Diagnosis and management of behavioral variant frontotemporal dementia

Abstract

Frontotemporal dementia was documented over a century ago. The last decade, however, has seen substantial changes in our conceptions of this increasingly recognized disorder. Different clinical variants have been delineated, the most common of which is the behavioral variant (bvFTD). Updated diagnostic criteria have been established. New histopathological findings and genetic etiologies have been discovered. Research continues to uncover molecular mechanisms by which abnormal proteins accumulate in degenerating brain tissue. Novel neuroimaging techniques suggest that functional networks are diminished in bvFTD that might be relevant to empathy and social behavior. Despite rapid advances in our understanding of bvFTD, the disease is still under-recognized and commonly misdiagnosed. The result is inappropriate patient care. Recognizing the various presentations of bvFTD and its histological and genetic subtypes might further diagnosis, treatment, and research.

Keywords: Behavioral variant frontotemporal dementia; FTD; Pick’s disease; bvFTD; frontotemporal dementia; frontotemporal lobar degeneration.

Figure 1

A T1 magnetic resonance image of the brain in axial, coronal, and sagittal cuts, shown in neurological convention, showing severe anterior predominant atrophy with hydrocephalus ex vacuo in a patient with advanced behavioral variant frontotemporal dementia (bvFTD).

Figure 2

Axial fluorodeoxyglucose positron emission tomography (FDG-PET) images of a patient with advanced bvFTD, shown in neurological convention, demonstrating hypometabolism in the anterior lobes bilaterally, but right more so than left. Image courtesy of Pia Ghosh of the UCSF Memory and Aging Center.