What is the definition of Rasmussen Encephalitis?

Rasmussen encephalitis is a chronic inflammatory neurological disease that usually affects only one hemisphere of the brain. It most often occurs in children under the age of 10, although adolescents and adults may also be affected. Rasmussen encephalitis is characterized by frequent and severe seizures, loss of motor skills and speech, paralysis on one side of the body (hemiparesis), inflammation of the brain (encephalitis), and mental deterioration. While the cause of Rasmussen encephalitis is unknown, there is evidence that in many patients it is an autoimmune disorder. Immune therapy and surgery may be used for treatment.

What are the alternative names for Rasmussen Encephalitis?

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What are the causes for Rasmussen Encephalitis?

The underlying cause of Rasmussen encephalitis remains unknown. Current evidence suggests that autoimmune processes are responsible for the damage to the brain cells. In most patients, the trigger of the abnormal immune response is unclear, although it may follow an otherwise minor bacterial or viral infection or head injury.  Rasmussen encephalitis is a sporadic disease. It has not been associated with any particular environments or populations and cannot be caught from others. Research is underway to help better understand the underlying cause.

What are the current treatments for Rasmussen Encephalitis?

Anti-epileptic drugs are usually not effective in controlling the seizures associated with Rasmussen encephalitis. Recent studies have shown some success with treatments that suppress or modulate the immune system, in particular those that use corticosteroids, intravenous immunoglobulin, or tacrolimus. Surgery to control seizures may be performed in later stages of the disease when neurological deficits stabilize. Surgical procedures, such as functional hemispherectomy (removal of half of the brain) and hemispherotomy (a surgical procedure for hemispheric disconnection), may reduce the frequency of seizures and also improve behavior and cognitive abilities.   
  • Journal: Neurology(R) neuroimmunology & neuroinflammation
  • Published —
Infratentorial MRI Findings in Rasmussen Encephalitis Suggest Primary Cerebellar Involvement.
  • Condition: anti-GABAB R Encephalitis
  • Journal: Medicine
  • Treatment Used: Etoposide with Immunotherapy
  • Number of Patients: 1
  • Published —
This case report describes a patient with anti-GABAB R encephalitis that was treated using etoposide and immunotherapy.