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Protease-resistant prion protein (PrP) has been considered diagnostic, and possibly causative, of all human prion diseases. These authors report identifying 11 patients, registered with the National Prion Disease Pathology Surveillance Center, with atypical prion disease that differed from previously defined cases in several ways, most notably in a preponderance of protease-sensitive PrP in the brain. The authors propose a new clinical entity: protease-sensitive prionopathy (PSPr).
The 11 patients described in this report presented with a milder and more slowly progressing phenotype than is typical in prion diseases, and some received a diagnosis of non-Alzheimer dementia. The authors suggest that PSPr is underreported because of this probab…