到第 6 个月时,所有 Bell 面瘫病例都会显示面部肌张力和运动恢复。到第 6 个月不存在一定程度的功能恢复,提示其他诊断。在不进行治疗的情况下,70% 的 Bell 面瘫病例会完全恢复正常功能,而 13% 会恢复到遗留轻微程度的联带运动,16% 会恢复到遗留重度面部联带运动伴面部表情显著受损。[1]Peitersen E. The natural history of Bell palsy. Am J Otol. 1982;4:107-111.http://www.ncbi.nlm.nih.gov/pubmed/7148998?tool=bestpractice.com人们认为,不治疗时,最终临床结果取决于面部神经的初始损伤程度、共病和解剖学因素,例如面神经管的直径。除了在 Bell 面瘫的急性弛缓阶段使用角膜保护措施外,当前的治疗流程以减少初始神经损伤程度为目标,缩短功能恢复的时间,避免长期发生重度面部联带运动。
口服皮质类固醇是急性治疗的主要方法,[26]Gronseth GS, Paduga R; American Academy of Neurology. Evidence-based guideline update: steroids and antivirals for Bell palsy. Neurology. 2012;79:2209-2213.http://www.neurology.org/content/79/22/2209.longhttp://www.ncbi.nlm.nih.gov/pubmed/23136264?tool=bestpractice.com几项大型前瞻性对照试验[27]Sullivan FM, Swan IR, Donnan PT, et al. A randomised controlled trial of the use of aciclovir and/or prednisolone for the early treatment of Bell's palsy: the BELLS study. Health Technol Assess. 2009;13:1-130.http://www.journalslibrary.nihr.ac.uk/hta/volume-13/issue-47http://www.ncbi.nlm.nih.gov/pubmed/19833052?tool=bestpractice.com[28]Hato N, Yamada H, Kohno H, et al. Valacyclovir and prednisolone treatment for Bell's palsy: a multicenter, randomized, placebo-controlled study. Otol Neurotol. 2007;28:408-413.http://www.ncbi.nlm.nih.gov/pubmed/17414047?tool=bestpractice.com[29]Engström M, Berg T, Stjernquist-Desatnik A, et al. Prednisolone and valaciclovir in Bell's palsy: a randomised, double-blind, placebo-controlled, multicentre trial. Lancet Neurol. 2008;7:993-1000.http://www.ncbi.nlm.nih.gov/pubmed/18849193?tool=bestpractice.com和 meta 分析证明了其益处;[30]Madhok VB, Gagyor I, Daly F, et al. Corticosteroids for Bell's palsy (idiopathic facial paralysis). Cochrane Database Syst Rev. 2016;(7):CD001942.http://onlinelibrary.wiley.com/doi/10.1002/14651858.CD001942.pub5/fullhttp://www.ncbi.nlm.nih.gov/pubmed/27428352?tool=bestpractice.com应当向所有患者提供这些治疗,最好是在症状发生 72 小时内。虽然禁用抗病毒单药治疗(除非是禁用皮质类固醇的例外病例),[26]Gronseth GS, Paduga R; American Academy of Neurology. Evidence-based guideline update: steroids and antivirals for Bell palsy. Neurology. 2012;79:2209-2213.http://www.neurology.org/content/79/22/2209.longhttp://www.ncbi.nlm.nih.gov/pubmed/23136264?tool=bestpractice.com[27]Sullivan FM, Swan IR, Donnan PT, et al. A randomised controlled trial of the use of aciclovir and/or prednisolone for the early treatment of Bell's palsy: the BELLS study. Health Technol Assess. 