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:
Wernicke encephalopathy can evolve into the chronic form of thiamine deficiency known as Korsakoff psychosis, characterized by:
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
- 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
- DWI/ADC: restricted diffusion can also be seen in the same regions
- MR spectroscopy: may show decreased or normal NAA with the notable presence of lactate
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:
- Leigh disease: mammillary bodies not involved
- metronidazole-induced encephalopathy: dentate nuclei, cranial nerve nuclei and splenium also involved