bluuebunny/crossref_metadata_2025_split huggingface dataset:
Assessment of vestibulo‐ocular reflexes in congenital nystagmus
Authors: Gresty Michael A. , Barratt Hilary J. , Page Nicholas G. R. , Ell Jonathan J. 
Published: January 2005

AbstractThe vestibulo‐ocular reflex and its suppression by fixation of a target rotating with the subject were tested in 18 subjects with congenital nystagmus using steps of constant velocity rotation and sinusoidal stimuli swept in frequency between 0.01 and 1.0 Hz. Responses to stopping stimuli were abnormal in waveform and of short duration in most subjects tested. This pattern was attributed to masking of the response by spontaneous eye movements and to adaptation. In contrast, during both oscillation in the dark and attempted suppression of the vestibulo‐ocular reflex, all subjects had nystagmus that was modulated with the stimulus during all frequencies of stimulation. The phase relationship of the nystagmus to the motion stimulus was the same as in normal subjects. Estimates of gain of the vestibulo‐ocular reflex response were not meaningful because of contamination of the vestibular response by the congenital nystagmus waveforms. Modulation of amplitude and reversal of nystagmus in phase with the vestibular stimulus at all frequencies of oscillation were shown most clearly during attempted suppression of the vestibulo‐ocular reflex. This finding is clinically useful because it establishes suppression as a test of the presence of vestibular function in congenital nystagmus.
Hyponatremia and cerebral infarction in patients with ruptured intracranial aneurysms: Is fluid restriction harmful?
Authors: Wijdicks E. F. M. , Vermeulen M. , Hijdra A. , van Gijn J. 
Published: January 2005

AbstractWe studied retrospectively the relationship between hyponatremia and cerebral infarction in 134 consecutive patients with aneurysmal subarachnoid hemorrhage. In 44 patients sodium levels fell below 135 mmol/L on at least two consecutive days between the second and the tenth day after the hemorrhage. Twenty‐five of these patients fulfilled the criteria for the syndrome of inappropriate secretion of antidiuretic hormane. Cerebral infarction developed in 27 of the 44 patients with hyponatremia and in 19 of the 90 patients with normal serum sodium levels (p < 0.001). Cerebral infarctions were more often fatal in patients with hyponatremia (p < 0.01).Twenty‐six of the 44 patients had been treated with fluid restriction to correct the serum sodium levels, and infarctions developed in 21. Fluid restriction to correct hyponatremia appears to be potentially dangerous in patients with aneurysmal subarachnoid hemorrhage.
Computed tomographic analysis of brain morphometrics in 30 healthy men, aged 21 to 81 years
Authors: Schwartz Michael , Creasey Helen , Grady Cheryl L. , DeLeo James M. , Frederickson Hal A. , Cutler Neal R. , Rapoport Stanley I. 
Published: January 2005

AbstractComputed transverse axial tomography (CT) was employed to examine brain morphometrics in 30 healthy men, aged 21 to 81 years. Seven consecutive CT slices 30 to 80 mm above the inferior orbitomeatal line were analyzed. CT numbers in grey and white matter regions were not correlated significantly with age (p<0.05), nor were right/left ratios for derived parameters. The volume of gray matter was correlated negatively with age (p>0.05), and the volume of cerebrospinal fluid correlated positively with age, in the seven slices. The volumes of the lateral and third ventricles were elevated in the elderly, and volumes of the thalamus and tenticular nucleus were reduced. The results demonstrate that brain atrophy, evidenced by a loss of grey matter and by dilatation of cerebrospinal fluid spaces, occurs in the healthy elderly, and provide baseline CT‐derived brain morphometric data for healthy men in relation to age.
The second sensory area in humans: Evoked potential and electrical stimulation studies
Authors: Lüders H. , Lesser R. P. , Dinner D. S. , Hahn J. F. , Salanga V. , Morris H. H. 
Published: January 2005

AbstractA patient with intractable seizures originating from a right frontal focus was evaluated for surgical treatment. This evaluation was carried out using a chronically implanted array of 96 stainless steel electrodes 1 cm apart and covering the perirolandic and frontal areas. Somatosensory evoked potentials and electrical stimulation of the subdural electrodes localized the primary sensory hand area. Evoked potentials of identical waveform but of lower amplitude and 2.4 ms longer latency were recorded in the inferior frontal gyrus immediately anterior to the face area of the motor strip. Electrical stimulation of that area elicited: (1) a “paralyzing” feeling in the left arm and face; (2) inhibition of rapid alternating movements of left fingers, left hand, and tongue; (3) inability to maintain a strong voluntary muscle contraction of the left hand or tongue; and (4) speech arrest. This appears to be the first report of a secondary sensory area in humans demonstrated by both electrical stimulation and evoked potential studies. Electrical stimulation showed that the secondary sensory area overlapped an area of complex motor control, suggesting that the secondary sensory area provides direct sensory feedback information for appropriate motor integration.
Myophosphorylase deficiency impairs muscle oxidative metabolism
Authors: Haller Ronald G. , Lewis Steven F. , Cook J. D. , Blomqvist C. G. 
Published: January 2005

