als and frontotemporal dysfunction a reviewals和额颞叶功能障碍进行审查.pdfVIP

Hindawi Publishing Corporation Neurology Research International Volume 2012, Article ID 806306, 9 pages doi:10.1155/2012/806306 Review Article ALS and Frontotemporal Dysfunction: A Review Eugene Y. Achi and Stacy A. Rudnicki Department of Neurology, University of Arkansas for Medical Sciences, Little Rock, AR 72205, USA Correspondence should be addressed to Stacy A. Rudnicki, sarudnicki@ Received 5 April 2012; Accepted 19 June 2012 Academic Editor: Brett Morrison Copyright © 2012 E. Y. Achi and S. A. Rudnicki. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Though once believed to be a disease that was limited to the motor system, it is now apparent that amyotrophic lateral sclerosis (ALS) may be associated with cognitive changes in some patients. Changes are consistent with frontotemporal dysfunction, and may range from mild abnormalities only recognized with formal neuropsychological testing, to profound frontotemporal dementia (FTD). Executive function, behavior, and language are the most likely areas to be involved. Screening helpful in detecting abnormalities includes verbal or categorical fluency, behavioral inventories filled out by the caregiver, and evaluation for the presence of depression and pseudobulbar affect. Patients with cognitive dysfunction have shortened survival and may be less compliant with recommendations regarding use of feeding tubes and noninvasive ventilation. Evolving knowledge of genetic and pathological links between ALS and FTD has allowed us to better understand the overlapping spectrum of ALS and FTD. 1. Introduction