Korsakoff syndrome

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Korsakoff syndrome
Other namesAlcoholic Korsakoff syndrome (AKS), Korsakov syndrome, Alcohol amnestic disorder
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Thiamine
Specialty Psychiatry

Korsakoff syndrome (KS) [1] is a disorder of the central nervous system characterized by amnesia, deficits in explicit memory, and confabulation. This neurological disorder is caused by a deficiency of thiamine (vitamin B1) in the brain, and it is typically associated with and exacerbated by the prolonged, excessive ingestion of alcohol. [2] Korsakoff syndrome is often accompanied by Wernicke encephalopathy; this combination is called Wernicke–Korsakoff syndrome. [2]

Contents

Korsakoff syndrome is named after Sergei Korsakoff, the Russian neuropsychiatrist who described it during the late 19th century. [3]

Signs and symptoms

There are seven major symptoms of Korsakoff syndrome, an amnestic-confabulatory syndrome:[ citation needed ]

Benon R. and LeHuché R. (1920) described the characteristic signs of Korsakoff syndrome with some additional features including: confabulation (false memories), fixation amnesia, paragnosia or false recognition of places, mental excitation, and euphoria. [7]

Thiamine is essential for the decarboxylation of pyruvate, and deficiency during this metabolic process is thought to cause damage to the medial thalamus and mammillary bodies of the posterior hypothalamus, as well as generalized cerebral atrophy. [8] These brain regions are all parts of the limbic system, which is heavily involved in emotion and memory.

KS involves neuronal loss, that is, damage to neurons; gliosis, which is a result of damage to supporting cells of the central nervous system, and bleeding also occurs in mammillary bodies. Damage to the medial dorsal nucleus or anterior nuclei of the thalamus (limbic-specific nuclei) is also associated with this disorder. Cortical dysfunction may have arisen from thiamine deficiency, alcohol neurotoxicity, and/or structural damage in the diencephalon. [9]

Originally, it was thought that a lack of initiative and a flat affect were important characteristics of emotional presentation in those affected. Studies have questioned this, proposing that neither is necessarily a symptom of KS. Research suggesting that people with Korsakoff syndrome are emotionally unimpaired has made this a controversial topic. It can be argued that apathy, which is a usual characteristic, reflects a deficit of emotional expressions, without affecting the experience or perception of emotion. [10]

KS causes deficits in declarative memory in most people, [11] but leaves implicit spatial, verbal, and procedural memory functioning intact. [12] People with KS have deficits in the processing of contextual information. Context memories refers to the where and when of experiences, and is an essential part of recollection. The ability to store and retrieve this information, such as spatial location or temporal order information, is impaired. [13] Research has also suggested that people with Korsakoff syndrome have impaired executive functions, which can lead to behavioral problems and interfere with daily activities. It is unclear, however, which executive functions are affected most. [14] Nonetheless, IQ is usually not affected by the brain damage associated with Korsakoff's syndrome. [15]

At first it was thought that those with KS used confabulation to fill in memory gaps. However, it has been found that confabulation and amnesia do not necessarily co-occur. Studies have shown that there is dissociation between provoked confabulation, spontaneous confabulation (which is unprovoked), and false memories. [11] That is, people affected could be led to believe certain things had happened which actually had not, but so could people without Korsakoff syndrome.[ citation needed ]

Causes

Conditions resulting in thiamine deficiency and its effects include chronic alcoholism and severe malnutrition. [16] Alcoholism may co-occur with poor nutrition, which in addition to inflammation of the stomach lining, causes thiamine deficiency. [17] Other causes include dietary deficiencies, prolonged vomiting, eating disorders, and the effects of chemotherapy. It can also occur in pregnant women who have a form of extreme morning sickness known as hyperemesis gravidarum. [18] Mercury poisoning can also lead to Korsakoff syndrome. [19] Though it does not always co-occur, this disorder can emerge frequently as a consequential result of Wernicke's encephalopathy. [20]

