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Year : 2013  |  Volume : 61  |  Issue : 1  |  Page : 100--101

Panda with "Bright eyes" in Wilson's disease

Pradeep Kallollimath, Madhu Nagappa, Sanjib Sinha, Jitender Saini, Parayil S Bindu, Arun B Taly 
 Department of Neurology, Neuroimaging and Interventional Radiology, National Institute of Mental Health and Neurosciences, Bangalore, India

Correspondence Address:
Sanjib Sinha
Department of Neurology, National Institute of Mental Health and Neurosciences, Hosur Road, Bangalore - 560 029
India




How to cite this article:
Kallollimath P, Nagappa M, Sinha S, Saini J, Bindu PS, Taly AB. Panda with "Bright eyes" in Wilson's disease.Neurol India 2013;61:100-101


How to cite this URL:
Kallollimath P, Nagappa M, Sinha S, Saini J, Bindu PS, Taly AB. Panda with "Bright eyes" in Wilson's disease. Neurol India [serial online] 2013 [cited 2021 Apr 19 ];61:100-101
Available from: https://www.neurologyindia.com/text.asp?2013/61/1/100/108052


Full Text

We have highlighted herein an interesting, hitherto unusual, imaging feature in Wilson's disease, a rare but treatable autosomal recessive disorder of copper metabolism.

A 16-year-old boy presented with progressive dysarthria, dysphagia, drooling, and declining scholastic performance of 1-year duration. Examination revealed bilateral dense Kayser-Fleischer rings, generalised rigidity, dystonia of the extremities, and short shuffling gait. Laboratory findings of reduced serum ceruloplasmin (3 mg/dl; N: 15-35), total copper (28 μg/dl; N: 75-160), and elevated 24-hour urinary copper (200 μg/24 hours; N: ≤70 mcg/24 hour) confirmed the diagnosis of Wilson's disease. He received de-coppering therapy with d-penicillamine and zinc sulphate as well as trihexyphenidyl and had symptomatic improvement.

Brain magnetic resonance imaging (MRI) showed hyperintense red nuclei in T2-weighted and FLAIR axial [Figure 1]a and b, and T2-weighted coronal [Figure 1]c sequences, which gave the appearance of "Bright eyes of Panda." The red nuclei appeared "bright" in diffusion weighted imaging [Figure 1]d and "dark" in apparent diffusion coefficient map [Figure 1]e suggesting restricted diffusion. This observation is different to the commonly seen "Face of Giant Panda" sign first described by Hitoshi et al. [1] This characteristic radiological sign is seen in 10% of patients with Wilson disease [2],[3] consisting of increased T2-weighted signals in the periaqueductal gray, substantianigra pars compacta, and mid-brain tegmentum sparing the red nuclei. MRI showing "Face of Giant Panda" in an unrelated patient [Figure 1]f with Wilson's disease is also provided. The exact pathogenesis of the "Face of Giant Panda" putated to be due to the deposition of heavy metals [4] such as iron and copper remains to be conclusively established.{Figure 1}

References

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2Taly AB, Meenakshi-Sundaram S, Sinha S, Swamy HS, Arunodaya GR. Wilson's disease: Description of 282 patients evaluated over 3 decades. Medicine (Baltimore) 2007;86:112-21.
3Sinha S, Taly AB, Ravishankar S, Prashanth LK, Venugopal KS, Arunodaya GR, et al. Wilson's disease: Cranial MRI observations and clinical correlation. Neuroradiology 2006;48:613-21.
4Rutledge JN, Hilal SK, Silver AJ, Defendini R, Fahn S. Study of movement disorders and brain iron by MR. AJR Am J Roentgenol 1987;149:365-79.