This article provides a summary of the SIGN guidance on the diagnosis and management of epilepsy. Continue reading for information on:
- Models of care
- Diagnosis, classification and investigation
- Status epilepticus
- Epilepsy and women's health
- Epilepsy in older people
- Epilepsy in people with learning disability
- Psychiatric comorbidity
- Mortality in epilepsy
- Information for patients and carers.
The following recommendations were highlighted by the SIGN guideline development group as the key clinical recommendations that should be prioritised for implementation.
The grade of recommendation ([A],[B],[C],[D]) relates to the strength of the supporting evidence on which the recommendation is based. It does not reflect the clinical importance of the recommendation. Good practice points are marked with a [✓].
See the full guideline for a key to grades of recommendation.
Diagnosis, Classification, and Investigation
- The diagnosis of epilepsy has important physical, psychosocial and economic implications for the patient. It is therefore important that the diagnosis is correct. It has been shown that a significant proportion of epilepsy diagnoses made by non-specialists are incorrect
- Epilepsy may be difficult to diagnose in the early stages, especially in the absence of a witnessed account. It is important to make the distinction between genetic generalised epilepsies and focal epilepsies, as this affects treatment choices, investigation, prognosis and counselling. Electroencephalogram (EEG) can aid classification of epileptic seizures and epilepsy syndromes.
- [C] The diagnosis of epilepsy should be made by an epilepsy specialist
- [C] A clear history from the patient and an eyewitness to the attack give the most important diagnostic information, and should be the mainstay of diagnosis.
- [C] The seizure type(s) and epilepsy syndrome should be identified
- [C] The distinction should be made between a focal epilepsy and a genetic generalised epilepsy.
- [C] EEG is not routinely indicated and cannot exclude a diagnosis of epilepsy
- [C] EEG should be used to support the classification of epileptic seizures and epilepsy syndromes when there is clinical doubt
- [C] EEG should be performed in young people with generalised seizures to aid classification and to detect a photoparoxysmal response
- [B] Short-term video-EEG, preferably with suggestion, should be available for the investigation and diagnosis of suspected epilepsy and non-epileptic attack disorder
- [C] Inpatient video EEG and other specialist investigations (including polysomnography with full EEG montages) should be available for patients who present diagnostic difficulties.
- [C] MRI is the modality of choice for brain imaging in patients with epilepsy
- [C] Brain imaging is not routinely required when there is a confident diagnosis of a genetic generalised epilepsy
- [D] CT has a role in the urgent assessment of seizures, or when MRI is contraindicated.
When to Start Antiepileptic Treatment
- [B] The decision to start antiepileptic drugs (AEDs) should be made by the patient and an epilepsy specialist
- AEDs should be offered after a first tonic-clonic seizure if:
- [B] the patient has had previous myoclonic, absence or focal seizures
- [B] the EEG shows unequivocal epileptic discharges
- [B] the patient has a structural cerebral disorder
- [D] the patient considers the risk of recurrence unacceptable
- Use of sodium valproate must take into account MHRA safety advice, issued in April 2018, on use of valproate medicines in women and girls of childbearing potential and the conditions of the Pregnancy Prevention Programme (advice available at gov.uk/drug-safety-update/valproate-medicines).
Choice of Formulation
- [A] In patients with focal onset seizures, lamotrigine is the drug of choice. Where lamotrigine is poorly tolerated, carbamazepine, and levetiracetam may be reasonable alternatives
- [A] In genetic generalised epilepsy or unclassified epilepsy, sodium valproate is the most effective antiepileptic drug
- [A] where sodium valproate is poorly tolerated or contraindicated, lamotrigine and topiramate are suitable alternatives
- [D] in women of childbearing age, levetiracetam or lamotrigine may be a reasonable alternative
- [C] Routine switching between different manufacturers of antiepileptic drugs should be avoided
- [✓] The adverse effect and interaction profiles should direct the choice of drug for the individual patient
- [✓] Sodium valproate should not be used in women and girls of childbearing potential unless there is no suitable alternative and a Pregnancy Prevention Programme is in place.
