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North West Clinical Neuroscience Partnership The Management of Adults with Epilepsy A North West Clinical Framework August 2001 (Final version) Contents Section 1 Section 2 Section 3 Introduction The North West Clinical Framework page 4 The Background Incidence and prevalence of epilepsy Diagnosis Investigation Classification of epilepsy Causation Status epilepticus Sudden death Medication Epilepsy in children and adolescents Epilepsy in women Epilepsy in older people Complex epilepsy Learning disability Intractable epilepsy Surgical treatment Non-epileptic attack disorder Impact of epilepsy Information Patterns of care Summary page 5 page 5 page 6 page 6 page 9 page 9 page 10 page 10 page 11 page 12 page 13 page 14 page 14 page 14 page 15 page 16 page 16 page 17 page 17 page 17 The Management of People with Epilepsy: Clinical Guidelines Introduction Diagnosis Not epilepsy Uncertain Epilepsy Treatment Starting antiepileptic drug therapy Management and follow-up Diagnosis uncertain Epilepsy Patient in remission with no side effects of treatment Patient having side effects of therapy or not in remission Refractory epilepsy Non-epileptic attacks Epilepsy Considering surgery Status epilepticus 2 page 19 page 19 page 21 page 22 page 22 page 23 page 23 page 25 page 25 page 26 page 26 page 27 page 28 page 29 page 29 page 31 page 33 Section 4 Section 5 Section 6 Section 7 Section 8 Section 9 The Adolescent with Epilepsy Services for children with epilepsy Adolescence Management of epilepsy Transition page 37 page 37 page 37 page 38 Women with Epilepsy Accuracy of diagnosis Selection of medication Contraception Pre-conception counselling Pregnancy Hormonal changes and seizures page 39 page 39 page 39 page 40 page 40 page 41 Epilepsy in Older People Who are “the elderly”? Diagnosis Implications Who should provide the service for older people? Epilepsy in Complex Situations Epilepsy in the context of learning disability Epilepsy with psychiatric or behavioural problems Refractory epilepsy page 42 page 42 page 43 page 44 page 45 page 47 page 48 The Epilepsy Service The Epilepsy Service The neurophysiology service page 51 page 56 Summary page 58 Appendix 1 North West Clinical Neuroscience Partnership page 63 Appendix 2 Participants and Advisors page 65 Appendix 3 Literature Survey page 66 References page 71 3 Section 1 Introduction The North West Clinical Framework This guidance has been produced by the North West Clinical Neuroscience Partnership as a Framework for the commissioning and provision of care for people with epilepsy in the North West of England and North Wales. It links all aspects of care in a single service with clearly identified roles and responsibilities. The North West Clinical Neuroscience Partnership is a partnership between the 3 Zonal Commissioners of Specialised Services in the North West, working on behalf of the Health Authorities and Primary Care Groups and Trusts in the North West, and the 3 Specialist Neuroscience Provider Trusts. The Partnership works in close cooperation with the North West Regional Specialised Commissioning Group and also includes representation from the Specialised Health Commissioning Service in Wales. The Partners have jointly agreed to develop a series of Clinical Frameworks and to work together to develop a Region-wide commissioning process which will ensure that the 7 million people of the North West and North Wales have access to a high quality Clinical Neuroscience service. The details of the Partners and the Partnership Agreement are included as Appendix 1. Guidelines for the management of people with epilepsy have been published by a number of organisations. The evidence based guidelines of the Scottish Royal Colleges (SIGN) were particularly helpful as a starting point in constructing the North West Framework. Many people have contributed to the development of this Framework. The process began in May 1999 with a meeting at which users and commissioners of the service were asked to address the question “What do I expect from an Epilepsy Service?” Following this a number of workshops and working groups have involved patients, carers, voluntary agencies, GPs, neurologists, neurophysiologists, neuropsychologists, neuropsychiatrists, specialists in learning disability, neurosurgeons and specialist nurses. The Partnership is extremely grateful to all those people who have given their time and expertise over the last 2 years: a list of participants in the process can be found in Appendix 2. It is planned to begin to use the Framework as a basis for commissioning in 2002 following any modification necessary in the light of consultation. The Partnership believes that the Framework will ensure the delivery of a high quality, reliable, accessible and sensitive service for people with epilepsy wherever they live in the North West and North Wales. August 2001 4 Section 2 The Background Incidence and prevalence of epilepsy Epilepsy is a relatively common condition characterised by a tendency to recurrent seizures. Although there are variations among ethnic (Wright et al 1) and socio-economic (Morgan et al2) groups within populations there is remarkable agreement that the incidence is about 50 per 100,000 per year over-all (Zarrelli et al3, MacDonald et al4). There is also agreement that prevalence in the population from age 10 to 60 remains steady at about 7 per 1000, with “active epilepsy” affecting 4-5 per 1000 (Wright et al1, MacDonald et al 4). The first seizure can occur at any age but onset is predominantly during childhood or older age. Not all children who develop epilepsy continue to have seizures into adult life and some adults enter remission. Thus in spite of a steady prevalence up to age 60 in the population the cumulative incidence continues to rise. After age 65 there is a marked rise in incidence from 85.9 per 100,000 to 135.4 in people over 85. (Wallace5). This means that about 30% of the population will have had epilepsy at some stage in their life (Anderson et al6). The General Practitioner with an average list size of about 2000 will see 1 person with a new diagnosis of epilepsy each year and will provide care for 10-20 people with epilepsy depending on the age and socioeconomic profile of the practice population. Some people have many seizures each day; others are free from seizures for months or years. Although the condition is chronic it does not always remain active throughout a person’s life. With appropriate treatment 68-86% of people with epilepsy can be free from seizures or have less than 1 seizure in 3 years (Cockerell et al7). Thus there are important distinctions between incidence and prevalence of epilepsy, prevalence of active epilepsy, and incidence and prevalence of the various types of epilepsy. Diagnosis The agreement on incidence and prevalence is more remarkable in the context of the difficulties that are encountered in defining and recognising seizures and hence reaching a diagnosis (Smith et al8, Zaidi et al9). Identification of the many different kinds of seizure, and diagnosis of the types of epilepsy depend upon the clinical history. Dependence on the clinical history as the basis of diagnosis places a relatively high value on the experience of the doctor taking the history, interpreting or amplifying the given description. Hart10 found that the time from the first seizure to diagnosis was more than 6 months in 50% of patients and over 2 years in 30%. Only about 25% of the people referred to neurology or epilepsy clinics with a possible 5 diagnosis of Epilepsy turn out to have that diagnosis (Smith et al8). Most have syncope (Zaidi et al9), (simple faints), or psychological, emotional, or personal problems. Syncope is often complicated by brief motor or ocular phenomena (Lempert et al11) which lead the unwary to an erroneous diagnosis of epilepsy. There is also good evidence to show that in at least 20% of patients given a diagnosis of epilepsy, the diagnosis is wrong (Smith et al8 , Zaidi et al9). The process of diagnosis is thus not easy. Investigation Diagnosis is rarely dependant on anything other than the history and a witness description but the prognosis and options for treatment are largely determined by the type (syndromic classification) of the epilepsy and by any identifiable cause (Semah et al12, Brodie and Dichter13). In looking for a cause or trying to establish the type of epilepsy, imaging of the brain (by CT or MR scanning) and neurophysiological studies, (electroencephalography, EEG), are necessary (King et al14). The EEG is particulary important in the classification of some forms of epilepsy. For those people with persisting or frequent seizures, especially partial seizures, further specialised imaging, neurophysiological and neuropsychological investigation may reveal a cause or show that surgical treatment could be of help (Devinsky15). Some findings, eg diffuse, low-grade glioma, might have no treatment implications but can explain the difficulty in controlling the seizures (Smith et al16). CT scanning is probably adequate for the identification of treatable causes of focal or localised epilepsy beginning in adult life although it will not reveal all lesions. MR scanning is more informative than CT scanning or Positron Emission Tomography (PET) and is essential when surgery is being considered (Heinz et al17, Helveston et al18). If MR is readily available it would be reasonable to use it rather than CT scanning in the investigation of epilepsy when imaging is considered necessary. Classification of epilepsy (Tables 1 and 2) Classification of the type of epilepsy is also based largely on the history and witness description of seizures but the EEG findings are very important in identifying some seizure types and syndromes. Doubts about the accuracy of descriptions (Samuel and Duncan19, Wulf20) or the interpretation of the description (Rinaldi et al21) have led to calls for a new classification which is easier to use outside specialist centres (Manford22, Everitt and Sander23). In spite of these difficulties, identification of the seizure type and syndrome is important because of the information they give on prognosis and on the most appropriate antiepileptic drug treatment (Devinsky15, Brown et al24). The extent of agreement on incidence and prevalence suggests that the diagnostic tools, though not perfect, are usable and helpful. 6 TABLE 1. (Modified from International League Against Epilepsy25) CLASSIFICATION OF EPILEPSIES AND EPILEPTIC SYNDROMES. 1 Localisation-related (focal, local, partial) epilepsies and syndromes 1.1. Idiopathic (with age-related onset) a. Benign childhood epilepsy with centrotemporal spikes b. Childhood epilepsy with occipital paroxysms 1.2. Symptomatic 1.3. Unknown as to whether the symptom is symptomatic or idiopathic 2. Generalized epilepsies 2.1. Idiopathic (with age-related onset, in order of age) a. Benign neonatal familial convulsions b. Benign neonatal convulsions c. Benign myoclonic epilepsy in infancy d. Childhood absence epilepsy (pyknoepilepsy) e. juvenile absence epilepsy f. Juvenile myoclonic epilepsy (impulsive petit mal) g. Epilepsy with grand mal (generalized tonic-clonic) seizures on awakening 2.2. Cryptogenic or symptomatic (in order of age) a. West’s syndrome (infantile spasms) b. Lennox-Gastaut syndrome c. Epilepsy with myoclonic-astatic seizures d. Epilepsy with myoclonic absence 2.3 Symptomatic 2.3.1. non-specific aetiology a. early myoclonic encephalopathy 2.3.2. specific syndromes a. epileptic seizures that complicate many disease states 3. Epilepsies and syndromes undetermined whether focal or generalized 3.1. with both generalised and focal seizures a. neonatal seizures b. Severe myoclonic epilepsy in infancy c. Epilepsy with continuous spike-wave during slow wave sleep d. Acquired epileptic aphasia (Landau-Kleffner syndrome) 4. Special syndromes a. Febrile convulsions 7 TABLE 2. (Modified from International League Against Epilepsy26) CLASSIFICATION OF EPILEPTIC SEIZURES ACCORDING TO CLINICAL TYPE. I. Partial (focal and local) seizures A. Simple partial seizures (consciousness not impaired) 1. With motor symptoms 2. With somatosensory or special sensory symptoms (simple hallucinations, such as tingling, light flashes, buzzing) 3. With autonomic symptoms or signs (e.g., epigastric sensation, pallor, sweating, flushing, piloerection, and pupillary dilatation) 4. With psychic symptoms (disturbances of higher cerebral function e.g. déjà vu, fear, distortion of time perception) B. Complex partial seizures (impairment of consciousness and often automatisms) 1. With simple partial onset followed by impairment of consciousness 2. With impairment of consciousness at onset C. Partial seizures evolving to secondarily generalized seizures (e.g., generalized tonic-clonic seizures) 1. Simple partial seizures evolving to generalized seizures 2. Complex partial seizures evolving to generalized seizures 3. Simple partial seizures evolving to complex partial seizures and further evolving to generalized seizures II. Generalized seizures (convulsive or nonconvulsive) A. Absence seizures (impairment of consciousness alone or with mild clonic, atonic, or tonic components and automatisms 1. Typical 2. Atypical B. Myoclonic seizures C. Clonic seizures D. Tonic seizures E. Tonic-clonic seizures F. Atonic seizures III. Unclassified seizures 8 Causation Epilepsy can be directly inherited through a single gene or can be associated with other clearly inherited conditions. While the inheritance of disorders directly causing epilepsy is uncommon there is a genetic influence in a high proportion of people with epilepsy (Steinlein27). Thus the incidence in children of a parent with epilepsy is 3 times that of the general population (Ottman et al28). Children of affected mothers are at greater risk than children of affected fathers (Ottman et al29). Some of the inherited degenerative or metabolic disorders are associated with seizures as a part of the much bigger clinical picture of delayed development or motor, sensory and intellectual decline. About 1/3 of people with learning disability have seizures (Sillanpaa 30, Graydon31). Infection, haemorrhage or trauma to the brain can cause epilepsy in children or adults: ischaemic stroke and some brain tumours are also prone to cause seizures, particularly in adults. Status epilepticus Status epilepticus is said to occur in more than 15% of patients with epilepsy at some stage in their life: either there is a series of seizures without return to normal in between, or a seizure lasts for more than 30 minutes. The event is recurrent in more than 13% of people (Fountain32). Other authors suggest a lower incidence drawing attention to the need for a precise definition and better management of repetitive seizures (Coeytaux et al33). Generalised convulsive status epilepticus Status epilepticus is as common when it is the first indication of an acute cerebral illness eg encephalitis as it is in the context of chronic epilepsy (Starreveld and Starreveld34). Particular risk factors are young age, low antiepileptic drug levels, fever in children and stroke in adults. Status epilepticus constitutes a medical emergency necessitating urgent parenteral medication and admission to hospital. If control is not achieved within 15 minutes, admission to an intensive care unit is essential so that muscle relaxants and assisted ventilation can be started. This allows the use of further antiepileptic medication which would suppress respiration. Status epilepticus still has a mortality of 7.6% (Coeytaux et al33) but this is largely related to the underlying cause (Starreveld and Starreveld34). There is general agreement that the recognition and early management of this condition is poor with misdiagnosis, delay in medication or inadequate doses contributing to the morbidity and mortality (Cascino et al35, Walker et al36). Care can be improved by the use of an algorithm, and education of staff in its use, even in the setting of a specialist service (Gilbert37). Nonconvulsive status epilepticus The definition, management and outcome of nonconvulsive status are more controversial. Until recently it was thought to be a relatively benign condition 9 characterised by prolonged periods of abnormal behaviour in older people which were reversed by the parenteral administration of benzodiazepines (Thomas38, Krumholz39). At least 4 types have now been described (Thomas38) and some authors suggest that up to 8% of comatose patients without signs of seizure activity have nonconvulsive status epilepticus (Towne et al40). Other authors feel that this observation is based on a misunderstanding of the EEG changes associated with coma (Niedermeyer and Ribiero41). In this situation it is not surprising that there is confusion over treatment. Seriously ill, comatose patients with EEG activity suggesting nonconvulsive status epilepticus do not appear to do well with aggressive medication to control the EEG; elderly patients displaying episodic confusion do respond to medication (Kaplan42). In the light of the difficulties with clinical diagnosis and EEG interpretation neurologists with expertise in epilepsy should be involved whenever suspicion of such a diagnosis arises. Sudden unexpected death Over the last few years it has become recognised that unexpected, sudden death is 2 or 3 times more common in people with epilepsy than in the population as a whole. Most authors believe that the deaths are seizure related (Opeskin et al43). Seizure related deaths might account for up to 40% of all deaths in people with chronic epilepsy (Tomson44). The mechanisms remain unclear but possible risk factors include male sex, age 20-40, generalised seizures, poor seizure control and poor compliance with medication (Langan45). Medication There is now a wide range of effective anticonvulsant or antiepileptic drugs. With appropriate selection and dosage about 70% of people