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Atypical social cognitive processing in premotor Huntington’s disease: a single case study

Published online by Cambridge University Press:  26 October 2015

T. Burke ,
D. Healy ,
P. Casey ,
O. Hardiman  and
N. Pender
T. Burke*
Affiliation:
Department of Psychology, Beaumont Hospital, Dublin 9, Ireland Academic Unit of Neurology, School of Medicine, Trinity College Dublin, Ireland
D. Healy
Affiliation:
Department of Neurology, Beaumont Hospital, Dublin 9, Ireland
P. Casey
Affiliation:
Department of Psychiatry, The Mater Misericordiae University Hospital, Dublin, Ireland
O. Hardiman
Affiliation:
Academic Unit of Neurology, School of Medicine, Trinity College Dublin, Ireland Department of Neurology, Beaumont Hospital, Dublin 9, Ireland
N. Pender
Affiliation:
Department of Psychology, Beaumont Hospital, Dublin 9, Ireland Academic Unit of Neurology, School of Medicine, Trinity College Dublin, Ireland Department of Psychology, Royal College of Surgeons in Ireland, Dublin, Ireland
*
*Address for correspondence: T. Burke, Department of Psychology, Lower Ground Floor, Beaumont Hospital, Dublin 9, Ireland. (Email: burket2@tcd.ie)
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Abstract

Background

We report the case of a 52-year-old male with pre-motor Huntington’s disease (HD) who has undergone detailed clinical and neuropsychological examination. This patient’s negative symptomatology and behavioural change are having a detrimental impact on his social, occupational and interpersonal life, in the absence of motor symptoms.

Methods

The patient has undergone repeat neuropsychological testing (T1 aged 50; T2 aged 52) with particular focus on executive function and social cognition on repeat testing.

Results

This case details a specific manifestation of HD relating to behavioural, psychiatric and social affective deficits.

Conclusions

This case illustrates how social cognitive changes can occur in HD, months and even years prior to the onset of motor features and how such unrecognized deficits can have a deleterious impact on an individual’s functional ability and lifestyle, before the disease is traditionally considered to have become manifest.

Keywords

Affect deficitempathyHuntington’s diseasesocial-cognition
Type
Case Report
Information
Irish Journal of Psychological Medicine , Volume 34 , Issue 1 , March 2017 , pp. 53 - 58
Copyright
© College of Psychiatrists of Ireland 2015 

