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Epilepsy Epilepsy Overview

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Epilepsy overview A NICE pathway brings together all NICE guidance, quality standards and materials to support implementation on a specific topic area. The pathways are interactive and designed to be used online. This pdf version gives you a single pathway diagram and uses numbering to link the boxes in the diagram to the associated recommendations. To view the online version of this pathway visit: http://pathways.nice.org.uk/pathways/epilepsy Pathway last updated: 04 March 2015. To see details of any updates to this pathway since its launch, visit: About this Pathway . For information on the NICE guidance used to create this path, see: Sources . Copyright © NICE 2015. All rights reserved NICE Pathways Pathways
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  • Epilepsy overview

    A NICE pathway brings together all NICE guidance, qualitystandards and materials to support implementation on a specifictopic area. The pathways are interactive and designed to be usedonline. This pdf version gives you a single pathway diagram anduses numbering to link the boxes in the diagram to the associatedrecommendations.

    To view the online version of this pathway visit:

    http://pathways.nice.org.uk/pathways/epilepsy

    Pathway last updated: 04 March 2015. To see details of any updates to this pathway since its launch,visit: About this Pathway. For information on the NICE guidance used to create this path, see:Sources.Copyright NICE 2015. All rights reserved

    NICEPathwaysPathways

    http://pathways.nice.org.uk/pathways/epilepsyhttp://pathways.nice.org.uk/pathways/epilepsy#content=view-info-category%3Aview-about-menuhttp://pathways.nice.org.uk/http://pathways.nice.org.uk/http://pathways.nice.org.uk/http://www.nice.org.uk/

  • Epilepsy overview NICE Pathways

    Epilepsy pathwayCopyright NICE 2015.

    Page 2 of 36

  • 1 Child, young person or adult presents with a suspected seizure

    No additional information

    2 Diagnosing epilepsy and supporting investigations

    See Epilepsy / Diagnosing epilepsy and supporting investigations

    3 Non-epileptic attack disorder suspected

    Where non-epileptic attack disorder is suspected, refer to psychological or psychiatric servicesfor further investigation and treatment.

    Using EEG to evaluate non-epileptic attack disorder

    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 people.

    For other recommendations on using electroencephalogram (EEG) when investigating epilepsy,see EEG in this pathway.

    4 Epilepsy diagnosed

    No additional information

    5 Information about epilepsy

    Giving information about epilepsy

    Everyone providing care or treatment for children, young people and adults with epilepsy shouldbe able to provide essential information.

    Provide information in formats, languages and ways that are suited to the child, young person oradult's requirements. Consideration should be given to developmental age, gender, culture andstage of life of the person.

    Epilepsy overview NICE Pathways

    Epilepsy pathwayCopyright NICE 2015.

    Page 3 of 36

    http://pathways.nice.org.uk/pathways/epilepsy/diagnosing-epilepsy-and-supporting-investigationshttp://pathways.nice.org.uk/pathways/epilepsy/diagnosing-epilepsy-and-supporting-investigations#content=view-node%3Anodes-electroencephalogram-eeg

  • Healthcare professionals have a responsibility to educate others about epilepsy so as to reducethe stigma associated with it. Provide information about epilepsy to all people who come intocontact with children, young people and adults with epilepsy, including school staff, social careprofessionals and others.

    Information to provide

    Give to the person and their families and/or carers, and ensure access to sources of,information about (where appropriate):

    epilepsy in generaldiagnosis and treatment options (see also diagnosis and treatment in this pathway)medication and side effects (see also anti-epileptic drugs in this pathway)seizure type(s), triggers and seizure controlmanagement and self-carerisk managementfirst aid, safety and injury prevention at home and at school or workpsychological issuessocial security benefits and social servicesinsurance issueseducation and healthcare at schoolemployment and independent living for adultsimportance of disclosing epilepsy at work, if relevant (if further information or clarification isneeded, voluntary organisations should be contacted)road safety and driving

    prognosis

    sudden death in epilepsy (SUDEP; see below)status epilepticus (see also status epilepticus in this pathway)lifestyle, leisure and social issues (including recreational drugs, alcohol, sexual activity andsleep deprivation)family planning and pregnancy (see also special considerations for women and girls withepilepsy in this pathway)voluntary organisations, such as support groups and charitable organisations, and how tocontact them.

    Epilepsy overview NICE Pathways

    Epilepsy pathwayCopyright NICE 2015.

    Page 4 of 36

    http://pathways.nice.org.uk/pathways/epilepsy/diagnosing-epilepsy-and-supporting-investigationshttp://pathways.nice.org.uk/pathways/epilepsy/managing-epilepsy-in-children-young-people-and-adultshttp://pathways.nice.org.uk/pathways/epilepsy/treating-epilepsy-with-anti-epileptic-drugs-aedshttp://pathways.nice.org.uk/pathways/epilepsy/treating-prolonged-or-repeated-seizures-and-convulsive-status-epilepticushttp://pathways.nice.org.uk/pathways/epilepsy/special-considerations-for-women-and-girls-with-epilepsyhttp://pathways.nice.org.uk/pathways/epilepsy/special-considerations-for-women-and-girls-with-epilepsy

  • When to give information about epilepsy

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

    Give appropriate information before important decisions are made (for example, regardingpregnancy or employment).

    Discuss the possibility of having seizures, and provide information on epilepsy before seizuresoccur, for those at high risk of developing seizures (such as after severe brain injury), with alearning disability, or who have a strong family history of epilepsy. For more information onpeople with learning disabilities, see people with learning disabilities in this pathway.

    Set aside adequate time in the consultation to provide information revisit in subsequentconsultations.

    Use checklists to remind children, young people and adults, and healthcare professionals, aboutinformation that should be discussed during consultations.

    Ensure that the child, young person or adult with epilepsy and their family and/or carers asappropriate knows how to contact a named individual when information is needed. This namedindividual should be a member of the healthcare team and be responsible for ensuring that theinformation needs of the child, young person or adult and/or their family and/or carers are met.

