Takaya Y, Waragai M, Ogawara K, Hayashi M
Department of Neurology, Kofu City Hospital.
Rinsho Shinkeigaku. 1996 Aug;36(8):996-9.
A 66-year-old woman with a 10 years' history of autosomal dominant hereditary spinocerebellar degeneration developed an atypical nystagmus. Neurological examination revealed cerebellar ataxia, absent tendon reflex, supranuclear upward gaze palsy, saccadic pursuit and nystagmus. Nystagmus was a dissociated type and appeared only in lateral gaze and the amplitude was prominent in adducting eyes. Brain MRI showed cerebellar hemispheric and vermal atrophy, and pontine atrophy. Neuro-otological examination revealed normal caloric test, but impaired optokinetic nystagmus and visual suppression test. The dissociated adduction nystagmus of this case may correspond to gaze evoked nystagmus and latent hypofunction of the abducens motor neurons.
一名患有常染色体显性遗传性脊髓小脑变性10年的66岁女性出现了非典型眼球震颤。神经系统检查发现小脑共济失调、腱反射消失、核上性向上凝视麻痹、眼球跳动性追踪和眼球震颤。眼球震颤为分离型，仅在侧方凝视时出现，内收眼的振幅明显。脑部MRI显示小脑半球和蚓部萎缩以及脑桥萎缩。神经耳科学检查显示冷热试验正常，但视动性眼球震颤和视觉抑制试验受损。该病例的分离性内收眼球震颤可能与凝视诱发性眼球震颤和展神经运动神经元潜在功能减退相对应。
