Progressive multifokale Leukoenzephalopathie bei Wegener'scher Granulomatose unter Therapie mit Cyclosporin A

Summary A female patient with Wegener's granulomatosis developed severe bone marrow depression after two years treatment with cyclophosphamide. Corticosteroids alone could not sufficiently suppress disease activity, therefore additive therapy with Cyclosporin A was started. Four weeks later the patient developed a central nervous system disorder with affective disturbances and progressive somnolence. However, inspite of intensive diagnostic procedures, no definite diagnosis could be established. After another two months she died. Post-mortem-examination showed progressive multifocal leukoencephalopathy. An association between immunosuppressive therapy and reactivation of JC-Virus is suggested.

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Abkürzungsverzeichnis PML Progressive multifokale Leukoenzephalopathie - WG Wegener'sche Granulomatose - CyA Cyclosporin A - (C)CT (Craniale)Computertomographie - NMR Nuclear Magnetic Resonance (Kernspintomographic) - ZNS Zentralnervensystem - AIDS Acquired Immuno-deficiency Syndrome