MULTIPLE DUCTLESS GLANDULAR SCLEROSIS 451 



relatively small. There were on the head only a few thin hairs, the eyebrows and the 

 lashes were absent, almost completely, as were also the hairs of the trunk. The lower jaw 

 was ill-developed, and there was especially a poor development of the clavicles. The mus- 

 culature was poorly developed, the genitalia were about one or two years behind in their 

 development, the skin was thin and dry, the nails were short and the anterior fontanelle 

 had not as yet closed, and certain of the temporary teeth were still present. There was a 

 falsetto voice; the intelligence was rather well developed. The individual made quite a 

 senile impression. At the age of eighteen, the boy died with symptoms that gave thought 

 to the diagnosis of "thymic or cardiac asthma." At autopsy was found an enlarged 

 thymus gland and atheromatous alteration of the mitral and aortic valves; the coronary 

 arteries were highly sclerotic. The capsule of the spleen was thickened, the kidneys were 

 fibrous, the walls of the intestines and stomach were so atrophied that they were almost 

 transparent. The liver was relatively large, the brain normal. The long tubular bones 

 were rather slender, but the ends much thickened. Especially the condyles of the femurs 

 and the arterial ends of the clavicles were swollen up. Ossification had been premature. 

 Microscopically the ductless glands (thyroid, hypophysis, testicles and suprarenals) were 

 apparently normal, there was no microscopical examination. 



Hutchinson observed a three-and-a-half-year-old-boy with the same disease. Hastings 

 Gilford saw this case when it was fifteen years of age and followed it to its death two years 

 later. There existed extreme emaciation, so that the bones and the tendons were very 

 prominent. The abdomen was large, the condyles of the tubular bones were rather thick- 

 ened, especially the distal epiphyses of the femurs, so that the patellas were pushed 

 forward. The fingers were short, the size of the hands corresponding to that of a three- 

 year-old child. The ossification represented that of a twenty-year-old youth. There 

 were symptoms of a mitral and aortic failure, and death occurred with symptoms of a 

 cardiac syncope. {There was no autopsy. 



The case of Variot and Pironneau was that of a fifteen-year-old girl. She weighed only 

 11.65 kg. like a two-year-old child was 102 cm. in height, size and dimensions corre- 

 sponding with those of a five-year-old child. The appearance was entirely senile. Apart 

 from isolated hairs on the head, the hair was entirely absent, even the hairs of the eyebrows 

 and lashes. The skin was thin and folded, the panninculus adiposus was entirely absent. 

 The muscles were relatively well developed, and stood up prominently through the thin 

 skin. The nails were very small, the tooth formation was very bad, and second dentition 

 was just appearing. Many teeth have fallen out without especial pain. The develop- 

 ment of the genitalia had remained behind, and breasts especially had not developed. 

 The intelligence was rather good. Up to the fifteenth month development had been en- 

 tirely normal, and from this time it had remained behind. X-ray examination showed 

 that the lower jaw was very atrophic and that the epiphysial junctures had for the most 

 part closed. Gilford Hastings reports also a case of Ransom that possibly belongs here. 

 It was concerned with a twenty-seven-year-old girl who was 135 cm. tall and weighed 16 

 kg. The father had been a heavy alcoholic. The girl's intelligence was feebly developed. 

 Diarrhea had existed from youth, and later there had been a diabetes. The genitalia in 

 their development represented those of a ten-year-old girl. The ovaries were absent. 

 The girl was markedly emaciated and looked pronouncedly senile. At autopsy there were 

 found fibrous changes in the spleen, lymph glands, the pancreas, kidneys, suprarenals, 

 thyroid, hypophysis, etc. The heart weighed 6 oz. (about 176 gm.) and there were athero- 

 matous changes in the aortic and mitral valves and in the aorta. 



The cases cited show the greatest similarity to one another. Already 

 Hastings Gilford remarked that his case and that of Hutchinson were so similar 



