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慢性多发性神经根神经病的一种非典型儿科表现

An Atypical Pediatric Presentation of a Chronic Polyradiculoneuropathy.

作者信息

Speer Wes, Szewczyk Christopher, Jacobson Ryan

机构信息

Psychiatry, Rush Medical College, Chicago, USA.

Neurology, Rush Medical College, Chicago, USA.

出版信息

Cureus. 2023 Aug 30;15(8):e44361. doi: 10.7759/cureus.44361. eCollection 2023 Aug.

Abstract

Here, we present a case of a 15-year-old male with polyradiculoneuropathy, which was diagnosed as chronic inflammatory demyelinating polyneuropathy (CIDP), who was refractory to initial treatment. The patient presented with a one-and-a-half-month history of decreased strength, most notable in the bilateral hip flexors and finger flexors/extensors, and areflexia. Electromyography and nerve conduction studies did not fulfill diagnostic criteria for a demyelinating polyneuropathy; however, the cerebrospinal fluid analysis demonstrated albuminocytologic dissociation and his physical exam was otherwise consistent with the diagnosis. He was treated with IV immunoglobulin (IVIg). He relapsed less than one month later with worsening weakness. Imaging revealed increased cauda equina enhancement when compared to the MRI from the previous admission, and labs were otherwise similar to the initial presentation. He was treated with a second course of IVIg in addition to high-dose IV methylprednisolone. Upon his second discharge, he was transitioned to oral corticosteroids, and at a follow-up visit one month later, he had fully regained his strength and demonstrated normal reflexes. This case highlights the variable nature of CIDP in its initial presentation, its course, and its response to treatment, particularly in young patients. Additionally, we would like to emphasize that this case of CIDP was in the context of chronic malnutrition and significant weight loss, which made the diagnostic picture more complex.

摘要

在此,我们报告一例15岁男性的多神经根神经病病例,该病例被诊断为慢性炎症性脱髓鞘性多发性神经病(CIDP),对初始治疗无效。患者有一个半月的肌力下降病史,最明显的是双侧髋屈肌和手指屈肌/伸肌,且腱反射消失。肌电图和神经传导研究未达到脱髓鞘性多发性神经病的诊断标准;然而,脑脊液分析显示蛋白细胞分离,且其体格检查在其他方面与诊断相符。他接受了静脉注射免疫球蛋白(IVIg)治疗。不到一个月后他复发,肌无力加重。影像学检查显示与上次入院时的MRI相比,马尾增强增加,实验室检查在其他方面与初始表现相似。除了大剂量静脉注射甲基强的松龙外,他还接受了第二疗程的IVIg治疗。第二次出院时,他改用口服皮质类固醇,在一个月后的随访中,他已完全恢复肌力,腱反射正常。该病例突出了CIDP在初始表现、病程及对治疗反应方面的多变性,尤其是在年轻患者中。此外,我们想强调的是,该例CIDP是在慢性营养不良和显著体重减轻的背景下发生的,这使得诊断情况更加复杂。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c6f6/10540090/fb8a1d0d7161/cureus-0015-00000044361-i01.jpg

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