Motor Neuron

Motor Neuron

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We just don’t cure, we care

Worldwide, Motor Neuron Disease occurs in 1-5 out of 100,000 people. The disease takes a huge toll on not only patients but also their families and loved ones.

Stem cell therapy is a revolutionary treatment method that can give hope to those suffering from MND. After undergoing this therapy, a majority of our patients reported clearer and louder speech, and improved tongue movements, reduced choking, improved swallowing, reduced saliva drooling and increased respiratory capacity.

A few patients showed better neck control. Better limbic movements were reported in a few patients. Improvement was also reported in the lower extremity function, ambulation, fine motor activities, static and dynamic standing and sitting balance.

Using Kaplan-Meier survival analysis, it was found that the survival duration of patients treated with in trathecal autologous stem cell transplantation was longer than that of patients who were not given stem cell therapy.

These are a group of neurological, degenerative disorders that destroy motor neurons, the cells responsible for voluntary muscle activity. Voluntary muscle activity includes breathing, walking, eating, speaking, and swallowing. Messages from nerve cells in the brain (upper motor neurons) are sent to those in the brain stem and spinal cord (lower motor neurons). From here, messages are transmitted to specific muscles. So while upper motor neurons are responsible for directing the lower motor neurons to produce movement such as breathing or walking, lower motor neurons carry our these directions by controlling movement in the specific parts of the body such as tongue, limbs, chest etc.

This means, disruptions in muscle activity can be on two levels. Between upper motor neurons and lower motor neurons disruption in signal transmission can cause the muscles to become stiff, movements to become slow and knee and ankle jerks to become overactive. When disruption is between lower motor neurons and the muscle, the muscles slowly weaken and start to waste away. There may be uncontrollable twitching called fasciculation and eventually a person may no longer be able to control voluntary muscle movement.

Amyotrophic lateral sclerosis ( ALS A.K.A. Lou Gehrig disease) is the most common motor neuron disease, accounting for approximately 85% of motor neuron disease. Other MNDs are post-polio syndrome (PPS), primary lateral sclerosis (PLS), progressive bulbar palsy and progressive muscular atrophy.

As to why motor neurons begin to lose their functions is still not known. Motor Neuron disease may be inherited or sporadic. Amyotrophic lateral sclerosis which affects both upper and lower motor neurons has inherited and sporadic forms and affects the arms, legs, and facial muscles.

If the MND is passed on from the parents, it can be Autosomal dominant meaning that a person needs to inherit only one copy of the defective gene from one affected parent to be at risk of the disease or Autosomal recessive meaning that the individual must inherit a copy of the defective gene from both parents. Such parents themselves do not show symptoms of MND. In X-linked inheritance, a woman becomes the carrier of the defective gene and passes it to her sons. Some inherited forms of ALS are caused by mutations in the SOD1 gene. This gene helps protect cells from oxidative damage.

Factors that may be related to the development of MNDs are:

  • Abnormally high levels of glutamate, which passes messages between nerves
  • May defect immune response, as a result of which the immune system may damage the nerves that control muscles, leading to a loss of muscle control
  • Environmental factors such as infections, toxic substances, and dietary deficiencies.

Symptoms appear first in the arms and hands, legs, or swallowing muscles. Mostly, those affected by MND will first experience weakness of muscles that control speech and chewing. Gradually, muscles of the limbs lose strength and he individual is unable to hold the body upright. Symptoms may also include stiff muscles, spasms, cramps and twitching. Eventually, when muscles of the diaphragm and chest grow weak breathing becomes impossible without mechanical support.

Motor Neuron Disease is prevalent among adults and children. In children symptoms are visible at birth or before the walking stage. Motor Neuron Disease incidence is more among men than women.

The symptoms of MNDs vary with the progression of the disease. The symptoms fall into three categories:

  • The initial stage – The symptoms develop slowly and subtly over time
  • The advanced stage – this affects certain body parts and their functions. One experiences more incidences of muscle spasms, weakness, respiratory problems, pains, difficulty in swallowing and speaking, difficulty in walking and maintaining balance.
  • The end stage – this stage is mainly defined by body paralysis, severely affecting daily activities and shortness of breath

Additional symptoms due to the stress of the disease include depression, anxiety and insomnia.

Currently there is no cure or standard treatment for MNDs. In the absence of a cure for Motor Neuron Disease, supportive treatment is the best way forward in maintaining quality of life. Clinics that provide expert consultation in neurology, physiotherapy, respiratory therapy etc play a very important role for those suffering from MND.

Patients and their caretakers need a multidisciplinary team experienced in management of progressive neurological disability. The team should comprise a physiotherapist to advise on mobility, postural support, and prevention of contractures; a speech therapist; an occupational therapist to provide aids such as wheelchair, mobile arm etc to maintain function and a dietitian to for nutritional advice.

Medication such as muscle relaxants may relieve stiffness and help with treating panic attacks. Anti-convulsants and anti-inflammatory drugs may help relieve pain and antidepressants can be used to treat depression. Rilutor is the standard drug that is currently being used for MND. Stronger medicines such as morphine may be needed eventually to address musculoskeletal abnormalities or pain.

Stem Cell Therapy

Stem cell therapy holds great promise in the treatment of Motor Neuron Disease. Stem cells, with their ability to differentiate into neural and glial cells, are crucial in the regeneration of the motor neuron cells.

Cells derived from the bone marrow give a good source of stem cells required neural regeneration. They contain both hematopoietic as well as mesenchymal progenitor cells. These cells are known to travel to the spinal cord and plant successfully at the site of the disease. They also release certain messenger molecules that further enhance protection of the motor neuron cells and protect them from destruction.

Cellular therapy is widely tested. It has regarded to be beneficial and safe in MND. Post stem cell therapy, majority of our patients reported clearer and louder speech, and improved tongue movements. Other symptoms like improved swallowing, reduced choking, increase respiratory capacity and reduced saliva drooling. A few showed better neck control, a few exhibited improved limb function. These improvements helps patients gain independence and improve their quality of life.

Stem cell therapy in combination with standard drug treatment and rehabilitation may lead to slowing down or halting of the progression in MND, especially in the initial stages of the disease.

Role of Stem cells in treatment of MND:

  • Migrated stem cells help in tissue repair.
  • Cell replacement is brought about by stem cells.
  • Increased cell population.
  • Help make a better micro-environment for neuron survival
  • Release certain messenger molecules, which enhance protection of the motor neuron cells and protect them from destruction.
Dr. Alok Sharma

Consultant Neurosurgeon

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