Van der Walt A, Buzzard K, Sung S, Spelman T, Kolbe S C, Marriott M, Butzkueven H, Evans A
Department of Neurology, Royal/Melbourne Hospital, Australia Melbourne Brain Centre, Department of Medicine at RMH, University of Melbourne, Australia/Centre for Neuroscience, Department of Anatomy and Neuroscience, University of Melbourne, Australia
Department of Neurology, Royal Melbourne Hospital, Australia.
Mult Scler. 2015 Dec;21(14):1847-55. doi: 10.1177/1352458515577690. Epub 2015 May 26.
The pathophysiology of multiple sclerosis (MS) tremor is uncertain with limited phenotypical studies available.
To investigate whether dystonia contributes to MS tremor and its severity.
MS patients (n = 54) with and without disabling uni- or bilateral upper limb tremor were recruited (39 limbs per group). We rated tremor severity, writing and Archimedes spiral drawing; cerebellar dysfunction (SARA score); the Global Dystonia Scale (GDS) for proximal and distal upper limbs, dystonic posturing, mirror movements, geste antagoniste, and writer's cramp.
Geste antagoniste, mirror dystonia, and dystonic posturing were more frequent and severe (p < 0.001) and dystonia scores were correlated with tremor severity in tremor compared to non-tremor patients. A 1-unit increase in distal dystonia predicted a 0.52-Bain unit (95% confidence interval (CI) 0.08-0.97), p = 0.022) increase in tremor severity and a 1-unit (95% CI 0.48-1.6, p = 0.001) increase in drawing scores. A 1-unit increase in proximal dystonia predicted 0.93-Bain unit increase (95% CI 0.45-1.41, p < 0.001) in tremor severity and 1.5-units (95% CI 0.62-2.41, p = 0.002) increase in the drawing score. Cerebellar function in the tremor limb and tremor severity was correlated (p < 0.001).
Upper limb dystonia is common in MS tremor suggesting that MS tremor pathophysiology involves cerebello-pallido-thalamo-cortical network dysfunction.
多发性硬化症(MS)震颤的病理生理学尚不确定,现有的表型研究有限。
研究肌张力障碍是否与MS震颤及其严重程度有关。
招募了有或无致残性单侧或双侧上肢震颤的MS患者(n = 54)(每组39条肢体)。我们对震颤严重程度、书写和阿基米德螺旋线绘图进行评分;评估小脑功能(SARA评分);使用全球肌张力障碍量表(GDS)评估上肢近端和远端、肌张力障碍姿势、镜像运动、对抗性手势和书写痉挛。
与无震颤患者相比,对抗性手势、镜像肌张力障碍和肌张力障碍姿势在震颤患者中更频繁、更严重(p < 0.001),且肌张力障碍评分与震颤严重程度相关。远端肌张力障碍增加1个单位预测震颤严重程度增加0.52个Bain单位(95%置信区间(CI)0.08 - 0.97,p = 0.022),绘图评分增加1个单位(95% CI 0.48 - 1.6,p = 0.001)。近端肌张力障碍增加1个单位预测震颤严重程度增加0.93个Bain单位(95% CI 0.45 - 1.41,p < 0.001),绘图评分增加1.5个单位(95% CI 0.62 - 2.41,p = 0.002)。震颤肢体的小脑功能与震颤严重程度相关(p < 0.001)。
上肢肌张力障碍在MS震颤中很常见,提示MS震颤的病理生理学涉及小脑 - 苍白球 - 丘脑 - 皮质网络功能障碍。