The basics Epilepsy by Dr Nivedita Bajaj , She is a Consultant Paediatrician working within NHS, currently employed by East and North Herts NHS Trust. Dr Bajaj has extensive experience in assessment and management of a wide range of neurodevelopmental conditions and neurodiabilities. She leads clinical autism service in her trust.
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2. Objectives
An overview of epilepsy
List the basic classification of epileptic
seizures
Getting key facts in the history
Being aware of general management
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Dr Nivedita Bajaj
3. Introduction
Most common neurological condition
Prevalence of 0.5 – 1.0%
Annual incidence in childhood is about 60 per
100,000, excluding neonatal seizures
Much more common in children with pre-existing
neurological disability
12-18% of children with mild learning disability,
21 – 36% of children with
severe learning disability and in up to 90% of children with
bilateral spastic cerebral palsy (GMFCS V)
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4. What is epilepsy?
A transient occurrence of signs and / or symptoms due
to abnormal excessive or synchronous neuronal activity
in the brain
Epilepsy is a disorder of the brain characterised
by an enduring predisposition to generate
epileptic seizures and by the neurobiological,
cognitive, psychological and social
consequences of this condition.
ILAE definition 4
Dr Nivedita Bajaj
5. Is it Epilepsy?
In the study of Uldall et al the misdiagnosis rate
was of the order of 40%.
The commonest reasons for misdiagnosis are:
a) Failure to take an accurate history.
b) Failure to think of possible differential diagnoses.
c) Undue reliance on the EEG.
d) Diagnosis may be difficult!
5Dr Nivedita Bajaj
6. NICE 2012
“all children, young people and adults with
a recent onset suspected seizure should
be seen urgently by a specialist”.
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7. Classification of the epilepsies
The International League Against Epilepsy
(ILAE) suggests a flexible approach with
diagnosis on 5 axes:
Axis 1 – description of the seizure.
Axis 2 – epileptic seizure type from an accepted list.
Axis 3 – syndrome from an accepted list if possible.
Axis 4 – aetiological diagnosis if known.
Axis 5 – degree of impairment caused by epilepsy.
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8. Aetiological classification
A specific cause for a child’s epilepsy is only
found in about 30% of cases
Genetic
Structural/Metabolic
Disorders of brain development (migration disorders,
hydrocephalus)
Damage to the brain caused by hypoxic-ischaemia, infection,
inflammation, etc.;
Space-occupying lesions such as hamartomas, arachnoid cysts
and tumours.
Metabolic disorders ranging from aminoacidopathies to
mitochondrial cytopathies
Unknown
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9. What triggers seizures?
No apparent reason
Certain triggers make a seizure more
likely
These are not the cause of epilepsy, but
may trigger a seizure
Examples: Tiredness, flashing lights, lack 9
10. Different types of seizures
There are over forty different types of
seizures.
Seizures can be divided into two main types:
Generalised seizures
Focal seizures
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11. Seizure types
Generalised seizures
These initially involve both hemispheres of the
brain. The person will be unconscious
Focal seizures
These arise from one part of the brain. Symptoms
and level of consciousness depend on the area of
the brain involved
Focal seizures account for around 60% of all
cases of epilepsy
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13. Generalised seizures
Tonic clonic seizures
Often preceded by a cry, the person becomes rigid and
may fall if standing. The muscles then relax and tighten
rhythmically causing the person to convulse. Breathing
may stop or become laboured and they may be
incontinent.
Tonic seizures
There is a general stiffening of muscles without
rhythmical jerking. There is loss of consciousness
causing the person to fall to the ground (usually
backwards) with consequent risk of injury.
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14. Generalised seizures
Clonic seizures
These are characterised by rhythmic or semi-
rhythmic muscle contractions typically involving
the upper extremities, neck and face.
Atonic seizures
There is a sudden loss of muscle tone, with
instantaneous collapse (usually forwards) often
resulting in facial or other injuries. People
normally recover quickly.
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15. Generalised seizures
Myoclonic seizures
Consist of sudden, brief muscle contractions,
either singly or in clusters that can affect any
muscle group.
Absence seizures
Usually last 5-10 seconds. They manifest as a
sudden onset of staring and impaired
consciousness with or without eye blinking and
lip smacking.
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16. Focal seizures
Simple partial seizure
Often referred to as a ‘warning’ or ‘aura’
No impairment of consciousness
Sometimes develop into other seizures
These can present with:
Rhythmical twitching of one limb, or part of
a limb
Unusual tastes, smells or sensations such
as pins and needles in a distinct part of
the body 16
17. Secondarily generalised seizures
Focal seizures may spread to involve the
whole of the brain and if this happens it is
called a secondarily generalised seizure
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18. epileptic encephalopathy
Epileptic activity itself may contribute to
disturbances of behavioural and cognitive
functioning, above and beyond that
expected from the underlying pathology.
