Beriberi is a
nervous systemThe nervous system is an organ system containing a network of specialized cells called neurons that coordinate the actions of an animal and transmit signals between different parts of its body. In most animals the nervous system consists of two parts, central and peripheral. The central nervous...
ailment caused by a
thiamine deficiency (deficiency of
vitamin B1Thiamine or thiamin or vitamin B1 , named as the "thio-vitamine" is a water-soluble vitamin of the B complex. First named aneurin for the detrimental neurological effects if not present in the diet, it was eventually assigned the generic descriptor name vitamin B1. Its phosphate derivatives are...
) in the diet.
ThiamineThiamine or thiamin or vitamin B1 , named as the "thio-vitamine" is a water-soluble vitamin of the B complex. First named aneurin for the detrimental neurological effects if not present in the diet, it was eventually assigned the generic descriptor name vitamin B1. Its phosphate derivatives are...
is involved in the breakdown of energy molecules such as
glucoseGlucose is a simple sugar and an important carbohydrate in biology. Cells use it as the primary source of energy and a metabolic intermediate...
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.
Etymology
The origin of the term is unclear, although several hypotheses have been suggested. One hypothesis is that it comes from a Sinhalese phrase meaning "weak, weak" or "I cannot, I cannot", the word being
reduplicatedReduplication in linguistics is a morphological process in which the root or stem of a word is repeated exactly or with a slight change....
for emphasis. Another hypothesis is that it is from the Arabic "bhur-bhari", meaning "sailor's asthma." In 1630, a Dutch physician named Jacob Bonitus encountered the disease while working in Java. In the first known description of Beriberi, he wrote, "A certain very troublesome affliction, which attacks men, is called by the inhabitants Beriberi (which means sheep). I believe those, whom this same disease attacks, with their knees shaking and the legs raised up, walk like sheep. It is a kind of paralysis, or rather Tremor: for it penetrates the motion and sensation of the hands and feet indeed sometimes of the whole body."
History
In Asia, where polished white rice (milled rice that has had its husk, bran, and germ removed) was the common staple food of the middle class, beriberi resulting from lack of vitamin B
1 was endemic. In 1884, Takaki Kanehiro, a British-trained Japanese medical doctor of the
Japanese NavyThe Imperial Japanese Navy was the navy of the Empire of Japan from 1869 until 1947, when it was dissolved following Japan's constitutional renunciation of the use of force as a means of settling international disputes...
, observed that beriberi was endemic among low-ranking crew who often were provided nothing but rice, but not among crews of Western navies and officers who consumed a Western-style diet.
In 1883, Kanehiro learned of a very high incidence of beriberi among cadets on a training mission from Japan to Hawaii, via New Zealand and South America that lasted for 9 months. On board, 169 men out of 376 developed the disease and 25 died. With the support of the Japanese Navy, he conducted an experiment in which another ship was deployed on the same route and under identical conditions, except that its crew was fed a diet of meat, fish, barley, rice, and beans. At the end of the voyage, this crew had suffered 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 Dr.
Christiaan EijkmanChristiaan Eijkman was a Dutch physician and professor of physiology whose demonstration that beriberi is caused by poor diet led to the discovery of vitamins...
, a
DutchThe Netherlands is a constituent country of the Kingdom of the Netherlands, located mainly in North-West Europe and with several islands in the Caribbean. Mainland Netherlands borders the North Sea to the north and west, Belgium to the south, and Germany to the east, and shares maritime borders...
physicianA physician is a health care provider who practices the profession of medicine, which is concerned with promoting, maintaining or restoring human health through the study, diagnosis, and treatment of disease, injury and other physical and mental impairments...
and pathologist, demonstrated that beriberi is caused by poor diet. He discovered that feeding unpolished rice instead of the polished variety to chickens helped to prevent beriberi in the chickens.
