Clinical commentary
Epileptic Disord 2009; 11 (3): 270-6

Panayiotopoulos syndrome
with coincidental brain lesions
A. Destina Yalçın, Hülya Ertaşoğlu Toydemir, Lale
Gündoğdu Çelebi, Hulki Forta
Copyright © 2019 John Libbey Eurotext. Téléchargé par KAROLINSKA INSTITUTET KIB le 12/02/2019.

Neurology Clinic, Şişli Etfal Education and Research Hospital, Şişli-Istanbul, Turkey
Received January 11, 2009; Accepted July 16, 2009

ABSTRACT – Purpose. To describe the clinical and electroencephalographic
features of three patients diagnosed with Panayiotopoulos syndrome with different lesions identified by cranial MRI investigation. Methods. Our study was
based on patients from the Epilepsy Outpatient Clinic of Şişli Etfal Education
Hospital in Istanbul, where a prospective study of Panayiotopoulos syndrome
was initiated in 1995. Records of our Epilepsy Outpatient Clinic revealed 53
patients with Panayiotopoulos syndrome. Among them were three with cranial
lesions identified by MRI. Results. The onset of the seizures in our patients ranged between five and eight years. The seizures included mainly autonomic
symptoms such as nausea, vomiting, pallor, mydriasis, urinary and fecal incontinence, and rarely hypersalivation. Autonomic partial status was detected in
one patient. The personal history of our patients revealed head trauma in two
and difficult birth history in one patient. Two patients described simple febrile
seizures. All patients had occipital spike or spike-wave complexes in their
EEGs. The background activity was normal. From the cranial MRI, one patient
had a neuroepithelial cyst, the second patient had a right occipital encephalomalasic lesion and the third patient had an arachnoid cyst located in the cisterna
magna associated with colpocephaly. Seizure frequency was low in general. All
patients except for one received carbamazepine treatment 450-600 mg/day.
None of our patients had seizures under antiepileptic treatment. Conclusions.
Children with Panayiotopoulos syndrome may have static MRI brain findings
which are likely to be coincidental and do not affect prognosis.
Key words: autonomic seizures, occipital epilepsy, Panayiotopoulos syndrome,
symptomatic epilepsy, cranial magnetic resonance imaging

270

lepsy syndrome after Rolandic epilepsy. In this study, we describe the
clinical and electroencephalographic
features of three patients diagnosed
with PS with different lesions detected
by cranial MRI investigation.

Methods
Our study was based on patients from
the Epilepsy Outpatient Clinic of Şişli
Etfal Education Hospital (Istanbul,
Turkey) where a prospective study of

Epileptic Disord Vol. 11, No. 3, September 2009

doi: 10.1684/epd.2009.0267

Correspondence:
Dr A.D. Yalçın
Selçuklar Sokak,
Özlem Apt,
51/14, 34335 Akatlar,
Istanbul, Turkey
<destinayalcin@yahoo.com>

An expert consensus has defined
Panayiotopoulos syndrome (PS) as a
benign age-related focal seizure disorder occurring in early and midchildhood, characterized by seizures
that are often prolonged, with predominantly autonomic symptoms,
and by an EEG that shows shifting
and/or multiple foci, often with occipital predominance (Ferrie et al., 2006).
PS is remarkably benign in terms of
seizure frequency and evaluation
(Covanis, 2006). It is the second
most common benign childhood epi-

Copyright © 2019 John Libbey Eurotext. Téléchargé par KAROLINSKA INSTITUTET KIB le 12/02/2019.

Panayiotopoulos syndrome with coincidental brain lesions

PS was initiated in 1995. Records of our Epilepsy
Outpatient Clinic revealed 53 patients with PS, among
them were three with cranial lesions identified by MRI
who were included in the study.
The personal and family history, neurological state, age of
onset, seizure type, duration and circadian distribution,
EEGs, neuroimages and school performance were all
reviewed. The parents and close relatives were asked
whether they had experienced seizures during their childhood or not. The ictal symptomatology was assessed from
the patients’ own descriptions and their first-degree relatives. Seizures were classified according to the
Commission of the International League Against Epilepsy
(Commission on Classification and Terminology of the
International League Against Epilepsy, 1989). All EEGs
were performed in paperless EEG machines (Medelec
DG compact 2000). Electrodes were placed according
to the 10-20 International System. All records included
three-minute long hyperventilation and intermittent photic stimulation in a selected stimulation range (0.5-30 Hz).
Ictal recordings were not performed because of the difficulty in provoking seizures under laboratory conditions.
Follow-up period for the three patients included regular
visits every three months and many EEG recordings ranging between every four to six years.

