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Journal of the Korean Academy of Rehabilitation Medicine 1997;21(1):87-94.
Sympathetic Skin Response in Patients with Duchenne Muscular Dystrophy.
Jung, Kwang Ik , Kang, Seong Woong , Moon, Jae Ho
Department of Rehabilitation Medicine, Yonsei University College of Medicine, Korea.
뒤시엔느형 근디스트로피에서의 교감신경 피부반응
정광익, 강성웅, 문재호, 김철*
연세대학교 의과대학 재활의학교실, 인제대학교 의과대학 재활의학교실*

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.

Key Words: Duchenne muscular dystrophy, Sympathetic skin response, Dystrophin, Myoepithelium, Sweat gland


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