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Figure 5. The EEG from a boy with rolandic epilepsy who developed continuous spike and
wave during slow-wave sleep.

Many children with CSWS have structural brain abnormalities. In others the condition
develops in previously normal children. It is a rare complication of benign childhood epilepsy
with centro-temporal spikes (rolandic epilepsy) and other idiopathic focal epilepsies of
childhood. Carbamazepine has been implicated as precipitating it in some cases. Treatment
is similar to LKS and, as for LKS, the condition appears to eventually remit, but usually
leaving significant neurodevelopmental problems.

Epilepsy with myoclonic absences
Epilepsy with myoclonic absences (EMA) is a rare epilepsy syndrome which can start at any
time in childhood. The hallmark is myoclonic absence seizures. These are similar to typical
absence seizures but with superimposed rhythmic myoclonic jerks mainly of the upper limbs.
The jerks can be mild or severe. GTCS and atonic seizures may also occur. The EEG shows
a normal background with 3 Hz generalised spike and wave discharges. Simultaneous EMG
demonstrates the myoclonic jerks. Marked asymmetries and focal abnormalities may be
apparent. Slowing of the background may occur later.

EMA is now classified as a genetic generalised epilepsy (GGE) by the ILAE but most
children with myoclonic absences have pre-existing neurodevelopmental problems and a
variety of underlying aetiologies, including structural brain defects and chromosomal
abnormalities. Impaired cognitive functioning commonly develops even in those children
who were normal prior to the onset of the seizures. There is some evidence that successful
treatment of the seizures prevents or minimises this. Drugs active against absence seizures in
general should be tried and drugs such as carbamazepine mainly active in focal epilepsies
should be avoided. Relative seizure resistance is the rule.

Some aetiologies associated with severe childhood epilepsies

Some pathological entities are associated with a high likelihood of intractability. These are
not epilepsy syndromes (although some are syndromes as used in the more general sense) but
for completeness will be briefly described here.