bluuebunny/crossref_metadata_2025_split huggingface dataset:
Effects of brain‐derived neurotrophic factor on motor dysfunction in wobbler mouse motor neuron disease
Authors: Ikeda Ken , Klinkosz Bogdan , Greene Tom , Cedarbaum Jesse M. , Wong Vivien , Lindsay Ronald M. , Mitsumoto Hiroshi 
Published: January 2005

AbstractBrain‐derived neurotrophic factor (BDNF) has been shown to promote the survival of developing motor neurons in vitro and to rescue motor neurons from axotomy‐induced cell death in vivo. In this study, we examined the effects of exogenous BDNF on the progression of wobbler mouse motor neuron disease (MND). After clinical diagnosis at age 3 to 4 weeks, 20 affected mice received subcutaneous injections of recombinant human BDNF (5 mg/kg, n = 10) or vehicle (n = 10), three times a week for 4 weeks. In a separate experiment done to conduct a histometric analysis of the C‐5 and C‐6 ventral roots and to determine the number of myelinated nerve fibers, 7 wobbler mice received identical BDNF treatment. In the 10 BDNF‐treated wobbler mice, grip strength declined at a slower rate (p < 0.03) and was twice as great as that of vehicle‐treated animals at the end of treatment (p < 0.01). In vivo biceps (p < 0.01‐) and in vitro muscle twitch tensions (p < 0.02) were also greater than those of vehicle‐treated mice. The biceps muscle weight was 20% greater (p < 0.05) and the mean muscle fiber diameter was significantly larger in BDNF‐treated mice (p < 0.001) because the number of small (denervated) muscle fibers was markedly reduced. The number of myelinated motor axons at the cervical ventral roots studied in the additional 7 affected mice was 25% greater with BDNF treatment (p < 0.0001). This study establishes that exogenous BDNF administration can retard motor dysfunction in a natural MND and diminish denervation muscle atrophy and motor axon loss.
Selectively distributed processing of visual object recognition in the temporal and frontal lobes of the human brain
Authors: Seeck M. , Schomer D. , Mainwaring N. , Ives J. , Dubuisson D. , Blume H. , Cosgrove R. , Ransil B. J. , Mesulam M.‐M. 
Published: January 2005

AbstractEvoked potentials to visually driven cognitive tasks were recorded through depth electrodes placed bilaterally within the amygdala, hippocampus, midtemporal and inferotemporal cortex, and lateral frontal cortex of 6 epileptic patients. Task‐related differential response patterns were used to identify the recording sites engaged by specific aspects of visual encoding. In this group of 6 patients, the amygdala was most frequently engaged in encoding the familiarity of faces; midtemporal and inferotemporal cortex, in encoding perceptual identity and object categorization; and lateral frontal cortex, in holding visual object information in working memory. The two aspects of encoding that most frequently engaged the hippocampal region were related to working memory and object categorization. The processing of complex visual knowledge is thus anatomically distributed but regionally specialized. These experiments also showed that identical input and output parameters can engage different areas of the brain depending on the nature of the instructional set.
Decreased CD3‐mediated interferon‐γ production in relapsing‐remitting multiple sclerosis
Authors: Brod Staley A. , Khan Mohammed , Bright John , Sriram Subramaniam , Marshall Gailen D. , Henninger Evelyn M. , Kerman Ronald H. , Wolinsky Jerry S. 
Published: January 2005

AbstractMultiple sclerosis (MS) is a chronic inflammatory disease of the central nervous system that has been postulated to be T‐cell mediated. We examined the proliferation and cytokine secretion of mononuclear cells after stimulation with OKT3 (anti‐CD3) monoclonal antibody concanavalin A, or ionomycin plus myristic acid palmityl ester in subjects with stable relapsing‐remitting MS. Control subjects demonstrated good proliferation to anti‐CD3 monoclonal antibody whereas subjects with relapsing‐remitting MS showed a significantly decreased anti‐CD3 monoclonal antibody‐mediated response. There was no difference in concanavalin or ionomycin plus myristic acid palmityl ester stimulation between control subjects and MS subjects. Secretion of interferon‐γ was significantly decreased and transforming growth factor‐β was significantly increased from cultures stimulated with anti‐CD3 monoclonal antibody, but not ionomycin plus myristic acid palmityl ester or concanavalin A, in MS patients compared to control subjects. Secretion of interleukin‐10 and tumor necrosis factor‐β was not different between control subjects and MS patients following stimulation with anti‐CD3 monoclonal antibody, concanavalin A, or ionomycin plus myristic acid palmityl ester, or of interleukin‐2 and interleukin‐4 following stimulation with anti‐CD3 monoclonal antibody or concanavalin A. An abnormality of signal transduction and secretion of the immunomodulatory molecule interferon‐γ may exist in MS via the CD3 T‐cell receptor complex.
Locomotor capacity of spinal cord in paraplegic patients
Authors: Dietz V. , Colombo G. , Jensen L. , Baumgartner L. 
Published: January 2005

