Spinal Muscular Atrophy is a  hereditary neuromuscular disease affecting the lower motor neurons. Weakness and atrophy(wasting)is caused by progressive degeneration of anterior horn cells of the spinal cord. In people with SMA, the proximal muscles—those closest to the center of your body—tend to be weaker than the distal muscles, which are located farther from the center. With time, muscle weakness usually gets worse. In AYURVEDA  this condition can be correlated to ‘MAMSA KSHAYA’.

SMA CLASSIFICATION

SMA is divided into five subtypes. Medical professionals categorize them according to life expectancy, severity, and age of onset. The subtypes are:

• Congenital SMA, or SMA type 0, is an uncommon form that affects a fetus prior to birth. Fetal movements usually decrease prior to birth. Infants with type 0 diabetes usually have respiratory failure and severe muscular weakness at birth. Most deaths occur either at birth or in the first month of life.

 

• SMA type 1 (severe SMA): Also known as Werdnig-Hoffman illness, type 1 accounts for about 60% of SMA cases. The first six months of infancy are characterized by symptoms such as hypotonia, or reduced muscular tone, and inadequate head control. Breathing and eating difficulties are also experienced by infants with type 1 SMA. Children diagnosed with type 1 SMA pass away before turning 2 if they do not receive breathing support.

 

• SMA type 2 (intermediate SMA): Between the ages of six and eighteen months, type 2 SMA symptoms (also known as Dubowitz disease) manifest. Hypotonia and progressively deteriorating muscle weakness are among the symptoms, with the affected legs being more affected than the arms. Type 2 SMA children may be able to sit up, but they are unable to walk. Seventy percent of type 2 diabetics will live to be 25 years old, and some will live into their 30s. The primary cause of death is respiratory disorders.

 

• SMA type 3 (mild): Following a child’s first 18 months of life, symptoms of type 3 SMA, also known as Kugelberg-Welander disease, start to appear. Type 3 symptoms include weakening in the muscles of the lower limbs, which makes walking difficult. Breathing problems are rare in people with type 3 MSA, and life expectancy is usually unaffected.

 

• SMA type 4(Adult):The mildest variant of SMA is( adult )type 4. Usually, it doesn’t show up until after the age of 21. Since the signs of muscle weakness develop gradually, most type 4 patients are still ambulatory. Usually, it has no effect on life expectancy.

ETIOLOGY  OF SMA

Since SMA is a hereditary disorder, for all forms of spine muscular atrophy, mutations (alterations) in the SMN1 (survivor motor neuron 1) gene are responsible. The severity of your illness depends on how many copies of the SMN2 gene you have.

SMN Protein is produced by a functional SMN1 gene . This protein is necessary for motor neurons to live and perform their functions. Your body produces less SMN protein when you have SMA, which causes your motor neurons to shrink and eventually die. Your brain is therefore unable to regulate voluntary motions, particularly those of your head, neck, chest, and legs.

 DIAGNOSIS OF SMA

The main tests involved are:

• Genetic Testing: Through the identification of SMN1 gene abnormalities, this blood test can confirm 95% of SMA cases.
• Electromyogram(EMG) or Nerve Conduction Study: Our muscles and nerves’ electrical activity is measured by these tests.
• Creatine Kinase Blood Test: This enzyme is released into your circulation by deteriorating muscles. However, in contrast to other neuromuscular illnesses, the levels are usually normal with SMA. type 1 but slightly elevated or normal  with remaining types.

ETIOLOGY AND SYMPTOMS OF SMA IN AYURVEDA(MAMSA KSHAYA)

Mamsakshaya is caused by beejabhaga avayava dushti (genetic causes/ chromosomes  is affected), which is why it is incurable from birth. Agnimandya, or the diminished ability of muscle tissue (mamsa dhatu) to change energy, will be present, resulting in disordered dhatu poshana (tissue nourishment). Vata will be vitiated since it is unable to synchronize the body’s regular functions.

The main issue is that they fail to thrive. Even if the patient lives, they will struggle to carry out their everyday tasks, such as walking, climbing stairs, and swallowing. Bala kshaya, Karma kshaya, Varna hani, Ojo kshaya, and Marana are some of the symptoms caused by  Beeja Dosha Janya Vyadhi(beeja means ovum and sperm) Udanavruta Prana Vata  and Majja Gata Vata.

A few symptoms, such as Anganam sosha (limb atrophy or emaciation), Sankocha (contraction), Kanja, Pangulya and Kubjatva (hand and foot lameness, hunchback, and shortness), are taken into consideration from Vata vyadhi Lakshanas, and few of these symptoms are also seen in spinal muscular atrophy.

TREATMENT OF SMA IN  AYURVEDA

The patient should be treated with Avaranahara Chikitsa in accordance with Ayurvedic principles, and then the Brimhana course of management should be followed.

