Managing Epilepsy in Primary Care

Epilepsy is defined as recurrent, unprovoked seizures.
These seizures are characterised by a brief disturbance in consciousness, behaviour, emotion, motor function or sensation.

Classification of seizures¹

Epileptic seizures and syndromes should now be classified along a multi-axial diagnostic scheme using axes of:

• Ictal phenomenology
• Seizure type
• Syndrome
• Aetiology
• Impairment.

The previous and still widely used classification by clinical type of seizure is now felt to be too inflexible. This classified seizures as:

• Simple partial (consciousness not impaired)
• Complex partial (consciousness impaired)
• Convulsive generalised (eg tonic-clonic)
• Non-convulsive generalised (eg absence seizure).

Prevalence of treated epilepsy is estimated at about 1 in 200 of the general populace.
Lifetime Prevalence is thought to be between 2 and 5%.
Annual incidence in a developed country is about 50/100,000 with peaks of onset in children and in the elderly.
An average G.P. will:

• Diagnose 1 or 2 new cases a year
• Care for about 10 people with epilepsy on treatment
• Care for a further 15-25 patients who have had seizures in the past but have never received treatment or who have now stopped treatment.

Following a first seizure, an individual may go to A&E or their local GP. An accurate history together with an eyewitness account, where available, is important to discern whether the 'attack' is indeed a seizure. A thorough physical examination should also be undertaken.

If a first epileptic seizure is suspected or where there is diagnostic uncertainty, the individual must be referred on to a specialist service (adult or paediatric neurology dependant on age) for full diagnosis. Specialist involvement is critical to prevent incorrect diagnosis or classification of epilepsy which in turn can lead to inappropriate treatment and persistence of seizures.

Following a first seizure, the health care team at this initial point of contact should be responsible for onward referral and providing information to the individual/carer/family about how to recognise any further seizures, first aid and the importance of reporting further attacks whilst awaiting diagnosis.

Symptoms

Enquire about:

• Background - previous head injuries, alcohol or drug use, previous strokes/febrile convulsions, family history of epilepsy
• Provoking factors - sleep deprivation, alcohol withdrawal, strobe or flashing lights
• Prodrome or aura - change in mood or behaviour preceding fit (prodrome) or early part of fit that precedes its other manifestations (aura) eg déjà-vu sensation, odd smells, visual disturbances
• Description of the attack - changes in consciousness, behaviour, abnormal movements, incontinence, tongue biting, skin colour, duration, circumstances, after-effects
• If recurrent episodes- frequency and relationship to sleep, menses etc
• Residual symptoms following attack - confusion, headache, aching limbs, temporary weakness of limbs (Todd's palsy).

Signs

Examination may be unremarkable.

• Check for any neurological or cardiac signs
• Look for evidence of systemic illness
• Skin examination may reveal neurofibromatosis or port wine stain (Sturge-Weber Syndrome)
• Developmental assessment (where appropriate).

Primary care investigations may yield few positive factors:

• Blood screen following first fit: U&E, glucose, LFT, Ca² , FBC, ESR
• Consider a toxicology screen
• Older patients may need CXR, additional blood tests or ECG if malignancy or cardiovascular causes are suspected.

Secondary care investigations include:

• Neuroimaging - MRI is the mode of choice where epilepsy is suspected but CT may be used in an acute situation or where MRI is not available or contraindicated.
• EEG should only be used where clinical history is suggestive of epilepsy to support diagnosis.
• Metabolic screening may be helpful in children.
• Neuropsychological assessment is sometimes used to evaluate impact on cognition, memory etc.

The differential for an epileptic seizure is extensive and includes:

• Vasovagal syncope ('faint')
• Psychogenic non-epileptic attacks (pseudoseizures)
• Hyperventilation and panic attacks
• Breath holding attacks (children)
• Complex migraine
• Vertigo
• Concussive convulsion
• Narcolepsy and catalepsy
• Transient ischaemic attack (TIA)
• Cardiac arrhythmia
• Hypnotic myoclonic jerks
• Night terrors
• Munchausen's syndrome.

Emergency care

See management of status epilepticus.

Ongoing management in general practice

After a new diagnosis and investigation, patients are usually commenced on treatment by their specialist.
All patients with epilepsy should have:

• An accessible point of contact to specialist services
• A care plan agreed between the individual/family/carers and primary and secondary care
• A named individual to contact for information if required.

