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57-467: The municipality was created by state law in 1948. In the early 1980s, Cubatão was one of the most polluted cities in the world, nicknamed "Valley of Death", due to births of brainless children and respiratory, hepatic and blood illnesses. High air pollution was killing forest over hills around the city. It was ranked the top ten dirtiest cities in the world by Popular Science . Around 0:00 BRT (3:00 GMT) on Saturday, 25 February 1984, an oil spill set

114-470: A persistent vegetative state (PVS), involves loss of cognitive functioning of the brain. A proposal by law professor David Randolph Smith, in an attempt to prove that neocortical death should legally be treated the same as brain death, involved PET scans to determine the similarities. However, this proposal has been criticized on the basis that confirming neocortical death by PET scan may risk indeterminacy. Anencephaly can be diagnosed before delivery with

171-480: A clear understanding on the functions of brainstem anatomical structures and how to test them. Brainstem stroke syndrome can cause a range of impairments including locked-in syndrome . Duret haemorrhages are areas of bleeding in the midbrain and upper pons due to a downward traumatic displacement of the brainstem. Cysts known as syrinxes can affect the brainstem, in a condition, called syringobulbia . These fluid-filled cavities can be congenital, acquired or

228-457: A high degree of accuracy. Although anencephaly is a fatal condition, the option of abortion is dependent on the abortion laws in the state. According to a 2013 report, 26% of the world's population reside in a country where abortion is generally prohibited. In 2012, Brazil extended the right of abortion to mothers with anencephalic fetuses. This decision is, however, receiving much disapproval by several religious groups. The case of baby Theresa

285-451: A major issue in personhood debates, across the board. In regards to anencephaly, those who oppose organ donation argue that it could open the door for involuntary organ donors such as an elderly person with severe dementia. Another point of contention is the number of children who would actually benefit. There are discrepancies in statistics; however, it is known that most anencephalic children are stillborn. Proposals have been made to bypass

342-478: A major public health issue. In 1999, it was found that for American children under the age of two who are waiting for a transplant, 30–50% die before an organ becomes available. Within the medical community, the main ethical issues with organ donation are a misdiagnosis of anencephaly, the slippery slope argument, that anencephalic neonates would rarely be a source of organs, and that it would undermine confidence in organ transplantation. Slippery slope concerns are

399-418: A protective effect. Research has suggested that, overall, female babies are more likely to be affected by the disorder. One issue concerning anencephalic newborns is organ donation . Initial legal guidance came from the case of Baby Theresa in 1992, in which the boundaries of organ donation were tested for the first time. Infant organs are scarce, and the high demand for pediatric organ transplants poses

456-673: A public health investigation and the Texas Neural Tube Defect Project. It was found, that neural tube defects in general, including spina bifida , and encephalocele had been occurring in Mexican-American women undetected for years in the area. Subsequently, multiple risk factors were found, foremost folic acid deficiency , low serum vitamin B12, high serum homocysteine levels, and obesity independently contributed to risk. Increasing dietary folate intake had

513-463: A result of new genetic research, some of these are, in fact, highly related in their root cause despite the widely varying set of medical symptoms that are clinically visible in the disorders . Anencephaly is one such disease, part of an emerging class of diseases called ciliopathies . The underlying cause may be a dysfunctional molecular mechanism in the primary cilia structures of the cell , organelles present in many cellular types throughout

570-456: A subtype of neural tube defect , folic acid may play a role in anencephaly. Studies have shown that the addition of folic acid to the diet of women of child-bearing age may significantly reduce, although not eliminate, the incidence of neural tube defects. Therefore, it is recommended that all women of child-bearing age consume 0.4 mg of folic acid daily, especially those attempting to conceive or who may possibly conceive, as this can reduce

627-454: A volunteer. There have been two state bills that proposed to change current laws regarding death and organ donation. California Senate Bill 2018 proposed to amend the UDDA to define anencephalic infants as already dead, while New Jersey Assembly Bill 3367 proposed to allow anencephalic infants to be organ sources even if they are not dead. Some genetic research has been conducted to determine

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684-453: Is a rare form of anencephaly characterized by malformed cranial bones, a median cranial defect, and a cranial protrusion called area cerebrovasculosa. Area cerebrovasculosa is a section of abnormal, spongy, vascular tissue admixed with glial tissue ranging from simply a membrane to a large mass of connective tissue , hemorrhagic vascular channels, glial nodules, and disorganized choroid plexuses . The most common type of anencephaly, where

