Variably protease-sensitive prionopathy

Author:

Kim Stella H.,Yu Melissa M.,Strutt Adriana M.

Abstract

Purpose of reviewPrion diseases are rare neurodegenerative diseases that are caused by abnormal pathogenic agents and can affect both humans and animals. These diseases are categorized as sporadic, inherited, or acquired by infection. Clinical manifestations include psychiatric symptoms, cognitive impairment, and parkinsonism, which are similar to those of other prion diseases and frontotemporal dementia variants.Recent findingsMore recently, scientists discovered a new sporadic prion disease called variably protease-sensitive prionopathy.SummaryThe following case discusses a patient presenting with sudden onset and rapid decline in cognitive, neurobehavioral, and motor functioning and his clinical journey including treatment interventions and diagnostic confirmation.

Publisher

Ovid Technologies (Wolters Kluwer Health)

Subject

Neurology (clinical)

Reference18 articles.

1. Centers for Disease Control and Prevention (CDC). CDC's Diagnostic Criteria for Creutzfeldt-Jakob Disease (CJD). Available at: cdc.gov/prions/cjd/diagnostic-criteria.html. Accessed February 11, 2015.

2. Sporadic human prion diseases: molecular insights and diagnosis

3. Phenotypic variability of sporadic human prion disease and its molecular basis: past, present, and future

4. Variably protease-sensitive prionopathy, a unique prion variant with inefficient transmission properties;Diack;Emerg Infect Dis,2014

5. Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein

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