Implementation

Local health communities should review their existing practice for epilepsy. The review should consider the resources required to implement the recommendations set out in the guideline, the people and processes involved, and the timeline over which full implementation is envisaged. It is in the interests of adults with epilepsy that the implementation timeline is as rapid as possible. Relevant local clinical guidelines, care pathways and protocols should be reviewed in the light of the guidance and revised accordingly. The guideline should be used in conjunction with the National Service Frameworks for long-term neurological conditions.

It also gives details of the grading scheme for the evidence and recommendations, the Guideline Development Group and the Guideline Review Panel, and technical detail on the criteria for audit. A quick reference guide for the diagnosis and management of the epilepsies in children and young people is available from the website (www.nice.org.uk/CG020childrenquickrefguide) or from the NHS Response Line (see below for ordering information). Full guideline The full guideline includes the evidence on which the recommendations are based, in addition to the information in the NICE guideline. It is published by the National Collaborating Centre for Primary Care. It is available from www.rcgp.org.uk, the NICE website (www.nice.org.uk/CG020) and the website of the National Electronic Library for Health (www.nelh.nhs.uk). Information for the public NICE has produced a version of this guidance for people with epilepsy. The information is available, in English and Welsh, from the NICE website (www.nice.org.uk/CG020). Printed versions are also available see below for ordering information. Related guidance National Institute for Clinical Excellence (2004) Newer drugs for epilepsy in adults. NICE Technology Appraisal Guidance no. 76. London: National Institute for Clinical Excellence. Available from: www.nice.org.uk/TA076 Review date The process of reviewing the evidence is expected to begin 4 years after the date of issue of this guideline. Reviewing may begin earlier than 4 years if significant evidence that affects the guideline recommendations is identified sooner. The updated guideline will be available within 2 years of the start of the review process.

Further information
Distribution This quick reference guide to the Institutes guideline on the diagnosis and management of the epilepsies contains the key priorities for implementation, summaries of the guidance, and notes on implementation. The distribution list for this quick reference guide is available from www.nice.org.uk/CG020adultsdistributionlist

NICE guideline
The NICE guideline, The epilepsies: the diagnosis and management of the epilepsies in adults and children in primary and secondary care, is available from the NICE website (www.nice.org.uk/CG020NICEguideline). The NICE guideline contains the following sections: Key priorities for implementation; 1 Guidance; 2 Notes on the scope of the guidance; 3 Implementation in the NHS; 4 Research recommendations; 5 Full guideline; 6 Related NICE guidance; 7 Review date.

Ordering information Copies of this quick reference guide can be obtained from the NICE website at www.nice.org.uk/CG020adultsquickrefguide) or from the NHS Response Line by telephoning 0870 1555 455 and quoting reference number N0739. Information for the public is also available from the NICE website or from the NHS Response Line (quote reference number N0741 for a version in English and N0742 for a version in English and Welsh). The quick reference guide for the diagnosis and management of the epilepsies in children is available from the NICE website (www.nice.org.uk/CG020childrenquickrefguide) or from the NHS Response Line (quote reference number N0740).

National Institute for Clinical Excellence

MidCity Place 71 High Holborn London WC1V 6NA www.nice.org.uk

N0739 1P 80k Oct 04 (OAK)

Issue date: October 2004

Quick reference guide

The epilepsies: diagnosis and management of the epilepsies in adults in primary and secondary care

TS ADU L

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D U LT S A A

DU
LT S
AD

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Clinical Guideline 20
October 2004 Developed by the National Collaborating Centre for Primary Care

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D U LT S A A

Contents

Contents
Information about this guide Key priorities for implementation Grading of the recommendations Outline care algorithm Diagnosis, investigation and classification Treatment and care Referral to tertiary care Regular structured review Prolonged or repeated seizures Information for adults with epilepsy and their family and/or carers Women with epilepsy Special groups Implementation Further information Ordering information 2 2 2 3 4 6 14 14 15 16 17 18 Back cover Back cover Back cover

Abbreviations used in this guide AED CT EEG anti-epileptic drug computed tomography electroencephalogram ESN FBC GDG epilepsy specialist nurse full blood count Guideline Development Group MRI SUDEP magnetic resonance imaging sudden unexpected death in epilepsy

This guidance is written in the following context: This guidance represents the view of the Institute, which was arrived at after careful consideration of the evidence available. Health professionals are expected to take it fully into account when exercising their clinical judgement. The guidance does not, however, override the individual responsibility of health professionals to make decisions appropriate to the circumstances of the individual patient, in consultation with the patient and/or guardian or carer.

National Institute for Clinical Excellence

MidCity Place 71 High Holborn London WC1V 6NA Website: www.nice.org.uk National Institute for Clinical Excellence, October 2004. All rights reserved. This material may be freely reproduced for educational and notfor-profit purposes within the NHS. No reproduction by or for commercial organisations is allowed without the express written permission of the National Institute for Clinical Excellence. Published by the National Institute for Clinical Excellence October 2004 ISBN: 1-84257-806-5 Printed by Oaktree Press Ltd, London

NICE Guideline: quick reference guide epilepsies in adults

This quick reference guide summarises the recommendations in the NICE guideline for the care of adults (people aged 18 or oldera) with epilepsy. The NICE guideline (www.nice.org.uk/CG020NICEguideline) also contains recommendations for the care of children and young people with epilepsy, which are summarised in a separate quick reference guide (see www.nice.org.uk/CG020childrenquickrefguide).
a It is recognised that there is a variable age range (1519 years) at which care is transferred between child and adult

health services by local healthcare trusts and primary care organisations.