Once stable, patients are usually discharged back to their GP and primary care team. Regular review of adults should occur at least annually (children should receive their structured review from their specialist).
Reviews should include:

• Occurence of further fits and fit frequency
• Drug compliance (check use of repeat prescriptions)
• Side-effects of drugs
• If fit-free for more than 2 years, discuss possibility of withdrawing medication
• Those on enzyme inducing drugs should have a FBC, U&E, LFT, Vit D levels, Serum Ca, ALP every 2-5 years although asymptomatic abnormalities in these test results should not be an automatic indication for altering medication.

Re-refer to specialist where:

• Poor control
• Seizures have continued for more than 5 years
• Side-effects or poor tolerance of current medication
• Concurrent illness complicating management
• Need for pre-conceptual advice
• Concerns about underlying cause of fits
• For withdrawal of anti-epileptic drugs (AEDs).

Patient education and information needs

After a new diagnosis of epilepsy and at future times, individuals with epilepsy, their families and carers will have many questions. Find out current understanding, what information they desire and acknowledge emotions surrounding diagnosis and treatment.
All patients should have access to information including:

• Diagnosis and treatment options
• Medication and side-effects
• Seizure type(s), triggers and control
• Management and self-care
• Risk management
• First-aid, safety and injury prevention at home, school and work
• Psychological issues
• Benefits and social services
• Insurance issues
• Implications for education and healthcare at school
• Employment and independent living for adults
• Importance of disclosure at work
• Road safety and driving
• Prognosis
• Sudden unexpected death in epilepsy (SUDEP)
• Status epilepticus
• Lifestyle, leisure and social issues (effects of recreational drugs, alcohol, sexual activity and sleep deprivation)
• Family planning and need for preconceptual counselling
• Voluntary organisations and support groups.

Any member of the health care team involved in their care should be able to access this information. Specialist epilepsy liaison nurses, where available, may be a useful resource.

The aim, as with many chronic conditions, is to involve and educate patients/families/carers so that they can be fully involved in all decision making and feel empowered to self-manage their condition.

Drugs^2,4,5,6

GPs are not routinely responsible for initiating AEDs or altering drug regimes, however it is important to be familiar with issues surrounding the choice and potential problems of medications.

• Treatment may not be needed after only 1 fit. 60% of adults who have had 1 fit will not have another (90% with a normal EEG). AEDs are normally initiated by a specialist following a second fit.
• Monotherapy is usually effective, but if unsuccessful a second drug may be slowly introduced. If this is ineffective it is usually slowly withdrawn and an alternative tried until control is achieved. The aim is for the minimum number of drugs possible as combination therapy increases risk of toxicity and introduces the risk of interactions between antiepileptics.
• Currently older AEDs (such as sodium valproate and carbamazepine) are preferred first line agents and newer AEDs (such as gabapentin, lamotrigine, topiramate, vigabatrin) are second line for those who have not received benefit from the older AEDs or where these older drugs would be unsuitable.
• Remember many anti-epileptic medications have interactions with other medicines. In particular, some AEDs are hepatic enzyme-inducers and can have significant effects on the metabolism of other drugs.

  Anti-epileptic medication7
  Hepatic enzyme inducers	Non-enzyme inducers
  Carbamazepine Oxycarbamazepine Phenobarbital Phenytoin Primidone Topiramate	Benzodiazepines Ethosuxamide Gabapentin Lamotrigine Levetiracetam Tigabine Valproate Vigabatrin

• Patients should be counselled on side effects.
• Current or future possibility of pregnancy should influence choice of drug in women of childbearing age.

Drug Monitoring

• Drug monitoring is not routinely recommended.
• Indications for AED levels include:
  • Concerns surrounding adherence
  • Suspected toxicity
  • Adjustment of phenytoin dose - a small rise in dose may lead to a large increase in blood levels but this offers a rough guide only as some patients will have good control with low levels whilst a few require (and tolerate) higher blood concentrations
  • Management of pharmacokinetic interactions
  • Specific clinical conditions (eg status epilepticus, organ failure, pregnancy).

