Neurolathyrism

Last updated
Neurolathyrism
Specialty Neurology
Symptoms Weakness, fatigue, paralysis of the legs, atrophy of leg muscles
Usual onsetGradual
DurationPermanent
Diagnostic method Based on symptoms and diet
FrequencyRare

Neurolathyrism, is a neurological disease of humans, caused by eating certain legumes of the genus Lathyrus . This disease is mainly associated with the consumption of Lathyrus sativus (also known as grass pea, chickling pea, kesari dal, or almorta) and to a lesser degree with Lathyrus cicera , Lathyrus ochrus and Lathyrus clymenum [1] containing the toxin ODAP.

Contents

This is not to be confused with osteolathyrism, a different type of lathyrism that affects the connective tissues. [2] Osteolathyrism results from the ingestion of Lathyrus odoratus seeds (sweet peas) and is often referred to as odoratism. It is caused by a different toxin (beta-aminopropionitrile) which affects the linking of collagen, a protein of connective tissues.

Another type of lathyrism is angiolathyrism which is similar to osteolathyrism in its effects on connective tissue. However, the blood vessels are affected as opposed to bone.

Signs and symptoms

The consumption of large quantities of Lathyrus seeds containing high concentrations of the neurotoxic glutamate analogue β-oxalyl-L-α,β-diaminopropionic acid (ODAP, also known as β-N-oxalyl-amino-L-alanine, or BOAA) causes paralysis, characterized by lack of strength in or inability to move the lower limbs, and may involve pyramidal tracts, producing signs of upper motor neuron damage. The toxin may also cause aortic aneurysm. [3] [4] A unique symptom of lathyrism is the atrophy of gluteal (buttocks) muscles. ODAP is a poison of the mitochondria, [4] leading to excess cell death, especially in motor neurons. [ citation needed ] Children can additionally develop bone deformity and reduced brain development. [5]

A related disease has been identified and named osteolathyrism, 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, aortic dissection, exostoses, and kyphoscoliosis and other skeletal deformities, apparently as the result of defective aging of collagen tissue. The cause of this disease is attributed to beta-aminopropionitrile, which inhibits the copper-containing enzyme lysyl oxidase, responsible for cross-linking procollagen and proelastin. BAPN is also a metabolic product of a compound present in sprouts of grasspea, pea and lentils. [6] Disorders that are clinically similar are konzo and lytico-bodig disease.[ citation needed ]

Causes

Glutamic acid L-Glutaminsaure - L-Glutamic acid.svg
Glutamic acid
Oxalyldiaminopropionic acid Beta-oxalylamino-L-alanine.svg
Oxalyldiaminopropionic acid

The toxicological cause of the disease has been attributed to the neurotoxin ODAP which acts as a structural analogue of the neurotransmitter glutamate. Lathyrism can also be caused by deliberate food adulteration.[ citation needed ]

Association with famine

Ingestion of legumes containing the toxin occurs despite an awareness of the means to detoxify Lathyrus. [7] Drought conditions can lead to shortages of both fuel and water, preventing the necessary detoxification steps from being taken, particularly in impoverished countries. [5] Lathyrism usually occurs where the combination of poverty and food insecurity leaves few other food options.[ citation needed ]

Diagnosis

There are no diagnostic criteria for neurolathyrism. Diagnosis is based on clinical features and exclusion of other diagnoses. [8]

Prevention

Eating the chickling pea with legumes having high concentrations of sulphur-based amino acids reduces the risk of lathyrism if such grain is available. Food preparation is also an important factor. Toxic amino acids are readily soluble in water and can be leached. Bacterial (lactic acid) and fungal (tempeh) fermentation is useful to reduce ODAP content. Moist heat (boiling, steaming) denatures protease inhibitors which otherwise add to the toxic effect of raw chickling pea through depletion of protective sulfur amino acids. During drought and famine, water for steeping and fuel for boiling are often also in short supply. Poor people sometimes know how to reduce the chance of developing lathyrism but face a choice between starvation and risking lathyrism. [5]

Epidemiology

This disease is prevalent in some areas of Bangladesh, Ethiopia, India and Nepal, [9] and affects more men than women. Men between 25 and 40 are particularly vulnerable. [5]

History

The first mentioned intoxication goes back to ancient India and also Hippocrates mentions a neurological disorder 46 B.C. in Greece caused by Lathyrus seed. [10]