Key priorities for implementation

The following recommendations have been identified as key priorities for implementation. Diagnosis All adults with a recent-onset suspected seizure should be seen urgentlya by a specialistb. This is to ensure precise and early diagnosis and initiation of therapy as appropriate to their needs. The seizure type(s) and epilepsy syndrome, aetiology and co-morbidity should be determined. Management Healthcare professionals should adopt a consulting style that enables the adult with epilepsy, and their family and/or carers as appropriate, to participate as partners in all decisions about their healthcare, and take fully into account their race, culture and any specific needs. All adults with epilepsy should have a comprehensive care plan that is agreed between the individuals, their family and/or carers as appropriate, and primary and secondary care providers. The AED (anti-epileptic drug) treatment strategy should be individualised according to the seizure type, epilepsy syndrome, co-medication and co-morbidity, the individuals lifestyle, and the preferences of the individual, and their family and/or carers as appropriate. Review and referral All individuals with epilepsy should have a regular structured review. In adults, this review should be carried out at least yearly by either a generalist or specialist, depending on how well the epilepsy is controlled and/or the presence of specific lifestyle issues. At the review, individuals should have access to: written and visual information; counselling services; information about voluntary organisations; epilepsy specialist nurses; timely and appropriate investigations; referral to tertiary services, including surgery as appropriate. If seizures are not controlled and/or there is diagnostic uncertainty or treatment failure, individuals should be referred to tertiary services soonc for further assessment. Special considerations for women of childbearing potential Women with epilepsy and their partners, as appropriate, must be given accurate information and counselling about contraception, conception, pregnancy, caring for children, breastfeeding and menopause.
a The Guideline Development Group considered that urgently meant within 2 weeks. b For adults, a specialist is defined throughout as a medical practitioner with training and expertise in the epilepsies. c The Guideline Development Group considered that soon meant being seen within 4 weeks.

Grading of the recommendations

The recommendations on pages 4 to 18 are evidence-based. The grading system used is shown below. Further information on the grading of the recommendations and the evidence used to develop the guideline is presented in the full guideline (see the back cover for details). A B C D N Directly based on category I evidence (meta-analysis of randomised controlled trials [RCTs] or at least one RCT) Directly based on category II evidence (at least one controlled study without randomisation or at least one other quasi-experimental study) or extrapolated from category I evidence Directly based on category III evidence (non-experimental descriptive studies) or extrapolated from category I or II evidence Directly based on category IV evidence (expert committee reports or opinions and/or clinical experience of respected authorities) or extrapolated from category I, II or III evidence Recommendation taken from NICE guideline or technology appraisal guidance

GPP Good practice point based on the clinical experience of the Guideline Development Group See the NICE guideline for further information (www.nice.org.uk/CG020NICEguideline).

NICE Guideline: quick reference guide epilepsies in adults

Key priorities for implementation

Information about this guide

Outline care algorithm

Outline care algorithm

Suspected seizure

Primary care

A&E (protocols in place for assessment) Initial screening by physician

Information obtained about the event GPP C Physical examination

Diagnostic doubt

Suspected epileptic seizure

Treatment with AEDs only in exceptional circumstances: see page 6

Referral to epilepsy specialist or other specialist (e.g. cardiologist)

Referral to specialist as soon as possible N (The GDG recommended within 2 weeks) Appropriate information provided at all stages: see page 16

Diagnosis by specialist C with investigations as necessary: see pages 45

Uncertain

Epilepsy

Non-epileptic attack disorder

Referral to psychological or psychiatric services

Further investigation, including assessment of other physical GPP causes (e.g. cardiac) (see page 5) or Referral to tertiary care: GPP see page 14

Investigation and classification by seizure type and epilepsy syndrome by specialist: see pages 45

Treatment: see pages 613 Women with epilepsy: see page 17 Special groups People with learning disabilities Black and ethnic minority groups Older people See page 18 Prolonged or repeated seizures Status epilepticus See page 15

Referral to tertiary care: see page 14

Regular structured review for all: see page 14 KEY: As necessary

NICE Guideline: quick reference guide epilepsies in adults

Diagnosis should be made by a specialist in the epilepsies C Detailed history of the attack: B from the person who had the attack + symptoms from eye-witness(es) to the attack Prospective recording (video and written) can be useful GPP Do not base the diagnosis on presence or absence of single features B Supporting investigations (EEG, neuroimaging) see below Give the person with epilepsy and their family and/or carers as appropriate an opportunity to discuss the diagnosis with an appropriate healthcare professional GPP See Appendix F of the NICE guideline (www.nice.org.uk/CG020) for an algorithm of differential diagnosis.

Investigations
EEG Use EEG: to support a diagnosis of epilepsy in adults in whom the clinical history suggests it C to help determine seizure type and epilepsy syndrome C to assess the risk of seizure recurrence after a first unprovoked seizure. B Do not use EEG: C to exclude a diagnosis of epilepsy in the case of probable syncope (risk of false-positive result) C in isolation to diagnose epilepsy. C Use standard EEG: GPP with photic stimulation and hyperventilation, with informed consent. If diagnosis or classification is still unclear, use: C long-term video or ambulatory EEG sleep EEG C repeated standard EEG (do not use in preference to sleep or sleep-deprived EEG). C EEG should be performed soona after it is requested. GPP
a The GDG considered that soon meant within 4 weeks.