AbstractThe induction of complex bilateral leg muscle activation combined with coordinated stepping movements is demonstrated in patients with complete paraplegia. This was achieved by partially unloading patients who were on a moving treadmill. In comparison to healthy subjects, the paraplegic patients displayed a less dynamic mode of muscle activation. In all other respects leg muscle electromyographic activity was modulated in a similar manner to that in healthy subjects. However, the level of electromyographic activity in the gastrocnemius (the main antigravity muscle during gait) was considerably lower in the patients. During the course of a daily locomotor training program, the amplitude of gastrocnemius electromyographic activity increased significantly during the stance phase, while inappropriate tibialis anterior activation decreased. Incompletely paraplegic patients benefited form the training with respect to performance of unsupported stepping movements on solid ground. In about half of completely paraplegic patients with low muscle tone, no beneficial effect of the training was seen. This may be due to an inhibitory effect on spinal neuronal activity by drugs patients were taking (e.g., prazosin, clonidine, cannabinoids). In this study intrathecal application of clonidine drastically reduced, while epinephrine enhanced locomotor muscle electromyographic activity. The results of this study promise to be significant in the treatment of paraplegic patients.
Outcome after spinal reirradiation for malignant epidural spinal cord compression
Authors: Schiff David , Shaw Edward G. , Cascino Terrence L. 
Published: January 2005

AbstractRadiotherapy is effective for most case of spinal cord compression. Although recurrent spinal cord compression is a common problem, little is known about whether reirradiation preserves neurologic function and what risk of radiation myelopathy it carries. To investigate this question, we reviewed patients at the Mayo Clinic between 1975 and 1992 undergoing two or more courses of radiotherapy to the same segment of the spinal column with radiographically documented epidural disease at the time of reirradiation to determine outcome as measured by the ability to walk and by survival. Fifty‐four patients met the study criteria. Radiation doses for the first course ranged from 2,250 to 5,400 cGy (median, 3,000 cGy), and total dose for all courses to the reirradiated spinal segment ranged from 3,650 to 8,089 cGy (median, 5,425 cGy). All patients were ambulatory following the first course of radiation, 40 (74%) were ambulatory at the onset of reirradiation, and 42 (78%) were ambulatory at the end of reirradiation. Thirty‐seven patients (69%) remained ambulatory at their last follow‐up 6 days to 80 months following reirradiation (median, 4.7 months). Five patients eventually became nonambulatory 6.5 to 35 months following reirradiation. Median survival for all patients following reirradiation was 4.2 months. We conclude that for cancer patients with progressive epidural disease following radiotherapy, reirradiation frequently preserves ambulation and carries minimal risk of radiation myelopathy during the patients' lifetime.
Gene dose of the ε4 allele of apolipoprotein E and disease progression in sporadic late‐onset alzheimer's disease
Authors: Frisoni Giovanni B. , Govoni Stefano , Geroldi Cristina , Bianchetti Angelo , Calabresi Laura , Franceschini Guido , Trabucchi Marco 
Published: January 2005

AbstractA strong association has been described in cross‐sectional studies between the ε4 allele of the apolipoprotein E and late‐onset Alzheimer's disease (LOAD). This study addresses the relationship between disease progression and the ε4 allele in 62 sporadic LOAD (onset at age 70 and over) patients. Disease progression was estimated retrospectively with the Mini‐Mental State Examination (MMSE) amounting to 4.7, 3.8, and 2.2 points per year (sex‐adjusted test for trend: p = 0.01) and with the Clinical Dementia Rating (CDR) to 0.76, 0.67, and 0.42 units per year (p = 0.03) in ‐/‐, ε4/‐, and ε4/ε4 patients. The proportion of patients with fast progression decreased (p ≤ 0.005) and with slow progression increased (p = 0.002) with increasing ε4 gene dose. These findings were confirmed in a smaller sample of 28 patients for whom longitudinal assessments of MMSE were available and when nonlinear progression of the disease was accounted for in a stratified analysis. These data suggest that disease duration might be longer in ε4 carriers and that this might at least partly account for the cross‐sectional association between the ε4 allele and LOAD.
First seizures associated with playing electronic screen games: A community‐based study in Great Britain
Authors: Quirk J. A. , Fish D. R. , Smith S. J. M. , Sander J. W. A. S. , Shorvon S. D. , Allen P. J. 
Published: January 2005

