More Severe Syndromes: Lennox-Gastaut and Related Conditions

The syndromes we'll look at in this chapter affect only a small number of children — but because they tend to follow a more challenging course, they deserve their own section. If you are facing one of these conditions, know that you are not alone, and that here too there are treatment options that keep improving.

Lennox-Gastaut Syndrome (LGS)

Lennox-Gastaut syndrome is a severe developmental and epileptic encephalopathy that usually begins between ages 3 and 5. It has three core features: more than one type of seizure (especially tonic seizures, atonic/drop seizures, and atypical absences), a characteristic slow spike-and-wave pattern on the EEG, and usually developmental delay.

In LGS, seizures are often resistant to medication, and control with a single drug is rarely possible; in most cases, more than one treatment needs to be used together. Because atonic (drop) seizures in particular carry a risk of injury, safety measures (such as a protective helmet) become important for these children.

Treatment Options in LGS

In LGS, the goal is often not complete freedom from seizures, but rather reducing the most harmful seizures (especially drop seizures) and improving the child's quality of life. Alongside medication, the ketogenic diet, vagus nerve stimulation (VNS), and — in selected patients — surgical options may come into play. In recent years, new medications approved specifically for LGS, along with cannabidiol, have made important additions to the treatment toolkit.

Sleep-Related Epileptic Encephalopathy (ESES / CSWS)

In this condition, the child's EEG shows almost continuous abnormal epileptic activity during sleep. Here's the interesting part: the seizures may be infrequent, or may not even be noticeable at all; yet this continuous electrical activity during sleep can cause a decline in the child's language, learning, attention, and behavior. That's why the first noticed sign is sometimes a sudden drop in the child's school performance or speech. The aim of treatment is to suppress this activity during sleep and protect the child's cognitive progress.

Landau-Kleffner Syndrome

In this rare syndrome, a child who had been developing normally gradually loses the ability to understand language and to speak (acquired epileptic aphasia). Families often suspect a hearing problem at first. The underlying cause is epileptic activity affecting the language centers. Early diagnosis and treatment are important for protecting language function; these children also benefit greatly from speech and language therapy.

Coping With Severe Syndromes

These conditions are among the most challenging for families, both medically and emotionally. Be patient with yourself and with your child.

These children are best supported through a team approach involving a pediatric neurologist, a physiotherapist, a speech and language therapist, a special education specialist, and, when needed, a dietitian.

Celebrate every small step of progress. Needing support along this path is not a weakness — it's a sign of strong parenting.