bluuebunny/crossref_metadata_2025_split huggingface dataset:
Wohlfart Kugelberg Welander Syndrome
Authors: Ross R.T. , Simpson Charles A. , Styles Salma 
Published: July 2016

SUMMARYA family is described with two brothers suffering from the Wohlfart Kugelberg Welander syndrome, and seven unaffected siblings. Another brother died in infancy from “a spinal disorder”. Both affected males developed symptoms in the third decade with weakness of the lower limb girdle, followed by the upper limb girdle. Both showed fasciculations, hyporeflexia, and purely lower motor neuron findings. EMG showed evidence of denervation and muscle biopsy showed neurogenic atrophy. Terminal motor latencies were slightly prolonged and one showed an elevated serum aldolase while both had elevated C.P.K.’s. Laboratory studies on some of the normal siblings were normal. The younger brother who has been seen over two years seems to have become slightly stronger.Dermatoglyphics and chromosomal analysis have been carried out on the patients and some of the normal siblings. All were normal except for the younger affected brother who has persistently showed a high percentage of abnormal cells, though the basic karyotype was normal, and abnormal dermatoglyphics. This is the only case we have found in the literature with chromosome abnormalities. This pedigree suggests a recessive inheritance, possibly x linked.
Wetherbee Ail
Authors: Powers James M. , Horoupian Dikran S. , Schaumburg Herbert H. 
Published: July 2016

SUMMARYThe neuropathological findings of a Farr family member consist of neuronal loss in the anterior horns and dorsal nuclei of Clarke, neuronal intracytoplasmic inclusions and posterior and lateral column demyelination. This report supports the role of familial amyotrophic lateral sclerosis as a link between common motor neuron disease and classical spinocerebellar degeneration.
Suppressive Effects of Various Amino Acids against Ouabain-Induced Seizures in Rats
Authors: Tsukada Y. , Inoue N. , Donaldson J. , Barbeau A. 
Published: July 2016

SUMMARY:The suppressive effect of various amino acids against ouabaininduced seizures was investigated in young female rats. The amino acids were injected into the left lateral ventricle 10 minutes prior to the intraventricular administration of 5 μg. of ouabain. Animals receiving 1.9 x 10-1M solutions of hypotaurine and of β- alanine were almost completely protected from the ouabain seizures. Administration of L-alanine and of glycine was also effective, although running and leaping seizures still occurred to some extent. Betaine reduced only clonic-tonic and whole body flexion and extension seizures. In contrast, L-proline exclusively suppressed clonic-tonic and focal clonic seizures. Rats injected with isethionic acid showed increases in incidence of running and leaping seizures while L-arginine in high concentrations caused aggravation in clonic-tonic seizures. L-cysteine, even in low concentrations, also brought about an increase in the occurrence and incidence of clonic-tonic seizures. The ED50of hypotaurine was 10.11 x 10-2M for running seizures and 4.63 x 10-2, M for clonictonic seizures; that of β -alanine was 14.01 x 10-2M for running seizures and 5.50 x 10-2M for clonic-tonic seizures. However, hypotaurine and β -alanine, the most effective compounds tested in the present studies, provided less protection than taurine previously examined by us under similar conditions (Izumi et al., 1973).
Electrical Stimulation of the Human Visual Cortex Preliminary Report
Authors: Talalla Andrew , Bullara Leo , Pudenz Robert 
Published: July 2016

SUMMARY:A feasibility study for the development of a human visual prosthesis has led several workers to observe the effects of electrical stimulation of the human visual cortex. Experience with such stimulations of three normal-sighted patients is reported. The results confirm some of the findings of other workers, but do not show that multiple phosphenes were experienced by our patients, using strictly limited parameters of stimulation.
Polyglycolic Acid Suture in Peripheral Nerve: An Electron Microscopic Study
Authors: Hudson Alan R. , Bilbao Juan M. , Hunter Daniel 
Published: July 2016

SUMMARY:The aim of this experiment was to investigate the reaction of peripheral nerve tissue to a synthetic absorbable suture material. Polyglycolic acid suture material was placed within the sciatic nerve of rats and the absorption of the material was investigated by means of electron photomicrographs. It was concluded that placement of polyglycolic acid into the peculiar environment of endoneurial tissues results in minimal scarring and in minimal disturbance of the surrounding nerve fibers. The material was progressively absorbed with minimal disturbance of intrafascicular structures.
The Chiari Malformation in Adults
Authors: Maroun F.B. , Jacob J.C. , Mangan M. 
Published: July 2016

