bluuebunny/crossref_metadata_2025_split huggingface dataset:
Human antiidiotypic antibody against opiate receptors
Authors: Roy Benjamin F. , Bowen Wayne D. , Frazier James S. , Rose John W. , McFarland Henry F. , McFarlin Dale E. , Murphy Dennis L. , Morihisa John M. 
Published: January 2005

AbstractSera containing antibodies toβ‐endorphin from 2 patients with major depressive disorder were shown to have antiidiotypic antibodies that specifically inhibited reactivity between anti‐β‐endorphin IgG andβ‐endorphin. Autologous and homologous antiidiotypic anti‐anti‐β‐endorphin IgG antibodies were isolated by affinity chromatography. The purified antiidiotypic antibody did not bindβ‐endorphin but competed with [125I]β‐endorphin for rat brain opiate receptors. Normal IgG that was similarly treated had negligible competitive effects. The antibody bound to the membrane preparation; such binding was inhibited by opiate receptor ligands. Binding of the antiidiotype to a 60,000‐dalton protein from rat brain was detected by Western immunoblot analysis. This protein corresponds in molecular weight to proteins proposed to be components of opiate receptors. These findings imply that immune reactivity to neuropeptides could contribute to psychiatric impairment.
Expression of nerve growth factor receptor in human peripheral neuropathies
Authors: Sobue Gen , Yasuda Takeshi , Mitsuma Terunori , Ross Alonzo H. , Pleasure David 
Published: January 2005

AbstractNerve growth factor (NGF) receptors in human sural nerve biopsies were detected immunohistochemically using a monoclonal anti‐human NGF receptor antibody. NGF receptors were not visualized within the endoneurium of normal adult nerves but were readily demonstrable within the endoneurium of nerves undergoing active axonal degeneration. Immunostaining of adjacent sections for S‐100β protein, a specific Schwann cell Marchker, suggested that Schwann cells expressed the NGF receptors induced by axonal degeneration. Residual Schwann cells in nerves in which axons were completely depleted also expressed NGF receptors. NGF receptors were not detected in relation to thinly myelinated regenerating axons, nor were they detected in the endoneurium of nerves that had undergone segmental demyelination and remyelination. The increased expression of NGF receptors in human axonal neuropathies may contribute to regeneration by NGF‐responsive neurons in these disorders.
Two cases of Wernicke's encephalopathy in children: An underdiagnosed complication of poor nutrition
Authors: Seear Michael D. , Norman Marchgaret G. 
Published: January 2005

AbstractWe describe the occurrence of Wernicke's encephalopathy in two children in whom the diagnosis was not suspected during life. Postmortem examination showed changes characteristic of the disease. This preventable and treatable condition is probably underdiagnosed in poorly nourished, nonalcoholic patients of all ages.
Recall and recognition memory in patients with Alzheimer's and Parkinson's diseases
Authors: Helkala Eeva‐Liisa , Laulumaa Veikko , Soininen Hilkka , Riekkinen Paavo J. 
Published: January 2005

AbstractAlzheimer's and Parkinson's diseases are associated with cognitive impairment, although the pattern of cognitive dysfunction is not identical. We investigated the recall and recognition memory of 18 patients with idiopathic Parkinson's disease (PD) and of 32 patients with probable Alzheimer' disease (AD). In the list‐learning test using Buschke's selective reminding method, PD patients showed superior delayed recognition memory capability compared to AD patients, whereas immediate recall did not differ in the two groups. In the delayed story recall, PD patients were also able to benefit from rehearsal and probe more than AD patients. The results suggest that PD patients are able to bind information better into long‐term storage than are AD patients. This may be due to better function in PD of the entorhinal cortex and hippocampus.
Loss of evoked potentials during spinal surgery due to spinal cord hemorrhage
Authors: Shukla R. , Docherty T. B. , Jackson R. K. , Weller R. O. , Sedgwick E. M. 
Published: January 2005

AbstractThe cortical somatosensory evoked potential (September) disappeared during corrective spinal surgery in a patient with muscular dystrophy. The patient died 18 hours after surgery. Autopsy revealed an intramedullary hemorrhage 4 mm in diameter in the posterior horn of the cervical spinal cord. Microscopically, hypoxic neurons were seen adjacent to the hemorrhagic area, implying that the lesion was at least 6 hours old. The hemorrhage corresponded to the loss of Septembers and confirms that spinal cord monitoring can detect such lesions.
Cerebrospinal fluid homovanillic acid and parkinsonism in Huntington's disease
Authors: Kurlan Roger , Goldblatt David , Zaczek Robert , Jeffries Keith , Irvine Carrie , Coyle Joseptemberh , Shoulson Ira 
Published: January 2005

