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To the Editor:
Boeve and Saper propose that REM sleep behavior disorder (RBD) be considered an early clinical marker for neurodegenerative diseases with α-synuclein aggregation,1 a proposal advanced earlier by Boeve et al.2
While RBD can be a sentinel event in these diseases, it is not specific for synucleinopathy, even in the universe of neurodegenerative disorders. As noted in their editorial, RBD has been reported in patients with progressive supranuclear palsy.3 We have followed several families in southeastern New England with Machado-Joseph disease (MJD) (SCA-3). The ataxin-3 protein which accumulates in MJD has an expanded polyglutamine domain, and is biochemically distinct from α-synuclein. In our patient population, clinical features suggestive of RBD were identified in a high proportion of affected individuals.4 This association has been confirmed with polysomnography by Iranzo et …
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