bluuebunny/crossref_metadata_2025_split huggingface dataset:
Age at onset of seizures in young children
Authors: Ellenberg Jonas H. , Hirtz Deborah G. , Nelson Karin B. 
Published: January 2005

AbstractAge at onset of seizures in young children and its relationship to factors such as prior neurological status and neurological outcome were examined. Of 52,360 children, 39,270 of whom were followed for the full 7 years, a total of 2,635 experienced one or more seizures between birth and 7 years of age. The incidence of nonfebrile convulsions was highest in the first year of life, especially in the first month. Children with neonatal seizures who later developed nonfebrile seizures did so early, two‐thirds by 6 months and three‐quarters by 1 year of age. Children with neurological or developmental abnormality assessed in the first year of life did not have their first seizure earlier than children without abnormality. Neurological abnormality in the first year of life before any seizure, and the presence of minor motor seizures, were associated with an increased rate of mental retardation and cerebral palsy at age 7, but early age at onset appeared to have little prognostic value regarding intellectual function, cerebral palsy, and epilepsy.
Deafness in Cockayne's syndrome: Morphological, morphometric, and quantitative study of the auditory pathway
Authors: Gandolfi A. , Horoupian D. , Rapin I. , Deteresa R. , Hyams V. 
Published: January 2005

AbstractThe auditory pathway of a 17‐year‐old deaf patient with Cockayne's syndrome was examined histologically. The cochlea showed marked atrophy of the spiral ganglion and attenuation of the cochlear division of the eighth cranial nerve. By means of the Computer Image Analyzer, the total number of neurons in the ventral cochlear nucleus was found to be reduced from 30,440 to 18,821. The mean diameter of the neurons in the ventral cochlear nucleus, medial dorsal olivary nucleus, and inferior colliculus was smaller than in a control patient, whereas in the medial geniculate nucleus and anterior transverse gyrus of Heschl, the neuronal size approximated the norm. The changes in the first three auditory relay nuclei were considered to represent transsynaptic atrophy caused by degeneration of the spiral ganglion and, possibly, the cochlear neuroepithelium. This histological report verifies that deafness in Cockayne's syndrome is largely sensorineural and that degeneration of spiral ganglion in humans can lead to a chain of trans‐synaptic degeneration in the ventral cochlear nucleus, medial dorsal olivary nucleus, and inferior colliculus.
Bilateral carotid artery occlusion resulting from giant cell arteritis
Authors: Howard George F. , Ho Sam U. , Kim Kwang S. , Wallach Jami 
Published: January 2005

AbstractA 65‐year‐old woman with subacute global deterioration of neurological function had bilateral occlusion of the internal carotid arteries demonstrated by angiography. Postmortem examination showed isolated giant cell arteritis involving the carotid siphon bilaterally.
Pure hemidystonia with basal ganglion abnormalities on positron emission tomography
Authors: Perlmutter Joel S. , Raichle Marcus E. 
Published: January 2005

AbstractWe present a patient with hemidystonia and an abnormality of the contralateral basal ganglion seen only with positron emission tomography. A 50‐year‐old sinistral man suffered minor trauma to the right side of his head and neck. Within 20 minutes he developed paroxysmal intermittent dystonic posturing of his right face, forearm, hand, and foot, with weaker contractions of the left foot, lasting several seconds and recurring every few minutes. Neurological findings between spells were normal. The following were also normal: electrolyte, calcium, magnesium, and arterial blood gas levels, and findings of drug screen, cerebrospinal fluid examination, electroencephalography with nasopharyngeal leads, computed tomographic scanning (initially and four weeks later), and cerebral angiography. Positron emission tomographic scanning revealed abnormalities in the left basal ganglion region, including decreased oxygen metabolism, decreased oxygen extraction, increased blood volume, and increased blood flow.
A serial study of peripheral blood T lymphocyte subsets in relapsing–remitting multiple sclerosis
Authors: Kastrukoff Lorne F. , Paty Donald W. 
Published: January 2005