2009;13:1-130.http://www.journalslibrary.nihr.ac.uk/hta/volume-13/issue-47http://www.ncbi.nlm.nih.gov/pubmed/19833052?tool=bestpractice.com[29]Engström M, Berg T, Stjernquist-Desatnik A, et al. Prednisolone and valaciclovir in Bell's palsy: a randomised, double-blind, placebo-controlled, multicentre trial. Lancet Neurol. 2008;7:993-1000.http://www.ncbi.nlm.nih.gov/pubmed/18849193?tool=bestpractice.com[31]Gagyor I, Madhok VB, Daly F, et al. Antiviral treatment for Bell's palsy (idiopathic facial paralysis). Cochrane Database Syst Rev. 2015;(11):CD001869.http://onlinelibrary.wiley.com/doi/10.1002/14651858.CD001869.pub8/fullhttp://www.ncbi.nlm.nih.gov/pubmed/26559436?tool=bestpractice.com[32]De Diego JI, Prim MP, De Sarriá MJ, et al. Idiopathic facial paralysis: a randomized, prospective, and controlled study using single-dose prednisone versus acyclovir three times daily. Laryngoscope. 1998;108:573-575.http://www.ncbi.nlm.nih.gov/pubmed/9546272?tool=bestpractice.com但在 Bell 面瘫的急性期,抗病毒药物疗法联合皮质类固醇可能提供额外的临床益处,尤其是对于重度至完全面瘫患者。[33]de Almeida JR, Al Khabori M, Guyatt GH, et al. Combined corticosteroid and antiviral treatment for Bell palsy: a systematic review and meta-analysis. JAMA. 2009;302:985-993.http://jama.jamanetwork.com/article.aspx?articleid=184482http://www.ncbi.nlm.nih.gov/pubmed/19724046?tool=bestpractice.com[34]Adour KK, Ruboyianes JM, Von Doersten PG, et al. Bell's palsy treatment with acyclovir and prednisone compared with prednisone alone: a double-blind, randomized, controlled trial. Ann Otol Rhinol Laryngol. 1996;105:371-378.http://www.ncbi.nlm.nih.gov/pubmed/8651631?tool=bestpractice.com在 Bell 面瘫的急性期,如果患者的电神经描记(ENoG,也称为诱发肌电图 [eEMG])表现为变性>90%,则从内耳道到茎乳孔,对骨性面神经管进行手术减压,在避免长期发生联带运动方面有益处。[35]Gantz BJ, Rubinstein JT, Gidley P, et al. Surgical management of Bell palsy. Laryngoscope. 1999;109:1177-1188.http://www.ncbi.nlm.nih.gov/pubmed/10443817?tool=bestpractice.com
皮质类固醇和抗病毒药物
来自随机对照试验 (RCT) 和 meta 分析的强有力证据支持在症状发生 72 小时内使用口服皮质类固醇,以便缩短成人完全恢复的时间,改善长期结果,无论面瘫的基线期严重程度如何。[26]Gronseth GS, Paduga R; American Academy of Neurology. Evidence-based guideline update: steroids and antivirals for Bell palsy. Neurology. 2012;79:2209-2213.http://www.neurology.org/content/79/22/2209.longhttp://www.ncbi.nlm.nih.gov/pubmed/23136264?tool=bestpractice.com[27]Sullivan FM, Swan IR, Donnan PT, et al. A randomised controlled trial of the use of aciclovir and/or prednisolone for the early treatment of Bell's palsy: the BELLS study. Health Technol Assess. 2009;13:1-130.http://www.journalslibrary.nihr.ac.uk/hta/volume-13/issue-47http://www.ncbi.nlm.nih.gov/pubmed/19833052?tool=bestpractice.com[28]Hato N, Yamada H, Kohno H, et al. Valacyclovir and prednisolone treatment for Bell's palsy: a multicenter, randomized, placebo-controlled study. Otol Neurotol. 