Hostname: page-component-cd9895bd7-7cvxr Total loading time: 0 Render date: 2024-12-31T15:00:30.212Z Has data issue: false hasContentIssue false
  • English
  • Français

Wilson's Disease: A Prospective Study of Psychopathology in 31 Cases

Published online by Cambridge University Press:  02 January 2018

T. R. Dening  and
G. E. Berrios
T. R. Dening
Affiliation:
Oxford Regional Health Authority, lately Research Fellow, Department of Psychiatry, University of Cambridge
G. E. Berrios*
Affiliation:
Department of Psychiatry, University of Cambridge Clinical School
*
∗Addenbrooke's Hospital, Hills Road, Cambridge CB2 2QQ
Get access Rights & Permissions [Opens in a new window]

Abstract

A prospective neuropsychiatric study of 31 consecutive subjects with Wilson's disease is reported. Exploratory factor analysis yielded four factors: neurological, cognitive, hepatic/depressive and psychiatric. Significant associations were found between a ‘psychopathic’ personality factor and neurological and dysarthria scores. The d’ and β coefficients of a signal-detection memory task dissociated: the former correlated only with Mini-Mental State and Benton visuospatial task; and the latter with depressive symptoms. Using discriminant function analysis, there was efficient classification of ‘psychopathic’ personality by dysarthria, and of individual depressive symptoms by disturbance of gait. Biochemical markers of hepatic dysfunction were significantly associated with certain depressive symptoms. No evidence emerged to support the putative association with schizophrenia-like psychosis.

Type
Papers
Information
The British Journal of Psychiatry , Volume 155 , Issue 2 , August 1989 , pp. 206 - 213
Copyright
Copyright © Royal College of Psychiatrists, 1989 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

Footnotes

∗

An abridged version of this paper was presented at the Quarterly Meeting of the Royal College of Psychiatrists, Cambridge, April 1988.