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References

Abu-Akel, A, Shamay-Tsoory, S (2011). Neuroanatomical and neurochemical bases of theory of mind. Neuropsychologia 49, 2971–2984.Google Scholar
Bowers, D, Blonder, XM, Heilman, KM (1999). The Florida Affect Battery Revised: Introduction to the Florida Affect Battery. neurology.ufl.edu/files/2011/12/florida-affect-battery-manual.pdf. Last accessed 7 August 2014.Google Scholar
Calder, AJ, Keane, J, Young, AW, Lawrence, AD, Mason, S, Barker, RA (2010). The relation between anger and different forms of disgust: implications for emotional recognition impairments in Huntington’s disease. Neuropsychologia 48, 2719–2729.Google Scholar
Damasio, AR, Maurer, RG (1978). A neurological model for childhood autism. Archives of Neurology 35, 777–786.CrossRefGoogle ScholarPubMed
Delis, DC, Kramer, JH, Kaplan, E, Holdnack, J (2004). Reliability and validity of the Delis-Kaplan executive function system: an update. Journal of the International Neuropsychological Society 10, 301–303.CrossRefGoogle ScholarPubMed
Dubois, B, Slachevsky, A, Litvan, I, Pillon, B (2000). The FAB: a frontal assessment battery at bedside. Neurology 55, 1621–1626.Google Scholar
Duff, K, Beglinger, LJ, Therlault, D, Allison, J, Paulsen, JS (2010). Cognitive deficits in Huntington’s disease on the repeatable battery for the assessment of neuropsychological status. Journal of Clinical and Experimental Neuropsychology 32, 231–238.Google Scholar
Ekman, P (1992). An argument for basic emotions. Cognition and Emotion 6, 169–200.CrossRefGoogle Scholar
Ekman, P (1993). Facial expression and emotion. American Psychologist 48, 384–392.CrossRefGoogle ScholarPubMed
Grace, J, Malloy, PF (2001). Frontal Systems Behaviour Scale: Professional Manual. Psychological Assessment Resources Inc.: Lutz, Florida.Google Scholar
Hart, EP, Dumas, EM, Giltay, EJ, Middelkoop, HAM, Roos, RAC (2013). Cognition in Huntington’s disease in manifest, pre-manifest and converting gene carriers over ten years. Journal of Huntington’s Disease 2, 137–147.CrossRefGoogle Scholar
Henley, SMD, Marianne, NJU, Frost, C, King, J, Tabrizi, SJ, Warren, JD (2012). Emotional recognition in Huntington’s disease: a systematic review. Neuroscience and Biobehavioural Reviews 36, 237–253.CrossRefGoogle ScholarPubMed
Huntington’s Disease Collaborative Research Group (1993). A novel gene containing a trinucleotide repeat that is expanded and unstable in Huntington’s disease chromosomes. Cell 72, 971–983.Google Scholar
Johnson, SA, Stout, JC, Solomon, AC, Langbehn, DR, Aylward, EH, Cruce, CB, Ross, CA, Nance, M, Kayson, E, Julian-Baros, E, Hayden, MR, Kieburtz, K, Guttman, M, Oakes, D, Shoulson, I, Beglinger, L, Duff, K, Penziner, E, Paulsen, JS; Predict-HD Investigators of the Huntington Study Group (2007). Beyond disgust: impaired recognition of negative emotions prior to diagnosis in Huntington’s disease. Brain 130, 1732–1744.CrossRefGoogle ScholarPubMed
Kenny, RA, Coen, RF, Frewen, J, Donoghue, OA, Cronin, H, Savva, GM (2013). Normative values of cognitive and physical function in older adults: findings from the Irish longitudinal study on aging. The American Geriatrics Society 61, 5279–5290.Google Scholar
Midlers, M, Crawford, JR, Lamb, A, Simpson, SA (2003). Differential deficits in expression recognition in gene-carriers and patients with Huntington’s disease. Neuropsychologia 41, 1484–1492.Google Scholar
Morey, LC (1991). The Personality Assessment Inventory Professional Manual. Psychological Assessment Resources: Odessa, Florida.Google Scholar
Morrison, PJ, Johnston, WP, Nevin, NC (1995). The epidemiology of Huntington’s disease in Northern Ireland. Journal of Medical Genetics 32, 524–530.CrossRefGoogle ScholarPubMed
Müller, KI, Bekkelund, SI (2013). Visual impairment and posterior cortical atrophy preceding rapid progressive dementia. BMJ Case Reports 2, 1757–1790.Google Scholar
Nasreddine, ZS, Philips, NA, Bédirian, V, Charbonneau, S, Whitehead, V, Collin, I, Cummins, JL, Chertkow, H (2005). The montreal cognitive assessment, MoCA: a brief screening tool for mild cognitive impairment. Journal of the American Geriatrics Society 53, 695–699.Google Scholar
Novak, MJ, Tabrizi, SJ (2010). Huntington’s disease. British Medical Journal 340, 34–40.CrossRefGoogle ScholarPubMed
Paulsen, JS, Long, JD (2014). Onset of Huntington’s disease: can it be purely cognitive? Movement Disorders 29, 1342–1360.CrossRefGoogle ScholarPubMed
Paulsen, JS, Smith, MM, Long, JD, The PREDICT HD Investigators and Coordinators of the Huntington’s Study Group (2013). Cognitive decline in prodromal Huntington’s disease: implications for clinical trials. Cognitive Neurology 84, 1233–1239.Google ScholarPubMed
Randolph, C (1998). Repeatable Battery for the Assessment of Neuropsychological Status Manual. The Psychological Corporation: San Antonio.Google Scholar
Rees, EM, Scahill, RI, Hobbs, NZ (2013). Longitudinal neuroimaging biomarkers in Huntington’s disease. Journal of Huntington’s Disease 2, 21–39.Google Scholar
Shamay-Tsoory, SG, Aharon-Peretz, J (2007). Dissociable prefrontal networks for cognitive and affective theory of mind: a lesion study. Neuropsychologia 45, 3054–3067.CrossRefGoogle ScholarPubMed
Snowden, JS, Austin, NA, Sembi, S, Thompson, JC, Craufurd, D, Neary, D (2008). Emotional recognition in Huntington’s disease and frontotemporal dementia. Neuropsychologia 46, 2638–2649.Google Scholar
Snowden, JS, Gibbon, ZC, Blackshaw, A, Doubleday, E, Thompson, J, Craufurd, D, Foster, J, Happé, F, Neary, D (2003). Social cognition in frontotemporal dementia and Huntington’s disease. Neuropsychologia 41, 688–701.CrossRefGoogle ScholarPubMed
Sprengelmeyer, R, Schroeder, U, Young, AW, Epplen, JT (2006). Disgust in pre-clinical Huntington’s disease: a longitudinal study. Neuropsychologia 44, 518–533.Google Scholar
Stone, VE, Baron-Cohen, S, Knight, RT (1998). Frontal lobe contributions to theory of mind. Journal of Cognitive Neuroscience 10, 640–656.Google Scholar
Walker, FO (2007). Huntington’s disease. Lancet 369, 218–228.Google Scholar