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Current thumbnail: Panayiotopoulos syndrome is an idiopathic, childhood-related, benign susceptibility to focal, mainly autonomic, seizures and autonomic status epilepticus. In this clinical summary, C P Panayiotopoulos, MD, PhD, FRCP, Consultant at the Department of Clinical Neurophysiology and Epilepsies of St. Thomas Hospital in London, England, details recent independent reports of more than 500 children, confirming the uniformity of Panayiotopoulos syndrome worldwide. Also reviewed in this summary are the wide spectrum of ictal autonomic manifestations, including ictal syncope, the marked variability of interictal EEG findings from normal to multifocal spikes, and the variable ictal EEG onset from the frontal or posterior regions.

Historical note and nomenclature
Panayiotopoulos syndrome is best described as early onset benign childhood seizure susceptibility syndrome with mainly autonomic seizures and autonomic status epilepticus. Well-documented current evidence indicates that descriptive terms referring to “occipital epilepsy” or “epilepsy with occipital spikes or occipital paroxysms” are incorrect and should be discouraged (Martinovic 2002; Panayiotopoulos 2002; Ohtsu et al 2003; Panayiotopoulos 2005).

Though the syndrome was initially identified in children with occipital paroxysms (with or without fixation-off sensitivity) or occipital spikes (Panayiotopoulos 1981; 1989a; 1989b), these may not occur in one third of patients whose EEG is normal or shows extraoccipital spikes only (Panayiotopoulos 1988; 1999b; 2002; Lada et al 2003; Ohtsu et al 2003; Panayiotopoulos 2005).

Though commonly referred as “occipital epilepsy” or “occipital seizure susceptibility” this also appears to be incorrect because:

1. Onset of seizures is mainly with autonomic symptoms and, particularly, emesis (80%), which are not occipital lobe manifestations
2. Of occipital symptoms, only deviation of the eyes may originate from the occipital regions, but this rarely occurs at onset. Visual symptoms are exceptional and not consistent in recurrent seizures.
3. Interictal occipital spikes may never occur
4. Ictal EEG have documented variable onset from the posterior or anterior regions (Oguni et al 1999; Panayiotopoulos 2002; 2004; 2005).|{diagram:CPEO10.bmp}{caption:Video-EEG of autonomic status epilepticus in a child with Panayiotopoulos syndrome}{label:Top: High amplitude spikes-slow waves are recorded from the bifrontal regions prior to the onset of the electrical discharge, which is also purely bifrontal. Bottom: First clinical symptoms with 3 to 4 coughs and marked tachycardia appeared 13 minutes from the onset of the electrical discharge, when this had become diffuse. (Figure courtesy of Dr M Koutroumanidis, Department of Clinical Neurophysiology, St Thomas’ Hospital, London, UK)}|

Panayiotopoulos described this syndrome through a 30-year prospective study that started in 1973 (Panayiotopoulos 1981; 1987; 1988; 1989a; 1989b; 1999a; 1999b; 2002). Initial publications included patients with EEG occipital paroxysms or occipital spikes that attracted the main attention, but later it became apparent that the same clinical manifestations and mainly ictal vomiting could occur in children with EEG extraoccipital spikes or normal EEG.

In 1981, Panayiotopoulos first documented fixation-off sensitivity of the occipital paroxysms in 4 patients of different epileptic syndromes. Two patients had what is now known as Panayiotopoulos syndrome; the third patient had a syndrome later described by Gastaut (Gastaut 1982) and now known as Gastaut-type childhood occipital epilepsy, and the fourth suffered from symptomatic occipital epilepsy (Panayiotopoulos 1981).|{diagram:CPEO1.bmp}{caption:EEG of occipital paroxysms in 4 children with different types of epileptic seizures}{label:Left top and bottom: EEG of 2 girls with Panayiotopoulos syndrome. Right top: EEG of a boy with frequent, brief visual seizures of elementary visual hallucinations and, occasionally, blindness. Right bottom: EEG a 15-year-old boy with symptomatic occipital lobe epilepsy. In routine EEG, high amplitude, continuous occipital sharp, and slow wave complexes (occipital paroxysms) occurred immediately after closing of the eyes, lasting as long as the eyes were closed. The EEG normalized immediately after opening the eyes and continued as long as the eyes were open, though some break in occipital spikes occurred. The activation of the occipital paroxysms was due to the elimination of central vision and fixation (left of the vertical bar, symbol of eyes with glasses). Occipital spikes were inhibited by fixation (right of the vertical bar, symbol of eyes without glasses). (Used with permission, Panayiotopoulos 1981)}| Subsequently, Panayiotopoulos documented the following:

• Ictal vomiting as a common but not exclusive autonomic seizure manifestation
• Frequent ictal autonomic manifestations with or without ictus emeticus
• Autonomic status epilepticus occurring in half of patients
• Mean age at onset of 5 years
• Excellent prognosis, with most patients having 1 to 3 seizures in total and remission occurring within 1 to 2 years from onset.
• High prevalence of approximately 6% of childhood non-febrile seizures
• Same seizures and prognosis irrespective of EEG findings
• Centrotemporal and other foci could appear together with occipital spikes in the same or subsequent EEG, and Rolandic seizures could occur at a later stage. Similar seizures with equally good prognosis could also occur in children with normal EEG or extraoccipital spikes (Panayiotopoulos 1988; 1989a; 1989b; 1999a; 1999b).

In Panayiotopoulos’ original study, ictal vomiting occurred only in 24 children out of 900 patients of all ages with epileptic seizures (Panayiotopoulos 1988). Twenty one (87.5%) were otherwise normal children (idiopathic cases constituting what is now considered as Panayiotopoulos syndrome), and 3 had symptomatic epilepsies. Seizures were mainly nocturnal. Ictal vomiting was always concurrent with other epileptic manifestations, more often deviation of the eyes and impairment of consciousness. The initial part of the ictus was short or prolonged for hours with frequent "marching" to hemiconvulsions and generalized seizures. The EEG of the 21 idiopathic cases showed great variations: 12 had occipital paroxysms or spikes alone or with extraoccipital spikes; 2 had central spikes and giant somatosensory evoked spikes; 2 had midline spikes; 1 had frontal spikes; 1 had brief generalized discharges; and 3 had consistently normal EEG.|{diagram:CPEO2.bmp}{caption:EEG of 3 children with extraoccipital spikes only or brief generalized discharges}{label:Left: EEG for this child had centrotemporal spikes and giant somatosensory evoked spikes (arrows indicate tapping of the left fingers). Middle: EEG of another child had scarce and brief generalized discharges of small spikes and slow waves. Right: EEG of this child had only midline spikes. (Used with permission, Panayiotopoulos 2002)}| Subsequent attention was focused on the predominant group with occipital spikes, which was established as “early onset benign childhood epilepsy with occipital paroxysms” (Panayiotopoulos 1989a; 1989b). The other group of 9 children with extraoccipital spikes or normal EEGs was re-evaluated much later (Panayiotopoulos 1999b); their clinical manifestations and outcome was similar to those patients with occipital spikes. Based on these results, it has been concluded that these 21 children, despite different EEG manifestations, suffered from the same disease, which is now designated as Panayiotopoulos syndrome to incorporate all cases irrespective of EEG localizations (Berg and Panayiotopoulos 2000; Caraballo et al 2000; Ferrie and Grunewald 2001; Oguni 2001; Koutroumanidis 2002; Martinovic 2002; Panayiotopoulos 2002; Lada et al 2003; Ohtsu et al 2003; Panayiotopoulos 2004; 2005).