The following year, Sir
Frederick HopkinsSir Frederick Gowland Hopkins OM FRS was an English biochemist who was awarded the Nobel Prize in Physiology or Medicine in 1929, with Christiaan Eijkman, for the discovery of vitamins. He also discovered the amino acid tryptophan, in 1901...
postulated that some foods contained "accessory factors"—in addition to proteins, carbohydrates, fats, and salt—that were necessary for the functions of the human body.
In 1901, Gerrit Grijns (May 28, 1865 – November 11, 1944), Dutch physician and assistant to
Christiaan EijkmanChristiaan Eijkman was a Dutch physician and professor of physiology whose demonstration that beriberi is caused by poor diet led to the discovery of vitamins...
in Netherlands Indies, correctly interpreted the disease as a deficiency syndrome. Indeed, it was later shown that beriberi results from the deficiency of
thiamineThiamine or thiamin or vitamin B1 , named as the "thio-vitamine" is a water-soluble vitamin of the B complex. First named aneurin for the detrimental neurological effects if not present in the diet, it was eventually assigned the generic descriptor name vitamin B1. Its phosphate derivatives are...
(vitamin B
1).
Dr.
Edward Bright VedderDr Edward Bright Vedder was a U.S. Army physician, a noted researcher on deficiency diseases, and a medical educator. He studied beriberi, a deficiency disease affecting the peripheral nerves, establishing an extract of rice bran as its proper treatment.-Biography:Vedder was born in New York City...
established (1910–13) an extract of rice bran as a treatment for beriberi.
Eijkman and Hopkins were awarded the 1929 Nobel Prize for Physiology or Medicine for the discovery.
Prevalence
Beriberi is rare in developed countries because most foods are now vitamin-enriched. Excluding the presence of arsenic in the environment (e.g. well water) one can get enough thiamine by eating a normal, healthy diet. Today, beriberi occurs mostly in patients who abuse alcohol. Drinking heavily can lead to poor nutrition, and excess alcohol makes it harder for the body to absorb and store thiamine.
General symptoms and effects
Its
symptomA symptom is a departure from normal function or feeling which is noticed by a patient, indicating the presence of disease or abnormality...
s include weight loss,
emotionEmotion is a complex psychophysiological experience of an individual's state of mind as interacting with biochemical and environmental influences. In humans, emotion fundamentally involves "physiological arousal, expressive behaviors, and conscious experience." Emotion is associated with mood,...
al disturbances, impaired sensory perception (
Wernicke's encephalopathyWernicke encephalopathy is a syndrome characterised by ataxia, ophthalmoplegia, confusion, and impairment of short-term memory.It is caused by lesions in the medial thalamic nuclei, mammillary bodies, periaqueductal and periventricular brainstem nuclei, and superior cerebellar vermis, often...
),
weaknessMuscle weakness or myasthenia is a lack of muscle strength. The causes are many and can be divided into conditions that have true or perceived muscle weakness...
and
painPain is an unpleasant sensation often caused by intense or damaging stimuli such as stubbing a toe, burning a finger, putting iodine on a cut, and bumping the "funny bone."...
in the limbs, and periods of irregular
heart rateHeart rate is the number of heartbeats per unit of time, typically expressed as beats per minute . Heart rate can vary as the body's need to absorb oxygen and excrete carbon dioxide changes, such as during exercise or sleep....
.
EdemaEdema or oedema ; both words from the Greek , oídēma "swelling"), formerly known as dropsy or hydropsy, is an abnormal accumulation of fluid beneath the skin or in one or more cavities of the body that produces swelling...
(swelling of bodily tissues) is common. It may increase the amount of
lactic acidLactic acid, also known as milk acid, is a chemical compound that plays a role in various biochemical processes and was first isolated in 1780 by the Swedish chemist Carl Wilhelm Scheele. Lactic acid is a carboxylic acid with the chemical formula C3H6O3...
and
pyruvic acidPyruvic acid is an organic acid, a ketone, as well as the simplest of the alpha-keto acids. The carboxylate ion of pyruvic acid, CH3COCOO−, is known as pyruvate, and is a key intersection in several metabolic pathways....
within the blood. In advanced cases, the disease may cause heart failure and death.
