Advertisement

Psychiatric Aspects of Wilson Disease

  • Sahil Munjal
  • Paula C. Zimbrean
Chapter
First Online:
Part of the Clinical Gastroenterology book series (CG)

Abstract

Psychiatric disturbances represent an important diagnostic and therapeutic issue in Wilson disease (WD). They represent a significant part of the clinical presentation of WD and can present at any point in the course of the illness. In this chapter, we are going to discuss relevant aspects of the clinical evaluation of psychiatric symptoms in WD, prevalence of psychiatric symptoms, significance of psychiatric symptoms, and treatment considerations.

Keywords

Wilson disease Psychiatry Depression Bipolar disorder Schizophrenia Psychiatric symptoms Treatment 
This is a preview of subscription content, log in to check access.

Bibliography

  1. 1.
    Wilson SAK. Progressive lenticular degeneration: a familial nervous disease associated with cirrhosis of the liver. Brain. 1912;34(4):295–507.CrossRefGoogle Scholar
  2. 2.
    Bornstein RA, McLean DR, Ho K. Neuropsychological and electrophysiological examination of a patient with Wilson disease. Int J Neurosci. 1985;26(3–4):239–47.CrossRefPubMedGoogle Scholar
  3. 3.
    Seniow J, Bak T, Gajda J, Poniatowska R, Czlonkowska A. Cognitive functioning in neurologically symptomatic and asymptomatic forms of Wilson disease. Mov Disord. 2002;17(5):1077–83.CrossRefPubMedGoogle Scholar
  4. 4.
    Portala K, Levander S, Westermark K, Ekselius L, von Knorring L. Pattern of neuropsychological deficits in patients with treated Wilson disease. Eur Arch Psychiatry Clin Neurosci. 2001;251(6):262–8.CrossRefPubMedGoogle Scholar
  5. 5.
    Rosselli M, Lorenzana P, Rosselli A, Vergara I. Wilson disease, a reversible dementia: case report. J Clin Exp Neuropsychol. 1987;9(4):399–406.CrossRefPubMedGoogle Scholar
  6. 6.
    Rathbun JK. Neuropsychological aspects of Wilson disease. Int J Neurosci. 1996;85(3–4):221–9.CrossRefPubMedGoogle Scholar
  7. 7.
    Saint-Laurent M. Schizophrenia and Wilson disease. Can J Psychiatr. 1992;37(5):358–60.CrossRefGoogle Scholar
  8. 8.
    Akil M, Brewer GJ. Psychiatric and behavioral abnormalities in Wilson disease. Adv Neurol. 1995;65:171–8.PubMedGoogle Scholar
  9. 9.
    Oder W, Grimm G, Kollegger H, Ferenci P, Schneider B, Deecke L. Neurological and neuropsychiatric spectrum of Wilson disease: a prospective study of 45 cases. J Neurol. 1991;238(5):281–7.PubMedGoogle Scholar
  10. 10.
    Kumawat BL, Sharma CM, Tripathi G, Ralot T, Dixit S. Wilson disease presenting as isolated obsessive-compulsive disorder. Indian J Med Sci. 2007;61(11):607–10.CrossRefPubMedGoogle Scholar
  11. 11.
    McDonald LV, Lake CR. Psychosis in an adolescent patient with Wilson disease: effects of chelation therapy. Psychosom Med. 1995;57(2):202–4.CrossRefPubMedGoogle Scholar
  12. 12.
    Carr A, McDonnell DJ. Wilson disease in an adolescent displaying an adjustment reaction to a series of life stressors: a case study. J Child Psychol Psychiatry. 1986;27(5):697–700.CrossRefPubMedGoogle Scholar
  13. 13.
    Crumley FE. Pitfalls of diagnosis in the early stages of Wilson disease. J Am Acad Child Adolesc Psychiatry. 1990;29(3):470–1.CrossRefPubMedGoogle Scholar
  14. 14.
    Srinivas K, Sinha S, Taly AB, Prashanth LK, Arunodaya GR, Janardhana Reddy YC, et al. Dominant psychiatric manifestations in Wilson disease: a diagnostic and therapeutic challenge! J Neurol Sci. 2008;266(1–2):104–8.CrossRefPubMedGoogle Scholar
  15. 15.
