Author: Dr. Vikram Chauhan

ABSTRACT

Limb Girdle muscular dystrophy is a group of diseases that causes weakness and wasting of the muscles commonly seen in arms and legs. Muscular System is an organ system consisting of skeletal, smooth and cardiac muscles. Muscular System permits the various movements, maintains the posture and circulates the blood throughout the body. There are 3 distinct types of muscles such as skeletal muscle, cardiac muscle, non striated muscle. Usually muscles provide strength, balance and heat to keep the body warm. There are 650 muscles in the human body, and made up of elastic tissue and small muscle fibers, each muscle fiber consists of tiny strands called fibrils which control the contraction of muscle fiber.

A tendon is a piece of connective tissue that connects muscle to a bone and when muscle is contracted it is pulled against the skeleton and creates a movement. The cells of cardiac muscles are X and Y shaped which are tightly connected by a special junction known as intercalated disk, they are finger-like projections. In this topic we are going to discuss its causes, symptoms and its ayurvedic management in detail further in this topic.

INTRODUCTION

Limb Girdle muscular dystrophy is a genetically heterogeneous group of muscular disease which is characterized by progessive wasting mainly affects hip and shoulder joints. It usually has an autosomal pattern of inheritance. In muscular dystrophy there are abnormal genes that interfere with the production of proteins which are needed to form a healthy muscle. This weakness and damage is due to lack of the protein known as dystrophin and deficiency of this protein causes problems in walking, swallowing, and in coordination in muscles.

It commonly affects all the sexes, the most common type is Duchenne and Becker dystrophy which is common in men. In the early stages limb girdle muscular dystrophy there is unusual Gait and makes running difficult. Muscle wasting may change in posture and in appearance of shoulder, arm, back. Weak shoulders tend to make shoulder blade sticks out and make scapular wings. In rare cases developmental delay and disability are seen in this disorder.

FACTS ABOUT LIMB GIRDLE MUSCULAR DYSTROPHY

1. In limb girdle muscular dystrophy is progessive as in this weakness continually worsen up
2. Limb girdle muscular dystrophy are neuromuscular disorders which are inherited.
3. The muscles which are commonly affected are close to the body.
4. Limb girdle muscular dystrophy affects the heart and muscles which are necessary for the breathing
5. It is an inherited disease and it can occur in all parts of the world.
6. There are 30 different types of limb girdle muscular dystrophy which are caused by mutations in different genes.
7. It is annually celebrated worldwide on 30th september.
8. Limb Girdle Dystrophy primarily affects voluntary muscles.
9. Individuals with limb girdle muscular dystrophy develop an abnormal curvature in the spine.
10. Elevated creatine kinase levels is the first sign of the limb girdle muscular dystrophy.  

CAUSES

1. Limb Girdle muscular dystrophy is caused by mutations in different genes.
2. It is based on the inheritance pattern where there are 2 types of pattern present : type 1 is autosomal dominant, and type 2 is autosomal recessive.
3. Calpainopathy is type 2 which causes mutation in the CAPN3 gene.
4. Dysferlinopathy is a limb girdle which is type 2B caused by mutation of the DYSF gene.

SYMPTOMS

The main sign of muscular dystrophy is progessive muscle weakness

Duchenne type of Muscular Dystrophy

It is the most common form which affects males more than females.

1. Waddling gait
2. Delayed growth
3. Muscle pain and stiffness
4. Trouble in jumping
5. Large calf muscles
6. Difficulty in sitting and raising

1. Becker muscular dystrophy

It is usually seen in teenagers and is milder and progresses slowly.

• Scoliosis
• Heart problems such as arrhythmia and cardiomyopathy
• Learning disabilities

DIAGNOSIS

1. Enzyme test
2. Genetic testing
3. Muscle biopsy
4. Electrocardiography and echocardiography
5. Lung monitoring test
6. Electromyography
7. MRI
8. Ultrasound

COMPLICATION

1. Trouble Walking
2. Trouble using arms
3. Contractures (shortening of muscles and tendons around joints)
4. Breathing problems
5. Scoliosis
6. Heart problems
7. Swallowing problems

TREATMENT

There is no cure for muscular dystrophy but in some diseases the extent of time can be increased which further provides strength to muscles and lung so therapies can be provided to provide relief to the patients.

