bluuebunny/crossref_metadata_2025_split huggingface dataset:
Proton magnetic resonance imaging in ischemic cerebrovascular disease
Authors: Salgado Efrain D. , Weinstein Meredith , Furlan Anthony J. , Modic Michael T. , Beck Gerald J. , Estes Melinda , Awad Issam , Little John R. 
Published: January 2005

AbstractProton magnetic resonance imaging (MRI) using a 0.6‐ or 1.5‐Tesla superconductive magnet was compared with high‐resolution computed tomography (CT) in 60 patients with transient ischemic attacks (TIAs) or brain infarction. MRI showed focal parenchymal changes in 84% of patients with TIAs, whereas CT showed similar changes in 42%. The sensitivity of MRI was also greater in patients with infarcts, but the difference between CT and MRI was not as great. Infarcts were usually better delineated by MRI regardless of location. However, MRI failed to reveal cortical infarcts that were clearly seen on contrast‐enhanced CT scans and was unable to clearly distinguish subacute from chronic hemorrhagic infarcts. MRI changes were best detected with T2‐weighted images and usually appeared as multiple areas of increased signal intensity in the subcortical and periventricular white matter. MRI changes often could not be correlated with the clinical history and neurological findings; identical changes have been seen in patients with no history of cerebrovascular disease.
Dendritic atrophy in children with Down's syndrome
Authors: Becker L. E. , Armstrong D. L. , Chan F. 
Published: January 2005

AbstractDendritic branching was evaluated in the visual cortex of 8 children with Down's syndrome and 10 controls, ranging in age from 4 months to 7 years and divided into infantile, late‐infantile, and juvenile groups. Camera lucida drawings of Golgi‐impregnated neurons were used for examining the following dendritic aspects: dendritic intersections as a function of distance from the cell body, point of maximum dendritic branching, number of branch orders, total number of branch segments, and total dendritic length. The number of intersections and the total dendritic length were above normal in the infantile period (6 months old or less) and dropped steadily to significantly below normal in the juvenile group (older than 2 years). These reductions contrasted with expanding dendritic arborization in normal children. The results suggest that the dendritic tree atophies in early childhood in Down's syndrome.
Gerstmann‐Sträussler‐Scheinker disease: Autopsy study of a familial case
Authors: Vinters Harry V. , Hudson Arthur J. , Kaufmann John C. E. 
Published: January 2005

AbstractPostmortem neuropathological findings in a patient with biopsy‐proved familial Gerstmann‐Sträussler‐Scheinker disease of eight years' duration included severe spongy change in the neocortex, extensive and often large amyloid deposits throughout the cerebral hemispheres and cerebellum, and severe astrocytic gliosis throughout all areas of gray and white matter within the brain. The degree of cortical spongy change was much greater than that in relatives who died with a similar clinical history, indicating the phenotypic heterogeneity in this familial disorder.
Neuropeptides in neurological disease
Authors: Beal M. Flint , Martin Joseph B. 
Published: January 2005

AbstractNeuropeptides are widely distributed in the central nervous system, where they serve as neuroregulators. Recent interest has focused on their role in degenerative neurological diseases. We describe the normal anatomy of neuropeptides in both the cerebral cortex and basal ganglia as a framework for interpreting neuropeptide alterations in Alzheimer's disease (AD), Huntington's disease, and Parkinson's disease. Concentrations of cortical somatostatin are reduced in AD and in dementia associated with Parkinson's disease. Concentrations of neuropeptide Y and corticotropin‐releasing factor are also reduced in AD cerebral cortex. The reduced cortical concentrations of somatostatin and neuropeptide Y in AD cerebral cortex may reflect a loss of neurons or terminals in which these two peptides are colocalized. In Huntington's disease, basal ganglia neurons in which somatostatin and neuropeptide Y are co‐localized are selectively preserved. Cerebrospinal fluid concentrations of neuropeptides in AD reflect alterations in cortical concentrations. Improved understanding of neuropeptides in degenerative neurological illnesses will help define which neuronal populations are specifically vulnerable to the pathological processes, and this could lead to improved therapy.
Transfection of human skeletal muscle cells with SV40 large T antigen gene coupled to a metallothionein promoter
Authors: Hurko Orest , McKee Linda , Zuurveld Judith G. E. M. 
Published: January 2005

