Rinne J O, Lee M S, Thompson P D, Marsden C D
University Department of Clinical Neurology, Institute of Neurology, London, UK.
Brain. 1994 Oct;117 ( Pt 5):1183-96. doi: 10.1093/brain/117.5.1183.
The presenting features and their subsequent evolution in 36 patients with pathologically proven or clinically probable corticobasal degeneration are described. The most common initial complaint was of a unilateral 'clumsy, stiff or jerky arm' (n = 20). Typically the arm was akinetic, rigid and apraxic. In about a third of these the arm was held in a striking and characteristic fixed dystonic posture. Jerking of the arm, due to action and stimulus-sensitive myoclonus accompanied these symptoms in about a third of the cases. The next most common presentation (n = 10) was difficulty walking due to clumsiness and loss of fine motor control of one leg due to apraxia or dysequilibrium, or a combination of both. Sensory symptoms in the affected arm heralded the onset of illness in three and accompanied a motor disturbance in two cases. Presentation with dysarthria or a behavioural syndrome were rare. The symptoms progressed slowly, usually involving first the ipsilateral arm and leg, but gradually spread to affect all four limbs. After a mean follow-up of 5.2 years (range 2-8 years) gait difficulties and a supranuclear ophthalmoplegia had emerged in most patients and dysarthria and pyramidal signs were common. Higher mental function was relatively preserved in most and a cortical sensory loss was evident in a quarter of cases.
本文描述了36例经病理证实或临床疑似皮质基底节变性患者的临床表现及其后续演变情况。最常见的初始症状是单侧“手臂笨拙、僵硬或抽搐”（n = 20）。典型表现为手臂运动不能、僵硬和失用。其中约三分之一的患者手臂呈现出显著且特征性的固定肌张力障碍姿势。约三分之一的病例中，手臂的抽搐是由动作和刺激敏感的肌阵挛引起的，这些症状同时存在。其次最常见的表现（n = 10）是由于笨拙以及一条腿因失用或平衡失调，或两者兼而有之导致的精细运动控制丧失而出现行走困难。患侧手臂的感觉症状在3例中预示疾病发作，2例伴有运动障碍。构音障碍或行为综合征的表现较为罕见。症状进展缓慢，通常首先累及同侧手臂和腿部，但逐渐蔓延至影响四肢。平均随访5.2年（范围2 - 8年）后，大多数患者出现步态困难和核上性眼肌麻痹，构音障碍和锥体束征也很常见。大多数患者的高级心理功能相对保留，四分之一的病例存在皮质感觉丧失。
