Lecture 6 - Epilepsy Paeds Flashcards
Broadly how does epilepsy have such a big impact on children who have it.
Children with epilepsy most commonly have slowed cognitive progression which can impact daily life (in the home, school, and socially) resulting in widespread psychological and cognitive impact.
Does epilepsy make a child less smart?
Cognitively epilepsy has been associated with a downward trend in IQ. Where the normal range is 80-115+, the mean IQ for children with epilepsy has often been found to fall within the lower end of this ‘normal’ range (85-90).
However, IQ is a gross and insensitive measure, it does not necessarily offer a complete picture of the difficulties faced by children with epilepsy. There are commonly subtle deficits (E.g., memory, executive, language, processing speed and behavioural symptoms) that result either DIRECTLY from the seizure activity or INDIRECTLY from the impact of epilepsy on the child’s home, school, and social functioning/life.
In many forms of epilepsy, there is slowed cognitive progress, that is rather then a decline in previously acquired abilities, though this can also occur particularly in epileptic encephalopathies.
It is also important to note that a ‘mean IQ’ does not represent everyone equally. Children are likely to have greater intellectual impairment when:
- Seizures have an early onsets, and the disorder is ongoing.
- Frequent seizures.
- Requires polytherapy
- Treatment resistant seizures (intractable), particularly if they are generalised.
- Status epilepticus/non-convulsive state
What is the difference between a decline and delay of cognitive development? Define both.
b
In epilepsy, are declines or delays in development more common?
b
Name the types of epilepsy associated with DECLINE (not just delay) in development/cognitive abilities.
Encephalitic encephalopathies (e.g., west syndrome and lennox-gastaut)
What is the lifetime prevalence of having a seizure? and during what years of life are they more likely to occur?
5%. Infants
Approx how many people in 1000 have epilepsy?
3-8 per 1000
Why might infants have a particular vulnerability to seizures?
Immature brain - GADA theory or secondary to underlying abnormality: - encephalopathy -haemorrhage -metabolic disorders - congenital cerebral malformation - neuronal migration disease (cortical dysplasia)
What neuropathological finding characterises Tuberous Sclerosis?
calcified lesions (‘tubers’) in the brain, often also have lesions on the skin. Sometimes tubers are static, other time new ones grow. May have a genetic component.
What is polymicrogyria?
Hyper-folding of the gyri
What is the definition of a ‘seizure’?
(ILAE and IBE) definition:
An epileptic seizure is a transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain.
Or SIMPLIFIED:
A period of abnormal electrical activity in the brain, usually with associated clinical symptoms.
What is the current (2014) operational definition of epilepsy? (ILAE)
Epilepsy is a disease of the human brain defined by any one of the following:
- At least 2 unprovoked seizures occurring >24h apart;
- One unprovoked seizure and probability of further future seizure (at least 60% risk) occurring in the next 10 years.
- Diagnosis of an epilepsy syndrome.
What is an ictal event?
Seizure event, an active seizure
What is another name of a seizure event?
Ictal event
What does ‘interictal’ mean?
Period between seizure events
What is the name for the period between seizures?
Interictal
What does ‘post-ictal’ mean?
Period immediately following a seizure event
What is the name for the period immediately following a seizure event
Post-ictal
What is an Aura?
A subjective phenomenon just prior to, or simultaneous with , the start of a seizure that can act as a warning.
These experiences rarely last more than a few seconds, but the nature of the Aura can be diagnostically relevant.
For example, Nausea/epigastric aura can indicate a temporal lobe epilepsy.
What are the three TIERS of epilepsy classification?
TIER 1: Seizure type (generalised, localised, undetermined)
TIER 2: Seizure aetiology (idiopathic, symptomatic, cryptogenic)
TIER 3: Syndrome (specific epilepsy syndromes, a cluster of signs & symptoms)
What are the 2 MAIN categories of type of seizure (TIER 1)?
- Partial seizure (AKA focal or local seizures, affecting only part of the brain)
- Generalised seizures (where clinical and EEG data indicate aberrant activity in both cerebral hemispheres simultaneously at onset.
Describe what a ‘generalised’ seizure is and outline the types of generalised seizures.
Generalised seizures are where clinical and EEG data indicate aberrant activity in both cerebral hemispheres simultaneously at onset.
TYPES:
Absence seizures ("petit mal") - characterised by EEG activity with minimal or no outward signs. Child will look phased out, for a brief period (1-2 --30 seconds), may have mild motor signs (e.g., humming or eye-deviation)
Tonic-Clonic seizures (“grand mal”) - The ‘typical’ seizure, combinations of muscle (tonic) tensing and jerking (clonic) movements.
Myoclonic seizures - Brief shock like twitching , which can be restricted to specific muscle groups (E.g., eye lid)
Clonic seizures - Small jerking movements (Without tonic rigidity)
Tonic seizures - Tensing/flexing, rigidity, increased tone of muscle
Atonic Seizures - Bodies muscles relax/go floppy, can occur in only some body parts, or everywhere as in a ‘drop attack’ (drop attacks may also occur in tonic)
Describe absent seizures
Absence seizures (“petit mal”) - characterised by EEG activity with minimal or no outward signs. EEG of 3hz with spikes-waves pattern. Child will look phased out, for a brief period (1-2 –30 seconds), may have mild motor signs (e.g., humming or eye-deviation)
What is ‘status epilepticus?’
A medical emergency, recurrent tonic-clonic without recovery of consciousness between attacks. EEG will show continuous, abnormal activity.
Most commonly occurs in severe epilepsy where there is poor medication adherence and/or epilepsy of encephalitic origins.
Repeated attacks of status epilepticus can affect cognition (highly damaging to brain). high morbidity.
