910 WALTEE TIMME 



dystrophy; and lie cites Friedreich that bone atrophy is not secondarily 

 due to disease of the bone through immobility of the- joint, but to nervous 

 and trophic influence. Eulenburg had a case which was combined with 

 acromegaly. Hlahn's conclusion is that because so many cases show bony 

 changes, there may be some connection between them. 



Bregman cites cases in which together with a "facies myopathique" 

 there was difficulty in looking up and in closing the eyes ; inequality of 

 the pupils and of the palpebral fissures. This is interesting from the fact 

 of the frequent involvement of the pineal gland ; in which condition just 

 such eye muscle difficulties arise (Bailey and Jelliffe). Bregman's cases 

 further showed internal glandular disturbances as follows : In Case 1 the 

 patient could not close the eyes properly and had nyctalopia; also there 

 was intense sweating of the extremities with cyanotic hands and a pulse of 

 100. In Case 2 there was marked prognathism, the upper teeth standing 

 prominently forward; the skin was mottled like marble. In Case 4 the 

 hands and feet were livid; there was large skeletal development of the 

 hands and feet in contrast to the rest of the skeleton,, and marked pro- 

 trusion of the upper jaw. In Case 5 there was extremely large body 

 growth. In these cases there is manifestly disturbance of both pituitary 

 and thyroid glands. 



Cestan and Lejonne publish two cases with contractures and state that 

 it is banal to say that contractures accompany all forms of progressive 

 muscular dystrophy, but that ordinarily the contractures are slight and 

 rarely sufficiently marked to alter the general attitude of the myopathic 

 patient. Schultze presents two cases, both with necropsv, of brother and 

 sister ; in the former there was a thinning of the long bones, the humerus 

 being thinner than the middle finger of a normal hand, and the medullary 

 cavity very small. The sister had a stiffness in all the joints of the body, 

 with atrophy of the bones. Schultze states that he had found only two 

 other instances in the literature in which was found bone atrophy with 

 muscular dystrophy, one a case of Friedreich's and the other Le Gendre's. 

 In all these cases the atrophy was a concentric one of the long bones with 

 no diminution in the length. In Le Gendre's case there was also an undue 

 hypertrophy of the genitals, with an enlarged prostate gland in a youth 

 aged 20 ; while Friedreich's case showed infantilism in the sexual organs, 

 voice and facial expression. The necropsies in Schultze' s cases showed no 

 apparent changes in the spinal cord, not even in the ganglion cells, and he 

 therefore concludes that we must look elsewhere than in the nervous system 

 for the cause of this "riddle-like disease/ 7 and advises us that we cannot 

 neglect the theory of predisposition, with accidental factors superimposed, 

 such as overstrain, trauma, underfeeding, infection and intoxication. 



O. With reported a familial type of the disease affecting three boys 

 in one family and sparing the four girls. What interests us in these cases 

 is the involvement of the tonsils, which were hypertrophied, a chronic 



