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Personality Disturbances in Amyotrophic Lateral Sclerosis: A Case Study Demonstrating Changes in Personality Without Cognitive Deficits

Published online by Cambridge University Press:  23 May 2014

Eric J. Waldron ,
Joseph Barrash ,
Andrea Swenson  and
Daniel Tranel
Eric J. Waldron*
Affiliation:
Department of Neurology, University of Iowa, Iowa City, Iowa
Joseph Barrash
Affiliation:
Department of Neurology, University of Iowa, Iowa City, Iowa Department of Psychology, University of Iowa, Iowa City, Iowa
Andrea Swenson
Affiliation:
Department of Neurology, University of Iowa, Iowa City, Iowa Iowa City Veterans Affairs Medical Center, Iowa City, Iowa
Daniel Tranel
Affiliation:
Department of Neurology, University of Iowa, Iowa City, Iowa Department of Psychology, University of Iowa, Iowa City, Iowa
*
Correspondence and reprint requests to: Eric Waldron, University of Iowa, Department of Neurology, 200 Hawkins Drive, 2007 RCP, Iowa City, IA 52242-1053. E-mail: eric-waldron@uiowa.edu
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Abstract

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. (JINS, 2014, 20, 1–8)

Keywords

Prefrontal cortexPersonality assessmentBehaviorNeuropsychologyMotor neuron diseaseLou Gehrig’s disease
Type
Case Study
Information
Journal of the International Neuropsychological Society , Volume 20 , Issue 7 , August 2014 , pp. 764 - 771
Copyright
Copyright © The International Neuropsychological Society 2014 

