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Wernicke–Korsakoff Syndrome

Medically Reviewed

A neurological disorder caused by thiamine deficiency, often seen in alcoholics, involving confusion and memory loss.

Overview

Wernicke–Korsakoff syndrome (WKS) is a serious neurological disorder that results from a deficiency of thiamine (vitamin B1), typically associated with chronic alcoholism. It is considered a two-stage condition comprising Wernicke's encephalopathy and Korsakoff's psychosis. Wernicke’s encephalopathy is the acute phase, characterized by confusion, ataxia, and ophthalmoplegia (eye movement abnormalities). If left untreated, it may progress to the chronic stage—Korsakoff’s psychosis—which involves severe memory deficits and confabulation. While Wernicke’s encephalopathy can be reversed with prompt treatment, Korsakoff’s psychosis is often permanent. Early recognition and thiamine supplementation are crucial to prevent irreversible brain damage.

Causes

The primary cause of Wernicke–Korsakoff syndrome is a deficiency of thiamine (vitamin B1), which is essential for carbohydrate metabolism and proper nerve function. The most common underlying factors include:

  • Chronic alcoholism: The leading cause of WKS due to poor dietary intake, reduced thiamine absorption, and impaired liver function affecting thiamine storage.

  • Malnutrition: Inadequate nutrition due to eating disorders, extreme diets, or gastrointestinal conditions such as Crohn's disease or gastric bypass surgery.

  • Prolonged vomiting: As seen in hyperemesis gravidarum or gastrointestinal obstruction.

  • Increased thiamine demand: During periods of high metabolic stress, such as infections, sepsis, or after carbohydrate loading in thiamine-deficient individuals.

Without sufficient thiamine, glucose metabolism in the brain is impaired, leading to neuronal injury, particularly in the thalamus, mammillary bodies, and brainstem.

Symptoms

The symptoms of Wernicke–Korsakoff syndrome can be grouped into two phases: the acute Wernicke’s encephalopathy phase and the chronic Korsakoff’s psychosis phase.

Wernicke’s Encephalopathy (Acute Phase)

This triad of symptoms is considered hallmark but is not always present in all patients:

  • Confusion: Disorientation, inattentiveness, and difficulties with concentration.

  • Ophthalmoplegia: Abnormal eye movements, including nystagmus (involuntary eye movement), double vision, or paralysis of the lateral rectus muscle.

  • Ataxia: Unsteady gait and difficulty with coordination, often due to cerebellar involvement.

Korsakoff’s Psychosis (Chronic Phase)

Develops in about 80% of patients who survive Wernicke’s encephalopathy and includes:

  • Anterograde amnesia: Inability to form new memories.

  • Retrograde amnesia: Loss of previously formed memories.

  • Confabulation: Fabrication of stories to fill memory gaps, often without awareness of doing so.

  • Lack of insight and apathy: Reduced emotional responsiveness and motivation.

Diagnosis

Wernicke–Korsakoff syndrome is primarily a clinical diagnosis, supported by history, physical examination, and, when needed, neuroimaging or laboratory tests. Diagnostic steps include:

  • Clinical history: Evaluation of risk factors such as chronic alcohol use, malnutrition, or recent gastrointestinal illness.

  • Neurological examination: Assessment of mental status, eye movements, gait, and coordination.

  • Thiamine level testing: Serum thiamine levels may be measured but are not always reliable for diagnosis.

  • MRI of the brain: May show characteristic lesions in the mammillary bodies, thalamus, and periaqueductal gray matter.

  • Neuropsychological testing: Useful in identifying memory deficits and cognitive impairment in suspected Korsakoff’s psychosis.

Because early treatment is critical, diagnosis should not be delayed while awaiting lab or imaging results if clinical suspicion is high.

Treatment

Treatment of Wernicke–Korsakoff syndrome focuses on immediate thiamine replacement and addressing underlying causes. Key components include:

Acute Management

  • Thiamine supplementation: High-dose intravenous thiamine (usually 500 mg IV three times daily for 2–3 days) followed by intramuscular or oral administration.

  • Glucose control: Thiamine should always be administered before glucose to avoid precipitating or worsening encephalopathy.

  • Hydration and electrolyte correction: Especially important in alcohol-dependent individuals who may have concurrent deficiencies (e.g., magnesium).

Supportive and Long-Term Care

  • Alcohol cessation programs: Counseling, rehabilitation, and support groups to prevent recurrence.

  • Nutritional rehabilitation: Balanced diet rich in vitamins and minerals.

  • Cognitive and occupational therapy: For patients with persistent cognitive deficits to improve functional independence.

Prognosis

The prognosis of Wernicke–Korsakoff syndrome depends on how early the condition is recognized and treated. Key points include:

  • Wernicke’s encephalopathy: If treated promptly with thiamine, symptoms may improve within days. Delay in treatment can lead to permanent damage.

  • Korsakoff’s psychosis: Often irreversible. Some patients may show partial improvement, but many have lasting memory impairments and require long-term care.

  • Mortality: High if Wernicke’s encephalopathy is left untreated, primarily due to complications like infections, malnutrition, and cardiac failure.

With early recognition and comprehensive management, the acute phase can be reversed, and progression to the chronic phase may be prevented. Long-term outcomes improve significantly when alcohol use is stopped and nutritional support is maintained.

Medical Disclaimer

The information provided on this page is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition.