bluuebunny/crossref_metadata_2025_split huggingface dataset:
Atrophy of mesial structures in patients with temporal lobe epilepsy: Cause or consequence of repeated seizures?
Authors: Cendes F. , Andermann F. , Gloor P. , Lopes‐Cendes I. , Andermann E. , Melanson D. , Jones‐Gotman M. , Robitaille Y. , Evans A. , Peters T. 
Published: January 2005

AbstractWe studied 70 epileptic patients by using magnetic resonance imaging volumetric measurements of amygdala (AM) and hippocampal formation (HF). Fifty patients presented with intractable temporal lobe epilepsy (TLE), 10 patients had focal extratemporal lobe epilepsy, and 10 had generalized epilepsy. In 91% of the 45 TLE patients without foreign tissue lesions, there was significant smallness of the AM and/or HF coinciding with the side of electroencephalographic seizure onset. No significant smallness or asymmetry was demonstrated in patients with focal extratemporal or generalized epilepsy. We performed a linear regression analysis, plotting the number of years of recurrent seizures and the estimated seizure frequency against the volumes of the AM and HF. There was no correlation between either of these two parameters and AM or HF volume (p > 0.9). There was also no correlation between the patient's age and volumetric measurements of AM or HF, nor did these measurements correlate with the occurrence of generalized seizures. On the other hand, patients with antecedent prolonged febrile convulsions in early childhood had significantly smaller AM and HF, compared with those without such a history (p < 0.001). The findings indicate that repeated seizures or longer duration of epilepsy do not cause increased atrophy of AM or HF that is measurable by volumetric magnetic resonance imaging.
Serum antibodies to Purkinje cells and dorsal root ganglia neurons in sensory neuronopathy without malignancy
Authors: Nemni R. , Camerlingo M. , Fazio R. , Casto L. , Quattrini A. , Mamoli D. , Lorenzetti I. , Canal N. , Mamoli A. 
Published: January 2005

AbstractAnti‐Purkinje cell antibodies (APCA), believed to be markers of paraneoplastic cerebellar degeneration in females, have been identified in the serum of 3 men with subacute sensory neuronopathies and no evidence of tumors 5 years after the onset of the neurological signs. By indirect immunohistochemistry on sections of rat cerebellum and dorsal root ganglia, the patients' IgG bound to the cytoplasms of both Purkinje cells and dorsal root ganglia neurons. By western blot analysis on whole human cerebellum and whole human dorsal root ganglia homogenates, the IgG from 2 patients bound to a 62‐kd protein in both homogenates and the IgG from 1 patient bound to a 110‐kd protein in the cerebellum homogenate only. Yo autoantibody test was negative in all patients. Our study provides evidence that non‐anti‐Yo APCA may be associated with subacute sensory neuronoopathies and are not necessarily markers of an underlying tumor. The previously described anti‐Yo APCA has only occurred in females with cancer.
The gp 120 glycoprotein of human immunodeficiency virus type 1 binds to sensory ganglion neurons
Authors: Apostolski Slobodan , McAlarney Terence , Quattrini Angelo , Levison Steven W. , Rosoklija Gorazd , Lugaressi Alessandra , Corbo Massimo , Sadiq Saud A. , Lederman Seth , Hays Arthur P. , Latov Norman 
Published: January 2005

AbstractUsing immunofluorescence microscopy we found that gp 120 binds to the surface of rat dorsal root ganglia neurons and human neuroblastoma cells but not to rat fibroblasts or glial cells. The binding of gp 120 to neurons was eliminated by pretreatment with trypsin, which removes cell‐surface proteins, but not with chloroform: methanol, which removes glycolipids. As control, neuronal staining by antisulfatide antibodies was eliminated by pretreatment with chloroform: methanol but not with trypsin. The gp 120 binding to neurons was also inhibited by the mouse monoclonal antibody 01, which binds to galactocerebroside and cross‐reactive glycoproteins. These studies suggest that the receptor for gp 120 on the surface of the dorsal root ganglia neurons is a glycoprotein. This interaction may mediate the effects of human immunodeficiency virus type 1 in sensory neuropathy.
Demonstration of human T‐cell lymphotrophic virus type I (HTLV‐I) from an HTLV‐I seronegative South Indian patient with chronic, progessive spastic paraparesis
Authors: Nishimura Masataka , Mingioli Elizabeth , McFarlin Dale E. , Jacobson Steven 
Published: January 2005

