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First-line treatments for West syndrome are ACTH tetracosactide, prednisolone and
vigabatrin20,21. ACTH/prednisolone is more effective than vigabatrin in controlling spasms
early on (at 14 days), but is similar in efficacy in long-term follow-up (12–14 months)22.
Vigabatrin appears to be particularly effective for infants who have West syndrome caused
by tuberous sclerosis. Vigabatrin appears to be safer than ACTH/prednisolone, with much
less serious and toxic side effects (see also Chapter 30), although the reported visual field
constriction in association with at least six months’ (and usually longer) exposure to this drug
is clearly of concern; the incidence of the visual field defect in children is unclear but is
considered to be approximately 2025%, although these data are predominantly based on
older children who received the drug for treating partial seizures and not in infants who were
treated for infantile spasms. A study evaluating the use of combination treatment with
ACTH/prednisolone and vigabatrin compared to ACTH/prednisolone alone (the ICISS –
International Collaborative Infantile Spasms Study) has just completed recruitment and early
reports are of improved early seizure control on combination treatment, but these are yet
unpublished. Other treatments that are of benefit include nitrazepam (which in a historic
comparator trial has been shown to have similar efficacy to ACTH, but fewer side effects23),
sodium valproate, topiramate, levetiracetam, zonisamide and pyridoxine. The ketogenic diet
should be considered in infants with West syndrome which is resistant to medication.
Children who have focal structural abnormalities and drug-resistant spasms should be
referred to an epilepsy surgery centre to consider whether they are suitable candidates for
resective epilepsy surgery.

Febrile convulsions (seizures)24

Defined as ‘convulsions with fever in children aged between six months and five years
without evidence of serious acute symptomatic brain disease (e.g. meningitis, encephalitis)’
(see also Chapter 8).

Although by definition children as young as six months of age may have febrile seizures, the
author would not accept the diagnosis in infants less than one year of age, and would consider
the following diagnoses first, and undertake the appropriate investigations:
 Meningitis/encephalitis
 Metabolic disorder
 Malformations of cortical development.

Clearly the number and type of investigations undertaken would depend upon the age of the
infant and whether the febrile seizure was ‘simple’ or ‘complex’ (complex means a seizure
which is focal, serial, longer than l5 minutes, or followed by a neurological deficit). For
example, a complex febrile seizure in a six-month-old infant should at least raise the
possibility that the child may be developing severe myoclonic epilepsy in infancy (SMEI,
described above) and would justify at least the exclusion of meningitis by CSF analysis and
urine culture. It would also merit neuroimaging (preferably MRI) to exclude or demonstrate
a structural lesion (including malformations of cortical development). If these investigations
are negative it would be appropriate to screen for SCN1A mutations. A simple febrile seizure
in a one-year-old with no obvious focus of infection would justify CSF and urine analysis
however. A simple febrile seizure in a two or three-year-old child with otitis media probably
needs no investigation and specifically there is no indication for undertaking an EEG in this
situation.

Frequently a complicated febrile seizure may actually represent a first epileptic seizure that
has been provoked by an intercurrent infection. Over the past few years, there has been the
identification of a ‘syndrome’ of generalised epilepsy and febrile seizures plus (GEFS+)
which may present with febrile seizures in the first two years of life. The word, ‘plus’ in this
syndrome refers both to the fact that febrile seizures may still occur after the age of five years