PurposeWhite matter hyperintensities (WMHs) are frequently found in elderly individuals with or without dementia. However, the association between WMHs and clinical presentations of dementia with Lewy bodies (DLB) has rarely been studied.MethodsWe conducted a retrospective analysis of patients with DLB registered in a dementia database. WMHs were rated visually using the Fazekas scale, and its associated factors including dementia severity, cognitive functions, neuropsychiatric symptoms, and core clinical features were compared among different Fazekas scores. Domains in the Clinical Dementia Rating (CDR), Cognitive abilities Screening Instruments (CASI), and Neuropsychiatric Inventory (NPI) were compared among different Fazekas groups after adjusting for age, sex, education, and disease duration.ResultsAmong the 449 patients, 76, 207, 110, and 56 had Fazekas score of 0, 1, 2, and 3, respectively. There was a positive association between dementia severity and WMHs severity, and the mean sums of boxes of the Clinical Dementia Rating (CDR-SB) were 5.9, 7.8, 9.5, and 11.2 (f = 16.84, p < 0.001) for the Fazekas scale scores 0, 1, 2, and 3, respectively. There was a negative association between cognitive performance and WMHs severity, and the mean CASI were 57.7, 45.4, 4.06, and 33.4 (f = 14.22, p < 0.001) for the Fazekas scale scores 0, 1, 2, and 3, respectively. However, WMHs were not associated with the core clinical features of DLB. After adjustment, all cognitive domains in CDR increased as the Fazekas score increased. In addition, performance on all cognitive domains in CASI decreased as the Fazekas score increased (all p < 0.001). Among neuropsychiatric symptoms, delusions, euphoria, apathy, aberrant motor behavior, and sleep disorders were significantly worse in the higher Fazekas groups compared to those in the group with Fazekas score of 0 after adjustment.ConclusionWMHs in DLB might contribute to deterioration of cognitive function, neuropsychiatric symptoms, and dementia stages. However, core clinical features were not significantly influenced by WMHs in DLB.