    If children, young people and adults, and families and/or carers, have not already found high-quality information from voluntary organisations and other sources, inform them of differentsources (using the Internet, if appropriate: see, for example, the website of the Joint EpilepsyCouncil of the UK and Ireland).

    Providing information about sudden unexpected death in epilepsy (SUDEP)

    Include information on sudden unexpected death in epilepsy (SUDEP) in literature on epilepsyto show why preventing seizures is important. Include tailored information on the person'srelative risk of SUDEP as part of the counselling checklist.

    The risk of SUDEP can be minimised by:

    optimising seizure controlbeing aware of the potential consequences of nocturnal seizures.

    Epilepsy overview NICE Pathways

    Epilepsy pathwayCopyright NICE 2015.

    Page 5 of 36

    http://pathways.nice.org.uk/pathways/epilepsy/special-considerations-to-be-taken-when-managing-epilepsy-in-specific-groups-of-people#content=view-node%3Anodes-children-young-people-and-adults-with-learning-disabilitieshttp://www.jointepilepsycouncil.org.ukhttp://www.jointepilepsycouncil.org.uk

  • Take account of the small but definite risk of SUDEP when tailoring information and discussions.

    Where families and/or carers have been affected by SUDEP, contact them to offer condolences,invite them to discuss the death, and offer referral to bereavement counselling and a SUDEPsupport group.

    Quality standards

    The following quality statements are relevant to this part of the pathway.

    4. Epilepsy care plan (children and young people)

    4. Epilepsy care plan (adults)

    6 Classification of epilepsy by seizure type and epilepsy syndrome andinvestigations to determine the cause of the epilepsy

    See Epilepsy / Classification of epilepsy by seizure type and epilepsy syndrome andinvestigations to determine the cause of the epilepsy

    7 Managing epilepsy in children, young people and adults

    See Epilepsy / Managing epilepsy in children, young people and adults

    8 Special considerations to be taken when managing epilepsy inspecific groups of people

    See Epilepsy / Special considerations to be taken when managing epilepsy in specific groups ofpeople

    9 Treating prolonged or repeated seizures and convulsive statusepilepticus

    See Epilepsy / Treating prolonged or repeated seizures and convulsive status epilepticus

    Epilepsy overview NICE Pathways

    Epilepsy pathwayCopyright NICE 2015.

    Page 6 of 36

    http://pathways.nice.org.uk/pathways/epilepsy/classification-of-epilepsy-by-seizure-type-and-epilepsy-syndrome-and-investigations-to-determine-the-cause-of-the-epilepsyhttp://pathways.nice.org.uk/pathways/epilepsy/managing-epilepsy-in-children-young-people-and-adultshttp://pathways.nice.org.uk/pathways/epilepsy/special-considerations-to-be-taken-when-managing-epilepsy-in-specific-groups-of-peoplehttp://pathways.nice.org.uk/pathways/epilepsy/treating-prolonged-or-repeated-seizures-and-convulsive-status-epilepticus

  • 10 Referral to a tertiary epilepsy service

    See Epilepsy / Referral to a tertiary epilepsy service

    11 A summary of where recommendations differ between children andyoung people and adults when diagnosing and managing epilepsy

    For differences in the recommendations between children and young people, and adults, atdifferent points of this pathway, see:

    Differences in recommendations for helping people to cope with epilepsy [See page 20]Differences in recommendations for what to do after a first seizure [See page 20]Differences in recommendations when diagnosing epilepsy [See page 22]Recommendations on where to perform investigations for children [See page 22]Differences in recommendations for using an EEG when diagnosing epilepsy [See page 22]Differences in recommendations for using an ECG when diagnosing epilepsy [See page 23]Differences in recommendations for the use of neuroimaging when investigating the causeof epilepsy [See page 23]Differences in recommendations for the use of blood tests and other investigations wheninvestigating the cause of epilepsy [See page 23]Differences in recommendations when starting treatment with AEDs [See page 24]Differences in recommendations for continuing treatment with AEDs [See page 24]Differences in recommendations for the use of psychological interventions [See page 24]Differences in recommendations for the use of the ketogenic diet [See page 25]Differences in recommendations for treating refractory convulsive status epilepticus [Seepage 25]Recommendations for referral to tertiary care that are specific for children [See page 24]Differences in recommendations for conducting a review [See page 26]

    Epilepsy overview NICE Pathways

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    http://pathways.nice.org.uk/pathways/epilepsy/referral-to-a-tertiary-epilepsy-service

  • Licensing indications

    Detailed below are the anti-epileptic drugs (AEDs) that have been recommended in thispathway but that do not currently have licensed indications for these seizures types orsyndromes or particular populations.

    Seizure type/syndrome

    Drug Details of licensing

    Clobazam

    At the time of publication, clobazam did not have UKmarketing authorisation for use in children younger than 3years (BNFC). There was insufficient experience of theuse of this drug in children younger than 6 years toenable any dosage recommendation to be made (SPC).It did have authorisation for adjunctive therapy forepilepsy, monotherapy under specialist supervision forcatamenial (menstruation) seizures (usually for 710days each month, just before and during menstruation),and cluster seizures (BNFC).

    Eslicarbazepineacetate

    At the time of publication, eslicarbazepine acetate did nothave UK marketing authorisation for use in childrenyounger than 18 years. It was not recommended owing toa lack of data on safety and efficacy (SPC).

    Gabapentin

    At the time of publication, gabapentin did not have UKmarketing authorisation for use in children younger than 6years and at doses over 50 mg/kg daily in childrenyounger than 12 years (BNFC). The use of gabapentinwas not recommended in children younger than 6 yearsowing to the lack of sufficient supporting data (SPC).

    Treatment ofrefractory focalseizures

    PregabalinAt the time of publication, pregabalin did not have UKmarketing authorisation for use in children (BNF).

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  • Pregabalin was not recommended for use in childrenyounger than 12 years and adolescents (1217 years)owing to insufficient data on safety and efficacy (SPC).