with epilepsy will have less than one seizure in three years (Cockerell et al 7). There is, however, no perfect drug which combines efficacy with freedom from sideeffects. Some people would rather experience an occasional seizure than endure persisting drowsiness or sluggishness. For others the balance is different; even an occasional seizure could risk the loss of employment or driving licence so they are willing to accept side-effects. The selection of medication depends upon the type of epilepsy, the age and gender of the patient and the individual choice of each patient once the possibilities have been explained (Devinsky15). Treatment with a single drug is usually adequate: Almost 50% of patients experience satisfactory control with monotherapy of the first drug used. If adequate doses of one drug do not achieve control the substitution of another single drug achieves control in 27% of that group. Only when monotherapy with therapeutic doses of 2 appropriate drugs has failed should combination therapy be tried. Although there is no clinical trial based evidence, when a second drug is to be added there is a theoretical advantage in choosing a drug with a different mechanism of action (Devinsky15). About 7% of patients who continue to 10 have seizures in spite of adequate trials of monotherapy will have control improved by the addition of a second drug (Kwan and Brodie46). Side effects of antiepileptic drugs are important in determining their usefulness in practice. Individual idiosyncratic reactions, acute sensitivity reactions, interference with the metabolism of other drugs and the chronic results of very long-term treatment are all factors to be taken into consideration when prescribing these medications. Choosing between anti-epileptic drugs (See also Pathway 2, page 23) (a) Partial onset seizures (simple partial, complex partial, secondary generalised tonic clonic). Carbamazepine is the conventional drug of first choice (Mattson et al47). Valproate is effective but concerns about cosmetic effects and teratogenic potential limit its use in women of childbearing age. Claims that Lamotrigine (Brodie et al48) and Oxcarbazepine (Dam et al49) posses similar efficacy and better tolerability than Carbamazepine are not established. There is evidence of the effectiveness of Levetiracetam as an add-on medication (Shorvon et al50). However, like Gabapentin (Datta and Crawford51) and Topiramate (Sachdeo et al52) these new compounds are potential first-line agents. (b) Generalised onset seizures (absences, myoclonus, tonic and atonic drop attacks, primary generalised tonic clonic seizures). Despite a lack of hard scientific evidence Valproate is accepted as the drug of first choice which produces a therapeutic dilemma in a woman of childbearing age. There is evidence that Lamotrigine (Motte et al53) and Topiramate (Biton et al54) are also effective in suppressing these types of seizures but they have not yet been in use for long enough to know whether the risk to a foetus is significantly different. Epilepsy in children and adolescents The incidence of epilepsy in children is relatively high and although it sometimes disappears before adulthood the cumulative prevalence by age 20 remains little changed for the next 30 years (Wallace et al 5). Thus most people with epilepsy in early adult life and middle age acquired their epilepsy in childhood. Although this framework is concerned with the management of epilepsy in adults it is clear that for many people with epilepsy diagnosis, investigation and treatment will have occurred prior to entry into adult medical care. The quality of this earlier care will be an important factor in determining the needs and options for care in adult life. The time of transfer from paediatric services to the adult service occurs at a time of great change in the individual. The change from school to 11 employment, from dependence on parents to independence, the need to consider contraception, the effects of alcohol and possibly drugs all occur within a few years. For some people the move from a paediatric service to the adult service is difficult. In order to review the diagnosis and treatment and introduce people to the adult service it is generally suggested that there should be joint clinics for adolescents run by the adult Epilepsy Service in conjunction with paediatricians (CSAG55). Epilepsy in women For women there is an additional set of factors related to reproductive health to be taken into account. In some women seizures are concentrated around the monthly period (Bauer et al56) or change behaviour at the time of the menopause (Harden et al57). The reasons for this are not fully understood and as yet there is no medication proven to improve the situation. It has also been noted that epilepsy is associated with reduced fertility and that some medications might exacerbate this (Wallace et al5): in particular some authors have described a link between Valproate and polycystic ovaries (Isojarvi et al58). For many years it has been known that the effectiveness of hormonal contraception is reduced by anticonvulsant drugs capable of hepatic enzyme induction, and that many, if not all, antiepileptic drugs are associated with an increased risk to the foetus (Yerby59). Although it has proved difficult to disentangle the various factors it appears that babies born to women with epilepsy and taking antiepileptic medication are 2-3 times more likely to have congenital abnormalities (Samren et al60). The same study suggested that if only major abnormalities were considered the relative risk for mothers with epilepsy and taking antiepileptic medication was even greater. A population survey in Iceland showed no increase in adverse events overall but found women taking antiepileptic medication were 2.7 times more likely to give birth to a baby with a major congenital abnormality (Olafsson et al 61). Lower birthweight and significantly reduced cognitive and intellectual function have been found to occur commonly in babies of mothers treated for epilepsy (Hvas et al62, Adab et al63). The risks are higher when more than one antiepileptic drug is being taken. Preconception advice and planning can reduce some of these problems (Betts and Fox64) but it seems unlikely that they can be eliminated if continuing medication is required. The Report on Confidential Enquiries into Maternal Deaths in the United Kingdom for the 3 year period 1994-199665 notes that 19 of the 268 maternal deaths during that period occurred in women with epilepsy. This is a higher figure than would be expected in either non pregnant women with epilepsy or pregnant women who do not have epilepsy. Aspiration was present in about 50%. The report recommends that relatives should be instructed in the care of the person during a seizure and that pregnant women who are at risk of seizures should be told of the risks and advised not to bathe alone. There is an understandable fear that the infant of a mother with epilepsy could be at risk of serious injury during a maternal seizure. In women who have 12 received counselling prior to birth the risk in the puerperium, though real, appears to be low (Fox and Betts66). It is now generally recommended that these issues should be addressed explicitly (American Academy of Neurology67), probably through special clinics run in conjunction with obstetricians or endocrinologists. Epilepsy in older people Epilepsy in older people also presents particular diagnostic and therapeutic difficulties. Most elderly people presenting with blackouts, dizzy spells or falls do not have epilepsy (Allcock and O’Shea68). Investigation for these symptoms would thus be more appropriately initiated in a cardiac, neurocirculatory or geriatric setting with referral to the epilepsy service limited to those patients thought to have epilepsy after investigation. However, epilepsy is common in old age. Recent studies have shown that over one third of people who have a new diagnosis of epilepsy and who take antiepileptic drugs are over the age of 60 (Tallis et al69). Seizures beginning at this age are partial seizures and they most commonly arise in the context of cerebrovascular disease (Hyoshi and Yagi70), but in one study up to a quarter had tumours (Hughes and Zialciata71). Seizures occuring in the first two weeks after a stroke are less likely to be followed by recurrent seizures than those occurring at a greater time after the stroke (Berges et al72). The diagnosis of epilepsy can be difficult. In 30% of people developing seizures after the age of 60 the diagnosis takes more than 1 year (DeToledo73). Although some of this is due to the presence of multiple symptoms and the reluctance of some older people to admit to problems, poor history taking and high staff turnover were identified as contributing factors. Occasionally non epileptic attack disorder presents for the first time at this age (Fakhoury et al74). As in younger people, video telemetry can be helpful in confirming or refuting a diagnosis of epilepsy (Drury et al75). The presence of other disease and use of medication, as well as altered drug metabolism in the elderly, can restrict the choice of antiepileptic drug. Nonethe-less complete control is still possible in around 70% of people (Koyama et al76,Stephen and Brodie77, Rowan78). In people with truly intractable seizures surgery can still be considered in older people. Results are not as good as in a younger age group but 52% of a group of people over 50 years old became seizure free after temporal lobectomy at the Mayo Clinic (Sirven et al79). Epilepsy has a profound effect on the lives of older people. Loss of confidence, injury and loss of independence in addition to the seizures, medication and coexisting conditions can pose major problems. A speedy, coordinated and informed response from health care providers is required to achieve the best possible quality of life (Stephen and Brodie77). 13 In view of the difficulties of diagnosis, the confounding effect of other diagnoses, the sensitivity to treatment and the major impact on independence, a clearly defined service for older people with epilepsy would be a great advantage. It is surprising then to find that older people are less likely than younger people to be referred to specialist clinics (Sander et al80). Complex epilepsy Learning Disability The Government White Paper, “Valuing People: A New Strategy for Learning Disability for the 21st Century”, recognises that people with learning disabilities have additional and often complex health care needs81. Up to 30% have seizures (Carvill et al82), the severity of the epilepsy being correlated directly with the severity of the learning disability. Diagnosis can be extremely difficult especially in the presence of stereotyped behaviours or involuntary movements (Paul83). Sometimes the seizures are easily controlled and are only a small part of the over-all picture but in 75% the seizures are difficult to control with frequent admissions to hospital, episodes of status epilepticus and injuries. Generalised tonic clonic seizures are most responsive to treatment whereas the prognosis for partial seizures is poor (Branford et al84). There is considerable interaction between the management of the epilepsy and the management of behavioural and cognitive problems related to the underlying condition (Espie et al85). It is clear that frequent seizure activity further reduces learning and is associated with more difficult behaviours, however there is a shortage of useful outcome and quality of life measures for this group of people (Espie et al86). Depending on the severity of the learning disability, care for epilepsy should be an integral part of the over-all care for people with a learning disability. Close cooperation with specialist epilepsy services is thought to improve management (Jenkins and Brown87). Overall it is felt that treatment and control of seizures has improved over the years (Pellock and Morton 88) but this is not borne out in all studies (Branford et al89). Currently services for people with learning disabilities in the UK are uneven and arrangements are often somewhat ad hoc and less satisfactory than desired. Lifelong informal carers report 40% more limiting health disorders than the general population with depression being 4 times as common among female carers. Neither the needs of the patients nor the needs of the carers are being met adequately (McGrother et al90). Intractable epilepsy Seizures prove difficult or impossible to control in a small number of people. Most often this occurs in people whose epilepsy is symptomatic of a known cerebral disorder (Devinsky15, Branford et al84). A review of the diagnosis and medication will show that about 25% do not have epilepsy and that almost 50% of those with refractory epilepsy have not had an adequate trial of 14 appropriate medication. The most common example is a failure to identify idiopathic generalised epilepsies and use Valproate for treatment. Careful adjustment of medication can reduce seizure frequency in this group (Smith et al8). Improving the extent to which patients adhere to treatment regimes also leads to an improvement in seizure control (Schmidt91). This still leaves a small number of people who will continue to have seizures in spite of taking adequate doses of appropriate medication. New medications and improved understanding of the causes and treatment of epilepsy might provide some help for these individuals in the future. Surgical treatment If the diagnosis is accurate and temporal lobe seizures remain intractable after adjustment of medication, detailed investigation might suggest that surgical treatment could be beneficial. Since the pioneering work of Wilder Penfield in Montreal over 70 years ago the investigative technology and the decision making process have changed considerably. In a relatively early series 41% of patients became free from seizures after surgery, with 58% managing 3 years without a seizure (Wass et al92). More recent series using better imaging and neurophysiological techniques have reported even better figures with over 70% seizure free (Smith et al93). Now investigation requires detailed clinical, neuroradiological, neurophysiological and neuropsychological assessment to localise the source of the seizures (MacKenzie et al94, Salanova et al95). With detailed MR imaging demonstrating a focal lesion and corroborating evidence from EEG and neuropsychology, including the Wada test, invasive electrode recordings can usually be avoided (Diehl and Luders96). If these tests give clear evidence of bilateral lesions it is unlikely that surgery would be helpful. Wieshmann et al 97 have shown that the more straightforward the investigative path to surgery the better the outcome. A successful outcome is more likely in patients who have a history of febrile seizures, a clearly focal origin for their seizures and in whom the resected specimen shows pathology (Salanova et al98). Initially outcome was measured largely in terms of freedom from seizures but there are now several measures of quality of life that have been used to show equally important gains from surgical treatment (Kellett et al99). Some people with intractable epilepsy, who were not thought likely to benefit from resective surgery, have had vagal nerve stimulators implanted. There is some evidence of a modest response but further assessment is required before the indications for its use are clear (Binnie100). Early reports of the use of stereotactic radiosurgery in the treatment of temporal lobe epilepsy have been promising with 80% seizure free at 2 years after treatment (Regis et al101). Longer term follow up will be important in determining the place of this technique in the treatment of epilepsy. Thus in carefully selected people who have a localised origin of their seizures in the temporal lobe and in whom language and memory function can be preserved, the results of surgery are good. For other people with intractable 15 epilepsy investigation may still suggest that surgery such as cortical resection or hemispherectomy may be of benefit. There is, however, less agreement on the place of surgery for intractable seizures without clearly localised pathology. Non-epileptic attack disorder In a small number of people thought to have intractable seizures further observation or investigation shows them to have a non-epileptic attack disorder or pseudoseizures. This condition results from a psychiatric or behavioural disorder: it is not a form of epilepsy and has no structural or pathophysiological basis (Moore and Baker102). It most frequently affects young women and is sometimes related to sexual abuse or assault in earlier life (Lancman et al103). Recognition can be extremely difficult especially if both epilepsy and pseudoseizures occur in the same person. Video EEG recording during and between episodes can be helpful in identifying seizures, pseudoseizures and the coincidence of both in the same individual (Devinsky et al104). Some pseudoseizures are manifestations of psychiatric disorders which require identification and treatment. Thus Neuropsychiatrists, neuropsychologists and neurologists are involved in the management of this group of people but the outcome is rather mixed. If there is no coexisting epilepsy or psychopathology, and the onset was only a few months before diagnosis, up to 70% will cease to have attacks (Lempert and Schmidt 105, Selwa et al106). The outcome appears to be better in children than adults (Irwin et al107). Over-all the success rate is much lower than 70% and more than half have persisting, long-term morbidity related to psychosocial problems (Lempert and Schmidt105). Impact of epilepsy A diagnosis of epilepsy has a profound effect on the life of an individual and on family members. The law places restrictions on driving, some occupations are seen to be unacceptably hazardous and many employers are wary of offering jobs to people who may have seizures in the work-place. People with more frequent seizures sometimes have difficulty with education or training and some are excluded from mainstream education (Fisher et al 108). These social burdens are made worse by a persisting perception of stigma or prejudice in society that is only partly related to the severity of the epilepsy (Baker et al109, Aziz et al110). Together with the unpredictability of seizures, the need to continue medication for a long period, and the associated side effects, epilepsy can constitute a significant disability and can give rise to considerable handicap (Baker et al111, Fisher et al108). It is thus crucial to make sure that the diagnosis is correct and that all possible measures to reduce the frequency of seizures, and the associated disability and handicap, are incorporated into services designed for people with epilepsy. 