AbstractTwelve patients with relapsing‐remitting multiple sclerosis and 3 control subjects were evaluated weekly for six months. Peripheral blood lymphocyte subsets were enumerated using monoclonal antibodies and analysis with a duallaser fluorescence‐activated cell sorter. Results were correlated with clinically assessed disease activity. Fluctuations occurred in the number of T lymphocytes (Leu 1+) and cells with the helper/inducer phenotype (Leu 3a/OKT4+) in both patients and controls. Fluctuations in the number of cells with the suppressor/cytotoxic (Ts/c) cell phenotype (OKT5, OKT8, Leu 2a+) also occurred. Variations in the relative numbers of cells labeled with all three Ts/c monoclonals did occur, but with OKT5 generally labeling fewer cells than OKT8 or anti–Leu 2a. When findings were correlated with disease activity, OKT5 was a more sensitive guage of disease activity than either OKT8 or anti–Leu 2a. When peripheral blood lymphocyte subsets were correlated with disease activity, the following patterns were observed: a reduction in the number of Ts/c cells without evidence of clinical disease (four episodes), the development of new symptoms suggestive of an acute relapse but without a reduction in the number of Ts/c cells (two episodes), and a reduction in the number of Ts/c cells associated with acute relapse (two episodes). In the two patients with acute relapse and a reduction in the number of Ts/c cells, clinical disease preceded a reduction in the number of Ts/c cells in 1 patient, whereas the two events occured simultaneously in the other patient. Taken together, these limited results indicate that in some cases a reduction in the number of Ts/c cells may be the result rather than the cause of disease activity and may represent an apiphenomenon.
Effect of high–dose intravenous steriod administration on contrast‐enhancing computed tomographic scan lesions in multiple sclerosis
Authors: Troiano Raymond , Hafstein Marino , Ruderman Marvin , Dowling Peter , Cook Stuart 
Published: January 2005

AbstractSix consecutive patients with multiple sclerosis and lesions contrast enhancing on computed tomographic scan were treated with high‐dose intravenous infusions of methylprednisolone. Double‐dose delayed computed tomographic scans were repeated at varying intervals during corticosteriod treatment. Contrast enhancement of sclerotic plaques was reduced or eliminated within as little as 8 hours after the first infusion. Rapid associated partial clinical improvement was noted in four patients with recent exacerbations of disease activity. Similar attenuation of contrastenhancing plaques and clinical improvement were less predictable following oral corticosteriod therapy in conventional dosage. These studies suggest that the dose, route of administration, and duration of corticosteriod therapy in multiple sclerosis may partially determine the effect of treatment.
Neuronal intranuclear inclusion disease in identical twins
Authors: Haltia Matti , Somer Hannu , Palo Jorma , Johnson William G. 
Published: January 2005

AbstractA pair of female identical twins exhibited slurred speech, nystagmus, and oculogyral spasms starting at age 11. The patients then had episodic rage, extrapyramidal and lower motor neuron abnormalities, and grand mal seizures, but retained largely normal intelligence, until death at age 21. Severe loss of nigral and craniospinal motor neurons was noted postmortem. Round, eosinophilic, autofluorescent inclusion bodies, 3 to 10 μm in diameter, were observed in the nuclei of mmilanost nerve cell types of the central and peripheral nervous systems and retina. Ultrastructurally the inclusions appeared as masses of filaments without a limiting membrane, the constituent filaments having a diameter of 8.5 to 9.5 nm. Histochemical results suggested the presence of proteins with a high content of tryptophan. Four similar cases have been reported previously under various designations. We propose the name neuronal intranuclear inclusion disease for the disorder.
Measles encephalomyelitis: Lack of evidence of viral invasion of the central nervous system and quantitative study of the nature of demyelination
Authors: Gendelman Howard E. , Wolinsky Jerry S. , Johnson Richard T. , Pressman Norman J. , Pezeshkpour Gholam H. , Boisset Gerardo F. 
Published: January 2005

