title: Congenital Zika syndrome with arthrogryposis: retrospective case series study.
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.
  
  
  METHODS
  Retrospective case series study.
  
  
  METHODS
  Association for Assistance of Disabled Children, Pernambuco state, Brazil.
  
  
  METHODS
  Seven children with arthrogryposis and a diagnosis of congenital infection presumably caused by Zika virus during the Brazilian microcephaly epidemic.
  
  
  METHODS
  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.
authors: Association for Assistance of Disabled Children, AACD, Recife, Brazil Barão de Lucena Hospital, HBL, Recife, Brazil vanessavdlinden@hotmail.com.
authors: Vanessa
authors: V
authors: van der Linden
authors: Association for Assistance of Disabled Children, AACD, Recife, Brazil Federal University of Pernambuco, UFPE, Recife, Brazil.
authors: Epitacio Leite Rolim
authors: EL
authors: Filho
authors: Federal University of Pernambuco, UFPE, Recife, Brazil.
authors: Otavio Gomes
authors: OG
authors: Lins
authors: Prof Fernando Figueira Integral Medicine Institute, IMIP, Recife, Brazil vanessavdlinden@hotmail.com.
authors: Ana
authors: A
authors: van der Linden
authors: Centro Diagnóstico Multimagem, Recife, Brazil Mauricio de Nassau University, Recife, Brazil.
authors: Maria de Fátima Viana Vasco
authors: Mde F
authors: Aragão
authors: Mauricio de Nassau University, Recife, Brazil University of Pernambuco, UPE, Recife, Brazil.
authors: Alessandra Mertens
authors: AM
authors: Brainer-Lima
authors: Prof Fernando Figueira Integral Medicine Institute, IMIP, Recife, Brazil.
authors: Danielle Di Cavalcanti Sousa
authors: DD
authors: Cruz
authors: Oswaldo Cruz University Hospital, HUOC, Recife, Brazil.
authors: Maria Angela Wanderley
authors: MA
authors: Rocha
authors: Oswaldo Cruz University Hospital, HUOC, Recife, Brazil.
authors: Paula Fabiana
authors: PF
authors: Sobral da Silva
authors: Oswaldo Cruz University Hospital, HUOC, Recife, Brazil.
authors: Maria Durce Costa Gomes
authors: MD
authors: Carvalho
authors: Barão de Lucena Hospital, HBL, Recife, Brazil.
authors: Fernando José
authors: FJ
authors: do Amaral
authors: Barão de Lucena Hospital, HBL, Recife, Brazil.
authors: Joelma Arruda
authors: JA
authors: Gomes
authors: Hospital Infantil Jorge de Medeiros Recife, Brazil.
authors: Igor Colaço
authors: IC
authors: Ribeiro de Medeiros
authors: Altino Ventura Foundation, FAV, Recife, Brazil Pernambuco's Eye Hospital, Recife, Brazil.
authors: Camila V
authors: CV
authors: Ventura
authors: Oswaldo Cruz University Hospital, HUOC, Recife, Brazil.
authors: Regina Coeli
authors: RC
authors: Ramos
mesh_terms: D001176:Arthrogryposis / Q000150:complications* / Q000000981:diagnostic imaging; D002114:Calcinosis / Q000000981:diagnostic imaging; D004576:Electromyography; D006801:Humans; D007596:Joints / Q000002:abnormalities / Q000000981:diagnostic imaging; D008279:Magnetic Resonance Imaging; D008831:Microcephaly / Q000150:complications* / Q000000981:diagnostic imaging; D012189:Retrospective Studies; D013116:Spinal Cord / Q000000981:diagnostic imaging; D013577:Syndrome; D014057:Tomography, X-Ray Computed; D014463:Ultrasonography; D000071243:Zika Virus Infection / Q000150:complications* / Q000151:congenital* / Q000000981:diagnostic imaging