Boy With Frequent, Severe Unilateral Headaches

The Case:
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?

The dialogue: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.

The disorder with the next shortest attacks is paroxysmal hemicrania. This is another rare headache disorder and occurs predominantly in females; its attacks typically last between 2 and 25 minutes. Absolute response to indomethacin is another characteristic feature.

Cluster headache is a more common headache disorder and is characterized by more prolonged attacks-typically between 45 minutes and 3 hours. Cluster headache occurs mainly in men, usually those older than 30 years. Also, cluster headaches are the most severe headaches in terms of pain intensity.

A migraine headache with atypical features that presents similarly to the other headaches we have discussed would probably last between 4 hours and 4 days (a typical duration for migraine). The only feature that would make such a migraine atypical would be the presence of autonomic symptoms (lacrimation, nasal congestion, ptosis, or conjunctival injection).

Finally, hemicrania continua causes moderate to severe headache pain that is almost constant but has fluctuations in severity. Spikes of severe pain may last up to 60 minutes; patients generally do not consider themselves headache-free between the severe spikes.

Primary care doctor: The duration of my patient's headaches is most similar to that of cluster headache, but he is much younger than the typical affected patient.

Headache specialist: Yes, the clinical symptoms you described are very typical of cluster headache.¹ The only unusual feature is the patient's age-14 years. Because of this atypical aspect of the presentation, the patient has suffered from chronic cluster headaches for 1 Z\x years without proper management. During this time, migraine and tension-type headache have been diagnosed, and his headaches have been managed accordingly. Thus, the treatment he has received has not resulted in any improvement.

Primary care doctor: What treatment plan would you recommend?

Headache specialist: Although cluster headaches are short-lasting, they are very severe and disabling. Patients with this disorder require special attention and intensive treatment. Cluster headaches may be successfully managed with 100% oxygen by face mask. Some antimigraine abortive medications are also effective. However, because of the short duration of attacks, oral abortive medications are ineffective in many patients. Moreover, patients usually don't have any warning signs that let them know that a cluster attack is imminent; instead, the headache begins suddenly and the pain reaches its greatest intensity within 1 to 2 minutes. Thus, it is best to choose medications that start to act immediately. Subcutaneous sumatriptan injection (4 or 6 mg) might be a good choice.² However, in patients with chronic cluster headache, such as this boy, the regular use of triptans may cause a rebound phenomenon and is not recommended.

Short-term use of corticosteroids might be beneficial. I typically use prednisone, two 5-mg tablets qid for 1 week, followed by a taper in which the total daily dose is reduced by 5 mg every other day until the patient is no longer receiving any medication. Alternatively, I sometimes use parenteral methylprednisolone, 80 mg IM. Another option is intramuscular or intravenous dihydroergotamine. Intranasal lidocaine drops can be used as an adjunctive treatment.^3,4Primary care doctor: What are the options for prophylaxis in this patient?

Headache specialist: I would first try the calcium channel blocker verapamil.⁵ If that agent is not effective, I would recommend lithium carbonate. In addition, studies have demonstrated that valproic acid and topiramate are highly effective in this setting.^6,7

I have found that histamine desensitization is a very effective treatment for chronic cluster headache.¹ The patient is given 21 bottles of histamine phosphate intravenously, each of which has been diluted in 250 mL of 0.9 normal saline (2.75 mg of histamine phosphate in the first bottle, and 5.5 mg in each of the remaining 20 bottles). This solution is titrated according to the patient's tolerance, starting at 20 mL per hour and increasing the infusion rate every 15 to 30 minutes by 10 mL per hour, up to a maximum rate of 125 mL per hour. This protocol can be administered on an outpatient basis and may produce fast and prolonged remission.

Outcome of this case
The patient was given lithium, intranasal lidocaine drops, 100% oxygen by face mask, and methylprednisolone acetate, 80 mg IM (single dose), in combination with histamine desensitization per protocol. As a result, the patient experienced complete remission of his headaches.

 

References:

REFERENCES:

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;45:1069-1072.
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.