AbstractMeasles encephalomyelitis appears to be an immune‐mediated parainfectious disorder, but it is unclear whether viral invasion of brain is an obligate step in its development. Immunocytochemical methods were used to search for virus antigen in formalin‐fixed, paraffin‐embedded central nervous system (CNS) tissues from 10 patients with measles encephalomyelitis and 12 patients who had died of measles without CNS involvement. All the CNS tissues studied were viral antigen negative. Similarly fixed CNS tissues from all of 6 patients with subacute sclerosing panencephalitis were viral antigen positive and served as controls. The pattern of perivenular demyelination was also determined in 4 cases of measles encephalomyelitis using antibodies to myelin associated glycoprotein and myelin basic protein and a Luxol fast blue stain. Areas of demyelination in serial sections were quantitated, and no morphometrical differences were found among tissues stained with the three stains.The data suggest the lack of virus replication in the CNS during encephalomyelitis or fatal measles without CNS symptoms. The pattern of loss of myelin associated glycoprotein and myelin basic protein in regions of perivenular demyelination resembles that reported in experimental allergic encephalomyelitis. This pattern of demyelination has been proposed to result from a primary attack on the myelin sheath rather than from direct involvement of the oligodendroglial cell.
Opioid‐induced epileptogenic phenomena: Anatomical, behavioral, and electroencephalographic features
Authors: Chugani Harry T. , Ackermann Robert F. , Chugani Diane C. , Engel Jerome 
Published: January 2005

AbstractRecent animal studies have indicated a possible role of opioids in epilepsy. Intraventricular opioid administration induces a prolonged nonconvulsive stuporous state characterized by epileptiform electroencephalographic patterns, and reversed by naloxone. In high doses, naloxone itself causes generalized clonic convulsions. We compared opioid‐induced and naloxone‐induced epileptogenic phenomena using quantitative 2‐deoxyglucose autoradiography in order to define the anatomical structures involved in these two different seizure types. When opioid‐induced seizures occurred, limbic structures were preferentially activated, but when naloxone‐induced clonic convulsions occurred, pyramidal and extrapyramidal motor areas and some limbic structures were activated. Based on the present experiments and currently available evidence, we speculate that opioid‐mediated epileptogenic phenomena are similar to those occurring during the postictal state of a fully kindled seizure, whereas naloxone‐induced epileptogenic phenomena are similar to the ictal state. Therefore, simple pharmacological manipulation of endogenous opioid systems may allow selective study of ictal and postictal phenomena.
Rebound nystagmus in olivocerebellar atrophy: A clinicopathological correlation
Authors: Bondar Roberta L. , Sharpe James A. , Lewis Anthony J. 
Published: January 2005

AbstractRebound nystagmus was recorded in a patient with cerebellar dysfunction. Neuropathological examination showed degeneration of the inferior olivary nuclei and the cerebellar cortex. The basis pontis and the brainstem tegmentum were spared. Rebound nystagmus is attributed to a smooth eye movement bias that is generated within intact vestibuloocular pathways to oppose gaze‐evoked centripetal drift of the eyes. The source of the rebound bias was not visual, because rebound nystagmus normally occurs in darkness, smooth pursuit was pursuit was paretic, and the olivocerebellar visual relay pathway was degenerated. An extraretinal, efference copy signal of the eye position error must detect the ocular drift and generate the rebound bias. The neuropathological findings indicate that rebound nystagmus can be a sign of involvement confined to the olivocerebellar circuit and that nonvisual signals of eye position are monitored independently of this cerebellar system.
“Locked‐in” state following Reye's syndrome
Authors: Kotagal Suresh , Rolfe Ursula , Schwarz Kathleen B. , Escober Wilfredo 
Published: January 2005

AbstractA 12‐year‐old boy with Reye's syndrome had an initial course complicated by increased intracranial pressure and systemic hypotension. He subsequently developed brainstem damage producing permanent apnea and a “locked‐in” state. Nevertheless, the normal architecture of sleep was retained, as was a high degree of intelligence.
Suprasegmentally induced motor unit activity in paralyzed muscles of patients with established spinal cord injury
Authors: Dimitrijevic Milan R. , Dimitrijevic Meta M. , Faganel Janez , Sherwood Arthur M. 
Published: January 2005

AbstractIn an attempt to demonstrate the presence of functional descending fibers in patients with clinically apparent functional spinal cord transection, we examined electromyographically recorded paralyzed leg muscle responses to the Jendrassik and other reinforcement maneuvers. Two patterns were observed: a low‐amplitude, short onset time reinforcement maneuver response (RMR) restricted to one to three muscle groups (RMR1), and a larger‐amplitude response with a longer onset time that occurred bilaterally in essentially all of the recorded muscles (RMR2). The responses imply preserved descending facilitory influence on isolated populations of motor units (RMR1) or on segmental interneuron pools (RMR2). Such findings indicate the presence of functioning fibers traversing the injured portion of the spinal cord in patients diagnosed as having a complete lesion. In such cases, it is possible for patients to initiate subclinical motor unit activity or suprasegmentally induced gross movement through reinforcement maneuvers, but not to control the amplitude or duration of the response.
Cerebral blood flow and regional potassium distribution during focal ischemia of gerbil brain
Authors: Mies G. , Kloiber O. , Drewes L. R. , Hossmann K.‐A. 
Published: January 2005

