Skip to main content
Log in

Wilson’s disease: cranial MRI observations and clinical correlation

  • Diagnostic Neuroradiology
  • Published:
Neuroradiology Aims and scope Submit manuscript

Abstract

Introduction

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.

Methods

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.

Results

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.

Conclusion

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

References

  1. King AD, Walshe JM, Kendall BE et al (1996) Cranial MR changes in Wilson’s disease. AJR Am J Roentgenol 167(6):1579–1584

    PubMed  CAS  Google Scholar 

  2. Saatci I, Topcu M, Baltaoglu FF et al (1997) MR findings in Wilson’s disease. Acta Radiol 38(2):250–258

    Article  PubMed  CAS  Google Scholar 

  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 44:365–370

    PubMed  CAS  Google Scholar 

  4. Magalhaes AC, Caramelli P, Menezes JR et al (1994) Wilson’s disease: MRI with clinical correlation. Neuroradiology 36(2):97–100

    Article  PubMed  CAS  Google Scholar 

  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 152–157

    Google Scholar 

  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 12:185–189

    Article  Google Scholar 

  7. Chu NS (1986) Sensory evoked potentials in Wilson’s disease. Brain 109:491–501

    Article  PubMed  Google Scholar 

  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):2059–2064

    PubMed  CAS  Google Scholar 

  9. Brewer GJ (2000) Recognition, diagnosis, and management of Wilson’s disease. Proc Soc Exp Biol Med 223:39–46

    Article  PubMed  CAS  Google Scholar 

  10. Walshe JM (1989) Wilson’s disease. In: Vinken PJ, Bruyn GW, Klawans HL (eds) Handbook of clinical neurology, vol 49. Elsevier, Amsterdam, pp 223–238

    Google Scholar 

  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):1064–1068

    PubMed  CAS  Google Scholar 

  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):2021–2027

    PubMed  Google Scholar 

  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):71–81

    Article  PubMed  CAS  Google Scholar 

  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):120–124

    PubMed  CAS  Google Scholar 

  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):638–643

    PubMed  CAS  Google Scholar 

  16. Grimm G, Oder W, Prayer L, Ferenci P, Madl C (1991) Comparison of functional and structural brain disturbances in Wilson’s disease. Neurology 41:272–276

    PubMed  CAS  Google Scholar 

  17. Prayer L, Wimberger P, Kramer J, Grimm G, Oder W, Imhof H (1990) Cranial MRI in Wilson’s disease. Neuroradiology 32(3):211–214

    Article  PubMed  CAS  Google Scholar 

  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):407–411

    PubMed  CAS  Google Scholar 

  19. Denny-Brown D (1964) Hepatolenticular degeneration (Wilson’s disease) – two different components. New Engl J Med 270:1149–1156

    Article  PubMed  CAS  Google Scholar 

  20. Sener RN (1993) Wilson’s disease: MRI demonstration of cavitations in the basal ganglia and thalami. Pediatr Radiol 23(2):157

    Article  PubMed  CAS  Google Scholar 

  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):401–405

    Article  PubMed  Google Scholar 

  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):171–174

    Article  PubMed  CAS  Google Scholar 

  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):635–638

    Article  PubMed  CAS  Google Scholar 

  24. Morgan MY (1998) Cerebral magnetic resonance imaging in patients with chronic liver disease. Metab Brain Dis 13(4):273–290

    Article  PubMed  CAS  Google Scholar 

  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):624–626

    Article  PubMed  CAS  Google Scholar 

  26. Drayer B, Burger P, Darwin R, Riederer S, Herfkens R, Johnson GA (1986) MRI of brain iron. AJR Am J Roentgenol 147(1):103–110

    PubMed  CAS  Google Scholar 

  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 6:67

    Google Scholar 

  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):749–756

    PubMed  CAS  Google Scholar 

  29. Sinha S, Jha DK, Sinha KK (2001) Wilson’s disease in Eastern India, a study of 49 cases. J Assoc Physicians India 49:881–884

    PubMed  CAS  Google Scholar 

  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 64:685–691

    PubMed  CAS  Google Scholar 

  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):531–536

    PubMed  Google Scholar 

  32. Thuomas KA, Aquilonius SM, Bergstrom K, Westermark K (1993) Magnetic resonance imaging of the brain in Wilson’s disease. Neuroradiology 35(2):134–141

    Article  PubMed  CAS  Google Scholar 

  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 98:557–563

    CAS  Google Scholar 

  34. Kendall, BE, Pollock, SS, Bass NM, Valentine AR (1981) Wilson’s disease. Clinical correction with cranial computed tomography. Neuroradiology 22(1):1–5

    Article  PubMed  CAS  Google Scholar 

Download references

Acknowledgements

We acknowledge the assistance of Dr. N. Vidya, Department of Neurology, for initiating the data collection. We are grateful to the members of staff of the Department of Neuroradiology for performing the MRI. Not the least, we are very thankful to our patients who consented to the study.

Conflict of interest statement

We declare that we have no conflict of interest.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to S. Sinha.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Sinha, S., Taly, A.B., Ravishankar, S. et al. Wilson’s disease: cranial MRI observations and clinical correlation. Neuroradiology 48, 613–621 (2006). https://doi.org/10.1007/s00234-006-0101-4

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00234-006-0101-4

Keywords

Navigation