16 Information Living with epilepsy is clearly not easy. With adequate information and understanding, people with epilepsy can have more control over their lives making decisions about employment, training, personal relationships, leisure and medication. There is a great deal of information produced by the epilepsy societies, pharmaceutical companies and internet providers. Our own survey (Appendix 3) identified more than 70 different publications used by neurologists and nurses in their clinics in the North West. In spite of this patients and families identify lack of information as one of their major concerns (Buck et al112). The opportunity to ask for advice from somebody other than the specialist is welcomed and while GPs are seen as helpful they are not seen as particularly knowledgeable (Chappell et al113). Specialist nurses are seen to be good communicators, appreciated by patients (Mills et al114), although evidence that understanding is greater among those who have seen the specialist nurse is restricted to those who have least knowledge of epilepsy (Ridsdale115). Patterns of care Traditionally much of the care for people with epilepsy has been provided by general practitioners with variable involvement of Neurologists, General Physicians, Psychiatrists or Geriatricians. Care in general practice has not always been ideal with most care being reactive (Chappell et al 113). Lack of confidence in their knowledge, lack of time and unfamiliarity with new drugs have been identified as barriers to providing epilepsy care in general practice (Thapar et al116). With major changes in available treatment and the introduction of Specialist Nurses there have been calls for better integration of the service and wider use of nurses (Mills et al117). Epilepsy clinics have developed where local clinicians have had a particular interest. Formal shared care arrangements have developed in some areas and the use of specialist epilepsy nurses has become widespread. There is, as yet, no evidence on the longer term impact of these changes on the quality of life of people with epilepsy. The organisation of care must focus on meeting the needs and wishes of the patient. Although levels of satisfaction with hospital and primary care are high, patients feel that they have to wait too long for a specialist appointment, that they do not have enough time with the doctor, that they would like more information and that they would like to talk to someone other than a doctor. They would like to see the same person on each visit to the clinic. Most would like their care shared between hospital and primary care (Jain et al118,Chapell and Smithson119). Summary Every GP will have about 10 people with epilepsy on the books. For most of these people the epilepsy will either be inactive or well controlled. An 17 accurate diagnosis is crucial but difficult and continued supervision is aimed at reducing the impact of the diagnosis and the frequency of seizures on the quality of life. After initial diagnosis continued supervision should be coordinated with good communication between all those involved. The needs of women, older people, adolescents and people with learning disabilities should be separately defined and the services designed to meet these needs. Planning care must be about ensuring that patients and families understand the situation and know where to get help at any time, whilst also ensuring that professionals providing care at every level have the skills and support they need to provide care to agreed standards. 18 Section 3 The Management of People with Epilepsy: Clinical Guidelines Introduction People with epilepsy, and the voluntary agencies representing their views, agree on the most important aspects of an epilepsy service. They are: Rapid referral and early appointment Referral to an expert Seeing the same doctor at each clinic visit Adequate, relevant information Continued supervision In the section that follows these points should be kept to the fore. A suspicion that a person might have epilepsy is a common reason for referral to a hospital clinic. Most of these people do not turn out to have epilepsy. It is important that the correct diagnosis is established quickly both because the misdiagnosis of epilepsy has unnecessary negative psychosocial and socioeconomic consequences and because other conditions require different investigation and treatment. If the diagnosis of epilepsy is confirmed the patient should have a complete diagnosis, appropriate counselling and continued care. This will involve multidisciplinary and multisector cooperation over a prolonged period. The team should include GPs, nurses, neurologists, neurophysiologists, neurosurgeons, neuroradiologists, neuropsychologists, neuropsychiatrists, social workers and voluntary agency workers. Currently these facilities are not widely available and, therefore, many patients are seen in busy general neurology or general medical clinics. Whatever the setting, however, we believe that concise guidelines will facilitate optimal care of these patients. The pathways that follow are generic. Additional guidance on meeting the needs of adolescents, women, older people, adults with learning disability and people with psychological or psychiatric problems follows in later sections. Diagnosis (Pathway 1) Most often individuals present to the GP or an Accident and Emergency Department with blackouts, funny turns, syncope or seizures. Only 20 to 25% of those referred on to hospital clinics will ultimately be shown to have epilepsy while approximately 60% have syncope (faints). Clinicians responsible for assessing these patients in A&E departments and primary 19 care should be familiar with this information and should know about the motor and ocular phenomena which commonly occur during syncope so that they avoid a misdiagnosis of epilepsy. When syncope occurs outwith expected circumstances a cardiology opinion should be sought. The identification of provoked seizures (alcohol/drug withdrawal, drug induced, concussive) is important because of the implications for future investigation and treatment. Avoidance of provocative factors, rather than drug treatment, is likely to be effective and the individual might retain the eligibility to drive. Clinicians seeing people with blackouts, funny turns and faints in primary care and A&E Departments should know the differential diagnosis of these symptoms. In particular they should recognise the significance of symptomatic seizures and be aware of the motor phenomena which frequently occur during syncope. If a diagnosis other than epilepsy is made, appropriate management should be determined by the relevant clinician. A complete diagnosis of a person with epilepsy requires differentiation of seizures from other causes of loss of consciousness/altered behaviour, distinction between acute symptomatic (provoked) seizures and a spontaneous tendency to recurrent seizures (epilepsy), classification of seizures and of epilepsy, and identification of the cause. This process demands expertise in history-taking, a knowledge of the differential diagnosis and of the utility and limitations of specialist investigations (EEG and cerebral imaging). The Epilepsy Service is the most likely place to find this expertise. Where an underlying neurological condition such as vascular disease or dementia is thought to be responsible for the seizures, referral to a neurology clinic could be appropriate in the first instance. Where epilepsy is suspected or established by the clinician, referral into the specialist Epilepsy Service is indicated. Where epilepsy is symptomatic referral to a neurology clinic or medical clinic could be appropriate. Because of the implications of a diagnosis of epilepsy and the possibility of further events people suspected of having epilepsy should be seen in the Epilepsy Service within 4 weeks of the first presentation. If there are other symptoms or signs to suggest that the seizure might be secondary to underlying cerebral pathology a more urgent referral is indicated. The Epilepsy Service should be designed to facilitate this. People thought to have epilepsy should have an appointment in the Epilepsy Service within 4 weeks from presentation. Those people whose signs or symptoms suggest an underlying cause should be referred urgently. 20 The Epilepsy Service should appointments when necessary. be designed to facilitate urgent Pathway 1: Diagnosis The initial clinical diagnosis falls into 3 categories: 1. Not epilepsy 2. Uncertain 3. Epilepsy 1. Not epilepsy The commonest condition is syncope. It should be emphasised that this is often complicated by brief motor or ocular phenomena which can lead the unwary to an erroneous diagnosis of epilepsy. The patient should be counselled about the physiological basis of symptoms, avoidance of provocative factors and postural manoeuvres that can prevent loss of consciousness when warning symptoms occur. They should be counselled regarding eligibility to drive and further care should be provided in primary care. If there is any possibility of a pathological basis for the syncope referral to a cardiologist is indicated. Patients who do not have epilepsy should have a clear explanation of the nature and implications of their symptoms. Any necessary further 21 referral should be arranged. The General Practitioner should receive the same information within 10 days. 2. Uncertain The diagnosis is sometimes uncertain. This is a difficult situation for the patient and family and requires sensitive management. Follow-up in the specialist Epilepsy Service, with any relevant investigation, gives the greatest opportunity for reaching a diagnosis. When a diagnosis has been made the appropriate course of action is followed as detailed in paragraph 1or 3. The patient, the GP and others involved should be kept fully informed at every stage. When the diagnosis is not clear, people who could have epilepsy should be followed up, and investigated as necessary, in the Epilepsy Service. Good, prompt communication with the patient and the GP is essential. 3. Epilepsy Following a confident diagnosis of epilepsy patients should be given detailed information about epilepsy and its implications for driving, employment/ education, contraception/pregnancy, and other issues relevant to them. Any investigations needed to refine the diagnosis should be requested and treatment started. Full information should be sent to the GP within 10 days. At the time of diagnosis people with epilepsy should receive a detailed explanation of the diagnosis and its implications, should have written information provided and should be introduced to the specialist epilepsy nurse. The GP should be informed within 10 days. The detailed classification of epilepsy is complex (see Tables 1 and 2, pages 7 and 8) but initially people with epilepsy may be divided on clinical and EEG grounds into 3 groups: (a) Generalised-onset epilepsies. The idiopathic generalised epilepsies commence in childhood/adolescence/early adulthood. They are genetically determined conditions associated with generalised spike and wave on the EEG. The brain is structurally normal and cerebral imaging is not required. People thought to have idiopathic generalised epilepsy should have an EEG but do not need cerebral imaging (CT/MR). (b) Unclassified epilepsy in patients under 30 years of age. These patients could have either idiopathic generalised or partial onset epilepsy consequent upon structural pathology. Both EEG and imaging are needed. 22 People with unclassified epilepsy beginning before the age of 30 should have a CT/MR scan and an EEG. (c) Partial onset epilepsy, or unclassified epilepsy in patients over 30 years of age. All these patients will have a partial onset epilepsy where structural pathology should be excluded by CT (or MR where it is available) in the first instance. An EEG is not required at this stage. People with partial seizures or with unclassified seizures starting after the age of 30 should have a CT/MR scan but do not need an EEG at this stage. The diagnosis of epilepsy in elderly people can be more difficult because of the broader differential diagnosis and the possible presence of more than one pathology. Elderly patients with unequivocal seizures are usually managed in a similar way to younger patients. If the diagnosis is uncertain, if the seizures prove to be refractory or medication is not tolerated they should be seen by a geriatrician in a “Medicine for the Elderly” clinic which is part of the epilepsy service. Initially older people with epilepsy, or suspected to have epilepsy, should be referred to a “Medicine for the Elderly” service where there is a physician with appropriate expertise. There should be close cooperation between this service and the Epilepsy Service. Treatment The aim of treatment is to reduce both the frequency of seizures and any disability or handicap resulting from the diagnosis of epilepsy. Individuals are only able to decide which course of action to take when they understand issues such as the effect on driving, employment, reproductive health and side effects of treatment. Information, counselling and support are all important parts of the management alongside the use of appropriate anticonvulsant or antiepileptic drugs. All people with epilepsy should be given full information about their epilepsy and their medication so that they can make informed decisions about their treatment and life-style. Starting antiepileptic drug therapy. A single drug should be introduced at a low dose and cautiously escalated to a standard maintenance dose. If seizures continue the dose should be increased to the maximum that can be tolerated before switching to an alternative single drug (monotherapy). Only when seizures continue despite adequate trials of monotherapy with 2 appropriate drugs in therapeutic doses, should combination therapy be started. Combination therapy should only be instituted within the framework of an Epilepsy Service. 23 When treatment is necessary monotherapy with an appropriate antiepileptic drug should be introduced. Combination therapy should only be used, after failure of monotherapy, within the agreed framework of the Epilepsy Service. PARTIAL SEIZURES GENERALISED OR UNCLASSIFIED SEIZURES MALE + FEMALE (NON-CHILD-BEARING) FEMALE (CHILD-BEARING) MALE + FEMALE (NON-CHILD-BEARING) FEMALE (CHILD-BEARING) SODIUM VALPROATE SODIUM VALPROATE OR LAMOTRIGINE CARBAMAZEPINE OR LAMOTRIGINE CARBAMAZEPINE OR LAMOTRIGINE Footnotes 1. Optimal treatment is not known. The chart reflects current practice. The SANAD study should determine optimal treatment. 2. Women of childbearing age who have generalised or unclassified seizures should be offered the choice of treatment after informed discussion. Further information to allow clearer advice may be available from studies currently in progress. Pathway 2: First Line Treatment of Newly Diagnosed Epilepsy Pathway 2 is based on the evidence reviewed earlier. More information is needed to determine the most appropriate anti-epileptic drug in each situation and to assess the place of the newer drugs. All patients with newly diagnosed epilepsy should have the situation explained to them and receive an invitation to take part in any relevant clinical trials (eg the “standard and new anti-epileptic drug study” (SANAD) which, hopefully, will determine optimal therapy for the future). Such invitations must be strictly within the conditions laid down by the ethics committee for that trial and should only be discussed in the context of full disclosure of known risks and benefits. Informed consent must be obtained Clinical trials are needed to determine best treatment. People requiring treatment should be invited to join such trials. Invitations to participate in trials should be strictly within the conditions laid down by the ethics committee and should only be discussed in the context of full disclosure of all known risks and benefits. Fully informed consent must be obtained. 24 Management and follow-up (Pathway 3A and 3B) People with epilepsy, or who might have epilepsy, should have continuing access to appropriate care and support. The nature and extent of this will vary according to the need for diagnosis, supervision of medication and control of seizures. People with epilepsy, or who might have epilepsy, should have continuing access to appropriate care and support. 1. Diagnosis uncertain (Pathway 3A) UNCERTAIN 4/12: CONSULTANT DIAGNOSIS NOT EPILEPSY ADVICE + DISCHARGE TO GP UNCERTAIN EPILEPSY FOLLOW-UP UNTIL DIAGNOSIS Pathway 3B REFERRAL Pathway 3A: Management and Follow Up - Diagnosis Uncertain Patients in whom the diagnosis is uncertain should be reviewed by the consultant 4 months after the first visit. If it is established that they do not have epilepsy, management should continue as above (pathway 1). If the diagnosis remains uncertain consultant follow-up will continue, the interval between visits depending on the frequency of events. The guidelines below (pathway 3B) should be followed if epilepsy is diagnosed. People with an uncertain diagnosis should continue to be followed-up in the Epilepsy Service. 