Category: Ankylosing Spondylitis

What is GNE MYOPATHY? – Causes, Diagnosis & Ayurvedic Treatment

Abstract

GNE myopathy is a hereditary illness that mostly affects the skeletal muscles. It is also known as HIBM, Nonaka myopathy, IBM2, and distal myopathy with rimmed vacuoles. The disease’s earliest symptoms develop between the ages of 20 and 40, and it affects both men and women at the same rate. Progressive muscular weakness, which often increases over time, decreasing grip strength, and frequent loss of balance are all symptoms of this illness. There is currently no cure for the condition, and therapy focuses on symptom management. Ayurveda, on the other hand, may aid in the management of this ailment.

Introduction

GNE myopathy is a hereditary muscle disorder in which GNE refers to the defective gene. It is a rare condition that affects around one person in a million individuals throughout the globe. It causes muscle weakness and atrophy in the legs and arms. The beginning of symptoms is generally in young adults, however, some people have seen a later onset. Foot drop is generally associated with increased tripping and a steppage gait at first. The problem worsens with time, resulting in proximal leg muscular weakness, difficulty ascending stairs or rising from a seated position and weakening of the hands and shoulder muscles.

How an you recognize myopathy’s indications and symptoms?

  • Disturbed gait and impaired stability are common in GNE myopathy patients, as are frequent falls, difficulties ascending stairs, jogging, and getting up from a sitting posture.
  • The muscles of the lower limbs are the first to be damaged, with the exception of the quadriceps, which seem to be largely unaffected.
  • Neck muscles might be impacted in the latter stages of the illness.
  • Finally, as the illness progresses, skeletal muscle function may be lost completely, leaving the patient completely reliant on others.

Acquired myopathies may exhibit symptoms that are comparable to hereditary myopathies, as well as the following

  • Muscle deterioration
  • Motor sluggishness
  • Impairment of the respiratory system
  • Dysfunction of the bulbar muscles (malfunction of the muscles responsible for swallowing and speech)

Causes of GNE myopathy

Causes

Changes (mutations) in the GNE gene, which codes for the enzyme glucosamine (UDP-N-acetyl)-2-epimerase/N-acetylmannosamine kinase, cause GNE myopathy. Infection, muscular damage, medicines, electrolyte imbalances, and thyroid illness are among the other reasons.

Diagnosis

GNE myopathy testing is appropriate for patients who have foot drop (in young adults) and distal extremity muscle weakness (in those who have advanced stages of the illness). Genetic testing is an excellent initial step in determining the severity of GNE myopathy in individuals. This includes the use of minimum or no invasive treatments, simply requiring a blood or saliva sample, and yielding results in hours or days.

Factors at Risk

Some myopathies, such as some forms of muscular dystrophy, are hereditary. Other myopathy risk factors include:

  • Myasthenia gravis, scleroderma, and thyroiditis are examples of autoimmune diseases.
  • Endocrine problems
  • Toxin exposure, such as pesticides and flame-retardant chemicals
  • Infections
  • Deficiency in vitamins
  • Antihistamines and long-term corticosteroids are among the drugs used.
  • Glycogen and lipid storage disorders are examples of metabolic diseases.

Treatment with Ayurveda

It is considered an incurable myopathy, with mortality occurring between the ages of 20 and 25. Modern science has limits in treating the ailment, but Ayurveda has a lot of promise in treating diseases that are incurable. Ayurveda may assist in maintaining a healthy range of motion for all of the body’s joints. Toxins are removed from the body, the doshas are balanced, and the body cells and muscles are rejuvenated to promote strength and flexibility. Yoga and meditation sessions are used in addition to treatment methods to increase blood circulation to all body parts and joints. These reduce the likelihood of acquiring additional medical problems while also enhancing respiration and other critical bodily processes.

A healthy food plan is also arranged for optimal nourishment that assists in muscle growth as well as overall body development. A well-balanced diet may assist to soothe inflamed doshas and bring the body’s critical systems and hormone levels back into equilibrium. Herbal decoctions for internal ingestion are given in addition to therapy regimens to assist ease symptoms. And that’s what the world’s leading Ayurveda industry, Planet Ayurveda offers us.

