What is the physiology of muscular dystrophy?
The term "muscular dystrophy" incorporates an assortment of hereditary disorders that lead to progressive, generalized disease of the muscle prompted by inadequate or missing glycoproteins in the muscle cell plasma membrane. [1] Muscular dystrophy is a non-communicable disorder with abundant variations. [2]
What are the types and symptoms of muscular dystrophy (MD)?
The symptoms include:
- weakness in your upper arm and lower leg muscles
- breathing problems
- heart problems
- shortening of the muscles in your spine, neck, ankles, knees, and elbows
What is the prognosis of Distal muscular dystrophy?
Patients usually present with myopathy of distal muscles, cardiomyopathy, difficulty in swallowing, and difficulty in speaking. There is no cure for distal muscular dystrophy but physical and occupational therapies may be helpful to manage the symptoms.
What triggers myasthenia gravis?
- Extreme tiredness or fatigue
- A medical condition or a disease
- Anxiety, stress and depression
- Certain medications like beta blockers, quinine, some types of anesthetics and antibiotics (2)
Is muscular dystrophy related to myasthenia gravis?
Further evidence for this hypothesis is afforded by occasional reports of patients with myasthenia gravis who have features suggestive of muscular dystrophy. 6-10 It is possible that this association is fortuitous, since myasthenia may occur concomitantly with many seemingly unrelated disorders.
What type of disease is myasthenia gravis?
Myasthenia gravis is caused by a problem with the signals sent between the nerves and the muscles. It's an autoimmune condition, which means it's the result of the immune system (the body's natural defence against infection) mistakenly attacking a healthy part of the body.
What are 3 types of muscular dystrophy?
Types of Muscular DystrophyDuchenne Muscular Dystrophy. ... Becker Muscular Dystrophy. ... Congenital Muscular Dystrophy. ... Myotonic Muscular Dystrophy. ... Limb-Girdle Muscular Dystrophy. ... Facioscapulohumeral Muscular Dystrophy. ... Emery–Dreifuss Muscular Dystrophy. ... Distal Muscular Dystrophy.More items...
What is myasthenia dystrophy?
What is myasthenia gravis? In myasthenia gravis, muscle weakness often first appears in the muscles of the face, neck and jaw. The arm and leg muscles are affected later. Myasthenia gravis (MG) is an autoimmune disease — a disease that occurs when the immune system attacks the body's own tissues.
What is the underlying cause of myasthenia gravis?
Myasthenia gravis is caused by an error in the transmission of nerve impulses to muscles. It occurs when normal communication between the nerve and muscle is interrupted at the neuromuscular junction—the place where nerve cells connect with the muscles they control.
What can trigger myasthenia gravis?
Myasthenia crisis may be caused by a lack of medicine or by other factors, such as a respiratory infection, emotional stress, surgery, or some other type of stress. In severe crisis, a person may have to be placed on a ventilator to help with breathing until muscle strength returns with treatment.
What diseases are similar to muscular dystrophy?
The diseases most frequently mistaken for muscular dystrophy were polymyositis and the syndrome of "benign hypotonia." Polymyositis, with its protean manifestations and variable course, may mimic all of the forms of muscular dystrophy so closely that differentiation becomes especially difficult.
What are the signs of muscular dystrophy in adults?
General Muscular Dystrophy SymptomsMuscle weakness.Difficulty walking.Frequent falling.Difficulty getting up from a lying or sitting position.Limited movement at certain joints (called contracture)Heart problems.Problems with breathing and swallowing.Muscle pain or stiffness.
What is the rarest form of muscular dystrophy?
Duchenne muscular dystrophy (DMD) is a rare muscle disorder but it is one of the most frequent genetic conditions affecting approximately 1 in 3,500 male births worldwide.
What is the most common muscular dystrophy?
Duchenne MD (DMD) DMD is the most common and severe form of MD among children, and it accounts for approximately half of MD cases. DMD occurs mostly in boys, usually between 3 and 5 years of age, and progresses rapidly. Most people with DMD are unable to walk by age 12 and may eventually need a respirator to breathe.
