Brief Communications
 

Creutzfeldt-Jakob disease and inclusion body myositis: Abundant disease-associated prion protein in muscle

Gabor G. Kovacs, MD PhD 1 2, Elisabeth Lindeck-Pozza, MD 1, Leila Chimelli, MD, PhD 3, Abelardo Q. C. Araújo, MD, PhD 4, Alberto A. Gabbai, MD, PhD 5, Thomas Ströbel, PhD 1, Markus Glatzel, MD 6, Adriano Aguzzi, MD, PhD 6, Herbert Budka, MD 1 *

1Institute of Neurology, University of Vienna, and Austrian Reference Centre for Human Prion Diseases, Vienna, Austria 2National Institute of Psychiatry and Neurology, Budapest, Hungary 3Department of Pathology, School of Medicine, Federal University of Rio de Janeiro 4Department of Neurology, School of Medicine, Federal University of Rio de Janeiro 5Department of Neurology, School of Medicine, Federal University of Sao Paulo, Brazil 6Institute of Neuropathology, University Hospital of Zürich, Zürich, Switzerland

email: Herbert Budka (h.budka@akh-wien.ac.at)

^*Correspondence to Herbert Budka, Institute of Neurology, AKH 4J, Wühringer Gürtel 18-20, POB 48, A-1097 Vienna, Austria

Funded by:
 European Union (EU) Project; Grant Number: TSELAB QLK2-CT-2002-81523
 EU Concerted Action PRIONET; Grant Number: QLK2-2000-CT-00837

Abstract

Pathologicalprion protein (PrPSc) is the hallmark of prion diseases affecting primarily the central nervous system. Using immunohistochemistry, paraffin-embedded tissue blot, and Western blot, we demonstrated abundant PrPSc in the muscle of a patient with sporadic Creutzfeldt-Jakob disease and inclusion body myositis. Extraneural PrPC-PrPSc conversion in Creutzfeldt-Jakob disease appears to become prominent when PrPC is abundantly available as substrate, as in inclusion body myositis muscle.

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Received: 16 June 2003; Revised: 11 September 2003; Accepted: 11 September 2003