Types
The main types of beriberi are:
- Wet beriberi affects the cardiovascular system, as well as other things.
- Dry beriberi and Wernicke-Korsakoff syndrome affect the nervous system.
- Infantile beriberi affects mostly children in developing countries.
Dry beriberi
Dry beriberi causes wasting and partial
paralysisParalysis is loss of muscle function for one or more muscles. Paralysis can be accompanied by a loss of feeling in the affected area if there is sensory damage as well as motor. A study conducted by the Christopher & Dana Reeve Foundation, suggests that about 1 in 50 people have been diagnosed...
resulting from damaged peripheral
nerveA peripheral nerve, or simply nerve, is an enclosed, cable-like bundle of peripheral axons . A nerve provides a common pathway for the electrochemical nerve impulses that are transmitted along each of the axons. Nerves are found only in the peripheral nervous system...
s. It is also referred to as
endemic neuritis. It is characterized by:
- Difficulty walking
- Tingling or loss of feeling (sensation) in hands and feet
- Loss of muscle function or paralysis of the lower legs
- Mental confusion/speech difficulties
- Pain
- Involuntary eye movements (nystagmus)
- Vomiting
Wet beriberi
Wet beriberi affects the
heartThe heart is a myogenic muscular organ found in all animals with a circulatory system , that is responsible for pumping blood throughout the blood vessels by repeated, rhythmic contractions...
; it is sometimes fatal, as it causes a combination of
heart failureHeart failure often called congestive heart failure is generally defined as the inability of the heart to supply sufficient blood flow to meet the needs of the body. Heart failure can cause a number of symptoms including shortness of breath, leg swelling, and exercise intolerance. The condition...
and weakening of the
capillaryCapillaries are the smallest of a body's blood vessels and are parts of the microcirculation. They are only 1 cell thick. These microvessels, measuring 5-10 μm in diameter, connect arterioles and venules, and enable the exchange of water, oxygen, carbon dioxide, and many other nutrient and waste...
walls, which causes the peripheral tissues to become edematous. It is also characterized by:
- Vasodilation leading to increased arteriovenous shunt
- Peripheral edema
- Paroxysmal nocturnal dyspnoea
- Increased heart rate
- Dyspnoea on exertion
- Swelling of the lower legs
Infantile beriberi
This type of beriberi is commonly found in children in developing countries.
Obvious signs and symptoms are crying, but not loudly and without tears. Untreated, it can prove fatal within 24 hours.
Exams and tests
A physical examination may show signs of congestive heart failure, which include:
- Difficulty breathing with neck veins
The jugular venous pressure is the indirectly observed pressure over the venous system...
that stick out
- Enlarged heart
- Fluid in the lungs
- Rapid heartbeat
- Swelling in both lower legs
- Confusion, memory loss, delusions, and lost sensitivity to vibrations may be witnessed on late-stage patients.
A neurological exam may show signs of:
- Changes in the gait
- Coordination problems
- Decreased reflexes
- Drooping of the eyelids
Blood tests will measure the amount of thiamine in the blood while urine tests will determine if thiamine is passing through the urine.
Treatment
The goal of treatment is to provide the thiamine the body is lacking. This is done with thiamine supplements which are given by injection or taken by mouth.
Other vitamins may also be recommended to help.
Subsequent blood tests will determine if the thiamine supplements are being effective.
Treatment for beriberi is with thiamine 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.
Causes
Beriberi is caused by a lack of
thiamineThiamine or thiamin or vitamin B1 , named as the "thio-vitamine" is a water-soluble vitamin of the B complex. First named aneurin for the detrimental neurological effects if not present in the diet, it was eventually assigned the generic descriptor name vitamin B1. Its phosphate derivatives are...
