Early onset benign occipital epilepsy synonym Panayiotopoulos syndrome

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This is another syndrome with age of onset between 1 and 14 years (mean 4-5 years). Estimates of prevalence have ranged from 0 to 0.6% of all children with epilepsy, and in one study it accounted for 28% of all benign focal epilepsies of childhood. Panayiotopoulos considers the condition to be due to diffuse maturation-related epileptogenicity activating emetic centres and the hypothalamus. The clinical presentation is distinctive. In the core syndrome, seizures take the form of eye deviation, nausea and vomiting, with subsequent evolution into clonic hemiconvulsions in some cases. They are often nocturnal and awareness may or nay not be altered. Other autonomic features occur including incontinence of urine, pallor, hyperventilation and headache. Typically, the seizures are prolonged, often lasting hours, and are therefore classified as episodes of status epilepticus (taking the form of absence or autonomic status epilepticus). Despite this high incidence of status, the prognosis of the syndrome is excellent and at least 50% of patients have only a total of 1-5 attacks. The interictal EEG shows occipital spikes, with a morphology similar to that in BECTS, and can be continuous. The EEG discharges are abolished by eye opening (the fixation-off phenomenon) and continue to be seen for years after the cessation of seizures. The boundaries of the syndrome are less well defined than in other syndromes. Some cases are included in which the prolonged seizures consist only of vomiting, or of syncopal symptoms, or prominent autonomic symptoms. This syndrome is often misdiagnosed as migraine, and also needs to be differentiated from other occipital epilepsies. The epilepsy usually remits over time without adverse sequaelae, although a minority of cases evolve to other forms of epilepsy. Usually, continuous antiepileptic drug treatment is not needed but a small night-time dose of carbamazepine, valproate or benzodiazepines will usually suppress all seizures. In the acute phase when seizures are prolonged, rectal diazepam should be given. Parents should be counselled about the condition and the acute management of the seizures.

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