bluuebunny/crossref_metadata_2025_split huggingface dataset:
Isolated Spinal Cord Arteritis
Authors: Feasby Thomas E. , Ferguson Gary G. , Kaufmann J.C.E. 
Published: July 2016

SUMMARY:This patient presented as a subacute progressive cervical myelopathy and the differential diagnosis included cervical spondylotic myelopathy and intramedullary mass. Microscopically, vascular lesions plus a patchy myelomalacia indicated a vasculitis. However, there was no suggestion of a generalized vasculitis at autopsy and the only supporting laboratory study was a raised erythrocyte sedimentation rate. It would seem that a vasculitis similar to polyarteritis nodosa or other collagen disease may be confined to the spinal cord.
Alpha Methyldopahydrazine as an Adjunct to Levodopa Therapy in Parkinson’s Disease
Authors: Paty D.W. , Jaatoul N. , Kertesz A. , McInnis W. 
Published: July 2016

SUMMARY:A double-blind, double-observer study was carried out in twenty-five patients with Parkinson’s disease. Alpha methyldopahydrazine in combination with L-dopa was compared to placebo with L-dopa. Combination therapy resulted in a reduction in L-dopa dosage to 1/3 of the amount required during the baseline. There were no side effects attributed directly to the alpha methyldopahydrazine. The overall incidence of side effects in the two groups was similar but the combination therapy significantly reduced the incidence of nausea and vomiting. The limiting factor in the combination therapy was the presence of L-dopa induced dyskinesias.
Analysis of 128 Patients with Angiogram in Acute Head Trauma
Authors: Cinesi M.P. , Miller J.D.R. , Grace M. , Ayers T.N. 
Published: July 2016

SUMMARY:A computerized analysis of 128 patients admitted with acute head injury and who underwent angiography is shown. Patients were divided into groups according to: age, sex, type of accident, state of consciousness and presence of localizing signs on admission, types of cerebral lesions on angiography, and discharge condition.There is a preponderance of young males in this series of patients, related mainly to MVA. A total of 71% of the patients had abnormal angiograms, but the incidence of normal and abnormal results did not correlate significantly with any of the chosen parameters.The same parameters were also analysed to assess their value as a prognostic index for the patient. The conclusion was drawn that the angiogram per se has no significant value as a prognostic tool, and that state of consciousness on admission is the best single index for prognosis.
Memory and Growth in the Superior Temporal Gyri
Authors: Akesson E.J. , Dahlgren W.J. , Hyde J.B. 
Published: July 2016

SUMMARY:The superior temporal gyri were measured in 33 infants and in 33 adults. In the adults, most right superior temporal gyri were larger. This asymmetry was not found in infants, a difference which suggests greater growth of the right superior temporal gyri in the population from which our sample was taken. The asymmetry may be related to the functional asymmetry found by Penfield: some of his patients reported re-experiencing of past sensory experiences with electrical stimulation of the temporal lobe. This response was more frequently evoked from the right hemisphere.
Cucumber Shaped and 35 nm Particles in Creutzfeldt-Jakob Disease
Authors: Horoupian Dikran S. , Ross R.T. 
Published: July 2016

SUMMARY:A 63 year old female with the ataxic form of Creutzfeldt-Jakob disease (CJD) is presented. In addition to amyloid plaques which were not associated with Alzheimer’s neurofibrillary tangles, rare profiles similar to those reported in Scrapie were also seen. To our knowledge, these profiles have never been observed in CJD and their presence in this condition adds a further morphologic similarity between the human and animal forms of subacute spongiform “viral” encephalopathies.
Roles of Cerebellum and Basal Ganglia in Initiation and Control of Movements
Authors: Brooks V.B. 
Published: July 2016

SUMMARY:Theories of function of the cerebellum and basal ganglia are examined in the light of recent experimental findings obtained with the local cooling method, and both are matched against clinical observations. Evidence is summarized for a programming and initiating role in monkeys’ elbow movements of the lateral, and to a lesser degree, intermediate, cerebellum. Cooling either nuclei affected movements, but neither seemed to be important for precentral cortical unit discharge accompanying compensation for suddenly applied load pulses. The globus pallidus seemed to be importantly involved in movement guidance in the absence of vision.
Biochemical Markers for Huntington's Chorea
Authors: McLean D.R. , Nihei T. 
Published: July 2016

