Wernicke encephalopathy, also known as Wernicke-Korsakoff syndrome, or alcoholic encephalopathy, if trying to avoid eponyms, 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.
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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 6,7:
alcohol abuse (up to 90% in industrialised countries 5)
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
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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 mammillary 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 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% 9.
History and etymology
It is named after:
Carl Wernicke: German neurologist and psychiatrist (1848-1905) 2
Sergei Korsakoff: Russian neuropsychiatrist (1854-1900) 2
Differential diagnosis
General imaging differential considerations include:
Leigh disease: mammillary bodies not involved
metronidazole-induced encephalopathy: dentate nuclei, cranial nerve nuclei and splenium also involved 10
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bilateral medial thalami abnormalities
central venous thrombosis