AbstractWe have undertaken to increase the proliferative capacity of cultured human skeletal myocytes by transfection with a plasmid construct that contains the immortalizing and transforming large T antigen gene of simian virus 40 (SV40) under the control of a zinc‐sensitive metallothionein promoter. This construct was chosen to permit rapid growth of transformants in zinc‐containing medium, which induces high levels of T antigen expression, and muscle‐specific differentiation after withdrawal of exogenous zinc, which reduces levels of T antigen. When grown in 100 μM Zn2+, transformed myocytes expressed the large T antigen, divided rapidly, and acquired an apparently unlimited proliferative capacity. Transfer of these cells to a zinc‐poor medium resulted in decreased T antigen immunofluorescence, growth rate, and saturation density as well as a return to a physiological spindle morphology. Despite transformation, these cells expressed differentiation markers characteristic of myoblasts: the B isoform of creatine kinase, and surface antigens 5.1H11, D5, and Thy 1 in the presence or absence of Zn2+. When grown to high density in a serum‐poor medium, these cells differentiated further into typical multinucleated myotubes that expressed the M isoform of creatine kinase and increased levels of surface antigen 5.1H11, creatine kinase, and nicotinic acetylcholine receptors, but no detectable Thy 1 antigen. The specific activity of these differentiation markers was higher when the cells were grown in the absence of added zinc. These results indicate that transformation of human skeletal myocytes with a regulatable SV40 large T antigen gene allows an increase of the proliferative capacity of these cells with preservation of their capacity to differentiate in a physiological manner.
Schwannoma presenting as lumbar disc disease in an adolescent boy
Authors: Lahat Eliezer , Rothman Allen S. , Aron Alan M. 
Published: January 2005

AbstractLumbar disc disease and spinal cord tumors occur relatively rarely in the pediatric age group. We report on a 16‐year‐old boy who presented with signs and symptoms of lumbar disc disease that failed to respond to conservative treatment. He was diagnosed preoperatively to have schwannoma of the S‐1 root.
Left thalamic infarction and disturbance of verbal memory: A clinicoanatomical study with a new method of computed tomographc stereotaxic lesion localization
Authors: Mori Etsuro , Yamadori Atsushi , Mitani Yohko 
Published: January 2005

AbstractA 41‐year‐old right‐handed man developed disturbances of language and memory after a discrete thalamic infarction. Detailed neuropsychological assessment revealed deficits in verbal fluency, word finding, confrontation naming, and anterograde memory for verbal material. High‐resolution computed tomography with stereotaxic lesion localization permitted the determination of the thalamic nuclei involved in the infarction. We suggest that the patient's problem in language and verbal memory reflected a defect in memory processing for verbal material during registration, retention, and retrieval, and that this defect probably resulted from a left anterior thalamic lesion affecting the ventrolateral nucleus, centromedian‐parafascicular nuclei complex, internal medullary lamina, or mamillothalamic tract.
Sickle cell anemia and central nervous system infarction: A neuropathological study
Authors: Rothman Steven M. , Fulling Keith H. , Nelson James S. 
Published: January 2005

AbstractCNS infarcts were demonstrated in 12 of 24 autopsied patients with sickle cell anemia. The infarcts occurred most extensively in the territory supplied by the distal branches of the internal carotid artery, especially the anterior–middle cerebral artery boundary zone. They were regularly associated with organizing and recanalizing thrombi involving the distal cervical and proximal intracranial divisions of the internal carotid system. On the basis of these findings, we infer that the pathogenesis of the infarction involves perfusion failure or intraarterial embolization in addition to intravascular sickling.
Structural determinants of electroencephalographic findings in acute hemispheric lesions
Authors: Schaul Neil , Green Lawrence , Peyster Robert , Gotman Jean 
Published: January 2005

