| H01812 | |
| H number | H01812 |
| Name | Rasmussen encephalitis; Rasmussen syndrome |
| Description | 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 |
| Network | - |
| Gene | CTLA4 (polymorphism) [HSA:1493] [KO:K06538] PDCD1 (polymorphism) [HSA:5133] [KO:K06744] |
| Pathogen | - |
| Env factor | - |
| Carcinogen | - |
| Drug | - |
| Comment | - |
| Other DBs | ICD-11: 8A62.Y ICD-10: G04.8 MeSH: D004660 |
| Reference | PMID:24457189 AUTHORS Varadkar S, Bien CG, Kruse CA, Jensen FE, Bauer J, Pardo CA, Vincent A, Mathern GW, Cross JH TITLE Rasmussen's encephalitis: clinical features, pathobiology, and treatment advances. JOURNAL Lancet Neurol 13:195-205 (2014) DOI:10.1016/S1474-4422(13)70260-6 PMID:21686702 AUTHORS Quesada CM, Urbach H, Elger CE, Bien CG TITLE Rasmussen encephalitis with ipsilateral brain stem involvement in an adult patient. JOURNAL BMJ Case Rep 2009:bcr08.2008.0648 (2009) DOI:10.1136/bcr.08.2008.0648 PMID:19615863 AUTHORS Bien CG, Schramm J TITLE Treatment of Rasmussen encephalitis half a century after its initial description: promising prospects and a dilemma. JOURNAL Epilepsy Res 86:101-12 (2009) DOI:10.1016/j.eplepsyres.2009.06.001 PMID:24269013 AUTHORS Takahashi Y, Mogami Y, Mine J, Imai K, Koide Y, Matsuda K, Akasaka N, Konishi T, Imamura A, Inoue Y TITLE Genetic variations of immunoregulatory genes associated with Rasmussen syndrome. JOURNAL Epilepsy Res 107:238-43 (2013) DOI:10.1016/j.eplepsyres.2013.09.004 PMID:12458050 AUTHORS Mantegazza R, Bernasconi P, Baggi F, Spreafico R, Ragona F, Antozzi C, Bernardi G, Granata T TITLE 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. JOURNAL J Neuroimmunol 131:179-85 (2002) DOI:10.1016/S0165-5728(02)00261-8 |