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Lathyrism

 

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Lathyrism



 
 
Lathyrism or Neurolathyrism is a neurological disease of humans and domestic animals, caused by eating certain legumes of the genus
Genus

A genus is a low-level taxonomic rank used in the classification of living and fossil organisms. The taxonomic ranks are domain , kingdom , phylum, class , order , family , genus, and species....
 Lathyrus
Lathyrus

The genus Lathyrus consists of the sweet peas and vetchlings, flowering plants in the legume family Fabaceae. There are approximately 160 species of Lathyrus; they are native to temperate areas, with a breakdown of 52 species in Europe, 30 species in North America, 78 in Asia, 24 in tropical East Africa, and 24 in temperat...
. This problem is mainly associated with Lathyrus sativus
Lathyrus sativus

Lathyrus sativus, is a legume commonly grown for human consumption and livestock feed in Asia and East Africa. It is a particularly important crop in areas that are prone to drought and famine, and is thought of as an 'insurance crop' as it produces reliable yields when all other crops fail....
 (also known as Grass pea, Kesari Dhal, Khesari Dhal or Almorta) and to a lesser degree with Lathyrus cicera
Lathyrus cicera

Lathyrus cicera is a species of Lathyrus known by the common names red pea and flatpod peavine. It is native to Europe, North Africa, and the Middle East, and it is known from other places as an introduced species....
, Lathyrus ochrus and Lathyrus clymenum containing the toxin
Toxin

A toxin is a poisonous substance produced by living cells or organisms. For a toxic substance not produced by living organisms, "toxicant" is the more appropriate term, and "toxics" is an acceptable plural....
 ODAP
Oxalyldiaminopropionic acid

Oxalyldiaminopropionic acid is the neurotoxin responsible for lathyrism....
.






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Lathyrism or Neurolathyrism is a neurological disease of humans and domestic animals, caused by eating certain legumes of the genus
Genus

A genus is a low-level taxonomic rank used in the classification of living and fossil organisms. The taxonomic ranks are domain , kingdom , phylum, class , order , family , genus, and species....
 Lathyrus
Lathyrus

The genus Lathyrus consists of the sweet peas and vetchlings, flowering plants in the legume family Fabaceae. There are approximately 160 species of Lathyrus; they are native to temperate areas, with a breakdown of 52 species in Europe, 30 species in North America, 78 in Asia, 24 in tropical East Africa, and 24 in temperat...
. This problem is mainly associated with Lathyrus sativus
Lathyrus sativus

Lathyrus sativus, is a legume commonly grown for human consumption and livestock feed in Asia and East Africa. It is a particularly important crop in areas that are prone to drought and famine, and is thought of as an 'insurance crop' as it produces reliable yields when all other crops fail....
 (also known as Grass pea, Kesari Dhal, Khesari Dhal or Almorta) and to a lesser degree with Lathyrus cicera
Lathyrus cicera

Lathyrus cicera is a species of Lathyrus known by the common names red pea and flatpod peavine. It is native to Europe, North Africa, and the Middle East, and it is known from other places as an introduced species....
, Lathyrus ochrus and Lathyrus clymenum containing the toxin
Toxin

A toxin is a poisonous substance produced by living cells or organisms. For a toxic substance not produced by living organisms, "toxicant" is the more appropriate term, and "toxics" is an acceptable plural....
 ODAP
Oxalyldiaminopropionic acid

Oxalyldiaminopropionic acid is the neurotoxin responsible for lathyrism....
. The lathyrism resulting from the ingestion of Lathyrus odoratus
Sweet pea

Sweet Pea is a flowering plant in the genus Lathyrus in the family Fabaceae , native to the eastern Mediterranean region from Sicily east to Crete....
 seeds (sweet peas) is often referred to as odoratism or osteolathyrism
Osteolathyrism