    Zonisamide

    At the time of publication, zonisamide did not have UKmarketing authorisation for use in children younger than18 years owing to insufficient data on safety and efficacy(SPC).

    Clobazam

    At the time of publication, clobazam did not have UKmarketing authorisation for use in children younger than 3years (BNFC). There was insufficient experience of theuse of this drug in children younger than 6 years toenable any dosage recommendation to be made (SPC).It did have authorisation for adjunctive therapy forepilepsy, monotherapy under specialist supervision forcatamenial (menstruation) seizures (usually for 710days each month, just before and during menstruation),and cluster seizures (BNFC).

    GTC

    Oxcarbazepine

    At the time of publication, oxcarbazepine did not have UKmarketing authorisation for GTC seizures (BNF). It hadauthorisation for focal seizures with or without secondaryGTC seizures (BNF).

    Absenceseizures

    Clobazam

    At the time of publication, clobazam did not have UKmarketing authorisation for use in children younger than 3years (BNFC). There was insufficient experience of theuse of this drug in children younger than 6 years toenable any dosage recommendation to be made (SPC).It did have authorisation for adjunctive therapy forepilepsy, monotherapy under specialist supervision forcatamenial (menstruation) seizures (usually for 710

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  • days each month, just before and during menstruation),and cluster seizures (BNFC).

    Lamotrigine

    At the time of publication, lamotrigine had UK marketingauthorisation for monotherapy of typical absenceseizures for those aged 212 years only. There was notauthorisation outside of this age range (BNF).

    Levetiracetam

    At the time of publication, levetiracetam did not have UKmarketing authorisation for use in absence seizures. Ithad authorisation for monotherapy and adjunctivetreatment of focal seizures with or without secondarygeneralisation and adjunctive therapy of myoclonicseizures in patients with JME and GTC seizures (BNF).

    Topiramate

    At the time of publication, topiramate did not have UKmarketing authorisation for use in absence seizures. Ithad authorisation for monotherapy and adjunctivetreatment of focal seizures and GTC seizures andadjunctive treatment for seizures associated withLennoxGastaut syndrome (BNF).

    Zonisamide

    At the time of publication, zonisamide did not have UKmarketing authorisation for use in absence seizures. Ithad authorisation for adjunctive therapy for adult patientswith refractory focal seizures, with or without secondarygeneralisation (BNF).

    Myoclonicseizures

    Clobazam

    At the time of publication, clobazam did not have UKmarketing authorisation for use in children younger than 3years (BNFC). There was insufficient experience of theuse of this drug in children younger than 6 years toenable any dosage recommendation to be made (SPC).It did have authorisation for adjunctive therapy for

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  • epilepsy, monotherapy under specialist supervision forcatamenial (menstruation) seizures (usually for 710days each month, just before and during menstruation),and cluster seizures (BNFC).

    Levetiracetam

    At the time of publication, levetiracetam did not have UKmarketing authorisation for monotherapy use inmyoclonic seizures. It had authorisation for monotherapyand adjunctive treatment of focal seizures with or withoutsecondary generalisation and adjunctive therapy ofmyoclonic seizures in patients with JME and GTCseizures (BNF).

    Topiramate

    At the time of publication, topiramate did not have UKmarketing authorisation for use in myoclonic seizures. Ithad authorisation for monotherapy and adjunctivetreatment of focal seizures and GTC seizures andadjunctive treatment for seizures associated withLennoxGastaut syndrome (BNF).

    Zonisamide

    At the time of publication, zonisamide did not have UKmarketing authorisation for use in myoclonic seizures. Ithad authorisation for use in adjunctive treatment ofrefractory focal seizures with or without secondarygeneralisation (BNF).

    Tonic or atonicseizures

    Lamotrigine

    At the time of publication, lamotrigine did not have UKmarketing authorisation for use in tonic or atonicseizures. It had authorisation for monotherapy andadjunctive treatment of focal seizures, GTC seizures andseizures associated with LennoxGastaut syndrome. Italso had authorisation for monotherapy of typicalabsence seizures for children aged 212 years (BNF,BNFC).

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  • Topiramate

    At the time of publication, topiramate did not have UKmarketing authorisation for use in tonic or atonicseizures. It had authorisation for monotherapy andadjunctive treatment of focal seizures and GTC seizuresand adjunctive treatment for seizures associated withLennoxGastaut syndrome (BNF).

    Infantile spasmsACTH(tetracosactide)

    At the time of publication, ACTH (tetracosactide) did nothave UK marketing authorisation for infantile spasms.Depot ampoules are not recommended in infants andchildren younger than 3 years owing to the presence ofbenzyl alcohol in the formulation (SPC).

    LennoxGastautsyndrome

    FelbamateAt the time of publication, felbamate did not have UKmarketing authorisation. There was no SPC available.

    Clobazam

    At the time of publication, clobazam did not have UKmarketing authorisation for use in children under 3 yearsof age (BNFC). There was insufficient experience of theuse of this drug in children younger than 6 years toenable any dosage recommendation to be made (SPC).It did have authorisation for adjunctive therapy forepilepsy, monotherapy under specialist supervision forcatamenial (menstruation) seizures (usually for 710days each month, just before and during menstruation),and cluster seizures (BNFC).

    Dravetsyndrome

    Topiramate

    At the time of publication, topiramate did not have UKmarketing authorisation for use in Dravet syndrome. Ithad authorisation for monotherapy and adjunctivetreatment of focal seizures and GTC seizures andadjunctive treatment for seizures associated withLennoxGastaut syndrome (BNF).

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  • Carbamazepine

    At the time of publication, carbamazepine did not haveUK marketing authorisation for BECTS/Panayiotopoulossyndrome and late-onset childhood occipital epilepsy(Gastaut type). It had authorisation for focal and GTCseizures (BNF).

    Clobazam

    At the time of publication, clobazam did not have UKmarketing authorisation for use in children younger than 3years (BNFC). There was insufficient experience of theuse of this drug in children younger than 6 years toenable any dosage recommendation to be made (SPC).It did have authorisation for adjunctive therapy forepilepsy, monotherapy under specialist supervision forcatamenial (menstruation) seizures (usually for 710days each month, just before and during menstruation),and cluster seizures (BNFC).