Management of Drug-resistant Epilepsy
- [C] Failure to respond to appropriate AEDs should prompt a review of the diagnosis of epilepsy and adherence to medication
- [D] Combination therapy should be considered when treatment with two first-line AEDs has failed or improved control occurs during the process of phased substitution
- [A] Carbamazepine, gabapentin, lacosamide, lamotrigine, levetiracetam, oxcarbazepine, perampanel, pregabalin, topiramate, sodium valproate, and zonisamide may be used in the adjunctive treatment of focal epilepsy
- [A] Lamotrigine, levetiracetam, ethosuximide, sodium valproate, and topiramate may be used in the adjunctive treatment of generalised epilepsy
- [B] The choice of drugs in combination should be matched to the patient's seizure type(s) and should, where possible, be limited to two or at most three AEDs
- [✓] Sodium valproate should not be used in women and girls of childbearing potential unless there is no suitable alternative and a Pregnancy Prevention Programme is in place.
Antiepileptic Drug Blood Levels
- [D] Routine monitoring of AED concentrations is not indicated. Measurement can sometimes be useful in the following circumstances:
- adjustment of phenytoin dose
- assessment of adherence
- assessment of toxicity
- situations where drug metabolism is likely to change, for example pregnancy
- otherwise unexplained loss of seizure control
- [✓] Antiepileptic drug blood level measurement is best supervised by an epilepsy specialist.
Management of Provoked Seizures
- [✓] When seizures are provoked by metabolic disturbances or drugs, attention should be directed to correction or withdrawal of the provocative factor
- [✓] Patients with seizures provoked by alcohol or substance misuse may benefit from referral to addiction services and other support agencies
- [B] Following an acute brain insult or neurosurgery, long-term prophylactic AED treatment is not indicated
- [C] Following an acute brain insult, antiepileptic drugs used to treat the provoked seizures should be withdrawn (unless unprovoked seizures occur later)
- [D] AED treatment is not indicated for concussive convulsions.
Antiepileptic Drug Adverse Effects
- [✓] AEDs should be commenced in a dose no higher than recommended by the manufacturer
- [C] Patients should be warned of common potential adverse effects and given clear instructions to seek medical attention urgently for symptoms including rash, bruising or somnolence with vomiting especially in the first weeks of treatment
- [D] Liver function and full blood count should not be monitored routinely
- [C] Patients taking AEDs should receive dietary and other lifestyle advice to minimise the risk of osteoporosis
- [B] The potential negative psychotropic effects of AEDs should be borne in mind when deciding on the most appropriate AED for an individual patient
- [✓] AED treatment should be used with caution in those with pre-existing behavioural or psychiatric conditions and epilepsy.
Antiepileptic Drug Withdrawal
- [A] Prognostic index indicators can be used to give an estimate of the risks of seizure recurrence following AED withdrawal
- [✓] The question of AED withdrawal should be discussed with people with epilepsy who are at least two years seizure free, so that they can make an informed choice. Factors to be discussed should include driving, employment, fear and risks of further seizures and concerns about prolonged antiepileptic drug treatment
- [✓] The rate of withdrawal of antiepileptic drugs should be slow, usually over a few months, and longer with barbiturates and benzodiazepines. One drug should be withdrawn at a time.
- [B] Referral for assessment for neurosurgical treatment should be considered if the epilepsy is drug resistant
- [D] Assessment as to suitability for a potentially curative resective procedure should be made before consideration of palliative procedures such as vagus nerve stimulation
- [C] Vagus nerve stimulation may be considered in adult patients who have been found to be unsuitable for resective surgery.
