A system of medicine / by many writers.

Date:: 1911

Credit: A system of medicine / by many writers. Source: Wellcome Collection.

Provider: This material has been provided by UCL Library Services. The original may be consulted at UCL (University College London)

56/944 page 32

$congenital. Sir W. Gowers has suggested the name abiotrophy as indicating a defective vital endurance, and in this sense the myopathies might bo included under such a title. History.—The first cases described were those now known as pseudo- liypertruphic paralysis, of which instances were recorded by Sir Charles Bell (in 1S:3()) and by Partridge (1847). Meryon, who gave a very accurate description of four boys in one family aftected by atrophy with contractures, published in 1852 an account of a necropsy, in which he states that the spinal cord and nerves were carefully examined microscopically, and not the slightest trace of disease was detected in the ganglion-cells of the grey matter and the anterior roots ; the only structural change observed was in the muscular Hbres, Avhich were broken down and converted into oil-globules and granular matter; of these changes he gave drawings. Meryon called the disease a granular and fatty degeneration of voluntary muscles. In isr).3 Little referred to two exemplary cases of pseudo- hypertrophic paralysis, which he observed in 1847 in two brothers with enlarged calves, talipes e(|uinus, lordosis on standing, kyphosis on sitting, and atrophy of the upper arms with large deltoids; at the necropsy the gastrocnemii were found large and fatty with traces only of muscle-tissue, but no mention is made of the spinal cord. In 1855 Duclienne ])ublish(Ml cases of progiessive muscular atrophy in adults and childicn, and descri))ed some of these cases in adults as beginning in tlic tmiik nuiscles or -lioiiMrr, and in the latter as affecting the facial nuiscles, and especially the ()rl)i(iilaris oris; but he ascribed them to lesions of the cord. Duchenne, in 1855, speaking on atrophic mus- culaire graisseuse, and on a necro})sy of a case by Cruveilhier, refers to Mei-yon's case, the figures illustrating which, Duchenne says, have a great similarity with the features he had seen in cases of atrophic musculaire graisseuse,'' and he dismisses the matter with the opinion that Meryon's case was one of a muscular affection commencing in infancy, of which state I have seen a go(jd number, and which I will call Paralysie atrophi(jue graisseuse de I'enfance, a disease which, from his description on J). 8:J9 {loc. cif.), was evidently infantile jjaralysis. Meryon, in 1804, described a necropsy on a second case in 1859, in \s liicli he states that not a trace of disease was found in the central nervous system, also that these cases may be recognised by the absence of any symptom of central disturbance, and that they are not the same as simple nuiscular atrophy, but are due to lesions of the muscles only, lie says, M. Duchenne has referred my first case to a category of disease which he designs as nervous in character, resulting from some antecedent febiile affection, and terminating in rapid recovery or in degeneration into fat of the affected muscles, and he calls this 'Atrophic graisseuse de I'enfance,' . . . but my cases have nothing in common with these. Meryon further shewed the hereditary nature of his cases ; and also stated that in one family there was an uncommon increase of the gastrocnemii. He further states, I am induced to believe in an idiopathic disease of the muscles, dependent perhaps on defective nutrition.$

A system of medicine / by many writers.

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