25 2. Epilepsy (Pathway 3B) When a diagnosis of epilepsy was established the individual should have been given detailed information and an appointment to see the specialist nurse. The nature and extent of continuing supervision will depend upon the individual circumstances. However, in the first few months after diagnosis all patients need access to advice and to continuing prescription of medication. In order to facilitate this the specialist nurse should receive copies of all correspondence. Assuming a knowledge of any other pre-existing diagnosis or medication, antiepileptic drug treatment should usually be initiated in the epilepsy clinic. The patient should be given sufficient medication to cover the titration period with clear written instructions covering dosage, side effects and sources of help and advice. The GP and specialist nurse should be given details immediately and at latest within 10 days. Further prescriptions should issued by the GP; the specialist nurse will be able to give information. Sometimes, particularly where full information is lacking, it is appropriate to ask the GP to issue the first prescription. In this situation communication with the GP should be immediate and should contain sufficient detail to enable the GP to titrate the dose correctly and to advise the patient on side effects. The specialist nurse will maintain patient records, supervise follow-up appointments, and should become the patients’ first point of contact in the Epilepsy Service, whoever issues the first prescription. Three months after the first appointment every person with epilepsy should see the specialist nurse to discuss their understanding of the diagnosis and so that further, individually relevant, information on driving, employment and life style can be given. This is particularly important for women of childbearing age. At this three month visit the patient will be assessed as being either in remission with no side-effects of treatment, or as continuing to have seizures and/or side-effects of treatment. People assessed as being in remission should be given clear instructions to contact the specialist nurse should they have a further seizure or develop side effects of medication. People with epilepsy should be seen by a specialist nurse 3 months after presentation to the Epilepsy Service. Further follow-up will be planned in the light of this appointment. a) Patients in remission with no side-effects of treatment Twelve months after the first visit each patient who has been free from seizures and has no side effects from treatment should have an appointment with the consultant/specialist. At this appointment the consultant/specialist will confirm that the person understands the diagnosis, the driving regulations and the need for long term treatment. Patients should be informed that if they achieve a 2 year remission they could be provided with an individualised estimate of the risk of relapse on drug withdrawal (MRC Antiepileptic Drug Withdrawal Study Group120) permitting informed decision-making. Continuing 26 supervision can then be undertaken in the primary care setting according to an agreed protocol which includes advice on reasons for re-referral; planning pregnancy, considering AED withdrawal and relapse of epilepsy. People whose epilepsy is in remission at 1 year should be seen by a consultant/specialist in the Epilepsy Service. After explanation arrangements should be made for annual review in the primary care setting. They should have clear instructions on what to do if they have a further seizure or side effects or need advice. TREATED EPILEPSY 3/12: NURSE + COUNSELLING IN REMISSION WITH NO SIDE EFFECTS NO REMISSION OR HAS SIDE EFFECTS CONSULTANT + NURSE DISCUSSION + PLAN DIAGNOSIS QUESTIONED NURSE SUPERVISION 12/12: CONSULTANT/ SPECIALIST 12/12: CONSULTANT REMAINS IN REMISSION Pathway 3A IN REMISSION WITH NO SIDE EFFECTS GP OR PRACTICE NURSE UNCHANGED COMBINED CONSULTANT + NURSE FOLLOW-UP 12/12: FOLLOW-UP IN PRIMARY CARE Pathway 4 Pathway 3B: Treated Epilepsy - Management and Follow Up In order to maintain shared care the GP or practice nurse should invite the patient for annual review and inform the specialist nurse of the outcome so that hospital records can be updated. In time this will be much easier when all sectors and disciplines have a single shared record and computerised data base. Good communication is essential to good care. People with epilepsy should receive written information giving details of agreed goals and plans. All those involved in care should have copies of this information and of all other communications within 10 days. Shared clinical records should be developed. b) Patients having side-effects of therapy or not in remission About half those taking antiepileptic drugs after the first visit will either continue to have seizures or have side effects from the treatment. These are 27 patients who report ongoing problems at the 3 month visit in Pathway 2B. At this stage the consultant and specialist nurse should discuss the patient and a treatment plan will be formulated for the following year. If the diagnosis is questioned the patient will join the beginning of Pathway 3A for further medical review. Otherwise, the patient should remain under nurse supervision until their appointment with the doctor at one year after the initial visit. At the one year review, if the patient has entered a remission and has no side effects of treatment then the ‘remission, no side-effects’ branch of Pathway 3B should be followed. Those continuing to have seizures or side effects of treatment should receive yearly medical assessments with additional nurse supervision as necessary. People continuing to have seizures or side effects of medication at 1 year should remain under annual review by the consultant with additional specialist nurse supervision as necessary. c) Refractory epilepsy (Pathway 4) Patients who have not responded to treatment or who have been referred with a label of drug-resistant epilepsy should have the diagnosis reviewed carefully by the consultant. This will identify the group of people who do not have epilepsy, identify opportunities to improve therapy, and identify people who might benefit from surgery. PATIENTS FROM PATHWAY 2 (EPILEPSY) OR PRESENTING DE NOVO WITH DRUG RESISTANT EPILEPSY DIFFICULT EPILEPSY REVIEW DIAGNOSIS NON-EPILEPTIC ATTACKS? EPILEPSY VIDEOTELEMETRY NEUROPSYCHIATRY NEUROPSYCHOLOGY NON-EPILEPTIC ATTACKS STOP ANTI-EPILEPTIC DRUGS INITIATE INTERVENTION NEUROPSYCHOLOGY NEUROPSYCHIATRY FOLLOW-UP REVIEW CLASSIFICATION EPILEPSY GENERALISED ONSET GENERALISED ONSET OPTIMISE THERAPY PARTIAL ONSET UNCERTAIN UNCERTAIN EEG MRI VIDEOTELEMETRY PARTIAL ONSET OPTIMISE THERAPY REVIEW IMAGING CONSIDER SURGERY Pathway 6 Pathway 4: Difficult epilepsy 28 Where the diagnosis is uncertain, investigation should include an up-to-date EEG and, if required, MRI and video-telemetry. The final diagnosis or classification may be modified according to the results. People who continue to have seizures in spite of medication should be reviewed thoroughly in the Epilepsy Service to confirm or refute the diagnosis. (i) Non-epileptic attacks (non-epileptic seizures, pseudo seizures) Recognising and establishing a diagnosis for non-epileptic attacks is extremely important. Such patients can usually be identified on clinical grounds. However, simultaneous video-EEG telemetry helps to confirm the diagnosis and can also identify patients with frontal lobe epilepsy and those with both epilepsy and pseudo seizures. When the diagnosis of epilepsy is removed the situation should be explained carefully to the patient, antiepileptic drugs should be withdrawn and appropriate intervention should be provided. Subsequent management of this heterogeneous group of patients requires the involvement of both neuropsychiatrist, for diagnosis of specific treatable underlying psychiatric disorders, and neuropsychologist for assessment/management of psychological factors predisposing to non-epileptic attacks. Patients with non epileptic attack disorder should be identified. After identification antiepileptic treatment should be withdrawn and help should be provided by a neuropsychologist and a neuropsychiatrist. It is important to try to reach a diagnosis for the cause of the attacks. (ii) Epilepsy Each patient with apparently drug-resistant epilepsy should be syndromically classified, using available evidence, as having generalised onset or partial onset epilepsy. The previous treatment history should be reviewed thoroughly to ensure that there have been adequate trials of appropriate drugs and that the medication has been taken regularly. Failure to recognise idiopathic generalised epilepsies and to treat with Valproate is the commonest example of suboptimal therapy. Ensuring that prescribed treatment is taken regularly is known to improve control. 29 In those with genuinely refractory conditions the following combinations of drugs are recommended: Generalised onset epilepsy: 2 of the following 3 drugs (Pathway 5A) Valproate, Topiramate Lamotrigine. Partial onset epilepsy: 2 drugs (Pathway 5B) Carbamazepine, Oxcarbazepine or Lamotrigine and one other. GENERALISED SEIZURES TONIC-CLONIC + MYOCLONIC SEIZURES ABSENCES EXISTING TREATMENT EXISTING TREATMENT SODIUM VALPROATE LAMOTRIGINE ADD 1. 2. 3. 4. LAMOTRIGINE TOPIRAMATE CLOBAZAM PHENOBARBITONE SODIUM VALPROATE ADD 1. 2. 3. 4. ADD SODIUM VALPROATE TOPIRAMATE CLOBAZAM PHENOBARBITONE 1. LAMOTRIGINE 2. ETHOSUXIMIDE LAMOTRIGINE ADD 1. SODIUM VALPROATE 2. ETHOSUXIMIDE Pathway 5A: Treatment of intractable epilepsy - Generalised seizures Whichever combinations are used drugs with different modes of actions are preferable because they could be synergistic and there is less likelihood that side-effects will be additive. People should only be considered to have intractable epilepsy after adequate trials of maximum tolerated doses of appropriate antiepileptic drugs singly and in combination. Patients with refractory partial onset seizures should have detailed MRI to maximise the possibility of detecting lesions. Some findings will have no 30 treatment implications but might explain the refractory nature of the condition, whereas other findings might indicate the presence of treatable lesions. PARTIAL SEIZURES EXISTING TREATMENT CARBAMAZEPINE, OXCARBAZEPINE, OR LAMOTRIGINE ADD IF FIRST DRUG CARBAMAZEPINE/ OXCARBAZEPINE IF FIRST DRUG LAMOTRIGINE ADD ADD 1. 2. 3. 4. 5. 6. TOPIRAMATE VALPROATE GABAPENTIN LEVETIRACETAM CLOBAZAM TIAGABINE 1. 2. 3. 4. 5. 6. TOPIRAMATE VALPROATE GABAPENTIN LEVETIRACETAM CLOBAZAM TIAGABINE Pathway 5B: Treatment of intractable epilepsy - Partial seizures d) Considering Surgery People with truly refractory temporal lobe epilepsy should be considered early for surgery. If possible the situation should be identified before adulthood so that the impact of epilepsy on education, employment, social and family relationships is minimised. Pathway 6 is an example of a pre-surgical investigation protocol that emphasises a non-invasive approach. Experience in Liverpool suggests that the more straightforward the pathway to surgery the better the outcome. Those with unilateral temporal lobe atrophy on MRI have an excellent chance of becoming free from seizures after surgery. Conversely the absence of atrophy or lesion on MRI indicates that there is little chance of a successful outcome from surgery. Patients should be counselled accordingly at an early stage of the investigation process. A decision to advise surgery can only be taken after a series of investigations and periods of observation. This process inevitably takes a considerable time but it should not be prolonged unnecessarily; from the beginning of the assessment process to surgery should not take more than 1 year unless at the request of the person with epilepsy. Some of the investigations are not 31 without risk: although the trend towards less invasive assessment techniques will reduce the risk it is unlikely to disappear altogether in the foreseeable future. People contemplating undergoing surgery should be fully aware of the risks of the procedures and of the length of time the assessment process will take. INTRACTABLE PARTIAL SEIZURES PROBABLY OF TEMPORAL LOBE ORIGIN ROUTINE INTERICTAL EEG QUANTITATIVE MRI BASELINE NEUROPSYCHOLOGY TEMPORAL LESION OR ATROPHY YES NO SEIZURE RECORDINGS +/INTRACRANIAL ELECTRODES* SEIZURES LATERALISED TO ONE TEMPORAL LOBE WADA TEST YES SATISFACTORY# YES TAILORED INVESTIGATION NO SURGERY NOT OFFERED NO SURGERY Pathway 4 Pathway 6: Pre-surgical evaluation (suspected temporal lobe seizure origin) * Used infrequently since the advent of more detailed MR studies # Satisfactory Wada test means acceptable contralateral memory and, ideally, impaired ipsilateral memory. 32 Assessment requires the collaboration of the neurologist, neurophysiologist, neuroradiologist, neuropsychologist, neuropsychiatrist and neurosurgeon. The surgery should only be undertaken by a surgeon experienced in the surgical treatment of epilepsy. There is no good guide to the volume of activity required to ensure that competence is maintained. It seems reasonable to suggest that experience should be maximised by concentrating the assessment in approved Centres and the surgery in the hands of 2-3 adult and 2 paediatric surgeons in the North West Region. All neurosurgeons taking part in the epilepsy surgery programme must agree to take part in the regular audit of their results. Results should be reviewed regularly at multidisciplinary meetings involving all Epilepsy Centres in the North West. There is much less agreement on the management of partial seizures originating in other parts of the brain, surgical treatment is sometimes possible but is less likely to produce sustainable benefits for the patient. Decisions should be made by the individual with epilepsy after all the information has been discussed with the specialised team referred to above, and with other carers. In the absence of proof of the efficacy of some procedures, and the waiting time for surgery of proven benefit for many people, it is not likely that the Epilepsy Service will support unproven procedures outside a properly organised clinical trial. Continuing assessment, advice and support must be provided following surgery. The follow up should ensure best possible treatment for any persisting seizures, identify opportunities to improve the quality of life, address any continuing concerns and provide a basis for the assessment of the efficacy and outcome of the surgical procedure. This process requires multidisciplinary involvement including neuropsychology and, sometimes, neurorehabilitation. Assessment for surgery is a very specialised process: it should only be undertaken in approved Epilepsy Centres with a dedicated, multidisciplinary team that jointly undertakes regular reviews of outcome. The surgery should be undertaken by a neurosurgeon with particular expertise in surgery for epilepsy. All participating neurosurgeons must agree to take part in regular outcome review. Following surgery multidisciplinary follow up, with assessment and support, must be provided. e) Status epilepticus Status epilepticus is a medical emergency. The continuation of seizures leads to neuronal damage that can give rise to persisting deficit or even death. Prolonged periods of unconsciousness even in an ITU setting are associated with considerable risks, and the underlying cause of the seizures might also cause increasing damage if not treated promptly. Thus the management of convulsive status epilepticus has 3 components: 33 1) Maintenance of ventilation, circulation and metabolic support 2) Urgent cessation of seizure activity 3) Identification and treatment of the cause Recognition of status epilepticus should be followed by immediate attention to airway, breathing and circulation. Any impairment of these vital functions should be corrected. The practical arrangements for the treatment of the seizures should continue simultaneously. The abolition of seizure activity requires intravenous administration of antiepileptic drugs. Currently Lorazepam should be given in a dose of 0.1mg/kg and repeated if seizures have not stopped within 15 minutes. Phenytoin (20mg/kg) should be used alongside the initial dose partly because of its efficacy in ending seizure activity when combined with a benzodiazepine but also as a means of easing the transition to oral antiepileptic medication after the episode of status epilepticus is over. If these measures are not adequate barbiturate anaesthesia should be used; this must be undertaken in an ITU with close supervision and careful monitoring of seizure activity on the EEG. Not infrequently the dose of antiepileptic drugs required to end seizure activity is sufficient to suppress respiration. Prolonged seizure activity, especially when combined with poor respiratory function can give rise to a profound systemic metabolic disturbance. In order to prevent, recognise and treat these problems patients should be admitted to an ITU immediately if recovery does not occur within 15 minutes. In practical terms this means transfer from the A&E department or ward to the ITU immediately status epilepticus has been diagnosed. Arrangement for transfer should not, however, interfere with treatment or monitoring in the meantime. A neurologist with expertise in epilepsy should always be consulted and should be consulted as early as possible. When there is an obvious brain injury as the cause of the seizures, appropriate investigations and treatment should be undertaken urgently. Such patients might be more sensitive to the sedative effects of benzodiazepines: it is recommended that particular attention is paid to ventilation and circulation and that phenytoin should be used as the first treatment in this group if facilities for assisted ventilation and circulatory support are not immediately available. If seizures are not controlled within 15 minutes there should be rapid progression to barbiturate anaesthesia and assisted ventilation with urgent transfer to a Neuroscience Centre. Status epilepticus is a medical emergency . Immediate treatment is required and should follow the agreed protocol. ITU facilities are essential and neurological advice must be sought urgently. 