Prion-Related Diseases Treatment & Management

Updated: Oct 27, 2014
  • 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

See the list below:

  • 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 (PrP C) to its abnormal counterpart (PrP res) either by directly binding PrP C or PrP res, or by redistributing, sequestering, or downregulating PrP C, thus preventing its conversion. Others aim to enhance the clearance of PrP res. Other targets include accessory molecules such as the laminin receptor precursor, which influences conversion (or cell-signaling molecules) that may be required for pathogenesis. [83]
  • Other promising therapeutic approaches aimed to block the production of PrP Sc are based on PrP RNA interference, passive or active immunization, dominant negative inhibition of PrP Sc formation, as well as inhibition of interactions between PrP Sc and other cofactors. An alternative strategy consists of combining gene therapy with cell therapy. [84]
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Consultations

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

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