Panayiotopoulos syndrome has been confirmed worldwide with a unique uniformity in all races (Panayiotopoulos 2002; 2005).

Clinical manifestations
Panayiotopoulos syndrome is a childhood-related idiopathic benign susceptibility to focal, mainly autonomic seizures and autonomic status epilepticus. The children have normal physical and neuropsychological development.

Seizure characteristics. Seizures comprise an unusual constellation of autonomic, mainly emetic symptoms, often with unilateral deviation of the eyes and other more conventional ictal manifestations. In a typical presentation, the child is fully conscious, able to speak and understand but complains “I feel sick,” looks pale, and vomits. One parent reported: “He told me that he felt sick, and on his way to the toilet his eyes and head turned to the right, and he was talking out of context, and then he was sick.”

Two thirds of the seizures start in sleep; the child may wake up with similar complaints while still conscious or else may be found vomiting, conscious, confused, or unresponsive. Oguni and colleagues documented from one ictal EEG that clinically, while asleep “he suddenly got up with both eyes open, vomited several times, and then showed a prolonged atonic state with cyanosis and irregular respiration for 3 minutes” (Oguni et al 1999).

The full emetic triad (nausea, retching, vomiting) culminates in vomiting in 74% of the seizures; in others only nausea or retching occur, and in a few, emesis may not be apparent. Other autonomic manifestations may occur concurrently or appear later in the course of the ictus. These include pallor and less often flushing or cyanosis, mydriasis, and less often miosis, coughing, cardiorespiratory and thermoregulatory alterations, incontinence of urine or feces, and modifications of intestinal motility. Hypersalivation (probably a concurrent Rolandic symptom) may occur. Headache and, more often, cephalic auras that may be autonomic manifestations occur, particularly at onset. Apnea and cardiac asystole may be common, but only exceptionally are these severe and potentially fatal without immediate medical intervention (Panayiotopoulos 2005; Verrotti et al 2005).

More conventional seizure-symptoms often ensue. The child gradually or suddenly becomes confused and unresponsive; exceptionally, consciousness may be preserved (6%). Eyes and often the head deviate to one side (60%), or eyes gaze widely open (12%). Other symptoms in order of prevalence are: speech arrest (8%), hemifacial spasms (6%), visual hallucinations (6%), oropharyngolaryngeal movements (3%), unilateral drooping of the mouth (3%), eyelid jerks (1%), myoclonic jerks (1%), ictal nystagmus, and automatisms (1%). The seizures commonly end with hemiconvulsions, often with Jacksonian march (19%) or generalized convulsions (21%). Hemiconvulsive (2%) or generalized convulsive status (2%) is exceptional.

Ictal syncope is an intriguing and important ictal feature of Panayiotopoulos syndrome. “Ictal syncope” is a descriptive term to denote transient loss of consciousness and postural tone that occurs in a seizure before or without convulsions. The child becomes “completely unresponsive and flaccid like a rag doll” before and often without convulsions or in isolation. In one report, “suddenly and without warning she collapsed and became unresponsive while talking to her teacher.... She complained of ‘dizziness,’ and then her eyes deviated to the left, she fell on the floor, and she became totally flaccid and unresponsive for 5 minutes” (Panayiotopoulos 2002; 2004; 2005).

The same child may have seizures with marked autonomic manifestations and seizures in which autonomic manifestations may be inconspicuous. The clinical seizure manifestations are roughly the same irrespective of EEG localizations, though there may be slightly less autonomic and slightly more focal motor features at onset in children without occipital spikes.

Duration of seizures. Nearly half (44%) of the seizures last for more than 30 minutes and up to 7 hours (mean, approximately 2 hours), constituting autonomic status epilepticus. Of the other half (54%) duration varies from 1 to 30 minutes with a mean of 9 minutes. Lengthy seizures are equally common in sleep and wakefulness. Even after the most severe seizures and status, the patient is normal after a few hours' sleep. There is no record of residual neurologic or mental abnormalities. The same child may have brief and lengthy seizures.

Circadian distribution. In two thirds (66%) of affected children, seizures occur only during sleep and particularly the first hour of sleep. In one-fifth (17.5%), seizures start while the child is awake. The remaining 16.5% have seizures both during sleep and awake. These figures may change with the inclusion of less typical cases such as those patients who manifest with mainly ictal syncope.

Age at onset and sex. Range is from 1 year to 14 years with a consistent median around the age of 5 years (mean 4.7, plus or minus 1.7 years). Three quarters (76%) occur between the ages of 3 years and 6 years. Boys and girls are equally affected.

Clinical vignette
Case 1. A 29-year-old woman had 2 nocturnal seizures in her life, both at age 6 years (Panayiotopoulos 1981). In the first fit she was found vomiting vigorously, eyes turned to one side, pale, and unresponsive. Her condition remained unchanged for 3 hours before she developed generalized tonic clonic seizures. She gradually improved, and by the next morning was normal. The second seizure occurred 4 months later. She awoke and told her mother that she wanted to vomit, and then vomited. Within minutes her eyes turned to the right. Her mother, who was on her left, asked, "Where am I?" "There, there," the child replied, indicating to the right. Ten minutes later she closed her eyes and became unresponsive. Generalized convulsions occurred 1 hour from onset. Thereafter she recovered quickly. Her EEGs showed occipital paroxysms, but this normalized by the age of 10 years.|{diagram:CPEO1.bmp}{caption:EEG of occipital paroxysms in 4 children with different types of epileptic seizures}{label:Left top and bottom: EEG of 2 girls with Panayiotopoulos syndrome. Right top: EEG of a boy with frequent, brief visual seizures of elementary visual hallucinations and, occasionally, blindness. Right bottom: EEG a 15-year-old boy with symptomatic occipital lobe epilepsy. In routine EEG, high amplitude, continuous occipital sharp, and slow wave complexes (occipital paroxysms) occurred immediately after closing of the eyes, lasting as long as the eyes were closed. The EEG normalized immediately after opening the eyes and continued as long as the eyes were open, though some break in occipital spikes occurred. The activation of the occipital paroxysms was due to the elimination of central vision and fixation (left of the vertical bar, symbol of eyes with glasses). Occipital spikes were inhibited by fixation (right of the vertical bar, symbol of eyes without glasses). (Used with permission, Panayiotopoulos 1981)}|

The patient and her sister had infrequent vasovagal syncopal episodes. After one such syncopal episode (retrospectively the possibility of ictal syncope should be considered) the patient was misdiagnosed as having temporal lobe epilepsy. We have communicated with her or her parents annually from 1976. She is well, successfully educated, and works in business administration.