Neuroimaging
Use neuroimaging (MRI/CT) to identify structural abnormalities that cause certain epilepsies. C Do not routinely request neuroimaging when a diagnosis of idiopathic generalised epilepsy has been made. C MRI MRI is the imaging investigation of choice for people with epilepsy. The use of MRI is particularly important for people: who have developed epilepsy as adults who have any suggestion of a focal onset from history, examination or EEG in whom seizures continue in spite of first-line medication. C MRI should be performed soona after it is requested. GPP
a The GDG considered that soon meant within 4 weeks.

NICE Guideline: quick reference guide epilepsies in adults

Diagnosis, investigation and classification

Diagnosis, investigation and classification

Diagnosis, investigation and classification continued

5

CT CT is an alternative to MRI: C if MRI is contraindicated or unavailable C in an acute situation, to determine whether a seizure has been caused by an acute neurological lesion or illness. GPP

Other tests and assessments

Consider blood tests (e.g. plasma electrolytes, glucose, calcium) to identify potential causes or significant comorbidity. GPP Perform a 12-lead ECG. GPP Refer to a cardiologist in cases of diagnostic uncertainty. GPP Refer for neuropsychological assessment when: MRI has identified abnormalities in areas associated with cognitive function the person with epilepsy is having educational or occupational difficulties the person with epilepsy complains of memory or other cognitive deficits and/or cognitive decline. D Classification Determine: seizure type(s), epilepsy syndrome, aetiology and co-morbidity. C Classify epileptic seizures and epilepsy syndromes: use a multi-axial diagnostic scheme (axes description of seizure; seizure type; syndrome and aetiology). D Provide the person with epilepsy with appropriate information about investigations, diagnosis and prognosis (see page 15) Refer to tertiary care as necessary (see page 14)

NICE Guideline: quick reference guide epilepsies in adults

Empowering people to manage their condition

Adults with epilepsy and their families and/or carers should be empowered to manage their condition as well as possible. GPP Adults should receive appropriate information and education about all aspects of epilepsy (see page 16). This may be best achieved and maintained through structured self-management plans. A If individuals wish to manage their condition more effectively, highlight the Expert Patients Programme (www.expertpatients.nhs.uk). GPP

Overall care
Provide an accessible point of contact with the specialist services. GPP Enable adults with epilepsy, and their family and/or carers as appropriate, to participate as partners in all decisions about their healthcare. D Take fully into account the race, culture and any specific needs (including the need for appropriate interpreters) of the person with epilepsy and of their family and/or carers as appropriate. D Establish a comprehensive care plan that: is agreed between the individual, family and/or carers (where appropriate) and primary and secondary care providers includes medical and lifestyle issues. GPP Epilepsy specialist nurses should be an integral part of the network of care of individuals with epilepsy. Their key roles are to support both epilepsy specialists and generalists, ensure access to community and multiagency services, and provide information, training and support to the individual, families and carers. D

Anti-epileptic drugs (AEDs)

Specialist supervision of AED treatment An epilepsy specialist should: recommend initiation of appropriate treatment plan continuation of treatment manage, or provide guidance for, withdrawal GPP Starting treatment The decision to start AED treatment should be made after full discussion of the risks and benefits, taking account of the persons epilepsy syndrome, prognosis and lifestyle. GPP The decision should be made between the person with epilepsy, their family and/or carers (as appropriate) and an epilepsy specialist. After a full discussion of the risks and benefits, some adults with epilepsy may choose not to take AED therapy. GPP AED therapy should only be started once the diagnosis of epilepsy is confirmed, except in exceptional circumstances that require discussion and agreement between the prescriber, the specialist and the individual and their family and/or carers as appropriate. GPP Treatment with AED therapy is generally recommended after a second epileptic seizure. A Consider AED treatment after a first unprovoked seizure if: the individual has a neurological deficit the EEG shows unequivocal epileptic activity the individual and/or their family and/or carers consider the risk of having a further seizure unacceptable brain imaging shows a structural abnormality. B

NICE Guideline: quick reference guide epilepsies in adults

Treatment and care

Treatment and care

Treatment and care continued

Choice of AED Choice of drug Factors to consider when tailoring treatment strategy to the individual Seizure type Epilepsy syndrome Co-medication Co-morbidity Lifestyle Preferences of the individual (and their family and/or carers, as appropriate)

See pages 812 for further details A Monotherapy and combination therapy Use monotherapy whenever possible. N If the first treatment is unsuccessful, try monotherapy with another drug. GPP Consider combination therapy if seizures continue after attempts with monotherapy. N If an AED has failed because of adverse effects or continued seizures, start the second drug (alternative firstline or second-line) and build up to an adequate or maximum-tolerated dose and only then taper off the first drug slowly. GPP If the second drug is unhelpful, taper either the first or second drug (depending on relative efficacy, side effects and tolerability) before starting another drug. GPP If trials of combination therapy do not bring about worthwhile benefits, revert to the regimen (monotherapy or combination therapy) that has provided the best balance between tolerability and reducing seizure frequency. N Use of the newer AEDs Newer AEDsa are recommended: for adults who have not benefited from treatment with the older AEDs (e.g. carbamazepine or sodium valproate) when the older AEDs are unsuitable because: of contraindications of potential interactions with other drugs (notably oral contraceptives) they have been poorly tolerated by the person with epilepsy the person is a woman of childbearing potential. N Continuing treatment Continuing AED therapy should be planned by a specialist; if management is straightforward, continuing AED therapy can be prescribed in primary care if local circumstances and/or licensing allow. GPP Continuing prescribing should: be part of the individuals agreed treatment plan (include details of how specific drug choices were made, drug dosage, possible side effects, and action to take if seizures persist) GPP take account of the needs of the person with epilepsy and their family and/or carers (as appropriate). GPP The formulation or brand of AED should not be changed (variations in bioavailability or different pharmacokinetic profiles may increase the potential for reduced effect or excessive side effects). D Carry out regular blood test monitoring only if clinically indicated. C Indications for monitoring AED blood levels: detection of non-adherence to the prescribed treatment suspected toxicity adjustment of phenytoin dose management of pharmacokinetic interactions specific clinical conditions (e.g. status epilepticus, organ failure or pregnancyb). D Carry out other blood tests as necessary, for example: clotting studies before surgery for adults taking valproate full blood count, electrolytes, liver enzymes, vitamin D levels, and other tests of bone metabolism every 25 years for adults taking enzyme-inducing drugs. GPP Asymptomatic minor abnormalities in blood test results are not necessarily an indication for changes in medication. GPP
a Gabapentin, lamotrigine, levetiracetam, oxcarbazepine, tiagabine, topiramate and vigabatrin, within their licensed indications. b Routine monitoring during pregnancy is not recommended; monitoring for dose adjustment may be needed if seizures increase or are likely to increase. D

