Continuum of frontal lobe impairment in amyotrophic lateral sclerosis.
OBJECTIVE
To identify the nature and prevalence of cognitive and behavioral abnormalities in patients with amyotrophic lateral sclerosis (ALS).


METHODS
Survey of clinical characteristics.


METHODS
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.


METHODS
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.
Murphy
Jennifer M
JM
Department of Neurology, ALS Center, University of California, San Francisco, CA 94117, USA. jennifer.murphy@ucsf.edu
Henry
Roland G
RG
Langmore
Susan
S
Kramer
Joel H
JH
Miller
Bruce L
BL
Lomen-Hoerth
Catherine
C
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications / Q000473:pathology*; D003072:Cognition Disorders / Q000209:etiology; D005625:Frontal Lobe / Q000000981:diagnostic imaging / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D001523:Mental Disorders / Q000209:etiology; D008875:Middle Aged; D009483:Neuropsychological Tests; D011859:Radiography