AppId is over the quota
On Wednesday, we challenged Well readers to figure out the diagnosis for a middle-aged woman with a pulsating whooshing sound in her head and a sharp stabbing pain on the left side of her neck and head. Nearly 400 readers wrote in with some very thoughtful assessments of this patient’s problem.
The correct diagnosis is…
Hemicrania continua
The only right answer we got came in around 11:15 a.m. from Sashank Prasad, a neuro-ophthalmologist from Brigham and Women’s Hospital in Boston. He says he sees a lot of headache patients because eye involvement is a common feature in many chronic headaches. It was a comment I had made to another reader, noting that the patient didn’t require surgery to get better, that helped him focus on hemicrania continua as the cause of this patient’s pain. One of the characteristics of this syndrome is that it is usually very sensitive to indomethacin, a type of medicine in the same family as ibuprofen and naproxen.
The Diagnosis:
Hemicrania continua is a type of daily headache first described in the early 1980s. It is characterized by the symptoms noted by this patient: persistent pain on one side of the head interspersed with episodes of much more severe pain that is often described as sharp or stabbing. The episodes are usually accompanied by other facial symptoms, including watery eyes, runny nose, eyelid swelling or constriction of the pupil.
Most patients with this type of headache improve when treated with indomethacin. A hemicrania continua headache will sometimes respond to other anti-inflammatory drugs — but response to indomethacin, in particular, is a defining characteristic of the syndrome.
It’s seen more commonly in women than in men and most commonly comes on in a patient’s 20s, though these headaches can start at any age.
How the Diagnosis Was Made:
When the patient came back to the office a few weeks later, I examined her and told her that I thought that she had something known as carotidynia, a pain syndrome caused by inflammation of the tissues of the carotid artery. The cause is unknown, but the condition most frequently occurs in patients with a history of migraine headaches. It is sometimes associated with an injury to the carotid, like a dissection or tumor, but several scans had not detected a problem like that. I had also read that carotidynia and pulsatile tinnitus were more common in patients with abnormal carotid arteries, and this patient, as I described in my previous post, had unusual twisting and meandering carotid arteries.
Carotidynia can usually be treated with medications used to prevent migraine headaches. The patient had already tried beta blockers, the most commonly used migraine preventing drug, but hadn’t tolerated it, so I suggested she try Topamax, a medication developed to prevent seizures, which has also been used successfully to prevent migraines. If these types of medications didn’t work, I told her, we could consider trying a nerve-blocking injection to the region. The patient left my office optimistic that finally she might have found a diagnosis and a treatment. She made an appointment to come back in a month.
A Lucky Break:
Meanwhile, back at the ranch, I was busy studying. Every 10 years internists have to take a test to maintain our certification with the American Board of Internal Medicine. It’s one way the board has to make sure we all stay up to date on the newest medical practices. I had been studying for the past 18 months to take this daylong test in November 2011.
As I was reading, I came across a reference to an unusual disease with a Victorian-sounding name. I didn’t remember it and went to Google to read more about it: hemicrania continua. The first site I clicked on was written by a patient who suffered from this disorder.
And suffer she had. Reading her symptoms was like talking with my patient. The headache was unilateral, constant, stabbing. As I moved on to the medical literature, I saw that my patient’s symptoms fulfilled all the diagnostic criteria for the diagnosis except for one. Patients with this disorder usually have eye symptoms like watery eyes, swollen eyelids or a unilateral constriction of the pupil.
Talking With the Patient:
Excited by my discovery, I couldn’t wait until our next appointment, so I called the patient. How was the Topamax working? I asked. Not so well, she told me. She had stopped taking it after a couple of weeks. It didn’t help the pain, and when she took it she felt “dumb as a rock.”
I told her that I had some new ideas about what might be causing her pain, but first I had a couple of questions. Did she have any eye watering or eyelid swelling when the pain in her head was most intense? Yes, she told me. Sometimes she felt as if she had a cold, just in her left eye. And did she ever notice anything different about the pupil in that eye? Yes, she said. When the pain was most severe she noticed that her pupils were often not the same size. No one had asked her about these symptoms, and they were so mild she hadn’t thought to mention them.
Now I was really excited. I explained my incidental finding and started her on a two-week course of indomethacin. If this was hemicrania continua, she would get better with this medication. I hung up the phone and mentally crossed my fingers.
How the Patient Fared:
A few weeks later we spoke again. How was she feeling? How was her headache? She laughed at the question. She felt great, she told me. Her voice was excited. Her joy was audible.
The headache was gone. Completely gone. She had taken the medicine for almost a week with no effect and had almost given up, when suddenly the headache just disappeared. Just like that. It was amazing, she told me.
Strangely, for reasons I can’t explain, the whooshing sound in her head also disappeared. It had been diminishing over the past several weeks, even before she started the new medication, and now she heard it only occasionally when she held her head in certain positions.She stopped the indomethacin after the two-week course. Her headaches hadn’t returned. But if they did, she told me, she knew what to do. She told me that she felt normal for the first time in years. Her blood pressure was well controlled on a single medication, but she’s hopeful that once she gets back into shape, she may not need it. In fact, she was getting ready to go for a hike. After not being able to exercise for years, she was working hard to get back into shape and back to her previous level of activity.
There’s a great line in baseball that I used to hear frequently quoted in my first career, when I was a television journalist. It was from Lefty Gomez, a New York Yankee. He said he’d rather be lucky than good. I guess that’s true in medicine. It was lucky I was studying. It was lucky I ran across this mention of this half-remembered disease. It’s humbling to know how easily I could have missed this diagnosis. Does it have to be a choice? Lucky or good? Frankly, I’d much rather be both.
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