MULTIGLANDULAR SYNDKOMES 909 



The disease lasts from 20 to 40 years, with periods of quiescence. Erb 

 states that this juvenile form is identical with Friedreich's hereditary 

 progressive muscular atrophy. These latter forms belong in the same 

 group with pseudohypertrophy. 



In one publication, Erb gives the results of the examination of the 

 available pathologic material of all these forms and says in summarizing 

 that all the noticeable changes are in the muscle fiber chiefly, from an 

 increase in volume to absolute disappearance of the muscle. There is an 

 increase in the muscle nuclei and these are both normally and abnormally 

 placed. There are clefts in the long axis of the muscle between the 

 muscle fibers and an increase of connective tissue is found with a later 

 deposit of fat cells and a thickening of the vessel walls. As a result of 

 the identical pathologic findings in all forms of this elusive muscle disease, 

 he proposes the classification of all these forms as types under the generic 

 term of dystrophia musculorum progressiva. This classification has 

 already been given earlier in this chapter. 



Among other types the next cases of importance in the development 

 of the semeiology of the disease were reported by Prager. Here the 

 patients, two in number, were children of first cousins, who themselves 

 were free of the disease. The importance of the citation of one of these 

 cases is in the fact that while the patient, a woman of 48, had always had a 

 waddling gait and a difficulty in going upstairs, yet after her first puer- 

 perium, which lasted three months, the difficulty in walking was so in- 

 tensified that she needed support. Her muscular system gradually under- 

 went the usual atrophies in the pectorals, latissimi, trapezius, rhomboids, 

 biceps, deltoids, triceps, and supinator loiigus ; but these atrophies were 

 disguised by masses of fat in the arms and legs. To these physical signs, 

 however, were appended the statements : ( 1 ) the patient was easily excited 

 and then developed a tremor; (2) there was difficulty in deglutition for 

 fifteen years; (3) dyspnea in cold weather, and (4) increase in stools. 

 Further on is the remark that the patient had a "siruma" on the neck and 

 complained of urticaria. That is, she was a hyperthyroid subject. We 

 shall find as we go along in the analysis of the symptoms as presented by 

 the author frequent indications of disturbance in the endocrin system. In 

 all probability, had such indications been then understood, there would 

 have resulted a far wider range of symptoms in the muscular dystrophies ; 

 for what to the older observers probably appeared negligible in this regard, 

 now assumes great importance. We shall see how more and more often in 

 later years investigators have described conditions present in progressive 

 muscular dystrophy, apart from the actual muscle changes, that are seem- 

 ingly part of the disease process ; which conditions have received but scant 

 reference in the earlier works, presumably because they seemed so utterly 

 adventitious. So Hahn states that skeletal anomalies are often seen and 

 the idea is now that these are part of the condition of progressive muscular 



