Cluster headache

Last revised by Ian Bickle on 27 Dec 2021

Cluster headaches are a particularly painful form of recurrent headache considered the most common trigeminal autonomic cephalalgias 1. This is also known by a variety of different names, for example; paroxysmal nocturnal cephalgia, histamine headache (also known as Horton headache), cranial autonomic syndrome3

Cluster headaches are fairly uncommon affecting 0.06 to 0.4% of the population 1. Men are more often affected (M: F 3:1) 1

Cluster headaches are characterized by severe unilateral anterior or lateral headache (frontal, orbital or temporal) 1. Headaches typically last from a quarter of an hour to a few hours (45 to 60 minutes is typical) and are associated with ipsilateral autonomic signs 1,2:

  • cutaneous: sweating, swelling
  • orbital: conjunctival injection, miosis, ptosis, lacrimation
  • nasal: congestion and rhinorrhea

They are clustered temporally and often described as having a 'clockwork' recurrence, occurring anywhere from many times a day to every second day 1.

The main role of imaging in patients with cluster headaches is to exclude other causes of a severe headache (see differential diagnosis below). SPECT, PET, MRI (functional MRI and voxel-based morphometry)  have been used in a research setting to attempt to elucidate the underlying pathophysiology of cluster headaches and other trigeminal autonomic cephalalgias 2

A detailed discussion of the treatment of cluster headaches is beyond the scope of this article, however, generally, treatment strategies can be divided into: 

  1. acute management
  2. preventative management
  3. interventional procedures

Acute management focuses on triptans (e.g. sumatriptan - subcutaneous or intranasal, zolmitriptan - oral) and inhaled oxygen 1

A variety of drugs have been shown to reduce the frequency of attacks, including verapamil, lithium, valproic acid, and topiramate 1

A variety of interventions are used in medically refractory cases or patients in whom medical therapy is not tolerated. Procedures include 1

Once the typical episodic and recurrent nature of the headaches becomes apparent and the stereotyped pattern of signs and symptoms evident the diagnosis can usually be made with a high degree of certainty. Initially, however, many other causes of a severe headache may be thought of as possible etiologies, including: 

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