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Cognition and Amyotrophic Lateral Sclerosis (ALS)
Journal article   Open access   Peer reviewed

Cognition and Amyotrophic Lateral Sclerosis (ALS)

D Irwin, Carol Lippa and J Swearer
American journal of Alzheimer's disease and other dementias, v 22(4), pp 300-312
01 Aug 2007
PMID: 17712161
url
https://doi.org/10.1177/1533317507301613View
Published, Version of Record (VoR)Maybe Open Access (Publisher Bronze) Open

Abstract

Amyotrophic lateral sclerosis (ALS) is classically described as a pure motor disease; however, there is growing evidence of a range of cognitive impairment. Cognitive abnormalities include deficiencies in frontal executive skills, varying from mild deficits to meeting criteria for diagnosis of frontotemporal dementia (FTD). Cognitive impairment occurs in sporadic and familial forms of ALS. Patients may present with cognitive deficits before, after, or at the onset of motor neuron disease. Structural and functional imaging studies have shown extramotor cortical degeneration corresponding to levels of frontal executive impairment on neuropsychologic testing. In addition, ALS and a subset of FTD patients display common pathological findings on immunohistochemistry staining. It is believed that these disorders represent a continuum between motor and nonmotor cortical degeneration. The purpose of this article is to review the literature on cognitive deficits in ALS. Identifying changes in cognition is critical for physicians and caregivers of ALS patients, as cognitive decline may interfere with patient compliance. Diagnosis and treatment of cognitive symptoms in ALS patients may improve quality of life.

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