bluuebunny/crossref_metadata_2025_split huggingface dataset:
Speculations on Disease States Induced by Excitatory Amino Acids
Authors: Turnbull John 
Published: July 2016

Abstract:There is much current interest in excitatory amino acids and their receptors because of their postulated involvement in several disorders of the nervous system. They function as neurotransmitters, but can act as neurotoxins in some situations. They have been implicated in the pathogenesis of cerebral hypoxic/ischemic and hypoglycemic damage, in epilepsy, in some degenerative diseases, and in some forms of neurotoxin-induced cerebral dysfunction. These diseases may reflect abnormality in a system which has evolved to provide synaptic plasticity essential for learning and memory. The purpose of this paper is to explore the ramifications of such a hypothesis.
Peripheral Neurological Complications of Aortoiliac Vascular Disease
Authors: D'Amour Monique L. , Lebrun Louise Hélène , Rabbat Antoine , Trudel Jules , Daneault Nicole 
Published: July 2016

Abstract:Six patients with an aortoiliac vascular disease and a peripheral neurological deficit are presented. Clinical and electromyographic findings revealed lumbosacral plexus, sciatic and femoral nerve lesions. A correlation is made between the level of the vascular lesion (aortic, aortoiliac or distally) and the type of peripheral nerve deficit observed. In a patient complaining of pain, weakness, or numbness in a leg, the differential diagnosis should include aortoiliac vascular disease. The peripheral neurological symptoms may be the initial manifestation of the vascular disease or may appear in the early post-operative period.
Metrizamide CT Scanning in Spinal Nerve Root Cysts
Authors: Goyal R.N. , Russell N.A. , Belanger J.M.E.G. , Benoit B.G. , Rawa M. 
Published: July 2016

Abstract:Two cases of lumbosacral root cysts of different etiology are reported. Their specific radiographic features are described using the combined technique of metrizamide myelography followed by computerized tomography of the spine. The terminology of intraspinal cysts is reviewed and their distinguishing features discussed.
Acetylcholine Receptor Antibodies in Myasthenia Gravis: Use of a Qualitative Assay for Diagnostic Purposes
Authors: Oger Joel , Kaufman Robin , Berry Kenneth 
Published: July 2016

ABSTRACT:We have modified the techniques of Lindstrom and of Tindall to measure serum acetylcholine receptor antibody using human antigen bound to 125I-alpha Bungarotoxin. By using 10 ä1 of serum and precipitating antigen-antibody complexes with an excess of staph A, we found that only one out of 43 patients with clinically diagnosed active generalized Myasthenia Gravis had no antibodies. In pooling these results with the results of tests done for diagnostic purposes we found positive results in 54/55 generalized active MG, 8/21 MG in remission, 16/37 ocular MG and 0/55 healthy controls. Two out of 38 non MG were also positive and their clinical diagnosis of botulism and penicillamine treated rheumatoid arthritis have been confirmed by a one year follow-up. Most of these sera were also tested for reactivity with fetal calf AchR. Six out of 49 samples positive with the human receptor were negative with calf receptor. We conclude that our technique is extremely useful for the diagnosis of Myasthenia Gravis and that fetal calf antigen cannot replace human antigen in the assay.
Familial Parkinson's Disease: Possible Role of Environmental Factors
Authors: Calne Susan , Schoenberg Bruce , Martin Wayne , Uitti Ryan J. , Spencer Peter , Calne D.B. 
Published: July 2016

ABSTRACT:We report here six families with Parkinson's disease in whom the onset of symptoms tended to occur at approximately the same time irrespective of the age of the patient. The mean difference in the time of onset in different generations was 4.6 years while the mean difference in age of onset in children and parents was 25.2 years. We construe this pattern of age separation within families as suggestive of an environmental rather than genetic cause. Support for this view derives from the lack of correlation between occurrence of the disease and the degree of consanguinity. We conclude that our findings are in accord with the hypothesis which attributes the cause of some cases of Parkinson's disease to early, subclinical environmental damage followed by age-related attrition of neurons within the central nervous system.
Interpretation of Single Band Patterns in CSF Protein Electrophoresis
Authors: Bass B.H. , Armstrong H. , Weinshenker B. , Ebers G.C. , Noseworthy J.H. , Rice G.P.A. 
Published: July 2016