SUMMARY:The uptake of dopamine (DA) by platelet rich plasma was assayed in 11 patients with Huntington's chorea (H. C). The results confirmed the previous observation that platelets from H.C. patients take up, at equilibrium, more dopamine than do platelets from normal control subjects. The mean difference was 50% higher at DA substrate concentrations of 0.11 mM.However, attempts to confirm the higher Na+- K+ATPase activity of erythrocyte ghosts from Huntington's chorea patients were unsuccessful.
Study of a Family with Progressive Ataxia, Tremor and Severe Distal Amyotrophy
Authors: Bouchard J. , Bedard P. , Bouchard R. 
Published: July 2016

SUMMARY:We have studied a large family of which seven members suffer from a progressive disease with onset in the first decade. The first symptoms were gait ataxia and clumsiness in all cases, followed by progressive development of severe distal amyotrophy reminiscent of Charcot-Marie-Tooth disease. In four patients a postural tremor which was relieved by pharmacological agents was also evident in the limbs or head.Cerebellar atrophy was confirmed on CT scan. Motor nerve conduction velocities were in the low normal range, while sensory nerve conduction was markedly decreased. All patients had impaired proprioception and vibration sense. The laboratory investigation revealed a normal CSF protein level and elevated serum bilirubin.The patients reported in this study apparently suffer from an original recessive form of spinal and olivocerebellar degeneration associated with a neuronal form of Charcot-Marie-Tooth disease.
A Preliminary Study of Dynamic Muscle Function in Hereditary Ataxia
Authors: Richards C. , Bouchard J.P. , Bouchard R. , Barbeau H. 
Published: July 2016

SUMMARY:Dynamic muscle function was evaluated in nine patients with Friedreich's ataxia (FA) and eight with autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS). The measurement of torque throughout maximum voluntary isokinetic knee movements was used to quantitatively describe muscle weakness in the ataxic patients. Both FA and ARSACS patients were shown to have decreased dynamic strength in comparison to normal values during knee extension and flexion movements at 30% /s. In the FA patients a lower torqueproducing capacity was seen in the older patients.The electromyographic (EMG) activity was recorded in lower extremity muscles during the movements. In the vastus lateralis (VL), deviations from the normal EMG activation pattern were described in both groups of patients. A reduced amplitude in the EMG activity in the medial hamstrings (MH) was seen in the majority of the patients. An index of coactivation was defined by comparing the EMG activity when a muscle lengthened (antagonistic) to the EMG activity when the same muscle shortened (agonistic) during the isokinetic contractions. In comparison to normal values increased coactivation indexes were present in the VL and MH in patients of both groups. The characteristics of dynamic muscle strength and the activation of agonistic and antagonistic muscles described in the present study will provide the basis of evaluation for the effects of therapy in these patients.
Pathomechanics and Management of Scoliosis in Friedreich Ataxia Patients: Preliminary Report
Authors: Allard P. , Duhaime M. , Raso J.V. , Thiry P.S. , Drouin G. , Geoffroy G. 
Published: July 2016

SUMMARY:The preliminary results based on a one year study on the evolution and management of scoliosis are presented. Twenty-one patients were followed in the Neuro- Muscular Disease Clinic at Ste- Justine Hospital where standardized spinal radiographs were taken periodically with the Scoliosis Chariot and the Throne. The short period of observation as well as the relatively small number of Friedreich ataxia patients followed requires that these results and the following remarks be interpreted with caution.Pathomechanics — Between the age groups I (5- 10 years) and 11(10-15 years), a substantial increase in the Cobb values occurs. Associated with it, an increase was observed in the thoracic and thoraco lumbar projected surface area indices. The relative rotation between the thoracic and lumbar segments was presumed to be the cause of the sudden increase in the Cobb measurements.For the non-ambulatory patients, a decrease in the lumbar lordosis towards a thoraco-lumbar kyphosis as well as a sudden increase in the sacral angle and a drop in the lumbosacral angle were associated with the seated posture assumed by the patient.Management — Prevention of the progression of established curves was our main objective. Careful examination of the spine, depending on the age of the child, in our preliminary study, stimulated early orthopaedic treatment in any curve of 20° or more. There was always concern for curves of 30° or more. In the growing child, bracing was recommended.In the older child, the curve was usually stable after sixteen years of age. Surgery was usually attempted in curves over 40° in the growing child. The same curve was usually stable after the growth period.For the non-ambulatory patients, the present study suggested the prescription of a molded seat with the following characteristics: i) a posterior lumbar support, ii) low thoracic lateral supports and iii) a slight inclination of the seating system. This was presumed to be beneficial in maintaining stability of the spine. Presently, an evaluation of such a device is under investigation.
The Cardiomyopathy of Friedreich's Ataxia Morphological Observations in 3 Cases
Authors: Lamarche J.B. , Côté M. , Lemieux B. 
Published: July 2016

