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Background: Sporadic Creutzfeldt-Jacob disease (sCJD) and non-convulsive status epilepticus (NCSE) may show similar clinical features and occur concomitantly. The diagnosis and treatment for patients with both sCJD and NCSE are challenging without current proposed management in clinical practice.Case presentation and review of literature: Two women (52 and 61 years old) developed subacute onset of progressive conscious change, unsteady gait and involuntary movements. The results of brain MRI (hyperintensities in cerebral cortex and basal ganglia) and persistent elevation of 14-3-3 and tau protein levels in cerebrospinal fluid (CSF) supported a diagnosis of sCJD. Electroencephalography showed epileptiform discharges with temporospatial evolution that met the diagnostic criteria of NCSE. Treatment with antiepileptic medication (AED) led to clinical improvement in one patient. We searched PubMed and MEDLINE for articles between 1977 and February 2018 and found the other 11 patients of sCJD who had concurrent NCSE.Conclusion: To distinguish between sCJD and NCSE, the involvement of basal ganglia on MRI and persistent elevation of 14-3-3 and tau proteins in CSF are suggestive of sCJD. Meanwhile, a fluctuated clinical course, epileptiform discharges with temporospatial evolution and clinical response to AED treatment are characteristics of NCSE. Treating NCSE in patients with sCJD is difficult but worth trying for the chance of clinical improvement.

Diagnosis and Treatment of Patients with Concurrent Non-Convulsive Status Epilepticus and Sporadic Creutzfeldt-Jakob Disease