bluuebunny/crossref_metadata_2025_split huggingface dataset:
Galactosylceramide lipidosis: Novel presentation as a slowly progressive spinocerebellar degeneration
Authors: Thomas P. K. , Halpern J.‐P. , King R. H. M. , Patrick D. 
Published: January 2005

AbstractA 34‐year‐old woman was initially seen because of a progressive neurological disorder suggestive of a spinocerebellar degeneration. This condition had a late infantile onset and was unassociated with visual impairment or dementia. Nerve conduction velocity was severely reduced. A left hemiparesis later developed. A computed tomographic scan revealed multiple periventricular hypodense lesions, suggestive of a leukodystrophy. Sural nerve biopsy demonstrated changes of a chronic demyelinating neuropathy, with inclusions typical of Krabbe's disease. This diagnosis was confirmed by the finding of reduced leukocyte galactocerebrosidase activity.
Cortical abnormalities in Alzheimer's disease
Authors: Foster Norman L. , Chase Thomas N. , Mansi Luigi , Brooks Rodney , Fedio Paul , Patronas Nicholas J. , Di Chiro Giovanni 
Published: January 2005

AbstractRegional cerebral glucose metabolism, an index of neuronal activity, was compared in 20 patients with Alzheimer's disease and 8 age‐matched normal volunteers by positron emission tomography following {18F}2‐fluoro‐2‐deoxy‐D‐glucose administration. Overall cortical glucose utilization in the Alzheimer's group was 10 to 49% below that of control individuals. The posterior parietal cortex and contiguous portions of posterior temporal and anterior occipital lobes were most severely affected; frontal cortex was relatively spared. This pattern of cortical involvement is consistent with the major clinical features of Alzheimer's disease. Comparison of patients with early and more advanced dementia suggested that a substantial decline in glucose metabolism occurs before cognitive impairment becomes evident; once the patient is symptomatic, however, small additional metabolic decrements are associated with a marked deterioration in intellectual function.
The induction and reversibility of cerebral acidosis in thiamine deficiency
Authors: Hakim A. M. 
Published: January 2005

AbstractRegional cerebral pH was determined autoradiographically using carbon 14‐labeled dimethyloxazolidinedione in normal rats, following various durations of thiamine deficiency and replenishment with thiamine when the clinical sequelae of the deficiency appeared. In our model the clinical sequelae of thiamine deficiency (opisthotonus) appeared on the average on day 18. Regional cerebral pH on day 12 was comparable to that in controls and ranged between 7.02 ± 0.03 and 7.09 ± 0.03 (mean ± SEM) in gray matter structures. On day 14 the pH in the inferior colliculus was 6.85 ± 0.08 and relative acidosis also appeared in thalamic structures. At opisthotonus the pH was 6.48 ± 0.17 in the mamillary body, 6.43 ± 0.14 in the vestibular nucleus, and 6.36 ± 0.14 in the medial dorsal nucleus of the thalamus (p < 0.01). One dose of thiamine replenishment at this stage transiently raised the pH in the inferior colliculus to 7.25 ± 0.19 and in the medial dorsal nucleus to 7.20 ± 0.13 (p < 0.01). Cerebral regions showing significant acidosis during thiamine deficiency coincided largely with those known to be histologically vulnerable and those previously reported to show a focal rise in local cerebral glucose utilization between days 11 and 14 of thiamine deficiency. This focal acidosis shown to occur in thiamine deficiency may be one mechanism contributing to the selective histological vulnerability in this model.
In vivo measurement of brain tumor pH using [11C]DMO and positron emission tomography
Authors: Rottenberg D. A. , Ginos J. Z. , Kearfott K. G. , Junck L. , Dhawan V. , Jarden J. O. 
Published: January 2005

AbstractIn vivo measurements of regional brain tissue/tumor pH (rpH) have been accomplished in 9 patients with primary or metastatic brain tumors using[11C]dimethyloxazolidinedione ([11C]DMO) and positron emission tomography. Tumor rpH values ranged from 6.88 to 7.26, whereas gray matter and white matter rpH values ranged from 6.74 to 7.09 and from 6.77 to 7.03, respectively. Our result, which are consistent with reported [14C]DMO autoradiographic measurements of brain and tumor pH, suggest that the pH microenvironment of brain tumors is not more “acidic” than that of normal gray or white matter.
Electrophysiological analysis of the mode of action of muscle relaxants in spasticity
Authors: Delwaide P. J. 
Published: January 2005

