Mechanism of Disease
Very little is understood about the disease mechanisms for these disorders. It is believed that a person who develops one of these rare neuroimmune disorders likely has a genetic predisposition to autoimmunity, and that there are environmental factors that interact with these genetics to trigger the disease. The specific genetics in each of these disorders is not completely understood and environmental factors have not been clearly identified. In the case of Multiple Sclerosis (MS), a relationship to decreased levels of vitamin D and diminished exposure to sunlight are being considered, but no other factors are suspected for these other neuroimmune disorders. It is believed that the immune system response could be to a viral, bacterial or fungal infection, and in the case of TM, a significant number of people have flu-like symptoms, a respiratory infection, or a child might have an ear infection preceding their attack. This immune response might explain why the immune system was revved up. However, it does not explain why the immune system becomes dysfunctional and attacks ‘self.’ Additionally, no one understands why some people have a good recovery from an attack, while others have no recovery.
The central nervous system is separated and protected from foreign agents by the blood brain barrier. For the immune system to attack anywhere in the central nervous system, cells from the immune system have to pass through this barrier. Thus, in the case of these disorders, not only does the immune system become confused, it also has to find a way to cross this protective barrier to get to the brain, the spinal cord and/or the optic nerves. These mechanisms are not very well understood.