bluuebunny/crossref_metadata_2025_split huggingface dataset:
Neurological autoimmune disease and the trimolecular complex of T‐lymphocytes
Authors: Hohlfeld Reinhard 
Published: January 2005

AbstractT‐lymphocytes recognize antigen in a trimolecular complex: The T‐cell receptor binds to a processed fragment of antigen that itself is bound to a major histocompatibility complex (MHC) molecule on the surface of an antigen‐presenting cell. The trimolecular complex controls antigen‐specific T‐cell activation in normal and abnormal immune reactions. Recent progress in myasthenia gravis (MG) and experimental autoimmune encephalomyelitis (EAE) exemplifies this, leading to the following conclusions: (1) Autoimmune T cells may act by interfering with immunoregulation (as in MG) or by directly mediating autoimmune damage (as in EAE), or both. (2) In both diseases, the autoimmune T cells are clonally heterogeneous but recognize only a limited number of epitopes on the autoantigen (acetylcholine receptor in MG; myelin basic protein in EAE). Many of these epitopes can be defined as short peptide fragments of antigen, bound to a particular type of MHC molecule. (3) The MHC determines which peptides are recognized by autoimmune T cells in a given patient or inbred animal strain. (4) The discovery of the limited repertoire of autoimmune T cells has allowed considerable progress in the immunotherapy of EAE, using either monoclonal antibodies or cytotoxic T cells directed against clonotypic determinants on the autoaggressive T cells. (5) One obstacle to this approach in human disease is the polymorphism of the MHC in the species and the commensurate heterogeneity of autoimmune T cells.
Bilateral central macrogyria: Epilepsy, pseudobulbar palsy, and mental retardation—a recognizable neuronal migration disorder
Authors: Kuzniecky Ruben , Andermann Frederick , Tampieri Donatella , Melanson Denis , Olivier Andre , Leppik I. 
Published: January 2005

AbstractThe neuronal migration disorders comprise several morphological entities that are recognizable during life using current imaging techniques. We studied 4 patients who had a characteristic bilateral central rolandic and sylvian macrogyria. The patients had pseudobulbar palsy with oromotor incoordination and developmental delay and were mildly retarded. Minor seizures developed between the ages of 8 and 9 years. Subsequently, atonic drop attacks became the predominant epileptic pattern. Epileptogenic electrographic abnormalities were secondary generalized or multifocal. The lesions were detected by computed tomography and magnetic resonance imaging in all patients. Bilateral symmetrical areas of thick cortex surrounding a large sulcus were seen. This syndrome consists of specific clinical, imaging, electroencephalographic, and epileptic features. It can be suspected clinically and confirmed by imaging studies. Callosotomy in two patients helped the intractable seizures.
Middle cerebral artery pial territory infarcts: A study of the lausanne stroke registry
Authors: Bogousslavsky Julien , van Melle Guy , Regli Franco 
Published: January 2005

AbstractUsing a standard protocol (Doppler ultrasonography, electrocardiography, brain computed tomography, and in selected cases angiography, echocardiography, and Holter monitoring) we studied the clinical characteristics and etiological factors in 380 patients with first stroke who had a corresponding infarct limited to the territory of the pial branches of the middle cerebral artery (MCA) on computed tomography. The presumed cause of infarction was large‐artery disease (≥ 50% carotid artery or MCA stenosis or occlusion) in one‐third of the patients and cardioembolism in one‐fourth of the patients. Half of 230 patients undergoing angiography showed evidence for distal occlusions suggesting artery‐to‐artery or cardiac emboli. Severe heart disease and potential cardiac sources of embolism were more common in patients with infarcts in the territory of the posterior (inferior) division of MCA than in patients with infarcts in the territory of the anterior (superior) division of MCA. Persisting functional disability on discharge was also more severe in patients with the former than in patients with the latter. The neurological picture was polymorphous and it could sometimes even be misleading, as in some patients with a classical lacunar syndrome or with aphasia and unexpected sparing of speech areas. However, a few clinical syndromes apparently specific for involvement of a single MCA branch could be delineated, and in some instances they were highly predictive of embolism from heart or proximal artery.
Cerebrospinal fluid transthyretin in the neonate and blood–cerebrospinal fluid barrier permeability
Authors: Larsen Paul D. , Delallo Leonard 
Published: January 2005

