bluuebunny/crossref_metadata_2025_split huggingface dataset:
Tropical spastic paraparesis: A model of virus‐induced, cytotoxic T‐cell–mediated demyelination?
Authors: Moore G. R. Wayne , Traugott Ute , Scheinberg Labe C. , Raine Cedric S. 
Published: January 2005

AbstractTropical spastic paraparesis is a neurological disorder that is most commonly seen in certain tropical (mainly Caribbean) areas and that presents as a progressive spastic paraparesis and urinary dysfunction. Recent studies have revealed an association between tropical spastic paraparesis and human T‐cell lymphotropic virus type I (HTLV‐I) infection. We report the results of a detailed morphological and immunocytochemical study of a patient with tropical spastic paraparesis. Lesions were restricted to the spinal cord and optic nerve, where demyelination, inflammation, and fiber loss were common features. Lymphocytes were seen closely applied to nerve fibers within which were changes resembling those seen in myelinated central nervous system cultures exposed to cytokines. Immunocyctochemically, HTLV‐I p19 core protein and a predominance of CD8+ (suppressor/cytotoxic) T cells and expression of class I major histocompatibility antigen were demonstrated in spinal cord lesions. It is postulated that cytotoxic T cells, either directly or via cytokines, induce lysis of the myelin sheath and subsequently the axon, resulting in a mixed picture of demyelination and axonal loss with secondary tractal degeneration. Despite this destruction, extensive remyelination was evident within affected areas of spinal cord.
Tumor necrosis factor in serum and cerebrospinal fluid of patients with multiple sclerosis
Authors: Franciotta D. M. , Grimaldi L. M. E. , Martino G. V. , Piccolo G. , Bergamaschi R. , Citterio A. , D'Eril G. V. Melzi 
Published: January 2005

AbstractWe measured levels of alpha–tumor necrosis factor (α‐TNF) in cerebrospinal fluid and serum samples from 50 drug‐free patients with multiple sclerosis, 25 patients with other neurological diseases, 27 patients with non‐neurological diseases, and 10 normal subjects. The most elevated levels of α‐TNF were found in patients with inflammatory or autoimmune diseases. Comparable serum levels of α‐TNF were detected in normal control subjects, patients with multiple sclerosis, and patients with degenerative neurological diseases. In patients with multiple sclerosis, α‐TNF levels were also unrelated to time elapsed between the occurrence of clinical exacerbation and the time of sample collection. Only 3 patients with chronic progressive multiple sclerosis had detectable α‐TNF in the cerebrospinal fluid. Our data do not support a role for elevated levels of circulating α‐TNF in the maintenance of the disease. However, we cannot rule out the possibility that a transient elevation of a α‐TNF triggers the cellular events leading to demyelination in multiple sclerosis.
Modification of central dopaminergic mechanisms by continuous levodopa therapy for advanced Parkinson's disease
Authors: Mouradian M. Maral , Heuser Isabella J. E. , Baronti Fabio , Chase Thomas N. 
Published: January 2005

AbstractThe management of fluctuations in motor function complicating advanced Parkinson's disease with continuously administered dopaminomimetics was studied in 12 patients. In response to 7 to 12 days of round‐the‐clock intravenous infusions of levodopa, fluctuations in motor performance gradually diminished, ultimately by more than 40°. The beneficial effect persisted for about 6 days after withdrawal of continuous parenteral treatment and resumption of standard oral therapy. Clinical improvement was associated with changes in several pharmacological indices: Acute dose‐response studies of intravenous levodopa showed a shift of the curve to the right in the immediate postinfusion phase compared to preinfusion studies; the therapeutic index improved significantly as patients demonstrated about 76° increased beneficial antiparkinsonian response with an equal degree of toxic dyskinetic effects; and the duration of action levodopa was prolonged by 30°. These results suggest that changes in central dopaminergic mechanisms contributing to motor complications in advanced Parkinson's disease can be modified by procedures that provide continuous dopamine replacement. Presumably these modifications underlie the gradual amelioration of motor fluctuations over several days of round‐the‐clock therapy. Results of the present study also suggest potential deleterious effects of chronic intermittent oral treatment in the development of motor complications and thus support the role of long‐term, continuous administration of dopaminomimetics.
Inhibition of mononuclear phagocytes reduces ischemic injury in the spinal cord
Authors: Giulian Dana , Robertson Claudia 
Published: January 2005

