慢性炎性脱髓鞘性多发性神经根神经病

GBS 典型病程小于 4 周。 在病程为 4-8 周的病例中，可能很难鉴别，因为这些病例趋于更像 CIDP 而非 GBS。[6]Oh SJ, Kurokawa K, de Almeida DF, et al. Subacute inflammatory demyelinating neuropathy. Neurology. 2003 Dec 9;61(11):1507-12.http://www.ncbi.nlm.nih.gov/pubmed/14663033?tool=bestpractice.com[7]Hughes R, Sanders E, Hall S, et al. Subacute idiopathic demyelinating polyradiculoneuropathy. Arch Neurol. 1992 Jun;49(6):612-6.http://www.ncbi.nlm.nih.gov/pubmed/1317700?tool=bestpractice.com

CIDP 的一些亚急性特征可能像在 GBS 中一样呈现单相。[8]Nevo Y, Pestronk A, Kornberg AJ, et al. Childhood chronic inflammatory demyelinating neuropathies: clinical course and long-term follow-up. Neurology. 1996 Jul;47(1):98-102.http://www.ncbi.nlm.nih.gov/pubmed/8710133?tool=bestpractice.com[9]Simmons Z, Wald JJ, Albers JW. Chronic inflammatory demyelinating polyradiculoneuropathy in children: I. Presentation, electrodiagnostic studies, and initial clinical course, with comparison to adults. Muscle Nerve. 1997 Aug;20(8):1008-15.http://www.ncbi.nlm.nih.gov/pubmed/9236792?tool=bestpractice.com

更频繁的前期感染、呼吸衰竭、颅神经损害和自主功能障碍。[1]Dyck PJ, Lais AC, Ohta M, et al. Chronic inflammatory polyradiculoneuropathy. Mayo Clin Proc. 1975 Nov;50(11):621-37.http://www.ncbi.nlm.nih.gov/pubmed/1186294?tool=bestpractice.com[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[5]Said G. Chronic inflammatory demyelinative polyneuropathy. J Neurol. 2002 Mar;249(3):245-53.http://www.ncbi.nlm.nih.gov/pubmed/11993521?tool=bestpractice.com

神经传导检查的脱髓鞘特性往往不那么严重。[48]Van den Bergh PY, Pieret F. Electrodiagnostic criteria for acute and chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve. 2004 Apr;29(4):565-74.http://www.ncbi.nlm.nih.gov/pubmed/15052622?tool=bestpractice.com

脑脊液 (CSF) 异常较少见，且 CSF 蛋白通常未升高。[1]Dyck PJ, Lais AC, Ohta M, et al. Chronic inflammatory polyradiculoneuropathy. Mayo Clin Proc. 1975 Nov;50(11):621-37.http://www.ncbi.nlm.nih.gov/pubmed/1186294?tool=bestpractice.com[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

与亚急性 CIDP 患者相比，GBS 患者的神经超声所显示的神经肿大可能较少。[73]Kerasnoudis A, Pitarokoili K, Behrendt V, et al. Bochum ultrasound score versus clinical and electrophysiological parameters in distinguishing acute-onset chronic from acute inflammatory demyelinating polyneuropathy. Muscle Nerve. 2015 Jun;51(6):846-52.http://www.ncbi.nlm.nih.gov/pubmed/25297575?tool=bestpractice.com

发作较早、无力进展缓慢、骨骼畸形（例如高弓足）和极远端无力。[74]Pareyson D. Differential diagnosis of Charcot-Marie-Tooth disease and related neuropathies. Neurol Sci. 2004 Jun;25(2):72-82.http://www.ncbi.nlm.nih.gov/pubmed/15221625?tool=bestpractice.com

电诊法特性包括传导速度一直减慢和无传导阻滞。[75]Lewis RA, Sumner AJ. The electrodiagnostic distinctions between chronic familial and acquired demyelinative neuropathies. Neurology. 1982 Jun;32(6):592-6.http://www.ncbi.nlm.nih.gov/pubmed/6283420?tool=bestpractice.com

