Complications in progressive muscular wasting diseases in the matured age.

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  • 筋萎縮性疾患成人患者の合併症の検討

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Abstract

In this study, we analyzed complications in the cases of slowly progressive muscular wasting diseases in the matured age. The subjects were 34 myotonic dystrophy (MyD), 26 limb-girdle muscular dystrophy (LG), 7 Becker muscular dystrophy (BMD), 10 fascioscapulohumeral muscular dystrophy (FSH), 4 distal myopathy (Dist) and 4 spinal muscular atrophy (SMA). All cases were in ages higher than 40 year-old.<br>High incidences of cataract and diabetes mellitus were observed in MyD. Malignant neoplasm occurred in three cases, but had no disease specificity. Vertebral osteoporosis and deformity were prominent in thoses cases of LG, BMD and FSH. LG and BMD also showed remarkable osteoporosis in both pelvic and ankle bones. In MyD, osteoporosis was observed more remarkably in ankle bone, whereas less remarkably in proxymal bones such as vertebrae and pelvis. In ECG study, the ischemic changes, myocardial damages and conduction blocks were observed in 23, 24 and 25% of all cases, respectively. The conduction block in MyD was the prominent incidence of 47%. Two cases had trace of cerebrovascular accident in the brain CT, and lacunar infarction was noted in 8 out of 56 cases. In MyD, the diffuse low density area in the white matter was seen in 24%, and moderate or severe brain atrophy in 76%. MyD also showed low IQ which had no significant correlations with age, duration of illness and the degree of brain atrophy.<br>In these cases with progressive muscular wasting, the complications are classified as the secondary changes of muscular wasting such as osteoporosis, smooth muscle disorder, cardiac muscle involvement which may be followed by cerebral embolism, and common symptoms with aging. MyD had complicated clinical features which was modified by the progelia and endocrinological and metabolic disorders.

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