(vitamin B
1). Thiamine occurs naturally in unrefined
cerealCereals are grasses cultivated for the edible components of their grain , composed of the endosperm, germ, and bran...
s and fresh foods, particularly
whole grainWhole grains are cereal grains that contain cereal germ, endosperm, and bran, in contrast to refined grains, which retain only the endosperm. Whole grains can generally be sprouted while refined grains generally will not sprout. Whole-meal products are made by grinding whole grains in order to make...
bread, fresh meat,
legumes, green vegetables, fruit, milk, etc. Beriberi is therefore common in people whose
dietIn nutrition, diet is the sum of food consumed by a person or other organism. Dietary habits are the habitual decisions an individual or culture makes when choosing what foods to eat. With the word diet, it is often implied the use of specific intake of nutrition for health or weight-management...
excludes these particular types of nutrition e.g. as a result of
famineA famine is a widespread scarcity of food, caused by several factors including crop failure, overpopulation, or government policies. This phenomenon is usually accompanied or followed by regional malnutrition, starvation, epidemic, and increased mortality. Every continent in the world has...
.
Beriberi may be found in people whose diet consists mainly of polished
white riceWhite rice is the name given to milled rice that has had its husk, bran, and germ removed. This alters the flavour, texture and appearance of the rice and helps prevent spoilage and extend its storage life. After milling, the rice is polished, resulting in a seed with a bright, white, shiny...
, which is very low in thiamine because the thiamin-bearing husk has been removed. It can also be seen in chronic
alcoholicAlcoholism is a broad term for problems with alcohol, and is generally used to mean compulsive and uncontrolled consumption of alcoholic beverages, usually to the detriment of the drinker's health, personal relationships, and social standing...
s (
Wernicke-Korsakoff syndromeWernicke–Korsakoff syndrome is a manifestation of thiamine deficiency, or beriberi. This is usually secondary to alcohol abuse...
),
Arsenic poisoningArsenic poisoning is a medical condition caused by increased levels of the element arsenic in the body. Arsenic interferes with cellular longevity by allosteric inhibition of an essential metabolic enzyme...
causes alterations in cellular metabolism resulting in blockage of thiamine use which results in thiamine deficiency without any dietary shortfall. The mechanism of arsenic neuropathy may be similar to the neuropathy of thiamine deficiency [Sexton and Gowdy 1963], whereby arsenic inhibits the conversion of pyruvate to acetyl coenzyme A and thus blocks the
Krebs cycleThe citric acid cycle — also known as the tricarboxylic acid cycle , the Krebs cycle, or the Szent-Györgyi-Krebs cycle — is a series of chemical reactions which is used by all aerobic living organisms to generate energy through the oxidization of acetate derived from carbohydrates, fats and...
.
The disease was often found in
AsiaAsia is the world's largest and most populous continent, located primarily in the eastern and northern hemispheres. It covers 8.7% of the Earth's total surface area and with approximately 3.879 billion people, it hosts 60% of the world's current human population...
n countries (especially in the 19th century and before), due to those countries' reliance on
white riceWhite rice is the name given to milled rice that has had its husk, bran, and germ removed. This alters the flavour, texture and appearance of the rice and helps prevent spoilage and extend its storage life. After milling, the rice is polished, resulting in a seed with a bright, white, shiny...
as a
staple foodA staple food is one that is eaten regularly and in such quantities that it constitutes a dominant portion of a diet, and that supplies a high proportion of energy and nutrient needs. Most people live on a diet based on one or more staples...
.
Thiamine deficiency causes neuropathy through
neuronA neuron is an electrically excitable cell that processes and transmits information by electrical and chemical signaling. Chemical signaling occurs via synapses, specialized connections with other cells. Neurons connect to each other to form networks. Neurons are the core components of the nervous...
death due to its effects upon
astrocyteAstrocytes , also known collectively as astroglia, are characteristic star-shaped glial cells in the brain and spinal cord...
s. This causes alterations in their glutamate uptake, through changes in the levels of the astrocytic glutamate transporters EAAT1 and EAAT2 creating
excitotoxicityExcitotoxicity is the pathological process by which nerve cells are damaged and killed by excessive stimulation by neurotransmitters such as glutamate and similar substances. This occurs when receptors for the excitatory neurotransmitter glutamate such as the NMDA receptor and AMPA receptor are...
