Korsakoff syndrome

See also: Wernicke–Korsakoff syndrome
Korsakoff syndrome
Other names: Alcoholic Korsakoff syndrome (AKS),[1] Korsakoff's amnesic syndrome,[2] Korsakov syndrome, alcohol amnestic disorder
Thiamine
SpecialtyPsychiatry
DurationLong-term[2]
CausesThiamine deficiency (vitamin B1)[2]
Risk factorsAlcoholism, malnutrition, eating disorder, chemotherapy, long term vomiting[2]
Diagnostic methodBased on symptoms after ruling out other possible cases[3]
Differential diagnosisHead injury, multiple sclerosis, schizophrenia, intoxication, seizure[3]
TreatmentThiamine, memory rehabilitation, institutionalization[3]
Frequency<2%[3]

Korsakoff syndrome (KS) is a disorder characterized by memory loss, tremor, disorientation, decreased inhibition, and vision problems.[2][3] This may result in the classic presentation of confabulation, were memory gaps are replaced with untrue information.[3] It is generally a long term, often permanent disorder.[2][3]

It occurs due to deficiency of thiamine (vitamin B1), often due to alcoholism, though other causes may also result in the condition.[2][3] The underlying mechanism involves damage to cells in the brain and spinal cord.[2] It is part of a spectrum of disease known as Wernicke–Korsakoff syndrome, together with Wernicke encephalopathy.[2] Diagnosis is based on symptoms after ruling out other causes.[3]

Treatment is initially with thiamine.[3] Electrolytes may also require replacement.[3] Long term measures may include memory rehabilitation and institutionalization.[3] Korsakoff syndrome occurs in about 80% of people with Wernicke encephalopathy that goes untreated.[4] Less than 2% of people are affected.[3] It is named after Sergei Korsakoff, a Russian neuropsychiatrist who described it in the late 19th century.[5]

Signs and symptoms

There are seven major symptoms of Korsakoff syndrome, an amnestic-confabulatory syndrome:

  1. anterograde amnesia, memory loss for events after the onset of the syndrome
  2. retrograde amnesia, memory loss extends back for some time before the onset of the syndrome
  3. amnesia of fixation, also known as fixation amnesia (loss of immediate memory, a person being unable to remember events of the past few minutes)[6][7][8]
  4. confabulation, that is, invented memories which are then taken as true, due to gaps in memory, with such gaps sometimes associated with blackouts
  5. minimal content in conversation
  6. lack of insight
  7. apathy – interest in things is quickly lost, and there is an indifference to change

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.[9]

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.[10] 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.[11]

Originally, it was thought that a lack of initiative and a flat affect were important characteristics of emotional presentation in sufferers. 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.[12]

KS causes deficits in declarative memory in most people,[13] but leaves implicit spatial, verbal, and procedural memory functioning intact.[14] 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.[15] 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.[16] Nonetheless, IQ is usually not affected by the brain damage associated with Korsakoff's syndrome.[17]

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.[13] That is, people affected could be led to believe certain things had happened which actually had not, but so could people without Korsakoff syndrome.

Causes

Conditions resulting in thiamine deficiency and its effects include chronic alcoholism and severe malnutrition.[18] Alcoholism may co-occur with poor nutrition, which in addition to inflammation of the stomach lining, causes thiamine deficiency.[19] 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.[20] Mercury poisoning can also lead to Korsakoff syndrome.[21] Though it does not always co-occur, this disorder can emerge frequently as a consequential result of Wernicke's encephalopathy.[22]

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.[11]

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.[23][24]

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.[25]

  • Age
  • Alcoholism
  • Chemotherapy
  • Dialysis
  • Extreme dieting
  • Genetic factors

Diagnosis

KS is primarily a clinical diagnosis; imaging and lab tests are not necessary.

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.[24] 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.[26][27]

Treatment

It was once assumed that anyone suffering from 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.[24] 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.

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.[28] 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.[29]

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.[30]

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.[29]

Epidemiology

Rates varies between countries, but it is estimated to affect around 12.5% of heavy drinkers.[31]

