MUSCULAR DYSTROPHY DISEASES

The weakness of muscles (myopathies) is the diseases interfere with chemical reactions involved in drawing bio-energy (Prana shakti) from food. Normal, fuel molecules derived from food must be broken down further inside each cell before they can be used by the cells; mitochondria to make the energy molecule ATP. The mitochondria inside each cell could be called the cells; “energies.” The metabolic muscle diseases are caused by problems in the way certain fuel molecules are processed before they enter the mitochondria, or by the inability to get fuel molecules in to mitochondria. Muscle required a lot of energy in the form of ATP to work properly. When energy levels become too low, muscle weakness and exercise intolerance with muscle pain or cramps may occur.
In a few metabolic muscle disorders, symptoms aren’t caused so much by a lack of energy, but rather by unused fuel molecules that build up inside muscle cells. This buildup may damage the cells, leading to chronic weakness. Metabolic muscle diseases that have their onset in infancy tend to be the most severe, and some forms are fatal. Those that begin in childhood or adulthood tend to be less severe, and changes in diet and lifestyle can help most people with the milder forms adjust.

What causes muscle weakness?
Most muscle weakness is caused by defects in the enzymes (Agnis) that control chemical reactions used to breakdown food. Enzyme defects are caused by flaws in the genes that govern production of the enzymes. A gene is a “recipe” or set of instructions for making a protein, such as an enzyme. A defect in the gene may cause the protein to be made incorrectly or not al all, leading to deficiency in the amount of that enzyme. Genes are passed from parents to children. Therefore genetic defects can be inherited.
In normal metabolism, food provides fuel that’s processed inside the cells, producing energy (ATP) for muscles contraction and other cellular functions. In metabolic myopathies, missing enzymes prevent motochondria from properly processing fuel, and no energy is produced for muscle function.
Muscular dystrophy refers to a group of genetic diseases characterized by progressive weakness and degeneration of the skeletal or voluntary muscles which control body movement. The muscles of the heart and some other involuntary muscles are also affected in some forms of muscular dystrophy, and a few forms involve other organs as well. The muscular dystrophies include many inherited disorders such as: Becker’s, Duchenne, Facioscapulo-humeral, Limb-girdle, Emery-Dreifuss, and Myotonic and myotonia congenita.

Most common neuro muscular disease among children
Most children are affected by muscular dystrophies, inflammation of muscles, cerebral palsy, motor problems related to injuries at the time of birth or thereafter, and also birth-related injuries to the arms and legs – mostly to the nerves in the arms.
The most common diagnosis would be spasticity in children, muscular dystrophy and different kinds of delays such as not walking, crawling and so on. Other less common problems are spinal muscular atrophy, neuropathies and so on.
Duchenne muscular dystrophy is the most common form affecting children, and myotonic muscular dystrophy is the most common form affecting adults. Muscular dystrophy can affect people of all ages. Although some forms first become apparent in infancy or childhood, other may not appear until middle age or later.
These diseases are distinguished from each other by the type of inheritance (sex-linked, dominant gene, recessive gene), the age when symptoms appear, and the types of symptoms that develop. Because these are inherited disorders, risks include a family history of muscular disease.

Symptoms of muscular dystrophy
Weakness is primary symptom

• Progressive frequent falls
• Delayed development of muscle motor skills
• Difficulty in walking (delayed walking)
• Difficulty using one or more muscle groups (the specific muscles affected depends on the type of dystrophy)
• Poor muscle tone and curved spine

Muscular dystrophy is diagnosed by serum CPK (an enzyme found in muscle) and EMG (electromyography). An ECG is also done to monitor changes in cardiac status.

This disease may also alter the results of the following tests:

• Myoglobin in urine / serum
• LDH (lactate dehydorgenase)
• Creatinine
• AST (aspartate aminotrasferase)

What are the complications?

• Joint contractures, deformities and scoliosis
• Permanent progressive disability
• Decreased functional ability to care for self
• Mental impairment and depressive manifestations
• Cardiomyopathy, respiratory failure and death

What is the prevention?
Women may be asymptomatic but still be carrier of gene for the disorder. Duchenne muscular dystrophy can be detected with about 95% accuracy by genetic studies performed during pregnancy

New treatment options
There is a big push to find a cure or at least modify the problems in different ways. There are newer treatment options.
Panchkarma therapies, specially developed molecules (mamsagni rasayan) and yogic techniques may be effective in the long term management of muscular weakness. It may help people to maintain muscle strength and function. In addition, some people with metabolic disorders have benefited from strict ayurvedic dietary changes. There is evidence that those with carbohydrate-processing problems may be helped by a natural high protein diet, while those with difficulty processing fats may do well on a natural diet high in carbohydrates and low in fats.
Ayurvedic herbs which safe guard the function of liver can be very effective in reversing heart failure in the patient of myopathies. Orthopedic appliances such as braces and wheelchairs can improve mobility and self care abilities. In some cases, surgery on spine or lower extremities may help improve function and slow deterioration.
Ayurveda considers this condition as mamsa – vata – kshaya (MVK) related to beeja dosha and impairments of mamsagni (muscle enzyme). It involves deranged vata and subsequent degeneration of muscles and structures related to it. Ayurvedic treatment might slow or stop the progressive of muscle degeneration. A combined strategy of age old Til Mash Pinda sweda (TMP), pizhichil, anuvasana vasti, mamsagni rasayana and yogic support is effective in the long term management of muscular weakness.

Mamsagni rasayana
The mamsagni G is gurvel, guggul and gold (G3) based new ayurvedic formula developed by Ayurveda Acharya Mukesh Dev Jain in 1998 for his care through research project on neuromuscular diseases. The active ingredients are bio-enhanced and standardized marich, triphala, gurvel, guggul, kuchla, parad and gold bhasma. This new compound molecule regulates muscular metabolism and useful as a safe ayurvedic medicine for the management of muscular dystrophies.
The marich promoted mamsagni (muscle enzyme system) triphala guggulu is well known srotorodhahara (correct membrane defect) and regulated fat metabolism. Kuchla boost the neuromuscular tone while the gold bhasma retards the process of muscular degeneration. Gurvel also called amrita (nectar) is best immunity modulating (rasayana) drug. The combined effect is neuro-muscular vata-pitta-kapha balancing which is evident by promising clinical results such as weight loss, decrease in blood CPK level, decrease in walking difficulty, increase in neuro-muscular tone

The practical therapeutic benefits of weight reduction are not only obvious but potentially greater i.e. improved muscle power/ weight ratio.

Dose: the therapeutic oral dose is 20 mg/kg body weight administered in two divided doses. The medicine can be given preferably before meals with honey or with dasmula ghrita.

Dr. Mukesh D Jain MD
Global ayurveda (Dec-Jan 2007)