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View this article online at: patient.info/doctor/beriberi
Beriberi
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, heatlabile vitamin that acts as a coenzyme on a number of metabolic pathways. The recommended daily intake is 1.2
mg. [1] 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'. [2]
Epidemiology
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. [3]
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. [6]
Risk factors
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. [11] A loop diuretic can also increase the risk. [12]
Presentation
History
Early features
Fatigue and apathy.
Irritability.
Drowsiness, depression, poor concentration.
Anorexia, nausea, vomiting, abdominal pain.
Later features
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).
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Examination
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.
Diagnosis
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.
Investigations
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. [13] Pre-loading and post-loading whole blood or erythrocyte
transketolase activity is also sometimes assessed. [14]
Other tests include blood thiamine level, pyruvate, lactate, alphaketoglutarate and glycosylate. [15]
Thiamine concentration in whole blood can now be measured using high-performance liquid
chromatography. [16]
In Wernicke's encephalopathy, the CSF is normal or protein is slightly elevated but, if left untreated,
blood pyruvate and blood transketolase will rise. [17]
MRI scan and, to a lesser extent CT, have been used to detect brain changes in Wernicke's
encephalopathy. [18]
The electroencephalogram (EEG) may show diffuse slowing. [19]
Echocardiogram should be used to assess heart failure.
Management
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: [20]
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. [21]
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. [21]
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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. [22] However,
some patients can go into standard cardiac failure and require input from a cardiologist. [23]
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 .
Complications
The heart failure of beriberi, if untreated, can be fatal. [24]
Anaphalaxis can result from parenteral treatment. [20]
Thiamine deficiency may occur with deficiencies of other B group vitamins which may need treatment
in their own right. [25]
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.
Prognosis
Mortality is rare and is usually associated with the wet form due to cardiac failure. [26] 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. [27]
Prevention [28]
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.
History
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. [29] 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. [30] Beriberi was not uncommon on long naval voyages and was thought to
have much in common with scurvy.
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In 1753 Royal Navy Surgeon James Lind demonstrated that the consumption of fresh lemons and oranges cured
scurvy. [31] 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. [32] 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 & 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. 2014;2014:506072. 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 Oct;16(4):687-9. doi: 10.4103/0972-2327.120467.
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26.
Osiezagha K, Ali S, Freeman C, et al; Thiamine deficiency and delirium. Innov Clin Neurosci. 2013 Apr;10(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 Jul;54(3):295-314.
Watson JT, El Bushra H, Lebo EJ, et al; Outbreak of beriberi among African Union troops in Mogadishu, Somalia. PLoS
One. 2011;6(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 Dec;61(5):519-25.
Mimouni-Bloch A, Goldberg-Stern H, Strausberg R, et al; Thiamine deficiency in infancy: long-term follow-up. Pediatr
Neurol. 2014 Sep;51(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 Jul;22(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 Mar;18(3):164-6. doi: 10.4103/0972-5229.128706.
Verma V, Donadee C, Gomez L, et al; Nonalcoholic Wernicke's EncephalopathyAssociated with Unintentional Weight
Loss, Cholecystectomy, and Intractable Vomiting: The Role of Dual Thiamine and Corticosteroid Therapy. Case Rep
Neurol Med. 2014;2014:430729. 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. 2012;11(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 3;2014. 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. 2014;2014:878915. 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. 2013;51(3):222-6.
Lu J, Frank EL; Rapid HPLC measurement of thiamine and its phosphate esters in whole blood. Clin Chem. 2008
May;54(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 23;7:1-6. 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 Feb;115(2):e233-8.
Cerase A, Rubenni E, Rufa A, et al ; CT and MRI of Wernicke's encephalopathy. Radiol Med. 2011 Mar;116(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 15;73(11):828-33. doi: 10.1212/WNL.0b013e3181b121f5. Epub 2009 Jul 1.
Drug Safety Update; Medicines and Healthcare products RegulatoryAgency (MHRA) 2007;1(2):8.
British National Formulary
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
Winter;13(4):495-9.
Bello S, Neri M, Riezzo I, et al; Cardiac beriberi: morphological findings in two fatal cases. Diagn Pathol. 2011 Jan 19;6:8.
doi: 10.1186/1746-1596-6-8.
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 OctNov;23(5):487-93. doi: 10.1177/0884533608323430.
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27. Thomson AD, Guerrini I, Marshall EJ; The evolution and treatment of Korsakoff's syndrome: out of sight, out of mind?
Neuropsychol Rev. 2012 Jun;22(2):81-92. doi: 10.1007/s11065-012-9196-z. Epub 2012 May 9.
28. Doung-ngern P, Kesornsukhon S, Kanlayanaphotporn J, et al; Beriberi outbreak among commercial fishermen, Thailand
2005. Southeast Asian J Trop Med Public Health. 2007 Jan;38(1):130-5.
29. Christiaan Eijkman Biographical; Nobelprize.org
30. Wolf G, Carpenter K; Early Research into the Vitamins: The Work of Wilhelm Stepp, History of Nutrition, The Journal of
Nutrition
31. Of the Prevention of the Scurvy; James Lind, ATreatise of the Scurvy, 1753
32. Lonsdale D; Areview of the biochemistry, metabolism and clinical benefits of thiamin(e) and its derivatives. Evid Based
Complement Alternat Med. 2006 Mar;3(1):49-59.
Disclaimer: This article is for information only and should not be used for the diagnosis or treatment of medical
conditions. EMIS has used all reasonable care in compiling the information but makes no warranty as to its
accuracy. Consult a doctor or other healthcare professional for diagnosis and treatment of medical conditions.
For details see our conditions.
Original Author:
Dr Laurence Knott
Current Version:
Dr Laurence Knott
Peer Reviewer:
Dr Helen Huins
Document ID:
1859 (v23)
Last Checked:
15/10/2014
Next Review:
14/10/2019
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