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 Volume 64, Number 5, March 08, 2005
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NEUROLOGY 2005;64:780-786
© 2005 American Academy of Neurology
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Idiopathic REM sleep behavior disorder

Toward a better nosologic definition

Maria Livia Fantini, MD, MSc, Luigi Ferini-Strambi, MD and Jacques Montplaisir, MD, PhD

From Centre d'étude du sommeil et des rythmes biologiques (Drs. Fantini and Montplaisir), Hôpital du Sacré-Coeur, Montréal; Sleep Disorders Center (Drs. Fantini and Ferini-Strambi), Department of Neurology, Università Vita-Salute San Raffaele, Milan, Italy; and Département de Psychiatrie (Dr. Montplaisir), Université de Montréal, Canada.

Address correspondence and reprint requests to Dr. Maria Livia Fantini, Sleep Disorders Center, Department of Neurology, IRSSC H San Raffaele, Via Stamira d'Ancona n. 20, 20127 Milan, Italy.

REM sleep behavior disorder (RBD) is a parasomnia characterized by a lack of motor inhibition during REM sleep leading to potentially harmful dream-enacting behaviors. RBD affects mainly older men and its prevalence in the general population is estimated to be around 0.5%. RBD may be idiopathic or associated with other neurologic disorders. A strong association between RBD and alpha-synucleinopathies has been recently observed, with the parasomnia often heralding the clinical onset of the neurodegenerative disease. The idiopathic form accounts for up to 60% of the cases reported in the three largest series of patients with RBD. Small clinical follow-up studies revealed that a proportion of these patients will eventually develop a parkinsonian syndrome or a dementia of Lewy bodies type in the years following the RBD diagnosis, while some patients will never show other neurologic signs within several decades from the RBD onset. Recent studies have looked at neurophysiologic and neuropsychological functions in idiopathic RBD and have found evidences of CNS dysfunction during both wakefulness and sleep. An impairment of the cortical activity, specific neuropsychological deficits, and signs of autonomic dysfunction have been observed in a variable proportion of these patients, challenging the concept of idiopathic RBD. Identifying subjects with a high risk of developing a neurodegenerative process may be crucial in order to develop early intervention strategies.




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