bluuebunny/crossref_metadata_2025_split huggingface dataset:
Alterations of Membrane Phosphorylation in Erythrocyte Membranes from Patients with Duchenne Muscular Dystrophy
Authors: Vickers J.D. , McComas A.J. , Rathbone M.P. 
Published: July 2016

SUMMARY:The phosphorylation of spectrin, hand 3 protein, and the phospholipids of erythrocyte membranes (ghosts) was examined in 10 patients with Duchenne muscular dystrophy (DMD) and in healthy age- and sex-matched controls. The rales of phosphorylation of spectrin and band 3 protein were significantly higher in ghosts prepared from patient blood than from control blood at both 30° C and 37° C. However, the mean increases in the rate of phosphorylation of both spectrin and band 3 protein in response to a temperature change from 30° C to 37° C were identical in gliosis from patient and controls. Phosphorylation of phospholipid and its temperature response did not differ between patients and controls. These results complement previous observations of differences in erythrocytes from patients with DMD. The similarity of the changes in phosphorylation of both spectrin and band 3 protein indicates a common cause, possibly their lipid environment.
Case Report and Ultrastructural Study of Intracranial Embryonal Carcinoma
Authors: Ejeckam Gerson , Norman Margaret G. , Ivan Leslie P. 
Published: July 2016

SUMMARY:A case of a primary intracranial embryonal carcinoma, the first with ultrastructural study, is reported. The tumor was associated with precocious puberty in a 6½-year-old female. Characteristic embryoid bodies were present. At the ultrastructural level three cell types were noted: undifferentiated, differentiated, and intermediate types. The undifferentiated showed scanty cytoplasmic organelles and numerous free polysomes, while the differentiated cells contained well-developed mitochondria, Golgi apparatus, rough endoplasmic reticulum, and some contained secretory granules. The intermediate cells possessed dilated and irregularly-shaped mitochondria but still retained large numbers of free polysomes. The authors suggest that intracranial germ cell tumors be named in conformity with germ cell tumors in other sites, and that terms such as “ectopic pinealoma” and “atypical teratoma of the pineal” be used no longer.
F-wave and Cervical Somatosensory Response Conduction from the Seventh Cervical Spinous Process to Cortex in Multiple Sclerosis
Authors: Eisen Andrew , Nudleman Kenneth 
Published: July 2016

SUMMARY:Transit (conduction) times from the wrist to the seventh cervical spinous process (C7) were measured by using the F-wave response (TA) and the cervical somatosensory response (SEPC). The mean values obtained in 25 controls were 10.1 ± 0.9 s-3 and 10.9 ± 1.0 s-3 respectively. The 0.8 ± 1.0 s-3 difference between the two methods represents central delay of SEPC, possibly at the level of the dorsal root ganglion. The mean onset latency of the cortical somatosensory response (SEPA) evoked by median nerve stimulation in the same subjects was 15.5 ± 1.0 s-3. Transit time from C7 to the cortex (TB) given by either SEPA - TA or SEPA - SEPC - 0.8, where 0.8 is the necessary corrective factor for central delay of SEPC, was 5.3 ± 1.0 s-3. These techniques were applied to 10 patients suspected of having multiple sclerosis, but without clinical evidence of involvement above C7. Six of the 10 cases showed prolongation of TB. In 4 of these, this could only be calculated by the F-wave method since SEPC was absent. It is concluded that transit times derived from either the F-wave or SEPC are equally valid and interchangeable. The absence of one response allows for its replacement by the other.
CSF Electrophoresis: An Adaptation Using Cellulose Acetate for the Identification of Oligoclonal Banding
Authors: Paty D.W. , Donnelly M. , Bernardo M.E. 
Published: July 2016

SUMMARY:An adaptation of cellulose acetate electrophoresis for studying concentrated cerebrospinal fluid is described. Two hundred and twenty-one patients have been studied, and the specificity for multiple sclerosis and sub-acute sclerosing panencephalitis is discussed. This has been positive for oligoclonal banding (OB) in 79% of patients with clinically definite multiple sclerosis.
Timing of the Electroretinogram Response and Dark Adaptation
Authors: Brunette Jean Real , Lafond Gilles 
Published: July 2016