How many types of muscular dystrophy are there?
There are 9 types of muscular dystrophy, with each type involving an eventual loss of strength, increasing disability, and possible deformity. The most well known of the muscular dystrophies is Duchenne muscular dystrophy (DMD), followed by Becker muscular dystrophy (BMD).
What is the life expectancy of someone with myasthenia gravis?
There is no cure for MG, but most people with the condition have a normal life span. Only 3 to 4 out of every 100 people with MG die because of MG. Years ago, early death occurred in over a third of people with MG. Today, if someone dies of MG, death is usually due to a myasthenic crisis or a thymoma.
Is myasthenia gravis a neurological disorder?
General Discussion. Myasthenia gravis is a neuromuscular disorder primarily characterized by muscle weakness and muscle fatigue. Although the disorder usually becomes apparent during adulthood, symptom onset may occur at any age.
Is myasthenia gravis a progressive disease?
In most people with myasthenia gravis, muscle weakness is temporary and reversible, and tends to wax and wane over time. Because the disorder doesn't lead to progressive loss of function or paralysis, the description progressive disease does not really apply. Rather, it's a disease of fluctuating weakness.
How long can you live with myasthenia gravis?
The age specific mortality rates were low below 50 years. After this age the mortality increased with age in both sexes; after 60 years more rapidly in men than in women. The overall survival rates three, five, 10, and 20 years from diagnosis were 85%, 81%, 69%, and 63% respectively.
Who is most likely to get myasthenia gravis?
Though this disease can affect people of any age, it's more common in women younger than 40 and in men older than 60.
What is the Difference Between Muscular Dystrophy and Myasthenia Gravis?
Myasthenia gravis is an autoimmune disorder characterized by the production of antibodies that block the transmission of impulses across the neuromuscular junction.
What are the limitations of muscular dystrophy?
In myasthenia gravis and muscular dystrophy, there are such limitations to the body’s ability to move. A progressive loss of muscle mass and the resultant loss of muscle strength are the hallmark features of muscular dystrophy. On the other hand, myasthenia gravis is an autoimmune disorder characterized by the production of antibodies that block the transmission of impulses across the neuromuscular junction. The key difference between the two disorders is, in myasthenia gravis the problem is at the level of neuromuscular junctions but in muscular dystrophy, the lesion is in the muscle.
Where is muscular dystrophy defect?
In muscular dystrophy, the defect is in the muscles but in myasthenia gravis, the defect is at the neuromuscular junction. This is the difference between the two disorders.
What are the clinical features of shortening of muscles and tendons?
Shortening of muscles and tendons. Clinical features are, · There is weakness of proximal limb muscles, extraocular muscles ,and bulbar muscles. · There are fatigability and fluctuation with regard to the muscle weakness. · No muscle pain. · Heart is not affected but the respiratory muscles can be affected.
Which muscles are weak?
There is weakness of proximal limb muscles, extraocular muscles, and bulbar muscles
Why do we do muscle biopsies?
Usually, muscle biopsies are done for the purpose of identifying the pathological variant of the muscular dystrophy.
What is the weakness of MG?
Weakness and fatigue in MG tend to fluctuate from day to day, and even during a single day. People with the disease are often strongest in the morning after a full night’s sleep and weakest in the evening. Exercise may also affect the weakness and fatigue experienced by those living with MG.
Where does weakness spread in MG?
In generalized MG, weakness tends to spread sequentially from the face and neck to the upper limbs, the hands, and then the lower limbs. It may become difficult to lift the arms over the head, rise from a sitting position, walk long distances, climb stairs, or grip heavy objects.
What is bulbar weakness?
About 15% of patients present with bulbar symptoms. Bulbar weakness tends to give speech a slurred, nasal quality. It also can lead to frequent choking spells and make eating unpleasant and tiresome. Limb weakness alone is highly uncommon and can be seen in only 5% of MG patients.
How long does it take for MG to progress?
