SympoSium: EpiLEpSy

Epilepsy in the teenagerRichard E Appleton

AbstractEpilepsy is the most common chronic neurological disease in adoles-

cence and at this time may change for a number of reasons, including the

natural history of the epilepsy syndrome, the underlying cause and the

effect of a changing lifestyle, and specifically one that is endeavouring to

achieve independence and self-empowerment. management begins with

making the correct diagnosis of epilepsy, the seizure type(s) and the epi-

lepsy syndrome and ensuring that an underlying cause has been consid-

ered and appropriately investigated. Holistic management is mandatory

but frequently difficult in view of the challenging issues at this time of

life. A teenager’s adherence with management (anticonvulsants, clinical

attendance and communication) is often brittle and fragile; healthcare

professionals must understand this and possess the necessary skills to

optimise their care and support their successful maturation into adult-

hood. These must include medical and communication (listening as well

as talking) skills. Finally, the young person with epilepsy should be seen

in an appropriate environment and their ongoing care should be elec-

tively planned, ideally through a specialised transitional service.

Keywords adolescence; care; children; epilepsy; hand-over; teenager;

transition

Is it epilepsy?

Paroxysmal episodes, including epileptic seizures, are common in the teenager or during adolescence, the period of life pragmati-cally defined as being 14–18 years of age. Although epilepsy is the most common neurological disorder of adolescence, other parox-ysmal events are frequently misdiagnosed as epileptic seizures, including vasovagal syncope, cardiac arrhythmias (specifically, the prolonged QT interval syndrome), psychogenic non-epileptic seizures and substance abuse. The most important point when assessing a teenager with epilepsy is to be as certain as possible that this is the correct diagnosis; a wrong diagnosis of epilepsy at this age may have significant and life-changing consequences.

How common is epilepsy?

Although the data are old, the incidence of a first epileptic sei-zure in a teenager is reported to be approximately 75 per 100,000 and the prevalence in children aged 15 years, 7–9 per 1000.

Richard E Appleton MB BS DCH MA(oxon) FRCP FRCPCH is a Consultant

Paediatric Neurologist at the Roald Dahl EEG Department, Paediatric

Neurosciences Foundation, Royal Liverpool Children’s Hospital

(Alder Hey), Liverpool, UK.

pAEDiATRiCS AND CHiLD HEALTH 19:5 23

What type of epilepsy is it (the epilepsy syndrome) and what is the cause?

Epilepsy in adolescence may already have had an onset in early childhood, may develop de novo, may remit spontaneously or may change for a number of reasons including: • the natural history of the specific epilepsy syndrome • the nature of the underlying cause of the epilepsy • the effects of an evolving independence that may result in an

erratic lifestyle and poor concordance (adherence) with both medication and clinic attendance.

It is important to understand and be aware of the different epilepsies in adolescence because of their different seizure types, causes, therapies and outcomes; details of the different epilepsy syndromes can be found elsewhere. The key points of some of the most common epilepsy syndromes are highlighted below. • Juvenile myoclonic epilepsy (JME): myoclonic seizures always occur but may not be recognised as a seizure by the teenager or their family. Their occurrence must always be asked about in any teenager who experiences a tonic-clonic seizure, which is the typical presentation of JME. • Juvenile absence epilepsy (JAE): although absences may oc-cur every day, they are never as frequent as those in childhood-onset absence epilepsy. In addition, the absences in JAE are more prolonged, often lasting for over a minute and are usually accompanied by more purposeful automatisms; speech may not be completely lost. This can result in an initial (wrong) diagnosis of complex partial seizures and the prescription of carbamazepine (CBZ), which may precipitate absence status. • Symptomatic focal (partial): characteristically, the initial seizures often occur during sleep, when the partial onset of the seizures may not have been witnessed. There should be a very low threshold for undertaking brain magnetic resonance imag-ing in any teenager aged 13 years or above who presents with nocturnal seizures, particularly if these are focal. • Progressive myoclonic epilepsies (PME): although rare, this group of epilepsies may initially present and follow an early course that can be characteristic of an idiopathic generalised (or, less likely focal), epilepsy and specifically JME. Increasing myoclonic seizures that are drug-resistant and/or the develop-ment of cognitive difficulties should raise the possible diagnosis of a PME.

How should epilepsy be treated in the teenager?