AbstractIn Huntington's disease (HD), normal or Decemberreased levels of homovanillic acid (HVA) in cerebrospinal fluid (CSF) have been reported but have not been analyzed with respect to severity of parkinsonism, which in certain cases may be a predominant feature of the illness. We obtained CSF by lumbar puncture from four groups of nonmedicated subjects: (1) those with HD in the earliest stages of illness (n = 51), (2) those with parkinsonism, including idiopathic (n = 10) and atypical forms (n = 4), (3) those with nonparkinsonian movement disorders (n = 19), and (4) normal volunteers (n = 4). HVA was determined by high‐pressure liquid chromatography with electrochemical detection, and motor signs were assessed in standardized fashion. The parkinsonian group had reduced levels of CSF HVA, but the other groups showed no significant differences. For those with HD, no correlation was found between HVA level and severity of parkinsonism, and there were no differences in HVA level between those subjects with (n = 14) of without (n = 37) prominent parkinsonism or between subjects whose age at illness onset was 30 years or less (n = 16) and those whose age at onset was over 30 (n = 35). Our findings indicate that in early, untreated HD, CSF HVA is in the normal range and does not correlate with the severity of parkinsonism. This observation supports neuropathological findings suggesting that parkinsonian features in HD are largely related to the loss of postsynaptic striatal dopamine receptors rather than to presynaptic nigral degeneration.
The neuroexcitotoxic amino acids glutamate and aspartate are altered in the spinal cord and brain in amyotrophic lateral sclerosis
Authors: Plaitakis Andreas , Constantakakis Evangelos , Smith Jeffrey 
Published: January 2005

AbstractBecause recent studies showed a systemic defect in glutamate metabolism in amyotrophic lateral sclerosis (ALS), we measured the levels of free amino acids in frontal and cerebellar cortex and two areas of spinal cord obtained at autopsy from 22 patients who died of this disease. Glutamate levels were significantly Decemberreased (by 21 to 40% of control values) in all areas investigated; cervical and lumbar spinal cord showed the greatest change. Aspartate levels were also significantly reduced (by 32 to 35%) in the spinal cord only. A positive correlation was shown between the changes of glutamate and aspartate as well as a significant alteration in the glutamate to glutamine ratio in the spinal cord of patients with ALS. Although we cannot exclude the possibility that these abnormalities may partly result from neuronal cell loss, the data suggest the presence of a generalized defect that may affect the neurotransmitter and metabolic pool of glutamate. The defect may be expressed more severely in the spinal cord than in other central nervous system areas. These results, taken together with the previously shown systemic abnormality, raise the possibility that distribution of glutamate between the intracellular and extracellular pool may be altered in ALS and may mediate the neurodegeneration.
Encephalitis among cree children in northern Quebec
Authors: Black Deborah N. , Watters Gordon V. , Andermann Eva , Dumont Charles , Kabay Michel E. , Kaplan Paige , Meagher‐Villemure Kathleen , Michaud Jean , O'gorman Gus , Reece Elena , Tsoukas Christos , Wainberg Marchk A. 
Published: January 2005

AbstractWe report a neurological disease among Cree Indian children in a northern Quebec village. The disease manifests as severe mental retardation, cerebral atrophy with white matter changes and calcifications, and systemic immunological abnormalities. Eleven cases are known in five families. The familial incidence of cases and the high degree of parental consanguinity suggest a genetic contribution. We propose that this entity may be caused by an unusual viral infection in a genetically vulnerable host.
Ataxia—ocular motor aprilaxia: A syndrome mimicking ataxia‐telangiectasia
Authors: Aicardi Jean , Barbosa Célia , Andermann Eva , Andermann Frederick , Morcos Rami , Ghanem Qais , Fukuyama Yukio , Awaya Yutaka , Moe Paul 
Published: January 2005

