Developmental and epileptic encephalopathy with spike-wave activation in sleep

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ORPHA:725OMIM:245570G40.4
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2Active trials3Specialists8Treatment centers

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Overview

Developmental and epileptic encephalopathy with spike-wave activation in sleep (DEE-SWAS) is a rare and serious brain condition that affects children. It is also sometimes called epileptic encephalopathy with continuous spike-and-wave during sleep (CSWS) or, when it mainly affects language, Landau-Kleffner syndrome. In this condition, the brain produces abnormal electrical activity — especially during sleep — that disrupts normal brain development. This unusual brain activity shows up on a test called an EEG (electroencephalogram) as continuous or near-continuous spike-wave patterns during non-REM sleep. The condition causes seizures, but the sleep-related brain activity is often what causes the most harm. Even without obvious seizures at night, the abnormal electrical bursts during sleep interfere with learning, memory, behavior, and language. Children may regress — meaning they lose skills they had already learned, such as speech or coordination. Treatment focuses on reducing the abnormal brain activity, especially during sleep, using anti-seizure medications, steroids, or other therapies. While there is no cure, early and aggressive treatment can help protect brain development and improve quality of life. The condition often improves after puberty, but many children are left with lasting learning or behavioral challenges.

Also known as:

Key symptoms:

Seizures, often occurring during sleepLoss of previously learned language or speech skillsLearning difficulties and intellectual regressionAttention problems and hyperactivityBehavioral changes such as aggression or mood swingsMemory problemsDifficulty understanding spoken languagePoor coordination or clumsinessSleep disturbancesDrooling or difficulty swallowing in some casesSocial withdrawal or autism-like behaviors

Clinical phenotype terms (30)— hover any for plain English
Interictal epileptiform activityHP:0011182Continuous spike and waves during slow sleepHP:0031491Focal aware seizureHP:0002349Focal hemiclonic seizureHP:0006813Atypical absence seizureHP:0007270Focal motor seizureHP:0011153EEG with frontal focal spike wavesHP:0012010EEG with centrotemporal focal spike wavesHP:0012557Psychotic mentationHP:0001345Focal clonic seizureHP:0002266
Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗OMIM ↗NORD ↗

FDA & Trial Timeline

4 events
Jul 2026Melatonin for the Treatment of DEE-SWAS

Hugo W. Moser Research Institute at Kennedy Krieger, Inc. — PHASE2

TrialNOT YET RECRUITING
Aug 2024Study of Patients with Electrical Status Epilepticus in Sleep

East Suffolk and North Essex NHS Foundation Trust

TrialRECRUITING
Aug 2019Repeated Oscillatory TMS Therapy of the Epileptogenic Cortical Area in Children With Focal CSWS

Children's Mercy Hospital Kansas City — NA

TrialRECRUITING
Mar 2019Sleep Related Memory Consolidation in Children With Age Related Focal Epilepsy.

University Hospital, Strasbourg, France — NA

TrialRECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

No FDA-approved treatments are currently listed for Developmental and epileptic encephalopathy with spike-wave activation in sleep.

2 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.

View clinical trials →

Clinical Trials

2 recruitingView all trials with filters →
N/A2 trials
Repeated Oscillatory TMS Therapy of the Epileptogenic Cortical Area in Children With Focal CSWS
N/A
Actively Recruiting
PI: Lalit Bansal, M.D. (Children's Mercy Hospital Kansas City) · Sites: Overland Park, Kansas · Age: 321 yrs
Sleep Related Memory Consolidation in Children With Age Related Focal Epilepsy.
N/A
Actively Recruiting
PI: Anne DE SAINT MARTIN, MD (Hôpitaux Universitaires de Strasbourg) · Sites: Nancy; Strasbourg · Age: 613 yrs

Specialists

3 foundView all specialists →
AE
Ann-Sofie Eriksson
Specialist
PI on 1 active trial
AM
Anne DE SAINT MARTIN
Specialist
PI on 1 active trial
LB
Lalit Bansal
KANSAS CITY, MO
Specialist
PI on 1 active trial

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Developmental and epileptic encephalopathy with spike-wave activation in sleep.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Developmental and epileptic encephalopathy with spike-wave activation in sleep

Disease timeline:

New recruiting trial: Sleep Related Memory Consolidation in Children With Age Related Focal Epilepsy.

A new clinical trial is recruiting patients for Developmental and epileptic encephalopathy with spike-wave activation in sleep

New recruiting trial: Repeated Oscillatory TMS Therapy of the Epileptogenic Cortical Area in Children With Focal CSWS

A new clinical trial is recruiting patients for Developmental and epileptic encephalopathy with spike-wave activation in sleep

New recruiting trial: Study of Patients with Electrical Status Epilepticus in Sleep

A new clinical trial is recruiting patients for Developmental and epileptic encephalopathy with spike-wave activation in sleep

Caregiver Resources

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Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.What is causing my child's condition — has a genetic cause been found?,How often should my child have EEG monitoring, and what are we looking for?,Which medication or treatment do you recommend first, and what are the side effects?,When should we consider steroids, and what are the risks?,What signs should prompt me to call you or go to the emergency room?,What educational and therapy supports should my child have right now?,Is there a chance my child's condition will improve after puberty, and what can we do to protect their development in the meantime?

Common questions about Developmental and epileptic encephalopathy with spike-wave activation in sleep

What is Developmental and epileptic encephalopathy with spike-wave activation in sleep?

Developmental and epileptic encephalopathy with spike-wave activation in sleep (DEE-SWAS) is a rare and serious brain condition that affects children. It is also sometimes called epileptic encephalopathy with continuous spike-and-wave during sleep (CSWS) or, when it mainly affects language, Landau-Kleffner syndrome. In this condition, the brain produces abnormal electrical activity — especially during sleep — that disrupts normal brain development. This unusual brain activity shows up on a test called an EEG (electroencephalogram) as continuous or near-continuous spike-wave patterns during non

At what age does Developmental and epileptic encephalopathy with spike-wave activation in sleep typically begin?

Typical onset of Developmental and epileptic encephalopathy with spike-wave activation in sleep is childhood. Age of onset can vary across affected individuals.

Are there clinical trials for Developmental and epileptic encephalopathy with spike-wave activation in sleep?

Yes — 2 recruiting clinical trials are currently listed for Developmental and epileptic encephalopathy with spike-wave activation in sleep on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.

Which specialists treat Developmental and epileptic encephalopathy with spike-wave activation in sleep?

3 specialists and care centers treating Developmental and epileptic encephalopathy with spike-wave activation in sleep are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.