Clinical lecture on a case of progressive muscular sclerosis (pseudo-hypertrophic muscular paralysis of duchenne). / by William Pepper.

  • Pepper William, 1843-1898.
Date:
[1871?]
Catalogue details

Licence: Public Domain Mark

Credit: Clinical lecture on a case of progressive muscular sclerosis (pseudo-hypertrophic muscular paralysis of duchenne). / by William Pepper. Source: Wellcome Collection.

Provider: This material has been provided by the Royal College of Physicians of Edinburgh. The original may be consulted at the Royal College of Physicians of Edinburgh.

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    of the surrounding connective tissue, and undergo a delusive increase in size and power.* Thus, I find that the fibrils in the gastrocnemii muscles of this patient are fully one-third wider than those in his left deltoid, and that some of the former have acquired the enor- mous size of jjj// in transverse diameter. Whether this transitory stage usually exists or not, the sclerotic change does not depart from its inevitable law of development. Already in this second stage we have seen patches of fat-globules appearing in the interfibril- lar spaces, and as the disease passes into the final stage, this fatty degeneration of the muscle advances with varying rapidity, even leading in some cases to such an accumulation of fat as to be visible to the unaided eye as yellowish streaks. This extreme condition is, however, very rare, and much more frequently the accumulation of fat is moderate. Even when very great, however, it is found to be far more due to in- crease of the interstitial fat than to a true fatty degen- eration of the muscular fibrils. Their nutrition must be, however, very gravely impaired, so that they un- dergo atrophy, and in many places entirely disappear, leaving their sheaths empty. This change coincides with the rapid extension of paralysis which character- izes the final stage of the disease. This detailed account of the muscular lesion naturally leads to the question of the pathology of this curious affection. It cannot be held that the disease depends upon or is essentially connected with any cerebral lesion. It is true that in a number of cases the patients have been idiotic, or at least exhibited marked impairment in intel- lectual development: in the present case, also, epileptic convulsions are present as a complication. It must, how- ever, be remembered that cerebral disturbances of any kind whatsoever are not uniformly present, that dis- orders of special sense are rare, and that some cases of the disease have been observed associated with a nor- mal state of the intellectual faculties ; and, finally, that in the one case where the nervous centres have been * This enlargement of the size of the fibrils of the gastrocnemii has also been observed by Leyden (loc. cit.).