    Eslicarbazepineacetate

    At the time of publication, eslicarbazepine acetate did nothave UK marketing authorisation for use in childrenyounger than 18 years. It was not recommended owing toa lack of data on safety and efficacy (SPC).

    BECTS/Panayiotopoulossyndrome andlate-onsetchildhoodoccipitalepilepsy(Gastaut type)

    Gabapentin

    At the time of publication, gabapentin did not have UKmarketing authorisation for BECTS/Panayiotopoulossyndrome and late-onset childhood occipital epilepsy(Gastaut type). It had authorisation for use in focalseizures with and without secondary generalisation (BNF)but it did not have UK marketing authorisation for use inchildren younger than 6 years and at doses over 50 mg/kg daily in children younger than 12 years (BNFC). Theuse of gabapentin was not recommended in childrenyounger than 6 years owing to the lack of sufficientsupporting data (SPC).

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  • Lacosamide

    At the time of publication, lacosamide did not have UKmarketing authorisation for BECTS/Panayiotopoulossyndrome and late-onset childhood occipital epilepsy(Gastaut type). It had authorisation for adjunctivetreatment of focal seizures with or without secondarygeneralisation (BNF).

    Lamotrigine

    At the time of publication, lamotrigine did not have UKmarketing authorisation for BECTS/Panayiotopoulossyndrome and late-onset childhood occipital epilepsy(Gastaut type). It had authorisation for monotherapy andadjunctive treatment of focal and GTC seizures, seizuresassociated with LennoxGastaut syndrome, andmonotherapy treatment of typical absence seizures inchildren aged 2 to 12 years (BNF).

    Levetiracetam

    At the time of publication, levetiracetam did not have UKmarketing authorisation for BECTS/Panayiotopoulossyndrome and late-onset childhood occipital epilepsy(Gastaut type). It had authorisation for monotherapy andadjunctive treatment of focal seizures with or withoutsecondary generalisation and adjunctive therapy ofmyoclonic seizures in patients with JME and GTCseizures (BNFC).

    Oxcarbazepine

    At the time of publication, oxcarbazepine did not have UKmarketing authorisation for BECTS/Panayiotopoulossyndrome and late-onset childhood occipital epilepsy(Gastaut type). It had authorisation for focal seizures withor without secondary GTC seizures (BNF).

    PregabalinAt the time of publication, pregabalin did not have UKmarketing authorisation for use in children (BNF).Pregabalin was not recommended for use in children

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  • younger than 12 years and adolescents (1217 years)owing to insufficient data on safety and efficacy (SPC).

    Tiagabine

    At the time of publication, tiagabine did not have UKmarketing authorisation for BECTS/Panayiotopoulossyndrome and late-onset childhood occipital epilepsy(Gastaut type). It had authorisation for focal seizures withor without secondary generalisation that are notsatisfactorily controlled by other antiepileptics (BNF).

    Topiramate

    At the time of publication, topiramate did not have UKmarketing authorisation for BECTS/Panayiotopoulossyndrome and late-onset childhood occipital epilepsy(Gastaut type). It had authorisation for monotherapy andadjunctive treatment of focal seizures and GTC seizuresand adjunctive treatment for seizures associated withLennox-Gastaut syndrome (BNF).

    Vigabatrin

    At the time of publication, vigabatrin did not have UKmarketing authorisation for BECTS/Panayiotopoulossyndrome. It can be prescribed in combination with otherepileptic treatment for focal epilepsy with or withoutsecondary generalisation (BNF).

    Zonisamide

    At the time of publication, zonisamide did not have UKmarketing authorisation for BECTS/Panayiotopoulossyndrome and late-onset childhood occipital epilepsy(Gastaut type). It had authorisation for adjunctive therapyfor adult patients with refractory focal seizures, with orwithout secondary generalisation (BNF).

    IGE ClobazamAt the time of publication, clobazam did not have UKmarketing authorisation for use in children younger than 3years (BNFC). There was insufficient experience of the

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  • use of this drug in children younger than 6 years toenable any dosage recommendation to be made (SPC).It did have authorisation for adjunctive therapy forepilepsy, monotherapy under specialist supervision forcatamenial (menstruation) seizures (usually for 710days each month, just before and during menstruation),and cluster seizures (BNFC).

    Lamotrigine

    At the time of publication, lamotrigine did not have UKmarketing authorisation for use in IGE. It hadauthorisation for monotherapy and adjunctive treatmentof focal and GTC seizures, seizures associated withLennoxGastaut syndrome, and monotherapy treatmentof typical absence seizures in children aged 2 to 12 years(BNF).

    Levetiracetam

    At the time of publication, levetiracetam did not have UKmarketing authorisation for IGE. It had authorisation formonotherapy and adjunctive treatment of focal seizureswith or without secondary generalisation and adjunctivetherapy of myoclonic seizures in patients with JME andGTC seizures (BNF).

    Topiramate

    At the time of publication, topiramate did not have UKmarketing authorisation for use in IGE. It hadauthorisation for monotherapy and adjunctive treatmentof focal seizures and GTC seizures and adjunctivetreatment for seizures associated with LennoxGastautsyndrome (BNF).

    ZonisamideAt the time of publication, zonisamide did not have UKmarketing authorisation for use in IGE. It hadauthorisation for adjunctive therapy for adult patients with

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  • refractory focal seizures, with or without secondarygeneralisation (BNF).

    Clobazam

    At the time of publication, clobazam did not have UKmarketing authorisation for use in children younger than 3years (BNFC). There was insufficient experience of theuse of this drug in children younger than 6 years toenable any dosage recommendation to be made (SPC).It did have authorisation for adjunctive therapy forepilepsy, monotherapy under specialist supervision forcatamenial (menstruation) seizures (usually for 710days each month, just before and during menstruation),and cluster seizures (BNFC).