AbstractWe report 14 patients with a slowly progressive syndrome featuring ataxia, choreoathetosis, and ocular motor Aprilaxia in both the horizontal and vertical planes. Although the neurological signs were indistinguishable from those of ataxia‐telangiectasia, the onset tended to be later and none of the patients had evidence of multisystemic involvement. Specifically, there was no tendency to frequent infections, and immunoglobulins, α‐fetoprotein, T‐ and B‐lymphocyte Marchkers, and chromosomes 7 and 14 were normal in all tested patients. The simultaneous absence of telangiectasias and of other nonneurological manifestations made ataxia‐telangiectasia an unlikely diagnosis. We suggest that these patients suffer from an unusual type of spinocerebellar degeneration. This syndrome has been observed in different populations from three continents, with a genetic pattern suggesting recessive autosomal inheritance.
The glutamate antagonist MK‐801 reduces focal ischemic brain damage in the rat
Authors: Park C. K. , Nehls D. G. , Graham D. I. , Teasdale G. M. , McCulloch J. 
Published: January 2005

AbstractExcessive activation of the N‐methyl‐D‐aspartate (NMDA) subtype of glutamate receptor has been implicated in the sequence of neurochemical events that results in irreversible neuronal damage in cerebral ischemia. The effects of the NMDA antagonist (+) ‐5‐methyl‐10,ll‐dihydro‐5H‐dibenzo[a,d]cyclohepten‐5,10‐imine maleate (MK‐801) upon the amount of ischemic brain damage has been assessed quantitatively in the lightly anesthetized rat. Focal cerebral ischemia was produced by the permanent occlusion of one middle cerebral artery (MCA), and the animals were killed 3 hours after the arterial occlusion. MK‐801 (0.5 mg/kg) was administered intravenously either 30 minutes prior to MCA occlusion or 30 minutes after the induction of ischemia. Pretreatment with MK‐801 reduced the volume of ischemic damage both in the cerebral cortex (by 38% compared with untreated rats with MCA occlusion; p < 0.01) and in the caudate nucleus (by 18% compared with controls; p < 0.05). Treatment with MK‐801, initiated 30 minutes after MCA occlusion, reduced the volume of ischemic damage in the cerebral cortex (by 52% compared with controls; p < 0.01). The volume of ischemic damage in the caudate nucleus was minimally influenced by MK‐801 treatment initiated after MCA occlusion. The antiischemic effects of MK‐801 were readily demonstrable despite the hypotension that MK‐801 induced in rats anesthetized with halothane (0.5%), nitrous oxide (70%), and oxygen (30%). The potency of MK‐801 in reducing ischemic brain damage, even when administered after the induction of ischemia, highlights the potential use of NMDA receptor antagonists for the treatment of focal cerebral ischemia in humans.
Absence status epilepticus with computed tomographic brain changes following metrizamide myelography
Authors: Obeid Tahir , Yaqub Basim , Panayiotopoulos Chrysostomos , Al‐Jasser Saleh , Shabaan Azzam , Hawass Nour El‐Din 
Published: January 2005

AbstractAbsence status epilepticus following metrizamide myelography was associated with computed tomographic scan evidence of a high concentration of the dye in brain gray matter. We suggest that absence status epilepticus is due to the direct effect of metrizamide on the cortex and that this clinically treatable condition may have escaped diagnosis in previous reports.
Dementia, parkinsonism, and motor neuron disease: Neurochemical and neuropathological correlates
Authors: Gilbert Joseptemberh J. , Kish Stephen J. , Chang Li‐Jan , Morito Caryl , Shannak Kathleen , Hornykiewicz Oleh 
Published: January 2005

AbstractThe neurochemical Marchkers for the major neurotransmitter systems were measured in the brain of a patient who died with a dementia‐parkinsonism‐motor neuron disease (DPMN) syndrome complex. Moderate neuronal loss in the substantia nigra, spongiform changes in the frontal cortex, and moderate anterior horn cell loss throughout the spinal cord were observed. A severe nigrostriatal dopamine deficiency provides the basis for the observed parkinsonian features. The dementia is unexplained.
Magnetic resonance imaging–based brain morphometry: Development and application to normal subjects
Authors: Filipek Pauline A. , Kennedy David N. , Caviness Verne S. , Rossnick Susan L. , Spraggins Thomas A. , Starewicz Piotr M. 
Published: January 2005

AbstractA semiautomated computerized method of in vivo morphometric analysis that is based upon high‐resolution three‐dimensional magnetic resonance imaging has been developed. This morphometric method is efficient and is of greater analytical precision than any other morphometric method currently applied to living human tissue. Including error inherent in image data acquisition, the aggregate error of the methodology, as estimated by the phantom studies, ranges from 4.5 to 9.6%, with incremental error above 4.5% a function of magnetic resonance slice thickness. This method was applied to magnetic resonance scans of 7 normal volunteers. The derived volumes of whole brain and of individual substructures were closely concordant with previously published volumes of normal fresh (unfixed) brains obtained post mortem. This morphometric methodology is potentially applicable to any structure or lesion that can be visualized by magnetic resonance imaging.
Hypoxia‐sensitive hyperexcitability of the intramuscular nerve axons in isaacs' syndrome
Authors: Oda Kenichiro , Fukushima Noriko , Shibasaki Hiroshi , Ohnishi Akio 
Published: January 2005