Case 2. This case involved diurnal autonomic status epilepticus with behavioral disturbances that would be difficult to attribute to seizure activity before the motor partial ictal events. A now 6-year-old normal boy had a seizure at age 4 years while traveling on a train with his parents who vividly described the event:

He was happily playing and asking questions when he started complaining that he was feeling sick, became very pale, and quiet. He did not want to drink or eat. Gradually, he was getting more and more pale, kept complaining that he felt sick, and became restless and frightened. Ten minutes from the onset, his head and eyes slowly turned to the left. The eyes were opened but fixed to the left upper corner. We called his name but he was unresponsive. He had completely gone. We tried to move his head but this was fixed to the left. There were no convulsions. This lasted for another 15 minutes, when his head and eyes returned to normal and he looked better, although he was droopy and really not there. At this stage he vomited once. In the ambulance, approximately 35 minutes from the onset, he was still not aware of what was going on, although he was able to answer simple questions with yes or no. In the hospital he slept for three quarters of an hour and gradually came around, but it took him another half to an hour before he became normal again.

EEG showed occipital paroxysms and MRI was normal. A similar prolonged episode, preceded by behavioral changes, occurred 8 months later at school. He received no medication. Since then he has been well.

Case 3. This case involved nocturnal and diurnal autonomic status epilepticus with frequent vomiting witnessed from onset. An 8-year-old boy of mixed race had 2 prolonged seizures at the age of 5 years. The first seizure occurred during a brief nap. He woke up and walked with “shaking feet” to his mother, complaining that he felt sick. Within 2 to 3 minutes, his eyes and subsequently his head turned to the extreme right. His mother recalls:

I asked him to look at me, and he would not. If I moved his head to the front, it would go back to the right. Within a minute he vomited, and his eyes started blinking, and there were also tiny jerks of his body, legs, and arms that lasted for a minute. He became unresponsive to anything I said to him. He then was rigid, and he went to a deep sleep like in a coma. In the hospital he continued to be in this unconscious state, ever so often just getting up to be sick, and straight back down again. He did not start to regain consciousness or be aware of people around until about 3 hours later. He was well the next morning and discharged home.

The second seizure occurred 6 months later in a ferryboat trip:

He told me that he felt sick, and on his way to the toilet his eyes and head turned to the right and he was talking out of context, and then he was sick. I thought he was having another fit. He was still able to converse with me in and out of sleep. He did not become unconscious, but he was continuously sick for several hours. By the time we arrived in a hospital 3 hours later, he was improving; he just seemed tired. The doctors told us that this was due to dehydration, for which treatment was provided. He was normal the next morning.

Awake EEG, 1 month after the first seizure, showed only 1 left-sided occipital and a possible frontal midline spike; the second EEG at age 8 showed infrequent central, frontal, and midline spikes during sleep.|{diagram:CPEO3.bmp}{caption:EEG showing occipital spikes of child aged 5 and 8 years}{label:Left: one left occipital and one probable midline spike were recorded at age 5 years. Right: central, frontal, and midline spikes were recorded in sleep EEG at age 8 years.}|

Case 4. This case involved a diurnal pure autonomic status epilepticus with midline spikes and subsequent Rolandic seizures with centrotemporal spikes. A 9-year-old boy returned from school one day looking tired and pale. Five minutes later, he complained of headache and became agitated and more pale. Within 5 minutes, he started banging his head on the wall and soon became unresponsive and floppy “like a rag doll,” incontinent of urine and feces with his eyes widely open and pupils markedly dilated. At this stage, he vomited vigorously. This condition continued on his way to the hospital where he arrived by ambulance half an hour from onset. Three hours later, he was still confused, partly unresponsive, pale, and quiet, and he vomited again. Recovery started 4 hours from onset. He did not convulse at any stage. He was apyrexial, and other autonomic functions were normal. He slept and was entirely normal the next morning, discharged home with the diagnosis “epileptic seizure? probably atypical migraine.”

EEG had midline spikes at central midline electrode. On followup exam 1 year later, he had 2 typical Rolandic seizures, and EEG showed centrotemporal spikes.|{diagram:CPEO4.bmp}{caption:EEG showing occipital spikes of a child at age 9 years}{label:(Left) Abundant midline spikes only were recorded in his first EEG. (Right) One year later, spikes were localized mainly in the right centrotemporal regions; some scattered right sided occipital spikes were also seen.}| At last followup, at age 11 years, he was well with no further seizures.

Case 5. This case involved seizures manifesting mainly with ictal syncope. A 7-year-old boy had from age 5 years approximately 12 episodes of collapse at school. All episodes were stereotyped but of variable duration from 2 to 35 minutes. While standing or sitting, he slumped forwards and fell on his desk or the floor and became unresponsive as if in “deep sleep.” There were no convulsions or other discernible ictal or postictal symptoms.

Four EEGs consistently showed frequent multifocal spikes predominating in the frontal regions.

Case 6. This case involved diurnal seizure with inconspicuous onset progressing to more dramatic events after sleep. A 5-year-old girl was pale and feeling unwell prior to leaving for school. Later that morning, her parents were informed that she was sick and retching. At home, after a brief natural sleep, she awoke with coughing and retching and asked for a glass of water. Within 10 minutes, she became unresponsive. Her eyes opened widely and she began convulsing. Convulsions were controlled 30 minutes later with intravenous administration of diazepam. She was back to normal the next morning. EEG showed random occipital and central spikes.|{diagram:CPEO5.bmp}{caption:EEG showing occipital spikes of a child at age 5 years}{label:Random occipital spikes mainly on the left and an independent right central spike are recorded in her EEG 2 days after her first prolonged seizure.}| Subsequently, she had an atypical evolution as reported elsewhere (Ferrie et al 2002).

Etiology
Usually, no family history exists of similar seizures, though siblings with Panayiotopoulos syndrome have been reported. Of 113 patients of Ferrie and colleagues, 2 had other siblings with Rolandic epilepsy, 1 had siblings with Panayiotopoulos syndrome, and 7% of the patients had relatives of the first degree with epilepsy (Ferrie et al 1997). In a more recent prospective study, 2 pairs of 66 patients were siblings, and a family history of epilepsy was found in 30.3% (Caraballo et al 2000). Of 42 patients described by Lada and colleagues, there were 2 pairs of siblings with Panayiotopoulos syndrome (Lada et al 2003).

