By Sandra G. Boodman
Special to The Washington Post
Right away, Lori White knew that something was very wrong.
The 44-year-old legal assistant at a Northern Virginia law firm had been working out with a personal trainer at her gym, executing a demanding and unfamiliar move. As she pulled down on a bar equipped with weights while simultaneously lunging forward, she felt an explosive pop in her head, immediately followed by a headache more crushing than any she had previously experienced.
For the next 10 minutes, White recalled, she sat on the floor, clutching her head and fearing she would throw up or pass out.
To her relief, the pain receded within a few hours. “I figured I’d just strained something,” she recalled. But within weeks of the 2005 episode, an alarming new problem surfaced: stabbing pains lasting five to 30 seconds in the front of her head, similar to the “brain freeze” that people sometimes experience while eating ice cream.
It took White three years to discover what had happened that day in the gym and two more to sort out what should be done about it – a confusing and sometimes contradictory process that involved specialists in the Washington area as well as Baltimore and Charlottesville, Va. Two weeks ago at Georgetown University Hospital, White underwent treatment that doctors hope will cure her problem.
“It’s been such a frustrating and painful battle,” she said, recalling the neurologists who speculated that her strange pains might be the result of caffeine, a cough or simply changing positions.
“For a long time,” she said, “I don’t think anyone really took me seriously.”
The piercing head pains seemed to be triggered by movement: coughing, sneezing, bending over, laughing or even singing. Sometimes a change in the weather or altitude would unleash them. Although intense, they were, at first, mercifully brief, and would vanish for weeks at a time.
After several months, White consulted a neurologist who practiced in Fairfax County, Va. The doctor performed a basic exam and took a history, but offered little in the way of advice or a diagnosis, White recalled.
A second neurologist, in Chevy Chase, Md., seemed similarly baffled. His advice: Avoid caffeine, which can make headaches worse, to see whether the problem cleared up.
It didn’t, so in 2008 White went to see a third neurologist. He ordered an MRI scan and told White she might have a “cough” headache, an unusual type triggered either by straining or, in some cases, by disorders involving the skull.
By then, White said, she worried that the cause of her headaches might be serious. When she received the MRI results, she saw a brief reference to a “borderline chiari.” After searching the Internet, she discovered that one of the causes of a cough headache is a Chiari 1 malformation of the skull.
These malformations occur when brain tissue from the cerebellum protrudes into the spinal canal, the result of a congenital deformity that might not appear until adulthood. In some cases the problem is mild, does not cause symptoms and is picked up as an incidental finding during imaging. In most cases the skull is abnormally small or misshapen, particularly at the back, according to the Mayo Clinic Web site, one of the sources White consulted. The protruding brain tissue can obstruct the flow of spinal fluid that bathes the brain and spinal cord, disrupting signals transmitted to other parts of the body and causing serious neurological problems, including paralysis.
Although unusual, Chiari malformations are more common than previously believed. Among those affected is singer Rosanne Cash, who underwent treatment for the condition three years ago.
Armed with pages of research, White returned to the third neurologist and asked him whether he thought a Chiari 1 malformation might account for her head pains.
White said the doctor looked at her MRI, glanced at the papers she had brought and dismissively replied, “It couldn’t be that.” He told her he had no idea what was causing her headaches and suggested she see a headache specialist.
“At this point,” White recalled, “I began to think I was losing my mind.”
She made an appointment with a Baltimore neurosurgeon for another opinion. He sent her for a second MRI and two weeks later called her with the results. During a brief conversation, he confirmed the Chiari diagnosis – the condition had in all likelihood been triggered by that gym exercise – and said he “would be happy to do surgery whenever you would like.”
White was flummoxed: When exactly did a person decide she wanted to undergo brain surgery? She had read that doctors do not necessarily operate on Chiari malformations, which are sometimes treated with medications and regular monitoring. And she wondered whether her problem might be caused by something else, particularly if the condition was “borderline,” as the first MRI indicated.
One of the decisions doctors and patients must make is when and whether to operate. Surgery involves cutting through the skull and carries obvious risks, including infection. But the disorder can be progressive, and waiting can result in complications that include the development of a syrinx, a cyst or cavity in the spinal cord that fills with fluid. In such cases, surgery is usually considered a necessity.
White made an appointment with a second neurosurgeon, this one at Georgetown, hoping to get a more definitive answer about what to do.
The specialist thought the location of her head pain – in the front, not the back, as is characteristic of Chiari – was puzzling. He wanted White to undergo further testing by a neuro-ophthalmologist, to rule out another condition first.
Those tests were negative, and last April White drove to Charlottesville to consult a third neurosurgeon to see if surgery was advisable. The specialist ordered an MRI of White’s cervical spine to check for evidence of progression, including a syrinx.
The Charlottesville doctor told her the tests revealed no syrinx and recommended she undergo a repeat MRI in a year. White said she then asked him for a drug to treat her head pain: In addition to the stabbing episodes, which occurred between 20 and 30 times a day, she had a constant headache. White said the surgeon looked at her uncomprehendingly before telling her he had not written a prescription in 25 years. He, too, suggested she see a headache specialist.
“I could not believe it,” she said. “Sometimes these pains would just bring me to my knees, and he treated me like I was just trying to get drugs. I was just so appalled by that.”
She headed home and visited a fifth neurologist, Mahan Chehrenama of the Neurology and Headache Treatment Center in Alexandria, Va. Chehrenama prescribed medications for the pain, examined the results of previous tests and ordered a different kind of MRI, this one designed to assess the flow of spinal fluid to check for blockages.
“She was the first doctor to really get it,” White said, relieved that someone was listening and answering her questions. Chehrenama told her that the latest scan had revealed a syrinx and that surgery within the next six months was necessary to prevent “an acute neurological event.” Without the operation, White could become paralyzed.
The procedure, called a decompression, would involve removing a small section of bone at the back of White’s skull as well as a portion of the top vertebrae, and opening the dura, the outermost membrane that covers the brain, to relieve pressure. A patch using a piece of cow heart would be sewn over the expanded opening; this would give the brain more room inside the skull, thereby restoring the proper flow of spinal fluid, eliminating the syrinx and, if all went well, hopefully, stopping her headaches.
So why had the MRI performed only weeks earlier failed to detect the syrinx?
“The study I did was different than the kind they did,” Chehrenama said. She regarded the location of White’s pains, which had puzzled several doctors, as inconsequential. “We see referred pain all the time,” she said.
Treating Chiari cases, said Chehrenama, who has seen 20 patients with the condition, can be tricky, because it requires balancing the risks of waiting against the risks of surgery. The syrinx tipped the balance: Without surgery White risked hydrocephalus, a buildup of fluid inside the skull that can have catastrophic consequences.
White finally thought she had the answer that had long eluded her: an unequivocal diagnosis and a definitive course of action.
She went back to Georgetown neurosurgeon Christopher Kalhorn, whom she had seen earlier and trusted, and scheduled surgery. The three-hour operation was performed Oct. 12; recovery is expected to take about two months.
“So far, so good,” White said last week. She still has a headache – to be expected for someone recuperating from brain surgery – but the brain-freeze pains have disappeared.
Although she found the differences of opinion and uncertainties about her diagnosis and treatment frustrating, the worst part, she said, was the dismissive attitude she too often encountered.
“I definitely learned that you have to be your own advocate,” White said. “If not, I could have wound up unable to walk.”