Muscular Dystrophy
Muscular dystrophy is a group of more than 30 inherited (genetic) diseases where muscle weakness is main cause of the disease. These conditions are a type of myopathy, a disease of the skeletal muscles. As time passes by, muscles shrink and become weaker, affecting your ability to walk and perform daily activities like brushing your teeth. Heart and lungs also can be affected from this.
Some forms of muscular dystrophy are recognizable at birth or develop during childhood. Some forms develop later during adulthood. Muscular dystrophy does not have any cure for now.
Types & Symptoms –
There are 30 types of muscular dystrophy, which vary in symptoms and severity.
Some important once are described as follows –
▪︎ Duchenne muscular dystrophy (DMD)
Duchenne muscular dystrophy (DMD) mainly affects children. The majority of individuals affected are children are males as comparison with females at birth.
The symptoms include –
• trouble walking
• loss of reflexes
• difficulty standing up
• poor posture
• bone thinning
• scoliosis, an abnormal curvature of the spine
• learning differences
• breathing difficulties
• swallowing problems
• lung and heart weakness
People with DMD typically have to start using a wheelchair before their teenage years.
▪︎ Becker muscular dystrophy (BMD)
Becker muscular dystrophy (BMD) is similar to DMD, except it’s severity range. This type is also more common in male children than females.
Muscle weakness occurs mostly in the arms and legs, with symptoms appearing between the ages of 11 and 25 years.
Other symptoms of BMD include –
• walking on the toes
• frequent falls
• muscle cramps
• trouble getting up from the floor
Many with this condition won’t choose to use a wheelchair until they’re in their mid-30s or older, and a small percentage of people never require one. Most people with BMD live until middle age or later.
▪︎ Congenital muscular dystrophy
Congenital muscular dystrophies are often recognizable between birth and 2 years old when parents begin to notice that their child’s motor functions and muscle control aren’t developing as they should.
Symptoms range from mild to severe. Symptoms vary and may include:
• muscle weakness
• poor motor control
• inability to sit or stand without support
• scoliosis
• foot deformities
• trouble swallowing
• respiratory issues
• vision problems
• speech problems
• learning differences
▪︎ Myotonic dystrophy
This form causes myotonia, which is an inability to relax your muscles after they contract. Myotonic dystrophy is also called Steinert’s disease or dystrophia myotonica.
Myotonic dystrophy can affect your:
• facial muscles
• central nervous system (CNS)
• adrenal glands
• heart
• thyroid
• eyes
• gastrointestinal tract
Symptoms most often appear first in the face and neck which includes –
• drooping muscles in the face, producing a thin, drawn look
• difficulty lifting the neck due to weak neck muscles
• difficulty swallowing
• droopy eyelids, or ptosis
• early baldness in the front area of the scalp
• poor vision, including cataracts
• weight loss
• increased sweating
It affects all sexes equally.This dystrophy type may also cause impotence and testicular atrophy in males. In females, it may cause irregular periods and infertility.
▪︎ Facioscapulohumeral muscular dystrophy (FSHD)
Facioscapulohumeral muscular dystrophy (FSHD) affects the muscles in the face, shoulders, and upper arms. This type of muscular dystrophy is also known as Landouzy-Dejerine disease.
FSHD may symptoms include –
• difficulty chewing or swallowing
• slanted shoulders
• a crooked appearance of the mouth
• a wing-like appearance of the shoulder blades
A smaller number of people with FSHD may develop hearing and respiratory problems. FSHD tends to progress slowly. Symptoms usually appear during teenage years, but they sometimes don’t appear until a person is in their 40s.
▪︎ Limb-girdle muscular dystrophy (LGMD)
Limb-girdle muscular dystrophy (LGMD) refers to a group of more than 20 inherited conditions. They cause weakening of the muscles and a loss of muscle bulk. The symptoms usually begin in the shoulders and hips but may also occur in the legs and neck.
The patient may find it hard to get up out of a chair, use stairs, and carry heavy items. They may also stumble and fall more easily.
▪︎ Oculopharyngeal muscular dystrophy (OPMD)
Oculopharyngeal muscular dystrophy (OPMD) causes weakness in your facial, neck, and shoulder muscles. Other symptoms include:
• drooping eyelids
• vision problems
• trouble swallowing
• voice changes
• heart problems
• difficulty walking
Individuals usually begin to experience symptoms in their 40s or 50s.
▪︎ Distal muscular dystrophy
Distal muscular dystrophy is also called distal myopathy. It’s a group of over six diseases affecting the muscles specifically the –
• forearms
• hands
• calves
• feet
The condition may also affect your respiratory system and heart muscles. The symptoms tend to progress slowly and include a loss of fine motor skills and difficulty walking.
Most people develop symptoms of distal muscular dystrophy between the ages of 40 and 60 years old.