Osteolathyrism is a collagen cross-linking deficiency brought on by dietary over-reliance on the seeds of Lathyrus sativus. It is commonly seen in combination with neurolathyrism and angiolathyrism in impoverished areas where famine demands reliance on a crop with known detrimental effects....
, which is caused by a different toxin (beta-aminopropionitrile) that affects the linking of collagen
Collagen

Collagen is the main protein of connective tissue in animals and the most abundant protein in mammals, making up about 25% to 35% of the whole-body protein content....
, a protein
Protein

Proteins are organic compounds made of amino acids arranged in a linear chain and joined together by peptide bonds between the carboxyl and amino groups of adjacent amino acid Residue ....
 of connective tissue
Connective tissue

Connective tissue is a form of fibrous biological tissue.It is one of the four types of tissue in traditional classifications .Collagen is the main protein of connective tissue in animals and the most abundant protein in mammals, making up about 25% of the total protein content....
s.

Symptoms

The consumption of large quantities of Lathyrus grain containing high concentrations of the glutamate analogue neurotoxin ß-oxalyl-L-a,ß-diaminopropionic acid
Oxalyldiaminopropionic acid

Oxalyldiaminopropionic acid is the neurotoxin responsible for lathyrism....
 (ODAP, also known as ß-N-oxalyl-amino-L-alanine, BOAA) causes paralysis, characterized by lack of strength in or inability to move the lower limbs. A unique symptom of lathyrism is the emaciation
Emaciation

Emaciation occurs when a organism loses substantial amounts of much needed fat and often muscle tissue, making that organism look extremely thin....
 of gluteal muscles (buttocks). ODAP is a poison of mitochondria leading to excess cell death, especially in motor neurons.

Prevalence

This disease is prevalent in some areas of Bangladesh
Bangladesh

, officially the People's Republic of Bangladesh is a country in South Asia. It is bordered by India on all sides except for a small border with Burma to the far southeast and by the Bay of Bengal to the south....
, Ethiopia
Ethiopia

Ethiopia , officially the Federal Democratic Republic of Ethiopia, is a landlocked country situated in the Horn of Africa. Ethiopia is bordered by Eritrea to the north, Sudan to the west, Kenya to the south, Somalia to the east and Djibouti to the northeast....
, India
India

India, officially the Republic of India , is a country in South Asia. It is the List of countries and outlying territories by total area country by geographical area, the List of countries by population country, and the most populous liberal democracy in the world....
 and Nepal
Nepal

Nepal , officially the Federal Democratic Republic of Nepal, is a landlocked country in South Asia and is the world's youngest republic. It is bordered to the north by the People's Republic of China, and to the south, east, and west by India....
, and affects more men than women. The reason for this is unclear, female hormones have been suggested to offer a measure of protection, but also less food allocation and less oxidatively stressful activity [work] are plausible.

Causes


The toxicological cause of the disease has been attributed to the neurotoxin
Neurotoxin

A neurotoxin is a toxin that acts specifically on nerve cells , usually by interacting with membrane proteins such as ion channels.Some sources are more general, and define the effect of neurotoxins as occurring at nerve tissue....
 ODAP
Oxalyldiaminopropionic acid

Oxalyldiaminopropionic acid is the neurotoxin responsible for lathyrism....
 which acts as a structural analogue of the neurotransmitter
Neurotransmitter

Neurotransmitters are chemistry which relay, amplify and modulate signals between a neuron and another cell . Neurotransmitters are packaged into vesicles that cluster beneath the membrane on the presynaptic side of a synapse, and are released into the synaptic cleft, where they bind to receptors in the membrane on the postsynaptic side of...
 glutamate. Ingestion of legumes containing the toxin results mostly from ignorance of their toxicity and usually occurs where the despair of poverty
Poverty

Poverty is the shortage of common things such as food, clothing, shelter and safe drinking water, all of which determine our quality of life. It may also include the lack of access to opportunities such as education and employment which aid the escape from poverty and/or allow one to enjoy the respect of fellow citizens....
 and malnutrition
Malnutrition

Malnutrition is a general term for a medical condition caused by an improper or inadequate diet and nutrition.According to the World Health Organization, hunger and malnutrition are the single gravest threats to the world's public health and malnutrition is by far the biggest contributor to child mortality, present in half of all cases....
 leaves little other food options. Lathyrism can also be caused by food adulteration.

