Muscular dystrophy refers to a group of more than 30 inherited (genetic) diseases that cause muscle weakness.

What is muscular dystrophy?

Muscular dystrophy refers to a group of more than 30 inherited (genetic) diseases that cause muscle weakness. These conditions are a type of myopathy, a disease of the skeletal muscles. Over time, muscles shrink and become weaker, affecting your ability to walk and perform daily activities like brushing your teeth. The disease also can affect your heart and lungs.

Some forms of muscular dystrophy are apparent at birth or develop during childhood. Some forms develop later during adulthood. Currently, there isn’t a cure.

Types of muscular dystrophy

There are many different types of MD, each with somewhat different symptoms. Not all types cause severe disability and many don’t affect life expectancy.

Some of the more common types of MD include:

Duchenne MD – one of the most common and severe forms, it usually affects boys in early childhood; people with the condition will usually only live into their 20s or 30s

Myotonic Dystrophy – a type of MD that can develop at any age; life expectancy isn’t always affected, but people with a severe form of myotonic dystrophy may have shortened lives

Facioscapulohumeral MD – a type of MD that can develop in childhood or adulthood; it progresses slowly and isn’t usually life-threatening

Becker MD – closely related to Duchenne MD, but it develops later in childhood and is less severe; life expectancy isn’t usually affected as much

Limb-Girdle MD – a group of conditions that usually develop in late childhood or early adulthood; some variants can progress quickly and be life-threatening, whereas others develop slowly

Oculopharyngeal MD – a type of MD that doesn’t usually develop until a person is between 50 and 60 years old, and doesn’t tend to affect life expectancy

Emery-Dreifuss Md – a type of MD that develops in childhood or early adulthood; most people with this condition will live until at least middle age

Pathophysiology

Multiple proteins are involved in the complex interactions of the muscle membrane and extracellular environment. For sarcolemmal stability, dystrophin and the dystrophin-associated glycoproteins (DAGs) are important elements.

The dystrophin gene is located on the short arm of chromosome X near the p21 locus and codes for the large protein Dp427, which contains 3685 amino acids. Dystrophin accounts for only approximately 0.002% of the proteins in striated muscle, but it has obvious importance in the maintenance of the muscle’s membrane integrity.

Dystrophin aggregates as a homotetramer at the costomeres in skeletal muscles, as well as associates with actin at its N-terminus and the DAG complex at the C-terminus, forming a stable complex that interacts with laminin in the extracellular matrix. Lack of dystrophin leads to cellular instability at these links, with progressive leakage of intracellular components; this results in the high levels of creatine phosphokinase (CPK) noted on routine blood workup of patients with Duchenne MD.

Less active forms of dystrophin may still function as a sarcolemmal anchor, but they may not be as effective a gateway regulator because they allow some leakage of intracellular substance. This is the classic Becker dystrophy. In both Duchenne and Becker MD, the muscle-cell unit gradually dies, and macrophages invade. Although the damage in MD is not reported to be immunologically mediated, class I human leukocyte antigens (HLAs) are found on the membrane of dystrophic muscles; this feature makes these muscles more susceptible to T-cell mediated attacks.

Selective monoclonal antibody hybridization was used to identify cytotoxic T cells as the invading macrophages; complement-activated membrane attack complexes have been identified in dystrophic muscles as well. Over time, the dead muscle shell is replaced by a fibrofatty infiltrate, which clinically appears as pseudohypertrophy of the muscle. The lack of functioning muscle units causes weakness and, eventually, contractures.

Other types of MDs are caused by alterations in the coding of one of the DAG complex proteins. The gene loci coding for each of the DAG complex proteins is located outside the X chromosomes. Gene defects in these protein products also lead to alterations in cellular permeability; however, because of the slightly different mechanism of action and because of the locations of these gene products within the body, there are other associated effects, such as those in ocular and limb-girdle type dystrophies (see the image below).

Causes

Genetic changes cause MD, and each type is due to a different set of mutations. However, all the mutations prevent the body from producing dystrophin, a protein essential for building and repairing muscles.

Although dystrophin makes up a small percent of the total proteins in muscles, it is an essential molecule for their normal function. It glues various parts of muscle tissue together and links them to the sarcolemma, or the outer membrane.

