[Treatment of multiple sclerosis by Azathioprine. About 77 cases studied during 10 years (author's transl)].
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Biomedical subjects
Publications and source records attributed to M Devic.
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The authors report a case of grave orthostatic hypotension (Shy-Drager's syndrome) with major postural disturbances. The biological test confirmed a catecholaminergic deficiency. After several drugs were tried unsucessfully, an association of L-Dopa and fractionated doses of mono-amine-oxydase inhibitor was proposed. The increase blood pressure was sufficient to block the diturbances of postural adaptation, without inducing hypertensive jerks. The functionnal result have been stable for three years, while the parkinsonian syndrome have shown little progression.
Five cases of neurological disorders occurring after extra-corporeal circulation are presented owing to the singular nature of the clinical picture made up by the essential element of a progressive brachial diplegia, free from any sensory disorder. The topography of the lesion--single medullary lesion or bilateral encephalic--is discussed. An anatomic document enables the elimination of any médullary involvement in one of the cases, thus pointing to a bilateral central lesion. The physiopathological problems are looked at.
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The authors describe the results of immunological assay of complement factors C3, C4 (the usual path of activation of complement) and of B factor (the alternate path of activation) in 61 multiple sclerosis patients not receiving corticoids, 52 normal controls and 217 patients with other neurological disorders. Hypocomplementaemia (fall in factor C3 related to a fall in total haemolytic activity) was found in 29.5 p. 100 of the patients not on corticotherapy at the first assay, and in 36 p. 100 of the patients when repeated assays were carried out. Hypocomplementaemia is significantly more frequent in multiple sclerosis than in the normal population (0 p. 100) and in neurological patients (9.6 p. 100). In 13.1 p. 100 of the multiple sclerosis patients there was a decrease in B factor: 50.3 p. 100 of the multiple sclerosis patients exhibited no quantitative abnormality of the main factors of complement (normocomplementary multiple sclerosis). The group of multiple sclerosis patients with hypocomplementaemia was characterized by the incidence of other abnormalities in the complement system: cleavage of the C3 factor and a fall in B factor in 60 p. 100 of the cases. A more frequent increase in IgE and measles antibodies was found also while the normocomplementary multiple sclerosis patients more frequently had higher levels of IgA. Genetically, the group with hypocomplementaemia is related to a significant increase in the incidence of the HL-A W18 group while the normocomplementary multiple sclerosis patients appear closely related to the HL-A7 group. Familial investigations show that hypocomplementaemia is usually present in the ascendents and collaterals and that it seems to be transmitted with the HL-A haplotypes. Four families gave evidence of transmission with the W18 group. This transmission sometimes occurs together with transmission of an increase in IgE and/or of measles antibodies. In two pedigrees, one of the ascendents carried in his serum an activator of the alternate path of complement. There does not appear to be any prognostic difference between the two groups. In multiple sclerosis with hypocomplementaemia, the facts suggest a complex immunological abnormality, transmitted genetically to the subject and existing prior to the illness, comprising both elements of deficient and excessive immune response. The recognized presence of a gene of immunological reactivity and of genes of synthesis of complement on the 6th chromosome, in proximity with genes of histocompatability (HL-A and M.L.C.) provides a theoretical basis for this supposition.
Twenty-five cases of amyotrophic lateral sclerosis occurring before the age of 40, collected between 1963 and 1973 in the Neurological Hospital, Lyons, have prompted the authors to make the following observations. -The incidence of disease seems to have been on the increase during this period both in absolute terms and relatively speaking (relative to the total number of patients hospitalized and to the total number of patients hospitalized for amyotrophic lateral sclerosis after the age of forty). -The 25 cases involved mostly women--the opposite of what was found in cases of amyotrophic lateral sclerosis occurring after forty. -Only one was possibly familial. -The onset is mostly with purely pyramidal features or of the topographically circumscribed peripheral type. Development appears to take longer than the average for amyotrophic lateral sclerosis. It would be worth while confirming these findings by analysis of other similar groups.
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A case of pure graphic disorder without any disorder of speech, reading or praxis is described. The conditions under which it occurred after surgery for frontal oligodendroglioma would seem to confirm that a single frontal lesion is involve affecting in particular the foot of F2. This case is compared with similar ones which have appeared in the literature and are classified within the wider category of kinesthetic disorders caused by frontal lobe lesion.
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The authors present a retrospective study of 670 histologically verified tumours of the neuraxis collected over 90 months in the Neurological Hospital, Lyons. The main facts concerning histological type, topography of the tumour, date of diagnosis, address, profession, O.B.O. and Rhesus groups, age and sex were codified for feeding into a computer and processed by sophisticated statistical methods. This analysis shows the homogeneity of topographical distribution in the brain of the different types of glioma, the special genetic code of meningiomas (predominance of A and B genes of the A.B.O. group), absence of the same factors for glioblastoma. Sex ratio analysis shows the contrast between definitely masculine tumours--glioblastomas and astrocytomas--and meningiomas and spongioblastomas. Analysis of the "age specific rate" separates tumours prevalent in the young, where incidence is a decreasing function of age (spongioblastoma, medulloblastoma, cerebellar astrocytoma) and tumours prevalent in adults which are mainly meningiomas and glioblastomas whose incidence is an increasing exponential function of age. Epidemiological analysis shows the difference in geographical distribution of glioblastomas and meningiomas. As far as glioblastomas are concerned, it would appear that on to the general rule of constant tumourincidence over the area as a whole, is superimposed another of high incidence in particular districts of the Beaujolais and Maconnais regions. The aetiological implications of these various findings are discussed.
The problem has been considered of the action of Lithium salts on the progress of pregnancy and on the product of the conception. Lithium can bring about serious troubles in morphogenesis in invertebrate animals. The results obtained in mammals in laboratory experiments differ from species to species but it seems that these animals can be given about 8 to 9 times the levels of Lithium that are used in man without causing teratogenic effects. A statistical review of the children born to human mothers taking Lithium in pregnancy shows up an increase in the incidence of cardio-vascular abnormalities. (7.8% instead of 0.04%). The infants that have received Lithium and have not been anatomically affected show no more developmental problems later than others.