Foundations · Year 1 · from Foundations
Case 1: Wernicke-Korsakoff Syndrome (Thiamine Deficiency)
Clinical Image
Source: Radiopaedia - Wernicke encephalopathy - CC BY-NC-SA 3.0
Case Presentation
A 52-year-old man with a history of alcohol use disorder is brought to the emergency department by police after being found confused and unsteady on the street. He has a disheveled appearance and poor nutritional status. On examination, he is disoriented to time and place, has horizontal nystagmus with bilateral lateral gaze palsy (ophthalmoplegia), and a wide-based ataxic gait. Vital signs show temperature 37.8C and heart rate 110. Laboratory studies reveal mildly elevated lactate at 3.2 mmol/L and macrocytic anemia (MCV 108 fL). Recognizing the classic triad of Wernicke encephalopathy (confusion, ophthalmoplegia, ataxia), the physician immediately administers IV thiamine (vitamin B1) before any glucose-containing fluids, as glucose administration without thiamine can precipitate or worsen Wernicke encephalopathy. MRI brain shows symmetric T2 hyperintensities in the medial thalami and periaqueductal gray, characteristic of Wernicke encephalopathy. With thiamine repletion, his eye findings resolve within days, but he develops Korsakoff syndrome with profound anterograde amnesia and confabulation.
Key Learning Points
- Thiamine pyrophosphate (TPP) is an essential cofactor for pyruvate dehydrogenase and alpha-ketoglutarate dehydrogenase, both critical enzymes linking glycolysis to the TCA cycle
- Without thiamine, pyruvate cannot enter the TCA cycle and is instead shunted to lactate; the brain is particularly vulnerable because it cannot use fatty acids and depends on glucose oxidation
- The classic triad of Wernicke encephalopathy is confusion, ophthalmoplegia, and ataxia; Korsakoff syndrome (irreversible memory impairment) develops if treatment is delayed