Rare disorder that causes vision loss is on the rise | Ascension

Rare disorder that causes vision loss is on the rise

Ciara Nobles experienced symptoms that progressed rapidly over a short period of time.

She started having migraines that wouldn’t go away with medication. That was followed by blurry vision and dizzy spells that increased in frequency and duration. And it only got worse when Nobles suddenly lost vision in one eye.

“I was at work looking at my computer screen when my left eye went completely black, '' said Nobles, 27, a medical assistant at Ascension Providence in Mobile. “I will never forget that day on December 13 because it was one of the scariest moments of my life.”

Nobles went from doctor to doctor, but no one could pinpoint the cause of her condition. The day she lost her vision, Nobles’ coworkers walked her across the street to an ophthalmologist’s office where he dilated her pupils to identify the reason for vision loss.

The ophthalmologist referred her to a local neurologist in Mobile. He conducted imaging studies that detected fluid buildup in her spine and brain. He recommended weekly spinal taps to remove the excess fluid. Nobles also consulted with an endocrinologist who was treating her for other health conditions.

When the fluid buildup and vision loss persisted for several months, Nobles was finally referred to Dr. Nathan Kohler, interventional neuroradiologist at Ascension Sacred Heart Advanced Brain and Spine Institute in Pensacola. He conducted diagnostic studies and found that she had intracranial hypertension, a disorder that causes a buildup of pressure around the brain.

Dr. Kohler said intracranial hypertension is an insidious disease because symptoms often mimic other health problems so patients often see different specialists before the correct diagnosis is confirmed.

“The disorder can occur when a narrowed vein leads to an accumulation of fluid in the brain and an increase in intracranial pressure,” Kohler explained. “The brain is like a toilet tank -- water only goes in or out one way -- but there’s no shut-off valve. When too much pressure builds up, it can damage the optic nerves.”

To release pressure, Dr. Kohler inserted a stent in Nobles’ brain to expand the narrowed vein. The procedure was done using a thin catheter inserted through a tiny incision in her upper leg and advanced up to the brain. The stent helps to regulate fluid in the vein to prevent buildup.

While intracranial hypertension is considered a rare disorder, Dr. Kohler said he’s seen a significant number of cases in the area. As the only neuroradiologist in the area performing this type of surgery, he’s performed 12 brain stent surgeries in the past year.

Nobles remembers the day she lost her vision and when it returned. One month after surgery, on April 7, she was driving her daughter to school when she started seeing flickers of light.

“I couldn’t believe that it was real,” she said. “”At first my vision was blurry, but as the weeks went by it returned. It’s been such a huge relief and weight lifted off my shoulders.”

For more information on Ascension Sacred Heart Brain and Spine Institute, visit ascension.org/sacredheartneuro.