Description of the product

1. Ashwagandha Ghrit

It’s great for muscular fatigue, stiffness in the neck or back, exhaustion, joint problems, and arthritis of any kind. It also helps with anxiety, tension, and depression. The main ingredient of this formulation includes Ashwagandha (Withania somnifera).

Dosage: 1 tsp twice a day in warm water

2. Musli Strength

Safed Musli (Chlorophytum borivilianum) and Gokshura (Tribulus terrestris) are used in this herbal medicine. It aids in the enhancement of growth hormone circulation in the blood. This also aids in the development of muscle growth and strengthening to help in reducing GNE myopathy effects.

Dosage: 1 capsule twice a day

3. Boswellia Curcumin

Boswellia Curcumin seems to be helpful in lowering inflammation, alleviating arthritis symptoms, and increasing muscular strength. Shallaki (Boswellia serrata) and Curcumin (curcuma longa) are the main ingredients in this herbal combination that can help to get relief from GNE myopathy.

Dosage: 2 capsules twice a day

4. Yogaraj Guggul

Yograj Guggulu contains herbs including Chitraka (Plumbago zeylanica), Shuddha Guggulu (Commiphora mukul), Ardraka (Zingiber officinale), Pippali (Piper longum), Haritaki (Terminalia chebula), and others that may help reduce pain and enhance joint and muscular mobility, which following useful to get rid from GNE myopathy.

Dosage: Take 2 tablets  twice a day

5. Atirasadi Churna

Gokshura (Tribulus terrestris), Ashwagandha (Withania somnifera), Nutmeg, Shatavari (Asparagus racemosus), Kali Musli (Curculigo orchioides), Safed Musli (Chlorophytum borivilianum), and Varahi Kand (Dioscorea bulbifera) are some of the components in Planet Ayurveda’s Atirasadi Churna. It primarily aids in the development of stamina, and the strengthening of weak muscles. Moreover, it helps in the reduction of tension, and the reduction of worry.

Dosage: 1 tsp twice a day with warm milk is the recommended dosage.

6. RasRaj Ras

It is an Ayurvedic medication for neuromuscular diseases, as well as nerve and muscle strengthening. Ashwagandha Churna (Withania somnifera), Rasa sindura (purified and processed Mercury), Swarna Bhasma (Gold Bhasma), Loha Bhasma (Iron Bhasma), Rajata Bhasma (Silver Bhasma), and Vanga Bhasma (Tin Bhasma) are some of the herbs found in it.

Dosage: 1 chewable tablet once a day.

7. Bala Ashwagandha Oil

The oil’s rejuvenating effects help muscles to relax, nerves to tone, and nerve conduction to improve. It is used to relax the muscles while gently stroking them.

Dosage: For external use only.

Contact my assistant to provide you the costing / ordering and delivery information at – costing.planetayurveda@gmail.com or call at +91-172-5214040 Or Check Website – www.PlanetAyurveda.com

Conclusion

The negative effects of long-term allopathic medicines and therapies that produce GNE Myopathy might be addressed with these herbal combinations. As a result of the inefficacy of allopathic therapy. Start with Planet Ayurveda to purchase a real Ayurveda product if you want to learn more about the therapy. They may be reached at herbalremedies123@yahoo.com or their clinic in Mohali, Punjab.

Duchenne Muscular Dystrophy (DMD)Treatment with Herbal Remedies

Abstract

Bones and muscles are the specialized connective tissues that provide shape to the body. Bones only provide a basic framework to the body but muscles are the basic structure that maintains the body in proper shape. Almost each and every corner or organ of the body is connected to muscles that are well instructed by brain cells to maintain neuromuscular coordination and perform various voluntary or involuntary tasks. But due to some inheritable or other causes will lead to loss of proper functioning of the muscles which results in multiple health issues leading to a group of disease affecting muscles known as myopathies or muscular dystrophies. Out of which the most common is Duchenne muscular dystrophy. In this article we are going to discuss duchenne muscular dystrophy along with its clinical features, causes and best treatment using Ayurveda.

