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Encyclopedia > Amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis
Classification and external resources
ICD-10 G12.2
ICD-9 335.20
OMIM 105400
DiseasesDB 29148
MedlinePlus 000688
eMedicine neuro/14  emerg/24 pmr/10
MeSH D000690

Amyotrophic Lateral Sclerosis (ALS, sometimes called Maladie de Charcot, or Lou Gehrig's Disease (US)) is a progressive, usually fatal, neurodegenerative disease caused by the degeneration of motor neurons, the nerve cells in the central nervous system that control voluntary muscle movement. As a motor neuron disease, the disorder causes muscle weakness and atrophy throughout the body as both the upper and lower motor neurons degenerate, ceasing to send messages to muscles. Unable to function, the muscles gradually weaken, develop fasciculations (twitches) because of denervation, and eventually atrophy because of that denervation. The patient may ultimately lose the ability to initiate and control all voluntary movement except of the eyes. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10) is a coding of diseases and signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or diseases, as classified by the World Health Organization (WHO). ... // G00-G99 - Diseases of the nervous system (G00-G09) Inflammatory diseases of the central nervous system (G00) Bacterial meningitis, not elsewhere classified (G01) Meningitis in bacterial diseases classified elsewhere (G02) Meningitis in other infectious and parasitic diseases classified elsewhere (G03) Meningitis due to other and unspecified causes (G04) Encephalitis, myelitis... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ... The Disease Bold textDatabase is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ... MedlinePlus (medlineplus. ... eMedicine is an online clinical medical knowledge base that was founded in 1996. ... Medical Subject Headings (MeSH) is a huge controlled vocabulary (or metadata system) for the purpose of indexing journal articles and books in the life sciences. ... Image File history File linksMetadata Download high resolution version (1606x2495, 814 KB) Stephen Hawking lors de la conférence de presse à la Bibliothèque nationale de France consécutive à linauguration du labo astronomie et particules de Paris VII et de la sortie française de son ouvrage « Et Dieu... Image File history File linksMetadata Download high resolution version (1606x2495, 814 KB) Stephen Hawking lors de la conférence de presse à la Bibliothèque nationale de France consécutive à linauguration du labo astronomie et particules de Paris VII et de la sortie française de son ouvrage « Et Dieu... Stephen William Hawking, CH, CBE, FRS, FRSA, (born 8 January 1942) is a British theoretical physicist. ... Not to be confused with physician, a person who practices medicine. ... Categories: People stubs | French physicians | 1825 births | 1893 deaths | History of medicine ... Henry Louis Lou Gehrig (June 19, 1903 â€“ June 2, 1941), born Ludwig Heinrich Gehrig[2], was an American baseball player in the 1920s and 1930s, who set several Major League records and was popularly called the The Iron Horse[2] for his durability. ... Neurodegenerative disease (Greek νέυρο-, néuro-, nerval and Latin dÄ“generāre, to decline or to worsen) is a condition in which cells of the brain and spinal cord are lost. ... In vertebrates, motoneurons (also called motor neurons) are efferent neurons that originate in the spinal cord and synapse with muscle fibers to facilitate muscle contraction and with muscle spindles to modify proprioceptive sensitivity. ... Structure of a skeletal muscle Skeletal muscle is a type of striated muscle, attached to the skeleton. ... Motor neuron disease (MND) is a term used to cover a number of illnesses of the motor neurone; amyotrophic lateral sclerosis (ALS), progressive muscular atrophy (PMA), spinal muscular atrophy (SMA), progressive bulbar palsy (PBP), and primary lateral sclerosis (PLS) are all forms of MND. MND is the term used internationally... Upper motor neurons are any neurons that carry motor information down to the final common pathway, that is, any neurons that are not directly responsible for stimulating the target muscle. ... Lower motor neurons (LMNs) are the motor neurons connecting the brainstem and spinal cord to muscle fibers, bringing the nerve impulses from the upper motor neurons out to the muscles. ... It has been suggested that this article or section be merged with Twitching. ... Atrophy is the partial or complete wasting away of a part of the body. ...


Cognitive function is generally spared except in certain situations such as when ALS is associated with frontotemporal dementia.[1] However, there are reports of more subtle cognitive changes of the frontotemporal type in many patients when detailed neuropsychological testing is employed. Sensory nerves and the autonomic nervous system, which controls functions like sweating, generally remain functional. Fronto-temporal dementias selectively affect the frontal lobe of the brain. ... This article or section is in need of attention from an expert on the subject. ...

History
Year Event
1850 English scientist Augustus Waller describes the appearance of shriveled nerve fibers
1869 French doctor Jean-Martin Charcot first describes ALS in scientific literature
1881 "On Amyotrophic Lateral Sclerosis" gets translated into English and published in a three-volume edition of Lectures on the Diseases of the Nervous System
1939 ALS becomes a cause célèbre in the United States when baseball legend Lou Gehrig's career—and, two years later, his life—is ended by the disease.
1950s ALS epidemic occurs among the Chamorro people on Guam
1991 Researchers link chromosome 21 to FALS (Familial ALS)
1993 SOD1 gene on chromosome 21 found to play a role in some cases of FALS
1996 Rilutek® becomes the first FDA-approved drug for ALS
1998 El Escorial is developed as the standard for confirming ALS
2001 Alsin gene on chromosome 2 found to cause ALS2

Contents

Augustus Waller may refer to: Augustus Volney Waller (1816 - 1870), British neurophysiologist Augustus Desiré Waller (1856 - 1922), scientist and son of Augustus Volney Waller. ... Categories: People stubs | French physicians | 1825 births | 1893 deaths | History of medicine ... Look up cause célèbre in Wiktionary, the free dictionary. ... Henry Louis Lou Gehrig (June 19, 1903 â€“ June 2, 1941), born Ludwig Heinrich Gehrig[2], was an American baseball player in the 1920s and 1930s, who set several Major League records and was popularly called the The Iron Horse[2] for his durability. ...

Epidemiology, causes and risk factors

ALS is one of the most common neuromuscular diseases worldwide, and people of all races and ethnic backgrounds are affected. One to 2 people per 100,000 develop ALS each year.[2] ALS most commonly strikes people between 40 and 60 years of age, but younger and older people can also develop the disease. Men are affected slightly more often than women.


"Familial ALS" accounts for approximately 5%-10% of all ALS cases and is caused by genetic factors. Of these, approximately 1 in 10 are linked to a mutation in copper/zinc superoxide dismutase (SOD1), an enzyme responsible for scavenging free radicals. An antisense drug trial is underway using a small interfering RNA molecule (siRNA) that inhibits the production of this mutant SOD1 protein.[3] For other uses, see Copper (disambiguation). ... General Name, symbol, number zinc, Zn, 30 Chemical series transition metals Group, period, block 12, 4, d Appearance bluish pale gray Standard atomic weight 65. ... Structure of the monomeric unit of human superoxide dismutase 2 The enzyme superoxide dismutase (SOD, EC 1. ... Human glyoxalase I. Two zinc ions that are needed for the enzyme to catalyze its reaction are shown as purple spheres, and an enzyme inhibitor called S-hexylglutathione is shown as a space-filling model, filling the two active sites. ... In chemistry free radicals are uncharged atomic or molecular species with unpaired electrons or an otherwise open shell configuration. ... Antisense molecules interact with complementary strands of nucleic acids, modifying expression of genes. ... For other uses, see RNA (disambiguation). ...


Although the incidence of ALS is thought to be regionally uniform, there are three regions in the West Pacific where there has in the past been an elevated occurrence of ALS. This seems to be declining in recent decades. The largest is the area of Guam inhabited by the Chamorro people, who have historically had a high incidence (as much as 143 cases per 100,000 people per year) of a condition called Lytico-Bodig disease which is a combination of ALS, Parkinsonism, and dementia.[4] Two more areas of increased incidence are the Kii peninsula of Japan and West Papua.[5][6] The Chamorros are an indigenous people of Guam and the Mariana Islands. ... Lytico-Bodig disease is a neurological disease that exists on the Island of Guam. ...


Although there have been reports of several "clusters" including three American football players from the San Francisco 49ers, three soccer-playing friends in the south of England,[7] and reports of conjugal (husband and wife) cases in the south of France,[8][9][10][11][12] these are statistically plausible chance events. Although many authors consider ALS to be caused by a combination of genetic and environmental risk factors, so far the latter have not been firmly identified, other than a higher risk with increasing age. United States simply as football, is a competitive team sport that is both fast-paced and strategic. ... City San Francisco, California Other nicknames Niners, The Red And Gold, Bay Bombers Team colors Cardinal red, metallic gold and black Head Coach Mike Nolan Owner Denise DeBartolo York and John York General manager Lal Heneghan Mascot Sourdough Sam League/Conference affiliations All-America Football Conference (1946-1949) Western Division... Soccer redirects here. ...


Cause and risk factors

Scientists have not found a definitive cause for ALS and the onset of the disease has been linked to several factors, including: a virus; exposure to neurotoxins or heavy metals; DNA defects; immune system abnormalities; and enzyme abnormalities. There is a known hereditary factor in familial ALS (FALS); however, there is no known hereditary component in the 90-95% cases diagnosed as sporadic ALS. An inherited genetic defect linked to a defect on chromosome 21 is believed to cause approximately 40% of familial cases of ALS. This mutation is believed to be autosomal dominant. The children of those diagnosed with familial ALS have a higher risk factor for developing the disease; however, those who have close family members diagnosed with sporadic ALS have no greater a risk factor than the general population [1]. It has been suggested that this article or section be merged into Dominance relationship. ...


Some causative factors have been suggested for the increased incidence in the western Pacific. Prolonged exposure to a dietary neurotoxin is one suspected risk factor in Guam; the neurotoxin is a compound found in the seed of the cycad Cycas circinalis,[13] a tropical plant found in Guam, which was used in the human food supply during the 1950s and early 1960s. Families Cycadaceae cycas family Stangeriaceae stangeria family Zamiaceae zamia family Leaves and male cone of Cycas revoluta Cycads are an ancient group of seed plants characterized by a large crown of compound leaves and a stout trunk. ... Binomial name Cycas circinalis L. Cycas circinalis is a type of cycad thought to be linked with the degenerative disease Amyotrophic Lateral Sclerosis (ALS). ...


According to the ALS Association, military veterans are at an increased risk of contracting ALS. In its report ALS in the Military,[14] the group pointed to an almost 60% greater chance of the disease in military veterans than the general population. For Gulf War veterans, the chance is seen as twice that of the general population in a joint study by the Veterans Affairs Administration and the DOD.[citation needed] This article does not cite any references or sources. ... The United States Department of Veterans Affairs (VA) is a government-run military veteran benefit system with Cabinet-level status. ...


Symptoms

Initial symptoms

The onset of ALS may be so subtle that the symptoms are frequently overlooked. The earliest symptoms are obvious weakness and/or muscle atrophy. This is followed by twitching, cramping, or stiffness of affected muscles; muscle weakness affecting an arm or a leg; and/or slurred and nasal speech. The twitching, cramping, etc. associated with ALS is a result of the dying muscle, therefore these symptoms without clinical weakness or atrophy of affected muscle is likely not ALS. A symptom is a manifestation of a disease, indicating the nature of the disease, which is noticed by the patient. ...


The parts of the body affected by early symptoms of ALS depend on which motor neurons in the body are damaged first. About 75% of people experience "limb onset" ALS. In some of these cases, symptoms initially affect one of the legs, and patients experience awkwardness when walking or running or they notice that they are tripping or stumbling more often. Other limb onset patients first see the effects of the disease on a hand or arm as they experience difficulty with simple tasks requiring manual dexterity such as buttoning a shirt, writing, or turning a key in a lock. Occasionally the symptoms remain confined to one limb; this is known as monomelic amyotrophy. Monomelic amyotrophy (also known as MMA, Hirayamas disease, Sobue disease or Juvenile nonprogressive amyotrophy) is an untreatable, focal, lower motor neuron disease that primarily affects young (15 - 25 year-old) males in India and Japan. ...


About 25% of cases are "bulbar onset" ALS. These patients first notice difficulty speaking clearly. Speech becomes garbled and slurred. Nasality and loss of volume are frequently the first symptoms. Difficulty swallowing, and loss of tongue mobility follow. Eventually total loss of speech and the inability to protect the airway when swallowing are experienced. In medicine, aspiration is the entry of secretions or foreign material into the trachea and lungs. ...


Regardless of the part of the body first affected by the disease, muscle weakness and atrophy spread to other parts of the body as the disease progresses. Patients experience increasing difficulty moving, swallowing (dysphagia), and speaking or forming words (dysarthria). Symptoms of upper motor neuron involvement include tight and stiff muscles (spasticity) and exaggerated reflexes (hyperreflexia) including an overactive gag reflex. An abnormal reflex commonly called Babinski's sign (the large toe extends upward as the sole of the foot is stimulated) also indicates upper motor neuron damage. Symptoms of lower motor neuron degeneration include muscle weakness and atrophy, muscle cramps, and fleeting twitches of muscles that can be seen under the skin (fasciculations). Around 15–45% of patients experience pseudobulbar affect, also known as "emotional lability", which consists of uncontrollable laughter, crying or smiling, attributable to degeneration of bulbar upper motor neurons resulting in exaggeration of motor expressions of emotion. Dysphagia () is a medical term defined as difficulty swallowing. ... Look up dysarthria in Wiktionary, the free dictionary. ... Spasticity is a disorder of the bodys motor system,and especially the Central Nervous Systems (CNS), in which certain muscles are continuously contracted. ... Hyperreflexia is defined as overactive or overresponsive reflexes. ... In medicine (neurology), the Babinski reflex or Babinski sign is a reflex that can identify disease of the spinal cord and brain. ... Labile affect or Pseudobulbar affect refers to the pathological expression of laughter, crying, or smiling. ...


To be diagnosed with ALS, patients must have signs and symptoms of both upper and lower motor neuron damage that cannot be attributed to other causes.


