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Late Manifestation of Wilson’s Disease: A Case Report


Authors: M. Vališ 1;  R. Taláb 1;  J. Bártová 2;  P. Hůlek 2;  J. Žižka 3;  J. Masopust 4
Authors‘ workplace: Neurologická klinika LF UK a FN Hradec Králové 1;  I. interní klinika LF UK a FN Hradec Králové 2;  Radiologická klinika LF UK a FN Hradec Králové 3;  Psychiatrická klinika LF UK a FN Hradec Králové 4
Published in: Cesk Slov Neurol N 2007; 70/103(3): 328-331
Category: Case Report

Overview

A case of a woman examined repeatedly at different medical workplaces because of a progressive tremor of the head is presented. This symptom started at the patient’s age of 55 years. The investigations have not revealed etiology of the clinical state so far. Previous therapy was also ineffective. The patient was sent for an examination to our department seven years after the symptoms had appeared. The liver biopsy showed an increased copper amount in the liver, and that led to the diagnosis of Wilson’s disease. The symptoms of the disease were atypical and occurred late. This case-report has confirmed possibility of a rare occurrence of this disease in elderly patients with dyskinesia without clear etiology.

Key words:
Wilson´s disease – tremor – liver biopsy – ceruloplasmin – magnetic resonance


Sources

1. Wilson SAK. Progressive lenticular degeneration: a familial nervous disease associated with cirrhosis of the liver. Brain 1912; 34: 295–507.

2. Mitchell AJ. Neuropsychiatry and behavioural neurology explained. London: Saunders 2004.

3. Sternlieb I, Van den Hamer CJ, Morell AG, Alpert S, Gregoriadis G, Scheinberg IH. Lysosomal defect of hepatic copper excretion Wilson’s disease (hepatolenticular degeneration). Gastroenterology 1973; 64: 99–105.

4. Gow PJ, Smallwood RA, Angus PW, Smith AL, Wall AJ, Sewell RB. Diagnosis of Wilson’s disease: an experience over three decades. Gut 2000; 46: 415–419.

5. Pulkrábek J. Wilsonova nemoc. In: Rektor I, Rektorová I et al. Centrální poruchy hybnosti v praxi. Praha: Triton 2003: 141–152.

6. Ross ME, Jacobsen IM, Dienstag JL, Martin JB. Late-onset Wilson’s disease with neurological involvement in the absence of Kayser- Fleischer rings. Ann Neurol 1985; 17: 411–413.

7. Saatci I, Topcu M, Baltaoglu FF, Kose G, Yalaz K, Renda Y et al. Cranial MR findings in Wilson’s disease. Acta Radiol 1997; 38: 250–258.

8. Vymazal J, Kvíčala V, Nevšímalová S, Mareček Z. Význam CT diagnostiky u Wilsonovy choroby. Česk Slov Neurol N 1982; 45: 148–153.

9. Brewer GJ, Askari F, Lorincz MT, Carlson M, Schilsky M, Kluin KJ et al. Treatment of Wilson disease with ammonium tetrathiomolybdate: IV. Comparison of tetrathiomolybdate and trientine in a double-blind study of treatment of the neurologic presentation of Wilson disease. Arch Neurol 2006; 63: 521–527.

10. Campos Franco J, Dominguez Santalla MJ, Tome Martinez de Rituerto S, Otero Anton E, Gonzalez Quintela A. Late-onset Wilson’s disease. An Med Interna 2003; 20: 416–418.

Labels
Paediatric neurology Neurosurgery Neurology

Article was published in

Czech and Slovak Neurology and Neurosurgery

Issue 3

2007 Issue 3

Most read in this issue
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