Management of Prolonged Seizures Including Status Epilepticus
- Emergency treatment should be sought or given once a seizure has persisted, or there are serial seizures, for five minutes or more
- Generalised tonic-clonic status epilepticus is a medical emergency with significant morbidity and a mortality of between 16 and 39%. Both morbidity and mortality can be exacerbated by inadequate or delayed treatment
- [D] EEG should be used for confirming diagnosis of and monitoring treatment effect in patients with status epilepticus. EEG should be available as an emergency intervention for all patients with treated or suspected status epilepticus
- [✓] Non-availability of EEG should not deter or delay treatment of patients with status epilepticus.
- [✓] As soon as possible:
- secure airway
- give oxygen
- assess cardiac and respiratory function
- secure intravenous (IV) access in large veins
- [B] Patients with prolonged tonic-clonic seizures that have lasted five minutes or more should be given:
- midazolam 10 mg buccally or intranasally, or
- lorazepam 4 mg IV if midazolam is unavailable, or
- diazepam 10 mg IV or rectally if midazolam and lorazepam are unavailable.
In-hospital Treatment (Following Failure of Initial Benzodiazepine)
- The use of sodium valproate is contraindicated in pregnancy and in women and girls of childbearing potential (see April 2018 safety advice from MHRA). In an emergency, the balance of risks and benefits means that the prime consideration is to provide optimal and rapid control of seizures.
- For maintenance or long-term treatment, the MHRA safety advice, issued in April 2018, on use of valproate medicines in women and girls of childbearing potential should be followed where possible. Specialist review of women and girls of childbearing potential currently using sodium valproate should be initiated following successful resolution of the seizure and continued use of sodium valproate in this group should be avoided
- [B] Administer a repeat dose of benzodiazepine in hospital after 10 minutes if there is no response
- [✓] collect blood for a full blood count, urea and electrolytes, liver function tests, calcium, glucose, clotting, AED levels, and storage for later analyses
- [✓] measure blood gases to assess extent of acidosis
- [✓] establish aetiology. Give 50 ml of 50% glucose IV if there is any suggestion of hypoglycaemia and IV thiamine (given as Pabrinex®, two pairs of ampoules) if there is any suggestion of alcohol misuse or impaired nutritional status
- [✓] For sustained control in patients with established epilepsy give the usual AED treatment orally or by nasogastric tube (or IV if necessary for phenytoin, sodium valproate, phenobarbital, levetiracetam, or lacosamide)
- [D] Within 30 minutes, if seizures continue:
- give sodium valproate 20–30 mg/kg IV 40 mg/min or phenytoin 18 mg/kg IV 50 mg/min with ECG monitoring. Rates of phenytoin infusion may need to be reduced if hypotension or arrhythmia occur in older people or where there is renal/hepatic impairment
- [✓] If the patient remains unresponsive after initial treatment, EEG should be utilised to differentiate between continued seizures and drug-induced sedation
- [✓] Clear policies should be in place to avoid confusion between doses, formulations, routes and rates of administration of fosphenytoin and phenytoin
- [D] If status persists, then within 60 minutes:
- admit the patient to an intensive treatment unit and administer general anaesthesia
- refer for specialist advice
- [D] EEG should be used to determine response to treatment.
Management of Older People with Epilepsy
- [✓] Any older person developing new-onset seizures should undergo cognitive function screening and assessment for the presence of cerebrovascular risk factors, with appropriate management thereafter
- [✓] When choosing an antiepileptic drug for an older person with newly diagnosed epilepsy, consideration of the following is paramount:
- adverse effect profile
- appropriate formulation
- dosing regimen in those with adherence issues
- drug interactions
- low starting dose
- slow titration schedule
- low maintenance dosing
- [B] Lamotrigine or possibly levetiracetam should be considered when starting AED treatment in older people with focal-onset seizures
- [C] Gabapentin is an alternative mono- or adjunctive therapy option in older people with epilepsy
- [✓] For older people with cognitive problems, an epilepsy care plan should be considered.
