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Cluster headache

Definition
CH is a primary headache disorder characterized by severe unilateral pain mainly in the first division of the trigeminal nerve accompanied by ipsilateral cranial autonomic symptoms and a sense of agitation and restlessness during attacks.
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Pathophysiology
CH is caused by the activation of trigeminovascular complex and trigeminal-autonomic reflex.
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Disease course
Clinical manifestations include excruciating, unilateral headache over the peri- and retro-orbital regions and the temple accompanied by ipsilateral lacrimation, eye redness, eye discomfort, ptosis, nasal congestion, rhinorrhea, aural fullness, throat swelling, flushing, sense of restlessness, and agitation. Episodic headaches may subsequently become chronic headaches or may remit with age.
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Prognosis and risk of recurrence
CH is not associated with increased mortality.
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Key sources
The following summarized guidelines for the evaluation and management of cluster headache are prepared by our editorial team based on guidelines from the European Academy of Neurology (EAN 2023), the American Headache Society (AHS 2016), and the American Academy of Family Physicians (AAFP 2013).
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Guidelines

1.Diagnostic investigations

History and physical examination: consider using a questionnaire consisting of the combination of typical headaches lasting < 180 minutes plus conjunctival injection or lacrimation to assess for CH.
C
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2.Medical management

Abortive therapy, triptans, EAN
Offer sumatriptan SC 6 mg using a self-injection device in patients with CH.
B
Consider offering 20 mg nasal spray in cases of needle phobia or side effects.
B
Offer zolmitriptan PO 5 mg
B
or nasal spray.
B

More topics in this section

  • Abortive therapy (oxygen)

  • Abortive therapy (octreotide)

  • Abortive therapy (dihydroergotamine)

  • Abortive therapy (lidocaine)

  • Preventative therapy (verapamil)

  • Preventative therapy (lithium)

  • Preventative therapy (corticosteroids)

  • Preventative therapy (triptans)

  • Preventative therapy (anticonvulsants)

  • Preventative therapy (melatonin)

  • Preventative therapy (ergotamine)

  • Preventative therapy (capsaicin)

  • Preventative therapy (galcanezumab)

  • Preventative therapy (warfarin)

  • Preventative therapy (oxygen)

  • Preventative therapy (other agents)

3.Nonpharmacologic interventions

Noninvasive vagus nerve stimulation
Offer noninvasive vagus nerve stimulation (3 self-administered consecutive 2-minute simulations ipsilateral to the headache) at the time of attack onset in patients with episodic CH. Consider offering noninvasive vagus nerve stimulation as an add-on treatment.
B
Do not offer noninvasive vagus nerve stimulation for chronic CH.
D

4.Therapeutic procedures

Sphenopalatine ganglion stimulation: as per EAN 2023 guidelines, offer sphenopalatine ganglion stimulation for aborting attacks and reducing attack frequency in patients with CH.
B

More topics in this section

  • Greater occipital nerve stimulation

  • Greater occipital nerve block

  • Deep brain stimulation