246 



De. PAGET, ON INSTANCES OF REMARKABLE ABNORMITIES 



Since the above was written, my brother has sent me the particulars of another case which 

 he has seen in the last few days, and which in many respects has a striking similarity to my 

 own. The following are his notes: "B, B., now 9 years old, tall and very slim like both his 

 parents. All his muscles, except those of the legs, appeared very small : but especially the 

 pectorales and latissimi dorsi were so, and the borders of his axillae felt quite unnaturally thin 

 and weak. He walked and ran with his loins extremely hollowed, and his belly projected ; 

 he shuffled in walking, and easily broke into a run, and was apt to fall on slight occasions. 

 Through inability to raise his thighs he could not walk up stairs : and when he was laid flat 

 on his back, he could rise only by turning over on his side, and then getting up on hands and 

 knees — something like as a horse does. His defect in the movements of the thighs was as if 

 he had no psoas muscles ; but I could not be sure of their absence, though they certainly were 

 very small. His thighs were as lank and weak as any other part of him ; but his legs, and 

 especially the calves, were as large, firm and muscular as those of one of the very strongest 

 boys of his age. When he stood erect there appeared a very slight curvature of the lumbar 

 spine to the right ; and when he stooped this became much more marked, and was accompanied 

 with an uprising of the soft parts at the right side of the curvature, as if the right sides of the 

 vertebrae in this region were rotated backwards. No other distortion appeared in any part. 



" No cause of the defective state of the muscles could, in this case, be assigned. The 

 parents of the patient are wealthy and in good station : they are both well-formed, but have 

 delicate health ; and some of the mother's family have been phthisical." 



There was no earlier notice of the defects than that the boy, from the first, had an awkward 

 gait. 



It is unnecessary to indicate all the points of resemblance between this case and the two 

 which have fallen under my own observation : but it puts beyond a doubt the reality of a 

 relation between the extraordinary development of the calves and the muscular deficiencies 

 elsewhere. 



It may be worthy of observation that no deformity of the chest existed in any of these 

 cases. Rokitansky*, and with him many orthopaedists, have referred chicken-breast (Pectus 

 carinatum) to atrophy or paralysis of the Pectorales and Serrati muscles. The cases here 

 related prove that whatever may be the influence of defect of these muscles, in occasioning the 

 deformity, when the bony and cartilaginous walls of the chest are unsound, the same deformity 

 will not ensue if the skeleton be sound, even though the muscles may be wholly wanting. 



We cannot penetrate very deeply into the Pathology of these cases. Our materials are too 

 scanty -f*. In the two boys who came under my own notice, it seems reasonable to conjecture 

 that the state of the mother's health during gestation was the primary cause of the muscular 

 defects in her off'spring. There is reason for this in the fact of her having had on these two 

 occasions (and on these only of her numerous pregnancies) a state of bodily disorder, which 

 manifested itself by definite and peculiar symptoms. But admitting the mother's ill-health to 



• Pathologische ATiatomie^ B. ii. p. 291. 

 f 1 can find on record no other such case of Congenital 

 defect of muscles ; nor is any known to Professor W. Vrolik 



of Amsterdam, the author of the most complete work on mal- 

 formations that has yet been published. 



