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电生理标准在区分儿童脱髓鞘性神经病方面是否有用?

Are electrophysiological criteria useful in distinguishing childhood demyelinating neuropathies?

作者信息

Potulska-Chromik Anna, Ryniewicz Barbara, Aragon-Gawinska Karolina, Kabzinska Dagmara, Seroka Andrzej, Lipowska Marta, Kaminska Anna M, Kostera-Pruszczyk Anna

机构信息

Department of Neurology, Medical University of Warsaw, Warsaw, Poland.

Neuromuscular Unit, Polish Academy of Sciences, Warsaw, Poland.

出版信息

J Peripher Nerv Syst. 2016 Mar;21(1):22-6. doi: 10.1111/jns.12152.

Abstract

Childhood chronic inflammatory demyelinating polyneuropathy (CIDP) needs to be differentiated from hereditary neuropathy. We aimed to validate existing CIDP nerve conduction study (NCS) criteria in a group of children with demyelinating neuropathies of chronic or subacute onset. Retrospective analysis of clinical and NCS results in 18 children with CIDP, 7 with hereditary neuropathy with pressure palsy (HNPP), and 24 with Charcot-Marie-Tooth 1a (CMT1a). AAN and EFNS electrodiagnostic CIDP criteria were fulfilled in 17 of 18 CIDP, 3 of 7 HNPP, and 23 of 24 CMT1a patients. A distal compound muscle action potential (dCMAP) of >9 ms was observed in 14 of 18 CIDP patients but not in any patients with HNPP. Abnormal median/normal sural SNAP (AMNS) and a 10 m/s difference between conduction velocities (CV) of two corresponding nerves were not observed in any CMT1a patients. NCS in CMT1a, HNPP, and CIDP reflect demyelination. dCMAP duration, sensory AMNS, and a 10 m/s CV difference parameter are most useful in the differential diagnosis of pediatric CIDP.

摘要

儿童慢性炎症性脱髓鞘性多发性神经病(CIDP)需要与遗传性神经病相鉴别。我们旨在验证一组慢性或亚急性起病的脱髓鞘性神经病患儿中现有的CIDP神经传导研究(NCS)标准。对18例CIDP患儿、7例遗传性压力性麻痹性神经病(HNPP)患儿和24例遗传性运动感觉性神经病1型(CMT1a)患儿的临床和NCS结果进行回顾性分析。18例CIDP患儿中有17例、7例HNPP患儿中有3例、24例CMT1a患儿中有23例符合美国神经病学学会(AAN)和欧洲神经病学学会(EFNS)的CIDP电诊断标准。18例CIDP患儿中有14例观察到远端复合肌肉动作电位(dCMAP)>9 ms,而HNPP患儿均未观察到。在任何CMT1a患儿中均未观察到异常正中神经/正常腓肠神经感觉神经动作电位(AMNS)以及两条相应神经传导速度(CV)相差10 m/s的情况。CMT1a、HNPP和CIDP的NCS均反映脱髓鞘改变。dCMAP持续时间、感觉AMNS以及CV相差10 m/s的参数在儿童CIDP的鉴别诊断中最有用。

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