beriberi

Definition

Beriberi is a disease caused by a deficiency of thiamine (vitamin B₁) that affects many systems of the body, including the muscles, heart, nerves, and digestive system. Beriberi literally means "I can't, I can't" in Sing-halese, which reflects the crippling effect it has on its victims. It is common in parts of southeast Asia, where white rice is the main food. In the United States, beriberi is primarily seen in people with chronic alcoholism.

Description

Beriberi puzzled medical experts for years as it ravaged people of all ages in Asia. Doctors thought it was caused by something in food. Not until the early 1900s did scientists discover that rice bran, the outer covering that was removed to create the polished white rice preferred by Asians, actually contained something that prevented the disease. Thiamine was the first vitamin identified. In the 1920s, extracts of rice polishings were used to treat the disease.

In adults, there are different forms of beriberi, classified according to the body systems most affected. Dry beriberi involves the nervous system; wet beriberi affects the heart and circulation. Both types usually occur in the same patient, with one set of symptoms predominating.

A less common form of cardiovascular, or wet beriberi, is known as "shoshin." This condition involves a rapid appearance of symptoms and acute heart failure. It is highly fatal and is known to cause sudden death in young migrant laborers in Asia whose diet consists of white rice.

Cerebral beriberi, also known as Wernicke-Korsakoff syndrome, usually occurs in chronic alcoholics and affects the central nervous system (brain and spinal cord). It can be caused by a situation that aggravates a chronic thiamine deficiency, like an alcoholic binge or severe vomiting.

Infantile beriberi is seen in breastfed infants of thiamine-deficient mothers, who live in developing nations.

Although severe beriberi is uncommon in the United States, less severe thiamine deficiencies do occur. About 25% of all alcoholics admitted to a hospital in the United States show some evidence of thiamine deficiency.

— Karen Ericson, RN

[Sinhalese, reduplication of beri, weakness.]

For more information on beriberi, visit Britannica.com.

Definition

Beriberi is a condition caused by severe prolonged deficiency of vitamin B1 (also known as thiamine). Beriberi refers to a constellation of heart, gastrointestinal, and nervous system problems from thiamine deficiency.

Description

Thiamine is found in a variety of foods, particularly whole grains, legumes, and pork. Thiamine serves as a coenzyme in the chemical pathway responsible for the metabolism of carbohydrates. Thiamine deficiency interferes with the metabolism of glucose and the production of energy.

Four major types of beriberi exist: wet beriberi, which affects primarily the cardiovascular system; dry beriberi, which affects primarily the nervous system; shoshin, which is a rapidly evolving and frequently fatal form of cardiovascular beriberi; and infantile beriberi, which tends to strike babies between the ages of one and four months who are breastfed by mothers who are severely thiamine deficient.

Demographics

Because so many foods in the United States and other western countries are vitamin enriched, beriberi is extremely rare. In developed countries, beriberi is primarily a complication of malnutrition secondary to alcoholism or gastrointestinal disorders. Because alcoholism affects more males than females, rates of beriberi in developed countries are higher among males. The syndrome of symptoms caused by thiamine deficiency in alcoholism is called Wernicke-Korsakoff syndrome.

In developing countries, where diets are more limited, beriberi is endemic. In some areas of Asia, people subsist on polished rice, in which the outer, more nutritious husk is removed. The rates of beriberi in these areas are quite high. In certain parts of Indonesia, the prevalence of beriberi among low-income families is as high as 66%. The majority of patients with beriberi are infants (ages 1–4 months) and adults.

Causes and symptoms

Symptoms of beriberi are caused by abnormal metabolism of carbohydrates throughout the body, resulting in a decreased production of energy, and particular injury to the heart muscle and the nervous system.

Symptoms of dry beriberi include:

• numbness, tingling, burning pain in extremities
• pain and cramping in the leg muscles
• difficulty with speech
• problems walking
• disturbed sense of balance

Symptoms of wet beriberi include:

• fast heart rate
• swollen feet and legs
• enlarged heart
• enlarged, tender liver
• shortness of breath
• congestion in the lungs

Symptoms of shoshin beriberi are the same as those of wet beriberi, but the onset is sudden, the progression is rapid, and the risk of death is very high.

