Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig’s Disease and Motor Neuron Disease, is a chronic inflammatory degenerative disease of the central nervous system, which continuously destroys nerve cells (motor neurons) that are responsible for movement. Due to the degeneration of nerve cells spasticity, muscle weakness, and further muscle atrophy occur.
The actual underlying cause is unknown, in some forms of ALS a certain hereditary disposition seems to exist, but there is also a sporadic form. In the endemic form there is a massive clustering in certain geographic regions (e.g., on the island Guam). ALS is a very rare disease; according to statistics one in 100,000 people is affected.
Time and again an association with the uptake of aluminum is assumed, but this is strictly denied by manufactures of beverage cans. It may be that external factors cause a chronic inflammation in people with inherited tendency to suffer from ALS, whereby immune cells attack and destroy nerve cells.
The progression of the disease runs at a different pace, that means, in early stages of the disease local nerve damages occur, which spread throughout the nerves that are left in the body. The progression decides on the prognosis.
Primary Lateral Sclerosis (PLS) is a neurodegenerative disease of the upper motoneurons that is regarded as a benign form of ALS. In contrast to ALS the life expectancy is not impaired and the course of disease mostly turns out to be rather benign. The cause may possibly be a faulty autoimmune reaction. The disease is not genetically predisposed, mostly begins between the age of 40 and 60 and manifests itself as a slowly progressing pyramidal tract syndrome. The first symptoms often are leg cramps, sometimes also language disorders and dysphagia. Within its course PLS can result in paralysis of the arms and legs; compulsive laughing and crying can also occur. As in ALS, apart from a purely symptomatic therapy, a treatment trial with autologous stem cells comes into consideration.
Symptoms and Progression of ALS
Due to the increasing movement disorders, swallowing and speech difficulties, as well as weakness and loss of movement control of arms and legs increasing restrictions of the patient’s quality of life arise. In principle all muscles can be affected except the sphincter, eye, and heart muscles. The sensation of sensual impressions such as pain, touch, temperature, taste, or sound normally remains unimpaired.
Therapy of ALS
The conventional medicine currently does not provide cure and the therapy is limited to the relief of symptoms. Death is often caused by an inflammation of the lungs, which is further promoted by the paralysis of respiratory muscles and the loss of swallowing functions. The usual remaining life expectancy after the diagnosis of ALS normally comes to 3 to 5 years.
To achieve a better quality of life the disease mechanism is counteracted medicinally with the administration of Riluzol.
Symptomatic treatment contains physiotherapy, mostly combined with occupational therapy, and speech therapy. The purpose of physiotherapy is to gather the remaining muscle strength and combat nervous muscle undersupply by stimulating areas affected by movement.
Speech therapy should improve the comprehensibility of language and support the swallowing function. Respiratory muscles are as well selectively strengthened and headed for. The occupational therapy in ALS patients especially concentrates on the support of hand functions.
Sooner or later the patient will, however, reach a situation of the highest level of care, including artificial ventilation. To guarantee the patient’s autonomy it is therefore recommendable to timely issue a patient decree.
Stem Cell Therapy of ALS
Mesenchymal Stem Cells (MSC) or rather Stromal Vascular Fraction (SVF) obtained from the patient’s own fat tissue can be used for experimental treatment of ALS. Stem cells are considered as the precursor cells of all other completed body cells. Today we know that the mode of action of mesenchymal stem cells is mainly in the modulation of the immune system and the stimulation of regeneration of tissues and blood vessels triggered by cytokines.
Stem cells have shown promising results also in several other autoimmune diseases and ALS both has an inflammatory and a degenerative component. Thus the application of stem cells in the therapy of amyotrophic lateral sclerosis proposes itself. Studies give hope that injecting mesenchymal stem cells into the areas affected or by systemic application a decrease of progression can be achieved. The stem cells could encourage stationary nerve stem cells to regenerate via cytokines and thereby counteract the destruction of further motor neurons.
In general, nerve cells can be cultivated from stem cells “artificially” in the laboratory. However, the question is how this can be realized inside the human body and to what extent stem cells have an effect on the progression of ALS.