NICE Guideline: quick reference guide epilepsies in adults

Withdrawing an AED Withdraw gradually (over 23 months or longer); be aware of possible seizure recurrence. D Longer for benzodiazepines (6 months or longer); be aware of drug-related withdrawal symptoms and/or seizure recurrence. GPP Withdraw one drug at a time. D Agree with the person with epilepsy and their family and/or carers a failsafe plan of action if seizures recur (last dose reduction reversed, medical help sought). GPP

Other interventions
Psychological interventions Consider use as adjunctive therapy not as an alternative to pharmacological treatment where either the individual or the specialist considers seizure control to be inadequate with optimal AED therapy. A Vagus nerve stimulation (VNS) Use VNS as an adjunctive therapy to reduce the frequency of seizures in adults who are refractory to AED therapy but who are not suitable for resective surgery including adults whose epileptic disorder is dominated by partial seizures (with or without secondary generalisation) or generalised seizures. A

Complex or refractory epilepsy refer to tertiary care (see page 14) Regular structured review (see page 14) Provide adults with epilepsy with appropriate information about care and treatment (see page 16)

The tables that follow provide a summary reference guide to pharmacological treatment. They were prepared from data available in July 2004. Prescribers should refer to the British National Formulary and Summary of Product Characteristics for full and up-to-date details of licensing (also see Table 3). The tables should be used alongside the technology appraisal guidance published on the use of newer AEDs in adults with epilepsy (available from the NICE website at www.nice.org.uk/TA076). All drugs are listed in alphabetical order.

NICE Guideline: quick reference guide epilepsies in adults

Treatment and care continued

Withdrawing treatment Discuss continuing or withdrawing AED treatment with adults who have been seizure free for at least 2 years. (Appendix H of the full guideline has tables for the prognosis of remission of seizures see www.nice.org.uk/CG020fullguideline) A The decision to withdraw medication should be taken by the individual, their family and/or carers (as appropriate), and the specialist after a full discussion of the risks and benefits of withdrawal. The discussion should include the persons risk of seizure recurrence on and off treatment and take account of his/her epilepsy syndrome, prognosis and lifestyle. A Withdrawal of AEDs must be managed by, or be under the guidance of, the specialist. GPP

Treatment and care continued

Table 1 Drug options by seizure type Other drugs that may be considered Acetazolamide Clonazepam Phenobarbitala Phenytoina Primidonea,c Drugs to be avoided (may worsen seizures) Tiagabine Vigabatrin

Seizure type Generalised tonicclonic

First-line drugs Carbamazepinea Lamotrigineb Sodium valproate Topiramatea,b

Second-line drugs Clobazam Levetiracetam Oxcarbazepinea

Absence

Ethosuximide Lamotrigineb Sodium valproate

Clobazam Clonazepam Topiramatea

Carbamazepinea Gabapentin Oxcarbazepinea Tiagabine Vigabatrin Carbamazepinea Gabapentin Oxcarbazepinea Tiagabine Vigabatrin Carbamazepinea Oxcarbazepinea

Myoclonic

Sodium valproate

Clobazam Clonazepam Lamotrigine Levetiracetam Piracetam Topiramatea Clobazam Clonazepam Levetiracetam Topiramatea Clobazam Clonazepam Levetiracetam Topiramatea Clobazam Gabapentin Levetiracetam Phenytoina Tiagabine Acetazolamide Phenobarbitala Phenytoina Primidonea,c Acetazolamide Phenobarbitala Primidonea,c

Tonic

Lamotrigineb Sodium valproate

Atonic

Lamotrigineb Sodium valproate

Carbamazepinea Oxcarbazepinea Phenytoina

Focal with/without secondary generalisation

Carbamazepinea Lamotrigineb Oxcarbazepinea,b Sodium valproate Topiramatea,b

Acetazolamide Clonazepam Phenobarbitala Primidonea,c

a Hepatic enzyme-inducing AED. b Should be used as a first choice under circumstances outlined in the NICE technology appraisal of newer AEDs for adults see page 7. c Should rarely be initiated if a barbiturate is required, phenobarbital is preferred. Table 3 summarises licensing status in July 2004. For current details on licensing, see the Summary of Product Characteristics for each drug and/or the British National Formulary.