AbstractActive surveillance by virtually all electroencephalographic departments throughout Great Britain identified 118 patients who had a first seizure while playing an electronic screen game during two 3‐month periods. Patients were divided into Group A (46 patients)—those for whom there was thought to be a definite causal relationship (type 4 photoparoxysmal response); Group B (25 patients)—those for whom there was a probable causal relationship (types 1–3 photoparoxysmal response, clinical evidence of photosensitivity, subsequent recurrent seizures on repeat exposure to electronic screen games, and/or occipital spikes in the resting electroencephalogram); and Group C (47 patients)—those for whom there was no apparent causal relationship. The number of patients in Group C did not exceed that expected by the chance occurrence of two common events (playing electronic screen games and incidence of epilepsy). Most (103/118) of the patients were in the age range of 7 to 19 years. Within this age group the annual incidence of first seizures triggered by playing electronic screen games (Groups A and B combined) was estimated to be 1.5/100,000.
Pallidotomy increases activity of motor association cortex in parkinson's disease: A positron emission tomographic study
Authors: Grafton Scott T. , Waters Cheryl , Sutton James , Lew Mark F. , Couldwell William 
Published: January 2005

AbstractStereotactic posteroventral pallidotomy can improve motor performance in Parkinson's disease. Interruption of inhibitory pallidal projections to ventrolateral thalamus, components of a cortical‐basal ganglia motor loop allows for this clinical benefit. We hypothesized that pallidotomy would lead to increased movement related activity in motor cortical areas receiving projections from ventrolateral thalamus. This was tested in 6 Parkinson's disease patients who underwent stereotactic posteroventral pallidotomy. Each patient was imaged with positron emission tomography (PET) measures of regional cerebral blood flow (rCBF) during performance of a simple prehension task and at rest. Scans were acquired before and 17 weeks after surgery. After pallidotomy, movement‐related changes of rCBF increased significantly in both the supplementary motor area (SMA) and premotor cortex but not in primary motor cortex. The results demonstrate the importance of pallidothalamic circuitry for regulating volitional movements and confirm that disruption of inhibitory input to the ventrolateral thalamus can augment movement‐related activity in motor association areas.
Movement‐related cortical potentials preceding repetitive and random‐choice hand movements in parkinson's disease
Authors: Touge T. , Werhahn K. J. , Rothwell J. C. , Marsden C. D. 
Published: January 2005

AbstractThe movement‐related cortical electroencephalographic potential was recorded from scalp electrodes in 8 patients with idiopathic Parkinson's disease studied at least 12 hours after withdrawal of their normal drug therapy, and compared with the results from a group of 8 age‐matched control subjects. Two types of self‐paced voluntary arm movements were examined: repetitive forward movement of a joystick, and random‐choice movements of the same joystick in which subjects had to choose freely the direction in which they were to move the stick (forward, backward, left, or right). In normal subjects, the movement‐related cortical potential was larger prior to random‐choice movements, whereas in the patients, the amplitude was the same in both tasks. The implication is that processes involved in self‐selection of movement are abnormal in Parkinson's disease. This may contribute to the difficulty that patients have in initiating voluntary movement in the absence of any external cues.
Oculomotor function in amyotrophic lateral sclerosis: Evidence for frontal impairment
Authors: Shaunak S. , Orrell R. W. , O'Sullivan E. , Hawken M. B. , Lane R. J. M. , Henderson L. , Kennard C. 
Published: January 2005

