KEGG   DISEASE: ラスムッセン脳炎
エントリ  
H01812                                                             
名称    
ラスムッセン脳炎;
ラスムッセン症候群
概要    
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.
カテゴリ  
免疫系疾患; 神経系疾患
階層分類  
ヒト疾患 [BR:jp08402]
 免疫系疾患
  その他の免疫系疾患
   H01812  ラスムッセン脳炎
 神経系疾患
  その他の神経系および感覚器系疾患
   H01812  ラスムッセン脳炎
ICD-11 による疾患分類 [BR:jp08403]
 08 神経系の疾患
  てんかんまたは発作
   8A62  てんかん性脳症
    H01812  ラスムッセン脳炎
指定難病 [jp08407.html]
 H01812
関連パスウェイ
hsa04660  T cell receptor signaling pathway
病因遺伝子 
CTLA4 (polymorphism) [HSA:1493] [KO:K06538]
PDCD1 (polymorphism) [HSA:5133] [KO:K06744]
リンク   
ICD-11: 8A62.Y
ICD-10: G04.8
MeSH: D004660
文献    
  著者
Varadkar S, Bien CG, Kruse CA, Jensen FE, Bauer J, Pardo CA, Vincent A, Mathern GW, Cross JH
  タイトル
Rasmussen's encephalitis: clinical features, pathobiology, and treatment advances.
  雑誌
Lancet Neurol 13:195-205 (2014)
DOI:10.1016/S1474-4422(13)70260-6
文献    
  著者
Quesada CM, Urbach H, Elger CE, Bien CG
  タイトル
Rasmussen encephalitis with ipsilateral brain stem involvement in an adult patient.
  雑誌
BMJ Case Rep 2009:bcr08.2008.0648 (2009)
DOI:10.1136/bcr.08.2008.0648
文献    
  著者
Bien CG, Schramm J
  タイトル
Treatment of Rasmussen encephalitis half a century after its initial description: promising prospects and a dilemma.
  雑誌
Epilepsy Res 86:101-12 (2009)
DOI:10.1016/j.eplepsyres.2009.06.001
文献    
  著者
Takahashi Y, Mogami Y, Mine J, Imai K, Koide Y, Matsuda K, Akasaka N, Konishi T, Imamura A, Inoue Y
  タイトル
Genetic variations of immunoregulatory genes associated with Rasmussen syndrome.
  雑誌
Epilepsy Res 107:238-43 (2013)
DOI:10.1016/j.eplepsyres.2013.09.004
文献    
  著者
Mantegazza R, Bernasconi P, Baggi F, Spreafico R, Ragona F, Antozzi C, Bernardi G, Granata T
  タイトル
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.
  雑誌
J Neuroimmunol 131:179-85 (2002)
DOI:10.1016/S0165-5728(02)00261-8
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