末端潜伏期指数、改良 F 波比率和复合肌肉动作电位波幅分散也可能存在区别。[76]Attarian S, Azulay JP, Boucraut J, et al. Terminal latency index and modified F ratio in distinction of chronic demyelinating neuropathies. Clin Neurophysiol. 2001 Mar;112(3):457-63.http://www.ncbi.nlm.nih.gov/pubmed/11222967?tool=bestpractice.com[77]Stanton M, Pannoni V, Lewis RA, et al. Dispersion of compound muscle action potential in hereditary neuropathies and chronic inflammatory demyelinating polyneuropathy. Muscle Nerve. 2006 Oct;34(4):417-22.http://www.ncbi.nlm.nih.gov/pubmed/16823858?tool=bestpractice.com

神经活检更常提示在 HMSN 中出现异常增厚、髓鞘折叠或者髓鞘形成不良的区域。[78]Sander S, Ouvrier RA, McLeod JG, et al. Clinical syndromes associated with tomacula or myelin swellings in sural nerve biopsies. J Neurol Neurosurg Psychiatry. 2000 Apr;68(4):483-8.http://jnnp.bmj.com/content/68/4/483.longhttp://www.ncbi.nlm.nih.gov/pubmed/10727485?tool=bestpractice.com

患者趋于老年化，更常见于男性，具有更缓慢的病程且仅呈现远端无力。[79]Latov N, Hays AP, Sherman WH. Peripheral neuropathy and anti-MAG antibodies. Crit Rev Neurobiol. 1988;3(4):301-32.http://www.ncbi.nlm.nih.gov/pubmed/2458847?tool=bestpractice.com[80]Ellie E, Vital A, Steck A, et al. Neuropathy associated with "benign" anti-myelin-associated glycoprotein IgM gammopathy: Clinical, immunological, neurophysiological pathological findings and response to treatment in 33 cases. J Neurol. 1996 Jan;243(1):34-43.http://www.ncbi.nlm.nih.gov/pubmed/8869385?tool=bestpractice.com

传导阻滞罕有发生。[80]Ellie E, Vital A, Steck A, et al. Neuropathy associated with "benign" anti-myelin-associated glycoprotein IgM gammopathy: Clinical, immunological, neurophysiological pathological findings and response to treatment in 33 cases. J Neurol. 1996 Jan;243(1):34-43.http://www.ncbi.nlm.nih.gov/pubmed/8869385?tool=bestpractice.com

电诊法发现远端潜伏期延长和传导速度减缓不成比例，这是明显特征。[81]Kaku DA, England JD, Sumner AJ. Distal accentuation of conduction slowing in polyneuropathy associated with antibodies to myelin-associated glycoprotein and sulphated glucuronyl paragloboside. Brain. 1994 Oct;117 (Pt 5):941-7.http://www.ncbi.nlm.nih.gov/pubmed/7953603?tool=bestpractice.com

基本上，在所有患者中通过酶联免疫吸附测定 (ELISA) 和蛋白质印迹均发现存在抗 MAG 抗体。[82]Eurelings M, Moons KG, Notermans NC, et al. Neuropathy and IgM M-proteins: prognostic value of antibodies to MAG, SGPG, and sulfatide. Neurology. 2001 Jan 23;56(2):228-33.http://www.ncbi.nlm.nih.gov/pubmed/11160960?tool=bestpractice.com[83]Pestronk A, Li F, Bieser K, et al. Anti-MAG antibodies: major effects of antigen purity and antibody cross-reactivity on ELISA results and clinical correlation. Neurology. 1994 Jun;44(6):1131-7.http://www.ncbi.nlm.nih.gov/pubmed/7516055?tool=bestpractice.com

相隔很远的髓鞘是典型的病理发现。[80]Ellie E, Vital A, Steck A, et al. Neuropathy associated with "benign" anti-myelin-associated glycoprotein IgM gammopathy: Clinical, immunological, neurophysiological pathological findings and response to treatment in 33 cases. J Neurol. 1996 Jan;243(1):34-43.http://www.ncbi.nlm.nih.gov/pubmed/8869385?tool=bestpractice.com

