Advanced Options for Rare Neurological Disorder

April Archote, 39, had a headache that just wouldn't go away. It felt like a vise tightening around her skull. Despite the pain, she managed to perform her job as a dental assistant in Arkansas. That is, until she started having trouble seeing the tips of her instruments.

It was June 2013 when April went to a local optometrist for an exam. She expected to get a new eyeglass prescription she hoped would reduce the headaches. Instead, the doctor told her she might have a brain tumor or a neurological disorder called idiopathic intracranial hypertension (IIH), sometimes known as pseudotumor cerebri.

Fortunately, April did not have a brain tumor. But her brain was acting as if she did. The telltale signs of IIH are similar to those of a brain tumor: blurry vision, swelling of the optic nerve, pulsating sounds or tinnitus within the head and headaches.

For an unknown reason, the two dural sinuses in April's brain were filling up with too much blood from excess spinal fluid. Spinal fluid naturally protects the brain and spinal cord. In April's case, the extreme pressure caused incapacitating symptoms.

As if the physical suffering wasn't enough, April experienced the added frustration that comes with having a rare condition that is often unrecognized in the medical community. Though recognition of IIH – often misdiagnosed – was an important first step for April, receiving effective treatment would prove a grueling journey.

Over the next 4 years, April sought help from several specialists. Physicians prescribed various treatment regimens. Some were ineffective. Others caused problematic side effects.

"Neurologists gave me pain meds for headaches," explains April. "They told me losing weight could help. I lost 60 pounds and my symptoms got worse."

April tried a medication commonly prescribed for patients with IIH. It caused an allergic reaction that led to a stroke. It took weeks of intensive therapy for April to regain her basic skills, and then, less than a year later, she suffered a second stroke.

"That's when my daughter and I moved back to Clever, Missouri, to live with my parents," she says.

Finally, in January 2017, the pressure in April's head grew so severe she was admitted to a hospital in nearby Springfield, Missouri. After 6 days without improvement, one of the physicians enlisted the support of the expert neurology team at The University of Kansas Health System.

April was transported there by ambulance. Neurologist Michael Rippee, MD, was waiting for her. Neuro-interventionalist Michael Abraham, MD, arrived soon after.

"They said I needed an emergency magnetic resonance angiogram (MRA) of my brain," remembers April. "It showed severe stenosis, or narrowing, of the two large sinuses of my brain. No one had ever told me that before."

April also underwent a lumbar puncture, often called a spinal tap. A needle was inserted between two vertebrae in her lower back to remove a small sample of cerebrospinal fluid. The spinal tap allowed technicians to measure the pressure inside April's skull. It was dangerously high.

Dr. Abraham scheduled April for surgery the next day.

One surgical option for IIH patients is a shunt that drains excess fluid from the spinal cord into the abdominal cavity, relieving pressure. But shunts have several downsides. They can cause infection or malfunction, require periodic replacement, and may not provide sufficient symptom relief.

"For April, I recommended a stent," says Dr. Abraham. "This minimally invasive approach involves guiding a metal stent up to the brain through an incision in the groin. Once in place, the stent is expanded, broadening the affected vein to improve blood flow."

The neurology team at The University of Kansas Health System has performed venous stenting for more than 70 IIH patients in the last 5 years. Not every patient is a candidate for the procedure.

April calls the procedure a success.

"It's been life-changing," she says. "I had a headache for 4 years, couldn't get out of bed, couldn't get around. Today, 1 year after the stent, I feel 75% better."

According to Dr. Abraham, "We hope the stent will prevent further vision loss and eliminate the turbulent blood flow that causes the whooshing sounds. But April needs to be monitored closely for vision changes."

That's why he referred April to the health system's neuro-opthalmologist, Thomas Whittaker, MD. He is well versed in April's condition and routinely checks her optic nerve for swelling, called papilledema.

Dr. Whittaker explains, "Because IIH can cause more than just headaches, patients benefit from the multidisciplinary approach at The University of Kansas Health System. We collaborate to ensure all of April's health concerns are addressed."

Thanks to the stenting, April's condition is under control, though she still has episodes of pain and vision disturbance.

"When I get sick with the flu or the barometric pressure changes, the pressure increases in my head," she says.

She knows she can call Dr. Abraham when she needs to. "He orders a spinal tap at a hospital near me, so I don't have to travel to Kansas City. The entire team is so willing to work with other healthcare facilities and doctors. They want what's best for the patient."

A spinal tap serves two purposes for someone with IIH. It not only measures the level of cerebrospinal fluid in the brain, it also drains excess fluid from the body. April says, "It's uncomfortable for a few minutes, but the relief is definitely worth it."

After an article about April appeared in her local paper, several people with IIH reached out to her. Today, she runs a support group that includes 35 people.

April encourages everyone with IIH to go see Dr. Abraham at The University of Kansas Health System, which was chosen as a site for the national multicenter clinical trial on surgical treatment of IIH set to begin shortly.

"Most people think they have to go to hospitals on the east coast," she says. "But Dr. Abraham and his team are performing these surgeries right here, close to home in Kansas City."