Sympathetic skin response, cutaneous silent period and nociceptive flexion reflex in Fabry disease

Author:

Bölük Cem,Akıncı Yasemin,Seyahi NurhanORCID,Uzun Adatepe Nurten,Gündüz Ayşegül

Abstract

Background & Objective: Fabry disease is one of the well-known causes of the small fiber neuropathy. This study aims to explore small and large nerve fiber functions in patients with Fabry disease without using any extra equipment in a standard neurophysiology laboratory. Methods: Patients with Fabry disease at our tertiary center were invited to the electrophysiology laboratory. Routine nerve conduction studies (NCS), sympathetic skin response (SSR), cutaneous silent period (CSP) and nociceptive flexion reflex (NFR) tests were performed. The same protocol was applied to matched healthy subjects. Results: Nineteen patients and 19 healthy controls were included. Carpal tunnel syndrome was diagnosed in 3 patients (15.9%). Palmar and plantar SSRs could not be obtained in 4 and 9 patients, respectively. Abnormal CSP responses in the upper and lower extremities were recorded in 2 and 7 patients, respectively. The NFR response was abnormal in 8 patients. In total, at least one of the SSR, CSP or NFR was abnormal in 73.7% of patients. Conclusions: The study showed that combining SSR, CSP and NFR tests along with NCS could help to determine any abnormality in small and large fiber functions in most patients. The overall sensitivity of all tests was approximately 70% compared with the clinical diagnosis of small fiber neuropathy. This study is unique in that it explored a combination of NCS, SSR, CSP and NFR tests in Fabry disease.

Publisher

ASEAN Neurological Association

Subject

Neurology (clinical),Neurology

同舟云学术

1.学者识别学者识别

2.学术分析学术分析

3.人才评估人才评估

"同舟云学术"是以全球学者为主线,采集、加工和组织学术论文而形成的新型学术文献查询和分析系统,可以对全球学者进行文献检索和人才价值评估。用户可以通过关注某些学科领域的顶尖人物而持续追踪该领域的学科进展和研究前沿。经过近期的数据扩容,当前同舟云学术共收录了国内外主流学术期刊6万余种,收集的期刊论文及会议论文总量共计约1.5亿篇,并以每天添加12000余篇中外论文的速度递增。我们也可以为用户提供个性化、定制化的学者数据。欢迎来电咨询!咨询电话:010-8811{复制后删除}0370

www.globalauthorid.com

TOP

Copyright © 2019-2024 北京同舟云网络信息技术有限公司
京公网安备11010802033243号  京ICP备18003416号-3