bluuebunny/crossref_metadata_2025_split huggingface dataset:
Molecular Interactions Modulating Neuronal Survival and Growth
Authors: Riopelle R.J. , Dow K.E. , Verge V.M.K. , Richardson P.M. 
Published: July 2016

ABSTRACT:The extracellular environment of the neuron provides a heterogeneous milieu of survival and growth modulating molecular species subserving regulatory signals that operate in development, meditate activity-dependent enduring changes in synaptic connectivity, and promote or inhibit survival and axonal regeneration following insult. Parallel distributed processing networks in neurons, activated by these molecular species, can likely be recruited selectively to serve specific needs of the organism.
Neuronal Loss in a Lower Motor Neuron Disease Induced by a Murine Retrovirus
Authors: Jolicoeur Paul 
Published: July 2016

ABSTRACT:An animal model of neuronal loss induced by a murine retrovirus is described. The neurological disease is manifested by a lower limb paralysis and is characterized as a spongiform myeloencephalopathy. The primary determinant of neurovirulence has been mapped within the viral env sequences and a secondary determinant is present within the LTR region. A model of pathogenesis, suggesting that the disease is receptor-mediated, is proposed.
Electroencephalography, Magnetic Resonance Imaging and Pathology in Patients Treated Surgically for Temporal Lobe Epilepsy
Authors: Feindel William , Robitaille Yvon , Tampieri Donnatella , Goossens Lutgart , Li Maria , Melançon Denis 
Published: July 2016

ABSTRACT:In a consecutive series of 40 patients selected by EEG studies for surgical treatment of temporal lobe seizures, magnetic resonance imaging showed structural lesions in 25% and signal abnormalities, usually in the mesial temporal region, in another 35%. Pathological changes included structural lesions in over 30% of patients and sclerosis of the amygdala in almost half of the series. These findings further substantiate the implication of the amygdala in the pathogenesis of temporal lobe seizures with automatism and amnesia.
Temporal Lobectomy: Review of 100 Cases with Major Hippocampectomy
Authors: Feindel William , Rasmussen Theodore 
Published: July 2016

ABSTRACT:One hundred consecutive patients between 1961 and 1980, with temporal lobectomy including excision of half or more of the hippocampal formation, have been analyzed for degree of reduction of seizures. Thirty-nine percent have become and remained seizure free for follow-up period of 2 to 24 years (median 12 years). Another 24 patients achieved a marked reduction in seizure tendency as defined in the report and 25 showed a lesser degree of improvement. In fifteen patients no change in seizure frequency was recorded. Thus in this series of 100 patients, a complete or marked reduction of the seizure tendency was achieved in 63%.
Relevance of Removal of Limbic Structures in Surgery for Temporal Lobe Epilepsy
Authors: Olivier André 
Published: July 2016

ABSTRACT:We have briefly reviewed the experimental and clinical evidence for the importance of the amygdala and hippocampal formation in temporal lobe epilepsy. More specifically, we have analyzed our own experience in patients with temporal lobe epilepsy investigated with intracerebral stereotaxic electrodes and operated by various modalities of resection. Our results, in agreement with previous experimental and clinical work, provide further evidence for an overwhelming predominance of limbic participation in temporal lobe epilepsy. As a result, more and more selective procedures are being carried out involving the mesial structures. However, this shift has been slow and progressive because of the proven value of cortico-amygdalo-hippocampectomy which provides excellent results on seizure tendency with low morbidity.
Familial periodic ataxia responsive to acetazolamide
Authors: Bouchard J.P. , Roberge C. , Gelder N.M. van , Barbeau A. 
Published: July 2016

AbstractTwocases,afather and son, of recurrent cerebellarataxiain the same family are reported, suggestingafamilial trait for the dysfunction. In the older male the onset of each episode (30-90 min.) was signalled by dysarthria which then progressed towards gait ataxia; the son presented closely similar clinical symptoms. Physical examination and blood chemistry revealed no obvious neurological deficit or biochemical abnormalities, with the exception of I-III and III-IV evoked auditory wave interpeak latencies, which were found markedly abnormal on the left side in the father but not in the son; the EEG of both individuals showed some diffuse, slow wave abnormalities. A low dose of acetazolamide, 250 mg daily, has successfully repressed recurrence of the attacks over the past six months. Temporary withdrawal for 14 days of the carbonic anhydrase inhibitor in the father coincided with two observed ataxic episodes.
Distribution of apolipoprotein E phenotypes in Friedreich's ataxia
Authors: Bouthillier D. , Nestruck A.C. , Milne R. , Sing C.F. , Barbeau A. , Davignon J. 
Published: July 2016

