Wernicke-Korsakoff Syndrome (WKS) is a neuropsychiatric syndrome caused by thiamine (vitamin B1) deficiency, commonly found in chronic alcoholism. This primarily alcohol-induced disorder is characterized by amnesia, confabulation, disorientation, and neurological findings. WKS occurs due to necrotic lesions to the mammillary bodies, thalamus, and brainstem. The syndrome itself consists of 2 components: Wernicke encephalopathy (WE), and Korsakoff’s amnesia (KA), hence the combined name Wernicke-Korsakoff Syndrome.
WKS is a clinical diagnosis. Although often described as a triad (oculomotor abnormalities, cerebellar dysfunction, and an altered mental state) in the classic literature, it is rare for all three clinical signs to be present. [11] Less than 16% of individuals with WKS will have all three signs. Neuroimaging findings may help in confirming the diagnosis but it is not diagnostic.
In 1997, Caine et al. proposed that a diagnosis can be made when patients have any 2
of the following 4 features:[12]
Classically, Wernicke encephalopathy is an acute presentation, while Korsakoff syndrome is a late, chronic manifestation of Wernicke encephalopathy that typically presents as anterograde and retrograde amnesia with preserved long-term memory.
Course | Symptoms | |
---|---|---|
Wernicke Encephalopathy (WE) | Acute, but reversible | The classic triad of: • Eye abnormalities (nystagmus, oculomotor paralysis, paralysis of conjugate gaze), CN VI palsy AKA lateral gaze palsy (lateral rectus) • Ataxia of stance and gait • Mental status changes, including withdrawal, confusion, retro/anterograde amnesia |
Korsakoff's Syndrome/Amnesia (KS)* | Chronic and only 20% of cases are reversible with treatment | • Anterograde amnesia • Confabulations • Personality changes |
Thiamine resupplementation is the primary treatment. Oculomotor deficits should resolve over hours to days, while cognitive impairment may resolve within days to weeks. Most patients will exhibit some residual deficits.[21]
Guideline | Location | Year | Website | |
---|---|---|---|---|
European Federation of Neurological Societies (EFNS) - Wernicke Encephalopathy | Europe | 2010 | - | Link |