Is Rasmussen encephalitis fatal?

Such patients may live for many years after cessation of the “active” phase of the disease. However, if seizures continue and neurologic deterioration continues, the disease can prove fatal. Figure 13.1 illustrates the typical clinical course of Rasmussen’s encephalitis.

How common is Rasmussen encephalitis?

The condition is very rare with an estimated 200-500 cases worldwide, and it typically affects children between the ages of 2-10. The inflammation association with Rasmussen’s encephalitis is thought to get progressively worse, peak and then subside, leaving stable function but permanent brain damage.

Is Rasmussen encephalitis autoimmune?

Rasmussen’s encephalitis has features of an autoimmune disease in which immune system cells enter the brain and cause inflammation and damage. Research is ongoing into the causes of this rare disease.

How is Rasmussen encephalitis diagnosed?

Rasmussen encephalitis may be diagnosed clinically based upon a thorough clinical evaluation, a detailed patient history, and a complete neurological evaluation including advanced techniques such as electroencephalography (EEG), and magnetic resonance imaging (MRI).

Is there a cure for Rasmussen encephalitis?

Surgery. Surgery still remains the only cure for the seizures caused by Rasmussen’s encephalitis. This has functional consequences because the only effective surgery remains complete disconnection of the affected hemisphere (hemidisconnection), either as (functional) hemispherectomy or hemispherotomy.

Does EEG show past seizure?

An EEG can usually show if you are having a seizure at the time of the test, but it can’t show what happens to your brain at any other time. So even though your test results might not show any unusual activity it does not rule out having epilepsy.

Is encephalitis a rare disease?

In all its forms, encephalitis is incredibly rare: herpes simplex encephalitis (HSE), for instance, affects approximately one in 1,000,000 children. Although there are clear treatment routes available, viral encephalitis is incredibly destructive.

How old do you have to be to have Rasmussen’s encephalitis?

Rasmussen’s encephalitis (RE) is a very rare, chronic inflammatory neurological disease that usually affects only one hemisphere (half) of the brain. It most often occurs in children under the age of 10 but can also affect adolescents and adults.

What happens to the brain with Rasmussen encephalitis?

As a result, the patient usually experiences frequent episodes of uncontrolled electrical disturbances in the brain that cause epileptic seizures (epilepsy), and progressive cerebral destruction.

Where does EPC occur in Rasmussen encephalitis?

EPC is characterized by a rapid, rhythmic succession of contractions and relaxations of a muscle or muscle group (myoclonus), particularly of the arms, legs, and face, that may occur singularly or in a repetitive, continuous series. In Rasmussen this occurs consistently on one side of the body opposite the side of the inflammation.

Is there a biomarker for Rasmussen’s encephalitis?

Advances in neuroimaging suggest that progression of the inflammatory process seen with MRI might be a good biomarker in Rasmussen’s encephalitis. For many patients, families, and doctors, choosing the right time to move from medical management to surgery is a real therapeutic dilemma.