Armstrong Richard A
Vision Sciences, Aston University, Birmingham, UK.
Clin Exp Optom. 2016 Nov;99(6):498-506. doi: 10.1111/cxo.12429. Epub 2016 Aug 23.
Corticobasal degeneration is a rare, progressive neurodegenerative disease and a member of the 'parkinsonian' group of disorders, which also includes Parkinson's disease, progressive supranuclear palsy, dementia with Lewy bodies and multiple system atrophy. The most common initial symptom is limb clumsiness, usually affecting one side of the body, with or without accompanying rigidity or tremor. Subsequently, the disease affects gait and there is a slow progression to influence ipsilateral arms and legs. Apraxia and dementia are the most common cortical signs. Corticobasal degeneration can be difficult to distinguish from other parkinsonian syndromes but if ocular signs and symptoms are present, they may aid clinical diagnosis. Typical ocular features include increased latency of saccadic eye movements ipsilateral to the side exhibiting apraxia, impaired smooth pursuit movements and visuo-spatial dysfunction, especially involving spatial rather than object-based tasks. Less typical features include reduction in saccadic velocity, vertical gaze palsy, visual hallucinations, sleep disturbance and an impaired electroretinogram. Aspects of primary vision such as visual acuity and colour vision are usually unaffected. Management of the condition to deal with problems of walking, movement, daily tasks and speech problems is an important aspect of the disease.
皮质基底节变性是一种罕见的进行性神经退行性疾病,属于“帕金森综合征”疾病组,该组还包括帕金森病、进行性核上性麻痹、路易体痴呆和多系统萎缩。最常见的初始症状是肢体笨拙,通常影响身体的一侧,伴有或不伴有僵硬或震颤。随后,疾病影响步态,并缓慢进展至影响同侧的手臂和腿部。失用症和痴呆是最常见的皮质体征。皮质基底节变性可能难以与其他帕金森综合征相区分,但如果存在眼部体征和症状,则可能有助于临床诊断。典型的眼部特征包括与表现失用症一侧同侧的扫视性眼球运动潜伏期延长、平稳跟踪运动受损以及视觉空间功能障碍,尤其是涉及空间而非基于物体的任务。不太典型的特征包括扫视速度降低、垂直凝视麻痹、视幻觉、睡眠障碍和视网膜电图受损。诸如视力和色觉等主要视觉方面通常不受影响。针对行走、运动、日常任务和言语问题处理该疾病的管理是该疾病的一个重要方面。