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Jennifer M. Murphy, PhD; Roland G. Henry, PhD; Susan Langmore, PhD; Joel H. Kramer, PsyD; Bruce L. Miller, MD; Catherine Lomen-Hoerth, MD, PhD

Arch Neurol. 2007;64(4):530-534.

Objective  To identify the nature and prevalence of cognitive and behavioral abnormalities in patients with amyotrophic lateral sclerosis (ALS).

Design  Survey of clinical characteristics.

Setting  Multidisciplinary clinic within a university medical center.

Patients  A volunteer sample of 30 new patients with ALS were recruited consecutively. Of those invited, 23 participants (20 with sporadic ALS and 3 with familial ALS) enrolled. Participants ranged in age from 27 to 80 years (mean age, 56.5 years); the education level ranged from 12 to 21 years (mean education level, 3.5 years of college); and 17 participants (74%) were male.

Main Outcome Measures  Neuropsychological tests, neurobehavioral interviews, and structured magnetic resonance imaging.

Results  Patients were classified into subtypes of frontotemporal lobar degeneration (n = 5), suspected Alzheimer disease (n = 1), and subthreshold variants of cognitive impairment (n = 2), behavioral impairment (n = 4), and cognitively and behaviorally normal (n = 11). Five neuropsychological tests, 2 behavioral abnormalities, and right hemisphere gray matter reductions differentiated patients into normal and abnormal groups.

Conclusions  In this sample, a sizable proportion of patients with ALS possess a range of behavioral and cognitive changes that lie on a spectrum of frontotemporal impairment. Right hemisphere atrophy may be a biomarker for cognitive impairment in patients with ALS.

Author Affiliations: Department of Neurology, ALS Center (Drs Murphy, Langmore, and Lomen-Hoerth), Department of Radiology, Center for Molecular and Functional Imaging (Dr Henry), and Department of Neurology, Memory and Aging Center (Drs Kramer and Miller), University of California, San Francisco.

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