Chapter 9
Prion diseases and rapidly progressive dementias
Leonel T. Takada,
Michael D. Geschwind,
Leonel T. Takada
University of Sao Paulo Medical School, Sao Paulo, Brazil
Search for more papers by this authorMichael D. Geschwind
University of California, San Francisco, San Francisco, CA, USA
Search for more papers by this authorLeonel T. Takada,
Michael D. Geschwind,
Leonel T. Takada
University of Sao Paulo Medical School, Sao Paulo, Brazil
Search for more papers by this authorMichael D. Geschwind
University of California, San Francisco, San Francisco, CA, USA
Search for more papers by this authorBook Editor(s):Michael D. Geschwind MD PhD,
Caroline Racine Belkoura PhD,
Michael D. Geschwind MD PhD
Memory and Aging Center, Department of Neurology, University of California, San Francisco, San Francisco, CA, USA
Search for more papers by this authorCaroline Racine Belkoura PhD
Department of Neurological Surgery, University of California, San Francisco, San Francisco, CA, USA
Search for more papers by this authorSummary
Rapidly progressive dementias (RPDs) are cognitive disorders with onset to dementia occurring in less than 2 years, although commonly dementia occurs over much shorter time periods, such as months. Human prion diseases (PrDs), such as Jakob–Creutzfeldt disease (CJD), are one of the most common etiologies of RPD and occur in sporadic, genetic, and acquired forms. The clinical features at presentation vary widely (e.g., cerebellar, motor, cognitive, behavioral, etc.). Diffusion-weighted brain MRI is currently the most sensitive test for diagnosis of CJD, whereas CSF and EEG can help confirm the diagnosis (although a purported highly specific CSF test is now clinically available in some countries). Treatment for CJD is currently symptomatic and there are no disease-modifying therapies. CJD is often confused with many other RPDs and vice versa. The acronym VITAMINS is a useful tool to assist in the differential diagnosis of RPD, particularly to identify potential treatable causes, which are important to catch as early as possible in order to provide appropriate therapies and minimize neurological decline.
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