AbstractTransient ischemia to the lumbar and sacral sections of the spinal cord of rabbit leads to a deterioration in neurological function that usually worsens 24 to 48 hours after injury. This decline in movement of the hindlimbs develops simultaneously with the appearance of mononuclear phagocytes in the gray matter of injured neural tissue. Chloroquine and colchicine inhibit phagocytic and secretory functions in mononuclear phagocytes. When given up to 6 hours after an induced ischemic lesion in rabbit, this drug combination decreased the number of mononuclear phagocytes found within the gray matter of damaged spinal cord, improved the recovery of function of the hindlimbs and bladder, preserved spinal somatosensory evoked potentials, and promoted the survival of motor neurons. In contrast, the glucocorticoid dexamethasone, a weak inhibitor of mononuclear phagocytes in vivo, did not reduce the number of inflammatory cells in the spinal cord and did not improve motor and bladder functions. The suppression of mononuclear phagocytes soon after ischemic injury may offer a new approach in the treatment of vascular disease in the central nervous system.
Antimyelin basic protein and antimyelin antibody‐producing cells in multiple sclerosis
Authors: Olsson Tomas , Baig Shahid , Jeberg Bo Hö , Link Hans 
Published: January 2005

AbstractThe B‐Cell response to myelin and myelin basic protein was studied in patients with multiple sclerosis and in patients with acute aseptic meningoencephalitis by using a nitrocellulose immunospot assay. This method allows detection of single cells producing antibodies. Twenty‐seven (79%) of 34 patients with multiple sclerosis had cells producing IgG antibodies against myelin, and 11 (57%) of 19 had cells producing IgG antibodies against myelin basic protein in cerebrospinal fluid (CSF), with mean values of 30 and 14 per 104 mononuclear cells, respectively. Total numbers of IgG‐producing cells occurred at a mean number of 75 per 104 CSF Cells. Cells producing antimyelin or anti–myelin basic protein antibodies of IgA or IgM isotypes were rarely found in CSF. Patients with acute aseptic meningoencephalitis less frequently showed CSF cells producing IgG antibodies against myelin and myelin basic protein. No cells producing antibodies against myelin or myelin basic protein were detected in peripheral blood of patients with multiple sclerosis or meningoencephalitis. Thus, a majority of patients with multiple sclerosis had CSF cells that produced IgG antibodies against myelin and myelin basic protein. These cells comprised a large proportion of the total IgG‐producing cells. A pronounced B‐cell response against autoantigens produced at the target for immune attack might be important in the pathogenesis of multiple sclerosis.
Immunocytochemical and ultrastructural studies of lower motor neurons in amyotrophic lateral sclerosis
Authors: Murayama Shigeo , Mori Hiroshi , Ihara Yasuo , Bouldin Thomas W. , Suzuki Kinuko , Tomonaga Masanori 
Published: January 2005

AbstractNeuronal inclusions in lower motor neurons in 23 cases of adult‐onset sporadic amyotrophic lateral sclerosis were studied immunocytochemically and ultrastructurally. Monoclonal and polyclonal antiubiquitin antibodies recognized four structures in the neuronal perikarya: (1) all Lewy body–like inclusions in 6 cases with a relatively short clinical course, (2) a small percentage of Bunina bodies in 4 cases with abundant Bunina bodies, (3) ill‐defined structures closely associated with Bunina bodies (Bunina body–related structures) in 15 cases, and (4) a focally aggregated meshwork of fine filamentous structures not associated with Bunina bodies in all cases. These four structures were not recognized by the antibodies raised against cytoskeletal proteins (neurofilament, tubulin, microtubule‐associated protein 2, and phosphorylated tau). Electron microscopy revealed Lewy body–like inclusions to be accumulations of randomly oriented filaments, approximately 15 nm in diameter, covered by fine granules. Bundles of coated filaments 12 nm in diameter that sometimes formed Bunina body–like structures were also observed in the perikarya. Immunoelectron microscopy showed the reaction product with antiubiquitin to be on the filaments, 15 nm in diameter, of Lewy body–like inclusions. Our study revealed the existence of two types of filaments in lower motor neurons of patients with amyotrophic lateral sclerosis: (1) ubiquitin‐positive, granule‐associated filaments, approximately 15 nm in diameter, that form Lewy body–like inclusions; and (2) 12 nm coated filaments that may be a candidate for another ubiquitin‐positive structure and possibly a precursor of Bunina bodies. These two types of filaments may represent early pathological changes of lower motor neurons in amyotrophic lateral sclerosis.
A disorder of axonal development, necrotizing myopathy, cardiomyopathy, and cataracts: A new familial disease
Authors: Lyon Gilles , Arita Fernando , Le Galloudec Eric , Vallée Louis , Mission Jean‐Paul , Ferrière Gérard 
Published: January 2005

