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Self-awareness of Motor Dysfunction in Patients with Huntington's Disease in Comparison to Parkinson's Disease and Cervical Dystonia

Published online by Cambridge University Press:  08 June 2011

Emilia J. Sitek*
Affiliation:
Department of Neurological and Psychiatric Nursing, Medical University of Gdansk, Poland Department of Neurology, St. Adalbert Hospital, Gdansk, Poland
Witold Sołtan
Affiliation:
Department of Neurology, St. Adalbert Hospital, Gdansk, Poland
Dariusz Wieczorek
Affiliation:
Department of Rehabilitation, Medical University of Gdansk, Poland
Michał Schinwelski
Affiliation:
Department of Neurological and Psychiatric Nursing, Medical University of Gdansk, Poland Department of Neurology, St. Adalbert Hospital, Gdansk, Poland
Piotr Robowski
Affiliation:
Department of Neurological and Psychiatric Nursing, Medical University of Gdansk, Poland Department of Neurology, St. Adalbert Hospital, Gdansk, Poland
Ralf Reilmann
Affiliation:
Department of Neurology, University of Muenster, Germany
Katarzyna Guzińska
Affiliation:
Department of Clinical Psychology and Neuropsychology, University of Gdansk, Poland
Michał Harciarek
Affiliation:
Department of Clinical Psychology and Neuropsychology, University of Gdansk, Poland
Wioletta Krysa
Affiliation:
Department of Genetics, Institute of Psychiatry and Neurology, Warsaw, Poland
Jarosław Sławek
Affiliation:
Department of Neurological and Psychiatric Nursing, Medical University of Gdansk, Poland Department of Neurology, St. Adalbert Hospital, Gdansk, Poland
*
Correspondence and reprint requests to: Emilia J. Sitek, St. Adalbert Hospital, Neurology Department, Al. Jana Pawla II 50, 80-462 Gdansk, Poland. E-mail: emsitek@gmail.com

Abstract

Individuals suffering from Huntington's disease (HD) have been shown to present with poor self-awareness of a variety of symptoms. The aim of this study was to better assess the self-awareness of motor symptoms and activities of daily living (ADL) impairment in HD, in comparison to Parkinson's disease (PD) and cervical dystonia (CD). In particular, the anosognosia/anosodiaphoria of involuntary movements has been investigated. Self-awareness was tested in 23 patients with HD by comparing patient and caregiver ratings in reference to clinical control groups (25 PD with dyskinesias, PDdys; 21 PD without dyskinesias, PDndys; and 20 with CD). Patients were assessed neurologically by relevant rating scales. Self-awareness was tested using a scale based on 15 films demonstrating 3 types of motor symptoms (chorea/dyskinesias, parkinsonism, torticollis) as well as the Self-Assessment Parkinson's Disease Disability Scale. General cognitive status, verbal learning, cognitive control, and mood were also analyzed. Our results indicate that self-awareness of choreic movements was affected more severely in HD than in PDdys, despite comparable cognitive status. Patient–proxy agreement on ADL impairment was roughly similar in all clinical groups. The results are discussed in the context of orbitofrontal–limbic pathology as a potential trigger of anosognosia/anosodiaphoria in individuals with HD. (JINS, 2011, 17, 788–795)

Type
Regular Articles
Copyright
Copyright © The International Neuropsychological Society 2011

