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Cluster Headache Clinical Presentation

  • Author: Michelle Blanda, MD; Chief Editor: Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS  more...
 
Updated: Dec 07, 2015
 

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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Contributor Information and Disclosures
Author

Michelle Blanda, MD Chair Emeritus, Department of Emergency Medicine, Summa Health System Akron City/St Thomas Hospital; Professor of Emergency Medicine, Northeastern Ohio Universities College of Medicine

Michelle Blanda, MD is a member of the following medical societies: American College of Emergency Physicians, Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Rima M Dafer, MD, MPH, FAHA Associate Professor, Department of Neurology and Neurological Surgery, Loyola University, Chicago Stritch School of Medicine

Rima M Dafer, MD, MPH, FAHA is a member of the following medical societies: American Academy of Neurology, American Heart Association, American Headache Society

Disclosure: Nothing to disclose.

Chief Editor

Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS Professor Emeritus of Neurology and Psychiatry, Clinical Professor of Medicine, Clinical Professor of Family Medicine, Clinical Professor of Neurosurgery, State University of New York Upstate Medical University; Neuroscience Director, Department of Neurology, Crouse Irving Memorial Hospital

Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS is a member of the following medical societies: American College of International Physicians, American Heart Association, American Stroke Association, American Academy of Neurology, American Academy of Pain Medicine, American College of Forensic Examiners Institute, National Association of Managed Care Physicians, American College of Physicians, Royal College of Physicians, Royal College of Physicians and Surgeons of Canada, Royal College of Surgeons of England, Royal Society of Medicine

Disclosure: Nothing to disclose.

Acknowledgements

Joseph Carcione Jr, DO, MBA Consultant in Neurology and Medical Acupuncture, Medical Management and Organizational Consulting, Central Westchester Neuromuscular Care, PC; Medical Director, Oxford Health Plans

Joseph Carcione Jr, DO, MBA is a member of the following medical societies: American Academy of Neurology

Disclosure: Nothing to disclose.

Steven C Dronen, MD, FAAEM Chair, Department of Emergency Medicine, LeConte Medical Center

Steven C Dronen, MD, FAAEM is a member of the following medical societies: American Academy of Emergency Medicine and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Ragasri Kumar, DO Resident Physician, Department of Neurology, Loyola University Medical Center

Disclosure: Nothing to disclose.

Edward A Michelson, MD Associate Professor, Program Director, Department of Emergency Medicine, University Hospital Health Systems in Cleveland

Edward A Michelson, MD is a member of the following medical societies: American College of Emergency Physicians, National Association of EMS Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Lori K Sargeant, MD Consulting Staff, Summa Emergency Associates, Inc

Lori K Sargeant, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Emergency Physicians, and Ohio State Medical Association

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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Cluster headache: Functional imaging shows activation of specific brain areas during pain. Courtesy of Wikipedia Commons.
Cluster headache (CH): Voxel-based morphometry (VBM) structural imaging shows specific brain area of CH patients' (hypothalamus) being different to non-CH patients' brains. Courtesy of Wikipedia Commons.
Non-rebreather oxygen mask with reservoir for the acute treatment of cluster headache. Courtesy of Wikipedia Commons.
 
 
 
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