Torticollis due to disinhibition of the vestibulo-collic reflex in a patient with Steele-Richardson-Olszewski syndrome
Torticollis due to disinhibition of the vestibulo-collic reflex in a patient with Steele-Richardson-Olszewski syndrome
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DOI：
10.1002/mds.870120311
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发表时间：
1997-05-01
影响因子：
8.6
通讯作者：
Lees, AJ
中科院分区：
文献类型：
--
作者：
Bisdorff, AR;Bronstein, AM;Lees, AJ
据报道，一名患者具有 Steele-Richardson-Olszewski 综合征的临床表现，并伴有不寻常的间歇性颈部扭转。他几乎没有随意的眼球运动，只有非常缓慢、低幅度的随意头部运动。然而，为了响应视动或前庭刺激，他在预期眼球震颤的慢相方向上发生了完全的眼睛偏差，没有观察到快相，并且由于扫视缺陷，偏差超过了前庭刺激的持续时间。在这些刺激期间，头部也完全转动，比尝试随意运动时更快，并且与眼睛类似地保持偏离。这表明该患者的颈部偏差是由于前庭结肠反射不受抑制和头部位置重置机制紊乱所致。对全身旋转的颈部肌电图反应表明，导致该患者斜颈的前庭结肠反射有大约 30 毫秒的短潜伏期。
A patient with the clinical picture of Steele-Richardson-Olszewski syndrome and an unusual intermittent neck twisting is reported. He had virtually no voluntary ocular movements and only very slow, low amplitude voluntary head movements. However, in response to optokinetic or vestibular stimulation, he developed full eye deviations in the direction of the slow phase of the expected nystagmus, No quick phases were observed, and the deviation outlasted the duration of the vestibular stimuli because of defective saccades. The head also turned fully during these stimuli, quicker than on attempted voluntary movements, and remained deviated similarly to the eyes. This suggests that the neck deviations in this patient were due to a disinhibited vestibulo-collic reflex and a disturbed head position resetting mechanism. Neck electromyographic responses in response to whole-body rotation indicated that the vestibulocollic reflex responsible for the torticollis in this patient had a short latency of similar to 30 ms.