Wilson's disease: Atypical imaging features

Wilson's disease is a genetic movement disorder with characteristic clinical and imaging features. We report a 17-year-old boy who presented with sialorrhea, hypophonic speech, paraparesis with repeated falls and recurrent seizures along with cognitive decline. He had bilateral Kayser Flescher rings. Other than the typical features of Wilson's disease in cranial MRI, there were extensive white matter signal abnormalities (T2 and FLAIR hyperintensities) and gyriform contrast enhancement which are rare imaging features in Wilson's disease. A high index of suspicion is required to diagnose Wilson's disease when atypical imaging features are present.