Presenter instructions

1. Presenter instructions • Do not delete this slide • This presentation contains interactive hyperlinks. There are hyperlinks to allow you to view the treatment options for each seizure and epilepsy syndrome type. • The hyperlinks (green underlined text in boxes) only work when the presentation is in ‘slide show mode’. In order to guarantee effective use of the hyperlinks you must ensure you are clicking exactly on the hyperlink (wait for the mouse arrow to turn to a hand). If the presenter clicks anywhere outside the hyperlink this will just move the presentation on and you will go onto the next consecutive slide. • The presenter should print out the presenter notes to deliver from.

2. The epilepsies Implementing NICE guidance January 2012 NICE clinical guideline 137

3. Updated guidance • This guideline updates and replaces ‘The epilepsies: the diagnosis and management of the epilepsies in adults and children in primary and secondary care’ (NICE clinical guideline 20, 2004). • This guidance also updates and replaces NICE technology appraisals 76 and 79. • New recommendations for pharmacological treatment have been added.

4. What this presentation covers • Background • Scope • Guideline recommendations • Costs and savings • Discussion • NICE Pathway, NHS Evidence and • National Prescribing Centre • Find out more

5. Background • Epilepsy: common neurological disorder characterised by recurring seizures. • The majority of people with active epilepsy can satisfactorily control recurrent seizures. • Optimal management improves health outcomes and minimises detrimental impacts on health. • Newer anti-epileptic drugs are currently being prescribed, so it is important that clinical and cost effectiveness are identified.

6. Epidemiology • The epilepsies comprise the most common serious neurological disorders. • Estimated to affect between 362,000 and 415,000 people in England. • Estimated incidence: 50 per 100,000. • Estimated prevalence of active epilepsy in the UK: 5−10 cases per 1000.

7. Scope • This guideline provides recommendations for the treatment of children, young people adults and older people with a diagnosis of any type of epilepsy. Treatments for neonates are not covered by this guideline. • It covers all seizure types and epilepsy syndromes. • Recommendations are relevant to healthcare professionals who have direct contact with, and make decisions concerning, the care of people with epilepsy.

8. Decision making Healthcare professionals should: • adopt a consulting style that enables people with epilepsy to participate as partners in decisions about their healthcare • take into account their race, culture and any specific needs. • [2004]

9. Information • People with epilepsy should have access to information about all aspects of their condition. This information should be provided in suitable formats. • Adequate time should be set aside at consultation to provide information, and checklists should be used to ensure all information is discussed. • In high-risk individuals, the possibility of having seizures should be discussed, and information about epilepsy should be provided before seizures occur. • [2004]

10. Sudden unexpected death in epilepsy (SUDEP) • Tailored information and discussion on a person’s relative risk of SUDEP should be provided. • The risk of SUDEP can be minimised by optimising seizure control and being aware of potential consequences of nocturnal seizures. • Where families/carers have been affected by SUDEP, healthcare professionals should contact them to offer their condolences and referral to bereavement counselling. • [2004]

11. Following a first seizure • People presenting at A&E should be screened initially with onward referral to a specialist when an epileptic seizure is suspected or if there is diagnostic doubt. • Protocols should be in place that ensure proper assessment in the emergency setting. • All people should be seen as soon as possible and within 2 weeks, by a specialist in the management of the epilepsies. • [2004]

12. Diagnosis • Diagnosis should be established by a specialist medical practitioner and the opportunity to discuss this diagnosis should be given. • The decision as to whether an epileptic seizure has occurred should be based on a description of the attack and different symptoms. • If the diagnosis cannot be clearly established, further investigations and referral to a tertiary specialist should be considered. • [2004]

13. Investigations: electroencephalogram (EEG): 1 • Those requiring an EEG should have the test performed soon (within 4 weeks) after requested, and it should only be performed to support a diagnosis of epilepsy. • An EEG should not be performed in the case of probable syncope or used to exclude a diagnosis of epilepsy. It should also not be used in isolation to make a diagnosis. • An EEG may be used to help determine seizure type and epilepsy syndrome. • [2004]

14. Investigations: electroencephalogram (EEG): 2 • In those presenting with a first unprovoked seizure, an EEG can be used to assess risk of seizure recurrence. • Repeated standard EEGs may be helpful when the diagnosis of epilepsy or the syndrome is unclear. These should not be used in preference to sleep or sleep-deprived EEGs. • Photic stimulation and hyperventilation should remain part of standard EEG assessment. • [2004]

15. Investigations: neuroimaging • Neuroimaging should be used to identify structural abnormalities. • MRI should be the imaging of choice and is important in those who develop epilepsy before the age of 2 or in adulthood, those who have a suggestion of focal onset and those in whom seizures continue. • CT should be used to identify underlying gross pathology if MRI is not available or if sedation for MRI is required. • [2004]

