Beriberi

Background

• Dry Beriberi: neuro symptoms caused by thiamine deficiency
• Wet Beriberi: cardiac symptoms caused by thiamine deficiency
• Infantile Beriberi: neuro/cardiac symptoms caused by thiamine deficiency in <1 year old infant

Causes

• Anything that causes thiamine (vitamin B1) deficiency: poor dietary intake, malabsorption, increased metabolic requirement
  • Chronic alcoholism, dieting/fasting/starvation, anorexia, vomiting/diarrhea, unbalanced TPN, GI surgery, malignancy, dialysis, AIDS, IBD, pancreatitis, liver disease, thyrotoxicosis

Pathophysiology

• Thiamine is a cofactor for enzymes required in:
  • Krebs cycle
  • Pentose phosphate pathway
  • Alpha-ketoglutarate dehydrogenase
  • Pyruvate dehydrogenase.
• Because thiamine is an important cofactor in critical pathways for energy production, deficiency results in lactic acidosis and alteration of brain metabolism.
• Thiamine is also important for lipid metabolism and may affect myelin sheath formation. This may explain peripheral neuropathy symptoms in dry beriberi.

Thiamine deficiency types

• Wernicke-Korsakoff syndrome
• Beriberi

Clinical Features

Dry Beriberi with symmetric peripheral neuropathy.

Bariatric beriberi with bilateral wrist drop and peripheral neuropathy after bariatric surgery for morbid obesity.

Dry Beriberi

• Symmetrical peripheral neuropathy (motor and sensory) mostly distal extremities

Wet Beriberi

• CHF, high output heart failure, cardiomegaly, peripheral edema, tachycardia, DOE/PND/orthopnea
• Can include neuropathy seen in Dry Beriberi

Infantile Beriberi

• CHF, cardiomegaly, tachycardia, cyanosis, dyspnea, weight loss, marasmus, vomiting, loud cry, nystagmus, seizure

Bariatric Beriberi

• Occurs 1-3 months post-bariatric surgery
• Causes are multifactorial, including low nutritional intake, poor baseline nutrition, persistent vomiting, malabsorption

Differential Diagnosis

Vitamin deficiencies

• Vitamin A deficiency
• Vitamin B deficiencies
  • Vitamin B1 deficiency (Thiamine)
    • Beriberi
    • Wernicke-Korsakoff syndrome
  • Vitamin B3 deficiency (Pellagra)
  • Vitamin B9 deficiency (Folate)
  • Vitamin B7 deficiency (Biotin)
  • Vitamin B12 deficiency
• Vitamin C deficiency (Scurvy)
• Vitamin D deficiency (Rickets)
• Vitamin E deficiency
• Vitamin K deficiency
• Zinc deficiency
  • Acrodermatitis enteropathica

High-output heart failure

• Hyperthyroidism
• Beriberi
• High-output heart failure from AV fistula
• AVM
• Paget disease
• Anemia
• Pregnancy

Evaluation

Chest x-ray showing cardiomegaly with mild pulmonary congestion and pleural effusion in a patient with wet beriberi.

• Clinical diagnosis

Management

If you suspect Beriberi then treat it! Diagnosis is clinical and difficult to confirm, treatment is simple/inexpensive/effective, there is little risk to treatment, and the risk of morbidity/mortality from not treating is high

1. Thiamine 50-100mg IV/IM q day x 7-14 days, then 10mg PO q day until complete recovery
2. Magnesium; hypomagnesemic state may be resistant to thiamine administration
3. Multivitamin (at risk for other vitamin deficiencies)

• Give thiamine BEFORE glucose in patients requiring glucose who are at risk for thiamine deficiency; glucose without thiamine can precipitate/worsen WE by driving thiamine intracellularly

Prevention

Vitamin Prophylaxis for Chronic alcoholics

• At risk for thiamine deficiency, but no symptoms: thiamine 100mg PO q day
• Give multivitamin PO; patient at risk for other vitamin deficiencies

Banana bag

The majority of chronic alcoholics do NOT require a banana bag

• Thiamine 100mg IV
• Folate 1mg IV (cheaper PO)
• Multivitamin 1 tab IV (cheaper PO)
• Magnesium sulfate 2mg IV
• Normal saline as needed for hydration

See Also

• Thiamine
• Thiamine deficiency

References

1. Donnino, Michael, et al. “Myths and misconceptions of wernicke’s encephalopathy: what every emergency physician should know.” Annals of emergency medicine. 2007. Vol 50, no 6. Pages 715-721.
2. Sechi, GianPietro; Serra, Alessandro. “Wernicke’s encephalopathy: new clnical settings and recent advances in diagnosis and management.” Neurology. Vol 6, May 2007. Pages 442-455