Emerging symptoms

Although the sequence of emerging symptoms and the rate of disease progression vary from person to person, eventually patients will not be able to stand or walk, get in or out of bed on their own, or use their hands and arms. Difficulty swallowing and chewing impair the patient's ability to eat normally and increase the risk of choking. Maintaining weight will then become a problem. Because the disease usually does not affect cognitive abilities, patients are aware of their progressive loss of function and may become anxious and depressed. A small percentage of patients go on to develop frontotemporal dementia characterized by profound personality changes; this is more common among those with a family history of dementia. A larger proportion of patients experience mild problems with word-generation, attention, or decision-making. Cognitive function may be affected as part of the disease process or could be related to poor breathing at night (nocturnal hypoventilation). Health care professionals need to explain the course of the disease and describe available treatment options so that patients can make informed decisions in advance. Fronto-temporal dementias selectively affect the frontal lobe of the brain. ...


As the diaphragm and intercostal muscles (rib cage) weaken, forced vital capacity and inspiratory pressure diminish. In bulbar onset ALS, this may occur before significant limb weakness is apparent. Bilevel positive pressure ventilation (frequently referred to by the tradename BiPAP) is frequently used to support breathing, first at night, and later during the daytime as well. It is recommended that long before BiPAP becomes insufficient, patients (with the eventual help of their families) must decide whether to have a tracheostomy and long term mechanical ventilation. Most patients do not elect this route, and instead choose palliative hospice care at this point. Most people with ALS die of respiratory failure or pneumonia, not the disease itself. Intercostal muscles are several groups of muscles that run between the ribs, and help form and move the chest wall. ... Bilevel Positive Airway Pressure (or BiPAP) is a method of respiratory ventilation used primarily in the treatment of sleep apnea and various lung diseases. ... Tracheotomy is a surgical procedure used to cut a hole in the trachea through which a small tube is inserted. ... Palliative care (from Latin palliare, to cloak) is any form of medical care or treatment that concentrates on reducing the severity of disease symptoms, rather than providing a cure. ... This article is about human pneumonia. ...


ALS predominantly affects the motor neurons, and in the majority of cases the disease does not impair a patient's mind, personality, intelligence, or memory. Nor does it affect a person's ability to see, smell, taste, hear, or feel touch. Control of eye muscles is the most preserved function, although some patients with an extremely long duration of disease (20+ years) may lose eye control too. Unlike multiple sclerosis, bladder and bowel control are usually preserved in ALS, although as a result of immobility and diet changes, intestinal problems such as constipation can require intensive management.


Diagnosis

No test can provide a definite diagnosis of ALS, although the presence of upper and lower motor neuron signs in a single limb is strongly suggestive. Instead, the diagnosis of ALS is primarily based on the symptoms and signs the physician observes in the patient and a series of tests to rule out other diseases. Physicians obtain the patient's full medical history and usually conduct a neurologic examination at regular intervals to assess whether symptoms such as muscle weakness, atrophy of muscles, hyperreflexia, and spasticity are getting progressively worse.


Because symptoms of ALS can be similar to those of a wide variety of other, more treatable diseases or disorders, appropriate tests must be conducted to exclude the possibility of other conditions. One of these tests is electromyography (EMG), a special recording technique that detects electrical activity in muscles. Certain EMG findings can support the diagnosis of ALS. Another common test measures nerve conduction velocity (NCV). Specific abnormalities in the NCV results may suggest, for example, that the patient has a form of peripheral neuropathy (damage to peripheral nerves) or myopathy (muscle disease) rather than ALS. The physician may order magnetic resonance imaging (MRI), a noninvasive procedure that uses a magnetic field and radio waves to take detailed images of the brain and spinal cord. Although these MRI scans are often normal in patients with ALS, they can reveal evidence of other problems that may be causing the symptoms, such as a spinal cord tumor, multiple sclerosis, a herniated disk in the neck, syringomyelia, or cervical spondylosis. Electromyography (EMG) is a technique for evaluating and recording physiologic properties of muscles at rest and while contracting. ... Nerve conduction velocity (NCV) is a measurement made during a Nerve conduction study (NCS), a test commonly used to evaluate function of the motor and sensory nerves of the body. ... Peripheral neuropathy is the term for damage to nerves of the peripheral nervous system, which may be caused either by diseases of the nerve or from the side-effects of systemic illness. ... In medicine, a myopathy is a neuromuscular disease in which the muscle fibers do not function for any one of many reasons, resulting in muscular weakness. ... MRI redirects here. ... Look up hernia in Wiktionary, the free dictionary. ... An uncollapsed syrinx (before surgery). ...


Based on the patient's symptoms and findings from the examination and from these tests, the physician may order tests on blood and urine samples to eliminate the possibility of other diseases as well as routine laboratory tests. In some cases, for example, if a physician suspects that the patient may have a myopathy rather than ALS, a muscle biopsy may be performed. For other uses, see Blood (disambiguation). ... This article is about the urine of animals generally. ...


Infectious diseases such as human immunodeficiency virus (HIV), human T-cell leukaemia virus (HTLV), Lyme disease, syphilis[15] and tick-borne encephalitis[16] viruses can in some cases cause ALS-like symptoms. Neurological disorders such as multiple sclerosis, post-polio syndrome, multifocal motor neuropathy, and spinal muscular atrophy also can mimic certain facets of the disease and should be considered by physicians attempting to make a diagnosis. There have been documented cases of a patient presenting with ALS-like symptoms, having a positive Lyme titer, and responding to antibiotics.[17] Lyme disease is particularly difficult to diagnose. Species Human immunodeficiency virus 1 Human immunodeficiency virus 2 Human immunodeficiency virus (HIV) is a retrovirus that causes acquired immunodeficiency syndrome (AIDS, a condition in humans in which the immune system begins to fail, leading to life-threatening opportunistic infections). ... ... Lyme disease, or borreliosis, is an emerging infectious disease caused by at least three species of bacteria from the genus Borrelia. ... Syphilis is a curable sexually transmitted disease caused by the Treponema pallidum spirochete. ... Tick-borne meningoencephalitis or Tick-borne encephalitis is a tick-borne viral infection of the central nervous system affecting humans as well as most other mammals. ... Post-polio syndrome (PPS) is a condition that frequently affects survivors of poliomyelitis, a viral infection of the nervous system, after recovery from an initial paralytic attack of the virus. ... Spinal Muscular Atrophy (SMA) is a term applied to a number of different disorders, all having in common a genetic cause and the manifestation of weakness due to loss of the motor neurons of the spinal cord and brainstem. ...


Because of the prognosis carried by this diagnosis and the variety of diseases or disorders that can resemble ALS in the early stages of the disease, patients should always obtain a second neurological opinion.


A study by researchers from Mount Sinai School of Medicine identified three proteins that are found in significantly lower concentration in the cerebral spinal fluid of patients with ALS than in healthy individuals. This finding was published in the February 2006 issue of Neurology. Evaluating the levels of these three proteins proved 95% accurate for diagnosing ALS. The three protein markers are TTR, cystatin C, and the carboxyl-terminal fragment of neuroendocrine protein 7B2). These are the first biomarkers for this disease and may be first tools for confirming diagnosis of ALS. With current methods, the average time from onset of symptoms to diagnosis is around 12 months. Improved diagnostic markers may provide a means of early diagnosis, allowing patients to receive relief from symptoms years earlier.[18] Neurology is a scientific journal published by the American Academy of Neurology. ...