EXTERNAL MEDICATION

• أودوارثانام

أودوارتانام is the upward massage with herbal powders and given in mild form, for the purpose of initial rookshana karma. It helps in movement of limbs and joints and maintains tri dosha equilibrium in the body. It’s usually done with JEEVANTHYADI CHURNAM,TRIPHALA CHOORNA etc. It also aids in detoxification and improved circulation.

 

 

• PODIKIZHI

Podi  كيزي is also given in a very mild form, which helps in joint mobility and flexibility, detoxification , circulation ,strengthening of muscles and nervous system etc.

 

 

• ABHYANGA

الأبيانغا is the massage done with medicinal oil. Here SAHACHARADI TAILA ,MAHAMASHA TAILA,BALALAKSHADI TAILA etc can be used. It also helps in balancing tridosha, enhance lymphatic stimulation ,eliminating fatigue, helps attain  a feeling of well being.

 

 

• نجاوارا كيزي

Njavara kizhi, or Shashtika Shali Pinda Sweda, is a traditional medicine made from heated boluses containing milk, brown rice and a variety of medicinal plants. It helps in the nourishment of body.

 

• MAMSA KIZHI

Goat meat along with njavara/brown rice ,milk, medicinal drugs is boiled to make  medicinal bolus. This process is special because it includes Brimhana (nourishing) in addition to Snehana (oleation) and Snehayukta Swedana (sudation).

 

 

• VASTHI

فاستي is the installation of medical enema. MUSTHADI RAJA YAPANA VASTHI,ANUVASANAM WITH DHANWANTHARAM OR SAHACHARADI VASTHI PAKAM is highly beneficial.

 

 

• NASAYAM

ناسيام is the administration of medicines through nostrils. KSHEERA BALA AND KARPASASTHYADI THAILAM is best in this disease.

 

 

• PIZHICHIL

During Pizhichil treatment, the patient’s diseased areas are squeezed with a special linen cloth that has been soaked into heated Kuzhambu, or a therapeutic oil mixture. PRABHANJANAM TAILA,SAHACHARADI TAILA,PANCHASNEHA TAILA are highly useful for it.

 

INTERNAL MEDICATION

KASHAYA KALPANA

• Indukantham Kashayam
• Ashtavargam Kashayam 
• Sahacharadi Kashayam
• Dhanadanayanadi Kashayam 
• Prasaranyadi ksheera kashayam (later stage)
• Drakshadi kashayam

GHRITHA KALPANA

• Indukantham Ghritham
• Narasimha Ghritham
• Mahasneha Ghritham

TAILA KALPANA

• Sahacharadi Vasthi Pakam
• Rasna Dashamooladi Thailam

 VATI KALPANA

• Vilwadi Gulika

PHYSICAL THERAPY IN SMA

Physiotherapy in SMA patients:

• Muscle strengthening exercises
• Coordination and balance exercises
• Range of motion exercises
• Walking exercises
• General conditioning exercises(whole body exercise)

SPEECH  THERAPY IN SMA

Activities used in speech therapy include, for instance:

• Exercises for the tongue and mouth: Your speech therapist will demonstrate movements and exercises to help you strengthen and coordinated movements of  your tongue and mouth. 
• Face expressions: Mastering facial expression control ,helps enhance your motor abilities. Your therapist may ask you to pucker your lips or grin before telling you to relax.
• Reading aloud can help to improve the communication between your mouth and brain if your speech impairment inhibits you from using your tongue and mouth in the correct ways.
• Playing word games: Research has indicated that crossword puzzles, word searches, and memory games help preserve cognitive function and sharpen thinking abilities.

SWALLOWING THERAPY IN SMA

• Conventional Swallowing Exercises: Your doctor will provide you with a customized regimen of throat exercises aimed at improving your weaknesses.
• Assessing and strengthening tongue strength is done with lingual manometry. Physicians can provide patients biofeedback as they exercise by using a pressure-sensing device.
• Pharyngeal Manometry: This innovative therapy assesses muscle strength and offers real-time biofeedback to enhance exercise performance using a pressure-sensing catheter.
• Endoscopic Biofeedback: The throat can be seen by inserting an endoscope through the nose. After that, patients view the real-time performance and results while practicing swallowing exercises and eating and drinking.

DIET AND NUTRITION

• Balanced diet and appropriate nutrition helps to maintain strength and weight.
• SMA patients will usually have deficiency of VITAMIN A,D ,E and K.So food and supplements compensating this deficiency should be taken. 
• Quercetin rich food is also highly beneficial.
• Electrolytes should be balanced, like calcium, magnesium, potassium, chloride etc seems highly beneficial.
• AVOID-Sugary food, Acidic food, High Sodium food, Trans fat, Too much Fasting in SMA.

 

DISCLAIMER: The information provided in this article is intended solely for educational  purposes. Treatment decisions should be made exclusively by a well-qualified Ayurvedic physician. Self-medication is strongly discouraged.