    Lamotrigine

    At the time of publication, lamotrigine did not have UKmarketing authorisation for use in juvenile myoclonicepilepsy. It had authorisation for monotherapy andadjunctive treatment of focal and GTC seizures, seizuresassociated with LennoxGastaut syndrome, andmonotherapy treatment of typical absence seizures inchildren aged 2 to 12 years (BNF).

    Levetiracetam

    At the time of publication, levetiracetam did not have UKmarketing authorisation for monotherapy use in JME. Ithad authorisation for monotherapy and adjunctivetreatment of focal seizures with or without secondarygeneralisation and adjunctive therapy of myoclonicseizures in patients with JME and GTC seizures (BNF).

    JME

    Topiramate

    At the time of publication, topiramate did not have UKmarketing authorisation for use in JME. It hadauthorisation for monotherapy and adjunctive treatmentof focal seizures and GTC seizures and adjunctive

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  • treatment for seizures associated with LennoxGastautsyndrome (BNF).

    Zonisamide

    At the time of publication, zonisamide did not have UKmarketing authorisation for use in JME. It hadauthorisation for adjunctive therapy for adult patients withrefractory focal seizures, with or without secondarygeneralisation (BNF).

    Clobazam

    At the time of publication, clobazam did not have UKmarketing authorisation for use in children younger than 3years (BNFC). There was insufficient experience of theuse of this drug in children younger than 6 years toenable any dosage recommendation to be made (SPC).It did have authorisation for adjunctive therapy forepilepsy, monotherapy under specialist supervision forcatamenial (menstruation) seizures (usually for 710days each month, just before and during menstruation),and cluster seizures (BNFC).

    Lamotrigine

    At the time of publication, lamotrigine had UK marketingauthorisation for monotherapy of typical absenceseizures for those aged 212 years only. There was noauthorisation outside this age range (BNF).

    Levetiracetam

    At the time of publication, levetiracetam did not have UKmarketing authorisation for use in absence syndromes. Ithad authorisation for monotherapy and adjunctivetreatment of focal seizures with or without secondarygeneralisation and adjunctive therapy of myoclonicseizures in patients with JME and GTC seizures (BNF).

    Absencesyndromes

    TopiramateAt the time of publication, topiramate did not have UKmarketing authorisation for use in absence syndromes. It

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  • had authorisation for monotherapy and adjunctivetreatment of focal seizures and GTC seizures andadjunctive treatment for seizures associated withLennoxGastaut syndrome (BNF).

    Zonisamide

    At the time of publication, zonisamide did not have UKmarketing authorisation for use in absence syndromes. Ithad authorisation for adjunctive therapy for adult patientswith refractory focal seizures, with or without secondarygeneralisation (BNF).

    Propofol

    At the time of publication, propofol did not have UKmarketing authorisation for status epilepticus but hadauthorisation for anaesthesia and sedation. Diprivan 2%,Propofol-Lipuro 2%, and Propoven 2% were not licensedfor use in children younger than 3 years; Diprofusor TCI('target controlled infusion') system was not licensed foruse in children (BNFC).

    Thiopentalsodium

    At the time of publication, thiopental sodium did not haveUK marketing authorisation for status epilepticus (only ifother measures fail, see section 4.8.2 in BNF), by slowintravenous injection (BNF). It is authorised for convulsivestates: 75 to 125 mg (3 to 5 ml of a 2.5% solution) givenby intravenous infusion (SPC).

    MidazolamAt the time of publication, midazolam injection did nothave UK marketing authorisation for status epilepticus(BNF, BNFC).

    Statusepilepticus

    Diazepam

    At the time of publication, diazepam did not have UKmarketing authorisation for the use of Rectubes andStesolid Rectal Tubes in children younger than 1 year(BNFC).

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  • Abbreviations: BECTS, benign epilepsy with centrotemporal spikes; BNF, British nationalformulary; BNFC, British national formulary for children; GTC, generalised tonicclonic; IGE,idiopathic generalised epilepsy; JME, juvenile myoclonic epilepsy; SPC, summary of productcharacteristics.

    Differences in recommendations for helping people to cope with epilepsy

    Recommendations specific for children and young people

    In children and young people, self-management of epilepsy may be best achieved throughactive child-centred training models and interventions.

    Recommendations specific for adults

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

    Differences in recommendations for what to do after a first seizure

    Recommendations specific for children and young people

    The information that should be obtained from the child or young person and/or parent or carerafter a suspected seizure is contained in appendix D of the epilepsy NICE guideline.

    It is recommended that all children and young people who have had a first non-febrile seizureshould be seen as soon as possible1 by a specialist in the management of the epilepsies toensure precise and early diagnosis and initiation of therapy as appropriate to their needs.

    Recommendations specific for adults

    The information that should be obtained from the adult and/or family or carer after a suspectedseizure is contained in appendix D of the epilepsy NICE guideline.

    It is recommended that all adults having a first seizure should be seen as soon as possible by aspecialist in the management of the epilepsies to ensure precise and early diagnosis andinitiation of therapy as appropriate to their needs.

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    http://guidance.nice.org.uk/CG137/Guidance/Appendices/DifferentialDiagnosis/pdf/Englishhttp://guidance.nice.org.uk/CG137/Guidance/Appendices/DifferentialDiagnosis/pdf/English

  • 1 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.

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  • Differences in recommendations when diagnosing epilepsy

    Recommendations specific for children and young people

    The diagnosis of epilepsy in children and young people should be established by a specialistpaediatrician with training and expertise in epilepsy.

    Recommendations specific for adults

    The diagnosis of epilepsy in adults should be established by a specialist medical practitionerwith training and expertise in epilepsy.

    Recommendations on where to perform investigations for children

    All investigations for children should be performed in a child-centred environment.

    Differences in recommendations for using an EEG when diagnosingepilepsy

    Recommendations specific for children and young people

    An electroencephalogram (EEG) should be performed only to support a diagnosis of epilepsy inchildren and young people. If an EEG is considered necessary, it should be performed after thesecond epileptic seizure but may, in certain circumstances, as evaluated by the specialist, beconsidered after a first epileptic seizure.