    Soltanzadeh A, Soltanzadeh P, Nafissi S, Ghorbani A, Sikaroodi H, Lotfi J. Wilson disease: a great masquerader. Eur Neurol. 2007;57(2):80–5.CrossRefPubMedGoogle Scholar
  16. 16.
    Ferenci P, Lockwood A, Mullen K, Tarter R, Weissenborn K, Blei AT. Hepatic encephalopathy--definition, nomenclature, diagnosis, and quantification: final report of the working party at the 11th world congresses of gastroenterology, Vienna, 1998. Hepatology. 2002;35(3):716–21.CrossRefPubMedGoogle Scholar
  17. 17.
    Frederick RT. Current concepts in the pathophysiology and management of hepatic encephalopathy. Gastroenterol Hepatol (N Y). 2011;7(4):222–33.Google Scholar
  18. 18.
    Rosenberg R, Renvillard SG, Hjerrild S. Organic delirious states and other psychiatric disorders: lessons for the hepatologists. Metab Brain Dis. 2013;28(2):235–8.CrossRefPubMedGoogle Scholar
  19. 19.
    Groeneweg M, Quero JC, De Bruijn I, Hartmann IJ, Essink-bot ML, Hop WC, et al. Subclinical hepatic encephalopathy impairs daily functioning. Hepatology. 1998;28(1):45–9.CrossRefPubMedGoogle Scholar
  20. 20.
    Siu AL, Bibbins-Domingo K, Grossman DC, Baumann LC, Davidson KW, Ebell M, et al. Screening for depression in adults: US preventive services task force recommendation statement. JAMA. 2016;315(4):380–7.CrossRefPubMedGoogle Scholar
  21. 21.
  22. 22.
    Kroenke K, Spitzer RL, Williams JB. The PHQ-9: validity of a brief depression severity measure. J Gen Intern Med. 2001;16(9):606–13.CrossRefPubMedPubMedCentralGoogle Scholar
  23. 23.
    Lowe B, Unutzer J, Callahan CM, Perkins AJ, Kroenke K. Monitoring depression treatment outcomes with the patient health questionnaire-9. Med Care. 2004;42(12):1194–201.CrossRefPubMedGoogle Scholar
  24. 24.
    Schaefer M, Gotthardt DN, Ganion N, Wohnsland S, Seessle J, Stremmel W, et al. Wilson disease: health-related quality of life and risk for depression. Clin Res Hepatol Gastroenterol. 2016;40(3):349–56.CrossRefPubMedGoogle Scholar
  25. 25.
    Spitzer RL, Kroenke K, Williams JB, Lowe B. A brief measure for assessing generalized anxiety disorder: the GAD-7. Arch Intern Med. 2006;166(10):1092–7.CrossRefPubMedGoogle Scholar
  26. 26.
    Lowe B, Decker O, Muller S, Brahler E, Schellberg D, Herzog W, et al. Validation and standardization of the Generalized Anxiety Disorder Screener (GAD-7) in the general population. Med Care. 2008;46(3):266–74.CrossRefPubMedGoogle Scholar
  27. 27.
    Kroenke K, Spitzer RL, Williams JB, Monahan PO, Lowe B. Anxiety disorders in primary care: prevalence, impairment, comorbidity, and detection. Ann Intern Med. 2007;146(5):317–25.CrossRefPubMedGoogle Scholar
  28. 28.
    Ketter TA. Strategies for monitoring outcomes in patients with bipolar disorder. Prim Care Companion J Clin Psychiatry. 2010;12(Suppl 1):10–6.CrossRefPubMedPubMedCentralGoogle Scholar
  29. 29.
    Texas Uo. Mood disorder questionnaire. Available from: http://www.dbsalliance.org/pdfs/MDQ.pdf
  30. 30.
    Prashanth LK, Taly AB, Sinha S, Arunodaya GR, Swamy HS. Wilson disease: diagnostic errors and clinical implications. J Neurol Neurosurg Psychiatry. 2004;75(6):907–9.CrossRefPubMedPubMedCentralGoogle Scholar
  31. 31.
    Carta MG, Sorbello O, Moro MF, Bhat KM, Demelia E, Serra A, et al. Bipolar disorders and Wilson disease. BMC Psychiatry. 2012;12:52.CrossRefPubMedPubMedCentralGoogle Scholar
  32. 32.
    Zimbrean PC, Schilsky ML. The spectrum of psychiatric symptoms in Wilson disease: treatment and prognostic considerations. Am J Psychiatry. 2015;172(11):1068–72.CrossRefPubMedGoogle Scholar
  33. 33.