As at any age, the treatment approach must be holistic, treat-ing the person and not just the seizures, although this may be somewhat challenging. These aspects will be discussed later. Choosing the most appropriate antiepileptic drug (AED) is clearly important and should be based on the epilepsy syndrome, or sei-zure type(s) when the syndrome has not been identified and the safety profile of the drug. The final choice and decision is that of the teenager and their family, based on full and accurate infor-mation provided by the clinician. Considerable anecdotal data, largely reinforced by the results of the largest randomised clini-cal trial of AEDs (the study of Standard And New Antiepileptic Drugs (SANAD)), indicate that sodium valproate (VPA) is the most effective and best-tolerated AED in treating the idiopathic

2 © 2009 Elsevier Ltd. All rights reserved.

SympoSium: EpiLEpSy

generalised epilepsy syndromes (including JME and JAE). How-ever, there are justifiable and potentially serious concerns about the use of this drug in young women, particularly if they are sexually active, irrespective of whether they are considering pregnancy. These concerns include the reported impairment on a child’s learning and communication skills, in the absence of any spinal or facial abnormalities, whose mothers received VPA during pregnancy. Lamotrigine (LTG) may be a useful alterna-tive although it is not considered to be particularly effective in suppressing the myoclonic seizures in JME. New data have also indicated that blood LTG levels may fall during pregnancy result-ing in breakthrough tonic-clonic and focal seizures. Accumulat-ing – although still somewhat limited – data on the use of LTG taken as monotherapy during pregnancy, suggest that this drug is not teratogenic. Finally, levetiracetam (LEV) is effective for tonic-clonic, myoclonic and focal seizures and it now has a spe-cific licence to be used as adjunctive treatment for young people aged 12 years and above with JME. There are no convincing data that it is effective in treating absence seizures. In treating partial (focal) seizures, SANAD suggested that LTG was as effective but slightly better tolerated (i.e. was associated with fewer adverse side effects) than CBZ or oxcarbazepine and significantly better tolerated than topiramate.

What are the important issues for teenagers with epilepsy?

Teenagers as a group have special needs and concerns. These are characteristically sharpened by having a chronic and unpredict-able illness; consequently, teenagers can justifiably be regarded as facing a difficult and challenging time. It is obviously impor-tant that these needs and concerns are addressed as successfully as possible as they negotiate and emerge through adolescence. Medical staff must have some appreciation of what the young person already understands about epilepsy and what else they may want (and need) to understand, including if they have not yet thought about it! Consequently, clinicians must have an in-terest in and expertise about epilepsy (including around adoles-cence) and must also be aware of many issues: • lifestyle factors that may influence seizure control, including

sleep-deprivation and that seizure control may worsen at the start of, or during, menstruation (‘catamenial’ epilepsy)

• the potential interactions of AEDs with alcohol, other recrea-tional drugs and oral contraceptives. It is important to let the young person know that alcohol may be consumed but clearly in moderation

• the effect of AEDs on fertility and pregnancy, including the potential teratogenic effect of AEDs. It is good clinical prac-tice for young women who could be sexually active to take 5 mg of folate daily as this may reduce the risk of neural tube defects

• the realistic possibility of discontinuing antiepileptic medication • driving regulations (able to apply for a driving licence having

either been seizure-free for 12 months or only ever having experienced seizures when asleep over at least a 3-year peri-od). If the individual is already driving and wants to withdraw an AED then they should stop driving as the medication is being withdrawn and for 6 months after it has been discontin-ued. They can then resume driving if remaining seizure-free

pAEDiATRiCS AND CHiLD HEALTH 19:5 23

• accurate and up-to-date knowledge over employment and career choices

• the physical risks and dangers of seizures and specifically how these risks may be increased by irregular adherence with an-tiepileptic medication; where appropriate, and specifically if brought up by the teenagers themselves, this may include dis-cussion on the increased mortality associated with epilepsy

• the risks of ‘passing epilepsy on’ to their own childrenFinally, and crucially, they must be able to communicate effec-tively with the young person, which is not always easy, and which is often an inherent rather than acquired talent, although training may improve this skill. Healthcare professionals must be patient, listen (and listen again) and give facts and informa-tion rather than ‘rushing-in’ to advise or recommend. They must also inform teenagers where they can obtain further information appropriate to their needs; this may be through the internet or through visiting local resource centres (e.g. Connexions, Career Advice and Job Centres), or both. Young people are disillusioned and irritated by doctors and nurses who do not understand epilepsy or adolescence, or always talk and do not listen (or both) and this may further reinforce their feelings of frustration and isolation. Finally, healthcare professionals should be able to differentiate a moody and uncommunicative teenager from a depressed teenager. Communication with teenagers, as well as gaining some insight into how they cope with having a chronic disorder experience, is outlined in detail in a recently-launched project, instigated by the Department of Health and in close col-laboration with the Royal College of Paediatrics and Child Health (the e-learning for health Adolescent Health Project).