If dystrophin is absent or deformed, this process does not work correctly. This weakens the muscles and can damage the muscle cells.

In DMD, dystrophin is almost entirely absent. Conversely, in BMD, dystrophin is smaller or in short supply.

Risk factors

Muscular dystrophy occurs in both sexes and in all ages and races. However, the most common variety, Duchenne, usually occurs in young boys. People with a family history of muscular dystrophy are at higher risk of developing the disease or passing it on to their children.

What are the symptoms of muscular dystrophy?

Muscle weakness is the primary symptom of muscular dystrophy. Depending on the type, the disease affects different muscles and parts of the body. Other signs of muscular dystrophy include:

Enlarged calf muscles.

Difficulty walking or running.

Unusual walking gait (like waddling).

Trouble swallowing.

Heart problems, such as arrhythmia and heart failure (cardiomyopathy).

Learning disabilities.

Stiff or loose joints.

Muscle pain.

Curved spine (scoliosis).

Breathing problems.

Complications

The complications of progressive muscle weakness include:

Trouble walking. Some people with muscular dystrophy eventually need to use a wheelchair.

Trouble using arms. Daily activities can become more difficult if the muscles of the arms and shoulders are affected.

Shortening of muscles or tendons around joints (contractures). Contractures can further limit mobility.

Breathing problems. Progressive weakness can affect the muscles associated with breathing. People with muscular dystrophy might eventually need to use a breathing assistance device (ventilator), initially at night but possibly also during the day.

Curved spine (scoliosis). Weakened muscles might be unable to hold the spine straight.

Heart problems. Muscular dystrophy can reduce the efficiency of the heart muscle.

Swallowing problems. If the muscles involved with swallowing are affected, nutritional problems and aspiration pneumonia can develop. Feeding tubes might be an option.

Muscular dystrophy diagnosis

A number of tests can help your doctor diagnose muscular dystrophy. Your doctor can perform:

Blood testing. High levels of serum creatine kinase, serum aldolase, and myoglobin may all signal the need for further testing to confirm or rule out muscular dystrophy.

Genetic testing. High levels of creatine kinase and signs of insufficient dystrophin may indicate a need for genetic testing. This type of testing looks for a large mutation of the dystrophin (DMD) gene. If there’s no large mutation, the next set of genetic tests will look for small mutations.

Electromyography (EMG). EMG measures the muscle’s electrical activity using an electrode needle that enters your muscle. It can help doctors to distinguish muscular dystrophy from a nerve disorder.

Neurological physical exam. This exam rules out nervous system disorders and identifies the state of muscle strength and reflexes.

Cardiac testing. Cardiac testing identifies heart problems that sometimes occur with muscular dystrophy. Tests include an echocardiogram to look at the structure of the heart.

Imaging tests. MRI and ultrasound help doctors see the amount of muscle inside the body.

Exercise assessments. Exercise assessments look at muscle strength, breathing, and how exercise affects the body.

Treatment

Right now, there’s no cure for the disease. But there are many treatments that can improve symptoms and make life easier for you and your child.

Your doctor will recommend a treatment based on the type of muscular dystrophy your child has. Some of them are:

Physical therapyuses different exercises and stretches to keep muscles strong and flexible.

Occupational therapy teaches your child how to make the most of what their muscles can do. Therapists can also show them how to use wheelchairs, braces, and other devices that can help them with daily life.

Speech therapy will teach them easier ways to talk if their throat or face muscles are weak.

Respiratory therapy can help if your child is having trouble breathing. They’ll learn ways to make it easier to breathe, or get machines to help.

Medicines can help ease symptoms. They include:

Eteplirsen (Exondys 51), golodirsen (Vyondys53), and vitolarsen (Viltepso) for treating DMD. They are injection medications that help treat individuals with a specific mutation of the gene that leads to DMD, specifically by increasing dystrophin production. Talk to your child’s doctor about possible side effects.

Anti-seizure drugs that reduce muscle spasms.

Blood pressure medicines thathelp with heart problems.

