Congenital Zika syndrome with arthrogryposis: retrospective case series study
Vanessa van der Linden1, Epitacio Leite Rolim Filho2, Otavio Gomes Lins3
1Association for Assistance of Disabled Children, AACD, Recife, Brazil Barão de Lucena Hospital, HBL, Recife, Brazil vanessavdlinden@hotmail.com.
Insights
Congenital Zika syndrome is linked to arthrogryposis in infants, presenting with neurological abnormalities rather than joint issues. This suggests a neurogenic origin for the condition, impacting motor neurons.
Area of Science:
- Neurology
- Pediatrics
- Infectious Diseases
Background:
- Congenital Zika syndrome has been associated with various neurological complications in newborns.
- Arthrogryposis, characterized by joint contractures, can have diverse etiologies.
Purpose of the Study:
- To investigate the clinical, radiological, and electromyographic features of children with arthrogryposis and presumed congenital Zika virus infection.
- To explore the potential neurogenic origin of arthrogryposis in the context of congenital Zika syndrome.
Main Methods:
- Retrospective case series of seven children diagnosed with congenital infection presumably caused by Zika virus and arthrogryposis.
- Analysis of clinical data, brain imaging (CT/MRI), spinal MRI, hip radiographs, and needle electromyography (EMG).
Main Results:
- All seven children exhibited brain abnormalities consistent with congenital infection, including cortical malformations, calcifications, reduced brain volume, and brainstem/cerebellar hypoplasia.
- Arthrogryposis affected the limbs in most cases; hip dislocations and knee subluxations were common.
- EMG revealed moderate remodeling of motor units and reduced recruitment, suggesting neurogenic involvement. Spinal MRI indicated thinning of the cord and reduced ventral roots in some patients.
Conclusions:
- Congenital Zika syndrome should be considered in the differential diagnosis of congenital infections causing arthrogryposis.
- The observed arthrogryposis is likely of neurogenic origin, stemming from chronic central and peripheral motor neuron involvement.
- Potential mechanisms include neuronal tropism or vascular disorders affecting motor neuron development.
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.
