PrP systemic amyloidosis

PrP systemic amyloidosis
Other namesPrion protein systemic amyloidosis, chronic diarrhea with HSAN, chronic diarrhea with hereditary sensory and autonomic neuropathy, prion disease associated with diarrhea and autonomic neuropathy
SpecialtyNeurology
Usual onsetAdulthood
CausesMutation of PRNP
TreatmentPalliative care
PrognosisLife expectancy up to 30 years after symptom development

PrP systemic amyloidosis is an extremely rare and unusual form of inherited prion disease. Unlike most prion diseases, PrP systemic amyloidosis is not isolated to the central nervous system. The prion amyloid has extensive peripheral involvement, finding its way to peripheral nerves and internal organs. The initial presentation of this disease involves chronic diarrhea and autonomic neuropathy before progressing into neurodegeneration.

This article is issued from Wikipedia. The text is licensed under Creative Commons - Attribution - Sharealike. Additional terms may apply for the media files.