Drugs that turn down the body’s immune systemcalled immunosuppressants; they may slow damage to muscle cells.

Steroids like prednisone and defkazacort (Emflaza) that slow down muscle damage and can help your child breathe better. They can cause serious side effects, such as weak bones and a higher risk of infections.

Creatine, a chemical normally found in the body that can help supply energy to muscles and improve strength for some people. Ask your child’s doctor if these supplements are a good idea for them.

Surgery can help with different complications of muscular dystrophy, like heart problems or trouble swallowing.

Scientists also are looking for new ways to treat muscular dystrophy in clinical trials. These trials test new drugs to see if they are safe and if they work. They often are a way for people to try new medicine that isn’t available to everyone. Your doctor can tell you if one of these trials might be a good fit for your child.

Taking Care of Your Child

It’s hard when your child loses strength and can’t do the things other kids can do. Muscular dystrophy is a challenge, but it doesn’t have to keep your child from enjoying life.

There are many things you can do to help them feel stronger and get the most out of life.

Eat right. A healthy, well-balanced diet is good for your child in general. It’s also important for helping them stay at a healthy weight, which can ease breathing problems and other symptoms. If it’s hard for them to chew or swallow, talk to a dietitian about foods that may be easier to eat.

Stay active.Exercise can improve your child’s muscle strength and make them feel better. Try low-impact activities like swimming.

Get enough sleep. Ask your doctor or therapist about certain beds or pads that can make your child more comfortable and rested.

Use the right tools. Wheelchairs, crutches, or electric scooters can help your child if they have trouble walking.

The disease will most likely have a big impact on your family. Remember that it’s OK to ask a doctor, counselor, family, or friends for help with any stress, sadness, or anger you may feel. Support groups are also good places to talk to other people who have lived with muscular dystrophy. They can help your child connect with others like them and give you and your family advice and understanding.

How can I prevent muscular dystrophy?

Unfortunately, there isn’t anything you can do to prevent getting muscular dystrophy. If you have the disease, these steps can help you enjoy a better quality of life:

Eat a healthy diet to prevent malnutrition.

Drink lots of water to avoid dehydration and constipation.

Exercise as much as possible.

Maintain a healthy weight to prevent obesity.

Quit smoking to protect your lungs and heart.

Get flu and pneumonia vaccines.

Vasovagal syncope is a condition that leads to fainting in some people.

Definition

Vasovagal syncope is a condition that leads to fainting in some people. It is also called neurocardiogenic syncope or reflex syncope. It’s the most common cause of fainting. It’s usually not harmful nor a sign of a more serious problem.

Many nerves connect with your heart and blood vessels. These nerves help control the speed and force of your heartbeat. They also regulate blood pressure by controlling whether your blood vessels widen or tighten.

Usually, these nerves coordinate their actions so you always get enough blood to your brain. Under certain situations, these nerves might give an inappropriate signal. This might cause your blood vessels to open wide. At the same time, your heartbeat may slow down. Blood can pool in your legs which leads to a drop in blood pressure, and not enough of it may reach the brain. If that happens, you may briefly lose consciousness. When you lie or fall down, blood flow to the brain resumes.

Vasovagal syncope is quite common. It most often affects children and young adults, but it can happen at any age. It happens to men and women in about equal numbers. Unlike some other causes of fainting, vasovagal syncope does not signal an underlying problem with the heart or brain.

Prevalence of Vasovagal syncope

The prevalence of syncope differs based on the clinical setting and the age of the patient. It is estimated that 3% of men and 3.5% of women experience syncope during their lifetime. Syncope has been estimated to account for 1% to 3% of emergency department visits and 1% to 6% of hospital admissions.

The prevalence of syncope increases with age and is estimated to be 0.7% in patients age 35 to 44 compared with 4% to 6% in patients 75 and older. In the Framingham study, the incidence of syncope increased after the age of 70, from 5.7 per 1,000 events person-years in men aged 60 to 69 to 11.1 per 1,000 events person-years in men 70 to 79.

Vasovagal syncope risk factors

Though neurocardiogenic syncope is very common and many people have one or more episodes in their lifetimes with no identifiable cause some risk factors that could contribute to neurocardiogenic syncope include:

Alcohol or (prescription or illegal) drug use: In some cases, alcohol or drugs can precipitate a fainting episode, especially if they cause dehydration, low blood sugar or low blood pressure.

