成功治疗Poems综合征后的长期神经传导改变:两例报告

W. Homenda, Szymon Kukulski
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引用次数: 0

摘要

POEMS综合征是一种罕见的疾病,表现为多神经病变和浆细胞病变,并伴有其他多系统症状。感觉运动脱髓鞘,轴突和混合型多神经病变是最令人困扰的问题,往往导致残疾的患者。在此,我们报告了两例自体造血干细胞移植成功治疗的患者。经治疗后POEMS综合征症状完全消失。神经传导研究用于对完全缓解的患者随访数年,以分析神经再生的动态。尽管患者的临床表现有所改善,但神经传导恢复不完全。两例患者上肢神经功能恢复程度均高于下肢。感觉神经传导损伤的改善或稳定之后,在给定的肢体中,类似的运动神经传导是动态的。此外,观察到电生理特征的恶化,完成了POEMS综合征治疗后复杂而微妙的传导变化。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Long-Term Nerve Conduction Changes after Successful Treatment of Poems Syndrome: Two Case Reports
The POEMS syndrome is a rare disorder manifesting with polyneuropathy and plasma cell dyscrasia accompanied by other multisystemic symptoms. Sensorimotor demyelinating, axonal and mixed types of polyneuropathy are the most disturbing problems of patients often leading to disability. Here we present two patients successfully treated with autologous hematopoietic stem cells transplantation. Symptoms of the POEMS syndrome entirely resolved after the treatment. Nerve conduction studies were used to follow-up patients in complete remission for few years to analyze dynamics of nerves regeneration. Despite patients clinical performance improvement, the recovery of nerve conduction was not complete. In both patients nerves of upper limbs function was restored to the greater extent than in lower limbs. Improvement or stabilization of impairment of sensory nerves conduction was followed by similar motor nerves conduction dynamic in a given limb. In addition, worsening of the electrophysiological features was observed, completing the picture of complex and subtle changes of conduction after the POEMS syndrome treatment.
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