Primary progressive myoclonus of aging

Movement Disorders : Official Journal of the Movement Disorder Society
Maria Alvarez, John N Caviness

Abstract

Myoclonus in older individuals usually occurs in the context of associated neurologic features which allow the diagnosis of the underlying disorder. We encountered 7 patients with a newly recognized myoclonus syndrome; we use the term primary progressive myoclonus of aging (PPMA) for this syndrome. Our purpose was to characterize the clinical and electrophysiological properties of this syndrome. Our database was searched for the presence of "myoclonus" in the physical examination. Medical records and laboratory data were retrospectively reviewed, including electrophysiology data. We applied our criteria for PPMA: (1) asymmetric symptomatic action myoclonus, (2) >/=65 years of age, (3) cortical myoclonus physiology, (4) no dementia, (5) no associated features of defined neurodegenerative disorders, and (6) no secondary cause found. Seven patients fulfilled criteria. Age at presentation ranged from 70 to 87 years. Mean duration from myoclonus onset to last follow-up was 2.9 years. Electrophysiology showed positive-negative back-averaged transients, consistent with cortical myoclonus. No patient demonstrated dementia. Brain imaging in all cases was unremarkable. PPMA is a unique syndrome with characteristic findings that different...Continue Reading

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Citations

Oct 1, 2011·Neuropsychopharmacology : Official Publication of the American College of Neuropsychopharmacology·Yoland SmithMahlon R DeLong
Feb 23, 2011·Therapeutic Advances in Neurological Disorders·Maja KojovicKailash Bhatia
May 28, 2011·Movement Disorders : Official Journal of the Movement Disorder Society·Petra KatschnigKailash P Bhatia
May 30, 2009·Current Opinion in Neurology·Willi Gerschlager, Peter Brown
Jul 30, 2019·Continuum : Lifelong Learning in Neurology·John N Caviness
Aug 15, 2017·Brain Sciences·Olaf Eberhardt, Helge Topka

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