van Gaalen Judith, van de Warrenburg Bart P C
Department of Neurology & Donders Institute for Brain, Cognition and Behaviour, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
Pract Neurol. 2012 Feb;12(1):14-24. doi: 10.1136/practneurol-2011-000108.
The clinical management of cerebellar ataxia is challenging, mainly because ataxia is a symptom of many neurological diseases. Many types of ataxia disorders are genetic and some are extremely rare. Here, the authors suggest a diagnostic approach to ataxia developed around a case of sporadic, late-onset, slowly progressive ataxia. Clinical information such as age of onset, rate of progression, family history and certain non-cerebellar features can narrow the differential diagnosis. Brain MRI is almost obligatory and may reveal valuable diagnostic clues. Having ruled out structural lesions, the two other most common diagnoses are inflammatory and degenerative (including genetic) disorders. Although only a minority of underlying diseases are treatable, there are still many options for supportive care.
小脑共济失调的临床管理具有挑战性，主要是因为共济失调是许多神经系统疾病的一种症状。许多类型的共济失调症是遗传性的，有些极为罕见。在此，作者围绕一例散发性、迟发性、缓慢进展性共济失调病例，提出了一种共济失调的诊断方法。诸如发病年龄、进展速度、家族史和某些非小脑特征等临床信息，可缩小鉴别诊断范围。脑部磁共振成像（MRI）几乎是必不可少的，可能会揭示有价值的诊断线索。排除结构性病变后，另外两种最常见的诊断是炎症性和退行性（包括遗传性）疾病。尽管只有少数潜在疾病是可治疗的，但支持性护理仍有很多选择。