    Walshe JM, Yealland M. Wilson disease: the problem of delayed diagnosis. J Neurol Neurosurg Psychiatry. 1992;55(8):692–6.CrossRefPubMedPubMedCentralGoogle Scholar
  34. 34.
    Frota NAF, Caramelli P, Barbosa ER. Cognitive impairment in Wilson disease. Dement Neuropsychol. 2009;3(1):16–21.CrossRefPubMedPubMedCentralGoogle Scholar
  35. 35.
    Wenisch E, De Tassigny A, Trocello JM, Beretti J, Girardot-Tinant N, Woimant F. Cognitive profile in Wilson disease: a case series of 31 patients. Rev Neurol (Paris). 2013;169(12):944–9.CrossRefGoogle Scholar
  36. 36.
    Riley D, Wiznitzer M, Schwartz S, Zinn AB. A 13-year-old boy with cognitive impairment, retinoblastoma, and Wilson disease. Neurology. 2001;57(1):141–3.CrossRefPubMedGoogle Scholar
  37. 37.
    Lauterbach EC, Cummings JL, Duffy J, Coffey CE, Kaufer D, Lovell M, et al. Neuropsychiatric correlates and treatment of lenticulostriatal diseases: a review of the literature and overview of research opportunities in Huntington’s, Wilson’s, and Fahr’s diseases. A report of the ANPA Committee on Research. American Neuropsychiatric Association. J Neuropsychiatry Clin Neurosci. 1998;10(3):249–66.CrossRefPubMedGoogle Scholar
  38. 38.
    Sinha S, Christopher R, Arunodaya GR, Prashanth LK, Gopinath G, Swamy HS, et al. Is low serum tocopherol in Wilson disease a significant symptom? J Neurol Sci. 2005;228(2):121–3.CrossRefPubMedGoogle Scholar
  39. 39.
    Medalia A, Isaacs-Glaberman K, Scheinberg IH. Neuropsychological impairment in Wilson disease. Arch Neurol. 1988;45(5):502–4.CrossRefPubMedGoogle Scholar
  40. 40.
    Isaacs-Glaberman K, Medalia A, Scheinberg IH. Verbal recall and recognition abilities in patients with Wilson disease. Cortex. 1989;25(3):353–61.CrossRefPubMedGoogle Scholar
  41. 41.
    Zimbrean PC, Schilsky ML. Psychiatric aspects of Wilson disease: a review. Gen Hosp Psychiatry. 2014;36(1):53–62.CrossRefPubMedGoogle Scholar
  42. 42.
    Loudianos G, Gitlin JD. Wilson disease. Semin Liver Dis. 2000;20(3):353–64.CrossRefPubMedGoogle Scholar
  43. 43.
    Depressive Disorders. Diagnostic and statistical manual of mental disorders: DSM Library: Arlington, VA: American Psychiatric Association. 2013.Google Scholar
  44. 44.
    Shanmugiah A, Sinha S, Taly AB, Prashanth LK, Tomar M, Arunodaya GR, et al. Psychiatric manifestations in Wilson disease: a cross-sectional analysis. J Neuropsychiatr Clin Neurosci. 2008;20(1):81–5.CrossRefGoogle Scholar
  45. 45.
    Carta MG, Farina GC, Sorbello O, Moro MF, Cadoni F, Serra A, et al. The risk of bipolar disorders and major depressive disorders in Wilson disease: Results of a case control study. Int Clin Psychopharmacol. 2012;28:e61.CrossRefGoogle Scholar
  46. 46.
    Zimbrean P, Seniow J. Cognitive and psychiatric symptoms in Wilson disease. Handb Clin Neurol. 2017;142:121–40.CrossRefPubMedGoogle Scholar
  47. 47.
    Dening TR, Berrios GE. Wilson disease: a longitudinal study of psychiatric symptoms. Biol Psychiatry. 1990;28(3):255–65.CrossRefPubMedGoogle Scholar
  48. 48.
    Svetel M, Potrebic A, Pekmezovic T, Tomic A, Kresojevic N, Jesic R, et al. Neuropsychiatric aspects of treated Wilson disease. Parkinsonism Relat Disord. 2009;15(10):772–5.CrossRefPubMedGoogle Scholar
  49. 49.
    Dening TR, Berrios GE. Wilson disease. Psychiatric symptoms in 195 cases. Arch Gen Psychiatry. 1989;46(12):1126–34.CrossRefPubMedGoogle Scholar
  50. 50.
    Sagawa M, Takao M, Nogawa S, Mizuno M, Murata M, Amano T, et al. Wilson disease associated with olfactory paranoid syndrome and idiopathic thrombocytopenic purpura. No To Shinkei. 2003;55(10):899–902.PubMedGoogle Scholar