患者对皮质类固醇、静脉注射免疫球蛋白或血浆置换没有反应，[80]Ellie E, Vital A, Steck A, et al. Neuropathy associated with "benign" anti-myelin-associated glycoprotein IgM gammopathy: Clinical, immunological, neurophysiological pathological findings and response to treatment in 33 cases. J Neurol. 1996 Jan;243(1):34-43.http://www.ncbi.nlm.nih.gov/pubmed/8869385?tool=bestpractice.com[84]Lunn MP, Nobile-Orazio E. Immunotherapy for IgM anti-myelin-associated glycoprotein paraprotein-associated peripheral neuropathies. Cochrane Database Syst Rev. 2016;(10):CD002827.https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD002827.pub4/fullhttp://www.ncbi.nlm.nih.gov/pubmed/27701752?tool=bestpractice.com[85]Gorson KC, Ropper AH, Weinberg DH, et al. Treatment experience in patients with anti-myelin-associated glycoprotein neuropathy. Muscle Nerve. 2001 Jun;24(6):778-86.http://www.ncbi.nlm.nih.gov/pubmed/11360261?tool=bestpractice.com 但是近期研究提示患者可能对利妥昔单抗治疗有反应。[86]Zivkovic SA. Rituximab in the treatment of peripheral neuropathy associated with monoclonal gammopathy. Expert Rev Neurother. 2006 Sep;6(9):1267-74.http://www.ncbi.nlm.nih.gov/pubmed/17009914?tool=bestpractice.com

CIDP 和 MGUS（通常是 IgA 或 IgG）患者趋于老龄化，具有更远端无力、更缓慢的病程和更多感觉受累。[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[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[33]Smith IS. The natural history of chronic demyelinating neuropathy associated with benign IgM paraproteinaemia. A clinical and neurophysiological study. Brain. 1994 Oct;117 (Pt 5):949-57.http://www.ncbi.nlm.nih.gov/pubmed/7953604?tool=bestpractice.com

不伴有 CIDP 的IgA/IgG MGUS 相较 IgM MGUS 对血浆置换的反应更好。

血清和尿液免疫固定电泳可检测到单克隆蛋白。[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[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[33]Smith IS. The natural history of chronic demyelinating neuropathy associated with benign IgM paraproteinaemia. A clinical and neurophysiological study. Brain. 1994 Oct;117 (Pt 5):949-57.http://www.ncbi.nlm.nih.gov/pubmed/7953604?tool=bestpractice.com

患者通常对免疫治疗反应不佳。[87]Dyck PJ, Low PA, Windebank AJ, et al. Plasma exchange in polyneuropathy associated with monoclonal gammopathy of undetermined significance. N Engl J Med. 1991 Nov 21;325(21):1482-6.https://www.nejm.org/doi/10.1056/NEJM199111213252105?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%3dwww.ncbi.nlm.nih.govhttp://www.ncbi.nlm.nih.gov/pubmed/1658648?tool=bestpractice.com

如果免疫球蛋白水平大于 15 g/L (＞1.5 g/dL)，进行血液恶性肿瘤检查。[88]Kyle RA, Therneau TM, Rajkumar SV, et al. Long-term follow-up of 241 patients with monoclonal gammopathy of undetermined significance: the original Mayo Clinic series 25 years later. Mayo Clin Proc. 2004 Jul;79(7):859-66.http://www.ncbi.nlm.nih.gov/pubmed/15244381?tool=bestpractice.com

可见远端非对称性无力和感觉症状或体征缺失。[89]Nobile-Orazio E, Cappellari A, Priori A. Multifocal motor neuropathy: current concepts and controversies. Muscle Nerve. 2005 Jun;31(6):663-80.http://www.ncbi.nlm.nih.gov/pubmed/15770650?tool=bestpractice.com

