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Synonyms: thiamine deficiency, aneurin deficiency, vitamin B1 deficiency
Beriberi is caused by deficiency of thiamine, also called aneurin and vitamin B1. This is a water-soluble, heat-labile vitamin that acts as a coenzyme on a number of metabolic pathways. The recommended daily intake is 1.2 mg.The cardiovascular, muscular, gastrointestinal and nervous systems can be affected.
The word 'beri' means weak in Sinhala, a language from Sri Lanka. Beriberi means very weak. Another translation is 'I cannot, I cannot'.
Beriberi was endemic in some areas of the world and may be related to the consumption of milled rice. The process removes the outer crust that contains the vitamin and, in Indonesia especially, the condition was very common. The disease tends to affect adults and infants between 1 and 4 months old. Improved nutrition, health propaganda and heightened public awareness all contributed to a reduction in prevalence since the 1980s.
Outbreaks occasionally occur in circumstances in which there are dietary restrictions and/or intake of thiamine antagonists.[4, 5] An outbreak in Israel in 2003 was traced to the use of soy-based milk formula.
In western societies, predisposing factors to thiamine deficiency include gastrointestinal disease and alcoholism, as the latter impairs absorption of the vitamin. Deficiency has been recorded in AIDS, after bariatric surgery and in hyperemesis gravidarum.[7, 8]It can occur (rarely) after any type of protracted vomiting state. Cases have been reported in which severe vomiting was associated with post-cholecystectomy and suspected Crohn's disease.[9, 10]Hypermetabolic states also predispose to deficiency and this includes fever, pregnancy, postoperative state, total parenteral nutrition and renal dialysis.A loop diuretic can also increase the risk.
- Fatigue and apathy.
- Drowsiness, depression, poor concentration.
- Anorexia, nausea, vomiting, abdominal pain.
- Paraesthesia, peripheral neuropathy, depressed tendon reflexes, loss of vibration sense.
- Tender leg muscles and muscle cramps.
- Congestive heart failure with dyspnoea, orthopnoea and oedema.
In infancy, beriberi can present as acute fulminating, acute cardiac, aphonic and pseudomeningitic. In the aphonic form there is hoarseness and a weak cry due to paralysis of the laryngeal nerve. In the pseudomeningitic variety there there are symptoms of nystagmus, vomiting and convulsions but normal cerebrospinal fluid (CSF).
Beriberi is often classified as 'dry beriberi' when features are neurological and muscular and 'wet beriberi' when there is predominantly heart failure. Physical findings include:
- Pallor and waxy skin.
- Signs of malnutrition and wasting.
- Listlessness, weakness.
- Tachycardia, hepatomegaly, cardiomegaly and peripheral oedema.
- Paraesthesia and peripheral neuropathy, including depressed tendon reflexes.
Two conditions caused by thiamine deficiency that are usually related to alcohol abuse in western societies are Wernicke's encephalopathy and Korsakoff's syndrome. See separate article Wernicke-Korsakoff Syndrome.
A high index of suspicion should be maintained for patients with chronic alcohol abuse, special diets and malnutrition.
Congestive heart failure may need to be excluded as may other vitamin deficiencies (vitamin B12, niacin) if neurological symptoms are present.
- Probably the best diagnostic test is a good clinical response to administration of intravenous thiamine.
- Another test (performed occasionally as it is expensive) is a thiamine loading test. This involves administering a test dose of thiamine. In deficient patients the urine excretion of thiamine and thiamine pyrophosphate is lower than normal.Pre-loading and post-loading whole blood or erythrocyte transketolase activity is also sometimes assessed.
- Other tests include blood thiamine level, pyruvate, lactate, alphaketoglutarate and glycosylate.
- Thiamine concentration in whole blood can now be measured using high-performance liquid chromatography.
- In Wernicke's encephalopathy, the CSF is normal or protein is slightly elevated but, if left untreated, blood pyruvate and blood transketolase will rise.
- MRI scan and, to a lesser extent CT, have been used to detect brain changes in Wernicke's encephalopathy.
- The electroencephalogram (EEG) may show diffuse slowing.
- Echocardiogram should be used to assess heart failure.
There is often a deficiency of more than one vitamin and so the patient must be carefully monitored as treatment starts.
Thiamine is available in both oral and parenteral form and the latter may be employed at first, with transfer to oral preparations later. However, the Medicines and Healthcare products Regulatory Agency (MHRA) advises that potentially serious allergic adverse reactions may occur during, or shortly after, parenteral administration. They recommend that:
- Use be restricted to patients in whom parenteral treatment is essential.
- Intravenous injections should be administered slowly over 10 minutes.
- Facilities for treating anaphylaxis should be available.
25-100 mg daily by mouth are recommended for chronic mild deficiency and 200-300 mg daily for more severe deficiency.
Vitamin B deficiency, other than vitamin B12 deficiency, is rare in the UK and should be treated with preparations containing thiamine (B1), riboflavin (B2) and nicotinamide. Parenteral administration followed by oral treatment are recommended for Wernicke's encephalopathy and Korsakoff's syndrome.
