Overview
Acute encephalopathy with inflammation-mediated status epilepticus, also known by its abbreviation AEIMSE or sometimes referred to as AERRPS (Acute Encephalitis with Refractory, Repetitive Partial Seizures) or FIRES (Febrile Infection-Related Epilepsy Syndrome), is a rare and serious brain condition. It typically affects previously healthy children or young adults who develop sudden, severe, and prolonged seizures (called status epilepticus) that are very difficult to control with standard seizure medications. The condition usually begins after a mild illness such as a fever or cold, followed by an intense inflammatory response in the brain. This inflammation triggers continuous or near-continuous seizures that can last days to weeks and may require intensive care treatment including medically induced coma. The exact cause of this condition is not fully understood, but it is believed to involve an abnormal immune or inflammatory response that attacks the brain. It is not typically inherited and appears to occur sporadically. Key symptoms include prolonged seizures, altered consciousness, confusion, and potential long-term brain damage. Treatment focuses on stopping the seizures, reducing brain inflammation, and supporting the patient through the acute crisis. Despite aggressive treatment, many patients experience lasting effects including ongoing epilepsy, cognitive difficulties, and behavioral changes. Research is ongoing to better understand the underlying mechanisms and develop more effective treatments. Early recognition and rapid treatment in a specialized center are critical for the best possible outcomes.
Key symptoms:
Prolonged seizures that are very hard to stopHigh fever or recent mild illness before seizure onsetLoss of consciousness or deep confusionRepetitive jerking movements of the body or faceDifficulty breathing during seizure episodesMemory problems after the acute phaseDifficulty speaking or understanding languageBehavioral changes such as irritability or aggressionOngoing epilepsy after the initial episodeLearning difficulties or intellectual declineWeakness or movement problemsPersonality changesFatigue and low energy levels
Sporadic
Usually appears on its own, not inherited from a parent
Variable
Can begin at different ages, from infancy through adulthood
Treatments
No FDA-approved treatments are currently listed for Acute encephalopathy with inflammation-mediated status epilepticus.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for Acute encephalopathy with inflammation-mediated status epilepticus at this time.
New trials open frequently. Follow this disease to get notified.
Specialists
View all specialists →No specialists are currently listed for Acute encephalopathy with inflammation-mediated status epilepticus.
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
Travel Grants
No travel grants are currently matched to Acute encephalopathy with inflammation-mediated status epilepticus.
Community
No community posts yet. Be the first to share your experience with Acute encephalopathy with inflammation-mediated status epilepticus.
Start the conversation →Latest news about Acute encephalopathy with inflammation-mediated status epilepticus
No recent news articles for Acute encephalopathy with inflammation-mediated status epilepticus.
Follow this condition to be notified when news becomes available.
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.What is the likely cause of the seizures and brain inflammation in my case?,What immunotherapy options are available, and what are the risks and benefits of each?,Would the ketogenic diet be appropriate, and how do we get started?,What is the long-term outlook for seizure control and cognitive recovery?,What rehabilitation services should we pursue, and how soon should they start?,Are there any clinical trials or research studies we could participate in?,What should our emergency seizure action plan look like, and what rescue medications should we have at home?
Common questions about Acute encephalopathy with inflammation-mediated status epilepticus
What is Acute encephalopathy with inflammation-mediated status epilepticus?
Acute encephalopathy with inflammation-mediated status epilepticus, also known by its abbreviation AEIMSE or sometimes referred to as AERRPS (Acute Encephalitis with Refractory, Repetitive Partial Seizures) or FIRES (Febrile Infection-Related Epilepsy Syndrome), is a rare and serious brain condition. It typically affects previously healthy children or young adults who develop sudden, severe, and prolonged seizures (called status epilepticus) that are very difficult to control with standard seizure medications. The condition usually begins after a mild illness such as a fever or cold, followed
How is Acute encephalopathy with inflammation-mediated status epilepticus inherited?
Acute encephalopathy with inflammation-mediated status epilepticus follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.