J Korean Acad Rehabil Med.  1997 Feb;21(1):87-94.

Sympathetic Skin Response in Patients with Duchenne Muscular Dystrophy

• Jung KI
• Kang SW
• Moon JH

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 patie nts 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 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 group. 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 dvstrophin at myoepithelium of sweat gland in DMD patients.

Keyword

Duchenne muscular dystrophy; Sympathetic skin response; Dystrophin; Myoepithelium; Sweat gland

MeSH Terms

Animals
Dystrophin
Humans
Membranes
Mice
Muscular Dystrophy, Duchenne*
Skin*
Sweat Glands
Dystrophin