| Author |
Van Der Linden, Ana Rocha, Maria Angela Wanderley Brainer-lima, Alessandra Mertens Carvalho, Maria Durce Costa Gomes Do Amaral, Fernando José Ramos, Regina Coeli Aragão, Maria De Fátima Viana Vasco Gomes, Joelma Arruda Ribeiro De Medeiros, Igor Colaço Filho, Epitacio Leite Rolim Ventura, Camila V. Van Der Linden, Vanessa Lins, Otavio Gomes Cruz, Danielle Di Cavalcanti Sousa Sobral Da Silva, Paula Fabiana |
| Description |
Author Affiliation: van der Linden V ( Association for Assistance of Disabled Children, AACD, Recife, Brazil Barão de Lucena Hospital, HBL, Recife, Brazil vanessavdlinden@hotmail.com.); Filho EL ( Association for Assistance of Disabled Children, AACD, Recife, Brazil Federal University of Pernambuco, UFPE, Recife, Brazil.); Lins OG ( Federal University of Pernambuco, UFPE, Recife, Brazil.); van der Linden A ( Prof Fernando Figueira Integral Medicine Institute, IMIP, Recife, Brazil vanessavdlinden@hotmail.com.); Aragão Mde F ( Centro Diagnóstico Multimagem, Recife, Brazil Mauricio de Nassau University, Recife, Brazil.); Brainer-Lima AM ( Mauricio de Nassau University, Recife, Brazil University of Pernambuco, UPE, Recife, Brazil.); Cruz DD ( Prof Fernando Figueira Integral Medicine Institute, IMIP, Recife, Brazil.); Rocha MA ( Oswaldo Cruz University Hospital, HUOC, Recife, Brazil.); Sobral da Silva PF ( Oswaldo Cruz University Hospital, HUOC, Recife, Brazil.); Carvalho MD ( Oswaldo Cruz University Hospital, HUOC, Recife, Brazil.); do Amaral FJ ( Barão de Lucena Hospital, HBL, Recife, Brazil.); Gomes JA ( Barão de Lucena Hospital, HBL, Recife, Brazil.); Ribeiro de Medeiros IC ( Hospital Infantil Jorge de Medeiros Recife, Brazil.); Ventura CV ( Altino Ventura Foundation, FAV, Recife, Brazil Pernambuco's Eye Hospital, Recife, Brazil.); Ramos RC ( Oswaldo Cruz University Hospital, HUOC, Recife, Brazil.); |
| Abstract |
Objective To describe the clinical, radiological, and electromyographic features in a series of children with joint contractures (arthrogryposis) associated with congenital infection presumably caused by Zika virus. Design Retrospective case series study. Setting Association for Assistance of Disabled Children, Pernambuco state, Brazil. Participants Seven children with arthrogryposis and a diagnosis of congenital infection presumably caused by Zika virus during the Brazilian microcephaly epidemic. Main outcome measures Main clinical, radiological, and electromyographic findings, and likely correlation between clinical and primary neurological abnormalities. Results The brain images of all seven children were characteristic of congenital infection and arthrogryposis. Two children tested positive for IgM to Zika virus in the cerebrospinal fluid. Arthrogryposis was present in the arms and legs of six children (86%) and the legs of one child (14%). Hip radiographs showed bilateral dislocation in seven children, subluxation of the knee associated with genu valgus in three children (43%), which was bilateral in two (29%). All the children underwent high definition ultrasonography of the joints, and there was no evidence of abnormalities. Moderate signs of remodeling of the motor units and a reduced recruitment pattern were found on needle electromyography (monopolar). Five of the children underwent brain computed tomography (CT) and magnetic resonance imaging (MRI) and the remaining two CT only. All presented malformations of cortical development, calcifications predominantly in the cortex and subcortical white matter (especially in the junction between the cortex and white matter), reduction in brain volume, ventriculomegaly, and hypoplasia of the brainstem and cerebellum. MRI of the spine in four children showed apparent thinning of the cord and reduced ventral roots. Conclusions Congenital Zika syndrome should be added to the differential diagnosis of congenital infections and arthrogryposis. The arthrogryposis was unrelated to the abnormalities of the joints themselves, but was possibly of neurogenic origin, with chronic involvement of central and peripheral motor neurones leading to deformities as a result of fixed postures in utero. Based on the neurophysiological observations, we suggest two possible mechanisms: tropism of neurones, with involvement of peripheral and central motor neurones, or a relation with vascular disorders. |