AbstractWe studied oxidative metabolism during bicycle exercise in 4 patients with myophosphorylase deficiency. Maximal oxygen uptake (VO2max) was low (14.0 ± 1.4ml·kg−1· min−1, mean ± SE) compared with that in normal subjects (37.7 ± 1.9; n = 12) and patients with myalgia (24.9 ± 1.8; n = 10). Carbohydrate oxidation, as estimated by the respiratory exchange ratio (R), was low relative to workload (max R, mean ± SE: McArdle's disease, 0.96 ± 0.02; normal subject, 1.13 ± 0.02; myalgia, 1.09 ± 0.02). Intravenous glucose administration increased maximal oxygen uptake about 20% in those with McArdle's disease, but both VO2max and R remained lower than in control subjects. These findings suggest that the capacity for dynamic exercise in McArdle's disease is limited by the availability of oxidative substrate, and indicate that blood glucose is unable to substitute fully for muscle glycogen as an oxidative fuel. We also found that exercise cardiac output (Q) was excessive relative to oxygen uptake in affected patients (δQ̇/δV̇O2, mean ± SE: McArdle's disease, 11.6 ± 1.7; normal subjects, 48 ± 0.2; myalgia, 5.6 ± 0.2). This hyperkinetic circulation in exercise may serve to increase the delivery of blood‐brone oxidative substrate to working muscle.
Myasthenia gravis: Reactivation of clinical disease and of automimmune factors after discontinuation of long‐term azathioprine
Authors: Hohlfeld Reinhard , Toyka Klaus V. , Besinger Uwe A. , Gerhold Beate , Heininger Kurt 
Published: January 2005

AbstractIn 15 patients with myasthenia gravis who were in stable clinical remission while receiving azathioprine, we monitored disease severity and serial autoantibody titers before and after discontinuation of azathioprine. Cellular immunoreactivity against tuberculin (PPD) and agianst Torpedo acetylcholine receptor (AChR) was measured serially in 11 patients. Eight of 15 patients (53%) had a clinical relapse after 3 to 11 months, necessitating the reinstitution of immunosuppressive treatment in 6 patients. Seven patients have remained clinically stable during an observation period of 20 to 40 months. Anti‐AChR autoantibody titers correlated closely with the clinical course in the majority of patients, and rese markedly in 7 of the 8 patients who relapsed. Cellular stimulation indices correlated less closely with the clinical severity. Only in 3 patients did the clinical score, antibody titer, and cellular stimulation index rise concurrently. In 4 patients who had high cellular stimulation indices after the discontinuation of azathioprine, it was possible to isolate AChR‐reactive inducer/helper T lymphocytes.
Axonal attenuation and secondary segmental demyelination in myeloma neuropathies
Authors: Ohi Takakazu , Kyle Robert A. , Dyck Peter James 
Published: January 2005

AbstractThe sural nerves of 5 patients with osteosclerotic myeloma and polyneuropathy, of 3 patients with multiple myeloma and polyneuropathy, and of 6 healthy subjects were studied by neuropathological, morphomemmtric, and teased‐fiber approaches to assess cellular (Schwann cell or axon) vulnerability and to explore the mechanism of segmental demyelination. As compared with controls, the nerves of both types of myeloma neuropathy demonstrated a statistically significant and marked loss of myelinated fibers and increased frequencies of axonal degeneration among teased fibers at statistically significant levels. The peaks of diameter histograms of myelinated fibers of osteosclerotic myeloma/polyneuropathy nerves were displaced to smaller diameter categories, suggesting fiber atrophy. Segmental demyelination and remyelination was clustered, as found in secondary demyelination. Large‐and intermediate‐diameter myelinated fibers of osteosclerotic myeloma/polyneuropathyl nerves had significantly smaller axon calibers relative to myelin spiral length seen in electron micrographs. The loss of myelinated fiber axons, the shift of the peaks of diameter histograms to smaller sizes, the lack of noticeable increased numbers of demyelinated axons, the clustered distribution of segmental demyelination, and the axonal attenuation suggest a special axonal or neuronal vulnerabilityl and appear to provide an explanation for the observed segmental demyelination. Whether axonal attenuation has a perikaryaeal or proximal genesis now needs to be determined.
γ‐Vinyl GABA: A double‐blind placebo‐controlled trial in partial epilepsy
Authors: Gram Lennart , Klosterskov Peter , Dam Mogens 
Published: January 2005