▪︎ Emery-Dreifuss muscular dystrophy (EDMD)
Emery-Dreifuss muscular dystrophy (EDMD) usually begins in childhood and tends to affect male children than females at birth. The symptoms include –
• weakness in the upper arm and lower leg muscles
• breathing problems
• heart problems
• shortening of the muscles in the spine, neck, ankles, knees, and elbows
The most common causes of limited life expectancy are progressive heart or lung issues.
Causes –
Genes contain the information your cells need to make proteins that control all of the different functions in the body. When a gene has a problem, your cells can make the wrong protein, the wrong amount of it, or a damaged protein.
Children could get muscular dystrophy even if neither of the parents had the disease. This happens when one of child genes gets a defect on its own. But it’s rare for someone to get it this way.
In people with muscular dystrophy, the broken genes are the ones that make the proteins that keep muscles healthy and strong. For example, those with Duchenne or Becker muscular dystrophies make too little of a protein called dystrophin, which strengthens muscles and protects them from injury.
Risk factors –
• This disease can occurs in males as well as in females 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.
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.
Diagnosis –
A number of tests can help your health provider to diagnose muscular dystrophy.
• Blood testing.
High levels of serum creatine kinase, serum aldolase, and myoglobin may indicate 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 health provider to differentiate between 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 health providers to evaluate the amount of muscle inside the body.
• Exercise assessments.
Exercise assessments also helps evaluating muscle strength, breathing, and how exercise affects the body.
Treatment –
Researchers are still looking for a way to cure muscular dystrophy as there is no sure cure for the disease. It’s symptoms get worse as the time passes by, but these treatments can help the diseasesd to live somehow-
• Physical and occupational therapies helps in strengthen and stretch muscles. These therapies can help you maintain function and range of motion.
• Speech therapy helps those where swallowing is the major issue.
• Corticosteroids, such as prednisone and deflazacort, play their role to slow disease progression.
• Surgery relieves tension on contracted muscles and corrects spine curvature (scoliosis).
• Heart assist devices, such as pacemakers, make a way to treat heart rhythm problems and heart failure.
• Medical devices, such as walkers and wheelchairs, can improve mobility and prevent falls.
• Respiratory care, such as cough-assist devices and respirators, helps the patient in breathing.
Ayurvedic Perspective –
Muscular dystrophy is described as the “Mamsashosha” in Ayurveda. According to Ayurveda, muscular dystrophy is due to imbalance of vata dosha and sapta dhatus (basic elements to maintain body structure). Sapta dhatus involve rasa, rakta, mamsa, meda, asthi, majja and shukra which builds our body, each sapta dhatu has its own digestive fire (Dhatwagni). Vitiation in digestive fire of any of these dhatu leads to improper absorption of nutrients which result in muscle weakness.
In Ayurveda for the management of muscular dystrophy, concept of the paraspar dhatu paka is of prime importance whereas Acharyas have mentioned specific chikitsa sootra for the condition.
In Ayurveda texts, while explaining the dhatupaka avastha clearly signifies the importance of Agni which is responsible for the formation of the next dhatus. Thus correction of agni should be done by administration of deepana and pachana dravyas in order to strengthen the process. Doshas must be balanced and metabolic toxins must be eliminated from dhatus through panchakarma.
The treatment protocol for muscular dystrophy in Ayurveda is as follows –
• Abhyanga
It is a nourishing massage to the body with warm herbal oil. The massage helps reduce muscle stiffness in the lower back and the whole body increases muscle movement and increases energy. The oil supplements the loss of fat tissues and adds to elasticity.
• Navarakizhi
It is also known as Shashtika Shali Pinda Sweda which includes sweat-inducing massage with medicated herbs and special medicated rice in combination with milk. This further reduces muscle stiffness and increases muscle energy.
• Panchakarma Vasti
Vasti is the enema therapy that is given through the rectum which helps reduce constipation, backache, joint pains. It also washes away toxins from our digestive systems.
• Rasayana
It is a combination of medicinal herbs, specific diets, tonics in specific dosages to heal the damaged muscle tissues to support the healing of the body.
Beneficial Herbs –
• Ashwagandha
• Guduchi
• Bala
• Musli
• Vidarikand
• Akarkara
• Gokshura
• Triphala
• Guggulu
• Arjuna
Importance of Yoga –
Yoga has also shown to improve a muscular dystrophy patient’s digestion and aid the elimination of toxins. In muscular dystrophy, yoga can help to cope up with symptoms and improve the quality of life. Regular practice of yoga can improve blood circulation and strengthens the weakening muscles.
▪︎ Useful Yogasanas
• Surya Namaskar
• Bhujangasana
• Gomukhasana
• Padhastasana
• Pawanmuktasana
• Parvatasana
• Paschimottanasana
• Padmasana
• Virbhadrasana
• Virasana
▪︎ Useful Pranayama
• Nadi Shodhana Pranayama
• Bhramari Pranayama
• Anuloma – Vilom Pranayama
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