bluuebunny/crossref_metadata_2025_split huggingface dataset:
Physiological effects of 4‐aminopyridine on demyelinated mammalian motor and sensory fibers
Authors: Bowe Constance M. , Kocsis Jeffery D. , Targ Elizabeth F. , Waxman Stephen G. 
Published: January 2005

AbstractThe selective response of demyelinated sensory fibers to 4‐aminopyridine (4‐AP) has been proposed as a mechanism underlying the reported paresthesias that complicate the use of this potassium‐channel blocking agent in clinical trials for the treatment of multiple sclerosis and neuromuscular disorders. To identify differences in the electrophysiological response of specific fiber types to the application of 4‐AP, rat ventral and dorsal spinal roots, demyelinated by intrathecal injections of lysophosphatidylcholine, were examined in vitro before and during potassium‐channel blockade. The compound action potentials of demyelinated ventral roots showed a prominent postspike negativity associated with a broadening of single action potentials following application of 4‐AP. Under similar conditions, whole root responses of demyelinated dorsal root axons also developed a late negativity, but individual fibers were observed to fire repetitively in response to a single stimulus. The data support the hypothesis that the prominent sensory dysfunctions reported in clinical trials of 4‐AP are due to the selective response characteristics of sensory fibers.
Regional cerebral blood flow in down's syndrome
Authors: Melamed Eldad , Mildworf Bracha , Sharav Tereza , Belenky Lev , Wertman Eliahu 
Published: January 2005

AbstractRegional cerebral blood flow was measured in 14 patients with Down's syndrome, in 46 patients with Alzheimer's disease and senile dementia of the Alzheimer type, and in 114 age‐matched controls, using the xenon 133 inhalation technique. Cerebral blood flow was reduced in 13 of 14 Down's patients by a mean of 16.8 ± 2.5% from expected age‐matched normal values. Degrees of regional cerebral blood flow reduction did not differ among the frontal, temporal, parietal, and occipital regions in both cerebral hemispheres. The regional cerebral blood flow Decemberreases were similar in magnitude and pattern to those in Alzheimer patients. These findings constitute an additional similarity between the two disorders.
Supratentorial malignant gliomas in childhood: A review of fifty cases
Authors: Dropcho Edward J. , Wisoff Jeffrey H. , Walker Russell W. , Allen Jeffrey C. 
Published: January 2005

AbstractFrom 1977 to 1986, 50 children aged 15 months to 18 years were treated for supratentorial malignant gliomas at the Memorial Sloan‐Kettering Cancer Center and the New York University Medical Center. Thirteen patients had glioblastoma multiforme, 29 had anaplastic astrocytomas, and 8 had malignant gliomas. In 10 patients the tumor evolved from a low‐grade lesion. Seven patients, including 2 patients with neurofibromatosis, developed multiple primary malignant neoplasms. The median time to tumor progression after surgery was 31 weeks, with local recurrence representing the mode of treatment failure in nearly all patients. Notable clinical features included symptomatic leptomeningeal metastasis (13 patients) and intratumoral hemorrhage (9 patients). The estimated median survival time for all 50 patients was 98 weeks, with a 3‐year survival rate of 32%. A trend toward longer survival was seen in patients 12 years of age or younger at diagnosis. There was no apparent correlation between survival and tumor histology or tumor location. Recommendations for management are presented.
Search for antibodies to galactocerebroside in the serum and cerebrospinal fluid in human demyelinating disorders
Authors: Rostami A. M. , Burns J. B. , Eccleston P. A. , Manning M. C. , Lisak R. P. , Silberberg D. H. 
Published: January 2005

AbstractTo determine if galactocerebroside (GalC) is a target antigen in the human demyelinating disorders multiple sclerosis, Guillain‐Barré syndrome, and chronic demyelinating inflammatory polyneuropathy, we examined the serum and cerebrospinal fluid from patients with these disorders and from control subjects using four assay systems. In none of these assays could we detect significant differences in anti‐GalC antibody titer between patients with demyelinating diseases and normal subjects or patients with demyelinating diseases and normal subjects or patients with other neurological disorders. Our data suggest that there is no humoral immune response to GalC in human demyelinating disorders.
Elevated serotonin and reduced dopamine in subregionally divided Huntington's disease striatum
Authors: Kish Stephen J. , Shannak Kathleen , Hornykiewicz Oleh 
Published: January 2005