    In children and young people, a sleep EEG is best achieved through sleep deprivation or theuse of melatonin.

    Recommendations specific for adults

    An EEG should be performed only to support a diagnosis of epilepsy in adults in whom theclinical history suggests that the seizure is likely to be epileptic in origin.

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  • Differences in recommendations for the use of neuroimaging wheninvestigating the cause of epilepsy

    Recommendations specific for children and young people

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

    Differences in recommendations for the use of blood tests and otherinvestigations when investigating the cause of epilepsy

    Recommendations specific for children and young people

    In children and young people, other investigations, including blood and urine biochemistry,should be undertaken at the discretion of the specialist to exclude other diagnoses, and todetermine an underlying cause of the epilepsy.

    Recommendations specific for adults

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

    Differences in recommendations for using an ECG when diagnosingepilepsy

    Recommendations specific for children and young people

    In children and young people, a 12-lead electrocardiogram (ECG) should be considered incases of diagnostic uncertainty.

    Recommendations specific for adults

    A 12-lead ECG should be performed in adults with suspected epilepsy.

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  • Differences in recommendations when starting treatment AEDs

    Recommendations specific for children and young people

    Anti-epileptic drug (AED) therapy in children and young people should be initiated by aspecialist.

    Recommendations specific for adults

    AED therapy should be initiated in adults on the recommendation of a specialist.

    Differences in recommendations for continuing treatment with AEDs

    Recommendations specific for children and young people

    Regular blood test monitoring in children and young people is not recommended as routine, andshould be done only if clinically indicated and recommended by the specialist.

    Recommendations specific for adults

    Regular blood test monitoring in adults is not recommended as routine, and should be doneonly if clinically indicated.

    Recommendations for referral to tertiary care that are specific for children

    In children, the diagnosis and management of epilepsy within the first few years of life may beextremely challenging. For this reason, children with suspected epilepsy should be referred totertiary services early, because of the profound developmental, behavioural and psychologicaleffects that may be associated with continuing seizures.

    Differences in recommendations for the use of psychological interventions

    Recommendations specific for children and young people

    Psychological interventions (relaxation, cognitive behaviour therapy) may be used in childrenand young people with drug-resistant focal epilepsy.

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  • Recommendations specific for adults

    Psychological interventions (relaxation, cognitive behaviour therapy, biofeedback) may be usedin conjunction with anti-epileptic drug (AED) therapy in adults where either the person or thespecialist considers seizure control to be inadequate with optimal AED therapy. This approachmay be associated with an improved quality of life in some people.

    Differences in recommendations for the use of the ketogenic diet

    Recommendations specific for children and young people

    Refer children and young people with epilepsy whose seizures have not responded toappropriate anti-epileptic drugs (AEDs) to a tertiary paediatric epilepsy specialist forconsideration of the use of a ketogenic diet.

    Recommendations specific for adults

    No recommendation has been made for adults.

    Differences in recommendations for treating refractory convulsive statusepilepticus

    Recommendations specific for children and young people

    Administer intravenous midazolam1 or thiopental sodium to treat children and young people withrefractory convulsive status epilepticus. Adequate monitoring, including blood levels of anti-epileptic drugs (AEDs), and critical life systems support are required. See also the suggestedprotocols in appendix F of the epilepsy NICE guideline.

    Recommendations specific for adults

    Administer intravenous midazolam, propofol or thiopental sodium to treat adults with refractoryconvulsive status epilepticus. Adequate monitoring, including blood levels of AEDs, and criticallife systems support are required. See also the suggested protocols in appendix F of theepilepsy NICE guideline.

    1 At the time of publication (January 2012), this drug did not have UK marketing authorisation for this indication and/

    or population (see Licensing indications [See page 8] for details). Informed consent should be obtained and

    documented in line with normal standards in emergency care.

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    http://publications.nice.org.uk/the-epilepsies-the-diagnosis-and-management-of-the-epilepsies-in-adults-and-children-in-primary-cg137/appendix-f-protocols-for-treating-convulsive-status-epilepticus-in-adults-and-children-adultshttp://publications.nice.org.uk/the-epilepsies-the-diagnosis-and-management-of-the-epilepsies-in-adults-and-children-in-primary-cg137/appendix-f-protocols-for-treating-convulsive-status-epilepticus-in-adults-and-children-adults

  • Differences in recommendations for conducting a review

    Recommendations specific for children and young people

    Children and young people should have a regular structured review with a specialist.

    For children and young people, the maximum interval between reviews should be 1 year, butthe frequency of reviews should be determined by the child or young person's epilepsy and theirwishes and those of the family and/or carers. The interval between reviews should be agreedbetween the child or young person, their family and/or carers as appropriate, and the specialist,but is likely to be between 3 and 12 months.

    Recommendations specific for adults

    Adults should have a regular structured review with their GP, but depending on the person'swishes, circumstances and epilepsy, the review may be carried out by the specialist.

    For adults, the maximum interval between reviews should be 1 year but the frequency of reviewwill be determined by the person's epilepsy and their wishes.

    Adults should have regular reviews. In addition, access to either secondary or tertiary careshould be available to ensure appropriate diagnosis, investigation and treatment if the person orclinician view the epilepsy as inadequately controlled.

    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.

    Glossary

    Absence seizures

    Seizures characterised by behavioural arrest associated with generalised spike wave activity onEEG.

    Adherence

    The extent to which the person's behaviour matches the prescriber's recommendations.Adherence emphasises the need for agreement and that the patient is free to decide whether ornot to adhere to the doctor's recommendation.

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  • Adjunctive treatment

    Where a medication is added to a first-line anti-epileptic drug (AED) for combination therapy.

    Aetiology

    The cause or origin of a disease or disorder as determined by medical diagnosis.

    Adult

    Aged 18 years and older.

    Anti-epileptic drug

    (AED) Medication taken daily to prevent the recurrence of epileptic seizures.

    Atonic seizures

    Generalised seizures characterised by sudden onset of loss of muscle tone.