16. Other tests • Measurement of serum prolactin is not recommended. • Appropriate blood tests should be considered. • A 12-lead ECG should be performed in adults with suspected epilepsy and children in cases of diagnostic uncertainty. • In cases of diagnostic uncertainty a referral to a cardiologist should be considered. • [2004]

17. Neuropsychological assessment This should be considered when it is important to evaluate learning disabilities and cognitive dysfunction. Referral for assessment is indicated: • when those with epilepsy are having educational or occupational difficulties • when an MRI had identified abnormalities • when a person complains of memory or other cognitive deficits or cognitive decline. • [2004]

18. Management • People should have an accessible point of contact with specialist services. • All those with epilepsy should have a comprehensive, agreed care plan. • Epilepsy specialist nurses should be an integral part of the network of care. • Healthcare professionals have a responsibility to educate others to reduce stigma. • [2004]

19. Pharmacological treatment: 1 • The anti-epileptic drug (AED) treatment strategy should be individualised. • The diagnosis of epilepsy should be evaluated if events continue despite an optimal dose of a first-line AED. • [2004]

20. Pharmacological treatment: 2 • Consistent supply of a particular manufacturer's AED preparation is recommended as different preparations of some AEDs may vary in bioavailability or pharmacokinetic profiles and care needs to be taken to avoid reduced effect or excessive side effects. (1) • People with epilepsy should be treated with a single AED wherever possible. • [2012] • [2004]

21. Pharmacological treatment: 3 • If an AED has failed, a second drug should be started and built up to adequate dose, then the first drug should be tapered off slowly. • Combination therapy should only be considered when attempts at monotherapy have not resulted in seizure freedom. • [2004]

22. Pharmacological treatment: 4 • If using carbamazepine, offer controlled-release carbamazepine preparations. • When prescribing sodium valproate to women and girls of present and future childbearing potential, discuss the possible risks of malformation and neurodevelopmental impairments. • [2012]

23. Initiation of pharmacological treatment • AED therapy should only start on the recommendation of a specialist and once the diagnosis of epilepsy is confirmed. • The decision to start therapy should be taken between the person and specialist after a full discussion of the risks and benefits to treatment. • When possible choose which AED to offer based on the epilepsy syndrome. If this is not clear, base the decision on the presenting seizure. • [2004] • [2012]

24. Epilepsy syndrome type Click on the boxes to view the treatment recommendations for each syndrome type. Treatment of childhood absence epilepsy, juvenile absence epilepsy or other absence epilepsy syndromes Benign epilepsy with centrotemporal spikes, Panayiotopoulos syndrome or late-onset childhood occipital epilepsy (Gastaut type) Dravet syndrome Lennox–Gastaut syndrome Refractory convulsive status epilepticus Juvenile myoclonic epilepsy Other epilepsy syndromes More syndrome types

25. Epilepsy syndrome type: 2 Click on the boxes to view the treatment recommendations for each syndrome type. Treatment of epilepsy with generalised tonic-clonic (GTC) seizures only Infantile spasms Convulsive status epilepticus in hospital Prolonged or repeated seizures and convulsive status epilepticus in the community Idiopathic generalised epilepsy Back to previous list To go to recommendations for seizure types

26. Seizure type Click on the boxes to view the treatment recommendations for each seizure/syndrome type. Focal seizures GTC seizures Absence seizure Myoclonic seizure Tonic or atonic seizures Back to epilepsy syndromes Click here to continue to non-pharmacological recommendations

27. First-line treatment of focal seizures • Offer carbamazepine or lamotrigine. • Offer levetiracetam, oxcarbazepine or sodium valproate (provided the acquisition cost of levetiracetam falls to at least 50% of June 2011- see presenter notes for more information) if carbamazepine and lamotrigine are unsuitable or not tolerated. If the first-line AED is ineffective, offer an alternative from these five AEDs. • Consider adjunctive treatment if second AED ineffective. For adjunctive treatment click here To go back to seizure/syndrome type slide click here • [2012]

28. Adjunctive treatment of refractory focal seizures • Offer carbamazepine, clobazam, gabapentin, lamotrigine, levetiracetam, oxcarbazepine, sodium valproate or topiramate. • If adjunctive treatment is ineffective or not tolerated discuss with or refer to a tertiary epilepsy specialist. • Other AEDs that may be considered by the specialist are eslicarbazepine acetate, lacosamide, phenobarbital, phenytoin, pregabalin, tiagabine, vigabatrin and zonisamide. To go back to seizure/syndrome type slide click here • [2012]