AbstractWe measured transthyretin levels in the cerebrospinal fluid (CSF) of newborn infants, older children, patients with viral and bacterial meningitis, and adults with increased CSF protein levels. Neonatal CSF transthyretin levels are elevated disproportionately in comparison to levels in the other groups. We conclude that increased levels of transthyretin in the CSF of neonates are not explained by increased permeability of the blood–CSF barrier.
Dementia in Parkinson's disease is related to neuronal loss in the medial substantia nigra
Authors: Rinne Juha O. , Mlic Jaana Rummukainen , Paljärvi Leo , Rinne Urpo K. 
Published: January 2005

AbstractRegional neuronal loss in the substantia nigra was studied in relation to extrapyramidal symptoms and dementia in 12 patients with idiopathic Parkinson's disease(PD) and in 18 control subjects. Four areas of the right substantia nigra were investigated at the level of the superior colliculus and caudal red nucleus. In Parkinson's disease, the percentages of neurons, from the medial to the lateral part of the substantia nigra, were reduced to 49°, 31°, 41°, and 25° of the control values. The number of neurons in the lateral part showed a negative correlation with the severity of rigidity and hypokinesia, whereas tremor was less noticeable in patients with few neurons. The degree of dementia of the patients had a significant correlation only with neuronal loss in the medial part of the substantia nigra, suggesting, in view of the topographical organization of the neurons in the substantia nigra, that intact projections to the caudate nucleus and limbic and cortical areas are a prerequisite for normal cognitive functioning and that their dysfunction leads to clinical dementia.
Deprenyl suppresses the oxidant stress associated with increased dopamine turnover
Authors: Cohen Gerald , Spina Mary Beth 
Published: January 2005

AbstractTissue glutathione disulfide (GSSG) was studied as an index of changes in redox state in the striatum. When increased turnover of dopamine was provoked in mice by injection of haloperidol (1 mg/kg), the concentration of GSSG in the striatum tripled. Deprenyl (2.5 mg/kg) suppressed the rise in GSSG by 71.9%. These results indicate that deprenyl suppresses an oxidant stress associated with increased dopamine turnover.
Abnormal cough reflex in canine acrylamide neuropathy
Authors: Hersch Mark I. , McLeod James G. , Sullivan Colin E. 
Published: January 2005

AbstractCoughing in response to irritation of the airways is a fundamental protective reflex that is dependent on rapidly adapting bronchopulmonary receptors and their vagal afferent fibers; reflex airway constriction, which is effected by vagomotor efferent fibers, usually accompanies coughing. Although dysfunction of vagally mediated cardiovascular and gastrointestinal reflexes is a well‐documented complication of autonomic neuropathy, to date there have been no studies of the effect of peripheral autonomic failure on the cough reflex. In the study reported here, we examined the effect of acrylamide‐induced neuropathy, a distal axonopathy, on the ventilatory and tracheomotor components of the cough reflex in conscious dogs. There was a reduction in the cough reflex in response to mechanical irritation of the large airways in the preclinical phase of the neuropathy, and the cough reflex was virtually abolished when the dogs had moderate neuropathy. Following withdrawal of the neurotoxin, there was a substantial recovery of the cough reflex in surviving animals. It is possible that the cough reflex may be reduced in patients with vagal neuropathy and that this might compromise protection of the airway.
Subdivisional involvement of nigrostriatal loop in idiopathic parkinson's disease and striatonigral degeneration
Authors: Goto Satoshi , Hirano Asao , Matsumoto Sadayuki 
Published: January 2005

AbstractA topographical immunocytochemical analysis was performed on the substantia nigra from patients with idiopathic Parkinson's disease and striatonigral degeneration. Antibodies to tyrosine hydroxylase, a marker for nigrostriatal dopaminergic neurons, and to calcineurin, a marker for striatonigral projection fibers, were used in this study. There was a marked depletion of dopaminergic neurons in the substantia nigra of parkinsonian patients compared with control subjects, the reduction being greater in the lateral portion than in the medial portion (p < 0.001). Calcineurin immunoreactivity was densely distributed throughout the substantia nigra of patients with Parkinson's disease and control subjects. The numbers of dopaminergic neurons and of calcineurin‐immunoreactive fibers were markedly reduced in the lateral portion of the substantia nigra in all patients with striatonigral degeneration. Our results suggest that many symptoms of these two diseases may be due to disruption of the functions of the putamen and the lateral protein of the substantia nigra, which have dense reciprocal connections as part of the dopamine‐related nigrostriatal loop.
The hippocampus in experimental chronic epilepsy: A morphometric analysis
Authors: Bertram Edward H. , Lothman Eric W. , Lenn Nicholas J. 
Published: January 2005