Results
Patient 1
A five-year-old boy, had a simple febrile seizure at the age
of two and a mild head trauma at the age of three. His
parents described three episodes in one year, each lasting
30 to 45 minutes, when the boy was five years old. They

always started with stomach ache and nausea which
caused the child to wake up, followed by pallor, mydriasis, vomiting and incontinence of urine and faeces.
Afterwards he became flaccid and unresponsive and
went to sleep. These episodes were interpretated as autonomic partial status. The cranial MRI revealed a neuroepithelial cyst located in the left frontal lobe (figure 1). The
EEG recorded just immediately after an episode showed
high amplitude spike-wave complexes on both occipital
regions prominent on the left side (figure 2). Background
activity was normal. The patient was followed without
treatment for four and a half years, during which time
the described episodes did not repeat. The subsequent
EEGs in the follow-up period showed a shift of the abnormality located within the same regions. No difficulties
were ever experienced at school. The patient is now
nine years old.
Patient 2
A seven-year-old girl, was born after a prolonged and difficult delivery. She was not cyanotic at birth. She had a
complicated head trauma at the age of three years. When
she was seven years old, she had three seizures in six
months; of which two were related to awakening. They
occurred 30 minutes after awakening, started with a feeling of nausea and dizziness and were combined with pallor and mydriasis. As the patient ran towards the toilet, she
fell down flaccidly. During the last seizure, the patient
woke up in the middle of the night, complained of feeling
unwell and was nauseous. She was pale, her pupils were
markedly dilated and she vomited repeatedly. Walking
towards the toilet, she again fell down flaccidly and
slept for several hours. Based on cranial MRI, a right
occipital encephalomalasic lesion was detected
(figure 3). Her first EEG showed spikes on the right occipital region with a normal background activity which was
prominent during sleep (figure 4). On further EEG recordings we also detected spikes on the left occipital region
appearing mostly independent from the right side.
Antiepileptic treatment of 600 mg/day of carbamazepine
was started and no seizures were observed in the followup period of six years. The treatment was terminated last
year when she was twelve years old and the patient is still
seizure free. Her school performance was always good.
Patient 3

Figure 1. A small neuroepithelial cyst located in the left frontal lobe
(Patient 1).

Epileptic Disord Vol. 11, No. 3, September 2009

She suffered from a simple febrile seizure at the age of
two. At the age of eight years she had three nearly identical diurnal seizures, each lasting 5-10 minutes. Seizures
began with a feeling of nausea, followed by pallor, mydriasis, vomiting and hypersalivation. At the end of the seizure, the child became flaccid and unresponsive. Her cranial MRI showed an arachnoid cyst located in the cisterna
magna associated with colpocephaly (figures 5, 6). Her

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A.D. Yalçın, et al.

Figure 2. The interictal EEG recording shows high amplitude spike-wave complexes on both occipital regions more prominent on the left side
(Patient 1).

repeated EEGs revealed occipital spike-wave complexes
appearing almost always simultaneously on both sides
(figure 7). Carbamazepine at a dose of 450 mg/day was
administered after the third seizure and she had no other
seizures in the follow-up period lasting four years. Her
performance at school was above average.

Figure 3. Right occipital encephalomalasic lesion (Patient 2).

