From the Guidelines
Wernicke's syndrome is a neurological disorder caused by thiamine (vitamin B1) deficiency, typically seen in alcoholics but also in those with severe malnutrition or absorption issues, and requires immediate administration of thiamine to prevent progression to permanent brain damage. This condition presents with the classic triad of confusion, ataxia (unsteady gait), and eye movement abnormalities (nystagmus, ophthalmoplegia) 1. According to the most recent guidelines, patients with both alcohol- and non-alcohol-related cirrhosis are prone to deficiencies in water-soluble vitamins, particularly thiamine, and post-mortem evidence of Wernicke’s encephalopathy is often observed, even in the absence of a history/clinical signs during life 1.
Key Considerations
- The condition can be reversed if caught early, but may cause permanent brain damage if treatment is delayed 1
- Thiamine must be given before glucose in suspected cases, as glucose administration alone can worsen the condition by depleting remaining thiamine reserves
- Treatment requires immediate administration of thiamine, usually 500mg intravenously three times daily for 2-3 days, followed by 250mg daily until symptoms improve, then oral thiamine 100mg daily indefinitely
- Early recognition and treatment are critical to prevent progression to Korsakoff syndrome, causing permanent memory impairment 1
Management
- High-dose parenteral thiamine supplementation is mandatory if Wernicke’s encephalopathy is suspected 1
- A course of oral multivitamin supplementation could be justified in patients with decompensated liver disease 1
- Vitamin/micronutrient deficiencies may cause a metabolic encephalopathy which can accompany but should not be confused with hepatic encephalopathy 1
From the Research
Definition and Causes of Wernicke's Syndrome
- Wernicke's encephalopathy (WE) is an acute neurological disorder resulting from thiamine deficiency 2.
- It is mainly related to alcohol abuse but can be associated with other conditions such as gastrointestinal disorders 2.
- Thiamine deficiency can also present with cardiovascular symptoms, called "wet beriberi" 2.
- Association with folate deficit can worsen the clinical picture 2.
Symptoms and Diagnosis
- Symptoms of Wernicke's encephalopathy include gait instability, dyspnoea, chest pain, vertiginous syndrome, nystagmus, diplopia, dysmetria, and sensitive and motor deficit in all four limbs with areflexia 2.
- A cerebral magnetic resonance imaging (MRI) can show typical findings of WE 2.
- Diagnosis is eminently clinical, with the difficulty of high clinical variability, and complementary tests are used to support the diagnosis and rule out other causes that can produce similar symptoms 3.
Treatment and Prognosis
- Treatment is based on the administration of thiamine, which should be started early, and parenterally at the appropriate doses, in all patients with compatible symptoms, without waiting to confirm the diagnosis 3.
- Early vitamin treatment in symptomatic patients improves prognosis 2.
- High doses of thiamine can lead to a great improvement of neurological and neuroradiological assessment, but cognitive impairment may still be severe 4.
- Untreated, WE can lead to coma or death, or progress to Korsakoff syndrome (KS) - a dementia characterized by irreversible loss of anterograde memory 5.