bluuebunny/crossref_metadata_2025_split huggingface dataset:
Motor conduction studies in guillain‐barré syndrome: Description and prognostic value
Authors: Cornblath David R. , Mellits E. David , Griffin John W. , McKhann Guy M. , Albers James W. , Miller Robert G. , Feasby Thomas E. , Quaskey Shirley A. ,   The Guillain‐Barré Syndrome Study Group
Published: January 2005

AbstractThe North American study of plasmapheresis in Guillain‐Barré syndrome (GBS) included early, standardized electrodiagnostic testing in 210 of the 245 patients. To determine the types of abnormalities and the relation to outcome, we analyzed the prospectively collected motor conduction data obtained during the first 30 days of illness. For each parameter of motor conduction data, means were computed based on institutional normal data. Mean distal motor and F wave latencies commonly were abnormal in these early studies. In univariate analysis, mean compound muscle action potential (CMAP) amplitude from distal and proximal stimulation and mean motor conduction velocity were found to be related to the four predetermined outcome measures. In multivariate analysis of the motor conduction velocity were found to be related to the four predetermined outcome measures. In multivariate analysis of the motor conduction parameters, mean distal CMAP amplitude was the single best predictor of prognosis; other measures did not add to its predictive value. In further multivariate analysis of all factors relating to outcome, mean distal CMAP amplitude was determined to be the most powerful predictor of outcome, followed by plasmapheresis. These two factors were always statistically significant over and above all other variables. Our results indicate that prognostic information can be obtained from motor conduction studies even when performed early in the course of GBS; a mean distal CMAP amplitude of 0 to 20% of the lower limit of normal is associated with a markedly increased probability of a poor outcome. Nevertheless, even in patients with a low mean distal CMAP amplitude, the predicted outcome is improved with plasmapheresis therapy.
Clinical and neuropathological findings in systemic lupus erythematosus: The role of vasculitis, heart emboli, and thrombotic thrombocytopenic purpura
Authors: Devinsky Orrin , Petito Carol K. , Alonso Daniel R. 
Published: January 2005

AbstractWe reviewed the medical records and autopsy reports of 50 patients with systemic lupus erythematosus to determine the clinical and neuropathological features of this disease. Neuropsychiatric disturbances were found in the majority (74%) of the patients, occurring as psychiatric illness only (5 patients), neurological idsorders only (15 patients), and both together (17 patients). Central nervous system (CNS) lesions were present in half the patients; embolic brain infarcts (10 patients) and CNS infections (8 patients) were the most common. Cardiac sources of emboli were Libman‐Sacks endocarditis (5 patients), chronic valvulitis (2 patients), and left side of heart mural thrombus (2 patients). There was no evidence of active CNS vasculitis. Clinical features of thrombotic thrombocytopenic purpura (TTP) developed during the terminal illness in 14 patients, 7 of whom also had pathological evidence of TTP. Correlation between neuropsychiatric disorders and brain lesions could be made in approximately half the patients. This study indicates that cardiac emboli from Libman‐Sacks endocarditis and TTP are common pathogenetic factors of CNS disease in systemic lupus erythematosus, whereas CNS vasculitis is rare.
Prolonged deterioration of ischemic brain energy metabolism and acidosis associated with hyperglycemia: Human cerebral infarction studied by serial 31P NMR spectroscopy
Authors: Levine Steven R. , Welch K. M. A. , Helpern J. A. , Chopp Michael , Bruce Randal , Selwa James , Smith Michael B. 
Published: January 2005

AbstractWe report on a patient with a large ischemic hemispherical stroke studied serially by 31P nuclear magnetic resonance spectroscopy. Persistent hyperglycemia was associated with prolonged acidosis in ischemic brain and failure of high‐energy phosphate metabolism to recover. These in vivo human data support the concept that hyperglycemia adversely affects ischemic brain metabolism, pH, and clinical outcome.
Subcortical neglect: Neuropsychological, SPECT, and neuropathological correlations with anterior choroidal artery territory infarction
Authors: Bogousslavsky Julien , Miklossy Judit , Regli Franco , Deruaz Jean‐Pierre , Assal Gil , Delaloye Bernard 
Published: January 2005

