Yamazaki K, Katayama S, Ishihara T, Hirata K
Department of Neurology, Dokkyo University School of Medicine.
Rinsho Shinkeigaku. 1994 May;34(5):489-92.
We reported a case of Fisher's syndrome with upbeat nystagmus. The syndrome manifested itself with the appearance of diplopia and ataxic gait preceded by cold-like symptoms. Although slight blephaloptosis, marked bilateral upward gaze palsy, and restricted abduction of the right eye were observed, light reflexes and Bell's phenomenon were normal. A slight decrease in the muscular power of the distal extremities, bilateral fingertip dysesthesia, and diminished sensation of vibration were observed. All muscle stretch reflexes were absent, and no pathologic reflexes were noted. Truncal ataxia and incoordination were observed. The cerebrospinal fluid showed albumino-cytologic dissociation. Upbeat nystagmus in five directions of gaze was recorded on the EOG, the smooth pursuit eye movement showed a staircase pattern, and dysmetria was present in the saccadic eye movement. The caloric test elicited a satisfactory response, but the visual suppression test produced poor suppression. Taken altogether, the test results suggest that the responsible lesions in this case were not only peripheral but also central, extending from the midbrain to the tegmentum pontis and the vestibulocerebellum.
我们报告了一例伴有向上性眼球震颤的费希尔综合征病例。该综合征表现为在出现类似感冒症状后出现复视和共济失调步态。尽管观察到轻度上睑下垂、明显的双侧向上凝视麻痹以及右眼外展受限,但光反射和贝尔现象正常。观察到远端肢体肌肉力量略有下降、双侧指尖感觉异常以及振动觉减退。所有肌肉牵张反射均消失,未发现病理反射。观察到躯干共济失调和不协调。脑脊液显示蛋白细胞分离。眼电图记录了五个注视方向的向上性眼球震颤,平稳跟踪眼球运动呈阶梯状模式,扫视眼球运动存在辨距不良。冷热试验引出满意反应,但视觉抑制试验抑制效果不佳。综合来看,测试结果表明该病例的责任病变不仅累及外周,还累及中枢,从中脑延伸至脑桥被盖和前庭小脑。