左右非同期性ベル現象を認めた進行性核上性麻痺

Asynchronous Bell's Phenomenon in Progressive Supranuclear Palsy

　開眼失行を伴う進行性核上性麻痺（PSP）の60歳，男性例のベル現象異常について報告した。閉眼によるベル現象は左右同期して出現したが，開眼は左右が同期せず，開眼側の眼位が正中に復しても，閉眼側のベル現象は持続していた。開閉眼動作での眼輪筋，上瞼挙筋と眼球運動の協同運動が左右の連合を含めて障害されていることを示唆していた。L-droxydopaの投与により動作障害は改善したが，ベル現象を含めて眼症候は不変であった。

A 60-year-old man suffering from progressive supranuclear palsy showed asynchronous Bell's phenomenon and apraxia of eye-lid opening. He showed synchronous upward rotation of the eye-ball at closing eyes. Although apraxia of eye-lid opening was observed in both sides, eye opening of the right side took longer duration than that of the opposite side. When he tried to open his eyes, unilate-ral Bell's phenomenon was sustained in the side of involuntary eye closing for a several seconds. Thesefindings indicated disorders of the coordination of eye openineg-and-closing, which regulate the or-bicural muscle of eye, levator muscle of upper eyelid and external oocular muscles. And the mechanism of synchronous eye movement between the botth sides was also disturbed. The administration of L-droxydopa was effective to gait disturbance, but not to ocular movement disorder including impaired Bell's phenomenon. The pharmacological results suggested different neuron system between locomo-tion control and conjugation affected in this disease.