AbstractIn 2 patients with infarction in the territory of the right anterior choroidal artery, hemiparesis, hemihypesthesia (in 1), and hemianopia or superior quadrantanopia were associated with severe multimodal hemineglect, without anosognosia, disorientation, or asomatognosia. Single‐photon emission tomography showed that marked hypoperfusion was not limited to the right posterior capsular region, but also involved the overlying parietal cortex, and to a lesser extent the frontal cortex. At autopsy in 1 patient, the infarct was nearly limited to the deep white matter of the temporal isthmus and the retrolenticular part of the internal capsule; only minute lesions were present in the globus pallidus, body of caudate, and amygdala. These findings are consistent with a disconnection phenomenon as the basis for subcortical neglect with ipsilateral deactivation of the parietofrontal cortex.
Peripheral neuropathy in myotonic dystrophy: A nerve biopsy study
Authors: Cros Didier , Harnden Patricia , Pouget Jean , Pellissier Jean Françlois , Gastaut Jean Louis , Serratrice Georges 
Published: January 2005

AbstractSural nerve biopsies from 13 unselected myotonic dystrophy patients and 6 normal controls were studied morphometrically. The myelinated fiber density was reduced in 11 of the 13 myotonic dystrophy patients, with preferential loss of large myelinated fibers. Unmyelinated fiber densities and diameters were normal. Teased fiber studies commonly revealed focal areas of remyelination and abnormal wrinkling of the myelin sheath. Measurement of internodal length disclosed features of both axonal regeneration and focal demyelination‐remyelination. These findings are consistent with a chronic axonopathy of moderate severity, possibly due to axonal atrophy.
Mild senile dementia of the alzheimer type: 2. Longitudinal assessment
Authors: Berg Leonard , Miller J. Philip , Storandt Martha , Duchek Janet , Morris John C. , Rubin Eugene H. , Burke William J. , Coben Lawrence A. 
Published: January 2005

AbstractForty‐three subjects with mild senile dementia of the Alzheimer type (SDAT, so‐called probable Alzheimer's disease) were studied longitudinally by serial administration of several tests to rate dementia. Comparison was made with 58 healthy elderly controls. The clinical diagnosis was confirmed in all 16 autopsies. At 5 years after entry into the study, the cumulative rate of nursing home placement was 73%, and of death, 30%, rising at 7 years to 84% and 44%, respectively. Four of six clinical measures of dementia were found to correlate with the natural history of SDAT. Several measures are better than a single one in carrying out longitudinal studies of the disorder.
Antimigraine drug interactions with serotonin receptor subtypes in human brain
Authors: Peroutka Stephen J. 
Published: January 2005

AbstractThe interactions of antimigraine agents with serotonin (5‐hydroxytryptamine, 5‐HT) receptor subtypes were analyzed in human frontal cortex membranes. The drugs studied included 5‐HT antagonists, beta‐adrenergic antagonists, and calcium channel blockers. At 5‐HT1A sites labeled by 3H‐8‐hydroxy‐2‐(N, N‐dipropylamino)‐tetralin, (–)pindolol, alprenolol, (–)propranolol, methysergide, cyproheptadine, and pizotifen are similar in that they display affinities of approximately 100 nM for this receptor. By contrast, only methysergide displays relatively high affinity (120 ± 60 nM), whereas all other drugs have affinities greater than 1,000 nM for non‐5‐HT1A sites labeled by 3H‐5‐HT in human cortex. Finally, at 5‐HT2 receptors labeled by 3H‐spiperone, cyproheptadine, methysergide, and pizotifen are extremely potent agents (affinity constants of 1 to 10 nM), whereas amitriptyline (23 ± 4 nM), verapamil (140 ± 50 nM), and nifedipine (320 ± 80 nM) are moderately potent. All other drugs are inactive at concentrations below 1,000 nM. These data demonstrate that most antimigraine drugs display high affinity for the 5‐HT1A and/or 5‐HT2 receptor subtypes in human brain. However, antimigraine efficacy cannot be explained by drug interactions with a single 5‐HT receptor subtype.
Motor neuron syndrome and monoclonal IgM with antibody activity against gangliosides GM1 and GD1b
Authors: Nardelli Ettore , Steck Andreas J. , Barkas Thomas , Schluep Myriam , Jerusalem Felix 
Published: January 2005