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References

Albanese, A., Barnes, M.P., Bhatia, K.P., Fernandez-Alvarez, E., Filippini, G., Gasser, T., Valls-Solè, J. (2006). A systematic review on the diagnosis and treatment of primary (idiopathic) dystonia and dystonia plus syndromes: Report of an EFNS/MDS-ES Task Force. European Journal of Neurology, 13, 433444.CrossRefGoogle ScholarPubMed
Brown, R.G. MacCarthy, B., Jahanashi, M., Marsden, C.D. (1989). Accuracy of self-reported disability in patients with Parkinsonism. Archives of Neurology, 46, 955959.CrossRefGoogle ScholarPubMed
Caracci, G., Mukherjee, S., Roth, S.D., Decina, P. (1990). Subjective awareness of abnormal involuntary movements in chronic schizophrenic patients. American Journal of Psychiatry, 147, 295298.Google Scholar
Choynowski, M., Kostro, B. (1980). Rey Auditory Verbal Learning Test Manual. Warsaw: PWN Press (in Polish).Google Scholar
Comella, C.L., Stebbins, G.T., Goetz, C.G., Chmura, T.A., Bressman, S.B., Lang, A.E. (1997). Teaching tape for the motor section of the Toronto Western Spasmodic Torticollis Scale. Movement Disorders, 12, 570575.CrossRefGoogle ScholarPubMed
Consky, E.S., Basinski, A., Bele, L., Ranawaya, R., Lang, A.E. (1990). The Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS): Assessment of validity and inter-rater reliability. Neurology, 40, 445.Google Scholar
Craufurd, D., Snowden, J. (2002). Neuropsychological and neuropsychiatric aspects of Huntington'a disease. In G. Bates, P.S. Harper, & L. Jones (Eds.), Huntington's Disease (pp. 6294). New York: Oxford University Press.Google Scholar
Deckel, A.W., Morrison, D. (1996). Evidence of a neurologically based “denial of illness” in patients with Huntington's disease. Archives of Clinical Neuropsychology, 11, 295302.CrossRefGoogle ScholarPubMed
Douaud, G., Behrens, T.E., Poupon, C., Cointepas, Y., Jbabdi, S., Gaura, V., Remy, P. (2009). In vivo evidence for the selective subcortical degeneration in Huntington's disease. Neuroimage, 46, 958966.CrossRefGoogle ScholarPubMed
Draganski, B., Thun-Hohenstein, C., Bogdahn, U., Winkler, J., May, A. (2003). “Motor circuit” gray matter changes in idiopathic cervical dystonia. Neurology, 61, 12281231.CrossRefGoogle ScholarPubMed
Duff, K., Paulsen, J.S., Beglinger, L.J., Langbehn, D.R., Wang, C., Stout, J.C., PREDICT-HD Investigators of the Huntington Study Group. (2010). “Frontal” behaviours before the diagnosis of Huntington's disease and their relationship to markers of disease progression: Evidence of early lack of awareness. Journal of Neuropsychiatry and Clinical Neurosciences, 22, 196207.CrossRefGoogle ScholarPubMed
European Huntington's Disease Network (EHDN) Cognitive Working Group (2009). EHDN neuropsychological assessment protocol-manual for test administration and scoring. Ulm, Germany: EDHN.Google Scholar
Fleming, A., Cook, K.F., Nelson, N.D., Lai, E.C. (2005). Proxy reports in Parkinson's disease: Caregiver and patient self-reports of quality of life and physical activity. Movement Disorders, 20, 14621468.CrossRefGoogle ScholarPubMed
Folstein, M.F., Folstein, S.E., McHugh, P.R. (1975). Mini-mental state. Journal of Psychiatric Research, 12, 189198.CrossRefGoogle ScholarPubMed
Goetz, C.G., Stebbins, G.T., Chmura, T.A., Fahn, S., Klawans, H.L., Marsden, C.D. (1995). Teaching tape for the motor section of the Unified Parkinson's Disease Rating Scale. Movement Disorders, 10, 263266.Google Scholar
Halliday, G.M., McRitchie, D.A., Macdonald, V., Double, K.L., Trent, R.J., McCusker, E. (1998). Regional specificity of brain atrophy in Huntington's disease. Experimental Neurology, 154, 663672.CrossRefGoogle ScholarPubMed
Heilman, K.M., Harciarek, M. (2010). Anosognosia and anosodiaphoria of weakness. In G.P. Prigatano (Ed.), The study of anosognosia. New York: Oxford University Press.Google Scholar
Ho, A.K., Gilbert, A.S., Mason, S.L., Goodman, A.O., Barker, R.A. (2009). Health-related quality of life in Huntington's disease: Which factors matter most? Movement Disorders, 24, 574578.CrossRefGoogle ScholarPubMed
Ho, A.K., Robbins, A.O., Barker, R.A. (2006). Huntington's disease patients have selective problems with insight. Movement Disorders, 21, 385389.CrossRefGoogle ScholarPubMed
Hoth, K.F., Paulsen, J.S., Moser, D.J., Tranel, D., Clark, L.A., Bechara, A. (2007). Patients with Huntington's disease have impaired awareness of cognitive, emotional and functional abilities. Journal of Clinical and Experimental Neuropsychology, 29, 365376.CrossRefGoogle ScholarPubMed
Huntington Study Group. (1996). Unified Huntington's Disease Rating Scale: Reliability and consistency. Movement Disorders, 11, 136142.CrossRefGoogle Scholar
