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Brain & Development 32 (2010) 4–9

Review article

Panayiotopoulos syndrome: An important childhoodautonomic epilepsy to be differentiated from occipital epilepsy

and acute non-epileptic disorders

Michael Michael a, Katerina Tsatsou a, Colin D. Ferrie b,*

a St. Thomas’ Hospital, London, UKb Leeds General Infirmary, Department of Paediatric Neurology, Clarendon Wing, Leeds General Infirmary, Leeds LS2 9NS, UK

Received 2 December 2008; received in revised form 1 March 2009; accepted 2 March 2009

Abstract

Panayiotopoulos syndrome is a common multifocal autonomic childhood epileptic disorder with significant clinical, pathophys-iological and management implications. It affects otherwise normal children with onset at around 3–6 years. It is characterized byseizures, often prolonged, with predominantly autonomic symptoms and mainly ictal vomiting. EEG shows shifting and/or multiplefoci, often with occipital dominance. Despite characteristic clinical and EEG manifestations Panayiotopoulos syndrome is oftenconfused with occipital epilepsy and acute non-epileptic disorders such as encephalitis, syncope, cyclic vomiting or atypical migraine.This review aims to describe Panayiotopoulos syndrome on the basis of independent major studies and provide clinical clues fordiagnosis and management.� 2009 Elsevier B.V. All rights reserved.

Keywords: Autonomic seizures; Autonomic status epilepticus; Multifocal epilepsy; Benign seizures

1. Introduction

Panayiotopoulos syndrome is an important, commonand well documented childhood susceptibility to epilep-tic seizures [1] formally recognized by the InternationalLeague against Epilepsy (ILAE) [2]. It is a model ofautonomic seizures and autonomic status epilepticusspecific to childhood [3,4], which is frequently misdiag-nosed as non-epileptic disorders including acute enceph-alitis, syncope and cyclic vomiting syndrome [5].Panayiotopoulos syndrome affects otherwise normalchildren. The electroencephalogram (EEG) [6,7] andmagnetoencephalogram [8,9] are the only tests thatmay be abnormal and these usually show multifocal epi-

0387-7604/$ - see front matter � 2009 Elsevier B.V. All rights reserved.

doi:10.1016/j.braindev.2009.03.002

* Corresponding author. Tel.: +44 1133922188.E-mail address: colindferrie@aol.com (C.D. Ferrie).

leptogenic abnormalities. Prognosis of Panayiotopoulossyndrome is usually excellent and prophylactic antiepi-leptic drug is often not needed.

Despite significant progress made in the delineationof its clinical and pathophysiological features as wellas the immense implications of its recognition in clinicalpediatric practice, there is still noteworthy misunder-standing of what Panayiotopoulos syndrome is and par-ticularly its erroneous identification with idiopathicchildhood occipital epilepsy of Gastaut or EEG occipi-tal paroxysms [10–12].

The purpose of this review is to describe the key clin-ical and EEG features of Panayiotopoulos syndromeand point at differential diagnostic matters that mayfacilitate its early diagnosis and management in clinicalpediatric practice. Table 1 shows cumulative data of 550patients with Panayiotopoulos syndrome from sevenlarge scale independent studies [1,13–18].

Table 1Data from 7 independent major studies of 550 patients with PS[1,13–18].

Clinical data

Female/male 293/257 ratio = 1.14Mean age at first seizure (range) 5.1(1–14)Mean age at last seizure (range) 6.6 (2–15)Febrile seizures 18% (4–45%)Ictal vomiting 72.2% (44–87%)Deviation of the eyes 68% (31–100%)Ending with hemi or generalizedconvulsions

50.2% (30–70%)a

Visual hallucinations 7.2% (2–13%)c

Autonomic status epilepticus 43% (24–64%)Asleep only 64% (52–84%)Awake only 24% (3–37%)Single seizure 40% (18–83%)Two–five seizures 48% (41–58%)a

More than 10 seizures 5% (3–6%)a

EEG data

Occipital spikes 78% (65–100%)b

Occipital paroxysms 48% (21–74%)Extra-occipital spikes in thesame or other EEG

32%a

Extra-occipital spike only or normal EEG 27%c

Epidemiological data

Prevalence in children aged 1–15 years withnon-febrile seizures

6%a

Panayiotopoulos syndrome/ICOE-G 192/27a

Panayiotopoulos syndrome/Rolandic epilepsy 308/649a

a Data are not provided in all cited reports.b This is considered as a prerequisite inclusion criterion in some of

the cited reports.c This is considered as an exclusion criterion in some of the cited

reports.

