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Antiepileptic Drug Treatment of Rolandic Epilepsy and Panayiotopoulos ADC Online First, published on September 8, 2014 as 10.1136/archdischild-2013-304211 Arch Dis Child: first published as 10.1136/archdischild-2013-304211 on 8 September 2014. Downloaded from Review Antiepileptic drug treatment of rolandic epilepsy and Panayiotopoulos syndrome: clinical practice survey and clinical trial feasibility Louise C Mellish,1 Colin Dunkley,2 Colin D Ferrie,3 Deb K Pal1 ▸ Additional material is ABSTRACT published online only. To view Background The evidence base for management of What is already known on this topic? please visit the journal online (http://dx.doi.org/10.1136/ childhood epilepsy is poor, especially for the most common specific syndromes such as rolandic epilepsy (RE) archdischild-2013-304211). ▸ UK management of rolandic epilepsy and and Panayiotopoulos syndrome (PS). Considerable 1King’s College London, Panayiotopoulos syndrome are not well known international variation in management and controversy London, UK and there is limited scientific basis for drug 2 about non-treatment indicate the need for high quality Sherwood Forest Hospitals, treatment or non-treatment. Notts, UK randomised controlled trials (RCT). The aim of this study 3 ▸ Paediatric opinion towards clinical trial designs Department of Paediatric is, therefore, to describe current UK practice and explore is also unknown and important to assess prior Neurology, Leeds General the feasibility of different RCT designs for RE and PS. Infirmary, Leeds, UK to further planning. Methods We conducted an online survey of 590 UK Correspondence to paediatricians who treat epilepsy. Thirty-two questions Professor Deb K Pal, covered annual caseload, investigation and management Department of Basic and practice, factors influencing treatment, antiepileptic drug Clinical Neuroscience, King’s What this study adds? College London, Institute of preferences and hypothetical trial design preferences. Psychiatry, Psychology & Results 132 responded (22%): 81% were Neuroscience, London paediatricians and 95% at consultant seniority. We ▸ There are suggested patterns of underuse of SE5 8AF, UK; estimated, annually, 751 new RE cases and 233 PS cases. EEG and neuropsychological assessment and [email protected] Electroencephalography (EEG) is requested at least half overuse of brain MRI; 40% of patients are Received 26 February 2014 the time in approximately 70% of cases; MRI brain at routinely untreated. Revised 1 August 2014 least half the time in 40%–65% cases and ▸ Half the respondents would be open to Accepted 4 August 2014 neuropsychological evaluation in 7%–8%. Clinicians head-to-head or active versus non-active design reported non-treatment in 40%: main reasons were low or placebo-controlled clinical trials with frequency of seizures and parent/child preferences. carbamazepine and levetiracetam as the Carbamazepine is the preferred older, and levetiracetam preferred active treatments. the preferred newer, RCT arm. Approximately one-half considered active and placebo designs acceptable, choosing seizures as primary and cognitive/behavioural There are almost 40 electroclinical epilepsy syn- http://adc.bmj.com/ measures as secondary outcomes. dromes defined by constellations of seizure type(s), Conclusions Management among respondents is age of onset, electroencephalography (EEG) and broadly in line with national guidance, although with clinical features, each requiring individual assess- possible overuse of brain imaging and underuse of EEG ment and management. The evidence base for man- and neuropsychological assessments. A large proportion agement of childhood epilepsies in the UK is of patients in the UK remains untreated, and clinicians detailed in the National Institute of Health and on September 28, 2021 by guest. Protected copyright. seem amenable to a range of RCT designs, with Care Excellence (NICE) Guidelines6 and by its carbamazepine and levetiracetam the preferred active drugs. Scottish equivalent, the Scottish Intercollegiate Guidelines Network (SIGN).7 This guidance pro- vides a care standard framework against which a recent national ‘Epilepsy12’ audit of childhood epi- INTRODUCTION lepsy care was conducted.8 However, the evidence Epilepsy affects 63 400 young people under base for antiepileptic drug treatment in these guide- 18 years of age in the UK.1 Seizures represent one lines remains poor for many common childhood of the top five avoidable reasons for admission of epilepsies, consisting of only a few high-quality ran- children to emergency departments in the UK.2 domised controlled trials (RCT).9 Much of the evi- Aside from seizures, cognitive and behavioural dence available to NICE is extrapolated from comorbidities cause a substantial impact affecting heterogeneous studies of seizures