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Wernicke-Korsakoff syndrome
Other Resources UpToDate PubMed

Wernicke-Korsakoff syndrome

Contributors: Alec T. Jackson, Kathryn Bartlett DO, Richard L. Barbano MD, PhD, Gerald F. O'Malley DO
Other Resources UpToDate PubMed

Synopsis

Emergent Care / Stabilization:
Acute Wernicke encephalopathy is a clinical diagnosis, and prompt treatment can produce recovery. The treatment is high-dose thiamine replacement to prevent progression to Korsakoff syndrome.

Diagnosis Overview:
Wernicke-Korsakoff syndrome is a collection of clinical manifestations that result from vitamin B1 (thiamine) deficiency. The acute clinical manifestations of thiamine deficiency are known as Wernicke encephalopathy (WE). The classic clinical triad for diagnosis is confusion, ophthalmoplegia, and ataxia. Korsakoff syndrome occurs due to chronic thiamine deficiency and is an irreversible manifestation of acute symptoms. Most often, this condition is noted in patient populations with inadequate nutrient absorption or intake of thiamine. Wernicke-Korsakoff syndrome can occur from any condition leading to thiamine deficiency. It is most commonly seen in patients with alcohol use disorder, anorexia nervosa and other eating disorders, a history of gastrointestinal surgery, conditions with hyperemesis, and any other condition causing malnutrition.

WE presents with confusion (disorientation, inattention, memory dysfunction), ophthalmoplegia (horizontal and vertical nystagmus), and ataxia (unsteady, wide-based gait with short-spaced steps). Acute WE is a clinical diagnosis, and prompt treatment can produce recovery. The treatment is high-dose thiamine replacement to prevent Korsakoff syndrome.

Korsakoff syndrome, also known as alcohol amnestic disorder, is a late neuropsychiatric manifestation of chronic WE. Memory loss is the most serious effect; the patient usually has severe impairment of short-term memory. Confabulations are also a common characteristic of patients with Korsakoff syndrome. In alcohol use disorder, thiamine deficiency and alcohol's direct neurotoxic effects combine to produce deficits in anterograde and retrograde memory and apathy. Recovery in Korsakoff syndrome patients is uncommon.

Codes

ICD10CM:
E51.2 – Wernicke's encephalopathy
F04 – Amnestic disorder due to known physiological condition
F10.26 – Alcohol dependence with alcohol-induced persisting amnestic disorder

SNOMEDCT:
21007002 – Wernicke's disease
69482004 – Korsakoff's psychosis

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Like all clinically diagnosed neurologic conditions, the differential is broad. The history and clinical picture can be used to narrow down this differential in conjunction with additional laboratory analysis, neurologic imaging, and possibly a lumbar puncture.

Toxicologic:
  • Serotonin toxicity
  • Alcohol intoxication (see Toxic alcohol poisoning)
  • Illicit drug use or adverse drug reactions
Metabolic:
  • Hypoglycemia
  • Hyperglycemia
  • Hepatic encephalopathy
  • Hyponatremia
  • Inborn errors of metabolism in children
Psychiatric:
  • Delirium
  • Psychosis or Drug-induced psychosis
Endocrine:
  • Thyroid disease (see Thyroiditis)
  • Other endocrine deficiencies – parathyroid, pancreas, pituitary, adrenal
Structural neurologic diseases:
  • Epidural intracranial hematoma
  • Subdural hematoma
  • Cerebral stroke (see, eg, Basilar artery occlusion)
  • Cerebral stroke
  • Third ventricle tumor
  • Paraneoplastic encephalomyelitis
  • Acute demyelinating encephalomyelitis
  • Normal pressure hydrocephalus
Neurodegenerative diseases:
  • Dementia
  • Alzheimer disease
  • Frontotemporal lobar degeneration
  • Corticobasal syndrome
  • Progressive supranuclear palsy
  • Miller-Fisher variant of Acute inflammatory demyelinating polyneuropathy
  • Seizure (eg, temporal lobe epilepsy) or post-ictal state
Infectious diseases:
  • Infectious Encephalitis (eg, Herpes simplex virus encephalitis)
  • Tertiary syphilis
  • Creutzfeldt-Jakob disease
  • Human immunodeficiency virus disease / Acquired immune deficiency syndrome
Vitamin deficiency / hematologic:
  • Vitamin B12 deficiency
  • Folate deficiency
  • Pernicious anemia

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed:03/29/2023
Last Updated:03/30/2023
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Wernicke-Korsakoff syndrome
A medical illustration showing key findings of Wernicke-Korsakoff syndrome (Wernicke Encephalopathy)
Copyright © 2024 VisualDx®. All rights reserved.