AbstractThe antiepileptic effect of γ‐vinyl γ‐aminobutyric acid (GABA), an irreversible GABA‐transaminase inhibitor, was investigated in an add‐on, placebo‐controlled, double‐blind, cross‐over, fixed‐dose trial. Twenty‐one patients suffering from difficult to control complex partial seizures participated; 18 patients completed the trial. Serum levels of concomitant antiepileptic drugs were kept constant throughout the trial. Three patients (17%) reduction in seizure frequency and in 8 (44%) the seizures were reduced by at least 50%. Two patients developed a modereat and 1 patient a marked increase in seizure frequency during treatment with γ‐vinyl GABA. Except for 2 patients, who had to discontinue the trial becuase of adverse effects of γ‐vinyl GABA, the participants were unable to discriminate between treatment regimens with regard to side effefts γ‐vinyl GABA seems to be a promosing new antiepileptic drug, and the first one to present convincing evidence of a GABAergic mechanism of action.
Anatomical basis for acquired fluent aphasia in children
Authors: VanDongen Hugo R. , Loonen M. Christa B. , VanDongen Krijn J. 
Published: January 2005

AbstractThree girls aged 9 to 11 years developed fluent aphasia associated with acute brain lesions. As localized by computed tomography, the abnormalities in all three resided in the posterior part of the left hemisphere, encroaching upon Wernicke's area.
Association between essential tremor and Parkinson's disease
Authors: Geraghty John J. , Jankovic Joseph , Zetusky Walter J. 
Published: January 2005

AbstractTo examine a possinle relationship of essential tremor to Parkinson's disease (PD), 130 patients with essential tremor were studied. Twenty‐five patients had both essential tremor and PD. These 25 patients were matched for age and duration of PD symptoms with 25 patients who had idiopathic PD. Bradykinesia, postural instability, and gait difficulty were more severe in the patients with idiopathic PD. Degree of tremor, rigidity, and fumctional disability did not differ in the two groups. The prevalence of PD in the population with essential tremor was 24 times greater than expected. This study suggests that some patients with essential tremor have genetically increased risk for PD.
Progressive multifocal leukoencephalopathy: Clinical and radiographic features
Authors: Krupp Lauren B. , Lipton Richard B. , Swerdlow Michael L. , Leeds Norman E. , Llena Jose 
Published: January 2005

AbstractBetween April 1982 and March 1984 7 pathologically confirmed cases of progressive multifocal leukoencephalopathy (PML) were diagnosed at our institution. Only 1 case had been seen in the preceding twenty years. Four patients had acquired immunodeficiency syndrome (AIDS). The others had chronic lymphocytic leukemia, Hodgkin's lymphoma and systemic lupus erythematosus. All patients presented with progressive neurological deficits. In most, the initial computed tomographic (CT) scan was disproportionately less abnormal than the clinical findings. In 5 patients the first CT scan revealed hypodensities of the cerebral white matter which lacked mass effect and did not enhance with contrast agent. The lesions were observed to enlarge progressively on CT scans but often lagged behind the rate ofcllinical evolution. Although 3 patients were treated with cytosine arabinoside, none improved. PML had similiar clinical, radiographic, and pathological features in the AIDS and non‐AIDs patients. Of 79 AIDS paatients cared for at our institution between December 1979 and December 1983, 3.8% had PML. PML should be suspected in AIDS patients in the presence of the characteristic CT features, especially when CT‐clinical dissociation occurs.
Reversible, late‐onset disulfiram‐induced neuropathy and encephalopathy
Authors: Borrett D. , Ashby P. , Bilbao J. , Carlen P. 
Published: January 2005

AbstractDisulfiram toxicity is a well‐recognized cause of peripheral neuropathy and encephalopathy, usually developing within a few months of the start of therapy. We describe a patient who insidiously developed a peripheral neuropathy and encephalopathy after thirty years of disulfiram ingestion. Both complications partially resolved after the medication was stopped.
Infantile cerebellar atrophy
Authors: Furman J. M. , Baloh R. W. , Chugani H. , Waluch V. , Bradley W. G. 
Published: January 2005

