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Treatment approaches for frontotemporal lobar degeneration (FTLD) are rapidly evolving with improved understanding of the disease. This brief review highlights recent advances.Early-onset dementia has a devastating impact on families and rids its victims of their most productive and rewarding years. Over the past 10 years, FTLD has emerged as the commonest cause of dementia under the age of 60 years, outstripping even Alzheimer's disease in prevalence. Remarkable progress has occurred in our understanding of FTLD both as a set of distinctive clinical syndromes and as a set of disorders with unique genetic and pathological profiles. Although there are no Food and Drug Administration-approved medications for FTLD, new evidence of specific genetic and neurochemical defects is beginning to provide a strong rationale for pharmacological treatment.Behavioral changes, which are common in behavioral variant frontotemporal dementia and semantic dementia, often respond to treatment with selective serotonin reuptake inhibitors. Memantine also holds promise to treat neuropsychiatric symptoms, but more prospective trials are needed.With better understanding of pathogenic molecular pathways involving microtubule-associated protein tau, progranulin and TDP-43, potential disease-modifying therapies are being studied in animal models and approaching human trials.