AbstractWe demonstrated that an IgM M‐protein from a patient with motor neuron syndrome had antibody activity against gangliosides GM1, GD1b, and asialo GM1. Studies with a sugar‐binding lectin suggested that the epitope in the patient's M‐IgM involved the Gal(β1‐3) GalNAc moiety. Immunohistological techniques demonstrated staining of axons in the lumbar roots, granular cells, and white matter in the cerebellum by the patient's M‐IgM. We propose that, in this case, an autoimmune mechanism of motor neuron syndrome associated with a monoclonal protein is most likely.
Positron emission tomographic scan investigations of Huntington's disease: Cerebral metabolic correlates of cognitive function
Authors: Berent Stanley , Giordani Bruno , Lehtinen Shirley , Markel Dorene , Penney John B. , Buchtel H. A. , Starosta‐Rubinstein Simon , Hichwa Richard , Young Anne B. 
Published: January 2005

AbstractFifteen drug‐free patients with early to mid‐stage Huntington's disease (HD) were evaluated with positron emission tomographic (PET) scans of 18F‐2‐fluro‐2‐deoxy‐D‐glucose uptake and quantitative measures of neurological function, learning, memory, and general intelligence. In comparison with a group of normal volunteers, the HD patients showed lower metabolism in both caudate (p < 0.001) and putamen (p < 0.001) on PET scans. A significant and positive relationship was found between neuropsychological measures of verbal learning and memory and caudate metabolism in the patient group but not in the normal group. Visual‐spatial learning did not reflect a similar pattern, but performance intelligence quotient was positively related to both caudate and putamen metabolism in the HD group. Vocabulary level was unrelated to either brain structure. Discussion focuses on these and other observed brain‐behaviour relationships and on the implications of these findings for general behaviors such as those involved in coping and adaptation.
Thalamic astasia: Inability to stand after unilateral thalamic lesions
Authors: Masdeu Joseph C. , Gorelick Philip B. 
Published: January 2005

AbstractInability to stand in the absence of motor weakness or marked sensory loss is usually considered to reflect midline cerebellar disease. However, the 15 patients reported here had astasia related to unilateral thalamic lesions, documented by autopsy and computed tomography in 2 patients and by computed tomography in 13. The lesions, including infarction (6), hemorrhage (7), and tumor (2), involved primarily the superoposterolateral portion of the thalamus, but spread the rubral region. Alert, with normal or near‐normal strength on isometric muscle testing and a variable degree of sensory loss, the patients could not stand and 7 of them could not sit up unassisted. They fell backwards or toward the side contralateral to the lesion. They appeared to have a deficit of overlearned motor activity of an axial and postural nature. In the vascular cases, the deficit improved in a few days or weeks. However, these patients had a tendency to sustain falls during the rehabilitation period.
Possible neurotransmitter basis of behavioral changes in alzheimer's disease
Authors: Palmer Alan M. , Stratmann Gary C. , Procter Andrew W. , Bowen David M. 
Published: January 2005

AbstractSerotonin, 5‐hydroxyindoleacetic acid, and homovanillic acid concentrations have been determined in 10 areas of the cerebral cortex from 17 subjects with Alzheimer's disease and 18 control subjects. The dopamine metabolite was not reduced in any area, whereas both indoleamines were reduced in the superior frontal, inferior temporal, and fusiform gyri, and the temporal pole. These areas and areas of the parietal cortex, where there were no changes in concentration, have not previously been reported on. We argue that the large loss of indoleamines from the frontal lobe (to 50–63% of control values) is rather unexpected based on other biochemical measurements and may relate to behavioral changes.
A potential role for excitotoxins in the pathophysiology of spinal cord injury
Authors: Faden Alan I. , Simon Roger P. 
Published: January 2005

AbstractIt has been proposed that endogenously released excitatory amino acids may contribute to injury of the central nervous system in a variety of disorders including certain neurodegenerative diseases, epilepsy, and cerebral ischemia. In the present studies we evaluated the hypothesis that excitatory amino acids, acting at the N‐methyl‐D‐aspartate (NMDA) receptor, contribute to secondary tissue damage following traumatic spinal cord injury. Administration of NMDA, adjacent to the trauma site, significantly worsened the outcome after thoracic cord injury in rats, whereas its stereoisomer, N‐methyl‐L‐aspartate (NMLA), was without effect. Systemic treatment with MK‐801—a selective, centrally active, NMDA antagonist—significantly improved neurological outcome after trauma. These findings extend the excitotoxin concept to central nervous system trauma and indicate that NMDA antagonists may be beneficial in the treatment of traumatic spinal cord injury.
Reduction of monocyte 5′nucleotidase activity by γ‐interferon in multiple sclerosis and autoimmune diseases
Authors: Armstrong Marilyn A. , Shah Soraya , Hawkins Stanley A. , Bell Aubrey L. , Roberts Stanley D. 
Published: January 2005

