TÀI LIỆU ĐỘNG KINH The Epilepsies

Pharmacological Update of Clinical Guideline 20

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

Final
Methods, evidence and recommendations

January 2012

Commissioned by the National Institute for
Health and Clinical Excellence


The Epilepsies
Preface

Preface
Dr Richard Roberts
Consultant Neurologist, Ninewells Hospital, Dundee
Chairman, SIGN 70 Diagnosis and management of epilepsy in adults (2003)
The inadequacies that have existed in the services, care and treatment for people with epilepsy are
well recognised. Important issues include misdiagnosis, inappropriate or inadequate treatment,
sudden unexpected death that might have been prevented, advice about pregnancy and
contraception and management of status epilepticus. Service provision for people with epilepsy has
been patchy and sometimes poor both in primary and secondary care. This is now changing. The
new General Medical Services (GMS) contract includes targets for epilepsy. The number of
specialists with expertise in epilepsy is increasing. There has been a great increase in the number of
epilepsy specialist nurses, and structured services for epilepsy across primary and secondary care are
emerging. At the same time a number of new antiepileptic drugs have been licensed.

This guideline is published, therefore, at a time when it is likely to have a major impact. The
recommendations on service provision, such as waiting times to see specialists and for investigations,
will be challenging for the service providers, as they have been in Scotland following similar
recommendations (SIGN Guideline 70). The guidance on the use of the newer antiepileptic drugs
confirms their important role in the treatment of epilepsy. Clear guidance is given in various specific
areas such as pregnancy and contraception, learning disability, young people, repeated seizures in
the community and status epilepticus. The importance of the provision of information for people
with epilepsy and their carers is stressed. If there is successful implementation of the
recommendations, there will be a great improvement in the care of people with epilepsy.
Dr Nick Kosky

Chairman, The epilepsies guideline 2012

Update 2012

Consultant Psychiatrist, Prison Mental Health Inreach Team and Medical Director, Dorset
Community Health Services

The first NICE guideline on the management of epilepsy in children and adults was published in 2004.
Published by the National Clinical Guideline Centre at
The Royal College of Physicians, 11 St Andrews Place, Regents Park, London, NW1 4BT
First published 2004
© National Clinical Guideline Centre – January 2012
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The Epilepsies
Preface

The guideline highlighted the inadequacies that existed in the services, care and treatment for
people with epilepsy, and made great progress in addressing relevant important issues misdiagnosis, inappropriate or inadequate treatment, sudden unexpected death that might have
been prevented, advice about pregnancy and contraception and management of status epilepticus.
Revisiting this guideline is timely. The NHS is facing major financial challenges, and it is vital that a
spotlight is kept on the need to further develop the still variable services for people with epilepsy.
The place of newly licensed drugs for epilepsy also needs careful consideration.

People with epilepsy remain at the centre of this guideline, and the need for services to consider the
needs of each individual, to not discriminate in provision and to work in partnership with people with
epilepsy and their carers is underlined.
Attention has been paid to ensure that the recommendations are written in clear language and are
accessible, and, we hope, useful to all. Supporting the written version is an online care pathway, and
quality standards are soon to be published. We remain committed to the care of people with
epilepsy and commend these guidelines to you in that light.

Partial Pharmacological Update of Clinical Guideline 20

Update 2012

The updated guideline reminds the reader of the need for properly resourced services, offering

appropriate levels of expertise, which allow timely access to assessment and treatment for people
with epilepsy. The primary scope of the guidelines was to consider the role of antiepileptic drugs,
especially given the impact of important, real-world studies such as SANAD. The role of established
and newly licensed drugs has been considered using novel statistical methods allowing comparison
of cost effectiveness – a process that has been much aided, as always, by a robust stakeholder
review process.


The Epilepsies
Foreword

Foreword
Dr Mayur Lakhani
Chairman-Elect, Royal College of General Practitioners until 2006
Founding Chairman of the National Collaborating Centre for Primary Care (2001-2004)
It gives me great pleasure to see the publication of the first major clinical practice guideline from the
National Collaborating Centre for Primary Care, hosted by the Royal College of General Practitioners.
As a practising GP, I am well aware of the challenges faced when dealing with patients with epilepsy.
It is well recognised that the care of patients with epilepsy is sub-optimal and more needs to be done
to improve clinical standards. GPs are faced with a complex set of issues on a regular basis including
giving advice to patients about epilepsy and driving, planning a pregnancy and the thorny issue of
withdrawal of anti- epileptic medication. In these and other areas, practical recommendations are
essential: It is therefore welcome to have this clear guidance which will support GPs to implement
the Quality and Outcomes Framework of the new General Medical Services contract. In addition the
guideline contains important recommendations about service for patients with epilepsy and the
organisation of care.
The Royal College of General Practitioners exists to promote the highest possible standards of
general medical care and it is committed to increasing support for GPs to enable them to do so. I
commend these guidelines to the health community as a whole and urge commissioners to support
its implementation. I would like to acknowledge the excellent work of the staff of National

Collaborating Centre for Primary Care and colleagues at the University of Leicester in producing this
guideline.

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Contents
Guideline development group members ......................................................................................15
Acknowledgements ....................................................................................................................20
1

Introduction ........................................................................................................................21
1.1

Definition of epilepsy .......................................................................................................... 21

1.2

Clinical aspects .................................................................................................................... 21

1.3

Epidemiology....................................................................................................................... 22

1.4

Cost of epilepsy ................................................................................................................... 23

1.5

Health Services for people with epilepsy ............................................................................ 24
1.5.1

Primary care......................................................................................................... 24

1.5.2

Secondary care .................................................................................................... 25

1.6

The SANAD trial ................................................................................................................... 25

1.7

Guideline aims..................................................................................................................... 26

1.8

Principles underlying the guideline development .............................................................. 26

1.9

Who should use this guideline? .......................................................................................... 27

1.10 Structure of guideline documentation................................................................................ 27
1.11 Guideline limitations ........................................................................................................... 28

1.12 Plans for updating the guideline ......................................................................................... 28
2

Methods ..............................................................................................................................30
2.1

Introduction ........................................................................................................................ 30

2.2

The developers .................................................................................................................... 30
2.2.1

The National Collaborating Centre for Primary Care........................................... 30

2.2.2

The National Clinical Guidelines Centre .............................................................. 30

2.2.3

The methodology team ....................................................................................... 30

2.2.4

The Guideline Development Group ..................................................................... 31

2.3

Developing key clinical questions (KCQs)............................................................................ 32

2.4

Identifying the evidence ..................................................................................................... 32

2.5

2.4.1

Literature search strategies ................................................................................. 32

2.4.2

Health economics ................................................................................................ 34

Reviewing and grading the evidence .................................................................................. 35
2.5.1

Methods for 2004 Guideline................................................................................ 35

2.5.2

Methods for 2012 Guideline................................................................................ 36

