Rasmussen encephalitis is a rare chronic neurological disorder, characterised by unilateral inflammation of the cerebral cortex, drug-resistant epilepsy, and progressive neurological and cognitive deterioration. It predominantly affects children, with the onset in adults having a milder course. Infection occurring around two weeks prior to onset is observed in 38% of the patients. There is evidence of autoimmune involvement in the pathogenesis. The immunopathology of this disease is attributed mainly to activated cytotoxic T cells. It has been reported that genetic variations of immunoregulatory genes associated with Rasmussen syndrome.
Category
Immune system disease; Nervous system disease
Brite
Human diseases in ICD-11 classification [BR:br08403]
08 Diseases of the nervous system
Epilepsy or seizures
8A62 Epileptic encephalopathies
H01812 Rasmussen encephalitis
Antibodies against GluR3 peptides are not specific for Rasmussen's encephalitis but are also present in epilepsy patients with severe, early onset disease and intractable seizures.
