Cheshire William P, Freeman Roy, Gibbons Christopher H, Cortelli Pietro, Wenning Gregor K, Hilz Max J, Spies Judith M, Lipp Axel, Sandroni Paola, Wada Naoki, Mano Akiko, Ah Kim Hyun, Kimpinski Kurt, Iodice Valeria, Idiáquez Juan, Thaisetthawatkul Pariwat, Coon Elizabeth A, Low Phillip A, Singer Wolfgang
Department of Neurology, Mayo Clinic, 4500 San Pablo Rd., Jacksonville, Florida 32224, USA.
Department of Neurology, Harvard Medical School, 330 Brookline Avenue, Boston, Massachusetts 02215-5400, USA.
Clin Neurophysiol. 2021 Feb;132(2):666-682. doi: 10.1016/j.clinph.2020.11.024. Epub 2020 Dec 22.
Evaluation of disorders of the autonomic nervous system is both an art and a science, calling upon the physician's most astute clinical skills as well as knowledge of autonomic neurology and physiology. Over the last three decades, the development of noninvasive clinical tests that assess the function of autonomic nerves, the validation and standardization of these tests, and the growth of a large body of literature characterizing test results in patients with autonomic disorders have equipped clinical practice further with a valuable set of objective tools to assist diagnosis and prognosis. This review, based on current evidence, outlines an international expert consensus set of recommendations to guide clinical electrodiagnostic autonomic testing. Grading and localization of autonomic deficits incorporates scores from sympathetic cardiovascular adrenergic, parasympathetic cardiovagal, and sudomotor testing, as no single test alone is sufficient to diagnose the degree or distribution of autonomic failure. The composite autonomic severity score (CASS) is a useful score of autonomic failure that is normalized for age and gender. Valid indications for autonomic testing include generalized autonomic failure, regional or selective system syndromes of autonomic impairment, peripheral autonomic neuropathy and ganglionopathy, small fiber neuropathy, orthostatic hypotension, orthostatic intolerance, syncope, neurodegenerative disorders, autonomic hyperactivity, and anhidrosis.
自主神经系统疾病的评估既是一门艺术，也是一门科学，需要医生具备最敏锐的临床技能以及自主神经学和生理学知识。在过去三十年中，评估自主神经功能的无创临床检测方法的发展、这些检测方法的验证和标准化，以及大量描述自主神经疾病患者检测结果的文献的增长，进一步为临床实践提供了一套有价值的客观工具，以辅助诊断和预后判断。本综述基于当前证据，概述了一套国际专家共识推荐意见，以指导临床自主神经电诊断检测。自主神经功能缺陷的分级和定位纳入了交感心血管肾上腺素能、副交感心血管迷走神经和汗腺运动检测的分数，因为没有单一检测足以诊断自主神经功能衰竭的程度或分布。综合自主神经严重程度评分（CASS）是一种针对年龄和性别进行标准化的有用的自主神经功能衰竭评分。自主神经检测的有效指征包括全身性自主神经功能衰竭、自主神经损伤的区域性或选择性系统综合征、周围性自主神经病变和神经节病变、小纤维神经病变、直立性低血压、直立不耐受、晕厥、神经退行性疾病、自主神经功能亢进和无汗症。
