Transverse myelitis (TM) is a localized inflammatory disorder of the spinal cord that results in weakness, numbness and bowel, bladder and sexual dysfunction. It is a rare disorder with an incidence of between one and eight new case per million people a year. TM is one of a group of disorders in which the immune system becomes abnormally activated and “attacks” and injures the nervous system. The inflammation is confined to the spinal cord and usually occurs only once.
At Johns Hopkins, we have established the only transverse myelitis clinical and research center in the United States. We have dramatically improved the treatments and care of TM patients. As part of that effort, in 2001 we formed an international group of academic centers dedicated to defining the critical clinical features and natural history of TM. One of the immediate benefits of this group has been the publication of a critical paper in the medical literature which defined diagnostic criteria of TM and proposed a strategy for diagnosing and treating patients. This paper has been of great service to treating physicians throughout the world who have utilized it to rapidly diagnose and treat patients. We are aware of many examples where this approach has resulted in paralyzed patients returning to normal or near normal function.
Much work still remains to understand the natural history and the pathogenesis of transverse myelitis. There are three areas that require advanced understanding and research:
- A common underlying theme of ALL autoimmune diseases is that the central function of the normal immune system is to attack foreign substances and to ignore self tissue. The central derangement of the abnormal immune system is to attack self tissue. It is critical to understand the events that trigger the immune system to become abnormally activated. What are the “triggers” and what is the genetic susceptibility that renders some patients susceptible to that “trigger” while others are relatively resistant?
- Once the immune system is “tricked” into thinking that the nervous system is a “target,” how does it injure the brain and spinal cord? What are the cell injury pathways? How can we block them to render the nervous system resistant to injury during this attack from the immune system? We have begun to identify novel strategies that allow the nervous system to withstand the immunologic attack. These strategies will ultimately be applicable to a wide array of neurologic disorders, including Alzheimer’s disease, Parkinson’s disease, traumatic spinal cord injury and stroke.
- How do we restore function to patients with neurologic injury and paralysis as a result of transverse myelitis? Following the inflammatory injury, many patients are left with a spinal cord injury in which nerve cells in the spinal cord have died, leading to permanent weakness, loss of sensation and bowel and bladder dysfunction. Recently, we have learned how to direct stem cells to become motor neurons AND how to begin to direct them to reconnect with muscle. This represents the only hope for restoration of function in patients with long-standing paralysis. Recently, we and others have proved that this is a realistic goal, and we believe it WILL be achieved in the future.