Objective: To describe challenges to patient counseling in REM sleep behavior disorder (RBD).

Background: RBD is a significant risk factor for development of Parkinson disease (PD), dementia with Lewy bodies, and multiple system atrophy. Although >90% with RBD in some studies may phenoconvert, there are as yet no means of predicting individual prognosis and the inter-related questions of what testing to perform and how best to counsel these patients remain unanswered.

Method: We reviewed English-language peer-reviewed literature on RBD clinical management and prognosis and on counseling those at risk in other neurodegenerative diseases.

Results: Guidelines for managing RBD focus largely upon symptomatic management, although AASM Practice Guidelines suggest a baseline neurological exam with attention to cognition and “extrapyramidal signs”, in addition to counseling patients and families about the possibility of later neurodegenerative disease [1]. Despite enormous strides in understanding associated clinical features that may influence risk of phenoconversion [2], there are as yet no appropriate physician guidelines for work-up or follow up that reflect this knowledge. Moreover, known PD gene mutations and risk alleles have been demonstrated in some with RBD but there are no guidelines on genetic testing nor patient/family counseling.

Conclusion: Current guidelines for clinical care do not yet reflect recent advances in understanding RBD. Therefore, in addition to rigorous diagnostic, symptomatic treatment, and safety considerations, we suggest a focus upon: (1) screening for common comorbidities that are actionable, such as depression, and (2) administration of a standardized battery of motor and non-motor neurological features that are associated with neurodegenerative disorders. We will present a testing panel from a longitudinal observational cohort “REM Behavior Disorder Associations with Parkinson’s Disease Study (RAPiDS)” at our center, including evaluation of olfaction, coordination, and other testing that may be adapted for the clinic, and will also discuss counseling offered by our multi-disciplinary team. A remaining question is whether to, and in whom to, recommend genetic testing or neuroimaging. This will require attention to careful genetic counseling and knowledge of patient preferences that could be informed as a first step by experience in Alzheimer’s disease, PD, and other neurodegenerative disorders.

References: [1] Aurora RN et al (2010) J Clin Sleep Med 6: 85-95. Best Practice Guide for the Treatment of REM Sleep Behavior Disorder [2] Postuma RB et al (2019) Brain 142: 744-759. Risk and predictors of dementia and parkinsonism in idiopathic REM sleep behavior disorder: a multicenter study

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MDS Abstracts - https://www.mdsabstracts.org/abstract/in-the-shadow-of-parkinson-disease-what-to-tell-patients-with-rem-sleep-behavior-disorder/