MEDICATIONS USED ARE

1. Corticosteroids which help muscle strength and delay the progression of this disease.
2. Heart medications such as ACE inhibitors or beta blockers
3. For Duchenne dystrophy Golodirsen is used for the treatment of this disease.

VARIOUS THERAPY

• Range of motion and stretching exercise
• Low impact aerobic exercise
• Braces slow down the progression of contractures.
• Breathing assistance helps to improve oxygen level when respiratory muscles weaken.
• Mobility Aids- helps to maintain mobility and independence.

AYURVEDIC MANAGEMENT

Bone Tissue is the 5th tissue which is formed from its previous tissue that is meda dhatu or fat tissue. When it acts upon normal tissue it forms the part of fat tissue and body hair, nails are formed as human excreta. When it is contaminated by doshas or ama it leads to many diseases known as asthi pradoshaja rogas. Asthi Pradoshaja rogas causes asthi bheda, asthi toda and many diseases. In ayurveda it is related to ashraya ashraya relationship as vata dosha resides in tissue and they tend to contaminate each other and when it gets contaminated it causes bone morbidity

When vata doshas increase the bone tissue decreases in quality and quantity. The main factor of hereditary disease is due to defected Beeja (ovum and sperm) and beejbhaga (chromosome) which is responsible for the defective or vitiated part of the body or organ. All the structures such as heart, spleen and muscles are derived from mother and known as matrija bhava. Movements everywhere in the body are carried by vata dosha, this vata dosha occupies the empty channels and affects the entire part of the body. When this vata moves to majja asthi gata vata it causes spitting type of pain in hands and joints, decrease in muscle strength and constant pain in the body in bones and bone marrow. In this condition mamsa dhatu agni gets increase which leads to muscle destruction and improper absor

SYMPTOMS

1. Mental retardation
2. Eyelid drooling
3. Difficulty in walking and standing
4. Delay in motor skills
5. Development issues

TREATMENT

In Ayurveda there are many effective remedies to improve the growth and quality of life, the treatment plans include

1. Panchkarma therapy
2. Abhyangam
3. Kashaya basti
4. Anuvasna basti
5. Til pinda swedana

These treatment therapy removes the toxins from the body and balances the body to provide strength and flexibility. Yoga and meditation helps to improve blood circulation to all the parts of joints. Balanced diet is also pacifying the aggravated doshas and balances the body’s vitals and the hormone system.

HERBS USEFUL FOR LIMB GIRDLE MUSCULAR DYSTROPHY

1. Ashwagandha

Ashwagandha (Withania Somnifera) is a powerful herb for immunity, muscle strength, bodybuilding and is antioxidant. It helps to relieve tissue depletion hence promotes strength. Ashwagandha boosts up immunity and increases the ability of the body to fight against viruses, and is used in respiratory tract infections. It is used in cognitive impairment disorder and bipolar disease.

2. Guduchi

It is known as Giloy which boosts up the immunity level and improves blood circulation. Helps in joint disorders and maintain metabolic health. Guduchi (Tinospora cordifolia) balances tridoshas and promotes digestion, and has a rejuvenative property. Giloy usually relieves burning sensation in the body and is used in the treatment of jaundice and liver diseases.

3. Gokshura

Gokshura (Tribulus terrestris) is used in increasing libido and increases testosterone level. This testosterone level increases muscle building in the body and strengthens the body. Gikshura has lithotriptic properties which are used to break down the urinary stones. Usually it promotes sperm production and ovulation and has anti-inflammatory properties and is used in anti obesity treatment.

4. Guggul

It acts as a natural blood cleanser, used as an inflammatory disease and removes toxins from the body. It helps to relieve muscle cramps and provides inflammatory disease of muscle. It helps to relieve joint inflammation and decreases joint inflammation, it strengthens the bones and has a good effect on the musculoskeletal system. Guggul has an anti-aging property.

