Abstract
This rare disease was first described as an independent affection by Dejerine and Sottas.1 Previous to their publication Gombault and Mallet2 published the account of an observation of a patient having the characteristics of this affection, but they considered it as a case of tabes. There are but three autopsies recorded, those of Dejerine and Sottas,1 of Dejerine and Thomas3 and the case of Pierre Marie studied by Boveri.4 This disease has been studied chiefly in France. The original descriptions of Gombault and Mallet, Dejerine and Sottas and a subsequent publication by Dejerine5 give a complete and thorough description of the affection from a pathological as well as a clinical standpoint. A good account is found in the work of Dejerine and Thomas,6 and it is briefly considered by Pierre Marie7 in his book on neurology. Short accounts are also found in

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