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A healthy 14 year old boy had an episode of fever (38.5°C) for 1 day followed, 2 days later, by a progressively worsening confusional state, with slurring of speech, dizziness, and unsteady gait lasting 5 hours. Three months later he had a tonic attack followed by a prolonged postcritical coma which lasted 3 days. On both occasions haematological and biochemical blood tests, brain CT and MRI, CSF examination, and EEG were normal. A few days later, he had another tonic attack of 1 minute, with a confusional state, nominal dysphasia, and left hemiplegia for 8 hours followed by stupor for 3 days. The patient was therefore admitted to our hospital where nominal dysphasia, dystonia, dysmetria, tremulousness, increased deep tendon reflexes, ankle clonus, nystagmus and an enlarged thyroid gland were noted. During his stay in hospital, the patient had a paroxysmal horizontal nystagmus with bilateral mydriasis followed by a tonic attack, involving the right side of the body, with subsequent drowsiness and right hemiplegia for about 10 hours. The next morning he seemed recovered after the nocturnal rest. During that episode several examinations were carried out. EEG recording during wakefulness and spontaneous sleep showed irregular slow waves of 2 Hz–3 Hz on the left frontal region. Brain CT and MRI showed a mild reduction of volume of the left nucleus caudatus, MR angiography was normal. Ictal brain SPECT showed left hemispheric hypoperfusion. Biochemical evaluation encompassing lactate, pyruvate, ketone bodies, amino acids, ammonia, platelets, and protein C and S plasma concentrations performed ictally as well as interictally were …