During the Spanish War of Independence 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.[ citation needed ]

During WWII, on the order of Colonel I. Murgescu, commandant of the Vapniarka concentration camp in Transnistria, the detainees - most of them Jews - were fed nearly exclusively with fodder pea. Consequently, they became ill from lathyrism. [11]

Modern occurrence

During the post Civil war period in Spain, there were several outbreaks of lathyrism, caused by the shortage of food, which led people to consume excessive amounts of almorta flour. [12]

In Spain, a seed mixture known as comuña [13] 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 β-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. [14]

Ronald Hamilton suggested in his paper The Silent Fire: ODAP and the death of Christopher McCandless that itinerant traveler Christopher McCandless may have died from starvation after being unable to hunt or gather food due to lathyrism-induced paralysis of his legs caused by eating the seeds of Hedysarum alpinum . [15] In 2014, a preliminary lab analysis indicated that the seeds did contain ODAP. [16] However, a more detailed mass spectrometric analysis conclusively ruled out ODAP, with a molecular weight of 176.13 and lathyrism, and instead found that the most significant contributor to his death was the toxic action of L-canavanine, with a molecular weight of 176.00, which was found in significant quantity in the Hedysarum alpinum seeds he was eating. [17]

Related Research Articles

<i>Lathyrus</i> Plant genus in the pea family Fabaceae

Lathyrus is a genus of flowering plants in the legume family Fabaceae, and contains approximately 160 species. Commonly known as peavines or vetchlings, 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 temperate South America. There are annual and perennial species which may be climbing or bushy. This genus has numerous sections, including Orobus, which was once a separate genus. The genus has numerous synonyms, including Pisum, the ancient Latin name for the pea.

<span class="mw-page-title-main">Chris McCandless</span> American hiker and explorer (1968–1992)

Christopher Johnson McCandless, also known by his pseudonym "Alexander Supertramp", was an American adventurer who sought an increasingly nomadic lifestyle as he grew up. McCandless is the subject of Into the Wild, a nonfiction book by Jon Krakauer that was later made into a full-length feature film.

<i>Lathyrus sativus</i> Species of plant in the pea family

Lathyrus sativus, also known as grass pea, cicerchia, blue sweet pea, chickling pea, chickling vetch, Indian pea, white pea and white vetch, 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. The seeds contain a neurotoxin that causes lathyrism, a neurodegenerative disease, if eaten as a primary protein source for a prolonged period.

<i>Into the Wild</i> (book) 1996 nonfiction book by Jon Krakauer

Into the Wild is a 1996 non-fiction book written by Jon Krakauer. It is an expansion of a 9,000-word article by Krakauer on Chris McCandless titled "Death of an Innocent", which appeared in the January 1993 issue of Outside. The book was adapted to a film of the same name in 2007, directed by Sean Penn with Emile Hirsch starring as McCandless. Into the Wild is an international bestseller which has been printed in 30 languages and 173 editions and formats. The book is widely used as high school and college reading curriculum. Into the Wild has been lauded by many reviewers, and in 2019 was listed by Slate as one of the 50 best nonfiction works of the past quarter-century.

<i>Vicia</i> Genus of flowering plants in the bean family Fabaceae

Vicia is a genus of over 240 species of flowering plants that are part of the legume family (Fabaceae), and which are commonly known as vetches. Member species are native to Europe, North America, South America, Asia and Africa. Some other genera of their subfamily Faboideae also have names containing "vetch", for example the vetchlings (Lathyrus) or the milk-vetches (Astragalus). The lentils are included in genus Vicia, and were formerly classified in genus Lens. The broad bean is sometimes separated in a monotypic genus Faba; although not often used today, it is of historical importance in plant taxonomy as the namesake of the order Fabales, the Fabaceae and the Faboideae. The tribe Vicieae in which the vetches are placed is named after the genus' current name. The true peas (Pisum) are among the closest living relatives of vetches.

<i>Lathyrus japonicus</i> Species of legume

Lathyrus japonicus, the sea pea, beach pea, circumpolar pea or sea vetchling, is a species of flowering plant in the legume family Fabaceae, native to temperate coastal areas of the Northern Hemisphere, and Argentina.

