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## Complete Summary

#### GUIDELINE TITLE

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

#### BIBLIOGRAPHIC SOURCE(S)

National Collaborating Centre for Primary Care. The diagnosis and
management of the epilepsies in adults and children in primary and
secondary care. London (UK): Royal College of General Practitioners;
2004 Oct. 525 p. \[324 references\]

#### GUIDELINE STATUS

This is the current release of the guideline.

### \*\* REGULATORY ALERT \*\*

#### FDA WARNING/REGULATORY ALERT

**Note from the National Guideline Clearinghouse**: This guideline
references a drug(s) for which important revised regulatory and/or
warning information has been released.

-   [December 12, 2007,
    Carbamazepine](http://www.fda.gov/medwatch/safety/2007/safety07.htm#carbamazepine):
    The U.S. Food and Drug Administration (FDA) has provided
    recommendations for screening that should be performed on specific
    patient populations before starting treatment with carbamazepine.

### COMPLETE SUMMARY CONTENT

 \*\* REGULATORY ALERT \*\*\
 SCOPE\
 METHODOLOGY - including Rating Scheme and Cost Analysis\
 RECOMMENDATIONS\
 EVIDENCE SUPPORTING THE RECOMMENDATIONS\
 BENEFITS/HARMS OF IMPLEMENTING THE GUIDELINE RECOMMENDATIONS\
 QUALIFYING STATEMENTS\
 IMPLEMENTATION OF THE GUIDELINE\
 INSTITUTE OF MEDICINE (IOM) NATIONAL HEALTHCARE QUALITY REPORT
CATEGORIES\
 IDENTIFYING INFORMATION AND AVAILABILITY\
 DISCLAIMER

### SCOPE

#### DISEASE/CONDITION(S)

Epilepsy including:

-   Focal/partial/localisation related epilepsies

-   Idiopathic generalised epilepsies

-   Status epilepticus

#### GUIDELINE CATEGORY

Diagnosis\
Evaluation\
Management\
Treatment

#### CLINICAL SPECIALTY

Family Practice\
Internal Medicine\
Medical Genetics\
Neurology\
Obstetrics and Gynecology\
Pediatrics

#### INTENDED USERS

Advanced Practice Nurses\
Nurses\
Patients\
Pharmacists\
Physician Assistants\
Physicians

#### GUIDELINE OBJECTIVE(S)

To offer evidence-based and best practice advice on the diagnosis,
treatment, and management of the epilepsies in children and adults in
primary and secondary care

#### TARGET POPULATION

Adults and children, over 28 days old, who have epilepsy and are seen in
primary or secondary care settings, including pregnant women,
women/girls of child-bearing age, and people with learning disabilities

**Exclusions**

The following populations are *[not]{.underline}* included*:*

-   Neonates (infants aged 28 days or under)

-   People with febrile convulsions

#### INTERVENTIONS AND PRACTICES CONSIDERED

**Evaluation/Diagnosis**

1.  Specialist assessment

2.  Full clinical history with eye witness account supported by
    corroborative evidence

3.  Physical examination (including cardiac, neurological/mental status,
    and development assessment)

4.  Referral, when appropriate

5.  Investigations, including:

    -   Electroencephalogram (EEG)

        -   Provocation techniques

    -   Neuroimaging (magnetic resonance imaging \[MRI\] and computed
        tomography \[CT\])

    -   Other tests

        -   Adults: serum prolactin, plasma electrolytes, glucose,
            calcium

        -   Children: blood and urine biochemistry

        -   12 lead electrocardiogram (ECG)

    -   Neuropsychological assessment

6.  Classification using multi-axial diagnostic scheme

**Treatment/Management**

**Overall Approach**

1.  Patient/parent/family/carer education

2.  Referral to specialist/support services as needed

3.  Comprehensive Care Plan

**Pharmacological Treatment**

1.  Anti-epileptic drugs (AEDs) (monotherapy/combination)

    -   Adults: gabapentin, lamotrigine, levetiracetam, oxcarbazepine,
        tiagabine, topiramate, and vigabatrin

    -   Children: gabapentin, lamotrigine, oxcarbazepine, tiagabine,
        topiramate, and vigabatrin

2.  Continuation of treatment

    -   Blood test monitoring, as needed

3.  Withdrawal of treatment

    -   Managed AED withdrawal

4.  Referral for complex or refractory epilepsy

**Psychological Treatment**

1.  Relaxation, cognitive behaviour therapy, biofeedback

**Other Treatments**

1.  Ketogenic diet (Not recommended in adults; considered as adjunctive
    treatment in children)

2.  Vagus nerve stimulation (VNS)

3.  Rectal diazepam or buccal midazolam\* for prolonged or rectal
    seizures

4.  Lorazepam for sustained epilepticus

5.  Refractory status epilepticus

    -   Adults: propofol and thiopental

    -   Children: midazolam or thiopental

    -   Individual treatment pathways

**Special Populations**

**Women**

1.  Specialised patient education regarding contraception, conception,
    pregnancy, caring for children, breastfeeding, and menopause

2.  Folic acid for women on AEDs

3.  Referral to genetic counseling as appropriate

4.  Vitamin K parenterally for newborns at delivery

**People with Learning Disabilities**

1.  Full clinical history with eye witness account supported by
    corroborative evidence

2.  Special investigations (e.g. imaging under anesthesia), as needed

3.  Risk assessment

**Young People with Epilepsy**

1.  Consulting style of medical management

2.  Multidisciplinary services

3.  Patient education and referral to support services

**Older People with Epilepsy**

1.  Interventions same as for general population

**People from Black and Minority Ethnic Groups**

1.  Additional communication and cultural information such as
    interpreters and translations of information about employment rights
    and driving

**Review**

1.  Structured review, dependent on patient

2.  Provide access to written and visual information, counseling,
    information about voluntary organizations, epilepsy specialist
    nurses, investigations, and referral, when appropriate

\* Note: Buccal midazolam is currently unlicensed for the treatment of
prolonged or repeated seizures.

#### MAJOR OUTCOMES CONSIDERED

-   Recurrence of epileptic seizures

-   Quality of life

-   Morbidity and mortality

### METHODOLOGY

#### METHODS USED TO COLLECT/SELECT EVIDENCE

Hand-searches of Published Literature (Primary Sources)\
Searches of Electronic Databases

#### DESCRIPTION OF METHODS USED TO COLLECT/SELECT THE EVIDENCE

**Literature Search Strategy**

The aim of the literature review was to seek to identify all available,
relevant published evidence in relation to the key clinical questions
generated by the Guideline Development Group (GDG). The prioritised key
clinical questions (KCQs) were turned into evidence-based questions
(EBQs) by the project lead and systematic reviewer. Literature searches
were conducted using generic search filters and modified filters
designed to best address the specific question being investigated.
Searches included both medical subject headings (MeSH terms) and
free-text terms. Details of all literature searches are available from
the National Collaborating Centre for Primary Care (NCC-PC), University
of Leicester.

The information librarian developed a search strategy for each question
with the assistance of the systematic reviewer and the project lead.
Searches were re-run at the end of the guideline development process,
thus including evidence published up to the end of December 2003.

Depending on the clinical area, some or all of the following databases
were searched: Cochrane Library (up to Issue 3, 2003) was searched to
identify any relevant systematic reviews, and for reports of randomised
controlled trials, MEDLINE (for the period January 1966 to November
2003, on the OVID interface), EMBASE (for the period January 1980 to
November 2003, on the OVID interface), the Cumulative Index of Nursing
and Allied Health Literature (for the period January 1982 to November
2003, on the Dialog DataStar interface), PsycINFO (for the period 1887
to September 2003, on the OVID and the Dialog DataStar interfaces), the
Health Management Information Consortium database (HMIC), the British
Nursing Index (BNI), and the Allied and Complementary Medicine Database
(AMED). Searches for nonsystematic reviews of the literature were
limited to 1997 to November 2003. This was a pragmatic decision that
draws on the search strategies used by the North Of England Evidence
Based Guideline Development Project. No systematic attempt was made to
search \"grey literature\" (such as conference proceedings, abstracts,
unpublished reports or trials, etc.).

Existing systematic reviews and meta-analyses relating to epilepsy were
identified. Recent (last 6 years) high quality reviews of the epilepsy
literature were also identified. New searches, including identification
of relevant randomised controlled trials (RCTs), were conducted in areas
of importance to the guideline development process, for which existing
systematic reviews were unable to provide valid or up to date answers.
The search strategy was dictated by the exact EBQ the GDG wished to
answer. Expert knowledge of group members was also drawn upon to
corroborate the search strategy.

The National Research Register (NRR), National Guidelines Clearinghouse
(NGC), New Zealand Guidelines Group (NZGG), and the Guidelines
International Network (GIN) were searched to identify any existing
relevant guidelines produced by other organisations. The reference lists
in these guidelines were checked against the methodology team\'s search
results to identify any missing evidence.

