Marta Bertamino1, Sara Signa2,3, Marco Veneruso4, Giulia Prato5, Roberta Caorsi6,7, Giuseppe Losurdo8, Federica Teutonico9, Silvia Esposito10, Francesca Formica11, Nicola Tovaglieri12, Maria A Nagel13, Giulia Amico4,14, Alice Zanetti1, Domenico Tortora15, Andrea Rossi15,16, Paolo Moretti1, Marco Gattorno6, Angelo Ravelli7, Mariasavina Severino15. 1. Physical Medicine and Rehabilitation Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy. 2. DINOGMI, University of Genoa, Genoa, Italy. sarasigna@gaslini.org. 3. Center for Autoinflammatory Diseases and Immunodeficiencies, IRCCS Istituto Giannina Gaslini, Genoa, Italy. sarasigna@gaslini.org. 4. DINOGMI, University of Genoa, Genoa, Italy. 5. Child Neuropsychiatry Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy. 6. Center for Autoinflammatory Diseases and Immunodeficiencies, IRCCS Istituto Giannina Gaslini, Genoa, Italy. 7. Rheumatology Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy. 8. Infectious Diseases Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy. 9. Child Neuropsychiatry Unit, Niguarda Hospital, Milan, Italy. 10. Developmental Neurology Unit, IRCCS Istituto Neurologico Carlo Besta, Milan, Italy. 11. Acquired Brain Injury Unit, Scientific Institute IRCCS E. Medea, Bosisio Parini, Italy. 12. Department of Pediatrics, Niguarda Hospital, Milan, Italy. 13. Department of Neurology, University of Colorado, Boulder, USA. 14. Laboratory of Genetic and Genomics, IRCCS Istituto Giannina Gaslini, Genoa, Italy. 15. Neuroradiology Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy. 16. DISSAL, University of Genoa, Genoa, Italy.
Abstract
OBJECTIVE: Post-varicella arterial ischemic stroke (AIS) is considered an uncommon cause of pediatric stroke that is considered a self-limiting, monophasic disease. However, in a subset of patients, disease recurs; the prevalence of vasculopathy or AIS recurrence, severity of clinical outcomes, and standardized therapies have not been well characterized. Herein, we determined the clinical-neuroradiological features, long-term evolution, and relationship between acute phase treatment and vasculopathy recurrence in a pediatric population with post-varicella AIS. METHODS: Clinical, laboratory, and neuroradiological features of 22 children with post-varicella AIS between 2010 and 2019 (16 males, mean age at stroke 4 years, range 1.7-10) were reviewed. Statistical analyses were performed using χ2 and Fisher exact tests. RESULTS: Of the 22 cases, mean time from varicella to stroke was 4.5 months with 3 cases presenting more than 12 months after rash; 21 (95%) were not vaccinated for varicella; 3 (13.6%) had posterior circulation involvement; and 5 (22.7%) had AIS or vasculopathy recurrence, of which 4 recurred 6.1 months to 2.8 years after initial clinical onset. Recurrence was associated with lack of antiviral treatment during the first episode (p = 0.02). CONCLUSIONS: Post-varicella AIS can occur months after rash making diagnosis challenging. Because recurrent vasculopathy was seen predominantly in cases not treated with antiviral therapy during initial presentation, it is important to rapidly diagnose post-varicella AIS through clinical criteria and/or virological testing then treat with antivirals to prevent recurrence.
OBJECTIVE: Post-varicella arterial ischemic stroke (AIS) is considered an uncommon cause of pediatric stroke that is considered a self-limiting, monophasic disease. However, in a subset of patients, disease recurs; the prevalence of vasculopathy or AIS recurrence, severity of clinical outcomes, and standardized therapies have not been well characterized. Herein, we determined the clinical-neuroradiological features, long-term evolution, and relationship between acute phase treatment and vasculopathy recurrence in a pediatric population with post-varicella AIS. METHODS: Clinical, laboratory, and neuroradiological features of 22 children with post-varicella AIS between 2010 and 2019 (16 males, mean age at stroke 4 years, range 1.7-10) were reviewed. Statistical analyses were performed using χ2 and Fisher exact tests. RESULTS: Of the 22 cases, mean time from varicella to stroke was 4.5 months with 3 cases presenting more than 12 months after rash; 21 (95%) were not vaccinated for varicella; 3 (13.6%) had posterior circulation involvement; and 5 (22.7%) had AIS or vasculopathy recurrence, of which 4 recurred 6.1 months to 2.8 years after initial clinical onset. Recurrence was associated with lack of antiviral treatment during the first episode (p = 0.02). CONCLUSIONS: Post-varicella AIS can occur months after rash making diagnosis challenging. Because recurrent vasculopathy was seen predominantly in cases not treated with antiviral therapy during initial presentation, it is important to rapidly diagnose post-varicella AIS through clinical criteria and/or virological testing then treat with antivirals to prevent recurrence.
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