KEGG   DISEASE: Wernicke encephalopathy
Entry
H01565                      Disease                                
Name
Wernicke encephalopathy;
Wernicke-Korsakoff syndrome
Description
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).
Category
Nervous system disease; Endocrine and metabolic disease
Brite
Human diseases [BR:br08402]
 Nervous system diseases
  Other nervous and sensory system diseases
   H01565  Wernicke encephalopathy
 Endocrine and metabolic diseases
  Other endocrine and metabolic diseases
   H01565  Wernicke encephalopathy
Human diseases in ICD-11 classification [BR:br08403]
 05 Endocrine, nutritional or metabolic diseases
  Nutritional disorders
   Undernutrition
    5B5A  Vitamin B1 deficiency
     H01565  Wernicke encephalopathy
Related
pathway
hsa04977  Vitamin digestion and absorption
Gene
SLC19A2 [HSA:10560] [KO:K14610]
Drug
Thiamine hydrochloride [DR:D02094]
Other DBs
ICD-11: 5B5A.10
ICD-10: E51.2
MeSH: D014899
OMIM: 277730
Reference
  Authors
Yahia M, Najeh H, Zied H, Khalaf M, Salah AM, Sofienne BM, Laidi B, Hamed J, Hayenne M
  Title
Wernicke's encephalopathy: A rare complication of hyperemesis gravidarum.
  Journal
Anaesth Crit Care Pain Med 34:173-7 (2015)
DOI:10.1016/j.accpm.2014.09.005
Reference
  Authors
Kantor S, Prakash S, Chandwani J, Gokhale A, Sarma K, Albahrani MJ
  Title
Wernicke's encephalopathy following hyperemesis gravidarum.
  Journal
Indian J Crit Care Med 18:164-6 (2014)
DOI:10.4103/0972-5229.128706
Reference
  Authors
Guerrini I, Thomson AD, Cook CC, McQuillin A, Sharma V, Kopelman M, Reynolds G, Jauhar P, Harper C, Gurling HM
  Title
Direct genomic PCR sequencing of the high affinity thiamine transporter (SLC19A2) gene identifies three genetic variants in Wernicke Korsakoff syndrome (WKS).
  Journal
Am J Med Genet B Neuropsychiatr Genet 137B:17-9 (2005)
DOI:10.1002/ajmg.b.30194
Reference
  Authors
Kopelman MD, Thomson AD, Guerrini I, Marshall EJ
  Title
The Korsakoff syndrome: clinical aspects, psychology and treatment.
  Journal
Alcohol Alcohol 44:148-54 (2009)
DOI:10.1093/alcalc/agn118
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