    Attack

    An episode in the course of an illness.

    Baseline

    The initial set of measurements at the beginning of a study (after run-in period whereapplicable), with which subsequent results are compared.

    Benign epilepsy with centrotemporal spikes

    (BECTS) An epilepsy syndrome of childhood (514 years) characterised by focal motor and/orsecondarily generalised seizures, the majority from sleep, in an otherwise normal individual,with centrotemporal spikes seen on EEG.

    Carer

    Someone other than a healthcare professional who is involved in caring for a person with amedical condition.

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  • Childhood absence epilepsy

    An epilepsy syndrome with an age of onset of 49 years, characterised by frequent absenceseizures associated with 3 Hz spike wave activity on EEG.

    Child

    Aged 28 days to 11 years.

    Children

    Aged 28 days to 11 years.

    Clinical presentation

    The description of the history and presentation of the clinical condition to the assessing medicalteam.

    Clinician

    A healthcare professional providing direct patient care (for example, doctor, nurse orphysiotherapist).

    Comorbidity

    Co-existence of more than one disease or an additional disease (other than that being studiedor treated) in a person.

    Concordance

    This is a recent term, the meaning of which has changed. It was initially applied to theconsultation process in which doctor and patient agree therapeutic decisions that incorporatetheir respective views, but now includes supporting patients in medicine-taking as well ascommunication when prescribing. Concordance reflects social values but does not addressmedicine-taking and may not lead to improved adherence.

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  • Continuous spike and wave during slow sleep

    (CSWS) An epilepsy syndrome with childhood onset, characterised by a plateau and regressionof cognitive abilities associated with dramatic increase in spike wave activity in slow wave sleep(> 85% of slow sleep). There may be few seizures at presentation.

    Convulsive status epilepticus

    When a convulsive seizure continues for a prolonged period (longer than 5 minutes), or whenconvulsive seizures occur one after the other with no recovery between. Convulsive statusepilepticus is an emergency and requires immediate medical attention.

    Dosage

    The prescribed amount of a drug to be taken, including the size and timing of the doses.

    Dravet syndrome

    Previously known as severe myoclonic epilepsy of infancy. An epilepsy syndrome with onset ininfancy, characterised by initial prolonged, typically lateralised, febrile seizures, subsequentdevelopment of multiple seizure types including myoclonic, absence, focal and generalisedtonicclonic seizures, with developmental plateau or regression.

    Electrocardiogram

    (ECG) A test that records the heart's electrical activity.

    Electroencephalogram

    (EEG) An investigation that involves recording the electrical activity of the brain. Electrodes areattached to standardised points on the person's head with collodion. Recordings are usuallytaken across two points.

    Epileptic seizure

    A transient occurrence of signs and/or symptoms, the result of a primary change to the electricalactivity (abnormally excessive or synchronous) in the brain.

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  • Epilepsy syndrome

    A distinctive disorder identifiable on the basis of a typical age of onset, seizure types, specificEEG characteristics, and often other features. Identification of epilepsy syndrome hasimplications for treatment, management and prognosis.

    Focal seizures

    Seizures that originate within networks limited to one hemisphere, discretely localised or morewidely distributed. Replaces the terms partial seizure and localisation-related seizure.

    Generalised seizures

    Seizures that originate in, and rapidly engage, bilaterally distributed networks. Such bilateralnetworks can include cortical and subcortical structures but do not necessarily include the entirecortex.

    Generalised tonicclonic (GTC) seizures

    A seizure of sudden onset involving generalised stiffening and subsequent rhythmic jerking ofthe limbs, the result of rapid widespread engagement of bilateral cortical and subcorticalnetworks in the brain.

    Genetic

    With reference to epilepsy the epilepsy is, as best as understood, the direct result of a knownor presumed genetic defect(s) in which seizures are the core symptom of the disorder.

    Ictal phenomenology

    Description or history of ictal events (seizures).

    Idiopathic

    A syndrome that is only epilepsy, with no underlying structural brain lesion or other neurologicalsigns or symptoms. These are presumed to be genetic in aetiology and are usually agedependent.

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  • Idiopathic generalised epilepsy

    (IGE) A well-defined group of disorders characterised by typical absences, myoclonic andgeneralised tonicclonic seizures, alone or in varying combinations in otherwise normalindividuals. The EEG is also characteristic, demonstrating a distinct pattern of generalisedpolyspike wave discharges and/or generalised spike wave. Presumed to have a geneticaetiology. The new classification of the International League Against Epilepsy (ILAE, 2010)suggests the terminology should change to genetic generalised epilepsy (GGE).

    Infantile spasms

    A specific seizure type presenting in the first year of life, most commonly between 3 and 9months. Spasms are brief axial movements lasting 0.22 seconds, most commonly flexor innature, involving flexion of the trunk with extension of the upper and lower limbs. They areoccasionally referred to as 'salaam seizures'.

    Intervention

    Healthcare action intended to benefit the patient, for example, drug treatment, surgicalprocedure or psychological therapy.

    Juvenile absence epilepsy

    An epilepsy syndrome with an age of onset of 913 years characterised by absence seizures,associated with 34 Hz spike wave on EEG. Generalised tonicclonic seizures may occur.

    Juvenile myoclonic epilepsy

    (JME) An epilepsy syndrome with an age of onset of 520+ years (peak 1016 years)characterised by myoclonic seizures that most commonly occur soon after waking. Absence andgeneralised tonicclonic seizures may occur in between 50 and 80% of people with JME. EEGdemonstrates 36 Hz generalised polyspike and wave activity, with photosensitivity in more than30% of people.

    Ketogenic diet

    A specific diet that is high in fat but low in carbohydrates and protein.

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  • LandauKleffner syndrome

    (LKS) A very rare epilepsy syndrome with an age of onset of 36 years characterised by loss oflanguage (after a period of normal language development) associated with an epilepsy ofcentrotemporal origin, more specifically bitemporal spikes on EEG with enhancement in sleep orcontinuous spike and wave during slow sleep.

