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).

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}

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 most frequent autonomic manifestation.

Eye Deviation

Versive deviation of the eyes is as common as vomiting and occurs in approximately 80% of patients. Eye deviation is frequently accompanied by head deviation.

Impairment of Consciousness

Consciousness is usually intact at seizure onset but becomes impaired in 80%–90% of cases as the seizures progress.

FREQUENT FEATURES OF SEIZURES

Unilateral Clonic or Tonic–Clonic Seizures

Unilateral clonic or tonic–clonic seizures at onset or following vomiting and eye deviation occur in 25%–30% of cases.

Secondarily Generalized Tonic–Clonic Seizures

Secondarily generalized tonic–clonic seizures usually follow seizures starting with focal motor manifestations. In one series of patients, this course was seen in nearly 40% of the cases.⁵

Status Epilepticus

Status epilepticus is usually nonconvulsive, lasts more than 30 minutes, and occurs in approximately 30% of cases in all series.^5,6,7,13

LESS FREQUENT (BUT NOT RARE) SYMPTOMS

Visual Symptoms

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  Migraine-like headaches

  
  
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  Incontinence of urine and faeces may occur when consciousness is impaired.

  
  
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  Syncope-like manifestations

EEG FEATURES

Electroencephalographic Findings

The most useful laboratory test is the electroencephalographic (EEG). Occipital spikes are bilateral and synchronous, often with voltage asymmetry, or unilateral (Fig. 26–1). In the awake EEG, occipital paroxysms of high amplitude with sharp- and slow-wave complexes that occur immediately after closing the eyes are typically registered. These paroxysms are eliminated, or markedly attenuated, when the eyes are opened, a phenomenon due to fixation of sensitivity.¹² It has been emphasized that extraoccipital spikes (centrotemporal, frontal, or parietal) may also occur in children with PS.^5,6,12,16 According to a recent consensus report, normal EEGs during sleep are exceptional.⁷

ETIOLOGY

As an idiopathic epilepsy syndrome, PS is by definition not linked to a remote symptomatic or acute symptomatic etiology. It is most likely genetically determined, although no gene or chromosomic locus has been found. Affected siblings with PS have been reported.⁵

There is a high prevalence of febrile seizures, ranging from 16% to 45%, in children with PS.^5,8,16 A family history of epilepsy is reported in 30.3% of the cases.⁵ The occurrence of several children with PS and Rolandic seizures and centrotemporal spikes typical of BCECTS,¹⁷ as well as siblings having either Rolandic epilepsy or PS favors a genetic link between these syndromes, perhaps expressed as a reversible functional derangement of the brain cortical maturation.^3,5,15,17

Recently, a family with atypical PS has been reported with an SCN1A missense mutation in the proband¹⁸ that was not found in either her brother with febrile seizures or her parents. Two siblings with relatively early onset of seizures, prolonged time over which many seizures have occurred and strong association with febrile precipitants even after the age of 5 years.¹⁹ These data indicate that SCN1A mutations when found contribute to a more severe clinical phenotype of PS.

PATHOPHYSIOLOGY

The basic mechanisms and pathophysiology of PS are largely unknown. The clinical findings suggest diffuse maturation-related cortical hyperexcitability.^7,16 While the majority of cases exhibit occipital EEG spikes, a significant number have spikes in other areas and spikes may appear in two brain areas at the same time or in the course of time in patients with PS and BCECTS.^15,17 In addition, the high frequency of ictal vomiting and other autonomic manifestations suggests that epileptic activity occurs in various cortical locations.

DIAGNOSTIC WORKUP

Neuroimaging and Other Laboratory Examinations