Batten disease
Apr. 29, 2024
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Absence (and tonic) status epilepticus with repetitive discharges of unclassified generalized epilepsy
Continuous recording from video-EEG of a 48-year-old woman with unclassified lengthy episode of nonconvulsive (absence and tonic) status epilepticus and GTCS from the age of 11 years. She realized from previous experiences that she was in nonconvulsive status epilepticus when she felt “weird, like in a trance, missing pieces of conversation,” and she went to the hospital. Despite written instruction, no diazepam was administered. She was alert, attentive, cooperative, and well behaved, but she seemed depressed. Movement and speech were normal. She was able to count with no errors except when the discharges between arrows occurred. During these discharges, eyes, and sometimes mouth, would tonically open with a slight retropulsion of the head. Video-EEG was recorded 7 hours from onset of symptoms, which ended with a generalized tonic-clonic seizure 1 hour later. (Contributed by Dr. C P Panayiotopoulos.)