Creutzfeldt-Jacob disease (CJD)

Six years after receiving a dura mater allograft (Tutoplast brand, Tutogen Medical, Germany) during surgery for Arnold-Chiari malformation, a 39 year old woman from Colorado, USA,  developed head ache, neck pressure and visual changes. Two months later her symptoms progressed with impaired motor skills and mental status and she needed a wheel chair or a walker. Slow dysarthria, hand tremor, and memory loss worsened and she died 4 months after the onset of symptoms. Postmortem brain histology showed spongiosis and a PrP-res pattern that were diagnostic of CJD, iatrogenic type. The 57 year old German donor of the dura had dysarthria, ataxia, a broad based gait and aggressive behavioral changes. He died 6 weeks after onset of symptoms. No postmortem brain histology was performed. The dura from the German donor was processed in Alchua, Florida, USA, in 0.1% sodium hydroxide (lower than usual concentration) and no pooling of donors took place during processing.