Advertisement

Neuroradiology

, Volume 36, Issue 2, pp 97–100 | Cite as

Wilson's disease: MRI with clinical correlation

  • A. C. A. Magalhaes
  • P. Caramelli
  • J. R. Menezes
  • L. S. Lo
  • L. A. Bacheschi
  • E. R. Barbosa
  • L. A. Rosemberg
  • A. Magalhaes
Diagnostic Neuroradiology

Abstract

We tried to establish possible correlations between clinical data and MRI in a group of patients with Wilson's disease. Eleven patients (6 male, 5 female), aged between 11 and 50 years old, with a duration of illness from 5 months to 32 years, were submitted to MRI on a 1.5 T System. Three patients were asymptomatic, two had mild neurological disturbances, two were moderately affected and the remaining four had a severe form of the disease. All were receiving D-penicillamine at the time of the study. In the most symptomatic patients there were abnormalities in five or more sites on MRI. The putamen was affected in all symptomatic patients, including five with dystonia. A striking feature was the peripheral location of high signal putaminal lesions on T2-weighted images. In five cases, lesions in the corpus striatum or substancia nigra explained the patient's Parkinsonian features. MRI is an efficient method for studying involvement of the central nervous system in Wilson's disease, and allows some interesting anatomoclinical correlations.

Key words

Wilson's disease MRI 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Kinnear Wilson SA (1912) Progressive lenticular degeneration: a familial nervous disease associated with cirrhosis of the liver. Brain 34:295–509Google Scholar
  2. 2.
    Grimm G, Prayer L, Oder W, et al (1991) Comparison of functional and structural brain disturbances in Wilson's disease. Neurology 41:272–276Google Scholar
  3. 3.
    Starosta-Rubinstein S, Young AB, Kluin K, et al (1987) Clinical assessment of 31 patients with Wilson's disease. Arch Neurol 44: 365–370Google Scholar
  4. 4.
    Abdollah A, Tampieri D, Melanson D (1991) Wilson's disease: computed tomography and magnetic resonance imaging findings. Can Assoc Radiol J 42:130–134Google Scholar
  5. 5.
    Aisen AA, Martel W, Gabrielson TO et al (1985) Wilson disease of the brain: MR imaging. Radiology 157:137–141Google Scholar
  6. 6.
    Hoori A, Hirose G, Kataoka S, et al (1990) Neuroradiological studies of Wilson's disease by computed tomography and magnetic resonance imaging. Rinsho Shinkei 30:7–16Google Scholar
  7. 7.
    Kulisevsky J, Ruscalleda J, Grau JM (1991) MR imaging of acquired hepatocerebral degeneration. AJNR 12:527–528Google Scholar
  8. 8.
    Lawler GA, Pennock JM, Steiner RE, et al (1983) Nuclear magnetic resonance (NMR) imaging in Wilson's disease. J Comput Assist Tomogr 7:1–8Google Scholar
  9. 9.
    Prayer L, Wimberger D, Kramer J, et al (1990) Cranial MRI in Wilson's disease. Neuroradiology 32:211–214Google Scholar
  10. 10.
    Valença A, Goulão A, Leitão O, et al (1989) A case of Wilson's disease studied using magnetic resonance: a new aproach? Acta Med Port 2:89–92Google Scholar
  11. 11.
    Adams RD, Victor M (1985) Tremor, myoclenus, spasms, and tics. In: Adams RD, Victor M (eds) Principles of neurology, 3rd edn. McGraw-Hill, New York, pp 53–89Google Scholar
  12. 12.
    Scheingerg IH, Sterlieb I (1984) Wilson's disease, 1st edn.. Saunders, New York, pp 25–69Google Scholar
  13. 13.
    Schulman S (1968) Wilson disease. In: Minckler J (ed) Pathology of the nervous system, 1st edn. MacGraw-Hill, New York, pp 1139–1151Google Scholar
  14. 14.
    Rothfus WE, Hirsch WL, Malatock JJ, Bergman I (1988) Improvement of cerebral CT abnormalities following liver transplantation in a patient with Wilson's disease. J Comput Assist Tomogr 12:138–140Google Scholar
  15. 15.
    Ikeda K, Sakata C, Nemoto H, et al (1991) Clinico-radiological correlation of Wilson's disease by magnetic resonance imaging, computed and positron emission tomography. Rinsho Shinkei 31:147–153Google Scholar
  16. 16.
    Hawkins RA, Mazziota JC, Phelps ME (1987) Wilson disease studied with FDG and positron emission tomography. Neurology 37:1707–1711Google Scholar
  17. 17.
    Barbosa ER, Comerlatti LR, Scaff M, Canelas HM (1985) Degeneração hepato-lenticular: aspectos diagnósticos em 95 casos. Arq Neuropsiq 43:234–242Google Scholar

Copyright information

© Springer-Verlag 1994

Authors and Affiliations

  • A. C. A. Magalhaes
    • 1
  • P. Caramelli
    • 2
  • J. R. Menezes
    • 1
  • L. S. Lo
    • 1
  • L. A. Bacheschi
    • 2
  • E. R. Barbosa
    • 2
  • L. A. Rosemberg
    • 1
  • A. Magalhaes
    • 1
  1. 1.Department of Radiology, Hospital das ClinicasUniversity of Sao PauloSao PauloBrazil
  2. 2.Department of Neurology, Hospital das ClinicasUniversity of Sao PauloSao PauloBrazil

Personalised recommendations