[P08.072] First Symptoms in Pathologically-Confirmed Corticobasal Degeneration, Progressive Supranuclear Palsy, and Frontotemporal Dementia with Motor Neuron Disease

Michael H. Rosenbloom, Suzee Lee, Shenly Glenn, Alvin Chan, Caroline A. Racine, Bruce Miller, San Francisco, CA

OBJECTIVE: To investigate first symptoms in a pathologically-confirmed sample of frontotemporal dementia (FTD) spectrum disorders. BACKGROUND: Early detection and diagnosis of FTD spectrum disorders is critical for providing adequate treatment and intervention. However, these disorders present with a wide variety of clinical symptoms (i.e. behavior, cognitive, motor) making an early and accurate diagnosis difficult. It is also increasingly important to recognize which early symptoms are predictive of specific underlying neuropathology (i.e. tau vs. TDP-43) as potential disease-modifying therapies become available. Comprehensive studies of first symptoms in pathologically-confirmed patients are lacking. DESIGN/METHODS: We performed retrospective chart reviews for patients evaluated at the UCSF Memory & Aging Center with pathologically-confirmed corticobasal degeneration (CBD; N=9), frontotemporal dementia with motor neuron disease (FTD-MND, N=8), and progressive supranuclear palsy (PSP, N=18). First symptoms were assessed by examining the first clinical and/or research neurological report available. Symptoms were assigned to the following categories: memory, executive dysfunction, language/speech, behavioral/psychiatric, motor, and sensory. Cases that had multiple symptoms at onset contributed data to multiple categories. RESULTS: CBD presented with a wide range of first symptoms (33% language, 33% behavior, 22% memory, 11% motor, 11% executive dysfunction, 11% sensory). FTD-MND was associated with a high predominance of language/speech complaints (63%) as first symptoms, while commonly associated features of FTD such as executive dysfunction (25%) and behavior (12%) were less frequent. PSP was as likely to present with motor symptoms (50%) as with language/speech complaints (49%), while behavior (16%) and executive dysfunction (6%) were less likely. CONCLUSIONS/RELEVANCE: This preliminary study suggests that pathologically-confirmed CBD can present with a wide variety of first symptoms, consistent with the multitude of clinical presentations associated with CBD pathology. Interestingly, speech & language complaints were common amongst FTD-MND and PSP, suggesting that these symptoms may assist with early diagnosis. Supported by: NIH
Category - Aging and Dementia - Clinical

Thursday, April 30, 2009 11:30 AM

Poster Session VIII: Aging and Dementia: Clinical III (11:30 AM-2:30 PM)