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19. Epilepsy syndromes
NICE (2012) gives greater weight to
epilepsy syndrome diagnosis in
determining treatment choice than seizure
type
Epilepsy syndrome is formed by the
seizure type, EEG characteristics, age at
onset, presence of underlying neurologic
abnormalities, imaging results and diurnal
pattern of seizures
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20. Diagnosis of Epilepsy
Clinical history
Eye witness account of the event
Medical examination
Results of any further investigations
should be interpreted with reference to the
history of the event
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21. History taking
Setting: sleep or awake
Stimulus: eg: frustration, fright, breath-holding
Aura (warning): precedes a focal seizure
Onset - the first event in a seizure
Course of seizure
Offset
Post-ictal condition
After effects: such as prolonged confusion,
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23. Investigations
No tests to diagnose epilepsy
Diagnosis of epilepsy is clinical
Investigations:
- may support the diagnosis
- help in the classification
- guide management
- identification of an underlying cause
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24. Blood glucose at the time of the seizure or
early morning
Serum calcium
12 lead ECG recording
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26. EEG
The EEG may be normal in children with epilepsy
and abnormal in children without.
An EEG should NOT be done if the clinical
diagnosis is probable syncope or non-epileptic
because of the possibility of a false positive
result.
NICE guidance 2012 is clear that the role of the
EEG is to provide supporting evidence
for the clinical diagnosis of epilepsy and it can help with epilepsy
classification or
epilepsy syndrome diagnosis.
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27. EEG
Ictal EEGs – Recording the EEG during seizures
is valuable in defining the epilepsy syndrome
and in providing information about site of onset.
This may be possible, if seizures are sufficiently
frequent, by recording a 24-hour ambulatory
EEG or by using video EEG telemetry.
Provocative tests – sleep deprivation,
hyperventilation, photic stimulation.
Invasive recording
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29. Magnetic Resonance Imaging (MRI)
Investigation of choice
Better sensitivity or specificity
Useful in identifying structural abnormalities in
children
Computerised Tomography (CT or CAT)
Preferred if patient is acutely unwell
Valuable in detecting acute intracranial
hematoma, fractures, intracranial calcification, or
when there is a contrindicaton to MRI
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Neuroimaging
30. Neuroimaging
NICE guidance 2012: children and young people
with epilepsies should undergo neuroimaging
with MRI if they have epilepsy and:
Are under two years of age.
Have a suggestion of focal onset on history,
examination or EEG (unless clear evidence of benign
focal epilepsy).
Have seizures that continue despite first line
medication.
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31. Treatment of epilepsy
Why should epilepsy be treated?
Mortality
Morbidity
Injuries and accidents
SUDEP
Learning
Behaviour
Status epilepticus
Quality of life issues
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32. Treatment of epilepsy
The main objectives of treatment:
To enhance quality of life by stopping all seizures
with minimal side effects
Where seizures cannot be stopped without side
effects:
Minimize the number of seizures, especially tonic
clonic, tonic and atonic seizures
Minimise adverse effects of treatment
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33. Anti epileptic drugs
Drug should be selected with reference to
seizure type/syndrome, age and sex of
the individual
Started at a low dose and increased
slowly to aid tolerance
Regime kept simple with once or twice
daily dosing
Drugs which might increase some
seizures should be avoided
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34. Epilepsy Surgery
Neurosurgery should be considered if:
The drug treatment has been ineffective
Diagnostic investigations point to focal onset
Evidence of medical, social and educational
disability due to the seizures, with the child’s quality
of life likely to improve after surgery
Acceptable risk-benefit ratio for the proposed
surgery
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35. Vagus nerve stimulation
• Pacemaker-like pulse
generator
• Bipolar lead with two
stimulating electrodes
• Battery life of
5-10 years
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36. How does it work?
Mild electrical pulses applied to the left vagus nerve
in the neck for transmission to the brain
Intermittent stimulation
Typically 30 sec on/5 min off
24 hours a day, 7 days a week
Magnetic empowerment
On-demand stimulation
Acute stimulation related side effects control
Simple out-patient programming
(dosing) by treating physician
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37. Ketogenic diet
The ketogenic diet is a high-fat, low-
protein and very low carbohydrate diet that
can be effective in children with drug
resistant epilepsy.