ABSTRACT:We have examined the diagnostic significance of finding one band in the immunoglobulin (IgG) region in spinal fluid protein electrophoresis. From January 1983 to January 1986, 855 consecutive CSF electrophoreses were performed on as many patients. A blinded observer identified a single band in the IgG region in 53 cases (6.2%). In only 14 patients (26%), were the clinical features ultimately felt to be due to clinically definite or possible multiple sclerosis (MS). The majority of patients with a single band had another neurological diagnosis (55%) or were neurologically normal (6%). Many of the neurological disorders in which a single band was found were not disorders in which an increased intrathecal synthesis of immunoglobulin or electrophoretic restriction would have been expected. A variety of conditions can produce a single band pattern. The significance of these patterns and the means by which they might be identified are described.
Gamma-Aminobutyric Acid (GABA) and Sepsis-Related Encephalopathy
Authors: Winder T.R. , Minuk G.Y. , Sargeant E.J. , Seland T.P. 
Published: July 2016

ABSTRACT:In order to determine whether disturbances in GABA homeostasis might play a role in the pathogenesis of sepsis-related encephalopathy, serum and brain tissue GABA concentrations from six areas of the brain (cortex, diencephalon, striatum, hippocampus, midbrain, and pons-medulla) were determined in a rat model of bacterial sepsis (cecal ligation and perforation). The results were compared to those obtained from sham operated control animals. All septic animals demonstrated clinical signs of encephalopathy and had elevated serum GABA levels (0.92 ± 0.3 uM versus 0.48 ± 0.15 in controls, p < 0.01). GABA content in the specific subcompartments of the brain, however, were similar in the two groups. These results indicate that although serum GABA levels are elevated during sepsis, GABA is unlikely to play an important role in the pathogenesis of sepsis-related encephalopathy.
Lymphocytic Adenohypophysitis
Authors: McDermott Michael W. , Griesdale Donald E. , Berry Kenneth , Wilkins G. Edward 
Published: July 2016

ABSTRACT:Lymphocytic adenohypophysitis (LAH) is an uncommon disorder in the spectrum of pituitary disease. Twenty-three cases proven by biopsy or at autopsy have been reported since 1962. We report 2 further cases and review the etiology, immunology and pathology of the disease. The diagnosis should be considered in afemale patient who presents during the post-partum period with the clinical picture of a non-functional or prolactin cell pituitary adenoma and evidence of hypopituitarism.
Treatment of Syringomyelia with a Syringosubarachnoid Shunt
Authors: Tator Charles H. , Briceno Carlos 
Published: July 2016

ABSTRACT:The surgical results in 40 patients with syringomyelia, treated with a syringosubarachnoid shunt or other procedures are reviewed. The principal indication for surgery was that of significant neurological deterioration. There were 12 patients with idiopathic syringomyelia without tonsillar ectopia, 12 with an associated Chiari malformation, 11 with post-traumatic syringomyelia and five patients with spinal arachnoiditis. There were 38 syringosubarachnoid shunts performed in 35 patients, and an excellent or good result was achieved in 26 patients (74.3%). In terms of the type of syringomyelia, the best results were obtained in the idiopathic group without tonsillar ectopia and in the post-traumatic group. A short duration of pre-operative symptoms favoured a better outcome, and in our opinion, early surgical treatment is indicated for all patients with neurological deterioration. All eight patients in whom a posterior fossa decompression was performed as the initial surgical procedure required a second operation, either a syringosubarachnoid or syringoperitoneal shunt to achieve neurological improvement or stabilization. Thus, the syringosubarachnoid shunt is an effective therapeutic modality for patients with syringomyelia, particularly for the idiopathic and post-traumatic groups. More than one surgical procedure may be required to achieve cessation of deterioration. Overall, excellent or good results were achieved in 29 (72.5%) of the 40 patients.
Spectral Analysis of the EEG in Craniocerebral Trauma
Authors: Moulton Richard J. , Marmarou Anthony , Ronen Jacob , Ward John D. , Choi Sung , Lutz Harry A. , Byrd Steven , Desalles Antonio , Maset Angelo , Muizelarr J. Paul , Young Harold F. 
Published: July 2016

