Centre de Référence Maladies Neuromusculaires Rares, Hôpital Pitié-Salpêtrière and Université Paris VI, Paris, France.
Eur J Neurol. 2011 Jun;18(6):899-905. doi: 10.1111/j.1468-1331.2010.03312.x. Epub 2010 Dec 29.
Some patients within the spectrum of chronic inflammatory demyelinating polyradiculoneuropathies (CIDP) have distal acquired demyelinating symmetric (DADS) neuropathy, usually associated with anti-myelin-associated-glycoprotein (MAG) IgM monoclonal gammopathy. The aim of this retrospective study was to investigate patients with DADS neuropathy without anti-MAG antibodies, and study their response to immunotherapy.
Patients were selected on the basis of (i) 'Definite CIDP' according to the EFNS/PNS Guideline criteria, (ii) The presence of disproportionately prolonged motor latencies resulting in a terminal latency index (TLI) ≤ 0.25 in at least two motor nerves and (iii) The absence of anti-MAG antibodies on ELISA. Response to immunotherapy was defined as persistent improvement by at least one point on the INCAT disability score.
Data from 146 CIDP patients were analysed, and 10 patients were included. Six had clinically pure sensory neuropathy, and four had sensorimotor neuropathy. Ataxia was present in nine patients, generalized areflexia in seven and postural tremor in two. Five of the 10 patients had abnormal sensory potentials only in the upper limbs. An associated condition was found in nine patients: two chronic lymphocytic leukaemias, four IgG monoclonal gammopathies (one associated with non-Hodgkin's lymphoma) and two IgM monoclonal gammopathies of unknown significance. Patients were mostly improved with intravenous immunoglobulin (IVIg), corticosteroids, plasma exchanges, or a combination thereof.
DADS neuropathy without anti-MAG antibodies is more likely to be considered a variant of CIDP. In addition, such patients should be systematically investigated for an associated haematological or immunological condition.
在慢性炎症性脱髓鞘性多发性神经病（CIDP）谱内的一些患者具有远端获得性脱髓鞘对称（DADS）神经病，通常与抗髓鞘相关糖蛋白（MAG）IgM 单克隆丙种球蛋白有关。本回顾性研究的目的是研究无抗 MAG 抗体的 DADS 神经病患者，并研究其对免疫治疗的反应。
根据 EFNS/PNS 指南标准，基于以下标准选择患者：（i）“明确的 CIDP”；（ii）存在不成比例地延长的运动潜伏期，导致终末潜伏期指数（TLI）≤0.25，至少有两个运动神经；（iii）ELISA 检测无抗 MAG 抗体。免疫治疗的反应定义为 INCAT 残疾评分至少提高 1 分。
分析了 146 例 CIDP 患者的数据，其中纳入了 10 例患者。6 例为临床纯感觉神经病，4 例为感觉运动神经病。9 例患者存在共济失调，7 例存在全身反射消失，2 例存在姿势性震颤。10 例患者中有 5 例仅上肢感觉电位异常。9 例患者存在相关疾病：2 例慢性淋巴细胞白血病，4 例 IgG 单克隆丙种球蛋白（1 例与非霍奇金淋巴瘤相关）和 2 例意义不明的 IgM 单克隆丙种球蛋白。患者大多通过静脉注射免疫球蛋白（IVIg）、皮质类固醇、血浆置换或联合治疗得到改善。
无抗 MAG 抗体的 DADS 神经病更可能被认为是 CIDP 的一种变体。此外，此类患者应系统地检查是否存在相关的血液学或免疫学疾病。
