Wernicke-Korsakoff Syndrome


Article Author:
Shweta Akhouri


Article Editor:
Edward Newton


Editors In Chief:
Rudolf Bickel
Abdolreza Saadabadi
Cornel Stanciu


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Frank Smeeks
Kristina Soman-Faulkner
Radia Jamil
Patrick Le
Sobhan Daneshfar
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Pritesh Sheth
Hassam Zulfiqar
Navid Mahabadi
Steve Bhimji
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Mark Pellegrini
James Hughes
Beata Beatty
Nazia Sadiq
Hajira Basit
Phillip Hynes
Tehmina Warsi


Updated:
3/15/2019 10:18:30 AM

Introduction

 When a patient presents with confusion, persistent memory, and learning deficits, one should suspect the presence of Wernicke-Korsakoff syndrome. This syndrome usually presents with a clinical triad consisting of altered mental status (i.e., confusion or dementia), nystagmus (or ophthalmoplegia), and ataxia. Unfortunately, less than a third of patients present with this clinical triad.[1][2][3]

To better understand this disorder, practitioners should think of Wernicke encephalopathy and Wernicke-Korsakoff syndrome as two separate syndromes: (1) Wernicke encephalopathy is characterized by an acute confusional state and often reversible clinical features, and (2) Wernicke-Korsakoff syndrome with persistent but often irreversible clinical features including dementia and gait abnormalities.[4]

Etiology

The cause of Wernicke-Korsakoff syndrome is a deficiency of thiamine or vitamin B1. Individuals with poor nutrition for any reason are at risk for this disorder. The most common social factor associated with Wernicke-Korsakoff syndrome is chronic alcohol abuse which leads to decreased absorption and utilization of thiamine. However, it is important to note that there are nonalcoholic causes of thiamine deficiency which can also produce Wernicke-Korsakoff syndrome. [5][6][7]These include individuals with malnutrition, starvation, schizophrenia, anorexia nervosa, prisoners of war, and terminal malignancies. In the past, baby formula which was deficient in thiamine also led to Wernicke-Korsakoff syndrome. Wernicke-Korsakoff syndrome also can develop during the first trimester of pregnancy in women who develop hyperemesis gravidarum. Another common cause is bariatric surgery and malignancies of the gastrointestinal (GI) tract.

Epidemiology

The prevalence is between 0% and 2% worldwide. Specific subpopulations have higher prevalence rates including people who are homeless, older individuals living alone or in isolation, and psychiatric inpatients. Prevalence is not connected to alcohol consumption per capita. For example, in France, a country that is well known for its consumption of wine, the prevalence is approximately 0.4%, while Australia has a prevalence of approximately 3%.[8]

Pathophysiology

Brain atrophy associated with Wernicke-Korsakoff Syndrome occurs in the mammillary bodies, the cerebellum, the thalamus, the frontal lobe, the periaqueductal grey, the walls of the third ventricle, and the floor of the fourth ventricle. In addition to the damage seen in these areas, there may be damage to the cortex, although this may be due to the direct toxic effects of alcohol as opposed to thiamine deficiency. 

The amnesia associated with Wernicke-Korsakoff Syndrome is a result of atrophy of the structures of the diencephalon (thalamus, hypothalamus, and mammillary bodies) and is similar to amnesia that is present as a result of damage to the medial temporal lobe. It has been argued that the memory impairment can occur as a result of damage along any part of the mammillothalamic tract, which may explain how Wernicke-Korsakoff Syndrome can develop in patients with damage exclusively to either the thalamus or the mammillary bodies.

History and Physical

An individual with Wernicke-Korsakoff syndrome will usually present with (1) ocular disturbances which may include diplopia, painless vision loss, or strabismus, (2) gait abnormalities that include a wide-based, short-stepped gait and an inability to stand or ambulate without assistance, and (3) mental status changes that include apathy, paucity of speech, and indifference to the environment. As the disorder progresses, other symptoms that may develop include agitation, anger, hallucinations, and confabulations.

Evaluation

The diagnosis of Wernicke-Korsakoff syndrome is made by the history and clinical findings supplemented with lab studies showing thiamine deficiency. Treatment aims to prevent the disorder from getting worse. One should maintain a high level of suspicion for thiamine deficiency to avoid precipitation of severe symptoms of Wernicke-Korsakoff syndrome. Prophylactic thiamine administration is relatively safe and should be started even if the diagnosis has not been confirmed.[9][10][11]

Treatment / Management

Unfortunately, unlike Wernicke encephalopathy, Wernicke-Korsakoff syndrome is a long-term disorder which is often progressive. It is very rare for the individual with Wernicke-Korsakoff syndrome to recover fully even with aggressive treatment. After thiamine treatment, the symptoms of encephalopathy will improve in 5 to 12 days. The patient should be offered oral thiamine and consulted for rehabilitation and treatment of other comorbid conditions. Most patients with Wernicke-Korsakoff syndrome need long-term care in a chronic care facility. Their prognosis is guarded.[12][13][14][15]

Differential Diagnosis

The differential diagnosis includes the following:

  • Anoxic encephalopathy
  • Alzheimer disease
  • Temporal lobe epilepsy
  • Concussive head injury
  • Dementia with Lewy bodies
  • Herpes simplex virus
  • Temporal lobe infarction
  • Transient global amnesia
  • Third ventricle tumor

Prognosis

Approximately 25% of patients with Wernicke-Korsakoff syndrome require long-term institutionalization. Patients depending on long-term care often have one or more comorbidity (somatic and psychiatric).

