Epidemiology

Prevalence

• Prevalence of Thiamine Deficiency in Patients with Stable Outpatients with Congestive Heart Failure
  • Prevalence of Thiamine Deficiency is Very Low in Stable Outpatients with Congestive Heart Failure (Clin Nutr, 2016) [MEDLINE]

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Etiology

Cases of Thiamine Deficiency in Developed Countries are Generally Associated with One of the Following

• Alcohol Abuse (see Ethanol)
• Fad Diet (Nutr Clin Pract, 2008) [MEDLINE] (Life Sci, 2014) [MEDLINE]
• Heart Failure with Prolonged Diuretic Use (see Heart Failure) (Congest Heart Fail, 2007) [MEDLINE]
• Bariatric (Weight Loss) Surgery (see Bariatric Surgery)
• Total Parenteral Nutrition (TPN) with Inadequate Thiamine Replacement (see Total Parenteral Nutrition)

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Physiology

Refeeding Syndrome May Exacerbate Thiamine Deficiency (see Refeeding Syndrome)

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Diagnosis

XXXXXX

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Clinical Manifestations

Infantile Beriberi

Epidemiology

• Occurs in Infants: usually manifests between 2-3 mo of age
• Mainly Affects Infants Who are Breastfed by Mothers with a Thiamine-Deficient Diet

Clinical Manifestations

• Cardiovascular Manifestations
  • Cardiomegaly
  • Sinus Tachycardia (see Sinus Tachycardia)
• Neurologic Manifestations
  • Agitation (see Agitation): may occur in older infants
  • Altered Mental Status (see Altered Mental Status): may occur in older infants
  • Aseptic Meningitis (see Meningitis): may occur in older infants
    • Cerebrospinal Fluid/Lumbar Puncture (LP) (see Lumbar Puncture): normal
  • Nystagmus (see Nystagmus): may occur in older infants
  • Purposeless Movements: may occur in older infants
  • Seizures (see Seizures): may occur in older infants
• Other Manifestations
  • Aphonic (Soundless) Cry: may occur in older infants
  • Cyanosis (see Cyanosis)
  • Dyspnea (see Dyspnea)
  • Loud Piercing Cry
  • Vomiting (see Nausea and Vomiting)

Dry Beriberi

Epidemiology

• Occurs in Adults
• Risk Factors
  • Bariatric Surgery (Annu Rev Nutr, 2013) [MEDLINE]
  • Total Parenteral Nutrition (TPN) Without Adequate Thiamine Supplementation (see Total Parenteral Nutrition) (Pediatrics, 1998) [MEDLINE]

Clinical Manifestations

• Neurologic Manifestations
  • Symmetrical Peripheral Neuropathy (see Peripheral Neuropathy): usually involves the distal extremities
    • Motor Deficits
    • Sensory Deficits

Wet Beriberi

Epidemiology

• Occurs in Adults
• Risk Factors
  • Bariatric Surgery (Annu Rev Nutr, 2013) [MEDLINE]
  • Total Parenteral Nutrition (TPN) Without Adequate Thiamine Supplementation (see Total Parenteral Nutrition) (Pediatrics, 1998) [MEDLINE]

Clinical Manifestations

• Cardiovascular Manifestations
  • Cardiomegaly
  • Cardiomyopathy with Congestive Heart Failure (CHF) (see Congestive Heart Failure)
  • Peripheral Edema (see Peripheral Edema)
  • Sinus Tachycardia (see Sinus Tachycardia)
• Neurologic Manifestations
  • Peripheral Neuropathy (see Peripheral Neuropathy)

Wernicke-Korsakoff Syndrome

Epidemiology

• Risk Factors
  • Bariatric Surgery (Ann Surg, 2008) [MEDLINE]
  • Total Parenteral Nutrition (TPN) Without Adequate Thiamine Supplementation (see Total Parenteral Nutrition) (Pediatrics, 1998) [MEDLINE]

Clinical Manifestations

• Wernicke’s Encephalopathy
  • General Comments
    • Acute Neurologic Emergency
    • Occurs Mainly in Alcoholics with Thiamine Deficiency
    • May Be Due to Impaired Synthesis of the Pentose Phosphate Pathway Enzyme, Erythrocyte Transketolase
  • Ataxia (see Ataxia)
  • Confusion (see Delirium)
  • Nystagmus (see Nystagmus)
    • Ophthalmoplegia (see Ophthalmoplegia)
• Korsakoff Syndrome
  • General Comments
    • Chronic Neurologic Syndrome Which Usually Occurs After Wernicke’s Encephalopathy
  • Confabulation
  • Deficits in Short-Term Memory (see Memory Loss)
  • Grossly Normal Cognition

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Treatment

Ethanol Withdrawal (see Ethanol)

• Standard Prophylaxis Regimen
  • Administer 100 mg/day IV (Usually in a “Banana Bag” Which Includes Folate and Multivitamin) x Several Days, Followed by 50-100 mg PO day
• Royal College of Physicians High-Dose Thiamine Prophylaxis Regimen for Wernicke’s Encephalopathy (Administered in Addition to Other B Vitamins, Ascorbic Acid, Potassium, Magnesium, and Phosphate) (Alcohol Alcohol, 2002) [MEDLINE]
  • Administer 250 mg IV qday x 3-5 Days

Thiamine Deficiency (Beriberi and/or Wernicke’s Encephalopathy)

• Standard Regimen
  • Administer 100 mg IV qday (Usually in a “Banana Bag” Which Includes Folate and Multivitamin) Until Patient is Tolerating a Balanced Oral Diet
  • However, Higher Thiamine Doses May Be Required in Patients Who Do Not Clinically Improve on the Standard Regimen
• Royal College of Physicians High-Dose Thiamine Regimen for the Treatment of Wernicke’s Encephalopathy (Administered in Addition to Other B Vitamins, Ascorbic Acid, Potassium, Magnesium, and Phosphate) (Alcohol Alcohol, 2002) [MEDLINE]
  • Administer 500 mg IV TID x 3 Days, Followed by 250 mg IV qday x 5 Days (or Until Clinical Improvement is Observed)

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References

General

• XXXX

Etiology

• Wernicke encephalopathy and beriberi during total parenteral nutrition attributable to multivitamin infusion shortage. Pediatrics. 1998;101(1):E10 [MEDLINE]
• The Royal College of Physicians report on alcohol: guidelines for managing Wernicke’s encephalopathy in the accident and Emergency Department. Alcohol Alcohol. 2002 Nov-Dec;37(6):513-21 [MEDLINE]
• Loop diuretic therapy, thiamine balance, and heart failure. Congest Heart Fail. 2007;13(4):244 [MEDLINE]
• Wernicke encephalopathy after bariatric surgery: a systematic review. Ann Surg. 2008;248(5):714 [MEDLINE]
• Characteristics of thiamin and its relevance to the management of heart failure. Nutr Clin Pract. 2008;23(5):487 [MEDLINE]
• Vitamin status after bariatric surgery: a randomized study of gastric bypass and duodenal switch. Am J Clin Nutr. 2009;90(1):15. Epub 2009 May 13 [MEDLINE]
• Nutrient deficiencies after gastric bypass surgery. Annu Rev Nutr. 2013;33:183-203 [MEDLINE]
• Current aspects of thiamine deficiency on heart function. Life Sci. 2014 Mar;98(1):1-5 [MEDLINE]
• Prevalence of thiamine deficiency in a stable heart failure outpatient cohort on standard loop diuretic therapy. Clin Nutr. 2016;35(6):1323 [MEDLINE]