Atypical evolution in childhood epilepsy with occipital paroxysms (Panayiotopoulos type)

We report, on two, school-age girls with clinical and electroencephalograph ic features of early onset childhood epilepsy with occipital paroxysms (CE OP) of the "Panayiotopoulos type" that showed atypical evolution. Neurologi cal examination and brain imaging were normal in both. One child presented at age 2.5 years episodes of oculocephalic deviation, and ictal vomiting du ring nocturnal sleep. The EEG showed left occipital spikes during wakefulne ss and sleep. One year later, frequent inhibitory seizures appeared in the lower limbs causing, "pseudoataxic gait". At the same time she presented wi th behavioral disturbances and aphasia. EEG showed bilateral spike-waves wh ile awake and continuous spike-waves during slow sleep (CSWSS). After switc hing AEDs to benzodiazepines, control of seizures along with improvement of behavior, and partial restoration of cognitive functions were achieved. Th e CSWSS disappeared and the last EEG at age 8 years only showed only isolat ed right occipital spikes. The other girl had a personal and familial history of febrile seizures. At 4 years of age she presented the first non-febrile seizures during sleep, w ith oculocephalic deviation and ictal vomiting, followed by a generalized t onic-clonic seizure. Partial control of seizures was obtained with antiepil eptic drugs. At age 7, the child began to have weekly episodes of oculoceph alic version, occasionally with secondary generalization. Repeated inhibito ry seizures and absences also appeared. EEG showed frequent bilateral spike s occupying predominantly the posterior regions while awake, and CSWSS. At 7.5 years the same electro-clinical picture persisted. Ethosuximide was add ed to sodim valproate and clobazam. Fifteen days later, the seizures disapp eared and the EEG showed less frequent bilateral occipital spikes. She is n ow 9 years old and she has been seizure-free for 18 months. Her present neu ropsychological profile shows mild mental retardation. The two children with typical electroclinical features of "Panayiotopoulos Type" CEOP developed an atypical evolution which, to our knowledge, has not been described previously.