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Journal of the Korean Academy of Rehabilitation Medicine 1990;14(1):7.
Clinical Study of Progressive Muscular Dystrophy
Jae Ho Moon, M.D., Hoon Jang, M.D., Ae Young Kim, M.D. , Jung Soon Shin, M.D.
Department of Rehabilitation Medicine, Yonsei University College of Medicine
진행성 근 디스트로피의 임상적 고찰
문재호, 장훈, 김애영, 신정순
연세대학교 의과대학 재활의학교실

Muscular dystrophy is usually considered to be a chronically progressive disease leading to disability in physical, psychological and social aspects. Management should include early diagnosis, establishment of a rehabilitation plan, maintenance of ADL and ambulation as long as possible, anticipation of complications, development of a program for prevention and supportive counselling of the patient and family.

We studied 106 progressive muscular dystrophy patients under the headings of type, electromyographic findings, family history, muscle enzyme levels, functional stage and vital capacity.

The results of these studies were as follows:

1) 106 patients comprised 66 cases of Duchemme type, 23 cases of limb-girdle type and 17 cases of facioscapulohumeral type.

2) Duration from symptom onset to initial visit was 3.7 years in Duchenne type, 8.1 years in limb-girdle type and 8.5 years in facioscapulohumeral type.

3) Suspected symptoms in family members were reported 30.3% in Duchenne type, 21.7% in limb-girdle type and 47.1% in facioscapulohumeral type.

4) Change of functional stage in Duchenne type was from 2.5 to 3.1 after 8.5 month follow up.

5) In the same age group, functional stage of the early visit group was higher and the rate of progression was slower than in the late visit group.

6) After intensive pulmonary care program, vital capacity increased in the group with high level of functional stage.

Key Words: Progressive muscular dystrophy, Functional stage, Vital capacity


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