AbstractHere we describe a human T‐cell lymphotopic virus type I (HTLV‐I) seronegative patient from South India with a chronic, progressive spastic paraparesis from which HTLV‐I has been isolated from peripheral blood lymphocytes. HTLV‐I pol and tax viral sequences were detected in DNA from fresh peripheal blood lymphocytes (PBL) by polymerase chain reaction (PCR) and liquid hybridization techniques. Southern blot analysis of the PCR products demonstrated a low copy number of HTLV‐I at the level of one viral copy per 10,000 fresh PBL. A long‐term CD4+ T‐cell line was established from PBL of this patient using recombinant interleukin‐2, OKT3, and feeder cells. DNA from these cultured lines was amplified and portions of the HTLV‐I long terminal repeat (U3), pol, env, and tax regions were sequenced (a total of 1,115 bp). The sequence data showed that the HTLV‐I associated with this patient was 98.8% homologous to prototype HTLV‐I. Southern blot analysis also confirmed the presence of full‐length HTLV‐I. These results indicate that HTLV‐I can be demonstrated in an HTLV‐I seronegative patient from south India with a chronic progressive neurological disorder.
Carotid endarterectomy: Trials and tribulations
Authors: Easton J. D. , Wilterdink J. L. 
Published: January 2005

AbstractSince its introduction 40 years ago, the value of carotid endarterectomy has been controversial. In the early 1980s, several clinical trials were initiated to determine the efficacy of this operation in patients with carotid stenoses who were either symptomatic or asymptomatic for retinal or hemispheric ischemia. In 1991, interim results were published for the North American Symptomatic Carotid Endarterectomy Trial (NASCET) and the European Carotid Surgery Trial (ECST), both reporting efficacy for surgery in patients with symptomatic carotid artery stenosis of greater than 70%. Subgroup analyses revealed variable risk groups. The Veterans Administration (VA) Symptomatic Trial (Cooperative Studies Program 309 of the Department of Veterans Affairs) terminated early because of these results and its findings were consistent with the results of the larger trials. NASCET and ECST continue for symptomatic patients with carotid stenoses between 30% and 69%. The results of three trials in asymptomatic patients, the Mayo asymptomatic trial, the Carotid Artery Stenosis with Asymptomatic Narrowing: Operation Versus Aspirin trial, and the VA Asymptomatic Trial (Cooperative Studies Protocol 167 of the Department of Veterans Affairs), have been reported. None showed a statistically significant benefit for surgery in the prevention of stroke or death. However, none was sufficiently large to exclude such a benefit. The large Asymptomatic Carotid Atherosclerosis Study is in progress. Differences in the results and design of these trials are discussed as are restrictions in the applicability of their results.
Indices of oxidative stress and mitochondrial function in individuals with incidental Lewy body disease
Authors: Dexter D. T. , Sian J. , Rose S. , Hindmarsh J. G. , Mann V. M. , Cooper J. M. , Wells F. R. , Daniel S. E. , Lees A. J. , Schapira A. H. V. , Jenner P. , Marsden C. D. 
Published: January 2005

AbstractBrain tissue from normal individuals with incidental Lewy bodies and cell loss in pigmented substantia nigra neurons (asymptomatic Parkinson's disease) and age‐matched control subjects without nigral Lewy bodies was examined biochemically. There was no difference in dopamine levels or dopamine turnover in the caudate and putamen of individuals with incidental Lewy body disease compared to control subjects. There were no differences in levels of iron, copper, manganese, or zinc in the substantia nigra or other brain regions from the individuals with incidental Lewy body disease compared to those from control subjects. Similarly, ferritin levels in the substantia nigra and other brain areas were unaltered. There was no difference in the activity of succinate cytochrome c reductase (complexes II and III) or cytochrome oxidase (complex IV) between incidental Lewy body subjects and control subjects. Rotenone‐sensitive NADH coenzyme Q1 reductase activity (complex I) was reduced to levels intermediate between those in control subjects and those in patients with overt Parkinson's disease, but this change did not reach statistical significance. The levels of reduced glutathione in substantia nigra were reduced by 35% in patients with incidental Lewy body disease compared to control subjects. Reduced glutathione levels in other brain regions were unaffected and there were no changes in oxidized glutathione levels in any brain region. Altered iron metabolism is not detectable in the early stages of nigral dopamine cell degeneration. There may be some impairment of mitochondrial complex I activity in the substantia nigra in Parkinson's disease. The marked reduction in nigral reduced glutathione levels suggests this to be an important early change in the process of oxidative stress underlying Parkinson's disease.
Oligodendrocytes in the early course of multiple sclerosis
Authors: Brück Wolfgang , Schmied Mascha , Suchanek Gerda , Brück Yvonne , Breitschopf Helene , Poser Sigrid , Piddlesden Sara , Lassmann Hans 
Published: January 2005