Febrile convulsions are frequent, approximately 17% ranging in various reports from 12% to 47%. There may be a high prevalence of abnormal birth deliveries (Panayiotopoulos 2002).

Pathogenesis and pathophysiology
Clinical and EEG findings of Panayiotopoulos syndrome indicate that there is a diffuse cortical hyperexcitability, which is maturation-related. This diffuse epileptogenicity may be unequally distributed, predominating in one area, which is often posterior. The preferential involvement of emetic and autonomic systems in general may be attributed to epileptic discharges generated at various cortical locations and influencing vulnerable emetic centres and the hypothalamus in children (Panayiotopoulos 2002; 2004; 2005).

Panayiotopoulos syndrome and all other benign childhood focal seizures, with Rolandic epilepsy as their main representative, are probably linked due to a common, genetically-determined, mild, and reversible functional derangement of the brain cortical maturational process that Panayiotopoulos proposed as "benign childhood seizure susceptibility syndrome" (Panayiotopoulos 1993; 1999a; 2002; 2005). The various EEG and seizure manifestations often follow an age- (maturation-) related localization (Panayiotopoulos 2002; 2005).

The pathogenetic links between Panayiotopoulos syndrome and Rolandic epilepsy are indicated by EEG and clinical data (Panayiotopoulos 1989a; 1999a; 2002; Ferrie et al 1997; Caraballo et al 2000; Covanis et al 2003; Lada et al 2003; Ohtsu et al 2003). Thus, in Panayiotopoulos syndrome:

• Children may also suffer from Rolandic seizures, either at the same, or a later age.
• Seizures often have concurrent symptoms of Rolandic epilepsy.
• Occipital and centrotemporal spikes, or functional spikes in other locations, may occur in the same EEG or, more often, in subsequent EEG. On other occasions, only extraoccipital spikes are recorded.|{diagram:CPEO6.bmp}{caption:Video-EEG samples of Panayiotopoulos-type seizures in child aged 5 and 7 years}{label:This child had 4 Panayiotopoulos type seizures from ages 5 to 7 years. EEG showed occipital and other spikes with brief generalized discharges that were asymptomatic. His first 2 seizures were diagnosed as gastroenteritis.}|

Epidemiology
Prevalence of Panayiotopoulos syndrome is around 13% of children 3 to 6 years old with one or more nonfebrile seizures and 6% of the age group of 1 to 15 years. Of the general population of children, 2 to 3 per thousand may be affected. These figures may be higher if cases with currently atypical clinical presentations are included (Panayiotopoulos 2002; 2005).|{diagram:CPEO7.bmp}{caption:Prevalence of Panayiotopoulos syndrome}{label:The chart shows prevalence of Panayiotopoulos syndrome in relation to Rolandic and Gastaut type childhood occipital epilepsy based on 4 independent studies of 606 children. The overall prevalence of Panayiotopoulos syndrome is probably much higher if cases with atypical clinical presentations are included. Also, prevalence of Panayiotopoulos syndrome with extraoccipital spikes is much higher because in 2 of these studies occipital spikes was a required inclusion criterion.}|

Prevention
Not applicable.

Differential diagnosis
Despite sound clinico-EEG manifestations, Panayiotopoulos syndrome escaped recognition for many years, for many reasons. Ictal vomiting is rarely considered as a seizure event. When this is associated with a deteriorating level of consciousness, followed by convulsions, encephalitis or other acute cerebral insults are the prevailing diagnoses at the acute stage. If the child is seen after complete recovery, atypical migraine, gastroenteritis, or a first seizure are likely diagnoses. Similarly, ictal syncope only recently has been recognized as an important clinical manifestation of Panayiotopoulos syndrome; ictal syncope may be misdiagnosed as cardiogenic syncope, pseudoseizure, or a more severe encephalopathic state (Panayiotopoulos 2002; 2004; 2005).

Panayiotopoulos syndrome is easy to diagnose because of the characteristic clustering of clinical seizure semiology, which is often supported by interictal EEG findings. The main problem is to recognize emetic and other autonomic manifestations as seizure events and not to dismiss them or erroneously to consider them as unrelated to the ictus and a feature of encephalitis, migraine, syncope, or gastroenteritis.|{diagram:CPEO6.bmp}{caption:Video-EEG samples of Panayiotopoulos-type seizures in child aged 5 and 7 years}{label:This child had 4 Panayiotopoulos type seizures from ages 5 to 7 years. EEG showed occipital and other spikes with brief generalized discharges that were asymptomatic. His first 2 seizures were diagnosed as gastroenteritis.}|

Of other epileptic conditions, the following should be considered:

1. In symptomatic causes of similar autonomic seizures and autonomic status epilepticus in children, there is often abnormal neurologic or mental symptomatology, abnormal brain imaging, and EEG background abnormalities.
2. Panayiotopoulos syndrome and Gastaut-type childhood occipital epilepsy have entirely different clinical manifestations despite common interictal EEG when occipital paroxysms occur.
3. Rolandic seizures have different clinical manifestations, and emesis has not been reported. However, recently Covanis and colleagues reported 24 children with EEG centrotemporal spikes and ictal emesis (Covanis et al 2003). Twenty (83%) of the 24 patients had ictal semiology typical of Panayiotopoulos syndrome, but 5 also had concurrent Rolandic symptoms, and 4 later developed pure Rolandic seizures. The other 4 patients (17%) had typical Rolandic seizures with concurrent ictus emeticus.
4. Cases of Panayiotopoulos syndrome with seizures occurring when the child is febrile may be diagnosed as febrile seizures, but again, this is of no prognostic significance.
5. Photosensitive occipital seizures may have similar symptoms of autonomic disturbances and ictal vomiting, but elementary visual hallucinations and other manifestations of visual seizures usually precede these (Guerrini et al 1995).

Improving diagnostic yield. Seizure clinical manifestations are probably more important than EEG. By increasing awareness, diagnosis is expected to improve. Referrals are often vague and include encephalitis, atypical migraine, gastroenteritis, or first prolonged seizure. The contribution of EEG technologists is crucial (Sanders et al 2004). In an ongoing prospective study, at the end of 3 years 228 consecutive children aged 1 to 14 years had one or more epileptic seizures. Fourteen patients (6.1%) had Panayiotopoulos syndrome diagnosed mainly on clinical grounds. This did not include 11 additional patients with possible Panayiotopoulos syndrome, either with atypical clinical and EEG features or inadequate information. Of the 14 children with typical Panayiotopoulos syndrome, only 3 were appropriately diagnosed on referral. Alarmingly, 9 patients were suspected of suffering from encephalitis, a diagnosis that demanded further invasive procedures such as lumbar puncture, erroneous treatment, and costly hospital admissions. For most of the patients, the correct diagnosis and management was prompted by the EEG technologist who obtained the appropriate history through a simple questionnaire while preparing the patient for an EEG (Sanders et al 2004).