AbstractWe descrine a family whose menbers have a dominantly inherited, early‐onset, nonprogressive syndrome that includes spontaneous upbeating nystagmus and mild gait ataxia. Magnetic resonance imaging showed localized atrophy of cerebellar cermis. Several families described in the literature resemble our family but differ in mode of inheritance, age at onset, rate of progression or clinical findings. We believe this family represents a unique type of inherited early‐onste atrophy of the cerebellar vermis.
Brain neurotransmitter abnormalities in neuronal intranuclear inclusion body disorder
Authors: Kish S. J. , Gilbert J. J. , Chang L. J. , Mirchandani L. , Shannak K. , Hornykiewicz O. 
Published: January 2005

AbstractBiochemical markers for the major neurotransmitter systems measured in the brain of a patients who died with neuronal intranuclear inclusion disease. A severe nigrostriatal dopamine deficiency constitutes the basis for the parkinsonian features in the patient. A marked loss of brain norepinephrine and serotonin was found in basal ganglia and hypothalamus. Brain amino acid (γ‐aminobutyric acid and glutamate) and cholinergic (choline acetyltransferase activity) systems were either normal or less severely affected.
Hypoglycemia: Causes, neurological manifestations, and outcome
Authors: Malouf Renée , Brust John C. M. 
Published: January 2005

AbstractDuring a 12‐month prospective study there were 125 visits to the Harlem Hospital Emergency Room for symptomatic hypoglycemia. Sixty‐five patients had obtundation, stupor, or coma; 38 had confusion or bizarre behavior; 10 were dizzy or tremulous; 9 had had seizures; and 3 had suffered sudden hemiparesis. Diabetes mellitus, alcoholism, and sepsis, alone or in combination, accounted for 90% of predisposing conditions; others included fasting, terminal cancer, gastroenteritis, insulin abuse, and myxedema. Average blood glucose level were lower among comatose than among obtunded patients, but overlap was considerable, and overall there was little correlation among cause, blood glucose levels, and symptoms. Although mortality was 11%, only one death was attributable to hypoglycemia per se, and only four survivors had focal neurological residua.
Basal ganglia glucose utilization after recent precentral ablation in the monkey
Authors: Dauth George W. , Gilman Sid , Frey Kirk A , Penney John B. 
Published: January 2005

AbstractIn the macaque monkey, unilateral ablation of areas 4 and 6 of Brodmann result initially in a signficant decrease of glucose metabolic activity in the ipsilateral caudate nucleus, putamen, globus pallidus, substantia nigra, and subthalamic nucleus. The contralateral hemisphere shows nonsignificant but consistently decreased activity in the caudate nucleus, putamen, and globus pallidus. Cerebral blood flow is decreased in the same pattern as the glucose metabolic activity. The change in glucose metabolic activity result from loss of neurons known to project directly from the cerebral cortex to the basal ganglia and also from indirect effect(diaschisis) in basal ganglia structures that do not receive connections from the cerebral cortex.
Nuerological finding in triosephosphate isomerase deficiency
Authors: Poll‐The Bwee Tien , Aicardi Jean , Girot Robert , Rosa R. 
Published: January 2005

AbstractTwo siblings with hemolytic anemia caused by triosephosphate isomerase deficiency developed a progressive neurological syndrome featuring dystonic movements, tremor, pyramidal tract signs, and evidence of spinal motor neuron involvement. Intelligence was unaffected. The findings in these patients and in 14 previously published cases indicate that neurological manifestations are an integral part of the disorder and suggest that specific structures in the basal ganglia, brainstem, and spinal cord bear the brunt of the pathological process, which does not affect the cerebral cortex.
Progressive encephalopathy in children with acquired immue deficiency syndrome
Authors: Epstien Leon G. , Sharer Leroy R. , Joshi Vijay V. , Fojas Myrna M. , Koenigsberger M. Richard , Oleske James M. 
Published: January 2005

AbstractWe are reporting the cases of four children with a diagnosis of acquired immune deficiency syndrome (AIDS) who demonstrated a progressive encephalopathy manifested by loss of motor milestones or intellectual abilities, and weakness with pyramidal tract signs. Two patients were ataxic. One child was cortically blind and suffered myoclonic jerks before his death. Two of the patients had isolated seizures at some time during the disease course. Two patients developed secondary microcephaly and all four patients showed cortical atrophy on computed tomographic scanning. Three of the patients died. Postmortem neuropathological findings included gross cortical atorphy, microglial nodules, and intranuclear inclusions, as are described in the subacute encephalitis reported in adult AIDS patients. We propose that the possible pathogenetic mechanisms of the encephalopathy include opportunistic or persistent viral infection of the brain.
Action of Lambert‐Eaton myasthenic syndrome IgG at mouse motor nerve terminals
Authors: Prior C. , Lang B. , Wray D. , Newsom‐Davis J. 
Published: January 2005

