Motor chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare and poorly described subtype of CIDP. We aimed to study their clinical and electrophysiological characteristics and response to treatment. From a prospective database of CIDP patients, we included patients with definite or probable CIDP with motor signs and without sensory signs/symptoms at diagnosis. Patients were considered to have pure motor CIDP (PM‐CIDP) if sensory conductions were normal or to have motor predominant CIDP (MPred‐CIDP) if ≥2 sensory nerve action potential amplitudes were abnormal. Among the 700 patients with CIDP, 17 (2%) were included (PM‐CIDP n = 7, MPred‐CIDP n = 10); 71% were male, median age at onset was 48 years (range: 13‐76 years), 47% had an associated inflammatory or infectious disease or neoplasia. At the more severe disease stage, 94% of patients had upper and lower limb weakness, with distal and proximal weakness in 4 limbs for 56% of them. Three‐quarters (75%) responded to intravenous immunoglobulins (IVIg) and four of five patients to corticosteroids including three of three patients with MPred‐CIDP. The most frequent conduction abnormalities were conduction blocks (CB, 82%) and F‐wave abnormalities (88%). During follow up, 4 of 10 MPred‐CIDP patients developed mild sensory symptoms; none with PM‐CIDP did so. Patients with PM‐CIDP had poorer outcome (median ONLS: 4; range: 22‐5) compared to MPred‐CIDP (2, range: 0‐4; P = .03) at last follow up. This study found a progressive clinical course in the majority of patients with motor CIDP as well as frequent associated diseases, CB, and F‐wave abnormalities. Corticosteroids might be considered as a therapeutic option in resistant IVIg patients with MPred‐CIDP.

中文翻译：

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运动性慢性炎性脱髓鞘性多发性神经病（CIDP）在17例患者中：临床特征，电生理研究和对治疗的反应。

运动性慢性炎性脱髓鞘性多发性神经病（CIDP）是一种罕见且描述不清的CIDP亚型。我们旨在研究其临床和电生理特征以及对治疗的反应。从CIDP患者的前瞻性数据库中，我们纳入了确诊或可能患有CIDP且具有运动体征且在诊断时没有感觉征兆/症状的患者。如果感觉传导正常，则认为患者具有纯运动CIDP（PM-CIDP），如果≥2个感觉神经动作电位幅度异常，则认为患者具有运动占优势的CIDP（MPred-CIDP）。在700名CIDP患者中，包括17名（2％）（PM-CIDP n = 7，MPred-CIDP n = 10）；男性为71％，发病年龄中位数为48岁（范围：13-76岁），伴发炎性或感染性疾病或瘤形成的比例为47％。在更严重的疾病阶段 94％的患者患有上肢和下肢无力，其中4％的肢体远端和近端无力，其中56％。四分之三（75％）的患者对静脉注射免疫球蛋白（IVIg）有反应，五分之四的患者对皮质类固醇有反应，其中三分之三的MPred-CIDP患者。最常见的传导异常是传导阻滞（CB，82％）和F波异常（88％）。在随访期间，每10名MPred-CIDP患者中有4名出现轻度的感觉症状。PM-CIDP都没有这样做。与MPred-CIDP（2，范围：0-4； PM-CIDP）相比，PM-CIDP患者的预后较差（ONLS中位数：4；范围：22-5）。四分之三（75％）的患者对静脉注射免疫球蛋白（IVIg）有反应，五分之四的患者对皮质类固醇有反应，其中三分之三的MPred-CIDP患者。最常见的传导异常是传导阻滞（CB，82％）和F波异常（88％）。在随访期间，每10名MPred-CIDP患者中有4名出现轻度的感觉症状。PM-CIDP都没有这样做。与MPred-CIDP（2，范围：0-4； PM-CIDP）相比，PM-CIDP患者的预后较差（ONLS中位数：4；范围：22-5）。四分之三（75％）的患者对静脉注射免疫球蛋白（IVIg）有反应，五分之四的患者对皮质类固醇有反应，其中三分之三的MPred-CIDP患者。最常见的传导异常是传导阻滞（CB，82％）和F波异常（88％）。在随访期间，每10名MPred-CIDP患者中有4名出现轻度的感觉症状。PM-CIDP都没有这样做。与MPred-CIDP（2，范围：0-4； PM-CIDP）相比，PM-CIDP患者的预后较差（ONLS中位数：4；范围：22-5）。P = .03）。这项研究发现了大多数运动CIDP患者以及常见的相关疾病，CB和F波异常患者的临床病程进展。皮质类固醇可能被认为是MPred-CIDP耐药的IVIg患者的治疗选择。