AbstractAllelic polymorphism at the apolipoprotein E (apo E) gene locus (alleles ɛ2, ɛ3 and ɛ4) is responsible for the existence of 6 discrete electrophoretic phenotypes of plasma apo E. Since the presence of the ɛ2 allele in the genotype tends to be associated with higher triglyceride levels, a study was undertaken to determine if a higher frequency of this allele could account for the presence of higher plasma triglycerides in subsets of patients with Friedreich's Ataxia. The frequency of the apo E phenotypes was determined in 37 subjects with Friedreich's Ataxia and compared with that of 102 normolipidemic and 102 hyperlipidemic individuals. There was no increased prevalence of the E3/2 phenotype and the ɛ2 allele in the Friedreich's sample as is found in a hyperlipidemic sample. Furthermore, the ɛ2 subset did not have significantly higher plasma triglycerides than the non-ɛ2 subset and the hypothesis was rejected. On the other hand, there was a trend for a decreased frequency of the E4/3 phenotype in the Friedreich's sample relative to the hyperlipidemic group but the difference did not reach statistical significance. The apo E phenotype distribution was also measured in a smaller sample of Charlevoix-Saguenay disease; this led to the discovery of two siblings with the relatively rare E2/2 phenotype and unexpectedly low levels of plasma lipid and lipoprotein concentrations. Plasma apolipoprotein E concentrations in both diseases were within the normal range except for subjects bearing the E2/2 phenotype.
The Encephalopathy of Sepsis
Authors: Jackson Alan C. , Gilbert Joseph J. , Young G. Bryan , Bolton Charles F. 
Published: July 2016

ABSTRACT:Twelve fatal cases of encephalopathy associated with sepsis were examined in a ten-year retrospective study. The sources of infection and organisms isolated were variable. Six of the patients had focal neurologic signs; five had seizures. The level of consciousness varied from drowsiness to deep coma, and electroencephalograms revealed diffuse or multifocal abnormalities. Computed tomographic head scans and cerebrospinal fluid examinations were usually unremarkable. Eight patients had disseminated microabscesses in the brain at autopsy. Four patients had proliferation of astrocytes and microglia in the cerebral cortex, a feature associated with metabolic encephalopathies. Additional findings included cerebral infarcts, brain purpura, multiple small white matter hemorrhages, and central pontine myelinolysis. Although sepsis may cause encephalopathy by producing disturbances in cerebral synaptic transmission and cerebral energy production through a toxic mechanism, bacterial invasion of the brain with the formation of disseminated microabscesses is also an important cause.
Electrophysiological Disorders in Multiple Sclerosis and Optic Neuritis
Authors: Sanders E.A.C.M. , Reulen J.P.H. , Hogenhuis L.A.H. , van der Velde E.A. 
Published: July 2016

ABSTRACT:Visual evoked response (VER), auditory brainstem evoked response (ABER), somatosensory evoked response (SSER), blink reflex and electronystagmographic (ENG) investigative methods were applied to a group of 89 patients with Multiple Sclerosis (MS) and Optic Neuritis (ON). The MS patients were classified as definite (n = 31), probable (n = 31) and possible (n = 27).The aim of this study was to determine the diagnostic value of the five electrophysiological tests in MS. VER and ABER recordings were found to reveal the highest number of asymptomatic abnormalities (33 and 31 percent respectively). The combination of VER, ABER and ENG revealed all possible electrophysiological disorders. As these tests are completely non-invasive it is proposed, that a combination of two of these three tests is useful for the detection of a second silent lesion in patients with suspected MS showing purely spinal signs (VER, ENG, ABER) and/or a history of uncomplicated ON (ABER, ENG).
Monitoring of Sensory Evoked Potentials During Surgery of Skull Base Tumours
Authors: Gentili F. , Lougheed W.M. , Yamashiro K. , Corrado C. 
Published: July 2016