2.6

Methods of combining studies (2012) ................................................................................ 37

2.7

Protocol for guideline evidence reviews for the partial update (2012).............................. 37

Types of studies ............................................................................................................................. 37
Types of participants ..................................................................................................................... 38
Types of interventions ................................................................................................................... 39

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Duration of studies ........................................................................................................................ 39
Posology ........................................................................................................................................ 39
Types of outcome measures and definitions ................................................................................ 39
Type of analysis ............................................................................................................................. 41
Use of unpublished data in the guideline ..................................................................................... 41
2.8

Grading of quality of evidence for outcomes (2012) .......................................................... 41

Inconsistency ................................................................................................................................. 42
Indirectness ................................................................................................................................... 42
Imprecision .................................................................................................................................... 42

2.9

2.8.1

Health economics methods ................................................................................. 44

2.8.2

Literature review for health economics .............................................................. 45

Developing recommendations ............................................................................................ 46

2.10 Research Recommendations............................................................................................... 48
2.10.1

Newly diagnosed seizures (focal and generalised) – monotherapy .................... 48

2.10.2

Epilepsy syndromes ............................................................................................. 48

2.10.3

Infantile spasms ................................................................................................... 49

2.10.4

Treatment of convulsive status epilepticus (i.e. not just refractory) .................. 49

2.10.5

AEDs and pregnancy ............................................................................................ 50

2.10.6

Ketogenic diet in adults ....................................................................................... 50

2.11 Prioritisation of recommendations for implementation .................................................... 51
2.12 The relationship between the guideline and the Technology Appraisals for the newer
antiepileptic drugs (AEDs) ................................................................................................... 51
2.13 The relationship between the guideline and National Service Frameworks ...................... 52
2.14 The relationship between the guideline and the Scottish Intercollegiate Guidelines
Network guidelines on epilepsy .......................................................................................... 52
2.15 External review ................................................................................................................... 53
2.16 Level of evidence table ....................................................................................................... 53
3

Key priorities for implementation ........................................................................................55

4

Guidance .............................................................................................................................57
4.1.1

Outline epilepsy care algorithms ......................................................................... 82

5

Audit Criteria .......................................................................................................................85

6

Principle of decision making .................................................................................................86
6.1

7

Who should be involved in the decision making process for adults and children with
epilepsy? ............................................................................................................................. 86

Diagnosis .............................................................................................................................87
7.1

Introduction ........................................................................................................................ 87

7.2

Establishing the diagnosis of epilepsy................................................................................. 87

7.3

Key features of the history and examination that allow epilepsy to be differentiated
from other diagnoses in adults and children ...................................................................... 88

7.4

What are the key features of the history and examination that allow an epileptic

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seizure to be differentiated from other causes of attack disorder in adults? .................... 91

8

9

7.5

The role of attack/seizure diaries in diagnosis in adults & children ................................... 91

7.6

The role of home video recording in making the diagnosis of epilepsy in adults and
children?.............................................................................................................................. 91

Investigations ......................................................................................................................93
8.1

Introduction ........................................................................................................................ 93

8.2

The role of EEG in making a diagnosis of epilepsy .............................................................. 93
8.2.1

How good is the standard EEG at differentiating between individuals who
have had an epileptic seizure and those who have had a non-epileptic
seizure? ................................................................................................................ 93

8.2.2

How good is the EEG at differentiating between individuals who have
different epilepsy seizure types and epilepsy syndromes? ................................. 98

8.2.3

How can the diagnostic yield of the standard interictal EEG be improved? ....... 98

8.2.4

What are the roles of long-term video-EEG and ambulatory EEG?................... 102

8.2.5

What is the role of provocation techniques and induction protocols? ............. 103

8.2.6

Does an abnormal EEG predict seizure recurrence? ......................................... 105

8.3

The role of neuroimaging in the diagnosis of epilepsy ..................................................... 107

8.4

The role of prolactin levels and other blood tests as an aid to diagnosis......................... 114

8.5

Cardiovascular tests as an aid to diagnosis ....................................................................... 116

8.6

What is the role of neuropsychological assessment in the diagnosis and management
of epilepsy? ....................................................................................................................... 116

Classification of seizures and epilepsy syndromes ............................................................... 119
9.1

Introduction ...................................................................................................................... 119

9.2

Classification of the epilepsies .......................................................................................... 119

9.3

What is the role of classification in adults and children with epilepsy? ........................... 129

10 Pharmacological treatment of epilepsy .............................................................................. 130
10.1 Introduction ...................................................................................................................... 130
Pharmacological treatment of epilepsy ...................................................................................... 131
10.2 How many times should monotherapy be tried before combination therapy is
considered? ....................................................................................................................... 131
10.2.1

When should AED treatment in adults and children be started?...................... 132

10.2.2

Who should start AED treatment in adults and children?................................. 136

10.2.3

In adults and children with epilepsy on AEDs does management of
continuing drug therapy by a generalist as opposed to a specialist lead to
different clinical outcomes? .............................................................................. 136

10.2.4

What is the role of monitoring in adults and children with epilepsy? .............. 137

10.2.5

What influences AED treatment concordance in adults and children? ............ 140

10.2.6

When and how should AED treatment be discontinued in adults and
children? ............................................................................................................ 141

10.2.7

In adults/children with epilepsy on AEDs does management of drug
withdrawal by a generalist as opposed to a specialist lead to different

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outcomes? ......................................................................................................... 147
10.2.8

New recommendations and link to evidence .................................................... 148

10.3 Monotherapy for newly diagnosed Focal Seizures ........................................................... 156
10.3.1

Introduction ....................................................................................................... 156

10.3.2

Methods of the evidence review ....................................................................... 156

10.3.3

Matrix of the evidence for adults ...................................................................... 156

10.3.4

Monotherapy for adults with newly diagnosed focal seizures.......................... 159

10.3.5

Individual patient data network meta-analysis as monotherapy for focal

epilepsy .............................................................................................................. 199

10.3.6

Health economic evidence of AEDs used as monotherapy for adults with
newly diagnosed focal epilepsy ......................................................................... 200

10.3.7

Monotherapy for children with newly diagnosed focal epilepsy ...................... 206

10.3.8

Health economic evidence of AEDs used as monotherapy for children with
newly diagnosed focal epilepsy ......................................................................... 209

10.3.9

New recommendations and link to evidence .................................................... 211

10.3.10 New research recommendations (for full list see section 2.11) ........................ 221
10.4 Therapy for refractory focal seizures ................................................................................ 222
10.4.1

Introduction ....................................................................................................... 222

10.4.2

Methods of the evidence review ....................................................................... 222

10.4.3

Matrix of the evidence....................................................................................... 222

10.4.4

Single AED therapy for refractory focal seizures ............................................... 226

10.4.5

Health Economic Evidence for single AED therapy for refractory focal
seizures .............................................................................................................. 228

