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Original Contribution
April 2007

Continuum of Frontal Lobe Impairment in Amyotrophic Lateral Sclerosis

Author Affiliations

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.

Arch Neurol. 2007;64(4):530-534. doi:10.1001/archneur.64.4.530

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.