AbstractA case of Isaacs' syndrome with Trousseau's phenomenon is reported. Myokymia, pseudomyotonia (difficulty relaxing after forceful contraction), and ischemia‐induced carpal spasm (Trousseau's phenomenon) were not abolished by nerve block distal to the cuff or by intravenous infusion of calcium. Inhalation of oxygen suppressed the pseudomyotonia and Trousseau's phenomenon, but myokymia persisted. Phenytoin abolished all types of abnormal discharges. The morphological abnormality in biopsied short peroneal muscle consisted of intraterminal and ultraterminal sprouting. We postulate that the trigger zone for abnormal discharge in our case is in the distal segment of the intramuscular nerve axon, including the nerve terminal. Hypoxia‐sensitive hyperexcitability of the axon membrane might be responsible for the generation of pseudomyotonia and Trousseau's phenomenon, although the mechanism underlying myokymia remains unknown.
A controlled clinical trial of baclofen as protective therapy in early huntington's disease
Authors: Shoulson Ira , Odoroff Charles , Oakes David , Behr Jill , Goldblatt David , Caine Eric , Kennedy Judith , Miller Charlyne , Bamford Kathryn , Rubin Allen , Plumb Sandra , Kurlan Roger 
Published: January 2005

AbstractWe carried out a controlled clinical trial to examine the potential of baclofen to slow the functional decline of patients with early Huntington's disease (HD). The basis of the trial was: (1) the hypothesis that excitatory amino acid neurotransmission mediates the neuronal degeneration of HD, (2) preclinical evidence that baclofen retards corticostriatal release of glutamate and aspartate, and (3) reports that baclofen produces short‐term clinical benefits in some HD patients. Sixty patients with early HD were randomized to chronic baclofen, 60 mg/day, or placebo treatments and followed systematically for up to 42 months. Total functional capacity was not favorably influenced by baclofen treatment. Factors that contributed, although nonsignificantly, to a more rapid rate of total functional capacity decline included younger age (< 35 years), earlier stage (stage I) of illness, paternal inheritance of the HD gene, and baclofen treatment. Our patients declined at a pace slower than that observed in other prospective studies, a finding likely due to selection criteria, avoidance of neuroleptic therapy, and strong psychosocial support.
Inclusion body myositis: The mumps virus hypothesis
Authors: Nishino Hiroshi , Engel Andrew G. , Rima Burt K. 
Published: January 2005

AbstractThe resemblance of the filamentous inclusions in inclusion body myositis (IBM) to mumps virus nucleoproteins and the report of immunoreactivity of the inclusions for mumps virus antigens have implicated the mumps virus in the etiology of IBM. We tested the mumps virus hypothesis by in‐situ hybridization with a cDNA probe specific for the mumps virus nucleocapsid gene, and immunocytochemically with antibodies against “soluble” and “viral” mumps antigens. The tests were performed on muscle specimens (IBM, 20; acid maltase deficiency, 4; chloroquine myopathy, 2; nonweak control subjects, 5) and mumps virus–infected and uninfected HEp‐2 cells. The in‐situ hybridization study showed a strong specific signal in the infected HEp‐2 cells but no specific signal in IBM, other myopathies, or nonweak control subjects. The immunocytochemical study showed specific binding of the antimumps antibodies to the infected HEp‐2 cells but demonstrated only nonspecific binding of these antibodies around rimmed vacuoles in IBM, acid maltase deficiency, and chloroquine myopathy. These studies cast doubt on the mumps hypothesis of IBM.
High‐dose leucovorin reverses acute high‐dose methotrexate neurotoxicity in the rat
Authors: Phillips Peter C. , Thaler H. Tzvi , Allen Jeffrey C. , Rottenberg David A. 
Published: January 2005