Diagnostic workup
By definition of an idiopathic syndrome, neurologic, mental, and high resolution MRI is normal. The most useful laboratory test is EEG. The most important determinant of the neurodiagnostic procedures is the state of the child at the time of first medical attendance:

1. The child has a brief or lengthy seizure of Panayiotopoulos syndrome but fully recovers prior to arriving in the accident and emergency department or being seen by a physician. A child with the distinctive clinical features of Panayiotopoulos syndrome, particularly ictus emeticus and lengthy seizures, may not need any investigations other than EEG. However, because approximately 10% to 20% of children with similar seizures may have brain pathology, an MRI may be needed.
   
2. The child with a typical lengthy seizure of Panayiotopoulos syndrome partially recovers while still in a postictal stage, tired, mildly confused, and drowsy on arrival to the accident and emergency department or when seen by a physician. The child should be kept under medical supervision until fully recovered, which usually occurs after a few hours of sleep. Then guidelines are the same as in (1) above
   
3. The child is brought to the accident and emergency department or is seen by a physician while ictal symptoms continue. This is the most difficult and challenging situation. There may be dramatic symptoms accumulating in succession, which demand rigorous and experienced evaluation. A history of a previous similar seizure is reassuring and may prevent further procedures.

Electroencephalography. EEG, commonly (90%) reveals functional, mainly multifocal, high amplitude, sharp and slow wave complexes. EEG is indispensable in the diagnosis of patients with Panayiotopoulos syndrome if clinical information is inadequate or emetic manifestations are inconspicuous. Electroencephalographers should be alerted by frequent multifocal spikes of a normal child with one or a few seizures.|{diagram:CPEO1.bmp}{caption:EEG of occipital paroxysms in 4 children with different types of epileptic seizures}{label:Left top and bottom: EEG of 2 girls with Panayiotopoulos syndrome. Right top: EEG of a boy with frequent, brief visual seizures of elementary visual hallucinations and, occasionally, blindness. Right bottom: EEG a 15-year-old boy with symptomatic occipital lobe epilepsy. In routine EEG, high amplitude, continuous occipital sharp, and slow wave complexes (occipital paroxysms) occurred immediately after closing of the eyes, lasting as long as the eyes were closed. The EEG normalized immediately after opening the eyes and continued as long as the eyes were open, though some break in occipital spikes occurred. The activation of the occipital paroxysms was due to the elimination of central vision and fixation (left of the vertical bar, symbol of eyes with glasses). Occipital spikes were inhibited by fixation (right of the vertical bar, symbol of eyes without glasses). (Used with permission, Panayiotopoulos 1981)}||{diagram:CPEO2.bmp}{caption:EEG of 3 children with extraoccipital spikes only or brief generalized discharges}{label:Left: EEG for this child had centrotemporal spikes and giant somatosensory evoked spikes (arrows indicate tapping of the left fingers). Middle: EEG of another child had scarce and brief generalized discharges of small spikes and slow waves. Right: EEG of this child had only midline spikes. (Used with permission, Panayiotopoulos 2002)}||{diagram:CPEO3.bmp}{caption:EEG showing occipital spikes of child aged 5 and 8 years}{label:Left: one left occipital and one probable midline spike were recorded at age 5 years. Right: central, frontal, and midline spikes were recorded in sleep EEG at age 8 years.}||{diagram:CPEO4.bmp}{caption:EEG showing occipital spikes of a child at age 9 years}{label:(Left) Abundant midline spikes only were recorded in his first EEG. (Right) One year later, spikes were localized mainly in the right centrotemporal regions; some scattered right sided occipital spikes were also seen.}||{diagram:CPEO5.bmp}{caption:EEG showing occipital spikes of a child at age 5 years}{label:Random occipital spikes mainly on the left and an independent right central spike are recorded in her EEG 2 days after her first prolonged seizure.}||{diagram:CPEO6.bmp}{caption:Video-EEG samples of Panayiotopoulos-type seizures in child aged 5 and 7 years}{label:This child had 4 Panayiotopoulos type seizures from ages 5 to 7 years. EEG showed occipital and other spikes with brief generalized discharges that were asymptomatic. His first 2 seizures were diagnosed as gastroenteritis.}|
There is a great EEG variability of functional focal spikes at various electrode locations (Panayiotopoulos 1988; 1999b; 2002; Oguni et al 1999; Covanis et al 2003; Lada et al 2003; Ohtsu et al 2003; Sanders et al 2004). All brain regions are involved though the posterior predominate.|{diagram:CPEO8.bmp}{caption:Prevalence of spike localization in Panayiotopoulos syndrome}{label:Diagram shows prevalence of spike localization as estimated from the EEG analysis of 47 patients with Panayiotopoulos syndrome. All but the anterior temporal regions are involved. Note that frontal spikes are second more frequent after the occipital, followed by central spikes. Right and left occipital and frontal regions are equally involved. Midline spikes occurred in 17%. (Used with permission Panayiotopoulos 2002)}| Two thirds (68%) of patients have at least one EEG with occipital spikes, which are often (64%) concurrent with extraoccipital spikes also in at least one EEG. The other third (32%) never show occipital spikes; instead they have extraoccipital spikes (21%) only, consistently normal EEG (9%), or brief generalized discharges only (2%). EEG with multifocal spikes in more than two and often many brain locations occur in one third (30%) of the patients; single spike foci are rare (9%). Cloned-like repetitive multifocal spike-wave complexes may be a characteristic features when they occur (19%). They are probably pathognomonic of the syndrome if recorded from otherwise normal children with a few epileptic seizures. They have never been studied or reported before in idiopathic epilepsies. On the contrary, multifocal repetitive spikes are considered to indicate a bad prognosis and a symptomatic etiology. Cloned-like repetitive multifocal spike-wave complexes do not determine prognosis as they equally occur in children with one or more seizures.|{diagram:CPEO9.bmp}{caption:Cloned-like repetitive multifocal spike-wave complexes in 2 children with Panayiotopoulos syndrome}{label:Consider carefully the EEG of these 2 cases. Cloned-like repetitive multifocal spike-wave complexes practically constitute electrical status epilepticus. However, these 2 children were otherwise normal; their academic performance was excellent and certainly above average, and they only had a few autonomic seizures or autonomic status epilepticus. (Used with permission Panayiotopoulos 2002)}|

Spikes are usually of high amplitude and morphologically similar to the centrogyral (Rolandic) spikes. They often show stable dipoles in the occipital regions (Yoshinaga et al 2005). However, small and even inconspicuous spikes may appear in the same or previous EEG of children with giant spikes. Though rare, small positive spikes or other unusual EEG spike configurations may occur.