电诊法检查提示多灶性传导阻滞为主要的脱髓鞘特性。[89]Nobile-Orazio E, Cappellari A, Priori A. Multifocal motor neuropathy: current concepts and controversies. Muscle Nerve. 2005 Jun;31(6):663-80.http://www.ncbi.nlm.nih.gov/pubmed/15770650?tool=bestpractice.com 感觉神经传导检查正常。

在50%~80% 的患者中发现抗 GM1 抗体。

未对皮质类固醇或血浆置换反应。

与多发性骨髓瘤、POEMS（多神经病、器官巨大症、内分泌病、单克隆丙种球蛋白病和皮肤损害）综合征、骨硬化性骨髓瘤、Waldenstrom 高丙种球蛋白血症和Castleman病相关的神经病变往往以远端为主。[90]Dispenzieri A, Suarez GA, Kyke RA. POEMS syndrome (osteosclerotic myeloma). In: Dyck PJ, Thomas PK, eds. Peripheral neuropathy. Philadelphia, PA: Elsevier Saunders; 2005:2453-69.[91]Kyle RA, Dyck PJ. Neuropathy associated with the monoclonal gammopathies. In: Dyck PJ, Thomas PK, eds. Peripheral neuropathy. Philadelphia, PA: Elsevier Sanders; 2005:2255-76. 与冷球蛋白血症和淋巴瘤相关的神经病变均可能为多灶性。[92]Bosch EP, Habermann TM, Tefferi A. Peripheral neuropathy with lymphoma, leukemia, and myeloproliferative disorders. In: Dyck PJ, Thomas PK, eds. Peripheral neuropathy. Philadelphia, PA: Elsevier Saunders; 2005:2489-503.

血清和尿液免疫固定电泳、定量免疫球蛋白水平、骨骼检查和骨髓活检通常会发现血液系统异常。[88]Kyle RA, Therneau TM, Rajkumar SV, et al. Long-term follow-up of 241 patients with monoclonal gammopathy of undetermined significance: the original Mayo Clinic series 25 years later. Mayo Clin Proc. 2004 Jul;79(7):859-66.http://www.ncbi.nlm.nih.gov/pubmed/15244381?tool=bestpractice.com

对于淋巴瘤，可能需要进行神经活检。[92]Bosch EP, Habermann TM, Tefferi A. Peripheral neuropathy with lymphoma, leukemia, and myeloproliferative disorders. In: Dyck PJ, Thomas PK, eds. Peripheral neuropathy. Philadelphia, PA: Elsevier Saunders; 2005:2489-503.

尽管轴突变性可能占主导，电诊法检查可能具有轴突变性和脱髓鞘特征。[90]Dispenzieri A, Suarez GA, Kyke RA. POEMS syndrome (osteosclerotic myeloma). In: Dyck PJ, Thomas PK, eds. Peripheral neuropathy. Philadelphia, PA: Elsevier Saunders; 2005:2453-69.[91]Kyle RA, Dyck PJ. Neuropathy associated with the monoclonal gammopathies. In: Dyck PJ, Thomas PK, eds. Peripheral neuropathy. Philadelphia, PA: Elsevier Sanders; 2005:2255-76.[92]Bosch EP, Habermann TM, Tefferi A. Peripheral neuropathy with lymphoma, leukemia, and myeloproliferative disorders. In: Dyck PJ, Thomas PK, eds. Peripheral neuropathy. Philadelphia, PA: Elsevier Saunders; 2005:2489-503.