10.4.6

Adjunctive therapy in children, young people and adults with refractory
focal seizures ..................................................................................................... 229

10.4.7

Health economic evidence of AEDs used as adjunctive therapy for adults
with refractory focal epilepsy ............................................................................ 268

10.4.8

Health economic evidence of AEDs used as adjunctive therapy for children
with refractory focal epilepsy ............................................................................ 273

10.4.9

New recommendations and link to evidence .................................................... 276

10.4.10 Research Recommendations (for full list see section 2.11) .............................. 283
10.5 Generalised Tonic-Clonic Seizures (GTCS)......................................................................... 284
10.5.1

Introduction ....................................................................................................... 284

10.5.2

Methods of the evidence review ....................................................................... 284

10.5.3

Matrix of the evidence....................................................................................... 284

10.5.4

Monotherapy for the treatment of generalised tonic-clonic seizures in
adults ................................................................................................................. 287

10.5.5

Individual patient data network meta-analysis as monotherapy for
generalised tonic-clonic epilepsy....................................................................... 308

10.5.6

Monotherapy for the treatment of generalised tonic-clonic seizures in
children .............................................................................................................. 310

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10.5.7

Adjunctive therapy for the treatment of generalised tonic-clonic seizures...... 310

10.5.8

Health economic evidence for AEDs used as adjunctive therapy in adults
with refractory generalised tonic-clonic seizures.............................................. 315

10.5.9

New recommendations and link to evidence .................................................... 317

10.6 Absence Seizures............................................................................................................... 322
10.6.1

Introduction ....................................................................................................... 322

10.6.2

Methods of the evidence review ....................................................................... 323

10.6.3

Matrix of the evidence....................................................................................... 323

10.6.4

AEDs for the treatment of absence seizures ..................................................... 323

10.6.5

New recommendations and link to evidence .................................................... 324

10.7 Myoclonic Seizures............................................................................................................ 329
10.7.1

Introduction ....................................................................................................... 329

10.7.2

Methods of the evidence review ....................................................................... 329

10.7.3

Matrix of the evidence....................................................................................... 329

10.7.4

Monotherapy for the treatment of myoclonic seizures .................................... 330

10.7.5

Adjunctive therapy for the treatment of myoclonic seizures ........................... 331

10.7.6

New recommendations and link to evidence .................................................... 333

10.8 Tonic or atonic seizures..................................................................................................... 340
10.8.1

Introduction ....................................................................................................... 340

10.8.2

Methods of the evidence review ....................................................................... 340

10.8.3

Matrix of the evidence....................................................................................... 340

10.8.4

New recommendations and link to evidence .................................................... 341

10.9 Infantile Spasms (West syndrome) ................................................................................... 345
10.9.1

Introduction ....................................................................................................... 345

10.9.2

Methods of the evidence review ....................................................................... 345

10.9.3

Matrix of the evidence for adjunctive therapy.................................................. 345

10.9.4

New recommendations and link to evidence .................................................... 352

10.9.5

New research recommendations (for full list see section 2.11) ........................ 356

10.10 Dravet syndrome (SMEI) ................................................................................................... 357
10.10.1 Introduction ....................................................................................................... 357
10.10.2 Methods of the evidence review ....................................................................... 357
10.10.3 Matrix of the evidence....................................................................................... 357
10.10.4 Adjunctive treatment of Dravet Syndrome (SMEI)............................................ 358
10.10.5 New recommendations and link to evidence .................................................... 359
10.10.6 New research recommendations (for full list see section 2.11) ........................ 362
10.11 Lennox-Gastaut Syndrome................................................................................................ 363
10.11.1 Introduction ....................................................................................................... 363
10.11.2 Methods of the evidence review ....................................................................... 363
10.11.3 Matrix of the evidence....................................................................................... 363

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10.11.4 Adjunctive treatment for Lennox-Gastaut syndrome ....................................... 364
10.11.5 Health economic evidence of AEDs used as adjunctive therapy for children
with Lennox-Gastaut syndrome ........................................................................ 369
10.11.6 New recommendations and link to evidence .................................................... 371
10.12 Benign epilepsy with centrotemporal spikes, Panayiotopoulos syndrome and lateonset childhood occipital epilepsy (Gastaut type) ............................................................ 376
10.12.1 Introduction ....................................................................................................... 376
10.12.2 Methods of the evidence review ....................................................................... 376
10.12.3 Matrix of the evidence....................................................................................... 376
10.12.4 Monotherapy for the treatment of adults and children with BECTS,
Panayiotopoulos syndrome and late onset childhood occipital epilepsy
(Gastaut type) .................................................................................................... 377
10.12.5 New recommendations and link to evidence .................................................... 380
10.13 Idiopathic Generalised Epilepsy (IGE) ............................................................................... 397
10.13.1 Introduction ....................................................................................................... 397
10.13.2 Methods of the evidence review of IGE ............................................................ 397
10.13.3 Matrix of the evidence....................................................................................... 398
Matrix of the evidence for childhood absence epilepsy, juvenile absence epilepsy and
other absence epilepsy syndromes ................................................................... 400
10.13.4 Monotherapy for the treatment of IGE in newly diagnosed patients ............... 401
10.13.5 Adjunctive therapy in children, young people and adults with IGE .................. 405
10.13.6 Health economic evidence for AEDs used as monotherapy in the treatment
of patients with newly diagnosed IGE ............................................................... 406
10.13.7 Monotherapy for the treatment of childhood absence epilepsy, juvenile
absence epilepsy and other absence epilepsy syndromes ................................ 409
10.13.8 Adjunctive therapy for the treatment of childhood absence epilepsy,
juvenile absence epilepsy and other absence epilepsy syndromes .................. 415

10.13.9 Monotherapy for the treatment of Juvenile Myoclonic Epilepsy (JME)............ 415
10.13.10 Monotherapy/adjunctive therapy for the treatment of juvenile myoclonic
epilepsy (JME) .................................................................................................... 417
10.13.11 Adjunctive treatment for for the treatment of of Juvenile Myoclonic
Epilepsy (JME) .................................................................................................... 418
10.13.12 AEDs for the treatment of epilepsy with generalised tonic clonic seizures
only .................................................................................................................... 419
10.13.13 Introduction ....................................................................................................... 419
10.13.14 Methods of the evidence review ....................................................................... 419
10.13.15 Matrix of the evidence....................................................................................... 419
10.13.16 New recommendations and link to evidence ................................................... 419
10.14 Other epilepsy syndromes ................................................................................................ 443
10.14.1 Introduction ....................................................................................................... 443
10.14.2 New recommendations and link to evidence .................................................... 443