Anxiety or panic disorders: Because syncope can be triggered by intense emotional stress, the heightened levels associated with these conditions could increase the risk of neurocardiogenic syncope.

Diabetes: Episodes of low blood sugar typically from skipping meals can bring on neurocardiogenic syncope. People with diabetes may experience these low blood sugar episodes more frequently, even when they eat regular meals.

Illnesses that affect your autonomic nervous system, such as Parkinson’s disease: Orthostatic hypotension, a sharp drop in blood pressure that happens when a person gets up from a bed or chair, can cause dizziness or fainting. This symptom is common in Parkinson’s and some other conditions.

Causes of Vasovagal syncope

Many triggers can cause vasovagal syncope, such as:

Standing for long periods

Too much heat

Intense emotion, such as fear

Intense pain

The sight of blood or a needle

Exercising for a long time

Older adults may have additional triggers, such as:

Urinating

Swallowing

Coughing

Having a bowel movement

Symptoms

Before a person faints, there are a number of warning signs. These are known as prodromal symptoms, and they usually occur within a few minutes of loss of consciousness. These include:

Blurry vision

Cold, clammy sweat

Feeling of warmth

Lightheadedness

Narrowing vision, or tunnel vision

Nausea

Pale skin

Uncontrollable yawning

If you notice any of these symptoms, you may have time to take steps to prevent injury, such as sitting down or moving away from furniture that could cause injury when you fall.

People who faint usually regain consciousness within a few seconds, since lying down improves blood pressure almost immediately. However, they should not stand up too soon since being active can lead to another episode.

Complications of Vasovagal syncope

Vasovagal syncope itself is generally not dangerous. Of course, fainting can be dangerous if it happens at certain times, like while driving.

Most people with rare episodes of vasovagal syncope can drive safely. If you have chronic syncope that is not under control, your doctor may advise against driving.

This is especially likely if you don’t usually have warning signs before you faint. Ask your doctor about what is safe for you to do.

Diagnosis and test

Diagnosing vasovagal syncope often begins with a physical examination. During the physical exam, your doctor will listen to your heart and take your blood pressure. He or she may also massage the main arteries in your neck to see if that causes you to feel faint.

Your doctor may also recommend several tests to rule out other possible causes of your fainting particularly heart-related problems. These tests may include:

Electrocardiogram: This test records the electrical signals your heart produces. It can detect irregular heart rhythms and other cardiac problems. You may need to wear a portable monitor for at least a day or as long as a month.

Echocardiogram: This test uses ultrasound imaging to view the heart and look for conditions, such as valve problems, that can cause fainting.

Exercise stress test: This test studies heart rhythms during exercise. It’s usually conducted while you walk or jog on a treadmill.

Blood tests: Your doctor may look for conditions, such as anemia, that can cause or contribute to fainting spells.

Tilt table test. If no heart problems appear to cause your fainting, your doctor may suggest that you undergo a tilt table test. During the test, you lie flat on your back on a table that changes positions, tilting you upward at various angles. A technician monitors your heart rhythms and blood pressure during the test to see if changing your posture affects them.

Treatment and medications

In most cases of vasovagal syncope, treatment is unnecessary. Your doctor may help you identify your fainting triggers and discuss ways you might avoid them.

However, if you experience vasovagal syncope often enough to interfere with your quality of life, your doctor may suggest trying one or more of the following remedies:

Medications: A drug called fludrocortisone acetate that’s normally used to treat low blood pressure may be helpful in preventing vasovagal syncope.

In some people, vasovagal syncope occurs with disturbing frequency even when all appropriate precautions are taken. For these individuals, drug therapy is often helpful.

Drugs that have been shown to be of some help include:

Midodrine, a vasoconstricting drug that dilates blood vessels

Norpace (disopyramide), an antiarrhythmic drug

Serotonin re-uptake inhibitors

Theophylline, typically used to treat asthma

While one or more of these drugs will often help reduce episodes of syncope, finding the “right” combination of drugs is usually a matter of trial and error. Patience is required on the part of both the doctor and patient in order to find the best therapy.

