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 Singhalese, 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.

Causes and symptoms

Thiamine is one of the B vitamins and plays an important role in energy metabolism and tissue building. It combines with phosphate to form the coenzyme thiamine pyrophosphate (TPP), which is essential in reactions that produce energy from glucose or that convert glucose to fat for storage in the tissues. When there is not enough thiamine in the diet, these basic energy functions are disturbed, leading to problems throughout the body.

Special situations, such as an over-active metabolism, prolonged fever, pregnancy, and breastfeeding, can increase the body's thiamine requirements and lead to symptoms of deficiency. Extended periods of diarrhea or chronic liver disease can result in the body's inability to maintain normal levels of many nutrients, including thiamine. Other persons at risk are patients with kidney failure on dialysis and those with severe digestive problems who are unable to absorb nutrients. Alcoholics are susceptible because they may substitute alcohol for food and their frequent intake of alcohol decreases the body's ability to absorb thiamine.

The following systems are most affected by beriberi:

• Gastrointestinal system. When the cells of the smooth muscles in the digestive system and glands do not get enough energy from glucose, they are unable to produce more glucose from the normal digestion of food. There is a loss of appetite, indigestion, severe constipation, and a lack of hydrochloric acid in the stomach.
• Nervous System. Glucose is essential for the central nervous system to function normally. Early deficiency symptoms are fatigue, irritability, and poor memory. If the deficiency continues, there is damage to the peripheral nerves that causes loss of sensation and muscle weakness, which is called peripheral neuropathy. The legs are most affected. The toes feel numb and the feet have a burning sensation; the leg muscles become sore and the calf muscles cramp. The individual walks unsteadily and has difficulty getting up from a squatting position. Eventually, the muscles shrink (atrophy) and there is a loss of reflexes in the knees and feet; the feet may hang limp (footdrop).
• Cardiovascular system. There is a rapid heartbeat and sweating. Eventually the heart muscle weakens. Because the smooth muscle in the blood vessels is affected, the arteries and veins relax, causing swelling, known as edema, in the legs.
• Musculoskeletal system. There is widespread muscle pain caused by the lack of TPP in the muscle tissue.

Infants who are breastfed by a thiamine-deficient mother usually develop symptoms of deficiency between the second and fourth month of life. They are pale, restless, unable to sleep, prone to diarrhea, and have muscle wasting and edema in their arms and legs. They have a characteristic, sometimes silent, cry and develop heart failure and nerve damage.

Diagnosis

A physical examination will reveal many of the early symptoms of beriberi, such as fatigue, irritation, nausea, constipation, and poor memory, but the deficiency may be difficult to identify. Information about the individual's diet and general health is also needed.

KEY TERMS

B vitamins— This family of vitamins consists of thiamine (B₁), riboflavin (B₂), niacin (B₃), pantothenic acid (B₅), pyridoxine (B₆), biotin, folic acid (B₉), and cobalamin (B₁₂). They are interdependent and involved in converting glucose to energy.

Coenzyme— A substance needed by enzymes to produce many of the reactions in energy and protein metabolism in the body.

Edema— An excess accumulation of fluid in the cells and tissues.

Enzyme— A protein that acts as a catalyst to produce chemical changes in other substances without being changed themselves.

Metabolism— All the physical and chemical changes that take place within an organism.

Peripheral neuropathy— A disease affecting the portion of the nervous system outside the brain and spinal chord. One or more nerves can be involved, causing sensory loss, muscle weakness and shrinkage, and decreased reflexes.

Thiamine pyrophosphate (TPP)— The coenzyme containing thiamine that is essential in converting glucose to energy.

There are many biochemical tests based on thiamine metabolism or the functions of TPP that can detect a thiamine deficiency. Levels of thiamine can be measured in the blood and urine and will be reduced if there is a deficiency. The urine can be collected for 24 hours to measure the level of thiamine excreted. Another reliable test measures the effect of TPP on red blood cell activity since all forms of beriberi affect the metabolism of red blood cells.

An electroencephalogram (EEG), which measures electrical activity in the brain, may be done to rule out other causes of neurologic changes. Observing improvements in the patient after giving thiamine supplements will also confirm the diagnosis.

Treatment

Treatment with thiamine reverses the deficiency in the body and relieves most of the symptoms. Severe thiamine deficiency is treated with high doses of thiamine given by injection into a muscle (intramuscular) or in a solution that goes into a vein (intravenously) for several days. Then smaller doses can be given either by injection or in pill form until the patient recovers. Usually there are other deficiencies in the B vitamins that will also need treatment.

The cardiovascular symptoms of wet beriberi can respond to treatment within a few hours if they are not too severe. Heart failure may require additional treatment with diuretics that help eliminate excess fluid and with heart-strengthening drugs like digitalis.

Recovery from peripheral neuropathy and other symptoms of dry beriberi may take longer and patients frequently become discouraged. They should stay active; physical therapy will also help in recovery.