Stopping medication

• May be considered after 2 years seizure free
• Refer for specialist advice before discontinuing as risk of further fit may have significant social and psychological consequences (eg driving, employment). Even patients who have been seizure-free for several years run a significant risk of seizure recurrence on drug withdrawal.

  Prognostic index for recurrence of seizures within one and two years after continuing AED treatment or starting slow withdrawal.8 Starting score (all individuals) -175 Age 16 or older: add 45 Taking more than one AED: add 50 Seizures after start of AED treatment: add 35 History of primary or secondarily generalized tonic-clonic seizures: add 35 History of myoclonic seizures: add 50 EEG in last year: not available: add 15 abnormal: add 20. Period free from seizures (t: no. of years) add 200/t. TOTAL SCORE T Divide total score by 100 and exponentiate z=e T/100 Probability of recurrence of seizures: Continued treatment: by one year 1-0.89z by two years 1-0.79z. Slow withdrawal: by one year 1-0.69z by two years 1-0.60z.

• Drugs usually withdrawn slowly over months under the care of the specialist.
• Where patients are receiving several AEDs, only one drug should be withdrawn at a time.

Certain patient groups will also have additional needs related to their epilepsy:

Epilepsy in women of reproductive age⁹

Contraception

• Some anti-epileptic medication cause harm to a developing fetus. Women need to be aware of this risk and good contraceptive advice given where pregnancy is not actively desired.
• AEDs that act as hepatic enzyme inducers can also interact with the contraceptive pill (both COC and POP) diminishing contraceptive efficacy. Again women should be counselled regarding this risk and be aware that hormonal methods of contraception are not a first-line choice.
• If possible, use an antiepileptic which is not a hepatic enzyme inducer.
• If this is unavoidable, use 50mcg oestrogen preparations in the combined pill and run 3 or 4 packs together ("tri-cycling") with a maximum of 4 pill free days. If breakthrough bleeding occurs, increase the dose still further to 80-100mcg. Barrier contraception should also be used.
• Avoid POP if on hepatic enzyme inducers or at least double the dose of POP where patient is unwilling or unsuitable for other methods.
• For emergency contraception, an intrauterine device is the most reliable option although a double dose of levonorgestrel can be an alternative.
• Depo-injections can be used and can be scheduled every 12 weeks as per normal use.

Pre-conception counselling

• Consider referral for specialist opinion before conception to reduce or change drug treatment if possible. Risk of newer drugs as yet not quantified but sodium valproate is associated with teratogenicity.⁵
• Counsel about the balance of possible harm done by medication compared with that due to poorly controlled seizures (to both mother and fetus).
• Fetal malformations (most commonly neural tube defects, cleft lip/palate and cardiac malformations) occur in 4% of those diagnosed with epilepsy but not taking medication and increases to 6% when taking medication (polytherapy with certain drugs can have a much higher risk). Note: where both parents have epilepsy, heredity risk for child is 15-20%.
• Recommend Folic acid 5 mg per day before conception and up to 12 weeks.

Epilepsy in pregnancy

• Pregnant women with epilepsy should be referred for specialist obstetric and epilepsy care.
• They should be offered antenatal screening (alpha-fetoprotein measurement and a second trimester ultrasound scan) in view of the increased risk of neural tube defects.
• Delivery should be arranged for a specialist centre that will be able to manage any fits (1-2% fit during delivery and a further 1-2% in the 48 hours following delivery).
• Drug dosage may need increasing if fit frequency increases during pregnancy. This may need reducing following delivery. Changes to medication should follow specialist advice only.
• Breastfeeding is generally safe and recommended with older AEDs and some of the newer AEDs taken in normal doses.
• Women on enzyme-inducing AEDs should receive 20mg/day Vitamin K from 36/40 gestation. Their babies should receive 1mg im at delivery with repeat at 28 days old.
• Epilepsy should be reviewed by the GP at the 6 week post-partum check and by a specialist at about 12 weeks following delivery.

Epilepsy in children and young people^1,10,2

The management of epilepsy in children and young people is extremely specialist with several syndromes specific to children. Some are 'benign' and will usually remit, others carry a very poor prognosis eg infantile spasms, Lennox-Gestaut syndrome. Primary care may be involved in supporting the family and coordinating multi-disciplinary team input.