ABSTRACT:Despite advances in instrumentation and the use of microsurgical techniques, neurosurgical procedures involving extensive areas of skull base or other critical areas of brain still carry significant risk for neurological injury. The use of intraoperative recording of sensory evoked potentials (SEP) has been advocated to monitor neurologic function during these major neurosurgical procedures to reduce the risk of injury to neural structures.This report summarizes our experience with intraoperative monitoring of SEP in over 200 patients, and details our findings in a group of 12 patients with skull base and posterior fossa tumours. Somatosensory evoked potentials (SSEP) were monitored in all patients, and brain stem auditory evoked potentials (BAEP) in five. While minor changes in BAEP and SSEP parameters were noted in most patients, significant changes occurred in five. Irreversible loss of BAEP in one patient was associated with complete hearing loss postoperatively. Marked, persistent alteration of both BAEP and SSEP was associated with postoperative brainstem dysfunction. No patient with stable BAEP and SSEP at the end of the procedure suffered additional neurological deficit.We conclude that intraoperative SEP monitoring may be valuable in minimizing neural injury during major neurosurgical procedures.
Craniopharyngiomas
Authors: Hoffman Harold J. 
Published: July 2016

ABSTRACT:A review has been carried out of those patients with a craniopharyngioma who were treated initially in our institution and had a microsurgical excision of their tumour during the period of time when newer and sophisticated modalities of investigation and treatment were available. Twenty-nine patients with craniopharyngioma were treated initially by microsurgical excision of their tumour at the Hospital for Sick Children from January 1976 to June 1985. In 21 a total removal of the tumour was performed, and there has been no clinical evidence of recurrence. No deaths were recorded in any of the 29 patients. The principal morbidity has resulted from endocrine deficits that these patients exhibit.
Intrathecal Synthesis of Autoantibodies to Myelin Basic Protein in Multiple Sclerosis
Authors: Catz Ingrid , Warren Kenneth G. 
Published: July 2016

ABSTRACT:A solid phase radioimmunoassay was used to detect anti-myelin basic protein (MBP) antibodies in the CSF and serum of multiple sclerosis (MS) patients and controls. CSF and serum samples were assayed prior to acid hydrolysis in order to detect free anti-MBP as well as after acid hydrolysis to measure the total (free and bound) amount of antibody. An anti-MBP index controlling for serum levels as well as the degree of breakdown of the blood brain barrier was used to estimate intrathecal synthesis of anti-MBP. MS patients with acute exacerbations or chronically progressive disease have significantly elevated levels of both free and total CSF anti-MBP. The anti-MBP index is also significantly increased in MS patients with both forms of active disease. Anti-MBP antibodies are intrathecally produced in MS patients with active disease.
High Dose Anticholinergic Therapy in Adult Dystonia
Authors: Lang Anthony E. 
Published: July 2016

ABSTRACT:Forty-four adult patients with various forms of dystonia were treated with anticholinergics slowly increased to maximum tolerated doses. Thirty-seven per cent of the 35 idiopathic dystonia patients had a moderate to marked improvement with an average dosage of 21.5 mg of trihexyphenidyl. Younger patients with a shorter duration of dystonia and those who tolerated higher doses tended to benefit most. However there were exceptions to all of these factors. None of the nine patients with symptomatic dystonias improved more than mildly and most had no benefit despite the use of dosages similar to those resulting in improvement in idiopathic dystonia patients. Side effects were common. These often forced drug withdrawal at lower doses than those which might have resulted in improvement. These results suggest that high dose anticholinergics should be the first line of therapy for disabling dystonia.
The Concurrence of Multiple Sclerosis and Amyotrophic Lateral Sclerosis
Authors: Hader Walter J. , Rozdilsky Bohdan , Nair Cheranada P. 
Published: July 2016

ABSTRACT:We report the clinical and pathological findings of the unusual combination of two idiopathic central nervous system diseases, multiple sclerosis and amyotrophic lateral sclerosis in a 56 year old physician with a twenty-seven year history of a disease initially characterized by relapses and remissions, followed by an eight year quiescent period. During the last year of life there was rapid deterioration with development of generalized weakness, atrophy, weight loss and fasciculations of body and tongue, and associated difficulty with swallowing and sudden respiratory failure. The autopsy confirmed characteristic “burned out” plaques of multiple sclerosis and anterior horn cell and axonal degeneration of amyotrophic lateral sclerosis.
Modification of Motor Output to Compensate for Unanticipated Load Conditions During Rapid Voluntary Movements
Authors: Lee R.G. , Lucier G.E. , Mustard B.E. , White D.G. 
Published: July 2016

