What is Wernicke-Korsakoff syndrome?

From the Guidelines

Wernicke-Korsakoff syndrome is a serious neurological disorder caused primarily by thiamine (vitamin B1) deficiency, most commonly seen in people with chronic alcoholism, and requires immediate administration of thiamine to prevent progression to coma or death. It consists of two related conditions: Wernicke's encephalopathy, an acute emergency characterized by confusion, abnormal eye movements, and difficulty with coordination; and Korsakoff syndrome, a chronic condition marked by severe memory problems and confabulation (making up stories) 1. The syndrome develops because thiamine is essential for brain function, particularly in areas controlling memory and movement 1.

Key Characteristics

• Wernicke's encephalopathy is an acute emergency that can progress to Korsakoff syndrome if left untreated 1
• Korsakoff syndrome is a chronic condition marked by severe memory problems and confabulation 1
• Thiamine deficiency is a major public health concern in several low- and middle-income countries 1
• Alcohol interferes with thiamine absorption and utilization while increasing the brain's thiamine requirements, creating a perfect storm for deficiency 1

Treatment

• Treatment requires immediate administration of thiamine, typically 500mg intravenously three times daily for 2-3 days, followed by 250mg daily for 3-5 days, then oral thiamine 100mg daily indefinitely 1
• Complete abstinence from alcohol is necessary for any meaningful recovery, though many patients will have permanent memory deficits despite treatment 1

Prevention

• Early suspicion and recognition of thiamine deficiency are needed to enable immediate initiation of therapy, as thiamine reserves are depleted as early as 20 days of inadequate oral intake 1
• Patients with severe alcoholic liver disease should be anticipated to have trace element and vitamin deficiency, including thiamine, and should be treated accordingly 1

From the FDA Drug Label

In the treatment of Wernicke-Korsakoff syndrome, thiamine hydrochloride has been administered IV in an initial dose of 100 mg, followed by IM doses of 50 to 100 mg daily until the patient is consuming a regular, balanced diet. Wernicke-Korsakoff syndrome is a condition that can be treated with thiamine hydrochloride.

• The treatment involves administering thiamine hydrochloride IV in an initial dose of 100 mg, followed by IM doses of 50 to 100 mg daily.
• The treatment should continue until the patient is consuming a regular, balanced diet 2.

From the Research

Definition and Causes of Wernicke-Korsakoff Syndrome

• Wernicke-Korsakoff syndrome (WKS) is a brain disorder caused by thiamine deficiency, with more than 90% of cases reported in alcohol-dependent patients 3.
• The syndrome is characterized by two phases: Wernicke's encephalopathy (WE), the acute phase, and Korsakoff's psychosis, the chronic phase 4, 5.
• Thiamine deficiency is the primary cause of WKS, and it can be treated effectively or prevented completely with proper diagnosis and treatment 5, 6.

Symptoms and Diagnosis

• Wernicke's encephalopathy is often difficult to detect, but early diagnosed cases are quite easy to cure 4.
• The symptoms of WKS include cognitive impairment, ataxia, oculomotor abnormalities, and confusion 3, 7.
• The diagnosis of WKS is often missed by clinicians, likely because patients do not present with the classic signs associated with the condition 7.

Treatment and Prevention

• The basic approach to the treatment of WKS is long-term parenteral administration of thiamine, often in high doses 4.
• The optimal thiamine dose required for the prevention and treatment of WKS is still unknown, and more research is needed to determine the most effective treatment regimen 3.
• Early treatment with thiamine can prevent the development of Korsakoff's psychosis and improve cognitive function 6, 7.
• Physicians should have a high index of suspicion for WKS and dose thiamine accordingly to prevent this potentially devastating disease 7.