AbstractIn 8 gerbils (Meriones unguiculatus) focal cerebral ischemia was produced by occlusion of the left common carotid artery and the opposite external carotid artery. After two hours blood flow was measured with iodoantipyrine labeled with carbon 14, and evaluated by means of quantitative autoradiography. Thereafter the same brain sections were stained for regional potassium by means of a histochemical technique. Changes in tissue potassium content were assessed by measuring the differences in optical densities in homotopic brain regions of the stained sections. The correlation between blood flow and tissue potassium level revealed that below a flow threshold of about 0.23 ml/gm/min, a definite potassium loss from the tissue was observed. The combination of autoradiographic methods with a technique for measuring the regional distribution of potassium may be useful in providing additional information on the occurrence of disturbed electrolyte homeostasis after the onset of focal ischemia.
Cranial nuclear magnetic resonance imaging in head trauma
Authors: Gandy Samuel E. , Snow Robert B. , Zimmerman Robert D. , Deck Michael D. F. 
Published: January 2005

AbstractNuclear magnetic resonance imaging revealed focal intracranial lesions consisting of extraaxial hematomas or nonhemorrhagic contusions in 3 patients with severe neurological disability following head trauma. In all 3, standard skull radiography and computed tomography had been unremarkable.
Neuroepidemiology
Authors: Kurtzke John F. 
Published: January 2005

AbstractEpidemiology is the study of the natural history of disease, which includes its frequency, severity, and course and the identification of “risk factors” that influence these aspects. Neuroepidemiology is that branch of epidemiology dealing with disorders that affect the nervous system. Frequency of disease is best measured by population‐based rates, which are ratios of the number of cases to the population at risk, expressed as cases per unit of population. Incidence and mortality rates refer, respectively, to new cases of, and deaths caused by, a disease per unit of time and population. Prevalence rate is the cross‐sectional count of cases of a disease present at a given time per unit of population. Comparison of rates often requires consideration of age‐specific or age‐adjusted rates rather than crude rates, all ages. Course of illness (survival, complications, recurrence) can be measured by life‐table methods. The annual incidence for disease and injury of the nervous system is about 2.5% (2,500 per 100,000 population) and the prevalence is about 9.5%. Excluding all traumatic, pain, headache, alcohol, psychiatric, and special‐sense disorders, neurological diseases have an annual incidence and prevalence rate, respectively, of 1.1 and 3.6% of the population.
Epidemiology of parkinsonism: Incidence, classification, and mortality
Authors: Rajput A. H. , Offord Kenneth P. , Beard C. Mary , Kurland Leonard T. 
Published: January 2005

AbstractAn epidemiological study of parkinsonism over a 13‐year period (1967 through 1979) is presented, updating previous reports on incidence and trend in the population of Rochester, Minnesota. The overall average annual incidence of parkinsonism per 100,000 population was 20.5, adjusted to the 1970 total United States population, which is virtually unchanged from previous observations. Incidences calculated for each calendar year (1967 through 1979) revealed no remarkable change following the 1976 swine flu vaccination program. There was no sex difference and the peak incidence occurred between ages 75 and 84 years. Idiopathic Parkinson's disease was the most common variant (86%), followed by drug‐induced parkinsonism (7%). There were no new cases of postencephalitic parkinsonism diagnosed during the study period. Relative frequency of other types of Parkinson's disease as identified by practicing neurologists is presented. For each case two age‐ and sex‐matched controls were selected from the Rochester population. The survival rates in the controls were comparable to the general population of the west north central region of the United States. The mortalities in the patients were significantly higher (p= 0.001) than the controls and were unchanged from previous rates reported from the same community. In the 69 (50%) patients treated with levodopa, the mortality was comparable to that in controls. The favorable outcome in these cases is attributed to bias resulting from selection of healthier patients for treatment.
Dementia and motor neuron disease: Morphometric, biochemical, and Golgi studies
Authors: Horoupian D. S. , Thal L. , Katzman R. , Terry R. D. , Davies P. , Hirano A. , DeTeresa R. , Fuld P. A. , Petito C. , Blass J. , Ellis J. M. 
Published: January 2005

