Gne Myopathy – Body Unlearning Its Functionalities
We begin learning about our bodies from the first day of our lives. We try to move hands, sit, move from one place to another, walk, and so on, just like people around us do, which are known as milestones. These are normal developmental progressions that occur during our infant phase, but some children do not attain their milestones at the appropriate time; this is a known fact. But what we’re going to discover today, is a disease that causes the unlearning of all these milestones after a person reached adulthood, which isn’t very commonly seen in our society.
GNE MYOPATHY or Hereditary inclusion body myopathy is a rare autosomal recessive disorder. The disease affects the skeletal muscles, which are essential for every voluntary movement in the body. GNE myopathy manifests during the period of 20s to 40s of a person’s life. As the initial symptoms like general weakness of muscles and frequent falls are not taken seriously by patients and have a great chance of misdiagnosing, this disease is diagnosed at a very late stage. This is a disease in which the body unlearns its function, going back to how it was like an infant.
As the disease manifests in or after the 20s, the person who was completely unaware of disabilities is now forced into such a situation. A cure for this disease is not found, but symptoms can be managed to an extent. Proper treatment can delay a person from being wheelchair-bound or bedridden which is usually expected in 10 to 20 years from the onset of the disease. The disease affects 1 in a million people and the majority of the patients suffer from misdiagnosis.
HOW AYURVEDA VIEW GNE MYOPATHY
In Ayurveda, myopathies are considered to be due to the “Mamsa dhatu gata Vata“, a condition in which Vata dosha is vitiated and causes depletion of muscle tissues (mamsa dhatu kshaya). Mamsa dhatu dushti/prakopa condition can also be considered for treatment purposes.
Rasa (plasma), Rakta (blood), mamsa (muscle tissues), Medha (fat-adipose tissue), Asthi (bone), majja (bone marrow), and shukra (semen) are the seven dhatus that make up our body. Any of this dhatus, when vitiated or depleted, causes problems to general wellbeing. Mamsa shosha, or mamsa dhatu depletion (muscle wasting), can cause restricted movement of the body. Dhatu kshaya (Tissue depletion) is frequently associated with Vata dosha vitiation. Either Vata vitiation caused tissue depletion or tissue depletion caused Vata vitiation. Both should be treated concurrently to recover. Treatment may not result in complete recovery in some cases, but it can prolong the disease process and provide quality of life even when suffering from a chronic disease. GNE Myopathy is classified as an “Adhi-Bala pravritta vyadi,” i.e., a disease passed down from parents, it falls under the Asadhya roga (impossible or incurable diseases) category. But the treatment can prolong the complete loss of muscle function for years with periodical treatments.
WHAT CAUSES GNE MYOPATHY
GNE gene, which encodes for the enzyme in charge of producing sialic acid (a sugar needed for muscle cells, to create energy and also is a key component of cell membranes) becomes altered and impacts the activity of GNE enzymes resulting in GNE Myopathy. A progeny has to inherit defective GNE genes from both parents to be affected by these ailments, otherwise, he or she is just an asymptomatic carrier of this disease.
SYMPTOMS SUGGESTING THE PRESENCE OF GNE MYOPATHY
- Progressive muscle wasting
- Disturbed gait and frequent falls
- Decreased stability
- Bilateral foot drops due to weakness of the Tibialis anterior muscle
- Difficulty in doing simple daily physical activities like climbing stairs, running, or making a tight grip on something with hands
- Weakness starts in the lower limbs excluding quadriceps muscles and within 10 to 20 years after the onset, the patient becomes incapable of any voluntary movements
HOW IS GNE MYOPATHY DIAGNOSED?
- Genetic Testing – For identifying gene mutation, but this alone doesn’t confirm GNE myopathy
- Muscle Biopsy – Helps in ruling out other types of myopathies and confirms the presence of the condition. Also, provide information about the stage of the disease
- MRI – Helps in understanding the extent of the spread of the disease
TREATMENT OPTIONS PROVIDED BY MODERN MEDICINE
As a complete cure is yet to be found, treatment focuses on delaying the complete loss of muscle strength and movability. Overuse or underuse can accelerate the disease process. Periodic physiotherapy and optimum daily movement of the muscles can delay muscle wasting.
Enzyme replacement therapy has shown a modest but positive effect on muscle strength, but the effect being small to negligible caused the research to be discontinued.
