Case of pseudo-hypertrophic paralysis (of Duchenne) occurring in an adult / by D. Dyce Brown.

  • Brown, D. Dyce.
Date:
1870
Catalogue details

Licence: Public Domain Mark

Credit: Case of pseudo-hypertrophic paralysis (of Duchenne) occurring in an adult / by D. Dyce Brown. Source: Wellcome Collection.

    8/12 (page 8)
    nearly all the voluntary muscles may be paralyzed, it does not follow that they should all be hypertrophied; or, in other words, the degree of paralysis does not proceed in the direct ratio of the hypertrophy. The description of the anatomical state of the muscles causing this apparent hypertrophy, I shall reserve till we finish the consideration of the general symptoms. (6.) Then comes a period when the progress of the disease comes to a standstill for a varying period, generally several years, during which time the hopes of the friends are apt to rise. But— (7.) After this stationary period, the disease starts afresh. The weakness of the lower limbs increases till the patients are unable to stand upright, and are confined to their bed or couch; the upper limbs become likewise enfeebled, and often become meagre and atrophied, so that the patients are in a nearly helpless condition, during which they are cut off by some intercurrent disease, such as phthisis. There are no cerebral symptoms which are uniformly present. In some cases there has been slowness of speech, with blunting and dulness of the intellectual powers, even idiocy, while in others the mind has been perfectly intact. The electro-muscular contractility is at first quite normal, and it is only in the advanced stage of the disease that this is dimin¬ ished or lost. The temperature of the enfeebled and hypertro¬ phied muscles is generally lower than normal, and the skin has sometimes a rosy or bluish tinge, but neither of these phenomena are constant. It is also to be observed that, in general, no pain is complained of, and there is neither hypersesthesia nor anaesthesia. The functions of the bladder and rectum remain normal, and the general health is only affected at an advanced stage of the disease. We come now to inquire what are the anatomical alterations which are found to exist in pseudo-hypertrophic paralysis. As to the state of the nervous centres, the results are purely negative. Only one autopsy has hitherto been obtained, and in it no lesion of the nervous centres was detected. Then, as to the condition of the muscles : this has been ascertained in the dead body, and in several other cases in the living body also. This latter has been accom¬ plished by a peculiar instrument, used by Dr Duchenne for extract¬ ing a piece of muscular tissue from the living body, and called by him u emporte-piece histologique.” In the first place, the apparent hypertrophy of the muscles has been found to be the result, not of increase in number or size of the muscular fibres, but of an hypertrophy of the connective tissue, which normally exists. This is what occurs in the second stage of the disease, namely, when the muscles begin to enlarge. Besides this hypertrophy of the ordinary connective tissue, there is also a formation of numerous fibres, with fine undulations. A little further on, in the course of the disease, disseminated among the connective and fibrous tissues, there are found fatty particles of different sizes, and sometimes drops of oil, in clusters.