741-410: Is known as the sulcus limitans . This separates the medial motor neurons from the lateral sensory neurons. Lateral to the sulcus limitans is the area of the vestibular system , which is involved in special sensation. Moving rostrally, the inferior, middle, and superior cerebellar peduncles are found connecting the midbrain to the cerebellum. Directly rostral to the superior cerebellar peduncle, there

798-531: Is lateral to the pyramids. Emerging from the anterolateral sulci are the CN XII ( hypoglossal nerve ) rootlets. Lateral to these rootlets and the anterolateral sulci are the olives . The olives are swellings in the medulla containing underlying inferior nucleary nuclei (containing various nuclei and afferent fibers). Lateral (and dorsal) to the olives are the rootlets for CN IX ( glossopharyngeal ), CN X ( vagus ) and CN XI ( accessory nerve ). The pyramids end at

855-425: Is responsible for cognition . The remaining structure is usually covered only by a thin layer of membrane—skin, bone, meninges, etc., are all lacking. With very few exceptions, infants with this disorder do not survive longer than a few hours or days after birth. The National Institute of Neurological Disorders and Stroke (NINDS) describes the presentation of this condition as follows: "A baby born with anencephaly

912-425: Is the area postrema whose functions include the control of vomiting . The pons meets the medulla at the pontomedullary junction. This region is supplied by the joining of the basilar , vertebral arteries . The posterior inferior cerebellar artery also joins from which a large number of perforating arteries arise. Lateral spinal arteries also emerge to supply the posterior surface of the medulla oblongata. In

969-639: Is the name of an A La Carte song from 1981. Piaçaguera is a location within the city of Cubatão in the state of São Paulo , Brazil. The Rodovia Cônego Domênico Rangoni highway, formerly known as the Piaçaguera-Guarujá highway, starts there. It connects the Via Anchieta to Guarujá , crossing the Bertioga canal. It is there that the climb through the Serra do Mar escarpment begins by way of

1026-439: Is the superior medullary velum and then the two trochlear nerves. This marks the end of the pons as the inferior colliculus is directly rostral and marks the caudal midbrain. Middle cerebellar peduncle is located inferior and lateral to the superior cerebellar peduncle, connecting pons to the cerebellum. Likewise, inferior cerebellar peduncle is found connecting the medulla oblongata to the cerebellum. The main supply of blood to

1083-464: Is usually blind, deaf, unaware of its surroundings and unable to feel pain. Although some individuals with anencephaly may be born with a main brain stem , the lack of a functioning cerebrum permanently rules out the possibility of ever gaining awareness of their surroundings. Reflex actions such as breathing and responses to sound or touch may occur." Folic acid has been shown to be important in neural tube formation since at least 1991, and as

1140-477: Is usually offered to women at a higher risk of having a child with a neural tube defect to discuss available testing. It is known that people taking certain anticonvulsants and people with insulin-dependent diabetes have a higher risk of having a child with a neural tube defect. Until recently, medical literature did not indicate a connection among many genetic disorders , both genetic syndromes and genetic diseases , that are now being found to be related. As

1197-539: The Santos-Jundiaí railway line . "Piaçaguera" is a term from the Tupi language that means "old port" by way of the conjunction of the two words "peasaba" ("port") and "ûera" ("velho"). In telecommunications, the city was served by Companhia Telefônica da Borda do Campo until 1975, when it began to be served by Telecomunicações de São Paulo . In July 1998, this company was acquired by Telefónica, which adopted

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1254-729: The United States , anencephaly occurs in about 1 out of every 4600 births. Rates may be higher among Africans with rates in Nigeria estimated at 3 per 10,000 in 1990 while rates in Ghana estimated at 8 per 10,000 in 1992. As of 2005, rates in China were estimated at 5 per 10,000. A high anencephaly rate of 19.7 per 10,000 live births was found in 1990/1991 in Brownsville, Texas. A cluster of cases made national headlines and prompted

1311-474: The Vivo brand in 2012. The company is currently an operator of cell phones, fixed lines, internet (fiber optics/4G) and television (satellite and cable). Anencephaly Anencephaly is the absence of a major portion of the brain , skull , and scalp that occurs during embryonic development . It is a cephalic disorder that results from a neural tube defect that occurs when the rostral (head) end of