Epilepsy in People with Learning Disability
- [D] People with learning disability should be treated with the same range of AEDs as the general population
- [✓] In the management of people with learning disability and epilepsy:
- adequate time should be allowed for the consultation
- the carer should know the patient and bring relevant information on seizure type, frequency, possible adverse effects of medication, general health and behaviour to the consultation
- information in an accessible form should be available to patients and carers
- there should be a multidisciplinary approach to treatment, delivered by professionals with an expertise in epilepsy, to improve quality of life
- community learning disability nurses have an important role in liaising between the specialist services and patients and carers
- [✓] In people with Down’s syndrome and dementia who develop seizures, quality of life, including negative impact of all seizure types and medication adverse effects, should guide treatment
- [✓] The quality of information brought to appointments is highly variable and therefore the validity of this information should be thoroughly checked
Epilepsy and Women's Health
- Women with epilepsy of childbearing potential need advice about contraception and pregnancy as well as information about epilepsy management. Those who have received such advice are likely to have more reliable contraception, better health during pregnancy and improved pregnancy outcomes
- In addition to seizure type or syndrome, the choice of AED for women may be influenced by factors including potential teratogenicity, interactions with hormonal methods of contraception, and potential cosmetic adverse effects
- [✓] Advice on contraception should be given to young women, ideally before they become sexually active
- [✓] For women with epilepsy not receiving antiepileptic drugs and for those receiving antiepileptic drugs that do not induce hepatic enzymes (other than lamotrigine), contraceptive options are the same as those for women in general
- [C] To minimise the risk of contraceptive failure, a woman using any combined hormonal contraception, or a combined oral contraceptive pill, or a progesterone-only pill should be prescribed an AED that does not induce hepatic enzymes
- Women with epilepsy should:
- [B] receive prepregnancy counselling at the time of diagnosis and at regular intervals during their management, especially if they are taking AED treatment
- [D] be reassured that most will have a normal pregnancy and delivery
- [C] have their diagnosis and treatment, if appropriate, reviewed by specialist services before conception; a concerted effort should be made to optimise seizure control and rationalise AED therapy prior to conception
- [D] be well informed about pregnancy and epilepsy-related issues, including smoking cessation, before conception
- [D] Women with epilepsy should be informed that sodium valproate is associated with a higher rate of teratogenicity compared to other AEDs
- [✓] Wherever possible sodium valproate should be avoided during pregnancy
- [✓] For women of childbearing potential, particularly those women contemplating pregnancy, other antiepileptic drugs should be considered in preference to sodium valproate. However, sodium valproate might be the only effective AED for some women, and this should not preclude its use
- [✓] Where sodium valproate is prescirbed for women or girls of childbearing potential:
- a pregnancy test should be carried out beforehand to exclude pregnancy (unless there are compelling reasons to indicate that there is no risk of pregnancy)
- the lowest dose possible should be used
- [✓] Given the morbidity and mortality risks associated with seizures (including sudden unexpected death in epilepsy) no AED should be discontinued during pregnancy unless this has been discussed with an epilepsy specialist
- [✓] Pregnancies in women with epilepsy should be supervised in an obstetric clinic with access to an obstetrician with a special interest in medical disorders in pregnancy and an epilepsy specialist
- [D] Women should be aware that their seizure pattern may change around the menopause
- [D] Hormone replacement therapy should be prescribed for the same indications as in women who do not have epilepsy
- For further guidance on epilepsy and women's health, refer to the full guideline.
