Rapid Eye Movement Sleep Behavior Disorder and Neurodegenerative Disease. 2015

Michael Joseph Howell, and Carlos Hugh Schenck
Department of Neurology, University of Minnesota, Minneapolis.

OBJECTIVE The dream enactment of rapid eye movement sleep behavior disorder (RBD) is often the first indication of an impending α-synuclein disorder, such as Parkinson disease, multiple-system atrophy, or dementia with Lewy bodies. OBJECTIVE To provide an overview of RBD from the onset of dream enactment through the emergence of a parkinsonian disorder. METHODS Peer-reviewed articles, including case reports, case series, retrospective reviews, prospective randomized trials, and basic science investigations, were identified in a PubMed search of articles on RBD from January 1, 1986, through July 31, 2014. RESULTS Under normal conditions, vivid dream mentation combined with skeletal muscle paralysis characterizes rapid eye movement sleep. In RBD, α-synuclein abnormalities in the brainstem disinhibit rapid eye movement sleep motor activity, leading to dream enactment. The behaviors of RBD are often theatrical, with complexity, aggression, and violence; fighting and fleeing actions can be injurious to patients as well as bed partners. Rapid eye movement sleep behavior disorder is distinguished from other parasomnias by clinical features and the demonstration of rapid eye movement sleep without atonia on polysomnography. Consistent with early neurodegeneration, patients with RBD demonstrate subtle motor, cognitive, and autonomic impairments. Approximately 50% of patients with spontaneous RBD will convert to a parkinsonian disorder within a decade. Ultimately, nearly all (81%-90%) patients with RBD develop a neurodegenerative disorder. Among patients with Parkinson disease, RBD predicts a non-tremor-predominant subtype, gait freezing, and an aggressive clinical course. The most commonly cited RBD treatments include low-dose clonazepam or high-dose melatonin taken orally at bedtime. CONCLUSIONS Treatment of RBD can prevent injury to patients and bed partners. Because RBD is a prodromal syndrome of Parkinson disease (or related disorder), it represents a unique opportunity for developing and testing disease-modifying therapies.

UI MeSH Term Description Entries
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D018450 Disease Progression The worsening and general progression of a disease over time. This concept is most often used for chronic and incurable diseases where the stage of the disease is an important determinant of therapy and prognosis. Clinical Course,Clinical Progression,Disease Exacerbation,Exacerbation, Disease,Progression, Clinical,Progression, Disease
D020187 REM Sleep Behavior Disorder A disorder characterized by episodes of vigorous and often violent motor activity during REM sleep (SLEEP, REM). The affected individual may inflict self injury or harm others, and is difficult to awaken from this condition. Episodes are usually followed by a vivid recollection of a dream that is consistent with the aggressive behavior. This condition primarily affects adult males. (From Adams et al., Principles of Neurology, 6th ed, p393) Rapid Eye Movement Sleep Behavior Disorder,Behavior Disorder, REM,Behavior Disorder, Rapid Eye Movement Sleep,REM Behavior Disorder,Behavior Disorders, REM,REM Behavior Disorders
D020734 Parkinsonian Disorders A group of disorders which feature impaired motor control characterized by bradykinesia, MUSCLE RIGIDITY; TREMOR; and postural instability. Parkinsonian diseases are generally divided into primary parkinsonism (see PARKINSON DISEASE), secondary parkinsonism (see PARKINSON DISEASE, SECONDARY) and inherited forms. These conditions are associated with dysfunction of dopaminergic or closely related motor integration neuronal pathways in the BASAL GANGLIA. Autosomal Recessive Juvenile Parkinsonism,Familial Juvenile Parkinsonism,Parkinsonian Syndrome,Parkinsonism,Parkinsonism, Experimental,Parkinsonism, Juvenile,Ramsay Hunt Paralysis Syndrome,Autosomal Dominant Juvenile Parkinson Disease,Autosomal Dominant Juvenile Parkinsonism,Autosomal Dominant Parkinsonism,Autosomal Recessive Juvenile Parkinson Disease,Autosomal Recessive Parkinsonism,Chromosome 6-Linked Autosomal Recessive Parkinsonism,Experimental Parkinson Disease,Experimental Parkinsonism,Experimental Parkinsonism, MPTP-Induced,Familial Parkinson Disease, Autosomal Recessive,Juvenile Parkinson Disease,Juvenile Parkinson Disease, Autosomal Dominant,Juvenile Parkinson Disease, Autosomal Recessive,Juvenile Parkinsonism, Autosomal Dominant,Juvenile Parkinsonism, Autosomal Recessive,MPTP-Induced Experimental Parkinsonism,Parkinson Disease 2,Parkinson Disease 2, Autosomal Recessive Juvenile,Parkinson Disease Autosomal Recessive, Early Onset,Parkinson Disease, Autosomal Dominant. Juvenile,Parkinson Disease, Experimental,Parkinson Disease, Familial, Autosomal Recessive,Parkinson Disease, Juvenile,Parkinson Disease, Juvenile, Autosomal Dominant,Parkinson Disease, Juvenile, Autosomal Recessive,Parkinsonian Diseases,Parkinsonian Syndromes,Parkinsonism, Early Onset, with Diurnal Fluctuation,Parkinsonism, Early-Onset, With Diurnal Fluctuation,Parkinsonism, Juvenile, Autosomal Dominant,Parkinsonism, Juvenile, Autosomal Recessive,Chromosome 6 Linked Autosomal Recessive Parkinsonism,Diseases, Experimental Parkinson,Dominant Parkinsonism, Autosomal,Experimental Parkinson Diseases,Experimental Parkinsonism, MPTP Induced,Experimental Parkinsonisms,Juvenile Parkinsonism,Juvenile Parkinsonism, Familial,Juvenile Parkinsonisms,MPTP Induced Experimental Parkinsonism,Parkinson Diseases, Experimental,Parkinsonism, Autosomal Dominant,Parkinsonism, Autosomal Recessive,Parkinsonism, Familial Juvenile,Parkinsonism, MPTP-Induced Experimental,Parkinsonisms, Experimental,Parkinsonisms, Juvenile,Recessive Parkinsonism, Autosomal
D062706 Prodromal Symptoms Clinical or physiological indicators that precede the onset of disease. Prodromal Characteristics,Prodromal Period,Prodromal Signs,Prodromal Stage,Prodromal States,Prodromal Syndromes,Characteristic, Prodromal,Characteristics, Prodromal,Period, Prodromal,Periods, Prodromal,Prodromal Characteristic,Prodromal Periods,Prodromal Sign,Prodromal Stages,Prodromal State,Prodromal Symptom,Prodromal Syndrome,Sign, Prodromal,Signs, Prodromal,Stage, Prodromal,Stages, Prodromal,State, Prodromal,States, Prodromal,Symptom, Prodromal,Symptoms, Prodromal,Syndrome, Prodromal,Syndromes, Prodromal

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