Abstract
ネマリンミオパチーは筋線維内に桿状体様構造物（ネマリン小体）をもつ稀な先天性ミオパチーであり，時に心筋症の合併が報告されている．今回我々は自然歴の終盤にて拡張型心筋症を併発したネマリンミオパチーの一剖検例を経験した．ミクロ所見では心筋線維は骨格筋線維と同様の全般的萎縮を認めた．電顕ではネマリン小体は骨格筋線維内に認めるのみであったが，心筋線維Z帯の変性が見られることや患者の第2子にも同様の心筋障害がみられることよりネマリンミオパチーに関連した心筋症と考えた．
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