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POEMS综合征伴急性脱髓鞘性多发性神经病:终末潜伏期指数增加及均匀性脱髓鞘

POEMS syndrome presenting with acute demyelinating polyneuropathy: increased terminal latency indices and uniform demyelination.

作者信息

Min Ju-Hong, Lee Dong Kyu, Lee Sujin, Kim Kihyun, Jeon Eun-Seok, Jang Hye Ryoun, Kim Byoung Joon

机构信息

Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Korea.

出版信息

Intern Med. 2013;52(13):1513-6. doi: 10.2169/internalmedicine.52.9335. Epub 2013 Jul 1.

Abstract

We herein report a case of polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS) syndrome presenting with acute Guillain-Barré syndrome (GBS)-like features. The patient was healthy, except for mild dilated cardiomyopathy. She was unable to walk within ten days of the onset of weakness. A nerve conduction study (NCS) showed length-dependent, symmetric and non-focal demyelinating features with increased terminal latency indices (TLIs) in the lower limbs. Following the administration of intravenous immunoglobulin infusion, the proximal weakness of the lower extremities improved; however, the pleural effusion was aggravated and ascites newly developed. On further work-ups, splenomegaly, M-protein and sclerotic bone changes were observed. This case suggests that, although rare, POEMS syndrome can present with acute demyelinating polyneuropathy resembling GBS and that characteristic NCS features such as increased TLI and uniform demyelination are helpful for the early diagnosis of POEMS syndrome.

摘要

我们在此报告一例呈现急性吉兰-巴雷综合征(GBS)样特征的多神经病、器官肿大、内分泌病、M蛋白和皮肤改变(POEMS)综合征病例。该患者身体健康,仅有轻度扩张型心肌病。在出现肌无力症状十天内,她就无法行走。神经传导研究(NCS)显示下肢存在长度依赖性、对称性且非局灶性脱髓鞘特征,终末潜伏期指数(TLIs)升高。静脉输注免疫球蛋白后,下肢近端肌无力有所改善;然而,胸腔积液加重,且新出现了腹水。进一步检查发现脾肿大、M蛋白和骨质硬化改变。该病例表明,尽管罕见,但POEMS综合征可表现为类似GBS的急性脱髓鞘性多发性神经病,且诸如TLI升高和均匀性脱髓鞘等特征性NCS表现有助于POEMS综合征的早期诊断。

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