Objectives: To detail the biological, clinical and neurocognitive characteristics differentiating bipolar disorder (BD) from frontotemporal dementia (FTD) and to investigate whether BD is a risk factor for FTD.Methods: A total of 16 studies were included in this systematic review. Five studies described biological and/or neurocognitive characteristics between patients with BD and FTD, and 11 studies investigated whether BD was a risk factor for FTD.Results: Individuals with FTD presented higher levels of serum neurofilament light chain, greater grey matter reduction in frontal, parietal and temporal lobes, and increased slow wave oscillations in channels F3, F4, T3, T5, T4 and T6 within an electroencephalogram (EEG), relative to individuals with BD. Patients with FTD presented greater deficits in executive function and theory of mind compared to patients with BD in a euthymic state, and more deficits in verbal fluency compared to patients with BD in a current mood episode. Patients with BD in a current mood episode showed greater impairment in attention, working memory, verbal memory and executive function relative to individuals with FTD. In addition, retrospective studies showed that 10.2%-11.6% of patients with behavioural variant FTD (bvFTD) had a preceding history of BD. Conclusion:Biological and neurocognitive characteristics help to distinguish between BD and FTD, and it may help to reach a more precise diagnosis. In addition, individuals with BD are at higher risk of developing FTD. More studies are needed to identify the predictors of the conversion between BD to FTD.

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