Article Access Statistics | | Viewed | 5922 | | Printed | 128 | | Emailed | 3 | | PDF Downloaded | 169 | | Comments | [Add] | | Cited by others | 1 | |
|

 Click on image for details.
|
|
|
NEUROIMAGE |
|
|
|
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
Date of Web Publication | 4-Mar-2013 |
Correspondence Address: Sanjib Sinha Department of Neurology, National Institute of Mental Health and Neurosciences, Hosur Road, Bangalore - 560 029 India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0028-3886.108052
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;61:100-1 |
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: Brain MRI showed hyperintense red nuclei in T2W/ FLAIR axial (a and b), and T2W coronal (c) sequences which gave the appearance of 'Bright eyes of Panda'. The red nuclei appeared 'bright' in diffusion weighted imaging (d) and 'dark' in apparent diffusion coefficient map (e) suggesting restricted diffusion. MRI showing 'Face of Giant Panda' in an unrelated patient (f) with WD is provided for comparison
Click here to view |
» References | |  |
1. | Hitoshi S, Iwata M, Yoshikawa K. Mid-brain pathology of Wilson's disease: MRI analysis of three cases. J Neurol Neurosurg Psychiatry 1991;54:624-6.  [PUBMED] |
2. | Taly 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.  [PUBMED] |
3. | 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;48:613-21.  [PUBMED] |
4. | Rutledge 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.  |
[Figure 1]
This article has been cited by | 1 |
Panda with “bright eyes”: a rare sign in Wilson disease |
|
| Elison SARAPURA-CASTRO, Jorge RAMÍREZ-QUIÑONES, Mario CORNEJO-OLIVAS | | Arquivos de Neuro-Psiquiatria. 2020; 78(8): 525 | | [Pubmed] | [DOI] | |
|
 |
|