ABSTRACT:The objectives of the present study were to evaluate the relationship between the fractional amplitudes of the EEG derived from power spectral analysis (PSA) of the electroencephalogram (EEG) and depth of coma measured clinically with the Glasgow Coma Score, and to assess the accuracy of PSA in predicting long-term outcome. Thirty-two patients rendered unconscious by blunt head injury (mean (GCS = 7) had intermittent EEG recordings daily from 1-10 days post injury. There was a significant correlation between fractional amplitude of the EEG and the GCS. The rate and magnitude of change in the EEG and GCS were also correlated. There were significant differences in PSA parameters between improved and deteriorated patient groups at the termination of monitoring (p = .02) and in the change of PSA parameters over time (p = .02). Using linear discriminant analysis of PSA parameters, the accuracy of outcome prognostication based on the six month outcome was approximately 75%. Accurate classification of outcome was possible in a number of patients in whom there was little or no change in the GCS during the period of monitoring.
Idiopathic Parkinson's disease: Revised Concepts of Cognitive and Affective Status
Authors: Taylor Ann E. , Saint-Cyr J.A. , Lang A.E. 
Published: July 2016

ABSTRACT:Assumptions regarding increased risk of dementia in Parkinson's disease and of depression mimicking the endogenous form are reviewed and challenged from the perspectives of recent findings in both the neuropsychological and anatomical domains. Evidence suggests that depression, while frequent, behaviourally resembles the reactive variety and that selective impairment of cognitive functions considered to depend upon the integrity of the frontal lobes accompanies this disorder. In this regard, it is speculated that the cognitive alterations seen in non-demented parkinson patients are the consequences of dysfunction of the caudate nucleus which contributes significantly to the normal activities processed through the frontostriate “complex loop”.
Familial Alzheimer's Disease
Authors: Sadovnick A.D. , Tuokko H. , Horton A. , Baird P.A. , Beattie B.L. , Spiegel Sanford M. 
Published: July 2016

ABSTRACT:A family with familial Alzheimer's disease (FAD) inherited as an apparent autosomal dominant trait is presented. Twelve individuals (6 females; 6 males) in 4 generations were affected. The disease had its onset in the late 30's, early 40's with death by age 50. Although FAD which appears to be transmitted as an autosomal dominant trait is relatively rare, such families must be identified and carefully counselled with respect to recurrence risks for subsequent generations. In this family, there are currently 20 members of Generation IV, aged 15-37, and 9 members of Generation V, aged 1-11. The majority of these individuals appear to have a 50% risk for developing this disease.
The Effects of Myoinositol on the Autonomic Neuropathy in the Streptozotocin Diabetic Rat - A Freeze Fracture Study
Authors: Monckton G. , Marusyk H. 
Published: July 2016

ABSTRACT:This report describes a freeze fracture study of rat dorsal sympathetic chain in normal, streptozotocin diabetic on normal diet, and a group fed a 1% myoinositol normal diet. In nonmyelinated fibres, the diabetic group had significant loss of particles on the P face ofjuxta-axonal Schwann cell membranes, whilst myelinated nerves showed a profound loss of particles on P and E faces. In the group with adjuvant myoinositol the particle numbers were normal in both types of nerve. Axonal plasma membranes showed increased numbers of particles in the P face of the nonmyelinated membranes in the diabetic, but were normal in the myoinositol group. Myelinated axonal membranes showed no significant difference between the diabetic and the normal. Rats on a myoinositol diet showed a significant decrease in the E face particles. These results are consistent with those described in the sciatic myelinated nerves by earlier authors and reinforce the view that myoinositol is an important membrane constituent as phosphatidyhnositol and is essential for the expression of normal protein particle numbers.
Magnetic Resonance Imaging of Meningio-Angiomatosis
Authors: Kuzniecky Ruben , Melanson Denis , Robitaille Yves , Olivier André 
Published: July 2016

ABSTRACT:We report the case of a patient with meningio-angiomatosis, a rare disorder of the cerebral cortex often associated with Von Recklinghausen's neurofibromatosis. Previous reports have described a variety of radiological findings but in general they have been of little value in the diagnosis of this disorder. We describe the magnetic resonance image, which identified a well-defined lesion. The imaging techniques are reviewed.
An Unusual Subacute Progressive Motor Neuronopathy with Myasthenia-like Features
Authors: Noseworthy J.H. , Rae-Grant A.D. , Brown W.F. 
Published: July 2016