AbstractEcto‐5′nucleotidase (5′NT) activity in the plasma membrane of peripheral blood monocytes from patients with multiple sclerosis (MS), rheumatoid arthritis (RA), myasthenia gravis (MG), motor neuron disease (MND), and from normal control subjects of similar age was determined by radioisotopic assay. The activity in unstimulated monocytes cultured for 24 and 48 hours was found to be higher than normal in 56% of patients with active relapsing MS, 29% of patients with RA, and 7% of patients in the MG/MND group. While the enzyme activity was reduced after cultivation of the cells with recombinant interferonrγ(γ‐IFN) in all of the groups studied, the percentage reduction was significantly greater in monocytes from patients with active relapsing MS who were in relapse at the time of sampling (p= 0.03). HLA‐DR expression was monitored using immunofluorescence staining with monoclonal antibody and showed that similar numbers of monocytes in all patient and control groups expressed this antigen. Thus, while high monocyte 5′NT activity was found principally in patients with active relapsing MS and in some patients with RA, the monocytes from patients with MS were, in addition, exquisitely sensitive to stimulation withγ‐IFN.
Functional hemispherectomy for treatment of epilepsy associated with hemiplegia: Rationale, indications, results, and comparison with callosotomy
Authors: Tinuper Paolo , Andermann Frederick , Villemure Jean‐Guy , Rasmussen Theodore B. , Quesney Luis Felipe 
Published: January 2005

AbstractHemispherectomy is effective in arresting seizures associated with maximal or near maximal hemiparesis. This procedure, however, carries an unacceptable 33% risk of late complications due to cerebral hemosiderosis. Anatomically partial but functionally complete hemispherectomy was devised to avoid these complications. The frontal or occipital lobes, or both, were left in place with the blood supply intact but with connections to commissures and brainstem divided. The central strip and parietal and temporal lobes were removed. Twenty patients were so treated with a follow‐up of 4 to 13 years (average, 7.3 years) in 14. Ten of these are seizure free, 1 had a single nOctoberurnal seizure, 1 had occasional focal twitching, and 2 had a worthwhile but lesser reduction in the seizure tendency. None has developed cerebral hemosiderosis, to date. In appropriately selected patients, functional hemispherectomy is an effective procedure preferable to callosotomy or to partial hemispherectomy. When there is no independent ictal discharge from the opposite hemisphere, arrest of seizures may be expected, leading to improvement in cognitive functioning (mean increase, 10 IQ points), social behavior, and a reduction in or discontinuation of anticonvulsant medication. In these patients, gait and hand use remain unchanged.
Asymbolia for pain: A sensory‐limbic disconnection syndrome
Authors: Berthier Marchcelo , Starkstein Sergio , Leiguarda Ramon 
Published: January 2005

AbstractWe describe the behavioral and neuroanatomical features of asymbolia for pain occurring in 6 patients following unilateral hemispheric damage secondary to ischemic lesions in 5 and traumatic hematoma in 1. In the absence of priMarchy sensory deficits, these 6 patients showed a lack of withdrawal and absent or inadequate emotional responses to painful stimuli applied over the entire body, as well as to threatening gestures. Five patients also failed to react to verbal menaces. Patients appeared unconcerned about the defect and seemed unable to learn appropriate escape or protective responses. Common associated abnormalities were rapidly resolving hemiparesis, cortical‐type sensory loss, unilateral neglect, and body‐schema disorders. Neuroradiological examination disclosed left hemispheric lesions in 4 patients and right hemispheric involvement in 2. Although lesion extension differed, the insular cortex was invariably damaged in all 6 patients. These findings suggest that insular damage may play a critical role in the development of the syndrome by interrupting connections between sensory cortices and the limbic system.
Clinically diagnosed Alzheimer's disease: Autopsy results in 150 cases
Authors: Joachim C. L. , Morris J. H. , Selkoe D. J. 
Published: January 2005

