Movement disorders in children with congenital Zika virus syndrome
Hélio van der Linden1, Laura Silveira-Moriyama2, Vanessa van der Linden3
1Rehabilitation Center Dr. Henrique Santillo, Pediatric Neurology, Goiania, GO, Brazil; Neurology Institute, Goiania, GO, Brazil.
Insights
Congenital Zika Virus Syndrome (CZVS) frequently causes disabling dystonic postures and movement disorders. These extrapyramidal signs, often overlooked, are crucial for effective rehabilitation and management strategies in affected children.
Area of Science:
- Neurology
- Pediatrics
- Infectious Diseases
Background:
- Congenital Zika Virus Syndrome (CZVS) results from prenatal Zika virus infection, causing neurological and developmental issues.
- While spasticity is recognized, detailed characterization of abnormal postures and movement disorders in CZVS is lacking.
Purpose of the Study:
- To investigate the spectrum of motor abnormalities, particularly movement disorders, in infants diagnosed with CZVS.
- To characterize the prevalence and types of extrapyramidal signs in a cohort of children with CZVS.
Main Methods:
- A prospective, cross-sectional study involving 21 infants with confirmed CZVS in Brazil.
- Neurological examinations by child neurologists and a movement disorders specialist, supplemented by video analysis and neuroimaging review.
- Longitudinal evaluations were conducted for a majority of participants (76.2%) at six-month intervals.
Main Results:
- Dystonic postures were highly prevalent (95.2%), including specific finger postures (e.g., "125" in 80.1%) and oromandibular dystonia (42.9%).
- Other observed motor abnormalities included extensor axial hypertonia (38.1%) and tremor (19%).
- Neuroimaging revealed cortical malformations (100%), with severe gyral simplification in 61.9%, and calcifications in the grey-white matter transition (81%) and basal ganglia (52.4%).
Conclusions:
- Dystonic postures and other extrapyramidal signs are common and potentially disabling in CZVS.
- These movement disorders are often neglected despite the focus on spasticity management.
- Recognizing extrapyramidal findings is essential for optimizing rehabilitation and management approaches for children with CZVS.
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.
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