Practical strategy for detecting and relieving cluster headaches

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Applied Evidence

Practical strategy for detecting and relieving cluster headaches

J Fam Pract. 2005 December;54(12):1035-1040

By

Stephen M. Adams, MD

Diagnosis, too often delayed, is not difficult given the typical pattern, and treatment options work for most patients.

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References

1. Bahra A, May A, Goadsby PJ. Cluster headache: a prospective clinical study with diagnostic implications. Neurology 2002;58:354-361.

2. D’Andrea G, Terrazzino S, et al. Elevated levels of circulating trace amines in primary headaches. Neurology 2004;62:1701-1705.

3. Cohen AS, Goadsby PJ. Functional neuroimaging of primary headache disorders. Curr Neurol Neurosci Rep 2004;4:105-110.

4. Lipton RB, Bigal ME, Steiner TJ, Silberstein SD, Olesen J. Classification of primary headaches. Neurology 2004;63:427-435.

5. Bahra A, Goadsby PJ. Diagnostic delays and mis-management in cluster headache. Acta Neurol Scand 2004;109:175-179.

6. van Vliet JA, Eekers PJ, Haan J, Ferrari MD. Dutch RUSSH Study Group. Features involved in the diagnostic delay of cluster headache. J Neurol Neurosurg Psychiatry 2003;74:1123-1125.

7. Purdy RA, Kirby S. Headaches and brain tumors. Neurol Clin 2004;22:39-53.

8. Carter DM. Cluster headache mimics. Curr Pain Headache Rep 2004;8:133-139.

9. May A. Headaches with (ipsilateral) autonomic symptoms. J Neurol 2003;250:1273-1278.

10. Nilsson Remahl AI, Laudon Meyer E, Cordonnier C, et al. Placebo response in cluster headache trials: a review. Cephalalgia 2003;23:504-510.

11. Moore K. Cluster headache: the challenge of clinical trials. Curr Pain Headache Rep 2002;6:52-56.

12. Treatment of acute cluster headache with sumatriptan. The Sumatriptan Cluster Headache Study Group. N Engl J Med 1991;325:322-326.

13. van Vliet JA, Bahra A, Martin V, et al. Intranasal sumatriptan in cluster headache: randomized placebo controlled double-blind study. Neurology 2003;60:630-633.

14. Hardebo JE, Dahlof C. Sumatriptan nasal spray (20 mg/dose) in the acute treatment of cluster headache. Cephalalgia 1998;18:487-489.

15. Bahra A, Gawel MJ, Hardebo JE, Millson D, Breen SA, Goadsby PJ. Oral zolmitriptan is effective in the acute treatment of cluster headache. Neurology 2000;54:1832-1839.

16. Matharu MS, Levy MJ, Meeran K, Goadsby PJ. Subcutaneous octreotide in cluster headache: randomized placebo-controlled double-blind crossover study. Ann Neurol 2004;56:488-494.

17. Leone M, D’Amico D, Frediani F, et al. Verapamil in the prophylaxis of episodic cluster headache: a double bline study versus placebo. Neurology 2000;54:1382-1385.

18. Kudrow L. Response of cluster headache attacks to oxygen inhalation. Headache 1981;21:1-4.

19. Magnoux E, Zlotnik G. Outpatient intravenous dihydroergotamine for refractory cluster headache. Headache 2004;44:249-255.

20. Bussone G, Leone M, Peccarisi C, et al. Double blind comparison of lithium and verapamil in cluster headache prophylaxis. Headache 1990;30:411-417.

21. Zebenholzer K, Wober C, Vigl M, Wessely P. Eletriptan for the short-term prophylaxis of cluster headache. Headache 2004;44:361-364.

22. Fogan L. Treatment of cluster headache. A double-blind comparison of oxygen v air inhalation. Arch Neurol 1985;42:362-363.

23. Ekbom K, Monstad I, Prusinski A, Cole JA, Pilgrim AJ, Noronha D. Subcutaneous sumatriptan in the acute treatment of cluster headache: a dose comparison study. The Sumatriptan Cluster Headache Study Group. Acta Neurol Scand 1993;88:63-69.

24. Gobel H, Lindner V, Heinze A, Ribbat M, Deuschl G. Acute therapy for cluster headache with sumatriptan: findings of a one-year long-term study. Neurology 1998;51:908-911.

25. Physicians’ Desk Reference. 57th ed. Montvale, NJ: Thomson PDR; 2003;1544.-

26. DHE 45 package insert; Novartis Pharmaceuticals AG. 2002.

27. Rozen TD. Olanzapine as an abortive agent for cluster headache. Headache 2001;41:813-816.

28. Freitag FG. Cluster headache. Primary Care; Clinics in Office Practice 2004;31(no 2), June.

29. Russell MB. Epidemiology and genetics of cluster headache. Lancet Neurol 2004;3:279-283.

30. Blau JN, Engel HO. Individualizing treatment with verapamil for cluster headache patients. Headache 2004;44:1013-1018.

31. Leandri M, Luzzani M, Cruccu G, Gottlieb A. Drug-resistant cluster headache responding to gabapentin: a pilot study. Cephalalgia 2001;21:744-746.

