Christin Bermudez Andrews
University of Miami-Miller School of Medicine, USA
Posters & Accepted Abstracts: J Neurol Neurophysiol
Cognitive and behavioral impairments in Amyotrophic Lateral Sclerosis (ALS) have gained recognition as important facets of the frontotemporal neuropathology. ALS-Frontotemporal Spectrum Disorder (ALS-FTSD) has been identified in up to 75% of patients with ALS, with approximately 15-41% meeting diagnostic criteria of Frontotemporal Dementia (FTD). The presence of ALS-FTSD has been linked to shorter survival and reduced quality of life, two primary outcomes measures in drug treatment and development. While we know that ALS and FTD may have a shared neuropathology and even arise from common genetic mutations, the majority of ALS research has been conducted exclusively with homogeneous groups. As we move towards genetic therapies for these diseases, expanding our understanding of ALS-FTSD with racially, culturally and linguistically diverse populations will be essential. The Edinburgh Cognitive and Behavioral ALS Screen (ECAS) and the ALS Cognitive Behavioral Screen (ALS-CBS) are two commonly used measures in ALS-FTSD research. While both have shown demonstrated strong sensitivity and specificity when applied to patient samples that reflect the original normative groups, their use with diverse populations remains limited. While both measures have undergone transcultural normative projects, review of resulting normative standards reveals a wide range of variability with significantly different psychometric properties. The ECAS and ALS-CBS are promising assessment tools. While translation and validation efforts have been beneficial, they largely reflect isolated efforts. A global normative project is needed to facilitate collaborative pooling of data. Additionally, linguistic, cultural and educational variables that influence test performance should be quantified and statistically controlled to improve generalizability.
Christin Bermudez Andrews has completed her PhD at Nova South Eastern University. She is currently working as an Assistant Professor of Clinical Neuropsychology at the University of Miami, Miller School of Medicine in Department of Neurology.
E-mail: Dr.christinandrews@outlook.com
