Utilising Therapeutic Hypothermia in the Control of Non-convulsive Status Epilepticus in a Patient with Creutzfeldt-Jakob Encephalopathy

摘要

Prion diseases are characterised by transmissible proteins which promote progressive neurological deterioration of their hosts. The most common prion disease is sporadic Creutzfeldt-Jakob disease (sCJD)¹. Approximately 90 per cent of patients with CJD exhibit myoclonus at some point during their illness¹. In a retrospective review of 1,384 patients with probable or definite CJD, 10 patients were admitted with an initial diagnosis of refractory non-convulsive status epilepticus (rNCSE)². We encountered a patient with biopsy proven CJD who presented with rNCSE. Therapeutic hypothermia was instrumental in temporarily controlling the patient’s status epilepticus in combination with antiepileptic medications.