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10.14.3 New research recommendations (for full list see section 2.11) ........................ 444
10.15 Prolonged seizures and convulsive status epilepticus ...................................................... 444
10.15.1 Introduction ....................................................................................................... 444
10.15.2 Methods of the evidence review ....................................................................... 445
10.15.3 Matrix of the evidence....................................................................................... 445
10.15.4 AEDs for the treatment of prolonged seizures and convulsive status
epilepticus in the community ............................................................................ 448
10.15.5 Treatment of prolonged seizures and convulsive status epilepticus in

children (community) ........................................................................................ 450
10.15.6 Treatment of acute repetitive seizures (children and adults) ........................... 452
10.15.7 Treatment of convulsive status epilepticus in adults in hospitals ..................... 452
10.15.8 Treatment of convulsive status epilepticus in children ..................................... 458
10.15.9 Treatment of refractory status epilepticus........................................................ 462
10.15.10 New recommendations and link to evidence .................................................... 465
10.15.11 New research recommendations (for full list see section 2.11) ........................ 475
10.16 Non-convulsive status epilepticus .................................................................................... 476
10.16.1 Introduction ....................................................................................................... 476
10.16.2 Methods of the evidence review ....................................................................... 476
10.16.3 AEDs for the treatment of non-convulsive Status Epilepticus (observational
study) ................................................................................................................. 477
10.16.4 New recommendations and link to evidence .................................................... 477
10.16.5 Generic prescribing ............................................................................................ 477
10.17 When should an individual with epilepsy be referred for assessment in a tertiary
centre? .............................................................................................................................. 477
10.17.1 Introduction ....................................................................................................... 477
11 The role of non-drug treatments in the management of the epilepsies ................................ 481
11.1 Introduction ...................................................................................................................... 481
11.2 Does the treatment of epilepsy in adults or children with psychological methods lead
to a reduction in seizure frequency and/or a better quality of life? ................................ 481
11.3 Ketogenic Diet ................................................................................................................... 482
11.3.1

Introduction ....................................................................................................... 482

11.3.2

Methods of the evidence review ....................................................................... 482

11.3.3

Matrix of the evidence....................................................................................... 482

11.3.4

New recommendations and link to evidence .................................................... 486

11.3.5

New research recommendations (for full list see section 2.11) ........................ 488

11.3.6

Ketogenic diet in adults ..................................................................................... 488

11.4 In people with drug resistant epilepsy, is vagus nerve stimulation (VNS) effective as
an adjunctive treatment?.................................................................................................. 488
12 Information needs of individuals, families, and carers......................................................... 493
12.1 Introduction ...................................................................................................................... 493

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12.2 Information needs of the individual with epilepsy, the family, the carer, and special
groups ............................................................................................................................... 493

12.3 What information is required at different stages of the care pathway............................ 496
12.4 What is the risk of SUDEP in individuals with epilepsy ..................................................... 501
13 Women of childbearing age with epilepsy .......................................................................... 504
13.1 Introduction ...................................................................................................................... 504
13.2 What information and counselling should be given and when? ...................................... 504
13.3 What issues should be considered in women who may become pregnant or who are
breast feeding? ................................................................................................................. 507
13.4 Increased risk of seizures during pregnancy or whilst breastfeeding............................... 508
13.5 Teratogenic effects of AEDs whilst pregnant .................................................................... 511
13.5.1

Introduction ....................................................................................................... 511

13.5.2

Methods of the evidence review ....................................................................... 511

13.5.3

Comparison between specific monotherapies on developmental /cognitive
outcomes ........................................................................................................... 516

13.5.4

Any monotherapy exposure versus no exposure in general population .......... 519

13.5.5

New recommendations and link to evidence .................................................... 528

13.5.6

New research recommendations (for full list see section 2.11) ........................ 533

13.6 Do AEDs interact with contraceptives?............................................................................. 533
13.7 Does epilepsy increase the risk of complications in pregnancy? ...................................... 537
13.7.1

Are women with epilepsy at increased risk of complications during the
pregnancy and labour? ...................................................................................... 538

13.7.2

When should screening for structural fetal anomalies be performed in
pregnant women with epilepsy? ....................................................................... 538

13.8 When should folic acid be started? .................................................................................. 539
13.9 What are the dangers of seizures in women who are pregnant or post-natal? ............... 539
13.10 What is the role of drug monitoring in pregnant women with epilepsy? ........................ 541
13.11 Should oral or parenteral vitamin K be used? .................................................................. 542
13.12 What is the risk of of inheriting epilepsy? ........................................................................ 542
13.13 What is the role of joint epilepsy and obstetric clinics in the care of women with
epilepsy who are pregnant?.............................................................................................. 543
14 Children, young people and adults with learning disabilities and epilepsy ........................... 544
14.1 Introduction ...................................................................................................................... 544
14.2 Who should manage and treat epilepsy in children, young people and adults with
learning disabilities?.......................................................................................................... 544
14.2.1

Do people with learning disabilities and epilepsy who receive care from a

specialist in learning disabilities and epilepsy compared with care from a
non-specialist have differences in processes and outcomes of care? .............. 544

14.3 Is making a diagnosis more difficult in people with learning disabilities? ........................ 545
14.3.1

Are the rates of misdiagnosis higher for people with learning disabilities and
epilepsy when compared with people with epilepsy who do not have

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learning disabilities? .......................................................................................... 545
14.3.2

What are the practical difficulties in establishing the diagnosis in this group? 545

14.4 Are there difficulties in doing investigations in this group? ............................................. 546
14.4.1

Are there a) difficulties in conducting investigations (EEG; neuroimaging); b)
difficulties in interpreting investigations (EEG; neuroimaging) in people with
learning disability and epilepsy when compared with people with epilepsy
who do not have learning disabilities? .............................................................. 546

14.5 What are the main factors to assess when making a care plan for an individual with

learning disabilities and epilepsy? .................................................................................... 547
14.6 Pharmacological management of people with epilepsy and learning disabilities ............ 547
14.6.1

Introduction ....................................................................................................... 547

14.6.2

Methods of the evidence review ....................................................................... 547

14.6.3

Matrix of the evidence....................................................................................... 547

14.6.4

New recommendations and link to evidence .................................................... 552

14.6.5

Is epilepsy more difficult to treat in people with learning disabilities? ............ 554

14.6.6

Likelihood of remission of seizures.................................................................... 554

14.7 What are the additional management issues in people with learning disabilities? ......... 555
14.7.1

Is there increased mortality in people with learning disabilities and

epilepsy? ............................................................................................................ 556

14.7.2

What management issues in people with learning disabilities do healthcare
practitioners and carers view as important? ..................................................... 557

15 Young people with epilepsy ............................................................................................... 558
15.1 Introduction ...................................................................................................................... 558
15.2 Is a different approach to management required in adolescence?.................................. 558
15.3 What are the factors that affect adherence to treatment in adolescents with
epilepsy? ........................................................................................................................... 558
15.4 Is there any evidence of effectiveness for any given strategies proposed to improve
outcomes for adolescents? ............................................................................................... 559
15.5 What are the special needs or information requirements of this group? ........................ 559
15.6 Should the diagnosis of epilepsy be revisited in this group? ............................................ 561
16 Older people...................................................................................................................... 563
16.1 Pharmacological management of epilepsy in older people .............................................. 563
16.1.1

