Rasmussen's Syndrome
What Is Rasmussen's Syndrome?
Rasmussen's syndrome is associated with slowly worsening neurological problems and seizures in children. It begins between 14 months - 14 years of age, and seizures are often the first problem to appear.
- Mild weakness of an arm or leg is the most common early symptom besides seizures.
- The weakness and other neurological problems often begin 1 to 3 years after the seizures start.
- Progressive weakness on one side (hemiparesis) and thinking/memory impairment are common.
- Language problems (aphasia) often occur if the disorder affects the side of the brain that controls most language functions, which is usually the left side.
Recent studies suggest that the cause of Rasmussen's syndrome is an autoimmune disorder (antibodies are produced against the body's own tissues) directed against receptors on the brain cells. The process may be triggered by a viral infection, and possibly other triggering causes. A blood test for certain antibodies can be helpful in making the diagnosis.
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Contact Our HelplineWhat Type of Seizures Are Seen?
Simple partial motor seizures are the most common type, but in 1 out of 5 children, the first seizure is an episode of partial or tonic-clonic status epilepticus.
How Is Rasmussen's Syndrome Diagnosed?
CT and MRI scans of the brain show evidence of a slow loss (atrophy) of brain substance.
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Brain ImagingHow Is Rasmussen's Syndrome Treated?
Treatment of this disease with seizure medicines is disappointing. Anti-inflammatory steroids may be effective, but additional studies are needed. Immunologic therapies (gamma globulin, plasmapheresis, rituximab) may be helpful in some cases.
In children with severe weakness and loss of touch and vision on the side of the body opposite the involved hemisphere of the brain, a surgical procedure called a functional hemispherectomy may be successful.
What Is the Outlook?
Rasmussen's syndrome is rarely fatal, but its effects can be devastating.
- The seizures are typically relentless and weakness and mental impairment often follow.
- Despite the drastic nature of hemispherectomy, where half of the brain is removed, the surgery can be very helpful in stopping the seizures and preventing mental impairment.
- Children adjust to hemispherectomy remarkably well. After the surgery, most can walk and run, although with a limp. Hand function on the side opposite to the surgery is often significantly impaired and fine motor skills are not possible. Even when the language hemisphere is removed, children typically regain a considerable amount of language skills. After age 6, people recover less well from the side effects of hemispherectomy.
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