ミオパチー罹患筋の微細構造に関する研究

Observations on the Fine Structure of the Human Skeletal Muscle in Myopathy

I．緒言

　最近，筋ジストロフィー症を含む各種ミオパチーについての関心が高まり，生化学的あるいは生理学的，形態学的な面からの新しい知見が報告されているが，いまだその発生病理などについては不明な点が多い。

　形態学的研究として，従来の光学顕微鏡による研究に加えて，近年の電子顕微鏡学の進歩により，細胞としての筋の微細構造と機能が明らかになり^1），ミオパチーにおける筋の変性過程が光学顕微鏡のレベルから電顕による微細構造まで，広角度から検討が加えられるようになつた。

Muscle biopsies from 16 patients with myopa-thy were observed with electron microscope.

Various stages of degeneration of muscle fiber were observed as follows: 1) Slight changes; abnormalities of Z disc in a certain sarcomere, e. g. thickening of Z disc material or irregularity of Z disc.

2) Mcderate changes; the enlargement of the interfibrillar spaces, and disarrangement of the myofilaments, frequently originating at the I-band.

3) Severe changes; sarcoplasmic pads contain-ing various cytoplasmic organelles with amor-phous material derived from degenerated myofil-aments and appearance of the structures which might be considered as lysosomes.

Following the advanced changes, both mito-chondria and sarcoplasmic reticulum were swol-len.

No abnormalities of nuclei or sarcolemma were noted except that nuclei occasionally appeared in the center of the fiber, which showed pyknotic degeneration.

Alterations of Z disc and myofilament were discussed.