Mental Status Complications

Global confusional often resolves gradually after treatment.

If an amnestic deficit is present, it may manifest as the signs of apathy and global confusion resolve.

One in five patients who demonstrate signs of the amnestic state after treatment has been initiated will have a complete with most patients having varying degrees of persistent learning and memory impairment.

Maximum recovery may take years and depends on abstinence from alcohol.

Once patients with Korsakoff psychosis have recovered, they do not demand alcohol, but they will accept it if offered.

Ataxic Complications

Approximately half of the patients recover completely from ataxic symptoms, and the other half have incomplete recovery, with a residual slow, shuffling, wide-based gait and the inability to tandem walk.

Vestibular dysfunction also improves about half the time.

Ocular Complications

Patients who recover do so in a particular sequence.

Improvement of ocular abnormalities is the most dramatic, usually occurring within hours of thiamine administration.

Failure of ocular abnormalities to respond to thiamine should raise doubt as to the diagnosis.

Vertical nystagmus may persist for months.

Fine horizontal nystagmus can persist indefinitely, but patients completely recover from sixth nerve palsies, ptosis, and vertical-gaze palsies.

Mortality 

Mortality may occur secondary to infections and hepatic failure, but some deaths are attributable to defects of prolonged thiamine deficiency.

The mortality rate is 10-15% in severe cases.

Prognosis depends on the stage of disease at presentation and time of treatment.

Pearls and Other Issues

Korsakoff syndrome often follows or accompanies Wernicke encephalopathy.

  • If treated quickly, Korsakoff syndrome development may be prevented with thiamine treatments.
  • Thiamine needs to be administered quickly in both dose and duration.
  • Thiamine treatment may result in noticeable improvements in mental status after only 2 to 3 weeks of therapy.
  • With treatment, Wernicke's encephalopathy will not necessarily progress to Wernicke-Korsakoff syndrome.

To reduce the risk of Wernicke-Korsakoff syndrome, limit the intake of alcohol and ensure that proper nutrition needs are met.

  • Proper nutrition which, in combination with thiamine supplements, may reduce the risk of development of Wernicke-Korsakoff syndrome.
  • Supplemental thiamine and good nutrition may help heavy drinkers who refuse to or are unable to quit.

Enhancing Healthcare Team Outcomes

Wernicke Korsakoff syndrome is often encountered by the primary care physician, nurse practitioner or internist. While the diagnosis is relatively simple, the management is unsatisfactory. The disorder is progressive and carries very high morbidity and mortality. The condition is best managed by a multidisciplinary team that includes a neurologist, mental health nurse, psychiatrist, gastroenterologist, ophthalmologist, and an intensivist. Despite optimal treatment, full recovery does not occur. the majority of patients end up in a long term care facility and have a guarded prognosis.[16]

 


Interested in Participating?

We are looking for contributors to author, edit, and peer review our vast library of review articles and multiple choice questions. In as little as 2-3 hours you can make a significant contribution to your specialty. In return for a small amount of your time, you will receive free access to all content and you will be published as an author or editor in eBooks, apps, online CME/CE activities, and an online Learning Management System for students, teachers, and program directors that allows access to review materials in over 500 specialties.

Improve Content - Become an Author or Editor

This is an academic project designed to provide inexpensive peer-reviewed Apps, eBooks, and very soon an online CME/CE system to help students identify weaknesses and improve knowledge. We would like you to consider being an author or editor. Please click here to learn more. Thank you for you for your interest, the StatPearls Publishing Editorial Team.

Wernicke-Korsakoff Syndrome - Questions

Take a quiz of the questions on this article.

Take Quiz
Which statement best describes the Wernicke-Korsakoff syndrome?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A 57-year-old man with a longstanding history of alcohol use presents with confusion, disorientation, and gait imbalance. Other than disorientation, bilateral paresis of conjugate gaze is noted. CT head is negative. Which vitamin deficiency is most likely?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
What part of the brain is associated with memory loss in Wernicke-Korsakoff syndrome?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
Deficiency of which of the following is associated with Wernicke-Korsakoff syndrome?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
In a 68 year old alcoholic male who presents with mental status changes, gait abnormalities and confusion, a preliminary diagnosis of Wernicke Korsakoff syndrome is made. Which ocular feature would one expect to find in these patients?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
An indigent patient presents in an emergency room with confusion, bilateral sixth nerve palsies, horizontal nystagmus, and gait ataxia. Which of the following is the most likely diagnosis?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up