Jankovic, J. (2005). Motor fluctuations and dyskinesias in Parkinson's disease: Clinical manifestations. Movement Disorders, 20, 1116.CrossRefGoogle ScholarPubMed
Kremer, B. (2002). Clinical neurology of Huntington's disease. In G. Bates, P.S. Harper, & L. Jones. (Eds.), Huntington's disease (pp. 2861). New York: Oxford University Press.Google Scholar
Leritz, E., Loftis, C., Crucian, G., Friedman, W., Bowers, D. (2004). Self-awareness of deficits in Parkinson disease. The Clinical Neuropsychologist, 18, 352361.CrossRefGoogle ScholarPubMed
Litvan, I., Bhatia, K.P., Burn, D.J., Goetz, C.G., Lang, A.E., McKeith, I., Wenning, G.K. (2003). Movement Disorders Society Scientific Issues Committee report: SIC Task Force appraisal of clinical diagnostic criteria for parkinsonian disorders. Movement Disorders, 18, 467486.CrossRefGoogle ScholarPubMed
Lyoo, C.H., Ryu, Y.H., Lee, M.S. (2010). Topographical distribution of cerebral cortical thinning in patients with mild Parkinson's disease without dementia. Movement Disorders, 25, 496499.Google Scholar
Martínez-Martín, P., Benito-León, J., Alonso, F., Catalan, M.J., Pondal, M., Tobias, A., Zamarbide, I. (2003). Patients’, doctors’, and caregivers’ assessment of disability using the UPDRS-ADL section: Are these ratings interchangeable? Movement Disorders, 18, 985992.CrossRefGoogle ScholarPubMed
Mathias, J.L. (2003). Neurobehavioral functioning of persons with Parkinson's disease. Applied Neuropsychology, 10, 5768.CrossRefGoogle ScholarPubMed
McRae, C., Diem, G., Vo, A., O'Brien, C., Seeberger, L. (2002). Reliability of measurements of patient health status: A comparison of physician, patient and caregiver ratings. Parkinsonism and Related Disorders, 8, 187192.CrossRefGoogle ScholarPubMed
Mestre, T., Ferreira, J., Coelho, M.M., Rosa, M., Sampaio, C. (2009). Therapeutic interventions for symptomatic treatment in Huntington's Disease. Cochrane Database of Systematic Reviews. (3), CD006456.Google ScholarPubMed
Montgomery, S.A., Asberg, M. (1979). A new depression scale designed to be sensitive to change. British Journal of Psychiatry, 134, 382389.CrossRefGoogle ScholarPubMed
Paulson, H.L., Stern, M.B. (1997). Clinical manifestations of Parkinson's disease. In R.L. Watts & W.C. Koller (Eds.), Movement disorders – neurologic principles and practice (pp. 183199). New York: McGraw-Hill.Google Scholar
Reilmann, R., Roos, R.A.C., Rosser, A., Grimbergen, Y., Kraus, P., Craufurd, D., Landwehrmeyer, G.B. (2009). A teaching film, video library and online certification for the Unified Huntington's Disease Rating Scale Total Motor Score. Aktuelle Neurologie, 36, 116.Google Scholar
Rosas, H.D., Lee, S.Y., Bender, A.C., Zaleta, A.K., Vangel, M., Yu, P., Hersch, S.M. (2010). Altered white matter microstructure in the corpus callosum in Huntington's disease: Implications for cortical “disconnection”. Neuroimage, 49, 29953004.Google Scholar
Schrag, A., Jahanshahi, M., Quinn, N. (2000). How does Parkinson's disease affect quality of life? A comparison with quality of life in the general population. Movement Disorders, 15, 11121118.3.0.CO;2-A>CrossRefGoogle ScholarPubMed
Shenker, J.I., Wylie, S.A., Fuchs, K., Manning, C.A., Heilman, K.M. (2004). On-line anosognosia. Unawareness for chorea in real time but not on videotape delay. Neurology, 63, 159160.CrossRefGoogle Scholar
Siegel, S., Castellan, N.J. (1988). Nonparametric statistics for the behavioral sciences. New York: McGraw-Hill.Google Scholar
Sitek, E.J., Slawek, J., Wieczorek, D. (2008). Self-awareness of deficits in Huntington's and Parkinson's disease [in Polish]. Psychiatria Polska, 42, 393403.Google ScholarPubMed
Snowden, J.S., Craufurd, D., Griffiths, H.L., Neary, D. (1998). Awareness of involuntary movements in Huntington disease. Archives of Neurology, 55, 801805.Google Scholar
Stroop, J.R. (1935). Studies on interference in serial verbal reactions. Journal of Experimental Psychology, 28, 643662.CrossRefGoogle Scholar
Tinaz, S., Courtney, M.G., Stern, C.E. (2011). Focal cortical and subcortical atrophy in early Parkinson's disease. Movement Disorders, 26(3), 436441.CrossRefGoogle ScholarPubMed
Vitale, C., Pellecchia, M.T., Grossi, D., Fragassi, N., Cuomo, T., Di Maio, L., Barone, P. (2001). Unawareness of dyskinesias in Parkinson's and Huntington's diseases. Neurological Sciences, 22, 105106.CrossRefGoogle ScholarPubMed
White, R.F., Vasterling, J.J., Koroshetz, W., Myers, R. (1992). Neuropsychology of Huntington's disease. In R.F. White (Ed.), Clinical syndromes in adult Neuropsychology (pp. 213252). Amsterdam, Netherlands: Elsevier.Google Scholar