There is usually no need for the usual diuretics and angiotensin-converting enzyme (ACE) inhibitors for heart failure as there is often improvement in 6 to 24 hours and marked improvement in a few days. However, some patients can go into standard cardiac failure and require input from a cardiologist.
If alcoholism is the underlying problem, a 3-stage approach is required:
- If alcohol is suddenly stopped, a withdrawal syndrome of delirium tremens may result. A reducing dose of benzodiazepine, usually chlordiazepoxide, should therefore be started during the withdrawal process, the starting dose being dependent upon the level of alcohol consumption.
- The next stage is to get the patient to admit that there is a problem of alcohol abuse.
- The final stage is to get the patient to enter into long-term management of the problem. For more information, see separate article Alcoholism and Alcohol Abuse - Management.
- The heart failure of beriberi, if untreated, can be fatal.
- Anaphalaxis can result from parenteral treatment.
- Thiamine deficiency may occur with deficiencies of other B group vitamins which may need treatment in their own right.
- Korsakoff's syndrome is considered more fully elsewhere. The essential features are anterograde and retrograde amnesia, often with disorientation and confabulation. The last usually makes the patient delightful but any check of the authenticity of stories will reveal them as confabulation.
- Wernicke's encephalopathy is also covered elsewhere. Patients are often apathetic and confused. There is ataxia and nystagmus and even coma may result.
Mortality is rare and is usually associated with the wet form due to cardiac failure.Morbidity is also rare and usually presents in the dry form with neurological symptoms. In both situations, recognition of the condition and early replacement with thiamine rapidly reverse organ dysfunction. Korsakoff's syndrome may, however, only be reversible to a small degree.
Adults or the families of children need education about the cause of the disease and how to prevent recurrence. In third world countries the child may be discharged back to the same environment.
Good dietary sources of thiamine are whole grains, lean pork and legumes. Thiamine is not present in fats, oils and refined sugars and is destroyed by heat, pasteurisation and ionising radiation. Freezing does not affect thiamin content of foods.
In developed countries the problem is usually one of the very difficult management of alcohol abuse. If there are other reasons, such as after bariatric surgery, supplements can be continued.
The disease was described by the Chinese around 2700 BC but the cause was unknown. In the 19th century it was realised that the diet needed protein, fat and most of the calories from carbohydrate but it was not until the late 19th and early 20th centuries that the need for other nutrients was appreciated. Christiaan Eijkman studied prisoners in the Dutch East Indies who were fed a diet of polished rice and he noted a high incidence of neurological disorders. He also found that either feeding prisoners the whole rice or adding the husk would reverse the disorder. In 1911 Funk confirmed such findings in birds and postulated substances called vital amines, later abbreviated to vitamins. Beriberi was not uncommon on long naval voyages and was thought to have much in common with scurvy.
In 1753 Royal Navy Surgeon James Lind demonstrated that the consumption of fresh lemons and oranges cured scurvy. He divided twelve sailors into four groups of three to try three treatments and a control group in what was almost the first randomised controlled trial on record. There was a similar naval controlled trial in the early 1880s. A young Japanese medical officer, Kanehiro Takaki, arranged that two ships left Japan on similar voyages but with different diets. The first ship served the usual fare of rice, with some vegetables and fish. The second also served the crew wheat and milk, in addition to more meat than was served on the first ship. The results were impressive with 25 deaths from beriberi on the first ship and none on the other. The Japanese Admiralty adopted the new diet for the entire navy.
In 1926 two biochemists, called Jansen and Donath, isolated a tiny amount of a substance they called aneurin but there was too little to be of much value. Commercial production did not start until 1937 but it achieved great importance in the 1950s with the demand for the fortification of food.
Further reading and references
Kuno T, Nakamura H, Endo Y, et al; Clinical history and colliquative myocytolysis are keys to the diagnosis of shoshin beriberi. Case Rep Pathol. 20142014:506072. doi: 10.1155/2014/506072. Epub 2014 May 7.
Prakasha SR, Mustafa AS, Baikunje S, et al; "Dry" and "wet" beriberi mimicking critical illness polyneuropathy. Ann Indian Acad Neurol. 2013 Oct16(4):687-9. doi: 10.4103/0972-2327.120467.
Osiezagha K, Ali S, Freeman C, et al; Thiamine deficiency and delirium. Innov Clin Neurosci. 2013 Apr10(4):26-32.
Neurological Disorders: Public Health Challenges; World Health Organization, 2006
Arnold D; British India and the "beriberi problem", 1798-1942. Med Hist. 2010 Jul54(3):295-314.
Watson JT, El Bushra H, Lebo EJ, et al; Outbreak of beriberi among African Union troops in Mogadishu, Somalia. PLoS One. 20116(12):e28345. doi: 10.1371/journal.pone.0028345. Epub 2011 Dec 21.
Fozi K, Azmi H, Kamariah H, et al; Prevalence of thiamine deficiency at a drug rehabilitation centre in Malaysia. Med J Malaysia. 2006 Dec61(5):519-25.