The titles and abstracts of records retrieved by the searches were
scanned for relevance to the GDG\'s clinical questions. Any potentially
relevant publications were obtained in full text. These were assessed
against the inclusion criteria and the reference lists were scanned for
any articles not previously identified. Further references were also
suggested by the GDG. Evidence submitted by stakeholder organisations
that was relevant to the GDG\'s KCQs, and was of at least the same level
of evidence as that identified by the literature searches, was also
included.

**Initial Review**

The searches were first sifted by the information librarian and
systematic reviewer to exclude papers that did not relate to the scope
of the guideline. The abstracts of the remaining papers were scrutinised
for relevance to the EBQ under consideration. Initially both the
systematic reviewer and project lead reviewed the abstracts
independently. This proved impractical as the guideline progressed and
the task was delegated to the systematic reviewer. The project lead was
asked to review the abstracts in cases of uncertainty.

The papers chosen for inclusion were obtained and assessed for their
methodological rigour against a number of criteria that determine the
validity of the results. These criteria differed according to study type
and were based on the checklists developed by the Scottish
Intercollegiate Guidelines Network (SIGN). Critical appraisal was
carried out by the systematic reviewer. To minimise bias in the
assessment a sample of papers was independently appraised by the project
lead. Further appraisal was provided by the GDG members at the relevant
GDG meeting.

#### NUMBER OF SOURCE DOCUMENTS

Not stated

#### METHODS USED TO ASSESS THE QUALITY AND STRENGTH OF THE EVIDENCE

Weighting According to a Rating Scheme (Scheme Given)

#### RATING SCHEME FOR THE STRENGTH OF THE EVIDENCE

**Levels of Evidence**

**Ia** - Systematic review or meta-analysis of randomised controlled
trials

**Ib** - At least one randomised controlled trial

**IIa** - At least one well-designed controlled study without
randomization

**IIb** - At least one well-designed quasi-experimental study, such as a
cohort study

**III** - Well-designed non-experimental descriptive studies,
case-control studies, and case series

**IV** - Expert committee reports, opinions and/or clinical experience
of respected authorities

**NICE** - National Institute for Clinical Excellence (NICE) guidelines
or Health Technology Appraisal programme

#### METHODS USED TO ANALYZE THE EVIDENCE

Review of Published Meta-Analyses\
Systematic Review with Evidence Tables

#### DESCRIPTION OF THE METHODS USED TO ANALYZE THE EVIDENCE

The data were extracted to a standard template on an evidence table. The
findings were summarised by the systematic reviewer into a series of
evidence statements and an accompanying narrative review. The project
lead independently assessed the accuracy of the derived evidence
statements. None of the evidence based questions (EBQs) required the
preparation of a quantitative synthesis (meta-analysis) by the project
team.

The evidence statements were graded by the systematic reviewer according
to the established hierarchy of evidence table presented in the above
section titled \"Rating Scheme for the Strength of the Evidence.\" This
system reflects the susceptibility to bias inherence in particular study
designs. The project lead independently assessed the accuracy of the
grading.

The type of EBQ dictates the highest level of evidence that may be
sought. For questions relating to therapy/treatment, the highest
possible level of evidence is a systematic review or meta-analysis of
randomized controlled trials (RCTs) (evidence level Ia) or an individual
RCT (evidence level Ib). For questions relating to prognosis, the
highest possible level of evidence is a cohort study (evidence level
IIb). For diagnostic tests, the highest possible level of evidence is a
test evaluation study using a quasi-experimental design that uses a
blind comparison of the test with a validated reference standard applied
to a sample of individuals who are representative of the population to
whom the test would apply (evidence level IIb). For questions relating
to information needs and support, the highest possible level of evidence
is a descriptive study using either questionnaire survey or qualitative
methods (III).

For each clinical question, the highest level of evidence was selected.
If a systematic review, meta-analysis, or RCT existed in relation to an
EBQ, studies of a weaker design were ignored.

Summary results and data are presented in the text of the original
guideline document. More detailed results and data are presented in the
evidence tables (Appendix F of the original guideline document).

#### METHODS USED TO FORMULATE THE RECOMMENDATIONS

Expert Consensus

#### DESCRIPTION OF METHODS USED TO FORMULATE THE RECOMMENDATIONS

The Guideline Development Group (GDG) met at six weekly intervals for 16
months to review the evidence identified by the methodology team, to
comment on its quality and completeness, and to develop recommendations
for clinical practice based on the available evidence. In order to
generate separate recommendations for adults and children the GDG was
divided into adult and child subgroups. Each subgroup met to discuss the
evidence reviews and to make preliminary recommendations. The final
recommendations were agreed by the full GDG.

For each key clinical question (KCQ), the recommendations were derived
from the evidence statements presented to the GDG. The link between the
evidence statement and recommendation was made explicit. The GDG were
able to reach their agreed recommendations through a process of informal
consensus.

Each recommendation was graded according to the level of evidence upon
which it was based using the established grading of recommendations
table presented in the section below labeled \"Rating Scheme for the
Strength of the Recommendations.\" For questions relating to
therapy/treatment, the best possible level of evidence (a systematic
review or meta-analysis or an individual randomized controlled trial
\[RCT\]) would equate to a grade A recommendation. For questions
relating to prognosis and diagnostic tests, the best possible level of
evidence (a cohort study) would equate to a grade B recommendation. For
questions relating to information needs and support, the best possible
level of evidence (descriptive study) would equate to a grade C
recommendation. It is important that the grading in such areas is not
treated as inferior to those of therapy as it represents the highest
level of relevant evidence.

#### RATING SCHEME FOR THE STRENGTH OF THE RECOMMENDATIONS

**Grades of Recommendation**

**A** - Based directly on level I evidence

**B** - Based directly on level II evidence or extrapolated from level I
evidence

**C** - Based directly on level III evidence or extrapolated from level
I or level II evidence

**D** - Based directly on level IV evidence or extrapolated from level
I, level II, or level III evidence

**A (NICE)** - Recommendation taken from National Institute for Clinical
Excellence (NICE) guideline or Technology Appraisal

**GPP** - Good practice points based on the clinical experience of the
Guideline Development Group (GDG)

#### COST ANALYSIS

**The Cost of Epilepsy Misdiagnosis**

The guideline authors examined the economic burden of misdiagnosis in
epilepsy from the Health Sector point of view. The results presented
must be treated with caution since they relied on various assumptions.
Despite this, they believe the results are important and highlight the
problem from an economic perspective. Even when the lower bands (and
probably underestimated) of the estimations are considered the value of
the resources wasted due to misdiagnosis is significant. Indirect costs
(costs of lost productivity) were not estimated for reasons already
mentioned. However, they may be large considering that one study
estimated the average indirect costs per person with epilepsy to be
3,770 pounds sterling due to unemployment and in another study 23/46
misdiagnosed were unemployed, or under threat of job loss.

Other important costs and consequences of misdiagnosis which are not
considered in this study (mainly for lack of reliable data) are the cost
of side effects caused by incorrect treatment on misdiagnosed persons,
and the forgone benefits in terms of health, quality of life, and
productivity for receiving the correct treatment. Also, they did not
consider the direct costs for the patients and their families (for
example, transport costs to the health center hospital). And the costs
of legal settlements for both misdiagnosed persons and the National
Health Service (NHS) (for example, during 2002/3 the families of
children misdiagnosed in Leicester and Coroners Inquests).

To summarise, the guideline authors found that the opportunity costs of
misdiagnosis are considerably high. This is especially true if the
developers account for the fact that a large amount of lost resources
could be used to give the correct treatment to misdiagnosed persons and
to improve the quality of the treatment received by patients with
epilepsy.

See Appendix G of the original guideline document for detailed
information on the cost analysis.

#### METHOD OF GUIDELINE VALIDATION

External Peer Review\
Internal Peer Review

#### DESCRIPTION OF METHOD OF GUIDELINE VALIDATION

The guideline has been developed in accordance with the Institute\'s
guideline development process. This has included allowing registered
stakeholders the opportunity to comment on the scope of the guideline,
the first draft of the full and short form guideline, and the final
draft of the guideline. In addition, the first draft was reviewed by
nominated individuals with an interest in epilepsy and an independent
Guideline Review Panel (GRP) established by the Institute.

The comments made by the stakeholders, peer reviewers and the GRP were
collated and presented anonymously for consideration by the Guideline
Development Group (GDG). All comments were considered systematically by
the GDG and the project team recorded the agreed responses.

### RECOMMENDATIONS

#### MAJOR RECOMMENDATIONS

Levels of evidence (Ia-IV) and grades of recommendations (A-D, A
\[NICE\], and GPP) are defined at the end of the Major Recommendations
field.