AbstractWe studied electroencephalograms and computed tomographic scans of 54 patients with acute hemispheric strokes. Electrographic parameters evaluated included field, amplitude, frequency, persistence, and reactivity of focal or lateralized slow‐wave activity. Ipsilateral and contralateral background activity were also assessed. Structural and clinical features studied were lesion size, density, mass effect, location, tissue involvement, deep structure involvement, level of consciousness, and outcome. The data were analyzed using computer sorting and the χ2 test. The field, amplitude, and frequency of focal slow‐wave abnormalities generally failed to show a specific association with structural details. Continuous focal abnormalities correlated with large lesions (p < 0.05), mass effect (p < 0.05), and altered state of consciousness (p < 0.05). Reactive focal abnormalities were associated with small lesions (p < 0.05) and the absence of mass effect (p < 0.02). Ipsilateral background activity abnormalities correlated with lesion size (p < 0.001) and mass effect (p < 0.01). Attenuation of ipsilateral background activity was more important than irregularity. Abnormal background activity contralateral to the lesion side was associated with alteration of consciousness (p < 0.05).
The pathophysiology of penicillamine‐induced myasthenia gravis
Authors: Kuncl Ralph W. , Pestronk Alan , Drachman Daniel B. , Rechthand Emanuel 
Published: January 2005

AbstractThe temporal course and pathophysiology of penicillamine‐induced myasthenia gravis were studied in detail in a typical case. Our results suggest that this disorder and idiopathic autoimmune myasthenia gravis share the same essential pathophysiological features, including the presence of anti–acetylcholine receptor (AChR) antibody, serum‐induced blockade of AChRs, antibody‐mediated accelerated degradation of AChRs, and a resultant quantitative reduction in available junctional AChRs. An initial severe reduction in junctional AChRs was reversed and the patient recovered, both within 8 months of stopping penicillamine. Our data suggest that penicillamine probably produced myasthenia gravis by initiating a new autoimmune response rather than by enhancing ongoing autoimmunity.
Cerebral glucose metabolism in the Lennox‐Gastaut syndrome
Authors: Theodore William H. , Rose Douglas , Patronas Nicholas , Sato Susumu , Holmes Mark , Bairamian Dikran , Porter Roger J. , Chiro Giovanni Di , Larson Steven , Fishbein Donn 
Published: January 2005

AbstractWe used positron emission tomography with fluorine 18–labeled 2‐deoxyglucose to study cerebral glucose metabolism in 10 patients with Lennox‐Gastaut syndrome who had normal neuroradiological studies. The scans showed decreased metabolic rates relative both to those in the caudate nucleus and to normal control values in 3 patients whose seizures began before the age of 1, as well as in a patient with hyperprolinemia. No patient had a region of persistent focal hypometabolism. Metabolic rates increased in parallel with increased electroencephalographic discharges in 1 patient; 3 patients had lower metabolic rates when the electroencephalogram showed epileptiform discharges and while the patients were taking barbiturates.
Defective biosynthesis of proteolipid protein in pelizaeus‐merzbacher disease
Authors: Koeppen Arnulf H. , Ronca Nicholas A. , Greenfield Edward A. , Hans Mary B. 
Published: January 2005

AbstractThe brain of an 18‐year‐old patient with Pelizaeus‐Merzbacher disease was examined by standard neuropathological and biochemical methods and by immunocytochemical and immunochemical techniques. Analysis revealed a lack of myelin‐specific lipids, but showed a residual immunoreactivity for myelin basic protein, myelin‐associated glycoprotein, and 2,3‐cyclic nucleotide‐3‐phosphodiesterase. Examination by immunocytochemistry and enzyme‐linked immunosorbent assay showed an absence of proteolipid apoprotein (lipophilin). The peripheral nervous system was normal. Pelizaeus‐Merzbacher disease in humans shares many neuropathological and biochemical features with X‐linked mutations in animals, e.g., the jimpy mouse and myelin‐deficient rat. The specificity of this protein deficiency in Pelizaeus‐Merzbacher disease gains additional support from the recent mapping of the lipophilin gene to the human X chromosome.
Absence seizures in children: Clinical and electroencephalographic features
Authors: Holmes Gregory L. , McKeever Mark , Adamson Megan 
Published: January 2005