AbstractWe measured the rostrocaudal distribution of serotonin, dopamine, and their metabolites in Huntington's disease striatum (caudate and putamen). Mean levels of serotonin or 5‐hydroxyindoleacetic acid were elevated in most striatal subdivisions, whereas concentrations of dopamine or its metabolite homovanillic acid were slightly to markedly reduced. Dopamine and serotonin were at control levels in the nucleus accumbens and substantia nigra. Whereas the above‐normal serotonin can most likely be accounted for by striatal atrophy, the reduced dopamine suggests either a marked down regulation of nigrostriatal dopamine neurons or an actual reduction in the arborization of the striatal dopamine neurons. As experimental animal data suggest, the relative excess of striatal serotonin or one of its metabolites may facilitate the neurodegenerative process in Huntington's disease striatum.
Chronic muscle weakness caused by Borrelia burgdorferi meningoradiculitis
Authors: Wokke J. H. J. , de Koning J. , Stanek G. , Jennekens F. G. I. 
Published: January 2005

AbstractA 19‐year‐old man developed chronic weakness of the lower limbs as the predominant manifestation of Borrelia burgdorferi infection of the nervous system. Spirochetes were demonstrated in the cerebrospinal fluid. The condition resolved following intravenous penicillin treatment.
Positron emission tomography studies of cerebral glucose metabolism in chronic partial epilepsy
Authors: Abou‐Khalil Bassel W. , Siegel George J. , Chris Sackellares J. , Gilman Sid , Hichwa Richard , Marshall Robert 
Published: January 2005

AbstractPositron emission tomography (PET) performed with {18F}‐2‐fluoro‐2‐deoxy‐D‐glucose ({18F}FDG) was used to measure local cerebral metabolic rate for glucose (lCMRGlc) interictally in 31 patients with chronic partial epilepsy and 16 age‐matched normal subjects. Hypometabolic zones were visualized in 25 patients (81%). Cortical lCMRGlc in hypometabolic zones was within 2 standard deviations of the mean for normal temporal cortex in all but 8 patients. However, in 24 patients asymmetry between the hypometabolic cortex and homologous contralateral cortex was more than 2 standard deviations above the mean cortical asymmetry for normals. There was good correlation between hypometabolic zones and electroencephalogram (EEG) foci in patients with unilateral well‐defined EEG foci. Diffuse or shifting EEG abnormalities were often associated with normal PET scans. Of 28 patients who underwent magnetic resonance imaging, 10 showed focal temporal lobe abnormalities corresponding to focal hypometabolism. While the {18F}FDG PET scan cannot currently localize an epileptogenic zone independently, the absence of focal hypometabolism or its presence contralateral to a presumed EEG focus suggests the need for additional electrophysiological data.
Alzheimer‐related neuronal protein A68: Specificity and distribution
Authors: Wolozin Benjamin , Davies Peter 
Published: January 2005

AbstractWe previously reported that the monoclonal antibody Alz‐50 recognizes a protein (A68) with an apparent molecular weight of 68,000 daltons in the brains of patients who died with Alzheimer's disease, but not in brain tissue from individuals free of neurological disease. We now report that substantial quantities of this protein are found in brain tissue only from patients with Alzheimer's disease, Although trace amounts of A68 may be detected in cases of Guam—Parkinson dementia complex and Pick's disease, accumulation of A68 in neuronal perikarya and particularly in neuronal processes appears to be a unique feature of Alzheimer's disease. A68 can readily be detected in cerebrospinal fluid after concentration and partial purification. These data, combined with the quantitation of immunoreactivity in other neurological diseases, suggest the usefulness of detection of A68 in cerebrospinal fluid for diagnosis of Alzheimer's disease.
Naltrexone decreases self‐injurious behavior
Authors: Herman Barbara H. , Kathryn Hammock M. , Bs Ann Arthur‐Smith, , Egan James , Chatoor Irene , Werner Alisa , Zelnik Nathaniel 
Published: January 2005

AbstractThe effect of naltrexone (0.5, 1.0, 1.5, and 2.0 mg/kg) on the frequency of self‐injurious behavior (SIB) was investigated in three male adolescents. The frequency of total SIB was reduced significantly in all three subjects; dose‐dependent Decemberreases (at 0.5, 1.0, and 1.5 mg/kg) in SIB frequency were observed in the two mentally retarded subjects. These data suggest a role for opioid peptides in SIB.
Alpha‐glucosidase deficiency and basilar artery aneurysm: Report of a sibship
Authors: Makos Mignon M. , McComb Rodney D. , Hart Michael N. , Bennett Donald R. 
Published: January 2005

