Chapter 26: Benign Occipital Epilepsies

OVERVIEW

Gastaut presented a series of 36 patients with seizures suggesting occipital lobe origin, associated migraine-like symptoms, and occipital paroxysms of spike-waves, and proposed this condition as a new epileptic syndrome in childhood.¹ The 1989 International League Against Epilepsy (ILAE) Commission named this syndrome "childhood epilepsy with occipital paroxysms" and included it in the group of localization-related idiopathic epilepsies together with benign childhood epilepsy with centrotemporal spikes (BCECTSs).² In 1989, two studies by Panayiotopoulos based on a long follow-up of his patients called attention to a specific cluster of symptoms present in what he called "benign nocturnal childhood occipital epilepsy (COE)."^3,4 Vomiting as an ictal symptom and "cerebral insult-like" partial status epilepticus including autonomic symptoms were the most striking clinical manifestations.^3,4 Thereafter, several authors preferred the eponymic nomenclature of "Panayiotopoulos syndrome" (PS) in order to include patients with and without occipital spikes or occipital ictal origins.^5,6,7

Our group proposed designating the first syndrome as "Gastaut type of benign childhood occipital epilepsy" in order to distinguish it from the "Panayiotopoulos type of benign childhood occipital epilepsy" or PS, which also manifests with occipital paroxysms.^5,6,8,9,10 These criteria were adopted by the Task Force on Classification and Terminology of the ILAE.¹¹

In this chapter, we consider the therapeutic management of benign occipital epilepsies recognized in the Classification and Terminology of ILAE¹¹ that include, early-onset benign COE "Panayiotopoulos type" (PS) and late-onset childhood occipital epilepsy "Gastaut type" (COE of Gastaut).

PANAYIOTOPOULOS SYNDROME

PS is a clearly recognized syndrome and the second-most frequent benign focal epilepsy syndrome in childhood after BCECTSs. PS is four to eight times more frequent than COE of Gastaut.⁸

Seventy-five percent of patients experience their first seizure between the ages of 3 and 6 years. PS affects boys and girls equally and in two-thirds of patients seizures occur only in sleep. Seizures may occur while awake and onset may be inconspicuous with pallor, agitation, nausea, and vomiting.⁸

The duration of the seizures is usually long, commonly lasting for more than 5 minutes, and in approximately 40% of cases lasts more than 30 minutes, constituting in those cases a focal or secondarily generalized status epilepticus. Three groups of symptoms are the most important in PS.^{5,6,7,12,13,14}

CLINICAL FEATURES

CORE CLINICAL FEATURES

Ictal Emetic Symptoms and Other Autonomic Manifestations

Ictal vomiting, which is relatively unusual in other epilepsies, occurs in approximately 80% of cases of PS. In seizures from wakefulness, other symptoms from the emetic spectrum such as nausea or retching occur during or before the vomiting.¹⁵ Pallor is the ...