Abstract
BACKGROUND: Frontotemporal dementia has recently been recognized as a common cause of young-onset dementia. OBJECTIVE: To review the current approach to the clinical evaluation, understanding of pathophysiology, and management of frontotemporal dementia. RESULTS: Two main clinical presentations are: (1) behavioral, with impulsive behaviors and disinhibition, change in personality such as apathy and indifference, and poor judgment, and (2) language, with a nonfluent aphasia with anomia (primary progressive aphasia), or a fluent aphasia with early loss of word meaning (semantic dementia). The differential diagnosis includes other neurodegenerative dementias, vascular and other conditions affecting the brain, and psychiatric diseases. Investigations, including neuropsychological testing, and structural and functional brain imaging, may help support the diagnosis. Recent advances in understanding the pathophysiology have suggested that most cases have underlying ubiquitin-positive inclusions, whereas some have tau-positive inclusions. Genetic mutations, particularly on chromosome 17 in the tau or progranulin genes, have been identified. Management includes a trial of symptomatic medications and a multifaceted approach, including environmental modification and long-term care planning. CONCLUSION: Medical researchers studying frontotemporal dementia aim to identify disease-modifying drugs and, ultimately, a cure for this devastating disease.