AbstractIn three patients dementia without neurofibrillary tangles or Pick bodies antedated amyotrophy by several years. The motor neuron disorder in two patients was characterized by terminal bulbar symptoms; in one it was similar to classic amyotrophic lateral sclerosis. In two patients, quantitative studies of selected regions of the cortex using a computerized image analyzer disclosed, as in patients with senile dementia of Alzheimer type, a marked reduction in the number of neurons, especially those larger than 90 μ2. The findings differed from those in Alzheimer dementia, however, in that the cells in the substantia innominata were not reduced and the levels of choline acetyltransferase and somatostatin‐like immunoreactivity, determined in one patient, were within normal limits. A variable degree of sponginess of the upper layers of the cortex was attributed to attrition of pyramidal cell dendrites, observed in the one patient in whom Golgi study was successful. Because of severe degeneration of the substantia nigra in all three, the disease in these patients may represent a subset of motor neuron disease or a multisystem atrophy.
Cerebral physiological and metabolic effects of hyperventilation in the neonatal dog
Authors: Young Richard S. K. , Yagel Susan K. 
Published: January 2005

AbstractTo clarify the changes that occur during marked hypocarbia in the neonate, we measured brain blood flow and metabolite levels after 90 minutes of hyperventilation in neonatal dogs. Brain blood flow decreased significantly in diencephalon, brainstem, and spinal cord but not in cerebral cortex or white matter. There was no substantial change in the electroencephalogram. Lactate concentrations, both in telencephalon and in superior sagittal sinus blood, increased significantly, although there was no alteration in levels of ATP or phosphocreatine. Marked hypocarbia in the neonatal dog produces an elevated brain lactate level that may be related to changes in glycolytic rate rather than to tissue ischemia or hypoxia.
Physiological responses to focal cerebral ischemia in humans
Authors: Powers William J. , Grubb Robert L. , Raichle Marcus E. 
Published: January 2005

AbstractPositron emission tomography (PET) was used to investigate the regional hemodynamic and metabolic changes that accompany focal reductions in cerebral blood flow to ischemic but uninfarcted regions of the brain. Studies were performed on 7 patients chosen from a larger group of subjects with transient ischemic attacks and normal computed tomographic findings, specifically because their PET studies showed decreased blood flow to the symptomatic hemisphere rather than symmetrical flow to the two hemispheres. In regions with decreased blood flow, the cerebral metabolic rate for oxygen was also decreased, but to a lesser degree. Cerebral blood volume, vascular mean transit time, and fractional extraction of oxygen from blood were all increased. These findings indicate that cerebral oxygen metabolism was maintained in the face of decreased blood flow by local compensatory mechanisms that included dilation of intraparenchymal blood vessels and increased transfer of oxygen from blood to tissue. Such knowledge of the physiological characteristics of ischemic, uninfarcted brain is important if PET is to be used clinically to differentiate reversible cerebral ischemia from irreversible infarction.
Fine structure of cortical tubers in tuberous sclerosis: A Golgi study
Authors: Huttenlocher Peter R. , Heydemann Peter T. 
Published: January 2005

AbstractThe fine structure of cerebral cortex, including cortical tubers, was studied in 3 patients with tuberous sclerosis. Tubers were found to consist of two predominant cell populations, astroglia and small multipolar (stellate) neurons. Both cell types tended to form aggregates within tubers, with glia more prominent in the subpial region. The stellate neurons of tubers had beaded or varicose dendrites with few dendritic spines. The findings suggest that neurons within tubers are an aberrant primitive cell type that fails to express the pyramidal cell shape and dendritic morphology that is characteristic of normal human neocortex. Cortex intervening between tubers had basically normal dendritic morphology. However, quantitative study showed a decrease in the length of dendritic branches of pyramidal neurons, as also observed in several other conditions manifested by mental retardation.