Also known as Rasmussen’s Encephalitis, this rare form of epilepsy is the possible result of an auto-immune reaction or an infection. The exact cause is not fully known.
Named after Theodore Rasmussen, a neurosurgeon who first described the encephalitis in 1958, scientists are still studying the causes.
Rasmussen's encephalitis is a rare disease that causes inflammation in one side of the brain, leading to seizures, brain damage, and loss of function in that hemisphere.
Rasmussen’s Syndrome most commonly affects children between the ages of 2 and 10, but it can also affect adolescents and adults as well.
Often the first sign of the onset is a seizure, so an EEG will frequently be the patient’s first line of treatment towards their diagnosis. Followed by an MRI for imaging.
Seizure types could be generalized tonic-clonic or most commonly focal. Patient’s can also clinically have weakness in their extremities as an early sign as well.
Over the course of months to a few years this condition involves a worsening inflammation (encephalitis) of one hemisphere of the brain. This inflammation will lead to worsening seizures and progressive and permanent damage to the hemisphere.
There’s no cure for Rasmussen’s encephalitis. Instead, the goal of treatment is to manage symptoms and inflammation.
Treatment may include:
Antiseizure medications to decrease the frequency and severity.
Immunotherapy given at the onset may help manage seizures or prevent further immune-related brain damage.
Hemispherectomy by surgically removing half of the brain. Most children who have this surgery have already lost the function of the affected hemisphere.
As with any condition early diagnosis can be beneficial to help manage the progressive symptoms.
As Neurodiagnostic Technologists being aware of the condition and it’s coorelated EEG findings is essential in being a good resource for our patients and their families.
Be sure to continue to follow along on the blog for more information in our EEG in Epilepsy series.
Roya Tompkins, MS, REEG/EP T, RPSGT
Resources: Cleveland Clinic
Epilepsy Foundation
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