Clinical manifestation of patients with isolated rapid eye movement sleep behavior disorder after modest-to-long disease duration

Abstract

Study Objectives: Patients with isolated rapid eye movement (REM) sleep behavior disorder (iRBD) are known to be at risk of neurodegenerative diseases but the time process of neurodegeneration remains unclear and its ethnic difference is rarely discussed. The objective of this study was to investigate the clinical manifestation of iRBD according to disease duration in a Korean cohort. Methods: Patients who had been diagnosed with iRBD for at least 5 years were tracked and those currently remaining as iRBD were invited for motor, autonomic, olfactory, color discrimination, and cognitive tests. Cross-sectional comparison between patients with iRBD with modest disease duration (5-9.9 years) and long disease duration (≥10 years) was conducted. Results: There were total of 198 patients with iRBD (135 males, 68.2%) tracked, whose average age at diagnosis was 65.9 years. Thirty-three had developed parkinsonism and 17 had developed dementia, resulting in a phenoconversion risk of 35.7% at 10 years. Hazard rate showed a gradually increasing trend over time, beginning from 2.1 conversions per year to 8.5 at 10 years. Patients with iRBD with a long disease duration (n = 19) had slightly higher motor scores than those with a modest duration (n = 82). However, they showed no difference in the frequency of motor abnormality or other clinical markers. Conclusions: There is a possible racial or geographical difference of phenoconversion risk. The progression of neurodegeneration might be very subtle in that patients with iRBD with longer disease duration do not necessarily show higher frequency of neurodegeneration markers.