AbstractVariations in four electrophysiological tests (H/M, T/M, vibratory inhibition, and recovery curve of Hoffmann's reflex following stimulation at the ankle) were studied following a single administration of four myorelaxant drugs: diazepam (10 mg intramuscularly), baclofen (20 mg intramuscularly), tizanidine (4 mg orally), and idrocilamide (60 mg intramuscularly). Fifty‐one spastic patients, divided into four groups, were tested. All four drugs reduced the H/M and T/M ratios very slightly. Only diazepam and tizanidine reinforced vibratory inhibition. Diazepam and tizanidine did not modify the abnormal recovery curves, however, whereas baclofen and idrocilamide did. Reinforcement of vibratory inhibition suggests an increase in presynaptic inhibition mediated by gamma‐aminobutyric acid; changes in recovery curves are likely due to modifications of interneuronal reactivity. Matching myorelaxants to the predominant pathophysiological abnormality detected by electrophysiological exploration may lead to better treatment of spasticity.
Hemispheric lateralization of spatial contrast sensitivity
Authors: Kobayashi Shotai , Mukuno Kazuo , Ishikawa Satoshi , Tasaki Yoshiaki 
Published: January 2005

AbstractVisuospatial contrast sensitivity was determined by the Arden grating chart in 23 patients with cerebral infarctions involving the primary visual cortex or visual association cortex. Subjects were classified into three groups according to their lesions: I, 6 patients with unilateral medical occipital or occipitotemporal lesions; II, 6 patients with left lateral parieto‐occipital lesions; and III, 11 patients with right lateral parieto‐occipital lesions.Contrast sensitivity was markedly reduced in Group III, especially in patients having hemispatial agnosia. Group I patients with hemispatial agnosia showed almost normal contrast sensitivity. Spatial contrast sensitivity appears to be more affected when the lesions has an influence on the nondominant lateral parieto‐occipital cortex.
Tetrabenazine induces acute dystonic reactions
Authors: Burke Robert E. , Reches Avinoam , Traub Michael M. , Ilson Joyce , Swash Michael , Fahn Stanley 
Published: January 2005

AbstractFour patients suffered acute dystonic reactions casued by tetrabenzine. Because dystonic reactions have previously been reported only after dopamine receptor blockade and not with dopamine deplection, it is likely that the ability of tetrabenazine to induce acute dystonia is due to its dopamine receptor blocking properties. Because tertrabenazine can induce acute dystonia even when combined with α‐methyl‐p‐tyrosine, presynaptic dopamine stores may not be necessary for these reactions to occur.
Polyneuropathy and IgM monoclonal gammopathy: Studies on the pathogenetic role of anti‐myelin‐associated glycoprotein antibody
Authors: Mendell J. R. , Sahenk Z. , Whitaker J. N. , Trapp B. D. , Yates A. J. , Griggs R. C. , Qurarles R. H. 
Published: January 2005

AbstractAttentionl has recently been directed toward patients having a polyneuropathy IgM anti‐Myelin‐associated glycoprotein (anti‐MAG) antibody. The possibility of a pathogenetic role for the anti‐MAG antibody in the evolution of the polyneuropathy and in the development of central nervous system signs, including tremor and ataxia, remains unresolved. In 5 patients with this syndrome whose clinical courses were followed closely, in 1 of whom a complete postmortem examination of the nervous system was performed, we made the folowing obsercvations: (1) the anti‐MAG antibody did not localize to the compact layer of the myelin sheath in affected nerves, but did realize to areas of myelin splitting; (2) anti‐MAG antibody present in the sural nerve of an affected individual for 7 years was not associated with progressive pathology; (3) anti‐MAG antibody was not deposited in the central nervous system of an affected individual, although the antibody did bind to these same tissues in vitro; (4) deposition of anti‐MAG antibody observed at postmortem examination did not correlate with the degree of pathological change; and (5) study of the peripheral nervous system favored a primary axonal neuropathy with secondary demyelination.
Regional cerebral blood flow decreases during hyperglycemia
Authors: Duckrow Robert B. , Beard Daniel C. , Brennan Robert W. 
Published: January 2005