mixing epilepsy about two-thirds of children with epilepsy.3 Indeed, types and age groups,10 with uncertain applicability the comorbidity-associated burden may outweigh to well-defined childhood epilepsy syndromes.11 To cite: Mellish LC, that of the seizures themselves4 and in some epilep- Furthermore, while there is now an emerging idea Dunkley C, Ferrie CD, et al. sies, comorbidities are stronger predictors of of how childhood epilepsy as a whole is managed Arch Dis Child Published 5 8 Online First: [please include quality-of-life than seizures. Hence, the compre- in the UK, there is little detail about the variation Day Month Year] hensive management of epilepsy necessitates the in management of specific epilepsy syndromes and doi:10.1136/archdischild- recognition and management of individual epilepsy paediatricians’ familiarity with them. Hence, a 2013-304211 syndromes and their specific comorbidities. more detailed survey of practice is justified. Copyright Article author (or theirMellish employer) LC, et al. Arch 2014. Dis Child Produced2014;0:1–6. doi:10.1136/archdischild-2013-304211 by BMJ Publishing Group Ltd (& RCPCH) under licence.1 Arch Dis Child: first published as 10.1136/archdischild-2013-304211 on 8 September 2014. Downloaded from Review In the absence of solid RCT evidence, there is widespread his- Table 1 NICE recommendations for management of RE/PS (rolandic epilepsy/ torical and geographical variation in recommendations for the Panayiotopoulos Syndrome) drug management of specific epilepsies. In childhood, rolandic NICE recommendations for rolandic epilepsy (BECTS)/Panayiotopoulos Syndrome epilepsy (RE, sometimes known as Benign Epilepsy with ▸ Diagnosis and investigation by paediatrician with expertise CentroTemporal Spikes or BECTS), a focal epilepsy, is the most ▸ Investigation to include EEG confirmation of electroclinical syndrome common syndrome, estimated to constitute 8%–25% of all ▸ Neuroimaging not usually indicated in BECTS 12 ▸ ‘Children, young people and adults with epilepsy should be given information childhood epilepsies and diagnosed in 9% of children with ’ 8 about their seizure type(s) and epilepsy syndrome, and the likely prognosis. epilepsy in the recent Epilepsy12 audit. RE, along with the ▸ ‘Discuss with the child or young person, and their family and/or carers, 13 closely related Panayiotopoulos syndrome (PS), are part of the whether AED treatment for BECTS spikes, Panayiotopoulos syndrome…is group of non-lesional focal epilepsies (box 1). Textbooks and indicated.’ older ‘expert opinion’ often advised conservative management ▸ ‘Be aware that carbamazepine and oxcarbazepine may exacerbate or unmask – of RE, that is, with no antiepileptic drugs (AED),14 17 but continuous spike and wave during slow sleep, which may occur in some children with RE/BECTS.’ neither the extent to which this conservative management is fol- lowed, nor its scientific rationale,14 18 19 are known. Moreover, Other AEDs considered recent experimental evidence suggests that focal EEG spikes First-line AEDs Adjunctive AEDs in tertiary care may disrupt simultaneous regional brain function20 and might 21 Carbamazepine Carbamazepine Eslicarbazepine acetate also impair long-term learning and memory consolidation in Lamotrigine Clobazam Lacosamide 22 sleep, prompting a re-evaluation of the ‘benign’ nature of this Levetiracetam Gabapentin Phenobarbital group of epilepsies. Oxcarbazepine Lamotrigine Phenytoin When treated, carbamazepine and lamotrigine are recom- Sodium valproate Levetiracetam Pregabalin Oxcarbazepine Tiagabine mended as first-line monotherapy by NICE (see table 1) and 61726 Sodium valproate Vigabatrin others, although the evidence base is acknowledged to be Topiramate Zonisamide 27–29 poor, and there are theoretical risks of carbamazepine AEDs, antiepileptic drugs; BECTS, Benign Epilepsy with CentroTemporal Spikes; NICE, exacerbating the seizures or EEG abnormality30 31 and also National Institute of Health and Care Excellence. speech production.32 International practice beyond the UK diverges widely,29 33 as does expert opinion on the subject.34 fi Box 1 Clinical Description of RE and PS Sulthiame is considered rst-line in Germany, Austria and Israel,35 36 although it may have adverse effects on cognition;37 sodium valproate in France; while levetiracetam is popular in Rolandic epilepsy the USA. Thus, the rationale for treatment versus non-treatment Rolandic epilepsy (RE), also known as Benign Epilepsy of is not established and the evidence base in favour of specific Childhood with Centro-Temporal Spikes (BECTS), is the most AEDs is unknown amid widespread national and international common epilepsy in childhood, with an incidence of up to 21 12 variation in practice. This equipoise scenario sets the stage for per 100 000 children
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