一例与抗CV2/CRMP5抗体存在时间关联的严重慢性进行性轴索性多神经根神经病病例。
A case of severe chronic progressive axonal polyradiculoneuropathy temporally associated with anti-CV2/CRMP5 antibodies.
作者信息
Hannawi Yousef, Goldsmith Corey E, Kass Joseph S, Olar Adriana, Ubogu Eroboghene E, Kalkonde Yogeshwar V
机构信息
Department of Neurology, Baylor College of Medicine, Houston, TX, USA.
出版信息
J Clin Neuromuscul Dis. 2013 Sep;15(1):13-8. doi: 10.1097/CND.0b013e3182a04538.
OBJECTIVES
The involvement of the peripheral nervous system by anti-CV2/CRMP5 paraneoplastic antibodies is typically encountered as a mixed sensorimotor polyneuropathy. We report a fatal case of severe chronic progressive axonal polyradiculoneuropathy in association with this antibody.
METHODS
Review of the patient's chart, nerve conduction/electromyographic studies, and nerve biopsy.
RESULTS
A 51-year-old man presented with a progressive quadriparesis over a 4-month period. Extensive evaluation for potential etiologies was significant only for positive anti-CV2/CRMP5 antibodies without detection of an underlying neoplasm. Despite multiple immunomodulatory therapies, the patient progressed and demonstrated electrodiagnostic evidence for a chronic axonal polyradiculoneuropathy with ongoing denervation. The patient eventually died of respiratory failure.
CONCLUSIONS
This case adds to the clinical spectrum of the peripheral nervous system involvement in patients with paraneoplastic anti-CV2/CRMP5 antibodies.
目的
抗CV2/CRMP5副肿瘤性抗体累及周围神经系统通常表现为混合性感觉运动性多发性神经病。我们报告1例与此抗体相关的严重慢性进行性轴索性多神经根神经病致死病例。
方法
查阅患者病历、进行神经传导/肌电图检查及神经活检。
结果
一名51岁男性在4个月内出现进行性四肢瘫。对潜在病因进行的全面评估仅发现抗CV2/CRMP5抗体呈阳性，未检测到潜在肿瘤。尽管接受了多种免疫调节治疗，患者病情仍进展，电诊断显示为慢性轴索性多神经根神经病且存在持续失神经支配。患者最终死于呼吸衰竭。
结论
该病例拓宽了副肿瘤性抗CV2/CRMP5抗体患者周围神经系统受累的临床谱。
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