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When does Oculopharyngeal muscular dystrophy usually begin?
GaleEncyofMedicine
usually begins in a person's thirties or forties, with weakness in the muscles controlling the eyes and throat. Symptoms include drooping eyelids, difficulty swallowing (dysphagia), and weakness progresses to other muscles of the face, neck
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∙ 13y ago
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Why is Duchenne muscular dystrophy also known as pseudohypertrophic muscular dystrophy?
calves will begin to swell, though with fibrous tissue rather than with muscle, and feel firm and rubbery; this condition gives DMD one of its alternate names, pseudohypertrophic muscular dystrophy
How is Muscular Dystrophy tested for?
In the 1860s, descriptions of boys who grew progressively weaker, lost the ability to walk, and died at an early age became more prominent in medical journals. In the following ten years, French neurologist Guillaume Duchenne gave a comprehensive account of 13 boys with the most common and severe form of the disease (which now carries his name - Duchenne muscular dystrophy). It soon became evident that the disease had more than one form, and that these diseases affected males of all ages. Nine diseases including Duchenne, Becker, limb girdle, congenital, facioscapulohumeral, myotonic, oculopharyngeal, distal, and Emery-Dreifuss are always classified as muscular dystrophy.
Why is breathing affected by muscular dystrophy?
First of all, it matters what type of MD you have, but generally since the disease weakens your muscles to the diaphragm, intercostal muscles and other muscles involved in breathing begin to die, and make breathing harder and harder.
When do symptoms of Facioscapulohumeral muscular dystrophy first occur?
most commonly begin in the teens or early twenties, though infant or childhood onset is possible. Symptoms tend to be more severe in those with earlier onset. The disease is named for the regions of the body most severely affected
Becker muscular dystrophy?
DefinitionBecker muscular dystrophy is an inherited disorder that involves slowly worsening muscle weakness of the legs and pelvis.Alternative NamesBenign pseudohypertrophic muscular dystrophy; Becker's dystrophyCauses, incidence, and risk factorsBecker muscular dystrophy is very similar to Duchenne muscular dystrophy, except that it gets worse at a much slower rate.The disorder is passed down through families (inherited). Having a family history of the condition raises your risk.Becker muscular dystrophy occurs in approximately 3 - 6 out of every 100,000 males. It is less common than Duchenne muscular dystrophy.SymptomsFemales rarely develop symptoms. Males will develop symptoms if they inherit the defective gene. Symptoms usually appear in boys at about age 12, but may begin later.Muscle weakness of the lower body, including the legs and pelvis area, slowly gets worse, causing:Difficulty walking that gets worse over time; by age 25-30 the person is usually unable to walkFrequent fallsDifficulty with running, hopping, and jumpingLoss of muscle massMuscle weakness in the arms, neck, and other areas is not as severe as in the lower body.Other symptoms may include:Breathing problemsCognitive problems (these do not get worse over time)FatigueLoss of balance and coordinationSigns and testsThe health care provider will do a nervous system (neurological) and muscle examination. A careful medical history is also important, because the pattern of symptom development resembles that of Duchenne's muscular dystrophy. However, Becker muscular dystrophy gets worse much more slowly.An exam may find:Abnormally developed bones, leading to deformities of the chest and back (scoliosis)Abnormality of heart muscle function (cardiomyopathy)Congestive heart failure or irregular heartbeat (arrhythmias) - rareMuscle deformities, including: Contractures of heels and legsAbnormal fat and connective tissue in calf musclesMuscle loss that begins in the legs and pelvis, then moves on to the muscles of the shoulders, neck, arms, and respiratory systemTests that may be done include:CPK blood testElectromyography (EMG) nerve testingMuscle biopsyor genetic blood testTreatmentThere is no known cure for Becker muscular dystrophy. The goal of treatment is to control symptoms to maximize the person's quality of life. Some doctors prescribe steroids to help keep a patient walking for as long as possible.Activity is encouraged. Inactivity (such as bed rest) can make the muscle disease worse. Physical therapy may be helpful to maintain muscle strength. Orthopedic appliances such as braces and wheelchairs may improve mobility and self-care.Genetic counseling may be recommended. Daughters of a man with Becker muscular dystrophy may carry the defective gene and could pass it onto their sons.Support GroupsYou can ease the stress of illness by joining a support group where members share common experiences and problems.See: Muscular dystrophy - support groupExpectations (prognosis)Becker muscular dystrophy leads to slowly worsening disability, although the degree of disability varies. Some men may need a wheelchair, while others may only need to use walking aids such as canes or braces.ComplicationsHeart-related complications such as cardiomyopathyLung failurePneumonia or other respiratoryinfectionsIncreasing and permanent disability, that leads to: Decreased ability to care for selfDecreased mobilityCalling your health care providerCall your health care provider if:Symptoms of Becker muscular dystrophy appearA person with Becker muscular dystrophy develops new symptoms (particularly feverwith cough or breathing difficulties)You are planning to start a family and you or other family members have been diagnosed with Becker muscular dystrophyPreventionGenetic counseling may be advised if there is a family history of Becker muscular dystrophy.ReferencesKliegman RM, Behrman RE, Jenson HB, Stanton BF. Muscular dystrophies. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 608.
