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Lennox – Gastaut syndrome
Lennox – Gastaut syndrome ( LGS ) is
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Source : Specchio N et al 2022 32 |
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a medically refractory epilepsy with |
childhood ; they generally have a good |
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onset before age 18 . |
prognosis and overlapping clinical and |
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It is characterised by multiple |
EEG features ( see table 2 ). Idiopathic |
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drug-resistant seizure types ( of which |
generalised epilepsies ( IGEs ) account |
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one must be tonic seizures ), develop- |
for 15-20 % of people with epilepsy . 33 |
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mental delay and typical EEG features |
Development and intellect are typ- |
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of diffuse slow spike-and-wave and |
ically normal , but there is a higher rate |
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generalised paroxysmal fast activity . 32 |
of comorbid mood disorders , ADHD |
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There are many different causes of |
and learning disabilities compared |
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LGS . Individuals with this diagnosis |
with the general population . |
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must be managed in conjunction with a paediatric neurologist because of the refractory nature of the condition . |
MANAGEMENT
REFER children and adolescents to a
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Self-limited focal epilepsies of childhood
Self-limited focal epilepsies ( SeLFEs )
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paediatrician or paediatric neurologist after a first suspected epileptic seizure . Refer more urgently if there is a deterioration in their behaviour , cog- |
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are responsible for 25 % of epilepsies in |
nition or development ; or if infantile |
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childhood . 32 |
spasms are suspected . |
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These focal epilepsies have an onset in childhood , with a characteristic age-dependent clinical pres- |
Seizure safety
After a seizure , provide the patient and
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Figure 9 . Typical absence seizure with generalised 3Hz spike – wave . |
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entation and EEG pattern , and normal examination ( see table 1 ).
It is presumed that genetic factors play a role in the aetiology , although a single gene cause is rarely found .
Not all patients diagnosed with a SeLFE will require ASM as seizures are often infrequent . If frequent seizures occur , they typically respond well to carbamazepine .
Some children may have overlapping clinical features of different SeLFEs or evolve from one syndrome to another .
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their family with seizure safety and first-aid education .
Individualise safety advice to each patient , noting that certain activities carry greater risk than others . Regardless , all children and adolescents with epilepsy should be able to live full and active lives .
It is a particularly distressing experience for a parent to witness their child having a seizure . Paediatric Epilepsy Network NSW has a seizure first-aid poster ( see figure 10 ) that explains how a witness should manage a person hav-
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Source : The Sydney Children ’ s Hospital Network 34 |
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Idiopathic generalised epilepsies
Idiopathic generalised epilepsies are
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ing a generalised tonic-clonic seizure or a focal seizure without awareness . 34
Each child with epilepsy needs a seizure management plan to provide to
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a group of epilepsies with onset in |
carers , daycare or school . This should |
Childhood absence epilepsy |
4-10 years |
Remits by early adolescence in 60 % Remainder evolve to another idiopathic generalised epilepsy |
Typical absence seizures , occurring daily to multiple times a day Bilateral tonic – clonic seizures may occur |
3Hz generalised spike – wave discharges ( see figure 9 ) provoked with hyperventilation |
Juvenile absence epilepsy |
9-13 years Often drug responsive but may require lifelong therapy |
Absence seizures occurring less than daily Bilateral tonic – clonic seizures in more than 90 % |
3-4Hz generalised spike – wave discharges Photoparoxysmal response * seen in 25 % |
Juvenile myoclonic epilepsy |
10-24 years |
Lifelong disorder requiring lifelong therapy |
Myoclonic seizures ( all ) Bilateral tonic – clonic seizures (> 90 %) Absence seizures ( onethird ) |
3-6Hz generalised spike – wave or polyspike – wave discharges Photoparoxysmal response * in more than 30 % |