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Korsakoff's syndrome

Korsakoff's syndrome

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Korsakoff's syndrome is a neurological disorder caused by the lack of thiamine (vitamin B1)
Thiamine
Thiamine or thiamin or vitamin B1 , named as the "thio-vitamine" is a water-soluble vitamin of the B complex. First named aneurin for the detrimental neurological effects if not present in the diet, it was eventually assigned the generic descriptor name vitamin B1. Its phosphate derivatives are...

 in the brain
Brain
The brain is the center of the nervous system in all vertebrate and most invertebrate animals—only a few primitive invertebrates such as sponges, jellyfish, sea squirts and starfishes do not have one. It is located in the head, usually close to primary sensory apparatus such as vision, hearing,...

. Its onset is linked to chronic alcohol abuse and/or severe malnutrition. The syndrome
Syndrome
In medicine and psychology, a syndrome is the association of several clinically recognizable features, signs , symptoms , phenomena or characteristics that often occur together, so that the presence of one or more features alerts the physician to the possible presence of the others...

 is named after Sergei Korsakoff
Sergei Korsakoff
Sergei Sergeievich Korsakoff was a Russian neuropsychiatrist.Sergei Korsakoff was the first of great Russian neuropsychiatrists. He studied medicine at the University of Moscow, graduated in 1875 and subsequently became physician to "Preobrazhenski" mental hospital. From 1876 to 1879 he gained...

, the neuropsychiatrist
Neuropsychiatry
Neuropsychiatry is the branch of medicine dealing with mental disorders attributable to diseases of the nervous system. It preceded the current disciplines of psychiatry and neurology, in as much as psychiatrists and neurologists had a common training....

 who popularized the theory.

Symptoms


There are six major symptoms of Korsakoff's syndrome:
  1. anterograde amnesia
    Anterograde amnesia
    Anterograde amnesia is a loss of the ability to create new memories after the event that caused the amnesia, leading to a partial or complete inability to recall the recent past, while long-term memories from before the event remain intact. This is in contrast to retrograde amnesia, where memories...

  2. retrograde amnesia
    Retrograde amnesia
    Retrograde amnesia is a loss of access to events that occurred, or information that was learned, before an injury or the onset of a disease....

    , severe memory loss
  3. confabulation
    Confabulation
    Confabulation is the process in which a memory is remembered falsely. Confabulations are indicative of a complicated and intricate process that can be led astray at any given point during encoding, storage, or recall of a memory. Two distinct types of confabulation are often distinguished...

    , that is, invented memories which are then taken as true due to gaps in memory sometimes associated with blackouts
  4. meager content in conversation
    Conversation
    Conversation is a form of interactive, spontaneous communication between two or more people who are following rules of etiquette.Conversation analysis is a branch of sociology which studies the structure and organization of human interaction, with a more specific focus on conversational...

  5. lack of insight
    Insight
    Insight is the understanding of a specific cause and effect in a specific context. Insight can be used with several related meanings:*a piece of information...

  6. apathy
    Apathy
    Apathy is a state of indifference, or the suppression of emotions such as concern, excitement, motivation and passion. An apathetic individual has an absence of interest in or concern about emotional, social, spiritual, philosophical or physical life.They may lack a sense of purpose or meaning in...

     - the patients lose interest in things quickly and generally appear indifferent to change.


These symptoms are caused by a deficiency of thiamine (vitamin B1)
Thiamine
Thiamine or thiamin or vitamin B1 , named as the "thio-vitamine" is a water-soluble vitamin of the B complex. First named aneurin for the detrimental neurological effects if not present in the diet, it was eventually assigned the generic descriptor name vitamin B1. Its phosphate derivatives are...

, which is thought to cause damage to the medial
Medial
- Medial magmas :In abstract algebra, a medial magma is a set with a binary operation which satisfies the identity \cdot = \cdot , or more simply, xy\cdot uv = xu\cdot yv...

 thalamus
Thalamus
The thalamus is a midline paired symmetrical structure within the brains of vertebrates, including humans. It is situated between the cerebral cortex and midbrain, both in terms of location and neurological connections...

 and possibly to the mammillary bodies of the hypothalamus as well as generalized cerebral atrophy
Cerebral atrophy
Cerebral atrophy is a common feature of many of the diseases that affect the brain. Atrophy of any tissue means loss of cells. In brain tissue, atrophy describes a loss of neurons and the connections between them...

.