Prevention

Recent research suggests that sulfur amino acid
Amino acid

In chemistry, an amino acid is a molecule containing both amine and carboxyl functional groups. These molecules are particularly important in biochemistry, where this term refers to alpha-amino acids with the general formula H2NCHRCOOH, where R is an organic substituent....
s have a protective effect against the toxicity of ODAP.

Food preparation is also an important factor. Toxic amino acids are readily soluble in water and can be leached. Bacterial (lactic acid
Lactic acid

Lactic acid , also known as milk acid, is a chemical compound that plays a role in several biochemistry processes. It was first isolated in 1780 by a Swedish chemist, Carl Wilhelm Scheele, and is a carboxylic acid with a chemical formula of C3H6O3....
) and fungal (tempeh
Tempeh

Tempeh, or tempe in Javanese language, is made by a natural culturing and controlled fermentation process that binds soybeans into a cake form....
) fermentation is useful to reduce ODAP content. Moist heat (boiling
Boiling

Boiling, a type of phase transition, is the rapid vaporization of a liquid, which typically occurs when a liquid is heated to its boiling point, the temperature at which the vapor pressure of the liquid is equal to the pressure exerted on the liquid by the surrounding environmental pressure....
, steaming
Steaming

Steaming is a method of cooking using steam. Steaming is considered a relatively healthier cooking technique and capable of cooking almost all kinds of food....
) denatures proteinase inhibitors which otherwise add to the toxic effect of raw grasspea through depletion of protective sulfur amino acids.

The underlying cause for excessive consumption of grasspea is a lack of alternative food sources. This is a consequence of poverty
Poverty

Poverty is the shortage of common things such as food, clothing, shelter and safe drinking water, all of which determine our quality of life. It may also include the lack of access to opportunities such as education and employment which aid the escape from poverty and/or allow one to enjoy the respect of fellow citizens....
 and political conflict. The prevention of lathyrism is therefore a socio-economic challenge.

Historical occurrence

The first mentioned intoxication goes back to ancient India and also Hippocrates
Hippocrates

Hippocrates of Cos II or Hippokrates of Kos - ancient Greek: ; Hippokr?tes was an Ancient Greece physician of the Age of Pericles, and was considered one of the most outstanding figures in the history of medicine....
 mentiones a neurological disorder 46 B.C. in Greece caused by Lathyrus seed. Lathyrism was occurring in regular basis.

During the Spain War of Independence
Peninsular War

The Peninsular War or Spanish War of Independence was a contest between First French Empire and the allied powers of Spain, the United Kingdom of Great Britain and Ireland, and Kingdom of Portugal for control of the Iberian Peninsula during the Napoleonic Wars....
 against Napoleon, grasspea served as a famine food. This was the subject of one of Francisco de Goya's famous aquatint prints titled Gracias a la Almorta ("Thanks to the Grasspea"), depicting poor people surviving on a porridge made from grasspea flour, one of them lying on the floor, already crippled by it.

In the film 'Ashes' [English title] by Andrzej Wajda
Andrzej Wajda

Andrzej Wajda is a Poland film director. Recipient of an honorary Academy Awards, he is one of the most prominent members of the Polish Film School....
 based on the novel Popioly
Popioly

Popioly can refer to the following:* The Ashes , a 1965 Polish film* Popioly, Kuyavian-Pomeranian Voivodeship, a former village in Poland* Popioly, Warmian-Masurian Voivodeship, a village in Poland...
 [Polish title] translated as 'Lost army' [English title] by Stefan Zeromski
Stefan Zeromski

Stefan Zeromski was a Poland novelist and dramatist. He was called the "conscience of Polish literature". He also wrote under the pen names: Maurycy Zych, J?zef Katerla and Stefan Iksmorez....
 spanning the period 1798-1812, a horse is poisoned by grain from a Spanish village. The footage of the horse losing control of its hindlegs suggests that it was fed with Almortas. The scene with the horse falling down a steep cliff is very graphic.

