Refractory status epilepticus seen in the early phase of sporadic Creutzfeldt-Jakob disease.

Abstract

A male patient in his 60s presented with subacute-onset progressively worsening encephalopathy, myoclonus, paranoia, behavioural changes, confusion and cognitive decline, all on a background of paranoid schizophrenia. Extensive investigations, including blood tests and cerebrospinal fluid analysis, were inconclusive. MRI of the brain demonstrated cortical ribboning, with high signals noted in the caudate nucleus and internal capsule bilaterally on T2/fluid-attenuated inversion recovery and diffusion-weighted imaging. Electroencephalogram identified widespread sharp wave discharges consistent with status epilepticus.