Brief generalized discharges of slow waves intermixed with small spikes may occur either alone (4%) or, more often, with focal spikes (15%).

The EEG spikes may be stimulus sensitive; occipital paroxysms are commonly (47%) activated by the elimination of central vision and fixation whereas centrogyral spikes may be elicited by somatosensory stimuli. Occipital photosensitivity is an exceptional finding.

Functional spikes in whatever location are accentuated by sleep. If a routine EEG is normal, a sleep EEG should be performed. There is no particular relationship between the likehood of an abnormal EEG and the interval since the last seizure. EEGs short or long after a seizure are equally likely to manifest with functional spikes that may occur only once in serial routine and sleep EEGs.

The background EEG is usually normal, but diffuse or localized slow wave abnormality may also occur in at least one EEG of 20% of patients, especially postictally. EEG abnormalities, particularly functional spikes, may persist after clinical remission for many years until mid-teens. Conversely, spikes may appear only once in one of a series of EEGs.

Frequency, location, and persistence of functional spikes do not determine clinical manifestations, duration, severity, and frequency of seizures or prognosis.

Ictal EEG. Ictal EEG reports are rare because of the infrequency of seizures, likewise the scarcity of ictal recordings of Rolandic seizures. The ictal discharge in Panayiotopoulos syndrome is characterized by rhythmic monomorphic decelerating theta or delta activity, which is markedly different than the episodic fast activity of visual seizures of the Gastaut-type late onset idiopathic childhood occipital epilepsy (Beaumanoir 1993; Vigevano and Ricci 1993; Oguni et al 1999; Vigevano et al 2000; Demirbilek and Dervent 2004, Panayiotopoulos 2004; 2005a). The onset of the ictal discharge is usually from the posterior brain regions, but frontal onset has also been documented (Oguni et al 1999; Panayiotopoulos 2004; 2005a). In the documented case, the patient was a child with interictal posterior and frontal spikes that were synchronous or independent (Oguni et al 1999). While asleep, “he suddenly got up with both eyes open, vomited several times, and then showed a prolonged atonic state with cyanosis and irregular respiration for 3 minutes.” The first EEG change consisted of periodic slow waves from the left frontal region for 3 seconds, followed by rhythmic generalized ictal discharge. Koutroumanidis and colleagues cited by Panayiotopoulos (Panayiotopoulos 2004; 2005) video-EEG documented autonomic status epilepticus of more than 1 hour in a child with Panayiotopoulos syndrome and interictal bifrontal high amplitude spikes (Koutroumanidis et al in press).|{diagram:CPEO10.bmp}{caption:Video-EEG of autonomic status epilepticus in a child with Panayiotopoulos syndrome}{label:Top: High amplitude spikes-slow waves are recorded from the bifrontal regions prior to the onset of the electrical discharge, which is also purely bifrontal. Bottom: First clinical symptoms with 3 to 4 coughs and marked tachycardia appeared 13 minutes from the onset of the electrical discharge, when this had become diffuse.}| The ictal discharge had clear onset from the frontal regions, and the first clinical symptoms of coughing appeared 15 minutes later when the discharge involved the whole brain. Subsequent clinical symptoms were tachycardia, ictus emeticus (without vomiting), and impairment of consciousness. No other ictal manifestations occurred.

Magnetoencephalography (MEG). Thirteen patients with adequate clinical and EEG follow-up documenting Panayiotopoulos syndrome were recently investigated with advanced MEG technology combined with MRI (Kanazawa et al 2005). Equivalent current dipoles clustered preferentially in cortical locations along the parietal-occipital, the calcarine or the Rolandic sulci. Equivalent current dipoles along the parietal-occipital sulcus were more often superior (affecting entirely the parietal cortex) than inferior. The equivalent current dipoles clustering was unilateral or bilateral, monofocal or multifocal. Despite the high prevalence of frontal spikes in surface EEG, no frontal equivalent current dipoles were detected with MEG.

Prognosis and complications
Panayiotopoulos syndrome is a remarkably benign condition despite high incidence of autonomic status epilepticus. One third (27%) have a single seizure only. Another half (47%) has 2 to 5 seizures. Only 5% have more than 10 seizures, and these may be frequent but outcome is again favorable (Oguni 2001). Seizures usually remit within 1 to 2 years from onset. The risk of developing epilepsy in adult life is probably no more than of the general population. However, one fifth (21%) may develop other types of infrequent, usually Rolandic (13%) seizures during childhood and early teens (Panayiotopoulos 2002). These are also age-related and remit before the age of 16 years. Atypical evolutions with absences and drop attacks like those occurring in Rolandic epilepsy are exceptional (Caraballo et al 2001; Ferrie et al 2002).

Management
Current practice parameter guidelines for febrile seizures, if appropriately modified, may be the basis for similar guidelines in Panayiotopoulos syndrome. Based on the risks and benefits of the effective therapies, continuous anticonvulsant therapy is not recommended for children with only 1 seizure or brief seizures. Most clinicians for recurrent seizures use carbamazepine. No evidence exists of superiority amongst monotherapy with phenobarbitone, carbamazepine, sodium valproate, or no treatment at all (Ferrie et al 1997).

Lengthy seizures are a medical emergency; rectal diazepam is prescribed for home administration.

Pregnancy
Not applicable.

Anesthesia
Not applicable.

References Cited
Beaumanoir A. Semiology of occipital seizures in infants and children. In: Andermann F, Beaumanoir A, Mira L, Roger J, Tassinari CA, editors. Occipital seizures and epilepsies in children. London: John Libbey and Company Ltd, 1993:71-86.**

Berg AT, Panayiotopoulos CP. Diversity in epilepsy and a newly recognized benign childhood syndrome. Neurology 2000;55:1073-4.

Caraballo RH, Astorino F, Cersosimo R, Soprano AM, Fejerman N. Atypical evolution in childhood epilepsy with occipital paroxysms (Panayiotopoulos type). Epileptic Disord 2001;3:157-62.

Caraballo RH, Cersosimo RO, Medina CS, Fejerman N. Panayiotopoulos-type benign childhood occipital epilepsy. A prospective study. Neurology 2000;55:1096-100.**

Covanis A, Lada C, Skiadas K. Children with Rolandic spikes and ictal vomiting: Rolandic epilepsy or Panayiotopoulos syndrome? Epileptic Disord 2003;5(3):139-43.

Demirbilek V, Dervent A. Panayiotopoulos syndrome: video-EEG illustration of a typical seizure. Epileptic.Disord 2004,6:121-4.

Ferrie CD, Beaumanoir A, Guerrini R, et al. Early-onset benign occipital seizure susceptibility syndrome. Epilepsia 1997:38:285-93.**

Ferrie CD, Grunewald RA. Panayiotopoulos syndrome: a common and benign childhood epilepsy [Commentary]. Lancet 2001;357:821-3.