SUMMARY:The time response of the Electroretinogram (ERG) is an essential part of the examination. Peak time of
response increases with dark adaptation and decreases with the intensity of stimulation. When working with constant amplitude during dark adaptation sensitivity increases but the intensity of stimulation decreases. It is essential to clarify the relationship of these two factors for valid interpretation of results. The present work suggests that the state of adaptation or retinal sensitivity affects amplitude, while peak time of the response is modified by the absolute value of the stimulating source whatever the state of adaptation. This observation has been done on rod responses and is valid as long as pigments remain unbleached and able to respond.
Dominantly Inherited Hypertrophic Neuropathy
Authors: Mongia S.K. , Ghanem Q. , Preston D. , Lewis A.J. , Atack E.A. 
Published: July 2016

SUMMARY:Clinical, electrophysiological, and histopathological studies of some members of a family with dominantly
inherited hypertrophic neuropathy are presented. Twenty-five members were studied. Seventeen were abnormal on clinical examination. Their ages varied from 2½ to 78 years. Age at onset in 14 of the 17 varied between 2½ and 56 years. Pes cavus and palpable nerve thickening were present in more than half of the affected individuals. All patients had areflexia. Fifteen of the 17 had distal motor weakness as well as mild to moderate sensory impairment. Motor weakness affecting the proximal hip and shoulder girdle muscles was seen in 13 patients. Four patients gave a history of trigeminal neuralgia. Motor nerve conduction velocities were markedly impaired in all the clinically affected members. These studies were normal in the 8 unaffected members. Motor conduction velocities of the proximal segment of the ulnar nerve were slower compared to the distal segment in almost all the affected members. There was no significant correlation between the degree of clinical disability and the extent of impairment in the motor nerve conduction velocities. Sural nerve biopsies were studied. These observations are discussed.
Hydranencephaly in Association with Roberts Syndrome
Authors: Ekong Chris E.U. , Rozdilsky Bohdan 
Published: July 2016

SUMMARY:A clinicopathological study in a case of Roberts syndrome (tetraphocomelia, cleft lip and palate, and phallic hypertrophy) is reported. This patient had hydranencephaly and imperforate anus, two additional congenital abnormalities so far not reported in this syndrome.
Platelet Dopamine Uptake in Huntington's Chorea and Gilles de la Tourette's Syndrome: Effect of Haloperidol
Authors: Butterworth Roger F. , Gonce Michel , Barbeau André 
Published: July 2016

SUMMARY:Uptake of 14C-dopamine by human platelets has been studied in two diseases, namely Gilles de la Tourette's syndrome and Huntington's chorea, in which abnormal metabolism of dopamine has been implicated. Platelets from untreated Huntington's chorea patients showed a small increase in Km and Vmax.; platelets from patients in all other groups showed an uptake identical with the controls. Haloperidol (10 -5M) was also shown to be a strong non-competitive inhibitor of 14C-DA uptake by platelets. This property is probably unrelated to the drugs' action in ameliorating the symptoms of Huntington's chorea which is likely related to the increase in cholinergic neuronal activity produced by neuroleptic blockade of dopamine receptors.
The Role of Cyclic Nucleotides in the CNS
Authors: Phillis John W. 
Published: July 2016

SUMMARY:On the basis of the information presented in this review, it is difficult to reach any firm decision regarding the role of cyclic AMP (or cyclic GMP) in synaptic transmission in the brain. While it is clear that cyclic nucleotide levels can be altered by the exposure of neural tissues to various neurotransmitters, it would be premature to claim that these nucleotides are, or are not, essential to the transmission process in the pre- or postsynaptic components of the synapse. In future experiments with cyclic AMP it will be necessary to consider more critically whether the extracellularly applied nucleotide merely provides a source of adenosine and is thus activating an extracellularly located adenosine receptor, or whether it is actually reaching the hypothetical sites at which it might act as a second messenger. The application of cyclic AMP by intracellular injection techniques should minimize this particular problem, although possibly at the expense of new difficulties. Prior blockade of the adenosine receptor with agents such as theophylline or adenine xylofuranoside may also assist in the categorization of responses to extracellularly applied cyclic AMP as being a result either of activation of the adenosine receptor or of some other mechanism. Ultimately, the development of highly specific inhibitors for adenylate cyclase should provide a firm basis from which to draw conclusions about the role of cyclic AMP in synaptic transmission. Similar considerations apply to the actions of cyclic GMP and the role of its synthesizing enzyme, guanylale cyclase.The use of phosphodiesterase inhibitors in studies on cyclic nucleotides must also be approached with caution. The diverse actions of many of these compounds, which include calcium mobilization and block of adenosine uptake, could account for many of the results that have been reported in the literature.
Haloperidol-Induced Dyskinesias in the Monkey
Authors: Bédard P. , Delean J. , Lafleur J. , Larochelle L. 
Published: July 2016

