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Wernicke-Korsakoff Syndrome

Updated : September 5, 2023





Background

Wernicke Korsakoff Syndrome or Wernicke Korsakoff psychosis is a psychiatric condition caused by Thiamine (Vitamin B1) deficiency. It is commonly observed in chronic alcoholics. However, non-alcoholic causes are often observed. Wernicke encephalopathy and Korsakoff syndrome are two distinct syndromes together as Wernicke-Korsakoff syndrome.

Clinical features of Wernicke encephalopathy include an acute state of confusion, usually reversible. While Wernicke encephalopathy, which leads to Korsakoff syndrome if not treated effectively, is characterized by gait abnormalities, delusional thinking, and memory loss, which are commonly irreversible.

Epidemiology

The incidence of Wernicke Korsakoff Syndrome ranges between 0-2%, as most cases go undiagnosed.

High prevalence rates are found in several subpopulations, such as homeless individuals, older people who live without their families or are isolated, and patients in psychiatric facilities.

It’s interesting to note that prevalence and the quantity of alcohol consumption are unrelated. In severe cases, the mortality rate is 10-15%.

Anatomy

Pathophysiology

Brain atrophy occurs predominantly in the mamillary bodies. It might occur in the region tectal plate, periaqueductal grey, dorsomedial thalami, and third ventricle walls. In contrast to impairment to these areas, damage to cortex may also occur; however, this damage may also result from the toxic effect of alcohol and not primarily because of the thiamine deficiency.

The impairment of memory results from damage caused to the mammillo-thalamic tract, which describes individuals who develop Wernicke Korsakoff Syndrome have damage to either the mamillary bodies or the thalamus.

The amnesia usually occurs from atrophy to the structures of the diencephalon (hypothalamus, thalamus, mammillary bodies); damage in the medial temporal lobe also produces similar amnesic conditions. Ocular-motor lesions result from damage to abducens nuclei and the eye movement centers in the midbrain-pons. Ataxia results from damage to superior vermis.

Etiology

Wernicke Korsakoff Syndrome is caused by thiamine deficiency (Vitamin B1). Individuals with poor nutrition are generally at risk. Chronic alcohol abuse is the most prevalent social factor associated with Wernicke Korsakoff syndrome, which results in decreased thiamine absorption and consumption.

Non-alcoholic cases of thiamine deficiency have also been reported in individuals with low socio-economic status, malnutrition, starvation, schizophrenia, anorexia nervosa, prisoners of war, and terminal malignancies. This syndrome can also develop in the first trimester of pregnancy in women who develop hyperemesis gravidum.

The other common causes are malabsorption of thiamine in conditions such as bariatric surgery, malignancies, and disorders of the gastrointestinal tract, systemic diseases such as Tuberculosis, AIDS, Uremia.

Genetics

Prognostic Factors

It is estimated that about 25% of the patients require prolonged hospitalization and have at least one comorbidity (psychiatric and somatic). Recovery after treatment requires time and depends on alcohol withdrawal.

The amnesic state is reversible in a few patients. Improvement of the ocular impairments usually resolves after administration of thiamine; if ocular impairments are not resolved, the re-evaluation of Wernicke Korsakoff Syndrome is required.

Mortality is due to hepatic failure and other infectious diseases; some deaths occur due to persistent thiamine deficiency.

Clinical History

Physical Examination

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

 

thiamine 

100 mg intravenously; later 50-100 mg/day intramuscularly or intravenously until a regular balanced diet is consumed



 
 

Media Gallary

References

https://www.ncbi.nlm.nih.gov/books/NBK430729/

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Wernicke-Korsakoff Syndrome

Updated : September 5, 2023




Wernicke Korsakoff Syndrome or Wernicke Korsakoff psychosis is a psychiatric condition caused by Thiamine (Vitamin B1) deficiency. It is commonly observed in chronic alcoholics. However, non-alcoholic causes are often observed. Wernicke encephalopathy and Korsakoff syndrome are two distinct syndromes together as Wernicke-Korsakoff syndrome.

Clinical features of Wernicke encephalopathy include an acute state of confusion, usually reversible. While Wernicke encephalopathy, which leads to Korsakoff syndrome if not treated effectively, is characterized by gait abnormalities, delusional thinking, and memory loss, which are commonly irreversible.

The incidence of Wernicke Korsakoff Syndrome ranges between 0-2%, as most cases go undiagnosed.

High prevalence rates are found in several subpopulations, such as homeless individuals, older people who live without their families or are isolated, and patients in psychiatric facilities.

It’s interesting to note that prevalence and the quantity of alcohol consumption are unrelated. In severe cases, the mortality rate is 10-15%.

Brain atrophy occurs predominantly in the mamillary bodies. It might occur in the region tectal plate, periaqueductal grey, dorsomedial thalami, and third ventricle walls. In contrast to impairment to these areas, damage to cortex may also occur; however, this damage may also result from the toxic effect of alcohol and not primarily because of the thiamine deficiency.

The impairment of memory results from damage caused to the mammillo-thalamic tract, which describes individuals who develop Wernicke Korsakoff Syndrome have damage to either the mamillary bodies or the thalamus.

The amnesia usually occurs from atrophy to the structures of the diencephalon (hypothalamus, thalamus, mammillary bodies); damage in the medial temporal lobe also produces similar amnesic conditions. Ocular-motor lesions result from damage to abducens nuclei and the eye movement centers in the midbrain-pons. Ataxia results from damage to superior vermis.

Wernicke Korsakoff Syndrome is caused by thiamine deficiency (Vitamin B1). Individuals with poor nutrition are generally at risk. Chronic alcohol abuse is the most prevalent social factor associated with Wernicke Korsakoff syndrome, which results in decreased thiamine absorption and consumption.

Non-alcoholic cases of thiamine deficiency have also been reported in individuals with low socio-economic status, malnutrition, starvation, schizophrenia, anorexia nervosa, prisoners of war, and terminal malignancies. This syndrome can also develop in the first trimester of pregnancy in women who develop hyperemesis gravidum.

The other common causes are malabsorption of thiamine in conditions such as bariatric surgery, malignancies, and disorders of the gastrointestinal tract, systemic diseases such as Tuberculosis, AIDS, Uremia.

It is estimated that about 25% of the patients require prolonged hospitalization and have at least one comorbidity (psychiatric and somatic). Recovery after treatment requires time and depends on alcohol withdrawal.

The amnesic state is reversible in a few patients. Improvement of the ocular impairments usually resolves after administration of thiamine; if ocular impairments are not resolved, the re-evaluation of Wernicke Korsakoff Syndrome is required.

Mortality is due to hepatic failure and other infectious diseases; some deaths occur due to persistent thiamine deficiency.

thiamine 

100 mg intravenously; later 50-100 mg/day intramuscularly or intravenously until a regular balanced diet is consumed



https://www.ncbi.nlm.nih.gov/books/NBK430729/

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