Prion Diseases - 25/10/18

Doi : 10.1016/j.ncl.2018.07.005

Boon Lead Tee, MD, MS ^a,^b, Erika Mariana Longoria Ibarrola, MD ^a,^c, Michael D. Geschwind, MD, PhD ^d,^⁎
^a Global Brain Health Institute, University of California, San Francisco, 675 Nelson Rising Lane, Suite 190, San Francisco, CA 94518, USA
^b Department of Neurology, Buddhist Tzu Chi General Hospital, No. 707, Section 3, Zhong Yang Road, Hualien City, Hualien County 97002, Taiwan
^c Dementia Department, National Institute of Neurology and Neurosurgery Manuel Velasco Suarez, Av. Insurgentes Sur 3877, Col. La Fama, Del. Tlalpan, Ciudad de México. C.P. 14269, Mexico
^d Memory and Aging Center, Department of Neurology, University of California, San Francisco, 675 Nelson Rising Lane, Suite 190, San Francisco, CA 94158, USA

^∗Corresponding author. Memory and Aging Center, University of California, San Francisco, 675 Nelson Rising Lane, Suite 190, San Francisco, CA 94158.Memory and Aging CenterUniversity of California, San Francisco675 Nelson Rising LaneSuite 190San FranciscoCA94158

Résumé

Prions diseases are uniformly fatal neurodegenerative diseases that occur in sporadic, genetic, and acquired forms. Acquired prion diseases, caused by infectious transmission, are least common. Most prion diseases are not infectious, but occur spontaneously through misfolding of normal prion proteins or genetic mutations in the prion protein gene. Although most prion diseases are not caused by infection, they can be transmitted accidentally. Certain infection control protocols should be applied when handling central nervous system and other high-risk tissues. New diagnostic methods are improving premortem and earlier diagnosis. Treatment trials have not shown improved survival, but therapies may be available soon.

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Keywords : Creutzfeldt-Jakob disease, CJD, Jakob-Creutzfeldt disease, Rapidly progressive dementia, Protein misfolding disorders, Transmissible spongiform encephalopathies