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Rasmussen Encephalitis: An Update.

Karla C Cay-Martinez¹, Richard A Hickman², Guy M McKhann Ii³

¹Department of Neurology, Columbia University Irving Medical Center, New York, New York.

Seminars in Neurology

|March 19, 2020

Summary

This summary is machine-generated.

Rasmussen encephalitis (RE) is a rare pediatric epilepsy causing severe neurological deficits. Hemispheric disconnection offers seizure freedom but has side effects, prompting research into alternatives.

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Area of Science:

Pediatric Neurology
Epileptology
Neuroimmunology

Background:

Rasmussen encephalitis (RE) is a rare, progressive pediatric epilepsy characterized by unilateral brain inflammation.
It leads to medically refractory focal epilepsy, hemiparesis, and cognitive decline.
The exact cause involves a cell-mediated immune response, but the specific antigen is unknown.

Purpose of the Study:

To review the current understanding of Rasmussen encephalitis, including its clinical presentation, pathology, and treatment.
To highlight the challenges in diagnosis and management of RE.
To discuss the efficacy and limitations of current treatments like hemispheric disconnection and emerging immunomodulatory therapies.

Main Methods:

Literature review of studies on Rasmussen encephalitis.
Analysis of clinical, pathological, and treatment data.
Synthesis of existing knowledge and identification of research gaps.

Main Results:

Rasmussen encephalitis typically affects one cerebral hemisphere but can cause secondary effects in the contralateral hemisphere.
Hemispheric disconnection is effective in achieving seizure freedom in 70-80% of patients but results in hemiparesis and hemianopia.
Evidence for disease modification with current immunomodulatory therapies is limited.

Conclusions:

Rasmussen encephalitis presents significant diagnostic and management challenges.
Hemispheric disconnection is a viable treatment for seizure control but has functional consequences.
Further research is needed to identify the inciting antigen and develop more effective disease-modifying therapies.