CLUSTER HEADACHE

Drawing of I. Rubloff entitled "indescribeable faceache". (c) 2005, I. Rubloff, all rights reserved

Timothy C. Hain, MD Page last modified: February 12, 2011

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Cluster headache is defined as excruciating unilateral head pain which occurs in brief episodes (15 minutes-2 hours). Clusters consist of headache that occur daily for 3 weeks to 3 months, then remit. Typically there are bouts in early July and early January. It may awaken one from sleep (migraine and tension usually won't). Some experience flushing, droopy eye, nasal stuffiness, or eye tearing. Men are affected 2 to 5 times more frequently than are women (reverse of migraine). Unlike the situation for migraine, menopause does not reduce the frequency and severity of headache in women. There probably are several mechanisms for same symptom complex - migraine variant, sphenopalatine neuralgia, and perhaps a genetic syndrome involving circadian rhythms..

The reason for the peculiar periodicity of cluster headache has recently been discussed (Pringsheim T, 2002). There are two genes -- the PER or period gene located on the X chromosome and the timeless (TIM) gene. Both genes affect the suprachiasmatic (STC) nucleus of the hypothalmus. These genes generate products that control circadian rhythms. It seems reasonable to hypothesize that in cluster headache there is a disturbance of this clock.

Paroxysmal hemicrania

Paroxysmal hemicrania (PH) is ocasionally mistaken for cluster. Both disorders are short-lived, unilateral, and accompanied by ipsilateral autonomic features. The PH patients show a female predominance, a shorter attack duration (2-30 min), and often a greater attack frequency (5 or more/day). A prompt response to indomethacin confirms the diagnosis (Lipton et al, 2003)

Treatment of Cluster:

• Analgesics -- rarely effective, even narcotics.
  • Indomethacin (25 TID)
• Intranasal Capsaicin.  While effective for prevention, this  is difficult to administer.
• Ergot and related medications
  • Ergot compounds and triptans work but may be addictive in this syndrome due to rebound and requirements for frequent administration. Would start with these anyway, as need a fast acting agent.
  • Sumatriptan -- might differentiate migraine from neuralgia. Intranasal is the most effective method (Markey, 2003). Injectable is the most reliable method.  See also Hardebo, 1998.
  • Zolmatriptan nasal spray (Markey, 2003)
  • DHE-45 (Intranasal).
• Intranasal lidocaine. A 4% solution administered by drops (not spray) has been reported successful. (Markey 2003)
• Lithium -- equivalent to verapamil in efficacy, but more side effects (Bussone et al, 1990).
• Oxygen (5-7 L flow x 10 min) also reportedly works in 50%, but minimal successes in author's patients.
• Verapamil (Calan) prophylaxis often works, but may be too slow in onset (2 week) to be useful acutely. Verapamil works best in large doses -- 240 to 480/day. When used in this dose, heart monitoring is suggested. (Blau et al, 2004; Bussone et al, 1990; Cohen et al, 2007)
• Steroids (Decadron 0.75 BID x 7 D). Other authors recommend prednisolone (Bahra et al, 2002)
• Surgical treatments
  • Trigeminal nerve section -- can be used in intractable chronic cases (Jarrar et al, 2003)
  • Occipital nerve stimulation (Burns). Used in intractable cases.
• Xyrem (sodium oxybutate)

Cluster headaches are extremely painful and the author's present practice is to use multiple medications simultaneously when it recurs. The author's usual protocol is to combine triptans with steroids and verapamil.  The triptans are used for pain control, while waiting for the steroids and verapamil to take effect.

A new and perhaps better approach to cluster was reported by Khatami et al.  Xyrem (Sodium oxybate), which induces extremely deep sleep, was reported to be effective in cluster(2011).  It is our thought that as clusters normally occur in the middle of the night, someone on Xyrem might simply sleep through them.  This seems to us to be potentially a better way of managing them than the medications above.

References:

• Bahra, A., et al. (2002). "Cluster headache: a prospective clinical study with diagnostic implications." Neurology 58(3): 354-61.
• Blau JN. and H. O. Engel (2004). "Individualizing treatment with verapamil for cluster headache patients." Headache 44(10): 1013-8.
• Burns B, Watkins L, goadsby P. Treatment of intractable chronic cluster headache by occipital nerve stimulation in 14 patients. Neurology 2009:72:341-345
• Bussone, G et al. (1990). "Double blind comparison of lithium and verapamil in cluster headache prophylaxis." Headache 30(7): 411-7.
• Cohen AS et al. (2007). "Electrocardiographic abnormalities in patients with cluster headache on verapamil therapy." Neurology 69(7): 668-75.
• Hardebo JE, Dahlof C. Sumatriptan nasal spray (20 mg/dose) in the acute treatment of cluster headache. Cephalgia 1998, 18:487-489
• Jarrar RG and others. Outcome of trigeminal nerve section in the treatment of chronic cluster headache. Neurology 2003:60:1360-62
• Lipton RB and others. Why headache treatments fail. Neurology 2003:60:1064-1070
• Khatimi R, Tararotti S, Siccoli MM, Bassetti CL, Sandor PS. Long term efficacy of sodium oxybate in 4 patients with chronic cluster headache. Neurology 2011:77:67-70
• Markey HL. Topical agents in the treatment of cluster headache. Current pain and headache reports 2003, 7: 139-143
• Pringsheim, T. (2002). "Cluster headache: evidence for a disorder of circadian rhythm and hypothalamic function." Can J Neurol Sci 29(1): 33-40.
• Shakra S, Becker WJ, Werner J, et al. Zolmitriptan nasal spray is effective, fast-acting and well tolerated during both short and long-term treatment. Neurology 2002, 58(suppl 3) A414.