Introduction

Duchenne muscular dystrophy (DMD) is the form of myopathy (the disease that affects muscles) that primarily affects boys. This condition usually begins in very young ones i.e. approximately in the 3-4 years old and then worsens gradually at a fast rate. The affected children start facing difficulties in standing and walking due to the progessive weakening of muscles of thigh and pelvis in the early stages of the disease. This condition is manifested due to the loss of dystrophin protein. Dystrophin is an essential protein which is required for the proper functioning of the muscle as it is the main constituent of muscle fiber’s cell membrane. The loss or absence of this protein will result in the characterization of a group of structural and mobility-related symptoms which constitutes this disease.

Duchenne Muscular Dystrophy

Sign and Symptoms

DMD is a disorder characterized by gradual muscle loss due to the death of muscle fibres or replacement by other connective tissue or fat that initially affects the muscles of thigh, pelvic area and calves slowly progressing towards muscles of shoulders, and upper extremities, neck and respiratory muscles. Muscle fatigue is the commonest sign observed.

The classical sign of DMD is the patient having difficulty in getting up from a lying or even sitting position. And this is known as the positive Gowers’ sign (that governs the weakness of proximal lower limb muscles), the child usually gets up by lying on his abdomen and trying to lift his body with the help of upper extremities due to the gradual loss of muscle strength in lower limbs.

The clinical features can be seen in the affected boys with Duchenne muscular dystrophy beginning to manifest even before five years of age, they face it very difficult in stepping or walking, the affected child usually tends to walk on toes due to the gradual shortening of Achilles tendon. In later stages, along with the growing age and enhancing dystrophy it becomes very much hard to walk and ultimately there is loss of ability to walk. Along with these mobility disorders the body undergoes various structural changes also due to the loss of muscles e.g. scoliosis is common.

Also, this muscle weakness will affect the heart muscles which leads to many defects in cardiac musculature. Other associated symptoms in later stages include difficulty in swallowing and talking and difficulty in breathing. Also there are some other non musculoskeletal disorders which may be found in association with DMD, dyslexia (learning disorder), aggravated risk of neurological diseases such as ADHD and weakness in specific cognitive skills which are considered to be as a result of dysfunctional dystrophin protein in brain.

Causes

  • It is a sex link inherited disorder, more specifically an X-linked recessive dystrophy, means there is a disturbance in the gene which is linked with X chromosome, which means that males are more affected as females can act as a carriers.
  • Only one third cases are reported due to mutation. Another two thirds are being inherited from the carrier mother.
  • This mutated gene is encoding a muscle protein named dystrophin.

Diagnosis

Diagnosis can be made on the clinical manifestations and more precisely genetic testing can be done to make a diagnosis at the time of birth. Moreover, the blood investigation is also suggestive of raised levels of creatine kinase. Other methods of testing include DNA testing, muscle biopsy and prenatal tests.

Complications

The prolonged duchenne muscular dystrophy can affect the multiple systems of the body, and a few major complications are listed as

  1. Scoliosis – deformity in the curvature of the spine when the back musculature is weakened and fully unable to support the vertebral column.
  2. Contractures– due to shortening of tendons, muscles or ligaments around joints, these will lead to joint deformity leading to mobility issues such as trouble walking or even standing.
  3. Severe Dyspnea– Breathing abnormalities occurs when muscular dystrophy involves the muscles of the diaphragm and chest muscles, (muscle supporting breathing process)
  4. Dysphagia– Difficulty in swallowing and speaking can occur when the muscles of the face and throat become weak.
  5. Cardiomyopathies– This disorder can also affect the heart musculature which can lead to various heart disorders and systemic problems.
  6. Paralysis- These conditions will gradually lead to complete loss of muscle function and result in paralysis of the body lower to the next after approx. the second decade of life.

Ayurvedic View

As this disease is a myopathy it involves the involvement of muscle tissues in the disease. In Ayurveda, mans dhatu is correlated with the various muscles of the body and the mansvaha sroto dushti (vitiation in microchanneled environment associated with the muscle tissue) is considered as the basic cause of all the myopathies or muscular dystrophies. And thus, Duchenne muscular dystrophy can be considered as mansvaha sroto dushti janya mamas dhatu kshya

Acharya Charka had mentioned the basic seats of manovaha srotas as sanayu (tendons) and twak (skin). Thus as discussed above the initial sign of this disease involves the shortening of the achilles tendon which results in mobility issues. The wrinkled, loose or flaccid skin can be generally seen in the parts affected. Also the kshay lakshan (signs of deficiency) of mamasdhatu as mentioned by Acharya Sushruta are as follows

There is gradual wasting of muscles of buttocks, pubic region, calves, thigh, cheeks, lips, axilla, chest, abdomen and neck. Malaise and loss in the elasticity of arteries and weight loss are also the general clinical signs of mansa dhatu kshaya.

