Overview
Wernicke-Korsakoff syndrome describes a spectrum of neuropsychiatric problems as a result of thiamine (vitamin B1) deficiency most commonly secondary to chronic excessive alcohol consumption. It describes the effects of Wernicke encephalopathy (WE) and Korsakoff syndrome.
Pathophysiology
Alcohol is thought to disrupt thiamine storage and metabolism which is needed for respiration, leading to inadequate ATP production. This leads to neurones being starved of energy and dying leading to brain atrophy.
Presentation
Wernicke encephalopathy (WE)
WE is characterised by the presence of the following triad:
- Ophthalmoplegia
- Confusion
- Ataxia
Korsakoff syndrome
If untreated, WE can lead to Korsakoff syndrome, which is characterised by memory problems:
- Anterograde amnesia – inability to form new memories
- Retrograde amnesia – inability to recall memories from before the event causing amnesia
- Confabulation – recalling false or erroneous memories without the intending to deceive
Management
Overview
Management involves treating the underlying cause. If the cause is chronic excessive alcohol consumption, this should be addressed along with giving oral, IM or IV thiamine along with B vitamins indefinitely.
Prognosis
If untreated, WE can lead to death in up to 20% of cases. Out of those who survive, around 85% develop Korsakoff syndrome. 25% of those with Korsakoff syndrome require long-term institutionalisation.
