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Treatment of Neurological Symptoms in Wilson Disease

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Management of Wilson Disease

Part of the book series: Clinical Gastroenterology ((CG))

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Abstract

Brain copper accumulation commonly causes neurological symptoms in Wilson disease (WD). Many of the neurological manifestations that are present in WD involve movement disorders such as tremor, dysarthria, parkinsonism, dystonia, and gait impairment. Current de-coppering therapy provides in most cases only partial improvement of the neurological aspects of WD, and the use of medications to treat residual neurological symptoms is commonly required to improve patients’ quality of life. The evidence assessing the efficacy of different therapeutic options for movement disorders in WD is limited. This chapter aims to review the most common neurological manifestations in WD and the therapeutic options currently available.

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Bibliography

  1. Brewer GJ, Yuzbasiyan-Gurkan V. Wilson disease. Medicine (Baltimore). 1992;71(3):139–64.

    Article  CAS  Google Scholar 

  2. Starosta-Rubinstein S, Young AB, Kluin K, Hill G, Aisen AM, Gabrielsen T, et al. Clinical assessment of 31 patients with Wilson disease. Correlations with structural changes on magnetic resonance imaging. Arch Neurol. 1987;44(4):365–70.

    Article  CAS  PubMed  Google Scholar 

  3. Dastur DK, Manghani DK, Wadia NH. Wilson disease in India. I. Geographic, genetic, and clinical aspects in 16 families. Neurology. 1968;18(1 Pt 1):21–31.

    Article  CAS  PubMed  Google Scholar 

  4. Taly AB, Meenakshi-Sundaram S, Sinha S, Swamy HS, Arunodaya GR. Wilson disease: description of 282 patients evaluated over 3 decades. Medicine (Baltimore). 2007;86(2):112–21.

    Article  Google Scholar 

  5. Huang CC, Chu NS. Wilson disease: clinical analysis of 71 cases and comparison with previous Chinese series. J Formos Med Assoc Taiwan Yi Zhi. 1992;91(5):502–7.

    PubMed  CAS  Google Scholar 

  6. Lorincz MT. Neurologic Wilson disease. Ann N Y Acad Sci. 2010;1184:173–87.

    Article  CAS  PubMed  Google Scholar 

  7. Merle U, Schaefer M, Ferenci P, Stremmel W. Clinical presentation, diagnosis and long-term outcome of Wilson disease: a cohort study. Gut. 2007;56(1):115–20.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. 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.

    PubMed  CAS  Google Scholar 

  9. Członkowska A, Litwin T, Chabik G. Wilson disease: neurologic features. Handb Clin Neurol. 2017;142:101–19.

    Article  PubMed  Google Scholar 

  10. Nazer H, Brismar J, al-Kawi MZ, Gunasekaran TS, Jorulf KH. Magnetic resonance imaging of the brain in Wilson disease. Neuroradiology. 1993;35(2):130–3.

    Article  CAS  PubMed  Google Scholar 

  11. Algin O, Taskapilioglu O, Hakyemez B, Ocakoglu G, Yurtogullari S, Erer S, et al. Structural and neurochemical evaluation of the brain and pons in patients with Wilson disease. Jpn J Radiol. 2010;28(9):663–71.

    Article  CAS  PubMed  Google Scholar 

  12. Zimbrean PC, Schilsky ML. Psychiatric aspects of Wilson disease: a review. Gen Hosp Psychiatry. 2014;36(1):53–62.

    Article  PubMed  Google Scholar 

  13. Machado A, Chien HF, Deguti MM, Cançado E, Azevedo RS, Scaff M, et al. Neurological manifestations in Wilson disease: report of 119 cases. Mov Disord Off J Mov Disord Soc. 2006;21(12):2192–6.

    Article  Google Scholar 

  14. Stremmel W, Meyerrose KW, Niederau C, Hefter H, Kreuzpaintner G, Strohmeyer G. Wilson disease: clinical presentation, treatment, and survival. Ann Intern Med. 1991;115(9):720–6.

    Article  CAS  PubMed  Google Scholar 

  15. Brewer GJ, Fink JK, Hedera P. Diagnosis and treatment of Wilson disease. Semin Neurol. 1999;19(3):261–70.

    Article  CAS  PubMed  Google Scholar 

  16. Hölscher S, Leinweber B, Hefter H, Reuner U, Günther P, Weiss KH, et al. Evaluation of the symptomatic treatment of residual neurological symptoms in Wilson disease. Eur Neurol. 2010;64(2):83–7.

