[Idiopathic childhood occipital epilepsy]

To describe the clinical and electroencephalographic (EEG) features, as well as the outcome of children diagnosed with idiopathic childhood occipital epilepsy (COE) in our hospital.

A retrospective review of the clinical records of children diagnosed with COE in the previous 10years was carried out with description of clinical and EEG features and neuroimaging studies. The outcome of patients followed-up for at least 5 years was also reviewed.

Ten children were studied: two with type I (Gastaut) COE, six with type II (Panayiotopoulos) COE, and two with intermediate forms of the disorder. Patients with type I COE suffered daytime seizures with visual symptoms (hallucinations and amaurosis) followed by versive motor partial complex seizures with secondary generalized seizures. Age of onset was late childhood and the seizures reappeared in adolescence when therapy was discontinued. Patients with type II COE had nocturnal seizures consisting of tonic deviation of the head and eyes, some degree of disturbance of consciousness and hypotony followed by vomiting and hemiclonic movements or generalized tonic-clonic seizures. In five children, the first presenting symptom was status epilepticus. In all patients the age of onset was between 1 and 4 years. The results of neuroimaging studies were normal. EEG records showed normal baseline activity with slow wave spikes in the occipital region that disappeared or were reduced by eye opening.

Our clinical and EEG findings are similar to those of other published studies. Type II COE frequently presents as status epilepticus and can be confused with other neurologic emergencies. Ictal EEG is useful to clarify the diagnosis. In type II COE, ictal symptomatology may overlap with migraine with aura. Although designated benign, patients with type I COE may develop learning problems and continue to have seizures throughout childhood.