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A novel subtype of Creutzfeldt-Jakob disease characterized by a small 6 kDa PrP fragment.

Krebs B, Bader B, Klehmet J, Grasbon-Frodl E, Oertel WH, Zerr I, Stricker S, Zschenderlein R, Kretzschmar HA

Center for Neuropathology and Prion Research, Ludwig-Maximilians-Universität München, National Reference Center for Transmissible Spongiform Encephalopathies, Feodor-Lynen-Str. 23, 81377, Munich, Germany, hans.kretzschmar@med.uni-muenchen.de.

We report on a novel subtype of Creutzfeldt-Jakob disease with a single proteinase K-resistant prion protein fragment of about 6 kDa in Western blots of brain homogenates. Clinically this patient showed a progressive spastic disorder and dementia over 3 years. No mutation of the prion protein gene was found. Since this patient had received a blood transfusion, an iatrogenic cause, albeit unlikely, cannot be ruled out. Future studies will have to be attentive to small prion protein fragments, which may cause or be associated with unusual clinical disease that might possibly only be diagnosed by immunoblotting of brain homogenates.

Published 13 July 2007 in Acta Neuropathol (Berl), 114(2): 195-9.
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