PET scans show that there is a decrease of glucose metabolism in the frontal, parietal and cingulated regions of the brain in those with Korsakoff syndrome. This may contribute to memory loss and amnesia. Structural neuroimaging has also shown the presence of midline diencephalic lesions and cortical atrophy. [9]

Structural lesions of the central nervous system, though rare, can also contribute to symptoms of KS. Severe damage to the medial dorsal nucleus inevitably results in memory deficit. Additionally, autopsies of people who had KS have showed lesions in both the midline and anterior thalamus, and thalamic infarctions. Bilateral infarctions to the thalamus can result in Korsakoff-induced amnesia as well. These findings imply damage to anterior thalamic nuclei can result in disruptive memory. [21] [22]

Risk factors

A number of factors may increase a person's risk to develop Korsakoff syndrome. These factors are often related to general health and diet. [23]

Diagnosis

KS is primarily a clinical diagnosis; imaging and lab tests are not necessary.[ citation needed ]

Prevention

The most effective method of preventing AKS is to avoid thiamine deficiency. In Western nations, the most common causes of such a deficiency are alcoholism and eating disorders. [22] Because these are behavioral-induced causes, Korsakoff syndrome is essentially considered a preventable disease. Thus, fortifying foods with thiamine, or requiring companies that sell alcoholic beverages to supplement them with B vitamins in general or thiamine in particular, could avert many cases. [24] [25]

Treatment

It was once assumed that anyone with KS would eventually need full-time care. This is still often the case, but rehabilitation can help regain some, albeit often limited, level of independence. [22] Treatment involves the replacement or supplementation of thiamine by intravenous or intramuscular 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. Treatment typically requires taking thiamine orally for 3 to 12 months, though only about 20 percent of cases are reversible. If treatment is successful, improvement will become apparent within two years, although recovery is slow and often incomplete.[ citation needed ]

As an immediate form of treatment, a pairing of intravenous or intramuscular thiamine with a high concentration of B-complex vitamins can be administered three times daily for 2–3 days. In most cases, an effective response will be observed. A single dose of 1 gram of thiamine can also be administered to achieve a clinical response. [26] In those who are seriously malnourished, the sudden availability of glucose without proper bodily levels of thiamine to metabolize is thought to cause damage to cells. Thus, the administration of thiamine along with an intravenous form of glucose is often good practice. [27]

Treatment for the memory aspect of KS can also include domain-specific learning, which when used for rehabilitation is called the method of vanishing cues. Such treatments aim to use intact memory processes as the basis for rehabilitation. Those who used the method of vanishing cues in therapy were found to learn and retain information more easily. [28]

People diagnosed with KS are reported to have a normal life expectancy, presuming that they abstain from alcohol and follow a balanced diet. Empirical research has suggested that good health practices have beneficial effects in Korsakoff syndrome. [27]

Epidemiology

Rates varies between countries, but it is estimated to affect around 12.5% of heavy drinkers. [29]

Related Research Articles

<span class="mw-page-title-main">Brain damage</span> Destruction or degeneration of brain cells

Neurotrauma, brain damage or brain injury (BI) is the destruction or degeneration of brain cells. Brain injuries occur due to a wide range of internal and external factors. In general, brain damage refers to significant, undiscriminating trauma-induced damage.

<span class="mw-page-title-main">Wernicke–Korsakoff syndrome</span> Combined presence of Wernickes encephalopathy (WE) and Korsakoffs syndrome

Wernicke-Korsakoff syndrome (WKS) is the combined presence of Wernicke encephalopathy (WE) and Korsakoff syndrome. Due to the close relationship between these two disorders, people with either are usually diagnosed with WKS as a single syndrome. It mainly causes vision changes, ataxia and impaired memory.

Encephalopathy means any disorder or disease of the brain, especially chronic degenerative conditions. In modern usage, encephalopathy does not refer to a single disease, but rather to a syndrome of overall brain dysfunction; this syndrome has many possible organic and inorganic causes.

