological benefit. (j Child Neurol 1987;2:140-150).