The UCSF screening exam effectively screens cognitive and behavioral impairment in patients with ALS

The University of California San Francisco (UCSF) Screening Battery provides clinicians with a uniquely tailored tool to measure ALS patients’ cognitive and behavioral changes, adjusting for dysarthria and hand weakness. The battery consists of the ALS-CBS (1 Woolley SC, York MK, Moore DH, Strutt AM, Murphy J, Schulz PE, et al. Detecting frontotemporal dysfunction in ALS: utility of the ALS Cognitive Behavioral Screen (ALS-CBS). Amyotroph Lateral Scler. 2010;11:303–11.[Taylor & Francis Online], [Web of Science ®] , [Google Scholar]), Written Fluency Test (2 Abrahams S, Leigh PN, Harvey A, Vythelingum GN, Grise D, Goldstein LH. Verbal fluency and executive dysfunction in amyotrophic lateral sclerosis. Neuropsychologia. 2000;38:734–47.[Crossref], [PubMed], [Web of Science ®] , [Google Scholar]), and a new revision of the Frontal Behavior Inventory (FBI-ALS) (3 Kertesz A, Davidson W, Fox H. Frontal behavioral inventory: diagnostic criteria for frontal lobe dementia. Can J Neurol Sci. 1997;24:29–36.[Crossref], [PubMed], [Web of Science ®] , [Google Scholar]). The validity of each component was tested by comparing results with a gold standard neuropsychological exam (GNE). Consensus criteria-based GNE diagnoses (4 Strong MJ, Grace GM, Freedman M, Lomen-Hoerth C, Woolley S, Goldstein LH, et al. Consensus criteria for the diagnosis of frontotemporal cognitive and behavioral syndromes in amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2009;10:131–46.[Taylor & Francis Online], [Web of Science ®] , [Google Scholar]) were assigned (n = 24) and concurrent validity was tested for each screening exam component. Results showed that each of the four cognitive and behavioral screening test components were significantly associated with diagnoses confirmed by GNE. GNE diagnoses were significantly associated with FBI-ALS negative score, written S-words score, and ALS-CBS cognitive score. The total FBI-ALS score and C-words tests were less predictive of GNE-diagnosed impairment. In conclusion, the UCSF Cognitive Screening Battery demonstrates good external validity compared with GNE in this modest sample, encouraging its use in larger investigations. These data suggest that this battery may provide an effective screen to identify ALS patients who will then benefit from a full examination to confirm their diagnosis.