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Diffusion Tensor Magnetic Resonance Imaging in the Analysis of Neurodegenerative Diseases
Published on: July 28, 2013
Do MRI features distinguish Wilson's disease from other early onset extrapyramidal disorders? An analysis of 100
L K Prashanth1, S Sinha, A B Taly
1Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India.
Abstract:
Magnetic resonance imaging (MRI) is frequently used in the evaluation of various extrapyramidal disorders. Among the plethora of MRI features in Wilson's disease (WD), only "face of the giant panda" sign has been recognized to distinguish WD from other early onset extrapyramidal disorders (EOEPD). To ascertain the value of various MRI features in differentiating neuropsychiatric form of WD from other EOEPD. This retrospective analysis included 100 patients (M:F = 56:44) of EOEPD (5-40 years), who had undergone MRI during Jan'03 to Nov'08. Their clinical features were recorded and the following MR sequences were analyzed: T1WI, T2WI, FLAIR. Fifty-six patients had WD (M:F = 28:30, age at onset: 14 +/- 6.8 years) and 44 had other EOEPD (M:F = 27:17, age at onset: 19 +/- 9.8 years) that included Huntington's disease--4, young-onset Parkinson's disease--7, mitochondrial disorders--2, Hallervorden-Spatz disease--8, non-Wilsonian hepatolenticular degeneration--2, toxic/metabolic disorder--1, and others--20. The duration of illness at the time of MRI was comparable (WD: 3.1 +/- 4.9 years; Other EOEPD: 2.8 +/- 2.4 years). MR signal characteristics varied in topography and severity in both the groups. All the patients of WD had signal abnormalities in MRI, as against 16/44 of the other EOEPD group. The following MR observations were noted exclusively in WD: "Face of giant panda" sign (14.3%), tectal plate hyperintensity (75%), central pontine myelinolysis (CPM)-like abnormalities (62.5%), and concurrent signal changes in basal ganglia, thalamus, and brainstem (55.3%). Besides "Face of giant panda" sign, hyperintensities in tectal-plate and central pons (CPM-like), and simultaneous involvement of basal ganglia, thalamus, and brainstem are virtually pathognomonic of WD.
Insights
Magnetic resonance imaging (MRI) can help diagnose Wilson's disease (WD) in early-onset extrapyramidal disorders (EOEPD). Specific MRI findings like the "face of the giant panda" sign and brainstem abnormalities are highly indicative of WD.
Area of Science:
- Neurology
- Radiology
- Medical Imaging
Background:
- Extrapyramidal disorders (EOEPDs) often require advanced imaging for diagnosis.
- Wilson's disease (WD) is a treatable genetic disorder that can cause neurological symptoms.
- Differentiating WD from other EOEPDs using MRI is crucial for timely intervention.
Purpose of the Study:
- To evaluate the diagnostic value of various MRI features in distinguishing neuropsychiatric Wilson's disease from other early-onset extrapyramidal disorders.
- To identify specific MRI markers that are pathognomonic for Wilson's disease.
Main Methods:
- Retrospective analysis of 100 patients (ages 5-40) diagnosed with EOEPD.
- Review of MRI sequences (T1WI, T2WI, FLAIR) and clinical features.
- Comparison of MRI findings between 56 patients with WD and 44 patients with other EOEPDs.
Main Results:
- All 56 WD patients exhibited MRI signal abnormalities, compared to 16 of 44 in the other EOEPD group.
- Exclusive MRI findings in WD included: "face of the giant panda" sign (14.3%), tectal plate hyperintensity (75%), central pontine myelinolysis (CPM)-like abnormalities (62.5%), and concurrent basal ganglia, thalamus, and brainstem signal changes (55.3%).
- These specific findings, beyond the "face of the giant panda" sign, are highly suggestive of WD.
Conclusions:
- MRI is a valuable tool for diagnosing Wilson's disease in the context of EOEPD.
- Specific MRI patterns, including tectal plate and central pons hyperintensities and simultaneous involvement of basal ganglia, thalamus, and brainstem, are virtually pathognomonic for WD.
- These findings aid in differentiating WD from other neurological conditions presenting with similar symptoms.
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