Year :
2024
| Month :
February
| Volume :
18
| Issue :
2
| Page :
OJ01 -
Full Version
Panda Sign: The Defining Feature of Wilson’s Disease
Published: February 1, 2024 | DOI: https://doi.org/10.7860/JCDR/2024/62078.19004
Ojas Mahajan, Anamika Giri, Sunil Kumar, Sourya Acharya
1. Postgraduate, Department of Medicine, Datta Meghe Institute of Higher Education and Research (Deemed to be University), Jawaharlal Nehru Medical College, Wardha, Maharashtra, India.
2. Postgraduate, Department of Medicine, Datta Meghe Institute of Higher Education and Research (Deemed to be University), Jawaharlal Nehru Medical College, Wardha, Maharashtra, India.
3. Professor, Department of Medicine, Datta Meghe Institute of Higher Education and Research (Deemed to be University), Jawaharlal Nehru Medical College, Wardha, Maharashtra, India.
4. Professor, Department of Medicine, Datta Meghe Institute of Higher Education and Research (Deemed to be University), Jawaharlal Nehru Medical College, Wardha, Maharashtra, India.
Correspondence Address :
Dr. Sunil Kumar,
Professor, Department of Medicine, DMIHER, Sawangi, Wardha-442001, Maharashtra, India.
E-mail: sunilkumarmed@gmail.com
Abstract
Wilson’s disease is an autosomal recessive disorder caused by a mutation of the Wilson’s (global) disease (ATP7B) protein produced by chromosome 13 (1). It manifests as an inability to excrete copper in bile, leading to copper deposition in the liver, brain, and other organs. Clinical signs and symptoms include liver dysfunction and neuropsychiatric disorders (2). While copper accumulation primarily occurs in the basal ganglia, it can affect all parts of the brain. Magnetic Resonance Imaging (MRI) is an effective tool for assessing disease severity and treatment response (3).
A 28-year-old male patient presented to the Emergency Department at Datta Meghe Institute of Higher Education and Research with complaints of tremors in the right lower and upper limbs for one year, diminished vision in both eyes for eight months, and speech slurring for five months. There were no significant past or family medical histories. Jaundice or cirrhosis, which would have indicated hepatic involvement, were absent. A slit-lamp examination revealed Kayser- Fleischer (KF) rings in both eyes. Laboratory investigations showed the following results: serum copper 70 mg/dL (normal range: 85- 150 mg/dL), serum ceruloplasmin 9.2 mg/dL (normal range: 15- 60 mg/dL), and 24-hour urinary copper 110 mg/day (normal range: 50-70 mg/day). Other routine investigations yielded normal results. Additionally, the patient displayed MRI findings. T2-weighted MRI images showed hyperintense regions in the bilateral thalami, brainstem, and middle cerebellar peduncles without diffusion restriction (Table/Fig 1),(Table/Fig 2). These MRI findings resembled the “face of the giant panda”, which is characteristic of Wilson’s disease. Similar MRI appearances can be observed in conditions such as Leigh’s disease, Japanese B encephalitis, methanol toxicity, extrapontine myelinolysis, malnourished patients, and in cases of rapid correction of hyponatraemia (4). The patient was started on daily tablet penicillamine 250 mg and tablet zinc 50 mg thrice a day.
The “Panda sign,” although seen in various conditions, is classically observed the present patients with Wilson’s disease, as described in this clinical case.
Keywords
Brain, Ceruloplasmin, Copper, Magnetic resonance imaging
Reference
| 1. | Patel M, Gupta A, Raja KK, Kumar S, Acharya S. Wilson’s disease presenting as lower cranial nerve involvement-gold is not glittering. Int J Res Rev. 2020;7(10):412-14.
| 2. | Atalar M, Bas¸ pinar N. “Face of the Giant Panda” Sign in Wilson Disease. Turk Norol Derg. 2019;25(3):175.
[ CrossRef] | 3. | Parekh JR, Agrawal PR. Wilson’s disease: ‘face of giant panda’ and ‘trident’ signs together. Oxford Med Case Reports. 2014;2014(1):16-17.
[ CrossRef] [ PubMed] | 4. | Vella S, Grech R. Highlighting an atypical cause of the face of the giant panda sign. BJR Case Rep. 2017;4:20170046. [ CrossRef] |
DOI: 10.7860/JCDR/2024/62078.19004
Date of Submission: Dec 13, 2022
Date of Peer Review: Mar 10, 2023
Date of Acceptance: Mar 27 2023
Date of Publishing: Feb 01, 2024
AUTHOR DECLARATION:
• Financial or Other Competing Interests: None
• Was Ethics Committee Approval obtained for this study? Yes
• Was informed consent obtained from the subjects involved in the study? Yes
• For any images presented appropriate consent has been obtained from the subjects. Yes
PLAGIARISM CHECKING METHODS:
• Plagiarism X-checker: Dec 19, 2022
• Manual Googling: Mar 11, 2023
• iThenticate Software: Mar 25, 2023 (10%)
ETYMOLOGY: Author Origin
EMENDATIONS: 5
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