M. Michael et al. / Brain & Development 32 (2010) 4–9 5

1.1. Classificattion and nomenclature

There are three formally recognized benign childhoodfocal epileptic syndromes: Rolandic epilepsy, Panayioto-poulos syndrome, and idiopathic childhood occipitalepilepsy of Gastaut [2,19]. Rolandic epilepsy (or benignchildhood epilepsy with centrotemporal spikes) has beenwell known to pediatricians for over 50 years. Idiopathicchildhood occipital epilepsy of Gastaut was initiallydescribed as ‘‘childhood epilepsy with occipital parox-ysms” but later because of the recognition of Panayioto-poulos syndrome, this changed to ‘‘late onset childhoodoccipital epilepsy (Gastaut type)”. Similarly, Panayioto-poulos syndrome was initially recognized as ‘‘early onsetbenign childhood epilepsy with occipital paroxysms”,later as ‘‘early onset benign childhood occipital epilepsy(Panayiotopoulos type)” and now as Panayiotopoulossyndrome because of convergent evidence that its clini-cal, EEG and pathophysiological spectrum are muchwider than occipital epilepsy as detailed in this review.

From the EEG point of view, an important point isthat centrotemporal, occipital, frontal, parietal and mid-line spikes are not specific of any syndrome as they alsooccur in 2–4% of normal and a significant proportion of

young patients with non-epileptic disorders [20]. Occip-ital paroxysms are long runs of occipital spikes thatmainly occur during eyes closed because of fixation-offsensitivity [21]. They are relatively rare and do not spec-ify a particular epileptic syndrome.

1.2. Panayiotopoulos syndrome is a childhood autonomic

epilepsy

Autonomic seizures and autonomic status epilepticusare the hallmarks of this syndrome which typically startsat a peak age of 3–6 years (range 1–14 years) with noapparent sex or race prevalence (Table 1) [4,22]. In atypical presentation of a daytime seizure the child feelssick, looks unwell and vomits. Then gradually con-sciousness becomes impaired, eyes deviate to one sideand the event may end with a convulsion.

Autonomic seizures are defined as epileptic seizurescharacterized by altered autonomic function of any typeat seizure onset or in which manifestations consistentwith altered autonomic function are prominent (quanti-tatively dominant or clinically important) even if notpresent at seizure onset. The altered autonomic functionmay be objective or subjective or both [4].

Ictal emesis (nausea, retching and vomiting) are thepredominant symptoms of autonomic seizures in Pana-yiotopoulos syndrome and occur in around 80% ofpatients (Table 1). Other autonomic manifestationsinclude pallor and less frequent cyanosis, mydriasisand less frequent miosis, cardiac, respiratory and ther-moregulatory abnormalities, hypersalivation, cephalgicauras and incontinence of urine and feces. Cardiorespi-ratory arrest has been reported in four out of around1000 cases; recovered completely [3,19]. Syncopal-likeattacks are an interesting feature of Panayiotopoulossyndrome that occur in at least one-fifth of seizures[1,3,4]. The child becomes ‘completely unresponsiveand flaccid like a rag doll’. They may be concurrent withother seizure symptoms or be the sole manifestation of aseizure [1,14]. They may occur while the patient is stand-ing, sitting, lying down or asleep and last from 1–2 minto half an hour. Restlessness, agitation, terror or quiet-ness, may appear at the onset of seizures, often in com-bination with other autonomic manifestations.

Autonomic seizures often progress to other moreconventional ictal symptoms and particularly impair-ment of consciousness, deviation of the eyes, hemicon-vulsions and less frequent generalized convulsions(Table 1). Visual hallucinations occur in about 10%patients, but nearly always happen after the onset ofautonomic manifestations; in only 1% of seizures arethey the first apparent symptom [1,16,17].

Nearly two-thirds of the seizures occur during daytimenaps or sleep. Typically, the autonomic seizures of Pana-yiotopoulos syndrome are of long duration and nearlyhalf of them last for more than half an hour constituting

6 M. Michael et al. / Brain & Development 32 (2010) 4–9

autonomic status epilepticus (Table 1). The others last onaverage 9 min with a range from 1 to 30 min.

