Ayurvedic Treatment for GNE Myopathy - Genetic Disorder

ABSTRACT

GNE myopathy is an autosomal distal myopathy which is rare in occurrence. This recessive myopathy usually starts in adult age characterized by moderate or slow progressive weakness of the distal muscles specifically affecting tibialis anterior muscle, sparing the quadriceps femoris. As this is a rare condition hence you might not find complete information on it anywhere. However we have tried to gather all the scattered data and present at one place. Other than details of the disease you must find its Ayurvedic management in this write up.

INTRODUCTION TO GNE MYOPATHY

HIBM, distal myopathy, IBM2 and Nonaka myopathy are the other names given to GNE myopathy. GNE myopathy specifically is a genetic disorder that is presented by the weakness of muscles which our body uses in day to day physical activities, i.e. skeletal muscles. Initial signs and symptoms of the disorder starts to come in observance between 20-40 years of age, however in some cases it might start in teen age. Men and women are both equally affected. As the disease progresses weakness of the muscles worsen and eventually resulting in repeated loss of balance and weaken grip strength.

AYURVEDIC ASPECT OF GNE MYOPATHY

In Ayurveda, genetic disorders are co related with beejdosha vikara which means defect in the embryo (genetic defect) resulting in genetic diseases. Four components are essential for the development of embryo, i.e. Rutu (Fertile period), Kshetra (Uterus and passages), Ambu (Nutrient fluid) and beej (Spermatozoa and ovum). Hence defect in any of them due to various factors like bacteria, fungi, viruses, industrial chemicals, alcohol, combustion products, ionizing radiation, ultraviolate radiation, and molecular oxygen can lead to genetic diseases. Other than this diet of the mother, vitiation of dosha, time factor and some unknown reasons can also cause genetic disorders.

CAUSES OF GNE MYOPATHY

Mutation of specific genes result in GNE myopathy. This mutated gene is normally responsible for the production of the enzyme that further is necessary for the production of SA (Sialic acid) which is required for the production of energy in all the cells along with the muscles cells. Hence abnormal mutation causes low or insignificant production of enzyme leading to low SA and hence weakness of muscles due to less energy which worsen with passing time.

SIGNS AND SYMPTOMS OF GNE MYOPATHY

There are specific complaints related to muscles only as the genetic defect causes effect on them most. Signs and symptoms starts to develop in adult age (20-30 years).

• Weakness of muscles
• Progressive muscle weakness
• In 10-20 years of onset of disease patient develops severe incapacitation
• Loss of balance frequently
• Weakened grip while holding objects
• Weakness of tibialis anterior muscle resulting in bilateral foot drop
• Disturbed gait causing frequent falls along with decreased stability
• Difficulty in climbing stairs
• Difficulty in getting up when seated and running
• Initially lower limb muscles are affected gradually affecting the upper limb muscles
• In worse cases even the neck muscles can be affected
  
  

DIAGNOSIS OF GNE MYOPATHY

Characteristic symptoms of weakness of distal extremity muscles along with foot drop will make a doctor suspicious of the GNE myopathy who goes for the further evaluation of the case history. Further patient is advised for genetic testing however certain other testing are done to confirm the GNE myopathy. Confirmative tests include muscle biopsy and MRI.

TREATMENT FOR GNE MYOPATHY

Similar to other genetic disorder there is no exact cure for the GNE myopathy and treatment is given to limit the disability and manage the signs and symptoms. Early diagnosis of the disease can be helpful in good prognosis of the case. Once the disease is diagnosed, patient is advised to avoid strenuous exercises as it can accelerate the progression of the disease. Conventionally the disorder is treated by giving supportive treatment for supplementation of SA (Sialic acid) along with the gene therapy. Certain physiotherapy and specific exercises are also advised to patient.

AYURVEDIC TREATMENT FOR GNE MYOPATHY

In Ayurveda, main aim is to rectify the vitiation of doshas, i.e. Vata, Pitta and Kapha along with management of signs and symptoms of the disease through herbal remedies.

