Rethinking Seizure Care Blog

Rasmussen’s Syndrome in Children

Posted by RSC Diagnostics on Feb 10, 2021

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Rasmussen’s syndrome is surrounded by mystery and is a rare form of epilepsy that has features of an autoimmune disorder where immune system cells fight against brain cell receptors causing inflammation and damage. Some believe it may stem from some type of viral infection. Rasmussen’s syndrome generally occurs in children and symptoms begin to show between 14 months to 14 years of age.

Part of the mystery surrounding Rasmussen’s syndrome is that it does not have any type of pattern and seizures do not tend to cluster in certain areas of the brain. It does not run in families so it does not likely have a genetic cause. This is why most believe that a viral infection is the culprit. 

What Are The symptoms?

Some of the early signs and symptoms are seizures and weakness in one or more of the limbs. As Rasmussen’s syndrome worsens, it can cause a number of neurological problems and recurrent seizures. Hemiparesis or ‘hemiplegia’ (progressive weakening on one side of the body) is common and memory/thinking impairments may be observed. Sometimes a child is unable to speak properly. These problems will generally start to manifest between one and three years, after the start of the seizures.

Can it Be Treated?

Currently, there are few treatment options for Rasmussen’s syndrome; anti-seizure medications or AEDs are not always helpful. In some cases, anti-inflammatory medication have been helpful, but whether they are truly effective is unknown since few studies have been performed.

Some patients may need to undergo a surgical procedure known as a functional hemispherectomy, where one-half of the brain is removed. It is a radical treatment and performed only on children who experience severe weakness as well as a loss of touch and vision on the side of the body opposite the involved hemisphere of the brain. This is a very difficult and drastic procedure, but it has been proven helpful in stopping the seizures and arresting further mental impairment.

After the surgery, most children can function very well. They are able to walk and run, although many of them will have a limp. They may also experience a reduction in their fine motor skills. However, most will be able to regain a large amount of the skills they lost, including language skills. Hemispherectomies have the best results for children under the age of six.

The good news is that Rasmussen’s syndrome is not often fatal, but that does not mean that it will be easy to live with. Even with treatment, the conditions can still be very difficult. Children tend to have a large number of seizures, the weakness and cognitive impairment often gets worse and MRI and CT scans may reveal atrophy of the brain.

Talk With Your Doctor

If your child is suffering from Rasmussen’s syndrome, your neurologist can help you to understand the treatment options appropriate for your child. Although surgical treatment is a huge step, it could prove to be beneficial to your child.





Topics: Pediatric Epilepsy