Wernicke-Korsakoff Syndrome - References

References

Gerridzen IJ,Joling KJ,Depla MF,Veenhuizen RB,Verschuur EML,Twisk JWR,Hertogh CMPM, Awareness and its relationships with neuropsychiatric symptoms in people with Korsakoff syndrome or other alcohol-related cognitive disorders living in specialized nursing homes. International journal of geriatric psychiatry. 2019 Mar 10;     [PubMed]
Yoon C,Gedzior J,DePry D, Wernicke-Korsakoff syndrome: Focus on low-threshold diagnosis and prompt treatment in the primary care setting. International journal of psychiatry in medicine. 2019 Mar 1;     [PubMed]
van Dongen LCM,Wingbermühle E,van der Veld WM,Vermeulen K,Bos-Roubos AG,Ockeloen CW,Kleefstra T,Egger JIM, Exploring the behavioral and cognitive phenotype of KBG syndrome. Genes, brain, and behavior. 2019 Feb 21;     [PubMed]
Polegato BF,Pereira AG,Azevedo PS,Costa NA,Zornoff LAM,Paiva SAR,Minicucci MF, Role of Thiamin in Health and Disease. Nutrition in clinical practice : official publication of the American Society for Parenteral and Enteral Nutrition. 2019 Jan 15;     [PubMed]
Doğan İG,Altiokka GU,Türker F,Saka B,Bilgiç B,Orhan EK, Wernicke's Encephalopathy due to Non-Alcoholic Gastrointestinal Tract Disease. Noro psikiyatri arsivi. 2018 Dec;     [PubMed]
Heirene R,John B,Roderique-Davies G, Identification and Evaluation of Neuropsychological Tools Used in the Assessment of Alcohol-Related Cognitive Impairment: A Systematic Review. Frontiers in psychology. 2018;     [PubMed]
McGlacken-Byrne D,Wallace E, Vitamin Deficiency and Systemic Failure: The Case For Greater Focus On Wernicke-Korsakoff Syndrome Irish medical journal. 2018 Dec 6;     [PubMed]
Arts NJ,Walvoort SJ,Kessels RP, Korsakoff's syndrome: a critical review. Neuropsychiatric disease and treatment. 2017;     [PubMed]
Nakamura ZM,Tatreau JR,Rosenstein DL,Park EM, Clinical Characteristics and Outcomes Associated With High-Dose Intravenous Thiamine Administration in Patients With Encephalopathy. Psychosomatics. 2018 Jul - Aug;     [PubMed]
Junghanns K,Wetterling T, [Alcohol withdrawal and its major complications]. Fortschritte der Neurologie-Psychiatrie. 2017 Mar;     [PubMed]
Latt N,Dore G, Thiamine in the treatment of Wernicke encephalopathy in patients with alcohol use disorders. Internal medicine journal. 2014 Sep;     [PubMed]
Isenberg-Grzeda E,Chabon B,Nicolson SE, Prescribing thiamine to inpatients with alcohol use disorders: how well are we doing? Journal of addiction medicine. 2014 Jan-Feb;     [PubMed]
Feeney GF,Connor JP, Wernicke-Korsakoff syndrome (WKS) in Australia: no room for complacency. Drug and alcohol review. 2008 Jul;     [PubMed]
Harper C, The neurotoxicity of alcohol. Human     [PubMed]
Day E,Bentham P,Callaghan R,Kuruvilla T,George S, Thiamine for Wernicke-Korsakoff Syndrome in people at risk from alcohol abuse. The Cochrane database of systematic reviews. 2004;     [PubMed]
Sanvisens A,Zuluaga P,Fuster D,Rivas I,Tor J,Marcos M,Chamorro AJ,Muga R, Long-Term Mortality of Patients with an Alcohol-Related Wernicke-Korsakoff Syndrome. Alcohol and alcoholism (Oxford, Oxfordshire). 2017 Jul 1;     [PubMed]

Disclaimer

The intent of StatPearls is to provide practice questions and explanations to assist you in identifying and resolving knowledge deficits. These questions and explanations are not intended to be a source of the knowledge base of all of medicine, nor is it intended to be a board or certification review of Addiction Medicine. The authors or editors do not warrant the information is complete or accurate. The reader is encouraged to verify each answer and explanation in several references. All drug indications and dosages should be verified before administration.

StatPearls offers the most comprehensive database of free multiple-choice questions with explanations and short review chapters ever developed. This system helps physicians, medical students, dentists, nurses, pharmacists, and allied health professionals identify education deficits and learn new concepts. StatPearls is not a board or certification review system for Addiction Medicine, it is a learning system that you can use to help improve your knowledge base of medicine for life-long learning. StatPearls will help you identify your weaknesses so that when you are ready to study for a board or certification exam in Addiction Medicine, you will already be prepared.

Our content is updated continuously through a multi-step peer review process that will help you be prepared and review for a thorough knowledge of Addiction Medicine. When it is time for the Addiction Medicine board and certification exam, you will already be ready. Besides online study quizzes, we also publish our peer-reviewed content in eBooks and mobile Apps. We also offer inexpensive CME/CE, so our content can be used to attain education credits while you study Addiction Medicine.