34 Seizures continuing > 15-30 minutes Immediate i/v bolus Lorazepam (0.1mg/kg) AND Phenytoin (20mg/kg) Seizures stopped Seizures continue 10-15 minutes Is it true status or pseudostatus ? Status ADMIT to ITU Give further bolus i/v Lorazepam. Maintain ventilation 10-15 minutes Seizures stopped Pseudostatus STOP ALL sedative drugs Seizures continue Establish adequate maintenance regime of Antiepileptic drugs. Treat aetiology of status Barbiturate (or other) anaesthesia until seizures cease on EEG. Maintain ventilation Pathway 7: Management of Status Epilepticus Some people are prone to recurrent episodes of status epilepticus or clusters of tonic-clonic (convulsive) seizures. In these circumstances family or carers can be taught to administer rectal Diazepam or other rescue medication. Such patients should have written guidelines supervised by an appropriately 35 qualified nurse and doctor. Social Services departments and Health Authorities will need to agree policy guidelines between themselves and the various private, voluntary and statutory providers of care, for the use of rectal Diazepam or other rescue medication. If control is not achieved within 15 minutes an ambulance should be called and one further dose of diazepam administered. Because Diazepam depresses respiration it is dangerous to give further doses outside hospital. 36 Section 4 The Adolescent with Epilepsy Services for children with epilepsy Epilepsy frequently begins in childhood and continues into adult life. It is not within the remit of this framework to describe the services available for children but it is clear that the needs and expectations of adults who developed epilepsy in childhood will be profoundly influenced by their earlier management. An epilepsy service should be available to all children with epilepsy and should deliver high quality care to approved standards in an acceptable manner. Adolescence The change from child to adult takes place over a prolonged but very variable period of time. During this transition many issues have to be addressed. Contraception, pregnancy, foetal risk, inheritance, parenting, driving, alcohol and recreational drugs, education and employment should all be discussed early enough to allow decisions to be made. The control of seizures continues to be a major goal. At a stage in life when continuing education, employment and personal relationships are posing new questions it is important to ensure that adequate, sensitive support, advice and counselling are available. Much of this support comes from the family but the interaction between members of the family is itself influenced by the epilepsy and can in some circumstances further delay psychosocial maturation (Alwash et al121). Parental stress is increased by the frequency of seizures, the number of medications and the number of visits to hospital (Camfield et al122). Fortunately the majority of young people with epilepsy seem to cope well and find the family supportive. In the same study, however, many were critical of the medical profession and of support services for people with epilepsy (Wilde and Haslam123). Chronic illness can affect an adolescent’s physical, cognitive and psychosocial development and adolescent development can influence the natural course and medical management of a chronic disease (Kaplan and Friedman 124). These considerations are particularly important in the context of epilepsy. Victimisation of children with epilepsy is common at secondary school (Wilde and Haslam123), self esteem is low and behavioural disturbance is more common (Hoare and Mann125). Evidence that formal psychiatric disorders are more common is conflicting (Kokkonen et al126, Alwash et al121). 37 The physical, psychosocial and cognitive development of the adolescent with epilepsy should be assessed and any necessary help provided. The needs of the whole family should be addressed. Management of epilepsy The frequency of seizures is one of the determinants of stress within the family and of psychosocial and developmental problems for the young person with epilepsy. Compliance with treatment regimes, including life style as well as medication influence the frequency of seizures (Otero and Hodes127). Kyngas128, in a large study in Finland showed that only 22% of adolescents with epilepsy complied fully with suggested health regimens and 34% reported poor compliance. Compliance with medication was highest and life style poorest. Good motivation, support from parents, physicians and nurses and a positive attitude were among the factors associated with higher compliance. For some adolescents with epilepsy there is evidence that formal structured counselling or cognitive behavioural therapy can reduce seizure frequency. Seizure control is an important part of the management of the adolescent with epilepsy. Counselling and cognitive behavioural therapy should be available. Transition “Transition from pediatric to adult health care is fraught with difficulties. On the one hand, the adult system is not properly prepared to receive patients who are survivors of the so-called childhood disorders. On the other hand, patients and families have difficulty leaving the protective environment created by pediatric caregivers, who in turn may have mixed feelings about letting patients go.” (Schidlow and Fiel129). The needs of adolescents are different from those of children and from those of adults. Abrupt transfer of people with epilepsy from the paediatric service to the adult service is unlikely to address those needs. A multidisciplinary transitional service which addresses the medical and psychosocial issues should be available to all children whose epilepsy continues into adult life. This service could be provided jointly by the paediatric and adult epilepsy services with a gradual change in emphasis. Alternatively a separate transitional service could be provided. There is no good information to support one arrangement rather than the other. If transitional clinics are like other clinics, the information given and the attitudes of staff are likely to be among the important factors in determining the success of such a clinic(Williams et al130). These factors should be monitored. A multidisciplinary transitional service, which addresses medical and psychosocial issues, should be available to all adolescents with epilepsy. 38 Section 5 Women with Epilepsy The services provided for women with epilepsy must reflect their particular needs. There is a general consensus on the issues to be addressed and a recognition that unless they are clearly identified in each locality they will not be met. There is, however, little or no evidence to suggest that any one way of designing the service meets the needs better than any other. The services provided for women with epilepsy must reflect their particular needs. Accuracy of diagnosis The diagnosis of epilepsy can be difficult. In particular it can be difficult to decide whether clinical attacks are due to epilepsy or non-epileptic attack disorder. Most people who experience non-epileptic attacks are women. The side effects of antiepileptic medication pose a greater threat to women especially during the years of reproductive life. An accurate diagnosis is thus extremely important in spite of the undoubted difficulties. It is important to have an accurate diagnosis in women of child-bearing age. Selection of medication As in all people with epilepsy the choice of antiepileptic medication is made on the basis of the type of epilepsy and the incidence of side effects. The aim of treatment should be to reduce the frequency and impact of seizures in a way that is acceptable to the individual. Within these general aims the choice of medication will be made by the woman. The choice will be influenced by consideration of contraception, preconception planning, pregnancy and the need for other medications. Treatment with antiepileptic drugs must take account of the need for contraception, planning of conception, pregnancy and infant care. Contraception Enzyme inducing antiepileptic drugs reduce the effectiveness of hormonal (oral and depot) contraceptives by increasing drug metabolism and lowering circulating concentration of the hormone. All women of child-bearing age, or 39 who are approaching this age, should be given information on the impact of antiepileptic medication on contraception. Higher dose pills containing at least 50 microgrammes of oestrogen or alternatively non-hormonal methods of contraception should be recommended. Although this issue should be raised by the doctor at the time of diagnosis, women with epilepsy find it easier to discuss the issues with an epilepsy nurse and an appropriate appointment should be made. Pre-conception counselling As many, if not all, anti-epileptic drugs can cause foetal damage and much of that damage probably occurs in the first weeks of pregnancy it is important to have taken all possible action to minimise the risks before pregnancy begins. Women should understand that the control of epilepsy may change during pregnancy and be advised about the risks to the foetus both of seizures and of antiepileptic drugs. In the time prior to conception seizure control should be achieved with the lowest dose of the smallest number of drugs possible. Wherever possible monotherapy should be used. Women should understand that the control of epilepsy may change during pregnancy and be advised about the risks to the foetus both of seizures and of antiepileptic drugs. Some of the teratogenic effects of antiepileptic drugs can be reduced by taking Folate. The United States Public Health Service recommends that, because not all pregnancy is planned, all women of child bearing potential should consume at least 0.4mg of folate each day to reduce the risk of neural tube defects (such as spina bifida) in the foetus. Women at high risk are recommended to take 4-5mg daily. It seems reasonable to suggest that at pre-conception counselling women with epilepsy should be advised to take 5 mg of Folate daily. At pre-conception counselling women should be advised to take 5 mg of Folate daily. Pregnancy With good pre-conception counselling most of the issues will have been identified and appropriate action will have been taken before pregnancy begins. There should be close co-operation between the Epilepsy Service and the obstetric service throughout pregnancy. Screening for foetal abnormalities by means of ultrasound scans and alpha-foetoprotein measurement in the first trimester can identify 95% of neural tube defects and heart anomalies. In the final month of pregnancy women with epilepsy should receive Vitamin K1 ,10mg daily, to reduce the risk of a bleeding disorder in the new-born baby. 40 Although small concentrations of some antiepileptic drugs are secreted in breast milk there is no general contra-indication to breast feeding. If the baby becomes irritable, drowsy or feeds poorly the situation should be reviewed. There should be close co-operation between the Epilepsy Service and the obstetric service throughout pregnancy. In the final month of pregnancy women with epilepsy should receive Vitamin K1 , 10mg daily, to reduce the risk of a bleeding disorder in the new-born baby. There is no general contra-indication to breast feeding. If the baby becomes irritable, drowsy or feeds poorly the situation should be reviewed. To reduce the risk of maternal mortality women should be aware of the risks associated with seizures during pregnancy and the importance of remaining on antiepileptic medication. Relatives should be instructed in what to do in the event of a seizure including the use of the recovery position once convulsive movements have ceased. Doctors should be aware of the increased risk of maternal mortality and should make adequate arrangements for the continued supervision throughout pregnancy and the first year thereafter. Each pregnant woman with epilepsy should be seen early in pregnancy and given information and advice on the risks of continuing seizures and the role of antiepileptic medication. Relatives should be instructed on what to do during a seizure and on the use of the recovery position. Hormonal changes and seizures Many women with epilepsy note that their seizures are worse around the time of menstruation. There is no consistent definition used to quantify this phenomenon and there is currently no therapeutic intervention of proven benefit. There are few studies of the effect of the menopause or hormone replacement therapy on the incidence or control of epilepsy and no consistent evidence of an adverse effect of either. Epilepsy is not a contraindication to the use of hormone replacement therapy. Any changes in seizure control should be treated as in other situations. 41 Section 6 Epilepsy in Older People Who are “the elderly”? Old age does not come on suddenly thus the definition of old age is somewhat arbitrary. Although the literature uses the term variably, sometimes referring to people over 60, for logistical and possibly biological reasons, it is reasonable to consider individuals over the age of 70 as requiring their own specialist service for epilepsy. This service should be closely associated with, or part of, the Epilepsy Service. The concurrence of epilepsy with other illnesses, particularly chronic disabling diseases that have an impact on mobility, cognitive function and continence increases sharply after age 70. This should not pre-empt neurologists seeing patients over the age of 70 or geriatricians seeing patients younger than this age. In particular patients under the age of 70 who have these additional problems could benefit from using the specialist epilepsy service for elderly people. Older people should have their own specialist service for epilepsy. Diagnosis Both the incidence and the prevalence of epilepsy increase in older age. The incidence of falls, dizzy spells and blackouts not due to epilepsy is also at its highest in this age group. The differential diagnosis is wide. Older people should have access to the broad range of clinical skills and diagnostic facilities required to make a positive diagnosis and determine best treatment. People who developed epilepsy in early life might continue to have partial or generalised seizures, or to take medication, through into old age. For this group of people there is no need for re-investigation but medication will need to be reviewed in the light of the development of any other conditions or the need for other medication. Regular review is even more important than in younger people. Seizures that begin in later life are partial and might or might not become secondarily generalised. They frequently have their origin in damaged cerebral tissue most often the result of cerebro-vascular disease but sometimes due to tumours or other cerebral pathology. It is in this group that diagnosis can be particularly complex with a wide differential diagnosis that includes not only neurological conditions. Physicians specialising in care for the elderly should work closely with neurologists with special expertise in managing epilepsy in older people, to ensure that all aspects of the service can be provided. 42 Implications As in the case of younger patients, older patients require: a) A clear diagnosis i) a positive diagnosis of epilepsy ruling out other causes of impairment or loss of consciousness ii) Diagnosis of underlying causes or precipitating factors iii) Diagnosis of concurrent illnesses b) good general management including information, counselling and education c) appropriate antiepileptic treatment with adequate monitoring of effects and side effects. To deliver these requirements the services provided should be similar to those provided for younger patients, but with some important differences. Pregnancy, contraception and lactation etc are no longer relevant. Other aspects of care assume lesser importance (occupational rehabilitation). However others assume considerably more importance and should be addressed explicitly by the Epilepsy Service: A) Differentiation of epilepsy from syncope: This is often difficult in older people and will frequently require access to all the facilities necessary for a full cardiovascular investigation including cardiac monitoring and tilt table testing. B) Rehabilitation: Many patients who have seizures lose confidence and are in danger of entering a vicious spiral of immobility in which loss of confidence leads to less mobility: this in turn leads to further loss of confidence. The service should therefore have ready access to falls rehabilitation and a full range of occupational therapy services with good links to the community. C) Concurrent illnesses: Many elderly people with seizures have them on the background of cardiovascular or cerebrovascular disease and will not infrequently have other illnesses. The service should be able to identify and address these concurrent problems. D) Cognitive impairment: In many people seizures will take place in the context of cerebrovascular or non-vascular degenerative cerebral disease. The Epilepsy Service, and in particular the clinic, should provide a sympathetic and supporting environment for such patients and their carers so that their management can be optimised despite the additional barrier of cognitive impairment. 