Introduction ....................................................................................................... 563

16.1.2

Methods of the evidence review ....................................................................... 563

16.1.3

Matrix of the evidence....................................................................................... 563

16.1.4

New recommendations and link to evidence .................................................... 569

17 People from black and minority ethnic groups .................................................................... 572
17.1 Introduction ...................................................................................................................... 572
17.2 What are the information and service provision needs of people from black and
minority ethnic groups? .................................................................................................... 572
18 The care process for people with epilepsy .......................................................................... 574

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The Epilepsies
Contents

18.1 Introduction ...................................................................................................................... 574
18.2 What features of the care process in primary care/shared care lead to improved
health outcomes for adults and children with epilepsy? .................................................. 574
18.2.1

What evidence is there regarding the quality of care currently provided in
primary care? ..................................................................................................... 575

18.2.2

What process of care has been proposed to improve outcomes for adults
and children with epilepsy in primary care? ..................................................... 576

18.3 What features of the care process in secondary and tertiary care lead to improved
health outcomes for adults and children with epilepsy? .................................................. 577
18.3.1

What evidence is there of the quality of care currently provided in
secondary/tertiary care? ................................................................................... 578

18.3.2

What process of care has been proposed to improve outcomes for adults
and children with epilepsy in secondary/tertiary care? .................................... 581

18.4 What features of the care process in A&E lead to improved health outcomes for
adults and children with epilepsy? ................................................................................... 583
18.4.1

Quality of care currently provided in and accident and emergency
departments (A&E) ............................................................................................ 583

18.4.2

What process of care has been proposed to improve outcomes for adults
and children with epilepsy in A&E? ................................................................... 585

18.5 How effective are individual/self management plans in adults and children with
epilepsy? ........................................................................................................................... 585
18.5.1

Introduction ....................................................................................................... 585

18.5.2

Do adults and children with epilepsy who are educated in self-management,
when compared with those who do not, have better health outcomes?......... 586

19 Glossary ............................................................................................................................ 588
20 Reference list..................................................................................................................... 606

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The Epilepsies
Guideline development group members

Guideline development group members
Guideline Development Group (GDG) members (2004)
Ms Kathy Bairstow, nominated by Epilepsy Action (British Epilepsy Association)
Patient Representative, Leeds
Ms Bernie Concannon, nominated by the Royal College of Nursing
Clinical Nurse Specialist (Paediatric Epilepsy), Birmingham Children’s Hospital
Mr Ian Costello, nominated by the Neonatal & Paediatric Pharmacists Group
Chief Pharmacist, Centre for Paediatric Research, School of Pharmacy, London
Dr Helen Cross, nominated by the Royal College of Paediatrics & Child Health
Senior Lecturer & Honorary Consultant in Paediatric Neurology, Institute of Child Health and Great
Ormond Street Hospital for Children, London
Professor John Duncan, nominated by the Royal College of Physicians
Professor of Neurology, The National Hospital for Neurology and Neurosurgery, London
Dr Amanda Freeman, nominated by the Royal College of Paediatrics and Child Health
Consultant Paediatrician, St Mary’s Hospital, Portsmouth

Ms Sally Gomersall, nominated by the National Society for Epilepsy
Patient Representative, Newark
Ms Jane Hanna, nominated by Epilepsy Bereaved
Patient Representative, Wantage
Mr William Harkness, nominated by the Society of British Neurological Surgeons
Consultant Neurological Surgeon, Great Ormond Street Hospital for Children, London
Dr Peter Humphrey, nominated by the Association of British Neurologists
Consultant Neurologist, The Walton Centre for Neurology & Neurosurgery, Liverpool
Dr Tanzeem Raza, nominated by the Royal College of Physicians
Consultant Physician, Royal Bournemouth Hospital
Mr Peter Rogan, nominated by the Joint Epilepsy Council
Patient Representative, Ormskirk
Dr Henry Smithson, nominated by the Royal College of General Practitioners
Guideline Development Group Lead
General Practitioner, York and Honorary Clinical Senior Lecturer, Hull York Medical School

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The Epilepsies
Guideline development group members

Guideline Development Group (GDG) members (2012)
Dr Amanda Freeman
Consultant Paediatrician, Department of Paediatrics, Queen Alexandra Hospital, Portsmouth.
Mrs Diane Flower
Lead Children's Epilepsy Specialist Nurse, Royal Gwent Hospital, Newport, South Wales, and
Children's Epilepsy Specialist Nurse, Bristol Royal Hospital for Children, Bristol.
Dr Greg Rogers

GP and General Practitioner with a Special Interest in Epilepsy [GPwSI] Eastern and Coastal Kent PCT
Professor Helen Cross
The Prince of Wales's Chair of Childhood Epilepsy, UCL-Institute of Child Health, Great Ormond Street
Hospital for Children & National Centre for Young People with Epilepsy. Head of Neurosciences Unit,
UCL-Institute of Child Health, London.
Professor Ian Chi Kei Wong
Director and Professor of Paediatric Medicines Research, Centre for Paediatric Pharmacy Research,
The School of Pharmacy, The University of London, UCL Institute of Child Health, Great Ormond
Street Hospital NHS Trust for Children (Until August 2011). Department of Pharmacology and
Pharmacy, Li Ka Shing Faculty of Medicine, University of Hong Kong.

Professor of Neurology, Department of Clinical and Experimental Epilepsy, UCL Institute of
Neurology, London. Consultant Neurologist, National Hospital for Neurology and Neurosurgery.
Medical Director, The Epilepsy Society
Dr Margaret Jackson
Consultant Neurologist, Newcastle Upon Tyne Hospitals NHS Trust
Mr Michael Harnor
Patient member. Retired university academic. Neurological charities trustee
Dr Nick Kosky (chair)
Consultant Psychiatrist, Prison Mental Health Inreach Team, Medical Director
Dorset Community Health Services, NHS Dorset
Dr Richard Appleton
Consultant Paediatric Neurologist. The Roald Dahl EEG Department Paediatric Neurosciences
Foundation. Alder Hey Children's NHS Foundation Trust, Liverpool.
Mrs Sally Gomersall
Patient member. Epilepsy Society Trustee and Epilepsy Bereaved Education & Awareness Manager
Mr Sean Mackey (until March 2010)
Independent Pharmacist consultant. Dalton

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Update 2012

Professor John Duncan


The Epilepsies
Guideline development group members

Head of Epilepsy Nursing Service, NHS Eastern and Coastal Kent Community services