**Principle of Decision Making**

**D** - Healthcare professionals should adopt a consulting style that
enables the individual 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.

**Coping with Epilepsy**

**GPP** - People with epilepsy and their families and/or carers should
be empowered to manage their condition as well as possible.

**A** - Adults should receive appropriate information and education
about all aspects of epilepsy. This may be best achieved and maintained
through structured self-management plans.

**A** - In children, self management of epilepsy may be best achieved
through active child-centred training models and interventions.

**GPP** - Healthcare professionals should highlight the Expert Patients
Programme
([www.expertpatients.nhs.uk](http://www.expertpatients.nhs.uk/)) to
individuals with epilepsy who wish to manage their condition more
effectively.

**Information**

**C** - Individuals with epilepsy and their families and/or carers
should be given, and have access to sources of, information about (where
appropriate):

-   Epilepsy in general

-   Diagnosis and treatment options

-   Medication and side effects

-   Seizure type(s), triggers, and seizure control

-   Management and self-care

-   Risk management

-   First aid, safety and injury prevention at home and at school or
    work

-   Psychological issues

-   Social security benefits and social services

-   Insurance issues

-   Education and healthcare at school

-   Employment and independent living for adults

-   Importance of disclosing epilepsy at work, if relevant (If further
    information or clarification is needed, voluntary organisations
    should be contacted.)

-   Road safety and driving

-   Prognosis

-   Sudden death in epilepsy (SUDEP)

-   Status epilepticus

-   Life style, leisure and social issues (including recreational drugs,
    alcohol, sexual activity, and sleep deprivation)

-   Family planning and pregnancy

-   Voluntary organisations, such as support groups and charitable
    organisations, and how to contact them

**GPP** - The time at which this information should be given will depend
on the certainty of the diagnosis and the need for confirmatory
investigations.

**GPP** - Information should be provided in formats, languages, and ways
that are suited to the individual\'s requirements. Consideration should
be given to developmental age, gender, culture, and stage of life of the
individual.

**GPP** - If individuals and families and/or carers have not already
found high quality information from voluntary organisations and other
sources, healthcare professionals should inform them of different
sources (using the Internet, if appropriate: see, for example, the
website of the Joint Epilepsy Council of the UK and Ireland,
[www.jointepilepsycouncil.org.uk](http://www.jointepilepsycouncil.org.uk/)).

**GPP** - Adequate time should be set aside in the consultation to
provide information, which should be revisited on subsequent
consultations.

**GPP** - Checklists should be used to remind both individuals and
healthcare professionals about information that should be discussed
during consultations.

**GPP** - Everyone providing care or treatment for individuals with
epilepsy should be able to provide essential information.

**GPP** - The person with epilepsy and their family and/or carers as
appropriate should know how to contact a named individual when
information is needed. This named individual should be a member of the
healthcare team and be responsible for ensuring that the information
needs of the individual and/or their family and/or carers are met.

**GPP** - The possibility of having seizures should be discussed, and
information on epilepsy should be provided 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.

**C** (adults)/**GPP** (children) - People with epilepsy should be given
appropriate information before they make important decisions (for
example, regarding pregnancy or employment).

**Sudden Death in Epilepsy (SUDEP)**

**C** - Information on SUDEP should be included in literature on
epilepsy to show why preventing seizures is important. Tailored
information on the individual\'s relative risk of SUDEP should be part
of the counselling checklist for people with epilepsy and their families
and/or carers.

**GPP** - The risk of SUDEP can be minimized by:

-   Optimising seizure control

-   Being aware of the potential consequences of nocturnal seizures

**C** - Tailored information and discussion between the individual with
epilepsy, family and/or carers (as appropriate) and healthcare
professionals should take account of the small but definite risk of
SUDEP.

**C** - Where families and/or carers have been affected by SUDEP,
healthcare professionals should contact families and/or carers to offer
their condolences, invite them to discuss the death, and offer referral
to bereavement counselling and a SUDEP support group.

**Following a First Seizure**

**GPP** - Individuals presenting to an Accident and Emergency department
following a suspected seizure should be screened initially. This should
be done by an adult or paediatric physician with onward referral to a
specialist\* when an epileptic seizure is suspected or there is
diagnostic doubt.

\*For adults, a specialist is defined throughout at a medical
practitioner with training and expertise in epilepsy. For children, a
specialist is defined throughout as a paediatrician with training and
expertise in epilepsy.

**D** - Protocols should be in place that ensure proper assessment in
the emergency setting for individuals presenting with an epileptic
seizure (suspected or confirmed).

**GPP** (adults) - The information that should be obtained from the
individual and/or family or carer after a suspected seizure is contained
in Appendix A of the original guideline document.

**GPP** (children) - The information that should be obtained from the
child and/or parent or carer after a suspected seizure is contained in
Appendix A of the original guideline document

**A \[NICE\]** (adults) - It is recommended that all people having a
first seizure should be seen as soon as possible\* by a specialist in
the management of the epilepsies to ensure precise and early diagnosis
and initiation of therapy as appropriate to their needs.

**A \[NICE\]** (children) - It is recommended that all children who have
had a first nonfebrile seizure should be seen as soon as possible\* by a
specialist in the management of the epilepsies to ensure precise and
early diagnosis and initiation of therapy as appropriate to their needs.

\*The Guideline Development Group considered that with a recent onset
suspected seizure, referrals should be urgent, meaning that patients
should be seen within 2 weeks.

**GPP** - At the initial assessment for a recent onset seizure, the
specialist should have access to appropriate investigations.

**C** - In an individual presenting with an attack, a physical
examination should be carried out. This should address the individual\'s
cardiac, neurological, and mental status, and should include a
developmental assessment where appropriate.

**GPP** - Essential information on how to recognise a seizure, first
aid, and the importance of reporting further attacks should be provided
to a person who has experienced a possible first seizure and their
family/carer/parent as appropriate. This information should be provided
while the individual is awaiting a diagnosis and should also be provided
to family and/or carers.

**Diagnosis**

**C** (adults) - The diagnosis of epilepsy in adults should be
established by a specialist medical practitioner with training and
expertise in epilepsy.

**C** (children) - The diagnosis of epilepsy in children should be
established by a specialist paediatrician with training and expertise in
epilepsy.

**GPP** (adults)/**C** (children) - Individuals and their families
and/or carers should be given an opportunity to discuss the diagnosis
with an appropriate healthcare professional.

**C** - A detailed history should be taken from the individual and an
eyewitness to the attack, where possible, to determine whether or not an
epileptic seizure is likely to have occurred.

**B** - The clinical decision as to whether an epileptic seizure has
occurred should then be based on the combination of the description of
the attack and different symptoms. Diagnosis should not be based on the
presence or absence of single features.

**GPP** - It may not be possible to make a definite diagnosis of
epilepsy. If the diagnosis cannot be clearly established, further
investigations and/or referral to a tertiary centre (see section on
Referral below) should be considered. Follow-up should always be
arranged.

**GPP** - Where non-epileptic attack disorder is suspected, suitable
referral should be made to psychological or psychiatric services for
further investigation and treatment.

**GPP** - Prospective recording of events, including video recording and
written descriptions, can be very helpful in reaching a diagnosis.

**Investigations**

**D** - Information should be provided to individuals and families
and/or carers as appropriate on the reasons for tests, their results and
meaning, the requirements of specific investigations, and the logistics
of obtaining them.

**GPP** (children) - All investigations should be performed in a child
centred environment.

**Electroencephalogram (EEG)**

**GPP** - Individuals requiring an EEG should have the test performed
soon (within 4 weeks) after it has been requested.

**C** (adults) - An EEG should be performed only to support a diagnosis
of epilepsy in adults in whom the clinical history suggests that the
seizure is likely to be epileptic in origin.

**C** (children) - An EEG should be performed only to support a
diagnosis of epilepsy in children. If an EEG is considered necessary, it
should be performed after the second epileptic seizure but may, in
certain circumstances, as evaluated by the specialist, be considered
after a first epileptic seizure.

**C** - An EEG should not be performed in the case of probable syncope
because of the possibility of a false-positive result.

**C** - The EEG should not be used to exclude a diagnosis of epilepsy in
an individual in whom the clinical presentation supports a diagnosis of
a non-epileptic event.

**C** - The EEG should not be used in isolation to make a diagnosis of
epilepsy.

**C** - An EEG may be used to help determine seizure type and epilepsy
syndrome in individuals in whom epilepsy is suspected. This enables
individuals to be given the correct prognosis.

**B** - In individuals presenting with a first unprovoked seizure,
unequivocal epileptiform activity shown on EEG can be used to assess the
risk of seizure recurrence.

**GPP** - For individuals in whom epilepsy is suspected, but who present
diagnostic difficulties, specialist investigations should be available.