SUMMARY:In the light of the recent finding of deposits of calcium salts and iron in myocardial cells in one case of Friedreich's ataxia, we have made a detailed morphological study of 3 new cases of this cardiomyopathy. Calcium deposits were not found in the muscle fibers but lipofuscin granules and deposits of iron were observed in our 3 cases. In addition to the usual findings of interstitial fibrosis, hypertrophy and degeneration of myocardial fibers, foci of segmental active muscle necrosis were constantly present. There is a possibility that Friedreich's ataxia could be a neurocardiac degenerative disease with a membrane defect which could be related to defective metabolism of vitamin E or other micronutrienls.
Friedreich's Ataxia and Oral Glucose Tolerance: II. The effect of ingested glucose on serum growth hormone in homozygotes, obligate heterozygotes and potential carriers of the Friedreich's Ataxia gene
Authors: Tolis G. , Mehta A. , Harvey C. , Andermann E. , Andermann F. , Barbeau A. 
Published: July 2016

SUMMARY:Homozygotes, obligatory heterozygotes and potential carriers of the Friedreich's Ataxia gene were tested with an oral glucose tolerance in order to assess a. the prevalence of abnormalities in glucose handling, b. the secretory pattern of two “diabetogenic” hormones, growth hormones and prolactin and c. to evaluate the possibility for detection of an abnormal trend in these hormones to be used as a genetic marker. Despite the high prevalence of glucose abnormalities horizontally and vertically in these families, the basal output and responses of these metabolic hormones to a glucose challenge were appropriate and thus not characteristic of
any of the above groups.
Leucocyte Glutamate Dehydrogenase in Various Hereditary Ataxias
Authors: Barbeau A. , Charbonneau M. , Cloutier T. 
Published: July 2016

SUMMARY:Leucocyte Glutamate Dehydrogenase (GDH) activity was measured in 44 patients with various forms of ataxia and 44 age and sex-matched normal controls. The only significant change found was a moderate decrease in activity in Freidreich's ataxia and a few patients with OPCA. This decreased activity is not primary to the disease but probably reflects a regulatory defect affecting mitochondrial membranes in these patients.
The Benzodiazepine Receptor: The Pharmacology of Emotion
Authors: Robertson H.A. 
Published: July 2016

SUMMARY:Anxiety seems to be an inherent and perhaps necessary component of civilization. However, the biological basis of anxiety has always been as obscure as the definition of anxiety itself. The importance of anxiety to our mental wellbeing was noted by Freud (1933) who considered it a “nodalpoint”. As in the case of schizophrenia, there have been as many hypotheses to explain anxiety as there have been investigators with different techniques for studying it. The benzodiazepines, introduced clinically in I960, are now the most widely-used anxiolytic drugs. From the time of their introduction, it was felt that an explanation of the mode of action of benzodiazepines might shed considerable light on the basis of anxiety. The discovery, in 1977, of a specific benzodiazepine receptor, uniquely localized in the CNS, was the major turning point in this search (Squires and Braestrup, 1977; Mohler and Okada, 1977).
Use of a Synaptosomal Model to Monitor Drug-Induced in vivo Changes in the γ-Aminobutyric Acid Content of Nerve Endings and its Relevance to Research on Antipsychotic Drugs
Authors: Wood J.D. 
Published: July 2016

SUMMARY:The validity of a synaptosomal model to monitor drug-induced changes in the y-aminobutyric acid (GABA) content of nerve endings is reviewed. Examples of data obtained using the model are presented and the possibility of using the model for research on antipsychotic drugs is discussed.
Trace Amines and Mental Disorders
Authors: Boulton Alan A. 
Published: July 2016

