Epilepsy Flashcards
What is a seizure?
A seizure is the transient occurrence of signs or symptoms due to abnormal excessive or synchronous neuronal activity in the brain
Seizures can manifest as a disturbance of consciousness, behaviour, cognition, emotion, motor function, or sensation
An isolated seizure can be caused by toxic, metabolic, structural, and infectious factors and should not be confused with epilepsy
What is epilepsy?
Epilepsy is a common neurological disorder in childhood. Seizures and epilepsy affect infants and children more than any other age group.
Epilepsy is a neurological disorder in which a person experiences recurring seizures
The International League Against Epilepsy describes epilepsy as a disease of the brain defined by any of the following conditions:
o At least two unprovoked seizures occurring more than 24 hours apart.
o One unprovoked seizure and a probability of further seizures similar to the general recurrence risk (at least 60%) after two unprovoked seizures, occurring over the next 10 years.
o Diagnosis of an epilepsy syndrome.
What is the aetiology of epilepsy?
A cause of epilepsy is only identified in about one third of people with the disorder:
o Structural — abnormalities visible on structural neuroimaging, for example stroke, trauma, or malformation of cortical development. The underlying basis for a structural abnormality can be genetic or acquired.
o Genetic — epilepsy resulting from a known or presumed genetic mutation (not necessarily inherited) in which seizures are a core symptom of the disorder, for example Dravet syndrome.
o Infectious — epilepsy results from a known infection in which seizures are a core symptom of the disorder (rather than seizures due to acute infection such as meningitis). Examples include tuberculosis, cerebral malaria, HIV, and congenital infection such as Zika virus.
o Metabolic — epilepsy results from a known or presumed metabolic disorder in which seizures are a core symptom of the disorder (which may occur as a result of a genetic defect). Examples include porphyria, amino-acidopathies, or pyridoxine deficiency.
o Immune — epilepsy that results directly from an immune disorder (where there is evidence of auto-immune mediated central nervous system inflammation) in which seizures are a core symptom of the disorder. Examples include anti-NMDA receptor encephalitis and anti-LG11 encephalitis.
What is the presentation of typical absences?
Typical absences (‘petit mal’ seizures): petit mal epilepsy is manifest by frequent (as many as 100 times per day or more) episodes of brief staring spells (lasting seconds at a time):
o Onset in childhood; attacks continuing into adult life are rare.
o A typical absence attack lasts only a few seconds. The onset and termination are abrupt. The child ceases what he or she is doing, stares, looks a little pale and may flutter the eyelids.
o Sometimes more extensive bodily movements occur (such as dropping the head forwards) and there may be a few clonic movements of the arms.
onThe interruption of the normal stream of consciousness is very brief and the child may be unaware of the attacks, as indeed may be the parents for some time after onset, assuming that the child is just day-dreaming.
o About one third of all children with petit mal will have one or more tonic-clonic convulsions.
What is infantile spasm?
This is West syndrome.
The typical movement in a spasm is of a sudden flexion (bending forward) in a tonic (stiffening) fashion of the body, arms and legs.
Sometimes, the episodes are different, with the arms and legs being flung outwards (these are called ‘extensor’ spasms). Sometimes the spasms may involve a flexor movement followed one or two seconds later by an extensor movement. Usually, they affect both sides of the body equally.
Typically, each episode lasts just 1 or 2 seconds after which there is a pause for between five and ten seconds which is then followed by a further spasm.
Occur in infants aged 4-8 months.
Consist of clusters of myoclonic spasms that occur when waking up.
What is Dravet’s syndrome?
Dravet’s syndrome includes severe myoclonic epilepsy of infancy with the onset of recurrent febrile and/or afebrile hemiclonic or generalised seizures, or status epilepticus, in a previously healthy infant.
Multiple seizure types develop which are generally resistant to AEDs. There is also developmental arrest or regression.
Onset up to 15 months of age may occur.
Mortality may be up to 15% by age 20 years.
What is juvenile myoclonic epilepsy?
o Occurs in the teen years.
o Early morning sudden myoclonic jerks, especially of the arms and shoulders.
o Often later develop generalised tonic-clonic (GTC) seizures.
o May be inherited as autosomal dominant.
What is Panayiotopoulos syndrome?
Panayiotopoulos syndrome is a common multifocal autonomic childhood epileptic disorder.
Affects otherwise normal children with onset at around 3-6 years.
Seizures are often prolonged, with predominantly autonomic symptoms and mainly ictal vomiting.
Electroencephalogram (EEG) shows shifting and/or multiple foci, often with occipital dominance.
Often confused with occipital epilepsy and acute non-epileptic disorders – e.g., encephalitis, syncope, cyclical vomiting or atypical migraine.
What is benign Rolandic epilepsy?
Also known as benign focal epilepsy.
It occurs in children aged 4-10 years and is more common in boys.
Nocturnal seizures that are characterised by facial twitching and aphasia.
Some children with benign Rolandic epilepsy may also have GTC seizures.
What is benign childhood epilepsy?
Some older children may have focal or generalised seizures.
The interictal EEG is characterised by large spike discharges over the Rolandic area of one hemisphere.
Is not associated with any structural lesion and has an excellent prognosis.
What are the differentials of epilepsy in children?
• Syncope
• Night terrors:
o These affect children aged between about 6 and 8 years, who suddenly awaken from a sound sleep, wide-eyed, screaming, and inconsolable.
o They are amnesic for the events the following morning. They seem to occur just as often in happy children as in children who are not doing well at school or in the family. Fortunately, they too pass quickly.
• Reflexic anoxic seizures:
o Affect younger children, aged between 1 and 2 years. A typical story is of a child who has some minor injury, or who is crossed in some way so that he or she becomes suddenly angry, upset or frightened. Such attacks terminate spontaneously without treatment.
- Febrile convulsions
- Fabricated or induced illness by carers
- Cardiac arrhythmias
- Migraine
- Sleep apnoea
- Narcolepsy
What are the investigations for epilepsy in children?
• EEG:
o Performed where seizure is likely to be epileptic in origin.
o Should not be used in isolation to make a diagnosis
o Performed after second seizure
o Photic stimulation and hyperventilation should remain part of standard EEE assessment and the child should be warned that these may induce a seizure.
- Neuroimaging such as MRI (first-line) to identify structural abnormalities.
- Blood and urine biochemistry
- 12-lead ECG
What is the management of epilepsy in children?
Essential information on how to recognise a seizure, reducing the risk of severe trauma during a seizure, appropriate first aid measures and the importance of reporting further attacks should be provided to a child or young person who has experienced a possible first seizure and to their family/carer/parent as appropriate.
This information should be provided while awaiting a diagnosis
Diagnosis in children should be established by a paeditrician.
Drug treatment:
o Use monotherapy whenever possible
o Treatment is associated with a small risk of suicidal thoughts and behaviour.
o Therapy initiated by a specialist.
o Treatment with AED therapy is generally recommended after a second epileptic seizure.
When should AED treatment be considered after a first seizure?
AED therapy should be considered and discussed after a first unprovoked seizure if:
- The child or young person has a neurological deficit.
- The EEG shows unequivocal epileptic activity.
- The child or young person and/or their family and/or carers consider the risk of having a further seizure unacceptable.
- Brain imaging shows a structural abnormality.
How can adherence to AED be optimised in children with epilepsy?
Educating children, young people and their families and/or carers in the understanding of their condition and the rationale of treatment.
Reducing the stigma associated with the condition.
Using simple medication regimens.
Positive relationships between healthcare professionals, the child or young person and their family and/or carers.
