Hélio van der Linden1, Laura Silveira-Moriyama2, Vanessa van der Linden3, André Pessoa4, Kette Valente5, Jonathan Mink6, Alex Paciorkowski7. 1. Rehabilitation Center Dr. Henrique Santillo, Pediatric Neurology, Goiania, GO, Brazil; Neurology Institute, Goiania, GO, Brazil. Electronic address: vanderlindenh@yahoo.com.br. 2. Fundação Espírita Américo Bairral, Itapira, SP, Brazil; Movement Disorder Unit, Department of Neurology, State University of Campinas, Sao Paulo, Brazil. 3. Hospital Barão de Lucena, Pediatric Neurology, Recife, PE, Brazil. 4. Hospital Infantil Albert Sabin, Fortaleza, CE, Brazil; State University of Ceará, Fortaleza, CE, Brazil. 5. Laboratory of Clinical Neurophysiology, Department of Psychiatry, Clinic Hospital - University of Sao Paulo (USP), Brazil. 6. Department of Neurology, Pediatrics, and Neuroscience, University of Rochester Medical Center, Rochester, NY, USA. 7. Deptartment of Neurology, Pediatrics, Biomedical Genetics, and Neuroscience, University of Rochester Medical Center, Rochester, NY, USA.
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.
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.
Authors: Yasmin Notarbartolo di Villarosa do Amaral; Jocieli Malacarne; Paloma Glauca Brandão; Patrícia Brasil; Karin Nielsen-Saines; Maria Elisabeth Lopes Moreira Journal: Front Psychiatry Date: 2021-08-30 Impact factor: 5.435
Authors: Lavínia Schuler-Faccini; Miguel Del Campo; Alfredo García-Alix; Liana O Ventura; Juliano André Boquett; Vanessa van der Linden; André Pessoa; Hélio van der Linden Júnior; Camila V Ventura; Mariana Carvalho Leal; Thayne Woycinck Kowalski; Lais Rodrigues Gerzson; Carla Skilhan de Almeida; Lucélia Santi; Walter O Beys-da-Silva; André Quincozes-Santos; Jorge A Guimarães; Patricia P Garcez; Julia do Amaral Gomes; Fernanda Sales Luiz Vianna; André Anjos da Silva; Lucas Rosa Fraga; Maria Teresa Vieira Sanseverino; Alysson R Muotri; Rafael Lopes da Rosa; Alberto Mantovani Abeche; Clairton Marcolongo-Pereira; Diogo O Souza Journal: Front Genet Date: 2022-03-08 Impact factor: 4.599