Therapies: Your doctor may recommend ways to decrease the pooling of blood in your legs. These may include foot exercises, wearing compression stockings or tensing your leg muscles when standing. You may need to increase salt in your diet if you don’t usually have high blood pressure. Avoid prolonged standing especially in hot, crowded places and drink plenty of fluids.

Surgery: Very rarely, inserting an electrical pacemaker to regulate the heartbeat may help some people with vasovagal syncope who haven’t been helped by other treatments.

Prevention of Vasovagal syncope

It may not be possible to completely prevent vasovagal syncope, but you may be able to cut down on how often you faint.

The most important step is to try and determine your triggers.

Do you tend to faint when you have your blood drawn, or when you watch scary movies? Or have you noticed that you feel faint when you’re overly anxious, or have been standing for a long time?

If you’re able to find a pattern, try to take steps to avoid or work around your triggers.

When you start to feel faint, immediately lie down or sit in a safe spot if you can. It could help you avoid fainting, or at least prevent injury due to a fall.

So sad as combined team of Custom officers and army vandalized a civilian vehicle in Lagos State.

According to Homepage news channel, it was learnt that a man simply identified as Baba Abe, was brutaly assaulted and his car vandalized by the combined team of the Nigeria military and Custom officers along Abgara/ Okokomaikao express road last night.

According to a local source, it was learnt that his Toyota Camry was also vandalized at the Custom check point after Agbara bust stop.

During further investigation by our sources it was disclosed that the reason of this evil act.

Was because of an accident which was caused by an okada rider who ran into their vehicle while they were returning from church program very close to the Custom check point.

The Custom men got angry over the incident as they said the incident was obstructing the free flow of vehicles along that assis.

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Manchester United woefully lost 0-4 against Brentford.

A shambolic Manchester United side conceded four goals in the opening 35 minutes in a 4-0 drubbing at Brentford on Saturday (Aug 13) as their woeful start to the Premier League season continued.

United’s seventh successive away league defeat was assured long before the interval as Brentford took ruthless advantage of a catalogue of errors by the visitors.

The rout began in the 10th minute when United keeper David de Gea allowed a weak shot by Josh Dasilva to slip past him.

Things got worse for United eight minutes later when De Gea played the ball out to former Brentford player Christian Eriksen who was caught in possession and Mathias Jensen slotted home.

When United’s defence failed to deal with a corner and Ben Mee glanced in a close-range header to make it 3-0 the Brentford fans were ecstatic while United’s new manager Erik ten Hag looked ashen-faced in his technical area.

Brentford’s fourth was a gem as Ivan Toney delivered a diagonal ball to Bryan Mbeumo from a counter-attack and Mbeumo calmly beat De Gea.

Ten Hag made three substitutions at halftime with Raphael Varane, Tyrell Malacia and Scott McTominay coming on but despite a slight improvement United offered little fight.

Brentford’s fans serenaded their players with “Hey Jude” at the final whistle while United’s players looked crestfallen as they trudged off rock bottom of the table having also lost their opener at home to Brighton & Hove Albion.

It was the first time since 1960 that United have conceded at least six goals in their first two matches of a top-flight season and the first time they have lost their opening two matches since 1992 when they went on to win the title.

This side, however, looks woefully inadequate to mount any kind of challenge and Ten Hag, who was taunted by the Brentford fans, appears to have a huge job on his hands.

The Dutchman is the first Manchester United manager to lose his first two games in charge since John Chapman in 1921.

It’s easy to dismantle this United side, just be organised and fight and you’re there,” said former United player and Sky Sports pundit Gary Neville said.

For Brentford, whose starting line-up cost in the region of 55 million compared to the more than 400 million of United’s, have picked up four points in their first two games.

It was just hard work. We knew the high press would affect them. It was clear to see that if you work hard, you get the result, Toney told Sky Sports.

We know they have great talent in there and have some players who can change the game. When you work like we did today and run all over them, you get a result like we did.

United has Cristiano Ronaldo back in their starting line-up but the Portuguese forward barely got a look in.