AbstractSaccadic eye movements, fixation, and smooth pursuit were recorded in 17 subjects with amyotrophic lateral sclerosis (ALS) and 11 age‐matched controls using a magnetic scleral search coil. Reflexive, remembered and antisaccades, and smooth pursuit at four target velocities were studied. Subjects with ALS showed significantly elevated error rates (distractibility) and latency in the antisaccade and remembered saccade paradigms but no abnormality of reflexive saccades. The frequency of small saccades that intruded on steady fixation (square‐wave jerks) was also increased in ALS subjects. Peak velocity gain of smooth pursuit and performance on the Wisconsin Card Sort Test did not differ significantly between the two groups. These findings are consistent with prefrontal dysfunction in ALS and provide an independent source of support for the thesis that the pathology of this condition invades frontal cortex.
Treatment of dural sinus thrombosis using selective catheterization and urokinase
Authors: Horowitz Michael , Purdy Phillip , Unwin Hal , Carstens George , Greenlee Ralph , Hise Joe , Kopitnik Tom , Batjer Hunt , Rollins Nancy , Samson Duke 
Published: January 2005

AbstractThrombosis of the cerebral dural venous sinuses, cortical draining veins, and deep cerebral veins is a rare clinical finding. Because of its low incidence and multiple etiologies, the optimum therapy for this condition will only be elucidated by a multicenter, randomized prospective study. At our institution, we favor early and aggressive management of cerebral venous sinus thrombosis with transfemoral, venous intradural infusions of the fibrinolytic agent urokinase. To date, treatment of only 13 patients using this technique has been reported in the English literature. This report adds 12 more such treated patients. Despite the presence of preinfusion infarcts in 5 patients, four of which were hemorrhagic, we incurred no major therapeutic morbidity. Functional sinus patency was achieved in 11 of 12 patients, with our only true failure occurring in an individual with symptoms of at least 2 months' duration. Good to excellent clinical outcome was achieved in 10 of 11 patients (one newborn had inadequate follow‐up).
Hu antigens: Reactivity with hu antibodies, tumor expression, and major immunogenic sites
Authors: Manley Geoffrey T. , Smitt Peter Sillevis , Dalmau Josep , Posner Jerome B. 
Published: January 2005

AbstractHuD, a human neuronal RNA‐binding protein, was the first identified member of a family of antigens that also includes HuC and Hel‐N1 (Hu antigens). The serum of all patients with anti‐Hu–associated paraneoplastic encephalomyelitis and sensory neuronopathy react with HuD and Hel‐N1, but the reactivity with HuC is unknown. In the current study we examined (1) the reactivity of anti‐Hu sera with HuD, HuC, and Hel‐N1; (2) the expression of HuD, HuC, and Hel‐N1 messenger RNA in small‐cell lung cancer of patients with and those without paraneoplastic encephalomyelitis/sensory neuronopathy; (3) the correlation between anti‐Hu serum reactivity with these three Hu antigens and the type of neurological symptoms; and (4) the major immunogenic sites of HuD. Our findings indicate that all anti‐Hu sera react with HuD, HuC, and Hel‐N1. However, only HuD is expressed in the small‐cell lung cancer tumors, indicating that among the three Hu antigens, HuD appears to play a central role in triggering the anti‐Hu immune response. No differences were identified regarding the reactivity of the anti‐Hu antibodies with HuD, HuC, and Hel‐N1 and the spectrum of neurological symptoms presented by the patients. Study of the major immunogenic sites of HuD resulted in the identification of two major immunodominant regions with at least two distinct epitopes recognized by the serum of all patients with anti‐Hu—associated paraneoplastic encephalomyelitis/sensory neuronopathy.
Recurrent stroke and thrombo‐occlusive events in the antiphospholipid syndrome
Authors: Levine Steven R. , Brey Robin L. , Sawaya Kara L. , Salowich‐Palm Leeza , Kokkinos James , Kostrzema Beata , Perry Mary , Havstad Suzanne , Carey John 
Published: January 2005

AbstractWe prospectively studied 81 consecutively identified patients with antiphospholipid antibodies (aPLs) who developed focal cerebral ischemia over a 7‐year period. The mean age of this cohort was approximately a decade younger than the average atherothromboembolic stroke victim and women were more commonly involved than men. The frequency of conventional stroke risk factors was lowest in the group of stroke patients with the highest levels of IgG cardiolipin immunoreactivity. Other serological abnormalities associated with aPL (false‐positive Veneral Disease Research Laboratory test, thrombocytopenia, prolonged activated partial thromboplastin time [aPTT]) were more common in the group with over 100 GPL units (high positive). Patients with the highest IgG anticardiolipin titers had the shortest times to subsequent thrombo‐occlusive events. The most common recurrent event was cerebral infarction, often occurring within the first year of follow‐up during a mean prospective follow‐up of 3 years. Over one‐half of the cohort had at least one recurrent thrombo‐occlusive event during follow‐up. This distinct syndrome of cerebral ischemia should be recognized for its younger age at onset, predominance of women, high risk of recurrent thrombo‐occlusive events, and the possible use of the IgG anticardiolipin antibody titer for prognosis.
Why do DNA testing? Practical and ethical implications of new neurogenetic tests
Authors: Bird Thomas D. , Bennett Robin L. 
Published: January 2005