What symptoms of congential muscular dystrophy begin in infancy?
marked by severe muscle weakness from birth, with infants displaying "floppiness" and very little voluntary movement. Nonetheless, a child with CMD may learn to walk, either with or without some assistive device, and live into young adulthood or beyond
What disease can be caused by a mutation in the dystrophin gene?
What Is Muscular Dystrophy?Muscular dystrophy (MD) is a genetic disorder that weakens the muscles that help the body move. People with MD have incorrect or missing information in their genes, which prevents them from making the proteins they need for healthy muscles. Because MD is genetic, people are born with the problem - it's not contagious and you can't catch it from someone who has it. MD weakens muscles over time, so children, teens, and adults who have the disease can gradually lose the ability to do the things most people take for granted, like walking or sitting up. Someone with MD might start having muscle problems as a baby or their symptoms might start later. Some people even develop MD as adults.Several major forms of muscular dystrophy can affect teens, each of which weakens different muscle groups in various ways:Duchenne (pronounced: due-shen) muscular dystrophy (DMD), the most common type of the disease, is caused by a problem with the gene that makes a protein called dystrophin. This protein helps muscle cells keep their shape and strength. Without it, muscles break down and a person gradually becomes weaker. DMD affects boys. Symptoms usually start between ages 2 and 6. By age 10 or 12, kids with DMD often need to use a wheelchair. The heart may also be affected, and people with DMD need to be followed closely by a lung and heart specialist. They can also develop scoliosis (curvature of the spine) and tightness in their joints. Over time, even the muscles that control breathing get weaker, and a person might need a ventilator to breathe.Becker muscular dystrophy (BMD), like DMD, affects boys. The disease is very similar to DMD, but its symptoms may start later and can be less severe. With BMD, symptoms like muscle breakdown and weakness sometimes don't begin until age 10 or even in adulthood. People with BMD can also have breathing, heart, bone, muscle, and joint problems. Many people with BMD can live long, active lives without using a wheelchair.Emery-Dreifuss (pronounced: em-uh-ree dry-fuss) muscular dystrophy (EDMD) typically starts causing symptoms in late childhood to early teens and sometimes as late as age 25. EDMD is another form of muscular dystrophy that affects mostly boys. It involves muscles in the shoulders, upper arms, and shins, and it often causes joint problems (joints can become tighter in people with EDMD). The heart muscle may also be affected.Limb-girdle muscular dystrophy (LGMD) affects boys and girls equally, weakening muscles in the shoulders and upper arms and around the hips and thighs. LGMD can begin as early as childhood or as late as mid-adulthood, and it often progresses slowly. Over time, a wheelchair might be necessary to get around. There are many different types of LGMD, each with its own specific features.Facioscapulohumeral (pronounced: fa-she-o-skap-you-lo-hyoo-meh-rul) muscular dystrophy (FSHD) can affect both guys and girls, and it usually begins during the teens or early adulthood. FSHD affects muscles in the face and shoulders and sometimes causes weakness in the lower legs. People with this type of MD might have trouble raising their arms, whistling, or tightly closing their eyes. How much a person with this form of muscular dystrophy is affected by the condition varies from person to person. It can be quite mild in some people.Myotonic (pronounced: my-uh-tah-nick) dystrophy (MMD) is a form of muscular dystrophy in which the muscles have difficulty relaxing. In teens, it can cause a number of problems, including muscle weakness and wasting (where the muscles shrink over time), cataracts, and heart problems.Congenital muscular dystrophy (CMD) is the term for all types of MD that show signs in babies and young children, although the MD isn't always diagnosed right away. Like other forms of MD, CMD involves muscle weakness and poor muscle tone. Occurring in both girls and boys, it can have different symptoms. It varies in how severely it affects people and how quickly or slowly it worsens. In rare cases, CMD can cause learning or intellectual disabilities.The life expectancy (in other words, how long a person may live) for many of these forms of muscular dystrophy depends on the degree to which a person's muscles are weakened as well as how much the heart and lungs are affected
What is the difference between muscle tone and atrophy?
While they often can be used to indicate the same thing, strictly speaking, "atrophy" means a loss of function due to disuse. "Dystrophy" is more general, meaning "other than normal". The distinction usually comes down to this: something that was originally normal will waste away via atrophy, while something that was never normal to begin with may suffer dystrophy.
What did the mother say the Stirrings usually begin with?
The mother said that the Stirrings usually begin with a slight twitching or trembling of the body.
How do all laws begin?
The laws made by Congress usually begin in Committees.
What process is in muscular atrophy?
Muscular atrophy is when the muscles in the body begin to break down on a cellular level due to lack of oxygen, disease, or simply lack of excercise. Don't confuse it with necrosis. Necrosis is complete cell death. Atrophy is just a loss of muscular integrity (i.e. getting fat and losing strength).
Do all emails have to begin with www?
Emails usually do not begin with www. Website URLs do.
