Abstract
Prion diseases are a diverse group of neurodegenerative disorders of humans and animals. Generally these are recognized as rapidly progressive cognitive disorders with additional neurological signs such as myoclonus, ataxia, and pyramidal and extrapyramidal dysfunction. Inherited forms of prion disease may be highly atypical with clinical durations up to 20 years and can mimic any other neurodegenerative disease in the earlier stages. MRI, CSF, and genetic analyses are particularly helpful investigations, although tissue biopsy may be needed in some patients. Symptom management strategies, experimental therapeutics, and public health measures are discussed.
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Further Reading
Collinge J. Prion diseases of humans and animals: their causes and molecular basis. Annu Rev Neurosci 2001 January 1;24:519–50.
Collinge J. Molecular neurology of prion disease. Journal of Neurology Neurosurgery and Psychiatry 2005 July;76(7):906–19.
Mead S. Prion disease genetics. European Journal of Human Genetics 2006 March;14(3):273–81.
Zerr I, Kallenberg K, Summers DM, Romero C, Taratuto A, Heinemann U et al. Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Brain 2009 October;132:2659–68.
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© 2011 Springer-Verlag London Limited
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Mead, S., Rudge, P. (2011). Prion Diseases. In: Hardiman, O., Doherty, C. (eds) Neurodegenerative Disorders. Springer, London. https://doi.org/10.1007/978-1-84996-011-3_10
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DOI: https://doi.org/10.1007/978-1-84996-011-3_10
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