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Korsakoff's syndrome Classification and external resources
Thiamine
ICD-10	F10.6
ICD-9	291.1, 294.0
DiseasesDB	14107
eMedicine	med/2405
MeSH	D020915

Korsakoff's syndrome or "Korsakov's syndrome" (Korsakoff's psychosis, amnesic-confabulatory syndrome), is a brain disorder caused by the lack of thiamine (vitamin B₁) in the brain. The syndrome is named after Sergei Korsakoff, the neuropsychiatrist who popularized the theory.

Contents 1 Symptoms 1.1 Signs 2 Treatment 3 Causes 4 Prevention 5 Case studies 6 In popular culture 7 References 8 External links

Symptoms

There are six major symptoms of Korsakoff's syndrome:

1. anterograde amnesia and
2. retrograde amnesia, severe memory loss
3. confabulation, that is, invented memories which are then taken as true due to gaps in memory sometimes associated with blackouts
4. meager content in conversation
5. lack of insight
6. apathy - the patients lose interest in things quickly and generally appear indifferent to change.

These symptoms are caused by a deficiency of thiamine (vitamin B₁), which is thought to cause damage to the medial thalamus and possibly to the mammillary bodies of the hypothalamus as well as generalized cerebral atrophy.^[1]

When Wernicke's encephalopathy accompanies Korsakoff's syndrome, the combination is called the Wernicke-Korsakoff syndrome. Korsakoff's is a continuum of Wernicke's encephalopathy, though a recognised episode of Wernicke's is not always obvious.

Korsakoff's involves neuronal loss, that is, damage to neurons; gliosis which is a result of damage to supporting cells of the central nervous system; and hemorrhage or bleeding in mammillary bodies. Damage to the dorsomedial nucleus of the thalamus is also associated with this disorder.

Signs

• Ataxia
• Apathy
• Retrograde and anterograde amnesia
• Confabulation
• Tremors
• Paralysis of muscles controlling the eye
• Lack of insight to the condition
• Coma

Treatment

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It was once assumed that anyone suffering from Korsakoff's syndrome would eventually need full time care. However, this is not the case and with proper treatment there is every chance that a person will remain independent and maintain a reasonable quality of life. ^[2] Treatment involves the replacement or suppletion of thiamine by intravenous (IV) or intramuscular (IM) injection, together with proper nutrition and hydration. However, the amnesia and brain damage caused by the disease does not always respond to thiamine replacement therapy. In some cases, drug therapy is recommended. If treatment is successful, improvement will become apparent within two years although recovery is slow and often incomplete.

Causes

Conditions resulting in the vitamin deficiency and its effects include chronic alcoholism, and severe malnutrition. Alcoholism is often an indicator of poor nutrition, which in addition to inflammation of the stomach lining, causes thiamine deficiency.^[3] As well as alcohol abuse, causes include dietary deficiencies, prolonged vomiting, eating disorders, or the effects of chemotherapy. It can also occur in pregnant women who have a form of extreme morning sickness known as hyperemesis gravidarum.^[4] Mercury poisoning can also cause it. It has also been caused by centipede (mukade) bites in Japan^[5].

Due to malnutrition and a lack of Thiamine, the hippocampus begins to decay, leaving holes that disallows one's rehearsed information within short term memory to transfer to long term memory (anterograde amnesia). However long term memory prior to the disorder can remain intact.

Prevention

The most effective method of preventing Korsakoff's syndrome is to avoid B vitamin/thiamine deficiency. In Western nations the most common cause of such deficiency is alcoholism. Thiamine was introduced to alcoholic beverages in the U.S. for a time, but this does not appear to have an effect on Korsakoff's syndrome, as the cause of Korsakoff's syndrome in chronic alcoholics is not a deficiency of thiamine in the diet, but rather a reduction in the body's ability to absorb thiamine in the intestine.^[4] In the U.S., government mandates to adding thiamine to alcoholic beverages have been blocked for this reason and also by political groups asserting that such supplementation would encourage alcohol use.^{citation needed}

Also, alcohol is, by itself, neurotoxic. It will cause neural damage taken in excess, especially in the hippocampus. The body responds to alcohol ingestion by releasing cortisol as a neuroprotective mechanism (animals that have been adrenalectomized may be killed by a fraction of the dose of alcohol that an unadrenalectomized animal may tolerate). Cortisol, specifically, has been shown to cause irreversible damage to the hippocampus when present in large amounts for extended periods of time.^[6] Alcohol in excess may be causal in and of itself in Korsakoff dementia regardless of thiamine addition to spirits. Alcohol toxicity is cumulative; cessation of its use, or never having used it, will reduce risk of korsakoff and other dementias.

