Springer Nature is making SARS-CoV-2 and COVID-19 research free. View research | View latest news | Sign up for updates

Relationship between striatal glucose consumption and copper excretion in patients with Wilson's disease treated withd-penicillamine


In 12 patients with Wilson's disease treated withd-penicillamine (DPA), the regional cerebral metabolic rate of glucose consumption of the lentiform and caudate nucleus was analysed using the18Fluo-rodeoxyglucose method and correlated with the clinical symptoms of the patients, the ceruloplasmin level, the serum level of free copper and the 24-h copper excretion. The more copper was eliminated, the higher was the basal ganglia glucose consumption. On the other hand, in seven patients who had been treated for more than 7 years a significant decline of the basal ganglia glucose consumption was observed, suggesting too low a maintenance dose of DPA.

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


  1. 1.

    Bornstein RA, Mc Lean DR (1985) Neuropsychological and electrophysiological examination of a patient with Wilson's disease. Int J Neurosci 26:239–257

  2. 2.

    Dopyns WB, Goldstein NP, Gordon H (1979) Clinical spectrum of Wilson's disease (hepatolenticular degeneration). Mayo Clin Proc 54:35–42

  3. 3.

    Duara R, Margolin RA, Robertson-Tchabo EA, London ED, Schwartz M, Renfrew JW, Koziarz BJ, Sundaram M, Grady C, Moore AM, Ingvar DH, Sokoloff L, Weingartner H, Kessler RM, Manning RG, Channing MA, Cutler NR, Rapoport SI (1983) Cerebral glucose utilization as measured by Positron emission tomography in 21 resting healthy men between ages of 21 and 83 years. Brain 106:761–775

  4. 4.

    Grimm G, Oder W, Prayer L, Ferenci P, Madl C (1990) Evoked potentials in assessment and follow-up of patients with Wilson's disease. Lancet 336:963–964

  5. 5.

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

  6. 6.

    Hefter H, Rautenberg W, Kreutzpaintner G, Arendt G, Freund H-J, Pichlmayr R, Strohmeyer G (1991) Does orthotopic liver transplantation heal Wilson's disease? Clinical followup of two liver transplanted patients. Acta Neurol Scand 84:192–196

  7. 7.

    Hefter H, Kuwert T, Langen H-J Arendt G, Stremmel W, Hennerici M, Herzog H, Feinendegen LE (1991) Measurement of regional cerebral glucose consumption by PET in patients with Wilson's disease: correlation with clinical symptoms and duration of therapy. In: Czlonkowska A, Van den Hamer CJA (eds) Proceedings of the 5th Symposium on “Wilson's disease”. Technical University, Delft, pp 147–157

  8. 8.

    Horoupian DS, Sternlieb I, Scheinberg IH (1988) Neuropathological findings in penicillamine-treated patients with Wilson's disease. Clin Neuropathol 7:62–67

  9. 9.

    Isaacs-Glaberman K, Medalia A, Scheinberg H (1989) Verbal recall and recognition abilities in patients with Wilson's disease. Cortex 25:353–361

  10. 10.

    Kuwert T, Lange HW, Langen K-J, Herzog H, Aulich A, Feinendegen LE (1990) Cortical and subcortical glucose consumption measured by PET in patients with Huntington's disease. Brain 113:1405–1423

  11. 11.

    Kuwert T, Hefter H, Scholz D, Milz M, Wei▪ P, Arendt G, Herzog H, Loken M, Hennerici M, Feinendegen LE (1992) Regional cerebral glucose consumption measured by positron emission tomography in patients with Wilson's disease. Eur J Nucl Med 19:96–101

  12. 12.

    Lange HW, Kuwert T, Rota E, Hefter H, Langen K-J, Herzog H, Aulich A, Hennerici M, Feinendegen LE (1990) Regional metabolic rate of glucose measured by 18F-DG PET in Huntington's disease, benign hereditary chorea and Wilson's disease. In: Benecke R, Conrad B, Marsden CD (eds) Motor disturbances II. Academic Press, London, pp 119–133

  13. 13.

    Lanzi G, Balottion U,Ottolini A (1981) Computerized cranial tomography in Wilson's disease: report of a case before and after penicillamine therapy. Ital J Neurol Sci 2:383–386

  14. 14.