AbstractThe neuropathology of demyelinating lesions in multiple sclerosis was studied in specimens obtained by diagnostic needle biopsy during early stages of the disease. The lesions were characterized by a chronic inflammatory reaction dominated by lymphocytes and macrophages, plaque‐like demyelination, and astroglial sclerosis. Oligodendrocytes within the lesions were studied by immunocytochemistry using antibodies against various myelin and oligodendroglia components. The expression of messenger RNA for proteolipid protein was determined by in situ hybridization. Our studies revealed that myelin‐oligodendrocyte glycoprotein is a sensitive and reliable marker for identification of oligodendrocytes in demyelinated plaques. The results suggest that in the early course of the disease in some patients, oligodendrocytes may largely be preserved, whereas in others oligodendroglial loss is pronounced. Loss of oligodendrocytes was only marginally related to the stage of demyelinating activity within the lesions. These findings indicate that the pathogenesis of demyelination may vary within different multiple sclerosis patients.
Alzheimer's disease and Lewy body disease: A comparative clinicopathological study
Authors: Lippa C. F. , Smith T. W. , Swearer J. M. 
Published: January 2005

AbstractThe exact nature of the relationship between Lewy body disease and Alzheimer's disease (AD) is unknown. To investigate this, we compared cases of pure Lewy body disease, mixed Lewy body disease with AD, and pure AD to see what pathological features were shared and how they differed. We counted neurons, Lewy bodies, diffuse and neuritic senile plaques, neurofibrillary tangles, and neuropil threads in the frontal and medial temporal cortex and hippocampus from 5 autopsied cases of Lewy body disease (without AD histopathology), 7 with combined Lewy body disease and AD, 6 with AD, and 5 age‐matched normal control subjects. Average neuronal counts in the cases with Lewy body disease were indistinguishable from those of control subjects, but higher than those for AD and combined Lewy body disease and AD. Diffuse plaque densities were similar in all disease forms. Neuritic senile plaques, neurofibrillary tangles, and neuropil threads were numerous in AD and combined Lewy body disease and AD, but sparse or absent in Lewy body disease and controls. Pure Lewy body disease and AD appear to be distinct clinicopathological entities except for the common feature of diffuse plaques in both disorders.
Influence of visual information on cerebellar saccadic dysmetria
Authors: Gaymard B. , Rivaud S. , Amarenco P. , Pierrot‐Deseilligny C. 
Published: January 2005

AbstractWe compared the accuracy of saccades made to seen or remembered visual targets in 3 patients with saccadic dysmetria due to cerebellar lesions. Saccadic dysmetria was worse for saccades to remembered targets and for saccades to flashed targets visible for only 150 msec (i.e., invisible at the time of saccade). Furthermore, no corrective saccades were made if the target was not visible. These results have implications for testing saccadic dysmetria and suggest new hypotheses about the control of saccadic amplitude.
Inborn errors of urea synthesis
Authors: Batshaw Mark L. 
Published: January 2005