Ferrie CD, Koutroumanidis M, Rowlinson S, Sanders S, Panayiotopoulos CP. Atypical evolution of Panayiotopoulos syndrome: a case report. Epileptic Disord 2002;4:35-42.

Gastaut H. Benign spike-wave occipital epilepsy in children. Rev Electroencephalogr Neurophysiol Clin 1982;12:179-201.

Guerrini R, Dravet C, Genton P, et al. Idiopathic photosensitive occipital lobe epilepsy. Epilepsia 1995;36:883-91.

Kanazawa O, Tohyama J, Akasaka N, Kamimura T. A magnetoencephalographic study of patients with Panayiotopoulos syndrome. Epilepsia 2005;46(7):1106-13.

Koutroumanidis M. Panayiotopoulos syndrome: a common benign but underdiagnosed and unexplored early childhood seizure syndrome [Editorial]. BMJ 2002;324:1228-9.

Koutroumanidis M, Ferrie CD, Rowlinson S, Sanders S. Video-EEG documentation of autonomic status epilepticus with frontal onset in Panayiotopoulos syndrome. In press.

Lada C, Skiadas K, Theodorou V, Covanis A. A study of 43 patients with Panayiotopoulos syndrome: a common and benign childhood seizure susceptibility. Epilepsia 2003;44:81-88.**

Martinovic Z. Panayiotopoulos syndrome or early-onset benign childhood occipital epilepsy. Epilepsia 2002;43:1268-72.

Oguni H. Panayiotopoulos syndrome. Lancet 2001;358:69.

Oguni H, Hayashi K, Imai K, Muto K, Osawa M. Study on the early-onset variant of benign childhood epilepsy with occipital paroxysms otherwise described as early-onset benign occipital seizure susceptibility syndrome. Epilepsia 1999;40:1020-30.**

Ohtsu M, Oguni H, Hayashi K, Funatsuka M, Imai K, Osawa M. EEG in children with early-onset benign occipital seizure susceptibility syndrome: Panayiotopoulos syndrome. Epilepsia 2003;44:435-42.**

Panayiotopoulos CP. Autonomic seizures and autonomic status epilepticus peculiar to childhood: diagnosis and management. Epilepsy Behav. 2004, 5: 286-295.

Panayiotopoulos CP. Benign childhood epilepsy with occipital paroxysms: a 15-year prospective study. Ann Neurol 1989a;26:51-6.**

Panayiotopoulos CP. Benign childhood focal seizures and related epileptic syndromes. In: Panayiotopoulos CP, editor. The Epilepsies: Seizures, Syndromes and Management. Oxford: Bladon Medical Publishing, 2005: 223-69.

Panayiotopoulos CP. Benign childhood partial epilepsies: benign childhood seizure susceptibility syndromes [editorial]. J Neurol Neurosurg Psychiatry 1993;56:2-5.

Panayiotopoulos CP. Benign childhood partial seizures and related epileptic syndromes. London: John Libbey and Company Ltd, 1999a.**

Panayiotopoulos CP. Benign nocturnal childhood occipital epilepsy: a new syndrome with nocturnal seizures, tonic deviation of the eyes, and vomiting. J Child Neurol 1989b;4:43-9.

Panayiotopoulos CP. Difficulties in differentiating migraine and epilepsy based on clinical and EEG findings. In: Andermann F, Lugaresi E, editors. Migraine and epilepsy. Boston: Butterworth, 1987:47-81.**

Panayiotopoulos CP. Extra-occipital benign childhood seizures with ictal vomiting and excellent prognosis. J Neurol Neurosurg Psychiatry 1999b;66:82-5.

Panayiotopoulos CP. Inhibitory effect of central vision on occipital lobe seizures. Neurology 1981;31:330-3.**

Panayiotopoulos CP. Panayiotopoulos syndrome: a common and benign childhood epileptic syndrome. London: John Libbey and Company, 2002.

Panayiotopoulos CP. Vomiting as an ictal manifestation of epileptic seizures and syndromes. J Neurol Neurosurg Psychiatry 1988;51:1448-51.**

Sanders S, Rowlinson S, Manidakis I, Ferrie CD, Koutroumanidis M. The contribution of the EEG technologists in the diagnosis of Panayiotopoulos syndrome (susceptibility to early onset benign childhood autonomic seizures). Seizure 2004, 13: 565-73.

Verrotti A, Salladini C, Trotta D, di Corcia G, Chiarelli F. Ictal cardiorespiratory arrest in Panayiotopoulos syndrome. Neurology 2005;64(10):1816-7.

Vigevano F, Lispi ML, Ricci S. Early onset benign occipital susceptibility syndrome: video-EEG documentation of an illustrative case. Clin Neurophysiol 2000;111(Suppl 2):S81-6.

Vigevano F, Ricci S. Benign occipital epilepsy of childhood with prolonged seizures and autonomic symptoms. In: Andermann F, Beaumanoir A, Mira L, Roger J, Tassinari CA, editors. Occipital seizures and epilepsies in children. London: John Libbey and Company Ltd, 1993:133-40.

Yoshinaga H, Koutroumanidis M, Shirasawa A, Kikumoto K, Ohtsuka Y, Oka E. Dipole analysis in panayiotopoulos syndrome. Brain Dev 2005,27:46-52.

ILAE.
ILAE Copyright Notice

Abbreviations
CT:computer tomography
EEG:Electroencephalography
MRI:magnetic resonance imaging

Synonyms
Benign childhood partial seizures with ictal vomiting and extraoccipital spikes
Benign nocturnal childhood occipital epilepsy
Early onset benign childhood epilepsy with occipital paroxysms
Early onset benign childhood occipital seizures
Early onset benign childhood seizure susceptibility syndrome with occipital and extraoccipital spikes
Early onset benign childhood susceptibility to autonomic seizures and autonomic status epilepticus
Early onset benign occipital seizure susceptibility syndrome
Early onset childhood epilepsy with occipital paroxysms (Panayiotopoulos type)
Panayiotopoulos syndrome
Panayiotopoulos type benign childhood occipital epilepsy

Associated disorders
Benign childhood seizure susceptibility syndrome
Late onset idiopathic childhood occipital epilepsy
Rolandic epilepsy
Rolandic seizures

Major keyword descriptors
autonomic seizures
autonomic status epilepticus
diurnal occipital seizures
eye deviation
fixation-off sensitivity
head deviation
hemiconvulsions
ictal autonomic symptoms
ictal behavioral abnormalities
ictal vomiting
ictus emeticus
ictal syncope
nocturnal occipital seizures
occipital epilepsy
occipital paroxysm
occipital seizures
occipital spikes
partial seizures
status epilepticus
tonic-clonic seizures
visual hallucinations

Minor keyword descriptors
childhood
convulsions
headache
seizures
sleep
spike

Age of presentation
02-05 years
06-12 years

Age of typical presentation
02-05 years
06-12 years

Population group(s) preferentially affected
none selectively affected

Occupation group(s) preferentially affected
none selectively affected

Sex
male=female

Family history
family history may be obtained

Heredity
heredity may be a factor

Glossary
early onset benign childhood seizures (Panayiotopoulos syndrome):a benign, idiopathic, localization-related syndrome of infrequent and often prolonged autonomic, mainly nocturnal seizures with a peak onset at 5 years often with multifocal EEG spikes.