AbstractGlycogen deposition in vascular smooth muscle has been demonstrated previously in alpha‐glucosidase deficiency but has not been clinically significant. Three sons of healthy, nonconsanguineous parents developed progressive proximal muscular weakness secondary to alpha‐glucosidase deficiency. Each patient developed a fusiform basilar artery aneurysm, which was complicated by fatal rupture in two patients and a cerebellar infarction in the third. Postmortem examination demonstrated severe vacuolation of skeletal muscle, liver, and vascular smooth muscle with accumulation of periodic acid‐Schiff–positive, diastase‐sensitive material. In the surviving brother, similar glycogen deposition was demonstrated in the smooth muscle of the superficial temporal artery. Basilar artery aneurysm formation in this sibship may be a consequence of alpha‐glucosidase deficiency.
Total lymphoid irradiation in multiple sclerosis: Blood lymphocytes and clinical course
Authors: Cook Stuart D. , Devereux Corinne , Troiano Raymond , Zito George , Hafstein Marino , Lavenhar Marvin , Hernandez Enrique , Dowling Peter C. 
Published: January 2005

AbstractWe have found a significant relationship between blood lymphocyte count and prognosis in 45 patients receiving either total lymphoid irradiation or sham irradiation for chronic progressive multiple sclerosis. Patients with sustained lymphocyte counts less than 900 mm−3 for prolonged periods after treatment showed less rapid progression over the ensuing 3 years than did patients with multiple sclerosis who had lymphocyte counts above this level (p < 0.01). Our results suggest that a simple laboratory test, the absolute blood lymphocyte count, may serve as a valuable barometer for monitoring the amount of immunosuppressive therapy needed to prevent progression in patients with multiple sclerosis, and possibly other autoimmune diseases.
Unilateral asterixis due to ipsilateral lesions in the pons and medulla
Authors: Peterson Donald I. , Peterson Gordon W. 
Published: January 2005

AbstractUnilateral asterixis was ipsilateral to the brainstem lesions in 2 patients. The finding of unilateral asterixis has been consistently associated with structural neurological disease, but contrary to previous reports it is not a reliable localizing or lateralizing finding.
Visual evoked potentials in patients with neuropathy and macroglobulinemia
Authors: Barbieri S. , Nobile‐Orazio E. , Baldini L. , Fayoumi Z. , Manfredini E. , Scarlato G. 
Published: January 2005

AbstractVisual evoked potentials were studied in 11 patients with neuropathy and macroglobulinemia. The P100 latency was increased bilaterally in 5 of the 6 patients whose IgM M‐proteins reacted with myelin‐associated glycoprotein (MAG) and in 1 of the other patients. In patients whose M‐protein bound to MAG, abnormal visual evoked potentials correlated with the presence of the M‐protein in the cerebrospinal fluid. Subclinical involvement of the central nervous system is frequent in patients with neuropathy and anti‐MAG M‐proteins and may be due to the binding of M‐proteins to central nervous system myelin.
Borderzone ischemia
Authors: Leblanc Richard , Lucas Yamamoto Y. , Tyler Jane L. , Diksic Mirko , Hakim Antoine 
Published: January 2005

AbstractPositron emission tomography was used to measure cerebral perfusion and metabolism in 7 patients with severe carotid stenosis. None of the patients had sustained a major stroke, and all had a normal neurological examination except 1 patient with a mild neurological deficit from a capsular lacuna. Cerebral blood flow (CBF), cerebral blood volume, the cerebral blood flow/cerebral blood volume ratio, oxygen metabolism, and the fractional extraction of oxygen by the brain were measured in the cerebral cortex of both hemispheres in the anterior and middle cerebral artery distributions, in the borderzone regions between the anterior and middle cerebral arteries (anterior borderzone), and between the middle cerebral and posterior cerebral arteries (posterior borderzone). Results obtained in patients were compared, using Student's t test, to those obtained in 6 neurologically normal, elderly volunteers. Cerebral blood flow and the cerebral blood flow/cerebral blood volume ratio were both significantly decreased (p < 0.025 and p < 0.005, respectively) in the anterior borderzone ipsilateral to the carotid stenosis. In the same region there was a tendency toward a rising fractional extraction of oxygen with the oxygen metabolism maintained in the low‐normal range. Cerebral blood flow was also significantly decreased (p < 0.05) in the contralateral anterior borderzone. Hemodynamic and metabolic variables in other vascular territories were not significantly different from values obtained in the control group. These data indicate that the anterior borderzone may be selectively vulnerable to severe carotid stenosis, which produces, in this region, diminished perfusion, lowered hemodynamic reserve, and a tendency towards a rising fractional extraction of oxygen.
Neuronal RNA in relation to alz‐50 immunoreactivity in alzheimer's disease
Authors: Doebler J. A. , Markesbery W. R. , Anthony A. , Davies P. , Scheff S. W. , Rhoads R. E. 
Published: January 2005