Etiology

SOD1

The cause of ALS is not known, though an important step toward answering that question came in 1993 when scientists discovered that mutations in the gene that produces the Cu/Zn superoxide dismutase (SOD1) enzyme were associated with some cases (approximately 20%) of familial ALS. This enzyme is a powerful antioxidant that protects the body from damage caused by superoxide, a toxic free radical. Free radicals are highly reactive molecules produced by cells during normal metabolism. Free radicals can accumulate and cause damage to DNA and proteins within cells. Although it is not yet clear how the SOD1 gene mutation leads to motor neuron degeneration, researchers have theorized that an accumulation of free radicals may result from the faulty functioning of this gene. Current research, however, indicates that motor neuron death is not likely a result of lost or compromised dismutase activity, suggesting mutant SOD1 induces toxicity in some other way (a gain of function).[19][20] Structure of the monomeric unit of human superoxide dismutase 2 The enzyme superoxide dismutase (SOD, EC 1. ... Lewis electron configuration of superoxide. ... In chemistry free radicals are uncharged atomic or molecular species with unpaired electrons or an otherwise open shell configuration. ... Structure of the coenzyme adenosine triphosphate, a central intermediate in energy metabolism. ...


Studies involving transgenic mice have yielded several theories about the role of SOD1 in mutant SOD1 familial amyotrophic lateral sclerosis. Mice lacking the SOD1 gene entirely do not customarily develop ALS, although they do exhibit an acceleration of age-related muscle atrophy (sarcopenia) and a shortened lifespan (see article on superoxide dismutase). This indicates that the toxic properties of the mutant SOD1 are a result of a gain in function rather than a loss of normal function. In addition, aggregation of proteins has been found to be a common pathological feature of both familial and sporadic ALS (see article on proteopathy). Interestingly, in mutant SOD1 mice, aggregates (misfolded protein accumulations) of mutant SOD1 were found only in diseased tissues, and greater amounts were detected during motor neuron degeneration.[21] It is speculated that aggregate accumulation of mutant SOD1 plays a role in disrupting cellular functions by damaging mitochondria, proteasomes, protein folding chaperones, or other proteins.[22] Any such disruption, if proven, would lend significant credibility to the theory that aggregates are involved in mutant SOD1 toxicity. However, it is important to remember that SOD1 mutations cause only 10% or so of overall cases and the etiological mechanisms may be distinct from those responsible for the sporadic form of the disease. Yet, the ALS-SOD1 mice remain the best model of the disease thus far. A genetically modified organism is an organism whose genetic material has been deliberately altered. ... Please wikify (format) this article as suggested in the Guide to layout and the Manual of Style. ... Structure of the monomeric unit of human superoxide dismutase 2 The enzyme superoxide dismutase (SOD, EC 1. ... Proteopathy (Proteo- [pref. ... In cell biology, a mitochondrion is an organelle found in the cells of most eukaryotes. ... Cartoon representation of a proteasome. ...


Other factors

Studies also have focused on the role of glutamate in motor neuron degeneration. Glutamate is one of the chemical messengers or neurotransmitters in the brain. Scientists have found that, compared to healthy people, ALS patients have higher levels of glutamate in the serum and spinal fluid. Laboratory studies have demonstrated that neurons begin to die off when they are exposed over long periods to excessive amounts of glutamate (excitotoxicity). Now, scientists are trying to understand what mechanisms lead to a buildup of unneeded glutamate in the spinal fluid and how this imbalance could contribute to the development of ALS. Failure of astrocytes to sequester glutamate from the extracellular fluid surrounding the neurones has been proposed as a possible cause of this glutamate-mediated neurodegeneration. Glutamate is the anion of glutamic acid. ... Neurotransmitters are chemicals that are used to relay, amplify and modulate electrical signals between a presynaptic and a postsynaptic neuron. ... Blood plasma is the liquid component of blood, in which the blood cells are suspended. ... Excitotoxicity is the pathological process by which nerve cells are damaged and killed by glutamate and similar substances. ... Astrocytes, also known as astroglia, are characteristic star-shaped cells in the brain. ...


Riluzole is currently the only FDA approved drug for ALS and targets glutamate transporters. Its very modest benefit to patients has bolstered the argument that glutamate is not a primary cause of the disease. The antibiotic ceftriaxone has demonstrated an unexpected effect on glutamate and appears to be a beneficial treatment for ALS in animal models. Ceftriaxone is currently being tested in clinical trials. Riluzole is a drug used to treat amyotrophic lateral sclerosis. ... Ceftriaxone (INN) (IPA: ) is a third-generation cephalosporin antibiotic. ...


Autoimmune responses which occur when the body's immune system attacks normal cells have been suggested as one possible cause for motor neuron degeneration in ALS. Some scientists theorize that antibodies may directly or indirectly impair the function of motor neurons, interfering with the transmission of signals between the brain and muscles. More recent evidence indicates that the nervous system's immune cells, microglia, are heavily involved in the later stages of the disease. Microglia cells positive for lectins Microglia are a type of glial cell that act as the immune cells of the Central nervous system (CNS). ...


In searching for the cause of ALS, researchers have also studied environmental factors such as exposure to toxic or infectious agents. Other research has examined the possible role of dietary deficiency or trauma. However, as of yet, there is insufficient evidence to implicate these factors as causes of ALS.


Future research may show that many factors, including a genetic predisposition, are involved in the development of ALS.


Treatment

No cure has yet been found for ALS. However, the Food and Drug Administration (FDA) has approved the first drug treatment for the disease: Riluzole (Rilutek). Riluzole is believed to reduce damage to motor neurons by decreasing the release of glutamate. Clinical trials with ALS patients showed that riluzole lengthens survival by several months, and may have a greater survival benefit for those with a bulbar onset. The drug also extends the time before a patient needs ventilation support. Riluzole does not reverse the damage already done to motor neurons, and patients taking the drug must be monitored for liver damage and other possible side effects. However, this first disease-specific therapy offers hope that the progression of ALS may one day be slowed by new medications or combinations of drugs. A small, open-label study recently suggested that the drug lithium which traditionally is used for the treatment of bipolar affective disorder may slow progression in both animal models and the human form of the disease.[23] However, further research is needed to establish whether the effect is real or not. FDA redirects here. ... Riluzole is a drug used to treat amyotrophic lateral sclerosis. ... This article is about the chemical element. ... Bipolar Affective Disorder, also known as manic depression, BPAD, or BP is a mood disorder resulting in unusually extreme highs and lows of an individuals mood, i. ...


Other treatments for ALS are designed to relieve symptoms and improve the quality of life for patients. This supportive care is best provided by multidisciplinary teams of health care professionals such as physicians; pharmacists; physical, occupational, and speech therapists; nutritionists; social workers; and home care and hospice nurses. Working with patients and caregivers, these teams can design an individualized plan of medical and physical therapy and provide special equipment aimed at keeping patients as mobile and comfortable as possible.


Physicians can prescribe medications to help reduce fatigue, ease muscle cramps, control spasticity, and reduce excess saliva and phlegm. Drugs also are available to help patients with pain, depression, sleep disturbances, and constipation. Pharmacists can give advice on the proper use of medications and monitor a patient's prescriptions to avoid risks of drug interactions. Spasticity is a disorder of the bodys motor system,and especially the Central Nervous Systems (CNS), in which certain muscles are continuously contracted. ... For the band, see Saliva (band). ... Phlegm (pronounced ) is sticky fluid secreted by the typhoid membranes of animals. ... On the Threshold of Eternity. ... Constipation, costiveness, or irregularity, is a condition of the digestive system where a person (or animal) experiences hard feces that are difficult to egest. ... Pharmacists are health professionals who practice pharmacy. ...


