The authors present a case of acute disseminated encephalomyelitis in a COVID-19 pediatric patient with positive SARS-CoV2 markers from a nasopharyngeal swab. A previously healthy 12-year-old-girl presented with a skin rash, headache, and fever. Five days after that, she had an acute, progressive, bilateral, and symmetrical motor weakness. She evolved to respiratory failure. Magnetic resonance imaging (MRI) of the brain and cervical spine showed extensive bilateral and symmetric restricted diffusion involving the subcortical and deep white matter, a focal hyperintense T2/ FLAIR lesion in the splenium of the corpus callosum with restricted diffusion, and extensive cervical myelopathy involving both white and gray matter. Follow-up examinations of the brain and spine were performed 30 days after the first MRI examination. The images of the brain demonstrated mild dilatation of the lateral ventricles and widespread widening of the cerebral sulci, complete resolution of the extensive white matter restricted diffusion, and complete resolution of the restricted diffusion in the lesion of the splenium of the corpus callosum, leaving behind a small gliotic focus. The follow-up examination of the spine demonstrated nearly complete resolution of the extensive signal changes in the spinal cord, leaving behind scattered signal changes in keeping with gliosis. She evolved with partial clinical and neurological improvement and was subsequently discharged.

Background: Paralysis of the diaphragm in newborn infants can lead to recurrent infections and life-threatening respiratory insufficiency. The clinical diagnosis of unilateral diaphragmatic paralysis has been reported in infants with laboratory evidence of congenital Zika virus infection and/or the congenital Zika syndrome (CZS) phenotype but no evaluation of phrenic nerve function has been described.All reported infants have had accompanying arthrogryposis. High infant mortality is reported. Methods: The causal mechanism of congenital diaphragmatic paralysis was evaluated in three infants with arthrogryposis as a manifestation of CZS (two of the three infants had laboratory evidence of ZIKV infection shortly after birth; the remaining infant had negative serology for ZIKV when first tested at 7 months of age). Electromyography and phrenic nerve compound muscle action potential (CMAP) were performed in all infants with diaphragmatic paralysis demonstrated on imaging studies. Results: All infants had evidence of moderate chronic involvement of peripheral motor neurons. Phrenic nerve CMAP was reduced on the side of the diaphragmatic paralysis in two infants and reduced bilaterally in the remaining infant who had primarily anterior involvement of the diaphragm. All three infants had multiple medical complications and one infant died at 18 months of age. Conclusion: Evaluation of three infants with CZS and diaphragmatic paralysis demonstrated phrenic nerve dysfunction. In these and other affected infants, arthrogryposis appears to be a constant co-occurring condition and health problems are significant; both conditions are likely due to involvement of the peripheral nervous system in some infants with CZS. K E Y W O R D S arthrogryposis, congenital infection, diaphragmatic paralysis, phrenic nerve, Zika virus

Background Congenital arthrogryposis (CA) consists of congenital joint contractures that affect at least two joints in different parts of the body. Approximately, 80% of CA cases are neurogenic, with changes to the formation, structure or functioning of the central and/or peripheral nervous systems. Most abnormalities are triggered either by motoneurons decreased activation in the corticospinal tract or by direct motoneurons injury. There had been few reports in the literature correlating congenital infection in humans with arthrogryposis until 2015. CA has recently been described associated with congenital Zika syndrome (CZS). Methods The objective of this study was to investigate and describe accurately the arthrogrypotic alterations in infants diagnosed with CZS and thus, suggest a possible pattern of orthopedic impairment. A total of 198 medical records of infants with CZS were evaluated. According to inclusion and exclusion criteria, 17 infants were included in the present study. Arthrogrypotic joints were orthopedically evaluated in four segments: right, left, upper, and lower limbs. All the four segments were assessed independently. Results Flexed wrists were the most frequently observed manifestation, associated with ulnar deviation (35.29%). Deformities were also commonly found in the third and fourth fingers (64.70%). Hip dislocation was found in 58.82% of the patients and talipes equinovarus and equinovalgus ankles were found in 29.41 and 23.52%. Conclusion There was a particular pattern of joint impairment related to CZS and arthogrypotic alterations of infants evaluated in this study.

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Introduction: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.Methodology: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.Results: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.Conclusions:We propose that congenital contractures associated with congenital Zika virus syndrome are caused by the malformation of upper and lower motor neurons during embryogenesis.