Medical mystery: Headaches that needed more than pills to resolve

Hard work
The young woman’s headaches were increasingly sending her to the emergency department for help.

At age 30, Chelsea had been struggling with migraine headaches for years. Her primary doctor had tried giving her a number of migraine medications, cycling between standard therapies as one became less effective or another’s side effects became intolerable. Still, every few months she came to our emergency department when she could bear the pain no more, asking us for the more potent therapies in our arsenal.

At one time, Chelsea could go half a year between visits to the emergency department. But then her headache episodes seemed to be coming more frequently and more severely. The throbbing right-sided pressure could no longer be controlled with her host of home medications, and only the powerful intravenous agents we could administer helped. Multiple CT scans revealed no abnormalities, though she could get reliable pain relief at the hospital.

One day in early spring, Chelsea was back at our emergency department, asking for the IV cocktail that had brought her relief so consistently. But this time, our examination revealed an underlying problem that required immediate attention.

The emergency department doctor assigned to Chelsea noted that she was resting in bed and covering her eyes to block the bright fluorescent lights that made her headache even worse. Her vital signs were unremarkable — heart rate, blood pressure, respiratory rate, and temperature were all within normal ranges. She moved all of her extremities equally and her neurologic exam revealed no deficits, weakness, or other abnormalities. Her heart was strong, regular, and steady, and her lungs clear and equal.

But a closer look revealed a problem that was even more serious than her pain.


Solution

The one abnormality on Chelsea’s exam was quite literally right in front of us – specifically, just behind her eyes. The ED team performed a fundoscopic exam, where the ophthalmoscope (next to the otoscope used to look in ears, noses, and mouths) is maneuvered to examine the interior surface of the eye. The exam revealed papilledema, an unusual finding that is caused by swelling of the nerves leaving the brain. Papilledema is a sign of increased intracranial pressure.

A lumbar puncture (spinal tap) was immediately performed, confirming markedly elevated pressure of the fluid surrounding the brain and spinal cord. The finding suggested a diagnosis of pseudotumor cerebri, a rare and dangerous condition of elevated intracranial pressure that is also known as idiopathic intracranial hypertension (IIH). Without proper diagnosis and treatment, this can lead to worsening headaches and even permanent vision loss.

But Chelsea’s testing was not yet finished. An even rarer condition associated with elevated intracranial pressure is cerebral venous sinus thrombosis (CVST), a blood clot in the veins that drain blood from the brain. CVST can cause seizures, strokes, and even as much as a 30 percent mortality rate, some studies suggest.

Chelsea was taken for immediate venography, a special type of imaging using timed contrast dye injection paired with CT or MRI to make difficult-to-see blood vessels more evident.  The test was again positive, identifying a large blood clot blocking blood flow out of the brain and raising the pressure within her skull.

Because her condition was so dire, Chelsea was taken to surgery to have the clot removed.  She was monitored in the hospital for several days without any complications, and recovered fully. We hope that her headaches will now be controllable with prescribed medications.

It’s likely that her papilledema developed over some time, but was not immediately evident, in part because her symptoms were attributed to migraines. The takeaway message for patients is that persistent headaches can sometimes be quite serious, and should be evaluated by a neurologist.

Rick Pescatore, D.O., is assistant director of research, Department of Emergency Medicine, at Inspira Health Network in Vineland. He soon will join Crozer Health Network in Chester, as director of Emergency Medicine Research.