Case report: Varicella associated neuropsychiatric syndrome (VANS) in two pediatric cases

Devika Dahiya; Claudia Marques Matos; Ming Lim; Ines Madureira; Sofia Duarte; Susan Byrne; Thomas Rossor

doi:10.1016/j.bbih.2023.100602

Case report: Varicella associated neuropsychiatric syndrome (VANS) in two pediatric cases

Brain Behav Immun Health. 2023 Feb 11:28:100602. doi: 10.1016/j.bbih.2023.100602. eCollection 2023 Mar.

Authors

Devika Dahiya¹, Claudia Marques Matos², Ming Lim^{3

4}, Ines Madureira⁵, Sofia Duarte², Susan Byrne^{1

6

7}, Thomas Rossor^{3

4}

Affiliations

¹ The School of Medicine and Department of Paediatrics, Royal College of Surgeons in Ireland, Department of Paediatric Neurology, Dublin 2, Ireland.
² Pediatric Neurology Department, Hospital Dona Estefânia, Centro Hospitalar Universitário Lisboa Central, Lisboa, Portugal.
³ Children's Neurosciences, Evelina London Children's Hospital at Guy's and St Thomas' NHS Foundation Trust, King's Health Partners Academic Health Science Centre, London, UK.
⁴ Department Women and Childrens Health, School of Life Course Sciences (SoLCS), Kings College London, UK.
⁵ Pediatric Rheumatology Unit, Hospital Dona Estefânia, Centro Hospitalar Universitário Lisboa Central, Lisboa, Portugal.
⁶ Department of Paediatric Neurology, Children's Health Ireland at Crumlin, Dublin 12, Ireland.
⁷ FutureNeuro, Royal College of Surgeons in Ireland, Dublin 2, Ireland.

Abstract

Background: Viral or bacterial infections can trigger auto-immune inflammatory reactions and conditions in children. Self-reactivity arises due to similarities in molecular structures between pathogenic microorganisms and regular body structures with consequent immune-cross reactions. Reactivation of latent Varicella Zoster Virus (VZV) infections can cause neurological sequalae, including cerebellitis, post-herpetic neuralgias, meningo/encephalitis, vasculopathy and myelopathy. We propose a syndrome caused by auto-immune reactivity triggered by molecular mimicry between VZV and the brain, culminating in a post-infectious psychiatric syndrome with childhood VZV infections.

Case presentation: Two individuals, a 6-year-old male and 10-year-old female developed a neuro-psychiatric syndrome 3-6 weeks following a confirmed VZV infection with intrathecal oligoclonal bands. The 6-year-old male presented with a myasthenic syndrome, behavior deterioration and regression in school, he was poorly responsive to IVIG and risperidone, however had a pronounced response to steroid treatment. The 10-year-old female presented with marked insomnia, agitation, and behavioral regression as well as mild bradykinesia. A trial of neuroleptics and sedatives resulted in a mild unsustained reduction in psychomotor agitation and IVIG was also unsuccessful, however the patient was very responsive to steroid therapy.

Conclusion: Psychiatric syndromes with evidence of intrathecal inflammation temporally related to VZV infections that are responsive to immune modulation have not been described before. Here we report two cases demonstrating neuro-psychiatric symptoms following VZV infection, with evidence of persistent CNS inflammation following the resolution of infection, and response to immune modulation.

Keywords: ADHD - Attention Deficit Hyperactivity Disorder, CNS – Central Nervous System; Auto-immune encephalitis; CSF – Cerebral Spinal Fluid, IVIG – Intravenous Immunoglobulin; Cross reactivity; Molecular mimicry; NG – Nasogastric, NMDA - N-methyl-D-aspartate; Neuropsychiatric syndrome; PANDAS - Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcus), PANS - Pediatric Acute onset Neuropsychiatric Syndrome; PET- FDG - positron emission tomography - fluorodeoxyglucose, VZV - Varicella Zoster Virus; Pediatric varicella zoster virus.

Publication types

Case Reports