Orphanet: Rasmussen subacute encephalitis
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Rasmussen subacute encephalitis

Disease definition

A rare inflammatory and autoimmune disease with epilepsy characterized by unilateral hemispheric atrophy, associated with drug-resistant focal epilepsy, progressive hemiplegia, and cognitive decline. The disease mainly affects children and begins with a prodromal period with mild hemiparesis or infrequent seizures lasting up to several years. The acute stage is marked by frequent seizures arising from one cerebral hemisphere, followed by a residual stage with persistent severe neurological deficits and relapsing epilepsy.

ORPHA:1929

Classification level: Disorder
  • Synonym(s):
    • Rasmussen syndrome
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: Childhood
  • ICD-10: G04.8
  • OMIM: -
  • UMLS: C0393484  C2930868
  • MeSH: C535291
  • GARD: -
  • MedDRA: -

Detailed information

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