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一名多灶性获得性脱髓鞘性感觉和运动性神经病(MADSAM)患者在对静脉注射免疫球蛋白产生良好临床反应后,其超声检查显示的多灶性神经增粗恢复正常。

Normalization of sonographical multifocal nerve enlargements in a MADSAM patient following a good clinical response to intravenous immunoglobulin.

作者信息

Tanaka Kanta, Ota Natsuko, Harada Yuzuru, Wada Ikko, Suenaga Toshihiko

机构信息

Department of Neurology, Tenri Hospital, Tenri, Japan.

Department of Clinical Pathology, Tenri Hospital, Tenri, Japan.

出版信息

Neuromuscul Disord. 2016 Sep;26(9):619-23. doi: 10.1016/j.nmd.2016.06.008. Epub 2016 Jun 14.

Abstract

Focal nerve enlargements at sites of conduction blocks can be visualized sonographically in patients with multifocal acquired demyelinating sensory and motor neuropathy (MADSAM). However, little is known about association between nerve morphological changes and treatment responses. Here we present a 73-year-old female MADSAM patient whose sonographical multifocal nerve enlargements normalized following a good treatment response. She was admitted to our department with progressive asymmetrical muscle weakness and sensory disturbances for 6 months. Ultrasonography revealed multifocal nerve enlargements at sites of electrophysiological demyelination. Intravenous immunoglobulin improved her symptoms and electrophysiological abnormalities. Six months later, ultrasonography revealed normalization of multifocal nerve enlargements. Contrary to our observations, one previous report described a MADSAM patient with persistent nerve enlargements at the sites of resolved conduction blocks. In this earlier patient, however, the time from onset to remission was approximately 30 months. Morphological changes of nerve enlargements in MADSAM may vary with treatment response.

摘要

在多灶性获得性脱髓鞘感觉和运动神经病(MADSAM)患者中,超声检查可以发现传导阻滞部位的局灶性神经增粗。然而,关于神经形态学变化与治疗反应之间的关联,我们知之甚少。在此,我们报告一名73岁的女性MADSAM患者,其在治疗反应良好后,超声检查发现的多灶性神经增粗恢复正常。她因进行性不对称肌无力和感觉障碍6个月入院。超声检查显示在电生理脱髓鞘部位有多灶性神经增粗。静脉注射免疫球蛋白改善了她的症状和电生理异常。6个月后,超声检查显示多灶性神经增粗恢复正常。与我们的观察结果相反,之前有一份报告描述了一名MADSAM患者,其在传导阻滞缓解部位仍存在持续性神经增粗。然而,在这名早期患者中,从发病到缓解的时间约为30个月。MADSAM中神经增粗的形态学变化可能随治疗反应而有所不同。

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