What is ESES?

ESES epilepsy (Electrical Status Epilepticus during Sleep) is a rare and complex form of epilepsy which primarily affects children, especially between ages three and ten. When it occurs, the child has multiple seizure episodes which is caused by erratic electrical activity throughout the child’s brain.

Some key findings sum up this condition are:

Seizure activity

ESES encompasses some increase in seizure threshold at sleep but with more subtle clinical features. In our practice, after the child suffers from insomnia and goes to sleep, it is quite common for them to have seizure episodes throughout the night, but when the child wakes up, they appear relatively calm and relaxed.

Cognitive decline

A key feature of ESES is significant learning and cognitive dysfunction. As a result, the child struggles to learn new things or even forgets what they have already learned, leading to behavioral and communication difficulties.Most seizures occur during the night. There is, however, a small, if not persistent, portion that occurs in the daytime: absence seizures, which last for a brief period, and myoclonic seizures at the front lead.

Causes and diagnostics

What causes ESES epilepsy is not known. It seems to have something to do with a problem involving activity in the thalamus, a part of the integrative brain that processes information from sensations. About 10% have a genetic origin, whereas almost 45% seem to originate from some kind of physical trauma to the tissues of the brain. But in most of the cases, the cause is undetermined.

Diagnosis is usually based on EEG during sleep, which demonstrates the typical seizure pattern present in ESES. 

Options for treatment: The options available, according to ESES, for epilepsy are:

• Medications: Sodium valproate, ethosuximide, and levetiracetam are the anti-seizure drugs commonly used. We can add steroids and immunomodulatory agents to the patient in some cases to help reduce seizures. 
• Dietary Interventions: Some patients with epilepsy have found the ketogenic diet, which is low in fat and high in food intake to be effective. 
• Surgical approach: If medications are not helpful surgical options should be considered.

Prognosis: Early diagnosis and treatment are essential for the best results. In most instances, children can live their lives and go through the learning and development tasks without much disruption as long as the treatment is initiated ear

To summarize:

Electrical Status Epilepticus during Sleep (ESES) is a rare epilepsy syndrome affecting children aged 3 to 10, characterized by erratic electrical brain activity and frequent seizures, primarily during sleep, with some occurring during the day presenting as absence or myoclonic seizures. This condition often leads to cognitive decline, learning difficulties, and behavioral issues. While the exact cause is unclear, it may involve thalamic activity, genetic factors, or brain trauma. Diagnosis is typically made through sleep EEG to identify characteristic seizure patterns. Treatment options include anti-seizure medications like sodium valproate and levetiracetam, steroids, ketogenic diets, and, in severe cases, surgery. Early diagnosis and treatment are critical for managing symptoms and enabling children to continue their learning and development with minimal disruption.