<span class="mw-page-title-main">Wernicke encephalopathy</span> Medical condition

Wernicke encephalopathy (WE), also Wernicke's encephalopathy, or wet brain is the presence of neurological symptoms caused by biochemical lesions of the central nervous system after exhaustion of B-vitamin reserves, in particular thiamine (vitamin B1). The condition is part of a larger group of thiamine deficiency disorders that includes beriberi, in all its forms, and alcoholic Korsakoff syndrome. When it occurs simultaneously with alcoholic Korsakoff syndrome it is known as Wernicke–Korsakoff syndrome.

<span class="mw-page-title-main">Mammillary body</span> Part of the limbic system

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. They consist of two groups of nuclei, the medial mammillary nuclei and the lateral mammillary nuclei.

<span class="mw-page-title-main">Alcoholic polyneuropathy</span> Medical condition

Alcoholic polyneuropathy is a neurological disorder in which peripheral nerves throughout the body malfunction simultaneously. It is defined by axonal degeneration in neurons of both the sensory and motor systems and initially occurs at the distal ends of the longest axons in the body. This nerve damage causes an individual to experience pain and motor weakness, first in the feet and hands and then progressing centrally. Alcoholic polyneuropathy is caused primarily by chronic alcoholism; however, vitamin deficiencies are also known to contribute to its development. This disease typically occurs in chronic alcoholics who have some sort of nutritional deficiency. Treatment may involve nutritional supplementation, pain management, and abstaining from alcohol.

Cerebral atrophy is a common feature of many of the diseases that affect the brain. Atrophy of any tissue means a decrement in the size of the cell, which can be due to progressive loss of cytoplasmic proteins. In brain tissue, atrophy describes a loss of neurons and the connections between them. Brain atrophy can be classified into two main categories: generalized and focal atrophy. Generalized atrophy occurs across the entire brain whereas focal atrophy affects cells in a specific location. If the cerebral hemispheres are affected, conscious thought and voluntary processes may be impaired.

Memory disorders are the result of damage to neuroanatomical structures that hinders the storage, retention and recollection of memories. Memory disorders can be progressive, including Alzheimer's disease, or they can be immediate including disorders resulting from head injury.

<span class="mw-page-title-main">Organic brain syndrome</span> Disorder of mental function whose cause is alleged to be known as physiological

Organic brain syndrome, also known as organic brain disease, organic brain damage, organic brain disorder, organic mental syndrome, or organic mental disorder, refers to any syndrome or disorder of mental function whose cause is alleged to be known as organic (physiologic) rather than purely of the mind. These names are older and nearly obsolete general terms from psychiatry, referring to many physical disorders that cause impaired mental function. They are meant to exclude psychiatric disorders. Originally, the term was created to distinguish physical causes of mental impairment from psychiatric disorders, but during the era when this distinction was drawn, not enough was known about brain science for this cause-based classification to be more than educated guesswork labeled with misplaced certainty, which is why it has been deemphasized in current medicine. While mental or behavioural abnormalities related to the dysfunction can be permanent, treating the disease early may prevent permanent damage in addition to fully restoring mental functions. An organic cause to brain dysfunction is suspected when there is no indication of a clearly defined psychiatric or "inorganic" cause, such as a mood disorder.

<span class="mw-page-title-main">Sergei Korsakov</span>

Sergei Sergeyevich Korsakov was a neuropsychiatrist from the Russian Empire, known for his studies on alcoholic psychosis. His name is lent to the eponymous Korsakov's syndrome and Wernicke–Korsakoff syndrome.

<span class="mw-page-title-main">Thiamine deficiency</span> Human disease

Thiamine deficiency is a medical condition of low levels of thiamine (Vitamin B1). A severe and chronic form is known as beriberi. The two main types in adults are wet beriberi and dry beriberi. Wet beriberi affects the cardiovascular system, resulting in a fast heart rate, shortness of breath, and leg swelling. Dry beriberi affects the nervous system, resulting in numbness of the hands and feet, confusion, trouble moving the legs, and pain. A form with loss of appetite and constipation may also occur. Another type, acute beriberi, found mostly in babies, presents with loss of appetite, vomiting, lactic acidosis, changes in heart rate, and enlargement of the heart.

