Wernicke-Korsakoff syndrome

• Disease Description (Causes and Symptoms)

  • Definition and causes: A neurological syndrome caused by vitamin B1 (thiamine) deficiency. It is a collective term for a condition in which acute Wernicke encephalopathy and Korsakoff syndrome, characterized by chronic memory impairment, occur together or sequentially. The most common cause is nutritional deficiency due to chronic alcohol consumption, but it can also occur due to prolonged fasting, severe vomiting, malabsorption after gastrointestinal surgery, or chemotherapy.
  • Pathophysiology: Thiamine is essential for carbohydrate metabolism and energy supply in the brain. When deficient, impaired energy metabolism causes selective damage to brain regions particularly sensitive to ischemia, including the reticular formation, mammillary bodies and dorsomedial thalamus, and central gray matter.
  • Main symptoms
    • Wernicke’s triad (acute phase): Confusion or altered consciousness, gait ataxia, and abnormal eye movements (nystagmus, exotropia, etc.). Abnormal eye movements and gait disturbances are common and progress acutely.
    • Korsakoff syndrome (chronic phase): Central memory impairment (especially impaired short-term storage of new events—anterograde amnesia), impaired recall, additive confabulation, and relatively preserved long-term procedural memory.
    • Other symptoms: Anxiety, reduced motivation, peripheral neuropathy, loss of appetite, weight loss, etc.

• Treatment

  • Emergency treatment (highest priority): If suspected, high-dose intravenous/intramuscular thiamine should be administered immediately at a hospital. According to clinical guidelines, during the initial acute phase, for example, thiamine 500 mg IV 2–3 times/day (some recommendations specify 500 mg IV TID) may be administered for several days, followed by dose reduction (e.g., switching to 250 mg IV/day or 100 mg PO/day). The dose and duration may vary according to local and hospital guidelines; follow the treating physician’s instructions.
  • Important principle: Avoid administering glucose (sugar) without administering thiamine beforehand or concurrently—glucose administration may worsen symptoms.
  • Additional treatment
    • Fluid and electrolyte correction, nutritional supplementation (high-protein diet and vitamin supplementation), treatment of alcohol withdrawal (if necessary), and treatment of complications (infection, coma, etc.).
    • In the long term, oral thiamine supplementation (e.g., 100 mg/day or more), nutritional management, abstinence from alcohol, and rehabilitation therapy (cognitive rehabilitation, occupational therapy, etc.).
  • Precautions: Eye movement abnormalities, altered consciousness, and ataxia may improve within a few days if treatment is prompt, but memory impairment may be permanent; therefore, early diagnosis and immediate treatment are essential.

• Checklist Before an Outpatient Visit (Including Example Questions)

  • Items to prepare (bring with you)
    • List of currently used medications (including prescription drugs, over-the-counter drugs, and supplements)
    • Recent blood test or imaging results, if available, and a copy of the hospital discharge records
    • Records of the amount and duration of alcohol consumption (as specific as possible), and records of dietary intake and weight changes
    • Records of when symptoms began and how they progressed (when they started, suddenly or gradually, aggravating factors, etc.), and episodes of falls, fainting, or decreased consciousness
    • History of allergies and past surgeries or gastrointestinal diseases
  • Information that is helpful to tell the doctor in advance during the outpatient visit
    • Recent alcohol consumption (daily and weekly amounts) and the time of the last drink
    • Whether glucose or vitamins were recently administered (during an emergency room or hospital visit)
    • Dietary intake and nutritional status (whether there has been rapid weight loss)
  • Example questions (questions patients can ask their doctor themselves)
    • "What thiamine dose will be administered now, and how long will it be administered?"
    • "What additional tests, such as an MRI or blood tests, are needed?"

• Examples of General Questions to Ask the Doctor

  • "Which symptoms are most likely to improve quickly with treatment, and which symptoms may remain permanently?"
  • "What are the side effects of thiamine administration, and in what situations should I return to the hospital?"
  • "In the long term, how long will vitamin supplementation or follow-up testing be needed?"
  • "What are the recommendations regarding daily activities, such as driving, returning to work, and resuming alcohol consumption?"

• Examples of Disease-Specific Questions

  • "Considering my case, including when my symptoms began and my alcohol history, how likely is my memory impairment (anterograde amnesia) to recover? What specific rehabilitation methods can help recovery?"
  • "I was given glucose in the emergency room. How can I confirm whether I received sufficient thiamine? Are additional tests or treatment needed?"

(Key Points)

• Clinical findings are the most important for diagnosis. Bilateral lesions in the mammillary bodies, thalamus, and other areas may be seen on MRI, but MRI may also be normal. Blood thiamine measurement and tests such as erythrocyte transketolase are available as supplementary tests, but when there is clinical suspicion, starting treatment without delay takes priority.
• Prevention consists of regular nutritional assessment and thiamine supplementation for high-risk groups (people with chronic alcohol use, patients with nutritional deficiencies, people after gastrointestinal surgery, etc.) and control of alcohol consumption.