Klockgether Thomas
Department of Neurology, University Hospital Bonn, and German Center for Neurodegenerative Diseases, Bonn, Germany.
Handb Clin Neurol. 2018;155:217-225. doi: 10.1016/B978-0-444-64189-2.00014-7.
Sporadic adult-onset ataxia (SAOA) is a nongenetic neurodegenerative disorder of the cerebellum of unknown cause which manifests with progressive ataxia. It is distinguished from hereditary ataxias and from acquired ataxias. SAOA also needs to be differentiated from multiple system atrophy of cerebellar type (MSA-C). Thus, the diagnosis of SAOA can only be made by exclusion. Although cerebellar ataxia is the prominent symptom in SAOA, patients often have additional nonataxia signs, including pyramidal tracts signs, decreased or absent ankle reflexes, sensory disturbances, mainly in the form of reduced vibration sense, and mild urinary symptoms that do not the fulfill the criteria for severe autonomic failure required for the diagnosis of MSA-C. Disease progression in SAOA is considerably slower than in MSA-C. Brain imaging typically shows isolated cerebellar atrophy. Nerve conduction studies provide evidence for polyneuropathy in about one-third of SAOA patients. As the etiology and pathogenesis of SAOA are unknown, there is no specific treatment approach to this condition.
散发性成人起病型共济失调（SAOA）是一种病因不明的非遗传性小脑神经退行性疾病，表现为进行性共济失调。它与遗传性共济失调和获得性共济失调不同。SAOA还需要与小脑型多系统萎缩（MSA-C）相鉴别。因此，SAOA的诊断只能通过排除法做出。虽然小脑共济失调是SAOA的突出症状，但患者通常还有其他非共济失调体征，包括锥体束征、踝反射减弱或消失、感觉障碍，主要表现为振动觉减退，以及轻度泌尿系统症状，但不符合MSA-C诊断所需的严重自主神经功能衰竭标准。SAOA的疾病进展比MSA-C慢得多。脑部影像学检查通常显示孤立性小脑萎缩。神经传导研究为约三分之一的SAOA患者存在多发性神经病提供了证据。由于SAOA的病因和发病机制尚不清楚，针对这种疾病没有特定的治疗方法。
