Rasmussen’s Syndrome– When It’s More Than Just the Seizures

In general, epilepsy can be described by the type of seizures a person has or by a term that describes the seizure type as well as other signs and symptoms, suspected cause, course or outlook. These terms are called Epilepsy Syndromes and can offer much more information to people with epilepsy and their families about what they may experience or expect. In this week’s newsletter, a study evaluating the effect of a treatment for Rasmussen’s Syndrome is presented as an example of new and hopeful research for some of the infrequent forms of epilepsy. The following information may help you better understand Rasmussen’s syndrome.

What is Rasmussen’s Syndrome?

Rasmussen’s syndrome is a condition that usually begins in childhood and can get progressively worse over time. Seizures are usually one of the first symptoms seen, but other neurological problems may occur as well. The seizures usually are a type of partial seizures but may progress to tonic clonic seizures. Seizure may occur in clusters or episodes of status epilepticus may occur (e.g. when seizures last too long or too many occur at once).

The seizures may affect areas of the brain involved with movement and there may be a gradually worsening of a person’s ability to use an arm and/or leg. The weakness is usually seen on the side of the body opposite from where the seizures arise in the brain. Other neurological problems may be seen such as problems with thinking, attention, memory, speech or other cognitive, behavior or mood problems.

Since there is currently no cure, children with Rasmussen’s will continue to live with this syndrome throughout their life.

How is it treated?

Treatment is first targeted to controlling seizures. However, usually standard seizure medicines don’t work well and multiple seizure medicines may be needed.

Treatment may also target the suspected cause of the disease. Rasmussen’s is an autoimmune disorder, which means that the body develops antibodies that attack its own immune system. This causes an inflammation of the brain (called encephalitis) that causes the seizures and other neurological problems. Steroid medicines may be used as well as other treatments aimed at helping the immune system.

If medicines don’t work, surgery can also be considered. Usually seizures in Rasmussen’s syndrome may arise from large areas of one side of the brain. Thus, surgery may involve removal of large parts of one side of the brain or almost one whole side. This type of surgery is called a hemispherectomy. Since important areas of the brain are affected, for example movement, speech or behavior, people must undergo testing to make sure that the surgery won’t cause further problems.

Removing the areas of the brain that are not working often helps stop the seizures or lessen the severity and number. It may also stop or slow down the development of other neurological problems. Problems that have already occurred from the encephalitis will not go away however. With proper rehabilitation and supports, many children will do well and can relearn some activities and have the chance of a better quality of life.

What should be done if a child is thought to have Rasmussen’s Syndrome?

• If the child has not been seen by an epilepsy specialist, it would be important to have them evaluated to make sure the diagnosis is correct and consider different treatments, including seizure medicines, immune therapy treatments, and surgery.
• An evaluation should include testing and treatment by a range of professionals to identify what types of problems the child is having, what can be done to help, what resources or supports many be needed at home or in school, and to help the child and family learn to manage the situation. Evaluations may include:
  • Physical and occupational therapists
  • Neuropsychologist
  • Speech therapist
  • Educational specialists
  • Social work and psychiatry
  • Nursing
  • Medical professionals
  • Neurosurgeon

Where can information about epilepsy syndromes be found?

• Visit www.epilepsy.com and you’ll find a listing of many epilepsy syndromes under All About Epilepsy and Seizures. Click here at Rasmussen's Syndrome for written information about this disease.

I hope you find this information helpful. From time to time we’ll highlight different epilepsy syndromes and resources for help. Send in your questions or areas you’d like to hear about!