PROGRESSIVE ATROPHIC MYOPATHY OF DISTAL ONSET

The authors present a 10 year old boy, who at the age of five began to have disturbs in the mobility of his hands. A few time later, a progressive atrophy of the thenar and hypothenar eminences with a predominance of the right side, appears. An electrodiagnostic revealed the absence of the reaction of degeneration. At the present, the same disorders and an outline of atrophy of the shouldergirdle are found. The mobility of the fingers is to disturbed that the movements of opposition of the thumb and the adduction of the other fingers are abolished. The strength of the hands is very diminished. There are no reflexive and sensitive disorders, nor fibrillary twitchings. Another electric study reveals the absence of a reaction of degeneration, and the rate of creatine in urine is very increased. With these semiologic facts the authors include this case among the primitive muscular dystrophies of distal begining. The patient was submited to a kinesiologic and medicative treatment comisting in oral administration of vitamin E and B,. After three months, they found a discreet improvement and great descent of the creatine in urine. The authors also make general considerations of this illness and specially of this strange form, of which only eight cases were described. They give a summary of them and a comparative synoptic table.