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Korsakoff psychosis

MedGen UID:
83883
Concept ID:
C0349464
Disease or Syndrome; Mental or Behavioral Dysfunction
Synonyms: Alcohol induced encephalopathy; Korsakoff Syndrome; Korsakoff's amnesic syndrome; Korsakoff's psychosis; Transketolase defect; Wernicke's encephalopathy; Wernicke-Korsakoff syndrome
Modes of inheritance:
Autosomal recessive inheritance
MedGen UID:
141025
Concept ID:
C0441748
Intellectual Product
Sources: HPO, OMIM, Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in homozygotes. In the context of medical genetics, autosomal recessive disorders manifest in homozygotes (with two copies of the mutant allele) or compound heterozygotes (whereby each copy of a gene has a distinct mutant allele).
Autosomal recessive inheritance (HPO, OMIM, Orphanet)
SNOMED CT: Korsakoff's psychosis (69482004); Wernicke-Korsakoff syndrome (69482004); Wernicke-Korsakov syndrome (69482004); Korsakoff psychosis (69482004); Korsakov psychosis (69482004)
 
OMIM®: 277730

Definition

behavior disorder caused by thiamine deficiency, most commonly due to chronic alcohol abuse; Wernicke's encephalopathy involves confusion, ataxia, nystagmus and ophthalmoplegia and is generally reversible by thiamine administration; Korsakoff's syndrome, involving severe anterograde and retrograde amnesia, may appear later and complete recovery is less frequent. [from CRISP]

Clinical features

Ptosis
MedGen UID:
2287
Concept ID:
C0005745
Disease or Syndrome
The upper eyelid margin is positioned 3 mm or more lower than usual and covers the superior portion of the iris (objective); or, the upper lid margin obscures at least part of the pupil (subjective).
Ataxia
MedGen UID:
849
Concept ID:
C0007758
Sign or Symptom
Cerebellar ataxia refers to ataxia due to dysfunction of the cerebellum. This causes a variety of elementary neurological deficits including asynergy (lack of coordination between muscles, limbs and joints), dysmetria (lack of ability to judge distances that can lead to under- oder overshoot in grasping movements), and dysdiadochokinesia (inability to perform rapid movements requiring antagonizing muscle groups to be switched on and off repeatedly).
Coma
MedGen UID:
1054
Concept ID:
C0009421
Disease or Syndrome
A coma is a deep state of unconsciousness. An individual in a coma is alive but unable to move or respond to his or her environment. Coma may occur as a complication of an underlying illness, or as a result of injuries, such as brain injury. A coma rarely lasts more than 2 to 4 weeks. The outcome for coma depends on the cause, severity, and site of the damage. People may come out of a coma with physical, intellectual, and psychological problems. Some people may remain in a coma for years or even decades. For those people, the most common cause of death is infection, such as pneumonia. NIH: National Institute of Neurological Disorders and Stroke.
Confusion
MedGen UID:
3587
Concept ID:
C0009676
Mental or Behavioral Dysfunction
Lack of clarity and coherence of thought, perception, understanding, or action.
Ophthalmoplegia
MedGen UID:
45205
Concept ID:
C0029089
Sign or Symptom
Paralysis of one or more extraocular muscles that are responsible for eye movements.
Peripheral neuropathy
MedGen UID:
18386
Concept ID:
C0031117
Disease or Syndrome
Your peripheral nerves are the ones outside your brain and spinal cord. Like static on a telephone line, peripheral nerve disorders distort or interrupt the messages between the brain and the rest of the body. . There are more than 100 kinds of peripheral nerve disorders. They can affect one nerve or many nerves. Some are the result of other diseases, like diabetic nerve problems. Others, like Guillain-Barre syndrome, happen after a virus infection. Still others are from nerve compression, like carpal tunnel syndrome or thoracic outlet syndrome. In some cases, like complex regional pain syndrome and brachial plexus injuries, the problem begins after an injury. Some people are born with peripheral nerve disorders. Symptoms often start gradually, and then get worse. They include . - Numbness. - Pain. - Burning or tingling. - Muscle weakness. - Sensitivity to touch. Treatment aims to treat any underlying problem, reduce pain and control symptoms. NIH: National Institute of Neurological Disorders and Stroke.
Psychosis
MedGen UID:
19568
Concept ID:
C0033975
Mental or Behavioral Dysfunction
Psychotic disorders are severe mental disorders that cause abnormal thinking and perceptions. People with psychoses lose touch with reality. Two of the main symptoms are delusions and hallucinations. Delusions are false beliefs, such as thinking that someone is plotting against you or that the TV is sending you secret messages. Hallucinations are false perceptions, such as hearing, seeing, or feeling something that is not there. Schizophrenia is one type of psychotic disorder. People with bipolar disorder may also have psychotic symptoms. Other problems that can cause psychosis include alcohol and some drugs, brain tumors, brain infections, and stroke. Treatment depends on the cause of the psychosis. It might involve drugs to control symptoms and talk therapy. Hospitalization is an option for serious cases where a person might be dangerous to himself or others.
Memory impairment
MedGen UID:
68579
Concept ID:
C0233794
Mental or Behavioral Dysfunction
An impairment of memory as manifested by a reduced ability to remember things such as dates and names, and increased forgetfulness.
Horizontal nystagmus
MedGen UID:
124399
Concept ID:
C0271385
Disease or Syndrome
Nystagmus consisting of horizontal to-and-fro eye movements.

