An Unusual Case of Ischaemic Strokes and Cerebral Aneurysms with Varicella Zoster Encephalitis in a Child with Combined Immunodeficiency

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Journal of Investigative Medicine





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Introduction To describe a novel presentation of clinical issues with combined immunodeficiency (CID). These patients are predisposed to atypical infections, which can have varied presentations and complications. Case description A 9-year-old male with CID and progressive failure to thrive associated with gradual developmental regression presented with a 2 day history of vomiting, diarrhoea, right shoulder rash, cough, intermittent abdominal distension, and altered mental status. His immunodeficiency was comprised of severe lymphopenia, absolute lymphocyte count of 1059, and specific antibody deficiency with inability to mount responses despite immunisation. No sick contacts. Abdominal x-ray resulted concerning for ileus. Initial labs were unremarkable except for: hypocalcemia, mild acidosis, and mild thrombocytosis. Abdominal CT showed enteroenteric intussusception and adenopathy. He underwent successful surgical reduction of the intussusception, appendectomy for appendicitis, and lymph node biopsy. Broad antibacterial and antifungal coverage were started after developing haemodynamic instability and worsened altered mental status from sepsis 4 days post-op. He became increasingly less interactive, with abnormal posturing of extremities along with mouth automatisms. A broad infectious work up was performed. MRI of the brain revealed severe cerebral atrophy, bilateral acute on chronic strokes with aneurysmal changes of the left internal carotid and basilar arteries. PCR studies on CSF resulted positive for varicella zoster virus. Acyclovir therapy was initiated. Subsequent lumbar punctures were negative for VZV. Discussion Herpesvirus infections can cause cerebral vascular remodelling, occlusion and ischemia leading to encephalopathy, seizures and stroke. Varicella infection-reactivation is associated with immunological status; particularly in neonates. This is the first paediatric case reported with CID and non-exposure varicella virus encephalitis associated with ischaemic strokes secondary to CNS fibromuscular dysplasia. Conclusion In lymphopenic patients with mental status changes, it is important to put varicella on the differential diagnosis despite a lack of known exposure, or usual skin manifestations.


Medicine and Health Sciences



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