SUMMARY:Haloperidol (0.25 mglkg i.m.) was injected daily for 6 months in six normal monkeys. Over a 24 hour period, the following symptoms could he observed: akathisia, circling, akinesia, choreoathetoid and dystonic movements, oro-facial dyskinesias and postural tremor with or without harmaline. Six months after cessation of haloperidol, harmaline-induced postural tremor could still be observed in all animals and oro-facial abnormal movements, in one monkey. The neuropathologic study of the experimental material did not disclose any alteration of the central nervous system.
The Possible Local Synthesis of Antibodies to Herpes Simplex Virus in Normal Cerebrospinal Fluid
Authors: Russell Anthony Science 
Published: July 2016

SUMMARY:We have used the technique of antibody mediated cell dependent immune lysis to examine paired samples of serum and CSF for antibody to herpes simplex virus. The 40 patients studied had no inflammatory disease of the nervous system, yet 20 of the CSF specimens did have antiviral antibody. This is an extremely sensitive technique for the detection of at least one type of antiviral antibody and “in vitro” is a very effective way of killing virus infected cells. There is no correlation between the level of antiviral antibody in the CSF with the total protein content, but the high CSF: serum antibody ratio in some subjects who are particularly susceptible to recurrent herpes infection raises the possibility that local stimulation and production of this antibody may occur.
Clinical Description and Roentgenologic Evaluation of Patients with Friedreich's Ataxia
Authors: Geoffroy G. , Barbeau A. , Breton G. , Lemieux B. , Aube M. , Leger C. , Bouchard J.P. 
Published: July 2016

SUMMARY:The 50 patients in this survey were classified by a panel of neurologists into 4 clinical sub-groups: Group la (“typical” Friedreich's ataxia, complete picture), Group lb (“typical” Friedreich's ataxia, incomplete picture), Group Ila (“atypical” Friedreich's ataxia, possible recessive Roussy-Levy syndrome), Group lib (heterogeneous ataxias). The clinical symptoms and signs were analyzed for each of these groups. A constellation of signs constantly present in Friedreich's ataxia and obligatory for diagnosis was described. Other important symptoms, such as the Babinski sign, kyphoscoliosis and pes cavus were found to be progressive, but not essential for the diagnosis at any given time. Finally, a host of other symptoms can only be called accessory. The progression of scoliosis was found to be an important tool in the differential diagnosis of ataxias. Our study also indicates, in contrast to the opinion of some authors, that absent deep tendon reflexes in the lower limbs and early dysarthria are essential in “typical” Friedreich's ataxia.
Electrocardiographic and Vectocardiographic Findings in Friedreich's Ataxia
Authors: Malo S. , Latour Y. , Cote M. , Geoffroy G. , Lemieux B. , Barbeau A. 
Published: July 2016

SUMMARY:Electrocardiographic and vectocardiographic changes are frequent in Friedreich's ataxia. In one of 35 patients both tests were normal. The vectocardiogram is more explicit in demonstrating the severity of the QRS changes with a right ventricular hypertrophy pattern present in 60% of cases. Serial examination and ECG tracings are recommended to monitor the cardiomyopathy in this progressive neurological disorder, in order to detect the onset of congestive heart failure, significant tachyarrythmias, or obstructive cardiomypathy.
Echocardiographic Findings in Friedreich's Ataxia
Authors: Gattiker H.F. , Davignon A. , Bozio A. , Batlle-Diaz J. , Geoffroy G. , Lemieux B. , Barbeau A. 
Published: July 2016

SUMMARY:Echocardiographic examination of 21 patients with Friedreich's ataxia (age 7 to 28 years) showed cardiac abnormalities in 90% of the cases. They were characterized by varying degrees of septal hypertrophy in 81%, left ventricular free wall hypertrophy in 61%, and a slight reduction of left ventricular internal dimension in 57% of the cases. Asymmetric septal hypertrophy (ASH) with a septal/left ventricular free wall ratio of over 1.3 was found in 29% of the cases, and systolic anterior motion (SAM) of the mitral valve in three patients. Two other patients showed evidence of a different type of cardiomyopathy with marked symmetric left ventricular hypertrophy and marked left ventricular enlargement.
Pathology of the Heart in Friedreich's Ataxia: Review of the Literature and Report of One Case
Authors: Sanchez-Casis G. , Cote M. , Barbeau A. 
Published: July 2016

