免疫介导的周围神经病.ppt

神内二病例讨论Immune mediated neuropathy – CIDP – MGUS病例特点v老年男性，慢性病程，反复缓解复发； v长期慢性腹泻病史； v自1998年2月开始双上肢麻木无力，1999年出 现四肢麻木活动无力，症状反复迁延复发， 无明确完全缓解期，使用激素和环磷酰胺等 有效； v查体可见双上肢伸肌肌力Ⅴ-级，双下肢肌力 Ⅴ级四肢肌张力和腱反射低下，双手掌指 关节以下、双踝关节以下针刺觉、音叉震动 觉减退 v肌电图：右胫前肌、右拇短外展肌可见巨大 电位，运动和感觉神经传导速度减慢 v诱发电位：VEP左侧各波潜伏期延长，BAEP左 耳Ⅰ波未引出，SEP双侧P15至N20潜伏期延长 ，左侧C7、Erb’s点及右侧Erb’s点波形未 引出双下肢SEP掴窝未引出波形 病例特点v脑脊液检查：细胞数2600/mm3, WBC2/mm3，生化：Pro76mg/dl, C- Glu3.1mmol/L, C-Cl 124mmol/L；GM1- IgM (+),GM1-IgG(-) v血GM1-IgM 1：200（参考值≤800）， GM1-IgG 1：50（参考值≤200） v尿本－周蛋白阴性 病例特点v血免疫全项均未见异常； v颈MRI：C5－6间盘后突，后缘骨刺压迫 脊髓，髓内可见长T2信号 病例特点定位诊断Ø周围神经：四肢麻木，针刺觉减退，提 示感觉神经小纤维受累；四肢远端音叉 觉减退，提示感觉神经大纤维受累；电 生理检查出现感觉神经传导速度慢、传 导阻滞。

四肢以远端为重的运动功能下 降，腱反射低下，无病理征，肌电图运 动神经传导速度慢，提示运动神经纤维 受累Ø运动神经元或神经根：电生理检查右胫 前肌可见巨大电位，多相电位增多，右 拇短外展肌可见巨大电位定位诊断定性诊断Ø 免疫介导的周围神经病 üIgM monoclonal gammopathy of undetermined significance üChronic inflammatory demyelinating polyneuropathy üParaneoplastic neuropathies未明意义的单克隆丙球蛋白病 （IgM monoclonal gammopathy of undetermined significance〕q发病时间大于2年，慢性迁延病程； q远端对称性感觉运动神经病； q肌电图脱髓鞘改变与运动神经元受累表 现； q脑脊液 GM1-IgM (+),GM1-IgG(-) q激素和环磷酰胺治疗有效 q腓肠神经活检：脱髓鞘改变，部分轴索 变性，血管周围少量淋巴细胞q A causal relation between demyelinating polyneuropathy and MGUS should be considered in a patient with: • (1)Demyelinating polyneuropathy according to the electrodiagnostic ANN criteria for idiopathic CIDP • (2)Presence of an M protein (IgM,IgG,or IgA), without evidence of malignant plasma cell dyscrasias like multiple myeloma , lymphoma, Waldenstrom’s macroglobulinemia, or amyloidosis. • (3) Family history negative for neuropathy. • (4) Age 30 yearsProposal for criteria for demyelinating polyneuropathy associated with MGUS The relation is definite when the following is present: q (1) IgM M protein with anti-MAG antibodies The relation is probable when at least three of the following are present in a patient without anti-MAG antibodies: q (1) Time to peak of the neuropathy 2 years q (2) Chronic slowly progressive course without relapsing or remitting periods q (3) Symmetrical distal polyneuropathy q (4) Sensory symptoms and signs predominate over motor featuresProposal for criteria for demyelinating polyneuropathy associated with MGUS Proposal for criteria for demyelinating polyneuropathy associated with MGUS q A causal relation is unlikely when at least three of the following are present in a patient without anti-MAG antibodies: q (1) Median time to peak of the neuropathy is within 1 year q (2) Clinical course is relapsing and remitting or monophasic q (3) Cranial nerves are involved q (4) Neuropathy is asymmetrical q (5) Motor symptoms and signs predominate q (6) History of preceding infection q Presence of abnormal median SNAP in combination with normal sural SNAP. CIDP诊断标准(Barohn,1989年)必须标准 v 进行性肌无力(缓慢进展,阶梯性或复发)2个月； v 对称性上肢或下肢的近端和远端肌无力； v 腱反射降低或消失。

必须排除标准，病人必须无： (1)临床出现纯感觉神经病,色素视网膜炎,药物接触; (2)实验室:低血清胆固醇,卟啉病,低血清B12, CSF中白细胞50 (3)电生理:有神经肌肉传递缺陷，肌病或前角细胞疾病 (4)神经活检显示血管炎，神经纤维肿胀，Amyloidosis, 细胞 器病等。