AbstractThe presence of hyperglycemia before brain ischemia increases stroke‐related morbidity and mortality in experimantal animals and humans. However, little is known of the effect of hyperglycemia on regional cerebral blood flow (rCBF). Acute hyperglycemia was induced in awake but restrained rats by intraperitoneal injection of 50% D‐glucose. Regional flow was determined using [14C]iodoantipyrine and quantitative autoradiography. Elevation of plasma glucose from 11 to 39 mM was associated with a 24% reduction in rCBF when compared with controls that received normal saline. Intraperitoneal D‐mannitol produced an elevation of plasma osmolality equivalent to that observed with glucose. However, rCBF was only reduced by 10%. Hyperglycemia appears to produce a global decrease in rCBF in awake rats that cannot be completely explained by the attendant increase in plasma osmolality. If a similar influence is present during brain ischemia, hyperglycemia could extend areas of critical flow limitation.
Eye movements in patients with absent voluntary horizontal gaze
Authors: Baloh R. W. , Furman J. , Yee R. D. 
Published: January 2005

AbstractDespite the complete absence of horizontal saccades, two patients with pontine gliomas had horizontal reflex eye movements within a range of ±20 degrees. The gain (peak eye velocity/peak stimulus velocity) and pahse of the vestibulo‐ocular reflex were normal, but the optokinetic gain was decreased. The latency, accuracy, and peak velocity of vertical saccades were normal. Apparently the voluntary gaze centers in the pontine reticular formation are not crucial for generating horizontal or vertical saccadic eye movements.
Stimulus rate determines regional brain blood flow in striate cortex
Authors: Fox Peter T. , Raichle Marcus E. 
Published: January 2005

AbstractIntravenous bolus administration of oxygen 15‐labeled water and positron emission tomography were used to measure changes in brain blood flow induced by two modes of photic stimuli over a wide range of repetition rates. These stimuli (patterned‐flash and reversingd checkerboard) were chosen in order to determine whether stimulus luminacne or stimulus frequency was reponsible for previously observed increased in blood flow in the striate cortex during photic stimulation. The response curves of blood flow change as a function of stimuls rate were nearlyl suggest that elementary stimulus variables, such as repetition rate, can have a major effect on local cerebral responses, as measured with positron emission tomography and other radiotracer methods.
Decreased morbidity from acute bacterial spinal epidural abscesses using computed tomography and nonsurgical treatment in selected patients
Authors: Leys D. , Lesoin F. , Viaud C. , Pasquier F. , Rousseaux M. , Jomin M. , Petit H. 
Published: January 2005

AbstractWe describe 5 patients with spinal epidural abscesses in whom computed tomographic scanning confirmed diagnosis with the use myelography. One patient required urgent surgery because of rapidly deteriorating neurological status, but the other 4 were treated nonsurgically. The medical treatment of these patients and 9 others described in the literature consisted of antibiotics administered parenterally for minimum of 8 weeks, followed by oral anitibiotic therapy. Early diagnosis with computed tomographic scanning and a benign neurological state at the state at the onset of treatment were associated with good results.
The cherry‐red spot in Tay‐Sachs and other storage diseases
Authors: Kivlin Jane D. , Sanborn George E. , Myers Garth G. 
Published: January 2005

AbstractThe disappearance of the cherry‐red spot in an otherwize typical patient with Tay‐Sachs disease is described and illustrated. This clinical onservation has rarely ben made, but it is consistent with the pathological findings of loss of retinal ganglion cells. Disappearance of the cherry‐red spot has also been observed in GM1 generalized gangliosidosis, the sialidoses known as the cherry‐red spot‐myoclonus syndrome and the Goldberg syndrome, and Niemann‐Pick, type C disease. Thus the absence of a cherry‐red spot in the neurologically impaired child or adult does not exclude these diagnoses.
Increased prevalence and titer of Epstein‐Barr virus antibodies in patients with multiple sclerosis
Authors: Sumaya Ciro V. , Myers Lawrence W. , Ellison George W. , Ench Yasmin 
Published: January 2005

