Also called benign epilepsy
of childhood with centro-temporal spikes
syndrome belongs to the localization related Idiopathic epilepsies. These
epileptic syndromes of childhood are characterized by focal seizures and focal
EEG anomalies. They are age related and are not associated with any recognizable
Seizures are usually rare and of short duration. They can however be
quite frequent particularly at the onset of the evolution. Seizure types may
vary from one child to the other but usually remain the same for each patient.
EEG findings are very typical and relatively easily recognizable. A
normal background activity associated with fairly high amplitude isolated spikes
and waves are the norm. The spikes and waves can be multifocal but are
classically in the centro-temporal regions. These abnormalities are exacerbated
during sleep but remain unchanged in their morphology. There are frequently
bursts of generalized spikes and waves seen. As in all partial idiopathic
epilepsies, there is suppression of the anomalies when the patient wakes up.
This is one of the most frequent forms of childhood epilepsies,
representing 15 to 20% of all children with epilepsy. The onset is usually
between 3 and 13 years with a maximum around 9 1/2 years. Usually the seizures
have their onset in the first decade and end in the second decade. As in almost
all epileptic syndromes, there is a male predominance.
Seizures frequency is quite variable, and usually seizures are rare.
A single seizure is reported in about 10% of known cases but it is likely
that a single nocturnal seizure may be missed and the patient may never know of
his/her condition. 2 thirds of the patients will have between 2 and 6 seizures
altogether. In the remaining 20% of cases the seizures can be very frequent, up
to several per day. There is no correlation between the seizure frequency, the
EEG anomalies, the response to treatment and the evolution which almost
invariably toward cessation of the seizures.
Seizures are usually very short in duration lasting between few seconds
and a couple of minutes. Nocturnal seizures tend to last longer than the ones
occurring during daytime. Most of the time (up to 75%) the seizures are solely
at night time. 15% of patients have purely diurnal seizures and the remaining
15% have seizures both during the day and the night. Often nocturnal seizures
occur toward the end of the night.
Up to 10% of children with Rolandic epilepsy had a febrile seizure(s).
About 5% of them have a history of perinatal difficulties. These factors are
unlikely to have any correlation with regard to the cause of the rolandic
These children do not have any neurological anomalies and some authors
believe that if the neurological exam is abnormal, the diagnosis of rolandic
epilepsy should be rejected. There are however rare cases reported where true
rolandic epilepsy coexists with a brain lesion.
Typical Rolandic seizure
The onset is often somato-sensitive. It consists of a unilateral
paresthesia involving the tongue, the lips the gums and the inside of the cheek.
Then there is a unilateral convulsion of the face, lips, tongue as well as the
muscles responsible for speech production. The convulsion can be tonic, clonic
or tonico-clonic. There is speech arrest (anarthria) and the child usually will
drool. During the episode, the level of consciousness remains normal and the
child will look scared.
Typically, a 5 to 10 year old child, previously perfectly healthy will
come to his parent, totally conscious, but unable to speak. He points to his
mouth, which is deviated to one, side drooling at the corner, and showing
jerking contractions. The spell does last more than 2 minutes and at the end the
child may be able to explain that it started by a funny feeling in the mouth,
either tingling or metallic taste on the tongue. This is so classical that the
diagnostic hardly requires an EEG.
On occasion the convulsion can spread to the arm, or even the leg. It can
switch side or become generalized if nocturnal (hardly ever will it become
generalized if it occurred during the day). Nocturnal seizures are the most
frequent ones and do often become generalized, and since they are rarely witness
right from the beginning, they are often mistaken for generalized seizures. The
EEG will then bring the diagnosis to light. These nocturnal seizures are often
brought to the mother s attention by vocal sounds coming from the child s
bedroom. She finds her child grunting or moaning with the mouth deviated to the
side or already in a full-blown generalized seizure. These nocturnal seizures
can last much longer than the typical diurnal ones. They may last up to half an
hour and may be followed by a transient Todd palsy (paralysis of the body part
that was involved during the seizure. This palsy resolves within hours to a day
The somato sensitive onset is probably very frequent, but may not be
described accurately by the young child. There are rare cases where they are the
only symptom of a seizure that does not evolve to a focal convulsion. Atypical
seizures can present as abdominal pain, sudden transient blindness, episodes of
dizziness, flashing lights or even absences. As a rule, the younger the child,
the more atypical the seizure can be.
Probably autosomal dominant with variable penetrance, this syndrome has a
40% positive family history.
In typical crisis, clinical diagnosis is easy and hardly requires an EEG.
If there are technical difficulties (young uncooperative child, no EEG services
in the community, etc) one may pass on the EEG. The EEG if obtained will
confirm the clinical diagnosis.
Only in atypical seizures will a CT scan be necessary. MRI or other
expensive investigations are not required.
No treatment is a very reasonable option. If there are only rare
nocturnal seizures, it is preferable to have these few seizures than to be on
If one decides to use medication, one will have to ascertain the
diagnosis since some medications could be exacerbating seizures if the child had
another syndrome such as Absence epilepsy or Continuous spikes waves of slow
sleep. Once the diagnosis is certain, there are several choices available:
-Carbamazepine (Tegretol) is
efficient in the majority of cases.
- Phenytoin (Dilantin) can
also be used in a single dose in the evening.
- Vigabatrine (Sabril) has
recently been shown to be almost as efficient as Tegretol in partial seizures.
Failure to control seizures with medications is not an unfavorable prognostic factor. One must always keep in mind that the control of the seizures is only to improve life style and does not change the evolution of the syndrome, which is almost always good. Basically all the children with rolandic epilepsy grow outof their seizures.