大多数患者对治疗的反应包括力量、感觉和步态的改善。 约 75%-80% 的患者在治疗的前几个月对 IVIG、 皮质类固醇或者血浆置换会有初始反应。 然而,反应可能不完全,并且患者可能会留有残留症状。 远端无力和感觉异常通常无法完全消退。
一些研究已着眼于远期预后,并发现超过 5-10 年后,大部分患者持续良好,其中 75% 的患者正常或者仅有轻微症状。[2]McCombe PA, Pollard JD, McLeod JG. Chronic inflammatory demyelinating polyradiculoneuropathy: clinical and electrophysiological study of 92 cases. Brain. 1987 Dec;110 (Pt 6):1617-30.http://www.ncbi.nlm.nih.gov/pubmed/3427403?tool=bestpractice.com[3]Barohn RJ, Kissel JT, Warmolts JR, et al. Chronic inflammatory demyelinating polyradiculoneuropathy: clinical characteristics, course, and recommendations for diagnostic criteria. Arch Neurol. 1989 Aug;46(8):878-84.http://www.ncbi.nlm.nih.gov/pubmed/2757528?tool=bestpractice.com[4]Hattori N, Misu K, Koike H, et al. Age of onset influences clinical features of chronic inflammatory demyelinating polyneuropathy. J Neurol Sci. 2001 Feb 15;184(1):57-63.http://www.ncbi.nlm.nih.gov/pubmed/11231033?tool=bestpractice.com[11]Gorson KC, Allam G, Ropper AH. Chronic inflammatory demyelinating polyneuropathy: clinical features and response to treatment in 67 consecutive patients with and without a monoclonal gammopathy. Neurology. 1997 Feb;48(2):321-8.http://www.ncbi.nlm.nih.gov/pubmed/9040714?tool=bestpractice.com[12]Bouchard C, Lacroix C, Plante V, et al. Clinicopathologic findings and prognosis of chronic inflammatory demyelinating polyneuropathy. Neurology. 1999 Feb;52(3):498-503.http://www.ncbi.nlm.nih.gov/pubmed/10025777?tool=bestpractice.com[32]Simmons Z, Albers JW, Bromberg MB, et al. Long-term follow-up of patients with chronic inflammatory demyelinating polyradiculoneuropathy, without and with monoclonal gammopathy. Brain. 1995 Apr;118 (Pt 2):359-68.http://www.ncbi.nlm.nih.gov/pubmed/7735878?tool=bestpractice.com[165]Sghirlanzoni A, Solari A, Ciano C, et al. Chronic inflammatory demyelinating polyradiculoneuropathy: long-term course and treatment of 60 patients. Neurol Sci. 2000 Feb;21(1):31-7.http://www.ncbi.nlm.nih.gov/pubmed/10938200?tool=bestpractice.com[166]van Doorn PA, Vermeulen M, Brand A, et al. Intravenous immunoglobulin treatment in patients with chronic inflammatory demyelinating polyneuropathy. Clinical and laboratory characteristics associated with improvement. Arch Neurol. 1991 Feb;48(2):217-20.http://www.ncbi.nlm.nih.gov/pubmed/1847041?tool=bestpractice.com
大约 50% 的患者会处于缓解期且无需任何治疗。 约 10% 的患者效果较差伴有重度残疾或死亡。
预后因素
一些研究已着眼于预后因素。[2]McCombe PA, Pollard JD, McLeod JG. Chronic inflammatory demyelinating polyradiculoneuropathy: clinical and electrophysiological study of 92 cases. Brain. 1987 Dec;110 (Pt 6):1617-30.http://www.ncbi.nlm.nih.gov/pubmed/3427403?tool=bestpractice.com[4]Hattori N, Misu K, Koike H, et al. Age of onset influences clinical features of chronic inflammatory demyelinating polyneuropathy. J Neurol Sci. 2001 Feb 15;184(1):57-63.http://www.ncbi.nlm.nih.gov/pubmed/11231033?tool=bestpractice.com[12]Bouchard C, Lacroix C, Plante V, et al. Clinicopathologic findings and prognosis of chronic inflammatory demyelinating polyneuropathy. Neurology. 1999 Feb;52(3):498-503.http://www.ncbi.nlm.nih.gov/pubmed/10025777?tool=bestpractice.com[32]Simmons Z, Albers JW, Bromberg MB, et al. Long-term follow-up of patients with chronic inflammatory demyelinating polyradiculoneuropathy, without and with monoclonal gammopathy. Brain. 1995 Apr;118 (Pt 2):359-68.http://www.ncbi.nlm.nih.gov/pubmed/7735878?tool=bestpractice.com[165]Sghirlanzoni A, Solari A, Ciano C, et al. Chronic inflammatory demyelinating polyradiculoneuropathy: long-term course and treatment of 60 patients. Neurol Sci. 2000 Feb;21(1):31-7.http://www.ncbi.nlm.nih.gov/pubmed/10938200?tool=bestpractice.com 不利的预后因素包括年龄较大、进行性病程、中枢神经系统受累、显示活性脱髓鞘的大量纤维,以及神经活检提示轴突缺失。有利的预后因素包括年龄较小、复发性或亚急性特征及近端无力。
对 IVIG 的反应
单相性或复发-缓解(非慢性进行性)病程以及在脑脊液蛋白中含量增加两倍提示对 IVIG 反应良好。[167]Tackenberg B, Lünemann JD, Steinbrecher A, et al. Classifications and treatment responses in chronic immune-mediated demyelinating polyneuropathy. Neurology. 2007 May 8;68(19):1622-9.http://www.ncbi.nlm.nih.gov/pubmed/17485651?tool=bestpractice.com 另一项研究提示,如果糖尿病患者罹患此病病情更为严重,但与非糖尿病患者相比,其对 IVIG 反应良好且较少复发。[168]Pedersen K, Pandolfo M, Mavroudakis N. Chronic inflammatory demyelinating polyneuropathy in a diabetic patient: deterioration after intravenous immunoglobulins treatment and favorable response to steroid treatment. Acta Neurol Belg. 2007 Mar;107(1):14-7.http://www.ncbi.nlm.nih.gov/pubmed/17569228?tool=bestpractice.com