AbstractWe report severe congenital encephalopathy and profound hypotonia associated with necrotizing myopathy, cardiomyopathy, and cataracts in 3 infants, including 2 sisters. Brain scans suggested agenesis of the corpus callosum. Neuropathological findings consisted of severe atrophy of the corpus callosum (not the usual agenesis with longitudinal callosal bundles), atrophy of the white matter, and absence of pyramidal tracts in the medulla. Multiple axonal swellings were present in the white matter and in Purkinje Cells. Except for the corpus subthalamicum, gray matter structures were preserved. These findings are considered to be the expression of a primary disorder of axonal development leading to a reduction in interneuronal synaptic contacts. It is suggested that the anomaly may be due to an extension of the normal phenomenon of axonal elimination, related to a primary defect of the axonal cytoskeleton. The concept of a primary axonal disorder may also apply to other, mostly familial, conditions with progressive atrophy of the cerebral white matter and corpus callosum.
Delineation of single‐word semantic comprehension deficits in aphasia, with anatomical correlation
Authors: Hart John , Gordon Barry 
Published: January 2005

AbstractIn 3 of 18 aphasic patients pure deficits in semantic comprehension at the single‐word level were defined through a series of tasks that excluded possible confounding deficits in auditory perception, visual perception, or speech production. In these pure cases, deficits were found at the superordinate, equivalence, and subordinate levels of single‐word semantic processing. Pure semantic deficits were found to be correlated with damage to the left posterior temporal and inferior parietal region; patients whose damage spared this area did not evince such deficits, and the converse was also true. This study confirms the existence of separable deficits in semantic comprehension and points conclusively to the left posterior temporal and inferior parietal region as being critical for semantic processing. This anatomical localization is in keeping with anatomical studies from nonhuman primates, suggesting that these regions may be concerned with multimodal processing and integration of language.
A large kindred with autosomal dominant Parkinson's disease
Authors: Golbe Lawrence I. , Di Iorio Giuseppe , Bonavita Vincenzo , Miller Douglas C. , Duvoisin Roger C. 
Published: January 2005

AbstractWe report two large kindreds with Parkinson's disease (PD) apparently inherited in autosomal dominant fashion. Forty‐one persons in four generations have been affected; we have examined 7 of them. The two kindreds originated in a single small town in southern Italy and therefore are probably related. The illness was typical for PD except for early onset at a mean age of 46.5 years and a rapid course that averaged 9.7 years from onset to death. Clinical appearance and response to levodopa were typical for PD. Only one instance of difinite nonpenetrance is known. Autopsy of 2 patients in one kindred showed the pathological changes typical of PD with Lewy bodies. Disease duration among affected persons who spent most of their lives in Italy was longer than for their affected US relatives, suggesting that exogenous agents may influence the course of this genetic illness. We conclude that what is probably a single gene without an additional environmental insult can cause the pathological changes typical of PD. Our findings therefore enhance the likelihood of significant genetic component in the cause of sporadic PD. By identifying a toxic gene product, future molecular genetic studies in our kindred(s) may provide insight into the pathogenesis of sporadic PD.
Detection of epileptiform activity by different noninvasive EEG methods in complex partial epilepsy
Authors: Goodin Douglas S. , Aminoff Michael J. , Laxer Kenneth D. 
Published: January 2005

AbstractThe diagnostic utility of different noninvasive electrode placements for deriving the electroencephalogram and detecting interictal epileptiform discharges was compared. Anterior temporal and nasopharyngeal electrodes in combination with routine scalp electrodes detected over 97% of the spikes, whereas recording from only standard electrode placements detected 58%. Minisphenoidal and surface sphenoidal electrodes were generally not helpful. In some circumstances, however, the use of surface sphenoidal electrodes provided important confirmatory information. In no case did the minisphenoidal electrodes provide unique information, and their use seems unjustified, although the inclusion of other nonstandard electrodes in the recording montage is important to increase the yield.
Immunohistochemical study of the striatal efferents and nigral dopaminergic neurons in parkinsonism‐dementia complex on guam in comparison with those in Parkinson's and Alzheimer's diseases
Authors: Goto Satoshi , Hirano Asao , Matsumoto Sadayuki 
Published: January 2005

