CREUTZFELDT-JAKOB-DISEASE IN A RECIPIENT OF CADAVERIC DURAL GRAFT - FIRST OBSERVATION IN AUSTRIA

Dura mater grafts can lead to Creutzfeldt-Jakob disease (CJD) as late complication (dura-CJD). So far 61 dura-CJD cases have been described worldwide. We report here the first dura-CJD case in Austria. A 50-yea r-old man had a traumatic open frontobasal skull fracture with tearing of dura mater in 1977. Reconstructive surgery used Lyodura(R) (Braun Melsungen AG, BRD). Lyodura(R) was derived from pooled cadaveric dura. Ten years after the dural transplantation, the patient developed gait ataxia, paresthesia of both legs, myoclonus and visual disturbance. C T was unremarkable. EEG showed diffuse unspecific changes. The patient died 5 months after onset of disease. Neuropathological examination s howed typical histopathology of CJD. Immunocytochemistry detected typi cal synaptic type prion protein (PrP) deposits and scattered PrP plaqu es in cerebral and cerebellar cortex, basal ganglia and spinal cord. C erebellar white matter contained numerous PrP miniplaques. This patter n is unusual for sporadic CJD, but is similar to that in CJD after hum an growth hormone treatment. In our patient and 13/19 earlier describe d cases with dural graft covering the cerebrum (''central inoculation' '), cerebellar disturbance was the initial symptom. Therefore, cerebel lar signs are characteristic as initial symptoms in iatrogenic CJD, ir respective of central (cerebral dura mater graft) or peripheral inocul ation (e.g. human growth hormone treatment). These data do not support the hypothesis that primary cerebellar symptoms in iatrogenic CJD aft er peripheral inoculation reflect migration of the infectious agent fr om the periphery via spinal cord and cerebellum to the cerebrum.