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.
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.
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.