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From the Department of Neurology (Dr. Ringholz), Emory University School of Medicine, Atlanta, GA; Department of Neurology (Dr. Appel), The Neurologic Institute, The Methodist Hospital, Houston, TX; Neurology Care Line (Drs. Mosnik and Schulz), Michael E. DeBakey VA Medical Center, Houston, TX; and Department of Neurology (Drs. Appel, Bradshaw, Cooke, Mosnik, and Schulz), Baylor College of Medicine, Houston, TX.
Address correspondence and reprint requests to Dr. Stanley H. Appel, Professor and Chairman, Methodist Neurological Institute, 6501 Fannin St., NB-302, Houston, TX 77030; e-mail: SAppel{at}tmh.tmc.edu
Objective: To investigate the prevalence and nature of cognitive changes associated with sporadic amyotrophic lateral sclerosis (ALS) using a large scale study.
Methods: Consecutive patients with sporadic ALS (n = 279) underwent comprehensive neurologic evaluation and neuropsychological testing. Testing data from normal controls (n = 129) were used for classification and comparison purposes.
Results: On non-motor, non-speed-dependent tasks, 51% of patients with ALS had evidence of cognitive impairment compared to 5% of controls. Cluster analysis suggested four patient subgroups: 49% intact, 32% with mild impairment, 13% with moderate impairment, and 6% with severe impairment. Forty-one patients (15%) met criteria for frontotemporal dementia (FTD). ALS patient subgroups, excluding the intact group, performed significantly lower on tests of executive function and memory than normal controls. Patients with more severe disease also had deficits in confrontation naming. Although memory function declined with increasing severity of overall cognitive impairment, only two patients had the severe memory loss typical of Alzheimer disease. Cognitive impairment was correlated with clinical measures of word-finding, phrase length, and motor programming. Cognitive impairment was not correlated with depression scores or severity or duration of motor or bulbar symptoms. Patients with bulbar vs limb-onset ALS were not different in either level of impairment or pattern of performance.
Conclusions: These data confirm the presence of cognitive impairment in 50% of patients with ALS and particularly implicate executive dysfunction and mild memory decline in the disease process. More severe impairment occurs in a subset of patients with ALS and has features consistent with FTD.
Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the August 23 issue to find the title link for this article.
The Vicki Appel/MDA ALS clinic is supported by the Muscular Dystrophy Association.
Disclosure: The authors report no conflicts of interest.
Received March 14, 2003. Accepted in final form May 13, 2005.
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