Symptoms of infantile beriberi include:

• restlessness
• difficulty sleeping
• diarrhea
• swollen arms and legs
• muscle wasting in arms and legs
• silent cry
• heart failure

Symptoms may coexist with other disorders due to thiamine deficiency such as Wernicke-Korsakoff encephalopathy. In such cases, confusion, memory loss, difficulty with eye movements, and even coma may occur.

Diagnosis

The first step to diagnosis includes taking a careful history to uncover a possible underlying cause for thiamine deficiency. Physical examination will demonstrate some of the expected signs of beriberi, such as swelling, decreased reflexes, decreased sensation, problems with walking or balance, etc.

Laboratory testing to demonstrate thiamine deficiency includes measurements of thiamine in the blood; tests of the activity of thiamine in whole blood or red blood cells (called transketolase activity), both before and after the administration of thiamine; measurements of the chemicals lactate and pyruvate in the blood (these will be increased in beriberi); and measurements of the amount of thiamine passed into the urine (this will be decreased in beriberi).

In some cases, the diagnosis of beriberi is made only after thiamine supplementation results in a resolution of the patient's symptoms.

Treatment team

Depending on how a patient enters the health care system, an emergency room physician, internal medicine physician, family practitioner, neurologist, gastroenterologist, or cardiologist may treat a patient for beriberi. A nutritionist should be consulted to develop a nutritional plan. If alcoholism is an underlying problem, the patient may need to enter an alcohol rehabilitation program. Physical therapy may help patients recover from the neurological complications of beriberi.

Treatment

When a patient has serious symptoms of thiamine deficiency, supplementation is usually started by giving thiamine through an IV or by intramuscular shots. Because magnesium is required for the proper functioning of thiamine, magnesium is usually administered through injections as well. After several days of this therapy, a multivitamin containing 5–10 times the usually recommended daily allowance of all the water-soluble vitamins, including thiamine, should be given for several weeks. Ultimately, the patient will be advised to follow a lifelong regimen of nutritious eating, with the regular diet supplying 1–2 times the recommended daily allowance of the water-soluble vitamins, including thiamine.

Recovery and rehabilitation

Recovery from the cardiovascular effects of beriberi is nearly always complete. Some of the neurological problems, however, may remain even after thiamine supplementation has been accomplished.

Prognosis

The longer a patient lives with a thiamine deficiency, the more severe the symptoms of beriberi. If untreated, beriberi is fatal. When treated with thiamine supplementation and a healthy diet, most of the symptoms of beriberi can be resolved.

Special concerns

Although beriberi is readily avoided with a healthy diet or successfully treated with thiamine supplementation and the initiation of a healthy diet, this is not always possible in developing countries where resources are scarce.

Resources

BOOKS

Brust, John C. "Nutritional Disorders of the Nervous System." In Cecil Textbook of Medicine, edited by Thomas E. Andreoli, et al. Philadelphia: W.B. Saunders Company, 2000.

Kinsella, Laurence A., and David E. Riley. "Nutritional Deficiencies and Syndromes Associated with Alcoholism." In Textbook of Clinical Neurology, edited by Christopher G. Goetz. Philadelphia: W.B. Saunders Company, 2003.

Russell, Robert M. "Vitamin and Trace Mineral Deficiency and Excess." In Harrison's Principles of Internal Medicine, edited by Eugene Braunwald, Anthony Fauci, et al. New York: McGraw-Hill, 2001.

Rosalyn Carson-DeWitt, MD

The result of severe and prolonged deficiency of vitamin B₁, still a problem in parts of south east Asia where the diet is high in carbohydrate and poor in vitamin B₁. In developed countries vitamin B₁ deficiency is associated with alcohol abuse; while it may result in beriberi, more commonly the result is central nervous system damage, the Wernicke-Korsakoff syndrome. In beriberi there is degeneration of peripheral nerves, starting in the hands and feet and ascending the arms and legs, with a loss of sensation and deep muscle pain. There is also enlargement of the heart, which may lead to oedema (wet beriberi), and death results from heart failure. Fatal heart failure may develop without the nerve damage being apparent (Shoshin or sudden beriberi). The name is derived from the Bahasa-Malay word for sheep, to describe the curious sheep-like gait adopted by sufferers.

A deficiency disease caused by lack of thiamin (vitamin B₁). Beriberi leads to a decreased appetite; gastrointestinal disturbances; peripheral nerve changes indicated by weakness of legs, cramping of calf muscles, and numbness of feet; heart enlargement; and mental confusion. Beriberi is treated with a daily dose of 25 mg of vitamin B₁. Alcoholics often suffer from a form of beriberi called ‘alcoholic pseudonephritis’, due to poor diet.