Update
2012

Mrs Tracey Truscott

Guideline Development Group (GDG) co-optees (2004)
Professor Gus Baker, nominated by the British Psychological Society
Professor of Neuropsychology, University of Liverpool
Professor Frank Besag, nominated by the Royal College of Psychiatrists
Consultant Psychiatrist, Bedfordshire & Luton Community NHS Trust and Visiting Professor of
Neuropsychiatry, University of Luton
Professor Shoumitro Deb, nominated by the Royal College of Psychiatrists
Professor of Neuropsychiatry and Intellectual Disability, University of Birmingham
Dr David Finnigan, nominated by PRODIGY
General Practitioner, Sowerby Centre for Health Informatics, University of Newcastle
Mr Andrew Green, nominated by the College of Occupational Therapists
Occupational Therapist, Frenchay Hospital, Bristol
Dr Jo Jarosz, nominated by the Royal College of Radiologists

Consultant Neuroradiologist, King’s College Hospital, London
Dr Andrew Lloyd Evans, nominated by the Royal College of Paediatrics and Child Health
Consultant Paediatrician, Royal Free Hospital, London
Dr David McCormick, nominated by the International League Against Epilepsy (ILAE)
Consultant Paediatrician, East Kent Hospitals NHS Trust, Kent
Mr James Oates, nominated by the Royal College of Nursing
Epilepsy Liaison Nurse (Adult), Hull Royal Infirmary
Dr Gillian Penney, nominated by the Royal College of Obstetricians and Gynaecologists
Senior Lecturer, Scottish Programme for Clinical Effectiveness in Reproductive Health, University of
Aberdeen
Ms Linda Perry, nominated by the National Centre for Young People with Epilepsy (NCYPE)
Director of Medical Services, NCYPE, St Piers Lane, Lingfield
Mr Martin Shalley, nominated by the British Association for Accident & Emergency Medicine
Consultant in A&E Medicine, Birmingham Heartlands Hospital
Professor Raymond Tallis, nominated by the British Geriatrics Society
Professor of Geriatric Medicine, University of Manchester

Professor Frank Besag
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Update 2012

Guideline Development Group (GDG) co-optees (2012)


The Epilepsies
Guideline development group members

Consultant Neuropsychiatrist Children’s Learning Disability Service. Twinwoods Health Resource

Centre, Bedford.
Dr Michael Marsh
Consultant in Obstetrics and Gynaecology, King's College Hospital, London
Dr Aza JJ Abdulla

Professor Tony Marson (External Peer Reviewer)
Professor of Neurology. University of Liverpool and Coordinating Editor Cochrane Epilepsy Group

Update 2012

Consultant Physician and Geriatrician. Department of Elderly Medicine, South London Healthcare
NHS Trust. Princess Royal University Hospital. Kent

Dr Catrin Tudur-Smith (External Peer Reviewer)
Senior Lecturer in Biostatistics. University of Liverpool and Statistical Editor Cochrane Epilepsy Group
Dr GP Sinha (External Peer Reviewer)
Consultant Paediatrician. Walsall Healthcare NHS Trust, Manor Hospital
National Collaborating Centre for Primary Care (NCC-PC) Project Team (2004)
Professor Richard Baker, Director, NCC-PC
Director, Department of Health Sciences, University of Leicester
Ms Janette Camosso-Stefinovic, Information Librarian, NCC-PC
Information Librarian, Department of Health Sciences, University of Leicester
Ms Nicola Costin, Systematic Reviewer, NCC-PC (January 2004 onwards)
Research Associate, Department of Health Sciences, University of Leicester
Ms Ariadna Juarez-Garcia, Health Economist, NCC-PC (May 2003 to July 2004)
Research Associate, Department of Health Sciences, University of Leicester
Ms Elizabeth Shaw, Senior Systematic Reviewer, NCC-PC
Research Fellow, Department of Health Sciences, University of Leicester
Dr Tim Stokes, Deputy Director, National Collaborating Centre for Primary Care, Leicester (NCC-PC)
Project Lead

Senior Lecturer in General Practice, Department of Health Sciences, University of Leicester
Dr Allan Wailoo, Health Economist, NCC-PC (until May 2003)
Lecturer in Health Economics, School of Health and Related Research, University of Sheffield

Dr Jennifer Hill (until March 2011)
Guidelines Operations Director
Ms Susan Latchem (from April 2011)
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Update 2012

National Clinical Guideline Centre Project team (2012)


The Epilepsies
Guideline development group members

Guidelines Operations Director
Ms Vanessa Delgado Nunes
Senior Research Fellow and Project Manager
Ms Julie Neilson

Ms Laura Sawyer
Senior Health Economist
Dr Grammati Sarri
Senior Research Fellow
Mr Carlos Sharpin
Senior Information Scientist and Research Fellow

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Update 2012

Senior Research Fellow


The Epilepsies
Acknowledgements

Acknowledgements
2004
The Guideline Development Group would like to thank Nancy Turnbull and Charmaine Larment of the
National Collaborating Centre for Primary Care, Royal College of General Practitioners for all their
hard work in arranging GDG meetings and supporting the guideline development process.
The Project Team would like to thank Ms Vicki Cluley, University of Leicester, for secretarial support
and Dr Ali Al-Ghorr and Dr Moray Nairn, Scottish Intercollegiate Guidelines Network, Edinburgh for
their help in sharing relevant searches and evidence reviews on the epilepsies in adults and children.
The team would also like to thank Dr Allan Wailloo, University of Sheffield for his initial health
economic input and Ms Nicola Costin for her help with the second draft.
2012

Partial Pharmacological Update of Clinical Guideline 20
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Update 2012

The Guideline Development Group and project team would like to thank Dr Lee-Yee Chong, Ms
Katrina Sparrow, Mrs Fulvia Ronchi, Ms Abigail Jones, Mr David Wonderling, Mr Tim Reason, Ms

Elisabetta Fenu, Mrs Liz Avital, Ms Hati Zorba and Dr Norma O’Flynn for all their help and support
throughout the guideline development process. The project team would also like to thank Professor
Tony Marson and Dr Catrin Tudur Smith for providing further data for the evidence analyses and for
acting as expert peer-reviewers to the guideline update.


The Epilepsies
Introduction

1 Introduction
1.1 Definition of epilepsy
2004
An epilepsy is defined as a neurological condition characterised by recurrent epileptic seizures
unprovoked by any immediately identifiable cause. An epileptic seizure is the clinical manifestation
of an abnormal and excessive discharge of a set of neurons in the brain 1.
Epilepsy should be viewed as a symptom of an underlying neurological disorder and not as a single
disease entity. The term ‘epilepsies’ is used in the title of the guideline to reflect this.

