Prion-Related Diseases Treatment & Management

Updated: Dec 18, 2017
  • Author: Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS; Chief Editor: Niranjan N Singh, MBBS, MD, DM, FAHS, FAANEM  more...
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Treatment

Medical Care

Discontinue any medication that could impair memory or cause confusion.

A number of potential therapeutic interventions are under current development, as discussed in Medication.

The transmissible spongiform encephalopathies are rapidly progressive neurodegenerative diseases, and outcome is inexorably fatal. No treatments have proven efficacious. Chemotherapeutic approaches have focused on blocking the conversion of the normal form of prion protein (PrPC) to its abnormal counterpart (PrPres) either by directly binding PrPC or PrPres, or by redistributing, sequestering, or downregulating PrPC, thus preventing its conversion. Others aim to enhance the clearance of PrPres. Other targets include accessory molecules such as the laminin receptor precursor, which influences conversion (or cell-signaling molecules) that may be required for pathogenesis. [84]

Other promising therapeutic approaches aimed to block the production of PrPSc are based on PrP RNA interference, passive or active immunization, dominant negative inhibition of PrPSc formation, as well as inhibition of interactions between PrPSc and other cofactors. An alternative strategy consists of combining gene therapy with cell therapy. [85]

Evidence is accumulating that PrPC may act as a receptor for protein aggregates and transduce neurotoxic signals in more common neurodegenerative disorders, such as Alzheimer's disease.

A general consensus on the role of PrPC and its physiological function within the brain is yet to be established. Lowering PrPC levels in the brain is predicted to be a powerful therapeutic strategy for the treatment of prion disease and the precise reason for PrPC's existence continues to remain enigmatic. 

Miguelez-Rodriguez et al. report that the existence of copathology significantly prolongs survival in patients with rapidly progressive dementia due to CJD. To further understand the molecular mechanisms behind prion diseases they suggest the study of major neurodegenerative-associated proteins in brains with CJD. [86]  

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Consultations

You may wish to consult with a neurologist and/or an infectious disease specialist.

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