Table 2 Drug options by epilepsy syndrome Epilepsy syndrome Childhood absence epilepsy First-line drugs Ethosuximide Lamotrigineb Sodium valproate Second-line drugs Levetiracetam Topiramatea Other drugs Drugs to be avoided (may worsen seizures) Carbamazepinea Oxcarbazepinea Phenytoin Tiagabine Vigabatrin Carbamazepinea Oxcarbazepinea Phenytoina Tiagabine Vigabatrin

Juvenile absence epilepsy

Lamotrigineb Sodium valproate

Levetiracetam Topiramatea

NICE Guideline: quick reference guide epilepsies in adults

Table 2 Drug options by epilepsy syndrome continued Epilepsy syndrome Juvenile myoclonic epilepsy First-line drugs Lamotrigineb Sodium valproate Second-line drugs Clobazam Clonazepam Levetiracetam Topiramatea Levetiracetam Other drugs Acetazolamide Drugs to be avoided (may worsen seizures) Carbamazepinea Oxcarbazepinea Phenytoina Tiagabine Vigabatrin Tiagabine Vigabatrin

Generalised tonicclonic seizures only

Carbamazepinea Lamotrigineb Sodium valproate Topiramatea,b

Acetazolamide Clobazam Clonazepam Oxcarbazepinea Phenobarbitala Phenytoina Primidonea,c Acetazolamide Clonazepam Phenobarbitala Primidonea,c Nitrazepam

Focal epilepsies: cryptogenic, symptomatic

Carbamazepinea Lamotrigineb Oxcarbazepinea,b Sodium valproate Topiramatea,b Steroidsd Vigabatrinb

Clobazam Gabapentin Levetiracetam Phenytoina Tiagabine Clobazam Clonazepam Sodium valproate Topiramatea Levetiracetam Topiramatea

Infantile spasms

Carbamazepinea Oxcarbazepinea

Benign epilepsy with centrotemporal spikes Benign epilepsy with occipital paroxysms Severe myoclonic epilepsy of infancy

Carbamazepinea Lamotrigineb Oxcarbazepinea,b Sodium valproate Carbamazepinea Lamotrigineb Oxcarbazepinea,b Sodium valproate Clobazam Clonazepam Sodium valproate Topiramatea,b Clobazam Clonazepam Ethosuximide Lamotrigineb Sodium valproate Steroidsd Lamotrigineb Sodium valproate Topiramatea,b Lamotrigineb Sodium valproate Steroidsd Clobazam Clonazepam Sodium valproate Topiramatea,b

Sulthiamee

Levetiracetam Topiramatea

Levetiracetam Stiripentole

Phenobarbitala

Carbamazepinea Lamotrigine Oxcarbazepinea Vigabatrin Carbamazepinea Oxcarbazepinea Vigabatrin

Continuous spike wave of slow sleep

Levetiracetam Topiramatea

LennoxGastaut syndrome

Clobazam Clonazepam Ethosuximide Levetiracetam Levetiracetam Topiramatea Lamotrigine Levetiracetam

Felbamatee

Carbamazepinea Oxcarbazepinea

LandauKleffner syndrome Myoclonic astatic epilepsy

Sulthiamee

Carbamazepinea Oxcarbazepinea Carbamazepinea Oxcarbazepinea

a Hepatic enzyme-inducing AED. b Should be used as a first choice under circumstances outlined in the NICE technology appraisal of newer AEDs for adults see page 7. c Should rarely be initiated if a barbiturate is required, phenobarbital is preferred. d Steroids: prednisolone or ACTH (adrenocorticotrophic hormone). e Not licensed in the UK, but available by importation. Table 3 summarises licensing status in July 2004. For current details on licensing, see the Summary of Product Characteristics for each drug and/or the British National Formulary.

NICE Guideline: quick reference guide epilepsies in adults

10

Treatment and care continued

Treatment and care continued

Table 3 Licensing of AEDsa

Drug Acetazolamide Carbamazepineb Clobazam

Details of licensing Indicated for use in conjunction with other AEDs including for tonicclonic and partial seizures. Indicated for use in generalised tonicclonic and partial seizures. Indicated for adjunctive therapy in epilepsy.

Clonazepam

Indicated for all forms of epilepsy and seizures. Especially absence seizures including atypical absence; primary or secondarily generalised tonicclonic, tonic or clonic seizures; partial seizures with elementary or complex symptomatology; various forms of myoclonic seizures, myoclonus and associated abnormal movements. Indicated primarily in absence seizures. May be used in combination with other AEDs when generalised tonicclonic seizures and other forms of epilepsy co-exist with absence seizures. No details. Indicated as add-on therapy for partial seizures and partial seizures with secondary generalisation in patients who have not achieved satisfactory control with or who are intolerant of standard anticonvulsants used alone or in combination. Indicated for simple partial seizures, complex partial seizures, secondarily generalised tonicclonic seizures, and primary generalised tonicclonic seizures. Also indicated for the treatment of seizures associated with LennoxGastaut syndrome. Indicated as adjunctive therapy in the treatment of partial-onset seizures with or without secondary generalisation in patients with epilepsy. Indicated for the treatment of partial seizures with or without secondarily generalised tonicclonic seizures. Indicated for use as monotherapy or adjunctive therapy in adults and in children of 6 years of age and above. Indicated for all forms of epilepsy, except absence seizures. Indicated for tonicclonic seizures, partial seizures, or a combination. Indicated for patients with myoclonus of cortical origin, irrespective of aetiology, and should be used in combination with other anti-myoclonic therapies. Indicated for generalised tonicclonic seizures and psychomotor epilepsy. Also can be used in partial or Jacksonian seizures, myoclonic jerks and akinetic attacks. Indicated for generalised, partial or other epilepsy. No details. Indicated as add-on therapy for partial seizures with or without secondary generalisation where control is not achieved by optimal doses of at least one other AED. Indicated for partial seizures with or without secondarily generalised seizures, seizures associated with LennoxGastaut syndrome, and primary generalised tonicclonic seizures. Treatment in combination with other AEDs for patients with resistant partial epilepsy with or without secondary generalisation; that is, where all other appropriate drug combinations have proved inadequate or have not been tolerated. Also for monotherapy in the treatment of infantile spasms.

