KEGG   DISEASE: ウェルニッケ脳症
エントリ  
H01565                                                             
名称    
ウェルニッケ脳症;
ウェルニッケ・コルサコフ症候群
概要    
Wernicke encephalopathy is a rare but serious neurological pathology due to a vitamin B1 deficiency. It is commonly associated with heavy alcohol consumption. Other clinical associations are with hyperemesis gravidarum, starvation, and prolonged intravenous feeding. Most patients present with the triad of ophthalmoplegia, ataxia, and confusion. It can be associated with life-threatening complication like central pontine myelinolysis. When Wernicke encephalopathy is suspected, treatment with high-dose parenteral thiamine (vitamin B1) should be given. If it is not treated promptly or if treatment is inadequate it can lead to death or to the chronic memory disorder, Korsakoff syndrome. Recent studies have suggested that an association exists between Wernicke encephalopathy and genetic polymorphisms in the thiamine transporter (SLC19A2).
カテゴリ  
神経系疾患; 内分泌代謝疾患
階層分類  
ヒト疾患 [BR:jp08402]
 神経系疾患
  その他の神経系および感覚器系疾患
   H01565  ウェルニッケ脳症
 内分泌代謝疾患
  その他の内分泌代謝疾患
   H01565  ウェルニッケ脳症
ICD-11 による疾患分類 [BR:jp08403]
 05 内分泌, 栄養, 代謝の疾患
  栄養疾患
   低栄養
    5B5A  ビタミンB1欠乏
     H01565  ウェルニッケ脳症
関連パスウェイ
hsa04977  Vitamin digestion and absorption
病因遺伝子 
SLC19A2 [HSA:10560] [KO:K14610]
治療薬   
チアミン塩化物塩酸塩 [DR:D02094]
ベンフォチアミン [DR:D01255]
オクトチアミン [DR:D01184]
ビスベンチアミン [DR:D01284]
プロスルチアミン [DR:D03286]
セトチアミン塩酸塩水和物 [DR:D03292]
チアミンジスルフィド [DR:D03299]
フルスルチアミン [DR:D03319]
フルスルチアミン塩酸塩 [DR:D03321]
チアミンジスルフィド硝化物 [DR:D04890]
リンク   
ICD-11: 5B5A.10
ICD-10: E51.2
MeSH: D014899
OMIM: 277730
文献    
  著者
Yahia M, Najeh H, Zied H, Khalaf M, Salah AM, Sofienne BM, Laidi B, Hamed J, Hayenne M
  タイトル
Wernicke's encephalopathy: A rare complication of hyperemesis gravidarum.
  雑誌
Anaesth Crit Care Pain Med 34:173-7 (2015)
DOI:10.1016/j.accpm.2014.09.005
文献    
  著者
Kantor S, Prakash S, Chandwani J, Gokhale A, Sarma K, Albahrani MJ
  タイトル
Wernicke's encephalopathy following hyperemesis gravidarum.
  雑誌
Indian J Crit Care Med 18:164-6 (2014)
DOI:10.4103/0972-5229.128706
文献    
  著者
Guerrini I, Thomson AD, Cook CC, McQuillin A, Sharma V, Kopelman M, Reynolds G, Jauhar P, Harper C, Gurling HM
  タイトル
Direct genomic PCR sequencing of the high affinity thiamine transporter (SLC19A2) gene identifies three genetic variants in Wernicke Korsakoff syndrome (WKS).
  雑誌
Am J Med Genet B Neuropsychiatr Genet 137B:17-9 (2005)
DOI:10.1002/ajmg.b.30194
文献    
PMID:19151162 (drug)
  著者
Kopelman MD, Thomson AD, Guerrini I, Marshall EJ
  タイトル
The Korsakoff syndrome: clinical aspects, psychology and treatment.
  雑誌
Alcohol Alcohol 44:148-54 (2009)
DOI:10.1093/alcalc/agn118
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