CNS nontumor
Toxic and metabolic disorders
Wernicke-Korsakoff syndrome

Author: Kymberly A. Gyure, M.D. (see Authors page)

Revised: 18 August 2017, last major update August 2015

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: Wernicke-Korsakoff syndrome [title]

Cite this page: Gyure, K.A. Wernicke-Korsakoff syndrome. PathologyOutlines.com website. http://pathologyoutlines.com/topic/cnswernickekorsakoff.html. Accessed September 19th, 2017.
General and terminology
  • Wernicke encephalopathy: neuropsychiatric syndrome resulting from thiamine (vitamin B1) deficiency
  • Korsakoff syndrome: a disproportionate impairment in memory relative to other features of cognition secondary to thiamine deficiency that usually follows or accompanies Wernicke encephalopathy
Epidemiology
  • Prevalence of approximately 1 - 3% in autopsy studies
  • Most cases in developed countries occur in the setting of alcoholism
  • Other clinical settings predisposing to Wernicke encephalopathy include a staple diet of polished rice, gastrointestinal surgical procedures leading to loss of portions of the GI tract, recurrent vomiting or chronic diarrhea, cancer chemotherapy and systemic diseases (renal failure with dialysis, AIDS)
Sites
  • Mammillary bodies, medial thalamus / wall of third ventricle, periaqueductal gray matter, midbrain tectum
Etiology
  • Thiamine deficiency leads to impaired function of enzymes using thiamine pyrophosphate as a coenzyme
  • Disruption in thiamine dependent metabolic processes (carbohydrate and lipid metabolism) leads to loss of osmotic gradients with resulting intracellular fluid accumulation (cytotoxic edema) and eventual blood brain barrier breakdown (vasogenic edema)
Clinical features
  • Wernicke encephalopathy: ocular abnormalities (nystagmus / ophthalmoplegia), mental status changes (confusion) and gait abnormalities (ataxia)
  • Korsakoff syndrome: loss of working memory with relative preservation of reference memory
Diagnosis
  • Mainly based on clinical and imaging findings
Laboratory
  • Measurement of thiamine or its metabolites in blood / erythrocytes
  • Red blood cell transketolase activity
Radiology description
  • Acute Wernicke encephalopathy: bilateral, symmetric T2 and FLAIR signal hyperintensities
Radiology images

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Hyperintense signal

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Shrinking of the cortical gyri

Prognostic factors
  • ~20% mortality rate
  • ~80% of individuals who survive Wernicke encephalopathy will develop Korsakoff syndrome
Case reports
Treatment
  • Parenteral thiamine administration, given before or with intravenous glucose
Gross description
  • Acute Wernicke encephalopathy: hemorrhages in mammillary bodies, medial thalami, periaqueductal gray matter
  • Chronic lesions: atrophy of affected structures
Gross images

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Cerebellar vermis atrophy not evident

Microscopic (histologic) description
  • Acute Wernicke encephalopathy: hemorrhages, vascular proliferation and edema in mammillary bodies, medial thalami and periaqueductal gray matter
  • Chronic lesions: mild neuronal loss with gliosis in affected structures
Microscopic (histologic) images

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Wernicke encephalopathy

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Atrophy of the cerebellar cortex

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Mamillary bodies

Differential diagnosis
  • Alcohol / drug intoxication
  • Bilateral paramedian thalamic infarcts
  • Other causes of acute encephalopathy (viral encephalitis, demyelinating disease)