Excessive fragmentary myoclonus in Machado-Joseph disease

Excessive fragmentary myoclonus in Machado-Joseph disease

Author Santos, Diogo Fernandes dos Autor UNIFESP Google Scholar
Pedroso, Jose Luiz Autor UNIFESP Google Scholar
Braga-Neto, Pedro Autor UNIFESP Google Scholar
Fontes Silva, Giselle Melo Autor UNIFESP Google Scholar
Coin de Carvalho, Luciane Bizari Autor UNIFESP Google Scholar
Prado, Lucila B. F. Autor UNIFESP Google Scholar
Barsottini, Orlando Graziani P. Autor UNIFESP Google Scholar
Prado, Gilmar Fernandes do Autor UNIFESP Google Scholar
Institution Universidade Federal de São Paulo (UNIFESP)
Abstract Objective: Machado-Joseph disease (MJD) is a neurodegenerative disease which usually presents several clinical findings including cerebellar ataxia and other extracerebellar features, such as Parkinsonism, dystonia, peripheral neuropathy, and lower motor neuron disease. Some data have demonstrated a high frequency of sleep disorders in these patients, including excessive daytime sleepiness (EDS), insomnia, obstructive sleep apnea (OSA), rapid eye movement (REM) sleep behavior disorder (RBD), and restless legs syndrome (RLS). Herein, we aimed to describe the high frequency of excessive fragmentary myoclonus (EFM) in MJD.Materials and methods: We recruited 44 patients with MJD and 44 healthy controls. All participants underwent an all-night polysomnography (PSG). EFM was evaluated and defined in accordance to the criteria of the American Academy of Sleep Medicine.Results: Half of the MJD patients (n = 22) had EFM diagnosed through PSG, though no healthy control participant presented this finding (P < .0001). in the MJD group, older participants and men had a higher frequency of EFM. There was no correlation between EFM and the following data: body mass index (BMI), apnea-hypopnea index (AHI), EDS, loss of atonia during REM sleep, periodic limb movements during sleep (PLMS), RLS, RBD, ataxia severity, the number of cytosine-adenine- guanine trinucleotide (CAG) repeats, disease duration, sleep efficiency, sleep fragmentation, and sleep stage percentages between patients with or without EFM.Conclusion: EFMis highly prevalent in patients with MJD. Our study demonstrates that EFM must be included in the clinical spectrum of sleep disorders in MJD patients. (C) 2014 Elsevier B.V. All rights reserved.
Keywords Machado-Joseph disease
Sleep disorders
Excessive fragmentary myoclonus
Spinocerebellar ataxia
Nonmotor symptoms
Language English
Date 2014-03-01
Published in Sleep Medicine. Amsterdam: Elsevier B.V., v. 15, n. 3, p. 355-358, 2014.
ISSN 1389-9457 (Sherpa/Romeo, impact factor)
Publisher Elsevier B.V.
Extent 355-358
Origin http://dx.doi.org/10.1016/j.sleep.2013.09.025
Access rights Closed access
Type Article
Web of Science ID WOS:000332769000012
URI http://repositorio.unifesp.br/handle/11600/37500

Show full item record


File Size Format View

There are no files associated with this item.

This item appears in the following Collection(s)




My Account