POEMS 与下列情况相关：下肢复合肌肉动作电位和感觉神经动作电位波幅降低更严重、时间弥散和传导阻滞更少、远端运动延迟更少、正中神经末端潜伏期指数更高、呈长度依赖模式的活动性去神经支配的更多证据。[93]Cui RT, Huang XS, Liu JX, et al. Electrophysiological characteristics of polyneuropathy in POEMS syndrome: comparison with CIDP. J Clin Neurophysiol. 2012 Aug;29(4):345-8.http://www.ncbi.nlm.nih.gov/pubmed/22854769?tool=bestpractice.com[94]Nasu S, Misawa S, Sekiguchi Y, et al. Different neurological and physiological profiles in POEMS syndrome and chronic inflammatory demyelinating polyneuropathy. J Neurol Neurosurg Psychiatry. 2012 May;83(5):476-9.http://www.ncbi.nlm.nih.gov/pubmed/22338030?tool=bestpractice.com[95]Mauermann ML, Sorenson EJ, Dispenzieri A, et al. Uniform demyelination and more severe axonal loss distinguish POEMS syndrome from CIDP. J Neurol Neurosurg Psychiatry. 2012 May;83(5):480-6.http://www.ncbi.nlm.nih.gov/pubmed/22396441?tool=bestpractice.com

典型的神经病变为远端，且感觉性多于运动性。[96]Uncini A, De Angelis MV, Di Muzio A, et al. Chronic inflammatory demyelinating polyneuropathy in diabetics: motor conductions are important in the differential diagnosis with diabetic polyneuropathy. Clin Neurophysiol. 1999 Apr;110(4):705-11.http://www.ncbi.nlm.nih.gov/pubmed/10378742?tool=bestpractice.com

近端糖尿病性神经病通常是非对称性的。

无力进展缓慢。[96]Uncini A, De Angelis MV, Di Muzio A, et al. Chronic inflammatory demyelinating polyneuropathy in diabetics: motor conductions are important in the differential diagnosis with diabetic polyneuropathy. Clin Neurophysiol. 1999 Apr;110(4):705-11.http://www.ncbi.nlm.nih.gov/pubmed/10378742?tool=bestpractice.com[97]Stewart JD, McKelvey R, Durcan L, et al. Chronic inflammatory demyelinating polyneuropathy (CIDP) in diabetics. J Neurol Sci. 1996 Oct;142(1-2):59-64.http://www.ncbi.nlm.nih.gov/pubmed/8902721?tool=bestpractice.com

电诊法检查主要提示糖尿病中轴突消失，但是可能出现继发性脱髓鞘特征。[29]Gorson KC, Ropper AH, Adelman LS, et al. Influence of diabetes mellitus on chronic inflammatory demyelinating polyneuropathy. Muscle Nerve. 2000 Jan;23(1):37-43.http://www.ncbi.nlm.nih.gov/pubmed/10590404?tool=bestpractice.com[96]Uncini A, De Angelis MV, Di Muzio A, et al. Chronic inflammatory demyelinating polyneuropathy in diabetics: motor conductions are important in the differential diagnosis with diabetic polyneuropathy. Clin Neurophysiol. 1999 Apr;110(4):705-11.http://www.ncbi.nlm.nih.gov/pubmed/10378742?tool=bestpractice.com[97]Stewart JD, McKelvey R, Durcan L, et al. Chronic inflammatory demyelinating polyneuropathy (CIDP) in diabetics. J Neurol Sci. 1996 Oct;142(1-2):59-64.http://www.ncbi.nlm.nih.gov/pubmed/8902721?tool=bestpractice.com[98]Haq RU, Pendlebury WW, Fries TJ, et al. Chronic inflammatory demyelinating polyradiculoneuropathy in diabetic patients. Muscle Nerve. 2003 Apr;27(4):465-70.http://www.ncbi.nlm.nih.gov/pubmed/12661048?tool=bestpractice.com

可能类似于多灶性 CIDP（Lewis-Sumner 变体）。[17]Lewis RA, Sumner AJ, Brown MJ, et al. Multifocal demyelinating neuropathy with persistent conduction block. Neurology. 1982 Sep;32(9):958-64.http://www.ncbi.nlm.nih.gov/pubmed/7202168?tool=bestpractice.com

尽管早期病变可见假性传导阻滞，电诊法检查提示轴突丧失。[99]Gorson KC. Vasculitic neuropathies: an update. Neurologist. 2007 Jan;13(1):12-9.http://www.ncbi.nlm.nih.gov/pubmed/17215723?tool=bestpractice.com

