Fact-sheet: Wernicke - Korsakoff encephalopathy


Updated on 08/15/2023 at 5:18 PM

Note : 0/10
Share

View all RADEOS cases associated with this fact-sheet

Definition

Encephalopathy related to vitamin B1 deficiency

Causes:

  • Chronic alcoholism, mainly
  • Other: prolonged fasting or vomiting,
  • gastroduodenectomy, parenteral nutrition, anorexia nervosa

Thiamine deficiency leads to osmotic disturbances causing intracellular and extracellular edema, since the metabolism of neurons located around the third ventricle and the cerebral aqueduct is largely dependent on this vitamin.

Clinical features

In the initial stage:

  • Oculomotor disturbances: nystagmus, oculomotor palsy, gaze palsy
  • Static cerebellar syndrome
  • Confusional state
  • Oppositional hypertonia

In the chronic stage: Korsakoff syndrome with confabulation, false recognition, and anterograde amnesia.

Laboratory findings

Markedly decreased vitamin B1 level

CT

  • No abnormality at the initial stage
  • At the chronic stage: atrophy of the mammillary bodies and diffuse cerebral atrophy

MRI

  • T1: hypointense
  • T2: hyperintense
  • T2-FLAIR: hyperintense
  • Diffusion: increased ADC (vasogenic edema) or decreased ADC (cytotoxic edema)
  • After gadolinium injection: possible but inconsistent enhancement of the mammillary bodies
  • Bilateral and symmetric involvement ++
    - posteromedial thalami
    - periaqueductal area
    - on either side of the third ventricle
    - mammillary bodies
  • Involvement of the cerebellum, red nuclei, corpus callosum, and cerebral cortex is less common.

At the chronic stage: atrophy of the mammillary bodies and diffuse cerebral atrophy.

Management

Emergency ++: IV vitamin B1 supplementation. Mainly preventive: - systematic vitamin B1 supplementation in every malnourished chronic alcoholic patient - systematic vitamin B1 administration with glucose infusion in a malnourished or alcoholic patient.

Favorable prognosis with treatment. Without treatment, mortality is high (estimated at 20%)

Differential diagnosis

  • Inflammatory or infectious demyelinating lesions
  • Deep cerebral vein thrombosis
  • Neuromyelitis optica
  • Behçet's disease
  • Central or extrapontine myelinolysis
  • Bithalamic infarction (Percheron artery territory)
  • Miller Fisher syndrome
  • Lymphoma of brain