ABSTRACT:Mechanisms responsible for load compensation during fast voluntary movements were investigated in 20 normal subjects trained to carry out rapid wrist flexions against a standard load. When an unanticipated increase in load occurred, there was a compensatory increase in agonist EMG and decrease in antagonist EMG. Unanticipated decreases in load produced reciprocal changes with a decrease in agonist EMG and an increase in antagonist EMG. The latency of these EMG changes was quite short and compatible with a spinal reflex mechanism rather than a long loop response. The results suggest that mechanisms exist at the spinal level to allow rapid modification of motor programs when unanticipated load conditions are encountered on initiation of movement.
Optic Neuritis and Myelopathy in Systemic Lupus Erythematosus
Authors: Oppenheimer Stephen , Hoffbrand B.I. 
Published: July 2016

ABSTRACT:The optic neuritis of systemic lupus erythematosus (S.L.E.) more frequently results in the persistence of a central scotoma or complete blindness after a single attack than demyelinating optic neuritis, although the initial clinical presentations may be identical. A significant number of patients, however, recover normal vision. Optic neuritis may be the presenting symptom of S.L.E. and as myelopathy may also occur in the course of the disease, confusion with multiple sclerosis may result, especially if there are no arthritic, cutaneous nor visceral manifestations. We report a case of lupus optic neuritis associated with anticardiolipin antibodies and a circulating lupus anticoagulant and suggest these may be a marker for vasculitic optic neuritis and play a role in its aetiology.
Free and Total Serum Valproate Concentrations: Their Relationship to Seizure Control, Liver Enzymes and Plasma Ammonia in Children
Authors: Farrell Kevin , Abbott Frank S. , Orr James M. , Applegarth Derek A. , Jan James E. , Wong Peter K. 
Published: July 2016

ABSTRACT:The relationships between total and free serum valproate (VPA) concentrations and seizure control, serum liver enzyme activity and plasma ammonia concentration were studied in 61 epileptic children. Enzymeimmunoassay (EMIT)Rmethods gave higher values of total VPA concentration than gas-liquid chromatography (GLC) methods. In over 80% of children with complete seizure control the ranges of total VPA concentration were 140-420 umol/L with GLC methods and 210-560 umol/L with EMIT methods. The range of free VPA concentrations in 78% of children with complete seizure control was 8.8-26.4 umol/L. Increased liver enzyme activity was observed in 6 of the 61 children and raised plasma ammonia concentration in 11 of 50 children. Plasma ammonia concentration was related to total serum VPA but was not related to free serum VPA. Increased serum liver enzyme activity was related to VPA dose per kg but not to free or total serum VPA concentration. Thus free VPA concentrations do not appear to be more useful than total VPA concentrations in predicting seizure control and do not correlate with liver enzyme activity or plasma ammonia concentration.
Early Onset Parkinson's Disease in Saskatchewan - Environmental Considerations for Etiology
Authors: Rajput A.H. , Uitti Ryan J. , Stern W. , Laverty W. 
Published: July 2016

ABSTRACT:The cause of idiopathic Parkinson's Disease (PD) is not known but it is believed to be related to some environmental agent(s). Given a long preclinical interval and onset of symptomatology around age 60 years, it becomes impossible to identify and analyze all prior environmental factors satisfactorily. To circumvent these difficulties we evaluated the childhood environment in those PD patients whose symptoms began at age 40 years or earlier. Twenty-one such cases were born and raised in the province of Saskatchewan. Nineteen of these 21 patients spent the first 15 years of life exclusively in rural Saskatchewan. Detailed population analysis indicates a strong predisposition to early onset idiopathic Parkinson's Disease (EPD) in those raised in rural areas (p = 0.0154). All but one case utilized exclusively well water for the first 15 years of life — a trait significantly different from that expected in the provincial population. It is concluded that rural Saskatchewan environments contribute to EPD and that well water used in childhood should be considered as a potential vehicle for the etiological agent.
The Age of Onset of Parkinson's Disease: Etiological Implications
Authors: Teräväinen H. , Forgach L. , Hietanen M. , Schulzer M. , Schoenberg Bruce , Calne D.B. 
Published: July 2016

