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.

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    Next, as to the state of the muscular fibres themselves, they are found (1.) discoloured, presenting a pale appearance, and resem¬ bling the subcutaneous cellulo-adipose tissue. (2.) The normal transverse striation becomes extremely fine, and in some parts hardly to be seen, till (3.) the transverse striation completely dis¬ appears. (4.) The transverse diameter of the muscular fibres be¬ comes very much diminished; there is, however, no parallelism between the diminution in the transverse diameter and the fineness of the striae, as it has been found that in fibres whose transverse diameter has been normal the striae have become very fine, and have even entirely disappeared. (5.) There is a marked diminu¬ tion in the number of muscular fibres in proportion to the quantity of hypertrophied cellulo-adipose tissue. Griesinger found that in one case the intermediate tissue occupied six or eight times larger space than the muscular fasciculi. If the muscular fibres had not really degenerated, the size of the muscles involved ought to be larger than it is, judging from the enormous hypertrophy of the intermediate connective and fibroid tissues. But there is good reason to believe that the muscular fibres do really degenerate and disappear, and that probably these undulated fibres, which exist among the connective or areolar tissue, are in reality what remains of empty sarcolemmata. The fatty globules, however, are not part of the muscular fibres in a state of fatty de¬ generation, but are entirely outside of them. The anatomical state of the muscles shows completely that it is an entirely different disease from that known as progressive fatty atrophy of the muscles. The symptoms of the disease are so marked, and so different from those of any other, that the diagnosis is hardly to be mistaken. What, then, is the ultimate cause of this muscular disease ? It has been already mentioned that no lesion of the brain has been found ; and although in some cases there was obtuseness of the intellectual faculties, even going the length of idiocy, yet in others there was no symptom of this sort, the mind being quite intact. Besides, as Dr Duchenne remarks, a cerebral lesion would not be sufficient to account for the morbid condition of the muscles. He also observes that, reasoning by analogy, we should not expect to find the seat of the disease in the spinal cord ; and at the autopsy above referred to, the spinal cord was found intact. Dr I)u- ehenne is inclined to place the seat of the disease in the vaso-motor nerves, and states that he cannot conceive of the production of such an increase of connective tissue, unless the vaso-motor nerves are involved and paralyzed. The facts that in some cases the skin has been of a rosy or bluish colour, and that the temperature of the affected parts is generally lowered, would seem to go in favour of this view. But no lesion of the ganglionic system has as yet been discovered. M. Bergeron believed that the paralysis of motor power was caused by the compression exercised on the muscular