AbstractThe clinical and electroencephalographic (EEG) features of absence seizures in children were evaluated using EEG frequency modulation radiotelemetry and videotape monitoring. The only seizures evaluated were those with a spike‐and‐wave or multiple spike‐and‐wave duration lasting at least 3 seconds. A total of 926 absence seizures (426 typical, 500 atypical) were reviewed in 54 patients. Abnormal interictal EEGs, multiple seizure types, mental retardation, or developmental delay were more likely in patients with atypical absence seizures than in patients with typical absence seizures. Both types of absence seizures usually had a clear onset and cessation. Atypical absence seizures lasted significantly longer than did typical absence seizures. Automatisms occurred more frequently in typical absence seizures than in atypical ones, while decreases in postural tone or tonic activity occurred more frequently in atypical absence seizures. Receptive and expressive speech were retained in some patients during both types of seizures. This study demonstrates that typical and atypical absence seizures are not discrete entities but rather form a continuum. No single clinical feature can adequately distinguish the two seizure types.
Clinical features and mechanism of occipital infarction
Authors: Pessin Michael S. , Lathi Ellen S. , Cohen Maurice B. , Kwan Eddie S. , Hedges Thomas R. , Caplan Louis R. 
Published: January 2005

AbstractTo clarify the clinical features and mechanism of infarction in the posterior cerebral artery territory, we investigated 35 consecutive patients who presented with homonymous visual field defects and occipital infarction documented by computed tomography. Cerebral angiographic findings in 23 patients, and the clinical features of rare transient ischemic attacks and maximal deficit occurring at stroke onset, were consistent with embolism of the posterior cerebral artery. Visual field defects were the only neurological abnormality in 17 patients; the remainder had additional findings. Three patients had a major brainstem stroke. Stroke in the posterior cerebral artery territory was found in a heterogeneous group of patients, although embolism was the most common stroke mechanism. Several distinct patient groups were identified: cardiac source embolism (10 patients), vertebrobasilar atheroma with local embolism (6), migraine (5), systemic illness with presumed coagulopathy (3), and “unknown source embolism” after negative cardiac investigation (11 patients). During follow‐up, 26 patients had no further neurological events (the majority on anticoagulation or antiplatelet treatment), 3 suffered new strokes, and 6 died.
Amino acids, glutathione, and glutathione transferase activity in the brains of patients with Alzheimer's disease
Authors: Perry Thomas L. , Yong Voon Wee , Bergeron Catherine , Hansen Shirley , Jones Karen 
Published: January 2005

AbstractWe measured the contents of amino acids and related amino compounds in autopsied brain from 22 patients with Alzheimer's disease (AD) and in cortical biopsy specimens from 2 other patients. The diagnosis of AD was established neuropathologically in all 24 patients by the presence of both neurofibrillary tangles and neuritic plaques in neocortex. The mean contents of γ‐aminobutyric acid (GABA), and of the GABA dipeptide homocarnosine, were significantly reduced in frontal and occipital cortices and in hippocampus of the autopsied brains of AD patients compared to control patients without neurological disease. However, GABA contents were normal in frontal cortex in biopsy samples from 2 patients. Phosphoethanolamine contents were significantly reduced at autopsy in frontal and occipital cortex, and in the substantia innominata. We found no evidence of a deficiency of glutamate, aspartate, or taurine in AD brain, as has been claimed. Glutathione contents and glutathione transferase activities were normal in frontal cortex and substantia innominata. The mechanism of neuronal death in patients with AD is unlikely to involve either insufficient synthesis of glutathione or failure to conjugate free radicals with glutathione.
Central sleep apnea and partial obstruction of the upper airway
Authors: Guilleminault Christian , Quera‐Salvá Maria Antonia , Nino‐Murcia German , Partinen Markku 
Published: January 2005

AbstractSeven men with central sleep apnea underwent polygraphic monitoring during sleep for at least 3 nights, in combination with other tests. Five patients had complaints of disturbed sleep; the other 2 were selected because they had central sleep apnea caused by bilateral brainstem lesions. The first 5 had a small upper airway, documented by cephalometric roentgenograms. Nasal continuous positive airway pressure, administered to improve the suspected respiratory load during sleep, eliminated the central sleep apnea in the first 5 patients but had, as expected, no positive effect on the central apnea of the 2 patients with brainstem lesions.
Oculogyric crisis: A syndrome of thought disorder and ocular deviation
Authors: John Leigh R. , Foley Joseph M. , Remler Bernd F. , Civil Richard H. 
Published: January 2005

