Childhood Epilepsies With a Good Outlook

The syndromes we will cover in this chapter share one common feature, summed up by a word in their name: "self-limited." This means that in most children the epilepsy improves and disappears on its own once a certain age is reached. These syndromes used to be called "benign," but because they can have mild effects on learning and behavior, the term "self-limited" is preferred today. Even so, the future is bright for the vast majority of children in this group.

Self-Limited Epilepsy With Centrotemporal Spikes (SeLECTS)

Self-limited epilepsy with centrotemporal spikes (SeLECTS; formerly known as benign rolandic epilepsy) is the most common self-limited epilepsy of childhood; it makes up about 6-7 percent of all childhood epilepsies. From here on, we will simply call it SeLECTS in the book.

Who Gets It, and How Does It Appear?

SeLECTS usually begins between the ages of 4 and 10, most often around age 7. These children are neurologically and mentally normal. The seizures typically occur during sleep or close to waking. Common signs include: stiffening of one side of the face, drooling, being unable to speak, strange sounds from the throat, and tingling in the tongue and cheek. The child often experiences these signs fully conscious, but the seizure may spread during sleep and turn into a tonic-clonic seizure.

Diagnosis and Treatment

On the EEG, centrotemporal spikes specific to this syndrome — which become more pronounced during sleep — are seen and support the diagnosis. The brain MRI is usually normal. When the seizures are infrequent and occur only during sleep, some doctors may prefer to monitor without starting medication, because the syndrome will improve on its own anyway. When medication is needed, the seizures are usually easily controlled with a single medicine.

An Encouraging Fact About SeLECTS

The seizures almost always disappear completely by mid-adolescence (usually by age 15-16).

The vast majority of children go on to live a healthy, seizure-free, medication-free life in adulthood. In this syndrome, some children may have mild difficulties with attention, language, or learning; for this reason, school performance should be monitored.

Self-Limited Epilepsy With Autonomic Seizures (Panayiotopoulos Syndrome)

This syndrome usually appears in children aged 3-6. Its most striking feature is that the seizures involve autonomic signs (relating to the body's involuntary functions). The child may suddenly turn pale, feel nauseated, or vomit; their eyes may turn to one side. The seizures can be long (sometimes more than half an hour), and this frightens families. However, the overall course of the syndrome is good, and the seizures usually improve on their own within a few years. In most children, the number of seizures is small.

Childhood Absence Epilepsy

We introduced absence seizures earlier; childhood absence epilepsy is a syndrome in which these seizures take center stage. It usually begins between the ages of 4 and 10, slightly more often in girls than in boys. The child has many brief zoning-out seizures a day.

Why Is Early Diagnosis Important?

In this syndrome, the child's intelligence is normal; however, "switching off" for a few seconds dozens of times a day makes it hard to follow lessons and learn. This is why the child may be wrongly labeled "inattentive" or "lazy." The correct diagnosis protects both the child's school success and their self-confidence.

Treatment and Course

Childhood absence epilepsy responds very well to correctly chosen medications, and the seizures are usually fully brought under control. A very important warning: some common epilepsy medications (especially certain medications that affect the sodium channel) can make absence seizures worse. This is exactly why the correct syndrome diagnosis and the correct choice of medication are vital. In a significant portion of children, the seizures disappear in adolescence.

Childhood Occipital Epilepsies With Visual Signs

In this group, the seizures start in the visual center at the back of the brain. During a seizure, the child may experience visual signs such as colored circles, lights, or temporary loss of vision; this may be accompanied by headache and vomiting. For this reason, it can sometimes be confused with migraine. The overall course is usually good.