2007;28:408-413.http://www.ncbi.nlm.nih.gov/pubmed/17414047?tool=bestpractice.com[29]Engström M, Berg T, Stjernquist-Desatnik A, et al. Prednisolone and valaciclovir in Bell's palsy: a randomised, double-blind, placebo-controlled, multicentre trial. Lancet Neurol. 2008;7:993-1000.http://www.ncbi.nlm.nih.gov/pubmed/18849193?tool=bestpractice.com[30]Madhok VB, Gagyor I, Daly F, et al. Corticosteroids for Bell's palsy (idiopathic facial paralysis). Cochrane Database Syst Rev. 2016;(7):CD001942.http://onlinelibrary.wiley.com/doi/10.1002/14651858.CD001942.pub5/fullhttp://www.ncbi.nlm.nih.gov/pubmed/27428352?tool=bestpractice.com[31]Gagyor I, Madhok VB, Daly F, et al. Antiviral treatment for Bell's palsy (idiopathic facial paralysis). Cochrane Database Syst Rev. 2015;(11):CD001869.http://onlinelibrary.wiley.com/doi/10.1002/14651858.CD001869.pub8/fullhttp://www.ncbi.nlm.nih.gov/pubmed/26559436?tool=bestpractice.com[36]Sullivan FM, Swan IR, Donnan PT, et al. Early treatment with prednisolone or acyclovir in Bell's palsy. N Engl J Med. 2007;357:1598-1607.http://www.ncbi.nlm.nih.gov/pubmed/17942873?tool=bestpractice.com[37]Axelsson S, Berg T, Jonsson L, et al. Prednisolone in Bell's palsy related to treatment start and age. Otol Neurotol. 2011;32:141-146.http://www.ncbi.nlm.nih.gov/pubmed/21099725?tool=bestpractice.com[38]Axelsson S, Berg T, Jonsson L, et al. Bell's palsy - the effect of prednisolone and/or valaciclovir versus placebo in relation to baseline severity in a randomised controlled trial. Clin Otolaryngol. 2012;37:283-290.http://www.ncbi.nlm.nih.gov/pubmed/22776019?tool=bestpractice.com[39]Berg T, Bylund N, Marsk E, et al. The effect of prednisolone on sequelae in Bell's palsy. Arch Otolaryngol Head Neck Surg. 2012;138:445-449.http://archotol.jamanetwork.com/article.aspx?articleid=1157682http://www.ncbi.nlm.nih.gov/pubmed/22652942?tool=bestpractice.com运动功能恢复:有高质量证据表明,在最多12 个月时的面部运动功能恢复方面,皮质类固醇比安慰剂或无治疗更有效。系统评价或者受试者>200名的随机对照临床试验(RCT)。在 Bell 面瘫中使用皮质类固醇的主要依据是利用它们的强力抗炎作用,根据推测能减轻神经水肿,使神经损伤减至最小。在儿童(年龄<16 岁)以及糖尿病控制不良、免疫缺陷、血压控制不良和有既往精神病史的患者中使用高剂量皮质类固醇应当谨慎。