Illustration captions
Diagram:CPEO1.bmp
caption:EEG of occipital paroxysms in 4 children with different types of epileptic seizures label:Left top and bottom: EEG of 2 girls with Panayiotopoulos syndrome. Right top: EEG of a boy with frequent, brief visual seizures of elementary visual hallucinations and, occasionally, blindness. Right bottom: EEG a 15-year-old boy with symptomatic occipital lobe epilepsy. In routine EEG, high amplitude, continuous occipital sharp, and slow wave complexes (occipital paroxysms) occurred immediately after closing of the eyes, lasting as long as the eyes were closed. The EEG normalized immediately after opening the eyes and continued as long as the eyes were open, though some break in occipital spikes occurred. The activation of the occipital paroxysms was due to the elimination of central vision and fixation (left of the vertical bar, symbol of eyes with glasses). Occipital spikes were inhibited by fixation (right of the vertical bar, symbol of eyes without glasses). (Used with permission, Panayiotopoulos 1981)

diagram:CPEO2.bmp
caption:EEG of 3 children with extraoccipital spikes only or brief generalized discharges
label:Left: EEG for this child had centrotemporal spikes and giant somatosensory evoked spikes (arrows indicate tapping of the left fingers). Middle: EEG of another child had scarce and brief generalized discharges of small spikes and slow waves. Right: EEG of this child had only midline spikes. (Used with permission, Panayiotopoulos 2002)

diagram:CPEO3.bmp
caption:EEG showing occipital spikes of child aged 5 and 8 years
label:Left: one left occipital and one probable midline spike were recorded at age 5 years. Right: central, frontal, and midline spikes were recorded in sleep EEG at age 8 years.

diagram:CPEO4.bmp
caption:EEG showing occipital spikes of a child at age 9 years
label:(Left) Abundant midline spikes only were recorded in his first EEG. (Right) One year later, spikes were localized mainly in the right centrotemporal regions; some scattered right sided occipital spikes were also seen.

diagram:CPEO5.bmp
caption:EEG showing occipital spikes of a child at age 5 years
label:Random occipital spikes mainly on the left and an independent right central spike are recorded in her EEG 2 days after her first prolonged seizure.

diagram:CPEO6.bmp
caption:Video-EEG samples of Panayiotopoulos-type seizures in child aged 5 and 7 years
label:This child had 4 Panayiotopoulos type seizures from ages 5 to 7 years. EEG showed occipital and other spikes with brief generalized discharges that were asymptomatic. His first 2 seizures were diagnosed as gastroenteritis.

diagram:CPEO7.bmp
caption:Prevalence of Panayiotopoulos syndrome
label:The chart shows prevalence of Panayiotopoulos syndrome in relation to Rolandic and Gastaut type childhood occipital epilepsy based on 4 independent studies of 606 children. The overall prevalence of Panayiotopoulos syndrome is probably much higher if cases with atypical clinical presentations are included. Also, prevalence of Panayiotopoulos syndrome with extraoccipital spikes is much higher because in 2 of these studies occipital spikes was a required inclusion criterion.

diagram:CPEO8.bmp
caption:Prevalence of spike localization in Panayiotopoulos syndrome
label:Diagram shows prevalence of spike localization as estimated from the EEG analysis of 47 patients with Panayiotopoulos syndrome. All but the anterior temporal regions are involved. Note that frontal spikes are second more frequent after the occipital, followed by central spikes. Right and left occipital and frontal regions are equally involved. Midline spikes occurred in 17%. (Used with permission Panayiotopoulos 2002)

diagram:CPEO9.bmp
caption:Cloned-like repetitive multifocal spike-wave complexes in 2 children with Panayiotopoulos syndrome
label:Consider carefully the EEG of these 2 cases. Cloned-like repetitive multifocal spike-wave complexes practically constitute electrical status epilepticus. However, these 2 children were otherwise normal; their academic performance was excellent and certainly above average, and they only had a few autonomic seizures or autonomic status epilepticus. (Used with permission Panayiotopoulos 2002)

diagram:CPEO10.bmp
caption:Video-EEG of autonomic status epilepticus in a child with Panayiotopoulos syndrome
label:Top: High amplitude spikes-slow waves are recorded from the bifrontal regions prior to the onset of the electrical discharge, which is also purely bifrontal. Bottom: First clinical symptoms with 3 to 4 coughs and marked tachycardia appeared 13 minutes from the onset of the electrical discharge, when this had become diffuse. (Used with permission, Koutroumanidis et al in press)

Permuted topics
Early onset benign childhood occipital epilepsy (Panayiotopoulos syndrome)
Early onset benign childhood occipital epilepsy
benign childhood occipital epilepsy, Early onset
childhood occipital epilepsy, Early onset benign
Panayiotopoulos syndrome
Early onset benign childhood seizures with occipital spikes (Panayiotopoulos syndrome)
childhood partial seizures with ictal vomiting and extraoccipital spikes, Benign
partial seizures with ictal vomiting and extraoccipital spikes, Benign childhood
ictal vomiting and extraoccipital spikes, Benign childhood partial seizures with
nocturnal childhood occipital epilepsy, Benign
childhood epilepsy with occipital paroxysms, Early onset benign
childhood occipital seizures, Early onset benign
childhood seizure susceptibility syndrome with occipital and extraoccipital spikes, Early onset benign
benign occipital seizure susceptibility syndrome, Early onset
occipital seizure susceptibility syndrome, Early onset benign
childhood epilepsy with occipital paroxysms (Panayiotopoulos type), Early onset
benign childhood occipital epilepsy, Panayiotopoulos type of

Related summaries
Benign childhood epilepsy with centrotemporal spikes
Idiopathic photosensitive occipital lobe epilepsy
Juvenile absence epilepsy
Juvenile myoclonic epilepsy
Late onset childhood occipital epilepsy (Gastaut type)
Myoclonic-astatic epilepsy of childhood

Differential diagnosis
encephalitis
acute cerebral insults
atypical migraine
symptomatic epilepsy with ictal vomiting
gastroenteritis
dehydration
Gastaut type of childhood occipital epilepsy
photosensitive occipital seizures
syncope