The confidence drained out of United from the moment De Gea allowed Dasilva’s shot to end up in the back of the net.

Eriksen, who got a mixed reception from Brentford’s fans, then inadvertently gifted his former club their second as United looked shellshocked by the energy of their hosts who went on to record their biggest top-flight victory since 1938.

Things will not get any easier for United whose next game is against Liverpool.

It was a horrible day, De Gea said. I should have saved the first shot and then the result may have been different.

Other teams concede one goal and win 5-1. We have to react much better. It was a poor performance.

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Two Ukrainian grain ship depart to Turkiye through Black Sea ports.

Two more ships carrying grain left from Ukraine’s Black Sea ports on Saturday (Aug 13), Turkiye defence ministry said, bringing the total number of vessels to depart the country under a UN-brokered deal to 16.

The Barbados-flagged Fulmar S left Ukraine’s Chornomorsk port, carrying 12,000 tonnes of corn to Turkiye southern Iskenderun province, the ministry said. The Marshall Island-flagged Thoe departed from the same port and headed to Turkiye Tekirdag, carrying 3,000 tonnes of sunflower seeds.

Another ship was to depart from Turkey on Saturday to Ukraine to buy grains, the statement said.

Ukraine’s infrastructure ministry said on Saturday that 16 ships carrying 450,000 tonnes of agriculture products had departed from Ukrainian sea ports since early August under the deal which ensured safe passage for vessels.

The agreement, signed by Ukraine, Russia, Turkey and the United Nations in July amid warnings of possible outbreaks of famine, allowed grain exports from Ukraine’s Black Sea ports to resume after being stalled for five months due to the war.

President Volodymyr Zelenskiy said that in less than two weeks, Ukraine had managed to export the same amount of grain from three ports as it had done by road for the whole of July.

This has already made it possible to reduce the severity of the food crisis, he said in a video address on Saturday.

Ukraine hopes to increase its maritime exports to over 3 million tonnes of grain and other agriculture products per month in near future.

Ukraine and Russia are major grains exporters, and the blockage of Ukrainian ports has trapped tens of millions of grain in the country, raising fears of severe food shortages and even outbreaks of famine in parts of the world.

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616 PDP members decamp to APC in Gombe state.

No fewer than 616 members of the Peoples Democratic Party (PDP), in Dukku Local Government Area of Gombe State on Saturday defected to the All Progressives Congress (APC).

An ex-chieftain of PDP, Alhaji Kwairanga Dukku, led the new entrants, including women and youths leaders from different wards within the local government area into the APC.

Dukku attributed their decision to the good and purpose-driven leadership style of Gov. Inuwa Yahaya.

Also speaking, Alhaji Bappah Maru, a former member of the PDP Elders’ Forum said the efforts of the present administration in the state in the provision of potable water in the area convinced him to join the APC.

Umaru said their decision to join the APC was to appreciate the governor’s people-oriented policies towards improving the well being of the people.

Welcoming the new members, Gov. Inuwa Yahaya said the APC-led state government had entrenched good governance.

Represented by the Commissioner for Finance and Economic Development, Alhaji Muhammad Magaji, Yahaya said the large number of people joining APC showed there was no hope for the opposition parties in the state in 2023.

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Bandits release 35 kidnapped victims after 3 weeks of abduction in Kaduna state.

Thirty-five persons kidnapped at Keke ‘B’ Millennium City in Chikun local government area of Kaduna State, have regained their freedom.

The victims, kidnapped about three weeks ago from their houses by bandits, were released by their abductors on Friday night after a payment of N2.5 million as ransom.

Meanwhile, one of the kidnap victims is still in captivity because his family was unable to meet the bandits’ demand of two motorcycles for his release.

Similarly, another kidnapped Doctor and 15 others, including women and children from Sabon Gero community also in Chikun local government area of the State, have also regained their freedom.

The leader of the Civilian JTF in the community, Uwasu Yunusa, confirmed the development to journalists in Kaduna, adding that no ransom was paid for their release.

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Devotees defy Osun state government warning, drink from contaminated river.

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The real reason the Northern Nigeria hate the Igbos, revealed in this exclusive interview.

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