The neuropathology of fatal encephalomyelitis in human Borna virus infection.

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.

The patient with unexplained elevated serum liver enzymes.

The pattern of elevated serum liver enzymes in symptomatic or asymptomatic patients allows for an initial classification of liver diseases into cholestatic or hepatocellular diseases. A female patient with extrahepatic cholestasis due to segmental bile duct strictures and a localized mass lesion within the pancreas is presented. Although many diagnostic procedures were performed in this case the diagnosis was not obtained before surgical laparotomy was initiated with bioptic sampling from bile ducts, lymph nodes and pancreatic tissue. Microscopic examination of the specimen revealed extensive biliary and pancreatic scarring together with periductal infiltrates composed of lymphocytes and plasma cells consistent with sclerosing cholangitis in systemic autoimmune pancreatitis. The patient completely recovered upon treatment with prednisone and azathioprine. The difficult approach to the final diagnosis is discussed in light of established and modern diagnostic tools.