Skip to main content
Log in

Wilson's disease clinical correlation with cranial computed tomography

  • Originals
  • Published:
Neuroradiology Aims and scope Submit manuscript

Summary

Cranial computed tomograms of 12 patients with proven Wilson's disease were correlated with clinical disturbances. CT abnormalities occurred only in the eight patients with neurological manifestations. The presence of atrophy and low density lesions of the basal ganglia shown on CT correlated well with clinical signs of extrapyramidal dysfunction. Cerebral atrophy and cerebellar cortical atrophy were only moderately related to the degree of intellectual dysfunction and ataxia, respectively; there were no specific clinical signs in cases with brainstem involvement. Abnormalities may be marked in scans taken within a year of neuropsychiatric symptoms, but the most severly abnormal CT scans occurred in patients with a relatively longer duration of untreated disease. Computed tomography provides the opportunity to follow the response of the abnormalities of the brain to cupruresis and can give some assistance in management.

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.

Similar content being viewed by others

References

  1. Barnes S, Hurst EW (1926) A further note on hepato-lenticular degeneration. Brain 49:36–60

    Google Scholar 

  2. Blackwood W (ed) (1976) Greenfield's neuropathology. 3rd ed. Year Book, Chicago, pp 171–177

    Google Scholar 

  3. Cuming JM (1948) The copper and iron content of brain and liver in the normal and in hepato-lenticular degeneration. Brain 71:410–415

    Google Scholar 

  4. Dobyns WB, Goldstein NP, Gordon H (1979) The clinical spectrum of Wilson's disease (hepato-lenticular degeneration). Mayo Clin Proc 54:35–42

    Google Scholar 

  5. Egger J, Lake BD, Wilson J (1981) Mitochondrial cytopathy. A multisystem disorder with ragged red fibres on muscle biopsy. Arch Dis Child (in press)

  6. Greenfield JG, Poynton FJ, Walshe FMR (1924) On progressive lenticular degeneration (hepato-lenticular degeneration). Q J Med 17:385–403

    Google Scholar 

  7. Hall K, Gardner-Medwin D (1978) Scan appearances in Leigh's disease (subacute necrotising encephalomyelitis). Neuroradiology 16:48–50

    Google Scholar 

  8. Harik CI, Donayan Post MJ (1981) Computed tomography in Wilson's disease. Neurology 31:107–110

    Google Scholar 

  9. Howard CP, Royce CE (1919) Progressive lenticular degeneration associated with cirrhosis of the liver (Wilson's disease). Arch Intern Med 24:497–508

    Google Scholar 

  10. Kendall BE, Claveria IE, Quiroga W (1977) C.A.T. in leukodystrophy and neuronal degeneration. In: du Boulay G, Moseley IF (eds) Computed axial tomography in clinical practice. Springer, Berlin Heidelberg New York, pp 191–202

    Google Scholar 

  11. Kim KS, Weinberg PE, Suh JH, Ho SU (1980) Acute carbon monoxide poisoning. Computed tomography of the brain. AJNR 1:399–403

    Google Scholar 

  12. Wilson SAK (1912) Progressive lenticular degeneration: A familial nervous dissase associated with cirrhosis of the liver. Brain 34:295–509

    Google Scholar 

  13. Merland JJ, Chiras J, Melki JP, Cassan JL (1978) Etude tomodensitometrique dans la maladie de Wilson. Neuroradiology 16:269–270

    Google Scholar 

  14. Nelson RF, Guzman DA, Grahovac Z, Howse DCN (1979) Computerized cranial tomography in Wilson's disease. Neurology (Minneap) 29:866–868

    Google Scholar 

  15. Ropper AH, Hatten HP Jr, Davies KR (1979) Computed tomography in Wilson's disease: report of two cases. Ann Neurol 15:102–103

    Google Scholar 

  16. Saigel RS, Seeger JF, Gabrielsen TO, Allen RJ (1979) Computed tomography in oculo-cranio somatic disease (Kearns-Sayre syndrome). Radiology 130:159–164

    Google Scholar 

  17. Sass-Kortsak A, Bearn AG (1978) Hereditary disorders of copper metabolism. In: Starburg JB, Wyngaarden JB, Fredrickson DS (eds) The metabolic basis of inherited disease. McGraw-Hill, New York, pp 1098–1126

    Google Scholar 

  18. Sax DS, Menzer L (1977) CT in Huntington's disease. Neurology 27:388

    Google Scholar 

  19. Schulman S (1968) Wilson's disease. In: Minckler J (ed) Pathology of the nervous system. McGraw-Hill, New York, pp 1139–1151

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Kendall, B.E., Pollock, S.S., Bass, N.M. et al. Wilson's disease clinical correlation with cranial computed tomography. Neuroradiology 22, 1–5 (1981). https://doi.org/10.1007/BF00344602

Download citation

  • Received:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00344602

Key words

Navigation