1368-449: The cerebrum to the medulla and to the cerebellum and also tracts that carry sensory signals to the thalamus . The pons is connected to the cerebellum by the cerebellar peduncles . The pons houses the respiratory pneumotaxic center and apneustic center that make up the pontine respiratory group in the respiratory center . The pons co-ordinates activities of the cerebellar hemispheres . The pons and medulla oblongata are parts of

1425-473: The fourth ventricle . In the floor of the fourth ventricle, various nuclei can be visualized by the small bumps that they make in the overlying tissue. In the midline and directly superior to the obex is the vagal trigone and superior to that it the hypoglossal trigone . Underlying each of these are motor nuclei for the respective cranial nerves. Superior to these trigones are fibers running laterally in both directions. These fibers are known collectively as

1482-429: The hindbrain that form much of the brainstem. The medulla oblongata , often just referred to as the medulla, is the lower half of the brainstem continuous with the spinal cord. Its upper part is continuous with the pons. The medulla contains the cardiac , dorsal and ventral respiratory groups , and vasomotor centres, dealing with heart rate , breathing and blood pressure . Another important medullary structure

1539-418: The medial geniculate nucleus of the diencephalon . The superior colliculus is positioned above the inferior colliculus, and marks the rostral midbrain. It is involved in the special sense of vision and sends its superior brachium to the lateral geniculate body of the diencephalon. The tegmentum which forms the floor of the midbrain, is ventral to the cerebral aqueduct . Several nuclei , tracts , and

1596-457: The metencephalon and the myelencephalon . The metencephalon will become the cerebellum and the pons. The more caudal myelencephalon will become the medulla . The brainstem plays important functions in breathing , heart rate , arousal / consciousness , sleep / wake functions and attention / concentration. There are three main functions of the brainstem: Ten of the twelve pairs of cranial nerves either target or are sourced from

1653-467: The neural tube fails to close, usually between the 23rd and 26th day following conception. Strictly speaking, the Greek term translates as "without a brain" (or totally lacking the inside part of the head), but it is accepted that children born with this disorder usually only lack a telencephalon , the largest part of the brain consisting mainly of the cerebral hemispheres, including the neocortex , which

1710-402: The obex , are the gracile and cuneate tubercles, respectively. Underlying these are their respective nuclei. The obex marks the end of the fourth ventricle and the beginning of the central canal . The posterior intermediate sulcus separates the gracile fasciculus from the cuneate fasciculus. Lateral to the cuneate fasciculus is the lateral funiculus . Superior to the obex is the floor of

1767-459: The pontine medulla junction, noted most obviously by the large basal pons . From this junction, CN VI ( abducens nerve ), CN VII ( facial nerve ) and CN VIII ( vestibulocochlear nerve ) emerge. At the level of the midpons, CN V (the trigeminal nerve ) emerges. Cranial nerve III (the oculomotor nerve ) emerges ventrally from the midbrain, while the CN IV (the trochlear nerve ) emerges out from

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1824-434: The reticular formation are contained here. The ventral tegmental area (VTA) is composed of paired cerebral peduncles . These transmit axons of upper motor neurons . The midbrain consists of: The pons lies between the midbrain and the medulla oblongata . It is separated from the midbrain by the superior pontine sulcus , and from the medulla by the inferior pontine sulcus . It contains tracts that carry signals from

1881-453: The spinothalamic tract ( pain , temperature, itch, and crude touch ). The parts of the brainstem are the midbrain, the pons, and the medulla oblongata; the diencephalon is sometimes considered part of the brainstem. The brainstem extends from just above the tentorial notch superiorly to the first cervical vertebra below the foramen magnum inferiorly. The midbrain is further subdivided into three parts: tectum , tegmentum , and

1938-411: The striae medullares . Continuing in a rostral direction, the large bumps are called the facial colliculi. Each facial colliculus , contrary to their names, do not contain the facial nerve nuclei. Instead, they have facial nerve axons traversing superficial to underlying abducens (CN VI) nuclei. Lateral to all these bumps previously discussed is an indented line, or sulcus that runs rostrally, and

1995-443: The ventral tegmental area . The tectum forms the ceiling. The tectum comprises the paired structure of the superior and inferior colliculi and is the dorsal covering of the cerebral aqueduct . The inferior colliculus is the principal midbrain nucleus of the auditory pathway and receives input from several peripheral brainstem nuclei, as well as inputs from the auditory cortex. Its inferior brachium (arm-like process) reaches to