AbstractIntravenous high‐dose methotrexate (HD‐MTX) reduces cerebral glucose metabolism and produces behavioral abnormalities and electroencephalographic slowing in an animal model of acute HD‐MTX neurotoxicity and in cancer patients undergoing HD‐MTX chemotherapy. We used our model of HD‐MTX neurotoxicity in the rat to determine if leucovorin (5‐formyltetrahydrofolate) reduces this neurotoxicity, and extended our characterization of this model to identify regional as well as global HD‐MTX treatment effects and to investigate HD‐MTX‐induced alterations in regional brain pH. Intravenous high‐dose leucovorin reversed the HD‐MTX‐induced decrease in cerebral glucose metabolism and associated behavioral and electroencephalographic abnormalities in the rat, but low‐dose leucovorin was ineffective. The major effect of HD‐MTX on cerebral glucose metabolism was a global reduction; however, smaller region‐specific treatment effects were identified in auditory, thalamic, and white matter structures. HD‐MTX did not alter regiona brain pH. These findings suggest a potential clinical role for high‐dose leucovorin in severe or prolonged acute HD‐MTX neurotoxicity and provide an important justification for the role of positron emission tomography in the early detection of clinical HD‐MTX neurotoxicity.
N‐methyl‐D‐aspartate antagonists: Ready for clinical trial in brain ischemia?
Authors: Albers Gregory W. , Goldberg Mark P. , Choi Dennis W. 
Published: January 2005

AbstractAntagonists of the N‐methyl‐D‐aspartate (NMDA) subclass of glutamate receptors may offer a new approach for the treatment of ischemic brain injury. This strategy is supported by a well‐developed scientific foundation and encouraging results in a variety of in vivo and in vitro experimental models. Several specific antagonists, including MK‐801, dextrorphan, dextromethorphan, and ketamine, have already been used at low doses in humans for other indications and are potential candidates for Phase I clinical trials.
Quantitation of cerebral atrophy in preclinical and end‐stage alzheimer's disease
Authors: de la Monte Suzanne M. 
Published: January 2005

AbstractMorphometric analysis of standardized gross cerebral slices from 16 patients with end‐stage Alzheimer's disease (AD), 14 controls without neuropathological lesions or neurological disease, and 4 neurologically intact nondemented patients with histopathological lesions of AD was used to measure cross‐sectional areas of cerebral cortex, white matter, subcortical nuclei, and the ventricular system. In AD, there was global cerebral atrophy of both cortex and white matter, selective atrophy of the amygdala and hippocampus, and ex vacuo hydrocephalus. In addition, in half the cases of AD, white matter atrophy was associated with overt histopathological evidence of patchy rarefaction of fibers and gliosis. Patients with preclinical AD had prominent and selective shrinkage of white matter comparable to that observed in AD, yet their cortical areas were normal. These observations suggest that white matter degeneration is an intrinsic component of AD. Moreover, its presence in preclinical AD where cortical atrophy is not evident indicates that cytoskeletal abnormalities associated with axonal degeneration may precede and perhaps cause the cortical atrophy observed in clinically manifested AD.
Normalization of short‐chain acylcoenzyme a dehydrogenase after riboflavin treatment in a girl with multiple acylcoenzyme a dehydrogenase—deficient myopathy
Authors: Didonato Stefano , Gellera Cinzia , Peluchetti Dionisio , Uziel Graziella , Antonelli Antonella , Lus Giacomo , Rimoldi Marco 
Published: January 2005

AbstractA 12‐year‐old girl was shown to have carnitine‐deficient lipid storage myopathy and organic aciduria compatible with multiple acylcoenzyme A (acyl‐CoA) dehydrogenase deficiency. In muscle mitochondria, activities of both short‐chain acyl‐CoA dehydrogenase (SCAD) and medium‐chain acyl‐CoA dehydrogenase (MCAD) were 35% of normal. Antibodies against purified SCAD, MCAD, and electron‐transfer flavoprotein were used for detection of cross‐reacting material (CRM) in the patient's mitochondria. Western blot analysis showed absence of SCAD‐CRM, reduced amounts of MCAD‐CRM, and normal amounts of electron‐transfer flavoprotein‐CRM. The patient, who was unresponsive to treatment with oral carnitine, improved dramatically with daily administration of 100 mg oral riboflavin. Increase in muscle bulk and strength and resolution of the organic aciduria were associated with normalization of SCAD activity and “reappearance” of SCAD‐CRM. In contrast, both MCAD activity and MCAD‐CRM remained lower than normal. These results suggest that in some patients with multiple acyl‐CoA dehydrogenase deficiency riboflavin supplementation may be effective in restoring the activity of SCAD, and possibly of other mitochondrial flavin‐dependent enzymes.