AbstractA comparative topographical immunohistochemical analysis was performed on the basal ganglia (including the substantia nigra) in Guamanian parkinsonism‐dementia complex, idiopathic Parkinson's disease (PD), and Alzheimer's disease (AD). The striatal projection neurons and their efferent fibers were examined by using antibodies to calcineurin, methionine‐enkephalin, and substance P. Tyrosine hydroxylase served as a marker for nigrostriatal dopaminergic neurons. The basal ganglia of patients with parkinsonism‐dementia complex reacted strongly with all of the antibodies and the reaction products exhibited a normal distribution pattern. These findings suggest that the striatal output system is well preserved in patients with this disease. Similar results were obtained in patients with AD or PD. However, as compared to the patients with AD or PD, patients with parkinsonism‐dementia complex showed severe reduction (> 90%) in the number of dopaminergic neurons in both the lateral and the medial portions of the substantia nigra. In view of the functional cortico‐subcortical loops, these findings could explain the parkinsonian features and in part the cognitive impairment that occur in parkinsonism‐dementia complex on Guam.
Successful chemotherapy for newly diagnosed aggressive oligodendroglioma
Authors: Macdonald David R. , Gaspar Laurie E. , Cairncross J. Gregory 
Published: January 2005

AbstractWe treated 3 patients with newly diagnosed aggressive oligodendroglioma with chemotherapy prior to radiotherapy and observed responses. All had residual or progressive tumor following initial surgery. We used a combination called PCV‐3 because recurrent anaplastic oligodendroglioma responds predictably to this regimen. Chemotherapy for response induction followed by radiotherapy for consolidation is feasible and effective initial treatment for this tumor.
Dominantly transmitted congenital indifference to pain
Authors: Landrieu P. , Said G. , Allaire C. 
Published: January 2005

AbstractTwo patients from a family with dominantly inherited indifference to pain were investigated. Perception of the other sensory modalities was normal as was the remainder of the neurological examination. Electrophysiological studies and morphometric evaluation of myelinated and unmyelinated fibers of nerve biopsy specimens were normal. This is the first morphometric study of peripheral nerve in dominantly inherited indifference to pain.
Cholinergic deficiency and frontal dysfunction in Parkinson's disease
Authors: Dubois Bruno , Pillon Bernard , Lhermitte Françlois , Agid Yves 
Published: January 2005

AbstractTo investigate the influence of Central cholinergic deficit on cognitive function in Parkinson's disease (PD), we compared the neuropsychological performance of a group of 20 patients who were treated with anticholinergic drugs (mean daily dose, 10.2mg) with that of a group of 20 patients who received no anticholinergics. The two groups were matched for all the variables of parkinsonism and levodopa therapy. At the dose used, there was no significant difference between the two groups of patients for intellectual, visuospatial, instrumental, and memory function. In contrast, in the group that received anticholinergics severe impairment was observed on tests believed to assess frontal lobe function. These results suggest that the lesion of the ascending cholinergic neurons, which has been demonstrated post mortem in PD, may play a role in the subcorticofrontal behavioral impairment of this disease.
Relation of hyperglycemia early in ischemic brain infarction to cerebral anatomy, metabolism, and clinical outcome
Authors: Kushner Michael , Nencini Patrizia , Reivich Martin , Rango Mario , Jamieson Dara , Fazekas Franz , Zimmerman Robert , Chawluk John , Alavi Abass , Alves Wayne 
Published: January 2005

AbstractWe studied the relation of serum glucose level measured in the first 12 hours of symptoms to the clinical findings, results of computed tomography (CT), and patterns of cerebral metabolism in 39 patients who had acute ischemic cerebral infarction. Structural damage was assessed by CT. Metabolic disruption was assessed using 18F‐fluorodeoxyglucose and positron emission tomography (PET). Median initial serum glucose concentration was 155 mg/dl (6.7 mM). Clinical recovery was significantly poorer in patients with initial serum glucose levels higher than the median (p < 0.05, chi square). PET tended to show normal results or minor abnormalities in patients with initial glucose levels less than the median, as opposed to lobar or multilobe abnormalities in patients with levels that were higher than the median (p < 0.05, Kendall's Tau b). The severity of hypometabolism in the ischemic region, expressed as the percent asymmetry of local cerebral glucose metabolism between homologous brain regions, was greater in patients with initial glycemia concentrations higher than the median (p < 0.001, t test). Relationships of serum glucose level with metabolic derangement and structural damage, but not outcome, held true in patients without a history of diabetes mellitus.
Polyneuropathy, ophthalmoplegia, leukoencephalopathy, and intestinal pseudo‐obstruction: POLIP syndrome
Authors: Simon Lynn T. , Horoupian Dikran S. , Dorfman Leslie J. , Marks Michael , Herrick Maie Kaarsoo , Wasserstein Philip , Smith Marion E. 
Published: January 2005