Modern occurrence

During the post-war period
Spain under Franco

Francisco Franco became the undisputed dictator of Spain when he defeated the Second Spanish Republic in the Spanish Civil War. Franco declared an official end of hostilities on April 1 1939, and reworked the name of the republic into the ?Spanish State,? a new moniker attempting to distinguish the new regime from both the monarchy and the republic...
 in Spain
Spain

Spain or the Kingdom of Spain , is a country located in Southern Europe on the Iberian Peninsula.The Spanish constitution does not establish any official denomination of the country, even though Espa?a , Estado espa?ol and Naci?n espa?ola are used interchangeably....
, there were several outbreaks of lathyrism, caused by the shortage of food, which led people to consume excessive amounts of Almorta flour.

In Spain, a seed mixture known as comuña consisting of Lathyrus sativus, L. cicera, Vicia sativa and V. ervilia provides a potent mixture of toxic amino acids to poison monogastric (single stomached) animals. Particularly the toxin beta-cyanoalanine from seeds of V. sativa enhances the toxicity of such a mixture through its inhibition of sulfur amino acid metabolism [conversion of methionine to cysteine leading to excretion of cystathionine in urine] and hence depletion of protective reduced thiols. Its use for sheep does not pose any lathyrism problems if doses do not exceed 50 percent of the ration.

Related conditions

A related disease has been identified and named osteolathyrism
Osteolathyrism

Osteolathyrism is a collagen cross-linking deficiency brought on by dietary over-reliance on the seeds of Lathyrus sativus. It is commonly seen in combination with neurolathyrism and angiolathyrism in impoverished areas where famine demands reliance on a crop with known detrimental effects....
, because it affects the bones and connecting tissues, instead of the nervous system. It is a skeletal disorder, caused by the toxin beta-aminopropionitrile (BAPN), and characterized by hernias, dissecting aortic aneurysm
Aortic aneurysm

An aortic aneurysm is a general term for any swelling of the aorta, usually representing an underlying weakness in the wall of the aorta at that location....
s, exostoses, and kyphoscoliosis
Ehlers-Danlos syndrome

Ehlers-Danlos syndrome is a group of rare genetic disorders affecting humans caused by a defect in collagen synthesis. Depending on the individual mutation, the severity of the syndrome can vary from mild to life-threatening....
 and other skeletal deformities, apparently as the result of defective aging of collagen
Collagen

Collagen is the main protein of connective tissue in animals and the most abundant protein in mammals, making up about 25% to 35% of the whole-body protein content....
 tissue
Connective tissue

Connective tissue is a form of fibrous biological tissue.It is one of the four types of tissue in traditional classifications .Collagen is the main protein of connective tissue in animals and the most abundant protein in mammals, making up about 25% of the total protein content....
. The cause of this disease is attributed to beta-aminopropionitrile, which inhibits the copper-containing enzyme
Enzyme

Enzymes are biomolecules that catalysis chemical reactions. Almost all enzymes are proteins. In enzymatic reactions, the molecules at the beginning of the process are called Substrate , and the enzyme converts them into different molecules, the products....
 lysyl oxidase
Lysyl oxidase

Lysyl oxidase also known as protein-lysine 6-oxidase is a protein which in humans is encoded by the LOX gene. Its inhibition can cause lathyrism, but at the same time, its upregulation by tumor cells may promote metastasis of the existing tumor, causing it to become malignant and cancer....
, responsible for cross-linking procollagen and proelastin. BAPN is also a metabolic product of a compound present in sprouts of grasspea, pea and lentils.

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