A 10 year-old female was evaluated for seizures. Nocturnal spells 1st developed when she was 3. She would awaken out of sleep with an abrupt nausea, vomiting and retching. She would become diaphoretic and pale and after about ½ hour would become sleepy return to bed awakening the next am without complaint. She was diagnosed with migraine. The episodes recurred and became noted during the day where she would develop attacks with recurrent vomiting that would subsequently become noted where she would “look to the right”. It occurred repeatedly and an opthamology evaluation was normal. She was diagnosed with a “seizure disorder” after “jerking” was noted on her right side at the end of her prolonged episode. An MRI and EEG were normal. A sleep-deprived EEG demonstrated occipital sharp waves and she was treated with CBZ with incomplete results. An event was captured during vEEG.
Figure: (Figure A) Interctal EEG demonstrating bilateral occpital spike and waves during light sleep. (Figure B) A left occipital seizure during photic stimulation captured during VEEG. Recording parameters include; longitudinal bipolar montage, sensitivity 7 uv, and filter settings of 1-70.
Panayiotopoulos syndrome (PS) is a recognized childhood epilepsy syndrome with an unusual seizure semiology in normal children1. Seizures occur with prominent autonomic symptoms lasting for ½ hour or longer. Migraine and cyclical vomiting syndrome may be diagnosed though when syncope, hemiclonic, or convulsive seizures are noted the association with seizures become clarified. EEG in patients with PS shows marked variability in terms of location and an EEG may be normal further hampering clinical recognition. When seen, occipital spikes predominate and are typically of high voltage (figure A). Seizures may show shifting onsets with scalp recording from anterior to posterior onset (figure B) though with similar clinical manifestations. The prolonged duration is characteristic and has been recognized as autonomic status epilepticus. The neuropsychological examination is normal excepting visual and visuoperceptual alterations and infrequently minor attention and memory disturbances2. Most patients have < 5 seizures though about 5% are recurrent. Treatment is usually not required and CBZ or VPA have been used. Prognosis is excellent and most remit in several years. An earlier age may be a negative predictor and our patient is now seizure free and off AEDs.
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