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儿童多发性神经病的临床和神经生理学谱。

Clinical and neurophysiological spectrum of polyneuropathies in children.

机构信息

Riga Stradins University, Latvia.

Children's Clinical University Hospital, Latvia.

出版信息

Neurol Neurochir Pol. 2020;54(5):466-470. doi: 10.5603/PJNNS.a2020.0068. Epub 2020 Sep 17.

DOI:10.5603/PJNNS.a2020.0068
PMID:32939748
Abstract

Peripheral neuropathy is a disorder of the peripheral nerves and results from a disturbance of structure and/or function of the peripheral sensory, motor and/or autonomic neurons. The possible aetiology of peripheral neuropathies is diverse, but inflammatory and hereditary diseases of the peripheral nerves predominate in childhood. The aim of this study was to determine the clinical and electrophysiological profile of large nerve fibre neuropathy detected by nerve conduction studies (NCS) in children over a 10-year period at the Children's Clinical University Hospital in Latvia. Based on NCS findings, 165 children between 2008 and 2018 were diagnosed with polyneuropathy. In our study, the majority of children had peripheral neuropathy due to acquired causes, mostly due to diabetes mellitus; roughly one in five of the patients had hereditary neuropathy. Almost half of the patients had motor deficits, which were more prevalent in toxic and inflammatory neuropathies. A little less than a third of patients complained of pain as well as presenting with autonomic dysfunction symptoms. The NCS demonstrated a demyelinating neuropathy in 52 cases (31%), an axonal neuropathy in 34 cases (21%), and mixed polyneuropathy in 79 cases (48%). Our study investigated the clinical and electrophysiological characteristics of polyneuropathies diagnosed with NCS in children. Most of the polyneuropathies in our study were hereditary and diabetic neuropathies with combined (myelin and axon) damage to nerve fibres. Almost all clinical symptoms of polyneuropathy were present in all aetiological groups.

摘要

周围神经病变是周围神经的一种紊乱,源于周围感觉、运动和/或自主神经元的结构和/或功能障碍。周围神经病的可能病因多种多样,但在儿童时期,炎症性和遗传性周围神经疾病更为常见。本研究旨在确定在拉脱维亚儿童临床大学医院的 10 年期间,通过神经传导研究(NCS)检测到的大纤维神经病的临床和电生理特征。基于 NCS 的发现,在 2008 年至 2018 年间,165 名儿童被诊断为多发性神经病。在我们的研究中,大多数儿童的周围神经病变是由获得性原因引起的,主要是由于糖尿病;约五分之一的患者患有遗传性神经病。几乎一半的患者有运动缺陷,这些缺陷在中毒性和炎症性神经病中更为常见。不到三分之一的患者有疼痛主诉,也有自主功能障碍症状。NCS 在 52 例(31%)中显示脱髓鞘神经病,在 34 例(21%)中显示轴突神经病,在 79 例(48%)中显示混合性多发性神经病。本研究调查了通过 NCS 诊断的儿童多发性神经病的临床和电生理特征。我们研究中的大多数多发性神经病是遗传性和糖尿病性神经病,伴有纤维的联合(髓鞘和轴突)损伤。几乎所有多发性神经病的临床症状都存在于所有病因组中。

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