29. First-line treatment of newly diagnosed GTC seizures • Offer sodium valproate. Be aware of teratogenic risks. • Offer lamotrigine if sodium valproate is unsuitable. Be aware that this AED may exacerbate myoclonic seizures. • Consider carbamazepine and oxcarbazepine. Be aware that these AEDs may exacerbate myoclonic or absence seizures. • [2012] For adjunctive treatment click here To go back to seizure/syndrome type slide click here

30. Adjunctive treatment of GTC seizures • Offer clobazam, lamotrigine, levetiracetam, sodium valproate or topiramate. • If there are absence or myoclonic seizures, or if juvenile myoclonic epilepsy (JME) is suspected, do not offer carbamazepine, gabapentin, oxcarbazepine, phenytoin, pregabalin, tiagabine or vigabatrin. To go back to seizure/syndrome type slide click here • [2012]

31. First-line treatment of absence seizures • Offer ethosuximide or sodium valproate. If there is a high risk of GTC seizures, offer sodium valproate first, unless it is unsuitable. • Offer lamotrigine if ethosuximide and sodium valproate are unsuitable, ineffective or not tolerated. For adjunctive treatment click here To go back to seizure/syndrome type slide click here • [2012]

32. Adjunctive treatment of absence seizures • Consider a combination of two of these three AEDs as adjunctive treatment: ethosuximide, lamotrigine or sodium valproate. • If ineffective or not tolerated, discuss with, or refer to, a tertiary epilepsy specialist and consider clobazam, clonazepam, levetiracetam, topiramate or zonisamide. • Do not offer carbamazepine, gabapentin, oxcarbazepine, phenytoin, pregabalin, tiagabine or vigabatrin. To go back to seizure/syndrome type slide click here • [2012]

33. First-line treatment of myoclonic seizures • Offer sodium valproate unless it is unsuitable. • Consider levetiracetam or topiramate if sodium valproate is unsuitable or not tolerated. Be aware that topiramate has a less favourable side-effect profile than levetiracetam and sodium valproate. For adjunctive treatment click here To go back to seizure/syndrome type slide click here • [2012]

34. Adjunctive treatment of myoclonic seizures • Offer levetiracetam, sodium valproate or topiramate. • If ineffective discuss with, or refer to, a tertiary epilepsy specialist and consider clobazam, clonazepam, piracetam or zonisamide. • Do not offer carbamazepine, gabapentin, oxcarbazepine, phenytoin, pregabalin, tiagabine or vigabatrin. To go back to seizure/syndrome type slide click here • [2012]

35. First-line treatment of tonic or atonic seizures • Offer sodium valproate. Be aware of teratogenic risks. For adjunctive treatment click here To go back to seizure/syndrome type slide click here • [2012]

36. Adjunctive treatment of tonic or atonic seizures • If first-line treatment with sodium valproate is ineffective or not tolerated, offer lamotrigine. • Discuss with a tertiary epilepsy specialist if adjunctive treatment is ineffective or not tolerated. Other AEDs that may be considered by the tertiary epilepsy specialist are rufinamide and topiramate. • Do not offer carbamazepine, gabapentin, oxcarbazepine, pregabalin, tiagabine or vigabatrin. To go back to seizure/syndrome type slide click here • [2012]

37. Treatment of infantile spasms • Discuss with, or refer to, a tertiary paediatric epilepsy specialist. • Offer a steroid (prednisolone or tetracosactide) or vigabatrin to infants with infantile spasms that are not due to tuberous sclerosis. • Offer vigabatrin to infants with infantile spasms due to tuberous sclerosis. If vigabatrin is ineffective, offer a steroid. To go back to seizure/syndrome type slide click here • [2012]

38. First-line treatment of Dravet syndrome • Discuss with, or refer to, a tertiary paediatric epilepsy specialist. • Consider sodium valproate or topiramate. For adjunctive treatment click here To go back to seizure/syndrome type slide click here • [2012]

39. Adjunctive treatment of Dravet syndrome • Discuss with a tertiary epilepsy specialist and consider clobazam or stiripentol. • Do not offer carbamazepine, gabapentin, lamotrigine, oxcarbazepine, phenytoin, pregabalin, tiagabine or vigabatrin. To go back to seizure/syndrome type slide click here • [2012]

40. First-line treatment of Lennox–Gastaut syndrome • Discuss with, or refer to, a tertiary paediatric epilepsy specialist. • Offer sodium valproate. For adjunctive treatment click here To go back to seizure/syndrome type slide click here • [2012]

