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patients with drug-resistant focal seizures, primary generalised tonic-clonic seizures, refractory
myoclonic and absence seizures and even refractory infantile spasms44,45. Its mechanism of
action, and therefore its reported adverse side effects, appears to be similar, but less severe, to
that of topiramate. Currently, in the UK, the drug only has a licence as an add-on treatment for
treating focal seizures in patients aged 18 years and above; however, it is expected to receive
a paediatric licence as adjunctive therapy within the near future (2013/14).

Rufinamide is licensed in the UK for treatment of seizures associated with Lennox-Gastaut
syndrome. A randomised double-blind placebo-controlled trial of 139 participants aged 430
years showed significant benefit in most seizure types, particularly atonic (‘drop’) and absence
seizures46.

Many other drugs have been used in paediatric epilepsy, usually in an attempt to control
multiple and refractory seizure types. Acetazolamide, a diuretic and carbonic anhydrase
inhibitor, is considered by many to be a useful add-on drug (usually in combination with
carbamazepine) in treating focal seizures47. Pyridoxine (vitamin B6) is clearly the treatment of
choice in the rare inherited disorder of pyridoxine-dependent seizures48, but it has also been
used in West syndrome (infantile spasms)49. A three-week trial of oral pyridoxine should also
be used in any child under 18 months of age with frequent seizures (including infantile spasms)
that have been resistant to ‘conventional’ AEDs. If there has been no obvious or sustained
response to pyridoxine, and there remains a high suspicion of pyridoxine-dependent epilepsy,
the child should then receive a three- or four-week course of pyridoxal phosphate. Biotin
should also be used in infants and young children with refractory seizures pending the result
of a serum biotinidase level. Folinic acid should also be used for any infant with neonatal-onset
seizures that have been resistant to both conventional antiepileptic medication and pyridoxine
and where no cause has been found for the epilepsy.

The high-fat, low-carbohydrate ketogenic diet is a historical treatment that has gained more
credibility as an effective management of children with drug resistant epilepsy50. A randomised
controlled trial has demonstrated definitive efficacy over no change in treatment. More relaxed
forms of the diet have raised the possibility of it being available to use over a wide age range.

Steroids, given either as prednisolone or less commonly hydrocortisone or
adrenocorticotrophic hormone (ACTH)51 (this drug is no longer available in the UK and has
been replaced by the synthetic steroid, tetracosactide, see Chapter 23), are frequently used in
treating different seizure types or epilepsy syndromes, including acute epileptic
encephalopathies. The mechanism of action of steroids is unclear but they may be very
effective, particularly in the following situations:

 infantile spasms (West syndrome)
 continuous spike-wave in slow sleep (CSWSS), also called electrical status epilepticus of

slow sleep (ESESS)
 Dravet syndrome
 Lennox-Gastaut syndrome, particularly in non-convulsive (atypical absence)) status
 other, rare epileptic encephalopathies.

Intravenous immunoglobulins have been used with varying (usually very limited), success in
intractable epilepsies including children with both the West,52,53 and Lennox-Gastaut
syndromes54,55. There are marked variations in the frequency of courses, duration of treatment