272

Discussion
Panayiotopoulos syndrome was first described as “benign
nocturnal childhood occipital epilepsy” in 1989
(Panayiotopoulos, 1989a, 1989b) and was cited in the
new classification proposed by the ILAE in the group of
idiopathic focal epilepsies of childhood together with
benign Rolandic Epilepsy and occipital epilepsy,
described by Gastaut (Engel, 2006). It can be best
described as a childhood-related idiopathic susceptibility
to partial seizures, mainly with autonomic ictal symptoms
and electroencephalographically with occipital and extraoccipital spikes (Panayiotopoulos, 2005; Covanis, 2006;
Ferrie et al., 2006; Koutroumanidis, 2007).
Age at onset varies between one and 12 years, but the first
seizure generally presents at five years (Panayiotopoulos,
1988, 1999; Oguni et al., 1999; Caraballo et al., 2000). In
our patients the seizures started between five and eight
years in concordance with other studies.
In PS the seizures generally occur during sleep (Yalçın
et al., 1997; Panayiotopoulos, 1999, 2005; Caraballo
et al., 2007). In a typical seizure, the child is fully conscious, able to speak, wakes up with a feeling of sickness
or nausea, looks pale and starts vomiting. Vomiting is the
definite ictal symptom of PS, occurring in 70% of the
cases (Kivity and Lerman, 1992; Beaumanoir, 1993;
Dalla Bernardina et al., 1993; Vigevano and Ricci,
1993; Maher et al., 1995; Fejerman, 1996; Ferrie et al.,
1997; Panayiotopoulos, 2005). Two of our three patients
woke up with initial symptoms of the seizure and in the

Epileptic Disord Vol. 11, No. 3, September 2009

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Panayiotopoulos syndrome with coincidental brain lesions

Figure 4. EEG recording during spontanous sleep showing spikes on the right occipital region (Patient 2).

second patient two seizures occurred within 30 minutes
after awakening. Vomiting was seen in all of our cases.
Other ictal autonomic symptoms include mydriasis and
less often miosis, cardiorespiratory and thermoregulatory
alternations, urinary and fecal incontinence, coughing

and hypersalivation (Kivity and Lerman, 1992;
Beaumanoir, 1993; Dalla Bernardina et al., 1993;
Vigevano and Ricci, 1993; Maher et al., 1995;
Fejermann, 1996; Ferrie et al., 1997; Yalçın et al., 1997;
Kivity et al., 2000; Caraballo et al., 2007;

Figure 6. Arachnoid cyst located in the cisterna manga (Patient 3).
Figure 5. Enlargement of the occipital horns of the lateral ventricules
(Patient 3).

Epileptic Disord Vol. 11, No. 3, September 2009

273

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A.D. Yalçın, et al.

Figure 7. The interictal EEG recording illustrates occipital spikes, synchronously appearing on both sides (Patient 3).

Koutroumanidis, 2007). For PS, the whole seizure may be
entirely autonomic, but commonly, other more conventional symptoms occur in the progress of the seizures.
These mainly include deviation of the eyes and
impairment of consciousness; visual hallucinations and
illusions may occur, but these are not present at onset.
Seizures of idiopathic childhood occipital epilepsy of
Gastaut are exclusively occipital and onset predominantly
consists
of
elementary
visual
hallucination
(Panayiotopoulos, 2005; Covanis, 2008). None of our
patients described a visual ictal symptom.
Another ictal symptom specific to PS is “ictal syncope”.
This is defined as loss of postural tonus and consciousness
and is detected in 20% of patients. During this episode,
the child becomes unresponsive, appears pale with blue
lips and falls down (Panayiotopoulos, 1992, 2005;
Caraballo et al., 2007; Koutroumanidis, 2007). In our
group, ictal syncope was described in all patients.
An unusual and crucial feature of PS is partial status epilepticus reported in 44% of affected children
(Panayiotopoulos, 2002, 2005; Koutroumanidis et al.,
2005). In our group it was diagnosed only in one patient.
For PS, seizures infrequently recur and end after one or
two years whether they are treated or not, as for
Rolandic epilepsy (Panayiotopoulos, 1988, 1989a,
1989b, 1992; Kivity and Lerman, 1992; Vigevano and
Ricci, 1993; Maher et al., 1995; Fejermann, 1996; Ferrie
et al., 1997). Frequency of the seizures or the presence of
partial status epilepticus do not affect this progression.

