Rapid eye movement sleep behavior disorder: devising controlled active treatment studies for symptomatic and neuroprotective therapy—a consensus statement from the International Rapid Eye Movement Sleep Behavior Disorder Study Group

Schenck, Carlos H.; Montplaisir, J.; Frauscher, Birgit; Högl, Birgit; Gagnon, Jean‐François; Postuma, Ronald B.; Šonka, Karel; Jennum, Poul; Partinen, Markku; Arnulf, Isabelle; Cock, Valérie Cochen De; Dauvilliers, Yves; Luppi, Pierre‐Hervé; Heidbreder, Anna; Mayer, Geert; Sixel‐Döring, Friederike; Trenkwalder, Claudia; Unger, Marcus M.; Young, Peter; Wing, Yun Kwok; Ferini‐Strambi, Luigi; Ferri, Raffaele; Plazzi, Giuseppe; Zucconi, Marco; Inoue, Yuichi; Iranzo, Álex; Santamaría, Joan; Bassetti, C.; Möller, Jens Carsten; Boeve, Bradley F.; Lai, Yuan-Yang; Pavlova, Milena; Saper, Clifford B.; Schmidt, Péter; Siegel, Judith M.; Singer, Carlos; Louis, Erik K. St.; Videnović, Aleksandar; Oertel, Wolfgang H.

doi:10.1016/j.sleep.2013.02.016

Cited by 204 publications

(139 citation statements)

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“…WD is a disease of metabolism; but it shares with PD not only the clinical phenomenology but also a similar topographical distribution of basal nuclei and brainstem lesions and the accumulation of metals in these locations 1 . The tegmental lesions in MRI and TCS do not prove a necessary causal connection between WD and RBD, as it is already established in PD and RBD 10 . But the presented cases provide evidence for both RBD as a phenomenon in early neurodegeneration, also in WD, and for tegmental lesions as a possible causal factor for RBD.…”

Section: Discussionmentioning

confidence: 77%

“…As such it could be of further interest in the recent efforts for development of symptomatic and neuroprotective therapy in RBD as early manifestation of PD and related syndromes 10 . Awareness of the possibility of RBD as presenting clinical symptom of WD allows for early diagnosis of special cases.…”

Section: Discussionmentioning

confidence: 99%

“…Early neuroprotective treatment of RBD in order to avoid or delay neurodegeneration in a-synucleinopathies has been one of the major challenges of neuropharmacological research 10 . In WD, highly effective treatment options are available, which allow slowing down or almost stopping the cascade of brain tissue destruction, provided the treatment is applied as soon as any disease activity appears 11 .…”

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confidence: 99%

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Wilson?s disease presenting as rapid eye movement sleep behavior disorder: a possible window to early treatment

Tribl

Bor‐Seng‐Shu

Trindade

et al. 2014

Arq. Neuro-Psiquiatr.

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Objective: To describe characteristics of REM sleep behavior disorder in Wilson's disease. Method: Questionnaire-based interviews (patients and relatives), neurological examinations, two-week prospective dream-diary, video-polysomnography, transcranial sonography, MRI. Results: Four Wilson's disease cases with REM sleep behavior disorder were described; three had REM sleep behavior disorder as initial symptom. All showed mesencephalic tegmental/tectal sonographic hyperechogenicities and two presented ponto-mesencephalic tegmental MRI hyperintensities. Conclusion: This first description of REM sleep behavior disorder in Wilson's disease in literature documents REM sleep behavior disorder as a possible presenting symptom of Wilson's disease and adds further evidence to the parallelism of Parkinson's disease and Wilson's disease in phenotype and brainstem topography, which ought to be further studied. REM sleep behavior disorder has prognostic relevance for neurodegeneration in a-synucleinopathies. In Wilson's disease, usefulness of early diagnosis and treatment are already well established. REM sleep behavior disorder in Wilson's disease offers a possible theoretical model for potential early treatment in this extrapyramidal and brainstem paradigm syndrome, previewing the possibility of neuroprotective treatment for REM sleep behavior disorder in "pre-clinical" Parkinson's disease.Keywords: Wilson's disease, parkinsonian syndromes, rapid eye movement sleep behavior disorder, parasomnias, tegmentum. RESUMOObjetivo: Descrever características do transtorno comportamental do sono REM (TCSR) na doença de Wilson (DW). Método: Aplicação de entrevistas, vídeo-polissonografia, sonografia transcraniana (STC), ressonância magnética (RM), diário de sonhos. Resultados: Descrevemos quatro casos de DW com TCSR. Três apresentaram o TCSR como primeira manifestação. Todos mostraram hiperecogenicidades mesencefálicas na STC, dois apresentaram hiperintensidades ponto-mesencefálicas na RM. Conclusão: Esta é a primeira descrição do TCSR na DW. Relatamos o TCSR como um sintoma inicial da DW. Acrescentamos prova para o paralelismo entre a doença de Parkinson e DW, com relação aos fenótipos e localização das lesões cerebrais. Nas alfa-sinucleinopatias, o TCSR tem relevância prognóstica quanto à neurodegeneração. Na DW, já conhecemos a importância de diagnóstico e tratamento precoces. O TCSR na DW oferece um modelo para antecipar o tratamento desta síndrome de acometimento dos núcleos basais e tronco, vislumbrando a possibilidade de tratamento neuroprotetor para a fase "pré-clínica" da DP.Palavras-chave: doença de Wilson, síndromes parkinsonianas, transtorno comportamental do sono REM, parassonias, tegmento.Wilson's disease (WD) causes tissue copper storage, especially in brain, cornea, and liver. Early stages of metal deposits in basal ganglia and brainstem in WD may be detected by transcranial sonography (TCS) 1 . WD's patients frequently suffer from disorders of sleep 2,3,4 , including altered REM sleep 2 , nevertheless REM slee...