A nutritional disease resulting from a deficiency of thiamine. Classically it is characterized by multiple neuritis, muscular atrophy, weakness, cardiovascular changes, and progressive edema.

Deficiency disease caused by lack of thiamin (vitamin B1). Beriberi leads to a decreased appetite; gastrointestinal disturbance; peripheral nerve changes indicated by weakness of legs, cramping of calf muscles, numbness of feet; heart irregularities (including tachycarditis), and mental confusion.

Beriberi is a disease that usually begins with a loss of feeling in the feet and then weakness and pain in walking. In many, but not all, cases the body then becomes swollen and in the most serious cases the heart begins to fail, and the patient becomes breathless and soon dies. The problem stems from an insufficient intake of the vitamin thiamin (or "thiamine") even though we require each day only about 1 milligram, which is equivalent to one 32,000th of an ounce. The word "beriberi" comes from Indonesia and may mean "weak" or "swelling," but there have been many other suggested meanings.

The disease used to be a serious problem in Far Eastern countries where white rice was the staple food and people ate only small quantities of supplementary foods. Husked rice grains provide a reasonable amount of this vitamin, but further processing, or "polishing" to rub off the bran and germ, removes most of the remaining thiamin. Washing the grains and boiling them leaves even less thiamin in the final cooked food. Unfortunately, brown (unpolished) rice goes rancid more quickly under tropical conditions and so has only a short storage life. In traditional peasant communities, where enough paddy (unhusked grain) would be pounded and winnowed each morning for the day ahead, this was not a problem. When inexpensive power machinery for milling and polishing rice was developed, this made the provisioning of the armed forces in particular much more convenient, but in Japan and other Asian countries it was followed by serious outbreaks of beriberi in the army and navy.

Infantile beriberi also has been a major cause of death among breast-fed infants in the Philippines and other communities where mothers are in a state of borderline, subclinical thiamin deficiency. Affected infants typically cease to pass urine and experience difficulty in breathing. Even those near death, however, respond dramatically to a dose of thiamin.

It is technically possible now to mix in with white rice a few vitamin-rich pellets manufactured to resemble rice grains. However, where rice-growing communities each have their own small village mill, it has been found impracticable to control such additions, which slightly increase the millers' costs. As an alternative, communities at risk can be supplied with inexpensive vitamin pills.

In developed countries thiamin deficiency is still a problem among alcoholics, partly because such addicts have highly abnormal diet patterns and partly because they seem to absorb the vitamin less efficiently. They also may show acute heart problems without any early symptoms of traditional beriberi. A small proportion progress to a syndrome with irreversible brain damage that requires indefinite hospitalization.

In many Western countries, millers are required to fortify white wheaten flour with thiamin (along with other micronutrients). Thus, even the population groups, such as alcoholics, who are eating an unbalanced diet are less likely to become deficient. It also has been suggested that alcoholic drinks should be fortified with thiamin. This would not be prohibitively expensive, but authorities have felt that, on balance, it would be undesirable because it would allow them to be marketed as "health drinks" despite the injurious effects associated with or caused by alcohol consumption, that is, automobile accidents, disruption of families, and a wide range of health problems.

Bibliography

Carpenter, K. J. Beriberi, White Rice, and Vitamin B. Berkeley: University of California Press, 2000.

Williams, R. R. Toward the Conquest of Beriberi. Cambridge, Mass.: Harvard University Press, 1961.

—Kenneth John Carpenter

The name given to thiamin deficiency in humans. See thiamin nutritional deficiency.

This article's citation style may be unclear. The references used may be made clearer with a different or consistent style of citation, footnoting, or external linking. (September 2009)

Beriberi
Classification and external resources
A sufferer – turn of the 20th century in southeast Asia
ICD-10	E51.1
ICD-9	265.0
DiseasesDB	14107
eMedicine	ped/229 med/221
MeSH	D001602

Beriberi (pronounced /bɛriˈbɛri/) is a nervous system ailment caused by a deficiency of thiamin (vitamin B1) in the diet. Thiamin is involved in the breakdown of energy molecules such as glucose and is also found on the membranes of neurons. Symptoms of beriberi include severe lethargy and fatigue, together with complications affecting the cardiovascular, nervous, muscular, and gastrointestinal systems.

