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In most children with Doose syndrome the first seizures are febrile or afebrile GTCS. These
are then followed by the characteristic seizure, the so-called myo-atonic seizure which
combines a symmetrical myoclonic jerk immediately followed by an atonic seizure, usually
causing a drop attack. Children with Doose syndrome may also have independent atonic and
myoclonic seizures and brief typical absence seizures. Episodes of non-convulsive status
epilepticus lasting hours or days occur in some children. Being an IGE, investigations other
than EEG are expected to be normal. Inter-ictal EEG may show rhythmic theta in the
parasagittal regions, with frequent clusters of generalised spike-wave discharges at 23 Hz
and/or polyspike or polyspike and wave discharges (Figure 4). These patterns may also be
ictal.

Figure 4. The EEG from a six-year-old boy with Doose syndrome.

The prognosis is variable. Many children (perhaps up to half) with the syndrome become free
of the drop attacks, although they may continue to have GTCS, and develop normally or near
normally. In others drop attacks may continue for years and learning difficulties become
apparent. It is these children with Doose syndrome who appear to have an epileptic
encephalopathy.
The response to AEDs varies from complete responses to marked drug resistance. Drugs
active against generalised epilepsies should be used. These include sodium valproate,
topiramate, levetiracetam and benzodiazepines. Lamotrigine may also be helpful but there is
concern that it can exacerbate myoclonic seizures. Drugs mainly active against focal seizures,
such as carbamazepine may exacerbate seizures and should be avoided. Doose syndrome
often responds very well to the ketogenic diet.
Landau-Kleffner syndrome and ECSWS (or ESES)
Landau-Kleffner syndrome (also called acquired epileptic aphasia) and epilepsy with
continuous spike and waves during slow-wave sleep (ECSWS) or epileptic encephalopathy
with electrical status epilepticus during slow-wave sleep (ESES) overlap and therefore will
be considered together. The Landau-Kleffner syndrome (LKS) usually starts in otherwise
normal children between five and seven years of age but can start much earlier or later. It is