Department of Neurology, University Hospitals Cleveland Medical Center, Case Western Reserve University School of Medicine, Cleveland, Ohio, 44106, USA.
Department of Neurology, Cleveland Clinic Foundation, Cleveland, Ohio, USA.
Muscle Nerve. 2019 Jun;59(6):658-664. doi: 10.1002/mus.26436. Epub 2019 Feb 25.
Chronic immune sensorimotor polyradiculopathy (CISMP) is a chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) variant affecting both sensory and motor nerve roots without evidence of peripheral nerve demyelination.
We report a case series of 9 patients with CISMP, identified from 2 tertiary centers. Clinical, electrodiagnostic, and neuroradiologic features, and treatment responses, were retrospectively reviewed.
Patients presented with sensorimotor deficits and hypo-/areflexia, predominantly involving lower extremities. Three had cranial nerve involvement. Electrodiagnostic findings in all patients localized to roots proximal to dorsal root ganglia, without evidence of peripheral nerve demyelination. Cerebrospinal fluid examination revealed an albuminocytologic association. Eight patients exhibited gadolinium enhancement and thickening of multiple spinal nerve roots and/or cranial nerves. All patients demonstrated good responses to immunotherapies.
CISMP is similar to CIDP in many aspects, but lacks typical electrodiagnostic findings of peripheral nerve demyelination. It is important to recognize this unusual and treatable entity. Muscle Nerve 59:658-664, 2019.
慢性免疫感觉运动性多神经根病（CISMP）是一种影响感觉和运动神经根的慢性炎症性脱髓鞘多神经根神经病（CIDP）变体，没有周围神经脱髓鞘的证据。
我们报告了 9 例 CISMP 患者的病例系列，这些患者是从 2 个三级中心中确定的。回顾性分析了临床、电诊断和神经影像学特征以及治疗反应。
患者出现感觉运动功能障碍和低反射/无反射，主要累及下肢。3 例有颅神经受累。所有患者的电诊断结果均定位于背根神经节近端的神经根，没有周围神经脱髓鞘的证据。脑脊液检查显示白蛋白细胞相关。8 例患者表现为多个脊神经根和/或颅神经的钆增强和增厚。所有患者对免疫治疗均有良好反应。
CISMP 在许多方面与 CIDP 相似，但缺乏周围神经脱髓鞘的典型电诊断发现。认识到这种不常见且可治疗的实体很重要。肌肉神经 59:658-664,2019。
