Abstract
Purpose
The aim of this study was to examine the structural-neurochemical abnormalities of the frontal white matter (FWM), deep gray matter nuclei, and pons in patients with Wilson’s disease (WD) using proton magnetic resonance spectroscopy (MRS) and diffusion-weighted imaging (DWI).
Materials and methods
Nine patients with WD and 14 age-matched controls were examined with MRS. N-Acetylaspartate (NAA), choline (Cho), and creatine (Cr) peaks were calculated. DWI scans from six WD patients and six controls were also obtained. The relative metabolite ratios and apparent diffusion coefficient (ADC) values of the WD patients were compared to those of the control subjects by using statistical measures.
Results
Measurements in the thalamus and pons showed significantly lower NAA/Cho and NAA/Cr ratios in the WD group than in the control group (P < 0.05). Thalamic and pontine Cho/Cr ratios in the patient group were significantly higher than those of the control group (P < 0.05). No statistically significant relation was found between the patient and control groups as a result of the MRS examinations of FWM and all ADC measurements (P > 0.05).
Conclusion
MRS is a noninvasive, valuable modality for detecting structural-neurochemical changes of the brain stem and deep gray matter in patients with WD. The contribution of DWI in these patients is limited.
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References
Tarnacka B, Szeszkowski W, Golebiowski M, Czlonkowska A. MR spectroscopy in monitoring the treatment of Wilson’s disease patients. Mov Disord 2008;23:1560–1566.
Tarnacka B, Szeszkowski W, Buettner J, Golebiowski M, Gromadzka G, Czlonkowska A. Heterozygous carriers for Wilson’s disease: magnetic spectroscopy changes in the brain. Metab Brain Dis 2009;24:463–468.
Alanen A, Kamu M, Penttinen M, Leino R. Magnetic resonance imaging and proton MR spectroscopy in Wilson’s disease. Br J Radiol 1999;72:749–756.
Kraft E, Trenkwalder C, Bergh FT, Auer DP. Magnetic resonance proton spectroscopy of the brain in Wilson’s disease. J Neurol 1999;246:693–699.
Van Wassenaer-van Hall HN, van den Heuvel AG, Jansen GH, Hoogenraad TU, Mali WP. Cranial MR in Wilson disease: abnormal white matter in extrapyramidal and pyramidal tracts. AJNR Am J Neuroradiol 1995;16:2021–2027.
Sinha S, Taly AB, Ravishankar S, Prashanth LK, Venugopal KS, Arunodaya GR, et al. Wilson’s disease: cranial MRI observations and clinical correlation. Neuroradiology 2006;48:613–621.
Saatci I, Topcu M, Baltaoglu FF, Köse G, Yalaz K, Renda Y, et al. Cranial MR findings in Wilson’s disease. Acta Radiol 1997;38:250–258.
King AD, Walshe JM, Kendall BE, Chinn RJ, Paley MN, Wilkinson ID, et al. Cranial MR imaging in Wilson’s disease. AJR Am J Roentgenol 1996;167:1579–1584.
Bican A, Bora I, Algin O, Hakyemez B, Ozkol V, Alper E. Neuroimaging in narcolepsy. Sleep Med 2010;11:225–226.
Soares DP, Law M. Magnetic resonance spectroscopy of the brain: review of metabolites and clinical applications. Clin Radiol 2009;64:12–21.
Schaefer PW, Grant PE, Gonzalez RG. Diffusion-weighted MR imaging of the brain. Radiology 2000;217:331–345.
Page RA, Davie CA, MacManus D, Miszkiel KA, Walshe JM, Miller DH, et al. Clinical correlation of brain MRI and MRS abnormalities in patients with Wilson disease. Neurology 2004;63:638–643.
Lucato LT, Otaduy MC, Barbosa ER, Machado AA, McKinney A, Bacheschi LA, et al. Proton MR spectroscopy in Wilson disease: analysis of 36 cases. AJNR Am J Neuroradiol 2005;26:1066–1071.
Kizkin S, Sarac K, Ozisik HI, Ozcan C. Central pontine myelinolysis in Wilson’s disease: MR spectroscopy findings. Magn Reson Imaging 2004;22:117–121.
Sener RN. Diffusion MRI findings in Wilson’s disease. Comput Med Imaging Graphics 2003;27:17–21.
