Min Young Gi, Ahmed Irad, Englezou Christina, Rajabally Yusuf A
Department of Translational Medicine, Seoul National University College of Medicine, Seoul, Republic of Korea.
Inflammatory Neuropathy Clinic, Department of Neurology, University Hospitals Birmingham, Birmingham, UK.
Muscle Nerve. 2025 Jul;72(1):66-70. doi: 10.1002/mus.28419. Epub 2025 Apr 22.
INTRODUCTION/AIMS: Incomplete forms of typical chronic inflammatory demyelinating polyneuropathy (CIDP) have recently been described, but their frequency and clinical characteristics are uncertain. This study aimed to describe a cohort of patients with incomplete typical CIDP.
We retrospectively analyzed 64 consecutive treatment-naïve patients with CIDP. Phenotypes were classified based on detailed motor examinations, and clinical, electrophysiological, and therapeutic characteristics were compared.
Nineteen (30%) subjects with typical CIDP presented with an incomplete phenotype; 12 (63.2%) exhibited a proximal arm-sparing pattern, 3 (15.8%) a distal arm-sparing pattern, 3 (15.8%) a pure paraparetic form, and 1 (5.2%) had a pure proximal form. In cases without full motor recovery, 11 (68.8%) maintained their original phenotype, while the rest transitioned to the complete (18.8%) or to another incomplete form (12.5%) due to involvement of previously unaffected segments. Subjects with incomplete typical CIDP had milder pre-treatment disability and weakness compared to those with the complete form, while other clinical and electrodiagnostic features were comparable. As opposed to the complete form, disability in incomplete typical CIDP at diagnosis showed no correlation with muscle strength.
Incomplete forms were observed in nearly one-third of subjects with typical CIDP. Incomplete typical CIDP represents a milder form of complete typical CIDP; however, its other disease characteristics, including treatment response, are similar, highlighting the importance of its proper prompt recognition as CIDP. Impairments beyond motor weakness, such as more diffuse proprioceptive loss, might play a role in the disability of patients with incomplete typical forms of CIDP.
引言/目的：近期已描述了典型慢性炎性脱髓鞘性多发性神经病（CIDP）的不完全形式，但其发生率和临床特征尚不确定。本研究旨在描述一组不完全典型CIDP患者。
我们回顾性分析了64例未经治疗的连续CIDP患者。根据详细的运动检查对表型进行分类，并比较临床、电生理和治疗特征。
19例（30%）典型CIDP患者表现为不完全表型；12例（63.2%）表现为近端上肢未受累模式，3例（15.8%）为远端上肢未受累模式，3例（15.8%）为单纯截瘫形式，1例（5.2%）为单纯近端形式。在运动未完全恢复的病例中，11例（68.8%）维持其原始表型，其余患者由于先前未受累节段受累而转变为完全型（18.8%）或另一种不完全形式（12.5%）。与完全型患者相比，不完全典型CIDP患者治疗前的残疾和虚弱程度较轻，而其他临床和电诊断特征相当。与完全型不同，不完全典型CIDP诊断时的残疾与肌肉力量无关。
近三分之一的典型CIDP患者观察到不完全形式。不完全典型CIDP代表完全典型CIDP的一种较轻形式；然而，其包括治疗反应在内的其他疾病特征相似，突出了及时正确识别其为CIDP的重要性。除运动无力外的损伤，如更广泛的本体感觉丧失，可能在不完全典型形式的CIDP患者的残疾中起作用。
