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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
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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
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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.
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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
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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
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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
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