Common form of childhood epilepsy. cf juvenile absence epilepsy.
One or more of:
Quite different from childhood absence epilepsy! Less nice.
High risk of serious developmental disorders if exposed in womb (up to 30-40% risk, including 5x higher risk of autism) and congenital malformations (10%), including:
In boys, pre-clinical data on transgenerational risks, and animal studies suggesting infertility.
Oral Valproate must not be started in new patients (male or female) younger than 55 years, unless two specialists independently consider and document that there is no other effective or tolerated treatment, or there are compelling reasons that the reproductive risks do not apply.”
Patient guide and checklist available.
Poorly understood complication of some epilepsies.
Clinically, global regression esp cognitive and behavioural, associated with paroxysmal epileptic activity during sleep, which may of course not be recognized. The classic example is Benign Rolandic Epilepsy, where continuous spike waves develop druing slow wave sleep (CSWS). Also seen in Landau Kleffner syndrome.
The problem can be unmasked by anti-epileptic medication, especially carbamazepine. Appears to be associated with brain pathologies eg polygyria, migrational disorders plus some chromosomal problems eg 8p-.
Diagnosis should be considered when unexpected cognitive impairment (eg memory, temperospatial skills, language) or behaviour changes (eg hyperactivity, aggression, disorientation). Motor impairments eg ataxia and dystonia have been described.
Dramatic increase in EEG abnormalities of any kind during sleep.
Traditional plus newer AEDs have been used, but evidence does not point to any one being superior to any other.
Steroids appear to be effective – unclear whether ACTH or hydrocortisone better.
Poor prognosis (ie long term neuropsychiatric problems) appear to be associated with longer duration of ESES, plus frontal neuropsychological deficits and frontal EEG anomalies.
[Epilepsy Research and Treatment 2012 http://dx.doi.org/10.1155/2012/642725]
Historically, people with epilepsy were “considered to have unique powers, even hailed as geniuses, regarded as having a sacred disease and leading sacred lives”. But then demonisation, persecution, social rejection. “Epileptic personality” described by psychiatry in 20th century. [Sacred lives, Ian Bone]
Self control is central to our self image and the manner in which we and society believe we should behave. Epilepsy jeopardises this. Patients often conceal. Guilt, loss of confidence and low mood common after seizures.
Definition: At least 2 unprovoked (or reflex) seizures, occurring more than 24hrs apart; else one unprovoked (or reflex) seizure and probability of further seizures similar to that seen in those who have had 2 unprovoked seizures (ie at least 60%); or recognized epilepsy syndrome. Also part of the definition is that epilepsy is considered “resolved” if age dependent syndrome and past applicable age, or else those who have been seizure free for 10 years and off medication for 5 years. (ILAE 2014)
Note that “seizure” does not have any real medical meaning! Transient signs/symptoms due to excessive or synchronous neuronal activity in brain (ILAE 2017) – but implies you can tell whether caused by abnormal brain activity, which can be hard!
First assessment:
Despite increasing sophistication, interpreting EEG remains an inexact science! Irregular background activity overlaps with detectable abnormality. Plus, only really picks up activity at surface of brain, and can miss simple partial seizures (but not tonic-clonic generalised). Review in 2000 did not find much evidence base. Requires dialogue between referrer and neurophysiologist. Diagnosis remains principally clinical – eg more than 1 tonic-clonic seizure, or multiple absences! Incidence of epileptiform activity in asymptomatic individuals appears to be about 1%, of which a few percent will develop epilepsy over the subsequent years. Abnormal activity is certainly more likely if structural abnormality, but still many will be and remain asymptomatic.<
50% of children with epilepsy have normal EEGs – so not a particularly useful test! Not only that, but in studies, at least 20% of “epileptics” were ultimately given a non-epileptic diagnosis! So request EEG with caution; should be used for confirming clinical opinion, or to guide treatment, not where symptoms are vague. Hence a firm diagnosis of epilepsy and then decisions about treatment may take some time; difficult for families to understand, but it is quite safe to be cautious esp considering the implications of a mistaken diagnosis.
If EEG is negative, then proceed to a sleep EEG (where child is woken by parents at 3am, kept awake then brought to department and allowed to fall asleep during monitoring). This has 80% sensitivity. Failing that, Medilog or Video with continous monitoring. This is good for distinguishing non-epileptic tonic-clonic seizures, but does not rule out co-existing epilepsy.
Good for:
Beware – an EEG finding of partial epilepsy may not have a surgical lesion! Must be used in conjunction with MRI. A generalized epilepsy may have multiple foci, so if you are unlucky to capture just one you will be misled. Similarly, if secondary generalization occurs rapidly, its partial nature may be missed.
Not usually useful to do after multiple seizures, unless type or frequency changes significantly, in which case a new syndrome/prognosis may have developed.</p>
<p><em>”The brain is subject to maturation; there are multiple protecting and triggering factors, often unpredictable. Seizures may be rare and easy to treat for months and years, but may become more frequent and difficult to control later on. But in many children a precise syndromic diagnosis can be made, and a good final prognosis can be expected in most cases.”</em></p>
All children with recurrent seizures should have an ECG with calculated QTc. Children under 2 with epilepsy or with recurrent focal seizures (other than CECTS) should have an elective MRI brain scan. In most other cases the course is predictable. A normal MRI does not rule out a small dysplastic lesion, equally the finding of a lesion does not mean that it is the cause of the epilepsy. However, at least you can exclude a tumour or malformation.</p>
Focal/partial often have acquired or congenital lesions, often specific precipitating factors eg sleep, startle!</p>
<p>Consider:</p>
Should not be started after first tonic-clonic generalized seizure. Try not to start before EEG done as may mask features.
When to start? Complex – consider:
The risk of accidents due to a seizure is marginal, probably because seizures mainly occur during times of reduced activity (eg somnolence, sleep, meals) rather than during intense physical or mental activity. Risk assessment should be done and documented including bathing, showering, swimming. NICE only specifically mentions with reference to epilepsy and learning disability. SIGN states that normal activities should be encouraged, but supervision requirements should be individualized taking into account type of activity and seizure activity.
“There is an old and unjustified prejudice that brain damage may result from seizures: this does not happen in the great majority, and usually only occurs in unpreventable and very special situations.” (cf severe migraine, frequent syncope).
For generalized, lamotrigine or levetiracetam. Not Valproate for girls (risk of congenital malformations) or boys (evidence of effect on fertility) unless nothing else works
Others:
For partial, use carbamazepine (CBZ) or lamotrigine. Else:
If 6 months therapy with 2 different but appropriate drugs does not control (at adequate doses), then refer to tertiary centre for re-think on diagnosis.
Routine drug level monitoring is not required.
Consider withdrawing treatment when seizure free for 2+ years. But treatment may also be aimed at reducing cognitive effects, not just seizures: evidence for this comes from surgically treated epilepsies where rapid cognitive-behavioural improvement can be seen; and the fact that behavioural problems are more common in the months leading up to the first fit before diagnosis. For example:
More efficient use of known drugs (doses, slow release preparations), side effects of drugs, and new therapeutic options (treatment of acute seizures with nasal or buccal midazolam (EPISTAT) by parents at home, or in schools) can make a big difference to families.
Temporal lobe epilepsies, most often due to mesial temporal sclerosis (and often refractory to medical therapy) can be treated surgically with excellent prognosis. An increasing number of intractable focal epilepsies can now be treated surgically.
Archives of Disease in Childhood 2005;90:5-10, T Deonna
SIGN guideline 81
Refer to tertiary if –
SPEN network has pathways for first seizure, new diagnosis, continuing seizures.
See also Living with Epilepsy