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周围神经成像显示的隐匿性1A型遗传性运动感觉神经病

Hidden Charcot-Marie-Tooth 1A as Revealed by Peripheral Nerve Imaging.

作者信息

Shibuya Kazumoto, Yoshida Toshiki, Misawa Sonoko, Sekiguchi Yukari, Beppu Minako, Amino Hiroshi, Suzuki Yo-Ichi, Suichi Tomoki, Tsuneyama Atsuko, Nakamura Keigo, Kuwabara Satoshi

机构信息

Department of Neurology, Graduate School of Medicine, Chiba University, Japan.

出版信息

Intern Med. 2019 Nov 1;58(21):3157-3161. doi: 10.2169/internalmedicine.3040-19. Epub 2019 Jul 10.

Abstract

Peripheral nerve imaging techniques have recently increasingly revealed their usefulness. We herein describe a man who had a subacute progression of symptom, diffuse and prominent proximal demyelination and conduction block, suggesting a diagnosis of inflammatory demyelinating polyneuropathy. Additional nerve imaging techniques revealed homogeneous and prominent nerve hypertrophy without proximal accentuation and the findings implied inherited polyneuropathies. Intravenous immunoglobulin was administered, and both the symptoms of weakness and findings of nerve conduction studies (NCS) improved. Subsequent genetic testing unveiled Charcot-Marie-Tooth 1A. To diagnose peripheral nerve disorders, a careful history, physical examination and NCS are essential diagnostic tools, but the findings of this case suggest the importance of nerve imaging techniques in clinical situations.

摘要

周围神经成像技术最近越来越显示出其有用性。我们在此描述一名男性,其症状呈亚急性进展,存在弥漫性且明显的近端脱髓鞘和传导阻滞,提示炎性脱髓鞘性多发性神经病的诊断。额外的神经成像技术显示神经均匀且明显肥大,无近端加重,这些发现提示遗传性多发性神经病。给予静脉注射免疫球蛋白后,肌无力症状和神经传导研究(NCS)结果均有所改善。随后的基因检测揭示为1A型遗传性运动感觉神经病。对于诊断周围神经疾病,仔细的病史、体格检查和NCS是必不可少的诊断工具,但该病例的发现提示神经成像技术在临床情况下的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6dd0/6875441/90952d0f7d0a/1349-7235-58-3157-g001.jpg

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