著明な寛解を示した非定型的筋ジストロフィーの1例—非定型的な原発性筋疾患考察における一般的問題について

AN ATYPICAL MUSCULAR DYSTROPHY WITH REMISSIONS

I．緒言

　主な筋疾患の疾患概念は一旦ほぼ確立されたかに思われたが，再検討をまつ点も少なくないことが示されつつあり，非定型的症例の集積はこの点からも重視されている。本論文の目的は分類困難な筋疾患の1例の報告と，その症例がいかなる疾患群に属するかを考察しつつ，非定型的な原発性筋疾患の疾病論的検討にあたつて生じる諸問題を一般的に論ずることである。

A case of myopathy which showed marked remis-sions in its course and a typical pathology of muscular dystrophy is described.

The eighteen years old male patient belonging to a family with finger anomalies noticed at the age of sixteen that his both legs were very fatigable. His legs became more and more weak until he was unable to go upstairs and to stand up from a chair unless helped by the upper limbs. His bilateral calves were enlarged tightly and tender. These difficulties became slight several months later and he was able to go upstairs and to stand up without help by the upper limbs. Additional episodes similar to one described above had occurred two times within one and a half year afterwards.

Muscle atrophy was seen at pelvic girdle, thigh and calf muscles symmetrically. Muscles of the bilateral upper arms were slightly atrophic, but no winging scapulae were observed. The patient climbed up with his knees and the weakness of the pelvic girdle gave rise to a characteristic waddling gait. No pseudohypertrophy, muscle twitchings, muscle tenderness, joint contracture were observed. The patient was otherwise entirely normal.

Electromyography was compatible with myopathic changes, serum creatine-phosphokinase activity was elevated more than 836 U. The muscle pathology was entirely identical to muscular dystrophy as seen in Fig. 2, where A-C and D-E show left m. rectus femoris and left m. gastrocnemius respectively. Not a single evidence of inflammation was revealed.

We have considered the possibility that this case might be muscular dystrophy rather than polymyositis. However final diagnosis was avoided since patients with muscular dystrophy never show such remissions as this case.