Letter to the Editor Epileptic Disord 2010; 12 (1): 91-4 Panayiotopoulos syndrome: a debate To the editor superbly illustrated by the syndrome of benign partial Yalcin et al. (2009) describe three patients with neuro- epilepsy with centro-temporal spikes, a syndrome that radiological abnormalities identifi ed on magnetic has robustly defi ed eponymous adoption for many resonance imaging (MRI), which they consider to be decades. “coincidental” and un-related to an underlying electro- clinical diagnosis of Panayiotopoulos syndrome Richard E. Appleton, Brian Tedman, (PS). While they propose that the MRI abnormalities Teresa Preston, Tam Foy demonstrated are coincidental and do not preclude The Roald Dahl EEG Department, the diagnosis of PS, there may be another explana- Paediatric Neurosciences Foundation Trust, tion. It could reasonably be argued that the radiolog- Alder Hey Children’s NHS Foundation Trust, ical abnormalities seen in their patients (particularly Liverpool L12 2AP, UK patients 2 and 3) are not coincidental and that these <[email protected]> patients have a symptomatic focal or multifocal epilepsy and not Panayiotopoulos syndrome. Response to the letter of R. Appleton, et al. Although an “expert consensus” has attempted to To the editor defi ne and classify the existence of PS (Ferrie et al., 2006), it does not necessarily follow that their opinion We welcome the comments of Appleton and his is correct. There appear to be ever-increasing reports colleagues regarding issues raised for a better under- of “atypical” cases of PS. On scientifi c grounds this standing of Panayiotopoulos syndrome (PS). must surely raise the question as to whether this does The authors argue that the 3 children we described represent a true “syndrome” as defi ned in both the had symptomatic focal or multifocal epilepsy not PS. English language and by the ILAE. This issue has been However, our fi ndings showed that the clinical and EEG recently addressed (Capovilla et al., 2009) and has raised manifestations of the 3 patients were not associated concerns over both the nosology and existence of the with a particular focus expected from their MRI abnor- syndrome, concerns shared by ourselves. Capovilla malities and prognosis was excellent with just a few and colleagues (2009) appropriately concluded that, autonomic seizures typical of PS. Similar cases have “Many theoretical and practical points, including also been described in rolandic epilepsy as we detail diagnostic, genetic, and pathophysiologic issues (still) in our report. remain unresolved for PS”. The most worrying aspect of this letter is that the authors In this EEG department, we have identifi ed only doubt even the existence of PS. Numerous indepen- three children over the past 12 months who would dent and prospective studies, including more than “fulfi ll” the electro-clinical criteria for PS; we are fully 500 children provided the evidence of this syndrome aware of the reported electro-clinical features of this (Michael et al., 2010). The ILAE commission recognizes syndrome. This is from a cohort of approximately the syndrome with the highest score of confi dence in 1,100 paediatric EEGs each year and with one of us a scale of 1-3 (Engel, 2006). The few atypical cases that (RA) directly involved with the management of over the authors refer to are those that may have more than 120 children presenting during the same year with de 10 seizures in their life but also with fi nal remission and novo epilepsy Over the same period, and using both more severe autonomic seizures that may be related to the accepted 1989 and proposed 2001 ILAE Classifi - contributing SCN1A mutations (Panayiotopoulos et al., cation, we have identifi ed 15 children with benign 2008). On the other hand, the authors contradict them- partial epilepsy with centro-temporal spikes (BECTS) selves by doubting the existence of PS as they are iden- and 11 patients with childhood-onset absence tifying PS in their practice at a prevalence of one out of epilepsy (CAE). 5 patients with rolandic epilepsy. We would suggest that, in the context of the proposed The relatively low prevalence of PS in their cohorts revised classifi cation of the epilepsies and epilepsy may be explained as follows: a signifi cant number syndromes and the absence of any genetic or of patients in their area may not be referred to them biochemical marker, great caution should be exercised because of misdiagnosis as encephalitis, migraine or before accepting and certainly over-expanding a new other non-epileptic paroxysmal disorders. Addition- and eponymous entity such as PS. It would also seem ally, their practice not to recommend an EEG after a entirely appropriate to simply describe the seizures fi rst seizure may result in missing one