A - 39 Diagnosing Behavioral Variant Frontotemporal Dementia in the Absence of Notable Cognitive Changes: the Value of Collateral Reports.

Abstract

Behavioral variant frontotemporal dementia (bvFTD) is a neurodegenerative disorder broadly characterized by insidious changes in psychosocial domains including personality/mood, behavior, and cognition. Early detection of bvFTD can be challenging in the absence of structural brain changes, cognitive deficits, and/or patient report. Collateral reports and behavioral observations become integral to diagnostic conceptualization. The patient is a 71-year-old White male who was seen by a neurologist for semi-involuntary fidgeting. His spouse also began noticing medication mismanagement and personality changes characterized by irritability, compulsive spending, and irrational thoughts, as well as uncharacteristic lapses in judgement and difficulty with planning and organization. Additional family reports endorsed new perseverative behaviors and intermittent confusion while operating familiar devices. An MRI of the brain demonstrated mild white matter disease. The patient demonstrated superior premorbid intellectual functioning and average-to-above average scores across most neuropsychological tests. Subtle deficits in maintaining set on executive tasks were noted. Behaviorally, speech was perseverative and tangential throughout the evaluation and he demonstrated low frustration tolerance. Collateral questionnaires indicated a decline in most activities of daily living and significant elevations in apathy, personal neglect, and loss of insight. While there were subtle changes in cognition, the patient's overall cognitive profile was intact. However, collateral reports, reported functional decline, and direct behavioral observations warranted concern for early stage bvFTD. Consistent with bvFTD literature, current neuropsychological assessments lack sensitivity for capturing deficits in the presence of behavioral and functional impairment. This case highlights the continued need to develop and validate naturalistic assessments to appropriately capture bvFTD.