What is Metachromatic Leukodystrophy?- Causes Symptoms & Ayurvedic Treatment

Abstract

This is an example of a disorder of lipid metabolism, a sulfatide lipidosis. Although there is some lipid storage in neurons in this disease, the basic process is that of myelin breakdown with the accumulation of galactosyl sulfatide and of certain other lipids which contain a galactosyl-3-sulfate moiety. The fundamental biochemical defect, in most cases, appears to be a deficiency of arylsulfatase-A (cerebroside sulfatase) with accumulation of sulfatides mainly in lysosomes. Rarer cases are due to multiple sulfatase deficiencies. This disorder is inherited as an autosomal recessive condition. Both CNS and PNS myelin are broken down. Today we will read about metachromatic leukodystrophy in brief.

Introduction

It is a rare inherited metabolic disorder in which the spinal cord and white matter are getting affected due to inappropriate growth or damage of myelin sheath which covers almost all nerve fibres of CNS and PNS. The accumulated sulfatide material appears as spherical granular masses 15-20 micro in diameter which stains positively with PAS and metachromatically with an acid cresyl violet stain using a frozen preparation. In the CNS, these masses may be either free in tissue spaces or within macrophages in the perivascular spaces. Axons and subcortical fibres are relatively spared. In the PNS, there is nerve fiber loss and segmental demyelination.

Cause

• Specific gene abnormality which causes white matter (myelin sheath) destruction of the brain.
• Genetic history or without genetic history a person develops this disease.

Risk Factor

• Family history of metachromatic leukodystrophy can increase the risk factor of this disease.

Symptoms

Metachromatic leukodystrophy usually becomes manifest clinically between the first and fourth year of life, although there are rare adult forms. Following are the symptoms:-

• Increase/lost muscle tone
• Tingling and numbness in hand or feet
• Burning or shooting pain in affected area
• Muscle weakness
• Movement changes
• Unable to eat
• Change in walking pattern
• Seizures may happen
• Abnormal eye movements
• Hear loss
• Speech loss
• Vision loss
• Behavioral changes
• Development of physical and mental health slowdown

Diagnosis

• The diagnosis is usually made by finding a decrease or absence of aryl sulfatase-A in the urine.
• Peripheral nerve biopsy can also be helpful in showing the sulfatide breakdown of myelin.

Ayurvedic Overview

In ayurveda we can correlate metachromatic leukodystrophy with imbalance of vata dosha and vija dusti (congenital factors coming from parents). Abnormal vata performs various afflictions to the body, thereby producing various illnesses. It also affects happiness, longevity and complexion. It alters the normal functioning of the brain, afflicts all sense faculties, deforms and destroys the embryo for long. As a result it gives rise to confusion, anxiety, fear and excessive delirium and finally stops the vital breath.

Treatment

• Ghee, oil, fat and marrow used as intake, massage and enema, unctuous fomentations, wind- free place, wrappers, meat soups, mild, sweet, sour and salty edibles and whatever is bulk-promoting is useful for the patients of vatika disorders.
• Poultices should be made with these very meats freed from bones, pounded and mixed with ghee, oil and sour things.
• Tubs filled with decoctions of leaves, milk and oil should be used for bath
• Sprinklings should also be used in patients of vata disorders after they are well-massaged.
• Meat of marshy and aquatic animals, dasamula, shatavari, kulattha, rasna, sesamum should be boiled with fat, sour curd in an earthen pitcher. With this tube formentation should be applied. The paste of these drugs should be used as poultice and ghee and oil cooked with the same should be used for massage and intake.
• There is no remedy for vatika disorders better than oil due to the presence of usna (hot), guru (dense), and snigdha (oily) properties. It becomes more effective after processing. Hence having been cooked with the drugs of vata alleviating groups hundred or thousand times it destroys the disorders located even in minute channels quickly.

Herbal Remedies for Metachromatic Leukodystrophy by Planet Ayurveda

Planet ayurveda provides a wide range of herbal medicines for many kinds of disorders. They follow strictly all the principles of ayurveda. They form different kinds of formulations like capsules, tablets, churna, leham, syrup etc. All the herbal medicines are GMP certified and 100% vegetarian. They show wonderful results in the human body and are safe to use. The formulations are made from pure, natural and effective herbs with no preservatives, additives or chemicals. At present there is no treatment for this disease, only supportive therapy will be given. Planet ayurveda provide herbal remedies for metachromatic leukodystrophy:-