2052-474: The Cart1-deficient mutants. Brainstem The brainstem (or brain stem ) is the posterior stalk-like part of the brain that connects the cerebrum with the spinal cord . In the human brain the brainstem is composed of the midbrain , the pons , and the medulla oblongata . The midbrain is continuous with the thalamus of the diencephalon through the tentorial notch , and sometimes

2109-418: The brain has entirely failed to form, except for the brain stem. Infants rarely survive more than one day after birth with holoanencephaly. The most severe type of anencephaly where area cerebrovasculosa and area medullovasculosa fill both cranial defects and the spinal column . Craniorachischisis is characterized by anencephaly accompanied by bony defects in the spine and the exposure of neural tissue as

2166-517: The brain stem, is dead. This bill was a result of much debate over the definition of death and is applicable to the debate over anencephaly. A related bill, the Uniform Anatomical Gift Act (UAGA), grants individuals and, after death, their family members the right to decide whether or not to donate organs. Because it is against the law for any person to pay money for an organ, the person in need of an organ transplant must rely on

2223-416: The brainstem from these nuclei. Diseases of the brainstem can result in abnormalities in the function of cranial nerves that may lead to visual disturbances, pupil abnormalities, changes in sensation, muscle weakness, hearing problems, vertigo, swallowing and speech difficulty, voice change, and co-ordination problems. Localizing neurological lesions in the brainstem may be very precise, although it relies on

2280-474: The brainstem is provided by the basilar arteries and the vertebral arteries . The human brainstem emerges from two of the three primary brain vesicles formed of the neural tube . The mesencephalon is the second of the three primary vesicles, and does not further differentiate into a secondary brain vesicle . This will become the midbrain. The third primary vesicle, the rhombencephalon (hindbrain) will further differentiate into two secondary vesicles,

2337-439: The brainstem nuclei. The nuclei of the oculomotor nerve (III) and trochlear nerve (IV) are located in the midbrain. The nuclei of the trigeminal nerve (V), abducens nerve (VI), facial nerve (VII) and vestibulocochlear nerve (VIII) are located in the pons. The nuclei of the glossopharyngeal nerve (IX), vagus nerve (X), accessory nerve (XI) and hypoglossal nerve (XII) are located in the medulla. The fibers of these cranial nerves exit

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2394-447: The brainstem. Other roles include the regulation of the central nervous system and the body's sleep cycle . It is also of prime importance in the conveyance of motor and sensory pathways from the rest of the brain to the body, and from the body back to the brain. These pathways include the corticospinal tract (motor function), the dorsal column-medial lemniscus pathway ( fine touch , vibration sensation , and proprioception ), and

2451-461: The causes of anencephaly. It has been found that cartilage homeoprotein (CART1) is selectively expressed in chondrocytes (cartilage cells). The CART1 gene to chromosome 12q21.3–q22 has been mapped. Also, it has been found that mice homozygous for deficiency in the Cart1 gene manifested acrania and meroanencephaly, and prenatal treatment with folic acid will suppress acrania and meroanencephaly in

2508-491: The city, costing US$ 1.2 billion so far. Although things have improved a lot, it is impossible to completely clean the soil and groundwater and while many large industries continue to work in such a small area, there will always be some pollution. Cubatão was mentioned as the "world's most polluted town" in the Jello Biafra - Sepultura collaboration "Biotech is Godzilla" on the group's 1993 album Chaos A.D. Cubatão

2565-419: The diencephalon is included in the brainstem. The brainstem is very small, making up around only 2.6 percent of the brain's total weight. It has the critical roles of regulating heart and respiratory function, helping to control heart rate and breathing rate . It also provides the main motor and sensory nerve supply to the face and neck via the cranial nerves . Ten pairs of cranial nerves come from

2622-463: The dorsal aspect of the midbrain. Between the two pyramids can be seen a decussation of fibers which marks the transition from the medulla to the spinal cord. The medulla is above the decussation and the spinal cord below. The most medial part of the medulla is the posterior median sulcus . Moving laterally on each side is the gracile fasciculus , and lateral to that is the cuneate fasciculus . Superior to each of these, and directly inferior to