HERBAL REMEDIES BY PLANET AYURVEDA FOR GNE MYOPATHY

Planet Ayurveda is a well known name in the field of herbal pharmaceuticals in India. Herbal products manufactured by Planet Ayurveda are used worldwide by the people owing the best quality and effectiveness of these products offered by the company. All the products manufactured at Planet Ayurveda are purely vegetarian, herbal, free from preservatives, additives, dye, colors, chemicals, and yeast which make them very effective in treating a complaint without any harmful effects exerted on body when taken as advised by doctors. For GNE myopathy Planet Ayurveda offers a specific combination of herbal remedies that are effective enough to manage the symptoms naturally.

Product List

1. Ashwagandha ghrit
2. Musli strength
3. Boswellia curcumin
4. Yogaraj guggulu
5. Atirasadi churna
6. Rasraj Ras
7. Bala Ashwagandha oil
   
   

Product Description

1. Ashwagandha Ghrit

This Ayurvedic formulation is in the form of ghee prepared using Ashwagandha (Withania somnifera) as the main ingredient. Ashwagandha ghrit balances vata, pitta and kapha dosha and help in treating muscle weakness and general debility in GNE myopathy.

Dosage- Take 1 teaspoon twice daily with warm water

2. Musli strength

Standardized extract of Safed musli (Chlorophytum borivilianum)and Gokshura (Tribulus terrestris) are used in the preparation of Musli strength capsules. As the name suggests these capsules help in gaining the muscle strength, has anti aging effect, removes weakness.

Dosage- Take 1 capsule twice daily after meals with plain water

3. Boswellia curcumin

These herbal capsules help in maintaining the muscle strength, reduce muscle injury and relieves the muscle soreness. It is prepared using the standardized extract of Shallaki (Boswellia serrata) and Haridra (Curcuma longa).

Dosage- Take 2 capsules twice daily after meals with plain water

4. Yograj guggul

It is combination of the herbs like Pippali (Piper longum), Ela (Eletteria cardamomum), Devdaru (Cedrus deodara), (Plumbago zeylanica), guggul (Commiphora mukul), Jeeraka (Cuminum cyminum), Mustaka (Citrus rotundus), Ardraka (Zingiber officinale), Haritaki (Terminalia chebula) etc. In GNE myopathy Yograj guggul help in relieving the muscle pain and soreness, helps in easing the walk and movements.

Dosage- Take 2 tablets twice daily after meals with plain water

5. Atirasadi Churna

Herbs like Shatavari (Asparagus racemosus), Safed musli (Chlorophytum borivilianum), Semal musli (Salmalia malabarica), Gokshura (Tribulus terrestris), Safed behmen (Centaurea behmen), Varahi kand (Dioscorea tuberose), Kesar (Crocus sativus) etc are used in the preparation of this herbal powder or churna. In GNE myopathy Atirasadi churna can help in treating the generalized weakness or debility, and also provide strength to the body.

Dosage- Take 1 teaspoon twice daily with warm milk after meals

6. Rasraj Ras

This tablet is an amazing combination of Abhrak bhasma (Calcined mica ash), Swarna bhasma (Calx of gold), Bang bhamsa (Calcined tin metal), Ras sindoor (Ayurvedic formulation), Ashwagandha (Withania somnifera), Laung (Piper longum) etc. Rasraj Ras is used in strengthening the nerves, muscles, hemiplegia, paralysis and cerebral disorders. It balances the Vata dosha.

Dosage- Chew 1 tablet daily once after meals

7. Balashwagandha oil

This amazing therapeutic oil helps in restoring and rejuvenating the strength and function of the muscles. It tones up the nerves and improve their conduction. Bala Ashwagandha oil is prepared using herbs like ashwagandha (Withania somnifera), Laksha (Laccifera lacca), Bala (Sida cordifolia), Manjishta (Rubia cordifolia), Sariva (Hemidesmus indicus), Durva (Cynodon dactylon) etc.

Direction- It is to be applied locally on the affected areas and massage gently.

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

GNE myopathy can be dreadful ailment for the person affected by it if not treated timely and with proper medications. Early diagnosis is the key for good prognosis. Genetic disorders might not be completely curable but with these herbal medicines definitely the level of disability can be decreased and patient can have better life.