Headache For Six Years Untill Correct Diagnosis Made
For six years, Lee J. Nelson searched in vain for the cause of the unrelenting headache centered like a bull’s-eye above the bridge of his nose.
He consulted nearly 60 doctors, none of whom could find a physical explanation for his pain. He took 100 different medications, but even powerful narcotics brought no more than temporary relief. One doctor who considered his headache a symptom of severe depression suggested electroshock; a specialist at Johns Hopkins proposed last-ditch brain surgery reserved for intractable psychiatric problems. So the day in 2003 that the Northern Virginia consultant found the answer to his baffling and rare medical problem in a 40-year-old article in the National Library of Medicine, he was overcome. “It described patients just like me,” Nelson recalled. “I started crying.”
For Nelson, now 55, and his wife, Neta, an executive at a small pharmaceutical company in Herndon, the discovery of that article in a British medical journal proved to be life-changing. It not only provided a diagnosis for a problem that had stumped dozens of specialists, but also described a surgical treatment for the malady that at times had driven Nelson to talk about suicide and his wife of nearly 30 years to contemplate divorce.
While Nelson credits Washington otolaryngologist Sonya Malekzadeh, one of the surgeons who performed an operation similar to the procedure outlined in the article, with saving his life, Malekzadeh regards Nelson’s unswerving determination as central to the case she considers among her most memorable.
“He was very persistent,” said Malekzadeh, an assistant professor of otolaryngology at Georgetown University who has co-written a paper about the case that has been submitted to a medical journal. “Without his help, I doubt we would have gotten to the end of it,” in part because his problem was so uncommon.
Ironically, it was also Nelson’s persistence — reflected in the extraordinary number of doctors he consulted — that may have prolonged his ordeal. “I saw just about every specialty,” he said, many only once because they offered neither “help nor hope.” “As the number got larger and larger I was inadvertently portraying desperation, and that’s a red flag,” Nelson noted. Doctors tend to regard such patients as having a psychiatric illness or angling for drugs.
One insurance company employee who reviewed his claims asked incredulously, “What is this: a hobby of yours?” Nelson recalled. His problem surfaced in the winter of 1997 after he recovered from a bad cold. He was left with a sharp headache, which his internist predicted would go away. When it didn’t, Nelson saw an otolaryngologist — the first of six — who ordered X-rays. They were normal. So were the MRIs and CT scans that followed.
Doctors subsequently ruled out a brain tumor, multiple sclerosis, chronic sinusitis, temporomandibular joint disorder, a nerve problem called atypical trigeminal neuralgia, epilepsy and migraines. “I kept saying this is an ENT [ear, nose and throat] problem,” Nelson said, “and ENTs kept saying there is no evidence of it.”
By the time he had seen about a dozen specialists, Nelson and his wife said, doctors had settled on a likely diagnosis: clinical depression. “I didn’t have other symptoms of depression, but as this went on, I did get deeply depressed,” Nelson said. He and his wife say they regarded depression as a consequence, not a cause, of his grinding headache. Nelson underwent psychotherapy and treatment that included numerous medications: anti-seizure drugs, antidepressants and even powerful antipsychotics. He tried biofeedback, acupuncture, hypnosis, Botox injections and a nerve block. He spent a total of six weeks in an inpatient head-pain unit affiliated with the University of Michigan. The couple say they spent tens of thousands of dollars that insurance didn’t cover.
The day an ophthalmologist at Johns Hopkins suggested cingulotomy, controversial psychosurgery reserved for intractable psychiatric problems, was arguably “the worst moment,” recalled Neta Nelson, who accompanied her husband to many appointments. “There were times I wondered, ‘How long can I do this?’ ” she remembered. “I cried many nights. There were times I would lie in bed, and just before I fell asleep I’d try and feel what it would be like to be Lee. It was a very difficult period.” Lee Nelson said he had pretty much given up — “I couldn’t tell the story again” — and planned to file for disability when his wife called Georgetown. Acting on her husband’s hunch that the problem was ENT-related, she sought an appointment with an otolaryngologist. Malekzadeh had the next open slot.
Lee Nelson vividly remembers their first meeting in November 2002. “She spent one and a half hours talking with me before she picked up an instrument,” he recalled. “I really felt like she was listening.” Malekzadeh remembers the meeting, too. “He was slightly desperate, very unhappy, but he seemed genuine, and he was there with his wife,” she recalled. “I didn’t think he was crazy or drug-seeking.” Her records show he was on a slew of medications at the time: the antipsychotic Seroquel, antidepressants, narcotic pain relievers, allergy medications and nasal steroid sprays. To her surprise, she was unable to examine the internal anatomy of his nose with an endoscope, a basic procedure that, surprisingly, none of the five other ENTs had attempted. His nasal passages were too narrow for the instrument.
After a variety of medicines failed to open them sufficiently, Malekzadeh told Nelson the best option might be exploratory sinus surgery. Two surgeries did reduce the pain. Unsure how to proceed, she sent Nelson to the University of Pennsylvania expert who had pioneered endoscopic sinus surgery. During the examination at Penn, a junior doctor probed a spot inside Nelson’s nose, eliciting an immediate response. “I almost flew out of the chair,” Nelson recalled. “I said, ‘That’s it! That’s the pain generator!’ The nugget I got was the name of the anatomical structure: the anterior ethmoid neurovascular complex.” The Philadelphia specialist told the couple he didn’t think it was germane. Neta Nelson insisted they head to the National Library of Medicine.
There on microfiche they found the 1963 article in the British Journal of Laryngology & Otology. A London surgeon described five cases very similar to Nelson’s in which a respiratory infection had caused swelling and compression of the ethmoid nerve in the sinuses, triggering a headache. The only permanent treatment appeared to involve removing bony tissue, thereby freeing the nerve from compression. “It was pretty classic,” said Malekzadeh, who had heard about such a problem during her training but had never seen a case.
On July 1, 2004, she and her colleague Suzette Mikula operated on Nelson. The pain, Nelson said, gradually lessened. “One day in January 2005 I woke up and realized there was no more pain. That was life-changing.” He hasn’t had the headache since. Although elated, Nelson said his recovery “got me into a whole other place of anger and frustration: Why did I have to go through this?” Malekzadeh considers it “a very gratifying case.” To Neta Nelson, the ordeal illustrates the importance of “never giving up. That’s the only take-home message.”
Lee Nelson said the experience has radically altered his view of doctors. “I’m very thankful I had the [financial] resources and the gray matter to do what I did,” he said. “But I think that a lot of physicians have lost their intellectual curiosity and don’t want to work with a patient.”
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