AbstractThe effect of intermittent seizures on the pryramidal neurons of the hippocampus is largely unknown. To determine whether recurrent seizures centered in the hippocampus can produce neuronal loss in this region, a morphometric analysis was performed from standardized sections of hippocampus using 5 groups of animals: (1) surgical control subjects, (2) rats kindled by the rapidly recurring hippocampal seizure (RRHS) paradigm, (3) kindled rats with a few additional limbic seizures (528 ° 66 seizures), (4) kindled rats with may limbic seizures (1,523 ° 130 seizures), and (5) rats experiencing limbic status epilepticus (SE) induced by “continuous” hippocampal stimulation. The RRHS and SE protocols induced significant neuronal loss in the CA1 region, but no evidence was found for additional cell loss with increasing numbers of intermittent seizures. These intermittent seizures were, however, associated with a significant thickening of the basal and apical dendritic fields of the CA1 region. These finding indicate that intermittent seizures produce no significant hippocampal neuronal loss and may result in a hypertrophy of CA1 dendritic fields.
Interfer beta augments supperssor cell function in multiple sclerosis
Authors: Noronha Avertano , Toscas Angela , Jensen Mark A. 
Published: January 2005

AbstractSuppressor cell function has been previously reported to be decreased in patients with progressive multiple sclerosis (MS). The abnormality could not be corrected in vitro and was present even after patients were treated with immunosuppressive agents. We now report that interferon beta augments suppressor function in vitro in progressive MS. Nonspecific suppressor cell function as measured in a concanavalin A (Con A) suppressor assay was reduced in 24 MS patients (mean percent suppression, 19.6 ± 2.2) when compared to 19 normal subjects (mean percent suppression, 35.0 ± 3.3). The data are highly significant (p < 0.001). When recombinant human interferon beta (103 units/ml) was added to lymphocyte cultures with Con A, Suppressor activity improved from 19.6 ± 2.2 to 37.8 ± 2.6 in MS (p < 0.001) and from 35.0 ± 3.3 to 46.2 ± 3.5 (p < 0.025) in control subjects. This study shows that recombinant interferon beta improves suppressor function in humans, an effect that is particularly significant in progressive MS.
Immunoregulatory properties of T‐cell lines derived from the systemic and intrathecal compartments: A phenotypic and functional study
Authors: Freedman Mark S. , Loertscher Rolf , Cashman Neil R. , Duquette Pierre , Blain Manon , Antel Jack P. 
Published: January 2005

AbstractTo determine whether immune regulation can differ within the intrathecal and systemic compartments, we compared phenotypic markers and functional properties of in vitro anti‐CD3 monoclonal antibody‐stimulated, interleukin 2‐expanded lymphoid cell lines simultaneously derived from peripheral blood and cerebrospinal fluid of individual donors (n = 9). We found that the proportions of total CD8+ T cells and of the putative CD8+ suppressor effector subset (CD28−) were lower in the cell lines derived from cerebrospinal fluid compared with cultures derived from peripheral blood (p p0.025 and p p0.005, respectively; paired t test), whereas the total CD4+ T‐cell proportion was higher (p p 0.025). For a donor subgroup with normal peripheral blood cell‐mediated activated suppressor function (63 ± 2), mean suppressor cell function mediated by unfractionated or CD8+ ‐enriched cells derived from cerebrospinal fluid was significantly lower (38 ± 7%; p 0.01, paired t test). For a donor subgroup with low peripheral blood cell‐mediated suppression (‐1 ± 10%), suppression mediated by cerebrospinal fluid cells was also low (9 ± 12%). Our results support the postulate that the immune response may be differentially regulated between the central nervous system and peripheral blood compartments.
Localization of epileptic foci using a large‐area magnetometer and functional brain anatomy
Authors: Tiihonen J. , Hari Dr R. , Kajola M. , Nousiainen U. , Vapalahti M. 
Published: January 2005

AbstractWe used a large‐area, 7‐channel, first‐order superconducting quantum interference device (SQUID) gradiometer to preoperatively determine the sites of epileptic foci in 2 patients with intractable temporal lobe seizures. The equivalent dipoles for the epileptic spikes were located with respect to external landmarks of the skull and in relation to the generation sites of magnetic auditory evoked responses. It was also possible, for the first time, to determine the location of the equivalent source using simultaneously measured data from seven locations only. The sites of the equivalent dipoles, in the right temporal lobe, agreed with the electrocorticographic and depth electrode recordings made during the operation.
A new point mutation within exon 5 of β‐hexosaminidase α gene in a Japanese infant with Tay‐Sachs disease
Authors: Nakano Takeshi , Nanba Eiji , Tanaka Akemi , Ohno Kousaku , Suzuki Yoshiyuki , Suzuki Kunihiko 
Published: January 2005

