ALS and Frontotemporal Dysfunction: A Review

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 recogni...

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Main Authors: Eugene Y. Achi, Stacy A. Rudnicki
Format: Article
Language:English
Published: Wiley 2012-01-01
Series:Neurology Research International
Online Access:http://dx.doi.org/10.1155/2012/806306
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author Eugene Y. Achi
Stacy A. Rudnicki
author_facet Eugene Y. Achi
Stacy A. Rudnicki
author_sort Eugene Y. Achi
collection DOAJ
description 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.
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spelling doaj-art-5f2fac408e364a5eafa4b16e959be5f92025-08-20T02:05:49ZengWileyNeurology Research International2090-18522090-18602012-01-01201210.1155/2012/806306806306ALS and Frontotemporal Dysfunction: A ReviewEugene Y. Achi0Stacy A. Rudnicki1Department of Neurology, University of Arkansas for Medical Sciences, Little Rock, AR 72205, USADepartment of Neurology, University of Arkansas for Medical Sciences, Little Rock, AR 72205, USAThough 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.http://dx.doi.org/10.1155/2012/806306
spellingShingle Eugene Y. Achi
Stacy A. Rudnicki
ALS and Frontotemporal Dysfunction: A Review
Neurology Research International
title ALS and Frontotemporal Dysfunction: A Review
title_full ALS and Frontotemporal Dysfunction: A Review
title_fullStr ALS and Frontotemporal Dysfunction: A Review
title_full_unstemmed ALS and Frontotemporal Dysfunction: A Review
title_short ALS and Frontotemporal Dysfunction: A Review
title_sort als and frontotemporal dysfunction a review
url http://dx.doi.org/10.1155/2012/806306
work_keys_str_mv AT eugeneyachi alsandfrontotemporaldysfunctionareview
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