The neuropathology of fatal encephalomyelitis in human Borna virus infection

Friederike Liesche, Viktoria Ruf, Saida Zoubaa, Gwendolyn Kaletka, Marco Rosati, Dennis Rubbenstroth, Christiane Herden, Lutz Goehring, Silke Wunderlich, Miguel Frederic Wachter, Georg Rieder, Ines Lichtmannegger, Willibald Permanetter, Josef G. Heckmann, Klemens Angstwurm, Bernhard Neumann, Bruno Märkl, Stefan Haschka, Hans Helmut Niller, Barbara SchmidtJonathan Jantsch, Christoph Brochhausen, Kore Schlottau, Arnt Ebinger, Bernhard Hemmer, Markus J. Riemenschneider, Jochen Herms, Martin Beer, Kaspar Matiasek, Jürgen Schlegel

Research output: Contribution to journalArticlepeer-review

43 Scopus citations


After many years of controversy, there is now recent and solid evidence that classical Borna disease virus 1 (BoDV-1) can infect humans. On the basis of six brain autopsies, we provide the first systematic overview on BoDV-1 tissue distribution and the lesion pattern in fatal BoDV-1-induced encephalitis. All brains revealed a non-purulent, lymphocytic sclerosing panencephalomyelitis with detection of BoDV-1-typical eosinophilic, spherical intranuclear Joest–Degen inclusion bodies. While the composition of histopathological changes was constant, the inflammatory distribution pattern varied interindividually, affecting predominantly the basal nuclei in two patients, hippocampus in one patient, whereas two patients showed a more diffuse distribution. By immunohistochemistry and RNA in situ hybridization, BoDV-1 was detected in all examined brain tissue samples. Furthermore, infection of the peripheral nervous system was observed. This study aims at raising awareness to human bornavirus encephalitis as differential diagnosis in lymphocytic sclerosing panencephalomyelitis. A higher attention to human BoDV-1 infection by health professionals may likely increase the detection of more cases and foster a clearer picture of the disease.

Original languageEnglish
Pages (from-to)653-665
Number of pages13
JournalActa Neuropathologica
Issue number4
StatePublished - Oct 1 2019

Bibliographical note

Publisher Copyright:
© 2019, The Author(s).


  • Borna disease virus (BoDV-1)
  • Bornavirus
  • Encephalitis
  • Virus
  • Zoonosis

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Clinical Neurology
  • Cellular and Molecular Neuroscience


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