AbstractBoth defective nucleic acid metabolism/protein synthesis and the expression of an abnormal antigen recognized by the Alz‐50 antibody may be involved in the neuronal degeneration of Alzheimer's disease (AD). A multiparametric analysis involving Alz‐50 immunocytochemistry and azure B–RNA microdensitometry was developed to compare nucleic acid alterations in Alz‐50‐positive neurons to those in Alz‐50‐negative neurons of the brain of AD patients. Alz‐50‐immunoreactive neurons of the hippocampal endplate (Rose's H3‐H5 fields) and the subiculum exhibited significantly lower (by approximately 30%) total RNA contents than negative neurons of the corresponding region. The mean RNA content of Alz‐50‐positive neurons of the AD brain was also reduced in comparison to that of Alz‐50‐negative neurons of agematched, nondemented controls, whereas there were no significant differences between negative neurons of AD patients and controls. The hippocampus of nondemented controls was also found to contain Alz‐50‐immunoreactive neurons, although 20‐to 30‐fold fewer than the hippocampus of AD patients. In the controls, there was also a tendency toward reduced RNA levels in Alz‐50‐positive versus ‐negative neurons. These data suggest a relationship between Alz‐50 immunoreactivity and defective nucleic acid metabolism in the AD brain.
Cyclosporine versus azathioprine in the long‐term treatment of multiple sclerosis—results of the german multicenter study
Authors: Kappos L. , Patzold U. , Dommasch D. , Poser S. , Haas J. , Krauseneck P. , Malin J.‐P. , Fierz W. , Graffenried B. U. , Gugerli U. S. 
Published: January 2005

AbstractIn a double‐blind controlled trial of 194 patients with clinically definite active multiple sclerosis, 98 were randomized to treatment with cyclosporine (CyA, 5 mg/kg/day), and 96 to treatment with azathioprine (Aza, 2.5 mg/kg/day). Eighty‐five patients in the CyA group and 82 in the Aza group completed a treatment period of 24 to 32 months in accordance with the study protocol. No significant differences could be detected between the two treatment groups at the end of the trial. Assessment was done by serial quantitative neurological examinations and Kurtzke's Expanded Disability Status Scale. Frequency of relapse and patient self‐evaluation also failed to show significant differences. Overall deterioration observed in both groups during the trial was only minor. The incidence of side effects in the CyA group was more than two times that in the Aza group. We conclude that CyA as a single agent cannot be the drug of final choice in long‐term immunosuppressive treatment of relapsing‐remitting and relapsing‐progressive multiple sclerosis.
Expression of immunoreactive major histocompatibility complex products in human skeletal muscles
Authors: Karpati George , Pouliot Yannick , Carpenter Stirling 
Published: January 2005

AbstractImmunoreactive class 1 and class 2 major histocompatibility complex gene products (MHCP) and β2 microglobulin (β2MG) were demonstrated by microscopic immunocytochemistry in cryostat sections of skeletal muscle biopsies of 67 patients with various neuromuscular diseases. Diagnoses included normal muscle, chronic partial denervation, Duchenne dystrophy, polymyositis, dermatomyositis, inclusion body myositis, and miscellaneous neuromuscular diseases. Normal mature muscle fibers did not express MHCP, but blood vessels showed both class 1 and 2 MHCP and β2MG. Regenerating muscle fibers showed consistent sarcolemmal class 1 MHCP expression irrespective of the disease. In polymyositis, the majority of extrafusal muscle fibers of most patients showed strong sarcolemmal class 1 MHCP expression. In dermatomyositis, muscle fibers situated either in perifascicular or in randomly clustered distribution revealed strong class 1 MHCP reactivity. In inclusion body myositis, scattered small clusters of muscle fibers were positive for class 1 MHCP. In polymyositis and inclusion body myositis, particularly strong class 1 MHCP expression was invariably seen in nonnecrotic muscle fibers partially invaded by lymphocytes whose cytotoxic effects are believed to be class 1 MHCP restricted. Factors or agents that trigger class 1 MHCP expression are presumed also to sensitize lymphocytes to muscle fibers in these diseases, but their identity remains obscure at this time. In dermatomyositis, the expression of MHCP in perifascicular muscle fibers and in areas of capillary loss may represent the triggering of MHCP expression by a nonspecific cellular stress reaction, in this case probably low‐grade ischemia.
Metabolic mapping of the oculomotor system in MPTP‐Induced parkinsonian monkeys
Authors: Ho Victor W. , Porrino Linda J. , Crane Alison M. , Burns Richard S. , Kopin Irwin J. , Sokoloff Louis 
Published: January 2005

