Infectious diseases and therapy · 2026
Long-Term Survival of Borna Disease Virus 1 (BoDV-1)-Encephalitis: An Exceptional Case with Valuable Insights for Pathophysiology and Disease Management.
СтатьяНазвание и аннотация: Перевод в очереди
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Grabova Denis, Wilkens Ida, Klein Matthias, Heck Suzette, Ruf Viktoria, Roeber Sigrun, Schmutzer-Sondergeld Michael, Brendel Matthias, Forbrig Robert, Klauss Helena, Katzdobler Sabrina, Höglinger Günter U, Dimitriadis Konstantin, Straube Andreas, Bauswein Markus, Kinzig Martina, Sörgel Fritz, Tappe Dennis, Masouris Ilias, Schöberl Florian
Аннотация
INTRODUCTION: Borna disease virus 1 (BoDV-1) encephalitis has a (sub)acute and mostly fatal course. Initial flu-like symptoms are typically followed by rapid progressive panencephalitis and death within weeks. No curative therapy exists so far. We present a long-term survivor with distinctive clinical, imaging, and pathophysiological features.
CASE PRESENTATION: A previously healthy male in his fifties was admitted in summer 2023 with (sub)acute encephalitis (cerebrospinal fluid, CSF: 132 cells/µl, no pathogen detection). Brain magnetic resonance imaging (MRI) demonstrated vasogenic edema affecting basal ganglia, temporal and limbic regions bilaterally. Despite high-dose steroids, the clinical condition of the patient slowly worsened. Three months later, BoDV-1 reactive antibodies were detected by elevated titers in CSF (1:320) and serum (1:5120). Treatment with favipiravir, corticosteroids, and mycophenolate mofetil (MMF) was initiated. After recovery for months, the patient developed progressive bilateral optic atrophy, cognitive decline, and a sleep disorder resembling Kleine-Levin syndrome. Translocator protein (TSPO)-PET revealed widespread microglial activation, confirmed by targeted cortical biopsy. Immunosuppressive therapy was escalated with anakinra, cyclophosphamide, and intrathecal dexamethasone, achieving temporary stabilization. In the summer of 2025, a severe brainstem syndrome emerged, accompanied by increased TSPO uptake in the brainstem and detection of BoDV-1 RNA in CSF for the first time. Despite another treatment with favipiravir and high-dose corticosteroids, the patient remained in a minimally conscious state.
DISCUSSION: This exceptional case with long-term survival in BoDV-1 encephalitis gives important insights: BoDV1-associated neuroinflammation leading to a slowly progressive decline can be visualized by TSPO-PET. Late BoDV-1 RNA detection and subacute re-exacerbation after prolonged survival provides evidence that BoDV-1 persists in human brain tissue.
CASE PRESENTATION: A previously healthy male in his fifties was admitted in summer 2023 with (sub)acute encephalitis (cerebrospinal fluid, CSF: 132 cells/µl, no pathogen detection). Brain magnetic resonance imaging (MRI) demonstrated vasogenic edema affecting basal ganglia, temporal and limbic regions bilaterally. Despite high-dose steroids, the clinical condition of the patient slowly worsened. Three months later, BoDV-1 reactive antibodies were detected by elevated titers in CSF (1:320) and serum (1:5120). Treatment with favipiravir, corticosteroids, and mycophenolate mofetil (MMF) was initiated. After recovery for months, the patient developed progressive bilateral optic atrophy, cognitive decline, and a sleep disorder resembling Kleine-Levin syndrome. Translocator protein (TSPO)-PET revealed widespread microglial activation, confirmed by targeted cortical biopsy. Immunosuppressive therapy was escalated with anakinra, cyclophosphamide, and intrathecal dexamethasone, achieving temporary stabilization. In the summer of 2025, a severe brainstem syndrome emerged, accompanied by increased TSPO uptake in the brainstem and detection of BoDV-1 RNA in CSF for the first time. Despite another treatment with favipiravir and high-dose corticosteroids, the patient remained in a minimally conscious state.
DISCUSSION: This exceptional case with long-term survival in BoDV-1 encephalitis gives important insights: BoDV1-associated neuroinflammation leading to a slowly progressive decline can be visualized by TSPO-PET. Late BoDV-1 RNA detection and subacute re-exacerbation after prolonged survival provides evidence that BoDV-1 persists in human brain tissue.