Overview
Acute encephalopathy with biphasic seizures and late reduced diffusion, often called AESD, is a rare brain condition that mainly affects young children, most commonly between 6 months and 4 years of age. It typically begins during a fever or viral infection, when a child suddenly develops prolonged seizures. After the initial seizures, the child may seem to improve for a few days, but then a second round of seizures occurs, usually around days 3 to 7 of the illness. During this second phase, brain MRI scans show a characteristic pattern called "reduced diffusion" in specific areas of the brain, particularly the white matter just under the cortex. This pattern helps doctors distinguish AESD from other types of childhood brain injury caused by fever or infection. The condition can lead to a range of outcomes. Some children recover well with only mild effects, while others may develop intellectual disability, epilepsy, or movement problems. The severity depends on how much brain tissue is affected. Treatment is mainly supportive, focusing on controlling seizures, reducing brain swelling, and managing the underlying infection. There is no specific cure for AESD. Researchers are studying ways to protect the brain during the critical early phase, including cooling therapy and certain medications, but no treatment has been proven to prevent the second phase of injury. Early recognition and prompt medical care are essential to give children the best chance of a good outcome.
Key symptoms:
Prolonged seizures during a feverLoss of consciousness or reduced awarenessA second cluster of seizures several days after the firstConfusion or altered mental state between seizure episodesWeakness on one or both sides of the bodyDifficulty speaking or understanding languageIntellectual disability or learning difficulties after recoveryMovement problems such as involuntary movements or poor coordinationEpilepsy developing after the acute illnessBehavioral changesDifficulty with fine motor skillsSwallowing difficulties in severe cases
Clinical phenotype terms (12)— hover any for plain English
Sporadic
Usually appears on its own, not inherited from a parent
Infantile
Begins in infancy, roughly 1 month to 2 years old
FDA & Trial Timeline
2 eventsEndari: FDA approved
To reduce the acute complications of sickle cell disease in adult and pediatric patients 5 years of age and older.
Ammonul: FDA approved
Adjunctive therapy in the treatment of acute hyperammonemia and associated encephalopathy in patients with deficiencies in enzymes of the urea cycle.
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Acute encephalopathy with biphasic seizures and late reduced diffusion.
View clinical trials →Clinical Trials
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Specialists
View all specialists →No specialists are currently listed for Acute encephalopathy with biphasic seizures and late reduced diffusion.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Financial Resources
1 resourcesAmmonul
Medicis Pharmaceutical Corp.
Travel Grants
No travel grants are currently matched to Acute encephalopathy with biphasic seizures and late reduced diffusion.
Community
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Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
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.How severe is the brain injury shown on my child's MRI, and what does this mean for their recovery?,What treatments are being used to protect my child's brain during this acute phase?,Will my child need long-term anti-seizure medication, and what are the side effects?,What developmental or learning challenges should I watch for as my child grows?,Should my child have genetic testing to look for risk factors, and would this affect treatment?,What rehabilitation services (physical therapy, speech therapy, occupational therapy) does my child need?,What should I do if my child has another seizure or develops a fever in the future?
Common questions about Acute encephalopathy with biphasic seizures and late reduced diffusion
What is Acute encephalopathy with biphasic seizures and late reduced diffusion?
Acute encephalopathy with biphasic seizures and late reduced diffusion, often called AESD, is a rare brain condition that mainly affects young children, most commonly between 6 months and 4 years of age. It typically begins during a fever or viral infection, when a child suddenly develops prolonged seizures. After the initial seizures, the child may seem to improve for a few days, but then a second round of seizures occurs, usually around days 3 to 7 of the illness. During this second phase, brain MRI scans show a characteristic pattern called "reduced diffusion" in specific areas of the brain
How is Acute encephalopathy with biphasic seizures and late reduced diffusion inherited?
Acute encephalopathy with biphasic seizures and late reduced diffusion follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Acute encephalopathy with biphasic seizures and late reduced diffusion typically begin?
Typical onset of Acute encephalopathy with biphasic seizures and late reduced diffusion is infantile. Age of onset can vary across affected individuals.
What treatment and support options exist for Acute encephalopathy with biphasic seizures and late reduced diffusion?
1 patient support program are currently tracked on UniteRare for Acute encephalopathy with biphasic seizures and late reduced diffusion. See the treatments and support programs sections for copay assistance, eligibility, and contact details.