Behavioral Variant Frontotemporal Dementia

William W. Seeley, MD Dementia p. 76-100 February 2019, Vol.25, No.1 doi: 10.1212/CON.0000000000000698
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PURPOSE OF REVIEW: This article describes the clinical, anatomic, genetic, and pathologic features of behavioral variant frontotemporal dementia (bvFTD) and discusses strategies to improve diagnostic accuracy, emphasizing common pitfalls to avoid. Key aspects of management and the future of diagnosis and care for the disorder are highlighted.

RECENT FINDINGS: BvFTD is a clinical syndrome, not a disease. Patients with the syndrome share core symptoms that reflect degeneration within the most consistently affected brain regions, but accompanying features vary and reflect the precise topography of regional degeneration. The clinician must distinguish a bvFTD syndrome from psychiatric illness and other neurodegenerative syndromes that feature a prominent behavioral component. Antemortem prediction of pathologic diagnosis remains imperfect but improves with careful attention to the clinical details. Management should emphasize prevention of caregiver distress, behavioral and environmental strategies, symptom-based psychopharmacology, and genetic counseling.

SUMMARY: BvFTD is an important and challenging dementia syndrome. Although disease-modifying treatments are lacking, clinicians can have a profound impact on a family coping with this disorder. Treatment trials are under way for some genetic forms of bvFTD. For sporadic disease, pathologic heterogeneity remains a major challenge, and ongoing research seeks to improve antemortem molecular diagnosis to facilitate therapeutic discovery.

Address correspondence to Dr William Seeley, 675 Nelson Rising Ln, San Francisco, CA 94158, bill.seeley@ucsf.edu.

RELATIONSHIP DISCLOSURE: Dr Seeley serves on the editorial boards of Acta Neuropathologica, Annals of Neurology, and NeuroImage: Clinical and as a consultant for Biogen; Merck & Co, Inc; and Third Rock Ventures, LLC. Dr Seeley receives research/grant support from the Bluefield Project to Cure FTD, the National Institutes of Health/National Institute on Aging (AG023501, AG019724), and the National Institutes of Health/National Institute of Neurological Disorders and Stroke (NS1104437, NS092474). Dr Seeley has provided expert medical testimony on legal cases related to violence in patients with neuropsychiatric illness.

UNLABELED USE OF PRODUCTS/INVESTIGATIONAL USE DISCLOSURE: Dr Seeley discusses the unlabeled/investigational use of medications for the treatment of behavioral variant frontotemporal dementia, none of which are approved by the US Food and Drug Administration.

© 2019 American Academy of Neurology