Published: 2022-10-26

A rare case of hepatolenticular degeneration with double panda sign on imaging

Fahad Mohammedamin Dadu, Sathish Kumar, Ram Kumar, Magesh Kumar


Wilson's disease is an inborn error of copper metabolism that is characterized by deficiency of ceruloplasmin, the serum transport protein for copper. Copper is collected in the liver, and after hepatic binding sites are saturated, it is released. Systemic disease then develops and there is abnormal accumulation of copper in the brain, particularly in the putamen and globus pallidus. Presenting this case of a 32-year-old male patient who presented with peculiar features for Wilson’s disease.


Dermatomyositis, Classical, Wilson's disease

Full Text:



De Bie P, Muller P, Wijmenga C, Klomp L. Molecular pathogenesis of Wilson and Menkes disease: correlation of mutations with molecular defects and disease phenotypes. J Med Genetics. 2017;44:673-88.

Walshe JM. Wilson’s disease. In: Vinken PJ, Bruyn GW, Klawans HL, editors. Handbook of Clinical Neurology. Amsterdam: Elsevier Sci. 1986;49:223-38.

Litwin T, Gromadzka G, Członkowska A. Gender differences in Wilson’s disease. J Neurol Sci. 2012;312:31-5.

Huster D. Wilson disease. Best Pract Res Clin Gastroenterol. 2010;24:531-9.

Singh P, Ahluwalia A, Saggar K, Grewal CS. Wilson's disease: MRI features. J Pediatr Neurosci. 2011;6(1):27-8.

Hitoshi S, Iwata M, Yoshikawa K. Mid-brain pathology of Wilson’s disease: MRI analysis of three cases. J Neurol Neurosurg Psychiatr. 1991;54:624-6.

Jacobs DA. The “double panda sign” in Wilson’s disease." Neurology. 2003;61(7):969.

Sener RN. Diffusion MR imaging changes associated with Wilson disease. AJNR Am J Neuroradiol. 2003;24:965-7.

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;26:1066-71.

SanjibS, Taly AB. Central Pontine Signal Changes in Wilson's Disease: Distinct MRI Morphology and Sequential Changes with De-Coppering Therapy. J Neuroimaging. 2007;17:286-91.