  51. 51.
    Chung YS, Ravi SD, Borge GF. Psychosis in Wilson disease. Psychosomatics. 1986;27(1):65–6.CrossRefPubMedGoogle Scholar
  52. 52.
    Shah N, Kumar D. Wilson disease, psychosis, and ECT. Convuls Ther. 1997;13(4):278–9.PubMedGoogle Scholar
  53. 53.
    Wichowicz HM, Cubala WJ, Slawek J. Wilson disease associated with delusional disorder. Psychiatry Clin Neurosci. 2006;60(6):758–60.CrossRefPubMedGoogle Scholar
  54. 54.
    Huang CC, Chu NS. Wilson disease: clinical analysis of 71 cases and comparison with previous Chinese series. Journal of the Formosan Medical Association = Taiwan yi zhi. 1992;91(5):502–7.PubMedGoogle Scholar
  55. 55.
    Practice guideline for the assessment and treatment of patients with suicidal behaviors. Am J Psychiatry. 2003;160(11 Suppl):1–60.Google Scholar
  56. 56.
    Dening TR, Berrios GE. Wilson disease: a prospective study of psychopathology in 31 cases. Br J Psychiatry. 1989;155:206–13.CrossRefPubMedGoogle Scholar
  57. 57.
    Freudenreich O, Holt DJ, Cather C, Goff DC. The evaluation and management of patients with first-episode schizophrenia: a selective, clinical review of diagnosis, treatment, and prognosis. Harv Rev Psychiatry. 2007;15(5):189–211.CrossRefPubMedGoogle Scholar
  58. 58.
    EASL Clinical Practice Guidelines. Wilson disease. J Hepatol. 2012;56(3):671–85.CrossRefGoogle Scholar
  59. 59.
    Cox DW. A screening test for Wilson disease and its application to psychiatric patients. Can Med Assoc J. 1967;96(2):83–6.PubMedPubMedCentralGoogle Scholar
  60. 60.
    Gedack M, Schönfeldt-Lecuona C, Kratzer W, Kassubek J, Connemann B. P03-538 – ceruloplasmin serum concentrations in a randomized population sample of 2,445 subjects. Eur Psychiatry. 2011;26:1708.CrossRefGoogle Scholar
  61. 61.
    Demily C, Parant F, Cheillan D, Broussolle E, Pavec A, Guillaud O, et al. Screening of Wilson disease in a psychiatric population: difficulties and pitfalls. A preliminary study. Ann General Psychiatry. 2017;16:19.CrossRefGoogle Scholar
  62. 62.
    Brewer GJ. Wilson disease. In: Frucht SJFS, editor. Movement disorder emergencies: diagnosis and treatment. Totowa: Humana Press; 2007. p. 195–208.Google Scholar
  63. 63.
    Finelli PF. Kayser-Fleischer ring: hepatolenticular degeneration (Wilson disease). Neurology. 1995;45(7):1261–2.CrossRefPubMedGoogle Scholar
  64. 64.
    Youn J, Kim JS, Kim HT, Lee JY, Lee PH, Ki CS, et al. Characteristics of neurological Wilson disease without Kayser-Fleischer ring. J Neurol Sci. 2012;323(1–2):183–6.CrossRefPubMedGoogle Scholar
  65. 65.
    Herring NR, Konradi C. Myelin, copper, and the cuprizone model of schizophrenia. Front Biosci (Schol Ed). 2011;3:23–40.Google Scholar
  66. 66.
    Gonzalez-Estecha M, Trasobares EM, Tajima K, Cano S, Fernandez C, Lopez JL, et al. Trace elements in bipolar disorder. J Trace Elem Med Biol. 2011;25(Suppl 1):S78–83.CrossRefPubMedGoogle Scholar
  67. 67.
    Bachmann H, Lossner J, Kuhn HJ, Biesold D, Siegemund R, Kunath B, et al. Long-term care and management of Wilson disease in the GDR. Eur Neurol. 1989;29(6):301–5.CrossRefPubMedGoogle Scholar
  68. 68.
    Modai I, Karp L, Liberman UA, Munitz H. Penicillamine therapy for schizophreniform psychosis in Wilson disease. J Nerv Ment Dis. 1985;173(11):698–701.CrossRefPubMedGoogle Scholar
  69. 69.
    Krim E, Barroso B. Psychiatric disorders treated with clozapine in a patient with Wilson disease. Presse Med. 2001;30(15):738.PubMedGoogle Scholar
  70. 70.