Where should the teenager with epilepsy be seen?

An important issue is how, and where, to continue reviewing the teenager when they reach the age of 16 or 17 years – the chrono-logical, but not always emotional and cognitive age at which they tend to be regarded as adults. This period is called the ‘transfer’ or ‘transition’ period, words which do not mean the same but are often perceived as such. Transition implies a planned and structured move from paediatric to adult care, which in practice should include appropriate preparation and discussion with the young person, before they are actually transferred to a different clinic or service for ongoing care. For obvious reasons, this tran-sitional period may be challenging for themselves as well as for their families and healthcare professionals. Ideally, the concept of transitional care should be introduced from 12 or 13 years of age, including in a specific teenager clinic within a paediatric department. Once the young person reaches 16 years of age there are then four options for their ongoing management. • To remain in paediatric care (whether in a specialist epilepsy or a general paediatric clinic). The only potential benefit of this option is to provide the young person with continuity of care. This is unsatisfactory for many reasons but include the follow-ing: first, because it is extremely unlikely that the paediatrician will have the knowledge and experience (and, therefore, con-fidence) of dealing with adult issues (sex, contraception, preg-nancy, driving and employment regulations) and because their communication skills are more likely attuned to either young children or their parents. Communicating with the teenager in their own right but still within a family unit demands a specific

3 © 2009 Elsevier Ltd. All rights reserved.

SympoSium: EpiLEpSy

approach, which many paediatricians may find uncomfortable. Second, because it would be wholly inappropriate for a 19, 20 or 21-year-old to be seen in a paediatric clinic, even if the clinic time was more tailored for the young person (e.g. in the evening). • To be discharged back to their GP. This is potentially unsatis-factory as the young person is denied continuity of specialist epi-lepsy care and advice at a critical time in their life. Although it is now a Department of Health directive that all GPs must annually audit all patients aged 16 years and above with epilepsy, in reality this might simply be a paper exercise. Consequently, these young people may remain on antiepileptic medication unnecessarily (as the epilepsy may have entered a spontaneous remission) and they may not receive appropriate or indeed any pre-conception advice and counselling (and this applies to both sexes). • To be referred to an adult physician or neurologist, neither of whom may necessarily have the knowledge or interest in man-aging epilepsy. The principle of adult practice is for follow-up appointments to be infrequent and brief and only to see patients if they have ‘problems’. Waiting for the young person to admit to ‘problems’ or express their concern may be optimistic, as well as being too late as and when any problem is disclosed. • To be transferred to a transitional service, whereby the young person’s care may be supervised in a specific clinic that operates separately from a paediatric or adult clinic and is able to pro-vide continuity of specialist epilepsy care that is also adolescent- specific. There is no precise definition of, or established criteria for a transitional service. One model that led to the inception of probably the first transitional epilepsy service in Liverpool in 1991 included the following: • joint supervision by a paediatric and adult neurologist – both of

whom have a specific interest in and knowledge of epilepsy • support from a nurse specialist(s) in epilepsy – ideally adult

but equally from both an adult and paediatric nurse specialist • access to advice from an employment adviser and psycholo-

gist (should this be necessary) and ideally on the same day as the young person’s clinic attendance

• based in the adult epilepsy clinic or department, because this will be where they receive their long-term specialist care.It is currently difficult, even impossible in some parts of the UK

to develop a transitional service because of the rigid framework and cost-effective pressures within the National Health Service. This is disappointing and surprising in view of the clear support for seamless specialist care for young people with epilepsy within a transitional format, as endorsed by the Scottish Intercollegiate Guideline Network, National Institute for Clinical Excellence and the National Service Framework (NSF). In March 2006, an NSF document entitled ‘Transition: getting it right for young people’ included the following:

‘These young people with neurological disorders and disabilities [defined as difficult or intractable epilepsy syndromes that differ from those seen in adult neurological practice] are the least well served as there is often no service available to take over their care when they leave paediatrics’.

Predictably, there are many issues that may militate against both the continuing functioning of pre-existing, and the development of new transitional clinics. These include: the per-sonalities of the staff supervising a transitional service; conflict

pAEDiATRiCS AND CHiLD HEALTH 19:5 23

between trusts (particularly foundation trusts); where a tran-sitional clinic should be based; and financial issues relating to consultant and epilepsy nurse sessions, and which hospital or primary care trust should be responsible for funding such a ser-vice. Primary care trust funding may be easier if the transitional clinic accepted referrals directly from GPs of young people who present with de novo epilepsy or whose epilepsy relapses having originally been under the care of (and discharged by) a paediatri-cian or paediatric neurologist, although such referrals are likely to represent only a minority of young people who require a tran-sitional epilepsy service. ◆

FurtHEr rEAdIng

Adab N, Kini u, Vinten J, et al. The longer term outcome of children

born to mothers with epilepsy. J Neurol Neurosurg Psychiatr 2004;

75: 1575–1583.