References

  1. ↑ "Korsakoff Syndrome - MeSH - NCBI". www.ncbi.nlm.nih.gov. Archived from the original on 2022-02-19. Retrieved 2021-12-20.
  2. 1 2 3 4 5 6 7 8 9 "Wernicke-Korsakoff Syndrome Information Page | National Institute of Neurological Disorders and Stroke". www.ninds.nih.gov. Archived from the original on 14 January 2022. Retrieved 20 February 2022.
  3. 1 2 3 4 5 6 7 8 9 10 11 12 13 Covell, Timothy; Siddiqui, Waquar (2022). "Korsakoff Syndrome". StatPearls. StatPearls Publishing. Archived from the original on 20 February 2022. Retrieved 20 February 2022.
  4. ↑ "Korsakoff Psychosis - Special Subjects". Merck Manuals Professional Edition. Archived from the original on 20 February 2022. Retrieved 20 February 2022.
  5. ↑ Vein, Alla (2009). "Sergey Sergeevich Korsakov (1854–1900)". J Neurol. 256 (10): 1782–3. doi:10.1007/s00415-009-5289-x. PMC 2758215. PMID 19690905.
  6. ↑ Nyssen R. (1960). "[Study of "amnesia of fixation" in Korsakoff's disease by non-repeated acquisition tests]". Acta Neurologica et Psychiatrica Belgica (in français). 60: 783–793. PMID 13730001.
  7. ↑ Nyssen R. (1957). "[Experimental contribution to the study of fixation amnesia in Korsakoff's syndrome of alcoholic origin]". Acta Neurologica et Psychiatrica Belgica (in français). 57 (8): 839–66. PMID 13478443.
  8. ↑ C. W. M. Whitty; O. L. Zangwill (22 October 2013). Amnesia: Clinical, Psychological and Medicolegal Aspects. Elsevier Science. p. 76. ISBN 978-1-4831-6514-1. Archived from the original on 4 August 2020. Retrieved 20 December 2021.
  9. ↑ Benon R., LeHuché R. (1920). "Cranial Injuries and Korsakoff's Psychosis" [Traumatismes crâniens et psychose de Korsakoff]. Archives Suisses de Neurologie, Neurochirurgie et Psychiatrie (in français): 319.
  10. ↑ Kolb, Bryan; Whishaw, Ian Q. (2003). Fundamentals of human neuropsychology. New York: Worth Publishers. p. 473. ISBN 978-0-7167-5300-1. OCLC 55617319.
  11. 1 2 Paller, K. A.; Acharya, A.; Richardson, Brian C.; Plaisant, Odile; Shimamura, Arthur P.; Reed, Bruce R.; Jagust, William J. (1997). "Functional neuroimaging of cortical dysfunction in alcoholic Korsakoff's syndrome". Journal of Cognitive Neuroscience. 9 (2): 277–293. doi:10.1162/jocn.1997.9.2.277. PMID 23962017. S2CID 7588861.
  12. ↑ 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.
  13. 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.
  14. ↑ 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.
  15. ↑ 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.
  16. ↑ Kessels, R. P. C.; Van Oort, R. (2009). "Executive dysfunction in Korsakoff's syndrome: time to revise the DSM criteria for alcohol-induced persisting amnestic disorder?". International Journal of Psychiatry in Clinical Practice. 13 (1): 78–81. doi:10.1080/13651500802308290. PMID 24946125. S2CID 36438009.
  17. ↑ Oscar-Berman, M. (Jun 2012). "Function and dysfunction of prefrontal brain circuitry in alcoholic Korsakoff's syndrome". Neuropsychol Rev. 22 (2): 154–69. doi:10.1007/s11065-012-9198-x. PMC 3681949. PMID 22538385.
  18. ↑ Carlson, Neil; Birkett, Melissa (2017). Physiology of Behavior. Pearson. p. 514. ISBN 978-0-13-408091-8.
  19. ↑ "What is Korsakoff's syndrome?". Alzheimer's Society. October 2008. Archived from the original on 2010-02-23. Retrieved 2021-12-20.
  20. ↑ Jasmin, Luc (13 February 2008). "Wernicke-Korsakoff syndrome". MedlinePlus Medical Encyclopedia. United States National Library of Medicine. Archived from the original on 4 July 2016. Retrieved 16 July 2009.
  21. ↑ ATSDR. 1999. Toxicological Profile for Mercury. Atlanta, GA:Agency for Toxic Substances and Disease Registry. http://www.atsdr.cdc.gov/toxprofiles/tp46.pdf Archived 2011-07-21 at the Wayback Machine
  22. ↑ 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.
  23. ↑ 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.
  24. 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.
  25. ↑ Rosenblum, Laurie B. (March 2011). "Korsakoff's Syndrome". NYU Langone Medical Center. Archived from the original on April 26, 2012. Retrieved February 12, 2012.
  26. ↑ 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.
  27. ↑ 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.
  28. ↑ Carlson, N. R. (2013). Physiology of behavior. Boston: Pearson. 547.
  29. 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. Archived from the original on 2021-09-23. Retrieved 2021-12-20.
  30. ↑ 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.
  31. ↑ 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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