sCJD mimics NCSE in ICU patients

Lapergue B et al.  Neurology 2010; 74:1995-1999.

10 patients with sCJD were admitted to neuro ICU with initial misdiagnosis of NCSE.  Mean age 64 +/- 13, with gait ataxis (7),cognitive impairment (5), myoclonus (1), visual (5) or auditory (1) hallucinations and sudden stroke like hemiparesis (2).  Disease duration was 106 days (+/- 30 days).  with EEG's strongly suggesting NCSE ( see article for examples).  EEG's responded to therapy with antiepileptic drugs,  On reexamination, EEG's did not show rhytmic activity, but rather periodic or semiperiodic sharp wave complexes with period of .5-1.0 seconds.  These were attenuated by auditory or painful stimuli.  They also fluctuated with drowsiness.  Importantly, there was no clinical improvement with EEG improvement.  MRI showed characteristic changes in 9/10 and 14,3,3 was seen in all patients.