ABSTRACT:The initial presentation and clinical course of this 60-year old woman suggested a diagnosis of myasthenia gravis. The subsequent development of tongue fasciculations and the lack of response to treatment made a diagnosis of amyotrophic lateral sclerosis (ALS) more likely despite the presence of conjugate gaze paresis and the absence of many of the typical clinical and electromyographic (EMG) findings seen in this condition. The pathological findings were consistent with either a motor neuronopathy or an unusual variant of ALS. We review the clinical and pathological features of this unusual case in this report.
Sphenoethmoidal Sinusitis Complicated by Cavernous Sinus Thrombosis and Pontocerebellar Infarction
Authors: Macdonald Robert L. , Findlay J. Max , Tator Charles H. 
Published: July 2016

ABSTRACT:An unusual case of sphenoethmoidal sinusitis complicated by cavernous sinus thrombosis, meningitis and pontine and cerebellar infarction is described. The patient presented with advanced intracranial complications which in retrospect caused delay in recognition and treatment of the underlying sphenoethmoidal sinusitis. Surgical drainage of the sinusitis was ultimately required. The pathogenesis of these complications is discussed, and the topic of sphenoid sinusitis reviewed in order to emphasize the numerous neurological manifestations of this disease.
Epilepsy: Relationships Between Electrophysiology and Intracellular Mechanisms Involving Second Messengers and Gene Expression
Authors: Gloor P. 
Published: July 2016

ABSTRACT:It is well known that pure absence epilepsy is a benign form of seizure disorder, while most others, particularly partial and convulsive seizures may have transient or permanent deleterious consequences and are more difficult to bring under therapeutic control by anticonvulsants. The hypothesis is proposed that the preservation of GABA-ergic inhibition in absence attacks and its breakdown in most other seizures may explain these differences. Breakdown of GABA-ergic inhibition allows NMDA receptors to become active. This opens the way for Ca2+ to enter the cell. Such Ca2+ entry is a long-lasting phenomenon. It is likely to be massive during most seizures except during absence attacks, and may therefore damage the neuron transiently or permanently. It may even destroy it. Ca2+ entry is also a crucial factor in the activation of the second messenger cascade which involves cytosolic as well as nuclear (genomic) components. Activation of this cascade converts short-lived electrophysiological processes occurring at the membrane into much longer-lasting intracellular processes. These may include plastic changes at the synaptic and receptor level and may account for kindling and the increasing therapy-resistance of long-standing seizure disorders. Changes resulting from massive Ca2+ entry into the neuron may explain why most seizures, except absence attacks, may have deleterious consequences of various kinds, some short-lived, some of longer duration, and some even permanent.
An Update on the Cellular and Molecular Biology of Brain Tumors
Authors: Cusimano Michael D. 
Published: July 2016

ABSTRACT:Patients with tumors of the central nervous system (CNS) remain difficult to treat despite recent advances in surgical, chemotherapeutic and radiotherapeutic techniques. A better understanding of the molecular and cellular biology of neoplasia is providing neuroscientists with a framework on which to devise novel therapies for these patients. It thus becomes imperative that neurologists and neurosurgeons be aware of these advances in basic science that may eventually have a positive impact on patient management. This paper reviews our present knowledge of the process of CNS oncogenesis and the roles that chemicals, viruses, oncogenes, growth inhibitor genes, and growth factors play in the process.
Partially Sequestered Fourth Ventricle: CT and MR Diagnosis of an Unusual Entity
Authors: Wagle V.G. , Melanson D. , Villemure J.G. 
Published: July 2016

ABSTRACT:Encystment of the fourth ventricle, due to occlusion of the aqueduct as well as the foramina of Magendie and Luschka has been described previously. Partial sequestration such as that encountered in two cases described is a less common entity. In these two cases, the aqueduct of Sylvius was occluded, but the basal foramina (Magendie and Luschka) were patent. We discuss this partial sequestration or communicating hydrocephalus of the fourth ventricle on the basis of MR and CT scan findings. Although computed tomography following water soluble cisternography did diagnose the entity indirectly MRI proved to be a superior tool. It permitted direct visualization of the basal foramina noninvasively.
La mère et l'enfant atteints de dystrophie myotonique de Steinert
Authors: Paris G. , Laframboise R. , Bouchard J.-P. 
Published: July 2016