1.2 Clinical aspects
2004
The clinical presentation depends on a number of factors, chiefly: the parts of the brain affected, the
pattern of spread of epileptic discharges through the brain, the cause of the epilepsy and the age of
the individual.2 The classification of the epilepsies is controversial and has tended to focus on both
the clinical presentation (type of epileptic seizure) and on the underlying neurological disorder
(epilepsies and epilepsy syndromes).3
Epilepsy is primarily a clinical diagnosis based on a detailed description of the events before, during
and after a seizure given by the person and/or witness. Electroencephalogram (EEG), magnetic
resonance imaging (MRI) and computed tomography (CT) are used to investigate individuals with
known and suspected epilepsy. The diagnosis of epilepsy requires that seizure type, epilepsy
syndrome and any underlying cause are determined.4 It can be difficult to make a diagnosis of

epilepsy and misdiagnosis is common.5
The UK National General Practice Study of Epilepsy found that 60% of people with epilepsy have
convulsive seizures, of which two thirds have focal epilepsies and secondarily generalised seizures
and the other third will have generalised tonic-clonic seizures.1,6,7 About one-third of cases have less
than one seizure a year, one-third have between one and 12 seizures per year and the remainder
have more than one seizure per month.8
In adults and children with epilepsy, most (70%) will enter remission (being seizure free for five years
on or off treatment) but 30% develop chronic epilepsy.9 The number of seizures in the 6 months
after first presentation is an important predictive factor for both early and long-term remission of
seizures.10
The UK National General Practice Study of Epilepsy found that the majority (60%) of people with
newly diagnosed or suspected epileptic seizures had epilepsy with no identifiable aetiology. Vascular
disease was the aetiology in 15% and tumour in 6%. Among older subjects the proportion with an
identifiable cause was much higher: 49% were due to vascular disease and 11% to tumours.6
The mainstay of treatment for epilepsy is antiepileptic drugs (AEDs) taken daily to prevent the
recurrence of epileptic seizures. Since the development of MRI there has been an increase in the
number of people identified with epilepsy who could benefit from surgery. There is also a need to
ensure provision of appropriate information to people with epilepsy and their carers. In the UK the
voluntary sector has an important role in helping people with epilepsy.11

Partial Pharmacological Update of Clinical Guideline 20
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The Epilepsies
Introduction

2012

Ensuring an accurate diagnosis is important for planning management. Although the primary aim is

to diagnose a recognisable electroclinical syndrome, it is recognised this may not be possible in a not
insignificant number of individuals. The exact syndrome diagnosis may not be readily apparent at
presentation. Moreover, in some, the cause may be of equal importance. A more descriptive
approach has been recommended, retaining the electroclinical syndromes where possible but where
underlying aetiology is taken into account 12. This has implications for treatment in an increasing
number of situations.

Update 2012

Since 2004, discussion with regard to the classification of the epilepsies has continued. With
advances in technology, particularly imaging and genetics, some of the older termininology eg
idiopathic/symptomatic/cryptogenic, has become redundant in general use. Furthermore, although
seizures may be focal or generalised in onset, such terminology cannot be applied to syndromes. The
terms partial, complex and simple are also replaced simply by focal.

1.3 Epidemiology
2004
The epilepsies comprise the most common serious neurological disorders in the UK. It affects
between 260,000 and 416,000 people in England and Wales (Appendix G).13
The incidence of epilepsy is about 50 per 100,000 per annum.14 The incidence is high in childhood,
decreases in adulthood and rises again in older people.6 The usual prevalence figure given for active
epilepsy in the UK is 5-10 cases per 1,000.11
Epidemiological studies consistently report a standardised mortality rate (SMR) of 2-4 for
epilepsy.15,16 In newly diagnosed epilepsy, death is largely attributable to the underlying disease (for
example, vascular disease, tumour). In chronic epilepsy, however, the main cause of excess mortality
is death during a seizure: sudden unexpected death in epilepsy (SUDEP).17 SUDEP is estimated to
account for 500 deaths a year in the UK and has been the subject of a recent National Sentinel
Clinical Audit.18
Epilepsy is not always associated with significant morbidity. Many people with epilepsy continue to
have highly productive and fruitful lives, in which the epilepsy does not interfere to a great extent.

However, there is an associated morbidity which may be significant in some individuals, and may be
due to the effects of seizures, their underlying cause and/or treatment. Epilepsy may sometimes
result in significant disability, social exclusion and stigmatisation. People with epilepsy commonly
encounter problems in the following areas: education; employment; driving; personal development;
psychiatric and psychological aspects and social and personal relationships.11 In addition, it is
important to recognise that people with epilepsy may have co-morbidities. For example, children
with epilepsy may have attentional difficulties or learning difficulties. 19
2012

Partial Pharmacological Update of Clinical Guideline 20
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Update 2012

Analysis of data from the Quality and Outcomes Framework (QOF) epilepsy diagnostic codes suggest
a prevalence of diagnosed epilepsy in people aged 18 and over of 1.15%. The use of data from
administrative databases such as the QOF, however, which incorporate non-validated epilepsy
diagnostic codes for the estimation of prevalence rates is fraught with difficulty and there is a
tendency for such databases to overestimate prevalence. There are no direct estimates of the
epilepsy prevalence for England. Some existing data using validated methods, suggest the prevalence


The Epilepsies
Introduction

An increasing population is the elderly, in whom the incidence of new onset epilepsy is increasing,
although the possibility of misdiagnosis also remains high 22. Special consideration needs to be given
when prescribing any medication within this population, not least because of drug interaction and
pharmacokinetic issues, and this similarly applies to antiepileptic medication. Increasing information
is also being gathered on the effect of antiepileptic drugs taken by a mother on the unborn child;

further data have to be accumulated to ensure accurate information on treatment and its possible
effects are given to a woman prior to conception so she is able to make choices 23.

1.4 Cost of epilepsy
2004
The medical cost to the NHS in 1992/1993 of newly diagnosed epilepsy in the first year of diagnosis
was calculated as £18 million and the total annual cost of established epilepsy estimated at £2 billion
(direct and indirect costs), over 69% of which was due to indirect costs (unemployment and excess
mortality).24
The costs of treating epilepsy are likely to increase given the new trends in prescribing patterns
towards newer and more expensive AEDs. One of the latest studies in the literature25 estimated that
the costs of prescribing costs in the community has risen three-fold in the last 10 years, from £26
million to £86 million, a yearly increase five times the rate of inflation. The author concluded that
this was largely explained by a rapid increase in the prescribing of newer AEDs. Over the period 1991
to 1999, the number of AED prescription items in England rose by 33%, and 42% of this increase was
accounted for by increased prescribing of new AEDs. The volume of older AEDs prescribed increased
from 4.8 million prescription items in 1991 to 5.7 million in 1999, compared with more than a
hundred-fold increase in prescribing of new AEDs from 5,400 to 721,000 over the same period.25

a

MacDonald BK, Cockerell OC, Sander JW, Shorvon SD. The incidence and prevalence of neurological disorders
in a prospective community based study in the United Kingdom. Brain 2000; 123:665-676
b

Purcell B, Gaitatzis A, Sander JW, Majeed A. Epilepsy prevalence and prescribing patterns in England and
Wales. Health Statistics 2002; 15: 23-31.
c

Chowdhury FA, Nashef L, Elwes RD. Misdiagnosis in epilepsy: a review and recognition of diagnostic

uncertainty. Eur J Neurol. 2008 Oct;15(10):1034-42.

