FTD Syndromes
Behavioral variant frontotemporal dementia
bvFTD is the most common FTD syndrome. It is characterized by alterations in social decorum and personal regulation including disinhibition, apathy, overeating, emotional blunting, personality changes toward coldness and submissiveness, repetitive motor behaviors, and impairment in judgment and insight. With these behavioral changes, deficits in executive control emerge and patients have problems with planning, organizing, shifting patterns and generating ideas.
As bvFTD may be misconstrued as a midlife problem of adjustment or a psychiatric disorder, patients often do not reach the neurologist until they have profound lapses in financial or interpersonal judgment. Unlike in AD, where family and acquaintances are often sympathetic to the patient due to the sparing of social decorum, colleagues and family may resent patients with bvFTD because of their rudeness, coldness and deficits in social modulation.
Approximately 15% of patients develop ALS, and extrapyramidal deficits are common, particularly as the disease progresses. Both structural and functional imaging are helpful and typically show greater abnormalities in the right than the left frontal regions. The ventral and medial frontal and insular regions are hit early in bvFTD, driving the disinhibition, apathy and eating disorder. This atrophy is usually evident by the time that the patient presents to the office.