Abstract
We reported the brain proton magnetic resonance spectroscopy (MRS) findings in 27 Wilson’s disease (WD) patients treated more than 6 years in neurological (nWD) and hepatic (hWD) subgroups. We investigated four hWD patients, with no improvement and eight with marked improvement; and eight nWD patients with marked improvement and seven with no improvement of clinical status. In nWD patients with improvement the MRS showed significantly higher Cho/Cr, Glx/Cr ratios levels. In hWD patients with no improvement the lower Cho/Cr and in nWD significantly lower NAA/Cr and higher Cho/Cr and Lip/Cr ratios were detected. In nWD patients with improvement the spectroscopic pattern, can be related to gliosis. In patients with no neurological improvement a persistent neuronal dysfunction can occur, perhaps as a result of copper or iron deposition.
Similar content being viewed by others
References
Bonavita S, Di Salle F, Tedeschi G (1999) Proton MRS in neurological disorders. Eur J Radiol 30:125–131
Bottomley P (1986) Spatial localization in NMR spectroscopy in vivo. Ann NY Acad Sci 508:333–348
Członkowska A, Galewicz A, Rodo M, Wehr H (1973) Observation on copper metabolism in Wilson’s disease. Acta Univ Carol Med 56:175–178
Haussinger D, Laubenberger J, vom Dahl S, Ernst T, Bayer S, Langer M, Gerok W, Henning J (1994) Proton magnetic resonance spectroscopy studies on human brain myo-inositol in hypo-osmolarity and hepatic encephalopathy. Gastroenterol 107:1475–1480
Hoogenraad T (1997) Wilson’s disease. WB. Saunders Company Ttd, London
Horoupian D, Sternlieb I, Scheinberg I (1988) Neuropathological findings in penicillamine—treated patients with Wilson’s disease. Clin Neuropathol 7:62–67
Kreis R, Ross B, Farrow N, Ackerman R (1992) Metabolic disorders of the brain in chronic hepatic encephalopathy detected with H-1 MR spectroscopy. Radiology 182:19–27
Lizardi-Cervera J, Almeda P, Guevara L, Uribe M (2003) Hepatic encephalopathy: a review. Ann Hepatol 2:122–130
Lucato LT, Barbosa M, Machado A, McKinney A, Bacheschi L, Scaff M, Cerri G, Leite C (2005) Proton MR spectroscopy in Wilson disease: analysis of 36 cases. Am J Neuroradiol 26:1066–1071
Medici V, Di Leo V, Lamboglia F, Bowlus C, Tseng S, D’Inca R, Irato P, Burra P, Martines D, Strunillo G (2007) Effect of penicillamine and zinc on iron metabolizm in Wilson’s disease. Scand J Gastroenterol 42:1495–1500
Page R, Davie F, MacManus D, Miszkiel K, Walshe J, Miller D (2004) Clinical correlation of brain MRI and MRS abnormalities in patients with Wilson’s disease. Neurology 63:638–643
Roh J, Lee T, Wie B (1994) Initial and follow up brain MRI findings and correlations with the clinical course in Wilson’s disease. Neurology 44:1064–1068
Sinha S, Taly B, Ravishankar S, Prashanth L, Venugopal K, Arunodaya G (2006) Wilson’s disease: cranial MRI observations and clinical correlations. Neuroradiology 48:613–621
Spahr L, Burkhard P, Grotzsch H, Hadengue A (2002) Clinical significance of basal ganglia alternations at brain MRI and 1H MRS in cirrhosis and role in the pathogenesis of hepatic encephalopathy. Metab Brain Dis 17:399–413
Tarnacka B, Szeszkowski W, Gołębiowski M, Członkowska A (2008) MR spectroscopy in monitoring the treatment of Wilson’s disease patients. Mov Disord 23:1560–1567
Taylor-Robinson SD, Sargentoni J, Marcus CD, Morgan MY, Bryant DJ (1994) Regional variations in cerebral proton spectroscopy in patients with chronic hepatic encephalopathy. Metab Brain Dis 9:347–359
Verma A, Saraswat V, Krishna R, Nath K, Thomas A, Gupta R (2008) In vivo 1H magnetic resonance spectroscopy-derived metabolite variations between acute-on-chronic liver failure and acute liver failure. Liver Int 1478:1095–1103
Acknowledgements
This work was supported by grant from the Polish Ministry of Education and Science (3P05B/119/23).
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Tarnacka, B., Szeszkowski, W., Gołębiowski, M. et al. Brain proton magnetic spectroscopy in long-term treatment of Wilson’s disease patients. Metab Brain Dis 25, 325–329 (2010). https://doi.org/10.1007/s11011-010-9214-x
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11011-010-9214-x