Forced Vital Capacity, Bulbar Onset, and Neuropsychological Impairment in Amyotrophic Lateral Sclerosis (ALS) (P04.231)

Abstract

Objective: To characterize cognitive involvement with neuropsychological tests measuring visuoconstruction (figure copy) and executive control/ mental search (letter F-word output) in patients with amyotrophic lateral sclerosis (ALS) to determine how limb versus bulbar onset and selected cognitive measures might be related to FVC. Background Previously a relationship between forced vital capacity (FVC) and cognitive involvement in ALS has been reported but the type of cognitive involvement has not been characterized nor the relationship of FVC and site of onset to that cognitive change. Design/Methods: 28 ALS patients were studied (age=61.76+12.26; MMSE=27.18+2.89; ALSFRS=29.42+9.48). Seated FVC was obtained and bulbar versus limb onset was determined by expert clinicians (LR, AD, THP). Neuropsychological functioning was assessed with subtests from the Philadelphia Brief Assessment of Cognition (PBAC), a neuropsychological protocol designed for to assess for dementia in Alzheimer9s disease and frontotemporal dementia phenotypes. Results: Simple correlations associated better FVC with limb rather than bulbar onset (r= 0.373, p 2 = 0.139, p 2 = 0.299, p 2 = 0.515, p Conclusions: These data suggest better erect FVC is associated with and limb rather than bulbar onset and better a figure copy test (visuoconstruction) greater output on letter fluency tests (executive control/ sustained mental search). Longitudinal assessment is necessary to determine the utility and clinical significance of these relatationships. Disclosure: Dr. Nieves has nothing to disclose. Dr. Eppig has nothing to disclose. Dr. Libon has nothing to disclose. Dr. Harris has nothing to disclose. Dr. Feldman has nothing to disclose. Dr. Paolone has nothing to disclose. Dr. Rojas has nothing to disclose. Dr. Deboo has nothing to disclose. Dr. Heiman-Patterson has nothing to disclose.