Nonconvulsive status epilepticus (NCSE) may have heterogeneous presentations and differential diagnosis may be particularly difficult because clinical signs coupled with periodic EEG pattern are most often subtle or non-specific. Moreover, few cases of NCSE have been previously described as the presenting symptom of sporadic Creutzfeldt–Jakob disease (sCJD) [1,4,5,7]. We describe a patient with a NCSE strongly, but erroneously, suggestive of a probable sCJD.
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