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Section: Discussionmentioning

confidence: 77%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

See 1 more Smart Citation

Wilson?s disease presenting as rapid eye movement sleep behavior disorder: a possible window to early treatment

Tribl

Bor‐Seng‐Shu

Trindade

et al. 2014

Arq. Neuro-Psiquiatr.

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“…Twenty‐one subjects with RBD (polysomnographically confirmed18) were evaluated with 18 F‐FDG‐PET, DAT‐SPECT, and olfactory testing. Per inclusion criteria, RBD subjects did not have parkinsonism19 or DLB20 at the time of the study.…”

Section: Methodsmentioning

confidence: 99%

FDG PET, dopamine transporter SPECT, and olfaction: Combining biomarkers in REM sleep behavior disorder

et al. 2017

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BackgroundIdiopathic REM sleep behavior disorder is a prodromal stage of Parkinson's disease and dementia with Lewy bodies. Hyposmia, reduced dopamine transporter binding, and expression of the brain metabolic PD‐related pattern were each associated with increased risk of conversion to PD. The objective of this study was to study the relationship between the PD‐related pattern, dopamine transporter binding, and olfaction in idiopathic REM sleep behavior disorder.MethodsIn this cross‐sectional study, 21 idiopathic REM sleep behavior disorder subjects underwent 18F‐fluorodeoxyglucose PET, dopamine transporter imaging, and olfactory testing. For reference, we included 18F‐fluorodeoxyglucose PET data of 19 controls, 20 PD patients, and 22 patients with dementia with Lewy bodies. PD‐related pattern expression z‐scores were computed from all PET scans.ResultsPD‐related pattern expression was higher in idiopathic REM sleep behavior disorder subjects compared with controls (P = 0.048), but lower compared with PD (P = 0.001) and dementia with Lewy bodies (P < 0.0001). PD‐related pattern expression was higher in idiopathic REM sleep behavior disorder subjects with hyposmia and in subjects with an abnormal dopamine transporter scan (P < 0.05, uncorrected).ConclusionPD‐related pattern expression, dopamine transporter binding, and olfaction may provide complementary information for predicting phenoconversion. © 2017 The Authors. Movement Disorders published by Wiley Periodicals, Inc. on behalf of International Parkinson and Movement Disorder Society.

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“…Based on the fact that iRBD diagnosed by polysomnography is by far the most specific prodromal sign of an evolving underlying synuclein disease (33), or even an indication of ongoing α-synuclein disease (4), patients with iRBD are a potentially important target population to study in upcoming trials, not only with substances to alleviate symptoms of RBD (34), but also with potentially diseasemodifying or neuroprotective substances. In addition, the long gap between the onset of iRBD and conversion to neurodegeneration (up to several decades) creates a need to identify subsets of patients who are going to convert in the near future, or who will convert to a more specific endpoint.…”

Section: Discussionmentioning

confidence: 99%

What the “man in the moon” can tell us about the future of our brains

Högl

2017

Ann. Transl. Med.

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Rapid eye movement sleep behavior disorder: devising controlled active treatment studies for symptomatic and neuroprotective therapy—a consensus statement from the International Rapid Eye Movement Sleep Behavior Disorder Study Group

Cited by 204 publications

References 93 publications

Wilson?s disease presenting as rapid eye movement sleep behavior disorder: a possible window to early treatment

Wilson?s disease presenting as rapid eye movement sleep behavior disorder: a possible window to early treatment

FDG PET, dopamine transporter SPECT, and olfaction: Combining biomarkers in REM sleep behavior disorder

What the “man in the moon” can tell us about the future of our brains

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