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免疫介导的周围神经病.ppt
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    参见附件(37KB)。

    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

    * 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

    * 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

    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

    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。

    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。

    定性诊断

    >免疫介导的周围神经病

    * IgM monoclonal gammopathyof undetermined significance

    * Chronic inflammatory demyelinating polyneuropathy

    * Paraneoplastic neuropathies

    未明意义的单克隆丙球蛋白病

    (IgM monoclonal gammopathyof 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

    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

    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

    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.

    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。