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A Rapidly Progressive Movement Disorder 

A Rapidly Progressive Movement Disorder
Chapter:
A Rapidly Progressive Movement Disorder
Author(s):

Susan H. Fox

and Marina Picillo

DOI:
10.1093/med/9780190607555.003.0028
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date: 15 April 2021

Prion diseases are a rare group of transmissible and untreatable encephalopathies that ultimately result in death after a short and rapidly progressive illness. The clinical features are variable but share a mix of cortical and subcortical features and a tendency for worsening at a speed that is typically faster than the monthly or yearly change seen in degenerative forms of dementia. Movement disorders represent a prominent feature of prion diseases and include cerebellar and extrapyramidal symptoms. Myoclonus is by far the most common involuntary movement in prion diseases. An awareness of the diagnosis is important to avoid the risk of iatrogenic transmission and to allow a discussion about prognosis with family and relatives.

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