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ALS
Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND) or—in the United States—Lou Gehrig's disease (LGD), is a rare, terminal neurodegenerative disorder that results in the progressive loss of both upper and lower motor neurons that normally control voluntary muscle contraction. ALS is the most common form of the motor neuron diseases. ALS often presents in its early stages with gradual muscle stiffness, twitches, weakness, and wasting. Motor neuron loss typically continues until the abilities to eat, speak, move, and, lastly, breathe are all lost. While only 15% of people with ALS also fully develop frontotemporal dementia, an estimated 50% face at least some minor difficulties with thinking and behavior. Depending on which of the aforementioned symptoms develops first, ALS is classified as ''limb-onset'' (begins with weakness in the arms or legs) or ''bulbar-onset'' (begins with difficulty in speaking or swallowing). Most cases of ALS (abo ...
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Lou Gehrig
Henry Louis Gehrig ( ; June 19, 1903June 2, 1941), also known as Heinrich Ludwig Gehrig, was an American professional baseball first baseman who played 17 seasons in Major League Baseball (MLB) for the New York Yankees (1923–1939). Gehrig was renowned for his prowess as a hitter and for his durability, which earned him the nickname "the Iron Horse", and he is regarded as one of the greatest baseball players of all time. Gehrig was an Major League Baseball All-Star Game, All-Star seven consecutive times, a Triple Crown (baseball), Triple Crown winner once, an American League (AL) Major League Baseball Most Valuable Player Award, Most Valuable Player twice and a member of six World Series List of World Series champions, champion teams. He had a career .340 batting average (baseball), batting average, .632 Slugging percentage, slugging average, and a .447 on-base percentage, on-base average. He hit 493 home runs and had 1,995 run batted in, runs batted in (RBIs). He is also one of ...
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Frontotemporal Dementia
Frontotemporal dementia (FTD), also called frontotemporal degeneration disease or frontotemporal neurocognitive disorder, encompasses several types of dementia involving the progressive degeneration of the brain's frontal lobe, frontal and temporal lobes. Men and women appear to be equally affected. FTD generally presents as a behavioral or language disorder with gradual onset. Signs and symptoms tend to appear in late adulthood, typically between the ages of 45 and 65, although it can affect people younger or older than this. There is currently no cure or approved symptomatic treatment for FTD, although some Off-label use, off-label drugs and behavioral methods are prescribed. Features of FTD were first described by Arnold Pick between 1892 and 1906. The name ''Pick's disease'' was coined in 1922. This term is now reserved only for the behavioral variant of FTD, in which characteristic Pick bodies and Pick cells are present. These were first described by Alois Alzheimer in 1911. ...
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Kennedy's Disease
Spinal and bulbar muscular atrophy (SBMA), popularly known as Kennedy's disease, is a rare, adult-onset, X-linked recessive lower motor neuron disease caused by trinucleotide CAG repeat expansions in exon 1 of the androgen receptor (AR) gene, which results in both loss of AR function and toxic gain of function. In men, the disease slowly progresses over decades with bulbar and lower motor neuron loss, muscle denervation, and direct skeletal muscle involvement.  The disease causes progressive muscle loss with weakness, fasciculations, and cramps. Weakness of the bulbar muscles follows causing difficulties in speech (dysarthria) and swallowing (dysphagia). Female carriers do not show symptoms.  Although there is no cure, supportive intervention can improve mobility and reduce complications. The prevalence of SBMA has been estimated at 2.6:100,000 males. There is no known cure for SBMA. Supportive care is focused on preventing disease complications and maintaining independen ...
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Fasciculation
A fasciculation, or muscle twitch, is a spontaneous, involuntary muscle contraction and relaxation, involving fine muscle fibers. They are common, with as many as 70% of people experiencing them. They can be benign, or associated with more serious conditions. When no cause or pathology is identified, they are diagnosed as benign fasciculation syndrome. Diagnosis The most effective way to detect fasciculations may be surface electromyography (EMG). Surface EMG is more sensitive than needle electromyography and clinical observation in the detection of fasciculation in people with amyotrophic lateral sclerosis (ALS). Deeper areas of contraction can be detected by electromyography (EMG) testing, though they can happen in any skeletal muscle in the body. Fasciculations arise as a result of spontaneous depolarization of a lower motor neuron leading to the synchronous contraction of all the skeletal muscle fibers within a single motor unit. An example of normal spontaneous depolari ...
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Multifocal Motor Neuropathy
Multifocal motor neuropathy (MMN) is a progressively worsening condition where muscles in the extremities gradually weaken. The disorder, a pure motor neuropathy syndrome, is sometimes mistaken for amyotrophic lateral sclerosis (ALS) because of the similarity in the clinical picture, especially if muscle fasciculations are present. MMN is thought to be autoimmune. It was first described in the mid-1980s. Unlike ALS, which affects both upper and lower motor neuron pathways, MMN involves only the lower motor neuron pathway, specifically, the peripheral nerves emanating from the lower motor neurons. Definitive diagnosis is often difficult, and many MMN patients labor for months or years under an ALS diagnosis before finally getting a determination of MMN. MMN usually involves very little pain; however, muscle cramps, spasms and twitches can cause pain for some people. MMN is not fatal, and does not diminish life expectancy. Many patients, once undergoing treatment, only experience ...
