Interpretation of MRI for Classical Signs of Hepatolenticular Degeneration

Background A 17-year-old male with severe dystonia and Parkinsonism-like features was referred for a magnetic resonance imaging (MRI) of the brain to evaluate the cause of his mental retardation. A positive family history with similar clinical complaints and progression was noted in two deceased elder siblings of the patient, suggesting a genetic link. Wilson’s disease was clinically suspected. Objectives The aim of this case report is to prepare the neurologists to interpretate classical MRI signs of Wilson’s disease, when clinical parameters are equivocal. Methods and Materials The patient was first evaluated clinically by a neurologist for mental retardation and neuropsychiatric disorders, and then MRI was ordered. MRI revealed classical double panda signs suggesting metabolic disease, mainly Wilson’s disease. Results Diagnosis was confirmed with supportive evidence from MRI, and treatment was started. Conclusion Neurologists must be familiar with classical double panda sign of Wilson’s disease on MRI in clinically doubtful cases.