AbstractA new point mutation within exon 5 of β‐hexosaminidase α subunit gene (guanine509→adenine; arginine170→glutamine) has been indentified as being responsible for the typical clinical and enzymological phenotype of infantile Tay‐Sachs disease in a Japanese infant. Expression of the mutant enzyme protein in the COS I cell system indicated that it is catalytically inactive and also is unstable. The patient is a compound heterozygote, and the exact abnormality in the other allele could not be identified except that it is not any of the other nine known mutations of the βhexosaminidase α. The data collectively suggest that the other allele is not producing stable messenger RNA (mRNA). The rapidly increasing number of mutations responsible for clinical and enzymological phenotypes and the very large number of statistically possible combinations among them for compound heterozygosity pose a serious pragmatic problem for classification and nomenclature of this group of rare genetic disorders.
Which risk factors predict the levodopa response in fluctuating Parkinson's disease?
Authors: Horstink M. W. I. M. , Zijlmans J. C. M. , Pasman J. W. , Berger H. J. C. , Korten J. J. , Van 't Hof M. A. 
Published: January 2005

AbstractThree putative risk factors to the pathogenesis of end‐of‐dose–related fluctuations–severity of Parkinson's disease (PD), duration of PD, and duration of levodopa therapy–were evaluated in 39 patients with PD by measuring response duration and response magnitude to a single levodopa dose. After discontinuing levodopa therapy for 12 hours, the motor status of each patient was assessed before a single dose of 100 mg of levodopa plus 25 mg of decarboxylase inhibitor was given and at five 45‐minute intervals thereafter. Multiple regression analysis showed that the response duration was best predicted by the severity of PD. The response magnitude was significantly correlated both to the duration of PD and to the duration of levodopa therapy. It is argued, however, that the response magnitude was predicted by the duration of levodopa therapy. Our results are in accordance with those of other recent studies and indicate that variances in levodopa response duration and in levodopa response magnitude are caused by their own differential risk factor.
Tyrosine hydroxylase—immunoreactive neurons in the temporal lobe in complex partial seizures
Authors: Zhu Zhen‐qing , Armstrong Dawna L. , Grossman Robert G. , Hamilton Winifred J. 
Published: January 2005

AbstractThe anterior mesial portion of the temporal lobe removed from 16 patients who underwent surgery for the treatment of complex partial seizures was found to contain tyrosine hydroxylase–immunoreactive neurons. The distribution of these neurons was correlated with the underlying neuropathological features. Ammon's horn sclerosis was present in 8 patients; a ganglioglioma, in 7 patients; and an infarction in the distribution of the middle cerebral artery, in 1 patient. Tyrosine hydroxylase–immunoreactive neurons were found in Ammon's horn of 6 of the 8 patients with Ammon's horn sclerosis, and in the subiculum and entorhinal cortex of all 8 patients with the same pathology. None of these neurons were found in Ammon's horn of the 7 patients with a ganglioglioma, but were found in the subiculum of 5 of the 7 patients and in the entorhinal cortex of all 7 patients. Tyrosine hydroxylase immunoreactivity was also studied in 13 control autopsy specimens. No tyrosine hydroxylase–immunoreactive neurons were found in Ammon's horn or the subiculum of any of the control specimens, but were found in the entorhinal cortex of 6 of the 13 specimens. The tyrosine hydroxylase–immunoreactive neurons in the mesial portion of the temporal lobe of patients with complex partial seizures may contribute to the increased levels of tyrosine hydroxylase found in neurochemical studies of specimens taken at temporal lobectomy.
Selective sparing of NADPH‐diaphorase–somatostatin–neuropeptide Y neurons in ischemic gerbil striatum
Authors: Uemura Yoshihiko , Kowall Neil W. , Beal M. Flint 
Published: January 2005