    Late-onset childhood occipital epilepsy (Gastaut type)

    Epilepsy with an age of onset in mid-childhood to adolescence with frequent brief seizurescharacterised by initial visual hallucinations, ictal blindness, vomiting and post-ictal headache.EEG typically shows interictal occipital spikes attenuated by eye opening.

    LennoxGastaut syndrome

    An epilepsy syndrome with an age of onset of 310 years characterised by multiple seizuretypes (including atonic, tonic, tonicclonic and atypical absence seizures), cognitive impairmentand specific EEG features of diffuse slow spike and wave (< 2 Hz) as well as paroxysmal fastactivity (10 Hz or more) in sleep.

    Monotherapy

    Use of a single drug in treatment.

    Myoclonic-astatic epilepsy

    (MAE) Also known as Doose syndrome. An epilepsy syndrome with an age of onset of 1860months, characterised by different seizure types with myoclonic and myoclonic-astatic seizuresseen in all, causing children to fall. The EEG shows generalised spike/polyspike and waveactivity at 26 Hz.

    Myoclonic seizures

    Sudden brief (

  • Non-convulsive status epilepticus

    A change in mental status or behaviour from baseline, associated with continuous seizureactivity on EEG, which is also seen to be a change from baseline.

    Non-epileptic attack disorder

    (NEAD) A disorder characterised by episodes of change in behaviour or movement, not causedby a primary change in electrical activity of the brain. Movements are varied, and the attackscan be difficult to differentiate from epileptic seizures.

    Older people

    Aged 65 years or older.

    Panayiotopoulos syndrome

    Epilepsy syndrome presenting in early childhood (mean 47 years) with rare seizures that areprolonged. Characterised by autonomic features including vomiting, pallor and sweatingfollowed by tonic eye deviation, impairment of consciousness with possible evolution intosecondary generalisation. Prognosis is excellent and treatment often unnecessary.

    Pharmacokinetic

    Pharmacokinetics is a term used to describe the way in which a drug is processed by the body,influencing absorption, metabolism, distribution and excretion.

    Polypharmacy

    Multiple different drugs used in a patient's treatment, which could include anti-epileptic drugs(AEDs).

    Polytherapy

    Two or more medications used in combination therapy. The pathway specifically refers to anti-epileptic drugs (AEDs).

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  • Prognosis

    A probable course or outcome of a disease. Prognostic factors are patient or diseasecharacteristics that influence the course of a disease. Good prognosis is associated with a lowrate of undesirable outcomes; poor prognosis is associated with a high rate of undesirableoutcomes.

    Quality of life

    A combination of a person's physical, mental and social wellbeing; not just the absence ofdisease.

    Specialist

    For children and young people: a paediatrician with training and expertise in epilepsy. Foradults: a medical practitioner with training and expertise in epilepsy.

    Sudden unexpected death in epilepsy

    (SUDEP) Sudden, unexplained, witnessed or unwitnessed, non-traumatic and non-drowningdeath in people with epilepsy, with or without evidence for a seizure, and excluding documentedstatus epilepticus, in which post-mortem examination does not reveal a toxicological oranatomic cause for death.

    Syncope

    A brief lapse in consciousness caused by transient reduction in blood flow to the brain. May becaused by many different factors, including emotional stress, vagal stimulation, vascular poolingin the legs, diaphoresis, or sudden change in environmental temperature or body position.

    Tertiary epilepsy specialist

    An adult neurologist who devotes the majority of their working time to epilepsy, is working in amultidisciplinary tertiary referral centre with appropriate diagnostic and therapeutic resources,and is subject to regular peer review.

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  • Tertiary paediatric epilepsy specialist

    A paediatric neurologist who devotes the majority of their working time to epilepsy, is working ina multidisciplinary tertiary referral centre with appropriate diagnostic and therapeutic resources,and is subject to regular peer review.

    Tertiary service

    Specialist care delivery unit, to which people may be referred from secondary care.

    Tonic

    Tonic seizures are epileptic seizures characterised by abrupt generalised muscle stiffeningpossibly causing a fall. The seizure usually lasts less than a minute and recovery is rapid.

    Tonicclonic

    Tonicclonic seizures are epileptic seizures characterised by initial generalised musclestiffening, followed by rhythmical jerking of the limbs, usually lasting a few minutes. The personmay bite their tongue and may be incontinent. They may feel confused or sleepy afterwards,and take a while to recover fully.

    Tonicclonic seizure

    An epileptic seizure characterised by initial generalised muscle stiffening, followed by rhythmicaljerking of the limbs, usually lasting a few minutes. The person may bite their tongue and may beincontinent. They may feel confused or sleepy afterwards, and take a while to recover fully.

    Young people

    Aged 12 to 17 years.

    Young person

    Aged 12 to 17 years.

    Sources

    Epilepsy. NICE clinical guideline 137 (2012)

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    http://guidance.nice.org.uk/CG137

  • Your responsibility

    The guidance in this pathway represents the view of NICE, which was arrived at after carefulconsideration of the evidence available. Those working in the NHS, local authorities, the widerpublic, voluntary and community sectors and the private sector should take it into account whencarrying out their professional, managerial or voluntary duties. Implementation of this guidanceis the responsibility of local commissioners and/or providers. Commissioners and providers arereminded that it is their responsibility to implement the guidance, in their local context, in light oftheir duties to avoid unlawful discrimination and to have regard to promoting equality ofopportunity. Nothing in this guidance should be interpreted in a way which would be inconsistentwith compliance with those duties.

    Copyright

    Copyright National Institute for Health and Care Excellence 2015. All rights reserved. NICEcopyright material can be downloaded for private research and study, and may be reproducedfor educational and not-for-profit purposes. No reproduction by or for commercial organisations,or for commercial purposes, is allowed without the written permission of NICE.

    Contact NICE

    National Institute for Health and Care ExcellenceLevel 1A, City TowerPiccadilly PlazaManchesterM1 4BT

    www.nice.org.uk

    [email protected]

    0845 003 7781

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    http://www.nice.org.ukmailto://[email protected]


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