强有力的证据建议不使用抗病毒单药治疗用于 Bell 面瘫,抗病毒单药治疗的结局劣于皮质类固醇单药治疗,与安慰剂相比没有益处。[29]Engström M, Berg T, Stjernquist-Desatnik A, et al. Prednisolone and valaciclovir in Bell's palsy: a randomised, double-blind, placebo-controlled, multicentre trial. Lancet Neurol. 2008;7:993-1000.http://www.ncbi.nlm.nih.gov/pubmed/18849193?tool=bestpractice.com[36]Sullivan FM, Swan IR, Donnan PT, et al. Early treatment with prednisolone or acyclovir in Bell's palsy. N Engl J Med. 2007;357:1598-1607.http://www.ncbi.nlm.nih.gov/pubmed/17942873?tool=bestpractice.com[31]Gagyor I, Madhok VB, Daly F, et al. Antiviral treatment for Bell's palsy (idiopathic facial paralysis). Cochrane Database Syst Rev. 2015;(11):CD001869.http://onlinelibrary.wiley.com/doi/10.1002/14651858.CD001869.pub8/fullhttp://www.ncbi.nlm.nih.gov/pubmed/26559436?tool=bestpractice.com运动功能恢复:有高质量的证据表明,在治疗结束时,在降低不完全恢复的患者比例方面,抗病毒治疗不如皮质类固醇有效。系统评价或者受试者>200名的随机对照临床试验(RCT)。
Meta 分析发现的中等质量证据表明,与单用皮质类固醇相比,抗病毒药物和皮质类固醇联合用药可减少 Bell 面瘫的长期后遗症,尤其是在最初存在完全或几乎完全性面瘫的患者中。[33]de Almeida JR, Al Khabori M, Guyatt GH, et al. Combined corticosteroid and antiviral treatment for Bell palsy: a systematic review and meta-analysis. JAMA. 2009;302:985-993.http://jama.jamanetwork.com/article.aspx?articleid=184482http://www.ncbi.nlm.nih.gov/pubmed/19724046?tool=bestpractice.com[31]Gagyor I, Madhok VB, Daly F, et al. Antiviral treatment for Bell's palsy (idiopathic facial paralysis). Cochrane Database Syst Rev. 2015;(11):CD001869.http://onlinelibrary.wiley.com/doi/10.1002/14651858.CD001869.pub8/fullhttp://www.ncbi.nlm.nih.gov/pubmed/26559436?tool=bestpractice.com[40]Goudakos JK, Markou KD. Corticosteroids vs corticosteroids plus antiviral agents in the treatment of Bell palsy: a systematic review and meta-analysis. Arch Otolaryngol Head Neck Surg. 2009;135:558-564.http://archotol.jamanetwork.com/article.aspx?articleid=410238http://www.ncbi.nlm.nih.gov/pubmed/19528403?tool=bestpractice.com[41]Quant EC, Jeste SS, Muni RH, et al. The benefits of steroids versus steroids plus antivirals for treatment of Bell's palsy: a meta-analysis. BMJ. 2009;339:b3354.http://www.bmj.com/content/339/bmj.b3354.longhttp://www.ncbi.nlm.nih.gov/pubmed/19736282?tool=bestpractice.com运动功能恢复:有中等质量证据表明,在治疗结束时,在降低面部功能不完全恢复的风险方面,皮质类固醇加抗病毒药物治疗比安慰剂更有效。受试者<200名的随机对照临床试验(RCT)、受试者>200名且方法学存在缺陷的随机对照临床试验(RCT)、方法学存在缺陷的系统评价或者高质量的观察性(队列)研究。重要的是,已经证明,将抗病毒药物疗法添加至皮质类固醇方案,不良事件没有增加。[31]Gagyor I, Madhok VB, Daly F, et al. Antiviral treatment for Bell's palsy (idiopathic facial paralysis). Cochrane Database Syst Rev. 2015;(11):CD001869.http://onlinelibrary.wiley.com/doi/10.1002/14651858.CD001869.pub8/fullhttp://www.ncbi.nlm.nih.gov/pubmed/26559436?tool=bestpractice.com在 Bell 面瘫中使用抗病毒药物的依据是,推测引发疾病的是病毒再活化,抗病毒药物早期给药可能降低病毒载量以及后续的轴突和施旺氏细胞损伤。
手术减压