These symptoms are very much similar to the features of Duchenne Muscular dystrophy. Hence the treatment protocol for the mamas dhatu kshaya must be used in this case for best results.

Treatment of Duchenne Muscular Dystrophy

Treatment plan according to Ayurveda for duchenne muscular dystrophy includes the following

1. Sanshodhan chikitsa

These include some procedures that are very much beneficial in the management of these variety of treatment

  1. Snehna karma (oilation therapy), external medicated massage is very much beneficial in treating the gradually increasing flaccidity and rigidity of muscles and enhancing the blood flow to the affected part will promote its functioning.
  2. Swedana karma (Sudation therapy): This modality of treatment focuses on the liquefaction of dosha from the extremities to the kosha (towards the centre of body i.e. gut) and thus enhancing the working efficiency of the extremities and is most effective in reducing stiffness. It is the best vata-kapha shamak (quenching vata and kapha) procedure.
  3. Vasti karma (therapeutic enema) with balya dravya and various ksheer vasti yogas will help in providing strength to the muscles and the therapeutic action upto the cellular levels helps in activating the muscle fibres and returning to their proper function.
  4. Nasya karma (Nasal administration of medicated oils) Nasal route is said to be the route to the brain. So, regular use of nasya with balya and medhya (nervine tonics) will help in improving the neuromuscular coordinations and thus reversing the early symptoms of the disease and also reducing the severity of the disease.

2. Shanshaman Chikitsa

Shanshaman Chikitsa includes the use of herbal formulation for strengthening the muscle fibres or correcting the neuromuscular coordination. The use of dhatu poshak dravyas along with balya (drugs used for proper feeding of body tissues and providing strength) must be done.

Planet Ayurveda Is A Globally Emerging Medicine Manufacturing Hub That Manufactures The Herbal Or Herbomineral Formulations With Strict Adherence To Ayurvedic Texts. The Products Formulated Here Are Obtained From 100% Natural Sources And Are Duly Purified And Processed Under The Guidance Of Our Expert Team Of Ayurvedic Physicians According To Ancient Time Tested Formulas.

Best Herbal Remedies as suggested by the expert team for the management of Duchene muscular dystrophy

  1. Musli Strength
  2. Ashwagandha Capsule
  3. Green essential
  4. Boswellia-Curcumin
  5. Suvarna Basant Malti Ras
  6. Atirasadi churna

Herbal Remedies for duchenne muscular dystrophy

Duchenne Muscular Dystrophy (DMD)

Products Description

1. Musli Strength

The ingredients of these capsules include extracts of safed musli (Asparagus adscendens) and gokshura (Tribulus terrestris). These both are considered as the best strength enhancers since ages.

Safed musli as described in Raj nighantu

Here, the word pushtibalprada means nourishing the body, and providing strength to the muscles. Also, the word shramhara here means it can also help in preventing or treating the over fatigue in muscle caused due the Duchene muscular dystrophy. Also, Acharya Charka had mentioned musli in sukrajanan mahaakshay, due to its dhatu vardhak (nourishing the bodily tissues deeply step by step from rasa to rakta and upto shukra) action because of its madhur-madhur-sheet sweet taste, aphrodisiac nature and cool potency) qualities that enhances the quality of body tissue which in process will also treat the altered mans dhatu (body tissue associated with muscles) due to the ongoing muscular dystrophy. Gokshura also has such properties that promote the health of muscles and also flushes out the toxins from the system to fast up healing the damage.