AbstractThe quantitative autoradiographic 2‐{14C}deoxyglucose method was used to map the distribution of alterations of local cerebral metabolic rate for glucose (lCMRGlc) in the oculomotor system of l‐methyl‐4‐phenyl‐1,2,3,6‐ tetrahydropyridine‐(MPTP)‐induced parkinsonian monkeys. The lCMRGlc was decreased in the frontal eye fields and in the paralamellar mediodorsal thalamus in the parkinsonian monkeys as compared to normal controls. No changes in lCMRGlc were observed in other areas of the oculomotor system. L‐Dopa therapy reversed the oculomotor symptoms in these monkeys, as well as the decrease of lCMRGlc in the frontal eye fields and the paralamellar mediodorsal thalamus. Because the frontal eye fields are known to be involved in the initiation of voluntary saccades, these findings suggest a functional anatomical basis for the oculomotor deficits in Parkinson's disease.
A new technique for interpreting the BAER in cochlear disease
Authors: Tomasulo Richard A. , Peele Pamela B. 
Published: January 2005

AbstractWe found, by studying 20 normal ears and 34 ears with cochlear disease, that the brainstem auditory evoked response (BAER) wave I latency was a good predictor of the I‐V interval (r = −0.67, p< 0.001), whereas hearing loss had little predictive value. The normal and hearingloss groups generated regression lines (wave I latency vs I‐V latency) that did not differ significantly from each other. A normal range of I‐V intervals can be established for any wave I latency, increasing the sensitivity of the BAER.
Immunotherapy of multiple sclerosis
Authors: Weiner Howard L. , Hafler David A. 
Published: January 2005

AbstractBased on the assumption that multiple sclerosis is an autoimmune disease, a number of clinical trials designed to suppress the immune system or to restore immune balance in multiple sclerosis have been attempted. Depending on the disease category, the clinical goals of immunotherapy differ. Therapeutic goals include improving recovery from acute attacks, preventing or decreasing the number of relapses, and halting the disease in its progressive stage. The ultimate goal of multiple sclerosis therapy is the early treatment of patients in an attempt to halt the onset of progression. Specific strategies of immunotherapy include generation of a suppressor influence, removal of helper/inducer cells, manipulation of activated T cells, manipulation of class II major histocompatibility complex‐bearing cells, alteration of lymphocyte traffic, extracorporeal removal of serum factors or cells, and manipulation of antigenspecific cells. Present treatment modalities are beginning to show some efficacy of nonspecific immunosuppression, but these treatments are limited by their toxicities. As the immunotherapy of multiple sclerosis moves to the next stage in the coming years, patients at an earlier stage of their disease will have to be treated, nontoxic forms of therapy developed, clinical trials lengthened, and a laboratory monitor of the disease developed. Given the positive effects of immunotherapy seen thus far in the disease, it is possible that appropriate immunotherapeutic intervention may provide effective treatment for the disease in the future.
The effects of tobacco smoking on smooth pursuit eye movements
Authors: Sibony Patrick A. , Evinger Craig , Manning Karen A. 
Published: January 2005

AbstractIt has recently been shown that tobacco smoking in normal human subjects induces a transient primary‐position upbeat nystagmus. We studied the effects of tobacco smoking on smooth pursuit eye movement and found defects in both vertical and horizontal tracking during the first 5 minutes after smoking one cigarette. The smooth pursuit defect consisted of a reduction in upward tracking velocity and the superposition of saccadic square‐wave jerks on both vertical and horizontal tracking eye movements. The degree of impairment in upward smooth pursuit correlated with the intensity of tobacco‐induced nystagmus present when recording in darkness. We suggest that these alterations are due to summation of nystagmus on normal tracking eye movements rather than primary defects in the smooth pursuit system.