The Chiari Malformation & Syringomyelia hidden neurological disorder - The Neurosurgery Center of Colorado

The Chiari Malformation & Syringomyelia, hidden neurological disorders – The Neurosurgery Center of Colorado

Posted by LIVING WELL Magazine on April 29, 2012 in Articles, Colorado, East Denver, Local Editions, Medical & Dental, Neurology

The Chiari Malformation & Syringomyelia: Neva’s struggle to overcome these hidden neurological disorders.

By John J. Oro, MD, The Neurosurgery Center of Colorado, East Denver LIVING WELL Magazine

Life was good. Neva was completing her graduate studies and had recently married her childhood sweetheart. Together, they enjoyed biking and hiking and were looking forward to starting a family. Then, the headaches began. At first, it was just a dull generalized ache, then, steadily, the pain concentrated in the back of her head. When getting up, she noted a brief dizziness. She pushed on, but other symptoms began to appear.

At an engagement with friends, she began to laugh. Oh, how she loved to laugh. But the headache stopped her short, and she started to avoid laughing. She noticed that lifting the laundry increased her headache and made her dizzy. Still, there were good days. Then, at the end of a vigorous bike ride, she developed an intense pressure in the back of her head that radiated behind her eyes. She doubled over in pain, her head feeling as though it would explode. She started hyperventilating and developed numbness around her lips. Deeply worried, her husband took her to the emergency room.

By the time they arrived at the ER, her headache began to ease, and her breathing rate returned to normal. The ER physician took a history and noted the increased demands on her life – a new marriage and pressure to complete her thesis. Her vital signs, neurological exam, and blood work were normal. She was diagnosed with a panic attack and an anxiety medication was prescribed. She went home, reduced her activity, avoided sports, and focused on her degree. She would have occasional headaches and dizziness, but they were mild in comparison.

A few months later, while looking in the upper cabinets of her kitchen, a strong feeling of dizziness and nausea overcame her. The pressure in the back of her head returned. Her arms became numb and her feet tingled. She went back to the ER where her vital signs, neurological exam, and blood work again were normal. Pain medication eased the headache. The history of her previous panic attack was reviewed, and she admitted that she had been pushing herself and not getting the rest she needed. She was diagnosed with anxiety and a psychological evaluation was recommended. Neva went home feeling irritated and did not schedule the visit with a psychologist. She coped with the recurring headaches by pulling away, staying in her bedroom, and waiting until the pain passed.

Soon, the stumbling began. The numbness in her arms and legs increased. She became moody and withdrew further. She made excuses to avoid her friends. Her personality was changing. She refused her husband’s suggestion to see the psychologist. Signs the marriage was under strain began to appear.

When she stumbled and fell, bruising her forehead and straining her neck, her husband had had enough. Back in the ER, the covering physician looked at the previous diagnoses of panic attack and anxiety. But the fall, neck pain, occipital headache, and stumbling suggested it was time to order a CT scan of her brain. Following a review of the scan, the radiologist recommended an MRI of her brain and cervical spine.

The diagnosis shocked everyone. There, in the center of her spinal cord was a large fluid filled cyst. It was surprising she could even walk. The radiologist also found a Chiari I malformation. The ER physician contacted the neurosurgeon on-call to discuss her condition. The neurosurgeon noted that the condition could be difficult to diagnose by symptoms alone and that some persons with symptoms were not diagnosed for several years. He also related that about 300,000 people in the U.S. have the Chiari I malformation, making the condition almost as common as multiple sclerosis.

The Chiari I malformation is a congenital crowding of the lower cerebellum and brainstem that can block the flow of spinal fluid from the brain cavity to the spinal canal. This can lead to malfunction of many neurological systems. While many affected persons have similar symptoms, the pattern often differs and can make the condition hard to recognize. In some persons, the blockage of spinal flow leads to the development of a fluid-filled cavity (a syrinx) within the spinal cord.

Neva was puzzled to learn the Chiari malformation had been present all her life. Maybe this explained why she had spent so much time in the nurse’s office for headaches while in grade school. There was little time for reflection. Neva searched for a center specializing in the Chiari malformation and syringomyelia and made arrangements to fly to Colorado for an appointment at The Chiari Care Center in Aurora.

During a two and a half-day visit, additional evaluations and studies were performed. Her scans were reviewed with her and her husband. She worried about the syrinx, but learned it would most likely decrease on its own once the crowding at the base of the skull was relieved. This was done through a surgical procedure, called posterior fossa decompression, performed at the back of the head to relieve the crowding and remove the pressure on the brain stem. She decided to proceed with surgery. After a three-hour operation and a few days in the hospital, Neva went home to recover with her family.

Seven years following surgery, Neva long ago completed her master’s degree and has a satisfying career. Now, when she and her husband go bike riding or go to the park, their two young daughters always accompanied them. Life, again, is good.

Contact The Neurosurgery Center of Colorado at 303-481-0035.

Oh my god, the first half of this reads exactly like my life up to this point. I'm in tears right now! I was busy with school, work, new marriage, starting my business... All my symptoms were either attributed to stress, anxiety, or fibromyalgia. My story is still in process - waiting for a neurosurgeon or neurologist to realize I need help. Hopefully my story will end like Neva's - with life being good again.