Juan CJ, Chen CY, Liu YJ, Chung HW, Chin SC, Hsueh CJ, et al. Acute putaminal necrosis and white matter demyelination in a child with subnormal copper metabolism in Wilson disease: MR imaging and spectroscopic findings. Neuroradiology 2005;47:401–405.
Da Costa MD, Spitz M, Bacheschi LA, Leite CC, Lucato LT, Barbosa ER. Wilson’s disease: two treatment modalities: correlations to pretreatment and posttreatment brain MRI. Neuroradiology 2009;51:627–633.
Kalra V, Mahajan S, Kesarwani PK. Rare presentation of Wilson’s disease: a case report. Int Urol Nephrol 2004;36:289–291.
Shannon KM. Movement disorders. In: Bradley WG, Daroff RB, Fenichel GM, Jankovic J, editors. Neurology in clinical practice. 4th edn. vol 2. Philadelphia: Butterworth Heinemann; 2004. p. 2159–2160.
Ala A, Walker AP, Ashkan K, Dooley JS, Schilsky ML. Wilson’s disease. Lancet 2007;369:397–408.
Pfeiffer RF. Wilson’s disease. In: Watts RL, Koller WC, editors. Movement disorders. 2nd edn. New York: McGraw-Hill; 2004. p. 779–797.
Machado AA, Deguti MM, Genschel J, Cançado EL, Bochow B, Schmidt H, et al. Neurological manifestations and ATP7B mutations in Wilson’s disease. Parkinsonism Relat Disord 2008;14:246–249.
Mak CM, Lam CW. Diagnosis of Wilson’s disease: a comprehensive review. Crit Rev Clin Lab Sci 2008;45:263–290.
Demirkiran M, Jankovic J, Lewis RA, Cox DW. Neurologic presentation of Wilson disease without Kayser-Fleischer rings. Neurology 1996;46:1040–1043.
Ross ME, Jacobson IM, Dienstag JL, Martin JB. Late-onset Wilson’s disease with neurological involvement in the absence of Kayser-Fleischer rings. Ann Neurol 1985;17:411–413.
Czlonkowska A, Rodo M, Gromadzka G. Late onset Wilson’s disease: therapeutic implications. Mov Disord 2008;23:896–898.
Czlonkowska A, Tarnacka B, Möller JC, Leinweber B, Bandmann O, Woimant F, et al. Unified Wilson’s disease rating scale: a proposal for the neurological scoring of Wilson’s disease patients. Neurol Neurochir Pol 2007;41:1–12.
Van Den Heuvel AG, Van Der Grond J, Van Rooij LG, Van Wassenaer-van Hall HN, Hoogenraad TU, Mali WPTM. Differentiation between portal-systemic encephalopathy and neu rodegenerative disorders in patients with Wilson’s disease: H-1 MR spectroscopy. Radiology 1997;203:539–543.
Jayasundar R, Sahani AK, Gaikwad S, Singh S, Behari M. Proton MR spectroscopy of basal ganglia in Wilson’s disease: case report and review of literature. Magn Reson Imaging 2002;20:131–135.
Tarnacka B, Szeszkowski W, Golebiowski M, Czlonkowska A. Metabolic changes in 37 newly diagnosed Wilson’s disease patients assessed by magnetic resonance spectroscopy. Parkinsonism Relat Disord 2009;15:582–586.
Yurtsever I, Hakyemez B, Taskapilioglu O, Erdogan C, Turan OF, Parlak M. The contribution of diffusion-weighted MR imaging in multiple sclerosis during acute attack. Eur J Radiol 2008;65:421–426.
Favrole P, Chabriat H, Guichard P, Woimant F. Clinical correlates of cerebral water diffusion in Wilson disease. Neurology 2006;66:384–389.
Smith AB, Smirniotopoulos JG, Rushing EJ, Goldstein SJ. Bilateral thalamic lesions. AJR Am J Roentgenol 2009;192:W53–62.
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An erratum to this article can be found online at http://dx.doi.org/10.1007/s11604-010-0546-6
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Algin, O., Taskapilioglu, O., Hakyemez, B. et al. Structural and neurochemical evaluation of the brain and pons in patients with Wilson’s disease. Jpn J Radiol 28, 663–671 (2010). https://doi.org/10.1007/s11604-010-0491-4
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DOI: https://doi.org/10.1007/s11604-010-0491-4