Nutritional Diseases

Prevalence: 1% to 3%

Age: onset ranges from 30 to 70 years

Thiamine

• Dietary sources: pork and whole grains

• Absorbed from duodenum, converted to thiamine pyrophosphate (TPP)

• TPP: important cofactor in breakdown of sugars and amino acids

• In central nervous system: affects myelin sheath and glucose metabolism, neurotransmitter synthesis, axonal transport

• Body storage capacity ~ 3 weeks

Diseases/conditions interfering with thiamine intake/metabolism

• Chronic alcoholism (most commonly)

• Liver disease

• Malnourishment or malabsorption states

• Gastrointestinal disease/obstruction

• Bariatric surgery

• Chronic hyperemesis

• Systemic illness

• Hyperalimentation

• Dialysis (long term)

Wernicke encephalopathy: ophthalmoplegia, discoordination, confusion

Korsakoff syndrome: anterograde amnesia, confabulation

• Symptoms relate to lesion of medial dorsal thalamus

Correct underlying illness (e.g., alcohol abstinence, medications, dietary supplementation)

Acute Wernicke encephalopathy: reversible with thiamine administration

Chronic Wernicke encephalopathy: partial to complete reversibility of symptoms possible

Korsakoff syndrome: irreversible (80% of cases)

Severe disease: 10% to 15% mortality