Timeline of Rapid Eye Movement Sleep Behavior Disorder in Overt Alpha-Synucleinopathies. 2021

Cole D Stang, and Aidan F Mullan, and Mania Hajeb, and Emanuele Camerucci, and Pierpaolo Turcano, and Peter Martin, and Michelle M Mielke, and Keith A Josephs, and James H Bower, and Erik K St Louis, and Bradley F Boeve, and Rodolfo Savica
Department of Neurology, Mayo Clinic, Rochester, MN, USA.

The aim was to analyze the timeline, prevalence, and survival of rapid eye movement (REM) sleep behavior disorder (RBD) in patients who developed alpha-synucleinopathies (Parkinson disease, dementia with Lewy bodies, and Parkinson disease dementia) compared with age- and sex-matched controls in a population-based incident-cohort study. We used a population-based, 1991 to 2010 incident-cohort study of alpha-synucleinopathies. A movement-disorder specialist reviewed medical records to confirm diagnoses. RBD was diagnosed by reported dream-enactment symptoms or polysomnography. Probable RBD and polysomnographically confirmed RBD were analyzed separately and combined. Among the 444 incident cases of alpha-synucleinopathy, 86 were clinically diagnosed with RBD (19.8%), including 30 (35%) by polysomnography and 56 (65%) as probable. The prevalence of idiopathic RBD at alpha-synucleinopathy diagnosis was 3.4%, increasing to 23.8% after 15 years. Cumulative lifetime incidence was 53 times greater in alpha-synucleinopathy patients than in controls (odds ratio [OR] = 53.1, 95% confidence interval [CI]: 13.0-217.2, p < 0.0001), higher in dementia with Lewy bodies than in Parkinson disease (OR = 2.57, 95% CI: 1.50-4.40, p = 0.0004), and higher in men than in women with Parkinson disease, dementia with Lewy bodies, or Parkinson disease dementia (OR = 3.70, 95% CI: 2.07-6.62, p < 0.0001), but did not increase mortality risk. Our cohort had RBD incidence of 3.4%. Overall RBD increased to 23.8% after 15 years, with an overall incidence of 2.5 cases per 100 person-years. With 53 times greater lifetime incidence in alpha-synucleinopathy patients than in controls, RBD was more likely to develop in dementia with Lewy bodies than in Parkinson disease or Parkinson disease dementia, and in men than in women, but did not increase mortality risk within our cohort. ANN NEUROL 2021;89:293-303.

UI MeSH Term Description Entries
D008297 Male Males
D009026 Mortality All deaths reported in a given population. CFR Case Fatality Rate,Crude Death Rate,Crude Mortality Rate,Death Rate,Age Specific Death Rate,Age-Specific Death Rate,Case Fatality Rate,Decline, Mortality,Determinants, Mortality,Differential Mortality,Excess Mortality,Mortality Decline,Mortality Determinants,Mortality Rate,Mortality, Differential,Mortality, Excess,Age-Specific Death Rates,Case Fatality Rates,Crude Death Rates,Crude Mortality Rates,Death Rate, Age-Specific,Death Rate, Crude,Death Rates,Determinant, Mortality,Differential Mortalities,Excess Mortalities,Mortalities,Mortality Declines,Mortality Determinant,Mortality Rate, Crude,Mortality Rates,Rate, Age-Specific Death,Rate, Case Fatality,Rate, Crude Death,Rate, Crude Mortality,Rate, Death,Rate, Mortality,Rates, Case Fatality
D010300 Parkinson Disease A progressive, degenerative neurologic disease characterized by a TREMOR that is maximal at rest, retropulsion (i.e. a tendency to fall backwards), rigidity, stooped posture, slowness of voluntary movements, and a masklike facial expression. Pathologic features include loss of melanin containing neurons in the substantia nigra and other pigmented nuclei of the brainstem. LEWY BODIES are present in the substantia nigra and locus coeruleus but may also be found in a related condition (LEWY BODY DISEASE, DIFFUSE) characterized by dementia in combination with varying degrees of parkinsonism. (Adams et al., Principles of Neurology, 6th ed, p1059, pp1067-75) Idiopathic Parkinson Disease,Lewy Body Parkinson Disease,Paralysis Agitans,Primary Parkinsonism,Idiopathic Parkinson's Disease,Lewy Body Parkinson's Disease,Parkinson Disease, Idiopathic,Parkinson's Disease,Parkinson's Disease, Idiopathic,Parkinson's Disease, Lewy Body,Parkinsonism, Primary
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000080874 Synucleinopathies Neurodegenerative disorders involving deposition of abnormal ALPHA-SYNUCLEIN in dopaminergic neurons and glial cells in the brain. Pathological aggregations of alpha-synuclein proteins results in LEWY BODIES and Lewy neurites; melanin granules in the SUBSTANTIA NIGRA and LOCUS COERULEUS; and glial cytoplasmic inclusions. Synucleinopathies are associated with mutation in the ALPHA-SYNUCLEIN (SNCA) gene on chromosome 4. PARKINSON DISEASE; LEWY BODY DISEASE with dementia; and MULTIPLE SYSTEM ATROPHY are prominent examples of synucleinopathy. a-Synucleinopathies,alpha Synuclein Pathology,alpha-Synucleinopathies,Synucleinopathy,a-Synucleinopathy,alpha Synuclein Pathologies,alpha Synucleinopathies,alpha-Synucleinopathy
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly
D000369 Aged, 80 and over Persons 80 years of age and older. Oldest Old
D015994 Incidence The number of new cases of a given disease during a given period in a specified population. It also is used for the rate at which new events occur in a defined population. It is differentiated from PREVALENCE, which refers to all cases in the population at a given time. Attack Rate,Cumulative Incidence,Incidence Proportion,Incidence Rate,Person-time Rate,Secondary Attack Rate,Attack Rate, Secondary,Attack Rates,Cumulative Incidences,Incidence Proportions,Incidence Rates,Incidence, Cumulative,Incidences,Person time Rate,Person-time Rates,Proportion, Incidence,Rate, Attack,Rate, Incidence,Rate, Person-time,Rate, Secondary Attack,Secondary Attack Rates
D015995 Prevalence The total number of cases of a given disease in a specified population at a designated time. It is differentiated from INCIDENCE, which refers to the number of new cases in the population at a given time. Period Prevalence,Point Prevalence,Period Prevalences,Point Prevalences,Prevalence, Period,Prevalence, Point,Prevalences

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