SUMMARY:The clinical features of the Chiari Malformation in seven adult patients are presented. It is suggested that the clinical syndromes associated with this malformation, in adults, can be classified as (a) compression of structures at the level of the foramen magnum (with or without radiologically demonstrable associated bony anomaly at the cranio-vertebral junction) (b) increased intracranial pressure or obstructive hydrocephalus and (c) intramedullary cervical cord syndrome. The usefulness of tomography, and demonstration of the vertebro-basilar circulation in the neuro-radiologic investigation of these patients is emphasized. The surgical procedures performed in the management of these patients are outlined.
The Effect of Diazepam on Presynaptic Inhibition in Patients with Complete and Incomplete Spinal Cord Lesions
Authors: Verrier M. , MacLeod S. , Ashby P. 
Published: July 2016

SUMMARY:The effect of diazepam on presynaptic inhibition in man has been examined in 5 patients with complete spinal transections and 7 patients with incomplete lesions. The inhibition of the H reflex by vibration applied to the tendo Achilles was used to assess presynaptic inhibition of the la monosynaptic pathway. Diazepam increased this inhibition in the patients with incomplete lesions, but had no significant effect on the inhibition in the patients with complete spinal transections.Evidently diazepam can enhance presynaptic inhibition in man. The effect, however, cannot be demonstrated in patients with longstanding complete spinal lesions possibly because of some alteration in the segmental presynaptic inhibitory mechanism in this group.
Pituitary Melanocorticotrophinoma with Amyloid Deposition
Authors: Bilbao Juan M. , Kovacs Kalman , Horvath Eva , Higgins Hubert P. , Horsey William J. 
Published: July 2016

SUMMARY:The light and electron microscopic features of a pituitary adenoma composed of adrenocorticotrophic hormone (ACTH) and melanocyte stimulating hormone (MSH) cells with perivascular amyloid deposition is reported. Histochemical and fine structural data indicate that this material is APUDamyloid and is present in the extra-cellular perivascular spaces. It is suggested that the differences in fine structure and of distribution of the amyloid in pituitary adenomas is dependent upon the cell of origin.
Principles Underlying New Methods for Chronic Neural Recording
Authors: Stein R.B. , Charles D. , Davis L. , Jhamandas J. , Mannard A. , Nichols T.R. 
Published: July 2016

SUMMARY:Chronic recording is possible from nerve fibers which have grown through holes in an insulating medium (regeneration electrodes) or which are enclosed by an insulating sheath (cuff
electrodes). Use of three electrodes in a balanced configuration permits good rejection
of electromyographic (EMG) signals and other sources of electrical interference (fluorescent lights, 60 Hz signals from the mains, etc.). Equations are derived and tested for predicting the
amplitude and form of the signals expected for a given cuff length and diameter. These equations can be used to design electrode units optimally for a given application. Finally, the use of transformers permits the neural signals to be carefully matched to the recording apparatus
and further optimizes the neural signal-to-noise and signal-to-EMG ratios. Use of these methods in several physiological and clinical applications, as well as potential abuses, are discussed.
The Effects of Motor Cortical Stimulation on the Excitability of Spinal Motoneurons in Man
Authors: Milner-Brown S.H. , Girvin J.P. , Brown W.F. 
Published: July 2016

SUMMARY:The pyramidal tract and particularly the direct corticomotoneuronal components (DCM) have become increasingly important in the higher primates. Minimal single pulse precentral stimulation in man evokes EMG discharges from the contralateral hand muscles with a latency of 18-21 milliseconds. The excitability changes produced by such cortical stimulation on the upper limb H-reflex has been observed to include a short duration early facilitation probably corresponding to the DCM input and a later, longer lasting facilitation mediated by the same and probably other corticofugal projections. Potentiation of the H-reflex in the upper limbs by means of postcentral excitation required much higher single pulse stimulus intensities and the changes in excitability produced on the spinal motoneurons could have been explained by physical extension of the stimulus current to the precentral region. Isometric contraction potentiated the H-reflex produced by combinations of precentral cortical and peripheral nerve stimulation but no direct evidence was found to support a possible transcortical basis for the V2 stretch reflex.
Physiological Basis of Cerebellar Dysmetria
Authors: Murphy John T. , Kwan C. , MacKay William A. , Wong Yiu C. 
Published: July 2016