AbstractInborn errors of urea synthesis can present in the newborn period as a catastrophic illness or later in childhood or adulthood with an indolent course punctuated by hyperammonemic episodes. Because symptoms mimic other neuropsychiatric disorders, it is common for there to be a delay in diagnosis, often with dire consequences. Diagnosis relies on the combination of clinical suspicion and the measurement of ammonium, lactate, and amino acids in plasma and organic acids and orotic acid in urine. Treatment involves nitrogen restriction combined with the stimulation of alternate pathways of waste nitrogen excretion. More recently liver transplantation has been performed as enzyme replacement therapy. The outcome is poor in children who survive prolonged neonatal hyperammonemic coma, with most manifesting developmental disabilities. The etiology of neuronal injury in this disorder is unclear but may involve some combination of ammonia/amino acid accumulation, neurotransmitter alterations, and excitotoxic injury. Gene therapy holds the promise of improved treatment in the future.
An immunohistochemical study on α‐ketoglutarate dehydrogenase complex in Parkinson's disease
Authors: Mizuno Yoshikuni , Matuda Sadayuki , Yoshino Hiroyo , Mori Hideo , Hattori Nobutaka , Ikebe Shin‐Ichirou 
Published: January 2005

AbstractWe report an immunohistochemical study of the mitochondrial α‐ketoglutarate dehydrogenase complex (KGDHC) in the substantia nigra in Parkinson's disease. The KGDHC, the three enzyme complex catalyzing the oxidation of α‐ketoglutarate to succinate through succinic semialdehyde, is the rate‐regulating enzyme of the TCA cycle. The mitochondrial toxin, MPP+, inhibits not only complex I but also the KGDHC. Therefore, we investigated this enzyme complex in Parkinson's disease. In the control patients (n = 6), the immunostaining of the melanized nigral neurons was generally uniform; most of the melanized neurons showed good immunostaining with some neurons showing somewhat reduced staining. In Parkinson's disease (n = 9), many melanized neurons showed reduced immunostaining for the KGDHC, and those neurons were more frequently seen in the lateral one‐third of substantia nigra. The decrease in the immunostaining for the KGDHC correlated roughly with the severity of degeneration. The KGDHC is more vulnerable to degeneration than complex II, III, and IV as noted in our previous immunohistochemical study. Even if secondary, the loss may play a role in the progression of the disease.
Mechanism of action of the anticonvulsant felbamate: Opposing effects on N‐methyl‐D‐aspartate and γ‐aminobutyric acidA receptors
Authors: Rho Jong M. , Donevan Sean D. , Rogawski Michael A. 
Published: January 2005

AbstractFelbamate is a promising new antiepileptic drug whose mechanism of action is unknown. In whole‐cell voltage clamp recordings from cultured rat hippocampal neurons, clinically relevant concentrations of felbamate (0.1–3 mM) inhibited N‐methyl‐D‐aspartate (NMDA) responses and potentiated γ‐aminobutyric acid (GABA) responses. Single‐channel recordings indicated that the effect on NMDA responses occurred via a channel blocking mechanism. Felbamate is the first anticonvulsant drug with dual actions on excitatory (NMDA) and inhibitory (GABA) brain mechanisms. This unique combination of effects could account for felbamate's broad spectrum of anticonvulsant activity in animal seizure models and its distinctive clinical efficacy and safety profile.
Childhood ataxia with diffuse central nervous system hypomyelination
Authors: Schiffmann Raphael , Moller Johanna R. , Trapp Bruce D. , Shih Henry H‐L. , Farrer Robert G. , Katz David A. , Alger Jeffry R. , Parker Colette C. , Hauer Peter E. , Kaneski Christine R. , Heiss John D. , Kaye Edward M. , Quarles Richard H. , Brady Roscoe O. , Barton Norman W. 
Published: January 2005

AbstractA significant number of patients with progressive leukodystrophy do not have a definitive diagnosis. This report describes the clinical, morphological, and biochemical characteristics of 4 unrelated girls with progressive ataxic diplegia of unknown etiology. These patients had normal development until the ages of 1.5 to 5 years. A diffuse confluent abnormality of the white matter of the central nervous system was present on computed tomography and magnetic resonance scans obtained early in the course of the illness. Dementia was not present and peripheral nerves were normal. All patients were evaluated for known metabolic and degenerative diseases and no abnormalities were observed. Light and electron microscopy of open‐brain biopsy specimens from 2 girls showed selective white matter abnormalities including hypomyelination, demyelination, and gliosis. Myelin‐specific proteins in the subcortical white matter were examined immunocytochemically and by Western blot analysis. The were of normal molecular size but were markedly reduced in quantity in both patients compared to control subjects. Lipid analysis revealed decreased levels of characteristic myelin lipids. When examined by magnetic resonance spectroscopic imaging, all patients showed a marked decrease of N‐acetylaspartic acid, choline, and creatine in white matter only. The magnetic resonance spectroscopic imaging profile is a unique diagnostic feature of this clinicopathological syndrome.
A splice junction mutation in a new myopathic variant of phosphoglycerate kinase deficiency (PGK North Carolina)
Authors: Tsujino Seiichi , Tonin Paola , Shanske Sara , Nohria Virinder , Boustany Rose‐Mary , Lewis Darrell , Chen Yuan‐Tuong , DiMauro Salvatore 
Published: January 2005