Physical therapy and special equipment such as assistive technology can enhance patients' independence and safety throughout the course of ALS. Gentle, low-impact aerobic exercise such as walking, swimming, and stationary bicycling can strengthen unaffected muscles, improve cardiovascular health, and help patients fight fatigue and depression. Range of motion and stretching exercises can help prevent painful spasticity and shortening (contracture) of muscles. Physical therapists can recommend exercises that provide these benefits without overworking muscles. Occupational therapists can suggest devices such as ramps, braces, walkers, and wheelchairs that help patients remain mobile. Physical therapy (or physiotherapy[1]) is the provision of services to people and populations to develop, maintain and restore maximum movement and functional ability throughout the lifespan. ... Assistive Technology (AT) is a generic term that includes assistive, adaptive, and rehabilitative devices and the process used in selecting, locating, and using them. ... Aerobic exercise refers to exercise that involves or improves oxygen consumption by the body. ... Swimmer redirects here. ... An exercise bicycle is a stationary bicycle used for exercise and not for travel. ... It has been suggested that this article or section be merged into Occupational therapy. ...


ALS patients who have difficulty speaking may benefit from working with a speech-language pathologist. These health professionals can teach patients adaptive strategies such as techniques to help them speak louder and more clearly. As ALS progresses, speech-language pathologists can recommend the use of augmentative and alternative communication such as voice amplifiers, speech-generating devices (or voice output communication devices) and/or low tech communication techniques such as alphabet boards or yes/no signals. These methods and devices help patients communicate when they can no longer speak or produce vocal sounds. With the help of occupational Therapists, speech-generating devices can be activated by switches or mouse emulation techniques controlled by small physical movements of, for example, the head, finger or eyes. It has been suggested that Speech pathology, Speech therapy, Phoniatrics be merged into this article or section. ... Augmentative and alternative communication (AAC) refers to an area of research, clinical, and educational practice. ...


Patients and caregivers can learn from speech-language pathologists and nutritionists how to plan and prepare numerous small meals throughout the day that provide enough calories, fiber, and fluid and how to avoid foods that are difficult to swallow. Patients may begin using suction devices to remove excess fluids or saliva and prevent choking. When patients can no longer get enough nourishment from eating, doctors may advise inserting a feeding tube into the stomach. The use of a feeding tube also reduces the risk of choking and pneumonia that can result from inhaling liquids into the lungs. The tube is not painful and does not prevent patients from eating food orally if they wish.


When the muscles that assist in breathing weaken, use of nocturnal ventilatory assistance (intermittent positive pressure ventilation (IPPV) or bilevel positive airway pressure (BIPAP)) may be used to aid breathing during sleep. Such devices artificially inflate the patient's lungs from various external sources that are applied directly to the face or body. When muscles are no longer able to maintain oxygen and carbon dioxide levels, these devices may be used full-time. Wikibooks has more about this subject: First Aid/CPR Cardiopulmonary resuscitation (CPR), is emergency first aid for an unconscious person on whom breathing and pulse cannot be detected. ... Bilevel Positive Airway Pressure (or BiPAP) is a method of respiratory ventilation used primarily in the treatment of sleep apnea and various lung diseases. ...


Patients may eventually consider forms of mechanical ventilation (respirators) in which a machine inflates and deflates the lungs. To be effective, this may require a tube that passes from the nose or mouth to the windpipe (trachea) and for long-term use, an operation such as a tracheotomy, in which a plastic breathing tube is inserted directly in the patient's windpipe through an opening in the neck. Patients and their families should consider several factors when deciding whether and when to use one of these options. Ventilation devices differ in their effect on the patient's quality of life and in cost. Although ventilation support can ease problems with breathing and prolong survival, it does not affect the progression of ALS. Patients need to be fully informed about these considerations and the long-term effects of life without movement before they make decisions about ventilation support. It must be pointed out that some patients under long-term tracheostomy intermittent positive pressure ventilation with deflated cuffs or cuffless tracheostomy tubes (leak ventilation) are able to speak. This technique preserves speech in some patients with long-term mechanical ventilation. Windpipe redirects here. ... Completed tracheotomy: 1 - Vocal cords 2 - Thyroid cartilage 3 - Cricoid cartilage 4 - Tracheal cartilages 5 - Balloon cuff A tracheotomy is a procedure performed by paramedics, emergency physicians and surgeons in order to secure an airway. ...


Social workers and home care and hospice nurses help patients, families, and caregivers with the medical, emotional, and financial challenges of coping with ALS, particularly during the final stages of the disease. Social workers provide support such as assistance in obtaining financial aid, arranging durable power of attorney, preparing a living will, and finding support groups for patients and caregivers. Home nurses are available not only to provide medical care but also to teach caregivers about tasks such as maintaining respirators, giving feedings, and moving patients to avoid painful skin problems and contractures. Home hospice nurses work in consultation with physicians to ensure proper medication, pain control, and other care affecting the quality of life of patients who wish to remain at home. The home hospice team can also counsel patients and caregivers about end-of-life issues. A social worker is a person employed in the administration of charity, social service, welfare, and poverty agencies, advocacy, or religious outreach programs. ... Palliative care is any form of medical care or treatment that concentrates on reducing the severity of the symptoms of a disease or slows its progress rather than providing a cure. ... A power of attorney or letter of attorney in common law systems or mandate in civil law systems is an authorization to act on someone elses behalf in a legal or business matter. ... A living will, also called will to live, advance health directive, or advance health care directive, is a specific type of power of attorney or health care proxy or advance directive. ...


Both animal and human research suggest calorie restriction (CR) may be contraindicated for those with ALS. Research on a transgenic mouse model of ALS demonstrates that CR may hasten the onset of death in ALS.[24] In that study, Hamadeh et al also note two human studies[25][26] that they indicate show "low energy intake correlates with death in people with ALS." However, in the first study, Slowie, Paige, and Antel state: "The reduction in energy intake by ALS patients did not correlate with the proximity of death but rather was a consistent aspect of the illness." They go on to conclude: "We conclude that ALS patients have a chronically deficient intake of energy and recommended augmentation of energy intake." (PMID 8604660) See also Negative calorie diet, very low calorie diet CRON redirects here. ... GMO redirects here. ...


Previously, Pedersen and Mattson also found that in the ALS mouse model, CR "accelerates the clinical course" of the disease and had no benefits.[27] Suggesting that a calorically dense diet may slow ALS, a ketogenic diet in the ALS mouse model has been shown to slow the progress of disease.[28]


The new discovery of RNAi has some promise in treating ALS. In recent studies, RNAi has been used in lab rats to shut off specific genes that lead to ALS. Cytrx Corporation has sponsored ALS research utilizing RNAi gene silencing technology targeted at the mutant SOD1 gene.[29] The mutant SOD1 gene is responsible for causing ALS in a subset of the 10% of all ALS patients who suffer from the familial, or genetic, form of the disease. Cytrx's orally-administered drug Arimoclomol is currently in clinical evaluation as a therapeutic treatment for ALS. In molecular biology, RNA interference (RNAi) is a mechanism in which the presence of small fragments of double-stranded RNA (dsRNA) whose sequence matches a given gene interferes with the expression of that gene. ... There are very few or no other articles that link to this one. ...


