뒤시엔느형 근디스트로피 환자의 포괄적 임상 고찰

Abstract

Objective: To evaluate clinical features in general and possible complications in Duchenne muscular dystrophy (DMD) which could be used for comprehensive rehabilitation management.

Method: One hundred and seventy-two patients with DMD were followed over 3 year period to provide clinical profile causing impairment and disability. We measured height, weight and manual muscle testing (MMT) when the patients visited the hospital. And we could measure pulmonary function, electrocardiogram (EKG), and intelligence quotient (IQ) test in cooporative patients.

Results: The median height and weight of DMD boys were normally distributed before age 12, but during the second decade height was markedly reduced, and weight was no longer normally distributed. The MMT measurement showed loss of strength in a fairly linear fashion according to increasing age, and extensor of lower extremities were weaker than flexors showing typical contractures of legs. There was a direct relationship between pulmonary function and MMT scores of upper extremities. There was a high occurrence (40%) of abnormal EKG, but none of the patients had a history of cardiovascular complication. DMD children suffered wide spectrum of psychological disturbance such as somatic complaints, attention and emotional problems in addition to expected psychological problems due to chronic disease and its progression, and 50.9% of them were below average on the IQ test.

Conclusion: These data on DMD subjects provide clinicians with useful information regarding the prevalence and severity of measurable impairment at different stages of the disease.