• December 12, 2007, 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.

GPP - Healthcare professionals should highlight the Expert Patients Programme (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).

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).

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

For information about availability, see the "Availability of Companion Documents" and "Patient Resources" fields below.

Electronic copies: Available in Portable Document Format [PDF] format from the National Institute for Clinical Excellence (NICE) Web site.

• 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.
• 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.
• 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.

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.

• 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.

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

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