KEGG   DISEASE: Rasmussen encephalitis
Entry
H01812                      Disease                                
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
Brite
Human diseases [BR:br08402]
 Immune system diseases
  Other immune system diseases
   H01812  Rasmussen encephalitis
 Nervous system diseases
  Other nervous and sensory system diseases
   H01812  Rasmussen encephalitis
Human diseases in ICD-11 classification [BR:br08403]
 08 Diseases of the nervous system
  Epilepsy or seizures
   8A62  Epileptic encephalopathies
    H01812  Rasmussen encephalitis
Related
pathway
hsa04660  T cell receptor signaling pathway
Gene
CTLA4 (polymorphism) [HSA:1493] [KO:K06538]
PDCD1 (polymorphism) [HSA:5133] [KO:K06744]
Other DBs
ICD-11: 8A62.Y
ICD-10: G04.8
MeSH: D004660
Reference
  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
Reference
  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
Reference
PMID:19615863 (drug)
  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
Reference
  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
Reference
PMID:12458050 (marker)
  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
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