SUMMARY:A primary control system for the arm position is formulated. The hypothesis that the cerebellum is a part of the system controller is checked by studying the nerve cells responses in the cerebellum, and motor cortex, to natural activation of muscular receptors. The results show that the cerebellum receives feedback information related to the speed of these receptors. The discussion concentrates on how the interruption of this feedback may result in excessive oscillations to instability. These observations are the base for evaluating how the cerebral lesions produce dismeasurements.
Generality of the Kindling Phenomenon: Some Clinical Implications
Authors: Pinel J.P.J. , Van Oot P.H. 
Published: July 2016

SUMMARY:The purpose of the present investigations was to explore the generality of the kindling phenomenon and its applicability to clinical situations. Whether local brain stimulation, electroconvulsive shock (ECS), or metrazol the consequence of periodic administration of convulsive agents was found to be the same; in each case repeated application of the agent resulted in the gradual development and intensification of convulsive symptoms (kindling). Moreover, in each case the resulting intensification was not specific to the agent being used and seemed to increase the responsiveness to convulsive agents in general. In the present studies this interaction was seen in the form of an intensified alcohol withdrawal syndrome observed 18 days after cessation of a series of metrazol injections, amygdaloid stimulations, or ECS. Thus, it appears that one of the hazards of the convulsive therapies is that they may induce enduring changes in brain function which leave the patient in a state of increased susceptibility to a variety of potentially convulsive agents.
CSF Electrophoresis in One Thousand Patients
Authors: Ebers G.C. , Paty D.W. 
Published: July 2016

SUMMARY:Agarose and/or cellulose acetate electrophoresis was performed on the CSF of one thousand patients. In patients with clinically definite multiple sclerosis (MS) (N = 267) 92.8% had oligoclonal banding (O.B.). In patients with possible MS (N - 283) O.B. was present in 31.1%. In patients with other neurological diseases (N = 450) O.B. was present in 8% (N = 36). Nineteen non-MS patients with positive O.B. had serum bands or disorders known to be associated with local immune response. The remaining 17 patients had no explanation for the oligoclonal banding. In the majority of these MS had not been a diagnostic consideration.CSF electrophoresis is the single most reliable laboratory lest in multiple sclerosis and deserves incorporation into the diagnostic criteria for the disease.
Confusion, Dysphasia, and Asterixis Following Metrizamide Myelography
Authors: Smith Michael S. , Laguna Jose F. 
Published: July 2016

SUMMARY:A patient exhibited confusion, dysphasia, and striking asterixis 18 hours after metrizamide myelography. The symptoms lasted for a day and a half. While metrizamide is a useful contrast medium, side effects are not unusual and can, on occasion, be quite severe.
Field Testing of an Ataxia Scoring and Staging System
Authors: Pourcher Emmanuelle , Barbeau André 
Published: July 2016

SUMMARY:The authors present a simple system for disability scoring and functional staging of an ataxic patient, based on modifications of a previous scheme advocated by De Falco and collaborators (1979). This system was tested under field conditions in 47 ataxic subjects and found to be useful and functional.
Hypertrophic Cardiomyopathy in Friedreich's Ataxia: Symmetric or Asymmetric?
Authors: Pasternac A. , Król R. , Petitclerc R. , Harvey C. , Andermann E. , Barbeau A. 
Published: July 2016

SUMMARY:We evaluated 15 patients with Friedreich's ataxia (FA) to define the incidence of myocardial involvement and the type of cardiomyopathy observed.All patients with FA had either ECG, vectocardiographic or echocardiography abnormalities, suggesting some degree of myocardial involvement. In contrast to reports indicating that asymmetric septal hypertrophy (ASH), often obstructive, is associated with FA, symmetric, concentric hypertrophic cardiomyopathy (SCH) was the predominant abnormality (sixty-seven percent of patients). Echocardiograms should be performed periodically in all FA patients since this technique allows the detection of cardiac hypertrophy.
Friedreich's Ataxia and Oral Glucose Tolerance: I. The effect of ingested glucose on serum glucose and insulin values in homozygotes, obligate heterozygotes and potential carriers of the Friedreich's ataxia gene.
Authors: Tolis G. , Mehta A. , Andermann E. , Harvey C. , Barbeau A. 
Published: July 2016

