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Central Nervous System - how is it affected by Marfan Syndrome?
Dural Ectasia
More than 60 percent of individuals with Marfan Syndrome experience dural ectasia.
The dura is the thin membrane made of connective tissue that covers the brain and the spinal cord. It contains spinal fluid called cerebrospinal fluid or CSF. Marfan Syndrome involves a weakening of connective tissue. Therefore, the dura in affected individuals is usually very weak. The membrane often swells or bulges under the pressure of spinal fluid. As the dura enlarges, it can push on the spine, widening the spinal canal or eroding the surrounding bones.
Individuals with Marfan Syndrome may go a long time without experiencing any of these symptoms. However, over time, the connective tissue in the central nervous system eventually weakens. The dura may end up rupturing and leaking spinal fluid into the body.
Symptoms include a pain or burning sensation in the stomach, lower back or legs. Headaches and a numbness or weakness in the legs are also common. When patients lie flat on their back, the symptoms usually decrease or disappear completely.
Degenerative Disk Disease
The backbone contains 26 bones that are called vertebrae. The soft disks between each vertebra in the back contain a substance that resembles jelly and provides cushioning. With age, these disks eventually wear down or shrink which is referred to as degenerative disk disease. This condition is also known as a herniated disk, slipped disc or ruptured disk. Lower back pain is most often caused by this condition.
Individuals with Marfan Syndrome can experience problems with degenerative disk disease at a much earlier age. The connective tissue starts to break down much faster than normal. The disks in their back may slip or rupture.
The disk can eventually wear down so much that they start to press against each other or against a nerve. This results in a very painful condition called a pinched nerve.
The worn disks can also form bony spurs. They can form in the space between discs or start to grow into the spinal canal. Patients will feel pain when the spurs start to press on the spinal cord or nerves.
Spinal Cysts
Spinal cysts are also very common for Marfan Syndrome patients. They are closed sacs filled with fluid that occur on the spine or in the facet joints. Facet joints are the small joints located behind and between each vertebrae to help stabilize your back. As a result of degeneration, fluid can build up over time. A small amount may eventually leak out and form cysts. Some cysts can apply pressure to the spinal cord and some will form inside the actual spinal cord.