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38. Ketogenic diet
High fat diet designed
to mimic starvation
Production of ‘ketone
bodies’
‘Classical diet’
‘MCT diet’
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39. Non-pharmacological
approaches to seizure control
Behaviour therapy
Avoidance of seizure triggers
Counter measures to provoking situations; e.g.
relaxation strategies for anxiety induced Sz
Life-style focused approaches
- Exercise
- Diet
- Compliance
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40. Status Epilepticus
Generalised convulsive (tonic clonic) status
epilepticus is a life-threatening medical
emergency characterised by:
A generalised convulsion lasting for 30
minutes or longer
or
Repeated tonic-clonic convulsions occurring
over a 30 minute period without recovery of
consciousness between each convulsion.
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41. SUDEP
The sudden unexpected, witnessed or
unwitnessed, non-traumatic, non-drowning
death with or without evidence for seizure.
500 deaths in the UK per year
Post mortem- no structural or toxicological
cause
suspected causes include hypoxia due to a
seizure or cardiac arrhythmia.
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42. Non-epileptic attack disorder (NEAD)
also referred to as psychogenic non-epileptic
seizures (PNES).
Indicators :
If the nature of the events change over time
There are multiple unexplained physical symptoms
There are unusually prolonged events
Refer for neurological assessment if NEAD is
suspected
Information for patient with NEAD can be helpful
for the person suffering these attacks and their
family to take away and reflect on
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43. Comorbidities
Learning disabilities: strong association
ADHD
Obsessive compulsive disorder
Autism
Behaviour difficulties: such as depression,
isolation, anxiety, fear,
The psychological effects
Motor disorders such as dyspraxia,
dystonia, ataxia, 43
44. Epilepsy and education
Some degree of educational problem is present
in 50%
Psychiatric disorders are common and under
diagnosed
Common problems: slow information
processing, attention deficit, memory
impairment, language deficit (comprehension,
word finding), poor motor planning, executive
dysfunction, etc.
Early identification and a multidisciplinary
approach (schools, educational psychologist,
CAMHS)
45. Epilepsy and education
Factors for poor educational progress
Early age of onset
Longer seizure history
Poor seizure control
Involvement of dominant hemisphere
Nocturnal attacks
High level of medications & poly-therapy
Absences from school
Low self-esteem & anxiety
Associated co-morbidities 45
46. Managing epilepsy in
adolescent girls
Effect on oral contraceptive (may need a higher
strength one).
Valproate can cause ovarian cysts.
Some drugs, especially valproate, are antifolate
and may therefore contribute to causing spinal
cord defects in the foetus.
• Several drugs are known to be teratogenic.
Valproate can cause weight gain.
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47. References:
International league against epilepsy: website
www.ilae.org.uk
Epilepsy action: website www.epilepsy.org.uk
National Institute for Clinical Excellence (2004)
The epilepsies diagnosis and management of
the epilepsies in children and young people in
primary and secondary care. London Oaktree
Press LTD
Epilepsy.com website
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48. More From Dr Nivedita Bajaj
https://drniveditabajaj.blogspot.co.uk/
https://drniveditabajaj.tumblr.com/
https://drniveditabajaj.blogspot.co.uk/2018
/03/dr-nivedita-bajaj-mbbs-nivedita.html
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Notes de l'éditeur
One in 200 people have epilepsy
Epilepsy affects 0.7- 0.8% of all school age children
The total number of children with active epilepsy at any point in time is approximately 61,000
Epilepsy is a clinical diagnosis
There is often no apparent reason why a seizure occurs at one time and not another
Some people find that certain triggers make a seizure more likely
These are not the cause of epilepsy, but may trigger a seizure on some occasions
the history is all-important in the differential diagnosis of paroxysmal events and also in the classification of the type of seizure. A number of important
components of the history of the episode must be obtained:
Many conditions mimick a seizure
An adjunctive therapy in reducing the frequency of seizures, who are refractory to AEDs and are not suitable for resective surgery.
Placement on right side induced cardiac arrhythmia. Carers can swipe magnet at the start of a seizure.
It was introduced in 1920 and was stimulated by the observation that fasting improved seizure control.
Behavour t: a seizure is considered part of a sequence of events. Changing the nature of events somewhere in that sequence can reduce the likelyhood of a seizure occuring.
Exercise: encourage to participate in a range of activities, give appropriate risk assessment and supervision. This is to enhance quality of life.
There are estimated to be 500 deaths in the UK per year due to SUDEP
Risk F- early onset epilepsy, specific epilepsy syndromes, high frequency of convulsive seizures, severity of a convulsive sz, polytherapy, subtherapeutic levels of AEDs, compliance, associated learning disability, being found alone in bed and in prone position.