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References

Abrahams, S., Goldstein, L.H., Suckling, J., Ng, V., Simmons, A., Chitnis, X., … Leigh, P.N. (2005). Frontotemporal white matter changes in amyotrophic lateral sclerosis. Journal of Neurology, 252, 321–331.Google Scholar
Achi, E.Y., & Rudnicki, S.A. (2012). ALS and frontotemporal dysfunction: A review. Neurology Research International, 2012, 1–9.Google Scholar
Barrash, J., Anderson, S.W., Hathaway-Nepple, J., Jones, R.D., & Tranel, D. (1997). The Iowa Scales of Personality Change. Iowa City, IA: University of Iowa Carver College of Medicine, Department of Neurology.Google Scholar
Barrash, J., Asp, E., Markon, K., Manzel, K., Anderson, S.W., & Tranel, D. (2011). Dimensions of personality disturbance after focal brain damage: Investigation with the Iowa Scales of Personality Change. Journal of Clinical and Experimental Neuropsychology, 33(8), 833–852.CrossRefGoogle ScholarPubMed
Barrash, J., Tranel, D., & Anderson, S.W. (2000). Acquired personality disturbances associated with bilateral damage to the ventromedial prefrontal region. Developmental Neuropsychology, 18, 355–381.CrossRefGoogle Scholar
Barson, F.P., Kinsella, G.J., Ong, B., & Mathers, S.E. (2000). A neuropsychological investigation of dementia in motor neurone disease (MND). Journal of the Neurological Sciences, 180, 107–113.Google Scholar
Bechara, A., Tranel, D., & Damasio, H. (2000). Characterization of the decision-making deficit of patients with ventromedial prefrontal cortex lesions. Brain, 123, 2189–2202.Google Scholar
Brooks, B.R., Miller, R.G., Swash, M., Munsat, T.L., & World Federation of Neurology Research Group on Motor Neuron Diseases. (2000). El Escorial criteria revisited: Revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders, 1(5), 293–299.CrossRefGoogle Scholar
Eslinger, P.J., & Damasio, A.R. (1985). Severe disturbance of higher cognition after bilateral frontal ablation: Patient EVR. Neurology, 35(12), 1731–1741.Google Scholar
Fukae, J., Motoi, Y., Komatsuzaki, Y., Takanashi, M., Mochizuki, H., & Mizuno, Y. (2005). [A 59-year-old woman with personality change and abnormal behavior followed by amyotrophy and dementia]. No To Shinkei, 57(9), 810–820.Google ScholarPubMed
Gibbons, Z.C., Snowden, J.S., Thompson, J.C., Happe, F., Richardson, A., & Neary, D. (2007). Inferring thought and action in motor neurone disease. Neuropsychologia, 45, 1196–1207.Google Scholar
Gibbons, Z.C., Richardson, A., Neary, D., & Snowden, J.S. (2008). Behaviour in amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis, 9, 67–74.Google Scholar
Girardi, A., MacPherson, S.E., & Abrahams, S. (2011). Deficits in emotional and social cognition in amyotrophic lateral sclerosis. Neuropsychology, 25(1), 53–65.Google Scholar
Gläscher, J., Adolphs, R., Damasio, H., Bechara, A., Rudrauf, D., Calamia, M., … Tranel, D. (2012). Lesion mapping of cognitive control and value-based decision-making in the prefrontal cortex. Proceedings of the National Academy of Sciences of the United States of America, 109, 14681–14686.Google Scholar
Grace, J., & Malloy, P. (2001). Frontal systems behavior scale. Lutz, FL: Psychological Assessment Resources, Inc.Google Scholar
Grossman, A.B., Woolley-Levine, S., Bradley, W.G., & Miller, R.G. (2007). Detecting neurobehavioral changes in amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis, 8, 56–61.CrossRefGoogle ScholarPubMed
Irwin, D., Lippa, C.F., & Swearer, J.M. (2007). Cognition and amyotrophic lateral sclerosis. American Journal of Alzheimer’s Disease & Other Dementias, 22(4), 300–312.Google Scholar
Lillo, P., Mioshi, E., Zoing, M.C., Kiernan, M.C., & Hodges, J.R. (2011). How common are behavioural changes in amyotrophic lateral sclerosis? Amyotrophic Lateral Sclerosis, 12, 45–51.Google Scholar
Lillo, P., Mioshi, E., Burrell, J.R., Kiernan, M.C., Hodges, J.R., & Hornberger, M. (2012). Grey and white matter changes across the amyotrophic lateral sclerosis-frontotemporal dementia continuum. Plos One, 7(8), e43993.Google Scholar
Lomen-Hoerth, C., Anderson, T., & Miller, B. (2002). The overlap of amyotrophic lateral sclerosis and frontotemporal dementia. Neurology, 59, 1077–1079.Google Scholar
Lomen-Hoerth, C., Murphy, J., Langmore, S., Kramer, J.H., Olney, R.K., & Miller, B. (2003). Are amyotrophic lateral sclerosis patients cognitively normal? Neurology, 60, 1094–1097.Google Scholar
Ludolph, A.C., Brettschneider, J., & Weishaupt, J.H. (2012). Amyotrophic lateral sclerosis. Current Opinion in Neurology, 25(5), 530–535.Google Scholar
Mackenzie, I.R.A. (2007). The neuropathology of FTD associated with ALS. Alzheimer Disease and Associated Disorders, 21(4), S44–S49.CrossRefGoogle ScholarPubMed
Meier, S.L., Charleston, A.J., & Tippett, L.J. (2010). Cognitive and behavioural deficits associated with the orbitomedial prefrontal cortex in amyotrophic lateral sclerosis. Brain, 133, 3444–3457.Google Scholar
Mioshi, E., Lillo, P., Yew, B., Hsieh, S., Savage, S., Hodges, J.R., … Hornberger, M. (2013). Cortical atrophy in ALS is critically associated with neuropsychiatric and cognitive changes. Neurology, 80(12), 1117–1123.Google Scholar
Neary, D., Snowden, J.S., & Mann, D.M.A. (2000). Cognitive change in motor neurone disease/amyotrophic lateral sclerosis (MND/ALS). Journal of the Neurological Sciences, 180, 15–20.CrossRefGoogle ScholarPubMed
Phukan, J., Pender, N.P., & Hardiman, O. (2007). Cognitive impairment in amyotrophic lateral sclerosis. Lancet Neurology, 6, 994–1003.CrossRefGoogle ScholarPubMed
Rascovsky, K., Hodges, J.R., Knowpman, D., Mendez, M.F., Kramer, J.H., Neuhaus, J., … Miller, B.L. (2011). Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain, 134, 2456–2477.Google Scholar
Ringholz, G.M., Appel, S.H., Bradshaw, M., Cooke, N.A., Mosnik, D.M., & Schulz, P.E. (2005). Prevalence and patterns of cognitive impairment in ALS. Neurology, 65, 586–590.Google Scholar
Schreiber, H., Gaigalat, T., Wiedemuth-Catrinescu, U., Graf, M., Uttner, I., Muche, R., & Ludolph, A.C. (2005). Cognitive function in bulbar- and spinal-onset amyotrophic lateral sclerosis: A longitudinal study in 52 patients. Journal of Neurology, 252, 772–781.Google Scholar
Talbot, K., & Ansorge, O. (2006). Recent advances in the genetics of amyotrophic lateral sclerosis and frontotemporal dementia: Common pathways in neurodegenerative disease. Human Molecular Genetics, 15(Spec No 2), R182–R187.CrossRefGoogle ScholarPubMed
Terada, T., Obi, T., Yoshizumi, M., Murai, T., Miyajima, H., & Mizoguchi, K. (2011). Frontal lobe-mediated behavioral changes in amyotrophic lateral sclerosis: Are they independent of physical disabilities? Journal of the Neurological Sciences, 309, 136–140.Google Scholar
Tranel, D. (2013). Introduction to Section V: Social neuroscience. In D.T. Stuss & R.T. Knight (Eds.), Principles of frontal lobe function (2nd ed., pp. 355–360). New York: Oxford University Press.Google Scholar
Tsermentseli, S., Leigh, P.N., & Goldstein, L.H. (2012). The anatomy of cognitive impairment in amyotrophic lateral sclerosis: More than frontal lobe dysfunction. Cortex, 48, 166–182.Google Scholar
Tsujimoto, M., Senda, J., Ishihara, T., Niimi, Y., Kawai, Y., Atsuta, N., … Sobue, G. (2011). Behavioral changes in early ALS correlate with voxel-based morphometry and diffusion tensor imaging. Journal of the Neurological Sciences, 307, 34–40.CrossRefGoogle ScholarPubMed
Wechsler, D. (2008). Wechsler Adult Intelligence Scale – fourth edition. San Antonio, TX: Pearson.Google Scholar
Wechsler, D. (2009). Wechsler Memory Scale – fourth edition. San Antonio, TX: Pearson.Google Scholar
Witgert, M., Salamone, R., Strutt, A.M., Jawaid, A., Massman, P.J., Bradshaw, M., … Schulz, P.E. (2010). Frontal-lobe mediated behavioral dysfunction in amyotrophic lateral sclerosis. European Journal of Neurology, 17, 103–110.CrossRefGoogle ScholarPubMed
Zalonis, I., Christidi, F., Paraskevas, G., Zabelis, T., Evdokimidis, I., & Kararizou, E. (2012). Can executive cognitive measures differentiate between patients with spinal- and bulbar-onset amyotrophic lateral sclerosis? Archives of Clinical Neuropsychology, 27, 348–354.Google Scholar
Zimmerman, E.K., Eslinger, P.J., Simmons, Z., & Barrett, A.M. (2007). Emotional perception deficits in amyotrophic lateral sclerosis. Cognitive & Behavioral Neurology, 20, 79–82.Google Scholar