SUMMARY:Glucose tolerance and insulin release were evaluated in 16 families with Friedrich's ataxia. Impaired glucose tolerance differed in incidence according to the method of evaluation, but was increased in number in parents' and siblings of Friedreich's cases. Insulin output was not quantitatively different from normal, although the insulin peak was often delayed. This finding, in association with impaired glucose tolerance, suggest a defect in glucose entry into cells.
Friedreich's Ataxia in Northern Italy II. Biochemical Studies in Cultured Cells
Authors: Bertagnolio B. , Uziel G. , Bottachi E. , Crenna G. , D’Angelo A. , Di Donato S. 
Published: July 2016

SUMMARY:Pyruvate and palmitate oxidations by cultured fibroblasts suspensions were measured in optimized conditions and proved to be within normal range in the cells from Friedreich's patients. However, when pyruvate oxidation was measured by direct assay of the pyruvate dehydrogenase complex, this enzyme activity proved to be significantly lower in Friedreich's than in controls' cells. These abnormalities were not observed when the cells were sonicated. Moreover, lipoamide dehydrogenase activity. Km and Vmax were within the normal range in Friedreich 's cells. These data suggest that the low activities of the PDH complex are not a primary defect in Friedreich's ataxia, but are more likely related lo membrane abnormalities in Friedreich's cells.
Correlation Between Serum Lipoamide Dehydrogenase Activity and Phosphatidylcholine Therapy in Friedreich's Ataxia
Authors: Melançon S.B. , Fontaine G. , Geoffroy G. , Vanasse M. , Dallaire L. , Potier M. 
Published: July 2016

SUMMARY:Serum lipoamide dehydrogenase activity and kinetics were studied in nine patients with Friedreich's ataxia before and three months after therapy with oral lecithin. Results disclosed a significant reduction in LAD inhibition by NADH in all patients after therapy. Three patients normalized their increased Km for lipoamide and one patient showed the opposite results after therapy. Two patients ceased lecithin after one month. All seven patients who remained in the trial group and one additional patient, showed subjective and objective signs of improvement in physical resistance. This study has offered some biochemical basis for the apparent clinical improvement in patients with Friedreich's ataxia who undergo lecithin therapy.
Protein Kinase Activity of Human Erythrocyte Membranes in Friedreich's Ataxia
Authors: Wong P. , Barbeau A. 
Published: July 2016

SUMMARY:Proteins of human erythrocyte membranes of Friedrich's ataxia patients and controls were examined by SDS-polyacrylamide gel electrophoresis before and after reduction withβ-mercaptoethanol. No difference could be detected in the composition of their state of aggregation. The protein kinase activity of human erythrocyte membranes of eleven Friedreich's ataxia patients and six controls was determined. No difference in their protein kinase activity could be detected. These results are discussed with respect to an involvement of a generalized membrane defect in Friedreich's ataxia.
Taurine Decreases Lesion Severity In the Hearts of Cardiomyopathy Hamsters
Authors: Azari Jamshid , Brumbaugh Peter , Barbeau André , Huxtable Ryan 
Published: July 2016

SUMMARY:Cardiomyopathic Syrian hamsters develop necrotic lesions consequent upon calcium overload from 60 days of age onward. Taurine, given as a 0.1M solution in place of drinking water for one month prior to sacrifice of animals of initial age 35 days, decreased the severity of subsequently developing cardiac lesions by 40%. Calcium concentration in the heart was decreased by 57%. Magnesium and iron concentrations were unaltered. Taurine given in a similar manner for 4 months had a protective effect, decreasing lesion severity by 21% and calcium concentration by 35%. Magnesium concentrations were increased by 12%. Compared to randombred animals, cardiomyopathic hamsters at one and two months of age have the same concentrations of calcium, magnesium and iron in the quadrants of the heart, except in the left ventricle, which has significantly higher concentration of calcium. Calcium concentrations are 70%, 1320% and 2100% higher respectively in one month, two month and five month old animals. Five month old animals differ slightly but significantly in iron (17% decrease) and magnesium concentrations (17% increase).Cardiomyopathic hamsters have insignificant differences in β-adrenergic receptor density compared to random-bred animals and have a significantly higher rate of taurine influx.
Bilateral Encephaloclastic Lesions In A 26 Week Gestation Fetus: Effect On Neuroblast Migration
Authors: Norman Margaret G. 
Published: July 2016

SUMMARY:A 26 week gestation fetus with bilaterally symmetrical encephaloclastic lesions in the cerebral hemispheres is described. Information about gestation allows dating of the cerebral insult to the 3-4th fetal month. The history and morphology in this case suggest that insults occurring at or before the 3-4th fetal month interfere with normal neuroblast migration, producing a cerebral scar containing a band of neuroblasts arranged without order or lamination.
Noradrenergic Function and Depression, Too Much or Too Little?
Authors: Maas J.W. , Huang Y. 
Published: July 2016

