뒤시엔느형 근디스트로피에서의 교감신경 피부반응

Abstract

Duchenne muscular dystrophy(DMD) is an X-linked recessive disease, caused by the mutation of dystrophin gene at Xp21. The dystrophin produced by this gene is therefore absent on the membrane of muscular fiber in the patients with DMD. Recently, it is known that the dystrophin has also been located on the myoepithelial layer of sweat gland in the mice.

We studied the sympathetic skin response(SSR) in a group of DMD patients and a control group to evaluate the function of sympathetic nerve and sweat gland in DMD patients.

Significant prolongation of latency of SSR in the palm and sole was noted in the group of DMD patients compared to the control group. However, there was no significant difference in the amplitude of SSR between two groups. In the patient group, the rise in latency of SSR was closely correlated with the duration of symptoms and weakly associated with the stage of the illness.

Therefore the latency of SSR may be a useful index in assessing the function of sympathetic nerve and sweat gland in DMD patients. These results could be a consequence of a lack of dystrophin at myoepithelium of sweat gland in DMD patients.