When Wernicke's encephalopathy
Wernicke's encephalopathy
Wernicke encephalopathy is a syndrome characterised by ataxia, ophthalmoplegia, confusion, and impairment of short-term memory.It is caused by lesions in the medial thalamic nuclei, mammillary bodies, periaqueductal and periventricular brainstem nuclei, and superior cerebellar vermis, often...

 accompanies Korsakoff's syndrome, the combination is called the Wernicke-Korsakoff syndrome
Wernicke-Korsakoff syndrome
Wernicke–Korsakoff syndrome is a manifestation of thiamine deficiency, or beriberi. This is usually secondary to alcohol abuse...

. 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
Gliosis
Gliosis is a proliferation of astrocytes in damaged areas of the central nervous system . This proliferation usually leads to the formation of a glial scar....

 which is a result of damage to supporting cells of the central nervous system
Central nervous system
The central nervous system is the part of the nervous system that integrates the information that it receives from, and coordinates the activity of, all parts of the bodies of bilaterian animals—that is, all multicellular animals except sponges and radially symmetric animals such as jellyfish...

; and hemorrhage or bleeding in mammillary bodies
Mammillary body
The mammillary bodies are a pair of small round bodies, located on the undersurface of the brain, that, as part of the diencephalon form part of the limbic system. They are located at the ends of the anterior arches of the fornix...

. Damage to the dorsomedial nucleus
Dorsomedial nucleus
Dorsomedial nucleus can refer to:* Dorsomedial hypothalamic nucleus* Medial dorsal nucleus of the thalamus...

 or anterior group of the thalamus
Thalamus
The thalamus is a midline paired symmetrical structure within the brains of vertebrates, including humans. It is situated between the cerebral cortex and midbrain, both in terms of location and neurological connections...

 (limbic-specific nuclei) is also associated with this disorder.

Signs

  • Apathy
    Apathy
    Apathy is a state of indifference, or the suppression of emotions such as concern, excitement, motivation and passion. An apathetic individual has an absence of interest in or concern about emotional, social, spiritual, philosophical or physical life.They may lack a sense of purpose or meaning in...

  • Ataxia
    Ataxia
    Ataxia is a neurological sign and symptom that consists of gross lack of coordination of muscle movements. Ataxia is a non-specific clinical manifestation implying dysfunction of the parts of the nervous system that coordinate movement, such as the cerebellum...

  • Coma
    Coma
    In medicine, a coma is a state of unconsciousness, lasting more than 6 hours in which a person cannot be awakened, fails to respond normally to painful stimuli, light or sound, lacks a normal sleep-wake cycle and does not initiate voluntary actions. A person in a state of coma is described as...

  • Confabulation
    Confabulation
    Confabulation is the process in which a memory is remembered falsely. Confabulations are indicative of a complicated and intricate process that can be led astray at any given point during encoding, storage, or recall of a memory. Two distinct types of confabulation are often distinguished...

  • Paralysis
    Paralysis
    Paralysis is loss of muscle function for one or more muscles. Paralysis can be accompanied by a loss of feeling in the affected area if there is sensory damage as well as motor. A study conducted by the Christopher & Dana Reeve Foundation, suggests that about 1 in 50 people have been diagnosed...

     of muscles controlling the eye
  • Retrograde
    Retrograde amnesia
    Retrograde amnesia is a loss of access to events that occurred, or information that was learned, before an injury or the onset of a disease....

     and anterograde amnesia
    Anterograde amnesia
    Anterograde amnesia is a loss of the ability to create new memories after the event that caused the amnesia, leading to a partial or complete inability to recall the recent past, while long-term memories from before the event remain intact. This is in contrast to retrograde amnesia, where memories...

  • Tremor
    Tremor
    A tremor is an involuntary, somewhat rhythmic, muscle contraction and relaxation involving to-and-fro movements of one or more body parts. It is the most common of all involuntary movements and can affect the hands, arms, eyes, face, head, vocal folds, trunk, and legs. Most tremors occur in the...

  • Anosognosia
    Anosognosia
    Anosognosia /æˌnɒsɒgˈnəʊsɪə/ is a condition in which a person who suffers disability seems unaware of the existence of his or her disability. Unlike denial, which is a defense mechanism, anosognosia is rooted in physiology...

