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Wilson's disease: MRI with clinical correlation

  • Diagnostic Neuroradiology
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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.

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References

  1. Kinnear Wilson SA (1912) Progressive lenticular degeneration: a familial nervous disease associated with cirrhosis of the liver. Brain 34:295–509

    Google Scholar 

  2. Grimm G, Prayer L, Oder W, et al (1991) Comparison of functional and structural brain disturbances in Wilson's disease. Neurology 41:272–276

    Google Scholar 

  3. Starosta-Rubinstein S, Young AB, Kluin K, et al (1987) Clinical assessment of 31 patients with Wilson's disease. Arch Neurol 44: 365–370

    Google Scholar 

  4. Abdollah A, Tampieri D, Melanson D (1991) Wilson's disease: computed tomography and magnetic resonance imaging findings. Can Assoc Radiol J 42:130–134

    Google Scholar 

  5. Aisen AA, Martel W, Gabrielson TO et al (1985) Wilson disease of the brain: MR imaging. Radiology 157:137–141

    Google Scholar 

  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 30:7–16

    Google Scholar 

  7. Kulisevsky J, Ruscalleda J, Grau JM (1991) MR imaging of acquired hepatocerebral degeneration. AJNR 12:527–528

    Google Scholar 

  8. Lawler GA, Pennock JM, Steiner RE, et al (1983) Nuclear magnetic resonance (NMR) imaging in Wilson's disease. J Comput Assist Tomogr 7:1–8

    Google Scholar 

  9. Prayer L, Wimberger D, Kramer J, et al (1990) Cranial MRI in Wilson's disease. Neuroradiology 32:211–214

    Google Scholar 

  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 2:89–92

    Google Scholar 

  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 53–89

    Google Scholar 

  12. Scheingerg IH, Sterlieb I (1984) Wilson's disease, 1st edn.. Saunders, New York, pp 25–69

    Google Scholar 

  13. Schulman S (1968) Wilson disease. In: Minckler J (ed) Pathology of the nervous system, 1st edn. MacGraw-Hill, New York, pp 1139–1151

    Google Scholar 

  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 12:138–140

    Google Scholar 

  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 31:147–153

    Google Scholar 

  16. Hawkins RA, Mazziota JC, Phelps ME (1987) Wilson disease studied with FDG and positron emission tomography. Neurology 37:1707–1711

    Google Scholar 

  17. Barbosa ER, Comerlatti LR, Scaff M, Canelas HM (1985) Degeneração hepato-lenticular: aspectos diagnósticos em 95 casos. Arq Neuropsiq 43:234–242

    Google Scholar 

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Magalhaes, A.C.A., Caramelli, P., Menezes, J.R. et al. Wilson's disease: MRI with clinical correlation. Neuroradiology 36, 97–100 (1994). https://doi.org/10.1007/BF00588068

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  • DOI: https://doi.org/10.1007/BF00588068

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