Neurology, Sunderland Royal Hospital, Sunderland, UK
Neurology, Sunderland Royal Hospital, Sunderland, UK.
Pract Neurol. 2022 Feb;22(1):57-59. doi: 10.1136/practneurol-2021-003070. Epub 2021 Nov 5.
Chronic immune sensory polyradiculopathy (CISP) is a rare variant of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We describe a man with isolated sensory ataxia whose initial investigations included normal nerve conduction studies and normal non-enhanced MR imaging of whole spine, but whose subsequent investigations showed delayed somatosensory evoked potential (SSEP) responses of the lower limbs, elevated cerebrospinal fluid (CSF) protein and lumbosacral nerve roots enhancement on MR imaging. We diagnosed CISP and he improved following intravenous immunoglobulin. Proposed diagnostic criteria for CISP are sensory symptoms with a polyneuropathic distribution without weakness, and normal motor and sensory nerve conduction and electromyography (EMG) studies, plus at least two of: abnormal SSEPs not due to central nervous system (CNS) involvement, MRI showing gadolinium enhancement or hypertrophy of the nerve roots, cauda equina or plexuses, and elevated CSF protein with normal cell count. Intravenous immunoglobulin is an effective treatment.
慢性免疫感觉性多发性神经病（CISP）是慢性炎症性脱髓鞘性多发性神经病（CIDP）的一种罕见变异型。我们描述了一位以孤立性感觉性共济失调为表现的男性患者，其初始检查包括正常的神经传导研究和整个脊柱的正常非增强性磁共振成像，但随后的检查显示下肢体感诱发电位（SSEP）反应延迟、脑脊液（CSF）蛋白升高和腰骶神经根磁共振成像增强。我们诊断为 CISP，他在接受静脉注射免疫球蛋白后有所改善。CISP 的拟议诊断标准为无无力的多发性神经病分布的感觉症状，以及正常的运动和感觉神经传导和肌电图（EMG）研究，加上以下至少两项：由于中枢神经系统（CNS）受累而不是由于中枢神经系统（CNS）受累而导致的异常 SSEP、MRI 显示神经根、马尾或丛的钆增强或肥大，以及脑脊液蛋白升高但细胞计数正常。静脉注射免疫球蛋白是一种有效的治疗方法。
