Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, UCL, Queen Square, London, United Kingdom.
Mov Disord. 2010 Jun 15;25(8):1061-7. doi: 10.1002/mds.23047.
Fixed dystonia is an uncommon but severely disabling condition typically affecting young women following a minor peripheral injury. There is no evidence of any structural lesions of the central nervous system nor any clear peripheral nerve or root damage. Electrophysiological techniques such as short intracortical inhibition, cortical silent period and a plasticity inducing protocol have revealed similarities but also differences compared to classical mobile dystonia. To further explore the pathophysiology of fixed dystonia we compared mental rotation of body parts and sensory temporal discrimination in 11 patients with fixed dystonia, 11 patients with classical mobile dystonia and 10 healthy controls. In the mental rotation task subjects were presented with realistic photos of left or right hands, feet and the head of a young women with a black patch covering the left or the right eye in six different orientations. Subjects had to verbally report the laterality of the presented stimuli. To assess sensory temporal discrimination subjects were asked to discriminate whether pairs of visual, tactile (electrical), or visuo-tactile stimuli were simultaneous or sequential (temporal discrimination threshold) and in the latter case which stimulus preceded the other (temporal order judgement). In accordance with previous studies patients with mobile dystonia were abnormal in mental rotation and temporal discrimination, whereas patients with fixed dystonia were only impaired in mental rotation. Possible explanations for this deficit may include the influence of the abnormal body posture itself, a shared predisposing pathophysiology for mobile and fixed dystonia, or a body image disturbance. These findings add information to the developing pathophysiological picture of fixed dystonia.
固定性肌张力障碍是一种罕见但严重致残的疾病，通常发生在年轻女性轻微外周损伤后。没有中枢神经系统任何结构损伤或任何明确的周围神经或神经根损伤的证据。电生理技术，如短程皮质内抑制、皮质静息期和诱导可塑性的方案，与经典的运动性肌张力障碍相比，揭示了相似性，但也有差异。为了进一步探讨固定性肌张力障碍的病理生理学，我们比较了 11 例固定性肌张力障碍患者、11 例经典运动性肌张力障碍患者和 10 例健康对照组的身体部位心理旋转和感觉时间辨别。在心理旋转任务中，受试者会看到一个年轻女性的真实照片，她的左眼或右眼被黑色眼罩遮住，照片呈现六个不同的方位。受试者需要口头报告呈现刺激的侧别。为了评估感觉时间辨别，要求受试者辨别视觉、触觉（电刺激）或视触觉刺激对是否同时呈现（时间辨别阈值），在后一种情况下，哪个刺激先于另一个刺激（时间顺序判断）。与先前的研究一致，运动性肌张力障碍患者在心理旋转和时间辨别方面存在异常，而固定性肌张力障碍患者仅在心理旋转方面存在障碍。这种缺陷的可能解释包括异常身体姿势本身的影响、运动性和固定性肌张力障碍的共同潜在病理生理学，或身体形象障碍。这些发现为固定性肌张力障碍的发展病理生理学提供了信息。