AbstractWe have studied the electrophsiological effects of IgG obtained from four patients with Lambert‐Eaton myasthenic syndrome (LEMS) (two with small cell carcinoma), using the mouse passive transfer model. Mice received LEMS or control IgG or plasma, 10 to 60 mg daily. Microeletrode intracellular recordings were from diaphragm muscle. LEMS IgG and plasma decreased end‐plate potential quantal content similarly, confirming IgG as the active factor. LEMS IgG was equally effective C5‐deficient mice, indicating that late complement components are not required. The time course of decline and recovery of quantal content closely followed that of the human IgG in the mouse serum, with time to half‐maximal effect of about 1.5 days in each case. Binding/dissociation of IgG or down/up regulation of the antigenic determinants, possibly Ca2+ channels, has a half‐life of between 2 and 36 hours. The results confirm our concepts that IgG antibody to nerve terminal determinants underlies the disorder of transmitter release in LEMS.
Leukoencephalopathy in diffuse hemorrhagic cerebral amyloid angiopathy
Authors: Gray F. , Dubas F. , Roullet E. , Escourolle R. 
Published: January 2005

AbstractWe have studied 12 patients with diffuse hemorrhagic cerebral amyloid angiopathy clinically and at postmortem examination. The brains in 8 patients had diffuse bilateral loss of myelin in the hemispheric white matter sparing the U fibers, corpus callosum, and internal capsules. The periventricular areas were predominantly affected. Microscopic examination of the white matter showed an association with subacute or chronic edematous lesions: spongiosis, swollen oligodendroglia, widening of the perivascular spaces with edema fluid or siderophages, hyalinization of the blood vessel walls, incomplete myelin loss, and astrocytic gliosis. Three of 8 autopsied patients had undergone computed tomographic examination, which showed bilateral hypodensity of the hemispheric white matter. The brains of 4 patients with illnesses of shorter duration showed only discrete but similar lesions in the centrum semiovale. These white matter changes are similar to those observed in Binswanger's subcortical encephalopathy. We suggest that a common mechanism of hypoperfusion of the distal white matter causes the leukoencephalopathy.
Metachromatic leukodystrophy manifesting as a schizophrenic disorder: Computed tomographic correlation
Authors: Finelli Pasquale F. 
Published: January 2005

AbstractA 32‐year‐old woman with a 12‐year history of schizophrenia demonstrated symmetrical bifrontal and biparietal periventricular hypodensities on computed tomographic scan. Sural nerve biopsy and urine and leukocyte enzyme assay confirmed the diagnosis of metachromatic leukodystrophy. The computed tomographic correlate in an adult with metachromatic leukodystrophy in whom the psychiatric manifestations were the predominant clinical feature is described.
Immunofluorescence study of patients with neuropathy and IgM M proteins
Authors: Takatsu Masami , Hays Arthur P. , Latov Norman , Abrams Gary M. , Nemni Raffaello , Sherman William H. , Nobile‐Orazio Eduardo , Saito Toyokazu , Freddo Lorenza 
Published: January 2005

AbstractImmunofluorescence histochemistry was used to study the pathogenesis of polyneuropathy in patients with an IgM M protein. Seventeen patients had an M protein that reacted with myelin‐associated glycoprotein (MAG), and their serum immunostained myelin sheaths of normal peripheral nerve of humans and certain other species. The staining was specific for the M protein idiotype and was abolished by prior absorption of serum with MAG. The sural nerve biopsy specimens from these 17 patients had pathological features of primary demyelination and deposits of IgM on the myelin sheaths. Sural nerve specimens of 2 patients with an M protein reactive with chondroitin sulfate showed axonal degeneration and diffuse deposits of IgM in the endoneurium. Serum of one of these patients immunostained connective tissue; the staining was specific for the M protein idiotype and was blocked by absorption of the serum with chondroitin sulfate. The antigenic specificity of the IgM M protein in another 9 patients with neuropathy is not known; however, sural nerve specimens obtained from some of the patients showed axonal degeneration and endoneurial deposits of IgM, and the serum IgM immunostained axons in some instances. The findings suggest that IgM M proteins may cause the neuropathy and that more than one autoantigen is involved.