External Capsular Hyperintensities in Wilson's Disease Presenting with Refractory Status Epilepticus

Wilson's disease is a genetic disorder characterized by abnormal accumulation of copper in the brain, liver, eye, and kidney. Various neuropsychiatric manifestations such as dystonia, parkinsonism, choreoathetosis, tremor, ataxia, personality disorders, mood changes, psychosis, cognitive abnormalities, sleep disorders, dystonia, tremor, ataxia, and seizures are known to occur in Wilson's disease. Status epilepticus is a very rare presentation of this disease. Here, we present a case of Wilson's disease occurring in an adolescent, who presented with refractory convulsive status epilepticus. His magnetic resonance imaging of the brain showed bilateral external capsular hyper intensities, with only subtle changes in other brain areas and the only clue to the presence of Wilson's disease was the presence of mildly deranged hepatic enzymes. The 24-h-urinary copper levels were subsequently found to be elevated in this patient.