What is Syringomyelia?

Before MJ’s MRI discovery in January Dan and I had never heard of Chiari Malformation or Syringomyelia – let alone have any idea what it was. So to understand our journey I thought it best to share some information with you that we had a crash course in when our son’s condition was discovered and surgery was scheduled within 4 weeks.

MJ has been diagnosed with 2 new neurological conditions (Autism being his first neurological diagnosis in September 2012). The first new one he was diagnosed with Chiari Malformation after an MRI on January 11, 2013.

MJ’s second new (and 3^rd total) neurological condition is Syringomyelia – diagnosed on 2/7/2013. His brain surgery was performed to alleviate some of the symptoms and possible complications due to both of these diagnosis.

What is Syringomyelia?

Syringomyelia is damage to the spinal cord due to the formation of a fluid-filled area (called a syrinx) within the cord.

The central nervous system is made up of the brain and spinal cord. The brain functions to receive nerve impulses from the spinal cord and cranial nerves. The spinal cord contains the nerves that carry messages between the brain and the body. Spinal cord injury can occur when there is damage to the cells within the spinal cord or when the tracts of nerves that run up and down the spinal cord are severed. Also known as: Syrinx

• MJ has a syrinx on his spine from T4 to T9 as well as fluid from T9 to L1



Picture from Scripps Health San Diego

 

 


Picture from Chiari Institute

 

Causes

In MJ’s case the fluid buildup seen in syringomyelia is a result of a birth defect (specifically, "chiari malformation," in which part of the brain pushes down onto the spinal cord at the base of the skull).

The fluid-filled cavity usually begins in the neck area. It expands slowly, putting pressure on the spinal cord and slowly causing damage.

What are the symptoms? 

• Gradual loss of muscle mass (wasting, atrophy) – MJ was diagnosed in 2012 with low muscle tone.
• Headache
• Muscle function loss, loss of ability to use arms or legs
• Numbness or decreased sensation – MJ had periodic numbness and pain in his feet.
• Decreased sense of pain or temperature
• Lessened ability to sense that the skin is being touched
• Neck, shoulders, upper arms, trunk -- in a cape-like pattern
• Slowly, but progressively, gets worse
• Pain down the arms, neck, or into the upper back – MJ has/had neck pain and back pain
• Weakness (decreased muscle strength, independent of exercise) in the arms or legs – MJ has this
• Uncoordinated movement – MJ has/had this

Treatment

The goals of treatment are to stop the spinal cord damage from getting worse and to maximize functioning. Surgery to relieve pressure in the spinal cord may be appropriate. Physical therapy may be needed to maximize muscular function.

MJ had a Chiari Decompression surgery to return flow of spinal fluid and slow progression of the Syringomyelia.

Possible complications

Without treatment, the condition will lead to:

• Continued or progressive loss of neurologic function
• Permanent disability including paralysis 

Prognosis

Untreated, the disorder gets worse very slowly, but it eventually causes severe disability. Surgical Chiari decompression usually stops the progression of the disorder, with about 50% of people showing significant improvement in neurologic function after surgical decompression.

As with the Chiari - surgery does not take away MJ’s Syringomyelia – It simply alleviates some of the brain compression and slows the progression of permanent neurological damage.

We will need to continue monitoring MJ’s condition to ensure his syrinx does not continue to grow.

MJ will have increase risks of brain and spinal cord injuries and possible symptoms that may make daily life more difficult.

**Information used from the following sources:

Chiari Institute, Scripps Health San Diego