A 14-year-old boy presents with frequent severe headaches characterized by sharp, throbbing pain behind his left eye and left temple. He has not experienced headaches on the right side of his head. He rates the severity of the pain as 8 or 9 on a 10-point visual analog scale. The pain may last from 30 minutes to 2 hours, with an average duration of 45 to 60 minutes. He usually has 8 or 9 headache attacks per day, and they frequently wake him up between 2 and 3 am. He says that once a headache is gone, he is headache-free until the next episode. He denies having any warning signs or symptoms that would indicate an approaching headache and denies physical exertion as a trigger; he says that the headaches occur suddenly and reach their greatest severity within 2 to 3 minutes. His parents comment that during a headache attack he usually prefers to sit quietly in a chair, although occasionally, when the pain is extremely severe, he may rock or pace. They, too, do not recall any triggering or precipitating factors.
The patient experienced his first headache episode almost 1 1/2 years earlier. He has had multiple headache attacks daily from the beginning, and there have been no major changes in the headache pattern since then. At different times, tension-type headache and migraine have been diagnosed.
He has attention deficit hyperactivity disorder; he had mild asthma and somatoform disorder when he was younger. His paternal grandmother had migraine. His regular medications include escitalopram, 10 mg/d; topiramate, 200 mg in the morning and 100 mg at night; and amphetamine/dextroamphetamine, 20 mg/d.
Physical and neurological findings are normal. MRI and CT scans of the head performed within the past 3 months did not reveal any abnormalities. Recent magnetic resonance angiography (MRA) also shows no pathological findings.
• How are the organic causes of this boy's headaches best ruled out?
• What headache syndromes might explain his headaches?
• Is there effective treatment?
Primary care doctor: What would you include in the differential diagnosis of frequent severe headaches in a 14-year-old boy?
Headache specialist: Aneurysm of a brain blood vessel is the most dangerous cause of a strictly unilateral headache of sudden onset—and occasionally, early in the course of a headache caused by aneurysm, its symptoms can mimic those of cluster headache. Thus, aneurysm should be foremost in the differential diagnosis. In this patient, I would also consider those headache disorders that possess the following qualities:
• Unilateral location of the pain.
• Comparatively short duration of pain.
• Multiple occurrences throughout the day.
• Symptoms associated with the headache (photophobia, phonophobia, lacrimation, nasal congestion, ptosis, and conjunctival injection).
The following entities have all or almost all of these specific characteristics:
• Cluster headache.
• Hemicrania continua.
• Chronic paroxysmal hemicrania.
• SUNCT syndrome (Short-lasting Unilateral Neuralgiform headache attacks with Conjunctival injection and Tearing).
• Migraine with atypical presentation.
Primary care doctor: What steps would you take to narrow the differential diagnosis?
Headache specialist: This patient's history reveals that his headache pain is of comparatively short duration (45 to 60 minutes) and is not triggered by physical exertion. This is important information because headaches associated with brain aneurysms are almost always related to exertion. The negative results of MRA definitively rule out aneurysm.
Further evaluation with cranial angiography is warranted in questionable cases in which elements of the history suggest a brain aneurysm (eg, strong association of headache with physical exertion, new onset of strictly unilateral pain) and the results of MRA are not completely clear because of artifacts—or are equivocal because of blood vessel positioning or the presence of a possible blood vessel loop that resembles an aneurysm.
Primary care doctor: How would you distinguish among the remaining nonorganic headache disorders in the differential?
Headache specialist: The key distinguishing feature is the duration of the pain. The other clinical features of the disorders in the differential are very similar.
Primary care doctor: What is the typical duration of an attack in these various forms of headache?
Headache specialist: Attacks are shortest in SUNCT syndrome, one of the rarest headache disorders. These headache attacks last only 5 seconds to 3 minutes; however, they may occur as often as 100 times a day.
1. Freitag FG. Cluster headache. Prim Care. 2004;31:313-329.
2. Gregor N, Schlesiger C, Akova-OztŸrk E, et al. Treatment of cluster headache attacks with less than 6 mg subcutaneous sumatriptan. Headache. 2005;45:
3. Mills TM, Scoggin JA. Intranasal lidocaine for migraine and cluster headaches. Ann Pharmacother. 1997;31:914-915.
4. Schurks M, Kurth T, de Jesus J, et al. Cluster headache: clinical presentation, lifestyle features, and medical treatment. Headache. 2006;46:1246-1254.
5. Blau JN, Engel HO. Individualizing treatment with verapamil for cluster headache patients. Headache. 2004;44:1013-1018.
6. Gallagher RM, Mueller LL, Freitag FG. Divalproex sodium in the treatment of migraine and cluster headaches. J Am Osteopath Assoc. 2002;102:92-94.
7. Leone M, Dodick D, Rigamonti A, et al. Topiramate in cluster headache prophylaxis: an open trial. Cephalalgia. 2003;23:1001-1002.