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Benign Rolandic Epilepsy

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1Benign Rolandic Epilepsy Empty Benign Rolandic Epilepsy on Sun Feb 21, 2010 6:07 am


A typical attack involves twitching, numbness, or tingling of the child's face or tongue (a partial seizure), which often interferes with speech and may cause drooling. These seizures last no more than 2 minutes and the child remains fully conscious. Sometimes the child also may have tonic-clonic seizures, usually during sleep. The seizures are usually infrequent, but they may occur in widely spaced clusters.

This syndrome represents about 15% of all epilepsies in children. The seizures begin at an average age of about 6 to 8 years (range 3-13) and are a bit more likely to affect boys. The children generally have normal intelligence, which is not affected by the seizures. The syndrome is more common in children who have close relatives with epilepsy.

Benign rolandic epilepsy (BRE) is named after the rolandic area of the brain, which controls movement in the affected part of the face. It is also called by other names, especially benign childhood epilepsy with centrotemporal spikes (BCECTS). That name refers to certain patterns ("spikes") seen on the child's EEG, which help to identify the syndrome. Other tests, such as the neurologic examination and MRI (if performed), are normal. While the condition is considered benign -- since most children with the condition outgrow the seizures -- during the period of time when the disorder is active, some children will have learning difficulties and behavioral problems. The problems typically disappear once the seizures stop and the EEG reverts to normal.

Because the seizures may be infrequent and usually occur at night, many children do not take any seizure medicines for BRE. Medication may be prescribed if a child has daytime seizures, a learning disorder, a mild mental handicap, or frequent seizures during sleep. The seizures usually can be controlled by any of the common seizure medicines. Neurontin (gabapentin), Trileptal (oxcarbazepine), Tegretol or Carbatrol (carbamazepine) are most often prescribed.

In almost every case, the seizures stop on their own by age 15.

The Genetics of BRE:

Benign rolandic epilepsy (BRE) and the centrotemporal spikes that identify it on an EEG are more common in siblings and other close relatives of children with this syndrome than in the general population. This suggests that a genetic tendency probably is involved in producing this form of epilepsy.

The likelihood that a person who inherits this tendency will actually develop the disease most likely depends on several factors, including the person's age. The likelihood is very low at birth, becomes almost certain between ages 4 and 16, and almost disappears after age 40. Scientists call this kind of pattern "age-dependent penetrance."

It wasn't possible to find definite evidence of a particular gene until the early 1990s, however, when a new gene-hunting technique called linkage studies began to be used. This technique traces patterns of heredity in large families at high risk for a particular disease. The scientists look for the location of a disease-causing gene mutation by identifying traits that are inherited along with it.

Linkage studies performed in families that include many people with BRE suggest that band 14 of the long arm of chromosome 15 (written by scientists as 15q14) is the most likely location for the gene responsible for some cases—but not all—of this syndrome. The band 15q14 appears to be involved in regulating the excitability of neurons (nerve cells) in the brain, so it may contribute to seizure susceptibility. Researchers have not discovered exactly how the gene produces BRE.

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