ABSTRACT:We have conducted a hospital-based survey of the age-specific prevalence of Parkinson's disease in 551 patients from Helsinki and Vancouver. We conclude that the disorder may be starting earlier than previously and we discuss the implications of this finding for the etiology of Parkinson's disease.
Inclusion Body Myositis Associated with Systemic Sarcoidosis
Authors: Danon Moris J. , Perurena Osvaldo H. , Ronan Salve , Manaligod Jose R. 
Published: July 2016

ABSTRACT:We report an autopsy study of a 64-year-old female with systemic sarcoidosis. In addition many muscles showed typical light and electron microscopic features of inclusion body myositis. To our knowledge this association has not been previously reported.
Neurological Abnormalities Associated with Severity of Dementia in Alzheimer's Disease
Authors: Huff F. Jacob , Growdon John H. 
Published: July 2016

Abstract:Abnormal findings on neurological examination were evaluated in 165 patients with a clinical diagnosis of Alzheimer's disease who attended a memory disorders clinic. Severity of dementia was measured by the Blessed Dementia Scale, and associations of abnormalities with dementia severity were evaluated using logistic regression. Presence of aphasia, apraxia, and primitive reflexes on neurological examination were strongly associated with severity, and weak associations were observed for abnormalities of muscle tone and gait. Among these associated neurological features, only aphasia and apraxia were present in mildly demented cases with sufficient frequency to suggest utility as diagnostic signs early in the course of the disease.
Cortical Biopsy Results in Alzheimer Disease: Correlation with Cognitive Deficits
Authors: Martin E.M. , Wilson R.S. , Penn R.D. , Fox J.H. , Clasen R.A. , Savoy S.M. 
Published: July 2016

Abstract:This paper reports neuropsychologic and pathologic data for eleven patients with a clinical diagnosis of probable Alzheimer disease (AD) according to recently proposed NINCDS criteria. In all cases, the clinical diagnoses were verified by cortical biopsy using histopathologic criteria for definite AD. Similar tissue samples from nine non-demented autopsied patients were also evaluated and none received an histopathologic diagnosis of AD. Correlations between cortical plaque counts and neuropsychologic test scores are also presented. These data have important implications for 1) the accuracy of clinical diagnosis in AD; 2) the validity of NINCDS criteria for the clinical and pathologic diagnosis of AD; and 3) the utility of cognitive test scores as indicators of disease severity.
Early Identification of Dementia
Authors: McGlone J. , Gupta S. 
Published: July 2016

Abstract:Currently the diagnosis of progressive organic dementia is one of exclusion offered only after cognitive decline is well advanced. Early in the disease process, memory complaints most often bring elderly people to the attention of their family physician .This behaviour represents the first step in the process of giving a patient the medical diagnosis of organic dementia, yet no systematic method has been developed to gather such subjective information and evaluate its medical significance. We are developing a memory complaints questionnaire and a brief non-threatening memory screening instrument to help identify organic dementia in its earliest stages. The screening device will be related to more sophisticated neuropsychological measures of dementia and it should be sensitive to mental deterioration over short periods of time. The instruments will document perceived and objective changes in memory and will help the physician attribute their findings to normal aging, depression and/or degenerative CNS disease.
Cognitive Assessment in Alzheimer's Disease: Cross-Sectional and Longitudinal Perspectives
Authors: Kaszniak Alfred W. , Wilson Robert S. , Fox Jacob H. , Stebbins Glenn T. 
Published: July 2016

Abstract:This paper describes select results of a longitudinal study of 62 mild to moderate Alzheimer's disease (AD) patients, in comparison to 60 age-matched healthy controls. Initial neurologic, radiologic, psychiatric, laboratory and cognitive examinations, required two full days, followed by one-day examinations at annual intervals. Of the total original sample, 31 AD patients and 39 controls could actually be followed for three annual examinations. Cognitive examination data confirmed cross-sectional (group discriminative) validity of memory and language measures, and showed the expected longitudinal deterioration in the AD sample, with controls maintaining consistent performance over the three years. However, those measures showing largest group differences at initial examination were not the best for tracking patient deterioration over time. Implications of these results for the selection of cognitive assessment measures are discussed.