Cluster Headache Clinical Presentation

Updated: Apr 26, 2017
  • Author: Michelle Blanda, MD; Chief Editor: Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS  more...
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Presentation

History

Attacks of cluster headache (CH) are typically short and occur with a clear periodicity, particularly during sleep or early morning hours, usually corresponding with onset of rapid eye movement (REM) sleep. [5, 9] Unlike migraine, CH is not preceded by aura and is not usually accompanied by symptoms such as nausea, vomiting, photophobia, or osmophobia. Typically, a patient experiences 1-2 cluster periods per year, each lasting 2 weeks to 3 months.

The International Headache Society (IHS) classifies CH as episodic or chronic on the basis of duration as follows [3] :

  • Episodic CH occurs in periods lasting from 7 days to 1 year; cluster attacks are separated by pain-free intervals at least 1 month long
  • Chronic CH persists for more than 1 year either without remission or with remissions shorter than 1 month; it is further divided into 2 subcategories, chronic CH from onset and chronic CH evolving from episodic CH

The pain of CH is manifested as follows:

  • Character - Excruciating, stabbing, sharp, and lancinating (as if the eye is being pushed out), rather than throbbing
  • Location – Unilateral, in the periorbital, retro-orbital, or temporal regions, though pain sometimes radiates to the cheek, jaw, occipital, and nuchal regions; the pain tends to remain on the same side during the cluster period but in rare cases may switch sides
  • Distribution - First and second divisions of the trigeminal nerve; approximately 18-20% of patients complain of pain in the extratrigeminal areas (eg, the back of the neck, along the carotid artery)
  • Onset – Sudden, peaking in 10-15 minutes
  • Duration - 5 minutes to 3 hours per episode
  • Frequency - May occur 1-8 times a day for as long as 4 months (often nocturnal)
  • Periodicity - Circadian regularity in 47%
  • Remission - Long symptom-free intervals occur in some patients; the length of these remissions averages 2 years but may range from 2 months to 20 years

Pain is accompanied by various cranial parasympathetic symptoms, including the following [5] :

  • Ipsilateral lacrimation (84-91%) or conjunctival injection
  • Nasal stuffiness (48-75%) or rhinorrhea
  • Ipsilateral eyelid edema
  • Ipsilateral miosis or ptosis
  • Ipsilateral forehead and facial perspiration (26%)

Alcoholic products and tobacco may precipitate an attack. Other triggers include hot weather, watching television, nitroglycerin, stress, relaxation, extreme temperatures, glare, allergic rhinitis, and sexual activity.

During an attack of CH, as many as 90% of patients may become agitated and extremely restless. They do not like to lie down to rest; instead, they prefer to pace or move around. In desperation, patients may rock, sit, pace, bang themselves against a hard surface, scream in pain, or crawl on the floor.

Structural lesions have been described with CH and should be suspected if the presentation is atypical. Atypical features may include the following:

  • Absence of a periodic pattern
  • Residual headache between exacerbations
  • Bilaterality
  • Incomplete or minimal response to standard therapy
  • Presence of lateralizing findings on examination (other than Horner syndrome)
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Physical Examination

Physical examination findings should be normal, except for certain findings that serve as hallmarks of CH. These accompanying findings are consistent with ipsilateral autonomic features characterized by cranial parasympathetic activation and sympathetic hypofunction. The presence of other abnormalities suggests another etiology for the headache.

Characteristic findings include the following:

  • Distinctive facial appearance - Leonine facies, multifurrowed and thickened skin with prominent folds, a broad chin, vertical forehead creases, and nasal telangiectasias
  • Parasympathetic overactivity - Ipsilateral lacrimation, conjunctival injection, rhinorrhea or congestion
  • Ocular sympathetic paralysis - Mild Horner syndrome (eg, ptosis, miosis, and anhidrosis), which may persist between attacks
  • Bradycardia
  • Facial flushing or pallor
  • Scalp and facial tenderness
  • Ipsilateral carotid tenderness (in some patients)

Patients often are in severe distress. They may lower the head and press on the site of pain, sometimes crying or screaming. Physical exercise may afford a degree of relief. In cases of especially severe or intolerable pain, patients may even threaten suicide.

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