REM sleep behavior disorder: Mimics and variants.

Rapid eye movement (REM) sleep behavior disorder (RBD) is a parasomnia with dream-enactment behaviors occurring during REM sleep and associated with the lack of the physiological REM sleep muscle atonia. It can be isolated and secondary to other neurological or medical conditions. Isolated RBD heralds in most cases a neurodegenerative condition due to an underlying synucleinopathy and consequently its recognition is crucial for prognostic implications. REM sleep without atonia on polysomnography is a mandatory diagnostic criterion. Different conditions may mimic RBD, the most frequent being obstructive sleep apnea during sleep, non-REM parasomnia, and sleep-related hypermotor epilepsy. These diseases might also be comorbid with RBD, challenging the evaluation of disease severity, the treatment choices and the response to treatment evaluation. Video-PSG is the gold standard for a correct diagnosis and will distinguish between different or comorbid sleep disorders. Careful history taking together with actigraphy may give important clues for the differential diagnosis. The extreme boundaries of RBD might also be seen in more severe and complex conditions like status dissociatus or in the sleep disorders' scenario of anti IgLON5 disease, but in the latter both clinical and neurophysiological features will differ. A step-by-step approach is suggested to guide the differential diagnosis.

Periodic Limb Movements During Sleep Mimicking REM Sleep Behavior Disorder: A New Form of Periodic Limb Movement Disorder.

Study Objectives: To describe a group of patients referred because of abnormal sleep behaviors that were suggestive of rapid eye movement (REM) sleep behavior disorder (RBD) in whom video-polysomnography ruled out RBD and showed the reported behaviors associated with vigorous periodic limb movements during sleep (PLMS). Aims and Methods: Clinical history and video-polysomnography review of patients identified during routine visits in a sleep center. Results: Patients were 15 men and 2 women with a median age of 66 (range: 48-77) years. Reported sleep behaviors were kicking (n = 17), punching (n = 16), gesticulating (n = 8), falling out of bed (n = 5), assaulting the bed partner (n = 2), talking (n = 15), and shouting (n = 10). Behaviors resulted in injuries in 3 bed partners and 1 patient. Twelve (70.6%) patients were not aware of displaying abnormal sleep behaviors that were only noticed by their bed partners. Ten (58.8%) patients recalled unpleasant dreams such as being attacked or chased. Video-polysomnography showed (1) frequent and vigorous stereotyped PLMS involving the lower limbs, upper limbs, and trunk (median PLMS index 61.2; median PLMS index in NREM sleep 61.9; during REM sleep only 8 patients had PLMS and their median PLMS index in REM sleep was 39.5); (2) abnormal behaviors (e.g., punching, groaning) during some of the arousals that immediately followed PLMS in NREM sleep; and (3) ruled out RBD and other sleep disorders such as obstructive sleep apnea. Dopaminergic agents were prescribed in 14 out of the 17 patients and resulted in improvement of abnormal sleep behaviors and unpleasant dreams in all of them. After dopaminergic treatment, follow-up video-polysomnography in 7 patients showed a decrease in the median PLMS index from baseline (108.9 vs. 19.2, p = .002) and absence of abnormal behaviors during the arousals. Conclusions: Abnormal sleep behaviors and unpleasant dreams simulating RBD symptomatology may occur in patients with severe PLMS. In these cases, video-polysomnography ruled out RBD and identified prominent PLMS followed by arousals containing abnormal behaviors. Our cases represent an objectively documented subtype of periodic limb movement disorder causing abnormal sleep behaviors.