AbstractDirect DNA tests for mutations in neurogenetic disorders are establishing a new era in diagnostic neurology. These highly accurate and specific tests will greatly improve diagnosis and genetic counseling, lead to more accurate assessment of prognosis, and reduce the cost of diagnostic evaluations. However, the use and interpretation of the tests can be complicated, and there are numerous social, psychological, and ethical implications of the testing that need to be recognized and anticipated. To provide the best care for our patients, neurologists need to understand the benefits and risks of this testing and properly apply it in the context of each individual clinical situation.
Benzodiazepine receptor binding in cerebellar degenerations studied with positron emission tomography
Authors: Gilman Sid , Koeppe Robert A. , Junck Larry , Kluin Karen J. , Lohman Mary , St. Laurent Roy T. 
Published: January 2005

AbstractWe used positron emission tomography with [11C]flumazenil to study gamma‐aminobutyric acid type A/benzodiazepine receptor binding quantitatively in the cerebral hemispheres, basal ganglia, thalamus, cerebellum, and brainstem of 72 subjects, including 14 with multiple system atrophy of the ataxic (olivopontocerebellar atrophy) type, 5 with multiple system atrophy of the extrapyramidal/autonomic (Shy‐Drager syndrome) type, 18 with sporadic olivoponto‐cerebellar atrophy, 15 with dominantly inherited olivopontocerebellar atrophy, and 20 normal control subjects with similar age and sex distributions. In comparison with data obtained from the normal control subjects, we found significantly Decemberreased ligand influx in the cerebellum and brainstem of multiple system atrophy patients of the olivopontocerebellar atrophy type and in patients with sporadic olivopontocerebellar atrophy, but not in patients with multiple system atrophy of the Shy‐Drager syndrome type. Despite these differences in ligand influx, benzodiazepine binding was largely preserved in the cerebral hemispheres, basal ganglia, thalamus, cerebellum, and brainstem in patients with multiple system atrophy of both types as well as those with sporadic or dominantly inhierited olivoponto‐cerebellar atrophy as compared with normal control subjects. The finding of relative preservation of benzodiazepine receptors indicates that these sites are available for pharmacological therapy in these disorders.
An italian kindred with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)
Authors: Ragno Michele , Tournier‐Lasserve Elisabeth , Fiori Mario G. , Manca Antonio , Patrosso Maria Cristina , Ferlini Alessandra , Sirocchi Giovanna , Trojano Luigi , Chabriat Hugues , Salvi Fabrizio 
Published: January 2005

AbstractVascular dementia is usually sporadic and associated with definite risk factors. Several cases also occur in a familial fashion, and may affect middle‐aged or even younger subjects. Recently, an autosomal dominant inheritance was demonstrated in two unrelated French families, the members of which were affected by storke‐like episodes culminating in progressive dementia. Genetic linkage analysis assigned the disease locus to chromosome 19q12. We report an additional kindred of Italian origin in which at least 16 subjects presented leukoencephalopathic alterations. Recurrent strokes, psychiatric disturbances, dementia, and in 2 members, tetraplegia and pseudobulbar palsy were the hallMarchks of this syndrome. Notably, 5 asymptomatic individuals had neuroradiological signs of leukoencephalopathy. Pathological examination of 1 subject revealed a widespread vasculopathy of the perforating arterioles, characterized by deposition of eosinophilic‐congophilic material that did not immunostain with antibodies against prion protein, β‐amyloid, cystatin C, transthyretin, or heat‐shock protein 70 and was similar to that described in the French families. Based on the maximum lod score, the most likely location for the disease locus was also mapped to chromosome 19q12, and found to coincide with the CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) locus. The present results confirm the existence of a nosologically distinct, autosomal dominant cerebrovascular disease, presenting with recurrent subcortical ischemic strokes independent of vascular risk factors.