Over a longer term, the symptoms of MG usually progress, reaching maximum or near-maximum severity within one to three years of onset in most people. In about 15% of people, the disease remains ocular, but in most it becomes oculobulbar or generalized.
Is juvenile MG a congenital myasthenic syndrome?
Compared to adult-onset MG, juvenile MG tends to progress more slowly and has a higher incidence of remission. Historically, many children given diagnoses of juvenile MG turned out to have a congenital myasthenic syndrome.
Does myasthenia gravis damage the heart?
Myasthenia gravis (MG) weakens and fatigues the body’s voluntary muscles (those we can move at will). It does not damage the musculature of the heart or the gastrointestinal tract.
What are the complications of myasthenia gravis?
Complications of myasthenia gravis. One of the most dangerous potential complications of MG is myasthenic crisis. This consists of life-threatening muscle weakness that can include breathing problems. Talk with your doctor about your risks.
How to diagnose muscle weakness?
making certain your eyes move properly. testing sensation in different areas of your body. testing motor functions, like touching your finger to your nose. Other tests that can help your doctor diagnose the condition include: repetitive nerve stimulation test.
What is the condition that causes weakness in the skeletal muscle?
Myasthenia gravis (MG) is a neuromuscular disorder that causes weakness in the skeletal muscles, which are the muscles your body uses for movement. It occurs when communication between nerve cells and muscles becomes impaired. This impairment prevents crucial muscle contractions from occurring, resulting in muscle weakness.
What is MG in medical terms?
MG is a neuromuscular disorder that’s usually caused by an autoimmune problem. Autoimmune disorders occur when your immune system mistakenly attacks healthy tissue. In this condition, antibodies, which are proteins that normally attack foreign, harmful substances in the body, attack the neuromuscular junction.
How rare is MG?
It’s a relatively rare condition that affects between 14 and 20 out of every 100,000 people in the United States .
What test is used to test for MG?
repetitive nerve stimulation test. blood testing for antibodies associated with MG. edrophonium ( Tensilon) test: a drug called Tensilon (or a placebo) is administered intravenously, and you’re asked to perform muscle movements under doctor observation. imaging of the chest using CT scans or MRI to rule out a tumor.
Why does muscle contracting fail?
The failure of muscles to contract normally occurs because they can’t respond to nerve impulses. Without proper transmission of the impulse, the communication between nerve and muscle is blocked and weakness results. Weakness associated with MG typically gets worse with more activity and improves with rest.
Who is at risk of developing myasthenia gravis?
Young patients are more commonly women, whereas older patients, over the age of 50, are more often men. People who inherit a tendency to develop an autoimmune condition are at increased risk of developing myasthenia gravis, so a patient with myasthenia gravis may have – or have a relative with – another autoimmune condition such as diabetes or thyroid disease. Occasionally, myasthenia gravis develops in people with rheumatoid arthritis who are given the drug penicillamine. In these cases, the myasthenia gravis symptoms usually disappear when the drug is stopped.
What is MG in medical terms?
Myasthenia gravis (sometimes abbreviated to MG) is a chronic, autoimmune condition that causes muscle weakness and excessive muscle fatigue. It is rare, affecting about 15 in every 100,000 people in the UK.
Can myasthenia gravis be relapsed?
The condition can vary in severity and distribution of weakness between individuals, and in an individual, the symptoms can fluctuate with relapses and remissions. Myasthenia gravis can resolve spontaneously, but for most people the condition persists for life. It can be life-threatening, but with modern treatments, 90 percent become symptom-free. Myasthenia gravis presents in two main forms, ocular myasthenia gravis and generalised myasthenia gravis.
Is myasthenia gravis inherited?
Myasthenia gravis is not an inherited condition and does not usually occur in families. This is in contrast to the congenital myasthenic syndromes that are genetic conditions (see below). However, it is thought that a person’s genetic make-up is one of perhaps many factors that lead them to develop myasthenia gravis, and it may occasionally be found in more than one family member.