Neurology >
Wernicke-Korsakoff Syndrome

“A neurological condition which is a combination of Wernicke’s encephalopathy (WE) and Korsakoff psychosis (KP)”

Risk Factors
  • Chronic alcohol misuse
  • Malnourishment
  • Gastrointestinal tract malignancy eg IBD
    Post-bariatric surgery
  • Hyperemesis gravidarum
Aetiology
  • WE:
    1. Chronic alcoholism
    2. Eating disorders
    3. Malnutrition
    4. Prolonged vomiting eg via chemotherapy, GI malignancy or hyperemesis gravidarum
  • KP:
    • Untreated Wernicke’s encephalopathy
Pathophysiology
  • WE:
    • Chronic alcoholism > Thiamine deficiency > Mitochondrial dysfunction and therefore oxidative toxicity> Brain tissue ischaemia and cell death in periaqueductal punctate > Wernicke’s (acute phase) > Korsakoff’s (chronic phase)
  • KP:
    • Thiamine deficiency > Hypothalamic damage and cerebral atrophy > Decreased ability to acquire new memories, confabulation (invented memory, owing to retrograde amnesia), lack of insight and apathy. Patients rarely recover
Clinical Presentation
  • WE:
    • Thiamine (vitamin B1) deficiency with a classical triad of:
      1. Acute confusional state which is reversible
      2. Ophthalmoplegia (nystagmus, lateral rectus, or conjugate gaze palsies)
      3. Ataxia
  • KP:
    • Persistent and irreversible state of
      1. Amnesia
      2. Confabulation
      3. Psychosis
Investigations
  • Blood to exclude reversible causes
  • ABG
  • CT
  • MRI
  • EEG to exclude non-convulsive status epilepticus
Management
  • WE:
    • Urgent replacement of thiamine
    • Supplement electrolytes and resume a balanced diet asap
  • KP:
    • A strategy of secondary harm prevention if thiamine replacement is ineffective: maintain oral thiamine, consider rehabilitation, treat co-morbidities, potential long-term residential care
Complications
  • KP:
    • Typically irreversible complications of memory loss

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