A banana bag is a bag of IV fluids containing vitamins and minerals. The bags typically contain thiamine, folic acid, and magnesium sulfate, and are usually used to correct nutritional deficiencies or chemical imbalances in the human body. The solution has a yellow color, hence the term "banana bag".

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Marchiafava–Bignami disease is a progressive neurological disease of alcohol use disorder, characterized by corpus callosum demyelination and necrosis and subsequent atrophy. The disease was first described in 1903 by the Italian pathologists Amico Bignami and Ettore Marchiafava in an Italian Chianti drinker. In this autopsy, Marchiafava and Bignami noticed that the middle two-thirds of the corpus callosum were necrotic. It is very difficult to diagnose and there is no specific treatment. Until 2008 only around 300 cases had been reported. If caught early enough, most patients survive.

<span class="mw-page-title-main">Medial dorsal nucleus</span>

The medial dorsal nucleus is a large nucleus in the thalamus.

Alcohol-related dementia (ARD) is a form of dementia caused by long-term, excessive consumption of alcoholic beverages, resulting in neurological damage and impaired cognitive function.

<span class="mw-page-title-main">Alcohol withdrawal syndrome</span> Medical condition

Alcohol withdrawal syndrome (AWS) is a set of symptoms that can occur following a reduction in alcohol use after a period of excessive use. Symptoms typically include anxiety, shakiness, sweating, vomiting, fast heart rate, and a mild fever. More severe symptoms may include seizures, and delirium tremens (DTs); which can be fatal in untreated patients. Symptoms start at around 6 hours after last drink. Peak incidence of seizures occurs at 24-36 hours and peak incidence of delirium tremens is at 48-72 hours.

Amnesia is a deficit in memory caused by brain damage or brain diseases, but it can also be temporarily caused by the use of various sedatives and hypnotic drugs. The memory can be either wholly or partially lost due to the extent of damage that was caused.

<span class="mw-page-title-main">Nutritional neuroscience</span> Scientific discipline

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In psychology, confabulation is a memory error consisting of the production of fabricated, distorted, or misinterpreted memories about oneself or the world. It is generally associated with certain types of brain damage or a specific subset of dementias. While still an area of ongoing research, the basal forebrain is implicated in the phenomenon of confabulation. People who confabulate present with incorrect memories ranging from subtle inaccuracies to surreal fabrications, and may include confusion or distortion in the temporal framing of memories. In general, they are very confident about their recollections, even when challenged with contradictory evidence.