**C** - Repeated standard EEGs may be helpful when the diagnosis of the
epilepsy or the syndrome is unclear. However, if the diagnosis has been
established, repeat EEGs are not likely to be helpful.

**C** - Repeated standard EEGs should not be used in preference to sleep
or sleep-deprived EEGs.

**C** - When a standard EEG has not contributed to diagnosis or
classification, a sleep EEG should be performed.

**GPP** - In children, a sleep EEG is best achieved through sleep
deprivation or the use of melatonin. (Note: Melatonin is not currently
licensed in the United Kingdom.)

**C** - Long-term video or ambulatory EEG may used in the assessment of
individuals who present diagnostic difficulties after clinical
assessment and standard EEG.

**C** - Provocation by suggestion may be used in the evaluation of
non-epileptic attack disorder. However, it has a limited role and may
lead to false positive results in some individuals.

**GPP** - Photic stimulation and hyperventilation should remain part of
standard EEG assessment. The individual and family and/or carer should
be made aware that such activation procedures may induce a seizure and
they have a right to refuse.

**Neuroimaging**

**C** - Neuroimaging should be used to identify structural abnormalities
that cause certain epilepsies.

**C** - Magnetic resonance imaging (MRI) should be the imaging
investigation of choice in individuals with epilepsy.

**C** - MRI is particularly important in those:

-   Who develop epilepsy before the age of 2 years or in adulthood

-   Who have any suggestion of a focal onset on history, examination, or
    EEG (unless clear evidence of benign focal epilepsy)

-   In whom seizures continue in spite of first-line medication

**GPP** - Individuals requiring MRI should have the test performed soon
(within 4 weeks).

**C** - Neuroimaging should not be routinely requested when a diagnosis
of idiopathic generalised epilepsy has been made.

**C** - Computed tomography (CT) should be used to identify underlying
gross pathology if MRI is not available or is contraindicated, and for
children in whom a general anaesthetic or sedation would be required for
MRI but not CT.

**GPP** - In an acute situation, CT may be used to determine whether a
seizure has been caused by an acute neurological lesion or illness.

**Other Tests**

**C** - Measurement of serum prolactin is not recommended for the
diagnosis of epilepsy.

**GPP** (adults) - In adults, appropriate blood tests (for example,
plasma electrolytes, glucose, calcium) to identify potential causes
and/or to identify any significant comorbidity should be considered.

**GPP** (children) - In children, other investigations, including blood
and urine biochemistry, should be undertaken at the discretion of the
specialist to exclude other diagnoses and to determine an underlying
cause of the epilepsy.

**GPP** (adults) - A 12 lead electrocardiogram (ECG) should be performed
in adults with suspected epilepsy.

**GPP** (children) - In children a 12 lead electrocardiogram should be
considered in cases of diagnostic uncertainty

**GPP** - In cases of diagnostic uncertainty, a referral to a
cardiologist should be considered.

**Neuropsychological Assessment**

**D** - Neuropsychological assessment should be considered in
individuals in whom it is important to evaluate learning disabilities
and cognitive dysfunction, particularly in regard to language and
memory.

**D** - Referral for a neuropsychological assessment is indicated:

-   When an individual with epilepsy is having educational or
    occupational difficulties

-   When an MRI has identified abnormalities in cognitively important
    brain regions

-   When an individual complains of memory or other cognitive deficits
    and/or cognitive decline

**Classification**

**D** - Epileptic seizures and epilepsy syndromes in individuals should
be classified using a multi-axial diagnostic scheme. The axes that
should be considered are description of seizure (ictal phenomenology),
seizure type, syndrome, and aetiology.

**C** - The seizure type(s) and epilepsy syndrome, aetiology, and
comorbidity should be determined, because failure to classify the
epilepsy syndrome correctly can lead to inappropriate treatment and
persistence of seizures.

**GPP** - Individuals with epilepsy should be given information about
their seizure type(s) and epilepsy syndrome, and the likely prognosis.

**Management**

**GPP** - People with epilepsy should have an accessible point of
contact with specialist services.

**GPP** - All people with epilepsy should have a comprehensive care plan
that is agreed between the individual, family and/or carers where
appropriate, and primary care and secondary care providers. This should
include lifestyle issues as well as medical issues.

**D** - Epilepsy specialist nurses (ESNs) should be an integral part of
the network of care of individuals with epilepsy. The key roles of the
epilepsy specialist nurses are to support both epilepsy specialists and
generalists, to ensure access to community and multi-agency services,
and to provide information, training, and support to the individual,
families, carers and, in the case of children, others involved in the
child\'s education, welfare, and well-being.

**GPP** - 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.

**Pharmacological Treatment**

*Appendix B of the original guideline document provides details on
prescribing for different seizure types and epilepsy syndromes.
Significant side effects are also described.*

**GPP** - Information that is provided about anti-epileptic drugs (AEDs)
needs to be in the context of that provided by the manufacturer, for
example, indications, side effects, and license status.

**A** - The AED treatment strategy should be individualised according to
the seizure type, epilepsy syndrome, comedication and comorbidity, the
individual\'s lifestyle, and the preferences of the individual and their
family and/or carers as appropriate (see Appendix B in the original
guideline document).

**GPP** - The diagnosis of epilepsy needs to be critically evaluated if
events continue despite an optimal dose of a first-line AED.

**D** - Changing the formulation or brand of AED is not recommended
because different preparations may vary in bioavailability or have
different pharmacokinetic profiles and, thus, increased potential for
reduced effect or excessive side effects.

**A \[NICE\]** - It is recommended that individuals should be treated
with a single antiepileptic drug (monotherapy) wherever possible. If the
initial treatment is unsuccessful, then monotherapy using another drug
can be tried. Caution is needed during the changeover period.

**GPP** - If an AED has failed because of adverse effects or continued
seizures, a second drug should be started (which may be an alternative
first-line or second-line drug) and built up to an adequate or maximum
tolerated dose and then the first drug should be tapered off slowly.

**GPP** - If the second drug is unhelpful, either the first or second
drug may be tapered, depending on relative efficacy, side effects, and
how well the drugs are tolerated before starting another drug.

**A \[NICE\]** - It is recommended that combination therapy (adjunctive
or \"add-on\" therapy) should only be considered when attempts at
monotherapy with AEDs have not resulted in seizure freedom. If trials of
combination therapy do not bring about worthwhile benefits, treatment
should revert to the regimen (monotherapy or combination therapy) that
has proved most acceptable to the individual, in terms of providing the
best balance between effectiveness in reducing seizure frequency and
tolerability of side effects.

**A \[NICE\]** (adults) - The newer AEDs gabapentin, lamotrigine,
levetiracetam, oxcarbazepine, tiagabine, topiramate, and vigabatrin,
within their licensed indications, are recommended for the management of
epilepsy in people who have not benefited from treatment with the older
antiepileptic drugs such as carbamazepine or sodium valproate, or for
whom the older antiepileptic drugs are unsuitable because:

-   There are contraindications to the drugs

-   They could interact with other drugs the person is taking (notably
    oral contraceptives)

-   They are already known to be poorly tolerated by the individual

-   The person is a woman of childbearing potential

**A \[NICE\]** (children) - The newer AEDs gabapentin, lamotrigine,
oxcarbazepine, tiagabine, topiramate, and vigabatrin (as an adjunctive
therapy for partial seizures), within their licensed indications, are
recommended for the management of epilepsy in children who have not
benefited from treatment with the older antiepileptic drugs such as
carbamazepine or sodium valproate, or for whom the older antiepileptic
drugs are unsuitable because:

-   There are contraindications to the drugs

-   They could interact with other drugs the child is taking (notably
    oral contraceptives)

-   They are already known to be poorly tolerated by the child

-   The child is currently of childbearing potential or is likely to
    need treatment into her childbearing years

**A \[NICE\]** - Vigabatrin is recommended as a first-line therapy for
the management of infantile spasms.

**Initiation of Pharmacological Treatment**

**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** (adults) - AED therapy should be initiated in adults on the
recommendation of a specialist.

**GPP** (children) - AED therapy in children should be initiated by a
specialist.

**GPP** - The decision to initiate AED therapy should be taken between
the individual, their family and/or carers (if appropriate) and the
specialist after a full discussion of the risks and benefits of
treatment. This discussion should take into account details of the
individual\'s epilepsy syndrome, prognosis and lifestyle.

**A** - Treatment with AED therapy is generally recommended after a
second epileptic seizure.

**B** - AED therapy should be considered and discussed with individuals
and their family and/or carers as appropriate 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

**GPP** - It should be recognised that some individuals (through their
families and/or carers, in some instances) may choose not to take AED
therapy following a full discussion of the risks and benefits.

**Continuation of Pharmacological Treatment**

**GPP** - Continuing AED therapy should be planned by the specialist. It
should be part of the individual\'s agreed treatment plan, which should
include details of how specific drug choices were made, drug dosage,
possible side effects, and action to take if seizures persist.