Insulin-like growth factor 1 has also been studied as treatment for ALS. Cephalon and Chiron conducted two pivotal clinical studies of IGF-1 for ALS, and although one study demonstrated efficacy, the second was equivocal, and the product has never been approved by the FDA. In January of 2007, the Italian Ministry of Health has requested INSMED corporation's drug, IPLEX, which is a recombinant IGF-1 with Binding Protein 3(IGF1BP3) to be used in a clinical trial for ALS patients in Italy. Insulin-like growth factor 1 (IGF-1) is a polypeptide protein hormone similar in molecular structure to insulin. ... IPLEX (mecasermin rinfabate [rDNA origin] injection) is a drug developed by INSMED corporation for the treatment of growth failure in children with severe primary IGF-I deficiency (Primary IGFD) or with growth hormone (GH) gene deletion who have developed neutralizing antibodies to GH. Due to a patent settlement, IPLEX is...


Methylcobalamin is being studied in Japan[30]: preliminary results show it significantly lengthens survival time of ALS patients. Chemical structure of Vitamin B12 The term vitamin B12 (or B12 for short) is used in two different ways. ...


Prognosis

Regardless of the part of the body first affected by the disease, it is usual for muscle weakness and atrophy to spread to other parts of the body as the disease progresses. It is important to remember that some patients with ALS have an arrested course with no progression beyond a certain point despite extensive follow-up. Such a pattern is particularly true for young males with predominant upper limb weakness especially on one side (so-called monomelic or Hirayama type motor neuron disease). Eventually people with ALS will not be able to stand or walk, get in or out of bed on their own, or use their hands and arms. In later stages of the disease, individuals have difficulty breathing as the muscles of the respiratory system weaken. Although ventilation support can ease problems with breathing and prolong survival, it does not affect the progression of ALS. Most people with ALS die from respiratory failure, usually within 3 to 5 years from the onset of symptoms. However, about 10 percent of those individuals with ALS survive for 10 or more years.


Notable people affected by ALS

Notable people living with ALS include:

Notable people who have died of ALS include: Stephen William Hawking, CH, CBE, FRS, FRSA, (born 8 January 1942) is a British theoretical physicist. ... Jason Becker, (born July 22, 1969) is an American neo-classical metal guitarist and composer. ... Orlando Thomas (born October 21, 1972) is a former defensive back who played in the NFL. Thomas played from 1995-2001. ...

This article is about the sport. ... Henry Louis Lou Gehrig (June 19, 1903 â€“ June 2, 1941), born Ludwig Heinrich Gehrig[2], was an American baseball player in the 1920s and 1930s, who set several Major League records and was popularly called the The Iron Horse[2] for his durability. ... This article is about the sport. ... James Augustus Catfish Hunter (April 8, 1946 – September 9, 1999), son of Abbott and Millie Hunter, was a prolific Major League Baseball right-handed starting pitcher between 1965 and 1979. ... Professor Morris Morrie Schwartz, BA, MA, Ph. ... Tuesdays with Morrie is a bestselling non-fiction book by American writer Mitch Albom, published in 1997 (ISBN 0-385-48451-8). ... Michael Joel Zaslow (November 1, 1944 – December 6, 1998) was an American actor. ... Jacob Koppel Jack Javits (May 18, 1904 – March 7, 1986) was a liberal New York politician. ... Charles Mingus (April 22, 1922 – January 5, 1979) was an American jazz bassist, composer, bandleader, and occasional pianist. ... For the film, see Leadbelly (film). ... Lane Smith, full name Walter Lane Smith (April 29, 1936 – June 13, 2005) was a U.S. character actor. ... Roy Lee Walford (June 29, 1924 - April 27, 2004) was an advocate of caloric restriction. ... Henry Agard Wallace (October 7, 1888 – November 18, 1965) was the 33rd Vice President of the United States (1941–45), the 11th Secretary of Agriculture (1933–40), and the 10th Secretary of Commerce (1945–46). ... Dennis Day (May 21, 1918 - June 22, 1988) Irish American singer who appeared for years on Jack Bennys radio and television shows. ... John Drury (January 4, 1927 – November 25, 2007) was a retired anchorman from Chicago, IL. He appeared on both WGN-TV and WLS-TV. Upon his retirement came the news that he was diagnosed with Amyotrophic Lateral Sclerosis, otherwise known as Lou Gehrigs disease. ... Sue Rodriguez (August 2, 1950-February 12, 1994) was an advocate of the right to die with dignity. ... Euthanasia (Greek, good death) is the practice of killing a person or animal, in a painless or minimally painful way, for merciful reasons, usually to end their suffering. ... Leeds United F.C. is the only professional association football club in Leeds. ... For other uses, see England (disambiguation). ... Donald George Revie, OBE, (10 July 1927 - 26 May 1989), was a football player for Leicester City, Hull City, Sunderland, Manchester City and Leeds United as a deep-lying centre forward. ... This article needs additional references or sources for verification. ... A musicologist is someone who studies musicology. ... Hans Keller (1919-1985) was an Austrian-born British musician and writer who made significant contributions to musicology and music criticism, and invented the method of Wordless Functional Analysis (in which a work is analysed in musical sound alone, without any words being heard or read). ... A musicologist is someone who studies musicology. ... Stanley Sadie CBE (October 30, 1930-March 21, 2005) was a British musicologist, music critic, and editor. ... Combatants Republic of Vietnam United States Republic of Korea Thailand Australia New Zealand The Philippines National Front for the Liberation of South Vietnam Democratic Republic of Vietnam People’s Republic of China Democratic Peoples Republic of Korea Strength US 1,000,000 South Korea 300,000 Australia 48,000... Prisoner of War camps Contents // Categories: Substubs | Prisons and detention centres ... Dieter Dengler (May 22, 1938 - February 7, 2001) was a United States Navy pilot during the Vietnam War. ... Rescue Dawn is a 2007 movie starring Christian Bale and Steve Zahn. ... Harry Browne (17 June 1933 – 1 March 2006) was an American libertarian writer, politician, and free-market investment analyst. ...

See also

Muscular Dystrophy Association (MDA) is a U.S. organization founded in 1950 which combats muscular dystrophy and diseases of the nervous system and muscular system in general by funding research, providing medical and community services, and educating health professionals and the general public. ... This article does not cite any references or sources. ... The ALS Therapy Development Institute[1] (ALS TDI) is a nonprofit biotechnology company seeking effective treatments for amyotrophic lateral sclerosis (ALS). ...

