[Reflections on a superacute myasthenic respiratory insufficiency treated by resuscitation and thymectomy].
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Biomedical subjects
Publications and source records attributed to J Boudouresques.
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Three children born of consanguineous parents had Lafora's disease and two of them had been studied clinically, pathologically and biochemically. The autosomal recessive transmission of the disease is confirmed by the familial study and the parents present no phenotypic anomaly of the disease. Electroclinical data are discussed with regard to the early differential diagnosis between this entity and idiopathic generalised epilepsy and myoclonic epilepsy without Lafora's bodies. Iodine spectrum of the cerebral and hepatic abnormal deposits suggest that Lafora's bodies mainly consist of long chains glycogen similar to amylopectin.
The authors report some observations about 228 cases of multiple sclerosis with secure diagnosis subdivided in severe (34,6 %), common (29,8 %) and benign after the tenth year of illness (35,6 %). These observations are in agreement with classical statistic reports concerning the natural disease history : higher female incidence (64 %), average age at onset about 29,5 years, 4 main first signs (ocular [42,5 %], motor [41,5 %], ataxia [36,3 %], sensory [35 %] and remitting course [82,5 %]). Follow up study of 81 "benign" forms after the tenth year of the disease allows us to look at the freqwuency of "secondarily worsening" forms (25,7 %) and "benign remaining" ones until the twentieth year (10,1 %).