Contents 1 Etymology 2 Causes 3 Symptoms and effects 4 Treatment 5 History 6 See also 7 References 7.1 Footnotes 7.2 General references 8 External links

Etymology

The origin of the word is from a Sinhalese phrase meaning "I cannot, I cannot", the word being doubled for emphasis.^[1]

Causes

Beriberi is caused by a lack of thiamine (vitamin B₁). Thiamin occurs naturally in unrefined cereals and fresh foods, particularly whole grain bread, fresh meat, legumes, green vegetables, fruit, and milk. Beriberi is therefore common in people whose diet excludes these particular types of nutrition e.g. as a result of famine.

Beriberi may be found in people whose diet consists mainly of polished white rice, which is very low in thiamin because the thiamin-bearing husk has been removed. It can also be seen in chronic alcoholics with an inadequate diet (Wernicke-Korsakoff syndrome), as well as being a rare side effect of gastric bypass surgery. If a baby is mainly fed on the milk of a mother who suffers from thiamin deficiency, then that child may develop beriberi as well.

The disease was often found in Asian countries (especially in the 19th century and before), due to those countries' reliance on white rice as a staple food.

Symptoms and effects

Its symptoms include weight loss, emotional disturbances, impaired sensory perception (Wernicke's encephalopathy), weakness and pain in the limbs, and periods of irregular heart rate. Edema (swelling of bodily tissues) is common. It may increase the amount of lactic acid and pyruvic acid within the blood. In advanced cases, the disease may cause heart failure and death.

• Wet beriberi affects the heart; it is sometimes fatal, as it causes a combination of heart failure and weakening of the capillary walls, which causes the peripheral tissues to become edematous.

• Dry beriberi causes wasting and partial paralysis resulting from damaged peripheral nerves. It is also referred to as endemic neuritis.

Treatment

Treatment for beriberi is with thiamin hydrochloride, either in tablet form or injection. A rapid and dramatic recovery within hours can be made when this is administered to patients, and their health can be improved within an hour of starting treatment. In emergency situations where concentrated thiamin supplements are unavailable, feeding the patient with a thiamin-rich diet (e.g. whole grain brown bread) will lead to recovery, though at a much slower rate.

History

In Asia, where polished white rice was the common staple food of the middle class, beriberi resulting from lack of vitamin B was endemic. In 1884, Takaki Kanehiro, a British-trained Japanese medical doctor of the Japanese Navy, observed that beriberi was endemic among low-ranking crew who often ate nothing but rice, but not among crews of Western navies and officers who consumed a Western-style diet. Kanehiro initially believed that lack of protein was the chief cause of beriberi. With the support of the Japanese Navy, he experimented using crews of two battleships; one crew was fed only white rice, while the other was fed a diet of meat, fish, barley, rice, and beans. The group that ate only white rice documented 161 crew with beriberi and 25 deaths, while the latter group had only 14 cases of beriberi and no deaths. This convinced Kanehiro and the Japanese Navy that diet was the cause of beriberi. This was confirmed in 1897, when Christiaan Eijkman discovered that feeding unpolished rice instead of the polished variety to chickens helped to prevent beriberi in the chickens. The following year, Sir Frederick Hopkins postulated that some foods contained "accessory factors"—in addition to proteins, carbohydrates, fats, et cetera—that were necessary for the functions of the human body.^[2]

In 1901, Gerrit Grijns (May 28, 1865 – November 11, 1944), Dutch physician and assistant to Christiaan Eijkman in Netherlands Indies, correctly interpreted the disease as a deficiency syndrome.^[3] Indeed, it was later shown that beriberi results from the deficiency of thiamin (vitamin B1).

Dr Edward Bright Vedder established (1910–13) an extract of rice bran as a treatment for beriberi.

Eijkman, a Dutch physician and pathologist, demonstrated that beriberi is caused by poor diet. His work led to the discovery of vitamins. Eijkman and Hopkins were awarded the 1929 Nobel Prize for Physiology or Medicine for the discovery.