274

The principle of a follow-up without treatment for
Rolandic epilepsy is also valid for these cases (Yalçın
et al., 1997; Panayiotopoulos, 1999; Covanis, 2006;
Koutroumanidis, 2007). An expert consensus statement
concluded that a prophylactic treatment with antiepileptic
drugs was probably best reserved for children whose seizures were unusually frequent, distressing, or otherwise
significantly interfering with the child’s life (Ferrie et al.,
2006). In our group, seizure frequency was also low. Our
first patient was followed without treatment. In two of our
cases, under low dose carbamazepine treatment, seizures
did not recur. For the second patient the treatment was
discontinued and she remained seizure-free.
The EEG, the most useful diagnostic test for PS, shows
functional spikes in many brain lesions predominantly in
occipital and centro-temporal location (Oguni et al.,
1999; Covanis et al., 2003; Lada et al., 2003; Ohtsu
et al., 2003). As for Rolandic epilepsy, the spikes in PS
tend to shift from one side to another. The EEGs of our
cases displayed normal background activity with highvoltage spikes and spike-waves over the occipital area.
Spikes located in regions other than the occipital area
were not detected.
Autonomic ictal signs and symptoms typical for PS
can also be observed in symptomatic cases.
A comprehensive study evaluating autonomic symptoms
and signs during partial epileptic seizures in 100 children
with symptomatic focal epilepsies provides crucial differences between symptomatic and idiopathic epilepsies in

Epileptic Disord Vol. 11, No. 3, September 2009

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Panayiotopoulos syndrome with coincidental brain lesions

childhood (Fogarasi et al., 2006). Regarding the most
important ictal manifestations, in the symptomatic group,
typical psychomotor or motor seizures predominate. In
this group, vomiting, the cardinal ictal symptom of PS,
usually occurs alone and seldomly with other autonomic
ictal symptoms. Vomiting also appears in the late
sequence of the seizure and frequently in the postictal
period. In the idiopathic group, including mainly PS, in
contrast, the autonomic signs or symptoms can be the
sole ictal manifestation and vomiting occurs early in the
seizure sequence.
Autonomic status epilepticus is described in 44% of
affected children with PS but not detected in the symptomatic group. Ictal syncope, a highly specific ictal symptom of PS, was described as atonic seizure only in four
children in the symptomatic group. The described autonomic ictal symptoms and signs in our patients were in
concordance with the idiopathic group.
Most authors consider cognitive, neurological and anatomical findings as the basis for the diagnosis of idiopathic
forms of epilepsy, especially for benign childhood focal
epilepsies, however, because of their high prevalence
they may incidentally occur in children with neurocognitive deficits or abnormal brain scans (Panayiotopoulos
et al., 2008). Since the appearance of modern neuroimaging procedures, abnormal findings have been reported in
patients with benign Rolandic epilepsy (Gelisse et al.,
1999, 2003; Lundberg et al., 1999). In our cases, the
MRI documented a left frontal neuroepithelial cyst, a
right occipital encephalomalasic lesion and an arachnoid
cyst located in the cisterna manga associated with colpocephaly. We believe that the MRI lesions are unrelated to
the seizures. The first patient’s left frontal neuroepithelial
cyst could be involved in epileptogenesis but the presence of exclusively occipital spikes in the repeated EEGs
is strong evidence against this possibility. The seizure
symptomatology of our second patient, who had a right
occipital encephalomalasic lesion, was not found to be
in concordance with seizures originating from the occipital lobe. These usually start with visual auras, commonly
including elementary visual hallucinations or, rarely, ictal
blindness. Contralateral head and eye deviation, blinking
and nystagmoid eye movements are described, especially
when the seizures progress (Panayiotopoulos, 2002).
None of these ictal symptoms were observed or described
in our patient. Despite discontinuation of antiepileptic
treatment she remained seizure-free, which could be
interpreted as strong evidence for the benignity of her seizures. Because of its nature and topography, the arachnoid cyst is unlikely to be epileptogenic. Most of the studies investigating the relationship between arachnoid cysts
and epilepsy concluded that there is no association
between them and arachnoid cysts can be incidentally
discovered by conducting an MRI of epileptic patients,
as in the case of our last patient (Yalçın et al., 2002;
Betting et al., 2006; Ozisik et al., 2008).

Epileptic Disord Vol. 11, No. 3, September 2009

We believe that our patients show the typical electroclinical traits of PS, despite cerebral lesions. The three
lesions reported here do not influence prognosis, thus
supporting the idiopathic nature and the benign course
of our patients.

□

Disclosures.
None.

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