Myoclonic status epilepticus with dystonia-like symptoms in patients with dementia: Report of two cases.

We report cases of two elderly women with dementia who presented with a new-onset seizure disorder characterized by subtle, rhythmic muscular contractions involving the buccolingual region and the four limbs, persistent jaw opening, and abnormal cervical posture that mimicked myoclonus-dystonia syndrome and oromandibular dystonia. The symptoms lasted several minutes to a few hours. Video-polygraphic recordings revealed an electromyographic (EMG) pattern of brief, shock-like muscular contractions consistent with myoclonus that correlated with a high-amplitude (70-90 µV), 11-14 Hertz, bilaterally symmetric electroencephalographic (EEG) rhythm over the frontocentral regions.