SUMMARY:A single case of typical Friedreich's ataxia was analyzed for cardiac changes and compared to the findings from the literature. Macroscopically, there was a cardiomegaly with some degree of ventricular hypertrophy and probable mild dilatation of the auricles. The more important and constant histologic changes were myocardial fibrosis and degeneration of the cardiac muscle cells. Granular deposits of calcium salts and iron were found in the muscle cells. A cardiomyopathy hypertrophic in type and occasionally obstructive appears to be an integral part of Friedreich's ataxia.
An Unusual Occurrence of Multiple Sclerosis in a Small Rural Community
Authors: Murray Thomas John 
Published: July 2016

SUMMARY:Ten patients with multiple sclerosis were found to have lived in close proximity in a Nova Scotia farming community of 150 people. All had drunk unpasteurized milk as children, were teetotallers, ate a high animal fat diet, and were well educated. Of greater interest was the observation that six of the ten cases were related in two family groups.The only time all patients lived in the community at the same time was in 1951 and 1952 during a polio outbreak. The relationship of polio to multiple sclerosis bears further study.The average age of the patients when they had measles was 11.8 years. Evidence suggests a link between risk of multiple sclerosis and both late onset of measles and pubertal age. Late onset of measles may be important in this cluster. Further epidemiological studies are needed to examine the age of onset of measles in M.S. cases.
Shy-Drager Syndrome. Neuropathological Correlation and Response to Levodopa Therapy
Authors: De Lean Jacques , Deck John H.N. 
Published: July 2016

SUMMARY:The post-mortem examination of the nervous system of a patient with Shy-Drager syndrome successfully treated with levodopa (Sharpe et al, 1972) revealed features of striato-nigral degeneration and amyotrophic lateral sclerosis, a cerebellar system degeneration and a loss of approximately 75% of sympathetic preganglionic neurons. Lewy bodies were not present and no detectable changes were observed in the sympathetic prevertebral ganglia.While the limited and transient beneficial effect of levodopa on the bradykinesia in our case is possibly due to the progressive loss of striatal dopaminergic receptors seen in striatonigral degeneration, we propose that in Shy-Drager syndrome, levodopa therapy benefits orthostatic hypotension because of a suppression of the central depressor action of this drug. This suppression is attributable to functional disconnection of sympathetic ganglia secondary to the loss of preganglionic neurons or to degeneration of central autonomic catechoiaminergic systems.
Intracranial Dysgerminomas
Authors: Leblanc G. , Francoeur J. , Copty M. , Contreras C. , Gagne F. 
Published: July 2016

SUMMARY:We have reported three cases of intracranial dysgerminoma. The origin of these tumors has been controversial for a long time. It is now accepted that they develop from germ cells. The diagnosis is often difficult to confirm because of the variety of signs. If their origin is now accepted, the best way to deal with these lesions is still controversial. They can be macroscopically removed if they are in the pineal region. Such treatment is not possible for suprasellar tumors. Since these tumors can metastasize, radiation should be administered as soon as a pathological diagnosis is obtained. It is our belief that it should be administered to the whole spinal axis.
Progress in Understanding and Treating Parkinson's Disease
Authors: Barbeau André 
Published: July 2016

SUMMARY:This review evaluates the long-term results of Levodopa therapy in Parkinson's disease upon quality of life, prolongation of survival and excess mortality. It also focuses on recent and new therapeutic approaches: Levodopa in comhindation with a Dopa-decarboxylase inhibitor or MAO-B inhibitor, dopamine agonists and an active tripeptide: L-prolyl-L-leucylglycine amide (M1F-I). It ends by looking at new avenues of etiological research in Parkinson's disease which may indicate specific accelerated ageing of catecholaminergic (pigmented) neuronal systems.
Integrative Versus Delay Line Characteristics of Cerebellar Cortex
Authors: MacKay W.A. , Murphy J.T. 
Published: July 2016