     - Lack of insight to or awareness of the condition

Treatment


It was once assumed that anyone suffering from Korsakoff's syndrome would eventually need full time care. This is still often the case, but rehabilitation can help regain some, often limited, level of independence. Treatment involves the replacement or supplementation of thiamine
Thiamine
Thiamine or thiamin or vitamin B1 , named as the "thio-vitamine" is a water-soluble vitamin of the B complex. First named aneurin for the detrimental neurological effects if not present in the diet, it was eventually assigned the generic descriptor name vitamin B1. Its phosphate derivatives are...

 by intravenous (IV) or intramuscular (IM) injection, together with proper nutrition
Nutrition
Nutrition is the provision, to cells and organisms, of the materials necessary to support life. Many common health problems can be prevented or alleviated with a healthy diet....

 and hydration. However, the amnesia and brain damage caused by the disease does not always respond to thiamine
Thiamine
Thiamine or thiamin or vitamin B1 , named as the "thio-vitamine" is a water-soluble vitamin of the B complex. First named aneurin for the detrimental neurological effects if not present in the diet, it was eventually assigned the generic descriptor name vitamin B1. Its phosphate derivatives are...

 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
Alcoholism
Alcoholism is a broad term for problems with alcohol, and is generally used to mean compulsive and uncontrolled consumption of alcoholic beverages, usually to the detriment of the drinker's health, personal relationships, and social standing...

 and severe malnutrition
Malnutrition
Malnutrition is the condition that results from taking an unbalanced diet in which certain nutrients are lacking, in excess , or in the wrong proportions....

. Alcoholism may be an indicator of poor nutrition, which in addition to inflammation
Inflammation
Inflammation is part of the complex biological response of vascular tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. Inflammation is a protective attempt by the organism to remove the injurious stimuli and to initiate the healing process...

 of the stomach lining, causes thiamine deficiency. Other causes include dietary deficiencies, prolonged vomiting, eating disorder
Eating disorder
Eating disorders refer to a group of conditions defined by abnormal eating habits that may involve either insufficient or excessive food intake to the detriment of an individual's physical and mental health. Bulimia nervosa, anorexia nervosa, and binge eating disorder are the most common specific...

s, or the effects of chemotherapy
Chemotherapy
Chemotherapy is the treatment of cancer with an antineoplastic drug or with a combination of such drugs into a standardized treatment regimen....

. It can also occur in pregnant women who have a form of extreme morning sickness known as hyperemesis gravidarum
Hyperemesis gravidarum
Hyperemesis gravidarum is a severe form of morning sickness, with "unrelenting, excessive pregnancy-related nausea and/or vomiting that prevents adequate intake of food and fluids." Hyperemesis is considered a rare complication of pregnancy but, because nausea and vomiting during pregnancy exist...

. Mercury poisoning
Mercury poisoning
Mercury poisoning is a disease caused by exposure to mercury or its compounds. Mercury is a heavy metal occurring in several forms, all of which can produce toxic effects in high enough doses...

 can also lead to Korsakoff's syndrome. It has also been caused by centipede
Centipede
Centipedes are arthropods belonging to the class Chilopoda of the subphylum Myriapoda. They are elongated metameric animals with one pair of legs per body segment. Despite the name, centipedes can have a varying number of legs from under 20 to over 300. Centipedes have an odd number of pairs of...

 (mukade) bites in Japan.

There is no specific treatment because the previous thiamine deficiency produces irreversible damage to the medial thalamic nuclei and mammillary bodies
Mammillary body
The mammillary bodies are a pair of small round bodies, located on the undersurface of the brain, that, as part of the diencephalon form part of the limbic system. They are located at the ends of the anterior arches of the fornix...

. Mammillary body atrophy may be visible on high-resolution MRI.

Prevention


The most effective method of preventing Korsakoff's syndrome is to avoid B vitamin/thiamine deficiency. In Western nations, the most common causes of such a deficiency are alcoholism and weight disorders. 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.

Case studies


A famous case study is recounted by Oliver Sacks
Oliver Sacks
Oliver Wolf Sacks, CBE , is a British neurologist and psychologist residing in New York City. He is a professor of neurology and psychiatry at Columbia University, where he also holds the position of Columbia Artist...

 in "The Lost Mariner" and "A Matter of Identity", which can be found in The Man Who Mistook His Wife for a Hat
The Man Who Mistook His Wife for a Hat
The Man Who Mistook His Wife for a Hat and Other Clinical Tales is a 1985 book by neurologist Oliver Sacks describing the case histories of some of his patients. The title of the book comes from the case study of a man with visual agnosia...

. Other cases include German entertainer Harald Juhnke
Harald Juhnke
Harald Juhnke , actually Harry Heinz Herbert Juhnke, was a well-known German actor, comedian and entertainer.-Life:...