SUMMARY:In this brief review it will be possible to mention only superficially the bioclinical, behavioral, neurochemical, neuropharmacological and neurophysiological evidence to support the view that some of the trace amines [meta- and paratyramine (m-TA, p-TA), β-phenylethylamine (PE) and tryptamine (T)] may play a significant role in the propagation of nervous impulses and perhaps be involved in the etiology of certain mental disorders. More detailed comments will be found in some recent papers and reviews (Axelrodet al, 1976; Boulton, 1974, 1976, 1978, 1979; Boulton and Baker, 1975; Boulton and Juorio, 1979; Faurbye, 1968; Mosnaim and Wolfe, 1978; Sandler and Reynolds, 1976; Wyatl et al, 1977).
Dietary Folic Acid and The Activity of Brain Cholinergic and γ-Aminobutyric Acid (GABA) Enzymes
Authors: Botez M.I. , Bachevalier Jocelyne , Tunnicliff G. 
Published: July 2016

SUMMARY:Charles River CD male rats were randomly divided into 3 groups of five each and placed on folate deficient, folate excess, and control diets respectively. Glutamate decarboxylase GA D y-aminohutyrate aminotransferase (GABA-T), choline acetyltransferase (ChAc), and acetylcholinesterase (AChE) were assayed in the rat brains after 6 weeks of dietary treatment. Neither folate deficiency nor folate supplementation influenced the enzymes associated with GABA and acetylcholine metabolism.
Balint's Syndrome
Authors: Hausser Chantal O. , Robert Françoise , Giard Normand 
Published: July 2016

SUMMARY:Balint's syndrome is usually attributed to bilateral parieto-occipital lesions. In several reported cases involvement of the frontal lobes was also documented and could be responsible for the “spasmodic fixation” often recorded in these patients. We report a case of Balint's syndrome in a patient with bilateral frontal and parieto-occipital metastases demonstrated by CTscan and confirmed by postmortem examination.
Decrease in the Rate of Protein Synthesis by Polysomes from Cultured Fibroblasts of Patients and Carriers with Duchenne Muscular Dystrophy
Authors: Boulé M. , Vanasse M. , Brakier-Gingras L. 
Published: July 2016

SUMMARY:Polysomes extracted from cultured fibroblast cells isolated from patients with Duchenne muscular dystrophy (DMD), carriers of the disease, and normal controls were used for in vitro measurement of protein synthesis in a wheat germ extract system. It was observed that polysomes from patients and carriers (seven of each aged 17 years or older) exhibited a 3-fold and a 1.5-fold decrease in the rate of protein synthesis, respectively, as compared with controls. These results are discussed with a view to developing a sensitive and easily available assay for the detection of DMD carriers.
Ouabain Induced Stereotyped Behavior in Rats
Authors: Davidson D.L.W. , Butterworth R.F. , Bélanger F. , Barbeau A. 
Published: July 2016

SUMMARY:Stereotyped behavior was inclined in rats with ouabain administered intraventricularly in doses of 2, 3 and 4 μg in 50 μl saline. Haloperiodol reduced the stereotyped behaviour. Monoamine turnover studies showed a reduction in concentration of norepinephrine in hippocampus and midbrain, an increase in norepinephrine turnover in the medulla oblongata and a reduction in dopamine turnover in the striatum. The interpretation of these finds is discussed. It is suggested that this model could be significant clinically as it demonstrates that impairment of Na+-K+-ATP'ase may result in behavioral abnormalities characterised by stereotypy.
Ouabain Induced Seizures: Site of Production and Response to Anticonvulsants
Authors: Davidson Duncan L.W. , Tsukada Yasuo , Barbeau André 
Published: July 2016

SUMMARY:Ouabain, an inhibitor of Na+-K+-ATP'ase, has been administered intraventricularly to rats to study the effect of impairment of membrane transport mechanisms on the genesis of seizures. Running and leaping seizures occur rapidly after injection oj ouabain in a low volume (10μl) when the maximal uptake of ouabain (39.8%) is in the hippocampus. Generalized clonic-lonic seizures are induced by higher volume injections (50μl) associated with wider distribution of ouabain, including the cerebellum and brainstem.Ouabain was injected into cerebral cortex, caudate nucleus, dorsal hippocampus, fastigeal nucleus, ventrolateral mesencephalic reticular formation and cerebellar cortex. The cerebellar injections produced both running and leaping and generalized clonic-lonic seizures. It is suggested that this results from decreased inhibitory effect of vermal and paravermal Purkinje cells on intra-cerebellar nuclei, which alters cerebellar influence on the reticular formation and the limbic system.Diphenylhydantoin, phenobarbitone, phenacemide, carbamezepine and clonazepam but not ethosuximide are effective against generalized clonic-lonic seizures, suggesting that this is a model for “grand mat” but not “petit mal” seizure mechanisms. It is furthermore suggested that running and leaping are subcortical, probably limbic, seizures that are most relevant as a model for temporal lobe seizures.
Computerized Assessment of Memory Performance in Dementia
Authors: Perez Francisco I. , Hruska Nancy A. , Stell Rebecca L. , Rivera Victor M. 
Published: July 2016