**GPP** - The needs of the individual and their family and/or carers as
appropriate should be taken into account when healthcare professionals
take on the responsibility of continuing prescribing.

**GPP** - If management is straightforward, continuing AED therapy can
be prescribed in primary care if local circumstances and/or licensing
allow.

**GPP** - The prescriber must ensure that the individual and their
family and/or carers as appropriate are fully informed about treatment
including action to be taken after a missed dose or after a
gastrointestinal upset.

**D** - Adherence to treatment can be optimised with the following:

-   Educating individuals and their families and/or carers in the
    understanding of their condition and the rationale of treatment

-   Reducing the stigma associated with the condition

-   Using simple medication regimens

-   Positive relationships between healthcare professionals, the
    individual with epilepsy and their family and/or carers

**C** (adults) - Regular blood test monitoring in adults is not
recommended as routine, and should be done only if clinically indicated.

**GPP** (children) - Regular blood test monitoring in children is not
recommended as routine, and should be done only if clinically indicated
and recommended by the specialist.

**D** - Indications for monitoring of AED blood levels are:

-   Detection of non-adherence to the prescribed medication

-   Suspected toxicity

-   Adjustment of phenytoin dose

-   Management of pharmacokinetic interactions

-   Specific clinical conditions, for example, status epilepticus, organ
    failure, and pregnancy

**GPP** - Examples of blood tests include:

-   Before surgery - clotting studies in those on valproate

-   Full blood count, electrolytes, liver enzymes, vitamin D levels, and
    other tests of bone metabolism (for example, serum calcium and
    alkaline phosphatase) every 2 to 5 years for adults taking
    enzyme-inducing drugs

**GPP** - Asymptomatic minor abnormalities in test results are not
necessarily an indication for changes in medication.

**Withdrawal of Pharmacological Treatment**

**A** - The decision to continue or 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. At the end of the discussion individuals, and their family
and/or carers as appropriate, should understand the individual\'s risk
of seizure recurrence on and off treatment. This discussion should take
into account details of the individual\'s epilepsy syndrome, prognosis,
and lifestyle.

**GPP** - Withdrawal of AEDs must be managed by, or be under the
guidance of, the specialist.

**A** - The risks and benefits of continuing or withdrawing AED therapy
should be discussed with individuals, and their families and/or carers
as appropriate, who have been seizure free for at least 2 years (see
Appendix H in the original guideline document for tables for the
prognosis for remission of seizures in adults).

**D** - When AED treatment is being discontinued in an individual who
has been seizure free, it should be carried out slowly (at least 2 to 3
months) and one drug should be withdrawn at a time.

**GPP** - Particular care should be taken when withdrawing
benzodiazepines and barbiturates (may take up to 6 months or longer)
because of the possibility of drug-related withdrawal symptoms and/or
seizure recurrence.

**GPP** - There should be a failsafe plan agreed with individuals and
their families and/or carers as appropriate, whereby if seizures recur,
the last dose reduction is reversed and medical advice is sought.

**Referral for Complex or Refractory Epilepsy**

**GPP** - All individuals with epilepsy should have access via their
specialist to a tertiary service when circumstances require.

**C** - Information should be provided to individuals and families
and/or carers as appropriate about the reasons for considering surgery.
The benefits and risks of the surgical procedure under consideration
should be fully explained before the individual\'s informed consent is
obtained.

If seizures are not controlled and/or there is diagnostic uncertainty or
treatment failure, individuals should be referred to tertiary services
soon (within 4 weeks) for further assessment. Referral should be
considered when one or more of the following criteria are present:

> **D** - The epilepsy is not controlled with medication within 2 years
>
> **GPP** - Management is unsuccessful after two drugs
>
> **GPP** - The individual is aged under 2 years
>
> **GPP** - An individual experiences, or is at risk of, unacceptable
> side effects from medication
>
> **GPP** - There is a unilateral structural lesion
>
> **GPP** - There is psychological and/or psychiatric comorbidity
>
> **GPP** - There is diagnostic doubt as to the nature of the seizures
> and/or seizure syndrome

**D** (children) - In children, the diagnosis and management of epilepsy
within the first few years of life may be extremely challenging. For
this reason, children with suspected epilepsy should be referred to
tertiary services early, because of the profound developmental,
behavioural, and psychological effects that may be associated with
continuing seizures.

**GPP** - Behavioural or developmental regression or inability to
identify the epilepsy syndrome in an individual should result in
immediate referral to tertiary services.

**GPP** - Individuals with specific syndromes such as Sturge-Weber
syndrome, the hemispheric syndromes, Rasmussen\'s encephalitis, and
hypothalamic hamartoma should be referred to a tertiary epilepsy
service.

**GPP** - Psychiatric comorbidity and/or negative baseline
investigations should not be a contraindication for referral to a
tertiary centre.

**GPP** - The tertiary service should include a multidisciplinary team
experienced in the assessment of individuals with complex epilepsy and
have adequate access to investigations and treatment by both medical and
surgical means.

**GPP** - The expertise of multidisciplinary teams involved in managing
complex epilepsy should include psychology, psychiatry, social work,
occupational therapy, counselling, neuroradiology, clinical nurse
specialists, neurophysiology, neurology, neurosurgery, and
neuroanaesthesia. Teams should have MRI and video telemetry facilities
available to them.

**GPP** - The neurosurgeon in the multidisciplinary team should have
specialist experience of and/or training in epilepsy surgery and have
access to invasive EEG recording facilities.

**Psychological Interventions**

**A** (adults) - Psychological interventions (relaxation, cognitive
behaviour therapy, biofeedback) may be used in conjunction with AED
therapy in adults where either the individual or the specialist
considers seizure control to be inadequate with optimal AED therapy.
This approach may be associated with an improved quality of life in some
individuals.

**A** (children) - Psychological interventions (relaxation, cognitive
behaviour therapy) may be used in children with drug-resistant focal
epilepsy.

**A** - Psychological interventions may be used as adjunctive therapy.
They have not been proven to affect seizure frequency and are not an
alternative to pharmacological treatment.

**Ketogenic Diet**

**C** (adults) - The ketogenic diet should not be recommended for adults
with epilepsy.

**C** (children) - The ketogenic diet may be considered as an adjunctive
treatment in children with drug-resistant epilepsy.

**Vagus Nerve Stimulation (VNS)**

**A** (adults) - Vagus nerve stimulation is indicated for use as an
adjunctive therapy in reducing the frequency of seizures in adults who
are refractory to antiepileptic medication but who are not suitable for
resective surgery. This includes adults whose epileptic disorder is
dominated by partial seizures (with or without secondary generalisation)
or generalised seizures.

**A** (children) - Vagus nerve stimulation is indicated for use as an
adjunctive therapy in reducing the frequency of seizures in children who
are refractory to antiepileptic medication but who are not suitable for
resective surgery. This includes children whose epileptic disorder is
dominated by partial seizures (with or without secondary generalisation)
or generalised seizures.

**Prolonged or Repeated Seizures in the Community**

**A** - An individual who has prolonged convulsive seizures (lasting 5
minutes or more) or serial seizures (three or more seizures in an hour)
in the community should receive urgent care and treatment.

**A** - Rectal diazepam is safe and effective in first-line treatment of
prolonged seizures and is recommended in the majority of cases.

**GPP** - For many individuals and in many circumstances, buccal
midazolam\* is more acceptable than rectal diazepam and is easier to
administer. It should be used according to an agreed protocol drawn up
by the specialist and only used following training.

\***Note**: Buccal midazolam is currently unlicensed for the treatment
of prolonged or repeated seizures.

**GPP** - Healthcare professionals should inform individuals, and their
families and/or carers, that buccal midazolam is currently unlicensed.

**GPP** - Treatment should be administered by trained clinical personnel
or, if specified by an individually agreed protocol drawn up with the
specialist, by family members or carers with appropriate training.

**GPP** - Care must be taken to secure the individual\'s airway and
assess his or her respiratory and cardiac function.

**GPP** - Depending on response and the individual\'s situation,
emergency services should be contacted, 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 individual\'s condition

**Treatment of Status Epilepticus**

**Convulsive Status Epilepticus**

**GPP** - In hospital, individuals with generalised tonic-clonic status
epilepticus should be managed immediately, as follows (with local
protocols being in place - see suggested guideline in Appendix C of the
original guideline document):

-   Secure airway

-   Give oxygen

-   Assess cardiac and respiratory function

-   Secure intravenous (IV) access in a large vein

**D** - Lorazepam should be used as a first-line treatment in status
epilepticus (see Appendix C of the original guideline document).

**Refractory Convulsive Status Epilepticus**

**D** - Treatment of refractory status epilepticus in secondary care
should follow the suggested guidelines (see Appendix C of the original
guideline document).