41. Adjunctive treatment of Lennox–Gastaut syndrome • Offer lamotrigine. • Discuss with a tertiary epilepsy specialist if lamotrigine ineffective or not tolerated. Other AEDs which may be considered: rufinamide and topiramate. • Do not offer carbamazepine, gabapentin, oxcarbazepine, pregabalin, tiagabine or vigabatrin. • Only offer felbamate in centres providing tertiary epilepsy specialist care and when all of the above AEDs have proved ineffective. To go back to seizure/syndrome type slide click here • [2012]

42. First-line treatment of benign epilepsy with centrotemporal spikes, Panayiotopoulos syndrome or late-onset childhood occipital epilepsy (Gastaut type) • Discuss whether AED treatment is indicated. • Offer carbamazepine or lamotrigine. • Offer levetiracetam, oxcarbazepine or sodium valproate (provided the acquisition cost of levetiracetam falls- see presenter notes for more information) if carbamazepine and lamotrigine are unsuitable or not tolerated. If the first AED tried is ineffective, offer an alternative from these five AEDs. For adjunctive treatment click here To go back to seizure/syndrome type slide click here • [2012]

43. Adjunctive treatment of benign epilepsy with centrotemporal spikes, Panayiotopoulos syndrome or late-onset childhood occipital epilepsy (Gastaut type) • Offer carbamazepine, clobazam, gabapentin, lamotrigine, levetiracetam, oxcarbazepine, sodium valproate or topiramate. • If ineffective or not tolerated discuss with, or refer to, a tertiary epilepsy specialist. Other AEDs that may be considered are eslicarbazepine acetate, lacosamide, phenobarbital, phenytoin, pregabalin, tiagabine, vigabatrin and zonisamide. To go back to seizure/syndrome type slide click here • [2012]

44. First-line treatment of idiopathic generalised epilepsy • Offer sodium valproate. • Offer lamotrigine if sodium valproate is unsuitable or not tolerated. • Consider topiramate but be aware that it has a less favourable side-effect profile than sodium valproate and lamotrigine. For adjunctive treatment click here To go back to seizure/syndrome type slide click here • [2012]

45. Adjunctive treatment of idiopathic generalised epilepsy • Offer lamotrigine, levetiracetam, sodium valproate or topiramate. • If ineffective or not tolerated discuss with, or refer to, a tertiary epilepsy specialist and consider clobazam, clonazepam or zonisamide. • Do not offer carbamazepine, gabapentin, oxcarbazepine, phenytoin, pregabalin, tiagabine or vigabatrin. To go back to seizure/syndrome type slide click here • [2012]

46. Treatment of juvenile myoclonic epilepsy First-line treatment: • offer sodium valproate • consider lamotrigine, levetiracetam or topiramate if sodium valproate is unsuitable or not tolerated Adjunctive treatment: • offer lamotrigine, levetiracetam, sodium valproate or topiramate • if ineffective or not tolerated discuss with, or refer to, a tertiary epilepsy specialist and consider clobazam, clonazepam or zonisamide • do not offer carbamazepine, gabapentin, oxcarbazepine, phenytoin, pregabalin, tiagabine or vigabatrin. To go back to seizure/syndrome type slide click here • [2012]

47. Treatment of epilepsy with GTC seizures only First-line treatment: • offer lamotrigine or sodium valproate • consider carbamazepine and oxcarbazepine. Adjunctive treatment: • offer clobazam, lamotrigine, levetiracetam, sodium valproate or topiramate. To go back to seizure/syndrome type slide click here • [2012]

48. Treatment of childhood absence epilepsy, juvenile absence epilepsy or other absence epilepsy syndromes First-line treatment: • offer ethosuximide or sodium valproate • offer lamotrigine if ethosuximide and sodium valproate are unsuitable, ineffective or not tolerated. Adjunctive treatment: • if ineffective consider a combination of two of the above three • if ineffective discuss with, or refer to, a tertiary epilepsy specialist and consider clobazam, clonazepam, levetiracetam, topiramate or zonisamide • do not offer carbamazepine, gabapentin, oxcarbazepine, phenytoin, pregabalin, tiagabine or vigabatrin. To go back to seizure/syndrome type slide click here • [2012]

49. Other epilepsy syndromes Refer to a tertiary paediatric epilepsy specialist all children and young people with continuous spike and wave during slow sleep, Landau–Kleffner syndrome or myoclonic-astatic epilepsy. To go back to syndrome type slide click here • [2012]

50. Continuation of pharmacological treatment • Maintain a high level of vigilance for treatment-emergent adverse effects. • Continuing AED therapy should be planned by the specialist. • Take the person’s needs into account. • If management is straightforward, continuing AED therapy can be prescribed in primary care. • Regular monitoring of blood tests is not recommended as routine. • [2012] • [2004]