Rasmussen subacute encephalitis

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ORPHA:1929G04.8
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Overview

Rasmussen encephalitis, also called Rasmussen subacute encephalitis or Rasmussen syndrome, is a rare inflammatory brain disease that mainly affects one side (hemisphere) of the brain. It most often begins in childhood, typically between ages 2 and 10, though it can occasionally start in teenagers or adults. The disease causes progressive inflammation and damage to one hemisphere of the brain, leading to worsening seizures, weakness on one side of the body, and a gradual decline in thinking and language abilities. The hallmark symptom is epilepsia partialis continua — frequent, repetitive seizures affecting one side of the body that are very difficult to control with standard seizure medications. Over time, the affected brain hemisphere shrinks (atrophies), and patients develop increasing weakness or paralysis on the opposite side of the body (hemiparesis). Cognitive abilities, including speech and memory, may also decline as the disease progresses. The exact cause of Rasmussen encephalitis is not fully understood, but it is believed to involve an autoimmune process where the body's immune system mistakenly attacks healthy brain tissue. Some researchers have found antibodies against brain proteins, particularly GluR3 (a glutamate receptor), in some patients, though this is not present in all cases. Treatment options include anti-seizure medications, immunotherapy (such as steroids, intravenous immunoglobulin, or plasma exchange) to reduce inflammation, and in many cases, surgery. The most effective surgical treatment is a hemispherectomy or hemispherotomy, which disconnects or removes the affected hemisphere to stop seizures. While this surgery can be life-changing, it does result in permanent weakness on one side and visual field loss.

Also known as:

Key symptoms:

Frequent seizures affecting one side of the bodyContinuous or near-continuous partial seizures (epilepsia partialis continua)Weakness or paralysis on one side of the bodyDifficulty speaking or understanding languageDecline in thinking and memory abilitiesLoss of motor skills on one sideVision loss in one half of the visual fieldPersonality or behavioral changesDifficulty with school performanceInvoluntary jerking movements of the face, arm, or legBrain shrinkage visible on MRIDifficulty walking or using one handTrouble with coordination on one side

Clinical phenotype terms (48)— hover any for plain English
Focal aware seizureHP:0002349Focal motor seizureHP:0011153EEG with focal spikesHP:0011193EEG with focal sharp slow wavesHP:0011195Subcortical cerebral atrophyHP:0012157Epilepsia partialis continuaHP:0012847Repeated focal motor seizuresHP:0032665Cerebral cortical hemiatrophyHP:0100308
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Rasmussen subacute encephalitis.

View clinical trials →

No actively recruiting trials found for Rasmussen subacute encephalitis at this time.

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Search ClinicalTrials.gov ↗Join the Rasmussen subacute encephalitis community →

Specialists

3 foundView all specialists →
KM
Kenneth D Laxer, M.D.
SAN FRANCISCO, CA
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 Rasmussen subacute encephalitis.

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Community

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.What stage of Rasmussen encephalitis is my child in, and how quickly is it progressing?,Would immunotherapy be helpful at this point, and what are the risks and benefits?,When should we consider hemispherectomy surgery, and what outcomes can we expect?,What seizure rescue medications should we have at home, and when should we call 911?,How will this disease affect my child's learning and development, and what support services are available?,Are there any clinical trials or new treatments being studied for Rasmussen encephalitis?,What rehabilitation services will my child need, and how can we access them?

Common questions about Rasmussen subacute encephalitis

What is Rasmussen subacute encephalitis?

Rasmussen encephalitis, also called Rasmussen subacute encephalitis or Rasmussen syndrome, is a rare inflammatory brain disease that mainly affects one side (hemisphere) of the brain. It most often begins in childhood, typically between ages 2 and 10, though it can occasionally start in teenagers or adults. The disease causes progressive inflammation and damage to one hemisphere of the brain, leading to worsening seizures, weakness on one side of the body, and a gradual decline in thinking and language abilities. The hallmark symptom is epilepsia partialis continua — frequent, repetitive seiz

How is Rasmussen subacute encephalitis inherited?

Rasmussen subacute encephalitis follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Rasmussen subacute encephalitis typically begin?

Typical onset of Rasmussen subacute encephalitis is childhood. Age of onset can vary across affected individuals.

Which specialists treat Rasmussen subacute encephalitis?

3 specialists and care centers treating Rasmussen subacute encephalitis are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.