| H01565 | |
| H番号 | 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 | 内分泌代謝疾患 |
| ネットワーク | - |
| 病因遺伝子 | SLC19A2 [HSA:10560] [KO:K14610] |
| 病原体 | - |
| 環境要因 | - |
| 発癌物質 | - |
| 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] |
| コメント | - |
| リンク | ICD-11: 5B5A.10 ICD-10: E51.2 MeSH: D014899 OMIM: 277730 |
| 文献 | PMID:26004883 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 PMID:24701066 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 PMID:16015585 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 PMID:19151162 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 |