, Volume 48, Issue 9, pp 613–621

Wilson’s disease: cranial MRI observations and clinical correlation

  • S. Sinha
  • A. B. Taly
  • S. Ravishankar
  • L. K. Prashanth
  • K. S. Venugopal
  • G. R. Arunodaya
  • M. K. Vasudev
  • H. S. Swamy
Diagnostic Neuroradiology



Study of MRI changes may be useful in diagnosis, prognosis and better understanding of the pathophysiology of Wilson’s disease (WD). We aimed to describe and correlate the MRI abnormalities of the brain with clinical features in WD.


MRI evaluation was carried out in 100 patients (57 males, 43 females; mean age 19.3±8.9 years) using standard protocols. All but 18 patients were on de-coppering agents. Their history, clinical manifestations and scores for severity of disease were noted.


The mean duration of illness and treatment were 8.3±10.8 years and 7.5±7.1 years respectively. MRI of the brain was abnormal in all the 93 symptomatic patients. The most conspicuous observations were atrophy of the cerebrum (70%), brainstem (66%) and cerebellum (52%). Signal abnormalities were also noted: putamen (72%), caudate (61%), thalami (58%), midbrain (49%), pons (20%), cerebral white matter (25%), cortex (9%), medulla (12%) and cerebellum (10%). The characteristic T2-W globus pallidal hypointensity (34%), “Face of giant panda” sign (12%), T1-W striatal hyperintensity (6%), central pontine myelinosis (7%), and bright claustral sign (4%) were also detected. MRI changes correlated with disease severity scores (P<0.001) but did not correlate with the duration of illness.


MRI changes were universal but diverse and involved almost all the structures of the brain in symptomatic patients. A fair correlation between MRI observations and various clinical features provides an explanation for the protean manifestations of the disease.


