Abstract
Aim of this study was to evaluate the rate of morphological liver and spleen abnormalities in patients with neurological clinical presentation of Wilson’s disease (WD). Fourteen patients with neurological presentation of WD divided into group A (5 patients who initiated chelating therapy <24 months from the first symptoms) and group B (9 patients whose therapy started ≥24 months after the initial symptoms) underwent abdominal MRI examination. Abnormal findings on abdominal MRI were present in 28% of patients with neurological form of WD. Significant hepatosplenomegaly was present in none of the patients from group A and in 4 (44%) patients from group B. In addition, macronodular liver cirrhosis and peritoneal effusion were evident in two and one patient from group B, respectively, and in none of the patients from group A. Our results suggest that severe portal hypertension and liver damage in patients with neurological presentation of WD might be reversible or do not even develop if chelating treatment is initiated <2 years after the onset of symptoms.
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References
Brewer GJ, Yuzbasiyan-Gurkan V (1992) Wilson disease. Medicine 71(3):139–164
Cheon JE, Kim IO, Seo JK, Ko JS, Lee JM, Shin CI, Kim WS, Yeon KM (2010) Clinical application of liver MR imaging in Wilson’s disease. Korean J Radiol 11:665–672
Hancu A, Mihai MC, Axelarad AD (2011) Wilson’s disease: a challenging diagnosis. Clinical manifestations and diagnostic procedures in 12 patients. Rev Med Chir Soc Med Nat Iasi 115(1):58–63
Kozić D, Svetel M, Petrović B, Dragašević N, Semnic R, Kostić VS (2003) MR imaging of the brain in patients with hepatic form of Wilson’s disease. Eur J Neurol 10:587–592
Walshe JM (1988) Diagnosis and treatment of presymptomatic Wilson’s disease. Lancet 20(2):435–437
Singh P, Ahluwalia A, Saggar K, Grewal CS (2011) Wilson’s disease. J Pediatr Neurosci 6(1):27–28
Walshe JM, Yealland M (1992) Wilson’s disease: the problem of delayed diagnosis. J Neurol Neurosurg Psychiatry 55:692–696
Miranda M, Brinck P, Roessler JL, Troncoso Sch M, Gonzales M, Alarcon T et al (1995) Wilson’s disease: a review apropos of a clinical experience in 16 patients. Rev Med Chil 123:1098–1107
Prashanth LK, Taly AB, Sinha S, Arunodaya GR, Swamy HS (2004) Wilson’s disease: diagnostic errors and clinical implications. J Neurol Neurosurg Psychiatry 75:907–909
Kozic D, Svetel M, Petrovic I, Sener RN, Kostic VS (2006) Regression of nodular liver lesions in Wilson’s disease. Acta Radiol 47:624–627
Akhan O, Akpinar E, Oto A, Koroglu M, Ozmen M, Akata D et al (2002) Unusual imaging findings in Wilson’s disease. Eur Radiol 12:S66–S69
Ko SF, Lee TY, Ng SH, Lin JW, Cheng YF (1998) Unusual imaging findings of Wilson’s disease in an asymptomatic 2-year-old girl. Abdom Imag 23:56–59
Mergo PJ, Ross PR (1998) Imaging of diffuse liver disease. Radiol Clin North Am 36:365–375
Hussain S, Reinhold C, Mitchell D (2009) Cirrhosis and lesion characterization at MR imaging. Radiographics 29:1637–1652
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This work has been supported by the Ministry of Science and Technological Development of Serbia, Scientific Project Number 175090.
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Kozic, D.B., Semnic, R., Petrovic, I. et al. Are irreversible morpholocical signs of portal hypertension in neurological form of Wilson’s disease associated with treatment delay? A pilot study. Acta Neurol Belg 112, 261–264 (2012). https://doi.org/10.1007/s13760-012-0025-1
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DOI: https://doi.org/10.1007/s13760-012-0025-1