Distribution patterns of demyelination correlate with clinical profiles in chronic inflammatory demyelinating polyneuropathy
Distribution patterns of demyelination correlate with clinical profiles in chronic inflammatory demyelinating polyneuropathy
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DOI：
10.1136/jnnp.72.1.37
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发表时间：
2002-01-01
影响因子：
11
通讯作者：
Hattori, T
中科院分区：
文献类型：
--
作者：
Kuwabara, S;Ogawara, K;Hattori, T
背景资料：慢性炎性脱髓鞘性多发性神经病（CIDP）是一种异质性疾病，具有广泛的临床范围，其特征是多灶性脱髓鞘，可累及远端神经末梢、中间神经节段和神经根。目的：探讨是否脱髓鞘沿着的过程中的神经的分布模式与CIDP.Methods患者的临床资料：运动神经传导进行了研究，对42例连续患者。根据脱髓鞘的生理学标准，在远端神经节段（远端模式）或中间神经节段（中间模式）或两者（弥漫模式）中确定脱髓鞘病变的存在。结果：患者分为远端型（n= 10）、中间型（n= 13）和弥漫型（n= 15），未分类型（n= 4）。远端型或弥漫型患者具有共同的临床特征，如亚急性发作、对称性症状、近端和远端肌肉无力。远端型患者对治疗有良好的反应，并有一个单相缓解过程，但弥漫型与治疗依赖性复发过程相关，反映了较长的疾病活动。血清TNF-α浓度仅在“弥漫性”患者亚组中升高，这可能与血神经屏障的破坏有关，因此，涉及中间节段。中间模式的特点是一个慢性过程中，不对称的症状，不太严重的残疾，和refractoriness的treatment.Conclusions：COP包括亚型病变的不同偏好沿着的过程中的神经。传导异常的分布模式可能有助于预测CIDP患者的预后。
Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a heterogeneous disorder having a wide clinical range, and is characterised by multifocal demyelination that can involve the distal nerve terminals, intermediate nerve segments, and nerve roots. Objective: To investigate whether the distribution patterns of demyelination along the course of the nerve correlate with clinical profiles in patients with CIDP.Methods: Motor nerve conduction studies were carried out on 42 consecutive patients. According to the physiological criteria for demyelination, the presence of a demyelinative lesion was determined in the distal nerve segments (distal pattern) or intermediate nerve segments (intermediate pattern), or in both (diffuse pattern). The serum concentration of tumour necrosis factor (TNF)-alpha was measured by immunoassay.Results: Patients were classified as having a distal (n= 10), intermediate (n= 13), or diffuse (n= 15) pattern, or were unclassified (n= 4). Patients with the distal or diffuse pattern had common clinical features such as subacute onset, symmetric symptoms, and weakness involving proximal as well as distal muscles. Patients with the distal pattern had a good response to treatment and a monophasic remitting course, but the diffuse pattern was associated with a treatment dependent relapsing course, reflecting longer disease activity. The serum TNF-alpha concentrations increased only in the "diffuse" subgroup of patients, and this might be associated with breakdown of the blood-nerve barrier and therefore, involvement of the intermediate segments. The intermediate pattern was characterised by a chronic course, asymmetric symptoms, less severe disability, and refractoriness to treatments.Conclusions: COP consists of subtypes with varying predilections for lesions along the course of the nerve. The distribution patterns of conduction abnormalities may be useful in the prediction of outcome of patients with CIDP.