References

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  2. ^ ALS Topic Overview. Retrieved on 2008-05-01.
  3. ^ Miller TM, Kaspar BK, Kops GJ, et al. "Virus-delivered small RNA silencing sustains strength in amyotrophic lateral sclerosis.". Ann. Neurol.. 
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  5. ^ S. Kuzuhara, Y. Kokubo P3-146Marked increase of parkinsonism-dementia (P-D) phenotypes in the high incidence amyotrophic lateral sclerosis (ALS) focus in the Kii peninsula of Japan. Alzheimer's and Dementia, Volume 2, Issue 3, Pages S417-S417
  6. ^ Spencer, On the decline and etiology of high-incidence motor system disease in West Papua (southwest New Guinea). Movement disorders (2005) vol.20 Suppl 12 -26
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  8. ^ Rachele MG, Mascia V, Tacconi P, Dessi N, Marrosu F (1998). "Conjugal amyotrophic lateral sclerosis: a report on a couple from Sardinia, Italy.". Ital J Neurol Sci. Apr;19 (2): 97–100. doi:10.1007/BF02427565. PMID : 10935845. 
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  12. ^ Corcia P, Jafari-Schluep HF, Lardillier D, Mazyad H, Giraud P, Clavelou P, Pouget J, Camu W (2003). "A clustering of conjugal amyotrophic lateral sclerosis in southeastern France.". Neurol. Apr;60 (4): 553–7. PMID : 12707069. 
  13. ^ Bains J, et al. (2002). "Isolation of various forms of sterol beta-D-glucoside from the seed of Cycas circinalis: neurotoxicity and implications for ALS-parkinsonism dementia complex".J. Neurochem. 82(3):516-28. PMID: 12153476.
  14. ^ ALS in the Military. The ALS Association (2007-05-17). Retrieved on 2008-05-01.
  15. ^ el Alaoui-Faris M, Medejel A, al Zemmouri K, Yahyaoui M, Chkili T (1990). "Amyotrophic lateral sclerosis syndrome of syphilitic origin. 5 cases.". Rev Neurol (Paris) 146 (1): 41–4. PMID : 2408129. 
  16. ^ Umanekii KG, Dekonenko EP (1983). "Structure of progressive forms of tick-borne encephalitis". Zh Nevropatol Psikhiatr Im S S Korsakova. 83 (8): 1173–9. PMID : 6414202. 
  17. ^ Hansel Y, Ackerl M, Stanek G. (1995). "ALS-like sequelae in chronic neuroborreliosis.". Wien Med Wochenschr. 145 (7-8): 186–8. PMID : 7610670. 
  18. ^ Pasinetti G, Ungar L, Lange D, Yemul S, Deng H, Yuan X, Brown R, Cudkowicz M, Newhall K, Peskind E, Marcus S, Ho L (2006). "Identification of potential CSF biomarkers in ALS". Neurology 66 (8): 1218–22. doi:10.1212/01.wnl.0000203129.82104.07. PMID 16481598. 
  19. ^ Reaume A, Elliott J, Hoffman E, Kowall N, Ferrante R, Siwek D, Wilcox H, Flood D, Beal M, Brown R, Scott R, Snider W (1996). "Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury". Nat Genet 13 (1): 43–7. doi:10.1038/ng0596-43. PMID 8673102. 
  20. ^ Bruijn L, Houseweart M, Kato S, Anderson K, Anderson S, Ohama E, Reaume A, Scott R, Cleveland D (1998). "Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1". Science 281 (5384): 1851–4. doi:10.1126/science.281.5384.1851. PMID 9743498. 
  21. ^ Furukawa Y, Fu R, Deng H, Siddique T, O'Halloran T (2006). "Disulfide cross-linked protein represents a significant fraction of ALS-associated Cu, Zn-superoxide dismutase aggregates in spinal cords of model mice". Proc Natl Acad Sci U S A 103 (18): 7148–53. doi:10.1073/pnas.0602048103. PMID 16636274. 
  22. ^ Boillée S, Vande Velde C, Cleveland D (2006). "ALS: a disease of motor neurons and their nonneuronal neighbors". Neuron 52 (1): 39–59. doi:10.1016/j.neuron.2006.09.018. PMID 17015226. 
  23. ^ Fornai F, Longone P, Cafaro L, et al (2008). "Lithium delays progression of amyotrophic lateral sclerosis". Proc. Natl. Acad. Sci. U.S.A. 105: 2052. doi:10.1073/pnas.0708022105. PMID 18250315. 
  24. ^ Hamadeh MJ, Rodriguez MC, Kaczor JJ, Tarnopolsky MA. Caloric restriction transiently improves motor performance but hastens clinical onset of disease in the Cu/Zn-superoxide dismutase mutant G93A mouse. Muscle Nerve. 2005 Feb;31(2):214-20. PMID 15625688.
  25. ^ Kasarskis EJ, Berryman S, Vanderleest JG, Schneider AR, McClain CJ. Nutritional status of patients with amyotrophic lateral sclerosis: relation to the proximity of death. Am J Clin Nutr. 1996 Jan;63(1):130-7. PMID 8604660.
  26. ^ Slowie LA, Paige MS, Antel JP. Nutritional considerations in the management of patients with amyotrophic lateral sclerosis (ALS). J Am Diet Assoc. 1983 Jul;83(1):44-7. PMID 6863783
  27. ^ Pedersen WA, Mattson MP. No benefit of dietary restriction on disease onset or progression in amyotrophic lateral sclerosis Cu/Zn-superoxide dismutase mutant mice. Brain Res. 1999 Jun 26;833(1):117-20. PMID 10375685.
  28. ^ Zhao Z, Lange DJ , Voustianiouk A, et al. A ketogenic diet as a potential novel therapeutic intervention in amyotrophic lateral sclerosis. BMC Neuroscience 2006, 7:29. (PMID 16584562). Media report on Zhao et al.
  29. ^ Allele-specific RNAi selectively silences mutant S...[Neurobiol Dis. 2006] - PubMed Result
  30. ^ Izumi Y, Kaji R (October 2007). "[Clinical trials of ultra-high-dose methylcobalamin in ALS]" (in Japanese). Brain Nerve 59 (10): 1141–7. PMID 17969354. 

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Further reading

  • ALS Hope Foundation. Retrieved on 2008-06-21. “Dedicated to the care and cure of people with Lou Gehrig's Disease. (from site page us.php)”
  • Lou Gehrig: The Official Web Site. CMG Worldwide. Retrieved on 2008-06-21. “The Official Web site of Lou Gehrig is an informational Web site intended to honor the life, the legend and the career of Lou Gehrig. (from site page siteinfo/index.htm)”
  • Patrick Aebischer; Ann C. Kato. "Playing defense against Lou Gehrig's Disease" (Paper), Scientific American, Verlagsgruppe Georg von Holtzbrinck, November 2007, pp. 86–93. Retrieved on 2008-06-21. "Researchers have proposed potential therapies for a paralyzing disorder once thought to be untreatable (sub-title)" 

This article incorporates in public domain text from The U.S. National Institute of Neurological Disorders and Stroke 2008 (MMVIII) is the current year, a leap year that started on Tuesday of the Common Era (or Anno Domini), in accordance with the Gregorian calendar. ... is the 172nd day of the year (173rd in leap years) in the Gregorian calendar. ... 2008 (MMVIII) is the current year, a leap year that started on Tuesday of the Common Era (or Anno Domini), in accordance with the Gregorian calendar. ... is the 172nd day of the year (173rd in leap years) in the Gregorian calendar. ... Aebischer, centre of the image Patrick Aebischer (1954 - ) is the current president of the EPFL. External links Patrick Aebischer at the EPFL Categories: Scientist stubs | Academic biography stubs ... Scientific American is a popular-science magazine, published (first weekly and later monthly) since August 28, 1845, making it the oldest continuously published magazine in the United States. ... 2008 (MMVIII) is the current year, a leap year that started on Tuesday of the Common Era (or Anno Domini), in accordance with the Gregorian calendar. ... is the 172nd day of the year (173rd in leap years) in the Gregorian calendar. ...