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References

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  2. 1 2 "Korsakoff Syndrome". Alzheimer's Association. Retrieved 24 January 2023.
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  10. ↑ Doulas, J.; Wilkinson, D. A. (1993). "Evidence of normal emotional responsiveness in alcoholic Korsakoff's syndrome in the presence of profound memory impairment". Addiction. 88 (12): 1637–1645. doi:10.1111/j.1360-0443.1993.tb02038.x. PMID   8130702.
  11. 1 2 Kessels, Roy P. C.; Kortrijk, Hans E.; Wester, Arie J.; Nys, Gudrun M. S. (1 April 2008). "Confabulation behavior and false memories in Korsakoff's syndrome: Role of source memory and executive functioning". Psychiatry and Clinical Neurosciences. 62 (2): 220–225. doi: 10.1111/j.1440-1819.2008.01758.x . PMID   18412846.
  12. ↑ Oudman, Erik; Van Der Stigchel, Stefan; Wester, Arie J.; Kessels, Roy P.C.; Postma, Albert (2011). "Intact memory for implicit contextual information in Korsakoff's amnesia". Neuropsychologia. 49 (10): 2848–2855. doi:10.1016/j.neuropsychologia.2011.06.010. PMID   21704050. S2CID   14415344.
  13. ↑ Parkin A. J.; Montaldi D.; Leng N. R.; Hunkin N. M. (1999). "Contextual cueing effects in the remote memory of alcoholic Korsakoff patients and normal subjects". The Quarterly Journal of Experimental Psychology. 42A (3): 585–596. doi:10.1080/14640749008401238. PMID   2236634. S2CID   11488963.
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  19. ↑ ATSDR. 1999. Toxicological Profile for Mercury. Atlanta, GA:Agency for Toxic Substances and Disease Registry. http://www.atsdr.cdc.gov/toxprofiles/tp46.pdf
  20. ↑ Pitel A. L.; Zahr N. M.; Jackson K.; Sassoon S. A.; Rosenbloom M. J.; Pfefferbaum A.; Sullivan E. V. (2011). "Signs of preclinical Wernicke's encephalopathy and thiamine levels as predictors of neuropsychological deficits in alcoholism without Korsakoff's syndrome". Neuropsychopharmacology. 36 (3): 580–588. doi:10.1038/npp.2010.189. PMC   3055684 . PMID   20962766.
  21. ↑ Rahme, R; Moussa, R; Awada, A; Ibrahim, I; Ali, Y; Maarrawi, J; Rizk, T; Nohra, G; Okais, N; Samaha, E (April 2007). "Acute Korsakoff-like amnestic syndrome resulting from left thalamic infarction following a right hippocampal hemorrhage". AJNR. American Journal of Neuroradiology. 28 (4): 759–60. PMC   7977335 . PMID   17416834.
  22. 1 2 3 Kopelman, MD; Thomson, AD; Guerrini, I; Marshall, EJ (Mar–Apr 2009). "The Korsakoff syndrome: clinical aspects, psychology and treatment". Alcohol and Alcoholism. 44 (2): 148–54. doi: 10.1093/alcalc/agn118 . PMID   19151162.
  23. ↑ Rosenblum, Laurie B. (March 2011). "Korsakoff's Syndrome". NYU Langone Medical Center. Archived from the original on April 26, 2012. Retrieved February 12, 2012.
  24. ↑ Harper, CG; Sheedy, DL; Lara, AI; Garrick, TM; Hilton, JM; Raisanen, J (Jun 1, 1998). "Prevalence of Wernicke-Korsakoff syndrome in Australia: has thiamine fortification made a difference?". The Medical Journal of Australia. 168 (11): 542–5. doi:10.5694/j.1326-5377.1998.tb139081.x. PMC   3391549 . PMID   9640303.
  25. ↑ Centerwall, BS; Criqui, MH (1978). "Prevention of the Wernicke-Korsakoff syndrome: a cost-benefit analysis". New England Journal of Medicine. 299 (6): 285–9. doi:10.1056/nejm197808102990605. PMID   96343.
  26. ↑ Carlson, N. R. (2013). Physiology of behavior. Boston: Pearson. 547.
  27. 1 2 Cook, CC (May–Jun 2000). "Prevention and treatment of Wernicke-Korsakoff syndrome". Alcohol and Alcoholism Supplement. 35 (1): 19–20. doi:10.1093/alcalc/35.Supplement_1.19. PMID   11304070. S2CID   45726575.
  28. ↑ Komatsu, Shin-Ichi; Mimura, Masaru; Kato, Motoichiro; Wakamatsu, Naoki; Kashima, Haruo (1 March 2000). "Errorless and Effortful Processes Involved in the Learning of Face-name Associations by Patients with Alcoholic Korsakoff's Syndrome". Neuropsychological Rehabilitation. 10 (2): 113–132. doi:10.1080/096020100389200. S2CID   145322185.
  29. ↑ Harper, C; Gold, J; Rodriguez, M; Perdices, M (1 February 1989). "The prevalence of the Wernicke-Korsakoff syndrome in Sydney, Australia: a prospective necropsy study". Journal of Neurology, Neurosurgery & Psychiatry. 52 (2): 282–285. doi:10.1136/jnnp.52.2.282. PMC   1032524 . PMID   2784828.
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