Abstract：
OBJECTIVE: Amyoplasia congenita is the most frequent type of arthrogryposis causing fetal hypokinesia, leading to congenital contractures at birth. The pathogenesis is thought to be impaired blood circulation to the fetus early in pregnancy, with hypotension and hypoxia damaging the anterior horn cells. In animal studies however a prenatal infection with a poliomyelitis-like viral agent was demonstrated. Congenital Zika virus syndrome (CZVS) has recently been described in infants with severe microcephaly, and in 10-25% of cases arthrogryposis.
METHODS: A search in PubMed for CZVS yielded 124 studies. After a selection for arthrogryposis, 35 papers were included, describing 144 cases. The studies were divided into two categories. 1) Those (87 cases) focussing on imaging or histological data of congenital brain defects, contained insufficient information to link arthrogryposis specifically to lesions of the brain or spinal motor neuron. 2) In the other 57 cases detailed clinical data could be linked to neurophysiological, imaging or histological data.
RESULTS: In category 1 the most frequent brain abnormalities in imaging studies were ventriculomegaly, calcifications (subcortical, basal ganglia, cerebellum), hypoplasia of the brainstem and cerebellum, atrophy of the cerebral cortex, migration disorders and corpus callosum anomalies. In category 2, in 38 of 57 cases clinical data were indicative of Amyoplasia congenita. This diagnosis was confirmed by electromyographic findings (13 cases), by MRI (37 cases) or histology (12 cases) of the spinal cord. The latter showed small or absent lateral corticospinal tracts, and cell loss and degeneration of motor neuron cells. Zika virus-proteins and flavivirus-like particles were detected in cytoplasm of spinal neurons.
CONCLUSIONS: The phenotype of arthrogryposis in CZVS is consistent with Amyoplasia congenita. These findings warrant search for an intrauterine infection with any neurotropic viral agent with affinity to spinal motor neurons in neonates with Amyoplasia.
METHODS: A search in PubMed for CZVS yielded 124 studies. After a selection for arthrogryposis, 35 papers were included, describing 144 cases. The studies were divided into two categories. 1) Those (87 cases) focussing on imaging or histological data of congenital brain defects, contained insufficient information to link arthrogryposis specifically to lesions of the brain or spinal motor neuron. 2) In the other 57 cases detailed clinical data could be linked to neurophysiological, imaging or histological data.
RESULTS: In category 1 the most frequent brain abnormalities in imaging studies were ventriculomegaly, calcifications (subcortical, basal ganglia, cerebellum), hypoplasia of the brainstem and cerebellum, atrophy of the cerebral cortex, migration disorders and corpus callosum anomalies. In category 2, in 38 of 57 cases clinical data were indicative of Amyoplasia congenita. This diagnosis was confirmed by electromyographic findings (13 cases), by MRI (37 cases) or histology (12 cases) of the spinal cord. The latter showed small or absent lateral corticospinal tracts, and cell loss and degeneration of motor neuron cells. Zika virus-proteins and flavivirus-like particles were detected in cytoplasm of spinal neurons.
CONCLUSIONS: The phenotype of arthrogryposis in CZVS is consistent with Amyoplasia congenita. These findings warrant search for an intrauterine infection with any neurotropic viral agent with affinity to spinal motor neurons in neonates with Amyoplasia.
摘要：
目的：先天性肌增生是导致胎儿运动功能减退的最常见的关节病类型，导致出生时的先天性挛缩。发病机制被认为是妊娠早期胎儿的血液循环受损，低血压和缺氧损害前角细胞。然而，在动物研究中，证实了脊髓灰质炎样病毒剂的产前感染。先天性寨卡病毒综合征（CZVS）最近在患有严重小头畸形的婴儿中被描述，在10-25%的病例中。
方法：在PubMed中搜索CZVS产生了124项研究。在选择关节病后，包括35篇论文，描述了144个案例。这些研究分为两类。1）那些（87例）集中于先天性脑缺陷的影像学或组织学资料，包含的信息不足，无法将节理病专门与大脑或脊髓运动神经元的病变联系起来。2)在其他57例中，详细的临床数据可能与神经生理学有关，成像或组织学数据。
结果：在第1类中，影像学检查中最常见的脑部异常是脑室扩大，钙化（皮质下，基底神经节,小脑)，脑干和小脑发育不全,大脑皮层萎缩,迁移障碍和call体异常。在第2类中，57例临床数据中有38例表明先天性肌增生。该诊断通过肌电图检查（13例）得到证实，经MRI（37例）或组织学（12例）检查脊髓。后者显示外侧皮质脊髓束小或缺失，以及运动神经元细胞的细胞丢失和变性。在脊髓神经元的细胞质中检测到寨卡病毒蛋白和黄病毒样颗粒。
结论：CZVS的关节病表型与先天性肌增生一致。这些发现保证了在患有肌增生的新生儿中寻找任何对脊髓运动神经元具有亲和力的嗜神经病毒剂的宫内感染。
方法：在PubMed中搜索CZVS产生了124项研究。在选择关节病后，包括35篇论文，描述了144个案例。这些研究分为两类。1）那些（87例）集中于先天性脑缺陷的影像学或组织学资料，包含的信息不足，无法将节理病专门与大脑或脊髓运动神经元的病变联系起来。2)在其他57例中，详细的临床数据可能与神经生理学有关，成像或组织学数据。
结果：在第1类中，影像学检查中最常见的脑部异常是脑室扩大，钙化（皮质下，基底神经节,小脑)，脑干和小脑发育不全,大脑皮层萎缩,迁移障碍和call体异常。在第2类中，57例临床数据中有38例表明先天性肌增生。该诊断通过肌电图检查（13例）得到证实，经MRI（37例）或组织学（12例）检查脊髓。后者显示外侧皮质脊髓束小或缺失，以及运动神经元细胞的细胞丢失和变性。在脊髓神经元的细胞质中检测到寨卡病毒蛋白和黄病毒样颗粒。
结论：CZVS的关节病表型与先天性肌增生一致。这些发现保证了在患有肌增生的新生儿中寻找任何对脊髓运动神经元具有亲和力的嗜神经病毒剂的宫内感染。
