Wernicke-Korsakoff syndrome

Wernicke encephalopathy, also referred as Wernicke-Korsakoff syndrome, is due to thiamine (vitamin B1) deficiency, and is typically seen in alcoholics.

On imaging, it is commonly seen on MRI as areas of symmetrical increased T2/FLAIR signal involving the mamillary bodies, dorsomedial thalami, tectal plate, periaqueductal area, and around the third ventricle.

It was originally described as characterised by the triad of:

  1. acute confusion
  2. ataxia
  3. ophthalmoplegia

Wernicke encephalopathy can evolve into the chronic form of thiamine deficiency known as Korsakoff psychosis, characterised by:

  1. memory loss (global amnesia)
  2. confabulation

The two terms are often concatenated to form Wernicke-Korsakoff syndrome.

Aetiology

Thiamine deficiency results from malnutrition or malabsorption, which can occur for a number of reasons 6,7:

  • alcohol abuse (up to 90% in industrialised countries 5)
  • starvation/fasting
  • prolonged total parental nutrition without supplementation
  • post bariatric surgery
  • hyperemesis gravidum
  • gastrointestinal malignancy
  • chronic dialysis
CT
  • usually normal
MRI
  • T2/FLAIR: symmetrically increased signal intensity in the:
  • T1 C+ (Gd): contrast enhancement can also be seen in the same regions, most commonly of the mamillary bodies 4
  • DWI/ADC: restricted diffusion can also be seen in the same regions 4
  • MR spectroscopy: may show decreased or normal NAA with the notable presence of lactate 4

Treatment of acute Wernicke-Korsakoff syndrome is with intravenous thiamine hydrochloride, along with other vitamins/minerals. Untreated, there is a high mortality of up to 20% 9.

It is named after 2:

  • Carl Wernicke: German neurologist and psychiatrist (1848-1905)
  • Sergei Sergeievich Korsakoff: Russian neuropsychiatrist (1853-1900)

General imaging differential considerations includes:

Toxic and metabolic encephalopathies
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Article Information

rID: 5627
Section: Syndromes
Synonyms or Alternate Spellings:
  • Wernicke encephalopathy
  • Alcoholic encephalopathy
  • Wernicke's encephalopathy
  • Thiamine deficiency
  • Vitamin B1 deficiency

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