References

Asberg, M., Perris, C., Schalling, D., et al (1978) The CPRS - development and applications of a psychiatric rating scale. Acta Psychiatrica Scandinavica (suppl. 271).Google Scholar
Beard, A. W. (1959) The association of hepatolenticular degeneration with schizophrenia. Acta Psychiatrica Neurologica Scandinavica, 34, 411–428.Google Scholar
Benton, A. L., Hannay, H. J. & Varney, N. R. (1975) Visual perception of line direction in patients with unilateral brain disease. Neurology, 25, 907–910.Google Scholar
Benton, A. L., Varney, N. R. & Hamsher, K. (1978) Visuospatial judgement: a clinical test. Archives of Neurology, 35, 364–367.CrossRefGoogle ScholarPubMed
Bornstein, R. A., McLean, D. R. & Ho, K. (1985) Neuropsychological and electrophysiological examination of a patient with Wilson's disease. International Journal of Neuroscience, 26, 239–247.Google Scholar
Canter, C. J., de la Torre, R. & Mier, M. (1961) A method of evaluating disability in patients with Parkinson's disease. Journal of Nervous and Mental Disease, 133, 143–147.Google Scholar
Davis, L. J. & Goldstein, N. P. (1974) Psychologic investigation of Wilson's disease. Mayo Clinic Proceedings, 49, 409–411.Google Scholar
Dening, T. R. (1985) Psychiatric aspects of Wilson's disease. British Journal of Psychiatry, 147, 677–682.Google Scholar
Dening, T. R. & Berrios, G. E. (1989) Wilson's disease: psychiatric symptomatology in 195 cases. Archives of General Psychiatry (in press).Google Scholar
Enderby, P. (1983) Frenchay Dysarthria Assessment. Windsor: NFER-Nelson.Google Scholar
Endicott, J., Spitzer, R. L., Fleiss, J. L., et al (1976) The Global Assessment Scale: a procedure for measuring overall severity of psychiatric disturbance. Archives of General Psychiatry, 33, 766–771.Google Scholar
Folstein, M. F., Folstein, S. E. & McHugh, P. R. (1975) ‘Mini-Mental State’: a practical method for grading the cognitive state of patients for the clinician. Journal of Psychiatric Research, 12, 189–198.Google Scholar
Goldberg, D. P. (1972) The Detection of Psychiatric Illness by Questionnaire. London: Oxford University Press.Google Scholar
Goldstein, N. P., Ewert, J. C., Randall, R. V., et al (1968) Psychiatric aspects of Wilson's disease (hepatolenticular degeneration): results of psychometric tests during long-term therapy. American Journal of Psychiatry, 124, 113–119.Google Scholar
Gouse, A. T. (1975) MD thesis. Yale University.Google Scholar
Griffiths, R. D. (1970) Personality assessment In The Psychological Assessment of Mental and Physical Handicap (ed. Mittler P.). London: Methuen.Google Scholar
Hayek, H. W., Knoll, E. & Widhalm, S. (1973) Über das organische Psychosyndrom bei Morbus Wilson. Monatsschrift Kinderheilkunde, 121, 679–683.Google Scholar
Hochhaus, L. (1972) A table for the calculation of d’ and β. Psychological Bulletin, 77, 375–376.Google Scholar
Karson, C. N. (1983) Spontaneous eye-blink rates and dopa-minergic systems. Brain, 106, 643–653.Google Scholar
Knehr, C. A. & Bearn, A. G. (1956) Psychological impairment in Wilson's disease. Journal of Nervous and Mental Disease, 124, 251–255.Google Scholar
Marsden, C. D. & Schachter, M. (1981) Assessment of extrapyramidal disorders. British Journal of Clinical Pharmacology, 11, 129–151.CrossRefGoogle ScholarPubMed
Medalia, A., Isaacs-Glaberman, K. & Scheinberg, I. H. (1988) Neuropsychological impairment in Wilson's disease. Archives of Neurology, 45, 502–504.Google Scholar
Miller, E. & Lewis, P. (1977) Recognition memory in elderly patients with depression and dementia: a signal detection analysis. Journal of Abnormal Psychology, 86, 84–86.Google Scholar
Miller, E., Berrios, G. E. & Politynska, B. (1987) The adverse effect of benzhexol on memory in Parkinson's disease. Acta Neurologica Scandinavica, 76, 278–282.CrossRefGoogle ScholarPubMed
Montgomery, S., Asberg, M., Jörnestedt, L., et al (1978) Reliability of the CPRS between the disciplines of psychiatry, general practice, nursing and psychology in depressed patients. Acta Psychiatrica Scandinavica, (suppl. 271), 29–32.Google Scholar
Rosselli, M., Lorenzana, P., Rosselli, A., et al (1987) Wilson's disease: a reversible dementia: case report. Journal of Clinical and Experimental Neuropsychology, 9, 399–406.CrossRefGoogle ScholarPubMed
Scheinberg, I. H., Sternlieb, I. & Richman, J. (1968) Psychiatric manifestations in patients with Wilson's disease In Wilson's Disease (ed. Bergsma D.). Birth Defects Original Articles Series , Vol. 4, No. 2. New York: The National Foundation.Google Scholar
Spitznagel, E. L. & Helzer, J. E. (1985) A proposed solution to the base rate problem in the kappa statistic. Archives of General Psychiatry, 42, 725–728.Google Scholar
SPSS Inc. (1986) SPSS-PC +. Chicago: SPSS.Google Scholar
Summers, W. K. (1978) A clinical method of estimating drug-induced delirium. Life Sciences, 22, 1511–1516.CrossRefGoogle ScholarPubMed
Tabachnick, B. G. & Fidell, L. S. (1983) Using Multivariate Statistics. New York: Harper & Row.Google Scholar
Tarter, R. E., Switala, J., Carra, J., et al (1987) Neuropsychological impairment associated with hepatolenticular degeneration (Wilson's disease) in the absence of overt encephalopathy. International Journal of Neuroscience, 37, 67–71.Google Scholar
Tyrer, P. & Alexander, J. (1979) Classification of personality disorder. British Journal of Psychiatry, 135, 163–167.Google Scholar
Tyrer, P. & Alexander, J. (1985) Revised Classification of Personality Using the Personality Assessment Schedule. Nottingham: Mapperly Hospital.Google Scholar
Webster, D. D. (1968) Clinical analysis of the disability in Parkinson's disease. Modern Treatment, 5, 257–282.Google Scholar
Wing, J. K., Cooper, J. E. & Sartorius, N. (1974) The Measurement and Classification of Psychiatric Symptoms. Cambridge: Cambridge University Press.Google Scholar
Submit a response

eLetters

No eLetters have been published for this article.