AbstractThe prevalence and titer of serum antibodies to several Epstein‐Barr virus (EBV) antigens were compared among patients with multiple sclerosis, healthy siblings of multiple sclerosis patients, patients with other neurological diseases, and healthy non‐blood‐related subjects. An increased antibody response to EBV antigens was noted rather consistently in the sera of the multiple sclerosis group in comparison with the control group. A greater number of reduced ratios of serum: CSF IgG antibody to EBV‐capsid antigen and antibody to EBV‐early antigen components than to adenovirus, a referemce or control virus, were found in the multiple sclerosis group. Reduced ratios of these Ebv antibodies were detected more frequently or showed a trend in this direction in multiple sclerosis patients compared with the group with other neurological diseases. Our findings extend the result of an earlier report and strengthen the association between EBV and multiple sclerosis.
Motor neuron disease and adult hexosaminidase a deficiency in two families: Evidence for multisystem degeneration
Authors: Mitsumoto Hiroshi , Sliman Robert J. , Schafer Irwin A. , Sternick Cary S. , Kaufman Benjamin , Wilbourn Asa , Horwitz Samuel J. 
Published: January 2005

AbstractWe studied three patients from two unrelated families with adult hexosaminidase A deficiency. A 30‐year‐old, non‐Jewish proband in the first family had juvenile amyotrophic lateral sclerosis that evolved to mild demetia, ataxia, and axonal (neuronal) motor‐sensory peripheral neuropathy. A 36‐year‐old Jewish proband in the second family had “pure” spinal muscular atrophy. One supposedly healthy brother of the first proband was found to have borderline IQ, mild spasicity, ataxia but no evidence of motor neuron disease. Marked cerebellar atrophy was detected by head scans in all three patients. In both probands electromyograms were chacteriszed prominent, complex repetitive discharges in many muscles. Hexosaminidase A activities against the artificial substrate were similar to those reported in infantile Tay‐Sachs disease; however, the hexosaminidase A level against GM2 substrates was higher than that found infantile Tay‐Sachs disease. The hexosaminidase A levels of the parents were in the heterozygous range. Motor neuron disease in our patients and in those previously described appears to be park of a multisystem degeneration of the nervous system.
Leptomeningeal metastases: Peripheral nerve and root involvement—clinical and electrophysiological study
Authors: Argov Zohar , Siegal Tali 
Published: January 2005

AbstractTwenty‐five patients with revently diagnosed leptomeningeal metastases underwent a prospective clinical and electrophysiological study to assess the mode and extent of peripheral nerve and spinal root invlovement. Motor conduction velocities and F wave latencies in the legs were found frequently to be disturbed compared with normal subjects and a control group of cancer patients. F wave changes were the most sensitive indicator of root involvement. Electrophysiological abnormalities were detected in 20 patients, compared with 15 who had abnormal clinical signs suggesting peripheral nerve dysfunction. Electrophysiological studies can be useful in the evaluation of patients with suspected leptomeningeal metastases, and repeated recording of F wave latencies may serve to indicate root recovery in a clinically stable patient.
Electroencephalographic spiking activity, drug levels, and seizure occurence in epileptic patients
Authors: Gotman J. , Marciani M. G. 
Published: January 2005

AbstractWe investigated the relationship among the electroencephalographic spiking rate, drug levels, and seizure occurence in 44 patients with focal epilepsy. Seizure occurence was continuously monitored by personnel or videorecording and spiking rate quantified by an atuomatic detection method. Results indicate that drug levels do not influence spiking rate, and spiking rate does not change before seizures but increases markedly after them, particularly secondarily generalized seizures. This increase can last several days and is observed during wakefulness and sleep. High or low spiking rates do not influence the occurence of seizures. We suggest that interictal spikes may passively reflect damage to the brain, a damage which worsened by further seizures. Spikes may not be directly related to seizure generation.
31P NMR study of cerebral metabolism during prolonged seizures in the neonatal dog
Authors: Young Richard S. K. , Osbakken Mary D. , Briggs Richard W. , Yagel Susan K. , Rice David W. , Goldberg Stuart 
Published: January 2005

AbstractThe effects of prolonged bicuculline‐induced seizures on cerebral blood flow and metabolism were determined in paralyzed, mechanically ventilated neonatal dogs. Transient changes occurring early in the course of status epilepticus included significant arterial hypertension, hypocarbia, elevation of plasma norepinephrine levels, and decline in brain glucose concentration. Cerebral blood flow remained elevated throughout the 45 minutes of seizure. Determination of cerebral metabolite values by in vivo phosphorus 31 nuclear magnetic resonance spectroscopy and by in vitro enzymatic analysis of frozen brain samples showed significant decreases in the level of phosphocreatine and relatively less change in ATP values. Progressive intracellular acidosis occurred, coincident with elevation of brain lactate concentrations. We conclude that the physiological and metabolic alterations that occur during prolonged seizures are not uniform, but change with time. Any hypothesis advanced to explain the mechanism of neuronal injury during prolonged seizures must take into account these temporally related changes.
Evoked potential testing in relatives of multiple sclerosis patients
Authors: Nuwer Marc R. , Visscher Barbara R. , Packwood James W. , Namerow Norman S. 
Published: January 2005

