Ocular MyopathyにMyopathia distalis tardaの合併した2症例

TWO CASES OF OCULAR MYOPATHY ASSOCIATED WITH MYOPATHIA DISTALIS TARDA

I．はじめに

　進行性全外眼筋麻痺は長く神経核性と考えられてきたが，KilohおよびNevin^1）の病理学的観察以来筋原性と考えられるにいたつている。その後McCauley^2），　Nico—laissenおよびBrodal^3）はbiopsyで，　BeckettおよびNetsky^4），　SchwarzおよびLiu^5）らは剖検例で同様の事実を確めている。TeasdallおよびSears^6），　Ess—len^7）らはさらに筋電図により検討を加え，これがocu—lar myopathyであることを確認している。

　ocular myopathyは従来きわめてまれなものとされ，その報告も少ないが，筋障害は眼筋のみにとどまらず，M．masseter （Bernhardt^8），　Kiloh　＆　Nevin^1）），や咽頭，喉頭筋（Bernhardt^8），　Thiel^9），　Walsh^10），　Teas—dal ＆ Sears^6）），顔面筋（Kiloh ＆ Nevin^1），　Teasdalら^6））ときには上肢または下肢の筋障害（Gowers^11），　Deso—gus^12），　Pelnár^13），　Poe^14）．　Martin^15），　Elliot^16），　Thiébautら^17），Gartuer ＆ Billet^18），　Kiloh　＆　Nevin^1），　Elliot，Hughes ＆ Turner^19），　Lees ＆ Liversedge^20））のくることが報告されている。

Two cases of ocular myopathy associated with late distal myopathy were reported. Case 1 was 52 years male has had slowly progres-sive ophthalmoplegia since age of 40 followed by weakness and atrophy of hands, forearms, difficulities of speech and swallowing and weakness of both lower extremities over 10 years period. Case 2 was 71 years male has had weakness and atrophy of left hand and leg followed by progressive ophthalmoplegia, difficulities of speech and swallowing, and of distal muscles of extremities.

Both cases showed total ophthalmoplegia, bilateral facial and palatal muscle weakness and atrophy of distal one third of upper and lower limbs. Myogenic atrophy was proved by electromyography and pathological studies on biopsied specimens.

Unequivocal hereditary occurrence of ocular myopathy associated with myopathia distalis tarda was obtained.

Presence of this unusual association of ocular myopathy and late distal myopathy was discussed from the literature.