ESES in Children: A Guide for Parents and Caregivers

Imagine a child who once thrived in school suddenly struggling with learning, memory, and behavior. The culprit? A rare neurological condition that disrupts the brain’s rhythm during deep sleep when it should be resting and restoring. This condition is known as Electrical Status Epilepticus during Slow-Wave Sleep, or ESES in children.

ESES in children is a severe, uncommon form of pediatric epilepsy that affects brain function particularly during non-REM deep sleep. Though it usually resolves around puberty, its long-term effects on cognition, learning, and behavior can be profound.

This blog explores what ESES epilepsy is, how it presents, the treatment options available, and what the future may hold for those affected. At Revive Research Institute, we’re committed to supporting individuals and families facing epilepsy through ongoing clinical trials because no one should navigate this journey without hope or options.

ESES in Children: What is ESES?

ESES, also referred to as Continuous Spikes and Waves during Slow-Wave Sleep (CSWS), is a childhood epilepsy syndrome first described in 1971. It presents a unique EEG pattern characterized by near-continuous epileptiform discharges during slow-wave (deep) sleep.

This disruption isn’t always accompanied by frequent seizures, which can delay diagnosis. But the real danger lies in its impact on neurodevelopment affecting how a child learns, behaves, and interacts with the world.

ESES In Children: Who is Affected?

ESES primarily affects children between the ages of 2 and 12, with a peak occurrence between 3 and 5 years old. It’s extremely rare, one study reported a prevalence of only 0.2% among children with epilepsy.

Symptoms to Watch For:

The symptoms of ESES in children may develop subtly over time and can often be mistaken for other developmental or behavioral disorders.

Seizures:

• These may be focal, generalized, or nocturnal
• Often less frequent than in other forms of epilepsy

Cognitive Impairment:

• Declines in attention, memory, and learning ability
• Struggles at school, inability to acquire new knowledge
• Possible language delays or regression

Behavioral Changes:

• Hyperactivity, irritability, aggression
• May mimic symptoms of autism spectrum disorders
• Trouble with social interactions

Motor & Language Difficulties:

• Loss of previously acquired motor skills
• Speech difficulties or aphasia

These changes often occur over weeks to months and may be more noticeable to teachers or caregivers than to medical professionals initially.

What Causes ESES?

While the exact cause of ESES in children remains unclear, several factors may play a role:

• Genetic predisposition: Family history may increase the risk of developing ESES.
• Structural brain abnormalities: Conditions like cortical dysplasia or brain lesions.
• Neurotransmitter imbalances: Disruptions in brain signaling chemicals.
• Immune system dysfunction: Inflammatory or autoimmune responses may contribute.

Risk Factors

Certain conditions may increase the likelihood of developing ESES in children:

• Early-onset seizure disorders
• Genetic or familial predisposition
• History of brain injury or malformations
• Neurodevelopmental conditions such as autism or intellectual disability

Also Read: Understanding Rolandic Epilepsy: Signs, Diagnosis, and Care

How is ESES Diagnosed?

Diagnosis of ESES in children often involves a comprehensive evaluation that includes:

Electroencephalogram (EEG)

• It is a key diagnostic tool that reveals continuous spike-wave activity during slow-wave sleep

Neuroimaging (MRI/CT)

• It helps identify any structural abnormalities in the brain

Neuropsychological Testing:

• It assesses cognitive development, memory, attention, and language skills

Medical History & Symptom Review:

• Includes behavioral and academic history

Because ESES in children is most visible during deep sleep, an overnight EEG or prolonged sleep study is often necessary for accurate diagnosis.

Treatment Options

Treatment of ESES in children is often challenging, as the condition can be resistant to traditional anti-epileptic medications. However, a combination of approaches may be effective:

Anti-Epileptic Drugs (AEDs)

• Common first-line treatments include valproic acid, levetiracetam, and ethosuximide

Benzodiazepines:

• Medications like clonazepam or clobazam help reduce EEG abnormalities during sleep

Corticosteroids:

• Prednisone or methylprednisolone are used in difficult-to-treat cases

Intravenous Immunoglobulins (IVIG)

• May be used as an alternative to steroids, particularly if an immune cause is suspected

Ketogenic Diet:

• A high-fat, low-carb diet has shown success in reducing seizures and improving EEG patterns

In some cases, surgical intervention may be considered if ESES is linked to a localized brain lesion.

Prognosis and Long-Term Outlook

The EEG abnormalities and seizures typically resolve after puberty. However, this doesn’t always mean full recovery.

Potential Long-Term Challenges:

• Persistent cognitive deficits (especially with delayed diagnosis or treatment)
• Behavioral issues such as anxiety, depression, or hyperactivity
• Speech and language delays
• Difficulty regaining academic and social skills

Children diagnosed with syndromes like Landau-Kleffner Syndrome, a condition related to ESES may face lifelong challenges, even after seizures subside.

Also Read: Epilepsy Life Expectancy: What You Need to Know

How Can Parents Support Their Child?

Early recognition and treatment of ESES in children can make a huge difference in the child’s long-term cognitive and behavioral development. Here’s what caregivers can do:

• Watch for changes in learning, memory, speech, or social behavior
• Follow through with EEG recommendations and neuropsychological testing
• Explore treatment options with your neurologist, including dietary therapies
• Consider therapy speech, occupational, and cognitive behavioral therapies can be valuable

Conclusion

Electrical Status Epilepticus during Slow-Wave Sleep is a rare but serious epilepsy syndrome that requires early recognition and tailored treatment. Though often temporary in its EEG presentation, the effects on a child’s learning and behavior can be long-lasting.

With prompt intervention, a multidisciplinary care team, and ongoing support, many children with ESES can lead fulfilling lives equipped with the tools they need to overcome the cognitive and emotional challenges they face.