HERBAL REMEDIES BY PLANET AYURVEDA FOR LIMB GIRDLE MUSCULAR DYSTROPHY

Planet Ayurveda is an ayurvedic clinic which has been serving humanity for the last 20 years and provides great treatment with great results. All the formulations prepared by planet ayurveda are made under strict guidance of M.D experts. Thes herbal remedies are free from any colors, resins, and preservatives. They are 100% pure medications made from plant extract. In this formulation no animal product is used and provides great benefits to patients without any side effects. For this disease Planet ayurveda provides herbal remedies which are providing great results.

PRODUCTS LIST

1. ASHWAGANDHA CAPSULES
2. MUSLI STRENGTH
3. SHATAVARI CAPSULES
4. BOSWELLIA CURCUMIN
5. BRAHMI CHYAWANPRASH

PRODUCTS DESCRIPTION

1. Ashwagandha Capsules

Ashwagandha capsules are single herbal formulations prepared by planet Ayurveda using single extract of Ashwagandha (Withania somnifera). It has a rejuvenating property that helps to maintain proper nourishment and supports the proper functioning of adrenal and reproductive systems. These capsules help to relieve muscles and calm down the mental stamina.

Dosage– 1-2 capsules twice daily with plain water after meals.

2. Musli Strength

Musli strength are polyherbal capsules formulated by planet ayurveda using ingredients such as Safed Musli (Chlorophytum borivilianum) and Gokshura (Tribulus terrestris). These capsules help to provide strength to the body and uplifts the power of the reproductive system. Helps to increase the quality and quantity of sperms, it gives strength to the bones and joints, this capsule has Gokshura which is giving power to the whole body.

Dosage– 1-2 capsules once or twice daily.

3. Shatavari Capsule

Shatavari Capsules are single herbal formulations prepared by planet ayurveda using standardized extract of Shatavari (Asparagus racemosus) maintains female health and reduces weakness, fatigueness. It helps to improve the female health and has a rejuvenating property. It helps to improve depression, genitourinary and has antimicrobial, antibacterial properties. Shatavri capsules reduces the risk of miscarriage and helps to developed various organs during pregnancy.

Dosage– 1-2 capsules twice daily with plain water after meals.

4. Boswellia Curcumin

Boswellia Curcumin are poly herbal capsules formulated by planet ayurveda using ingredients such as Shallaki (Boswellia serrata), Curcumin (Curcuma longa). These capsules have anti-inflammatory properties and provide joint relief, used as an arthritis supplement. It helps to reduce the stiffness and swelling in the joints, Curcumin helps to cleansing property and can fight against any type of arthritis, increases mobility and reduces the stiffness.

Dosage– 1 capsule twice daily after meals with plain water.

5. Brahmi Chyawanprash

Brahmi Chyawanprash is classical ayurvedic products which is formulated by planet ayurveda using ingredients such as Bhumi Amla (Phyllanthus niruri), Jivanti (Leptadenia reticulata), Jeerak (Cuminum cyminum), Ashwagandha (Withania somnifera) and many more. It helps as an immunomodulatory, memory boosting and rejuvenating property. It helps to improve and enhance the health issues which boosts up energy and doesn’t let toxins get accumulated.

Dosage– 1-2 teaspoons twice daily after meals.

CONCLUSION

Limb Girdle muscular dystrophy is a group of diseases that causes weakness and wasting of the muscles commonly seen in arms and legs. In muscular dystrophy there are abnormal genes that interfere with the production of proteins which are needed to form a healthy muscle. When it is contaminated by doshas or ama it leads to many diseases known as asthi pradoshaja rogas. Asthi Pradoshaja rogas causes asthi bheda, asthi toda and many diseases. When it is contaminated by doshas or ama it leads to many diseases known as asthi pradoshaja rogas. Asthi Pradoshaja rogas causes asthi bheda, asthi toda and many diseases. Planet ayurveda provided many herbal remedies which provided great relief without any side effects.