Wernicke-Korsakoff Syndrome

The-Dangers-of-Korsakoff-Syndrome-2

Wernicke-Korsakoff Syndrome (WKS) classically, but not always, presents with the clinical triad of confusion, ataxia, and nystagmus. It is best conceptualized as 2 distinct syndromes, with one being characterized by an acute/subacute confusional state and often reversible findings of Wernicke encephalopathy (a type of delirium) and the other by persistent and irreversible findings of Korsakoff dementia. (See Clinical and Workup.)

In 1881, Carl Wernicke first described an illness that consisted of paralysis of eye movements, ataxia, and mental confusion, in 3 patients. The patients, 2 males with alcoholism and a female with persistent vomiting following sulfuric acid ingestion, exhibited these findings, developed coma, and eventually died. On autopsy, Wernicke detected punctate hemorrhages affecting the gray matter around the third and fourth ventricles and aqueduct of Sylvius. He felt these to be inflammatory and therefore named the disease polioencephalitis hemorrhagica superioris.

Sergei Korsakoff, a Russian psychiatrist, described the disturbance of memory in the course of long-term alcoholism in a series of articles from 1887-1891. He termed this syndrome psychosis polyneuritica, believing that these typical memory deficits, in conjunction with polyneuropathy, represented different facets of the same disease. In 1897, Murawieff first postulated that a single etiology was responsible for both syndromes.

Wernicke-Korsakoff Syndrome (WKS) classically, but not always, presents with the clinical triad of confusion, ataxia, and nystagmus. It is best conceptualized as 2 distinct syndromes, with one being characterized by an acute/subacute confusional state and often reversible findings of Wernicke encephalopathy (a type of delirium) and the other by persistent and irreversible findings of Korsakoff dementia. (See Clinical and Workup.)

In 1881, Carl Wernicke first described an illness that consisted of paralysis of eye movements, ataxia, and mental confusion, in 3 patients. The patients, 2 males with alcoholism and a female with persistent vomiting following sulfuric acid ingestion, exhibited these findings, developed coma, and eventually died. On autopsy, Wernicke detected punctate hemorrhages affecting the gray matter around the third and fourth ventricles and aqueduct of Sylvius. He felt these to be inflammatory and therefore named the disease polioencephalitis hemorrhagica superioris.

Sergei Korsakoff, a Russian psychiatrist, described the disturbance of memory in the course of long-term alcoholism in a series of articles from 1887-1891. He termed this syndrome psychosis polyneuritica, believing that these typical memory deficits, in conjunction with polyneuropathy, represented different facets of the same disease. In 1897, Murawieff first postulated that a single etiology was responsible for both syndromes.

Etiology

A deficiency of thiamine (vitamin B-1) is responsible for the symptom complex manifested in Wernicke-Korsakoff syndrome, and any condition resulting in a poor nutritional state places patients at risk. The structural lesions associated with Wernicke-Korsakoff syndrome are more likely to manifest in patients with malnutrition. However, it is crucial to recognize that Wernicke-Korsakoff syndrome also appears in patients without exposure to alcohol. The occurrence of WKS in patients without alcohol use disorders is well described in the literature and cited in the section below.

The following are associated with Wernicke-Korsakoff syndrome:

  • Chronic alcoholism – There may be a synergistically destructive effect of alcohol and thiamine deficiency that contributes to the Wernicke-Korsakoff syndrome. Alcohol interferes with active gastrointestinal transport, and chronic liver disease leads to decreased activation of thiamine pyrophosphate from thiamine, as well as decreased capacity of the liver to store thiamine.
  • Bariatric surgery – Wernicke encephalopathy can present as early as 2 weeks after surgery; recovery typically occurs within 3-6 months of initiation of therapy but may be incomplete if this syndrome is not recognized promptly and treated (the highest risk is in young women with vomiting)
  • Nutritional deficiency and certain diets
  • Starvation – Persons with anorexia nervosa,schizophrenia, or terminal cancer; prisoners of war
  • Thiamine-deficient formula
  • Hyperemesis gravidarum – In a study of 49 cases of Wernicke encephalopathy in pregnancy, pregnancy loss attributable to Wernicke encephalopathy was nearly 48% [12]
  • Gastric malignancy, inflammatory bowel disease
  • Intestinal obstruction, including abdominal abscess
  • Plastic surgery – Panniculectomy
  • Systemic diseases – Malignancy, disseminated tuberculosis, acquired immunodeficiency syndrome (AIDS),  uremia, stem cell transplantation
  • Iatrogenic – Intravenous hyperalimentation (without thiamine supplementation), refeeding after starvation, chronic hemodialysis
  • Encephalitic infections or infarctions affecting the mammillary bodies or hippocampus Rare reports – Rare reports
  • Infants breastfed by mothers with inadequate intake of thiamine
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