Infantile beriberi is treated by giving thiamine to both the infant and the breast feeding mother until levels are normal.

In Wernicke-Korsakoff syndrome, thiamine should be given intravenously or by injection at first because the intestinal absorption of thiamine is probably impaired and the patient is very ill. Most of the symptoms will be relieved by treatment, though there may be residual memory loss.

Excess thiamine is excreted by the body in the urine, and negative reactions to too much thiamine are rare. Thiamine is unstable in alkali solutions, so it should not be taken with antacids or barbiturates.

Alternative treatment

Alternative treatments for beriberi deal first with correcting the thiamine deficiency. As in conventional treatments, alternative treatments for beriberi stress a diet rich in foods that provide thiamine and other B vitamins, such as brown rice, whole grains, raw fruits and vegetables, legumes, seeds, nuts, and yogurt. Drinking more than one glass of liquid with a meal should be avoided, since this may wash out the vitamins before they can be absorbed by the body. Thiamine should be taken daily, with the dose depending on the severity of the disease. Additional supplements of B vitamins, a multivitamin and mineral complex, and Vitamin C are also recommended. Other alternative therapies may help relieve the person's symptoms after the thiamine deficiency is corrected.

Prognosis

Beriberi is fatal if not treated and the longer the deficiency exists, the sicker the person becomes. Most of the symptoms can be reversed and full recovery is possible when thiamine levels are returned to normal and maintained with a balanced diet and vitamin supplements as needed.

Prevention

A balanced diet containing all essential nutrients will prevent a thiamine deficiency and the development of beriberi. People who consume large quantities of junk food like soda, pretzels, chips, candy, and high carbohydrate foods made with unenriched flours may be deficient in thiamine and other vital nutrients. They may need to take vitamin supplements and should improve their diets.

Dietary Requirements

The body's requirements for thiamine are tied to carbohydrate metabolism and expressed in terms of total intake of calories. The current recommended dietary allowances (RDA) are 0.5 mg for every 1000 calories, with a minimum daily intake of 1 mg even for those who eat fewer than 2,000 calories in a day. The RDA for children and teenagers is the same as for adults: 1.4 mg daily for males over age eleven, and 1.1 mg for females. During pregnancy, an increase to 1.5 mg daily is needed. Because of increased energy needs and the secretion of thiamine in breast milk, breast feeding mothers need 1.5 mg every day. In infants, 0.4 mg is advised.

Food Sources

The best food sources of thiamine are lean pork, beef, liver, brewer's yeast, peas and beans, whole or enriched grains, and breads. The more refined the food, as in white rice, white breads, and some cereals, the lower the thiamine. Many food products are enriched with thiamine, along with riboflavin, niacin, and iron, to prevent dietary deficiency.

During the milling process, rice is polished and all the vitamins in the exterior coating of bran are lost. Boiling the rice before husking preserves the vitamins by distributing them throughout the kernel. Food enrichment programs have eliminated beriberi in Japan and the Phillipines.

Like all B vitamins, thiamine is water soluble, which means it is easily dissolved in water. It will leach out during cooking in water and is destroyed by high heat and overcooking.

Resources

PERIODICALS

Ryan, Ruth, et al. "Beriberi Unexpected." Psychosomatics May-June 1997: 191-294.

Beriberi

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

BERIBERI

BERIBERI. 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.

See also Dietary Assessment ; Dietary Guidelines ; Disease: Metabolic Diseases ; Rice: Rice as a Food ; Rice: The Natural History of Rice ; Vitamins: Overview ; Wheat .

Kenneth John Carpenter

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.

Beriberi

Thiamin, or vitamin B₁, is a water-soluble vitamin that plays a role in energy production (through the synthesis of adenosine triphosphate [ATP]) and nerve conduction. (ATP is the major source of energy that the human body utilizes to do work.) Thiamin is found in abundance in foods such as lean pork, legumes , and yeast. In contrast, polished (white) rice, white flour, refined sugars, fats, and oils are foods lacking this vitamin. People at risk for thiamin deficiency include those who consume large quantities of alcohol and those who live in impoverished conditions, for such people are deficient in substantial amounts of vitamins and minerals .

Beriberi is a clinical manifestation of thiamin deficiency. Symptoms include nervous system abnormalities (e.g., leg cramps, muscle weakness), limb swelling, elevated pulse, and heart failure. Wernicke-Korsakoff syndrome is a related condition (with symptoms such as a jerky gait, disorientation, and impaired short-term memory) that occurs among alcoholics.

see also Nutritional Deficiency; Vitamins, Water-Soluble.

Kheng Lim

Bibliography

Morgan, Sarah L., and Weinsier, Roland L. (1998). Fundamentals of Clinical Nutrition, 2nd edition. St. Louis, MO: Mosby.

Kane, Agnes B., and Kumar, Vinay (1999). "Environmental and Nutritional Pathology." In Robbins Pathologic Basis of Disease, 6th edition. Philadelphia: W. B. Saunders.

beriberi 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.