AbstractEvoked potential (EP) tests were obtained in 110 neurologically normal first‐degree relatives of patients with multiple sclerosis. Visual EP tests were performed in all relatives; brainstem auditory and median nerve somatosensory EP tests were performed in 67 relatives. The relatives had a mean visual EP P100 latency that was significantly longer than that for normal subjects controlled for age and gender. Asymmetries were seen in results from individual MS relatives, including interocular visual EP P100 differences of up to 14 ms, and interarm somatosensory Erb‐N18 differences of up to 3.0 ms. We identified 19 pairs of patients and relatives who were HLA identical and 18 other pairs who were HLA double nonmatched. EP asymmetries were seen more often in the HLA identical siblings than in the HLA double nonmatched siblings. This suggests that subclinical, focal, and electrophysiological changes do occur in relatives of MS patients, especially if they share HLA types with the patients. Since less than 2% of siblings of MS patients would be expected to eventually develop clinical MS, these small subclinical electrophysiological changes are not expected to be a sign of the future appearance of clinical MS. Clinicians should be aware not to overinterpret small EP changes in relatives of MS patients.
Cytomegalovirus and herpes simplex virus ascending myelitis in a patient with acquired immune deficiency syndrome
Authors: Tucker Tarvez , Dix Richard D. , Katzen Carolyn , Davis Richard L. , Schmidley James W. 
Published: January 2005

AbstractProgressive ascending myelitis was the presenting feature of the acquired immune deficiency syndrome (AIDS) in a homosexual man who also had Kaposi's sarcoma, Pneumocystis pneumonia, and disseminated cytomegalovirus (CMV) infection. Neuropathological studies showed profuse cytomegalic cells throughout the brain and spinal cord, but no inflammatory response. At postmortem examination, CMV and herpes simplex virus, type 2 (HSV‐2), were recovered from multiple sites throughout the central nervous system (CNS). HSV‐2 was isolated from the anus, but from no other extraneural site; in contrast, pathology typical of CMV was also seen in the liver, gastrointestinal tract, adrenals, and lungs. Although histopathological evidence suggesting prior CMV infection has been seen in the brains of AIDS patients, the virus has never been cultured from the CNS in these immunosuppressed hosts, nor has it been known to infect the spinal cord. The absence of an inflammatory response suggests that the pathogenesis of CNS viral infections is altered in AIDS patients. Evidence for CMV infection of the CNS in AIDS patients is no longer circumstantial.
Serum prolactin and cortisol levels in evaluation of pseudoepileptic seizures
Authors: Pritchard Paul B. , Wannamaker Braxton B. , Sagel Julius , Daniel Charlene M. 
Published: January 2005

AbstractIn 6 patients with epilepsy, a twofold increase in serum prolactin levels followed true epileptic seizures, but no significant change followed pseudoepileptic attacks in 6 other patients. Serum prolactin concentration is a useful biochemical marker to distinguish between epileptic and pseudoepileptic seizures. Serum cortisol levels also increased after epileptic seizures, but diurnal and individual variations render the cortisol level a less reliable indicator of such attacks.
Human cutaneous mechanoreceptors during regeneration: Physiology and interpretation
Authors: Mackel Robert 
Published: January 2005

AbstractPercutaneous microneurography was used to record activity from single cutaneous afferents reinnervating the glabrous skin of the hand. Recordings were obtained from 6 months to 10 years after complete transsection of the median and/or ulnar nerves with subsequent repair. Transitional properties in the discharge behavior and receptive field characteristics of regenerating afferents were detected during the early stages of regeneration, when axonal maturation was still in progress; these may contribute to the slow time course of sensory recovery. Properties of reinnervated receptors long after nerve repair, when regeneration can be considered complete, indicated a correlation between electrophysiological findings and the final state of clinical recovery. The insights into the neural basis of sensory deficits provided by single‐unit microneurography are not yielded by conventional study of the compound action potentials. This technique reveals new information about the pathophysiological nature of peripheral nerve injury and leads to a reinterpretation of clinical deficits.