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脊髓空洞症、肢体肥大与交感神经过度活跃:一种罕见的关联。

Syringomyelia, limb hypertrophy and sympathetic overactivity: a rare association.

作者信息

Khanra Dibbendhu, Ray Sayantan, Sonthalia Nikhil, Talukdar Arunansu

机构信息

Department of General Medicine, Medical College Hospital, Kolkata, West Bengal, India.

出版信息

BMJ Case Rep. 2012 Jun 14;2012:bcr0320126092. doi: 10.1136/bcr.03.2012.6092.

Abstract

A 32-year-old man presented with uniform enlargement of right upper limb for 6 years. Examination revealed painful disorganised elbow joint along with sensory impairment in the affected limb with weakness of small muscles. The patient was given empirical antileprotic therapy from outside without any benefit. Ultrasonography showed pan-hypertrophic nature of local tissues. Although axonal type of sensorimotor neuropathy involving right ulnar and median nerve was detected in nerve conduction velocity study, biopsy of the same failed to confirm any axonal degeneration or evidence of leprosy. Considering the nature of sensory abnormality MRI of cervical spine was done which revealed a syrinx extending from C3 till D2. Tests for vasomotor tone showed positive results on the affected limb. Syringomyelia can rarely give rise to pan-hypertrophy of limb due to sympathetic overactivity, which is infrequently reported in literatures and deficient in logical grounds.

摘要

一名32岁男性,右上肢均匀性肿大6年。检查发现肘关节疼痛、结构紊乱,患肢感觉障碍,小肌肉无力。患者在外接受了经验性抗麻风治疗,但无任何效果。超声检查显示局部组织呈全肥厚性。尽管神经传导速度研究检测到累及右尺神经和正中神经的轴索性感觉运动神经病,但对其进行活检未能证实任何轴索变性或麻风证据。考虑到感觉异常的性质,对颈椎进行了磁共振成像检查,结果显示一个从C3延伸至D2的空洞。血管舒缩张力测试显示患肢结果为阳性。脊髓空洞症很少会因交感神经过度活跃而导致肢体全肥厚,这在文献中鲜有报道且缺乏合理依据。

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本文引用的文献

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