Wernicke–Korsakoff Syndrome

Primer

Wernicke-Korsakoff Syndrome (WKS) is a syndrome due to thiamine (vitamin B1) deficiency, commonly found in chronic alcoholism. This primarily alcohol-induced amnestic disorder results in necrotic lesions to the mammillary bodies, thalamus, and brainstem. The syndrome itself consists of 2 components: Wernicke’s encephalopathy (WE), and Korsakoff’s amnesia (KA), hence the combined name Wernicke-Korsakoff Syndrome.

Terminology

Wernicke's vs. Korsakoff's

Wernicke's Encephalopathy Korsakoff's Syndrome/Amnesia
  • 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
  • Chronic and only 20% of cases is reversible with treatment
  • Anterograde amnesia and confabulations
  • Cannot occur during an acute delirium or dementia and must persist beyond usual duration of intoxication or withdrawal management

Physical Exam

In patients with chronic alcoholism with only an ataxic gait and stance found on physical exam, and no other neurological findings, has alcoholic cerebellar degeneration (midline cerebellar degeneration).

Investigations

  • CBC, lytes, Cr
  • GGT - indicator of acute liver injury from EtOH use, for detecting people drinking > 4 drinks/day
  • AST, ALT, ALP, GGT
    • AST > ALT x 3 (in EtOH hepatitis)
    • AST/ALT ratio also > 2
  • INR
  • B12 and thiamine

Treatment

See also article: Alcohol Use Disorder

It is important to supplement patients with PO or IV thiamine. Thiamine 100mg TID should be prescribed as ongoing supplementary therapy in alcohol use disorder patients identified as at risk for thiamine deficiency. [1] For the more severe cases, 200mg IV thiamine x 3 days, followed by oral supplementation is appropriate. Those with suspected Wernicke’s encephalopathy should receive thiamine 100mg IM/IV x 3 days, then 300mg PO x 3-12 months. Don’t forget to give fluids as well!

References

1) RebelEM:Should You Prescribe Oral Thiamine for Chronic Alcoholics?