Abstract:
Frontotemporal dementia is the second most common younger-onset dementia affecting people under age 65 after Alzheimer’s disease. Here, we review the clinical, cognitive, neuroimaging and pathological profiles in the main subtypes of frontotemporal dementia: behavioral-variant frontotemporal dementia and primary progressive aphasia, which is further subdivided into semantic dementia, progressive nonfluent aphasia and logopenic progressive aphasia. While no disease-modifying treatments have yet been discovered, several symptomatic treatments are currently in use, with variable efficacy. In this chapter we consider the available pharmacological, behavioral, cognitive and carer interventions which have been investigated in frontotemporal dementia. We focus specifically on the types of patients likely to benefit from these interventions and known contraindications for their use. Finally we highlight the importance of accurate diagnosis and clear clinicopathological associations to facilitate the development of effective therapies in this disorder. While unfortunately the management of this devastating condition is currently challenging, a surge of research into frontotemporal dementia over the past two decades offers hope for improved treatment on the horizon.