Appleton RE, Chadwick D, Sweeney A. managing the teenager with

epilepsy: paediatric to adult care. Seizure 1997; 6: 27–30.

Appleton RE, Neville B. Teenagers with epilepsy. Arch Dis Child 1999;

81: 76–79.

Castle GF, Fishman LS. Seizures in adolescent medicine. Pediatr Clin

North Am 1973; 20: 819–835.

Cooper JE. Epilepsy in a longitudinal study of 5000 children. BMJ 1965;

i: 1020–1022.

David TJ. Transition from the paediatric clinic to the adult service.

J R Soc Med 2001; 94: 373–374.

Department of Health, National Service Framework. improving the

transition of young people with long term conditions from

children’s to adult health services. ‘Transition: getting it right

for young people’ (best practice guideline). London: Department

of Health, march 2006. Available at: www.dh.gov.uk/en/

policyandguidance/Heathandsocialservicecaretopics/Childrenservices/

Transitionfromchildrentoadultservices/index.

Eklund pG, Sivberg B. Adolescents’ lived experiences of epilepsy.

J Neurosci Nurs 2003; 35: 40–47.

Epilepsy in older children and adolescents. in: Roger J, Bureau m,

Dravet Ch, Genton p, Tassinari CA, Wolf p, eds. Epileptic syndromes

in infancy, childhood and adolescence. 3rd edn. Eastleigh: John

Libbey & Co, 2002; p. 315–385.

macleod S, Appleton RE. Neurological disorders presenting mainly in

adolescence. Arch Dis Child 2007; 92: 170–175.

marson AG, Al Kharusi A, Alwaidh m, et al. The SANAD Study of

efficacy of carbamazepine, gabapentin, lamotrigine, oxcarbazepine

or topiramate for treatment of partial epilepsy: an unblended

randomised controlled trial. Lancet 2007; 369: 1000–1015.

marson AG, Al Kharusi A, Alwaidh m, et al. The SANAD Study of

effectiveness of valproate, lamotrigine or topiramate for generalised

and unclassifiable epilepsy: an unblended randomised controlled

trial. Lancet 2007; 369: 1016–1026.

National institute of Clinical Excellence. The diagnosis and management

of the epilepsies in adults and children in primary and secondary

care. Clinical Guideline 20. London: NiCE, october 2004. Available

at: www.nice.org.uk.

Rasalam AD, Hailey H, Williams JHG, et al. Characteristics of fetal

anticonvulsant syndrome associated autistic disorder. Dev Med

Child Neurol 2005; 47: 551–555.

Royal College of paediatrics and Child Health website. Adolescent Health

project. Available at: www.rcpch.ac.uk/AHp. (accessed oct 2008).

4 © 2009 Elsevier Ltd. All rights reserved.

SympoSium: EpiLEpSy

Scottish intercollegiate Guidelines Network (SiGN). Diagnosis and

management of epilepsies in children and young people. Guideline

81. Edinburgh: SiGN, march 2005. Available at: www.sign.ac.uk.

Smith pEm. The teenager with epilepsy. BMJ 1998; 317: 960–961.

Viner R. Transition from paediatric to adult care. Bridging the gap or

passing the buck? Arch Dis Child 1999; 81: 271–275.

Practice points

• Epilepsy is probably the most common chronic neurological

disorder of the mid to late teenage years

• Syncope, cardiac arrhythmias, psychogenic non-epileptic

seizures and substance abuse are frequently misdiagnosed as

epilepsy in adolescence

pAEDiATRiCS AND CHiLD HEALTH 19:5 23

• Seizure-control may deteriorate or improve for many reasons

during this period, including through the natural history of

the epilepsy, poor or non-concordance with antiepileptic

medication and lifestyle changes

• in teenagers presenting with de novo epilepsy, always

consider an underlying cause and investigate appropriately;

remember that brain tumours and a progressive myoclonic

epilepsy may present with epileptic seizures at this age

• Know about the specific risks and benefits of the different

AEDs that may be particularly relevant at this age and

discuss these with the teenager

• Listen to what the teenager says and, if requested, see them

alone without their parents

• Give accurate facts and information (about epilepsy, its

treatment and where to find advice), rather than giving advice

and recommendations; this must include appropriate advice

on sexual behaviour, contraception, driving and career options

5 © 2009 Elsevier Ltd. All rights reserved.