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Rasmussen Encephalitis

A rare progressive unilateral encephalitis of childhood causing intractable epilepsy.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Nöroloji department. Book Appointment →

What is Rasmussen Encephalitis?

Rasmussen encephalitis is a rare progressive autoimmune disease that begins between ages 2-12 (mean 6) and selectively involves a single cerebral hemisphere; incidence is 0.2-0.4/million children.

T-cell–mediated immune attack against neurons and glial cells causes progressive cortical atrophy, intractable focal motor seizures (especially epilepsia partialis continua), progressive hemiparesis, hemianopia, and cognitive decline.

Diagnosis is established by MRI (progressive unilateral atrophy), EEG (lateralized slowing), and brain biopsy. Treatment includes IVIG, plasmapheresis, immunosuppressants (tacrolimus), high-dose steroids, and ultimately functional hemispherectomy. Hemispherectomy stops seizures in 65-85%.

Symptoms

Refractory focal motor seizures
Epilepsia partialis continua
Progressive hemiparesis
Hemianopia (loss of half visual field)
Loss of language (in dominant hemisphere)
Cognitive decline
Behavioral problems

Risk Factors

Childhood (peak 2-12)
Recent viral infection (hypothesis)
GluR3 receptor antibody (some patients)
T-cell–mediated autoimmune mechanism
Triggering vaccine or trauma (rare)
No known genetic predisposition
Equal sex distribution

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • First focal motor seizure (especially child >2)
  • Cluster of long seizures
  • Progressive hemibody weakness
  • Loss of language
  • Behavioral and cognitive change

Treatment Methods

01
Brain MRI (progressive unilateral atrophy)
02
EEG monitoring
03
IVIG (2 g/kg every 4 weeks)
04
Plasmapheresis
05
Tacrolimus or rituximab
06
Anticonvulsants (multiple combinations)
07
Functional hemispherectomy (definitive)

Which Department to Visit?

You can visit our Nöroloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.