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BMC Proceedings

Open Access

Short-term therapy with corticosteroids can lead to progressive multifocal leukoencephalopathy

  • Fatima Barbara König2,
  • Marija Djukic1,
  • Joachim Gerber1,
  • Stefan Schweyer3,
  • Wolfgang Brück2,
  • Stefan Schreiber5,
  • Reinhardt Rüchel4 and
  • Holger Schmidth1Email author
BMC Proceedings20082(Suppl 1):P33

Published: 23 September 2008


SarcoidosisAspergillosisProgressive Multifocal LeukoencephalopathyProgressive Multifocal LeukoencephalopathyImmune Deficiency Syndrome

Progressive multifocal leukoencephalopathy (PML) is a disease of the central nervous system caused by JC virus infection. As a general rule PML patients suffer from an acquired or congenital immune deficiency syndrome. Here we present the history of a 78-year-old female patient who was treated with corticosteroids (25–75 mg Prednisolone) for suspected pulmonary sarcoidosis. The patient developed an acute brachio-facial hemiparesis. The cerebral imaging showed focal demyelination but an analysis of the cerebrospinal fluid did not point towards an inflammatory cause of these alterations. Assuming a cerebral manifestiation of the pre-diagnosed sarcoidosis, the dose of the glucocorticosteroids was increased. Soon after admission, the patient's pulmonary capacity deteriorated sharply and she required intubation with artificial ventilator therapy. She subsequently died due to complicating fungal pneumonia. While pathological findings could confirm the diagnosis of pulmonary aspergillosis, the neuropathological workup surprisingly revealed PML as the underlying reason for her neurological symptoms. This unfavourable disease course highlights that even short-term immunosuppression with routine dosages of glucocorticosteroids may lead to PML, despite the lack of indicative clues in the CSF.

Authors’ Affiliations

Department of Neurology, University Medicine Göttingen, Göttingen, Germany
Department of Neuropathology, University Medicine Göttingen, Göttingen, Germany
Department of Pathology, University Medicine Göttingen, Germany
Department of Microbacteriology, University Medicine Göttingen, Göttingen, Germany
Department of Neurology, Humboldt University Berlin, Berlin, Germany


© König et al; licensee BioMed Central Ltd. 2008

This article is published under license to BioMed Central Ltd.