Pure autonomic failure (PAF) is a progressive syndrome of neurogenic orthostatic hypotension, widespread anhidrosis, urinary retention, and constipation without other neurologic manifestations. It is generally considered a peripheral ganglionic synucleinopathy. Natural history studies have described risk factors for the conversion of PAF to Parkinson's disease, multiple system atrophy, or dementia with Lewy bodies, yet the early stages of PAF are not well characterized. We present a patient with unilateral anhidrosis, contralateral facial flushing and hyperhidrosis consistent with Harlequin syndrome that, over 6 years, progressed to PAF, suggesting that PAF may present with focal autonomic impairment prior to generalized autonomic failure.

中文翻译：

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表现为丑角综合征的纯自主神经功能衰竭

纯自主神经功能衰竭 (PAF) 是一种神经源性直立性低血压、广泛无汗、尿潴留和便秘的进行性综合征，没有其他神经系统表现。它通常被认为是外周神经节突触核蛋白病。自然史研究已经描述了 PAF 转化为帕金森病、多系统萎缩或路易体痴呆的风险因素，但 PAF 的早期阶段并未得到很好的表征。我们介绍了一名患有单侧无汗、对侧面部潮红和多汗的患者，与 Harlequin 综合征一致，6 年后进展为 PAF，表明 PAF 可能在全身性自主神经功能衰竭之前出现局灶性自主神经损伤。