Ethosuximide Felbamate Gabapentin

Lamotrigine

Levetiracetam Oxcarbazepineb

Phenobarbitalb Phenytoinb Piracetam Primidoneb Sodium valproate Sulthiame Tiagabine Topiramateb Vigabatrin

a Information from the Summary of Product Characteristics for each drug and/or the British National Formulary. The British National Form July 2004. Please refer to the British National Formulary and Summary of Product Characteristics for current information on these drugs b Hepatic enzyme-inducing AED.

11

NICE Guideline: quick reference guide epilepsies in adults

Age below which use is unlicensed Monotherapy Unlicensed No age limit specified Unlicensed Adjunctive treatment No age limit specified No age limit specified < 3 years but can be used in children aged 6 months to 3 years in exceptional cases No age limit specified

No age limit specified

No age limit specified

No age limit specified

Unlicensed

< 6 years

< 12 years

< 2 years

Unlicensed < 6 years

< 16 years < 6 years

No age limit specified No age limit specified Unlicensed

No age limit specified No age limit specified < 16 years

No age limit specified No age limit specified

No age limit specified No age limit specified

Unlicensed

< 12 years

< 6 years No age limit specified

< 2 years No age limit specified

mulary was accessed for the purposes of this guideline in .

NICE Guideline: quick reference guide epilepsies in adults

12

Treatment and care continued

Treatment and care continued

Table 4 Side effects of drug treatment in adults that may be clinically significant GPP
The following selected list of side effects that may be clinically significant was developed from the Summary of Product Characteristics and the British National Formulary on behalf of the GDG by Professor JS Duncan and Professor JWAS Sander of University College London. The list was developed to help the practising clinician; it should not be considered exhaustive. For full details of side effects, the prescriber should refer to the British National Formulary and the Summary of Product Characteristics for each drug. Drug Acetazolamide Carbamazepinea Significant side effects include: Some loss of appetite, depression, tingling feeling in the extremities, polyuria, thirst, headache, dizziness, fatigue, irritability, and occasional instances of drowsiness. Allergic skin reactions, including urticaria, which may be severe. Accommodation disorders, for example blurred vision, diplopia, ataxia and nausea. Particularly at the start of treatment, or if the initial dose is too high, certain types of adverse reaction occur very commonly or commonly. Drowsiness has been reported. Tolerance may develop, especially during prolonged use. Somnolence and fatigue have been observed: such effects are usually transitory and disappear spontaneously as treatment continues or with dosage reduction. With certain forms of epilepsy, an increase in the frequency of seizures during long-term treatment is possible. Mild side effects, which are usually transient, may occur initially. These include headache, nausea and drowsiness. Other adverse reactions reported include weight loss and irritability. The most common possible side effects are somnolence and dizziness. A common side effect is fatigue. Headache has also been reported. Skin rash, which generally appears within 8 weeks of starting treatment and resolves on withdrawal. Adverse experiences reported include drowsiness, diplopia, dizziness, headache, insomnia, tiredness, fever (associated with a rash as part of a hypersensitivity syndrome) and agitation, confusion and hallucinations. Most common reported undesirable effects include dizziness and somnolence. Other undesirable effects include irritability, insomnia, ataxia, tremor, headache and nausea. Very common undesirable effects include diplopia, headache and nausea. Common undesirable effects include skin rash, ataxia and confusion. Drowsiness, lethargy and mental depression. Hypersensitivity reactions including skin rash. Common undesirable effects include drowsiness, ataxia and slurred speech and these are usually dose related. Coarsening of facial features, gingival hyperplasia, and hirsutism may occur rarely. Some haemopoetic complications have been reported including some anaemias (these usually respond to folic acid). Motor twitchings, dyskinesias (rare), tremor (rare), and mental confusion have all been observed. Reported effects (incidence of between 1% and 3%) include weight increase, insomnia, somnolence, nervousness, depression and (incidence less than 1%) diarrhoea and rash. Most common side effects include drowsiness and listlessness but these generally occur only at the beginning of treatment. Other effects have been reported but are usually transient. On occasions, an idiosyncratic reaction may occur which involves these symptoms in an acute and severe form necessitating withdrawal. Sedation and tremor have been reported occasionally. Transient hair loss, which may sometimes be dose related, has often been reported. Regrowth normally begins within 6 months. Increase in weight may also occur. Severe liver damage has been very rarely reported. Encephalopathy and pancreatitis may occur rarely. Also, hyperammonaemia without change in liver function tests may occur frequently and is usually transient. Blood dyscrasias may occur frequently and the blood picture return to normal when the drug is discontinued. Sodium valproate has been associated with amenorrhoea and irregular periods. Any menstrual problems should be reported to the GP and neurologist. Sodium valproate is associated with a higher risk of fetal malformations if taken in pregnancy. Dizziness, tiredness, nervousness (non-specific), tremor, concentration difficulties and depressed mood. Headache, somnolence, dizziness, paraesthesia and weight decrease. Increased risk of nephrolithiasis. Difficulty with memory and concentration/attention has been reported. Cases of eye reactions secondary acute angle closure glaucoma presenting as painful red eye or acute myopia have rarely been associated with topiramate occurring within 1 month of starting treatment. Somnolence is very common, whilst nausea, agitation, aggression, irritability and depression are common. Psychosis has been reported as uncommon. Visual field defects have been reported in one in three people taking vigabatrin with onset usually after months to years of treatment. Any person who has concerns about this should talk to their GP and neurologist. Visual field tests should be done every 6 months in patients on vigabatrin.

