Koh Jasmine Shimin, Tung James Wei Min, Tan-Yu Genevieve Lynn Yu, Umapathi Thirugnanam
Department of Neurology, National Neuroscience Institute, 11 Jalan Tan Tock Seng, Singapore 308433.
Lee Kong Chian School of Medicine, Nanyang Technological University, 59 Nanyang Dr., Experimental Medicine Building, Singapore 636921.
Case Rep Neurol Med. 2020 Feb 20;2020:6595086. doi: 10.1155/2020/6595086. eCollection 2020.
Chronic immune sensory polyradiculopathy (CISP) is an uncommon and treatable inflammatory disorder of the proximal sensory nerve roots. Patients typically present with severe sensory ataxia, normal motor examination, unsteady gait, and normal nerve conduction studies (NCS). We describe an elderly man who presented with a two-week history of progressive numbness of both legs and recurrent falls. He had hyporeflexia, normal strength, severe proprioceptive, and vibration sense loss in both lower limbs and was unable to stand or walk because of severe sensory ataxia. The NCS and MR scan of the spine were normal. Tibial somatosensory evoked potentials revealed proximal conduction defect and localized the pathology to the lumbar sensory nerve roots proximal to the dorsal root ganglion. Cerebrospinal fluid showed cytoalbuminergic dissociation suggestive of inflammation. CISP was diagnosed; he was given aggressive immunotherapy consisting sequentially of corticosteroids with mycophenolate mofetil and three cycles of intravenous immunoglobulin after which he regained independent mobility. Unlike previous reports where patients presented months-years after symptom onset and improved after single-line immunotherapy, our patient presented fairly acutely and made dramatic improvement only after aggressive combination therapy. We urge physicians to recognize this uncommon neurologic cause of sensory ataxia where early aggressive treatment is crucial for better functional outcomes.
慢性免疫性感觉性多神经根病(CISP)是一种罕见但可治疗的近端感觉神经根炎性疾病。患者通常表现为严重的感觉性共济失调、运动检查正常、步态不稳以及神经传导研究(NCS)正常。我们描述了一名老年男性,他有两周双下肢进行性麻木和反复跌倒的病史。他下肢反射减弱、肌力正常、严重本体感觉和振动觉丧失,由于严重的感觉性共济失调而无法站立或行走。脊柱的NCS和磁共振扫描均正常。胫神经体感诱发电位显示近端传导缺陷,并将病变定位至背根神经节近端的腰段感觉神经根。脑脊液显示细胞蛋白分离,提示存在炎症。诊断为CISP;给予积极的免疫治疗,依次使用皮质类固醇联合霉酚酸酯以及三个疗程的静脉注射免疫球蛋白,之后他恢复了独立活动能力。与之前报道的患者在症状出现数月至数年之后就诊且经单一免疫治疗后病情改善不同,我们的患者就诊时病情相当急,仅在积极的联合治疗后才取得显著改善。我们敦促医生认识到这种导致感觉性共济失调的罕见神经病因,早期积极治疗对于获得更好的功能结局至关重要。