Lytico-bodig (also Lytigo-bodig) disease, Guam disease, or amyotrophic lateral sclerosis-parkinsonism-dementia (ALS-PDC) is a neurodegenerative disease of uncertain etiology endemic to the Chamorro people of the island of Guam in Micronesia. Lytigo and bodig are Chamorro language words for two different manifestations of the same condition. ALS-PDC, a term coined by Asao Hirano and colleagues in 1961, reflects its resemblance to amyotrophic lateral sclerosis (ALS), Parkinson's disease, and Alzheimer's disease.

β-Methylamino-<small>L</small>-alanine Chemical compound

β-Methylamino-L-alanine, or BMAA, is a non-proteinogenic amino acid produced by cyanobacteria. BMAA is a neurotoxin. Its potential role in various neurodegenerative disorders is the subject of scientific research.

Osteolathyrism, sometimes referred to as odoratism, is a form of the disease Lathyrism. The disease results from the ingestion of Lathyrus odoratus seeds. The toxin found in the sweet peas is (beta-aminopropionitrile), which affects the linking of collagen, a protein of connective tissues. The condition results in damage to bone and mesenchymal connective tissues. Osteolathyrism occurs in people in combination with neurolathyrism and angiolathyrism in areas where famine demands reliance on a crop with known detrimental effects. It occurs in cattle and horses with diets overreliant upon the grass pea. Prominent symptoms include skeletal deformities and bone pain.

<span class="mw-page-title-main">Oxalyldiaminopropionic acid</span> Chemical compound

Oxalyldiaminopropionic acid (ODAP) is a structural analogue of the neurotransmitter glutamate found in the grass pea Lathyrus sativus. It is the neurotoxin responsible for the motor neuron degeneration syndrome lathyrism.

<i>Archidendron pauciflorum</i> Species of plant

Archidendron pauciflorum, commonly known as djenkol, jengkol or jering is a species of flowering tree in the pea family, Fabaceae. It is native to Southeast Asia, where the seeds are a popular dish. They are mainly consumed in Indonesia, Thailand, Myanmar, and Vietnam, prepared by frying, boiling, or roasting, and eaten raw. The beans are mildly toxic due to the presence of djenkolic acid, an amino acid that causes djenkolism. The beans and leaves of the djenkol tree are traditionally used for medicinal purposes such as purifying the blood. To date, djenkol is traded on local markets only.

<span class="mw-page-title-main">Gachas</span> Andalusian staple dish

Gachas is an ancestral basic dish of central and southern Spain. It is a gruel whose main ingredients are flour, water, olive oil, garlic, paprika and salt.

<i>Lathyrus cicera</i> Species of legume

Lathyrus cicera is a species of wild pea known by the common names red pea, red vetchling 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. This is a hairless annual herb producing a slightly winged stem. The leaves are each made up of two leaflike linear leaflets 3 to 6 centimeters long. They also bear branched, curling tendrils. The inflorescence holds a single pea flower 1 to 1.5 centimeters wide which is a varying shade of red. The fruit is a hairless dehiscent legume pod.

<span class="mw-page-title-main">Aminopropionitrile</span> Chemical compound

Aminopropionitrile, also known as β-aminopropionitrile (BAPN), is an organic compound with both amine and nitrile functional groups. It is a colourless liquid. The compound occurs naturally and is of interest in the biomedical community.

Divicine (2,6-diamino-4,5-dihydroxypyrimidine) is an oxidant and a base with alkaloidal properties found in fava beans and Lathyrus sativus. It is an aglycone of vicine. A common derivative is the diacetate form (2,6-diamino-1,6-dihydro-4,5-pyrimidinedione).

Angiolathyrism is a form of Lathyrism disease. It is mainly caused the consumption of Lathyrus sativus and to a lesser degree by Lathyrus cicera, Lathyrus ochrus and Lathyrus clymenum containing the toxin ODAP. The main chemical responsible is β-Aminopropionitrile, which prevents collagen cross-linking, thus making the blood vessel, especially the tunica media, weak. This can result in Cystic medial necrosis or a picture similar to Marfan syndrome. The damaged vessels are at an increased risk of dissection.

<i>Hedysarum alpinum</i> Species of legume

Hedysarum alpinum is a species of flowering plant in the legume family known by the common name alpine sweetvetch. It is called masu in the Iñupiaq language. It has a circumpolar distribution, occurring throughout the northern latitudes of the Northern Hemisphere. In North America it is widespread in Canada and the northernmost United States, including Alaska.

