Department of Medicine, Neurology Section, UT Health Science Center at Tyler, Tyler, TX, United States.
Department of Neurology, UT Southwestern Medical Center, Dallas, TX, United States.
Handb Clin Neurol. 2024;200:275-282. doi: 10.1016/B978-0-12-823912-4.00005-0.
A number of the well-recognized autoimmune and paraneoplastic neurologic syndromes commonly involve the autonomic nervous system. In some cases, the autonomic nerves or ganglia are primary targets of neurologic autoimmunity, as in immune-mediated autonomic ganglionopathies. In other disorders such as encephalitis, autonomic centers in the brain may be affected. The presence of autonomic dysfunction (especially gastrointestinal dysmotility) is sometimes overlooked even though this may contribute significantly to the symptom burden in these paraneoplastic disorders. Additionally, recognition of autonomic features as part of the clinical syndrome can help point the diagnostic evaluation toward autoimmune and paraneoplastic etiologies. As with other paraneoplastic disorders, the clinical syndrome and the presence and type of neurologic autoantibodies help to secure the diagnosis and direct the most appropriate investigation for malignancy. Optimal management for these conditions typically includes aggressive treatment of the neoplasm, immunomodulatory therapy, and symptomatic treatments for orthostatic hypotension and gastrointestinal dysmotility.
许多公认的自身免疫性和副肿瘤性神经综合征通常涉及自主神经系统。在某些情况下，自主神经或神经节是神经自身免疫的主要靶标，如免疫介导的自主神经节病变。在其他疾病中，如脑炎，大脑中的自主中枢可能受到影响。尽管自主功能障碍（特别是胃肠道动力障碍）可能会显著增加这些副肿瘤性疾病的症状负担，但有时会忽略其存在。此外，将自主特征识别为临床综合征的一部分有助于将诊断评估指向自身免疫和副肿瘤性病因。与其他副肿瘤性疾病一样，临床综合征以及神经自身抗体的存在和类型有助于确定诊断，并为恶性肿瘤提供最合适的检查。这些疾病的最佳治疗方法通常包括积极治疗肿瘤、免疫调节治疗以及体位性低血压和胃肠道动力障碍的对症治疗。
