History. It was almost a footnote the way Carl Wernicke described the first cases that
would later be recognized as Wernicke’s encephalopathy. Characterized by a triad of acute confusion, ophthalmoparesis, and ataxia, this clinical syndrome is often the consequence of acute depletion of thiamine stores. In his original report, Wernicke described 3 patients with these symptoms in an educational textbook. About the same time, a similar report was published by a Russian physician named Sergei Korsakoff, but it would be two decades before the similarities between these reports were identified by scholars.
Not everyone exhibits the full triad of symptoms. As we review in this week’s BrainWaves podcast, a rule of thumb to remember is a rule of thirds: one-third of patients may only have one-third of the triad of symptoms and one-third have all three manifestations. But nearly 20% of all patients with laboratory of pathologic evidence of Wernicke’s may meet zero criteria. So you really have to be looking for it if you expect to make the diagnosis. Another accepted definition of Wernicke’s encephalopathy in the literature is the addition of nutritional deficiency to the proposed triad.
Thiamine, or vitamin B1, is a water-soluble molecule that serves as a cofactor in a variety of metabolic pathways. Because deficiency of thiamine is so rare in western cultures, you’d think that it would be difficult to deplete your body’s stores. According to the US Food and Drug Administration, the human body only requires 1.2mg of thiamine per day in order to prevent deficiency. HOWEVER, the human body is unable to physically synthesize this molecule, so all thiamine must be acquired in the diet. Second, we are only capable of storing 2-3 weeks of this invaluable substance. Thirdly, even in “thiamine-rich” foods, the quantity of thiamine is still pretty low. Fortunately, most foods are fortified with vitamins, including B1, so that complications of deficiency may be prevented.
Depletion of thiamine stores can occur in a variety of conditions, as we discuss in our BrainWaves episode. Acute depletion results in Wernicke’s syndrome with significant neurologic manifestations. More subacute or chronic insufficiency may result in dilated cardiomyopathy (“wet beriberi”) and other forms of congestive heart failure, anemia, peripheral neuropathy, cognitive impairment, and a number of other conditions. So keep those stores replete and check out the latest BrainWaves podcast!
The content in this episode was vetted and approved by Michael Rubenstein.
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