Panayiotopoulos, syndrome

(Panayiotopoulos occipital epilepsy, idiopathic nocturnal epilepsy)

Quite frequent form of benign partial epilepsy, with early onset. It typically begins between 3 and 5 years of age (although cases have been reported starting between 1 to 15 years). The crisis is typically autonomic and occurs in 2/3 cases during nighttime sleep or a nap: the child wakes up saying that it feels unwell, vomit, is confused. If the crisis occurs outside a sleep period, there is a sudden change in behavior with pallor, mydriasis, repeated vomiting, major sweats. There is sometimes a deviation of gaze and mental clouding or even loss of consciousness. The crisis ends with some  uni- or bilateral clonic movements. The child then falls asleep and wakes up without sequelae. The EEG shows occipital spike-wave during the crisis and the MRI is normal. The remission is often spontaneous 2-3 years after the start of the attacks. Some children  have only one crisis.

In case of frequent crises: treatment with valproate of Na or carbamazepine.

Differential diagnosis: BECOP (Benign Epilepsy of Childhood with Occipital Paroxysms)


Anesthetic implications:

management of a small child with seizures, side effects of the antiepileptic treatment.


References:


Updated: November 2018