(Kiosk 6) Pediatric Stroke Secondary to Varicella Encephalitis: A Case Report

Case Diagnosis: Varicella Encephalitis

Case Description or Program Description: 17-year-old male with a history of depression, recurrent herpes labialis presented to acute care with 1 day history of altered mental status, left facial droop, urinary incontinence, and emesis. Physical exam notable for right sided eye medial deviation and anisocoria, left hemiparesis, positive pronator drift, upgoing right side plantar reflex, and significant distractibility.

Setting: Acute Inpatient Rehabilitation Hospital

Assessment/Results: On admission, an MRI revealed infarction in the right ACA territory, as well as areas surrounding the basal ganglia. A contrast-enhanced MRI suggested the presence of large vessel vasculitis or granulomatous disease. Further investigation through lumbar puncture indicated varicella encephalitis, and a CT scan was performed to check for a paraneoplastic syndrome, which came back negative for lymphoma. These results, along with low CD4 lymphocyte count and immunoglobulin levels, suggested primary immunodeficiency. Genetic workup suggested diagnosis of XMEN syndrome, which is associated with T-cell dysfunction. The patient was transitioned to an acute rehabilitation unit for gait instability, poor balance, restlessness, and impulsivity. Clonidine was initiated for dysexecutive syndrome, but was eventually replaced with propranolol and gabapentin, which led to significant improvement in impulsivity. The patient was discharged with functional independence for daily activities but required supervision for safety.

Discussion (relevance): Varicella encephalitis is a rare complication of Varicella-Zoster infection, with an estimated incidence of 0.1-0.2% in children and adolescents. The exact pathogenesis of varicella encephalitis is not fully understood, but it is thought to be related to direct viral invasion of the central nervous system or an immune-mediated response to the virus. In the setting of his primary immunodeficiency, it is suspected to be a result of reactivated virus from cold sores about 2 weeks prior to admission. Varicella encephalitis can cause significant cognitive and physical impairments, including difficulty with memory, attention, language, and balance.

Conclusions: A comprehensive rehabilitation program can lead to significant improvement in functional abilities and quality of life for patients with varicella encephalitis. Propranolol and Gabapentin may be effective tools for treatment of impulsivity and restlessness in pediatric varicella encephalitis.