Perdur Radhakantha Adiga was an Indian endocrine biochemist, reproductive biologist, INSA Senior Scientist and an Astra chair professor of the Indian Institute of Science. He was known for his researches on vitamin-carrier proteins and Lathyrus sativus and was an elected fellow of the Indian Academy of Sciences and the Indian National Science Academy. The Council of Scientific and Industrial Research, the apex agency of the Government of India for scientific research, awarded him the Shanti Swarup Bhatnagar Prize for Science and Technology, one of the highest Indian science awards for his contributions to Medical Sciences in 1980.

Lathyrism is a condition caused by eating certain legumes of the genus Lathyrus. There are three types of lathyrism: neurolathyrism, osteolathyrism, and angiolathyrism, all of which are incurable, differing in their symptoms and in the body tissues affected.

Lathyrism is a class of neurological disease of humans.

References

  1. ↑ "Medical problems caused by plants: Lathyrism" Archived 2012-02-04 at the Wayback Machine at Prince Leopold Institute of Tropical Medicine online database
  2. ↑ Ahmad, Kamal (1982). Adverse Effects of Foods. Springer, Massachusetts: Springer US. pp. 71–2. doi:10.1007/978-1-4613-3359-3_8. ISBN   978-1-4613-3359-3 . Retrieved 30 June 2020.
  3. ↑ William Howlett (2012). Neurology in Africa. pp. 248–249.
  4. 1 2 "Lathyrism". Egton Medical Information Systems Limited. 22 March 2010.
  5. 1 2 3 4 "Lathyrism | Doctor". patient.info. 22 March 2010.
  6. ↑ COHN, D.F. (1995) "Are other systems apart from the nervous system involved in human lathyrism?" in Lathyrus sativus and Human Lathyrism: Progress and Prospects. Ed. Yusuf H, Lambein F. University of Dhaka. Dhaka pp. 101-2.
  7. ↑ Jahan, K.; Ahmad, K. (June 1984). "Detoxification of Lathyrus Sativus". Food and Nutrition Bulletin. 6 (2): 1–2. doi: 10.1177/156482658400600213 . ISSN   0379-5721.
  8. ↑ Bick, Atira S.; Meiner, Zeev; Gotkine, Marc; Levin, Netta (June 2018). "Using Advanced Imaging Methods to Study Neurolathyrism". Israel Medical Association Journal . 18 (6): 341–345. ISSN   1565-1088. PMID   27468527.
  9. ↑ Spencer P. S.; Ludolph A. C.; Kisby G. E. (July 1993). "Neurologic diseases associated with use of plant components with toxic potential". Environmental Research. 62 (1): 106–113. Bibcode:1993ER.....62..106S. doi:10.1006/enrs.1993.1095. PMID   8325256.
  10. ↑ Mark V. Barrow; Charles F. Simpson; Edward J. Miller (1974). "Lathyrism: A Review". The Quarterly Review of Biology. 49 (2): 101–128. doi:10.1086/408017. JSTOR   2820941. PMID   4601279. S2CID   33451792.
  11. ↑ isurvived.org: The Holocaust in Romania Under the Antonescu Government, by Marcu Rozen.
  12. ↑ "AZCOYTIA, Carlos (2006): "Historia de la Almorta or el veneno que llegó con el hambre tras la Guerra Civil Española". HistoriaCocina". Historiacocina.com. Retrieved 2013-09-23.
  13. ↑ The etymological origin of this name is from "común" (common) in its meaning of mixture, referring to the mix of seeds obtained when cleaning the grain and which contaminate the main grain, generally wheat.
  14. ↑ J.E. Hernández Bermejo; J. León (1994). Neglected crops 1492 from a different perspective. Rome: Food and Agriculture Organization of the United Nations. ISBN   92-5-103217-3. Archived from the original on 2019-01-29. Retrieved 2006-08-07.
  15. ↑ Ronald Hamilton (September 12, 2013). "ODAP and the death of Christopher McCandless".
  16. ↑ "How Chris McCandless Died". The New Yorker. September 12, 2013.
  17. ↑ "How Chris McCandless Died: An Update". The New Yorker. February 11, 2015.
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