Frontotemporal dementia: diagnosis, deficits and management

Abstract

Frontotemporal dementia (FTD) is a progressive neurologic syndrome with diverse clinical presentations and attendant underlying pathologies. Psychiatric prodrome, neuropsychiatric symptoms and language difficulties are common in FTD, but the diversity of presentation raises unique diagnostic challenges that can significantly impact patient care and counsel for caregivers regarding clinical status and prognosis. While neuropsychiatric symptom measures are helpful, more sensitive assessments delineating the specific behavioral and linguistic deficits accompanying FTD are needed. Comprehensive clinical assessment in combination with evaluation of language, socio-emotional functioning, cognition and neuroimaging aid in accurate and early diagnosis and treatment planning. In what follows, we review each of the FTD syndromes, highlight current research investigating the cognitive, behavioral and socio-emotional deficits observed with this disease, address common diagnostic challenges and summarize best practices associated with management of FTD.

Keywords: differential diagnosis; frontotemporal dementia; neuropsychology; primary progressive aphasia; socio-emotional functioning.

Figure 1. T1-weighted structural MRI in patients with behavioral variant frontotemporal dementia, semantic variant primary progressive aphasia and nonfluent variant primary progressive aphasia

(A) Behavioural variant frontotemporal dementia (B) semantic variant frontotemporal dementia and (c) nonfluent variant primary progressive aphasia. Images are in radiological view (right hemisphere is on the left side of the image).