. Other changes include those to the
GABAGabâ or gabaa, for the people in many parts of the Philippines), is the concept of a non-human and non-divine, imminent retribution. A sort of negative karma, it is generally seen as an evil effect on a person because of their wrongdoings or transgressions...
transporter subtype GAT-3, GFAP,
glutamine synthetaseGlutamine synthetase is an enzyme that plays an essential role in the metabolism of nitrogen by catalyzing the condensation of glutamate and ammonia to form glutamine:Glutamate + ATP + NH3 → Glutamine + ADP + phosphate...
, the water channel protein
Aquaporin 4Aquaporin 4 also known as AQP4 is protein which in humans is encoded by the AQP4 gene. AQP4 belongs to the aquaporin family of integral membrane proteins that conduct water through the cell membrane.- Function :...
. These create
lactic acidosisLactic acidosis is a physiological condition characterized by low pH in body tissues and blood accompanied by the buildup of lactate especially D-lactate, and is considered a distinct form of metabolic acidosis. The condition typically occurs when cells receive too little oxygen , for example...
, brain edema,
oxidative stressOxidative stress represents an imbalance between the production and manifestation of reactive oxygen species and a biological system's ability to readily detoxify the reactive intermediates or to repair the resulting damage...
,
inflammationInflammation is part of the complex biological response of vascular tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. Inflammation is a protective attempt by the organism to remove the injurious stimuli and to initiate the healing process...
, and
white matterWhite matter is one of the two components of the central nervous system and consists mostly of myelinated axons. White matter tissue of the freshly cut brain appears pinkish white to the naked eye because myelin is composed largely of lipid tissue veined with capillaries. Its white color is due to...
impairment.
A rare condition known as genetic beriberi is passed down through families. People with genetic beriberi lose the ability to absorb thiamine from foods. This can happen slowly over time and symptoms occur when the person is an adult. However, because doctors may not consider beriberi in non-alcoholics, this diagnosis is often missed.
Beriberi can occur in breast-fed infants when the mother's body is lacking in thiamine. The condition can also affect infants who are fed unusual formulas that don't have enough thiamine.
Getting
dialysisIn medicine, dialysis is a process for removing waste and excess water from the blood, and is primarily used to provide an artificial replacement for lost kidney function in people with renal failure...
and taking high doses of diuretics can raise the risk of beriberi. It is also occasionally diagnosed in patients having undergone roux-en-y gastric bypass or other enteric diversion weight-loss surgery.
See also
- Korsakoff's syndrome
Korsakoff's syndrome is a neurological disorder caused by the lack of thiamine in the brain. Its onset is linked to chronic alcohol abuse and/or severe malnutrition...
and Wernicke's encephalopathyWernicke encephalopathy is a syndrome characterised by ataxia, ophthalmoplegia, confusion, and impairment of short-term memory.It is caused by lesions in the medial thalamic nuclei, mammillary bodies, periaqueductal and periventricular brainstem nuclei, and superior cerebellar vermis, often...
– also caused by thiamine deficiency.
External links
- www.wrongdiagnosis.com : beriberi
- Medical Encyclopedia, Medline
MEDLINE is a bibliographic database of life sciences and biomedical information. It includes bibliographic information for articles from academic journals covering medicine, nursing, pharmacy, dentistry, veterinary medicine, and health care...
, National Institutes of HealthThe National Institutes of Health are an agency of the United States Department of Health and Human Services and are the primary agency of the United States government responsible for biomedical and health-related research. Its science and engineering counterpart is the National Science Foundation...
.
- L Arturo Batres, MD. Beriberi.EMedicine.com