SUMMARY:In order to determine which of two general models (“tapped delay line” or “integrator”) provides a more accurate description of mammalian Purkinje cell (P-cell) activation by natural stimulation, the spatial and temporal characteristics of a population of neurons in cerebellar cortex responsive to small controlled stretches of forelimb muscles were examined in awake, locally anesthetized cats. Stretch of a single wrist muscle excited P-cells over a distance of about 1 mm in the long axis of a folium, a span which is at most half the length of parallel fibers. Both granule cells and molecular layer interneurons were excited over a wider zone than P-cells.Furthermore, P-cells across a response zone all fired on the average at the same time, as determined by computing peristimulus cross-interval histograms from pairs of simultaneously recorded neurons. Consistent delays could only be demonstrated in the minimal response latencies as measured from peristimulus time histograms. These delays, however, were longer than could be ascribed to parallel fiber conduction velocity.No evidence, therefore, was found in cat cerebellum to support the “tapped delay line” model, which postulates the successive activation of P-cells as an excitatory volley travels along a parallel fiber beam. Instead, an integrative mode of operation seems to predominate: a relatively wide substratum of activated granule cells simultaneously activates a narrower focus of P-cells centrally situated with respect to the granule cell population. The role of inhibitory interneurons in promoting the “integrator” model is discussed.
Ultrastructural Classification of Pituitary Adenomas
Authors: Horvath E. , Kovacs K. 
Published: July 2016

SUMMARY:Forty-eight surgically removed pituitary adenomas have been investigated hy electron microscopy. Distinct differences in fine structural appearances permitted their separation into 5 classes: 1) Growth hormone, (GH)-cell tumors; 2) Prolactin cell tumors; 3) Mixed adenomas composed of GH and prolactin cell's; 4) Adrenocorticotrophic-Melanocyte stimulating hormone (ACTH-MSH) cell tumors; 5) Undifferentiated cell adenomas. Densely and sparsely granulated tumors were distinguished within forms appeared to represent well defined entities, they may be variants of the same tumor differing only in pace of hormone production and/or release. Number and size of secretory granules varied considerably among tumors composed of the same cell type indicating that pituitary tumor classification cannot be based solely on granule morphology. This classification takes into account morphogenesis with emphasis on clinical features and structure-function relationship.
Cerebral Blood Flow in Patients with Intracranial Pressure Elevation due to Traumatic Brain Edema
Authors: Tweed W. A. , Overgaard Jørn 
Published: July 2016

SUMMARY:The object of this study was to determine if traumatic brain edema (BE) and increased intracranial pressure (ICP) reduce cerebral blood flow (CBF). Two groups of patients were studied, one with slight BE and ICP less than 20 mm Hg., the other with pronounced BE and ICP over 20 mm Hg. Although ICP was higher and cerebral perfusion pressure lower in pro-nounced edema there was only a small and non-significant reduction in CBF and no difference in cerebro-vascular resistance. Since traumatic BE does not increase resistance to blood flow through the brain, cerebral perfusion can be maintained if an adequate perfusion pressure is established. This in turn, demands the monitoring and control of ICP.
Neurophysiology of a Central Baroreceptor Pathway Projecting to Hypothalamic Vasopressin Neurons
Authors: Jhamandas Jack H. , Renaud Leo P. 
Published: July 2016

Abstract:Controversy exists as to the neural network whereby peripheral arterial baroreceptor information is transmitted to vasopressin (VP)-secreting neurons of the hypothalamic supraoptic nucleus (s.o.n.). In vivo electrophysiological studies in the rat were undertaken to characterize the selective depression of VP cell activity consequent to activation of peripheral baroreceptors. Electrical stimulation of the diagonal band of Broca (DB) in the rat evoked a similar selective inhibition of vasopressinergic neurons of the s.o.n. Local application of bicuculline, a GABA antagonist, abolished both the DB-evoked and baroreceptor-induced inhibition of VP-secreting neurons. In addition, recordings from DB neurons antidromically activated from the s.o.n. displayed an increase in firing consequent to baroreceptor activation, coinciding with the suppression of firing in s.o.n. VP neurons. These observations collectively indicate that an intrinsic GABA projection arising in the DB cell group selectively inhibits vasopressinergic neurons of the s.o.n. and that this pathway mediates peripheral arterial baroreceptor activity that influences the release of VP in the neurohypophysis. These data may be of critical importance in our understanding the etiology of those forms of experimental hypertension where abnormalities in central baroreceptor pathways have been implicated but not proven.