Abstract
Background
Congenital Zika Virus Syndrome (CZVS) denotes the neurologic and developmental sequelae
of congenital infection of the Zika virus. While prior studies have detailed the associated
clinical phenotypes, new findings continue to be identified. Abnormal postures and
movements have been previously described in children with CZVS, but not in detail.
Objective
To examine a cohort of infants with CZVS and characterize the spectrum of motor abnormalities,
especially movement disorders.
Design
Cross-sectional prospective study of 21 infants with confirmed CZVS.
Setting
Single-center cohort of 32 patients with serologically confirmed CZVS cared for in
a referral center in Brazil.
Participants
21 children (67% female), evaluated by two child neurologists and one movement disorders
specialist, with clinical and laboratory diagnosis of CZVS aged between 16 and 30 months,
with a mean age of 16 months at the time of the last examination.
Main outcome(s) and measure(s)
Prospective neurologic examination by a team of three neurologists, including one
movement disorders specialist. Sixteen (76.2%) children had a longitudinal evaluation
with a six-month interval. The same team of experts analyzed recorded videos of all
patients to characterize motor abnormalities and movement disorders. Neuroimaging
findings were also analyzed to correlate with clinical findings.
Results
Twenty (95.2%) patients presented with dystonic postures, including “125” posture
of the fingers in 17 (80.1%), “swan neck” posture of the fingers in three (18.8%),
oromandibular dystonia in nine (42.9%), extensor axial hypertonia in eight (38.1%)
and internal rotation of the shoulder posture in two (9.5%). Four (19%) patients had
tremor. All children had malformations of cortical development, and in 13 (61.9%),
the pattern was consistent with a severe and diffuse gyral simplification. Seventeen
children (81%) had calcification in the transition of grey and white matter, whereas
11 (52.4%) patients had basal ganglia calcifications.
Conclusion and relevance
In our series, dystonic postures and other extrapyramidal signs were frequent and
potentially disabling. Although children with CZVS are assessed and treated for spasticity,
dystonia and other movement disorders remain neglected. This study emphasizes that
extrapyramidal findings may potentially influence optimal strategies for rehabilitation
and management.
Keywords
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Article Info
Publication History
Published online: July 16, 2020
Accepted:
June 30,
2020
Received in revised form:
June 25,
2020
Received:
May 22,
2020
Identification
Copyright
© 2020 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.