1. SHILAJIT CAPSULES
2. BOSWELLIA+CURCUMIN
3. NEUROGENIE CAPSULES
4. ASHWAGANDHADI GHRIT
5. CHANDRAPRABHA VATI
6. VASANT KUSUMAKAR RAS

1. Shilajit Capsules

It is a single herbal capsule which is made from shilajit (asphaltum). It is sticky material found from Himalaya’s rocks. It is an antioxidant containing fulvic acid which prevents the tau protein (which damages the brain cell) hence reducing inflammation in neurological disorders. It has memory enhancing properties because it contains dibenzo-alpha-pyrones molecules which increase your thinking capacity. It lowers the blood sugar levels in the body. It also supports fertility. It has anti-ageing properties due to containing many minerals. It nourishes all the cells of our body.

Dose : 2 capsules twice daily, with plain water or milk after meals

2. Boswellia+Curcumin

It is a poly herbal capsule manufactured from shallaki (Boswellia serrata) and haridra (curcuma longa). Boswellia contains anti-inflammatory features which inhibit the cytokines secretion and help in reducing inflammation. It also has an antioxidant property which saves brain neurons and reduces the wrinkles on skin. It purifies the blood and helps in blood formation in the body. It also applies on cuts and bruises because it is a natural antibiotic. It prevents colon and skin tumors.

Dose : 1 capsule twice daily, after meals with plain water.

3. Neurogenie Capsules

It is polyherbal capsules made from ashwagandha (withania somnifera), and brahmi (bacopa monnieri). Ashwagandha is a tonic, potent spermatogenic and rejuvenating. It is sweet, bitter and astringent in taste and light, hot and unctuous in effect. It is a highly acclaimed herb for its tonic effect on the brain and the nervous system. It acts as an anti-stress and adaptogenic medicine. It has anti inflammatory, analgesic, aphrodisiac, and mildly sedative properties. It is also an immune-modulator, antioxidant and regulates all the physiological functions. Brahmi itself is a tonic for improving memory. It pacifies all three doshas mainly kapha and vata. It is also a tranquiliser, a muscle relaxant, an anticonvulsant, antipyretic and carminative. It is also a blood purifier

Dose: 1-2 Capsules twice daily, with plain water, after meals.

4. Ashwagandhadi Ghrit

It is a classical ayurvedic product made from ashwagandha (withania somnifera), bibhitaki (terminalia bellirica), cow’s ghee etc. This medicated ghee contains antioxidants. This acts as rasayan (rejuvenating), balya (tonic) and atishukrala (potent spermatogenic). It helps in nourishing all the cells of the body and is highly effective on the nervous system. This product reduces the inflammation from the body and provides sound sleep. This relaxes the brain.

Dose: 3 to 6 grams mixed with mishri or as guided by the physician.

5. Chandraprabha Vati

It is herbal tablets made from shilajit (asphaltum), guggul (commiphora mukul), haridra (curcuma longa), amla (emblica officinalis) etc. This herbal product is hot in effect. It pacifies all three doshas in the body. This contains calcium, iron, magnesium and a volatile oil. This is widely used in various male and female disorders. This helps in reducing pain, swelling and tenderness of inflamed joints. This also helps one in healing the ulcers. This also allays the stubborn and recurring infections of the urinary tract.

Dose : 1-2 tablets twice or thrice daily with lukewarm water

6. Vasant Kusmakkar Ras

It is a classical ayurvedic product made from bhasma of gold, bhasma of silver, bhasma of pearl, bhasma of coral etc. It acts as an anti-ageing and rejuvenative. It is widely used to treat frequent urination problems. It enhances skin complexion, memory, strength and concentration. It calms nervous excitement. It acts as a tranquilizer in case of depression or anxiety. It enhances the blood in the body.

Dose : 1 pill twice a day to be chewed.

Contact Planet Ayurveda Support Team to provide you the costing / ordering and delivery information at – costing.planetayurveda@gmail.com or Call at 0172-521-4030 (India), +91-172-521-4030 (Outside India) or Whatsapp at (+91) 9915-593-604

Conclusion

Metachromatic leukodystrophy is a lipid metabolism disorder in which the spinal cord and white matter get affected. In ayurveda it is correlated with vata dushti and vija dushti (congenital factors). There is no treatment for this inherited disease yet but in ayurveda we can manage this condition with some supportive therapy. We can use herbs like ashwagandha, brahmi, guggul, amla, haridra etc which is a tonic for our brain system and acts as an immune modulator. We can use different kinds of medicated oil/ghee to reduce vata because ghee is dense, oily and easily absorbs other herbs properties on its own.