See also

• Edward Bright Vedder
• Korsakoff's syndrome – also caused by thiamin deficiency

References

Footnotes

1. ^ Beriberi, Information about Beriberi
2. ^ Jack Challem (1997). "The Past, Present and Future of Vitamins"
3. ^ Grijns, G. (1901) Over polyneuritis gallinarum. I. Geneesk. Tijdscht. Ned. Ind. 43, 3-110

General references

• Angstadt JD, Bodziner RA (2005). "Peripheral polyneuropathy from thiamin deficiency following laparoscopic Roux-en-Y gastric bypass". Obes Surg 15 (6): 890–2. doi:10.1381/0960892054222759. PMID 15978166. 
• Hawk A (2006). "The great disease enemy, Kak'ke (beriberi) and the Imperial Japanese Army". Mil Med 171 (4): 333–9. PMID 16673750. http://findarticles.com/p/articles/mi_qa3912/is_200604/ai_n16350305. 
• Diagnosing Beriberi in Emergency Situations, by Prof Mike Golden, Aberdeen University. (n.d.)
• McIntyre N, Stanley NN (1971). "Cardiac beriberi: two modes of presentation". Br Med J 3 (5774): 567–9. PMID 5571454. 
• Mouly S, Khuong MA, Cabie A, Saimot AG, Coulad JP (1996). "Beri-Beri and thiamin deficiency in HIV infection". AIDS 10 (8): 931–2. doi:10.1097/00002030-199607000-00024. PMID 8828758. 
• Shivalkar B, Engelmann I, Carp L, De Raedt H, Daelemans R (1998). "Shoshin syndrome: two case reports representing opposite ends of the same disease spectrum". Acta Cardiol 53 (4): 195–9. PMID 9842404. 
• Haiti: Mysterious Prison Ailment Traced to U.S. Rice – Jeb Sprague and Eunida Alexandra. Inter Press Service (IPS). 17 January 2007.
• Weise Prinzo Z, de Benoist B (2002). "Meeting the challenges of micronutrient deficiencies in emergency-affected populations". Proc Nutr Soc 61 (2): 251–7. doi:10.1079/PNS2002151. PMID 12133207. 

External links

• www.wrongdiagnosis.com : beriberi
• Medical Encyclopedia, Medline, National Institutes of Health.
• L Arturo Batres, MD. Beriberi.EMedicine.com

v • d • e Nutrition disorders (E40-68, 260-269) Hypoalimentation/ malnutrition Protein-energy malnutrition Kwashiorkor · Marasmus · Catabolysis Avitaminosis B vitamins B1: Beriberi/Wernicke's encephalopathy(Thiamine deficiency) · B2: Ariboflavinosis · B3: Pellagra(Niacin deficiency) · B6: Pyridoxine deficiency · B7: Biotin deficiency · B9: Folate deficiency · B12: Vitamin B12 deficiency Other vitamins A: Vitamin A deficiency/Bitot's spots · C: Scurvy · D: Hypovitaminosis D/Rickets/Osteomalacia · E: Vitamin E deficiency · K: Vitamin K deficiency Mineral deficiency Zinc · Iron · Magnesium · Chromium · Selenium (Keshan disease) · Manganese · Molybdenum · Copper · Calcium · Potassium Hyperalimentation Overweight · Obesity Childhood obesity · Obesity hypoventilation syndrome · Abdominal obesity Vitamin poisoning Hypervitaminosis A · Hypervitaminosis D · Hypervitaminosis E Mineral overload see inborn errors of metal metabolism, toxicity nutrition navs: disorders, symptoms+signs/eponymous

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Dansk (Danish)
n. - beriberi

Nederlands (Dutch)
beriberi, ziekte veroorzaakt door gebrek aan vitamine B

Français (French)
n. - béribéri

Deutsch (German)
n. - Beriberi, Reisesserkrankheit

Ελληνική (Greek)
n. - (ιατρ.) μπέρι-μπέρι

Italiano (Italian)
beriberi

Português (Portuguese)
n. - beribéri (m) (Med.)

Русский (Russian)
бери-бери

Español (Spanish)
n. - beriberi

Svenska (Swedish)
n. - beriberi

中文（简体）(Chinese (Simplified))
脚气病

中文（繁體）(Chinese (Traditional))
n. - 腳氣病

한국어 (Korean)
n. - 각기

日本語 (Japanese)
n. - 脚気

العربيه (Arabic)
‏(الاسم) مرض البري بري نتيجه نقص الفيتامين ب‏

עברית (Hebrew)
n. - ‮ברי-ברי (מחלה)‬

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