importance of considering the aeti- |
cognitive comorbidities , but the |
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ology to optimise management and |
demand for these services can result |
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prognosis . 7 |
in long waiting times before the |
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A |
The multi-level classification system is summarised in figure 7 .
Seizure type is classified by onset into focal , generalised or unknown .
Focal-onset seizures originate in one area or one hemisphere of the brain , compared with generalised sei-
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assessment is undertaken .
CHILDHOOD EPILEPSY SYNDROMES
Infantile spasms syndrome
INFANTILE spasms syndrome is a
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zures that rapidly engage a widespread |
severe epilepsy syndrome of infancy |
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bilateral network . 26 |
associated with developmental pla- |
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Focal-onset seizures are further |
teauing and regression . It has a diverse |
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classified into ‘ aware ’ ( previously |
range of causes . |
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‘ simple partial seizures ’) or ‘ impaired |
The classic triad is epileptic |
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awareness ’ ( previously ‘ complex partial |
spasms , developmental plateauing / |
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seizures ’). Both focal-onset and gen- |
regression and hypsarrhythmia ( cha- |
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Figure 5 . EEG . |
B |
eralised-onset seizures can be further described , as listed in figure 4 .
Individuals with generalised epilep-
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otic , high-amplitude , epileptic EEG pattern ) on EEG , but infants may not have all three features . 28 |
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sies have an EEG demonstrating gen- |
Epileptic spasms are brief contrac- |
Source : Scheffer IE et al 7 |
eralised spike – wave activity and may have a range of different generalised seizure types ( see figure 8 ).
Those with focal epilepsies have an EEG demonstrating focal discharges and have focal-onset seizures . Individ-
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tions of the trunk muscles lasting less than three seconds and resulting in truncal flexion , extension or a mixed appearance . 29 Spasms tend to cluster and typically occur as the infant wakes or drifts off to sleep . The move- |
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uals with combined generalised and |
ment may be more subtle , such as |
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focal epilepsy have both generalised |
head nods . |
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and focal seizures and EEG features . |
Early recognition and urgent refer- |
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An epilepsy syndrome refers to a |
ral to a paediatric neurology service for |
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type of epilepsy with characteristic |
prompt investigation and management |
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clinical features occurring together , |
can optimise developmental outcomes . |
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and incorporates the age of epilepsy onset and remission , seizure types , EEG features , imaging features , comor- |
Dravet syndrome
Dravet syndrome , also known as severe
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Figure 7 . Framework for classification of the epilepsies . International League Against Epilepsy , 2017 . |
bidities and response to medications . Several common childhood epilepsy syndromes are described .
Comorbidities of epilepsy
Children with epilepsy may have learning
, psychological , behavioural , movement , orthopaedic , gastrointestinal and
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myoclonic epilepsy of infancy , is an early-onset , medically refractory epilepsy syndrome , that requires management by a paediatric neurologist .
A pathogenic variant in the SCN1A gene is identified in more than 85 % of patients . 30
The typical presentation is an infant
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sleep problems . 7 |
with a prolonged hemiclonic febrile |
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demonstrable EEG abnormalities and |
include a waxing and waning pat- |
are doubts about the diagnosis . 20 |
There is a higher prevalence of |
seizure , sometimes after vaccination . |
are a result of psychiatric dysfunc- |
tern of movements , sideways head |
Accurate diagnosis is essential to |
psychological disorders , learning dis- |
These infants continue to experience |
tion . They most commonly occur in older adolescent females . 20 Psychogenic stressors may or may not be identifiable . |
shaking , thrusting movements , eyelid closure with resistance to passive eye opening , and rapid recovery after a prolonged event . 22 |
avoid inappropriate interventions .
CLASSIFICATION
IN 2017 , the International League
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abilities and neurodevelopmental disorders ( including ADHD and autism spectrum disorder ) in children with epilepsy . 27 |
recurrent febrile seizures — often with mild fevers or even in response to hot baths or showers . Developmental delay becomes apparent after the age of one . |
Features that help differentiate |
Recording an event on EEG is the |
Against Epilepsy updated its classi- |
Ideally , neuropsychological test- |
Myoclonic and afebrile seizures tend to |
these events from epileptic seizures |
definitive diagnostic test when there |
fication of epilepsies to highlight the |
ing should be performed to identify |
occur at this stage . |