Myoclonus is brief, involuntary twitching of a muscle or a group of muscles. ... The Unverricht-Lundborg Disease (ULD) is the most common type of PME (Progressive Myoclonus Epilepsy). ... Juvenile myoclonic epilepsy (JME), also known as Janz syndrome, is a fairly common form of idiopathic generalized epilepsy, representing 5-10% of all epilepsies. ... A headache (cephalgia in medical terminology) is a condition of pain in the head; sometimes neck or upper back pain may also be interpreted as a headache. ... Familial hemiplegic migraine (FHM) is an autosomal dominant classical migraine subtype that typically includes hemiparesis (weakness of half the body) during the aura phase. ... Cluster headaches are rare, extremely painful and debilitating headaches that occur in groups or clusters. ... A vascular headache is a headache where blood vessel swelling or disturbance is causing the pain. ... Tension headaches, which were renamed tension-type headaches by the International Headache Society in 1988, are the most common type of primary headaches. ... It has been suggested that this article or section be merged with Coronary heart disease. ... A transient ischemic attack (TIA, often colloquially referred to as mini stroke) is caused by the temporary disturbance of blood supply to a restricted area of the brain, resulting in brief neurologic dysfunction that usually persists for less than 24 hours. ... Amaurosis fugax is a type of transient ischaemic attack (TIA). ... Transient global amnesia (TGA), is an anxiety-producing temporary loss of short-term memory. ... Cerebrovascular disease is damage to the blood vessels in the brain, resulting in a stroke. ... Middle cerebral artery syndrome is a condition where the blood supply from the middle cerebral artery is restricted, leading to a reduction of the function of the portions of the brain supplied by that vessel. ... Anterior cerebral artery syndrome is a condition where the blood supply from the anterior cerebral artery is restricted, leading to a reduction of the function of the portions of the brain supplied by that vessel. ... Posterior cerebral artery syndrome is a condition where the blood supply from the posterior cerebral artery is restricted, leading to a reduction of the function of the portions of the brain supplied by that vessel. ... Fovilles syndrome is caused by the blockage of the perforating branches of the basilar artery in the region of the brainstem known as the pons. ... Millard-Gubler syndrome is a syndrome of unilateral softening of the brain tissue arising from obstruction of the blood vessels of the pons, involving the sixth and seventh cranial nerves and fibers of the corticospinal tract, and is associated with paralysis of the abducens (including diplopia, internal strabismus, and loss... Lateral medullary syndrome (also called Wallenbergs syndrome) is a disease in which the patient has difficulty with swallowing or speaking or both owing to one or more patches of dead tissue (known as an infarct) caused by interrupted blood supply to parts of the brain. ... Webers Syndrome (superior alternating hemiplegia) is characterized by the presence of an oculomotor nerve palsy and contralateral hemiparesis or hemiplegia. ... There are very few or no other articles that link to this one. ... This article is about the sleeping disorder. ... Hypersomnia is excessive amount of sleepiness. ... Sleep apnea is a sleep disorder characterized by pauses in breathing during sleep. ... This article needs cleanup. ... Ondines Curse, also called congenital central hypoventilation syndrome (CCHS) or primary alveolar hypoventilation, is a respiratory disorder that is fatal if untreated. ... For other uses, see Narcolepsy (disambiguation). ... Cataplexy is a medical condition which often affects people who have narcolepsy, a disorder whose principal signs are EDS (Excessive Daytime Sleepiness), sleep attacks, sleep paralysis, hypnagogic hallucinations[1] and disturbed night-time sleep. ... Kleine-Levin Syndrome, or KLS, is a rare sleep disorder characterized by episodes of near-constant sleep and altered behavior. ... Circadian rhythm sleep disorders are a family of sleep disorders affecting the timing of sleep. ... Delayed sleep-phase syndrome (DSPS) is a chronic disorder of sleep timing. ... Advanced sleep phase syndrome (ASPS) is a sleep disorder in which patients feel very sleepy early in the evening (e. ... Idiopathic intracranial hypertension (IIH), sometimes called benign intracranial hypertension (BIH) or pseudotumor cerebri (PTC) is a neurological disorder that is characterized by increased intracranial pressure (ICP), in the absence of a tumor or other intracranial pathology. ... Normal pressure hydrocephalus (NPH) is a chronic type of communicating hydrocephalus whereby the increase in intracranial pressure (ICP) due to accumulation of cerebrospinal fluid (CSF) becomes stable and that the formation of CSF equilibrates with absorption. ... Idiopathic intracranial hypertension (IIH), sometimes called benign intracranial hypertension (BIH) or pseudotumor cerebri (PTC) is a neurological disorder that is characterized by increased intracranial pressure (ICP), in the absence of a tumor or other intracranial pathology. ... Encephalopathy literally means disease of the brain. ... Herniation, a deadly side effect of very high intracranial pressure, occurs when the brain shifts across structures within the skull. ... Cerebral edema (cerebral oedema in British English) is an excess accumulation of water in the intra- and/or extracellular spaces of the brain. ... Reyes syndrome is a potentially fatal disease that causes numerous detrimental effects to many organs, especially the brain and liver. ... An uncollapsed syrinx (before surgery). ... Syringobulbia is a medical condition when syrinxes, or fluid filled cavities, affect the brainstem. ... Morvans syndrome (or fibrillary chorea) is a rare disease named after nineteenth century French physician Augustin Marie Morvan (1819-1897). ... Spinal cord compression develops when the spinal cord is compressed by a tumor, abscess or other lesion. ...

  Results from FactBites:
 
Amyotrophic Lateral Sclerosis - MSN Encarta (1054 words)
Amyotrophic Lateral Sclerosis (ALS, sometimes called Maladie de Charcot, or Lou Gehrig's Disease (US)) is a progressive, usually fatal, neurodegenerative disease caused by the...
Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s Disease, is a fatal neurodegenerative disorder that is characterized by the selective loss of motor neurons in...
Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig’s disease, progressive, fatal disease of the motor neurons (nerve cells) that control the skeletal muscles of the body.
AMYOTROPHIC LATERAL SCLEROSIS (ALS) - EDGAR CAYCE Health Database (1535 words)
Amyotrophic lateral sclerosis is defined as a degenerative disease of the pyramidal tract and lower motor neurons, characterized by motor weakness and the spastic condition of the limbs associated with muscular atrophy, fibrillary twitching, and final involvement of nuclei in the medulla.
In the readings, amyotrophic lateral sclerosis is treated much in the same manner as multiple sclerosis, and the readings give a similar cause in both conditions.
This is on multiple sclerosis specifically but, as already mentioned, probably has a strong bearing on the etiology of amyotrophic lateral sclerosis.
  More results at FactBites »

 
 

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