Korsakow-Syndrom

Wernicke encephalopathy, also referred as Wernicke-Korsakoff syndrome, is a form of 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 mammillary bodies, dorsomedial thalami, tectal plate, periaqueductal area and/or around the third ventricle.

Clinical presentation

It was originally described as characterized by the triad of:

  • acute confusion
  • ataxia
  • ophthalmoplegia (most commonly horizontal nystagmus and conjugate gaze palsies)
  • Wernicke encephalopathy can evolve into the chronic form of thiamine deficiency known as Korsakoff psychosis, characterized by:

  • memory loss (global amnesia)
  • confabulation
  • The two terms are often concatenated to form Wernicke-Korsakoff syndrome.

    Pathology

    Etiology

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

    • alcohol abuse (up to 90% in industrialised countries )
    • starvation/fasting
    • prolonged total parenteral nutrition without supplementation
    • postbariatric surgery
    • hyperemesis gravidarum
    • gastrointestinal malignancy
    • chronic dialysis

    Radiographic features

    In acute stages, hemorrhage, necrosis, and edema may be present. In chronic stages, atrophic changes may be present especially involving the mamillary bodies.

    CT

    Usually normal.

    MRI

    Treatment and prognosis

    Treatment of acute Wernicke-Korsakoff syndrome is with intravenous thiamine hydrochloride, along with other vitamins/minerals, and treatment of the underlying cause (e.g. alcohol cessation). Untreated, there is high mortality of up to 20% .

    History and etymology

    It is named after:

    • Carl Wernicke: German neurologist and psychiatrist (1848-1905)
    • Sergei Korsakoff: Russian neuropsychiatrist (1854-1900)

    Differential diagnosis

    General imaging differential considerations include:

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