Australian Doctor 12th July Issue 2024 | Page 31

HOW TO TREAT 31
ausdoc . com . au 12 JULY 2024

HOW TO TREAT 31

Aware
Focal onset Generalised onset Unknown onset
Motor onset
Automatisms Atonic Clonic Epileptic spasms Hyperkinetic Myoclonic Tonic
Impaired awareness
Non-motor onset
Autonomic Behaviour arrest Cognitive Emotional Sensory
Motor
Tonic-clonic Clonic Tonic Myoclonic Myoclonic tonic-clonic Myoclonic atonic Atonic Epileptic spasms
Non-motor ( absence )
Typical Atypical Myoclonic Eyelid myoclonia
Motor
Tonic-clonic Epileptic spasms
Non-motor
Behaviour arrest
Unclassified
Source : Fisher RS et al 2017 8
Focal to bilateral tonic-clonic
Figure 4 . International League Against Epilepsy Classification of seizure types , 2017 .
Figure 6 . MRI machine ( A ) and MRI head coil with mirror ( B ) to watch movies .
Figure 8 . EEG demonstrating generalised ( A ) and focal ( B ) pattern .
importance of considering the aeti-
cognitive comorbidities , but the
ology to optimise management and
demand for these services can result
prognosis . 7
in long waiting times before the
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-
assessment is undertaken .
CHILDHOOD EPILEPSY SYNDROMES
Infantile spasms syndrome
INFANTILE spasms syndrome is a
zures that rapidly engage a widespread
severe epilepsy syndrome of infancy
bilateral network . 26
associated with developmental pla-
Focal-onset seizures are further
teauing and regression . It has a diverse
classified into ‘ aware ’ ( previously
range of causes .
‘ simple partial seizures ’) or ‘ impaired
The classic triad is epileptic
awareness ’ ( previously ‘ complex partial
spasms , developmental plateauing /
seizures ’). Both focal-onset and gen-
regression and hypsarrhythmia ( cha-
Figure 5 . EEG .
B
eralised-onset seizures can be further described , as listed in figure 4 .
Individuals with generalised epilep-
otic , high-amplitude , epileptic EEG pattern ) on EEG , but infants may not have all three features . 28
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-
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-
uals with combined generalised and
ment may be more subtle , such as
focal epilepsy have both generalised
head nods .
and focal seizures and EEG features .
Early recognition and urgent refer-
An epilepsy syndrome refers to a
ral to a paediatric neurology service for
type of epilepsy with characteristic
prompt investigation and management
clinical features occurring together ,
can optimise developmental outcomes .
and incorporates the age of epilepsy onset and remission , seizure types , EEG features , imaging features , comor-
Dravet syndrome
Dravet syndrome , also known as severe
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
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
sleep problems . 7
with a prolonged hemiclonic febrile
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
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 .