Clobazam Clonazepam

Ethosuximide Gabapentin Lamotrigine

Levetiracetam Oxcarbazepinea Phenobarbitala Phenytoina

Piracetam Primidonea

Sodium valproate

Tiagabine Topiramate

Vigabatrin

a Hepatic-enzyme-inducing drugs

13

NICE Guideline: quick reference guide epilepsies in adults

Indications for referral to a tertiary epilepsy service Refer immediately GPP Behavioural or developmental regression Epilepsy syndrome cannot be identified GPP Refer soona Consider when one or more of the following are present: D AEDs do not control seizures within 2 years two AEDs have been tried unsuccessfully GPP there are, or there is a risk of, unacceptable side effects of medication GPP there is a unilateral structural lesion GPP there is psychological and/or psychiatric co-morbidity GPP there is diagnostic doubt about seizure type and/or syndrome GPP

a The GDG considered that soon meant being seen within 4 weeks Note: psychiatric co-morbidity and/or negative baseline investigations should not be a contraindication for referral to a tertiary centre GPP

Provide regular structured review: usually, by the GP or by the specialist, depending on the person with epilepsys circumstances, epilepsy or preferences D at least once a year; frequency will depend on persons epilepsy and preference. D Refer to secondary or tertiary care if: D epilepsy is inadequately controlled (in the view of the specialist or the person with epilepsy) there are specific medical or lifestyle issues (for example, pregnancy or drug cessation). D At the review Consider treatment: effectiveness tolerability side effects adherence N Discuss the treatment plan and potential lifestyle issues GPP Ensure access to: information (see page 16) counselling services epilepsy specialist nurses timely and appropriate investigations referral to tertiary care (including surgery) where appropriate D

NICE Guideline: quick reference guide epilepsies in adults

14

Regular structured review

Regular structured review

Referral to tertiary care

Referral to tertiary care

Prolonged or repeated seizures

Prolonged or repeated seizures

Prolonged or repeated seizures in the community Convulsive seizures lasting 5 minutes or longer or three or more seizures in an hour Action Secure the airway GPP

Give urgent care and treatment A

Assess respiratory and cardiac function GPP Give rectal diazepam in most cases A or buccal midazolama an alternative to rectal diazepam These drugs should be given by a trained healthcare professional, or by a trained family member or carer GPP according to the individual agreed protocol drawn up by the specialist Call emergency services if required by the situation or the response to treatment, and particularly if: seizures develop into status epilepticus there is a high risk of recurrence this is the first episode there may be difficulties monitoring the persons condition GPP

a Currently unlicensed for the treatment of prolonged or repeated seizures inform the individual and their family and/or carers as appropriate. GPP

Status epilepticus Convulsive status epilepticus Generalised tonicclonic status epilepticus in hospital: manage immediately (local protocols should be in place) GPP See the suggested treatment guidelines in Appendix C of the full guideline (available from the NICE website: www.nice.org.uk/CG020fullguidance) D If the whole protocol or intensive care is required, consult tertiary care GPP Formulate individual treatment pathway for adults who have recurrent convulsive status epilepticus GPP Non-convulsive status epilepticus Non-convulsive status epilepticus is uncommon and management is less urgent see suggested treatment guidelines in Appendix C of the full guideline (available from the NICE website: www.nice.org.uk/CG020fullguidance) GPP

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NICE Guideline: quick reference guide epilepsies in adults

 Everyone providing care or treatment should be able to provide essential information. GPP Provide information in formats, languages and ways that are suited to the individuals requirements. Consider developmental age, gender, culture and stage of life. GPP Provide information before the person makes important decisions. C Set aside adequate time in the consultation to provide information. GPP Use checklists to remind both individuals and healthcare professionals about information that should be discussed during consultations. GPP Repeat the information at later consultations. GPP Ensure the person with epilepsy and/or their family or carers know how to contact a named member of the healthcare team to get the information they need. GPP Refer the person with epilepsy and/or their family or carers to sources of high-quality information (using the Internet, if appropriate: see, for example, the website of the Joint Epilepsy Council of the UK and Ireland, www.jointepilepsycouncil.org.uk). GPP Discuss the possibility of having seizures, and provide information on epilepsy, before seizures occur for people at high risk of developing seizures (such as after severe brain injury), people with a learning disability, or people who have a strong family history of epilepsy GPP Information to provide General information about epilepsy What epilepsy is Diagnosis Reasons for tests and what the results mean Seizure type and syndrome Prognosis Sudden unexpected death in epilepsy (SUDEP see below) Psychological issues Managing risk Self care Seizures Type(s) Triggers Control Treatment options AEDs, including indications, side effects, and licence status Action to be taken after a missed dose or after a gastrointestinal upset Reasons for referral (e.g. for surgery) Lifestyle Employment Independent living Insurance issues Disclosing epilepsy at work (refer to voluntary organisations for further information) Child care Driving Alcohol Recreational drugs Sexual activity Sleep deprivation Family planning Safety First aid Safety in the home and at work Status epilepticus Road safety Support Support organisations (including contact details) Claiming benefits Support from social services Issues for women Contraception Pregnancy Breastfeeding Menopause

