Cluster headaches are a particularly painful form of recurrent primary headache disorder, considered the most common trigeminal autonomic cephalalgia 1.
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Terminology
Cluster headaches have been known by a variety of different names, including paroxysmal nocturnal cephalgia, histamine headache, Horton headache (or Horton syndrome), or cranial autonomic syndrome 3.
Epidemiology
Cluster headaches are rare, affecting 0.06 to 0.4% of the population 1. Men are more often affected (M: F 3:1) 1.
Clinical presentation
Cluster headaches are characterized by severe unilateral anterior or lateral headache (frontal, orbital or temporal) 1. Headaches last between 15-180 minutes (45-60 minutes is typical) and are associated with ipsilateral autonomic signs 1,2:
cutaneous: sweating, periorbital 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.
Radiographic features
The main role of imaging in patients with cluster headaches is to exclude secondary causes (especially pituitary lesions) or other causes of a severe headache (see differential diagnosis below) 4. 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.
Treatment and prognosis
A detailed discussion of the treatment of cluster headaches is beyond the scope of this article, however, generally, treatment strategies can be divided into:
acute management
preventative management
interventional procedures
Acute management
Acute management focuses on triptans (e.g. sumatriptan - subcutaneous or intranasal, zolmitriptan - oral) and inhaled 100% FiO2 oxygen 1.
Preventative management
A variety of drugs have been shown to reduce the frequency of attacks, including verapamil, lithium, valproic acid, and topiramate 1.
Interventional procedures
A variety of interventions are used in medically refractory cases or patients in whom medical therapy is not tolerated. Procedures include 1:
ablative procedures on the trigeminal nerve (e.g. glycerol rhizotomy, trigeminal section, radiosurgery
trigeminal microvascular decompression
occipital nerve stimulation or greater occipital nerve injection
Differential diagnosis
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:
structural lesions (e.g. tumors or hemorrhages)
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