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Post-polio Syndrome
Post-polio syndrome (PPS, poliomyelitis sequelae) is a group of latent symptoms of poliomyelitis (polio), occurring in more than 80% of polio infections. The symptoms are caused by the damaging effects of the viral infection on the nervous system and typically occur 15 to 30 years after an initial acute paralytic attack. Symptoms include decreasing muscular function or acute weakness with pain and fatigue. The same may also occur years after a nonparalytic polio infection. The precise mechanism that causes PPS is unknown. It shares many features with chronic fatigue syndrome, but unlike that disorder it tends to be progressive and can cause loss of muscle strength. Treatment is primarily limited to adequate rest, conservation of available energy, and supportive measures, such as leg braces and energy-saving devices such as powered wheelchairs, analgesia (pain relief), and sleep aids. Signs and symptoms After a period of prolonged stability, some people who recover from pol ...
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Respiratory Failure
Respiratory failure results from inadequate gas exchange by the respiratory system, meaning that the arterial oxygen, carbon dioxide, or both cannot be kept at normal levels. A drop in the oxygen carried in the blood is known as hypoxemia; a rise in arterial carbon dioxide levels is called hypercapnia. Respiratory failure is classified as either Type 1 or Type 2, based on whether there is a high carbon dioxide level, and can be acute or chronic. In clinical trials, the definition of respiratory failure usually includes increased respiratory rate, abnormal blood gases (hypoxemia, hypercapnia, or both), and evidence of increased work of breathing. Respiratory failure causes an altered state of consciousness due to ischemia in the brain. The typical partial pressure reference values are oxygen Pa more than 80 mmHg (11 kPa) and carbon dioxide Pa less than 45 mmHg (6.0 kPa). Cause A variety of conditions that can potentially result in respiratory failure. The etiologie ...
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Neuroimaging
Neuroimaging is the use of quantitative (computational) techniques to study the neuroanatomy, structure and function of the central nervous system, developed as an objective way of scientifically studying the healthy human brain in a non-invasive manner. Increasingly it is also being used for quantitative research studies of brain disease and psychiatric illness. Neuroimaging is highly multidisciplinary involving neuroscience, computer science, psychology and statistics, and is not a medical specialty. Neuroimaging is sometimes confused with neuroradiology. Neuroradiology is a medical specialty that uses non-statistical brain imaging in a clinical setting, practiced by radiologists who are medical practitioners. Neuroradiology primarily focuses on recognizing brain lesions, such as vascular diseases, strokes, tumors, and inflammatory diseases. In contrast to neuroimaging, neuroradiology is qualitative (based on subjective impressions and extensive clinical training) but sometime ...
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Respiratory Failure
Respiratory failure results from inadequate gas exchange by the respiratory system, meaning that the arterial oxygen, carbon dioxide, or both cannot be kept at normal levels. A drop in the oxygen carried in the blood is known as hypoxemia; a rise in arterial carbon dioxide levels is called hypercapnia. Respiratory failure is classified as either Type 1 or Type 2, based on whether there is a high carbon dioxide level, and can be acute or chronic. In clinical trials, the definition of respiratory failure usually includes increased respiratory rate, abnormal blood gases (hypoxemia, hypercapnia, or both), and evidence of increased work of breathing. Respiratory failure causes an altered state of consciousness due to ischemia in the brain. The typical partial pressure reference values are oxygen Pa more than 80 mmHg (11 kPa) and carbon dioxide Pa less than 45 mmHg (6.0 kPa). Cause A variety of conditions that can potentially result in respiratory failure. The etiologie ...
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Feeding Tubes
A feeding tube is a medical device used to provide nutrition to people who cannot obtain nutrition by mouth, are unable to swallow safely, or need nutritional supplementation. The state of being fed by a feeding tube is called gavage, enteral feeding or tube feeding. Placement may be temporary for the treatment of acute conditions or lifelong in the case of chronic disabilities. A variety of feeding tubes are used in medical practice. They are usually made of polyurethane or silicone. The outer diameter of a feeding tube is measured in French units (each French unit equals  mm). They are classified by the site of insertion and intended use. Medical uses There are dozens of conditions that may require tube feeding (enteral nutrition) to prevent or treat malnutrition. Conditions that necessitate feeding tubes include prematurity, failure to thrive (or malnutrition), neurologic and neuromuscular disorders, inability to swallow, anatomical and post-surgical malformations o ...
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Skeletal Muscles
Skeletal muscle (commonly referred to as muscle) is one of the three types of vertebrate muscle tissue, the others being cardiac muscle and smooth muscle. They are part of the somatic nervous system, voluntary muscular system and typically are attached by tendons to bones of a skeleton. The skeletal muscle cells are much longer than in the other types of muscle tissue, and are also known as ''muscle fibers''. The tissue of a skeletal muscle is striated muscle tissue, striated – having a striped appearance due to the arrangement of the sarcomeres. A skeletal muscle contains multiple muscle fascicle, fascicles – bundles of muscle fibers. Each individual fiber and each muscle is surrounded by a type of connective tissue layer of fascia. Muscle fibers are formed from the cell fusion, fusion of developmental myoblasts in a process known as myogenesis resulting in long multinucleated cells. In these cells, the cell nucleus, nuclei, termed ''myonuclei'', are located along the inside ...
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Dysarthria
Dysarthria is a speech sound disorder resulting from neurological injury of the motor component of the motor–speech system and is characterized by poor articulation of phonemes. It is a condition in which problems effectively occur with the muscles that help produce speech, often making it very difficult to pronounce words. It is unrelated to problems with understanding language (that is, dysphasia or aphasia), although a person can have both. Any of the speech subsystems ( respiration, phonation, resonance, prosody, and articulation) can be affected, leading to impairments in intelligibility, audibility, naturalness, and efficiency of vocal communication. Dysarthria that has progressed to a total loss of speech is referred to as anarthria. The term ''dysarthria'' was formed from the Greek components ''dys-'' "dysfunctional, impaired" and ''arthr-'' "joint, vocal articulation". Neurological injury due to damage in the central or peripheral nervous system may result in wea ...
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