Cluster Headache
Cardinal Presentations / Presenting Problems, Neurological
First 5 Minutes
- Neither non-opioid nor opioids are effective treatments.
- High incidence of suicidal ideation and self-injurious behaviour.
Context
- Very low prevalence <1%.
- Affects primarily males aged 20-40.
- Typically attacks are “clustered” and can last between 15 min to 3 hours with periods of remissions in between.
- Strictly unilateral with ipsilateral cranial autonomic signs.
- Triggers include alcohol, PDE5 inhibitors, high altitude, weather changes and circadian rhythm disruption.
Diagnostic Process
Rule out headache red flags:
1. rapid onset
2. thunderclap headaches
3. neurodeficits
4. neck pain with/without fever
5. age > 50
6. worse with positional changes or Valsalva
7. new/worsening headaches
Diagnostic criteria based on the ICHD-3:
- At least five attacks fulfilling criteria B-D.
- Severe or very severe unilateral orbital, supraorbital and/or temporal pain lasting 15-180 minutes (when untreated)1.
- Either or both of the following:
- at least one of the following symptoms or signs, ipsilateral to the headache:
- conjunctival injection and/or lacrimation.
- nasal congestion and/or rhinorrhoea.
- eyelid oedema.
- forehead and facial sweating.
- miosis and/or ptosis.
- a sense of restlessness or agitation.
- at least one of the following symptoms or signs, ipsilateral to the headache:
- Occurring with a frequency between one every other day and 8 per day2.
- Not better accounted for by another ICHD-3 diagnosis.
Recommended Treatment
- First Line Therapy
- O2 inhalation via non-rebreather face mask at 12-15 L/min for at least 15 min.
- Sumatriptan 6mg subcutaneous OR 20mg intranasal contralateral to side of pain.
- Zolmitriptan 10mg oral or intranasal contralateral to side of pain.
- Second Line Therapy
- Lidocaine 1mL of 4% solution intranasal ipsilateral to side of pain.
- Ergotamine 2mg sublingual q30min to a maximum dose of 6mg.
- Dihydroergotamine 1mg IV bolus q1hr to a maximum of 3mg.
- Octreotide 100 mcg subcutaneous.
Criteria For Hospital Admission
Hospital admission not required.
Quality Of Evidence?
High
We are highly confident that the true effect lies close to that of the estimate of the effect. There is a wide range of studies included in the analyses with no major limitations, there is little variation between studies, and the summary estimate has a narrow confidence interval.
Moderate
We consider that the true effect is likely to be close to the estimate of the effect, but there is a possibility that it is substantially different. There are only a few studies and some have limitations but not major flaws, there are some variations between studies, or the confidence interval of the summary estimate is wide.
Low
When the true effect may be substantially different from the estimate of the effect. The studies have major flaws, there is important variations between studies, of the confidence interval of the summary estimate is very wide.
Justification
Recommendations are based on observational data from a limited number of small studies.
Related Information
OTHER RELEVANT INFORMATION
GPnotebook Podcast – https://gpnotebook.com/podcasts/neurology/ep-62-cluster-headaches
Reference List
Leone M, May A. Acute treatment of cluster headache attacks. In: Leone M, May A, eds. Cluster Headache and other Trigeminal Cephalgias. 1st ed. Springer; 2020: 131-134. Accessed November 29, 2023.
https://doi.org/10.1007/978-3-030-12438-0Leone M, May A. Classification and clinical features. In: Leone M, May A, eds. Cluster Headache and other Trigeminal Cephalgias. 1st ed. Springer; 2020: 11-21. Accessed November 29, 2023.
https://doi.org/10.1007/978-3-030-12438-0Lund NLT, Petersen AS, Fronczek R, Tfelt-Hansen J, Belin AC, Meisingset T, et al. Current treatment options for cluster headache: limitations and the unmet need for better and specific treatments – a consensus article. J Headache Pain. 2023;24(1):121. https://doi.org/10.1186/s10194-023-01660-8
Di Sabato F, Giacovazzo M. Management of cluster headache in the emergency department. J Headache Pain. 2005;6(4):294-297.
https://doi.org/10.1007/s10194-005-0212-z
RESOURCE AUTHOR(S)
DISCLAIMER
The purpose of this document is to provide health care professionals with key facts and recommendations for the diagnosis and treatment of patients in the emergency department. This summary was produced by Emergency Care BC (formerly the BC Emergency Medicine Network) and uses the best available knowledge at the time of publication. However, healthcare professionals should continue to use their own judgment and take into consideration context, resources and other relevant factors. Emergency Care BC is not liable for any damages, claims, liabilities, costs or obligations arising from the use of this document including loss or damages arising from any claims made by a third party. Emergency Care BC also assumes no responsibility or liability for changes made to this document without its consent.
Last Updated Dec 15, 2023
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