**C** (adults) - In adults, propofol or thiopental should be used to
control refractory status epilepticus. Adequate monitoring, including
blood levels of thiopental, and critical life systems support is
required (see Appendix C of the original guideline document).

**C** (children) - In children, midazolam or thiopental should be used
to control refractory status epilepticus. Adequate monitoring, including
blood levels of thiopental, and critical life systems support is
required (see Appendix C of the original guideline document).

**GPP** - Regular medication should be continued at optimal doses and
the reasons for status epilepticus should be investigated.

**GPP** - As the treatment pathway progresses, the expertise of an
anaesthetist/intensivist should be sought.

**GPP** - If either the whole protocol or intensive care is required the
tertiary centre should be consulted.

**GPP** - An individual treatment pathway should be formulated for
people who have recurrent convulsive status epilepticus.

**Non-convulsive Status Epilepticus**

**GPP** - Non-convulsive status epilepticus is uncommon and management
is less urgent. A suggested guideline can be found in Appendix C of the
original guideline document.

**Women with Epilepsy**

**C** - In order to enable informed decisions and choice, and to reduce
misunderstandings, women with epilepsy and their partners, as
appropriate, must be given accurate information and counselling about
contraception, conception, pregnancy, caring for children and
breastfeeding, and menopause.

**C** - Information about contraception, conception, pregnancy, or
menopause should be given to girls and women in advance of sexual
activity, pregnancy, or menopause, and the information should be
tailored to their individual needs. This information should also be
given, as needed, to people who are closely involved with girls and
women with epilepsy. These may include an individual\'s family and/or
carers.

**GPP** - All healthcare professionals who treat, care for, or support
women with epilepsy should be familiar with relevant information and the
availability of counselling.

**A \[NICE\]** (adults) - In women of childbearing potential, the risk
of the drugs (see section on AEDs in the original guideline document)
causing harm to an unborn child should be discussed and an assessment
made as to the risks and benefits of treatment with individual drugs.
There are currently few data on which to base a definitive assessment of
the risks to the unborn child associated with newer drugs. Specific
caution is advised in the use of sodium valproate because of the risk of
harm to the unborn child.

**A \[NICE\]** (children) - In girls of childbearing potential,
including young girls who are likely to need treatment into their
childbearing years, the risk of the drugs (see section on AEDs in the
original guideline document) causing harm to an unborn child should be
discussed with the child and/or her carer, and an assessment made as to
the risks and benefits of treatment with individual drugs. There are
currently few data on which to base a definitive assessment of the risks
to the unborn child associated with newer drugs. Specific caution is
advised in the use of sodium valproate because of the risk of harm to
the unborn child.

**GPP** - Prescribers should be aware of the latest data on the risks to
the unborn child associated with AED therapy when prescribing for women
and girls of childbearing potential.

**D** - All women on AEDs should be offered 5 mg per day of folic acid
before any possibility of pregnancy.

**A \[NICE\]** (adult) - In women of childbearing potential, the
possibility of interaction with oral contraceptives should be discussed
and an assessment made as to the risks and benefits of treatment with
individual drugs.

**A \[NICE\]** (children) - In girls of childbearing potential,
including young girls who are likely to need treatment into their
childbearing years, the possibility of interaction with oral
contraceptives should be discussed with the child and/or her carer, and
an assessment made as to the risks and benefits of treatment with
individual drugs.

**GPP** - In women of childbearing potential, the risks and benefits of
different contraceptive methods, including hormone-releasing
intra-uterine devices (IUDs), should be discussed.

**D** - If a woman taking enzyme-inducing AEDs chooses to take the
combined oral contraceptive pill, a minimum initial dose of 50
micrograms of oestrogen is recommended. If breakthrough bleeding occurs,
the dose of oestrogen should be increased to 75 micrograms or 100
micrograms per day, and \"tricycling\" (taking three packs without a
break) should be considered.

**D** - The progesterone-only pill is not recommended as reliable
contraception in women taking enzyme-inducing AEDs.

**D** - Women taking enzyme-inducing AEDs who choose to use depot
injections of progesterone should be informed that a shorter repeat
injection interval is recommended (10 weeks instead of 12 weeks).

**D** - The progesterone implant is not recommended in women taking
enzyme-inducing AEDs.

**GPP** - The use of additional barrier methods should be discussed with
women taking enzyme-inducing AEDs and oral contraception or having depot
injections of progesterone.

**D** - If emergency contraception is required for women taking
enzyme-inducing AEDs, the dose of levonorgestrel should be increased to
1.5 mg and 750 micrograms 12 hours apart.

**Pregnancy**

**C** - Women with epilepsy need accurate information during pregnancy,
and the possibility of status epilepticus and SUDEP should be discussed
with all women who plan to stop AED therapy (see sections on withdrawal
in the original guideline document).

**GPP** - All pregnant women with epilepsy should be encouraged to
notify their pregnancy, or allow their clinician to notify the
pregnancy, to the United Kingdom (UK) Epilepsy and Pregnancy Register
([www.epilepsyandpregnancy.co.uk](http://www.epilepsyandpregnancy.co.uk/)).

**GPP** - In all women with epilepsy, seizure freedom during pregnancy
should be sought.

**GPP** - The clinician should discuss with the woman the relative
benefits and risks of adjusting medication to enable her to make an
informed decision. Where appropriate, the woman\'s specialist should be
consulted.

**D** - Women with generalised tonic-clonic seizures should be informed
that the fetus may be at relatively higher risk of harm during a
seizure, although the absolute risk remains very low, and the level of
risk may depend on seizure frequency.

**D** - Women should be reassured that there is no evidence that simple
partial, complex partial, absence, and myoclonic seizures affect the
pregnancy or developing fetus adversely unless they fall and sustain an
injury.

**B** - Women should be reassured that an increase in seizure frequency
is generally unlikely in pregnancy or in the first few months after
birth.

**C** - Generally, women may be reassured that the risk of a
tonic-clonic seizure during the labour and the 24 hours after birth is
low (1 to 4%).

**D** - Routine monitoring of AED levels in pregnancy is not
recommended. If seizures increase or are likely to increase, monitoring
of AED levels may be useful to plan or anticipate the extent of change
of dose adjustment needed.

**B** - Women with epilepsy should be informed that although they are
likely to have healthy pregnancies, their risk of complications during
pregnancy and labour is higher than for women without epilepsy.

**GPP** - Care of pregnant women should be shared between the
obstetrician and the specialist.

**GPP** - Pregnant women who are taking AEDs should be offered a
high-resolution ultrasound scan to screen for structural anomalies. This
scan should be performed at 18 to 20 weeks\' gestation by an
appropriately trained ultrasonographer, but earlier scanning may allow
major malformations to be detected sooner.

**GPP** - The risk of seizures during labour is low, but it is
sufficient to warrant the recommendation that delivery should take place
in an obstetric unit with facilities for maternal and neonatal
resuscitation and treating maternal seizures.

**C** - All children born to mothers taking enzyme-induced AEDs should
be given 1 mg of vitamin K parenterally at delivery.

**D** - Genetic counselling should be considered if one partner has
epilepsy, particularly if the partner has idiopathic epilepsy and a
positive family history of epilepsy.

**GPP** - Although there is an increased risk of seizures in children of
parents with epilepsy, individuals with epilepsy should be given
information that the probability that a child will be affected is
generally low. However, this will depend on the family history.

**GPP** - Advanced planning, including the development of local
protocols for care, should be implemented in obstetric units that
deliver babies of women with epilepsy.

**GPP** - Joint epilepsy and obstetric clinics may be convenient for
mothers and healthcare professionals but there is insufficient evidence
to recommend their routine use.

**GPP** - It is, however, important that there should be regular
follow-up, planning of delivery, and liaison between the specialist or
epilepsy team and the obstetrician or midwife.

**Breastfeeding**

**GPP** - All women with epilepsy should be encouraged to breastfeed,
except in very rare circumstances. Breastfeeding for most women taking
AEDs is generally safe and should be encouraged. However, each mother
needs to be supported in the choice of feeding method that best suits
her and her family.

**GPP** - Prescribers should consult Appendix 5 of the British National
Formulary when prescribing AEDs for women who are breastfeeding. The
decision on whether to continue AED therapy should be made between the
woman and the prescriber and should be based on the risks and benefits
of breastfeeding against the potential risks of the drug affecting the
child.

**After the Birth**

**GPP** - Parents of new babies or young children should be informed
that introducing a few simple safety precautions may significantly
reduce the risk of accidents and minimise anxiety. An approaching birth
can be an ideal opportunity to review and consider the best and most
helpful measures to start to ensure maximum safety for both mother and
baby.

**C** - Information should be given to all parents about safety
precautions to be taken when caring for the baby (see Appendix D of the
full guideline).