43 Who should provide the epilepsy service for older people ? No one discipline or sector of the health service has all the facilities or skills required to provide a comprehensive service for older people with epilepsy. A multidisciplinary team including nurses, therapists, doctors, psychologists, psychiatrists and social workers, and linking all sectors is needed. Members of the team should have knowledge not only of epilepsy but also of other concurrent medical problems that affect older people. The service must, therefore, be closely related to general medical services for older people as well as to the epilepsy and neurology services. Within the framework outlined above, the service could be led by a neurologist or by a general physician or geriatrician with expertise in the management of older people with epilepsy. A multidisciplinary team including nurses, therapists, doctors, psychologists, psychiatrists and social workers, and linking all sectors is needed. The service should be led by a neurologist or by a general physician or geriatrician with expertise in the management of older people with epilepsy. 44 Section 7 Epilepsy in Complex Situations Much of the process of diagnosis and treatment of people with epilepsy is complex. Earlier sections have described the approach to many of the issues. Sometimes it is the context in which care is provided, or in which decisions are taken, that makes for complexity. This section is concerned with some of those situations. There are three areas of particular interest: Epilepsy in the context of Learning Disability Epilepsy in people with psychiatric or behavioural symptoms The assessment of people with refractory epilepsy Epilepsy in the context of learning disability People with learning disabilities do not always receive the range and quality of care that should be available. This is probably in part due to a lack of training and awareness on the part of neurologists and GPs. In some areas the service is poorly staffed. The control of seizures is an important factor in quality of life for this group of people. They should be entitled to the same concern and consideration given to other people with epilepsy. People with learning disability are entitled to the same care and consideration as that given to other people with epilepsy. Each person with a learning disability and epilepsy should have a written health care plan co-ordinated by a nurse. The health care plan should include regular health screening as well as an epilepsy management plan. It should be reviewed annually. Most care is currently provided by specialists in Learning Disability with some input from GP and Neurologist. Where there is interest and commitment this can work well. It is clearly not appropriate that the quality of care should depend only on individual enthusiasm. Each person with a learning disability should be assured of care of a satisfactory standard that is accessible and dependable. Each person with a learning disability and epilepsy should have a named GP, a named specialist in learning disability, a named nurse and a named Neurologist, responsible for providing necessary care. 45 Each Learning Disability consultant should be part of a team that includes a neurologist able to give advice and support if the control of seizures is at all problematic. Each neurologist providing support to a patient with a learning disability should be part of the Learning Disability Team and the Epilepsy Service, should know the name of the relevant specialist in learning disability and keep other members of the team informed about the management of the patient. Shared or common clinical records or joint clinics could aid communication. One neurologist may link with more than one District. It is desirable that the neurologist should have a special interest in epilepsy. Where this is not possible the team should have an additional link to a named Neurology Centre and a named neurologist with special expertise in epilepsy. Health professionals involved in providing care for people with learning disability and epilepsy should work as a team with clear goals, good communication and mutual respect. The use of shared clinical records should be explored. The team should include a learning disability consultant and a neurologist with a special interest in epilepsy. The service to people with learning disabilities could be improved by improving communication and support among those providing and using the service. The views of people with learning disabilities should be sought through approved techniques. Carers should receive recognition of the role they fulfil, should receive adequate support and information and should have their own needs addressed. Written agreements and agreed protocols could aid communication. Many people with learning disability take antiepileptic medication, sometimes in combination with antipsychotic drugs. All people taking medication, and their carers, must have a printed list of medication along with information about side effects and possible interactions. In addition they should know who to contact if problems arise. Although measures of outcome have yet to be agreed the quality of the service should be monitored. The views of people with learning disability, their advocates and carers should be taken into account. The views of people with learning disabilities should be sought and fully taken into account. They and their carers should receive printed details of current medication and copies of agreed goals and plans together with a list of contact addresses and telephone numbers to give access to appropriate information, advice and support at all times. In each District a Consultant in Learning Disability and a Consultant Neurologist should be identified and invited to describe the service for people with a learning disability. This service should conform to the pattern described in this report. Along with patients, carers and their 46 representatives, they should define standards against which the service is to be measured. Further improvement in the service could be achieved through better training for Neurologists and GPs in the assessment and treatment of people with a learning disability. This may require changes in post-graduate training programmes and the undergraduate curriculum. These issues should be explored with post-graduate and undergraduate deans. Post graduate and Undergraduate Deans should be invited to review the curriculum with a view to improving training in the assessment and treatment of people with learning disability. Epilepsy with psychiatric or behavioural problems People with Epilepsy occurring in the context of a psychiatric illness such as depression or schizophrenia do not require services separate from those for other people with epilepsy. The context of the treatment will be determined by the psychiatrist on the basis of the severity of the psychiatric illness. Because of the potential interaction of medications and the not infrequent uncertainty about the interpretation of some symptoms, close liaison between Neurologist, Psychiatrist and GP is essential. Clinical notes should contain details of all those involved in care along with contact numbers so that advice can be obtained at any time. People with epilepsy occurring in the context of a psychiatric illness should be managed in the same way as other people with epilepsy. Close liaison between neurologist and psychiatrist is essential. The clinical notes should contain the names and contact details of all people involved in care. People with severe behavioural disturbance in whom the question of epilepsy arises do need separate facilities. Sometimes it is difficult to know whether the person has epilepsy or nonepileptic attacks, sometimes genuine epilepsy is difficult to control, and sometimes it is difficult to know whether the behavioural disturbance is a manifestation of undoubted epilepsy. The behavioural disturbance determines the site of care. Facilities should be registered under the Mental Health Act, staffed largely by psychiatry trained nursing and therapy staff, and have an experienced epilepsy specialist as part of the team. This range of requirements suggests that such an assessment unit will be part of a psychiatry unit in close proximity to a Neuroscience Centre with the full range of neuro-physiology, neuropsychiatry, neuroradiology and neuropsychology services. Patients with severe behavioural disturbance in whom the question of epilepsy arises should be managed by an experienced team including a neuropsychiatrist, a neurologist with special expertise in epilepsy, a neuropsychologist, specialised neurophysiology and neuroradiology departments, and specialised therapists. The team must have access to 47 premises registered under the Mental Health Act. Ideally the unit would be part of a neuropsychiatry facility adjacent to, or part of, a neuroscience centre. The number of patients who would benefit from this type of unit is unknown but thought to be small. In the first instance one unit capable of assessing 6-8 patients should be sufficient for the North West of England and North Wales. In order to maintain standards and accountability the unit should be part of mainstream NHS care with clinical governance, audit and staff appraisal linking into the larger unit. It is suggested that this assessment unit should run in parallel with the adult assessment unit for people with refractory epilepsy and be sited in, or adjacent to, a Neuroscience Centre (see below). If the scale proves too small to attract and keep trained and experienced staff, or the cost is excessive, it might be necessary to consider a single facility for the North of England. At present it seems possible that by placing the unit in the context of a larger service it will be possible to achieve the flexibility for staff and the sharing of overheads necessary to make the proposal practicable. A single 8 bedded adult assessment unit for people with behavioural disturbance and epilepsy should be commissioned in the North West. The unit should be part of the mainstream care and take part in audit, training and clinical governance activities of the larger epilepsy and psychiatry services. It should be run in parallel with the assessment unit for refractory epilepsy. In order to ensure that such an arrangement works smoothly to the best advantage of the patient there will need to be formal joint planning of this part of the service with clearly defined roles and responsibilities. Individual patient protocols or pathways should be written for each patient within the context of the generic pathway for the group. A named individual should be responsible for the co-ordination of care and for liaison with other staff, relatives, and carers. Appropriate printed information should be given to the patient and carers. The GP should be kept informed of changes in location or management plan and given clear information and advice at the time of discharge. A named consultant should be responsible for the coordination of care in assessment units, within agreed protocols, and for liaison with members of the team, the patient and carers. The GP should be kept informed and given clear advice at the time of discharge from the unit. Refractory epilepsy A small number of people have epilepsy that proves resistant to all attempts at control. This group requires further assessment facilities in which a specialised team will attempt to confirm the diagnosis, optimise antiepileptic drug therapy, investigate possible causes, decide whether surgical treatment has a place, and identify people with pseudo-seizures or non-epileptic 48 attacks. Some of these functions can be performed as part of the work of an Epilepsy Clinic but for some patients prolonged admission is required in order to make a diagnosis or monitor changes in treatment. Such an assessment unit should be an integral part of the Epilepsy Service using the specialised neuro-physiology, neuro-radiology, neuropsychology and neuropsychiatry services of an Epilepsy Centre. When there are frequent seizures, the diagnosis is in doubt, medication is withdrawn and there is the possibility of status epilepticus, immediate medical, nursing and intensive care help should be available. This, together with the need for the full range of specialised investigative and support facilities, suggests that siting in close proximity to a Neuroscience Centre would be an advantage. Acute wards are not, however, a good place for prolonged admission or for this kind of observation and monitoring. A separate facility should be designated for this group of people. Where skills and facilities are available to allow separation of the presurgical assessment from the longer-term observation while still maintaining standards in both, geographical separation could be acceptable. The prolonged assessment unit would still need to be an integral part of the Epilepsy Service and it would have to be demonstrated that the risks of professional and social isolation could be overcome. The pre-surgical assessment unit should be on the site of a Neuroscience Centre. Currently it is anticipated that a unit of 10-12 beds would be adequate for an Adult Assessment Unit providing for the needs of the people of North West England and North Wales. It should be part of the mainstream NHS epilepsy network in order to ensure accountability and quality of service through the processes of audit, training and clinical governance established in the larger unit. Some of the problems experienced by people with pseudo-seizures require the intervention of a neuropsychiatrist and a neuropsychologist. If the unit providing the neuropsychiatric service for people with behavioural disturbance and epilepsy (see above) were to be reasonably close to this assessment unit there could be some sharing of expertise and resources. A 10-12 bed adult assessment unit should be commissioned for people with refractory epilepsy who require prolonged admission. This unit should ideally be in close proximity to a neuroscience centre with intensive care facilities and should be an integral part of the Epilepsy Service. Proximity to the assessment unit for people with epilepsy and behavioural disturbance would also be an advantage. The management of people with Epilepsy continues over a long period. Continuity of approach and, as far as is possible, of staff is desirable. Generic and patient specific protocols should be developed to allow patients to flow through the various parts of the service while remaining under the care of the same GP and specialist. This will demand good communication and flexibility of consultant staff who may have to supervise the care of patients in a variety of settings. The three Epilepsy Centres in the North West should work 49 together to develop the protocols and working practices necessary for the successful running of the assessment units. Generic and patient specific protocols should be developed to allow patients to move through different parts of the service while remaining under the care of the same team. Agreements between the Epilepsy Centres will be necessary to develop and monitor working practices in the assessment units. A small number of people with epilepsy and/or behavioural problems are so badly affected that they need some form of long term supervision. People in this group usually have either learning disabilities or other serious impairment of neurological function. It is likely to be the associated condition rather than the epilepsy that determines the need for care and thus the nature and place of care. If it is at all possible, people should be enabled to live at home or in the community. Villages and institutions should be a place of last resort. Wherever care is provided it should be considered as part of the Epilepsy Service and conform to the same standards expected of other parts of the Epilepsy Service. 