    Article  PubMed  Google Scholar 

  17. Litwin T, Dušek P, Członkowska A. Symptomatic treatment of neurologic symptoms in Wilson disease. Handb Clin Neurol. 2017;142:211–23.

    Article  PubMed  Google Scholar 

  18. Louis ED. Diagnosis and management of tremor. Contin Minneap Minn. 2016;22(4 Movement Disorders):1143–58.

    Google Scholar 

  19. Ondo W. Essential tremor: what we can learn from current pharmacotherapy. Tremor Hyperkinetic Mov N Y N. 2016;6:356.

    Google Scholar 

  20. Svetel M, Kozić D, Stefanova E, Semnic R, Dragasevic N, Kostic VS. Dystonia in Wilson disease. Mov Disord Off J Mov Disord Soc. 2001;16(4):719–23.

    Article  CAS  Google Scholar 

  21. Marsden CD, editor. Movement disorders: neurology 2: Buttwerworth-Heinemann Oxford, UK; 2013. 394 p.

    Google Scholar 

  22. Deuschl G. Dystonic tremor. Rev Neurol (Paris). 2003;159(10 Pt 1):900–5.

    CAS  Google Scholar 

  23. Fasano A, Bove F, Lang AE. The treatment of dystonic tremor: a systematic review. J Neurol Neurosurg Psychiatry. 2014;85(7):759–69.

    Article  PubMed  Google Scholar 

  24. Fahn S, Jankovic J, Hallett M. Principles and practice of movement disorders, vol. 3330: Elsevier Health Sciences Saunders Elsevier Philadelphia, USA; 2011.

    Google Scholar 

  25. Pellizzari R, Rossetto O, Schiavo G, Montecucco C. Tetanus and botulinum neurotoxins: mechanism of action and therapeutic uses. Philos Trans R Soc Lond B Biol Sci. 1999;354(1381):259–68.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  26. Dressler D, Altenmueller E, Bhidayasiri R, Bohlega S, Chana P, Chung TM, et al. Strategies for treatment of dystonia. J Neural Transm Vienna Austria 1996. 2016;123(3):251–8.

    CAS  Google Scholar 

  27. Thenganatt MA, Jankovic J. Treatment of dystonia. Neurother J Am Soc Exp Neurother. 2014;11(1):139–52.

    Article  CAS  Google Scholar 

  28. Maltese M, Martella G, Madeo G, Fagiolo I, Tassone A, Ponterio G, et al. Anticholinergic drugs rescue synaptic plasticity in DYT1 dystonia: role of M1 muscarinic receptors. Mov Disord Off J Mov Disord Soc. 2014;29(13):1655–65.

    Article  CAS  Google Scholar 

  29. Fahn S. High dosage anticholinergic therapy in dystonia. Neurology. 1983;33(10):1255–61.

    Article  CAS  PubMed  Google Scholar 

  30. McFarland NR. Diagnostic approach to atypical Parkinsonian syndromes. Contin Minneap Minn. 2016;22(4 Movement Disorders):1117–42.

    Google Scholar 

  31. da Silva-Júnior FP, Carrasco AE a B, da Silva Mendes AM, Lopes AJT, Nobre E Souza MA, de Bruin VMS. Swallowing dysfunction in Wilson disease: a scintigraphic study. Neurogastroenterol Motil Off J Eur Gastrointest Motil Soc. 2008;20(4):285–90.

    Article  Google Scholar 

  32. Członkowska A, Tarnacka B, Litwin T, Gajda J, Rodo M. Wilson disease-cause of mortality in 164 patients during 1992–2003 observation period. J Neurol. 2005;252(6):698–703.

    Article  PubMed  Google Scholar 

  33. Wilson DC, Phillips MJ, Cox DW, Roberts EA. Severe hepatic Wilson disease in preschool-aged children. J Pediatr. 2000;137(5):719–22.

    Article  CAS  PubMed  Google Scholar 

  34. Iorio R, D’Ambrosi M, Mazzarella G, Varrella F, Vecchione R, Vegnente A. Early occurrence of hypertransaminasemia in a 13-month-old child with Wilson disease. J Pediatr Gastroenterol Nutr. 2003;36(5):637–8.

