Overwhelming Bornavirus Encephalitis Resulting in a Lethal Outcome

Background: Encephalitis due to Borna disease virus 1 (BoDV-1) is a zoonosis associated with high lethality and endemic in parts of Germany and bordering countries. Over the last 3 years, BoDV-1 infections in humans have been described at an increasing frequency.

Methods: We report on two patients who died due to overwhelming encephalitis. The diagnosis of the BoDV-1 encephalitis was made post mortem on autopsy material of the brain.

Results: Patient 1: Following nonspecific symptoms, a 13-year-old girl was admitted with symptoms of acute disorientation, fever, and neurological signs such as progressive somnolence with missing swallowing reflexes, dysarthria, and gait disorder.

CSF exam revealed a lymphomonocytic pleocytosis. The detailed pathogen diagnostics remained inconspicuous, as did an evaluation of antineuronal antibodies. The cMRI demonstrated increasing signal alterations of the basal ganglia and a pronounced brain edema. After 3 weeks of intensive medical care, the patient died due to a lower entrapment of the brain stem.

Post mortem immunohistochemical examination of the brain revealed cells with positive staining against the nucleoprotein of BoDV-1. This was confirmed by detection of BoDV-1 RNA by in-situ hybridization and PCR.

Patient 2: A 12-year-old boy was admitted to our intensive care unit after non-specific symptoms such as fever and neurological deterioration (somnolence, dysarthria, gait disorder). Clinical, chemistry and imaging findings as well as the course of events were nearly identical to patient 1. The boy died due to therapy refractory raised intracranial pressure.

A total of 4 years later, the autopsy material of the brain revealed retrospectively Borna disease virus encephalitis.

Conclusion: In any encephalitis with an overwhelming course, a Borna disease virus infection should be suspected especially in the endemic areas of Southern Bavaria, Thuringia, and Saxony/Saxony-Anhalt, as well as parts of Austria, Liechtenstein, and Switzerland. The corresponding diagnosis in the CSF and serum should be initiated.

Publikationsverlauf

Artikel online veröffentlicht:
28. Oktober 2021