REM sleep behavior disorder in narcolepsy: A secondary form or an intrinsic feature?

Abstract

Disrupted nighttime sleep is one of the pentad of symptoms defining Narcolepsy. REM sleep behavior disorder (RBD) largely contributes to night sleep disruption and narcolepsy is the most common cause of secondary RBD. However, RBD linked to narcolepsy (N-RBD) has been insufficiently characterized, leaving unsolved a number of issues. Indeed, it is still debated whether N-RBD is an intrinsic feature of narcolepsy, as indubitable for cataplexy, and therefore strictly linked to the cerebrospinal fluid hypocretin-1 (CSF hcrt-1) deficiency, or an associated feature, with a still unclear pathophysiology. The current review aims at rendering a comprehensive state-of-the-art of N-RBD, highlighting the open and unsettled topics. RBD reportedly affects 30-60% of patients with Narcolepsy type 1 (NT1), but it may be seen also in Narcolepsy type 2 (NT2). When compared to idiopathic/isolated RBD (iRBD), N-RBD has been reported to be characterized by less energetic and quieter episode, which however occur with the same probability in the first and the second part of the night and sometime even subcontinuously. N-RBD patients are generally younger than those with iRBD. N-RBD has been putatively linked to wake-sleep instability due to CSF hcrt-1 deficiency, but this latter by itself cannot explain completely the phenomenon as N-RBD has not been universally linked to low CSF hcrt-1 levels and it may be observed also in NT2. Therefore, other factors may probably play a role and further studies are needed to clarify this issue. In addition, therapeutic options have been poorly investigated.

Keywords: Disrupted sleep; Isolated RBD; Narcolepsy; REM sleep behaviour disorder; Secondary RBD.