TY - JOUR
T1 - Personality disturbances in amyotrophic lateral sclerosis
T2 - A case study demonstrating changes in personality without cognitive deficits
AU - Waldron, Eric J.
AU - Barrash, Joseph
AU - Swenson, Andrea
AU - Tranel, Daniel
N1 - Publisher Copyright:
Copyright © 2014 INS. Published by Cambridge University Press.
PY - 2014
Y1 - 2014
N2 - Patients with amyotrophic lateral sclerosis (ALS) often show deficits on neuropsychological tests that tap functions related to the integrity of the prefrontal lobes. Various aspects of personality are also known to be mediated by prefrontal regions, particularly ventromedial prefrontal cortex (vmPFC). Other than apathy, personality changes have not been widely reported in patients with ALS, although clinical observations indicate such changes might be relatively common. Here, we report on a middle-aged woman with bulbar onset ALS (diagnosed 06/2011, examined in Spring, 2012) whose neuropsychological exam did not reveal cognitive deficits. She performed normally on tests of executive functioning. Self-report measures of mood and personality were unremarkable. However, significant personality changes subsequent to disease onset were reported by her husband and two daughters, and these changes were quantified with the Iowa Scales of Personality Change. Results show that personality disturbance may manifest in the absence of notable cognitive changes in ALS, and careful assessment of personality may be important for documenting early neurobehavioral changes in some ALS patients. Findings also show that patients with ALS may not have good insight into personality changes, underscoring the importance of acquiring collateral information. More generally, the results provide further evidence that ALS may compromise the integrity of ventromedial prefrontal regions.
AB - Patients with amyotrophic lateral sclerosis (ALS) often show deficits on neuropsychological tests that tap functions related to the integrity of the prefrontal lobes. Various aspects of personality are also known to be mediated by prefrontal regions, particularly ventromedial prefrontal cortex (vmPFC). Other than apathy, personality changes have not been widely reported in patients with ALS, although clinical observations indicate such changes might be relatively common. Here, we report on a middle-aged woman with bulbar onset ALS (diagnosed 06/2011, examined in Spring, 2012) whose neuropsychological exam did not reveal cognitive deficits. She performed normally on tests of executive functioning. Self-report measures of mood and personality were unremarkable. However, significant personality changes subsequent to disease onset were reported by her husband and two daughters, and these changes were quantified with the Iowa Scales of Personality Change. Results show that personality disturbance may manifest in the absence of notable cognitive changes in ALS, and careful assessment of personality may be important for documenting early neurobehavioral changes in some ALS patients. Findings also show that patients with ALS may not have good insight into personality changes, underscoring the importance of acquiring collateral information. More generally, the results provide further evidence that ALS may compromise the integrity of ventromedial prefrontal regions.
KW - Behavior
KW - Lou Gehrig's disease
KW - Motor neuron disease
KW - Neuropsychology
KW - Personality assessment
KW - Prefrontal cortex
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U2 - 10.1017/S1355617714000459
DO - 10.1017/S1355617714000459
M3 - Article
C2 - 24854881
AN - SCOPUS:84913612612
SN - 1355-6177
VL - 20
SP - 764
EP - 771
JO - Journal of the International Neuropsychological Society
JF - Journal of the International Neuropsychological Society
IS - 7
ER -