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

2. Ashwagandha Capsule

These capsules are being formulated with the standardised extract of Ashwagandha (Withania somnifera). Ashwagandha is a well known herb for promoting the overall strength of the body. Acharyas had described the many health benefits of it. Some of which play a very key role in treating Duchenne muscular dystrophy. The uses mentioned in text for Ashwagandha are as:

In this shloka the word balya signifies the ultimate efficiency of the herb in providing strength to the musculature, also the word rasayani means having some rejuvenating properties, (the herb is rich in antioxidants and promotes the better scavenging of free radicals and other endotoxins from the body). The other word shukarla signifies its role in nourishing the dhatus by the same procedure as discussed in musli. Thus with these properties along with its shleshmasothakshayupha (decreasing the aggravated kapha, reducing swelling or painful conditions and treating the dystrophy), this is the perfect formulation for these types of dystrophies.

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

3. Green Essentials

These capsules formulated by the Planet Ayurveda team for the betterment of essential vitamins and nutrients for the disease. Green Essentials contains grape seeds (Vitis vinifera), wheat grass (Triticulum aestivum), spirulina and amalaki (Emblica officinalis) like herbs which are a rich source of required nutrients for the body. The person affected with Duchenne muscular dystrophy most of the time is unable to cope with the required nutrition due to dysphagia . These capsules will facilitate the completion of requirements of the body along with boosting the immune system of the body.

Dosage– 1 capsule twice daily after meals with water.

4. Boswellia Curcumin

Boswellia-Curcumin is the one of the best formulations made by the Planet Ayurveda team with the goodness of extracts of two magnificent herbs i.e. boswellia and curcumin. The useful part used in Boswellia serrata (also known as shallaki), is the gum resin extracted from it (which is also mentioned as kundru in Ayurvedic texts). The properties of kundru as mentioned in texts are as:

Here, the word kaphanailan means it is beneficial in quenching both the kapha and vata dosha, which are very much beneficial for this muscular dystrophy. The herb also has anti-inflammatory and analgesic effects which also support the condition symptomatically.

The other ingredient Curcumin (Curcuma longa) which is known as haridra in ayurvedic texts has multi-dimensional beneficial actions on encouraging the health and healing power (immune response) of the body. The qualities of Haridra as mentioned in Ayurvedic texts:

Here in this phrase, the word krimighna can be understood as the quality of haridra to scavenge the various endotoxins from the body by its rich antioxidant nature of its phytochemicals. Also the term kaphapitanut in the shloka, signifies the property of curcumin to pacify the vitiated kapha and pitta condition, which will support digestion.

Thus, these both ingredients in combination makes a perfect match that makes the body more able to fight Duchenne muscular dystrophy.

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

5. Suvarna Basant Malti Ras

As discussed above there is depletion of mamsa dhatu due to mansvaha sroto dushti. And Acharyas had mentioned suvarna (purified gold) preparations are best for dhatu kshaya avastha (state of depletion of body tissue i.e. muscle tissue). Suvarna Basant Malti ras is a classical formulation with gold as the main ingredient along with other ingredients that are beneficial in nourishing the depleted mamsa dhatu as a result of duchenne muscular dystrophy along with enhancing the overall health of the body. This preparation also has a role in pacifying vitiation of vata and vata is considered as the main dosha involved in various neurological disorders, thus supporting better neuromuscular coordination of the tissues.

Dosage– 1 tablet once daily to be chewed, after meals.

6. Atirasadi Churna

Atirasadi churna is an herbal formulation available in powder form. It consists of the ingredients that have the best dhatu poshak effects. These ingredients include safed musli, ashwagandha , shatavari (Asparagus racemosus) and kesar (saffron) like herbs which aids the nourishment of bodily tissues and is also a great strength provider for muscles. Moreover, the powder form of these herbs facilitate in the better absorption and fine nature of the extracts allowing the extending the reach upto cellular level and thus magnifying the effects of this formulation in Duchene muscular dystrophy.

Dosage– 1 teaspoon twice daily with lukewarm water after meals.

Contact Planet Ayurveda to provide you the costing / ordering and delivery information at – costing.planetayurveda@gmail.com or call at +91-172-5214030 Or Check Website – www.PlanetAyurveda.com

Conclusion

There is no treatment for Duchenne muscular dystrophy with allopathic medicine. Whereas Ayurveda being an ancient science of life, treating such ailments since ages through a holistic approach of therapeutic procedures and herbal formulations. So, this is the best time to shift to Ayurveda mode of treatment for managing this type of dystrophies.