Pediatric Neurology Service, Hospital da Restauração, Recife, Brazil.
Pediatric Neurology Service, Hospital Barão de Lucena, Recife, Brazil.
J Clin Neurophysiol. 2023 May 1;40(4):350-354. doi: 10.1097/WNP.0000000000000893. Epub 2021 Sep 6.
Congenital Zika virus syndrome is a distinct pattern of birth defects in fetuses infected by the Zika virus. It presents a broad clinical spectrum that includes occurrences of microcephaly, hypertonia, dysphagia, hyperexcitability, seizures, and arthrogryposis. Imaging findings show neuronal migration disorders.
Case reports have suggested that arthrogryposis has a neurogenic cause. We analyzed needle electromyography and nerve conduction examinations on 77 patients aged 2-24 months presenting highly probable congenital Zika virus syndrome, with or without arthrogryposis.
All those with arthrogryposis presented with chronic muscle denervation in the electromyography examination. Similarly, children with single or reversible joint abnormalities at birth showed the same findings. Denervation in the paravertebral musculature was found in all of the children with diaphragmatic paralysis or thoracic deformities.
We propose that congenital contractures associated with congenital Zika virus syndrome are caused by the malformation of upper and lower motor neurons during embryogenesis.
先天性寨卡病毒综合征是寨卡病毒感染胎儿的一种独特的出生缺陷模式。它表现出广泛的临床谱系，包括小头畸形、高张力、吞咽困难、过度兴奋、癫痫发作和关节挛缩。影像学表现显示神经元迁移障碍。
病例报告提示关节挛缩具有神经源性原因。我们分析了 77 名 2-24 个月大的高度疑似先天性寨卡病毒综合征患儿的针极肌电图和神经传导检查，这些患儿伴有或不伴有关节挛缩。
所有伴有关节挛缩的患儿在肌电图检查中均表现为慢性肌肉去神经支配。同样，出生时伴有单个或可恢复关节异常的患儿也有同样的发现。所有存在膈肌麻痹或胸壁畸形的患儿均出现椎旁肌去神经支配。
我们提出，与先天性寨卡病毒综合征相关的先天性挛缩是由于胚胎发育过程中上下运动神经元的畸形所致。
