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Case Studies Issue: Hemicrania Continua

By Ira M. Turner, M.D.
The Headache Care and Research Center, Island Neurological Associates, PC, Plainview, NY

THE CASE

Joan, a 35-year-old female with a nine-month history of continuous left-sided headache, was referred to a headache specialist. She described a pain of mild-to-moderate severity on the left side of her forehead, temporal region, and back of her head and neck, which was present from waking in the morning until going to sleep at night. There was also a dull pressure associated with the pain. Numerous times per day the pain worsened, becoming sharp and jabbing for several seconds at a time, and radiating from the left temporal region to the left eye. On some occasions these jabs and jolts were associated with tearing from the left eye.

Joan had no relief with various over-the-counter remedies, with the exception of naproxen which achieved a minimal improvement of the dull continuous pain. She had been treated with two courses of oral antibiotics without help and a CT of her sinuses was reported as unremarkable. Trials of sumatriptan, eletriptan and a preparation of butalbital and APAP (containing acetaminophen), were reportedly of no benefit.

The headache specialist performed a neurological examination, which was entirely normal. Blood tests were also unrevealing. An MRI of the brain and MR angiography of the cervical and intracranial vessels were normal as well.

Joan was diagnosed with hemicrania continua and prescribed indomethacin 25 mg three times per day. Two days later the headaches were gone. After two months of treatment, this therapy was gradually reduced and Joan has (so far) remained headache-free.

DISCUSSION

Hemicrania continua is an uncommon (but by no means rare) headache disorder. The exact prevalence is undetermined and many cases are never correctly diagnosed. It is more common in women than men, though there is disagreement as to the size of this gender gap. The combination of two headache types is common in hemicrania continua. There is always a continuous, strictly unilateral pain of moderate severity that never changes side. In other words, untreated patients have no headache-free intervals. Superimposed on the chronic pain are intermittent sharp or jabbing sensations (jabs and jolts) on the same side. These can recur multiple times per day.

A number of associated cranial symptoms are commonly present on the same side as the pain, particularly during the jabbing exacerbations. These symptoms are similar to, though less severe than, those that accompany cluster headache and can include tearing from the eye, redness of the eye, drooping of the eyelid, change in the size of the pupil, nasal congestion and nasal discharge. Some patients also experience some nausea, as well sensitivity to light and noise, as with migraine.

Hemicrania continua is exquisitely responsive to indomethacin. In fact, many physicians feel that a positive response to indomethacin is required in order to establish the diagnosis. Unfortunately, because of possible gastrointestinal and renal problems that may occur with this medication, long-term usage may be problematic. In cases where different medications must be tried, there have been reports of successful treatment with other nonsteroidal anti-inflammatory agents (although similar adverse effects may occur) and with lamotrigine and topiramate.

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