1.3. Panayiotopoulos syndrome is a multifocal epilepsy as

documented with EEG and magnetoencephalography

The EEG is the most useful test. Interictal EEG isusually abnormal with mainly multifocal spikes (80%of patients) that often shift from one region toanother in sequential EEGs of the same child(Fig. 1) [1,6,15–17]. Occipital spikes predominate butdo not occur in a third of patients. Occipital parox-ysms are reported in nearly half the patients (Table1) but fixation-off sensitivity is rare (less than 10%)[23]. A single routine EEG is normal in 10% ofpatients, and a few children have consistently normalwake EEGs before a diagnostic sleep recording. Sleeptypically accentuates the spike abnormalities, and pho-tosensitivity is nearly always absent. Spikes may per-sist for many years after clinical remission or appearonly once despite multiple EEGs.

EEG dipole analysis [7] and magnetoencephalogra-phy [8,9,24] have documented the multifocal epilepto-genic potential of Panayiotopoulos syndrome alongthe parieto-occipital, calcarine and central sulci or inthe frontal lobes.

In the few reported ictal EEGs, the ictal discharge hasstarted from various brain regions, more often posteriorthan anterior, becomes diffuse and lasts for many min-utes before the first clinical symptoms become apparent[14,25–29].

1.4. The differential diagnosis of Panayiotopoulos

syndrome is mainly from non-epileptic disorders

The main difficulty in diagnosing Panayiotopoulossyndrome is that emetic and other autonomic manifesta-tions are often dismissed as unrelated to epileptic sei-zures or erroneously considered as features ofencephalitis, migraine, syncope or gastroenteritis. Thisis also the reason for the belated recognition of this com-mon syndrome [1,5,30,31]. A history of a previous sim-ilar seizure or full recovery after a few hours of sleep isreassuring and may help to avoid unnecessary investiga-tions and other treatments [1,23,31]. Conversely,patients with non-epileptic episodic autonomic distur-bances may be misdiagnosed as Panayiotopoulos syn-drome particularly considering the high incidence offunctional spikes in non-epileptic children [20].

Ten to twenty percent of patients with autonomic sei-zures have cerebral lesions as identified by abnormalneurological or mental state examination, abnormalbrain imaging and background EEG abnormalities. Itis because of these cases that brain imaging is indicated.

Panayiotopoulos syndrome is significantly differentfrom the idiopathic childhood occipital epilepsy of Gas-

taut with which it is still misdiagnosed [10–12]. Overlap-ping cases are more common with rolandic epilepsy thanwith idiopathic childhood occipital epilepsy of Gastaut[19]. As in any other epileptic syndrome, atypical casesor unusually severe cases may be difficult to diagnosewithout thorough clinical assessment and long follow-up.

1.5. Idiopathic childhood occipital epilepsy of Gastaut is a

pure occipital epilepsy

The idiopathic childhood occipital epilepsy of Gas-taut is a relatively rare form of pure occipital epilepsythat equally affects boys and girls with a peak age atonset at 8–11 years (range 3–15 years) [32–35]. It affectsotherwise normal children (idiopathic) and manifestswith ictal symptoms of occipital lobe origin. These aresubjective, objective or both. Visual subjective symp-toms mainly include elementary and less often complexvisual hallucinations, blindness and visual illusions.Objective oculomotor symptoms are tonic deviation ofthe eyes, oculoclonic movements or nystagmus andrepetitive eyelid closures or eyelid fluttering. Seizuresmay spread to more anterior brain regions generatingsymptoms from the temporal, parietal and frontal lobesand may progress to secondarily hemi- or generalizedconvulsions. Ictal vomiting may occur with progressionto the non-dominant temporal lobe [36]. Post-ictal head-ache, sometimes undistinguished from migraine, occursin more than half of patients with idiopathic childhoodoccipital epilepsy of Gastaut.

Elementary visual hallucinations are by far the com-monest ictal manifestation. They develop quickly withinseconds and generally do not last more than 1–3 min.They are usually multicolored and circular and are almostalways stereotyped in morphology, colors, location,movement and other specific features for each patient [34].

Seizures are usually frequent, brief and mainly diur-nal. Consciousness is intact during the visual symptoms(simple focal seizures), but may be disturbed or lost inthe course of the seizure, usually before or at the timeof eye deviation or convulsions.

In a typical presentation of idiopathic childhoodoccipital epilepsy of Gastaut the child has frequentvisual seizures of multicolored and circular patterns thatdevelop within seconds, do not last more than 1–2 minand are followed by severe headache.

Seizures are usually spontaneous but they may alsobe precipitated by lights, television or video games[32–35].

