<症例>拡張型の心筋症を伴ったKugelberg-Welander病の1症例

概要

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A 45-year-old female with Kugelberg-Welander (K-W) disease associated with dilated type cardiomyopathy is reported. She had difficulty in walking from the age of about 35. On physical examination, she exhibited systolic murmur and cardiomegaly. The neurological examination demonstrated muscle weakness, atrophy of the trunk and of the proximal portion of the extremities. On laboratory examination, the serum creatinine kinase level was found to be elevated. Electromyography showed neuropathic changes. Muscle biopsy specimen showed neuropathic and slightly myopathic changes. Electrocardiography showed no abnormalities of the conduction system. Left ventriculography revealed dilatation of ventricular cavity. Endomyocardial biopsy specimen showed degeneration of myocardial cells and proliferation of interstitial fibers. Loss of myofibrils and increased number of mitochondria were observed in electron microscopic examination. She was diagnosed as having K-W disease associated with dilated type cardiomyopathy, Seventeen cases of K-W disease with cardiomypathy were reported in the literature to date. However. this is the first case of K-W disease with dilated cardiomypathy without abnormalities in the conduction system. We found that electron microscopic examination of biopsied endomyocardium was necessary to reveal the occult myocardio pathy in the K-W disease.
近畿大学の論文
1991-12-25