Polysomnographic diagnosis of REM sleep behavior disorder: a change is neededCesari, Matteo; Heidbreder, Anna; St. Louis, Erik K; Sixel-Döring, Friederike; Bliwise, Donald L; Baldelli, Luca; Bes, Frederik; Fantini, Maria Livia; Iranzo, Alex; Knudsen-Heier, Stine; Mayer, Geert; McCarter, Stuart; Nepozitek, Jiri; Pavlova, Milena; Provini, Federica; Santamaria, Joan; Sunwoo, Jun-Sang; Videnovic, Aleksandar; Högl, Birgit; Jennum, Poul; Christensen, Julie A E; Stefani, Ambra
doi: 10.1093/sleep/zsac276pmid: 36519899
We welcome the remarks by Ferri and colleagues regarding the practical application of the International rapid eye movement (REM) sleep Behavior Disorder (RBD) Study Group (IRBDSG) video-polysomnography (vPSG) guidelines for RBD diagnosis. As highlighted in their letter, the rich body of literature continuously confirming isolated RBD (iRBD) as a neurodegenerative disease in the prodromal phase is based on polysomnography (PSG)-confirmed RBD cases, underlying the “necessary objective role played by the PSG.” Starting from this shared view, we would like to drive attention to the fact that the list of concerns raised by Ferri et al. (with the exception of point 8) also applies to the current ICSD-3 criteria for RBD and for other sleep disorders, for example, narcolepsy [1]. No return to the previous ICSD-2 criteria is desirable, as it is clear that mimics [2, 3] and unawareness of disease [4] make a diagnosis of RBD based on clinical history not possible. Ferri et al. refer to the common knowledge that capturing an RBD episode is difficult, and support this affirmation with unpublished data from the center of one of the authors, based on the application of a scale [5] developed to assess RBD symptoms’ severity in patients already diagnosed with RBD, and not for identifying RBD episodes. Moreover, it is unclear if the entire video recording during REM sleep was analyzed, or if only video segments with an associated muscular activity or movement artifact were inspected. The latter method would miss some RBD episodes, particularly if muscular activity in the upper extremities is not recorded [6]. According to the IRBDSG guidelines, an RBD episode consists of one or more motor events and/or vocalizations that can be interpreted as related to dream enactment [7]. Thus, this can include not only complex but also simple movements (e.g. punching or more subtle movements) and vocalizations representing dream enactment behaviors. The vPSG guidelines, which provide minimal requirements for video acquisition, shorten the odds of capturing an RBD episode, as demonstrated by previous studies with high-quality standards for video acquisition and analysis. In a large cohort of iRBD patients from Barcelona, a second PSG (because of insufficient REM sleep, absent REM sleep without atonia, or absence of RBD episodes) was needed in 15.8% [4]. Another study analyzing movements and vocalizations in iRBD reported purposeful motor episodes in all patients except one, who, however, had intelligible sleep talking and had only 19 minutes of REM sleep [8]. In RBD patients with overt alpha-synucleinopathies assessed with two nights of vPSG, at least three complex RBD movements were visible each night [9]. Data from these previous studies are in line with the experience from several centers that, in most cases, at least one RBD episode can be detected each night, in particular when applying the IRBDSG vPSG guidelines; thus in most cases, repeated vPSG would not be necessary. We thank Ferri et al. for bringing up insurance coverage issues in the context of RBD diagnosis, which have far-reaching implications for RBD patients. Despite being a relevant point to be considered, health insurance coverage should not determine how guidelines are developed. On the contrary, guidelines provide evidence-based guidance and should guide changes in health insurance policies for routine diagnostic procedures. As an example, MRI is required to diagnose multiple sclerosis [10], despite not being readily available worldwide. The diagnostic and treatment implications of a diagnosis of multiple sclerosis do not allow diagnostic uncertainty. The same applies to iRBD, being a prodromal synucleinopathy with relevant clinical and ethical implications related to its diagnosis. In the absence of documented RBD episodes, a diagnosis of provisional RBD is possible based on a clinical judgment if REM sleep without atonia and a clinical history of RBD are present [7], similarly to the cases with typical behaviors during vPSG and clinical history of RBD, but with preserved atonia during REM sleep [1]. Considering the variability of REM sleep-related metrics needed to establish a diagnosis of RBD (i.e. REM sleep duration, REM sleep without atonia, and dream enactment behaviors), we acknowledge the different significance of missing REM sleep without atonia versus missing a RBD episode in reaching RBD diagnosis. Future revisions of the IRBDSG vPSG guidelines may improve this classification. However, it allows recognition and prompts symptomatic treatment of cases not fully meeting RBD diagnostic criteria, at the same time highlighting the need for future confirmation. The IRBDSG vPSG guidelines for RBD diagnosis underline the need to distinguish between provisional and definite RBD, due to the relevant implications of an RBD diagnosis [11, 12], both as isolated and in the context of other diseases. We disagree with Ferri et al. that the application of the IRBDSG guidelines would interfere with RBD diagnosis. On the contrary, the guidelines will improve accurate diagnosis and the quality of clinical and research data, ultimately benefitting patient care. Application of IRBDSG guidelines will not impede, but speed up advancements in RBD knowledge, paving a path for acquiring good quality and harmonized data to provide the basis of collaborative multicenter studies, and allowing reproducibility of findings. The recently published IRBDSG vPSG guidelines for RBD diagnosis represent the initial IRBDSG guideline, which will continue to evolve based on the scientific advances in RBD research. These authors believe that the IRBDSG vPSG guidelines for RBD diagnosis and the RBD medical and scientific community are poised to advocate for changes in health insurance coverage policies, as vPSG remains needed for a definite RBD diagnosis. Funding agencies and companies performing clinical trials should be aware of the importance of video documentation of RBD episodes for a definite RBD diagnosis to avoid selection bias impairing the quality of clinical trials and research studies. A definite iRBD diagnosis is particularly relevant due to the implications of iRBD as prodromal synucleinopathy. Disclosure Statement None related to this work. References 1. American Acedamy of Sleep Medicine. International Classification of Sleep Disorders . 3rd ed. Darien, IL: AASM ; 2015 . 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