    Kulaksizoglu IB, Polat A. Quetiapine for mania with Wilson disease. Psychosomatics. 2003;44(5):438–9.CrossRefPubMedGoogle Scholar
  71. 71.
    Kontaxakis V, Stefanis C, Markidis M, Tserpe V. Neuroleptic malignant syndrome in a patient with Wilson disease. J Neurol Neurosurg Psychiatry. 1988;51(7):1001–2.CrossRefPubMedPubMedCentralGoogle Scholar
  72. 72.
    Chroni E, Lekka NP, Tsibri E, Economou A, Paschalis C. Acute, progressive akinetic-rigid syndrome induced by neuroleptics in a case of Wilson disease. J Neuropsychiatry Clin Neurosci. 2001;13(4):531–2.CrossRefPubMedGoogle Scholar
  73. 73.
    Bleakley S. Identifying and reducing the risk of antipsychotic drug interactions. Prog Neurol Psychiatry. 2012;16(2):20–4.CrossRefGoogle Scholar
  74. 74.
    Woerwag-Mehta S, Hindley P, Hedderly T, Dhawan A. Complex psychiatric presentation in adolescent onset Wilson disease. BMJ Case Rep. 2011;2011 http//casereports.bmj.com/content/2011/bcr.01.2010.2628.full.pdfGoogle Scholar
  75. 75.
    Loganathan S, Nayak R, Sinha S, Taly AB, Math S, Varghese M. Treating mania in Wilson disease with lithium. J Neuropsychiatry Clin Neurosci. 2008;20(4):487–9.CrossRefPubMedGoogle Scholar
  76. 76.
    Vaishnav P, Gandhi HA. Electro Convulsive Therapy in Psychiatric Manifestations in Wilson disease. Indian J Psychol Med. 2013;35(2):206–8.CrossRefPubMedPubMedCentralGoogle Scholar
  77. 77.
    Walter G, Lyndon B. Depression in hepatolenticular degeneration (Wilson disease). Aust N Z J Psychiatry. 1997;31(6):880–2.CrossRefPubMedGoogle Scholar
  78. 78.
    Gill HH, Shankaran K, Desai HG. Wilson disease: varied hepatic presentations. Indian J Gastroenterol. 1994;13(3):95–8.PubMedGoogle Scholar
  79. 79.
    Knott A, Dieperink E, Willenbring ML, Heit S, Durfee JM, Wingert M, et al. Integrated psychiatric/medical care in a chronic hepatitis C clinic: effect on antiviral treatment evaluation and outcomes. Am J Gastroenterol. 2006;101(10):2254–62.CrossRefPubMedGoogle Scholar
  80. 80.
    Minden SL, Ding L, Cleary PD, Frankel D, Glanz BI, Healy BC, et al. Improving the quality of mental health care in multiple sclerosis. J Neurol Sci. 2013;335(1–2):42–7.CrossRefPubMedGoogle Scholar
  81. 81.
    Wilson Disease Centers of Excellence. Available from: https://www.wilsonsdisease.org/for-patients-families/centers-of-excellence
  82. 82.
    Sorbello O, Riccio D, Sini M, Carta M, Demelia L. Resolved Psychosis after liver transplantation in a patient with Wilson disease. Clin Pract Epidemiol Ment Health. 2011;7:182–4.CrossRefPubMedPubMedCentralGoogle Scholar
  83. 83.
    Lankarani KB, Malek-Hosseini SA, Nikeghbalian S, Dehghani M, Pourhashemi M, Kazemi K, et al. Fourteen years of experience of liver transplantation for Wilson disease; a report on 107 cases from Shiraz, Iran. PLoS One. 2016;11(12):e0167890.CrossRefPubMedPubMedCentralGoogle Scholar
  84. 84.
    Al-Hilou H, Hebbar S, Gunson BK, Claridge LC, Syn WK, Holt AP. Long term outcomes of liver transplantation for Wilson disease: a single centre experience. Gut. 2012;61:A210.CrossRefGoogle Scholar
  85. 85.
    Geissler I, Heinemann K, Rohm S, Hauss J, Lamesch P. Liver transplantation for hepatic and neurological Wilson disease. Transplant Proc. 2003;35(4):1445–6.CrossRefPubMedGoogle Scholar
  86. 86.
    Stracciari A, Tempestini A, Borghi A, Guarino M. Effect of liver transplantation on neurological manifestations in Wilson disease. Arch Neurol. 2000;57(3):384–6.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of PsychiatryYale University School of MedicineNew HavenUSA

Personalised recommendations

Cite chapter

Buy options