third of patients doi : 10.1684/epd.2010.0299 on the basis of the electro-clinical features; this is with PS manifesting with a single seizure. Epileptic Disord Vol. 12, No. 1, March 2010 91 jleepd00416_cor3.indd 91 3/11/2010 12:54:45 PM Letter to the Editor In regard to nomenclature proposed by the ILAE, like epilepsy with occipital paroxysms” of Gastaut many others, we follow the eponymic term (Panayio- (ICOE-G); multifocal spikes suggested symptomatic topoulos syndrome) rather than “early onset benign epilepsies with poor prognosis. childhood occipital epilepsy” because the latter may be misleading as one third of children with PS do The veracity of Panayiotopoulos’s initial descriptions not have occipital epileptogenicity in semiological has, over the last two decades, been confi rmed in clinical seizure onsets, ictal and interictal EEG or large and long term studies from Europe, Japan and magnetoencephalography. South America. The published database on which our knowledge of PS is now based includes over 500 cases Destina Yalçin, Hülya Ertasoglu Toydemir, (Michael et al., 2010); there are few epilepsy syndromes Lale Gündogdu Çelebi, Hulki Forta which are better characterised. What emerges are a Neurology Clinic, remarkably uniform clinical picture and a diagnosis Sisli Etfal Education and Research Hospital, which is strikingly useful in helping predict prognosis Sisli-Istanbul, Turkey and dictate management. These independent researchers have joined together to produce consensus statements on PS (Ferrie et al., 2006) and autonomic status epilepticus (Ferrie Invited editorial comment et al., 2007). PS is a model of autonomic seizures and autonomic status epilepticus specifi c to childhood (Ferrie et al., 2006, 2007; Koutroumanidis et al., 2007; Panayiotopoulos syndrome: learning Panayiotopoulos et al., 2008; Michael et al., 2010). lessons from atypical cases Affected children are otherwise normal and the EEG and magnetoencephalogram are the only tests expected to be abnormal, usually showing multifocal Panayiotopoulos with his original report of ictal epileptogenic abnormalities. Ictal EEG recordings both vomiting (Panayiotopoulos, 1988) and his related studies confi rm the clinical manifestations of autonomic status (Panayiotopoulos, 1989, 1999) opened new chapters epilepticus and that it is associated with epileptiform in paediatric epileptology. He described autonomic discharges arising from various cortical areas (Iannetti seizures and autonomic status epilepticus specifi c to et al., 2009). PS is a common childhood epilepsy, childhood. These were common and mainly occurred probably affecting one child for every 2-3 with rolandic in otherwise normal children whose prognosis in epilepsy. Moreover, it is the most common cause of terms of seizure remission and development was non-febrile, non-convulsive status epilepticus in excellent. Their EEG was characterised by spikes and childhood (Okanishi et al., 2008). Prognosis is usually sharp waves occurring in variable localisations. These excellent; around two third of children have less than children are now classifi ed as having Panayiotopoulos 5 seizures. Prophylactic antiepileptic drug medication syndrome (PS) (Ferrie et al., 2006; Koutroumanidis is often not needed. et al., 2007). However, there was initial scepticism and There are some “atypical cases” of PS. These include resistance to these fi ndings, including from infl uential children who have frequent, but otherwise typical epileptologists because: seizures of PS and whose EEGs are typical. Their ultimate – ictal vomiting had been considered as extremely prognosis, in terms of eventual seizure remission, is rare and hitherto had been mainly described in the same as that of “typical cases”. In statistical terms neurosurgical series of adult patients. In children it was when considering seizure frequency, they lie within generally not considered as having an epileptic origin; the “expected distribution” but relatively distant from the median. Other “atypical cases” include – autonomic status epilepticus was not recognised around 5 reported children who developed other as a diagnostic entity; the proposition that it might be seizure types and unexpected reversible cognitive a common occurrence in a benign seizure disorder and behavioural problems, sometimes associated challenged orthodox concepts of status epilepticus; with continuous spike- and- slow wave during sleep. – it implied that paediatricians had been failing Similar cases are well recognised and are probably to diagnose signifi cant numbers of children with more common in benign rolandic epilepsy. This, and epilepsy, instead erroneously labelling then as having other shared features between these two common diverse non-epileptic disorders such