• Always consider the physical, psychological and social needs as well as the purely medical needs of a child.
• Ensure an inclusive style of consulting.
• Good multiagency communication is critical.
• A named clinician should oversee the transition of an adolescent patient from paediatric to adult services.
• At the transition to adult services, diagnosis and treatment should be formally reviewed.
• Information appropriate to the child's level of understanding should be supplied about their condition including potential consequences of epilepsy on lifestyle without over-limiting the child.
• Drugs used in children are more likely to be used off-license and without good trial evidence.
• 25% of children with epilepsy have special educational needs, of which a fifth have moderate to severe learning difficulties.

Epilepsy and learning disability ^1,2

The same standard of care should be applied to those with learning difficulties and epilepsy as for those with epilepsy alone.

• Management and treatment of epilepsy should be undertaken by a specialist working within a multi-disciplinary team.
• Diagnosis is often difficult - eyewitness accounts and corroborative video evidence may help.
• Choice of AEDs should consider likely side-effects impacting on cognition or behaviour.
• Risk assessment to include bathing and showering, preparation of food, use of electrical items, impact of epilepsy on a social setting etc.
• Balanced decisions regarding the suitability of independent living.

Epilepsy in elderly people¹¹

• Development of epilepsy is common in later life.
• The elderly are particularly vulnerable to injuries during seizures.
• The clinical situation and diagnosis are often complicated by a range of neurodegenerative, cerebrovascular, neoplastic and psychiatric comorbitities.
• Drug interactions and side-effects are particularly common. AEDs should be chosen with this in mind. Lamotrigine and gabapentin appear better tolerated than carbamazepine in elderly people.
• Loss of confidence, concerns surrounding stigmatisation and reduced independence may lead to premature admission to nursing homes and residential care. Appropriate support and education may help to counter this.
• The prognosis in terms of seizure control appears better in the elderly than in younger populations so optimal management requires rapid assessment, diagnosis and effective treatment.

Drivers¹²

• Any patient should be advised to stop driving and notify DVLA and motor insurance company following a seizure. Document your advice.
• DVLA recommends avoiding driving for 1 year after a seizure.
• If only nocturnal seizures for last 3 years, patient may drive.
• If medication is being withdrawn, must stop driving until remain seizure-free for 6 months off medication.
• Group 2 license holders (light goods & public service vehicles) need to be seizure free for 10 years.
• Cyclists should be advised to avoid cycling in traffic.

Practice Management of Epileptics

The nGMS Contract recommends certain clinical indicators are collected to monitor the care of epileptic patients.¹³In order to achieve quality points (currently a maximum of 15 points)the following are required

• A practice register of patients aged over 18 and receiving drug treatment for epilepsy(Epilepsy 5)
• A recall system for ensuring regular review(either opportunistic or linked to medication review)
• Record of seizure frequency in the last 15 months (Epilepsy 6)
• Recorded medication review in the last 15 months (Epilepsy 7)
• Percentage of epilepsy register who have been seizure-free for 12 months (Epilepsy 8).

A simple proforma (usually in the form of a computer template) can be developed.
Suggested points to cover-

• Regular medication review for adults (annual is optimum)
• Medication side effects, dosage and frequency
• Type and number of fits in the last year, document date of last fit
• Preconception and contraception counselling in women
• Driving advice.

• Social stigmatisation
• Psychosocial problems
• Developmental problems in children with early onset seizures
• Specific cognitive and learning difficulties impacting on education if not recognised
• AED side-effects
• Physical trauma secondary to seizures
• Risk of fetal malformation associated with AEDs
• SUDEP - approximately 500 deaths pa in UK.

• Variable risk of recurrence after first seizure:
  • Lowest risk (13-40%) in those with a provoked seizure, normal EEGs and no identifiable cause for first seizure.
  • Highest risk(90%) in those with an abnormal EEG with epileptic discharge and neurological defects.
  • Overall approximately 30-40% with highest risk in first year.
  • Treatment with AEDs halves recurrence risk.
• Remission of seizures is common - nine years on from initial seizure, 70% will be seizure free for last 3 years and only 30% still on medication.
• Withdrawal of medication is frequently successful.
• Mortality rates of double to quadruple those of the general population - highest in children with symptomatic epilepsy and those with learning or physical disability. Underlying condition, suicide, accidents, status epilepticus but particularly SUDEP contribute to mortality.