Mimouni-Bloch A, Goldberg-Stern H, Strausberg R, et al; Thiamine deficiency in infancy: long-term follow-up. Pediatr Neurol. 2014 Sep51(3):311-6. doi: 10.1016/j.pediatrneurol.2014.05.010. Epub 2014 May 15.
Milone M, Di Minno MN, Lupoli R, et al; Wernicke encephalopathy in subjects undergoing restrictive weight loss surgery: a systematic review of literature data. Eur Eat Disord Rev. 2014 Jul22(4):223-9. doi: 10.1002/erv.2292. Epub 2014 Apr 25.
Kantor S, Prakash S, Chandwani J, et al; Wernicke's encephalopathy following hyperemesis gravidarum. Indian J Crit Care Med. 2014 Mar18(3):164-6. doi: 10.4103/0972-5229.128706.
Verma V, Donadee C, Gomez L, et al; Nonalcoholic Wernicke's Encephalopathy Associated with Unintentional Weight Loss, Cholecystectomy, and Intractable Vomiting: The Role of Dual Thiamine and Corticosteroid Therapy. Case Rep Neurol Med. 20142014:430729. doi: 10.1155/2014/430729. Epub 2014 Jan 19.
Hesami O, Beladimoghaddam N, Assarzadegan F, et al; Wernicke's encephalopathy in a non-alcoholic Patient: Difficulties of early diagnosis and treatment. Iran J Neurol. 201211(4):159-61.
Ramsi M, Mowbray C, Hartman G, et al; Severe lactic acidosis and multiorgan failure due to thiamine deficiency during total parenteral nutrition. BMJ Case Rep. 2014 Jun 32014. pii: bcr2014205264. doi: 10.1136/bcr-2014-205264.
Misumida N, Umeda H, Iwase M; Shoshin beriberi induced by long-term administration of diuretics: a case report. Case Rep Cardiol. 20142014:878915. doi: 10.1155/2014/878915. Epub 2014 Jul 3.
Chatterjea M et al; Textbook of Medical Biochemistry, 2012
Michalak S, Michalowska-Wender G, Adamcewicz G, et al; Erythrocyte transketolase activity in patients with diabetic and alcoholic neuropathies. Folia Neuropathol. 201351(3):222-6.
Lu J, Frank EL; Rapid HPLC measurement of thiamine and its phosphate esters in whole blood. Clin Chem. 2008 May54(5):901-6. doi: 10.1373/clinchem.2007.099077. Epub 2008 Mar 20.
Al-Attas O, Al-Daghri N, Alokail M, et al; Metabolic Benefits of Six-month Thiamine Supplementation in Patients With and Without Diabetes Mellitus Type 2. Clin Med Insights Endocrinol Diabetes. 2014 Jan 237:1-6. doi: 10.4137/CMED.S13573. eCollection 2014.
Fattal-Valevski A, Kesler A, Sela BA, et al; Outbreak of life-threatening thiamine deficiency in infants in Israel caused by a defective soy-based formula. Pediatrics. 2005 Feb115(2):e233-8.
Cerase A, Rubenni E, Rufa A, et al; CT and MRI of Wernicke's encephalopathy. Radiol Med. 2011 Mar116(2):319-33. doi: 10.1007/s11547-011-0618-x. Epub 2011 Jan 12.
Fattal-Valevski A, Bloch-Mimouni A, Kivity S, et al; Epilepsy in children with infantile thiamine deficiency. Neurology. 2009 Sep 1573(11):828-33. doi: 10.1212/WNL.0b013e3181b121f5. Epub 2009 Jul 1.
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Shenoy V, Patil PV, Nagar V et al; Congestive cardiac failure and anemia in a 15-year-old boy Journal of Postgraduate Medicine, Vol. 51, No. 3, July-September, 2005, pp. 225-227
Dinicolantonio JJ, Lavie CJ, Niazi AK, et al; Effects of thiamine on cardiac function in patients with systolic heart failure: systematic review and metaanalysis of randomized, double-blind, placebo-controlled trials. Ochsner J. 2013 Winter13(4):495-9.
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Blass D; Neurochemical Mechanisms in Disease, 2010
Wooley JA; Characteristics of thiamin and its relevance to the management of heart failure. Nutr Clin Pract. 2008 Oct-Nov23(5):487-93. doi: 10.1177/0884533608323430.
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Doung-ngern P, Kesornsukhon S, Kanlayanaphotporn J, et al; Beriberi outbreak among commercial fishermen, Thailand 2005. Southeast Asian J Trop Med Public Health. 2007 Jan38(1):130-5.
Christiaan Eijkman Biographical; Nobelprize.org
Wolf G, Carpenter K; Early Research into the Vitamins: The Work of Wilhelm Stepp, History of Nutrition, The Journal of Nutrition
Of the Prevention of the Scurvy; James Lind, A Treatise of the Scurvy, 1753
Lonsdale D; A review of the biochemistry, metabolism and clinical benefits of thiamin(e) and its derivatives. Evid Based Complement Alternat Med. 2006 Mar3(1):49-59.
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