**C** - Parents should be reassured that the risk of injury to the
infant caused by maternal seizure is low.

**People with Learning Disabilities**

**GPP** - People with learning disabilities should receive the same
support and care for their epilepsy as the general population. In
addition, those with learning disabilities need the care of the learning
disabilities team.

**C** - Learning disabilities are a common association with epilepsy.
The management and treatment of the epilepsy should be undertaken by a
specialist, working within a multidisciplinary team.

**Diagnosis**

**C** - It can be difficult to diagnose epilepsy in people with learning
disabilities, and so care should be taken to obtain a full clinical
history. Confusion may arise between stereotypic or other behaviours and
seizure activity.

**D** - It is important to have an eye witness account supplemented by
corroborative evidence (for example, a video account), where possible.

**GPP** - Clear, unbiased reporting is essential. Witnesses may need
education to describe their observations accurately.

**Investigations**

**GPP** - Those with learning disabilities may require particular care
and attention to tolerate investigations.

**D** - Facilities should be available for imaging under anaesthesia, if
necessary.

**C** (children) - In the child presenting with epilepsy and learning
disability, investigations directed at determining an underlying cause
should be undertaken.

**Management**

**D** - In making a management plan for an individual with learning
disabilities and epilepsy, particular attention should be paid to the
possibility of adverse cognitive and behavioural effects of AED therapy.

**A \[NICE\]** - The recommendations on choice of treatment and the
importance of regular monitoring of effectiveness and tolerability are
the same for those with learning disabilities as for the general
population.

**B** - Every therapeutic option should be explored in individuals with
epilepsy in the presence or absence of learning disabilities.

**GPP** - Healthcare professionals should be aware of the higher risks
of mortality for people with learning disabilities and epilepsy and
discuss these with individuals, their families, and/or carers.

**C** - All individuals with epilepsy and learning disabilities should
have a risk assessment including:

-   Bathing and showering

-   Preparing food

-   Using electrical equipment

-   Managing prolonged or serial seizures

-   The impact of epilepsy in social settings

-   SUDEP

-   The suitability of independent living, where the rights of the
    individual are balanced with the role of the carer

**Young People with Epilepsy**

**C** - The physical, psychological, and social needs of young people
with epilepsy should always be considered by healthcare professionals.
Attention should be paid to their relationships with family and friends,
and at school.

**GPP** - Healthcare professionals should adopt a consulting style that
allows the young person with epilepsy to participate as a partner in the
consultation.

**GPP** - Decisions about medication and lifestyle issues should draw on
both the expertise of the healthcare professional and the experiences,
beliefs, and wishes of the young person with epilepsy as well as their
family and/or carers.

**GPP** - During adolescence a named clinician should assume
responsibility for the ongoing management of the young person with
epilepsy and ensure smooth transition of care to adult services, and be
aware of the need for continuing multi-agency support.

**D** - Multidisciplinary services provided jointly by adult and
paediatric specialists have a key role in the care of the young person
with epilepsy. This can facilitate the transition from paediatric to
adult services and aid in the dissemination of information.

**D** - Before the transition to adult services is made, diagnosis and
management should be reviewed and access to voluntary organisations,
such as support groups and epilepsy charities, should be facilitated.

**D** - The information given to young people should cover epilepsy in
general and its diagnosis and treatment, the impact of seizures and
adequate seizure control, treatment options including side effects and
risks, and the risks of injury. Other important issues to be covered are
the possible consequences of epilepsy on lifestyle and future career
opportunities and decisions, driving and insurance issues, social
security and welfare benefit issues, sudden death and the importance of
adherence to medication regimes. Information on lifestyle issues should
cover recreational drugs, alcohol, sexual activity, and sleep
deprivation.

**D** - The diagnosis and management of epilepsy should be reviewed
during adolescence.

**Older People with Epilepsy**

**A \[NICE\]** - The recommendations on choice of treatment and the
importance of regular monitoring of effectiveness and tolerability are
the same for older people as for the general population.

**People from Black and Minority Ethnic Groups**

**D** - People from black and minority ethnic groups may have different
cultural and communication needs and these should be considered during
diagnosis and management. The need for interpretation should be
considered alongside other means of ensuring that an individual\'s needs
are appropriately met.

**D** - An interpreter should have both cultural and medical knowledge.
Interpreters from the family are generally not suitable because of
issues such as confidentiality, privacy, personal dignity, and accuracy
of translation.

**D** - Information, including information about employment rights and
driving, should be available in an appropriate format or through other
appropriate means for people who do not speak or read English.

**Review**

**D** - Adults and children with epilepsy should have a regular
structured review and be registered with a general medical practice.

**D** (adults) - Adults should have a regular structured review with
their general practitioner (GP), but depending on the individual\'s
wishes, circumstances, and epilepsy, the review may be carried out by
the specialist.

**D** (children) - Children should have a regular structured review with
a specialist.

**D** (adults) - For adults, the maximum interval between reviews should
be 1 year but the frequency of review will be determined by the
individual\'s epilepsy and their wishes.

**GPP** (children) - For children, the maximum interval between reviews
should be 1 year, but the frequency of reviews should be determined by
the individual\'s epilepsy and their wishes and those of the family
and/or carers. The interval between reviews should be agreed between the
individual, their family and/or carers as appropriate, and the
specialist, but is likely to be between 3 and 12 months.

**D** (adults) - Adults should have regular reviews. In addition, access
to either secondary or tertiary care should be available to ensure
appropriate diagnosis, investigation, and treatment if the individual or
clinician view the epilepsy as inadequately controlled.

**D** (adults) - Adults with well-controlled epilepsy may have specific
medical or lifestyle issues (for example, pregnancy or drug cessation)
that may need the advice of a specialist.

**D** - If the structured review is to be conducted by the specialist,
this may be best provided in the context of a specialist clinic.

**A \[NICE\]** - Treatment should be reviewed at regular intervals to
ensure that individuals with epilepsy are not maintained for long
periods on treatment that is ineffective or poorly tolerated and that
concordance with prescribed medication is maintained.

**GPP** - Annual review should include an enquiry about side effects and
a discussion of the treatment plan to ensure concordance and adherence
to medication

**D** - 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, and referral to tertiary services including surgery,
where appropriate.

**Definitions**:

**Levels of Evidence**

**Ia** - Systematic review or meta-analysis of randomized controlled
trials

**Ib** - At least one randomized controlled trial

**IIa** - At least one well-designed controlled study without
randomization

**IIb** - At least one well-designed quasi-experimental study, such as a
cohort study

**III** - Well-designed non-experimental descriptive studies,
case-control studies, and case series

**IV** - Expert committee reports, opinions and/or clinical experience
of respected authorities

**NICE** - NICE guidelines or Health Technology Appraisal programme

**Grades of Recommendations**

**A** - Based directly on level I evidence

**B** - Based directly on level II evidence or extrapolated from level I
evidence

**C** - Based directly on level III evidence or extrapolated from level
I or level II evidence

**D** - Based directly on level IV evidence or extrapolated from level
I, level II, or level III evidence

**A (NICE)** - Recommendation taken from NICE guideline or Technology
Appraisal

**GPP** - Good practice points based on the clinical experience of the
GDG

#### CLINICAL ALGORITHM(S)

Algorithms are provided in the original guideline document for the
outline of care for adults and for children.

### EVIDENCE SUPPORTING THE RECOMMENDATIONS

#### TYPE OF EVIDENCE SUPPORTING THE RECOMMENDATIONS

The type of supporting evidence is provided for each recommendation (see
\'Major Recommendations\' field).

### BENEFITS/HARMS OF IMPLEMENTING THE GUIDELINE RECOMMENDATIONS

#### POTENTIAL BENEFITS

-   The correct classification of seizure type and epilepsy syndrome

-   Appropriate investigations, appropriate treatment, and information
    about the likely prognosis of the seizure type and/or syndrome

-   Reduced mortality and morbidity

-   Improved quality of life

#### POTENTIAL HARMS

**Diagnosis**

Individuals misdiagnosed with epilepsy may experience social and
financial deprivation as a result of having the wrong diagnostic label
and from side-effects of antiepileptic medication. In addition, there
may be a risk of unnecessary teratogenicity as a result of
Anti-epileptic drug (AED) therapy in women incorrectly diagnosed as
having epilepsy. In a small number of cases, individuals may die
prematurely because the correct diagnosis was not made, and a serious
condition was neither diagnosed nor treated. Individuals who have
symptoms due to epileptic seizures but who are wrongly diagnosed as
having psychiatric or associated disorders are disadvantaged from being
labeled with an incorrect diagnosis and by the effects of continuing
seizure activity because AEDs are not used. It is therefore crucial that
specialists involved in diagnosing epilepsy take great care to establish
the correct diagnosis.

**Side Effects of Drug Treatments**

-   Refer to Table 4 in Appendix B for side effects of drug treatment
    for adults.

