Department of Neurology, University of Rostock, Germany.
Autoimmun Rev. 2012 Jan;11(3):196-202. doi: 10.1016/j.autrev.2011.05.012. Epub 2011 May 18.
Various case series of patients with autoimmune demyelinating disease affecting both the central and peripheral nervous system (CNS and PNS), either sequentially or simultaneously, have been reported for decades, but their frequency is considerably lower than that of the "classical" neurological autoimmune diseases affecting only either CNS or PNS, such as multiple sclerosis (MS), chronic inflammatory demyelinating polyneuropathy (CIDP) or Guillain-Barré-Syndrome (GBS), and attempts to define or even recognize the former as a clinical entity have remained elusive. Frequently, demyelination started with CNS involvement with subsequent PNS pathology, in some cases with a relapsing-remitting course. Three potential mechanisms for the autoimmune etiology of these conditions can be discussed: (I) They could be caused by a common autoimmunological reactivity against myelin antigens or epitopes present in both the central and peripheral nervous system; (II) They could be due to a higher general susceptibility to autoimmune disease, which in some cases may have been caused or exacerbated by immunomodulatory treatment, e.g. b-interferon; (III) Their co-occurrence might be coincidental. Another example of an autoimmune disease variably involving the central or peripheral nervous system or both is the overlapping and continuous clinical spectrum of Fisher syndrome (FS), as a variant of GBS, and Bickerstaff brainstem encephalitis (BBE). Recent data from larger patient cohorts with demonstration of common autoantibodies, antecedent infections, and results of detailed clinical, neuroimaging and neurophysiological investigations suggest that these three conditions are not separate disorders, but rather form a continuous spectrum with variable central and peripheral nervous system involvement. We herein review clinical and paraclinical data and therapeutic options of these disorders and discuss potential underlying common vs. divergent immunopathogenic mechanisms.
几十年来，已经报道了许多影响中枢神经系统（CNS）和周围神经系统（PNS）的自身免疫性脱髓鞘疾病的病例系列，这些疾病要么是先后发生，要么是同时发生，但它们的频率明显低于仅影响 CNS 或 PNS 的“经典”神经自身免疫性疾病，如多发性硬化症（MS）、慢性炎症性脱髓鞘性多发性神经病（CIDP）或格林-巴利综合征（GBS），并且试图定义甚至识别前者作为一种临床实体仍然难以捉摸。脱髓鞘通常从 CNS 受累开始，随后出现 PNS 病理学，在某些情况下具有复发性缓解病程。这些疾病的自身免疫病因有三种潜在机制可以讨论：（I）它们可能是由针对存在于中枢和周围神经系统中的髓鞘抗原或表位的共同自身免疫反应引起的；（II）它们可能是由于对自身免疫性疾病的普遍易感性增加所致，在某些情况下，这种易感性可能是由免疫调节治疗（例如β干扰素）引起或加重的；（III）它们的同时发生可能是偶然的。另一个自身免疫性疾病可变地涉及中枢或周围神经系统或两者的例子是重叠和连续的临床谱 Fisher 综合征（FS），作为 GBS 的一种变体，和 Bickerstaff 脑干脑炎（BBE）。来自更大患者队列的最近数据表明存在共同自身抗体、前驱感染以及详细临床、神经影像学和神经生理学研究的结果表明，这三种情况不是单独的疾病，而是形成具有可变中枢和周围神经系统受累的连续谱。本文综述了这些疾病的临床和临床前数据以及治疗选择，并讨论了潜在的共同与不同免疫发病机制。
