伴有神经病变和前庭反射消失综合征的小脑性共济失调（CANVAS）中的汗腺去神经支配
Sweat Gland Denervation in Cerebellar Ataxia with Neuropathy and Vestibular Areflexia Syndrome (CANVAS).
作者信息
Umeh Chizoba C, Polydefkis Michael, Chaudhry Vinay, Zee David S
机构信息
Department of Neurology Brigham and Women's Hospital and Harvard Medical School Boston Massachusetts USA.
Department of Neurology The Johns Hopkins Hospital Baltimore Maryland USA.
出版信息
Mov Disord Clin Pract. 2016 Apr 5;4(1):46-48. doi: 10.1002/mdc3.12355. eCollection 2017 Jan-Feb.
Abstract
Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) is a newly recognized disorder characterized by cerebellar ataxia, nonlength-dependent sensory impairment, and bilateral vestibular loss. Sudomotor dysfunction has been described in CANVAS; however, the underlying pathology is not well characterized. To describe novel histopathological features of this syndrome, 2 siblings are presented who had CANVAS with unique findings of sweat gland denervation. Skin biopsy testing was performed to assess sudomotor structure and revealed markedly reduced sweat gland nerve fiber density below the 2.5th percentile in both patients. These histopathological findings suggest that postganglionic sudomotor dysfunction is an additional feature of CANVAS.
摘要
小脑性共济失调伴神经病和前庭反射消失综合征（CANVAS）是一种新发现的疾病，其特征为小脑性共济失调、非长度依赖性感觉障碍和双侧前庭功能丧失。CANVAS中曾有过汗腺功能障碍的描述；然而，其潜在病理特征尚未明确。为描述该综合征新的组织病理学特征，本文介绍了2例患有CANVAS且有汗腺去神经支配独特表现的同胞患者。进行了皮肤活检检测以评估汗腺运动结构，结果显示两名患者的汗腺神经纤维密度均显著降低，低于第2.5百分位数。这些组织病理学发现表明，节后汗腺运动功能障碍是CANVAS的另一个特征。
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