AbstractOne hundred fifty autopsy brains from patients with clinically diagnosed Alzheimer's disease (AD) were examined pathologically. The brains were received consecutively over a 3‐year period from numerous sources as part of research program in which one brain half was frozen for biochemical studies and the other half was fixed in formalin One hundred thirty‐one (87%) of the 150 cases fulfilled histological criteria for AD, with or without additional findings, such as Parkinson's disease or stroke. At least a minimal degree of amyloid angiopathy was found in every brain showing histopathological abnormalities of AD. Twenty‐three (18%) of the 131 AD brains had Lewy bodies in neurons of the substantia nigra. Thirteen of the 19 non‐AD cases were diagnosed as other neurodegenerative disorders. In only 2 cases was no histological correlate for the patient's dementia found. We conclude that (1) the many physicians who diagnosed these cases did so highly accurately; (2) degenerative changes in the substantia nigra were more common in patients with AD than has been reported for the general aged population; (3) amyloid angiopathy was a constant accompaniment of AD, although its severity varied widely; (4) vascular dementia was rarely clinically misdiagnosed as AD; (5) neuropathological findings were insufficient to account for the clinical syndrome of dementia in less than 2% of cases; (6) the histological criteria established by the National Institutes of Health/American Association of Retired Persons Research Workshop on the Diagnosis of Alzheimer's Disease worked well in assessing this large series.
A treatable multifocal motor neuropathy with antibodies to GM1 ganglioside
Authors: Pestronk A. , Cornblath D. R. , Ilyas A. A. , Baba H. , Quarles R. H. , Griffin J. W. , Alderson K. , Adams R. N. 
Published: January 2005

AbstractWe report 2 patients with a treatable, immune‐mediated motor polyneuropathy associated with antibodies to defined neural antigens. In these patients asymmetrical weakness developed in one arm and progressed over 2 to 3 years to involve the other arm, legs, and trunk. Both patients were initially diagnosed as having lower motor neuron forms of amyotrophic lateral sclerosis. However, repeated electrophysiological testing eventually showed multifocal conduction blocks in motor but not sensory fibers compatible with patchy selective demyelination. Serum testing by thin‐layer chromatography and enzyme‐linked immunosorbent assay revealed that both patients had high titers of antibody directed against GM1 and other gangliosides. Initial therapeutic trials of prednisone (100 mg daily for 4 to 6 months) and plasmapheresis were unsuccessful. Treatment with cyclophosphamide, however, was followed by Marchked improvement in strength in both patients.
Multiple sclerosis: Involvement of interferons in lesion pathogenesis
Authors: Traugott Ute , Lebon Pierre 
Published: January 2005

AbstractTo investigate a possible role of interferons (IFNs) in lesion pathogenesis, central nervous system tissue from multiple sclerosis patients and control subjects was stained by immunocytochemical techniques in combination with monoclonal antibodies or polyclonal antisera for the demonstration of IFN‐α, IFN‐β, and IFN‐γ. The results were correlated to lesion activity, to the presence of class I and class II major histocompatibility antigen‐positive astrocytes, and to the composition of cellular infiltrates. IFNs were detectable in active but not in inactive chronic multiple sclerosis lesions. In acute multiple sclerosis plaques, all three types of IFN were widely distributed on glial elements and infiltrating cells. In active chronic multiple sclerosis, labeling of cells for IFN‐α, IFN‐β, and IFN‐γ was most pronounced at the lesion edge and displayed distinct distribution patterns. Overall, IFN‐γ was more common than IFN‐α, IFN‐β, and was predominantly found on astrocytes. IFN‐α, was detectable mainly on macrophages. Distribution of IFN‐β, partially overlapped with that of IFN‐γ and IFN‐β, in that it was present on some astrocytes and on some macrophages. IFNs were rare in normal white matter remote from lesions and in the gliotic lesion center. La antigen and human leukocyte antigen ABC on astrocytes were distributed similarly to IFN‐γ and IFN‐β, respectively. These findings indicate that IFN‐γ may play a role in active lesion growth in multiple sclerosis, whereas IFN‐α and IFN‐β may exert some local immunosuppressive effect. IFNs were also detectable in central nervous system tissue from patients with some other neurological diseases but were virtually absent from tissue from normal control subjects.
Inflammatory lesions of peripheral nerve in a patient with human T‐lymphotropic virus type I‐associated myelopathy
Authors: Said Gérard , Goulon‐Goeau Catherine , Lacroix Catherine , Fève Anaic , Descamps Hélène , Fouchard Marchie 
Published: January 2005

AbstractIn a patient with tropical spastic paraparesis associated with a positive titer for human T‐lymphotropic virus type I, electrophysiological study detected a mixed, axonal and demyelinating, multifocal neuropathy. Perineural and perivascular infiltrates, moderate axon loss, wallerian degeneration, and demyelinating lesions of isolated fibers were present in the nerve biopsy specimen. These inflammatory lesions resembled those found in the central nervous system of patients with tropical spastic paraparesis.