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神内二病例讨论

神内二病例讨论. Immune mediated neuropathy CIDP MGUS. 病例特点. 老年男性，慢性病程，反复缓解复发； 长期慢性腹泻病史； 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； 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. 病例特点. 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

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神内二病例讨论

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  1. 神内二病例讨论 Immune mediated neuropathy CIDP MGUS

  2. 病例特点 • 老年男性，慢性病程，反复缓解复发； • 长期慢性腹泻病史； • 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； • 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

  3. 病例特点 • 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 • 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。

  4. 病例特点 • 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(-)。 • 血GM1-IgM 1：200（参考值≤800），GM1-IgG 1：50（参考值≤200）。 • 尿本－周蛋白阴性

  5. 病例特点 • 血免疫全项均未见异常； • 颈MRI：C5－6间盘后突，后缘骨刺压迫脊髓，髓内可见长T2信号

  6. 定位诊断 • 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。

  7. 定位诊断 • 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。

  8. 定性诊断 • 免疫介导的周围神经病 • IgM monoclonal gammopathy of undetermined significance • Chronic inflammatory demyelinating polyneuropathy • Paraneoplastic neuropathies

  9. 未明意义的单克隆丙球蛋白病（IgM monoclonal gammopathy of undetermined significance〕 • 发病时间大于2年，慢性迁延病程； • 远端对称性感觉运动神经病； • 肌电图脱髓鞘改变与运动神经元受累表现； • 脑脊液 GM1-IgM (+),GM1-IgG(-) • 激素和环磷酰胺治疗有效。 • 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

  10. Proposal for criteria for demyelinating polyneuropathy associated with MGUS • 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 years

  11. Proposal for criteria for demyelinating polyneuropathy associated with MGUS The relation is definite when the following is present: • (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: • (1)Time to peak of the neuropathy > 2 years • (2)Chronic slowly progressive course without relapsing or remitting periods • (3)Symmetrical distal polyneuropathy • (4)Sensory symptoms and signs predominate over motor features

  12. Proposal for criteria for demyelinating polyneuropathy associated with MGUS • A causal relation is unlikely when at least three of the following are present in a patient without anti-MAG antibodies: • (1)Median time to peak of the neuropathy is within 1 year • (2)Clinical course is relapsing and remitting or monophasic • (3)Cranial nerves are involved • (4)Neuropathy is asymmetrical • (5)Motor symptoms and signs predominate • (6)History of preceding infection • Presence of abnormal median SNAP in combination with normal sural SNAP.

  13. CIDP诊断标准(Barohn,1989年) 必须标准 • 进行性肌无力(缓慢进展,阶梯性或复发)2个月； • 对称性上肢或下肢的近端和远端肌无力； • 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。

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