AbstractWe describe 5 individuals (from three separate families) with a progressive neurological disorder characterized by sensorimotor peripheral polyneuropathy, cranial neuropathies (external opthalmoplegia, deafness), and the syndrome of chronic intestinal pseudo‐obstruction. Magnetic resonance imaging showed widespread abnormality of the cerebral and cerebellar white matter in the 2 patients studied. Autopsy examination in 3 revealed widespread endoneurial fibrosis and demyelination in the peripheral nervous system, possibly secondary to axonal atrophy, and poorly defined changes in cerebral white matter (leukoencephalopathy). The cranial nerves and spinal roots were less severely involved and the neurons in the brainstem and spinal cord were intact. The fatal gastrointestinal dysmotility was due to a severe visceral neuropathy. We suggest that these patients manifested a hereditary disorder with distinctive clinical, radiological, and neuropathological features, and propose the acronym POLIP to emphasize the distinctive tetrad of polyneuropathy, opthalmoplegia, leukoencephalopathy, and intestinal pseudo‐obstruction.
Synaptic transmission in human neocortex removed for treatment of intractable epilepsy in children
Authors: Wuarin Jean‐Pierre , Kim Yang In , Cepeda Carlos , Tasker Jeffrey G. , Walsh John P. , Peacock Warwick J. , Buchwald Nathaniel A. , Dudek F. Edward 
Published: January 2005

AbstractSynaptic transmission to pyramidal cells was studied in slices of neocortex resected from infants and children (n = 10, age 8 months to 13 years) undergoing surgical treatment for intractable epilepsy. Most specimens were from the least abnormal area of the resection. Stable intracellular recordings could be obtained for up to 8 hours. Most of the recorded neurons had electrophysiological characteristics similar to those of regular‐firing pyramidal cells and were in layers III to V, which was confirmed by intracellular staining with Lucifer yellow. Local extracellular stimulation evoked a sequence of excitatory and inhibitory postsynaptic potentials. Kynurenic acid, a broad‐spectrum excitatory amino acid antagonist, depressed the evoked excitatory postsynaptic potential. After application of the gamma‐aminobutyric acid antagonist, bicuculline (10–30 μM), extracellular stimulation induced large excitatory postsynaptic potentials and epileptiform bursts. Spontaneous bursts occasionally occured in bicuculline. This effect of bicuculline was observed in all the tissue samples, even those from infant patients (n = 4, age 8–16 months). Kynurenic acid depressed or abolished both spontaneous and stimulation‐induced bursts. The competitive antagonist for N‐methyl‐D‐aspartate receptors, DL‐2‐amino‐5‐phosphonopentanoic acid decreased the duration of bicuculline‐induced bursts. These data provide evidence that, similar to rat and cat neocortex, excitatory and inhibitory amino acids are important transmitters to pyramidal cells in immature human neocortex.
Quadriceps myopathy: Forme fruste of Becker muscular dystrophy
Authors: Sunohara Nobuhiko , Arahata Kiichi , Hoffman Eric P. , Yamada Hitoshi , Nishimiya Jin , Arikawa Eri , Kaido Misako , Nonaka Ikuya , Sugita Hideo 
Published: January 2005

AbstractWe examined dystrophin, the protein product of the Duchenne muscular dystrophy gene, in muscle biopsy specimens from 4 male patients with quadriceps myopathy, all of whom showed a mild and slowly progressive myopathy confined to the quadriceps muscles. All 4 patients had clear abnormalities of dystrophin, and were diagnosed as having Becker muscular dystrophy by both immunofluorescence and immunoblot examinations; that is, dystrophin of an abnormal molecular mass was visualized in muscle cryosections as “patchy” or discontinuous immunostaining at the surface membrane of the muscle fibers. One patient had a brother who showed widespread myopathic changes consistent with typical Becker muscular dystrophy. We conclude that the syndrome called quadriceps myopathy includes a group of forme fruste Becker muscular dystrophy.