再次行神经传导检查提示轴突丧失的典型特性。

可能需要进行神经活检以记录神经炎症证据。

寻找与开始使用药物相关的发病状况。

无力往往以远端为主。[100]London Z, Albers JW. Toxic neuropathies associated with pharmaceutic and industrial agents. Neurol Clin. 2007 Feb;25(1):257-76.http://www.ncbi.nlm.nih.gov/pubmed/17324727?tool=bestpractice.com[101]Herskovitz S, Schaumberg HH. Neuropathy caused by drugs. In: Dyck PJ, Thomas PK, eds. Peripheral neuropathy. Philadelphia, PA: Elsevier Saunders; 2005:2553-83.

神经活检可能提示神经周或施旺细胞溶酶体内含物伴有胺碘酮和哌克昔林。[100]London Z, Albers JW. Toxic neuropathies associated with pharmaceutic and industrial agents. Neurol Clin. 2007 Feb;25(1):257-76.http://www.ncbi.nlm.nih.gov/pubmed/17324727?tool=bestpractice.com[101]Herskovitz S, Schaumberg HH. Neuropathy caused by drugs. In: Dyck PJ, Thomas PK, eds. Peripheral neuropathy. Philadelphia, PA: Elsevier Saunders; 2005:2553-83.

神经病变以远端为主。

寻找相关特性：胃肠道神经病、眼肌麻痹、恶病质、听力受损或者脑白质病变。[102]Bedlack RS, Vu T, Hammans S, et al. MNGIE neuropathy: five cases mimicking chronic inflammatory demyelinating polyneuropathy. Muscle Nerve. 2004 Mar;29(3):364-8.http://www.ncbi.nlm.nih.gov/pubmed/14981734?tool=bestpractice.com

肌肉活检提示多个线粒体缺失而致的线粒体病理学（细胞色素氧化酶阴性纤维和粗糙的红色纤维）。

实验室检测提示血清乳酸中毒和血清胸苷水平显著升高。[102]Bedlack RS, Vu T, Hammans S, et al. MNGIE neuropathy: five cases mimicking chronic inflammatory demyelinating polyneuropathy. Muscle Nerve. 2004 Mar;29(3):364-8.http://www.ncbi.nlm.nih.gov/pubmed/14981734?tool=bestpractice.com

对于异染性脑白质营养不良症和 Krabbe 脑白质营养不良，主要特征可能是多神经病（易与 CIDP 混淆）。[103]Thomas PK, Goebel HH. Lysosomal and peroxisomal disorders. In: Dyck PJ, Thomas PK, eds. Peripheral neuropathy. Philadelphia, PA: Elsevier Saunders; 2005:1845-82.

在肾上腺脑白质营养不良中，以中枢神经系统症状和体征为主。

对于各种脑白质营养不良，电诊法结果更为一致。[103]Thomas PK, Goebel HH. Lysosomal and peroxisomal disorders. In: Dyck PJ, Thomas PK, eds. Peripheral neuropathy. Philadelphia, PA: Elsevier Saunders; 2005:1845-82.

以头部 MRI 结果为主，神经活检通常提示各种脑白质营养不良中的施旺细胞内含物。

探寻感染（如 HIV、肝炎、Lyme 病）的症状和体征。

HIV 检查、肝炎血清学或者 Lyme 血清学可能呈阳性。

脑脊液细胞增多，超过 10 个细胞/mm^3。[30]Cornblath DR, McArthur JC, Kennedy PG, et al. Inflammatory demyelinating peripheral neuropathies associated with human T-cell lymphotropic virus type III infection. Ann Neurol. 1987 Jan;21(1):32-40.http://www.ncbi.nlm.nih.gov/pubmed/3030188?tool=bestpractice.com[31]Simpson DM, Olney RK. Peripheral neuropathies associated with human immunodeficiency virus infection. Neurol Clin. 1992 Aug;10(3):685-711.http://www.ncbi.nlm.nih.gov/pubmed/1323749?tool=bestpractice.com

