Page 104 - ILAE_Lectures_2015

(b) an undetermined but probably small proportion of patients with any type of BCSSS that
may also suffer typical generalized convulsive or absence seizures either during the active phase
of BCSSS or more often at a later stage

(c) an undetermined but probably small proportion of patients with syndromes of IGE including
childhood absence epilepsy that may also have focal spikes or typical seizures of BCSSS38,62,84.

Management of benign childhood focal seizures

Short- and long-term treatment strategies of benign childhood focal seizures are empirical. In
the acute stage, control of the seizure is paramount. On the rare occasions that the child is
febrile, treatment of the underlying illness is also important. Long-lasting convulsive seizures
(>10 minutes) or convulsive status epilepticus (>30 minutes), although rare, constitute a
genuine paediatric emergency that demands appropriate and vigorous treatment – as for
prolonged febrile seizures and febrile status epilepticus. Benzodiazepines, in intravenous, rectal
or buccal preparations, are used to terminate status epilepticus. Early parental intervention is
more effective than late emergency treatment. Autonomic status epilepticus needs thorough
evaluation for proper diagnosis and assessment of the neurological/autonomic state of the child.
Aggressive treatment should be avoided because of the risk of iatrogenic complications83.

Continuous antiepileptic medication is not usually recommended. Although there are effective
therapies that could prevent the occurrence of additional seizures, potential adverse effects may
not commensurate with the benefit. The risks of recurrent seizures are small, the potential side
effects of drugs appear to outweigh the benefits and there is no convincing evidence that any
therapy will alleviate the possibility of recurrences.

Decisions on management must take into account the following:

(a) Most children have excellent prognosis: 10–30% may have only a single seizure and 60–
70% may have less than 10 in total. However, 10–20% of children may have frequent seizures,
which are sometimes resistant to treatment.

(b) Remission of benign childhood focal seizures is expected in all patients by the age of 15–
16 years at the latest.

(c) There is no evidence that the long-term prognosis is worse in untreated children, although
they may not be protected against seizure recurrences.

(d) Some children become frightened, even by simple focal seizures, and some parents are
unable to cope with the possibility of another fit despite firm reassurances.

(e) Persistence and frequency of EEG functional spikes do not predict clinical severity,
frequency or degree of liability to seizures

(f) In contrast to the other phenotypes of BCSSS, patients with ICOE-G often suffer from
frequent seizures and therefore prophylactic AED treatment may be mandatory.

Secondarily GTCS are probably unavoidable without medication. Continuous prophylaxis
consists of daily monotherapy using any AED that has proven efficacy in focal seizures. Most
authorities recommend carbamazepine though this drug may exaggerate seizures in a minority
of children with BCSSS including PS119. Recently, sulthiame has been revived as an excellent
drug for the treatment of benign childhood epilepsy with centrotemporal spikes with EEG
normalisation189,190 but this may be associated with cognitive abnormalities191. Of the newer