Chronic dysimmune neuropathy
Chronic dysimmune neuropathy
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慢性免疫失调性神经病
作者：
M. Busby;M. Donaghy
摘要。慢性免疫功能障碍神经病（CDN）是一种临床异质性的多神经病变，由其推定的免疫介导的病因联合起来。目前，这类神经病被分类为慢性炎症性脱髓鞘性多神经病变（CIDP）、多灶性运动神经病（MMN）和与血清副蛋白相关的神经病（副蛋白血症神经病）。这种分类不能识别其他独特的综合征，并且受到亚组内部异质性和亚组之间重叠的限制。我们通过对102名未选择的CDN患者的连续系列回顾来完善这种临床亚分类，这些患者转诊给一位神经科医生。我们将CDN分为6种临床亚型：一种感觉共济失调组；三个运动感觉亚组（慢性运动感觉脱髓鞘神经病，亚急性运动感觉脱髓鞘神经病和多灶性运动感觉神经病）；两个纯运动亚组（对称纯运动脱髓鞘神经病和多灶性运动神经病）。这种亚分类允许识别不同的综合征，并有助于解决异质性和重叠的问题。这些亚组之间的区别对患者管理具有直接的实际意义。虽然类固醇对大多数亚组都有益，但对于纯运动综合征来说并非如此，这两种症状都应该用静脉注射免疫球蛋白治疗。慢性发展的运动感觉脱髓鞘神经病变患者对类固醇的反应不如亚急性发作的患者好。发现老年亚急性运动感觉脱髓鞘神经病变与癌症之间存在关联。在任何临床亚组中，无论是否存在相关的副蛋白或神经特异性抗体，患者的行为都相似。
Abstract. The Chronic Dysimmune neuropathies (CDN) are a clinically heterogeneous group of polyneuropathies united by their presumed immune mediated aetiology. At present such neuropathies are classified as Chronic Inflammatory Demyelinating Polyneuropathy (CIDP), Multifocal Motor Neuropathy (MMN) and the Neuropathies in association with serum Paraproteins (Paraproteinaemic Neuropathies). This classification fails to recognise other distinctive syndromes and is limited by heterogeneity within, and overlap between, subgroups. We have refined this clinical subclassification by a review of a consecutive series of 102 unselected patients with CDN referred to a single neurologist. We recognise 6 clinical subtypes of CDN: one sensory ataxic group; three motor-sensory subgroups (chronic motor sensory demyelinating neuropathy, subacute motor sensory demyelinating neuropathy and a multifocal motor sensory neuropathy); and two pure motor subgroups (symmetric pure motor demyelinating neuropathy and multifocal motor neuropathy). This subclassification allows distinct syndromes to be recognised and helps resolve problems of heterogeneity and overlap. Distinction between these subgroups is of immediate practical relevance to patient management. Although steroids are beneficial for most of the subgroups, this is not so for both of the pure motor syndromes which should be treated with intravenous immunoglobulin. Patients with chronic development of Motor Sensory Demyelinating Neuropathy respond less well to steroids than those with a subacute onset. An association was found between elderly patients with Subacute Motor Sensory Demyelinating Neuropathy and carcinomas. Within any clinical subgroup patients behave similarly regardless of the presence of associated paraproteins or nerve specific antibodies.