TREATMENT OPTIONS IN AYURVEDA FOR GNE MYOPATHY
- Treatment focuses on bringing back Vata into its normalcy and replenishing muscle tissues.
- Panchakarma procedures like abhyanga (oil massage), shashtikashali pinda Swetha (massage using a heated bolus of medically prepared shashtika/Navara rice), vasti (enema with medicated liquids) are a few external procedures done for muscle strength and to pacify Vata dosha.
- Internal medications consist of properties that can pacify Vata dosha and nourish the mamsa dhatu i.e., muscle tissues.
HERBAL REMEDIES FROM PLANET AYURVEDA
Planet Ayurveda is a well-known ayurvedic medicine distributor with a global presence. All of our medications are chemical-free and completely safe to consume. All pharmaceuticals used are carefully sourced to ensure that the medicines we produce are of the highest quality. All of the medications we recommend for this condition have been carefully chosen by our most trusted doctors to make your journey with GNE Myopathy easier than ever. These medications will help your body maintain muscular function for a much longer period of time.
1. Ashwagandha Ghrit
Ashwagandha ghrit is a classical ghee-based ayurvedic medication, consisting of Withania somnifera as its main ingredient. Ashwagandha, also known as ‘Indian ginseng’ enriched with numerous withanolides, alkaloids, and sitoindosides, is an excellent immunomodulator, adaptogen and that helps build body and muscle strength. The ghee as a medium is excellent to facilitate the digestion and absorption of the drug resulting in faster improvement of muscle functions.
Dosage: 1 teaspoon in warm water twice a day.
2. Musli Strength
Musli (Chlorophytum borivilianum) and Gokshura (Tribulus Terrestris) are drugs used in a vast number of diseases. The standard extract of these both are made into capsules which are made from 100%vegetarian raw materials. Musli contains over 25 alkaloids and is known to nourish the muscles, increase muscle strength, and promote overall health. Gokshura containing steroidal saponins is a great immunomodulator and strengthening drug.
Dosage: Take one capsule twice a day.
3. Boswellia+Curcumin
Shallaki (Boswellia serrata) and Haridra (turmeric, Curcuma longa) are two amazing drugs with so many health benefits. Effects of turmeric are innumerable and shallaki is a body replenishing tonic for overall health improvement and avoiding degenerative changes. A combination of both drugs that contain phytoconstituents such as Acetyl-11-keto-β-boswellic acid, curcumin, etc. can decelerate the disease process and provide you with a high-quality life.
Dosage: 2 capsules twice a day is recommended.
4. Yograj Guggul
Yograja guggul is a classical medicine that is used for generations in India.it is a polyherbal tablet enriched with chitraka (Plumbago zeylanica), pippali (Piper longum), Yamani (Trachyspermum ammi), etc. Phytoconstituents augmented to this medicine through all 29 ingredients help to balance Vata dosha, increase muscular strength, and improve general health.
Dosage: 2 tablets twice a day is recommended.
5. Atirasadi Churna
Ashwagandha (Withania somnifera), Nutmeg (Myristica fragrans), Kali Musli (Curculigo orchioides), Safed Musli (Chlorophytum borivilianum), Gokshura (Tribulus terrestris), etc. are among the ingredients of this amazing formulation. This is a polyherbal medicine in powder form with innumerable active components like saponins, withanolides, alkaloids, etc that can boost your immunity, rejuvenate the body and help you rebuild muscle mass.
Dosage: 1 teaspoon powder twice daily with warm milk.
6. Rasraj Ras
This is a classical Herbo mineral medicine in tablet form, adapted from Siddha yoga sangraha. The effect of this medicine in neuromuscular conditions was proved with centuries of use. It has noteworthy analgesic, antioxidant and immunomodulating properties.
Dosage: once daily, to be chewed.
7. Balaswagandhadi Thailam
This is a medicated oil prepared from Bala (Sida cordifolia), Ashwagandha (Withania somnifera), and many more drugs, used for external application. Bala and ashwagandha contain natural steroids in it to accelerate your healing process. The oil provides nourishment and strength to the muscles, bones, joints, and pacifies Vata dosha.
Method Of Application: 15 to 30 minutes before a lukewarm water bath, This should be used for external application.
Conclusion
Ayurveda has changed many lives over the centuries. Now you can also experience the magic of elixirs we prepare as per the guidance of our great ancestors. Add Planet Ayurveda into your life as your most devoted companion, and see how your life changes with the finest quality medicines made in the field of ayurvedic pharmacy.