2679-488: The homozygous inactivation of the NUAK2 kinase leads to anencephaly in humans. Animal models indicate a possible association with deficiencies of the transcription factor TEAD2 . A woman who has had one child with a neural tube defect such as anencephaly has about a 3% risk of having another child with a neural tube defect, as opposed to the background rate of 0.1% occurrence in the population at large. Genetic counseling

2736-773: The human body. The cilia defects adversely affect "numerous critical developmental signaling pathways" essential to cellular development and, thus, offer a plausible hypothesis for the often multi-symptom nature of a large set of syndromes and diseases. Known ciliopathies include primary ciliary dyskinesia , Bardet–Biedl syndrome , polycystic kidney and liver disease , nephronophthisis , Alström syndrome , Meckel–Gruber syndrome , and some forms of retinal degeneration . Anencephaly can often be diagnosed before birth through an ultrasound examination. The maternal serum alpha-fetoprotein ( AFP screening ) and detailed fetal ultrasound can be useful for screening for neural tube defects such as spina bifida or anencephaly. Meroanencephaly

2793-606: The legal and ethical issues surrounding organ donation. These include waiting for death to occur before procuring organs, expanding the definition of death, creating a special legal category for anencephalic infants, and defining them as non-persons. In the United Kingdom , a child born with anencephaly was reported as the country's youngest organ donor. Teddy Houlston was diagnosed as anencephalic at 12 weeks of gestation. His parents, Jess Evans and Mike Houlston, decided against abortion and instead proposed organ donation. Teddy

2850-413: The medial part of the medulla is the anterior median fissure . Moving laterally on each side are the medullary pyramids . The pyramids contain the fibers of the corticospinal tract (also called the pyramidal tract), or the upper motor neuronal axons as they head inferiorly to synapse on lower motor neuronal cell bodies within the anterior grey column of the spinal cord . The anterolateral sulcus

2907-615: The result of a tumor. Criteria for claiming brainstem death in the UK have developed in order to make the decision of when to stop ventilation of somebody who could not otherwise sustain life. These determining factors are that the patient is irreversibly unconscious and incapable of breathing unaided. All other possible causes must be ruled out that might otherwise indicate a temporary condition. The state of irreversible brain damage has to be unequivocal. There are brainstem reflexes that are checked for by two senior doctors so that imaging technology

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2964-524: The risk to 0.03%. It is not advisable to wait until pregnancy has begun, since, by the time a woman knows she is pregnant, the critical time for the formation of a neural tube defect has usually already passed. A physician may prescribe even higher dosages of folic acid (5 mg/day) for women having had a previous pregnancy with a neural tube defect. Neural tube defects can follow patterns of heredity , with direct evidence of autosomal recessive inheritance. As reported by Bruno Reversade and colleagues,

3021-400: The shantytown Vila Socó on fire, killing 93 people according to official figures of the government, though the actual death toll may be more than 200. The contamination of workers with persistent organic pollutants put the now defunct Rhodia into Greenpeace 's top 10 world's worst corporate crimes ever in its report to Rio Summit in 1992. Strong efforts were made to diminish pollution in

3078-468: The vault of the skull fails to form. Craniorachischisis occurs in about 1 of every 1000 live births, but various physical and chemical tests can detect neural tube closure during early pregnancy. There is no cure or standard treatment for anencephaly. Prognosis is extremely poor, as many anencephalic fetuses do not survive birth and infants that are not stillborn will usually die within a few hours or days after birth from cardiorespiratory arrest . In

3135-514: Was born on 22 April 2014, in Cardiff , Wales, and lived for 100 minutes, after which his heart and kidneys were removed. His kidneys were later transplanted into an adult in Leeds . Teddy's twin, Noah, was born healthy. There are four different concepts used to determine brain death : failure of heart, failure of lungs, whole brain death, and neocortical death. Neocortical death, similar to

3192-411: Was still alive. By the time she died nine days after birth, her organs had deteriorated past the point of being viable. The Uniform Determination of Death Act (UDDA) is a model bill, adopted by many US states, stating that an individual who has sustained either 1) irreversible cessation of circulatory and respiratory functions or 2) irreversible cessation of all functions of the entire brain, including

3249-414: Was the beginning of the ethical debate over anencephalic infant organ donation. The story of baby Theresa remains a focus of basic moral philosophy. Baby Theresa was born with anencephaly in 1992. Her parents, knowing that their child was going to die, requested that her organs be given for transplantation. Although her physicians agreed, Florida law prohibited the infant's organs from being removed while she

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