Frontotemporal lobar degeneration (FTLD) consists of a group of neuropathologically defined entities characterized by specific protein inclusions in neurons and glia. Canonical presentations of FTLD encompass a set of syndromes, grouped under the term “frontotemporal dementia” (FTD), with prominent behavioral, speech, and language changes. These syndromes include the behavioral variant of FTD, the nonfluent agrammatic variant, and the semantic variant of primary progressive aphasia.1 Evidence from several clinical-pathologic studies suggests that the clinical manifestations of FTLD often exceed the description of the canonical syndromes and include a myriad of cognitive, neuropsychiatric, and motor changes.1–3