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Research paper
Wernicke-Korsakoff syndrome not related to alcohol use: a systematic review
  1. Simon J Scalzo1,
  2. Stephen C Bowden1,2,
  3. Margaret L Ambrose1,
  4. Greg Whelan3,4,
  5. Mark J Cook2,4
  1. 1Melbourne School of Psychological Sciences, The University of Melbourne, Melbourne, Victoria, Australia
  2. 2Department of Clinical Neurosciences, St. Vincent's Hospital, Melbourne, Victoria, Australia
  3. 3Addiction Services, The Melbourne Clinic, Richmond, Victoria, Australia
  4. 4Department of Medicine, The University of Melbourne, Melbourne, Victoria, Australia
  1. Correspondence to Professor Stephen C Bowden, Melbourne School of Psychological Sciences, Redmond Barry Building, University of Melbourne, VIC 3010, Australia; sbowden{at}unimelb.edu.au

Abstract

Objective Although Wernicke-Korsakoff syndrome (WKS) is a common condition, diagnosis remains difficult. WKS not associated with alcohol is rare and thought to present differently to alcohol-related WKS. We conducted a systematic review of WKS not related to alcohol to enhance understanding of WKS not related to alcohol and WKS in general.

Objective A systematic review was conducted of case reports, published in English, of Wernicke's encephalopathy and WKS in patients without a history of alcohol-use disorder. Main data sources: MEDLINE, Index Medicus. Eligible cases totaled 623. Publication dates ranged from 1867 to 2014. Comparisons of clinical presentation were made with published data on samples comprising, almost exclusively, alcohol-related WKS.

Results A wide array of illnesses precipitated WKS. When diagnosis of WKS was performed postmortem, non-alcohol-related cases presented a similar number of signs of the classic triad as alcohol-related cases (p=0.662, Cohen's w=0.12) but more signs when diagnosed antemortem (p<0.001, Cohen's w=0.46). The most common sign was altered mental state. Korsakoff syndrome or ongoing memory impairment was reported in 25% of non-alcohol-related WKS, although cognitive status was not explicitly reported in many cases. When duration of memory impairment was reported, 56% had clinically obvious memory impairment lasting beyond the period of acute presentation. Non-alcohol-related WKS was more often associated with female gender, younger age, shorter duration of precipitating illness and better survival rate compared to alcohol-related WKS.

Conclusions Thiamine deficiency in the absence of an alcohol-use disorder can cause the full clinical spectrum of WKS, including chronic cognitive impairment and Korsakoff syndrome.

  • CLINICAL NEUROLOGY
  • NEUROPSYCHIATRY
  • ALCOHOL-RELATED PROBLEMS
  • MEMORY
  • EVIDENCE-BASED NEUROLOGY

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