AbstractIn 3 patients who suffered oculogyric crises, mental changes accompanied upward deviation of the eyes. In 1 patient, whom we studied in detail, the mental disturbance consisted of a disorder of attention in which pathological fixation of a thought occurred. During the period of upward eye deviation, all functional types of conjugate eye movements were present in the upper field of gaze, suggesting an imbalance of the vertical gaze–holding mechanism. The eyes could be driven down only by a combined blink and downward saccade. Both the thought disorder and the ocular deviation responded promptly to anticholinergic agents. We propose that the disorders of thought and eye movement in oculogyric crisis are linked by a pharmacological imbalance common to both.
Alzheimer's disease: Glutamate depletion in the hippocampal perforant pathway zone
Authors: Hyman Bradley T. , Van Hoesen Gary W. , Damasio Antonio R. 
Published: January 2005

AbstractThe perforant pathway is the primary source of cortical input to the hippocampal formation. Its cells of origin, in the entorhinal cortex, are destroyed in Alzheimer's disease. Because the principal neurotransmitter of the perforant pathway's excitatory action is thought to be glutamate, we microdissected a portion of the pathway's terminal zone and assayed the excised tissue for glutamate. There was an 83% decrease in the level of free glutamate in subjects with Alzheimer's disease as compared to control subjects not affected by dementia (p < 0.005). We believe that this diminution in the glutamate content is a direct neurochemical correlate of perforant pathway destruction and that disruption of this crucial corticolimbic pathway contributes to the memory dysfunction in Alzheimer's disease.
Multifocal CNS demyelination following peripheral inoculation with herpes simplex virus type 1
Authors: Kastrukoff Lorne F. , Lau Allen S. , Kim Seung U. 
Published: January 2005

AbstractThe peripheral inoculation of herpes simplex virus type 1 (HSV 1) in experimental animals induces central nervous system (CNS) demyelinating lesions, but the potential relevance of this model to multiple sclerosis is lessened by the unifocal nature of the lesion. In this study, inbred strains of mice were selected on the basis of varying resistance to mortality following lip inoculation with virus. A spectrum of CNS pathology was observed, ranging from focal collections of inflammatory cells at the trigeminal root entry zone in resistant strains (C57BL/6J), to unifocal demyelinating lesions in moderately resistant strains (BALB/cByJ), to multifocal demyelinating lesions throughout the brain in susceptible strains (A/J). Findings from viral titration studies of the CNS support a direct cytolytic effect of virus in the development of demyelinating lesions at the trigeminal root entry zone but cannot exclude an immune‐mediated component. Furthermore, 50% tissue‐culture‐infective doses, immunofluorescence, and electron microscopic studies of primary cultures of oligodendrocytes, derived from the three strains of adult mice, identify differences in resistance to HSV 1 infection in vitro, suggesting that differences at this level may also contribute to the pathological appearance. Multifocal lesions in A/J mice were first observed when the infectious virus could no longer be isolated from the CNS and may be the result of an immune‐mediated process “triggered” by the acute CNS infection in susceptible strains of mice.
Ataxia after severe head injury: The pathological substrate
Authors: Susan Chester C. , Reznick Barry R. 
Published: January 2005

AbstractAtaxia is a common finding in patients who have recovered from severe head injury. This report delineates the clinical and pathological findings in a patient who recovered from a head injury but was left with ataxia of gait. A lesion of the superior cerebellar peduncle, demonstrated post mortem 2½ years after injury, was the only explanation for the ataxia. This lesion is part of the spectrum of changes seen in diffuse axonal injury thought to be due to shearing forces which tear white matter at the time of the initial injury.
Giant axonal neuropathy: Correlation of clinical findings with postmortem neuropathology
Authors: Thomas C. , Love S. , Powell H. C. , Schultz P. , Lampert P. W. 
Published: January 2005

AbstractWe report the clinical and postmortem neuropathological findings in a case of long‐standing giant axonal neuropathy. The patient, a caucasian male with kinky hair, was first seen at 4 years of age because of increasing unsteadiness of gait. Clinical examination showed nystagmus, cerebellar ataxia, distal sensory loss, and weakness. A sural nerve biopsy at 8 years of age revealed giant axonal neuropathy. The patient became increasingly demented and was incapacitated by weakness and ataxia; he died at 18 years of age. Histological examination of the brain and spinal cord showed numerous Rosenthal fibers, a distal axonopathy that most severely affected the corticospinal tracts, middle cerebellar peduncles, and posterior columns, and olivocerebellar degeneration.