AbstractIn the present study we investigated the relative vulnerability of neuronal subsets in the striatum to ischemia that was induced by bilateral transient ligation of the common carotid arteries in gerbils. After 4 days of survival, brains were evaluated using histochemical methods (NADPH‐diaphorase and silver degeneration procedures) and neurochemical methods with radioimmunoassays for somatostatin‐, neuropeptide Y–, and substance P–like immunoreactivity and measurements of amino acids using high‐pressure liquid chromatography with electrochemical detection. NADPH‐diaphorase–positive neurons were strikingly preserved in the ischemic dorsolateral portion of the striatum, in which there was severe neuronal loss. There was no significant depletion of NADPH‐diaphorase‐positive neurons in the striatum or cerebral cortex. Concentrations of neuropeptide Y–like and somatostatin‐like immunoreactivity were unchanged despite a significant 25% depletion of substance P–like immunoreactivity and gamma‐aminobutyric acid. Ischemic brain damage may be mediated by a neurotoxic effect of glutamate acting at the N‐methyl‐D‐aspartate (NMDA) receptor. Previous studies of NMDA excitotoxin lesions in rat striatum have shown a sparing of neurons containing NADPH‐diaphorase, somatostatin, and neuropeptide Y. The similar sparing of these neurons following ischemic lesions in gerbil striatum provides further evidence that NMDA receptor activation may play a role in ischemic injury.
Net production of cerebrospinal fluid is decreased by SCH‐23390
Authors: Boyson Sally J. , Alexander Alice 
Published: January 2005

AbstractA high density of binding sites for the ligands 3H‐SCH‐23390 and 3H‐SKF‐83566 has been found in the choroid plexus. Although these sites have similar pharmacology to D1 dopamine receptors, the high‐affinity component of 3H‐SCH‐23390 binding in the choroid plexus has been identified as the 5‐HT1c subtype of serotonin receptor. We investigated the possible role of these receptors in modulating the production of cerebrospinal fluid (CSF) in rats. (R) SCH‐23390 produced up to a 50% decrease in net CSF production, compared to saline. This compound is partial agonist at 5‐HT1c serotonin receptors, and an antagonist at D1 dopamine receptors. The (S) enantiomer of SCH‐23390 (SCH‐23388) was ineffective. Drugs interacting with receptors for neurotransmitters in the choroid plexus may hold promise for the treatment of patients with increased intracranial pressure, including those with mass lesions, head trauma, acute or chronic hydrocephalus, or pseudotumor cerebri.
Human central nervous system primitive neuroectodermal tumor expressing nerve growth factor receptors: CHP707M
Authors: Baker David L. , Reddy Usha Rani , Pleasure Samuel , Hardy Mattie , Williams Marge , Tartaglione Margaret , Biegel Jaclyn A. , Emanuel Beverly S. , Presti Patrizia Lo , Kreider Barbara , Trojanowski John Q. , Evans Audrey , Roy Amit R. , Venkatakrishnan Gita , Chen Jie , Ross Alonzo H. , Pleasure David 
Published: January 2005

AbstractA primitive neuroectodermal tumor (PNET) presented as a cerebral hemispheric mass in a 33‐year‐old man. Bone marrow metastases were discovered 11 months later. A cell line (CHP707m) was derived from these metastases. In culture, the cells showed features of neuronal differentiation, forming short neurites and synthesizing low‐molecular‐weight neurofilament protein. Northern blotting showed the tumor cells express nerve growth factor (NGF) receptor messenger RNA, and fluorescence‐activated cell‐sorting demonstrated NGF receptors on the cell surface. Western blotting showed CHP707m NGF receptors are truncated. The receptors are functional; they bind iodine 125–labeled mouse NGF with an affinity of 1.6 × 10−9 M, and short‐term treatment with NGF induces expression by the tumor cells of the proto‐oncogene, c‐fos. Although CHP707m is the first central nervous system PNET cell line proven to express NGF receptors, immunohistological survey of tissue sections prepared from human central nervous system PNETs showed that 13 of 35 contained NGF receptor‐positive tumor cells. Thus, more than one‐third of such tumors might be responsive to the effects of NGF.
Drop attacks with Meniere's syndrome
Authors: Baloh R. W. , Jacobson K. , Winder T. 
Published: January 2005

AbstractWe report the clinical features of 12 patients with drop attacks associated with Meniere's syndrome. Each described a sensation of being pushed, thrown, or knocked to the ground or a sudden illusion of movement of the environment that led to a fall. These episodes were not accompanied by symptoms of their typical attacks of Meniere's syndrome. The drop attacks occurred early and late in the course of the disease; they were the initial manifestation in 1 patient. In the majority the episodes spontaneously remitted, although Meniere's syndrome continued to progress. These attacks probably result from a sudden mechanical deformation of the otolithic membrane of the utricle or saccule due to pressure gradients within the inner ear. They are important to recognize because their prognosis is relatively benign compared to other causes of drop attacks.