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BENIGN ROLANDIC EPILEPSY

Also called benign epilepsy of childhood with centro-temporal spikes

 

            This 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 anatomical lesion. They spontaneously subside. The children do not have any neurological or intellectual deficit. They often have family history of idiopathic epilepsy.

            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 seizures.

             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 or two).

             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.

 

Genetic factors

             Probably autosomal dominant with variable penetrance, this syndrome has a 40% positive family history.

 

Investigations

             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.

 

Treatment

             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 medication.

            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 out” of their seizures.