Oculomotor function in multiple system atrophy: Clinical and laboratory features in 30 patients

Oculomotor function in multiple system atrophy: Clinical and laboratory features in 30 patients
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DOI:
10.1002/mds.21999
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发表时间:
2008-05-15
期刊:
影响因子:
8.6
通讯作者:
Bronstein, Adolfo
Bronstein, Adolfo
中科院分区:
医学1区
文献类型:
--
作者:
Anderson, Tim;Luxon, Linda;Bronstein, Adolfo

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我们回顾了30例可能多发性系统萎缩(MSA)患者的临床和实验室眼肌运动特征,其中22例为MSA- p, 8例为MSA- c。6例患者也进行了尸检,其中4例确诊MSA, 2例排除MSA(帕金森病和进行性核上性麻痹)。临床检查显示如下异常:过度的方波抽搐——30例中有21例;轻度垂直核上凝视性麻痹-8 / 30;注视诱发性眼球震颤——30例患者中12例,其中3例无小脑功能障碍的眼外证据;定位下拍性眼球震颤-10 / 25;轻度或中度跳囊性低度数-22 / 30;失败(“分手”)的顺利追求——30人中有28人;降低VOR抑制-16 / 24。眼电成像和热量测试没有增加显著的额外信息。在表现为动刚性综合征的患者中,很难区分特发性帕金森病与MSA-P和其他非典型帕金森素引起的疾病。我们的研究结果表明,过度的方波抽搐、轻度至中度的视跳低、VOR抑制受损、自发性眼球震颤或定位性下搏动眼球震颤可能是动眼病的“危险信号”或MSA存在的线索。此外,临床慢速扫视,或中度至重度凝视限制的存在,提示非MSA的诊断。(c) 2008运动障碍学会。
We reviewed the clinical and laboratory oculomotor features in 30 patients with probable multiple system atrophy (MSA), 22 with MSA-P and 8 with MSA-C. Six patients were also examined post mortem, MSA being confirmed in four and excluded in two (Parkinson's disease and progressive supranuclear palsy). Clinical examination showed the following abnormalities; excessive square wave jerks-21 of 30 patients; mild vertical supranuclear gaze palsy-8 of 30; gaze-evoked nystagmus-12 of 30 patients, three of whom had no extraocular evidence of cerebellar dysfunction; positioning downbeat nystagmus-10 of 25; mild or moderate saccadic hypometria-22 of 30; impaired ("broken up") smooth pursuit-28 of 30; reduced VOR suppression-16 of 24. Electro-oculography and caloric testing did not add significant extra information. In patients presenting with an akinetic-rigid syndrome it can be difficult to differentiate idiopathic Parkinson's disease from MSA-P and other causes of atypical parkinsonisin. Our findings suggest that the presence of excessive square wave jerks, mild - moderate hypometria of saccades, impaired VOR suppression, spontaneous nystagmus or positioning downbeat nystagmus may be oculomotor "red flags" or clues to the presence of MSA. Further, the presence of clinically slow saccades, or moderate-to-severe gaze restriction, suggests a diagnosis other than MSA. (c) 2008 Movement Disorder Society.