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From the Centre d'etude du sommeil (J.F.G., M.V., J.M.-M., J.M., R.B.P.), Hopital du Sacre-Coeur, Montreal; Department of Neurology (R.B.P.), McGill University, Montreal General Hospital, Montreal, Quebec; Département de psychiatrie (J.F.G., J.M.), Université de Montréal, Canada; and Sleep Disorders Center (M.L.F.), Department of Neurology, Università Vita-Salute San Raffaele, Milan, Italy.
Address correspondence and reprint requests to Dr. Ronald B. Postuma, Department of Neurology, L7-305 Montreal General Hospital, 1650 Cedar Ave., Montreal, Quebec, Canada H3G 1A4 ronald.postuma{at}mcgill.ca
Objective: Idiopathic REM sleep behavior disorder (RBD) is a potential preclinical marker for the development of neurodegenerative diseases, particularly Parkinson disease (PD) and Lewy body dementia. However, the long-term risk of developing neurodegeneration in patients with idiopathic RBD has not been established. Obtaining an accurate picture of this risk is essential for counseling patients and for development of potential neuroprotective therapies.
Methods: We conducted a follow-up study of all patients seen at the sleep disorders laboratory at the Hôpital du Sacré Coeur with a diagnosis of idiopathic RBD. Diagnoses of parkinsonism and dementia were defined according to standard criteria. Survival curves were constructed to estimate the 5-, 10-, and 12-year risk of developing neurodegenerative disease.
Results: Of 113 patients, 93 (82%) met inclusion criteria. The mean age of participants was 65.4 years and 75 patients (80.4%) were men. Over the follow-up period, 26/93 patients developed a neurodegenerative disorder. A total of 14 patients developed PD, 7 developed Lewy body dementia, 4 developed dementia that met clinical criteria for AD, and 1 developed multiple system atrophy. The estimated 5-year risk of neurodegenerative disease was 17.7%, the 10-year risk was 40.6%, and the 12-year risk was 52.4%.
Conclusions: Although we have found a slightly lower risk than other reports, the risk of developing neurodegenerative disease in idiopathic REM sleep behavior disorder is substantial, with the majority of patients developing Parkinson disease and Lewy body dementia.
Abbreviations: DSM-IV = Diagnostic and Statistical Manual of Mental Disorders, 4th edition; LBD = Lewy body dementia; MMSE = Mini-Mental State Examination; MSA = multiple system atrophy; PD = Parkinson disease; PSG = polysomnogram; RBD = REM sleep behavior disorder; UPDRS = Unified Parkinson's Disease Rating Scale.
e-Pub ahead of print on December 24, 2008, at www.neurology.org.
Supported by a Canadian Institutes of Health Research grant to J.M. and J.F.G., and by a grant from the Fonds de la recherche en santé du Québec to R.P.
Disclosure: J.Y. Montplaisir received personal compensation as consultant (Boehringer Ingelheim, Servier, Shire Biochem), speaker (Boehringer, Shire), and received financial support for research activities from Sanofi Synthelabo, GlaxoSmithKline. R.B. Postuma, J.F. Gagnon, M. Vendette, M.L. Fantini, and J. Massicotte-Marquez have nothing to disclose.
Received May 21, 2008. Accepted in final form September 18, 2008.
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