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cluster headache (suicide headache)

Classification: - most common of the trigeminal autonomic cephalgias Etiology: - smoking is a risk factor Epidemiology: 1) primarily affects men (72%) [3] - females may be more severely affected than men [13] 2) onset generally in late 20s, but may occur at any age Clinical manifestations: 1) main feature is periodicity a) cluster period lasts 2-3 months; 6-8 weeks [5] b) recurs every 1-2 years; 2-6 months [5] c) average remission is about 2 years d) may occur seasonally [5] e) 20% of patients do not have remission [3] or remission does not last > 1 month 2) attacks of 15 minutes to 3 hours duration - occur 1-8 times daily (mean duration 1 hour [5]) - longest duration of the trigeminal autonomic cephalgias - headaches repeat for 6-8 weeks, then disapper for months to years 3) nocturnal attacks occur in > 50% 4) not associated with aura 5) pain reaches a peak in about 10-15 min & lasts 45-60 min 6) pain [3] a) generally unilateral, excruciating, penetrating, non-throbbing & maximally behind eye b) retro-orbital pain (92%) or periorbital pain c) temporal 70%, trigeminal d) pain may also occur in teeth, forehead, jaw, cheek, neck e) pain unilateral during a single episode, but may change side from episode to episode 7) autonomic features are present a) due to sympathetic block & parasympathetic overactivation b) ipsilateral lacrimation (tearing) c) injection of conjunctiva d) nasal stuffiness or rhinorrhea e) ptosis (may become permanent) f) miosis g) periorbital swelling h) bradycardia 8) case presenting with photophobia, nausea [5] 9) scalp, face & carotid artery may be tender 10) restlessness, pacing [9] Diagnostic criteria: 1) at least 5 attacks fulfulling criteria 2) severe unilateral orbital, supraorbital &/or temporal pain lasting 15 minutes to 3 hours untreated 3) either a) at least one sign/symptom ipsilateral to the headache - conjunctival injection &/or lacrimation - nasal congestion &/or rhinorrhea - eyelid edema - forehead & facial perspiration - forehad & facial flushing - sensation of fullness in the ear - miosis &/or ptosis b) a sense of restlessness or agitation 4) attach frequency of 1 every other day to 8/day when a cluster occurs 5) not better explained by other diagnosis [5] Laboratory: - erythrocyte sedimentation rate & serum C-reactive protein for suspected temporal arteritis Radiology: - MRI neuroimaging of patients with suspected cluster headache to exclude structural lesions [5] - head CT if intracranial hemorrhage is suspected Differential diagnosis: - primary stabbing headache - duration seconds, but may be up to 1-2 minutes - no associated cranial autonomic features (lacrimation or tearing) Management: 1) prophylaxis is mainstay of therapy a) verapamil* or other calcium channel blocker b) lithium carbonate 600-900 mg/day in divided doses c) combination of verapamil & lithium best for chronic cluster headaches d) methysergide 1] effective early in the course of disease 2] least effective in later years e) ergotamine is effective for nocturnal attacks f) 40 mg prednisone tapered over 3 weeks g) anticonvulsants may be useful -> gabapentin, valproate, topiramate h) galcanezumab (weak recommenation) [15] i) melatonin 10 mg in the evening j) no strong recommendations [15] k) continue prophylaxis for 4-6 weeks after remission [4] 2) abortive therapy a) 100% oxygen inhalation [9] (weak recommendation) [15] - 5-8 L/min for 10 minutes; - 12 L/min by face mask for 15 minutes [6] 80% effective b) triptan for acute headadche (weak recommendation) 1] sumatriptan SC 2] zolimitriptan nasal 3] ergotamine suppository c) glucocorticoids to break cycle & as bridge therapy: - dexamethasone 8 mg - prednisone 100 mg PO QD for 5 days, follow with taper of 20 mg every 3 days [12] d) intranasal lidocaine 4% e) capsaicin in ipsilateral nostril f) modified trigeminal cisternal injection of glycerol for chronic cluster headache g) chlorpromazine may help some patients [4] h) olanzapine (Zyprexa) 5-10 mg PO QD PRN [4] i) opiates when other agents fail j) hand-held vagus nerve stimulator k) no strong recommendations [15] 3) surgical intervention a) chronic cluster headache b) not indicated for episodic cluster headache * drug of choice [4]

Specific

Horton's cephalgia

General

trigeminal autonomic cephalgia

References

  1. Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 1032-33
  2. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 633-34
  3. Journal Watch 22(7):56, 2002 Bahra A et al Cluster headache: a prospective clinical study with diagnostic implications. Neurology 58:354, 2002 PMID: 11839832
  4. Prescriber's Letter 9(8):46 2002
  5. Medical Knowledge Self Assessment Program (MKSAP) 14, 15, 16, 17, 18. American College of Physicians, Philadelphia 2006, 2009, 2012, 2015, 2018. - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  6. Cohen AS et al, High-Flow Oxygen for Treatment of Cluster Headache JAMA. 2009;302(22):2451-2457. PMID: 19996400 http://jama.ama-assn.org/cgi/content/short/302/22/2451
  7. Francis GJ, Becker WJ, Pringsheim TM. Acute and preventive pharmacologic treatment of cluster headache. Neurology. 2010 Aug 3;75(5):463-73 PMID: 20679639
  8. Petersen AS, Barloese MC, Jensen RH. Oxygen treatment of cluster headache: a review. Cephalalgia. 2014 Nov;34(13):1079-87 PMID: 24723673
  9. Nesbitt AD and Goadsby PJ. Cluster headache. BMJ 2012; 344:e2407 PMID: 22496300
  10. The Sumatriptan Cluster Headache Study Group. Treatment of acute cluster headache with sumatriptan. N Engl J Med. 1991 Aug 1;325(5):322-6. PMID: 1647496
  11. Ashkenazi A, Schwedt T. Cluster headache--acute and prophylactic therapy. Headache. 2011 Feb;51(2):272-86 PMID: 21284609
  12. Obermann M, Nagel S, Ose C et al. Safety and efficacy of prednisone versus placebo in short-term prevention of episodic cluster headache: A multicentre, double-blind, randomised controlled trial. Lancet Neurol 2021 Jan; 20:29. PMID: 33245858 https://www.thelancet.com/journals/laneur/article/PIIS1474-4422(20)30363-X/fulltext
  13. Kneisel K Cluster Headache Hits Women Harder, but Is Often Misdiagnosed. Awareness of sex differences in symptoms, triggers may increase diagnosis and treatment in women. MedPage Today December 21, 2022 https://www.medpagetoday.com/neurology/generalneurology/102351 - Fourier C, Ran C, Steinberg A et al Sex Differences in Clinical Features, Treatment, and Lifestyle Factors in Patients With Cluster Headache. Neurology. 2022. Dec 21 PMID: 36543572 https://n.neurology.org/content/early/2022/12/21/WNL.0000000000201688
  14. NEJM Knowledge+ - Hoffmann J, May A. Diagnosis, pathophysiology, and management of cluster headache. Lancet Neurol. 2018 Jan;17(1):75-83. PMID: 29174963 Review.
  15. Sico JJ, Antonovich NM, Ballard-Hernandez J et al 2023 U.S. Department of Veterans Affairs and U.S. Department of Defense Clinical Practice Guideline for the Management of Headache. Ann Intern Med. 2024 Oct 29. PMID: 39467289