    Leon MJ de, Ferris SH, George AE, Reisberg B, Christman DR, Kricheff II, Wolf AP (1983) Computed tomography and positron emission transaxial tomography evaluations of normal aging and Alzheimer's disease. J Cereb Blood Flow Metab 3:391–394

  15. 15.

    Leon MJ de, George AE, Ferris SH, Christman DR, Fowler JS, Gentes CI, Brodie J, Reisberg B, Wolf AP (1984) Positron emission tomography -and computed tomography assessments of the aging human brain. J Comput Assist Tomogr 8:88–94

  16. 16.

    Marsden CD (1987) Wilson's disease. Q J Med 248:959–966

  17. 17.

    Medalia A, Isaacs-Glaberman K, Scheinberg IH (1988) Neuropsychological impairment in Wilson's disease. Arch Neurol 45:502–504

  18. 18.

    Oder W, Grimm G, Kollegger H, Ferenci P, Schneider B, Deeke L (1991) Neurological and neuropsychiatric spectrum of Wilson's disease: a prospective study of 45 cases. J Neurol 238:281–287

  19. 19.

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

  20. 20.

    Rosselli M, Lorenzana P, Rosselli A, Vergara I (1987) Wilson's disease, a reversible dementia: case report. J Clin Exp Neuropsychol 9:399–406

  21. 21.

    Saito T (1987) Presenting symptoms and natural history of Wilson's disease. Eur J Pediatr 146:261–265

  22. 22.

    Scheinberg IH, Sternlieb I (1984) Wilson's disease. Saunders, Philadelphia

  23. 23.

    Seitz RJ, Kleinschmidt A, Kuwert T, Nebeling B, Stöcklin G, Hefter H (1992) Partial persistance of dopamine D2 receptors in the metabolically depressed striatum in Wilson's disease. Movement Disorders 7 [Suppl 1]:144

  24. 24.

    Snow BJ, Bhatt M, Wayne Martin WR, Calne DLDB (1990) The nigrostriatal dopaminergic pathway in Wilson's disease studied with positron emission tomography. J Neurol Neurosurg Psychiatry 54:12–17

  25. 25.

    Starosta-Rubinstein S, Young AB, Kluin K, Hill G, Aisen AM, Gabrielsen T, Brewer GJ (1987) Clinical assessment of 31 patients with Wilson's disease: correlations with structural changes on magnetic resonance imaging. Arch Neurol 44:365–370

  26. 26.

    Stremmel W, Meyerrose KW, Niederau C, Hefter H, Kreutzpaintner G, Strohmeyer G (1991) Wilson disease: clinical presentation, treatment, and survival. Ann Intern Med 115:720–726

  27. 27.

    Tatsch K, Schwarz J, Oertel WH, Kirsch C-M (1991) Spect imaging of dopamine D2 receptors with 123IIBZM: initial experience in controls and patients with Parkinson's syndrome and Wilson's disease. Nucl Med Commun 12:699–707

  28. 28.

    Volder A de, Sindic CJM, Goffinet AM (1988) Effect ofd-penicillamine treatment on brain metabolism in Wilson's disease: a case study. J Neurol Neurosurg Psychiatry 59:947–949

  29. 29.

    Walshe JM (1976) Wilson's disease. In: Vinken PJ, Bruyers GW (eds) Handbook of clinical neurology, vol 27. Elsevier, New York, pp 379–414

  30. 30.

    Walshe JM, Gibbs KR (1987) Brain copper in Wilson's disease. Lancet II:1030

  31. 31.

    Williams JB, Walshe JM (1981) Wilson's disease. An analysis of the cranial computerized tomography appearences found in 60 patients and the change in response to treatment with chelating agents. Brain 104:735–752

  32. 32.

    Wilson SAK (1912) Progressive lenticular degeneration. A familial nervous disease associated with cirrhosis of the liver. Brain 34:295–309

Download references

Author information

Correspondence to H. Hefter.

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Hefter, H., Arendt, G., Kuwert, T. et al. Relationship between striatal glucose consumption and copper excretion in patients with Wilson's disease treated withd-penicillamine. J Neurol 241, 49–53 (1993).

Download citation

Key words

  • Wilson's disease
  • Cerebral glucose metabolism
  • d-Penicillamine
  • Positron emission tomography