ABSTRACT:The Mother and Infant with Myotonic Dystrophy Pregnancy and delivery present a number of risks for the mother suffering from myotonic dystrophy, and for her infant. Most of the time, she does not even know that she is affected by the disease and a carrier of the gene. We review the complications of pregnancy and delivery in myotonic patients, and propose a simple management with specific items for each gestational period. The child of a dystrophic mother has a 50% risk of inheriting the abnormal gene. He may also exhibit a developmental and malformation syndrome called "congenital myotonic dystrophy". From the beginning, he may show respiratory distress, thereafter inability to swallow and severely hypotonia. Later, he may demonstrate mental retardation. Some of the most obvious signs found in neonates in our practice are illustrated. We also add a few tests to the list of those already recommended for these children.
La dystrophie myotonique: II nuptialité, fécondité et transmission du gène
Authors: Veillette Suzanne , Perron Michel , Mathieu Jean 
Published: July 2016

ABSTRACT:Myotonic Dystrophy: II Marriage, Fertility and Gene Transmission. A sociological study, using a representative sample (N = 218) of the myotonic dystrophy population, made possible an analysis of the relationship between certain demographic characteristics (marriage, fertility) and the mechanisms involved in the transmission of the deleterious gene. The results show a clear differentiation between the marriage rate of women and men affected by myotonic dystrophy. Whereas men show a considerable decline in marriage eligibility, women continue to marry at a young age and in a proportion almost equal to that of the unaffected population. The study also indicates that the average fertility rate among married patients in the Saguenay-Lac-Saint-Jean exceeds the fertility rate reported from affected populations found in other countries. Our study shows an above-normal male fertility rate. This demographic fact explains the overrepresentation of male transmitters noted in the affected population. However, if present trends remain unchanged, women will be more likely to transmit myotonic dystrophy to future generations. These conclusions illustrate how the transmission and spread of a dominant gene follow a pattern that cannot be dissociated from the socio-cultural characteristics taken as a whole, particularly demographic characteristics. They also provide us with useful avenues for setting up future prevention programmes.
Myotonic Dystrophy in Quebec: Geographical Distribution and Concept of Genetic Homogeneity
Authors: Laberge Claude 
Published: July 2016

ABSTRACT:The geographical distribution relative to place of residence of patients with myotonic dystrophy (MD) and admitted to a Quebec hospital during a five year period (1980-1984) is presented and discussed. The sample consists of 72 males and 68 females of varying ages over 10 years. Analysis of the data shows a North Shore distribution of patients in a cline from Saguenay-Lac-St-Jean, through Quebec City and to Montreal. However, a low prevalence is apparent on the South Shore, east of Québec City, for which an historical and genealogical explanation are discussed. This geographic distribution favours the hypothesis of genetic homogeneity for the MD gene in the Quebec population. A stronger second argument comes from genealogical studies of 10 families sampled from the Chicoutimi Muscular Dystrophy Clinic. Genealogical paths traced to ancestors who founded Charlevoix for these 10 families go back to a cluster of 25 founders, one of whom must have been the carrier of the MD gene. The probative third argument for genetic homogeneity comes from the allellic distribution of the apolipoprotein E (ApoE) gene in the Québec City, Saguenay and in families with MD. The ApoE locus is on chromosome 19 and closely linked to MD. In MD-affected individuals there is a linkage disequilibrium for the e4 allelle while non-MD members of these families show allellic frequencies not differing significantly from the control population. This suggests that the gene that is carried by all MD patients (at least in Northeast Québec) came through a “founder effect” and was carried in -cis on the chromosome 19 of the first carrier. Consequences of this genie homogeneity in relation to studies of penetrance and phenotypic variation in clinical expressivity are discussed.
Cerebral Edema Associated with Meningioma
Authors: Lee Shih-Tseng , Hsueh Swei 
Published: July 2016

ABSTRACT:Cerebral edema associated with meningiomas is a rather common phenomenon. A patient with a small meningioma presented with severe cerebral edema, out of proportion to the size of the tumor. During surgery the meningioma was removed and the adjacent edematous brain tissue biopsied. The histopathoiogical study showed conspicuous plasma cell and lymphocyte infiltration in both the tumor and the cerebral edematous tissue. The pathological findings are presented, and the possible mechanism for cerebral edema associated with meningioma is discussed.