Case studies

A famous case study is recounted by Oliver Sacks in "The Lost Mariner", which can be found in The Man Who Mistook His Wife for a Hat.

Other cases include that of artist Charles Blackman^[7] and entertainer Graham Kennedy.^[8]

In popular culture

• In the fictional show House, episode "Histories", the clinic patient suffers from Korsakoff's as a result of malnutrition.
• In the novel Mona Lisa Overdrive, the character Slick was incarcerated in a "chemo-penal unit" with "induced Korsakov's" (sic).
• In the British movie The Wrong Box, Peter Sellers plays a doctor suffering from Korsakoff's syndrome, who is reduced to signing reports of more than questionable medical content, while telling everybody of his former lavish Mayfair office, where only the Lords and Counts used to be his clientèle.

References

External links

• Strangers is a play by Edward Einhorn based on the syndrome.
• The Merck Manual: Function And Dysfunction Of The Cerebral Lobes - Amnesias - Korsakoff's syndrome
• Simple description of Korsakoff's syndrome
• Citizendium's article on Korsakoff's syndrome

v • d • e WHO ICD-10 mental and behavioral disorders (F · 290–319) Neurological/symptomatic Dementia (Alzheimer's disease, multi-infarct dementia, Pick's disease, Creutzfeldt-Jakob disease, Huntington's disease, Parkinson's disease, AIDS dementia complex, Frontotemporal dementia, Elopement, Sundowning, Wandering) · Delirium · Post-concussion syndrome · Organic brain syndrome Psychoactive substance alcohol (drunkenness, alcohol dependence, alcoholic hallucinosis, Alcohol withdrawal, delirium tremens, Korsakoff's syndrome, alcohol abuse) · opioids (opioid dependency) · sedative/hypnotic (benzodiazepine withdrawal) · cocaine (cocaine dependence) · general (Intoxication, Drug abuse, Physical dependence, Withdrawal) Psychotic disorder Schizophrenia (disorganized schizophrenia)  · Schizophreniform disorder  · Schizotypal personality disorder · Delusional disorder · Folie à deux · Schizoaffective disorder Mood (affective) Mania · Bipolar disorder · Clinical depression · Cyclothymia · Dysthymia Neurotic, stress-related and somatoform Anxiety disorder (Agoraphobia, Panic disorder, Panic attack, Generalized anxiety disorder, Social anxiety, Social phobia) · OCD · Acute stress reaction · PTSD · Adjustment disorder · Conversion disorder (Ganser syndrome) · Somatoform disorder (Somatization disorder, Body dysmorphic disorder, Hypochondriasis, Nosophobia, Da Costa's syndrome, Psychalgia)  · Neurasthenia Physiological/physical behavioral Eating disorder: Anorexia nervosa · Bulimia nervosa Sleep disorder: Dyssomnia (Hypersomnia, Insomnia) · Parasomnia (REM behavior disorder, Night terror) · Nightmare Sexual dysfunction: Erectile dysfunction · Premature ejaculation · Vaginismus · Dyspareunia · Hypersexuality · Female sexual arousal disorder Postpartum depression · Postnatal psychosis Adult personality and behavior Personality disorder · Passive-aggressive behavior · Kleptomania · Trichotillomania · Voyeurism · Factitious disorder · Munchausen syndrome · Ego-dystonic sexual orientation · Fetishism Mental retardation Mental retardation Psychological development (developmental disorder) Specific: speech and language (expressive language disorder, aphasia, expressive aphasia, receptive aphasia, Landau-Kleffner syndrome, lisp) · Scholastic skills (dyslexia, dysgraphia, Gerstmann syndrome) · Motor function (developmental dyspraxia) Pervasive: Autism · Rett syndrome · Asperger syndrome Behavioral and emotional, childhood and adolescence onset ADHD · Conduct disorder · Oppositional defiant disorder · Separation anxiety disorder · Selective mutism · Reactive attachment disorder · Tic disorder · Tourette syndrome · Speech (stuttering · cluttering)

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