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Cluster headache

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Updated 2024 VA/DoD guidelines for the management of cluster headache.

Background

Overview

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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Guidelines

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 American Society of Interventional Pain Physicians (ASIPP/NASS/AAPM/ASRA/IPSIS 2024), the United States Department of Defense (DoD/VA 2024), the European Academy of Neurology (EAN 2023), the American Headache Society (AHS 2016), and the American Academy of Family Physicians (AAFP ...
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Diagnostic investigations

History and physical examination: as per AAFP 2013 guidelines, consider using a questionnaire consisting of the combination of typical headaches lasting < 180 minutes plus conjunctival injection or lacrimation to assess for CH.
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Medical management

Abortive therapy, triptans: as per DoD/VA 2024 guidelines, consider offering sumatriptan SC 6 mg or intranasal zolmitriptan 10 mg for the short-term treatment of CH.
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  • Abortive therapy (oxygen)

  • Abortive therapy (octreotide)

  • Abortive therapy (dihydroergotamine)

  • Abortive therapy (lidocaine)

  • Abortive therapy (other agents)

  • 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)

Nonpharmacologic interventions

Behavioral interventions: as per DoD/VA 2024 guidelines, insufficient evidence to recommend biofeedback and smartphone application-based HR variability monitoring, CBT, mindfulness-based therapies, or progressive muscle relaxation for the treatment and/or prevention of headache.
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  • Dietary modifications

  • Alternative and complementary therapies

Therapeutic procedures

Sphenopalatine ganglion stimulation: as per DoD/VA 2024 guidelines, avoid offering sphenopalatine ganglion stimulation for the treatment of CH.
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  • Greater occipital nerve stimulation

  • Greater occipital nerve block (indications)

  • Greater occipital nerve block (technical considerations)

  • Deep brain stimulation

  • Vagus nerve stimulation