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Previous Volume 349:1812-1820 November 6, 2003 Number 19 Next

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ABSTRACT

Background In patients with sporadic Creutzfeldt–Jakob disease, pathologic disease-associated prion protein (PrP^Sc) has been identified only in the central nervous system and olfactory-nerve tissue. Understanding the distribution of PrP^Sc in Creutzfeldt–Jakob disease is important for classification and diagnosis and perhaps even for prevention.

Methods We used a highly sensitive method of detection — involving the concentration of PrP^Sc by differential precipitation with sodium phosphotungstic acid, which increased the sensitivity of Western blot analysis by up to three orders of magnitude — to search for PrP^Sc in extraneural organs of 36 patients with sporadic Creutzfeldt–Jakob disease who died between 1996 and 2002.

Results PrP^Sc was present in the brain tissue of all patients. In addition, we found PrP^Sc in 10 of 28 spleen specimens and in 8 of 32 skeletal-muscle samples. Three patients had PrP^Sc in both spleen and muscle specimens. Patients with extraneural PrP^Sc had a significantly longer duration of disease and were more likely to have uncommon molecular variants of sporadic Creutzfeldt–Jakob disease than were patients without extraneural PrP^Sc.

Conclusions Using sensitive techniques, we identified extraneural deposition of PrP^Sc in spleen and muscle samples from approximately one third of patients who died with sporadic Creutzfeldt–Jakob disease. Extraneural PrP^Sc appears to correlate with a long duration of disease.

Source Information

From the Institute of Neuropathology and National Reference Center for Prion Diseases, University Hospital of Zurich, Zurich, Switzerland.

Dr. Glatzel and Mr. Abela contributed equally to the article.

Address reprint requests to Dr. Aguzzi at the Institute of Neuropathology, University Hospital of Zurich, Schmelzbergstr. 12, CH-8091 Zurich, Switzerland, or at adriano{at}pathol.unizh.ch.

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Extraneural Pathologic Prion Protein
Favereaux A., Perret-Liaudet A., Vital A., Aguzzi A., Glatzel M.
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N Engl J Med 2004; 350:732-733, Feb 12, 2004. Correspondence

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