Major or Mild Frontotemporal Neurocognitive Disorder

Abstract

Frontotemporal neurocognitive disorder (NCD) constitutes the leading form of NCD in patients younger than age 65. It can lead to the behavioral-variant frontotemporal NCD or pure cognitive changes, as in primary progressive aphasia. Although patients with behavioral variant have relatively preserved memory and language function, they display major deficits in insight, judgment, working memory, and executive functions. Patients with primary progressive aphasia most commonly have difficulty with naming and/or word finding. Other common presenting symptoms include hesitancy or nonfluent speech production, motor speech abnormalities (such as dysarthria), impaired language comprehension, and repetition. Primary progressive aphasia has been divided into three separate subsyndromes: nonfluent/agrammatic, semantic, and logopenic variants. The clinical presentation is determined by the anatomical areas involved and the pathognomonic atrophy. An accurate clinical diagnosis helps to predict probable neuropathology, which is essential for targeted treatment planning. In this chapter, we review the primary symptomatology of these frontotemporal NCD variants, diagnostic and treatment challenges, and comorbidity complexity that needs to be considered in differential diagnosis.