, artist Charles Blackman
Charles Blackman
Charles Blackman is one of the best known Australian artists still living today, especially for the famous Schoolgirl and Alice in Wonderland series of the 1950s...

, and entertainer Graham Kennedy
Graham Kennedy
Graham Cyril Kennedy, AO was an Australian radio, television and film performer, often called Gra Gra and The King of Australian television.-Childhood:...

.

In popular culture

  • Strangers is a play by Edward Einhorn
    Edward Einhorn
    Edward Einhorn is an American playwright, theater director, and novelist, noted for the comic absurdism of his drama and the imaginative richness of his literary works....

     based on the syndrome.
  • In the Half-Life 2
    Half-Life 2
    Half-Life 2 , the sequel to Half-Life, is a first-person shooter video game and a signature title in the Half-Life series. It is singleplayer, story-driven, science fiction, and linear...

     mod Korsakovia
    Korsakovia
    Korsakovia is a single-player mod for Valve Corporation's Half-Life 2. It was developed by thechineseroom, known for the mod "Dear Esther," and released for download free of charge on September 20, 2009....

    , by thechineseroom, the player character Christopher is under treatment for Korsakoff's Syndrome.
  • In the television show House, season 1 episode "Histories," Dr. House diagnoses a patient with Korsakoff's syndrome after she gives different explanations for a sprained wrist to different interns who independently take her history.
  • Internet comedy sketch group Derrick Comedy
    Derrick Comedy
    Derrick Comedy is an Internet sketch comedy group from New York University. The group has a large YouTube following and was mentioned in Rolling Stone in July 2007. Their video "Bro Rape" has garnered over 8 million views...

     features a man with Korsakoff's syndrome in their video "Progression of a Mad Hatter".
  • In the episode "Keeping the Faith" of the television show Royal Pains first aired on 06/17/10, Korsakoff's syndrome is offered as a diagnosis for a female who had previously undergone a stomach reduction surgery.
  • In the novel Now Wait for Last Year
    Now Wait for Last Year
    Now Wait for Last Year is a 1966 science fiction novel by Philip K. Dick. The novel describes a future where the planet Earth has allied with an extraterrestrial species known as 'Starmen in their war against another species, the Reegs.-Plot summary:...

    by Philip K. Dick, The main character's wife is diagnosed with the syndrome after years of drug and alcohol abuse.
  • It is probable that Boston Corbett
    Boston Corbett
    Thomas P. "Boston" Corbett was the Union Army soldier who shot and killed Abraham Lincoln's assassin, John Wilkes Booth. He disappeared after 1888, but circumstantial evidence suggests that he died in the Great Hinckley Fire in 1894, although this remains impossible to substantiate.-Early...

    , the man who shot John Wilkes Booth
    John Wilkes Booth
    John Wilkes Booth was an American stage actor who assassinated President Abraham Lincoln at Ford's Theatre, in Washington, D.C., on April 14, 1865. Booth was a member of the prominent 19th century Booth theatrical family from Maryland and, by the 1860s, was a well-known actor...

    , suffered from Korsakoff's syndrome later in life due to his pre-and-post war profession as a hatter, and his eventual madness.
  • Davey Volner winner of the 81st Glascock Prize
    Glascock Prize
    The Glascock Poetry Prize is awarded to the winner of the annual Kathryn Irene Glascock Intercollegiate Poetry Contest at Mount Holyoke College...

     wrote a poem titled "Korsakoff's Syndrome".
  • Dr. Butz, the attending physician in the movie, Critical Care (1997), requests the etiology and symptoms of Korsakoff's syndrome of his resident, Dr. Ernst.
  • In the novel Mona Lisa Overdrive
    Mona Lisa Overdrive
    Mona Lisa Overdrive is a cyberpunk novel by William Gibson published in 1988 and the final novel of the Sprawl trilogy, following Neuromancer and Count Zero. It takes place eight years after the events of Count Zero and is set, as were its predecessors, in The Sprawl...

    by William Gibson
    William Gibson
    William Gibson is an American-Canadian science fiction author.William Gibson may also refer to:-Association football:*Will Gibson , Scottish footballer...

    , as punishment for stealing cars prior to the events in the novel, the character Slick Henry was subjected to a process that induced Korsakoff's syndrome in him. He features the episodic memory loss characteristic of the syndrome, and builds cybernetic sculptures as therapy.

External links