Muscular Atrophy Considered as a Syjnptoni 253 



thigh become affected. The muscles of the hand and fore- 

 arm ma)' become involved after a lapse of 3'ears, producing 

 the peculiar " main en griffe " so characteristic of the Du- 

 chenne- Aran type. This form of atrophy begins as a rule in 

 early life, is a family disease, attacks and progresses uniformly 

 on both sides, produces a double club foot, is attended at 

 times with slight disturbances of sensation and vasomotor 

 changes, and retains the tendon reflexes to a late stage. 



The pathology of these forms has been the subject of long 

 and earnest controversy. The peripheral or myopathic origin 

 was stubbornly held by Friedreich and the German school, 

 while Cruveilhier, Charcot, Lockhart Clarke and others clung 

 to the central or spinal origin theory. The latter is now the 

 one universally accepted. 



The ventral cornua of gray matter present the results of a 

 subacute inflammatory process leading to complete or partial 

 destruction of the ganglion cells, sclerotic changes in the 

 neuroglia, blood-vessel changes, cell proliferation, etc. The 

 contraction of the newly formed connective tissue may even 

 lead to the formation of cavities in the gray matter. ( See 

 Plate, fig. 4.) The ventral spinal roots are affected second- 

 arily, likewise some of the efferent nerve fibres. Charcot's 

 theory, then, is as follows : Atrophy of the muscular fibers is 

 the direct result of irritation, which, beginning in the gang- 

 lion cells of the ventral cornua, is propagated through the 

 ventral spinal roots and efferent nerves to the muscular fiber. 

 Friedreich's theory was that the primary insult was a myositis 

 with secondary changes as ascending neuritis of the peripheral 

 nerve trunks, which terminated in a chronic myelitis. 



The pathology of the Charcot-Tooth or peroneal type is still 

 sub judice. Hoff"man of Heidelberg has studied this form verv 

 carefully and has declared it to be of neuritic origin. He 

 therefore, has proposed to designate it ''progressive neurotic 

 muscular atrophy. ' ' Other observers still cling to the spinal 

 theory, and until definitely proven by careful microscopical 

 examination that it is primarily a disease of the peripheral 

 nerves it may be classed among the atrophies of spinal origin. 



I have under observation a case of this type of atrophy in 



