Head and Neck Department-ENT Department, Policlinico Umberto I University of Rome Sapienza, Italy.
Autoimmun Rev. 2012 Dec;12(2):323-8. doi: 10.1016/j.autrev.2012.05.008. Epub 2012 Jun 8.
To review our current knowledge of the etiopathogenesis of Bell's palsy, including viral infection or autoimmunity, and to discuss disease pathogenesis with respect to pharmacotherapy.
Relevant publications on the etiopathogenesis, clinical presentation, diagnosis and histopathology of Bell's palsy from 1975 to 2012 were analysed.
Bell's palsy is an idiopathic peripheral nerve palsy involving the facial nerve. It accounts for 60 to 75% of all cases of unilateral facial paralysis. The annual incidence of Bell's palsy is 15 to 30 per 100,000 people. The peak incidence occurs between the second and fourth decades (15 to 45 years). The aetiology of Bell's palsy is unknown but viral infection or autoimmune disease has been postulated as possible pathomechanisms. Bell's palsy may be caused when latent herpes viruses (herpes simplex, herpes zoster) are reactivated from cranial nerve ganglia. A cell-mediated autoimmune mechanism against a myelin basic protein has been suggested for the pathogenesis of Bell's palsy. Bell's palsy may be an autoimmune demyelinating cranial neuritis, and in most cases, it is a mononeuritic variant of Guillain-Barré syndrome, a neurologic disorder with recognised cell-mediated immunity against peripheral nerve myelin antigens. In Bell's palsy and GBS, a viral infection or the reactivation of a latent virus may provoke an autoimmune reaction against peripheral nerve myelin components, leading to the demyelination of cranial nerves, especially the facial nerve. Given the safety profile of acyclovir, valacyclovir, and short-course oral corticosteroids, patients who present within three days of the onset of symptoms should be offered combination therapy. However it seems logical that in fact, steroids exert their beneficial effect via immunosuppressive action, as is the case in some other autoimmune disorders. It is to be hoped that (monoclonal) antibodies and/or T-cell immunotherapy might provide more specific treatment guidelines in the management of Bell's palsy.
回顾贝尔面瘫的病因发病机制,包括病毒感染或自身免疫,并讨论药物治疗与疾病发病机制的关系。
对 1975 年至 2012 年贝尔面瘫的病因发病机制、临床特征、诊断和组织病理学相关文献进行分析。
贝尔面瘫是一种特发性周围性面神经麻痹,占所有单侧面神经麻痹的 60%75%。贝尔面瘫的年发病率为 1530/10 万。发病高峰为 2 至 4 decades(15~45 岁)。贝尔面瘫的病因不明,但病毒感染或自身免疫性疾病被认为是可能的发病机制。潜伏的疱疹病毒(单纯疱疹、带状疱疹)可能从颅神经节重新激活而导致贝尔面瘫。针对髓鞘碱性蛋白的细胞介导自身免疫机制被认为与贝尔面瘫的发病机制有关。贝尔面瘫可能是一种自身免疫性脱髓鞘性颅神经炎,在大多数情况下,它是格林-巴利综合征的单神经炎变异型,格林-巴利综合征是一种神经系统疾病,其特征是针对周围神经髓鞘抗原的细胞介导免疫。在贝尔面瘫和格林-巴利综合征中,病毒感染或潜伏病毒的重新激活可能引发针对周围神经髓鞘成分的自身免疫反应,导致颅神经脱髓鞘,特别是面神经。鉴于阿昔洛韦、伐昔洛韦和短期口服皮质类固醇的安全性,应向症状出现后 3 天内的患者提供联合治疗。然而,皮质类固醇通过免疫抑制作用发挥其有益作用似乎是合乎逻辑的,就像在其他一些自身免疫性疾病中一样。人们希望(单克隆)抗体和/或 T 细胞免疫疗法能够为贝尔面瘫的治疗提供更具体的治疗指南。