SUMMARY:Antithetical hypotheses as to CNS noradrenergic function in depressed patients can be constructed from results of pharmacological studies of the effects of antidepressant drugs. The experimental data supporting each of these opposing propositions is briefly reviewed in this paper. Finally, the results of clinical studies of noradrenergic function in depressed patients are noted and discussed in terms of
these disparate hypotheses.
Autonomic Neuropathy in the Streptozotocin Diabetic Rat
Authors: Monckton George , Pehowich Enid 
Published: July 2016

SUMMARY:An ultrastructural study of the sympathetic ganglia, dorsal sympathetic chain, and Auerbach's plexus is described in the streptozotocin diabetic rat. The observations cover the first six weeks following induction of diabetes. Degenerative changes began to appear in the axonal plasma membranes and axoplasm of unmyelinated fibers at 24 hours and were widespread by two weeks. Axonal sprouting and regenerating axons began to appear and increase in number through the 6th week. Chromatolysis in ganglion cells in sympathetic ganglia became apparent by two weeks as did degenerative changes in axons of Auerbach's plexus. This somewhat later appearance of changes in ganglion cells and Auerbach's plexus suggested the primary changes occurred in the postganglionic fibers. The cycle of change from degeneration to regeneration in the areas examined suggests either a possible toxic effect of strepiozotocin or a combination of metabolic disturbances as a cause of this neuropathy.
An Electrophysiological Quantitation of the Cubital Tunnel Syndrome
Authors: Odusote Kayode , Eisen Andrew 
Published: July 2016

SUMMARY:Four hundred and ninetytwo ulnar nerves were studied in 237 patients with cubital tunnel syndrome and 233 subjects without clinically apparent ulnar neuropathy. Terminal motor and sensory latencies, motor and sensory conduction velocities, sensory action potential amplitudes and dispersions, and needle electromyography were analysed by a 0 to 10 rating — the EMG score. The patients were clinically graded from 1 (least severe) to 4 (most severe). The median EMG scores of these were 0.9/10 (N=70): 1.6/10 (N=81); 4.7/10 (N=46); and 7.1/10 (N=56), respectively. The median EMG score for the controls was 0.6/10 (N=239) and 14.7% had asymptomatic ulnar entrapments. This incidence reached 30% in controls who were 60years or older. In 25 other controls, the above sulcus sensory action potential was analysed. Its mean dispersion was 4.9 ± 1.0 s-3. This measurement was significantly prolonged in 84.6% of 13 studies in which the EMG score was 0/10. These patients had either sensory symptoms only or an additional mild sensory deficit. Comparison of the EMG score obtained with sequential studies would allow one to objectively evaluate improvement or deterioration of the clinical state, giving a rational means of determining the need for surgery.
Non-Association of Friedreich's Ataxia and HLA Based on Five Families
Authors: Chamberlain S. , Walker J.L. , Sachs J.A. , Sachs J.A. , Wolf E. , Festenstein H. 
Published: July 2016

SUMMARY:HLA-A, B, and C typing was carried out on five families with classical Friedreich's Ataxia (F.A.). Segregation patterns in a total of 16 offspring. 10 with the disease and 6 without, indicated no association between F.A. and HLA.
The Neurological Complications of Brucellosis
Authors: Larbrisseau A. , Maravi E. , Aguilera F. , Martinez-Lage J.M. 
Published: July 2016

SUMMARY:Neurological complications of brucellosis have seldom been reported. Typical cases of meningoencephalitis, myelitis, and peripheral nerve involvement due to brucellosis, which have been collected over the past ten years, are described. Only four cases of extradural brucellar abcesses have been published previously. We now add four new observations. A CSF electrophoresis was done in the cases of meningoencephalitis and meningomyelitis and showed a marked increased of gamma globulin. This has never been reported previously.
Functional Implications of Changes in the Senescent Brain: A Review
Authors: Beck Charles H.M. 
Published: July 2016

SUMMARY:The morphological, chemical, and physiological changes in the brain accompanying old age are reviewed. The deterioration of the striatal and hypothalamic dopaminergic systems were implicated in the onset of age related Parkinsonian-like slowing of performance and altered affect. Cholinergic hippocampal and neocortical systems were chemically and physiologically abnormal in the aged. The implications for slowed cognitive processing and persistance of the memory trace are presented.