MRI Wilson’s disease 


  1. 1.
    King AD, Walshe JM, Kendall BE et al (1996) Cranial MR changes in Wilson’s disease. AJR Am J Roentgenol 167(6):1579–1584PubMedGoogle Scholar
  2. 2.
    Saatci I, Topcu M, Baltaoglu FF et al (1997) MR findings in Wilson’s disease. Acta Radiol 38(2):250–258PubMedCrossRefGoogle Scholar
  3. 3.
    Starosta-Rubinstein S, Young AB, Kluin K et al (1987) Clinical assessment of 31 patients with Wilson’s disease. Correlations with structural changes on MRI. Arch Neurol 44:365–370PubMedGoogle Scholar
  4. 4.
    Magalhaes AC, Caramelli P, Menezes JR et al (1994) Wilson’s disease: MRI with clinical correlation. Neuroradiology 36(2):97–100PubMedCrossRefGoogle Scholar
  5. 5.
    Schwab RS, England AC (1969) Projection technique for evaluating surgery in Parkinson’s disease. In: Gillingham FJ, Donaldson MC (eds) Third symposium on Parkinson’s disease. Livingstone, Edinburgh, pp 152–157Google Scholar
  6. 6.
    Meenakashi-Sundaram S, Taly AB, Kamat V, Arunodaya GR, Rao S, Swamy HS (2002) Autonomic dysfunction in Wilson’s disease – a clinical and electrophysiological study. Clin Auton Res 12:185–189CrossRefGoogle Scholar
  7. 7.
    Chu NS (1986) Sensory evoked potentials in Wilson’s disease. Brain 109:491–501PubMedCrossRefGoogle Scholar
  8. 8.
    Selwa LM, Vanderzant CW, Brunberg JA, Brewer GJ, Drury I, Beydoun A (1993) Correlation of evoked potential and MRI findings in Wilson’s disease. Neurology 43(10):2059–2064PubMedGoogle Scholar
  9. 9.
    Brewer GJ (2000) Recognition, diagnosis, and management of Wilson’s disease. Proc Soc Exp Biol Med 223:39–46PubMedCrossRefGoogle Scholar
  10. 10.
    Walshe JM (1989) Wilson’s disease. In: Vinken PJ, Bruyn GW, Klawans HL (eds) Handbook of clinical neurology, vol 49. Elsevier, Amsterdam, pp 223–238Google Scholar
  11. 11.
    Roh JK, Lee TG, Wie BA, Lee SB, Park SH, Chang KH (1994) Initial and follow-up brain MRI findings and correlation with the clinical course in Wilson’s disease. Neurology 44(6):1064–1068PubMedGoogle Scholar
  12. 12.
    van Wassenaer-van Hall HN, van den Heuvel AG, Jansen GH, Hoogenraad TU, Mali WP (1995) Cranial MR in Wilson disease: abnormal white matter in extrapyramidal and pyramidal tracts. AJNR Am J Neuroradiol 16(10):2021–2027PubMedGoogle Scholar
  13. 13.
    Giagheddu M, Tamburini G, Piga M et al (2001) Comparison of MRI, EEG, EPs and ECD-SPECT in Wilson’s disease. Acta Neurol Scand 103(2):71–81PubMedCrossRefGoogle Scholar
  14. 14.
    Oder W, Prayer L, Grimm G et al (1993) Wilson’s disease: evidence of subgroups derived from clinical findings and brain lesions. Neurology 43(1):120–124PubMedGoogle Scholar
  15. 15.
    Page RA, Davie CA, MacManus D et al (2004) Clinical correlation of brain MRI and MRS abnormalities in patients with Wilson disease. Neurology 63(4):638–643PubMedGoogle Scholar
  16. 16.
    Grimm G, Oder W, Prayer L, Ferenci P, Madl C (1991) Comparison of functional and structural brain disturbances in Wilson’s disease. Neurology 41:272–276PubMedGoogle Scholar
  17. 17.
    Prayer L, Wimberger P, Kramer J, Grimm G, Oder W, Imhof H (1990) Cranial MRI in Wilson’s disease. Neuroradiology 32(3):211–214PubMedCrossRefGoogle Scholar
  18. 18.
    Barbosa ER, Caramelli P, Bacheschi LA et al (1993) Wilson’s disease: magnetic resonance imaging (MRI) with clinical correlations in 16 cases. Rev Paul Med 111(3):407–411PubMedGoogle Scholar
  19. 19.
    Denny-Brown D (1964) Hepatolenticular degeneration (Wilson’s disease) – two different components. New Engl J Med 270:1149–1156PubMedCrossRefGoogle Scholar
  20. 20.
    Sener RN (1993) Wilson’s disease: MRI demonstration of cavitations in the basal ganglia and thalami. Pediatr Radiol 23(2):157PubMedCrossRefGoogle Scholar
  21. 21.
    Juan CJ, Chen CY, Liu YJ et al (2005) Acute putaminal necrosis and white matter demyelination in a child with subnormal copper metabolism in Wilson disease: MR imaging and spectroscopic findings. Neuroradiology 47(6):401–405PubMedCrossRefGoogle Scholar
  22. 22.
    Mochizuki H, Kamakura K, Masaki T et al (1997) Atypical MRI features of Wilson’s disease: high signal in globus pallidus on T1-weighted images. Neuroradiology 39(3):171–174PubMedCrossRefGoogle Scholar
  23. 23.
    Engelbrecht V, Schlaug G, Hefter H, Kahn T, Modder U (1995) MRI of the brain in Wilson disease: T2 signal loss under therapy. J Comput Assist Tomogr 19(4):635–638PubMedCrossRefGoogle Scholar
  24. 24.
    Morgan MY (1998) Cerebral magnetic resonance imaging in patients with chronic liver disease. Metab Brain Dis 13(4):273–290PubMedCrossRefGoogle Scholar
  25. 25.
    Hitoshi S, Iwata M, Yoshikawa K (1991) Mid-brain pathology of Wilson’s disease: MRI analysis of three cases. J Neurol Neurosurg Psychiatry 54(7):624–626PubMedCrossRefGoogle Scholar
  26. 26.
    Drayer B, Burger P, Darwin R, Riederer S, Herfkens R, Johnson GA (1986) MRI of brain iron. AJR Am J Roentgenol 147(1):103–110PubMedGoogle Scholar
  27. 27.
    Meenakshi-Sundaram S, Mahadevan A, Taly AB, Arunodaya GR, Swamy HS, Shankar SK (2003) Pathology of Wilson’s disease: revisited (abstract). Ann Indian Acad Neurol 6:67Google Scholar
  28. 28.
    Alanen A, Komu M, Penttinen M, Leino R (1999) Magnetic resonance imaging and proton MR spectroscopy in Wilson’s disease. Br J Radiol 72(860):749–756PubMedGoogle Scholar
  29. 29.
    Sinha S, Jha DK, Sinha KK (2001) Wilson’s disease in Eastern India, a study of 49 cases. J Assoc Physicians India 49:881–884PubMedGoogle Scholar
  30. 30.
    Polson RJ, Rolles K, Calne RY, Williams R, Marsden D (1987) Reversal of severe neurological manifestations of Wilson’s disease following orthotopic liver transplantation. Q J Med 64:685–691PubMedGoogle Scholar
  31. 31.
    van Wassenaer-van Hall HN, van den Heuvel AG, Algra A, Hoogenraad TU, Mali WP (1996) Wilson disease: findings at MR imaging and CT of the brain with clinical correlation. Radiology 198(2):531–536PubMedGoogle Scholar
  32. 32.
    Thuomas KA, Aquilonius SM, Bergstrom K, Westermark K (1993) Magnetic resonance imaging of the brain in Wilson’s disease. Neuroradiology 35(2):134–141PubMedCrossRefGoogle Scholar
  33. 33.
    Prashanth LK, Taly AB, Sinha S et al (2005) Prognostic factors in patients presenting with severe neurological forms of Wilson’s disease. Q J Med 98:557–563Google Scholar
  34. 34.
    Kendall, BE, Pollock, SS, Bass NM, Valentine AR (1981) Wilson’s disease. Clinical correction with cranial computed tomography. Neuroradiology 22(1):1–5PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2006

Authors and Affiliations

  • S. Sinha
    • 1
  • A. B. Taly
    • 1
  • S. Ravishankar
    • 2
  • L. K. Prashanth
    • 1
  • K. S. Venugopal
    • 1
  • G. R. Arunodaya
    • 1
  • M. K. Vasudev
    • 2
  • H. S. Swamy
    • 1
  1. 1.Department of NeurologyNational Institute of Mental Health and Neurosciences (NIMHANS)BangaloreIndia
  2. 2.Department of Neuroimaging and Interventional RadiologyNational Institute of Mental Health and Neurosciences (NIMHANS)BangaloreIndia

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