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Late‐onset asymmetric myoclonus: An emerging syndrome
Author(s) -
Katschnig Petra,
Massano João,
Edwards Mark J.,
Schwingenschuh Petra,
Cordivari Carla,
Bhatia Kailash P.
Publication year - 2011
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.23676
Subject(s) - myoclonus , corticobasal degeneration , medicine , movement disorders , neuroscience , psychology , pediatrics , neurological disorder , degenerative disease , age of onset , central nervous system disease , dementia , pathology , disease
Background: Asymmetric cortical myoclonus is typically thought to be associated with either contralateral cortical structural lesions or degenerative disorders such as corticobasal degeneration when onset is in middle‐aged or aged adults. This view has been challenged after a recent case series brought to light a syndrome of senile‐onset, asymmetric cortical myoclonus not associated with any such identifiable disorders, thus, named “primary progressive myoclonus of aging.” This is rare and no other reports have been published; hence, further such cases need to be highlighted. Case reports: Here, we describe 3 patients with some similarities, namely, adult‐onset, asymmetric myoclonus that is most likely to be cortical, with an unremarkable thorough diagnostic workup, but with younger age at onset and longer follow‐up time. Conclusions: This report expands on previous phenotypical descriptions attempting to further develop and refine this possible diagnostic entity. © 2011 Movement Disorder Society