    Article  PubMed  Google Scholar 

  35. Tissières P, Chevret L, Debray D, Devictor D. Fulminant Wilson disease in children: appraisal of a critical diagnosis. Pediatr Crit Care Med J Soc Crit Care Med World Fed Pediatr Intensive Crit Care Soc. 2003;4(3):338–43.

    Google Scholar 

  36. Roberts EA, Socha P. Wilson disease in children. Handb Clin Neurol. 2017;142:141–56.

    Article  PubMed  Google Scholar 

  37. Noureen N, Rana MT. Neurological Wilson disease in children: a three years experience from Multan. JPMA J Pak Med Assoc. 2011;61(8):743–8.

    PubMed  Google Scholar 

  38. Członkowska A, Tarnacka B, Möller JC, Leinweber B, Bandmann O, Woimant F, et al. Unified Wilson disease rating scale – a proposal for the neurological scoring of Wilson disease patients. Neurol Neurochir Pol. 2007;41(1):1–12.

    PubMed  Google Scholar 

  39. Leinweber B, Möller JC, Scherag A, Reuner U, Günther P, Lang CJG, et al. Evaluation of the Unified Wilson Disease Rating Scale (UWDRS) in German patients with treated Wilson disease. Mov Disord Off J Mov Disord Soc. 2008;23(1):54–62.

    Article  Google Scholar 

  40. Volpert HM, Pfeiffenberger J, Gröner JB, Stremmel W, Gotthardt DN, Schäfer M, et al. Comparative assessment of clinical rating scales in Wilson disease. BMC Neurol. 2017;17(1):140.

    Article  PubMed  PubMed Central  Google Scholar 

  41. Roberts EA, Schilsky ML, American Association for Study of Liver Diseases (AASLD). Diagnosis and treatment of Wilson disease: an update. Hepatol Baltim Md. 2008;47(6):2089–111.

    Article  CAS  Google Scholar 

  42. European Association for Study of Liver. EASL clinical practice guidelines: Wilson disease. J Hepatol. 2012;56(3):671–85.

    Article  Google Scholar 

  43. Ranjan A, Kalita J, Kumar S, Bhoi SK, Misra UK. A study of MRI changes in Wilson disease and its correlation with clinical features and outcome. Clin Neurol Neurosurg. 2015;138:31–6.

    Article  CAS  PubMed  Google Scholar 

  44. King AD, Walshe JM, Kendall BE, Chinn RJ, Paley MN, Wilkinson ID, et al. Cranial MR imaging in Wilson disease. AJR Am J Roentgenol. 1996;167(6):1579–84.

    Article  CAS  PubMed  Google Scholar 

  45. Litwin T, Dzieżyc K, Poniatowska R, Członkowska A. Effect of liver transplantation on brain magnetic resonance imaging pathology in Wilson disease: a case report. Neurol Neurochir Pol. 2013;47(4):393–7.

    PubMed  CAS  Google Scholar 

  46. Kim TJ, Kim IO, Kim WS, Cheon JE, Moon SG, Kwon JW, et al. MR imaging of the brain in Wilson disease of childhood: findings before and after treatment with clinical correlation. Am J Neuroradiol. 2006;27(6):1373–8.

    PubMed  CAS  Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Neurology, Yale University, New Haven, CT, USA

    Ana Vives-Rodriguez, Daphne Robakis & Nigel S. Bamford

  2. Department of Pediatrics, Yale University, New Haven, CT, USA

    Nigel S. Bamford

  3. Department of Cellular and Molecular Physiology, Yale University, New Haven, CT, USA

    Nigel S. Bamford

Authors
  1. Ana Vives-Rodriguez
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  2. Daphne Robakis
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  3. Nigel S. Bamford
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Corresponding author

Correspondence to Ana Vives-Rodriguez .

Editor information

Editors and Affiliations

  1. Yale New Haven Transplantation Center, Yale University, New Haven, Connecticut, USA

    Michael L. Schilsky

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Vives-Rodriguez, A., Robakis, D., Bamford, N.S. (2018). Treatment of Neurological Symptoms in Wilson Disease. In: Schilsky, M. (eds) Management of Wilson Disease . Clinical Gastroenterology. Humana Press, Cham. https://doi.org/10.1007/978-3-319-91527-2_6

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  • DOI: https://doi.org/10.1007/978-3-319-91527-2_6

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  • Publisher Name: Humana Press, Cham

  • Print ISBN: 978-3-319-91526-5

  • Online ISBN: 978-3-319-91527-2

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