证据显示,如果症状发作 2 周内,电神经描记 (ENoG) 显示两侧之间的差异>95%,则 Bell 面瘫后自发恢复到满意的(House-Brackmann 分级 [HBG] I 或 II 级)面部功能会减少 50%。在一项研究中,在系列 ENoG 证明变性为 90% 至 94% 的时间点 24 小时内,通过颅中窝入路对病理性压缩部位(在 94% 的病例中为内耳道孔)和附近节段进行减压,可使满意结果增加 30% 或更多。[16]Fisch U. Surgery for Bell's palsy. Arch Otolaryngol. 1981;107:1-11.http://www.ncbi.nlm.nih.gov/pubmed/7469872?tool=bestpractice.com第二项试验证明,诊断为 Bell 面瘫的患者,如果表现为重度或完全性弛缓性麻痹(即:HBG V 级或 VI 级),ENoG 应答表明与对侧的健侧相比变性>90%,并且肌电图 (EMG) 不存在自主运动单元电位,则包括内耳道孔在内的手术减压可使长期结果出现有临床意义和统计学意义的改善。[35]Gantz BJ, Rubinstein JT, Gidley P, et al. Surgical management of Bell palsy. Laryngoscope. 1999;109:1177-1188.http://www.ncbi.nlm.nih.gov/pubmed/10443817?tool=bestpractice.com在这项研究中,符合入组标准、除了药物疗法之外在症状发作 2 周内接受减压术的患者中,91% 最终进展至 HBG I 或 II 级,而仅接受药物疗法的患者中为 42%。[35]Gantz BJ, Rubinstein JT, Gidley P, et al. Surgical management of Bell palsy. Laryngoscope. 1999;109:1177-1188.http://www.ncbi.nlm.nih.gov/pubmed/10443817?tool=bestpractice.com[42]McAllister K, Walker D, Donnan PT, et al. Surgical interventions for the early management of Bell's palsy. Cochrane Database Syst Rev. 2013;(10):CD007468.http://onlinelibrary.wiley.com/doi/10.1002/14651858.CD007468.pub3/fullhttp://www.ncbi.nlm.nih.gov/pubmed/21328293?tool=bestpractice.com两项研究都受患者自我选择手术的影响而有偏差;这个局限性加上技术难度和相关的减压手术风险,例如听力受损、脑脊液漏和医源性面神经损伤,难以对其使用达成共识。[43]Baugh RF, Basura GJ, Ishii LE, et al. Clinical practice guideline: Bell's palsy. Otolaryngol Head Neck Surg. 2013;149(3 Suppl):S1-S27.http://oto.sagepub.com/content/149/3_suppl/S1.longhttp://www.ncbi.nlm.nih.gov/pubmed/24189771?tool=bestpractice.com尽管有人认为减压术对 Bell 面瘫没有益处,但结论是基于经乳突入路,内耳道孔并没有减压。[44]May M, Klein SR, Taylor FH. Idiopathic (Bell's) facial palsy: natural history defies steroid or surgical treatment. Laryngoscope. 1985;95:406-409.http://www.ncbi.nlm.nih.gov/pubmed/3982183?tool=bestpractice.com
对于符合下列标准的 Bell 面瘫患者,应当考虑由有经验的神经-耳科医生进行手术减压:
对面神经手术减压的依据是:将限制面神经管骨大约切除 180 度,并且从耳门到茎乳孔切除神经外膜,可为神经提供肿胀的空间,减少病毒引发的压迫性神经病和后续的缺血性神经损伤进展。
保护眼睛
在 Bell 面瘫的弛缓阶段,预防暴露性角膜病变至关重要。
白天应当戴眼镜而不是眼罩保护受累眼,根据需要使用人工泪液。在夜间,在涂抹温和的眼用润滑软膏后,可以使用无刺激的胶带将眼睑固定闭合;[45]The College of Optometrists (UK). Facial palsy (including Bell's palsy). March 2016. http://www.college-optometrists.org/ (last accessed 7 September 2017).http://www.college-optometrists.org/en/utilities/document-summary.cfm/docid/63AD5CC8-EB0F-41CB-89508DC2D43560FB还是应当避免使用眼罩。
眼科诊疗的适应症包括:唯一能看东西的眼睛受累,怀疑暴露性角膜炎,角膜感觉降低或缺失。对于缺少 Bell 现象(保护性反射,也就是在试图闭眼时眼球向上和向外旋转)的患者或早期快速恢复功能、预后差的患者(就诊时存在完全弛缓性麻痹、年龄较大的成人、味觉异常和糖尿病),应当强烈考虑早期上睑重量放置或睑缝合术(两者均是可逆的)。[23]Gilden DH. Clinical practice. Bell's palsy. N Engl J Med. 2004;351:1323-1331.http://www.ncbi.nlm.nih.gov/pubmed/15385659?tool=bestpractice.com