SUMMARY:Memory deficits in demented patients were studied by applying a computerized system to titrate individual memory performance. Two delayed matching to sample tasks were used. ABA1 involved random visual shapes used as a measure of visual memory. ABA 2 involved nonsense syllable trigrams used as a measure of verbal memory. A Human Test System panel with a display screen was utilized. This screen was a rear view projection window divided into four independent 3 x 2 sections arranged in a 2 x 2 matrix. The sample stimulus appeared on the upper left window for one second. The delay interval between stimulus sample and choices varied by 7 seconds dependent upon patient performance per trial. A Iso utilized was a color distractor task. Task parameters, data acquisition, retrieval, and graphic print-outs were all computerized. Individual titration levels reflected the severity of neurological impairment. Computerized assessment enabled subtle changes in memory performance to be readily identified. Implications for use of the system as a screening device in the assessment of mental changes in the elderly are apparent.
Trigeminal Neuralgia in Aqueduct Stenosis
Authors: Tucker William S. , Fleming Ross , Taylor Ferelith A. , Schutz Hart 
Published: July 2016

SUMMARY:Trigeminal neuralgia was the presenting symptom in two patients with aqueduct stenosis, hydrocephalus, and raised intracranial pressure. Treatment of the hydrocephalus resulted in the remission of pain in both patients.
Lipotropin, Melanotropin and Endorphin: In Vivo Catabolism and Entry into Cerebrospinal Fluid
Authors: Pezalla P.D. , Lis M. , Seidah N.G. , Chrétien M. 
Published: July 2016

SUMMARY:Anesthetized rabbits were given intravenous injections of either beta-lipotropin (beta-LPH), betamelanotropin (beta-MSH) or betaendorphin. The postinjection concentrations of these peptides in plasma and cerebrospinal fluid (CSF) were measured by radioimmunoassay (RIA). The plasma disappearance half-times were 13.7 min for beta-LPH, 5.1 min for beta-MSH, and 4.8 min for beta-endorphin. Circulating beta-LPH is cleaved to peptides tentatively identified as gamma-LPH and beta-endorphin. Each of these peptides
appeared in the CSF within 2 min postinjection. The maximum CSF to plasma ratios were 0.08 for beta-LPH, 1.48 for beta-MSH, and 0.23 for beta-endorphin.
The Uptake of 3H(G)L Leucine into Single Muscle Fibers in Charcot-Marie-Tooth Disease
Authors: Monckton George , Marusyk Halyna 
Published: July 2016

SUMMARY:In previous studies, the incorporation of 3H(G)L-leucine into muscles of patients with Charcot-Marie-Tooth (CMT) disease was shown to he increased in comparison with that observed in motor neuron disease (MND). To determine the cause of the increased uptake in CMT, studies of single fiber leucine incorporation have been undertaken. The results of this study indicate that the increased incorporation is into those muscle fibers which are undergoing regeneration following reinnervation. These results do not support the thesis that there is an associated myopathic process in CMT.
Fundamental Nature of Human Infant's Brain Asymmetry
Authors: Wada Juhn A. , Davis Alan E. 
Published: July 2016

SUMMARY:Morphological speech zone asymmetry in man cannot be due to environmental or developmental factors after birth. The functional implication of such a finding is not yet clear. Morphological asymmetry of the human brain is paralleled by electrophysiological evidence of cerebral hemispheric asymmetries. The results of our analysis of 50 infants suggest that clear occipital-temporal coherency asymmetry similar, but not identical to the adult pattern, also exists at or near birth. These asymmetries are generated by stimuli with no verbal content and in infants who presumably have no or an undeveloped capability for language. It is suggested that language is only a part of much more fundamental asymmetries which include the processing of auditory and visual information. Our results, and those of others, are consistent with the assumption that the left hemisphere is more able to relate stimuli to past experience, either short or long-term, while the right hemisphere is more able to process stimuli which are not easily identifiable or referable. These capabilities would not be based on language, and hence would be expected to develop independently and possibly before speech. The demonstration that reversing electrophysiological asymmetries can be generated with non-speech stimuli in the visual and auditory modalities, and in neonates, supports such an assumption.