32. Hering-Hanit R, Gadoth N. The use of baclofen in cluster headache. Curr Pain Headache Rep 2001;5:79-82.

33. D’Andrea G, Perini F, Granella F, Cananzi A, Sergi A. Efficacy of transdermal clonidine in short-term treatment of cluster headache: a pilot study. Cephalalgia 1995;15:430-433.

34. Lainez MJ, Pascual J, Pascual AM, Santonja JM, Ponz A, Salvador A. Topiramate in the prophylactic treatment of cluster headache. Headache 2003;43:784-789.

35. Hering R, Kuritzky A. Sodium valproate in the treatment of cluster headache: an open clinical trial. Cephalalgia 1989;9:195-198.

36. El Amrani M, Massiou H, Bousser MG. A negative trial of sodium valproate in cluster headache: methodological issues. Cephalalgia 2002;22:205-208.

37. Albertyn J, Barry R, Odendall CL. Cluster headache and the sympathetic nerve. Headache 2004;44:183-185.

Practice recommendations

Learn to recognize the distinctive pattern of cluster headaches that in most cases, even if some symptoms suggest migraine, will readily reveal this disorder (C).
100% oxygen by face mask and injected sumatriptan (Imitrex) are effective choices to terminate an acute attack (B).
Verapamil (Calan) or corticosteroids abort cluster cycles and prevent further attacks (B).

You interview a “migraine” sufferer who hopes to find the relief that has so far eluded him. During the history taking, he reports experiencing the premonitory aura typical of migraine as well as photophobia and occasionally nausea. One description, though, raises a question about the cause of his headaches: he says the intense pain recurs at roughly the same time of day and lasts for about 45 minutes; he is unable to concentrate on anything but the pain and he paces ceaselessly until it abates. The odds now favor a diagnosis of cluster headache.

Given that symptoms of migraine and cluster headaches overlap, and that migraine is more prevalent than cluster headache, recognizing the latter requires sensitivity to its key attributes described in this article.

Careful selection of therapeutic agents will hasten resolution of acute pain and prevent recurrences. Some drugs may be combined for quicker onset of action.

Telltale characteristics of cluster headaches

As shown in the opening example, cluster headache is diagnosed primarily by history (see Source of cluster headaches).

Recognize the pattern. Its most striking feature is the unmistakable pattern of repeated bouts of pain that “cluster” at the same time of the day or night.

Each attack is extremely intense and brief, typically lasting 15 to 180 minutes.

For patients with the episodic variety of cluster headaches, the pattern of repeated headaches may last days to weeks, then resolve spontaneously. The relatively few patients with the chronic form of the disorder experience an unending cycle of daily or near daily headaches.

Attacks may occur during the day or night, and often wake the patient from sleep.¹

Pain is always unilateral. Location of the pain varies among patients but is usually within the distribution of the trigeminal nerve. Almost all patients (92%) have retro-orbital pain, and most (70%) also have pain in the temporal region. Pain is present in the upper teeth, jaw, forehead, or cheek half of the time. Less common sites of pain are the ears, lower jaw, neck and shoulder.¹

FAST TRACK

Cluster headaches have a striking pattern of intense and brief attacks that recur at the same time of the day or night

Look for associated findings. Pain is accompanied by signs and symptoms of ipsilateral autonomic dysfunction. Lacrimation on the affected side is the most common associated feature. Rhinorrhea or a blocked nasal passage, red eye, and swelling or pallor of the forehead or cheek are often found bilaterally but are clinically dominant on the symptomatic side.

Restlessness occurs during an attack and the patient often prefers to pace about, in striking contrast to the migraine sufferer, who avoids activity so as not to exacerbate the pain. Half of cluster headache sufferers experience nausea, photophobia, or phonophobia during attacks. A smaller number (14%) report an aura similar to that of a migraine. Ninety percent of cluster headache patients who drink alcohol say it triggers headache while they are in the midst of a cluster cycle.¹

Delay in diagnosis common. The 2004 revision of the International Classification of Headache Disorders, reflects our improved understanding of and ability to identify these disorders.⁴ The mean time to diagnosis of cluster headache has decreased from 22 years in the 1960s to 2.6 years in the 1990s, reflecting much better recognition of the syndrome.⁵ Nevertheless most patients consult three primary care physicians before a diagnosis is made. The time between the first episode and diagnosis ranged from 1 week to 48 years (median 3 years) in one recent study.⁶

Overlap of migraine and cluster symptoms may lead to misdiagnosis. Factors contributing to diagnostic delay include photophobia or phonophobia, nausea, an episodic attack pattern and a younger age at onset (P<.01). Correct diagnosis is further complicated in that 26% of cluster headache suffers also report a history of migraine headaches.¹ The Key differentiating factor between the two headaches types is the predictable pattern of repeated, intense, brief head pain.

Rare underlying causes. A very few patients with headaches have brain tumors. Headache is present in 50% to 60% of newly diagnosed brain tumors, but is usually accompanied by other signs or symptoms. It is the only presenting symptom in approximately 8% of cases. Most headaches due to tumors are clinically similar to tension headache (77%), and some mimic migraine (9%). Rapidly growing tumors are more likely to be associated with constant unremitting headache. Rarely brain tumors may produce pain syndromes similar to cluster headache.⁷ Other causes of secondary cluster headache include infections, vascular abnormalities, and head trauma.