Korsakoff's psychosis and syndrome

Korsakoff’s syndrome is a condition that mainly affects chronic alcoholics. It is due to the direct effects of alcohol or to the severe nutritional deficiencies that are associated with chronic alcoholism, and has been seen in association with vitamin B deficiency. The syndrome also occurs in other severe brain disturbances, e.g. paralysis, dementia, brain damage, infections and poisonings. In chronic alcoholism the condition usually occurs following delirium tremens.

The syndrome is characterized by a severe memory defect, especially for recent event, for which the patient compensates by confabulation - the reciting of imaginary experiences. Psychologists working with these patients often have great problems sorting out what is truth and what is lies of what patients say - this is story-telling on a high level. Other symptoms may include delirium, anxiety, fear, depression, confusion, delusions and insomnia; painful extremities, sometimes bilateral wrist drop, more frequent bilateral foot drop with pain or pressure over the long nerves. Confabulation, a requisite for the classic syndrome, is not always present in mild conditions. With or without polyneuropathy. Korsakoff’s description of 1887 was of a case with polyneuritis.

An early mention of the condition was provided in 1822 by James Jackson (1777-1867), physician at Massachusetts General Hospital and professor at Boston Medical School, in a review of the peripheral neuritis of alcoholism. Subsequently, in 1868, Sir Samuel Wilks, physician at Guy's Hospital, London, gave an account of the characteristic mental symptoms in an article on alcoholic paraplegia.

Korsakoff in his doctoral thesis called the condition “cerebropathia psychica toxaemica.” The eponymic term Korsakoff’s psychosis was introduced by Friedrich Jolly (1844-1904). Korsakoff, however, gave priority to the Swedish physician Magnus Huss (1807-1890).

Korsakoff emphasised the association of alcoholic polyneuropathy with a specific pattern of mental disturbance as follows: "This mental disorder appears at times in the form of sharply delineated irritable weakness of the mental sphere, at times in the form of confusion with characteristic mistakes in orientation for place, time and situation, and at times as an almost pure form of acute amnesia, where the recent memory is most severely involved, while the remote memory is well preserved . . . Some have suffered so widespread memory loss that they literally forget everything immediately."

When the eponym was established, the term "Korsakoff's psychosis" was used for the combination of alcoholic polyneuropathy and characteristic mental disturbance, while "Korsakoff's syndrome" was the non-alcoholic form, with or without polyneuritis, which may result from acquired conditions, such as head injury, brain tumours and encephalitis.

The natural history of the disorder in 245 affected persons, in 82 of whom autopsy was undertaken, was documented by Victor et al. in 1971. Blass and Gibson in 1977 identified a genetically determined disturbance of transketolase, a thiamine pyrophosphate-binding factor in fibroblasts from affected persons. It is now assumed that persons who are homozygous for this defects are at increased risk of developing thiamine deficiency in circumstances of dietary inadequacy, notably alcoholism. In this way Korsakoff psychosis can be regarded as an autosomal recessive disorder, which manifests in genetically predisposed persons in whom alcoholism leads to thiamine deficiency.

• James Jackson:
  On a peculiar disease resulting from the use of ardent spirits.
  New England Journal of Medicine and Surgery and the collateral branches of science, Boston. 1822, 2: 351-353.
• M. Huss:
  Alcoholismus chronicus eller kronisk alkoholsjukdom.
  2 parts; Stockholm, 1849, 1851.
  German translation by Gerhard von Dem Busch (1791-1868), Leipzig, 1852.
  This work earned Huss a Monthyon prize from the Institut de France.
• Sir Samuel Wilks (1824-1911):
  Alcoholic paraplegia.
  Medical Times and Gazette, London, 1868, 2: 467-472.
• S. S. Korsakoff:
  Ob alkogol’nom paraliche.
  [Disturbance of psychic activity in alcoholic paralysis and its relation to the disturbance of the psychic sphere in multiple neuritis of nonalcoholic origin.]
  Vestnick klin. psychiat neurol, Moscow, 1887, volume 4, No. 2: 1-102.
  Ezhened. klin. Gaz., 1889, 9: 85, 115, 136.
  English translation in: Neurology, Cleveland, Ohio, 1955, 5: 395-406. Psychic disorder in conjunction with multiple neuritis (Psychosis Polyneuritica S Cerebropathia Psychica Toxaemica).
  Medizinskoje Obozrenije, 1889, 31, No. 13. Über eine besondere Form psychiser Störung kombiniert mit multipler Neuritis.
  Archiv für Psychiatrie und Nervenkrankheiten, Berlin, 1890, 21: 669-704. Über Erinnerungsstörungen (Pseudoreminiszenzen) bei polyneuritischen Psychosen.
  Allgemeine Zeitschrift für Psychiatrie, Berlin, 1891, 47.
• F. Jolly:
  Irrthum und Wahnsinn. 1893.
• J. P. Blass, G. E. Gibson:
  Abnormality of a thiamine-requiring enzyme in patients with Wernicke-Korsakoff syndrome.
  New England Journal of Medicine, Boston, 1977, 297: 1367-1370.