筋緊張性ジストロフィー症の１剖検例　舌筋の病態を中心に

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タイトル別名
  • An Autopsy Case of Myotonic Dystrophy. With Special Reference to Tongue Muscles.
  • With Special Reference to Tongue Muscles
  • 舌筋の病態を中心に

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説明

Myotonic dystrophy is a hereditary multisystem disease characterized by myotonia and muscle atrophy. Histopathological study of an autopsy case, especially lesions of tongue muscles, is presented. The present case was a 50-year-old man with a sister similarly disordered. He was admitted with complaints of paropsis and myopathy, accompanied with obesity, frontal alopecia, diabetes, and conscious disturbance. Electromyogram revealed myotonic discharges and confirmed the diagnosis of myotonic dystrophy by muscle biopsy. He died of pneumonia based on advanced myopathy 2 years after the first examination. An autopsied tongue macroscopically showed atrophy in the anterior region with yellowish cut surfaces. Histological examination revealed severe atrophy of tongue muscles with nuclear and myofibrillar changes and replacement by fat cells. Morphometric analysis demonstrated a little heavier myopathy in the posterior region than in the anterior and middle regions of the tongue. The other skeletal, smooth and cardiac muscles showed various stages of myopathy.

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