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

Abstract

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), Written Fluency Test (2), and a new revision of the Frontal Behavior Inventory (FBI-ALS) (3). The validity of each component was tested by comparing results with a gold standard neuropsychological exam (GNE). Consensus criteria-based GNE diagnoses (4) 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.

Key words::

• ALS
• frontotemporal lobar dementia
• cognitive
• behavioral
• screen

Acknowledgements

This research was conducted with the approval of the UCSF Internal Review Board and in accordance with the ethics standards of the Helsinki Declaration of 1975, revised in 1983. This research was funded in part by the ALS Association.

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

Supplementary material available online

Supplementary Appendix.