-   Refer to Table 5 in Appendix B for side effects of drug treatment
    for children.

### QUALIFYING STATEMENTS

#### QUALIFYING STATEMENTS

The guideline documentation and recommendations are subject to various
limitations. The National Institute for Clinical Excellence (NICE), the
commissioner of this work, is primarily concerned with the National
Health Service in England and Wales and is not able to make
recommendations for practice outside the National Health Service (NHS).
It is important to stress that social services, educational services and
the voluntary sector have an important role to play in the care of
people with epilepsy and this guideline is highly relevant to these
agencies.

### IMPLEMENTATION OF THE GUIDELINE

#### DESCRIPTION OF IMPLEMENTATION STRATEGY

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 original guideline document and the
\"Major Recommendations\" section of this summary, the people and
processes involved, and the timeline over which full implementation is
envisaged. It is in the interests of people 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 this guidance and revised accordingly.

This guideline should be used in conjunction with the National Service
Frameworks for children, older people, and for long-term neurological
conditions.

Suggested audit criteria are listed in Section 6 of the original
guideline document. These can be used as the basis for local clinical
audit, at the discretion of those in practice.

The following have been identified as key priorities for implementation

**Diagnosis**

-   All individuals with a recent onset suspected seizure should be seen
    urgently^a^ by a specialist^b^. 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
    comorbidity should be determined.

**Management**

-   Healthcare professionals should adopt a consulting style that
    enables the individual 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 individuals 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 anti-epileptic drug (AED) treatment strategy should be
    individualised according to the seizure type, epilepsy syndrome,
    comedication and comorbidity, the individual\'s lifestyle, and the
    preferences of the individual, their family and/or carers as
    appropriate.

**Review and Referral**

-   All individuals with epilepsy should have a regular structured
    review. In children, this review should be carried out at least
    yearly (but may be between 3 and 12 months by arrangement) by a
    specialist. 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 if
    appropriate.

-   If seizures are not controlled and/or there is diagnostic
    uncertainty or treatment failure, individuals should be referred to
    tertiary services soon^c^ 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
being seen within 2 weeks.

^b^ For adults, a specialist is defined throughout as a medical
practitioner with training and expertise in epilepsy. For children, a
specialist is defined throughout as a paediatrician with training and
expertise in epilepsy.

^c^ The Guideline Development Group considered that \"soon\" meant being
seen within 4 weeks.

#### IMPLEMENTATION TOOLS

Audit Criteria/Indicators\
Clinical Algorithm\
Patient Resources\
Quick Reference Guides/Physician Guides

For information about
[availability](#identifying-information-and-availability), see the
\"Availability of Companion Documents\" and \"Patient Resources\" fields
below.

### INSTITUTE OF MEDICINE (IOM) NATIONAL HEALTHCARE QUALITY REPORT CATEGORIES

#### IOM CARE NEED

Getting Better\
Living with Illness

#### IOM DOMAIN

Effectiveness\
Patient-centeredness

### IDENTIFYING INFORMATION AND AVAILABILITY

#### BIBLIOGRAPHIC SOURCE(S)

National Collaborating Centre for Primary Care. The diagnosis and
management of the epilepsies in adults and children in primary and
secondary care. London (UK): Royal College of General Practitioners;
2004 Oct. 525 p. \[324 references\]

#### ADAPTATION

Not applicable: The guideline was not adapted from another source.

#### DATE RELEASED

2004 Oct

#### GUIDELINE DEVELOPER(S)

National Collaborating Centre for Primary Care - National Government
Agency \[Non-U.S.\]

#### SOURCE(S) OF FUNDING

National Institute for Clinical Excellence (NICE)

#### GUIDELINE COMMITTEE

Guideline Development Group

#### COMPOSITION OF GROUP THAT AUTHORED THE GUIDELINE

*Committee Members*: Ms Kathy Bairstow, Patient Representative, Leeds;
Ms Bernie Concannon, Clinical Nurse Specialist Paediatric Epilepsy,
Birmingham Children\'s Hospital; Mr Ian Costello, Chief Pharmacist,
Centre for Paediatric Research, School of Pharmacy, London; Dr Helen
Cross, Senior Lecturer & Honorary Consultant in Paediatric Neurology,
Institute of Child Health and Great Ormond Street Hospital for Children,
London; Professor John Duncan, Professor of Neurology, The National
Hospital for Neurology and Neurosurgery, London; Dr Amanda Freeman,
Consultant Paediatrician, St Mary\'s Hospital, Portsmouth; Ms Sally
Gomersall, Patient Representative, Newark; Ms Jane Hanna, Patient
Representative, Wantage; Mr William Harkness, Consultant Neurological
Surgeon, Great Ormond Street Hospital for Children, London; Dr Peter
Humphrey, Consultant Neurologist, The Walton Centre for Neurology &
Neurosurgery, Liverpool; Dr Tanzeem Raza, Consultant Physician, Royal
Bournemouth Hospital; Mr Peter Rogan, Patient Representative, Ormskirk;
Dr Henry Smithson, Guideline Development Group Lead General
Practitioner, York

#### FINANCIAL DISCLOSURES/CONFLICTS OF INTEREST

All Guideline Development Group (GDG) members made a formal
\"Declaration of Interests\" at the start of the guideline development
and provided updates throughout the development process.

#### GUIDELINE STATUS

This is the current release of the guideline.

#### GUIDELINE AVAILABILITY

Electronic copies: Available in Portable Document Format \[PDF\] format
from the [National Institute for Clinical Excellence (NICE) Web
site](http://www.nice.org.uk/page.aspx?o=227586).

#### AVAILABILITY OF COMPANION DOCUMENTS

The following are available:

-   The epilepsies: the diagnosis and management of the epilepsies in
    adults and children in primary and secondary care. NICE guideline.
    2004 Oct. 73 p. Available in Portable Document Format (PDF) from the
    [National Institute for Clinical Excellence (NICE) Web
    site](http://www.nice.org.uk/page.aspx?o=229249).

-   The epilepsies: diagnosis and management of the epilepsies in adults
    in primary and secondary care. Quick reference guide. 2004 Oct.
    18 p. Available in Portable Document Format (PDF) from the [National
    Institute for Clinical Excellence (NICE) Web
    site](http://www.nice.org.uk/page.aspx?o=228024).

-   The epilepsies: diagnosis and management of the epilepsies in
    children and young people in primary and secondary care. Quick
    reference guide. 2004 Oct. 18 p. Available in Portable Document
    Format (PDF) from the [National Institute for Clinical Excellence
    (NICE) Web site](http://www.nice.org.uk/page.aspx?o=228042).

Print copies: Available from the National Health Service (NHS) Response
Line 0870 1555 455. ref: N0739 and N0740. 11 Strand, London, WC2N 5HR.

Additionally, Audit Criteria can be found in Section 6 of the [original
guideline document](http://www.nice.org.uk/page.aspx?o=227586).

#### PATIENT RESOURCES

The following is available:

-   The diagnosis and care of children and adults with epilepsy.
    Understanding NICE guidance information for people with epilepsy,
    their families and carers, and the public. London: National
    Institute for Clinical Excellence. 2004 Oct. 68 p. Available in
    Portable Document Format (PDF) from the [National Institute for
    Clinical Excellence (NICE) Web
    site](http://www.nice.org.uk/page.aspx?o=228044).

Print copies: Available from the National Health Service (NHS) Response
Line 0870 1555 455. ref: N0741. 11 Strand, London, WC2N 5HR.

Please note: This patient information is intended to provide health
professionals with information to share with their patients to help them
better understand their health and their diagnosed disorders. By
providing access to this patient information, it is not the intention of
NGC to provide specific medical advice for particular patients. Rather
we urge patients and their representatives to review this material and
then to consult with a licensed health professional for evaluation of
treatment options suitable for them as well as for diagnosis and answers
to their personal medical questions. This patient information has been
derived and prepared from a guideline for health care professionals
included on NGC by the authors or publishers of that original guideline.
The patient information is not reviewed by NGC to establish whether or
not it accurately reflects the original guideline\'s content.

#### NGC STATUS

This NGC summary was completed by ECRI on February 7, 2005. The
information was verified by the guideline developer on March 23, 2005.
This summary was updated by ECRI on April 21, 2005 following the release
of a public health advisory from the U.S. Food and Drug Administration
(FDA) regarding Trileptal (oxcarbazepine). This summary was updated by
ECRI on November 16, 2006, following the FDA advisory on Lamictal
(lamotrigine). This summary was updated by ECRI Institute on January 10,
2008, following the U.S. Food and Drug Administration advisory on
Carbamazepine.

#### COPYRIGHT STATEMENT

This NGC summary is based on the original guideline, which is subject to
the guideline developer\'s copyright restrictions.

### DISCLAIMER

#### NGC DISCLAIMER

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Date Modified: 11/10/2008