A 31-year-old man has the sudden onset of right periorbital pain. The pain worsens over the next 5 minutes and then remains at an excruciating, deep, non-flucuating level for the next 45 minutes. He has a friend drive him to the emergency department. While waiting in line to be seen over the next hour, the pain gradually subsides. When seen by the physician's assistant, he is afebrile and normotensive with pulse 80/min and respiratory rate 15/min. The pain is unilateral. He has homolateral lacrimation, reddening of the right eye, nasal stuffiness, and right lid ptosis. He is nauseated.

Over the next 2 months, he has several more similar episodes, each involving the right side. However, the next episode does not occur until a year later.

Questions:

7.1 What is the most likely diagnosis?

He has cluster headache, a vascular headache syndrome characterized by periorbital or, less commonly, temporal pain that begins without warning and reaches a crescendo over 3 to 5 minutes. Attacks last from 30 minutes to 2 hours and remain at maximal levels for about 45 minutes before tapering off. Pain is often excruciating and is deep, nonfluctuating, and explosive in quality; only rarely is it pulsatile. This episodic pain is characterized by one to three short-lived attacks of periorbital pain per day over a 4- to 8-week period, followed by a pain-free interval that averages 1 year. The chronic form, which may begin de novo or several years after an episodic pattern has become established, is characterized by the absence of sustained periods of remission. Each type may transform into the other. The male:female ratio is 8:1.

7.2 Is pharmacologic therapy indicated?

Once an attack has started, administration of 100% oxygen can help. Preventive therapy can consist of several options. A 10-day course of prednisone may help end a cluster headache series. For chronic cluster headache, calcium channel blockers or lithium may be useful. If patients can learn when attacks are likely to occur, they can take ergotamine 1 to 2 hours before the predicted event.

7.3 What if he had a recurring headache building up over 4 to 72 hours, maintained for several hours to days, and relieved by sleep, characterized by a pulsating quality and unilateral location, nausea, vomiting, and/or other symptoms including photophobia, light-headedness, visual disturbances, paresthesias, and vertigo? What therapy would be indicated?

Migraine is the most common cause of vascular headache. About 15% of women and 6% of men are affected. Migraine can occur without aura (common migraine) or with aura (classic migraine). There are multiple theories for migraine etiology. Mild migraines may respond to NSAIDS. Moderate to severe migraines may be treated with triptans, because stimulation of 5-HT1 receptors can stop an acute migraine attack. Ergotamine and dihydroergotamine are nonselective receptor agonists, while triptans are selective 5-HT1 receptor agonists (e.g., naratriptan, rizatriptan, sumatriptan, zolmitriptan).