Neurology, Internal Medicine, Sakura Medical Center, Toho University, Sakura, Japan,
Department of Uro-Neurology, The National Hospital for Neurology and Neurosurgery, Queen Square, London, United Kingdom.
Eur Neurol. 2020;83(1):80-86. doi: 10.1159/000506983. Epub 2020 Apr 22.
We report the case of a 52-year-old Japanese man who, while he had no cerebellar ataxia or parkinsonism, was revealed to have silent cerebellar hypoperfusion/mild cerebellar atrophy and sacral autonomic disorder. His sacral autonomic disorder was urinary retention without marked prostate hyperplasia. Urodynamics-sphincter electromyography revealed detrusor hyperactivity with impaired contraction and neurogenic changes of the sphincter motor unit potentials. Although he did not have a motor disorder, these features suggested possible multiple system atrophy-cerebellar (MSA-C) form. The present case report suggests that neuroimaging helps in diagnosing "premotor" MSA-C form in situ.
我们报告了一例 52 岁的日本男性病例,该患者虽无小脑共济失调或帕金森病,但被发现存在安静性小脑灌注不足/轻度小脑萎缩和骶部自主神经障碍。其骶部自主神经障碍为尿潴留,无明显前列腺增生。尿动力学-括约肌肌电图显示逼尿肌活动过度,收缩功能受损,以及括约肌运动单位电位的神经源性改变。尽管该患者没有运动障碍,但这些特征提示可能为多系统萎缩-小脑型(MSA-C)。本病例报告提示神经影像学有助于诊断原位“前驱”MSA-C 型。