AbstractWe report on a 12‐year‐old boy with the myopathic form of phosphoglycerate kinase (PGK) deficiency, and unique kinetic and physical characteristics of the mutant enzyme (PGK North Carolina). A G‐to‐T substitution at the 5′ end of intron 4 was identified in the PGK gene of this patient. The mutation destroys the consensus sequence GT at the 5′ splice junction of the intron. Activation of a cryptic splice site within intron 4 causes the insertion into the transcript of a 30‐bp fragment at the 5′ end of intron 4. This insertion results in ten additional amino acids within the “nose” of the PGK molecule, but does not generate a frameshift or a premature stop codon.
Posterior temporal epilepsy: Electroclinical features
Authors: Duchowny M. , Jayakar P. , Resnick T. , Levin B. , Alvarez L. 
Published: January 2005

AbstractIn the course of evaluating children with posterior temporal lobe epilepsy with subdural electrodes, we observed that their seizures commonly arose from basal rather than convexity foci and that they followed a stereotyped clinical sequence. Seizures characteristically began with behavioral arrest that coincided with basal temporal seizure discharges and progressed to motor signs as the seizure activity spread to the ipsilateral cortical convexity. Behavioral automatisms were observed in approximately half the patients, but were never the first or most prominent ictal manifestation. Focal lesions were identified preoperatively in 7 patients. We performed tailored temporal lobe resections in 14 patients, 10 (71%) of whom were seizure free (N = 9) or had occasional auras (N = 1) at a mean follow‐up of 2 years. These findings suggest that in childhood, posterior temporal seizures frequently arise from basal cortex and have a consistent and recognizable ictal and electrographic semiology. In medically refractory patients, tailored temporal resection is an effective therapy.
Pretreatment with intraventricular basic fibroblast growth factor decreases infarct size following focal cerebral ischemia in rats
Authors: Koketsu Naoki , Berlove David J. , Moskowitz Michael A. , Kowall Neil W. , Caday Cornelio G. , Finklestein Seth P. 
Published: January 2005

AbstractBasic fibroblast growth factor is a polypeptide with potent multipotential trophic effects on central nervous system cells, including neurons, glia, and endothelial cells. In particular, it promotes the survival of a wide variety of brain neuron in vitro, and protects these neurons against the effects of several neurotoxins, including excitatory amino acids, hypoglycemia, and calcium ionophore. Since lack of substrate delivery, excitatory amino acid toxicity, and calcium entry into cells appear to be important processes in neuronal death after ischemia, we tested the hypothesis that pretreatment with basic fibroblast growth factor limits infarct size in a model of focal cerebral ischemia in vivo. Mature male Long‐Evans rats received either continuous intraventricular infusion of basic fibroblast growth factor (1.2μ/day; with or without heparin, added to stabilize the growth factor) or vehicle alone for 3 days before focal ischemic infarcts were made in the right lateral cerebral cortex by permanent distal middle cerebral artery occlusion and temporary (45‐minute) bilateral carotid occlusion. Intraoperative measurements of core temperarure, arterial blood pressure and blood gases, blood glucose concentration, and hematocrit, and postoperative measurements of temperature revealed no differences among vehicle‐ versus basic fibroblast growth factor–treated animals. Twenty‐four hours later, animals were killed, brains were removed and stained to visualize cortical infarcts, and infarct volume was determined by image analysis. Overall, we found a 25% reduction in infarct volume in basic fibroblast growth factor– (N = 25) versus vehicle‐treated (N = 23) animals (p < 0.01). This reduction was not enhanced by the addition of heparin. These data show that pretreatment with intraventricular basic fibroblast growth factor limits infarct size following focal cerebral ischemia in rats.