10%-25% 的患者患有多发性单神经病或者远端对称性多神经病。[26]Hahn AF, Hartung HP, Dyck PJ. Chronic inflammatory demyelinating polyradiculoneuropathy. In: Dyck PJ, Thomas PK, eds. Peripheral neuropathy. 4th ed. Philadelphia, PA: Elsevier Saunders; 2005:2221-53.[27]Kissel JT, Mendell JR. Chronic inflammatory demyelinating polyradiculoneuropathy. In: Mendell JR, Kissel JT, Cornblath DR, eds. Diagnosis and management of peripheral nerve disorders. New York, NY: Oxford University Press; 2001:173-91.[28]Reid VA, Black KR, Menkes DL, et al. Chronic inflammatory demyelinating polyradiculoneuropathy: Diagnosis and management. In: Cros D, ed. Peripheral neuropathy. A practical approach to diagnosis and management. Philadelphia, PA: Lippincott Williams & Wilkins; 2001:85-107.

抗核抗体、抗双链 DNA 抗体和抗史密斯 (Sm) 抗体检测呈阳性。

补体水平可能较低。

神经活检可能提示血管炎证据 。[26]Hahn AF, Hartung HP, Dyck PJ. Chronic inflammatory demyelinating polyradiculoneuropathy. In: Dyck PJ, Thomas PK, eds. Peripheral neuropathy. 4th ed. Philadelphia, PA: Elsevier Saunders; 2005:2221-53.[27]Kissel JT, Mendell JR. Chronic inflammatory demyelinating polyradiculoneuropathy. In: Mendell JR, Kissel JT, Cornblath DR, eds. Diagnosis and management of peripheral nerve disorders. New York, NY: Oxford University Press; 2001:173-91.[28]Reid VA, Black KR, Menkes DL, et al. Chronic inflammatory demyelinating polyradiculoneuropathy: Diagnosis and management. In: Cros D, ed. Peripheral neuropathy. A practical approach to diagnosis and management. Philadelphia, PA: Lippincott Williams & Wilkins; 2001:85-107.

颅神经病最常见。

急性或亚急性多灶性感觉运动神经病罕见。[26]Hahn AF, Hartung HP, Dyck PJ. Chronic inflammatory demyelinating polyradiculoneuropathy. In: Dyck PJ, Thomas PK, eds. Peripheral neuropathy. 4th ed. Philadelphia, PA: Elsevier Saunders; 2005:2221-53.[27]Kissel JT, Mendell JR. Chronic inflammatory demyelinating polyradiculoneuropathy. In: Mendell JR, Kissel JT, Cornblath DR, eds. Diagnosis and management of peripheral nerve disorders. New York, NY: Oxford University Press; 2001:173-91.[28]Reid VA, Black KR, Menkes DL, et al. Chronic inflammatory demyelinating polyradiculoneuropathy: Diagnosis and management. In: Cros D, ed. Peripheral neuropathy. A practical approach to diagnosis and management. Philadelphia, PA: Lippincott Williams & Wilkins; 2001:85-107.

结节病肉芽肿在神经活检中罕有发现。

可能出现神经内膜或神经外膜炎症性浸润。[26]Hahn AF, Hartung HP, Dyck PJ. Chronic inflammatory demyelinating polyradiculoneuropathy. In: Dyck PJ, Thomas PK, eds. Peripheral neuropathy. 4th ed. Philadelphia, PA: Elsevier Saunders; 2005:2221-53.[27]Kissel JT, Mendell JR. Chronic inflammatory demyelinating polyradiculoneuropathy. In: Mendell JR, Kissel JT, Cornblath DR, eds. Diagnosis and management of peripheral nerve disorders. New York, NY: Oxford University Press; 2001:173-91.[28]Reid VA, Black KR, Menkes DL, et al. Chronic inflammatory demyelinating polyradiculoneuropathy: Diagnosis and management. In: Cros D, ed. Peripheral neuropathy. A practical approach to diagnosis and management. Philadelphia, PA: Lippincott Williams & Wilkins; 2001:85-107.