- Psychiatric comorbidities in people with epilepsy are common but may go undiagnosed and untreated. Major depression is the main psychiatric comorbidity in people with epilepsy with rates of 24% recorded
- [D] Screening for depression and suicidality should be considered in all patients with epilepsy
- [D] The NDDI-E, HADS-D, BDI-II or PHQ-2 can all be used to screen for depression in adults with depression and epilepsy. The NDDI-E may be superior for detecting severe depression and suicidal ideation
- [✓] When screening identifies the presence of possible psychiatric comorbidity, people with epilepsy should be referred to an appropriately trained mental healthcare professional for further assessment and, where relevant, treatment
- [✓] Screening tools used by non-mental healthcare professionals should be brief, easily administered and easily understood
- [✓] Although not specifically validated for use in epilepsy, consideration should be given to using screening tools such as the Glasgow Anxiety Scale or the Glasgow Depression Scale for People with Learning Disability in patients with intellectual disability
- [✓] Assessment of anxiety and depression should, where relevant, be considered as part of a multidisciplinary approach to patient-centred care
- [✓] Treatment of anxiety and depression should, where relevant, be considered as part of a multidisciplinary approach to patient-centred care
- [D] Treatment with antidepressants should be considered in patients with epilepsy and comorbid depression.
- [B] Healthcare professionals and patients should aim for complete seizure freedom to reduce the risk of sudden unexpected death in epilepsy
- [D] Adherence to the prescribed AED regime should be strongly encouraged and the patient asked to report any adverse effects that might compromise adherence in order to reduce the risk of increased mortality and morbidity
- [✓] Patients with active seizures, i.e. who have sustained seizures, and in particular generalised tonic-clonic seizures, in the past year, should be assessed by a specialist physician and epilepsy nurse specialist
- [✓] The apparent increase in SUDEP in people with frequent nocturnal seizures should be highlighted to patients and nocturnal supervision could be considered
- [✓] Patients admitted to video-EEG units who will have their antiepileptic drugs reduced must be warned of the risk of SUDEP, although the risk is low
- [✓] It is desirable that video-EEG units should monitor oxygen saturation levels as well as ECG and EEG
- [✓] Video-EEG units must have adequate staff levels to respond immediately should the patient become apnoic or exhibit a significant cardiac arrhythmia
- [D] Counselling about the risks of SUDEP should be considered for patients with epilepsy at an appropriate time for the patient and by an appropriate healthcare professional (consultant neurologist, physician with an interest in epilepsy, specialist registrar, or epilepsy nurse specialist).
Models of Care
- The care of people with epilepsy is provided in primary, secondary, and tertiary settings
- [D] A structured management system for epilepsy should be established in primary care. As with other chronic diseases, an annual review is desirable
- [D] The annual review should be facilitated by specialist epilepsy nurses, linking primary care to the hospital system (shared care)
- [D] The shared care management system adopted should seek to:
- identify all patients with epilepsy, register/record basic demographic data, validate the classification of seizures and syndromes
- make the provisional diagnosis in new patients, provide appropriate information and refer the patient to a specialist centre
- monitor seizures, aiming to improve control by adjustment of medication or re-referral to hospital services
- minimise adverse effects of medications and their interactions
- facilitate structured withdrawal from medication where appropriate, and if agreed by the patient
- introduce non-clinical interventions, and disseminate information to help improve the quality of life for patients with epilepsy
- address specific women's issues, and
- address the needs of patients with learning disabilities.
Information for Patients and Carers
- A checklist to help healthcare providers to give patients and carers information they may find helpful at the key stages of the patient journey is included in the full guideline. The checklist includes specific information requirements relating to:
- general epilepsy information
- antiepileptic drugs (AEDs)
- seizure triggers
- first aid
- issues for women
- possible psychosocial consequences
- sources of support.
- [✓] Information should be given in an appropriate manner with sufficient time to answer questions
- [✓] Information should be repeated over time and reinforced to ensure understanding
- [✓] Patients should be given information to take home in the most suitable format, for example leaflets, factsheets, a DVD, or specialised material for people with learning disability, making adjustments for patients from black and minority ethnic groups. All information and literature provided should be subject to regular review
- [✓] Where appropriate, information about bilingual, and culturally-sensitive epilepsy materials and support services should be given
- [✓] Healthcare professionals should be aware that the cultural differences and belief systems of patients from black and minority ethnic groups may have an impact on levels of understanding, management of the condition and adherence to medication and treatment.