50 Section 8 The Epilepsy Service The earlier sections have described the components necessary for the provision of care for people with epilepsy. This section proposes a system or model to deliver that care which brings together the component parts and defines the level and quality of care to be expected in each setting. This model is a framework within which clinicians at the local level will work out the local details: it does not set out to prescribe those details. The Epilepsy Service leads will look for opportunities to work with interested clinicians to take this process forward. It is important to recognise that this is a single integrated system in which all parts play an important role both in planning and providing care. It is also important to recognise the extent to which care is to be provided in the community by people who are trained and supported through the Epilepsy Service. Figure 1 shows the outline of the Service and Table 3 gives more details. MEDICAL CENTRE COMMUNITY SATELLITE Intermediate Specialist (GP) Specialist Nurse (Community) Neurologist Clinical Asst (GP) Paediatrician Specialist Nurse EEG DGH SATELLITE REGIONAL CENTRE GP Practice Nurse Neurologist Paediatric Neurologist Neurosurgeon Specialist Nurse Neuro-psychologist Neuro-psychiatrist Learning Disability Neurophysiology Neuroradiology Neuropathology Self Help Group * - nurse provided service Figure 1. The Epilepsy Service 51 Annual Follow-Up Rapid Access Diagnosis Medical Follow-Up Adolescent Preconception* Rapid Access Diagnosis Medical Follow-Up Adolescent Preconception* Rapid Access Diagnosis Medical Follow-Up Adolescent Surgical NEAD Pre-surgical Psychiatry Learning Disability Behavioural The Epilepsy Service will be coordinated by a lead clinician assisted by a small team of clinicians from the various sectors and sites. They will be responsible for the development of local arrangements and protocols, the setting and maintenance of standards, training and audit programmes and planning future changes in the provision of care. The lead clinician will be one of a number of lead clinicians in neuroscience services in the North West reporting to the North West Clinical Neuroscience Partnership through the Director of the Partnership. In designing the service and setting standards the requirements of people with epilepsy and their families should be the guiding principles. To ensure that these requirements are understood, and to monitor the extent to which they are being met, the Epilepsy Service will develop a users forum that will have an important part to play in shaping the Service. The development of this forum will be one of the early tasks for the Service. In the meantime the standards that follow have incorporated the requirements of people with epilepsy and the voluntary agencies consulted in the preparation of this Framework. As a general principle patients should have access to all the diagnostic and investigative skills and facilities required to reach a diagnosis and prescribe appropriate treatment. They should also be followed up as close to home as possible. This means enabling access as far into the Centre as is needed and providing skills as far out into the community as possible. It is envisaged that within each general practice partnership the GP and the practice nurse will provide annual follow up of the 70% of people with epilepsy whose epilepsy is stable and who have no side effects from their medication (about 50 patients per practice). In providing this care they will have the support of the rest of the Epilepsy Service and in particular will look to the Community satellite for practical day-to-day advice. The Community satellite is likely to be the place where most people have the diagnosis confirmed. It will also provide more specialised advice and support for people with proven, uncomplicated epilepsy within one PCT and could be PCT based (about 100 new patients per year). The Intermediate specialist would be a GP from the PCT/group who has received additional training, probably through attachment to the District Satellite or Regional Centre. Treatment should be limited to monotherapy with first-line antiepileptic drugs. The nurse will be an appropriately trained epilepsy specialist nurse who will retain regular contact with the District Satellite and the Centre as well as supporting practice nurses in their role. It is likely that one specialist nurse at this level would service several Community satellites depending on the size of the practices, PCTs and Districts. A major part of the role will be ensuring that the practice nurses have the necessary skills and support to provide care to the 70% of patients who receive their care at practice level. When the diagnosis is not straightforward, when investigation is required or when there are problems with treatment the District Satellite 52 should be used. Staff in this Satellite will support about 5 Community Satellites depending on local organisation and population distribution. EEG and CT scanning will be available allowing a confident diagnosis and classification for most patients. At this level medication will be reviewed and, if indicated, combination therapy might be used. If the diagnosis remains in doubt or control of seizures is not possible patients should be referred to the Regional Epilepsy Centre for further consideration. Ideally one neurologist in each District level neurology service should have particular expertise in the management of people with epilepsy. Currently this is not possible. All neurologists have considerable experience of epilepsy and until more neurologists with special expertise in this field are available they will provide the service in most Districts. The neurologist at District level should have a close link with a neurologist with such an interest at the Regional Epilepsy Centre, and should be ready to ask for further advice. In any event the neurologist at the District Satellite should be part of the Epilepsy Service and agree to the responsibilities for training and standards set by the Service. The nurse will be a specialist epilepsy nurse, appropriately trained and supervised. Although she will have a clinical case load she will be responsible for maintaining the nursing role in the Community Satellites in the District. Depending on the geography and size it might be necessary to have more than one nurse at this level. The Regional Epilepsy Centre should be regarded as a support for the rest of the Service. It is also the place where more complex issues are addressed in the context of multidisciplinary teamwork and the place where research is coordinated. In particular assessment of patients with intractable seizures, patients with behavioural or psychiatric problems, patients in whom the diagnosis remains in doubt, patients with nonepileptic attack disorder and patients who are thought to be candidates for surgery should all be seen by the team from the Regional Epilepsy Centre. Training should be a high priority with a strong commitment to maintaining and improving skills throughout the Epilepsy Service. In order to avoid isolation and to have comparable practices for outcome evaluation, audit and governance the three Centres in the North West should develop joint training, audit and governance procedures. The Regional Epilepsy Centres must also develop listening opportunities to hear feedback from all other parts of the Service. In order to avoid isolation and to have comparable practices for outcome evaluation, audit and governance the three Centres in the North West should develop joint training, audit and governance procedures. Validated literature giving consistent information should be available to people with epilepsy and their family or carers in each part of the Epilepsy Service. 53 The Epilepsy Service Site Medical Centre (1ocare) Care Initial presentation Annual follow up (FU) of definite epilepsy with good control and no complications (Population 10,000) Community Satellite (Population 100,000) Probable epilepsy referred for diagnosis FU definite epilepsy with no complications Preconception advice Table 3. Expectations and Standards Patient Expectations Prompt appointment Provisional diagnosis Explanation Appropriate treatment or referral Professional Expectations Support Information CME Easy access to next stage Good communication Standards Appointment <3 weeks Diagnosis confirmed Diagnosis explained Treatment explained Lifestyle advice FU information Referral if needed Written information provided 54 Support Information Training Good communication Prompt response Leadership Diagnosis given No diagnosis of epilepsy or use of antiepileptic drugs without referral Witness interviewed Patient expectations met Dr attends training session in Epilepsy Service each year Definite diagnosis or onward referral Monotherapy Good control Nurse sees all patients Patient expectations met Nurse/Dr attend 4 Epilepsy Service approved training sessions each year District Satellite (Population 500,000) Regional Centre (Population 1.5 - 3 million) ? Epilepsy ? cause Treatment failure Preconception advice ? Epilepsy Non Epileptic Attack Disorder Behavioural disturbance and epilepsy ? cause of epilepsy Treatment failure Surgery assessment Surgery Multidisciplinary care Research/Trials Appointment <3 weeks Investigations same day Results explained Diagnosis confirmed Diagnosis explained Treatment explained Lifestyle advice FU explained Written information provided Appointment <6 weeks Investigations explained Diagnosis confirmed Diagnosis explained Options explained Treatment explained FU explained Ethical approval and consent for trials Written information provided 55 Support Information Training Good communication Leadership Interdisciplinary commitment Teamwork Good communication Information Training Leadership Feedback Syndromic diagnosis made Appropriate use of investigation/drugs Appropriate referral back to community Nurse sees all patients Patient expectations met Nurse/Dr attend 4 Epilepsy Service approved training sessions each year Syndromic diagnosis made Appropriate use of investigation/drugs Results comparable with other centres Patients expectations met All staff involved in and receiving regular training Research protocols followed The neurophysiology service There are few uses for EEG outside the Epilepsy Service. Such non-epilepsy EEG uses are in the context of neurology. It is therefore reasonable to organise EEG services to maximise their availability to the Epilepsy Service. The classification of some forms of epilepsy is dependent on a good EEG properly reported. This facility must be available to the District Satellite. Video-telemetry and depth electrode recordings take place in assessment centres and the Regional Epilepsy Centres. These techniques are more demanding of technical time and expertise as well as the knowledge and experience of the neurophysiologists. Technical staff and neurophysiologists are in short supply. A network of services planned and coordinated in conjunction with the Epilepsy Service is likely to offer the best use of limited resources. Recruitment, training and maintenance of standards should be the responsibility of the Centre. The EEG service is an important part of the Epilepsy Service and should be organised in a way that maximises its availability and usefulness to the Epilepsy Service. 56 Section 9 Summary Presentation and Diagnosis Clinicians seeing people with blackouts, funny turns and faints in primary care and A&E Departments should know the differential diagnosis of these symptoms. In particular they should recognise the significance of symptomatic seizures and be aware of the motor phenomena which frequently occur during syncope. Where epilepsy is suspected or established by the clinician, referral into the specialist Epilepsy Service is indicated. Where epilepsy is symptomatic referral to a neurology clinic or medical clinic could be appropriate. People thought to have epilepsy should have an appointment in the Epilepsy Service within 4 weeks from presentation. Those people whose signs or symptoms suggest an underlying cause should be referred urgently. The Epilepsy Service should appointments when necessary. be designed to facilitate urgent Patients who do not have epilepsy should have a clear explanation of the nature and implications of their symptoms. Any necessary further referral should be arranged. The General Practitioner should receive the same information within 10 days. When the diagnosis is not clear, people who could have epilepsy should be followed up, and investigated as necessary, in the Epilepsy Service. Good communication with the patient and the GP is essential. At the time of diagnosis people with epilepsy should receive a detailed explanation of the diagnosis and its implications, should have written information provided and should be introduced to the specialist epilepsy nurse. The GP should be informed within 10 days. People thought to have idiopathic generalised epilepsy should have an EEG but do not need cerebral imaging (CT/MR). People with unclassified epilepsy beginning before the age of 30 should have a CT/MR scan and an EEG. People with partial seizures or with unclassified seizures starting after the age of 30 should have a CT/MR scan but do not need an EEG at this stage. 57 Initially older people with epilepsy, or suspected to have epilepsy, should be referred to a “Medicine for the Elderly” service where there is a physician with appropriate expertise. There should be close cooperation between this service and the Epilepsy Service. Treatment When treatment is necessary monotherapy with an appropriate antiepileptic drug should be introduced. Combination therapy should only be used, after failure of monotherapy, within the agreed framework of the Epilepsy Service. Clinical trials are needed to determine best treatment. People requiring treatment should be invited to join such trials. Invitations to participate in trials should be strictly within the conditions laid down by the ethics committee and should only be discussed in the context of full disclosure of all known risks and benefits. Fully informed consent must be obtained. Continuing Management People with epilepsy, or who might have epilepsy, should have continuing access to appropriate care and support. People with an uncertain diagnosis should continue to be followed-up in the Epilepsy Service. People with epilepsy should be seen by a specialist nurse 3 months after presentation to the Epilepsy Service. Further follow-up will be planned in the light of this appointment. People whose epilepsy is in remission at 1 year should be seen by a consultant/specialist in the Epilepsy Service. After explanation arrangements should be made for annual review in the primary care setting. They should have clear instructions on what to do if they have a further seizure or side effects or need advice. Good communication is essential to good care. People with epilepsy should receive written information giving details of agreed goals and plans. All those involved in care should have copies of this information and of all other communications within 10 days. Shared clinical records should be developed. People continuing to have seizures or side effects of medication at 1 year should remain under annual review by the consultant with additional specialist nurse supervision as necessary. 58 People who continue to have seizures in spite of medication should be reviewed thoroughly in the Epilepsy Service to confirm or refute the diagnosis. Patients with non epileptic attack disorder should be identified. After identification antiepileptic treatment should be withdrawn and help should be provided by a neuropsychologist and a neuropsychiatrist. It is important to try to reach a diagnosis for the cause of the attacks. People should only be considered to have intractable epilepsy after adequate trials of maximum tolerated doses of appropriate antiepileptic drugs singly and in combination. Assessment for surgery is a very specialised process: it should only be undertaken in approved Epilepsy Centres with a dedicated, multidisciplinary team who jointly undertake regular reviews of outcome. The surgery should be undertaken by a neurosurgeon with particular expertise in surgery for epilepsy. All participating neurosurgeons must agree to take part in regular outcome review. Following surgery multidisciplinary follow up, with assessment and support, must be provided. Status epilepticus is a medical emergency . Immediate treatment is required and should follow the agreed protocol. ITU facilities are essential and neurological advice must be sought urgently. The Adolescent with Epilepsy An epilepsy service should be available to all children with epilepsy and should deliver high quality care to approved standards in an acceptable manner. The physical, psychosocial and cognitive development of the adolescent with epilepsy should be assessed and any necessary help provided. The needs of the whole family should be addressed. Seizure control is an important part of the management of the adolescent with epilepsy. Counselling and cognitive behavioural therapy should be available. A multidisciplinary transitional service, which addresses medical and psychosocial issues, should be available to all adolescents with epilepsy. Women with Epilepsy The services provided for women with epilepsy must reflect their particular needs. 59 It is particularly important to have an accurate diagnosis in women of child-bearing age. Treatment with antiepileptic drugs must take account of the need for contraception, planning of conception, pregnancy and infant care. Women should understand that the control of epilepsy may change during pregnancy and be advised about the risks to the foetus both of seizures and of antiepileptic drugs. At pre-conception counselling women should be advised to take 5 mg of Folate daily. There should be close co-operation between the Epilepsy Service and the obstetric service throughout pregnancy. In the final month of pregnancy women with epilepsy should receive Vitamin K1 , 10mg daily, to reduce the risk of a bleeding disorder in the new-born baby. There is no general contra-indication to breast feeding. If the baby becomes irritable, drowsy or feeds poorly the situation should be reviewed. Each pregnant woman with epilepsy should be seen early in pregnancy and given information and advice on the risks of continuing seizures and the role of antiepileptic medication. Relatives should be instructed on what to do during a seizure and on the use of the recovery position. Older people with Epilepsy Older people should have their own specialist service for epilepsy. Physicians specialising in care for the elderly should work closely with neurologists with special expertise in managing epilepsy in older people, to ensure that all aspects of the service can be provided. A multidisciplinary team including nurses, therapists, doctors, psychologists, psychiatrists and social workers, and linking all sectors is needed. The service could be led by a neurologist or by a general physician or geriatrician with expertise in the management of older people with epilepsy. People with Learning Disability People with learning disability are entitled to the same care and consideration as that given to other people with epilepsy. Each person with a learning disability and epilepsy should have a written health care 60 plan co-ordinated by a nurse. The health care plan should include regular health screening as well as an epilepsy management plan. It should be reviewed annually. Each person with a learning disability and epilepsy should have a named GP, a named specialist in learning disability, a named nurse and a named Neurologist, responsible for providing necessary care. Health professionals involved in providing care for people with learning disability and epilepsy should work as a team with clear goals, good communication and mutual respect. The use of shared clinical records should be explored. The team should include a learning disability consultant and a neurologist with a special interest in epilepsy. The views of people with learning disabilities should be sought and fully taken into account. They and their carers should receive printed details of current medication and copies of agreed goals and plans together with a list of contact addresses and telephone numbers to give access to appropriate information, advice and support at all times. In each District a Consultant in Learning Disability and a Consultant Neurologist should be identified and invited to describe the service for people with a learning disability. This service should conform to the pattern described in this report. Along with patients, carers and their representatives, they should define standards against which the service is to be measured. Post graduate and Undergraduate Deans should be invited to review the curriculum with a view to improving training in the assessment and treatment of people with learning disability. Epilepsy in Disturbance the Context of Psychiatric Illness or Behavioural People with epilepsy occurring in the context of a psychiatric illness should be managed in the same way as other people with epilepsy. Close liaison between neurologist and psychiatrist is essential. The clinical notes should contain the names and contact details of all people involved in care. Patients with severe behavioural disturbance in whom the question of epilepsy arises should be managed by an experienced team including a neuropsychiatrist, a neurologist with special expertise in epilepsy, a neuropsychologist, specialised neurophysiology and neuroradiology departments, and specialised therapists. The team must have access to premises registered under the Mental Health Act. Ideally the unit would be part of a neuropsychiatry facility adjacent to, or part of, a neuroscience centre. 