EEG of a 10-year-old girl with autonomic seizures and autonomic status epilepticus identical to those of PS (case 60 in ref⁴). The neurological findings and development were normal. However, the EEG had features that were markedly different to the idiopathic cases (rectangle). This prompted an MRI examination, which showed an extensive dysembryoblastic neuroepithelial tumour in the right temporo-parieto-occipital regions, corresponding to severe EEG abnormalities in the same areas.

Since 7 years of age, she had experienced about 10 brief nocturnal seizures lasting 5–10 min each. During these episodes, she jumps out of bed, calls her parents, and complains of feeling sick and of headache. She screams and vomits, her eyes stare and roll, her eyelids blink, her pupils are dilated and she sweats profusely.

A dramatic diurnal autonomic status epilepticus occurred at school. She complained of frontal headache and she had gone out for fresh air when she started feeling “funny”. She screamed that she was hot, and she was sweating and vomiting. Subsequently, she became vacant, her speech slurred slightly, her eyes twitched and she dribbled a lot. She asked for water, but did not drink. She talked “gobbledegook” and gradually got worse. Her head deviated to the left and she became unresponsive. This was followed by a series of left hemiconvulsions lasting 5–10 min each. Convulsions were stopped with intravenous diazepam. She gradually recovered. The neurological examination was normal. She was amnesic of the events the next morning and she looked well, complaining only of photophobia.

The CT brain scan was normal. CSF and other relevant investigations were normal. She received triple therapy for encephalitis for 4 days after which she was discharged home.