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Updated: Aug 11, 2026
Diffusion Tensor Magnetic Resonance Imaging in the Analysis of Neurodegenerative Diseases
Published on: July 28, 2013
Mid-brain pathology of Wilson's disease: MRI analysis of three cases
S Hitoshi1, M Iwata, K Yoshikawa
1Department of Neurology, Faculty of Medicine, University of Tokyo, Japan.
Abstract:
MRI scans were obtained from three patients with Wilson's disease, all of whom showed rigidity and dysarthria; two also showed tremor and dystonia. Two had been treated with D-penicillamine for seven and 14 years, respectively and their neurological abnormalities had improved, but the third patient had not been treated. T2-weighted MRI of the mid-brain in all three revealed the characteristic "face of the giant panda" sign, consisting of high signal intensity in the tegmentum except for red nucleus, preservation of signal intensity of the lateral portion of the pars reticulata of the substantia nigra and hypo-intensity of the superior colliculus. The clinical significance of these MRI abnormalities is discussed.
Insights
The "face of the giant panda" sign on MRI scans is a key indicator of Wilson's disease neurological involvement. This distinctive mid-brain pattern aids in diagnosing this genetic disorder.
Area of Science:
- Neurology
- Radiology
- Genetics
Background:
- Wilson's disease is a genetic disorder causing copper accumulation.
- Neurological symptoms like rigidity and dysarthria are common in Wilson's disease.
- Magnetic Resonance Imaging (MRI) is crucial for diagnosing neurological manifestations.
Observation:
- Three patients with Wilson's disease presented with varying neurological symptoms including rigidity, dysarthria, tremor, and dystonia.
- Two patients showed improvement after D-penicillamine treatment, while one remained untreated.
- T2-weighted MRI scans revealed a specific mid-brain abnormality in all three patients.
Findings:
- The characteristic MRI finding, termed the "face of the giant panda" sign, was observed in all patients.
- This sign involves specific signal intensities in the mid-brain structures: high in the tegmentum (excluding the red nucleus), preserved in the substantia nigra pars reticulata, and low in the superior colliculus.
- The MRI findings were consistent across patients regardless of treatment status or symptom severity.
Implications:
- The "face of the giant panda" sign is a valuable diagnostic marker for Wilson's disease.
- This MRI sign can help in early detection and assessment of neurological involvement.
- Further research can explore the correlation between this sign and disease progression or treatment response.
