WCHD
Wernicke–Korsakoff syndrome (WKS), also known as Wernicke's encephalopathy and Korsakoff's psychosis, is a manifestation of thiamine (vitamin B1) deficiency, typically associated with prolonged alcoholism. The syndrome is a combination of two separate conditions, which can occur independently or together: Wernicke's encephalopathy and Korsakoff's psychosis. This complex is a serious and potentially life-threatening condition but is preventable and treatable if diagnosed early.
Etiology
WKS is primarily caused by a deficiency in thiamine (vitamin B1), which is crucial for brain metabolism. The deficiency can result from malnutrition, the consumption of large amounts of alcohol (which interferes with thiamine absorption and storage), or both. Other risk factors include chronic illnesses, cancer, HIV/AIDS, and prolonged vomiting or eating disorders.
Pathophysiology
Thiamine is essential for glucose metabolism in the brain. Its deficiency leads to a decrease in the production of adenosine triphosphate (ATP), causing a disruption in brain function. This disruption results in the characteristic lesions found in the mammillary bodies, thalamus, and other areas of the brain associated with WKS. These lesions can cause the symptoms associated with Wernicke's encephalopathy and Korsakoff's psychosis.
Clinical Features
Wernicke's encephalopathy is characterized by the classic triad of symptoms: confusion, ataxia (lack of muscle coordination), and ophthalmoplegia (eye movement abnormalities). However, not all patients will present with this triad. Korsakoff's psychosis is primarily marked by severe memory loss, confabulation (fabricating detailed, believable stories about experiences or situations that have not occurred), and hallucinations.
Diagnosis
Diagnosis of WKS is primarily clinical, based on history and physical examination. Laboratory tests to measure thiamine levels can be supportive but are not always definitive. Brain imaging, such as MRI or CT scan, can show characteristic changes but is not required for diagnosis.
Treatment
The primary treatment for WKS is the administration of high doses of thiamine, preferably before glucose is given to prevent worsening of symptoms. Treatment also involves addressing the underlying cause, such as alcohol cessation and nutritional support. Early treatment can lead to significant improvement, but some symptoms, particularly those related to Korsakoff's psychosis, may be irreversible.
Prevention
Prevention of WKS involves adequate nutritional intake, including supplementation of thiamine in individuals at risk, such as heavy alcohol users, patients with chronic illnesses, or those with malnutrition.
Prognosis
The prognosis of WKS varies. With prompt treatment, individuals with Wernicke's encephalopathy can experience rapid improvement. However, Korsakoff's psychosis has a more guarded prognosis, with many patients experiencing persistent memory problems despite treatment.
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Contributors: Prab R. Tumpati, MD