Department of Neurology, Penn State College of Medicine, Hershey, Pennsylvania, USA.
Department of Neurology, Dartmouth-Hitchcok Medical Center, Lebanon, New Hampshire, USA.
Muscle Nerve. 2021 Jan;63(1):10-21. doi: 10.1002/mus.27048. Epub 2020 Sep 14.
Autonomic neuropathies represent a complex group of disorders that preferentially target autonomic fibers and can be classified as either acute/subacute or chronic in onset. Acute-onset autonomic neuropathies manifest with such conditions as paraneoplastic syndromes, Guillain-Barre syndrome, Sjögren syndrome, infection, or toxins/chemotherapy. When the presentation is acute, immune-mediated, and without a secondary cause, autoimmune autonomic ganglionopathy is likely, and should be considered for immunotherapy. Of the chronic-onset forms, diabetes is the most widespread and disabling, with autonomic impairment portending increased mortality and cardiac wall remodeling risk. Acquired light chain (AL) and transthyretin (TTR) amyloidosis represent two other key etiologies, with TTR amyloidosis now amenable to newly-approved gene-modifying therapies. The COMPASS-31 questionnaire is a validated outcome measure that can be used to monitor autonomic severity and track treatment response. Symptomatic treatments targeting orthostatic hypotension, among other symptoms, should be individualized and complement disease-modifying therapy, when possible.
自主神经病是一组复杂的疾病，主要影响自主神经纤维，可分为急性/亚急性或慢性起病。急性起病的自主神经病表现为副肿瘤综合征、格林-巴利综合征、干燥综合征、感染或毒素/化疗等疾病。当表现为急性、免疫介导且无继发原因时，可能是自身免疫性自主神经节神经病，应考虑免疫治疗。在慢性起病的形式中，糖尿病最常见且致残，自主神经损伤预示着死亡率增加和心脏壁重构风险。获得性轻链 (AL) 和转甲状腺素蛋白 (TTR) 淀粉样变性是另外两个主要病因，现在 TTR 淀粉样变性可采用新批准的基因修饰治疗。COMPASS-31 问卷是一种经过验证的结局测量工具，可用于监测自主神经严重程度和跟踪治疗反应。针对直立性低血压等症状的对症治疗应个体化，并在可能时补充疾病修饰治疗。
