Palmowski A, Jost W H, Osterhage J, Prudlo J, Käsmann B, Schimrigk K, Ruprecht K W
Augenklinik und Poliklinik, Universität des Saarlandes Homburg.
Klin Monbl Augenheilkd. 1995 Mar;206(3):170-2. doi: 10.1055/s-2008-1035424.
Amyotrophic lateral sclerosis (ALS), a neurodegenerative disorder of unknown origin, was thought to spare the extraocular muscles. Extraocular involvement has recently been reported to occur in the late stages of ALS following respiratory insufficiency.
We report on two patients with ALS who were referred for screening of oculomotor impairment in ALS. Orthoptic examination in a 64-year-old woman with peripheral ALS revealed retraction of the upper eye lids as well as impaired abduction and upgaze in both eyes, developing prior to respiratory insufficiency. A 50-year-old man with bulbar ALS was found to have bilateral impairment of upgaze as well as a negative Bell's phenomenon. Horizontal pursuit was interrupted by compensatory saccades, vertical fixating saccades were slightly hypometric. These oculomotor changes were also seen prior to respiratory insufficiency.
These findings provide further evidence of early oculomotor involvement in ALS, e.g. prior to respiratory failure and prior than previously suspected. Oculomotor impairment may occur in both the peripheral and the bulbar type of ALS.
肌萎缩侧索硬化症（ALS）是一种病因不明的神经退行性疾病，以往认为不累及眼外肌。最近有报道称，眼外肌受累发生在ALS呼吸功能不全的晚期。
我们报告了两名因ALS动眼神经功能障碍筛查而转诊的患者。对一名64岁患有周围性ALS的女性进行的眼位检查显示，上眼睑退缩，双眼外展和上视功能受损，这些症状在呼吸功能不全之前就已出现。一名50岁患有延髓性ALS的男性被发现有双侧上视功能障碍以及贝尔现象阴性。水平跟踪被代偿性扫视打断，垂直注视性扫视略小于正常。这些动眼神经变化在呼吸功能不全之前也已出现。
这些发现进一步证明了ALS早期动眼神经受累，例如在呼吸衰竭之前以及比之前怀疑的更早阶段。动眼神经功能障碍可能发生在周围性和延髓性ALS中。