Recent clinical studies

Etiology

Chawla J, Kvarnberg D
Handb Clin Neurol 2014;120:891-914. doi: 10.1016/B978-0-7020-4087-0.00059-0. PMID: 24365359
Wirz-Justice A, Schröder CM, Gasio PF, Cajochen C, Savaskan E
Am J Geriatr Psychiatry 2010 Jan;18(1):33-41. doi: 10.1097/JGP.0b013e3181b0467a. PMID: 19910886
Jauhar P, Montaldi D
Alcohol Alcohol Suppl 2000 May-Jun;35(1):21-3. PMID: 11304072
Lal S, Wood PL, Kiely ME, Etienne P, Gauthier S, Stratford J, Ford RM, Dastoor D, Nair NP
Neurobiol Aging 1984 Winter;5(4):269-74. PMID: 6531064
Markowitsch HJ, Kessler J, Bast-Kessler C, Riess R
Int J Neurosci 1984 Dec;25(1-2):145-59. PMID: 6526588

Diagnosis

Kent PL
Appl Neuropsychol Adult 2017 May-Jun;24(3):232-251. Epub 2016 Apr 18 doi: 10.1080/23279095.2015.1135798. PMID: 27088230
Chawla J, Kvarnberg D
Handb Clin Neurol 2014;120:891-914. doi: 10.1016/B978-0-7020-4087-0.00059-0. PMID: 24365359
Jauhar P, Montaldi D
Alcohol Alcohol Suppl 2000 May-Jun;35(1):21-3. PMID: 11304072
Yarde WL, Kepes JJ, O'Boynick P
Kans Med 1995 Spring;96(1):22-3, 34. PMID: 7666636
Tarter RE
Dis Nerv Syst 1975 Apr;36(4):185-7. PMID: 1116426

Therapy

Kumar S
Neurol India 2004 Sep;52(3):307-9. PMID: 15472417
Jauhar P, Montaldi D
Alcohol Alcohol Suppl 2000 May-Jun;35(1):21-3. PMID: 11304072
Leigh D, McBurney A, McIlwain H
Br J Psychiatry 1981 Aug;139:153-6. PMID: 7306754
Tarter RE
Dis Nerv Syst 1975 Apr;36(4):185-7. PMID: 1116426

Prognosis

Leigh D, McBurney A, McIlwain H
Br J Psychiatry 1981 Aug;139:153-6. PMID: 7306754

Clinical prediction guides

Wirz-Justice A, Schröder CM, Gasio PF, Cajochen C, Savaskan E
Am J Geriatr Psychiatry 2010 Jan;18(1):33-41. doi: 10.1097/JGP.0b013e3181b0467a. PMID: 19910886

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