The EEG is the most useful test. Brain MRI is neededto exclude symptomatic occipital epilepsy that some-times occur in neurologically and cognitively normalchildren. The typical and EEG shows occipital spikeseither randomly or in long clusters of occipital parox-ysms with or without fixation-off sensitivity. All patients

Fig. 1. Despite similar clinical features, the interictal EEG shows great variability between these five patients. Patient A: EEG with bi-occipitalparoxysms. Patient B: EEG with bi-temporal and unilateral central and frontal spikes. Patient C: EEG with bi-frontal and unilateral central spikes.Patient D: EEG with repetitive bi-occipital-frontal spikes. Patient E: EEG with brief generalized discharges.

M. Michael et al. / Brain & Development 32 (2010) 4–9 7

with reflex photosensitive occipital lobe seizures haveposterior photoparoxysmal responses during photicstimulation.

There are numerous ictal EEGs of visual seizures.These show fast rhythms, fast spikes or both entirelylocalized to an occipital electrode [25,32,33,37–41].

1.6. The differential diagnosis of idiopathic childhoodoccipital epilepsy of Gastaut is mainly from migraine and

symptomatic occipital epilepsy

Idiopathic childhood occipital epilepsy of Gastaut isoften mistaken for migraine with aura and basilar

8 M. Michael et al. / Brain & Development 32 (2010) 4–9

migraine though these are easy to differentiate if all clini-cal elements are properly assessed and synthesized despitethe severe headache and vomiting that may follow theattacks in both of them. Visual seizures develop fast, areshort and consist of colored and circular patterns. Visualaura of migraine develops slowly over minutes, is long-lasting for 10–20 min and consists of mainly linear, zig-zag achromatic patterns [42–44]. Basilar migraine developgradually over 4 min, bilateral and blurring or blindness isassociated with other neurological symptoms such as ver-tigo, ataxia, diplopia and decreased hearing.

The differential diagnosis of idiopathic childhoodoccipital epilepsy of Gastaut from symptomatic occipi-tal epilepsies may be impossible without high resolutionbrain MRI for [45,46].

Idiopathic childhood occipital epilepsy of Gastaut isdistinctive from Panayiotopoulos syndrome. Seizureonset is primarily with visual symptoms in idiopathicchildhood occipital epilepsy of Gastaut and with auto-nomic manifestations in Panayiotopoulos syndrome [19].

1.7. Prognosis of Panayiotopoulos syndrome andidiopathic childhood occipital epilepsy of Gastaut

Panayiotopoulos syndrome appears to be remarkablybenign with half of children having a single or less thanfive seizures and 90% of patients going into completeremission within 1–2 years of onset [1,13,15–17]. Theothers may have frequent seizures, protracted active sei-zure period and may develop rolandic and less oftenoccipital or other seizures but these are also age-relatedand remit. Recently, SCN1A mutations have beenreported in a child [47] and two siblings [48] with rela-tively early onset of seizures, prolonged duration ofactive seizures which were often prolonged and whichhad a strong association with febrile precipitants evenafter the age of 5 years. This may indicate that Panayio-topoulos syndrome is genetically heterogeneous withSCN1A mutations contributing to a more severe clinicalphenotype. Cognitive function has not been assessedwith detailed psychological testing and this should beaddressed particularly for those with the severe formsof Panayiotopoulos syndrome.

The prognosis of idiopathic childhood occipital epi-lepsy of Gastaut is unclear but it is likely that aroundhalf of patients go into remission within 2–4 years ofonset [33–35]. The others continue to have visual sei-zures with infrequent secondarily GTCS well into adultlife. Seizures show a dramatically good response to car-bamazepine in more than 90% of patients.

1.8. Management

The great majority of children with Panayiotopoulossyndrome do not need antiepileptic drug treatment

[3,17,19]. However, prophylactic treatment may be indi-cated if a child has multiple recurrences or in the unli-kely event of parental insistence [49]. Conversely, inidiopathic childhood occipital epilepsy of Gastaut treat-ment is necessary because seizures are frequent and sec-ondarily GTCS are probably unavoidable in untreatedpatients. All antiepileptic drugs licensed for mono-therapy in focal seizures are probably appropriate,though most authorities prefer carbamazepine. Despiteits excellent prognosis, Panayiotopoulos syndrome usu-ally has a dramatic impact on parents who are con-cerned regarding the nature, cause and the impact ofthe events on their child’s development as well as thelack of appropriate information [50]. Parents need spe-cific information about Panayiotopoulos syndrome, inwhich seizures may have dramatic features and last formany hours. Parental anxiety is frequently compoundedby physicians’ uncertainty over diagnosis, managementand prognosis [50].

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