A Case of Progressive Muscular Sclerosis, with a Paper on the Same

William Ingalls, S. G. Webber
1870 Boston Medical and Surgical Journal  
Read before the Suffolk District Medical Society, Sept. 24th, 1870. J. S., of Irish parentage, five years and two months old, was admitted into "The Children's Hospital" on the 2d September, 1870. To the age of three years ho was quite a healthy child, but at about that period he began to move and act as though he had less strength than usual, and by degrees his mother came to acknowledge that such was really the case ; this condition increased and the spinal column became very weak, and the "
more » ... nward crook " of it was noticed by her.. Two weeks before admission ho had a whitlow upon the fore-finger of his left hand, and one week before, he had a fall while attempting to run upon a sidewalk ; from the first event the mother dated the special failing of his health, which she thought was increased by the second, and the evident decrease of his vital powers induced her to seek for him the benefits of the Hospital. When first seen by the writer, the child was sitting in a corner of his bed, a soft pillow being behind him, he being in such a position that if a line had been carried from-the end of tho spine, over it, to the back of his head and continued on the same curve, it would have formed a circle, or nearly so. On tho day after admission, Dr. Webber saw the patient with me, and at once recognized the disease* Wo caused him to stand upon the floor, and he walked a few steps in a tottling or shambling manner. The spine presented a regular and exaggerated curve inward, from the third or fourth dorsal vertebra to the sacrum. This shape comes well under the name given by Duchenne-"saddle-back." A perfect nie-turc of this child may bo seen in Duchenne's book, electrisation localisée, 2ud edition, 1861, p. 355. The head, while he was standing, and indeed while he was in any position which might have been called upright, gave to the spectator tho idea that it was large and heavy, for tho chin rested upon the sternum, and was inclined moro to the right shoulder than to the left. A copy from Duchcnne is presented. The muscles of the calves were largely developed, and the nates seemed to be so.
doi:10.1056/nejm187011170832001 fatcat:hkelt652one3dnswivalkm3v4i