61 A single 8 bedded adult assessment unit for people with behavioural disturbance and epilepsy should be commissioned in the North West. The unit should be part of the mainstream care and take part in audit, training and clinical governance activities of the larger epilepsy and psychiatry services. It should be run in parallel with the assessment unit for refractory epilepsy. A named consultant should be responsible for the coordination of care in assessment units, within agreed protocols, and for liaison with members of the team, the patient and carers. The GP should be kept informed and given clear advice at the time of discharge from the unit. Refractory Epilepsy A 10-12 bed adult assessment unit should be commissioned for people with refractory epilepsy who require prolonged admission. This unit should ideally be in close proximity to a neuroscience centre with intensive care facilities and should be an integral part of the Epilepsy Service. Proximity to the assessment unit for people with epilepsy and behavioural disturbance would also be an advantage. Generic and patient specific protocols should be developed to allow patients to move through different parts of the service while remaining under the care of the same team. Agreements between the Epilepsy Centres will be necessary to develop working practices in the assessment units. In order to avoid isolation and to have comparable practices for outcome evaluation, audit and governance the three Centres in the North West should develop joint training, audit and governance procedures. Information Validated literature giving consistent information should be available to people with epilepsy and their family or carers in each part of the Epilepsy Service. The EEG Service The EEG service is an important part of the Epilepsy Service and should be organised in a way that maximises its availability and usefulness to the Epilepsy Service. 62 Appendix 1 North West Clinical Neuroscience Partnership The Partnership came into being on September 1st 2000. Goal To minimise the impact of damage to the nervous system on the life of individuals and society through: 1) Seeking and using opportunities for prevention 2) Ensuring prompt access to appropriate, expert care following acute illness or injury 3) Developing paths of care which guarantee appropriate, accessible and sensitive care for people with chronic conditions 4) Supporting all providers of care whether lay or professional 5) Increasing understanding, in the NHS and wider society, of the impact of damage to the nervous system on the lives of affected individuals 6) Involving patients, carers and voluntary organisations in decision making, planning and evaluation of services. Working Together Trusts and Commissioners will work together to develop strategies to achieve the above goals. They will: 1) Together agree a North West Service Framework for the provision of Clinical Neuroscience services 2) Together encourage the development of North West Commissioning for the Clinical Neurosciences. In order to achieve this they agree to: 1) Share activity and outcome information 2) Develop a joint programme of benchmarking and audit 63 3) Develop a common understanding of manpower and training requirements 4) Ensure that proposals and job plans for new senior clinicians (Consultants and Nurse Consultants) are shared at an early stage and in all cases prior to appointment 5) Share development plans and capital bids above £100,000 at an early stage and at latest, prior to submission of a business case or service plan 6) Explore mechanisms for joint evaluation and introduction of new drugs and technologies 7) Define the information requirements to support co-operative working and develop the information system to deliver them. Monitoring This is a co-operative venture and it is not anticipated that individual groups or organisations will be less than fully committed. In the event of a dispute, the group will determine an acceptable procedure, possibly involving the RSCG, to resolve the issue. Conclusion This partnership represents an agreement between the three Tertiary Centre Trusts and the three Zonal Neuroscience Commissioners to work together to ensure that the population of the North West and North Wales has equitable access to a Clinical Neuroscience service which will meet their needs and will be both effective and efficient in its use of resources. Members (As at Sept 1st, 2000) Chief Executive, Preston Acute Hospitals NHS Trust Chief Executive, Salford Royal Hospitals Trust Chief Executive, Walton Centre for Neurology and Neurosurgery Specialised Commissioning Lead, Neurosciences, Greater Manchester Zone Specialised Commissioning Lead, Neurosciences, Lancashire and South Cumbria Specialised Commissioning Lead, Neurosciences, Merseyside and Cheshire Member, Specialist Health Services Commisioners for Wales Regional Specialised Commissioning Manager, NW Regional Office, NHS Clinical Director, Neurosciences, Preston Director of Neurosciences, Greater Manchester Medical Director, Walton Centre Chief Executive of Health Authority as Chair, (Sefton nominated from 2000) 64 Appendix 2 Participants and Advisors Kathy Bairstow Gus Baker Elizabeth Berry Sue Blake David Chadwick Paul Cooper Ivor Crook John Duncan Susan Duncan Paul Eldridge Jacqui Howard Bev Kain Mike Kerr Doug McDonald Tahir Majeed Ian Minshall Douglas Mitchell Martin Murphy Kieran O’Driscoll Simon Parsons Richard Pickles Hamira Riaz Peter Richardson Peter Rogan Stephen Rowe Angela Russell Joe Russell Simon Shorvon David Smith Ray Tallis Brian Tedman Ajay Thapar Philip Tidswell Udo Weishman Janine Winterbottom British Epilepsy Association Neuropsychologist, Liverpool Neuropsychologist, Manchester British Epilepsy Association Neurologist, Liverpool Neurologist, Manchester GP, Greater Manchester Neurologist, London Neurologist, Manchester Neurosurgeon, Liverpool Learning Disabilities, NW Regional Office, NHS Epilepsy Nurse, Preston Learning Disabilities, Cardiff GP, Cheshire Neurologist, Preston GP, Cheshire Neurologist, Preston Medical Director, St Helens & Knowsley HA Neuropsychiatrist, Liverpool Psychiatry, David Lewis Centre GP, Wrexham Neuropsychologist, Manchester Neurosurgeon, Manchester Secretary, Joint Epilepsy Council Learning Disabilities, Greater Manchester Preston Preston Neurologist, London Neurologist, Liverpool Geriatrician, Manchester Neurophysiologist, Liverpool GP, Manchester Neurologist, Preston Neurologist, Liverpool Epilepsy Nurse, Liverpool 65 Appendix 3 Literature Survey There is general agreement that people with epilepsy need accurate information given in a form and at a time that makes it readily understood. Contact with clinicians is relatively short and infrequent even now the number of specialist nurses has increased. Written information has been used as an adjunct to explanations given by clinicians and has the advantage that it can be turned to from time to time, between clinic appointments, as questions arise. The numerous web sites dealing with aspects of epilepsy and its treatment are also available as a source of information to professionals and general population alike. A working group reviewed the literature in use in the epilepsy clinics in the North West and compiled a database of the contents and relevance of that literature. Initially it was hoped that it would be possible to recommend a limited number of items which could be agreed for use in all sectors, thus reducing the possibility of confusion. The survey showed some 70 items in use with the voluntary organisations producing the majority. Although conversations with the Joint Epilepsy Council (the coordinating body for the more than 20 voluntary organisations in the UK concerned with epilepsy) were helpful they could hold out no hope of cooperation among the constituent organisations to rationalise the range of literature available. The database is included in this report as an example. It will become out of date very rapidly. It should serve to indicate both that there is a large volume of literature available and that much work will have to be undertaken on a regular basis to ensure that people with epilepsy have appropriate information and support. Literature must be available in the epilepsy service in the North West and North Wales and should be offered at the outset along with an opportunity for subsequent explanation at a meeting with the specialist nurse. The information should be reviewed for accuracy and usefulness by a member of the Epilepsy Service and all new literature should be assessed prior to introduction. It is difficult if not impossible to review all the information available on web sites. People should be encouraged to discuss with clinicians any questions that arise from visits to internet sites. 66 Available From Learning Disability Children Adolescents Older People BEA BEA Yes No No Yes No Yes No No No No No No Janssen Cilag Janssen Cilag No No No No No No No Yes No No No No Diagnosing Epilepsy NSE NSE Yes No No No No No No No No No No No Diagnosis of Epilepsy Hart & Rogan MREA Yes Yes Yes No No No No No No No No No NSE NSE Yes Yes No No No No No No No No No No Hanscomb & Hughes MREA, Bookshop Yes Yes Yes No No No No No No No No No Epilepsy – Complementary Therapies NSE NSE No No No Yes No No No No No No No No Epilepsy - Guide for Patients and Carers BBSF BBSF Yes Yes Yes No Yes Yes Yes Yes No No Yes Yes Epilepsy and Children BEA Epilepsy and Driving BEA BEA Yes No No No No No No No No Yes No No BEA Yes No No No Yes No No No No No No Epilepsy and Driving and Travel No NSE NSE No No No No Yes No No No No No Yes No Epilepsy and Education BEA BEA Yes No No No No No Yes No No Yes Yes Yes Epilepsy and Employment BEA BEA No No No No No Yes No No No No No No Epilepsy and Everyone BEA BEA Yes Yes Yes No Yes Yes Yes No No No Yes Yes Epilepsy and Inheritance BEA BEA No No No No No No No No No No No No Epilepsy and Learning Difficulties Rowe & Rogan MREA, Sanofi Winthrop Yes Yes No No No No Yes No Yes Yes No No Epilepsy and Learning Disabilities BEA BEA Yes Yes No No No No Yes No Yes No No No Epilepsy and Learning Disabilities (NSE) NSE NSE No No No No No No No No Yes No No No Epilepsy and Leisure NSE NSE No No No No No No Yes No No No No No Complementary Treatment Fact Sheet Contraception: Advice before Pregnancy Drug Treatment of Epilepsy Epilepsy General Medical Information Treatment Surgical Treatment CompleWomen mentary Driving Employment Life-style & Therapy Epilepsy Author Title Appendix 3. Literature Survey 67 Title Epilepsy and Pregnancy/caring for young children Epilepsy and Self Management Appendix 4. Epilepsy and Surgery Literature Epilepsy and the Young Adult Survey 1 General Medical Information Treatment Surgical Treatment CompleWomen mentary Driving Employment Life-style & Therapy Epilepsy Author Available From Learning Disability Children Adolescents Older People BEA BEA No No No No No No No Yes No No No No BEA BEA No No No No No No BEA BEA Yes No No No No No Yes No No No No No Yes No No Yes Yes Yes No No Yes No No No No No No No No No Yes Yes Yes Yes Yes Yes Yes Yes No Yes Yes No BEA BEA Epilepsy Bereaved BEA Epilepsy Bereaved BEA No No No No No No No Yes No No Yes No NSE NSE No No No No No No No No No No No No Epilepsy Explained Laidlaw MREA, Bookshop Yes Yes Yes No Yes Yes Yes Yes No No No No Epilepsy in Adolescence (Companion’s Guide) Baker & Rogan MREA, Sanofi Winthrop Yes Yes No No Yes No Yes No No No Yes No Epilepsy in Adulthood (Companion’s Guide) Baker & Rogan MREA, Sanofi Winthrop Yes Yes No Yes Yes No Yes Yes No No No No Epilepsy in Childhood (Companion’s Guide) Baker & Rogan MREA, Sanofi Winthrop Yes Yes Yes No No No Yes No No Yes No No Appleton & Gibbs Bookshop No No No No No No No No No Yes Yes No Wallace Bookshop No No No No No No No No No Yes No No Tallis Bookshop No No No No No No No No No No No Yes Epilepsy and Women Epilepsy and Safety Epilepsy at School Epilepsy in Childhood and Adolescence Epilepsy in Children Epilepsy in Elderly People Epilepsy in Later Life JEC JEC Yes No Yes No Yes Yes Yes No No No No Yes Epilepsy in Later Life (Companions Guide) Baker & Rogan MREA, Sanofi Winthrop Yes Yes No No Yes Yes Yes No No No No Yes Kramer Bookshop No No No No No No No No No No No Yes Hopkins & Appleton BEA, Bookshop Yes Yes Yes Yes Yes Yes Yes Yes No Yes Yes Yes Epilepsy in the Elderly Epilepsy the Facts Appendix 3. Literature Survey 68 Author Available From Rogan MREA, BEA BEA BEA Butler, Kerr & Todd NSE GlaxoWellcome NSE Facts about Epilepsy in Later Life NSE NSE Facts about Epilepsy Surgery NSE NSE Title Epilepsy,The Detective’s Story Epilepsy: Alcohol and Street Drugs Epilepsy: The Plain Facts Explaining Epilepsy First Aid for Epilepsy NSE NSE Hand in Hand Appleton BEA, MREA Illustrated Encyclopedia of Epilepsy Chadwick Illustrated Junior Encyclopedia of Epilepsy Learning about Epilepsy Appleton MREA, BEA Beran MREA, Bookshop Living with Epilepsy BEA, MREA, Bookshop General Medical Information Treatment Surgical Treatment CompleWomen mentary Driving Employment Life-style & Therapy Epilepsy Learning Disability Children Adolescents Older People Yes No No No No No Yes No No Yes No No No No No No No No Yes No No No Yes No Yes Yes No No No No Yes No Yes Yes No No Yes No No No No No No No No No No No Yes Yes No No No No Yes No No No No Yes No No Yes No No No No No No No No No No No No No No No No No No No No No No No No No No No No No No No Yes No Yes Yes Yes No Yes Yes Yes Yes No No Yes Yes Yes Yes No No No No Yes No No Yes Yes No No No No No No No No No No Yes No No Yes No No No No No No No No Yes Yes Yes Yes No No No No No No No No No No No Chadwick & Usiskin Living with Epilepsy(Fenwick) Fenwick BEA, Bookshop BEA, Bookshop My Mum has Epilepsy Llewellyn MREA, BEA Yes No No No No No Yes No No Yes No No Non Epileptic Attacks NSE NSE No No No No No No No No No No No No Photosensitive Epilepsy (BEA) BEA BEA Yes No No No No No No No No No No No Photosensitive Epilepsy (NSE) NSE NSE Yes Yes No No No No No No No No No No Pregnancy and Childcare NSE NSE No No No No No No No Yes No No No No Pregnancy and Delivery Janssen Cilag Janssen Cilag No No No No No No No Yes No No No No Appendix 3. Literature Survey 69 Title Author Available From General Medical Information Treatment Surgical Treatment CompleWomen mentary Driving Employment Life-style & Therapy Epilepsy Questions about Anti Epileptic NSE Drugs NSE Yes Yes No No No No Safety in the Home NSE NSE No No No No No Sport and Leisure BEA BEA No No No No No Sudden Death in Epilepsy NSE NSE No No No No SUDEP Fact Sheet BEA BEA No No No Surgery and Type of Operation Swimming and Epilepsy Janssen Cilag MREA Janssen Cilag MREA No No No Travel Fact Sheet BEA BEA No Treatment of Epilepsy BEA BEA Understanding Epilepsy Walker and Shorvon Family Doctor, BMA NSE Learning Disability Children Adolescents Older People No No No No No No No No No No Yes No No No Yes No No No No No No No No No No No No No No No No No No No No No No Yes No No No No No No No No No No No No No No Yes No No No No No No No No No No Yes No No No No No Yes Yes Yes Yes No No Yes No No Yes Yes Yes Yes Yes Yes No Yes Yes Yes Yes No No Yes Yes Vagus Nerve Stimulation NSE No No Yes No No No No No No No No No What difference does it make Danny? Young MREA, Bookshop No No No No No No No No No Yes No No Women and Epilepsy Betts & Crawford BEA, Bookshop No No No No No No No Yes No No Yes No Women with Epilepsy: Factsheet WWE Campaign WWE Campaign No No No No No No No Yes No No No No Appendix 3. Literature Survey 70 References 1 Wright J, Pickard N, Whitfield A et al. A population-based study of the prevalence, clinical characteristics and effect of ethnicity in epilepsy. Seizure 9: 309-313, 2000 2 Morgan CLI, Ahmed Z and Kerr MP. Social deprivation and prevalence of epilepsy and associated health usage. J Neurol Neurosurg Psychiatry 69: 1317, 2000 3 Zarrelli MM, Beghi E, Rocca WA et al. Incidence of epileptic syndromes in Rochester, Minnesota: 1980-1984. Epilepsia 40: 1708-1714, 1999. 4 MacDonald BK, Cockerell OC, Sander JWAS et al. The incidence and lifetime prevalence of neurological disorders in a prospective communitybased study in the UK. Brain 123: 665-676, 2000. 5 Wallace H, Shorvon S and Tallis R. 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