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Nervous system

Cluster headache

Cluster headache or suicide headache is an excruciating, strictly one-sided pain syndrome with attacks that last between 15-180 minutes and that are accompanied by marked ipsilateral cranial autonomic symptoms, such as lacrimation and conjunctival injection.

Jorge Muniz | MedComic

Cluster headache or suicide headache is an excruciating, strictly one-sided pain syndrome with attacks that last between 15-180 minutes and that are accompanied by marked ipsilateral cranial autonomic symptoms, such as lacrimation and conjunctival injection.

  • M/C type of trigeminal autonomic cephalgia (TACs)

Classification

ational Headache Society (IHS) classification of primary headaches:

  • Migraine
  • Tension headache
  • Trigeminal autonomic cephalalgias (of which cluster headache is the most prominent variety)
  • Group 4 “other primary headache disorders”: Consists of 10 rare headache syndromes with well-established primary nature
    • Benign exertional headaches
    • Primary stabbing headaches (“jabs and jolts syndrome”)
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Primary headache disorders. | Lee, V., Ang, L. L., Soon, D., Ong, J., & Loh, V. (2018). The adult patient with headache. Singapore medical journal, 5

Trigeminal autonomic cephalgias (TACs):

  • Cluster headache (M/C)
  • Paroxysmal hemicrania (PH)
  • Hemicrania continua (HC)
  • Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT)
Clinical features of the trigeminal autonomic cephalalgias: a comparison of cluster headache with the related trigeminal autonomic cephalalgia syndromes
Clinical features of the trigeminal autonomic cephalalgias: a comparison of cluster headache with the related trigeminal autonomic cephalalgia syndromes | May A. (2018). Hints on Diagnosing and Treating Headache. Deutsches Arzteblatt international, 115(17), 299–308. https://doi.org/10.3238/arztebl.2018.0299

Pathophysiology

Trigeminal-autonomic reflex with parasympathetic activation (hallmark feature):

  • Trigeminal-autonomic reflex: Consists of a brainstem connection between the trigeminal nerve and facial cranial nerve parasympathetic outflow
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Schematic pathway representation summarizing the trigeminal-parasympathetic reflex | Cluster headache. (2018). Nature Reviews Disease Primers, 4(1), 18007. https://doi.org/10.1038/nrdp.2018.7

Presentation

Headache:

Short lasting attacks of unilateral severe headache with severe ipsilateral cranial autonomic symptoms.
  • Unilateral severe episodic headaches
  • Restlessness during attacks
  • Ipsilateral autonomic symptoms
  • Location: Periorbital
  • Duration: < 3 hours (short-duration)
    • 8-10 weeks of symptoms followed by symptom-free interval
  • Stabbing/boring quality
  • Nocturnal pain, precipitated by alcohol
  • Associated features:
    • Photophobia

Ipsilateral cranial autonomic symptoms:

  • Conjunctival injection and/or lacrimation
  • Nasal congestion and/or rhinorrhea
  • Eyelid oedema
  • Forehead & facial sweating
  • Aural fullness
  • Ptosis/miosis
Patient with cluster headache during a right-sided painful attack
Photograph of a patient with cluster headache during a right-sided painful attack: Note the ipsilateral ptosis and miosis. Additionally there is obvious ipsilateral lacrimation and rhinorrhoea (see upper lip) | Reprinted from Benoliel R and Sharav Y. Trigeminal autonomic cephalgias (TACs). In: Y Sharav and R Benoliel (eds) Orofacial pain and headache. Edinburgh: Mosby Elsevier, 2008; pp.223–254

Diagnosis

International Classification of Headache Disorders-3 beta (2013) diagnostic criteria for cluster headache:

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International Classification of Headache Disorders-3 beta (2013) diagnostic criteria for cluster headache | Ravishankar K. (2018). Classification of Trigeminal Autonomic Cephalalgia: What has Changed in International Classification of Headache Disorders-3 Beta?. Annals of Indian Academy of Neurology, 21(Suppl 1), S45–S50. doi:10.4103/aian.AIAN_350_17

Management

Oxygen therapy:

  • 100% oxygen in 10l/min for 10-15 min
  • Sumatriptan 6mg SC

Prophylaxis:

  • Short-term prevention: Steroids
  • Long-term prevention: Verapamil > Lithium

Summary:

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