The National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK.
Behav Neurol. 1992;5(4):229-32. doi: 10.3233/BEN-1992-5405.
The clinical features of 67 patients with probable Steele-Richardson-Olszewski syndrome have been analysed. Bradykinesia was present in 92.5% of cases at the time of initial assessment and the two commonest presenting complaints were walking difficulties and stiffness. Only 11 patients had a supranuclear gaze palsy when first seen. Forty-nine patients developed cognitive impairment in the course of their illness based on neuropsychological testing. In addition to the classical clinical findings occasional other findings included focal dystonia, tonic inhibition of levator palpebrae and blepharospasm, respiratory disturbances and impaired hearing. Only nine cases responded at all to levodopa therapy.
分析了 67 例可能的 Steele-Richardson-Olszewski 综合征患者的临床特征。在初次评估时，92.5%的病例存在运动迟缓，最常见的两个首发症状是行走困难和僵硬。只有 11 名患者在初次就诊时出现核上性眼球运动麻痹。根据神经心理学测试，49 名患者在病程中出现认知障碍。除了经典的临床发现，偶尔还会发现其他一些表现，包括局灶性肌张力障碍、提上睑肌紧张性抑制和眼睑痉挛、呼吸障碍和听力受损。只有 9 例对左旋多巴治疗有反应。