神经病变伴有甲状腺功能减退或甲状腺功能亢进常以远端和感觉为主，通常可随甲状腺功能的纠正而改善。[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[40]Van den Bergh PY, Hadden RD, Bouche P, et al. European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society - first revision. Eur J Neurol. 2010 Mar;17(3):356-63.http://www.efns.org/fileadmin/user_upload/guidline_papers/EFNS_guideline_2010_inflammatory_demyelinating_polyradiculoneuropathy.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/20456730?tool=bestpractice.com[42]Cornblath DR, Asbury AK, Albers JW, et al. Research criteria for diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP): Report from the Ad Hoc Subcommittee of the AAN AIDS Task Force. Neurology. 1991 May;41(5):617-8.http://www.ncbi.nlm.nih.gov/pubmed/2027473?tool=bestpractice.com

甲状腺功能检查。[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[40]Van den Bergh PY, Hadden RD, Bouche P, et al. European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society - first revision. Eur J Neurol. 2010 Mar;17(3):356-63.http://www.efns.org/fileadmin/user_upload/guidline_papers/EFNS_guideline_2010_inflammatory_demyelinating_polyradiculoneuropathy.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/20456730?tool=bestpractice.com[42]Cornblath DR, Asbury AK, Albers JW, et al. Research criteria for diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP): Report from the Ad Hoc Subcommittee of the AAN AIDS Task Force. Neurology. 1991 May;41(5):617-8.http://www.ncbi.nlm.nih.gov/pubmed/2027473?tool=bestpractice.com

通常，临床表现包括远端轴突感觉神经病、小纤维神经病、腕管综合征或者自主神经病变。 已有多灶性神经病变伴脱髓鞘特征的罕见临床表现报告。[104]Gutierrez A, Turkewitz LJ, Correa H, et al. Primary systemic amyloidosis presenting with demyelinating features. Neurol Neurophysiol Neurosci. 2006 Dec 15:6.http://www.ncbi.nlm.nih.gov/pubmed/17260083?tool=bestpractice.com[105]Briemberg HR, Amato AA. Transthyretin amyloidosis presenting with multifocal demyelinating mononeuropathies. Muscle Nerve. 2004 Feb;29(2):318-22.http://www.ncbi.nlm.nih.gov/pubmed/14755500?tool=bestpractice.com

神经活检出现淀粉样蛋白。[106]Kyle RA, Kelly JJ, Dyck PJ. Amyloidosis and neuropathy. In: Dyck PJ, Thomas PK, eds. Peripheral neuropathy. Philadelphia, PA: Elsevier Saunders; 2005:2427-51.

尽管轴突变性可能占主导，电诊法检查可能具有轴突变性和脱髓鞘特征。[104]Gutierrez A, Turkewitz LJ, Correa H, et al. Primary systemic amyloidosis presenting with demyelinating features. Neurol Neurophysiol Neurosci. 2006 Dec 15:6.http://www.ncbi.nlm.nih.gov/pubmed/17260083?tool=bestpractice.com[105]Briemberg HR, Amato AA. Transthyretin amyloidosis presenting with multifocal demyelinating mononeuropathies. Muscle Nerve. 2004 Feb;29(2):318-22.http://www.ncbi.nlm.nih.gov/pubmed/14755500?tool=bestpractice.com[106]Kyle RA, Kelly JJ, Dyck PJ. Amyloidosis and neuropathy. In: Dyck PJ, Thomas PK, eds. Peripheral neuropathy. Philadelphia, PA: Elsevier Saunders; 2005:2427-51.

进行转甲状腺素蛋白突变分析以确定家族性淀粉样变性病。[105]Briemberg HR, Amato AA. Transthyretin amyloidosis presenting with multifocal demyelinating mononeuropathies. Muscle Nerve. 2004 Feb;29(2):318-22.http://www.ncbi.nlm.nih.gov/pubmed/14755500?tool=bestpractice.com