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Update 2012

to be between 0.7 to 0.8% for the whole population a bBased on a population in England of
51,810,000 in 2009 (< this would suggest there are between 362,000 and
415,000 people with epilepsy in England. In addition, there will be individuals, estimated to be a
further 5-30%, so amounting to up to another 124,500, who have been diagnosed with epilepsy, but
in whom the diagnosis is incorrect c.The rate of learning disability in the epilepsy population remains
high; in particular children with early onset epilepsy are highly likely to experience
neurodevelopmental compromise 20. Even in those with later onset, numbers with any degree of
learning disability are thought to be underestimated. The prevalence of behaviour disorder in
children with epilepsy also remains high. The British child and adolescent mental health survey,
questioning 10,438 children in the UK age 5-15 years, found a prevalence of behaviour disorder in
children with ‘pure’ epilepsy to be up to three times that of another chronic disorder (diabetes,
10.2%) or the general population (9.3%) and in ‘epilepsy plus’, almost six times (56%) 21. Both may be
compounded by medication and must therefore be taken into consideration when discussing
medication to use.


The Epilepsies
Introduction

2012

Update 2012

Since 2004, a further five AEDs have become licensed for use in the UK for the treatment of epilepsy.
A more recent cost analysis estimated the total cost of epilepsy in Europe in 2004 was 15.5 billion
Euros; the cost of antiepileptic drug use being €400,000 26. Economic cost however is only one aspect
to be considered when discussing the cost of epilepsy to the individual. Lost employment, hospital
visits and overall life disruption/quality of life need to be carefully considered. Studies reviewing
quality of life of individuals with epilepsy highlight important determinants to be seizure freedom
and medication side effects amongst others27. Seizure freedom should be strived for in each
individual who presents with epilepsy, although not at the expense of excessive side effects. Choices
of anti-epileptic medication therefore have to measured and tailored to the individual, informed by
data available from the existing evidence base.

1.5 Health Services for people with epilepsy
2004
Since 1953 six major reports11,18,28-31 have made recommendations to improve services for people
with epilepsy in the UK, but these services remain patchy and fragmented.13 The Department of
Health has recently published an action plan32 to improve services for people with epilepsy in
response to the National Sentinel Clinical Audit (SUDEP report).18
A key aim of the audit was to establish whether deficiencies in the standard of clinical management
or overall package of healthcare could have contributed to deaths. The issues raised by the SUDEP
report as they relate to primary and secondary care are summarised here.
2012

1.5.1

Primary care
2004
General practitioners (GPs) have a central role in the provision of medical care to adults with
epilepsy. The new GP contract includes quality markers, and hence financial incentive, for the
management of epilepsy in primary care. They also have an important, although more limited, role
in the management of epilepsy in children. A GP who has a list of 2,000 people can expect to care for

between 10 to 20 people with epilepsy who are on treatment and to see one to two new cases per
year.11
The SUDEP report found that the main problems in primary care for people with epilepsy were: lack
of timely access to skilled specialists; sparse evidence of structured care plans; triggers for referral
were sometimes missed, and there were failures of communication between primary and secondary
care.18

Partial Pharmacological Update of Clinical Guideline 20
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Update 2012

Since 2004, the clinical guideline recommendations have provided a framework by which epilepsy
services can be improved. However services remain patchy; a further report in 2008 by the All Party
Parliamentary Group on epilepsy (wasted money, wasted lives) recognised that in some areas many
of the recommendations as published in 2004 had not been implemented, and that an early review
was required as to the progress of implementation of the NICE guidelines in England & Wales.
Furthermore, the wider need for training was also recognised. Currently HQIP in collaboration with
the British Paediatric Neurology Association and the Royal College of Paediatrics and Child Health
have initiated a national audit of childrens services (Epilepsy12), measured against various
performance measures as defined by the 2004 guideline, due to publish in 2014.


The Epilepsies
Introduction

2012

1.5.2

Update 2012

Who takes primary responsibility for individuals with epilepsy may depend on local networks of care.
In children, responsibility remains primarily within secondary care. Training has been standardised
with courses through the British Paediatric Neurology Association and others. Transition of care into
adulthood may prove problematic however, as differing groups of individual adults may fall within
the remit of differing professional groups and teams eg adults with learning disability, and the
elderly. Some Primary Care Trusts have developed the role of the GP with a special interest in the
epilepsies (GPSIES) who are responsible for individuals with epilepsy. Defined care pathways for
individuals presenting with seizures are recommended, from initial diagnosis to complex care (NICE
2004).

Secondary care
2004
The majority of people with epilepsy receive most of their initial care in secondary care and those
whose seizures are not well controlled continue to receive ongoing care in secondary care. The
SUDEP report identified deficiencies in care provided to both adults and children in secondary care.18
A majority of adults (54%, 84/158) had inadequate care, which led to the conclusion that 39% of
adult deaths were considered potentially or probably avoidable. The main deficiencies identified
were (in descending order of frequency): inadequate access to specialist care, inadequate drug
management, lack of appropriate investigations, no evidence of a package of care, inadequate
recording of histories, adults with learning difficulties ‘lost’ in transfer from child to adult services,
and one or more major clinical management errors.
A majority of children (77%, 17/22) had inadequate care, which led to the conclusion that 59% of
deaths in children were considered potentially or probably avoidable. The main deficiencies
identified were (in descending order of frequency): inadequate drug management, inadequate
access to specialist care, and inadequate investigations.
There was concern that documentation was poor in both primary and secondary care; only 1% of
hospital records for adults showed that SUDEP had been discussed.
2012

Update 2012

Criteria by which individuals should be referred into tertiary care were included in the 2004
guideline. Care of individuals with epilepsy will be optimised where these guidelines are followed and
care pathways are in place. Audit of care is yet to be undertaken however; HQIP in collaboration with
British Paediatric Neurology Association and the Royal College of Paediatrics and Child Health have
initiated an audit of 12 outcomes from the NICE guideline to be conducted throughout the UK in
children (Epilepsy 12) to be complete by 2014.

1.6 The SANAD trial

Partial Pharmacological Update of Clinical Guideline 20
25

Update 2012

The SANAD trial was a pragmatic, randomised, unblinded, parallel group clinical trial comprising two
arms (one comparing new AEDs with carbamazepine and the other comparing newer AEDs with
sodium valproate). It was commissioned and sponsored by the NHS R&D Health Technology
Assessment Programme, but also supported by the pharmaceutical companies with AEDs included in
the study, who contributed approximately 20% of the total costs of the study. It received appropriate
multicentre and local ethics and research committee approvals, and patients gave informed consent
to inclusion and to long-term follow-up. It also achieved the involvement of a large number of