Cluster Headache

Ovid: 5-Minute Sports Medicine Consult, The

Cluster Headache
Kyle D. Parish
  • A primary headache disorder characterized by recurrent attacks of excruciating, short-lasting unilateral headache typically localized to the periorbital and temporal area. Disorder is associated with signs of ipsilateral autonomic dysfunction such as conjunctival injection, lacrimation, rhinorrhea or nasal congestion, facial diaphoresis, miosis, ptosis, and eyelid edema.
  • Attacks last 15–180 min if left untreated.
  • 2 types exist:
    • Episodic: 1 or 2 episodes per year separated by a pain-free interval of at least 1 mo; account for 80% of all cases
    • Chronic: Ongoing attacks of headaches for 1 yr or more without more than 1 mo of remission; represent the remaining 20% of cases
  • System(s) affected: Nervous
  • Synonym(s): Horton's headache; Erythromelalgia of the head; Histaminic cephalalgia; Ciliary or migrainous neuralgia; Erythroprosopalgia of Bing; Petrosal neuralgia of Gardner; Hemicrania periodica neuralgiformis; Sphenopalatine, Vidian, or Sluder neuralgia
  • Predominant age: Most often affects young adults; average age of onset occurs after 30 yrs.
    • A significant diagnostic delay has been reported in most cases (median of 3-yr delay).
    • Symptoms are rare in children.
    • Decrease is often noted after age 70.
  • Predominant gender: Male > Female (reported at just higher than 2:1)
Affects ∼0.05–0.1% of adults
Risk Factors
  • Male gender
  • Age between 20 and 40 yrs
  • Alcohol use
  • Tobacco use, particularly cigarette smoking
  • History of head injury
  • Family history of headaches
  • Shift work
  • Nitroglycerin use
Genetic aspects have been noted in twin studies, but no clear locus or transmission mode has been established.
General Prevention
  • Abstinence from alcohol and tobacco, especially during headache bouts
  • Regular sleep cycle
  • Exact cause is currently unknown.
  • PET scan and functional MRI studies have established a fundamental role of the hypothalamus in the pathophysiology of cluster headaches.
Commonly Associated Conditions
  • Tobacco use
  • Sleep apnea
  • Seasonal allergic rhinitis
  • Suicidal ideation
At least 5 attacks fulfilling the following criteria (1,2)[C]:
  • Severe or very severe unilateral periorbital and/or temporal pain lasting 15–180 min if untreated
  • Headache accompanied by at least one of the following (ipsilateral) symptoms:
    • Conjunctival injection and/or lacrimation
    • Nasal congestion and/or rhinorrhea
    • Eyelid edema
    • Facial sweating
    • Miosis and/or ptosis
    • A general sense of restlessness or agitation
  • Attacks from 1 every other day to 8 per day
  • Not attributed to another disorder
Diagnosis generally is made through history alone.
Physical Exam
  • Few distinguishing features outside active bout
  • Signs or trigeminal autonomic dysfunction ipsilateral to side of headache during attack
  • Restless and agitated behavior (pacing and rocking while holding head in hands)
Diagnostic Tests & Interpretation
Useful only to rule out diagnosis included in differential
In most cases normal, but used to exclude other diagnosis and/or for individuals not responding to appropriate therapy
Differential Diagnosis
  • Migraine headache
  • Paroxysmal hemicrania
  • Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome
  • Hemicrania continua
  • Trigeminal neuralgia
  • Intracranial tumor or bleed
  • Orbital tumor or infection
  • Sinusitis
  • Carotid dissection
  • Temporal arteritis
  • Herpes zoster


Ongoing Care
Avoidance of alcohol is highly recommended during bouts because it is the only known dietary trigger.
Patient Education
  • Little research is available to assess the effectiveness of lifestyle modifications, but exercise, relaxation techniques, biofeedback, and smoking cessation have been suggested to improve quality of life.
  • There is no evidence that exercise or athletic participation is contraindicated during an acute bout of cluster headache, but severity of headache may limit ability of individual participation during episode.
  • Very unpredictable course:
    • Recurrent attacks
    • Prolonged remission
  • Episodic type can evolve to chronic type.
  • Total remission has been described.
  • 339.00 Cluster headache syndrome, unspecified
  • 339.01 Episodic cluster headache
  • 339.02 Chronic cluster headache

This website uses cookies to improve your experience. We'll assume you're ok with this, but you can opt-out if you wish. Accept Read More