SUDEP There should be tailored information and discussion about the individuals relative risk of SUDEP information should be part of the counselling checklist for adults with epilepsy and their families and/or carers. C The risk of SUDEP can be minimised by optimising seizure control and being aware of the potential consequences of nocturnal seizures. GPP Where families and/or carers have been affected by SUDEP, healthcare professionals should contact them to offer their condolences, invite them to discuss the death, and offer referral to bereavement counselling and a SUDEP support group. C Healthcare professionals have a responsibility to educate others about epilepsy so as to reduce the stigma associated with it. They should provide information about epilepsy to all people who come into contact with people with epilepsy, including school staff, social care professionals and others. GPP

NICE Guideline: quick reference guide epilepsies in adults

16

Information for adults with epilepsy and their family and/or carers

Information for adults with epilepsy and their family and/or carers

Women with epilepsy

Women with epilepsy

Women (and, if appropriate, their family and/or carers or others closely involved) should be given information about contraception, conception, pregnancy and breastfeeding. Information should be given in advance of sexual activity or pregnancy. C Issues to be considered Contraception Risks and benefits of different contraceptive methods (including hormone-releasing IUDs). GPP Potential interaction with AEDs. N For women taking hepatic enzyme-inducing AEDs: the progesterone-only contraceptive pill is not recommended D the progesterone implant is not recommended D combined oral contraceptive pill: a minimum initial dose of 50 micrograms oestrogen is recommended; if breakthrough bleeding occurs, increase the oestrogen dose to 75 or 100 micrograms/day, and consider tricycling D depot injections of progesterone: inform the woman that a 10-week (instead of 12-week) repeat injection interval is recommended D discuss additional barrier methods. GPP Emergency contraception: the dose of levonorgestrel should be increased to 1.5 mg and 750 micrograms 12 hours apart. D Potential harmful effects of AEDs on the unborn child Be aware of the latest data on the risk to the unborn child associated with AED therapy and the availability of counselling. GPP Discuss the potential risk of harm to the unborn child associated with different AEDs with women of childbearing potential, and assess the risks and benefits of the individual drugs. N Offer 5 mg/day folic acid to women taking AEDs before there is any possibility of pregnancy. D Pregnancy Care of pregnant women should be shared between the obstetrician and the epilepsy specialist. GPP Encourage notification of pregnancy to the UK Epilepsy and Pregnancy Register (www.epilepsyandpregnancy.co.uk). GPP Issues to be considered and information to be provided include the following: need for folate D possible effects of epilepsy on the pregnant woman and the fetus AEDs during pregnancy N GPP breastfeeding and epilepsy, including breastfeeding while taking AEDs risk of SUDEP and status epilepticus if AEDs are discontinued C genetic counselling D need for vitamin K for the newborn child. C After the birth Discuss safety precautions in caring for the baby (see Appendix D of the full guideline: www.nice.org.uk/CG020fullguideline). C For further information about care during pregnancy, see the NICE guideline (www.nice.org.uk/CG020NICEguideline)

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NICE Guideline: quick reference guide epilepsies in adults

People with learning disabilities

People with epilepsy who have learning disabilities should receive the same support and care as the general population of people with epilepsy. They also need the care of the learning disabilities team. GPP The management and treatment of epilepsy in a person who has learning disabilities should be undertaken by a specialist, working within a multidisciplinary team. C Diagnosis and investigation Confusion may arise between seizure activity and stereotypical or other behaviours. C Obtain eye-witness accounts plus corroborative evidence (e.g. a video account), where possible: D witnesses may need education to describe observations accurately. GPP Particular care and attention may be needed to help the person tolerate investigations. GPP Facilities should be available for imaging under anaesthesia, if necessary. D Consider neuropsychological assessment in people with epilepsy in whom learning disabilities and cognitive dysfunction should be evaluated, particularly in regard to language and memory. D Management In developing the care plan, consider the possibility of adverse cognitive and behavioural effects of AED therapy. D Explore every therapeutic option. B There is a higher mortality risk for people with epilepsy and learning disabilities discuss this with the individual, their family and/or carers GPP Arrange a risk assessment, which includes: bathing and showering preparing food using electrical equipment SUDEP managing prolonged or serial seizures the impact of epilepsy in social settings independent living. C

People from black and minority ethnic groups

Consider whether the person has different cultural and communication needs, including need for an interpreter. D An interpreter should have cultural and medical knowledge; a family member is not usually suitable (because of issues of confidentiality, privacy, personal dignity and accuracy of translation). D Provide information (including information on employment rights and driving) in an appropriate format or through other means for people who do not speak or read English. D

Older people
The recommendations on the choice of treatment and importance of regular monitoring of effectiveness and tolerability are the same as for the general population. N

Young people with epilepsy

Transfer to adult care Multidisciplinary services provided jointly by adult and paediatric specialists have a key role in the care of the young person with epilepsy. They can facilitate the transition from paediatric to adult services and aid in the dissemination of information. D During adolescence a named clinician should assume responsibility for the ongoing management of the young person with epilepsy. This clinician should: ensure smooth transition of care to adult services be aware of the need for continuing multi-agency support. GPP Review diagnosis and management of epilepsy during the young persons adolescence. D Before the transition to adult services is made: review diagnosis and management facilitate access to voluntary organisations, such as support groups and epilepsy charities. D
NICE Guideline: quick reference guide epilepsies in adults 18

Special groups

Special groups