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Cluster Headache

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  1. See Also
    1. Acute Recurrent Headache
    2. Migraine Headache
    3. Headache
  2. Pathophysiology
    1. Migraine Variant
  3. Types
    1. Cluster Headache
      1. Episodic (90%)
        1. Remission periods last at least one month
      2. Chronic (10%)
        1. Headaches occur for more than one year
        2. Remissions last <1 month
    2. Cluster Headache Variants
      1. Hemicrania continua (or Paroxysmal hemicrania)
  4. Epidemiology
    1. More common in men
    2. Onset in men occurs in ages 20 to 40
    3. Onset in women peaks in the 60s
      1. More common in black women
  5. Risk Factors
    1. Tobacco abuse
    2. Family History of Headache
    3. Head Injury
    4. Shift work
  6. Symptoms
    1. Characteristics
      1. Deep pain
      2. Burning or stabbing type pain
    2. Severity: Excruciating pain ("Suicide Headache")
      1. Patient may even consider Suicide
    3. Location
      1. Unilateral Headache
      2. Orbital, supraorbital or temporal pain
      3. Radiates to upper teeth, jaw or neck
    4. Timing
      1. At least 5 attacks within 10 days
      2. Multiple daily episodes may occur (up to 8 per day)
      3. Headaches last 15 minutes to 180 minutes
      4. Usually recur at same time of day each day
      5. May awaken patient from sleep (esp. onset of REM)
      6. Remissions for months to years
    5. Triggers
      1. Sleep Apnea
      2. Vasodilators
        1. Nitroglycerin
        2. Alcohol
    6. Associated with at least one of the following
      1. Lacrimation
      2. Ipsilateral facial Flushing or sweating
      3. Ipsilateral Nasal discharge
      4. Affected eye red with dilated Conjunctival vessels
      5. Restlessness, pacing or rocking head in hands
      6. Horner's Syndrome
        1. Ipsilateral Ptosis
        2. Ipsilateral pupillary constriction (Miosis)
  7. Evaluation
    1. See Headache Evaluation
  8. Differential Diagnosis
    1. Migraine Headache
      1. Common features do not distinguish from cluster
        1. Aura occurs in 14% of cluster Headaches
        2. Photophobia occurs in >50% of cluster Headaches
    2. Hemicrania continua (or Paroxysmal hemicrania)
      1. Cluster-type Headache with brief duration
      2. Responds well to Indomethacin
    3. Orbital Myositis
      1. Similar to cluster Headache with longer duration
  9. Management: Abortive Treatment (See Migraine Treatment)
    1. First line agents
      1. Sumatriptan (Imitrex)
        1. Subcutaneous: 6 mg SC
        2. Intranasal 20 mg
      2. Oxygen Inhalation
        1. Dose: 7 liters per minute for 15 minutes
    2. Other agents
      1. Intranasal Lidocaine 4-10% solution
        1. Dose: 1 ml intranasally
          1. Aplied with cotton swab bilaterally for 5 minutes
        2. May be repeated twice in 15 minutes prn
        3. Relieves pain within 5-15 minutes
        4. Costa (2000) Cephalalgia 20:85
      2. Intranasal Dihydroergotamine 0.5 mg bilateral nares
        1. Reduces Headache severity
        2. Does not decrease cluster frequency or duration
        3. Andersson (1986) Cephalalgia 6:51
      3. Intranasal Capsaicin
        1. Applied to ipsilateral nostril bid for 7 days
        2. Marks (1993) Cephalalgia 13:114
      4. Indomethacin
        1. Dose: 25-50 mg tid prn
        2. Effective also in cluster Headache variants
  10. Management: Prophylaxis for Episodic Cluster Headaches
    1. See Migraine Prophylaxis
    2. First-line agents
      1. Corticosteroids
        1. Dose: 60 mg on days 1-3
        2. Tapering over 10-14 days
      2. Verapamil
        1. Start: 80 mg PO tid
        2. Increase to 120 to 160 mg PO tid
    3. Other agents
      1. Anticonvulsants
        1. Valproic Acid
        2. Topiramate (Topamax)
        3. Gabapentin (Neurontin)
      2. Indomethacin
        1. Dose: 25-50 mg tid
    4. Avoid agents with serious adverse effects
      1. Methylsergide
        1. No longer recommended due to systemic fibrosis
  11. Management: Prophylaxis for Chronic Cluster Headaches
    1. Verapamil
      1. Start: 80 mg PO tid
      2. Increase to 120 to 160 mg PO tid
    2. Lithium
      1. Dose: 300-600 mg/day initially (Maximum 900 mg/day)
      2. Base dose on serum Lithium levels
    3. Microvascular decompression surgery
  12. Management: Nonpharmacologic measures
    1. Relaxation techniques
    2. Cognitive-behavior therapy
    3. Treat comorbid Mood Disorders
    4. Alcohol cessation
  13. References
    1. Beck (2005) Am Fam Physician 71:717
    2. Dalessio (2001) Postgrad Med 109(1):69
    3. Dodick (2000) Cephalalgia 20(9):787
    4. Zakrzewska (2001) Br J Oral Maxillofac Surg 39:103

Cluster Headache (C0009088)

Definition (MSH)A primary headache disorder that is characterized by severe, strictly unilateral PAIN which is orbital, supraorbital, temporal or in any combination of these sites, lasting 15-180 min. occurring 1 to 8 times a day. The attacks are associated with one or more of the following, all of which are ipsilateral: conjunctival injection, lacrimation, nasal congestion, rhinorrhea, facial SWEATING, eyelid EDEMA, and miosis. (International Classification of Headache Disorders, 2nd ed. Cephalalgia 2004: suppl 1)
ConceptsDisease or Syndrome (T047)
ICD9346.2
MSHD003027
BasqueHORTONEN ZEFALEA
DanishCluster-hovedpine/Hortons syndrom
DutchCluster headache
EnglishBING-HORTON SYNDROME, Chronic Cluster Headache, Chronic Cluster Headaches, Ciliary Neuralgia, Ciliary Neuralgias, Cluster headache, Cluster Headache Syndrome, Cluster Headache Syndromes, Cluster headaches, ERYTHROPROSOPALGIA, HEADACHE HISTAMINE, HEADACHE VASOMOTOR, Histamine Cephalgia, Histamine Cephalgias, Histamine headache, HORTON SYNDROME, HORTON'S HEADACHE, Horton's neuralgia, Horton's Syndrome, Hortons Syndrome, Migrainous neuralgia, Neuralgic Migraine, Neuralgic Migraines, Vasomotor headache
FinnishCLUSTER PAANSARKY
FrenchCephalee de Horton
GermanCluster-Kopfschmerz
Hungariangorcsos fejfajas
ItalianCefalea a grappolo
NorwegianCLUSTER-HODEPINE/HORTONS SYNDROM
PortugueseCefaleia de Horton/cluster
Spanishcefalalgia histaminica, cefalea acuminada, Cefalea arracimada, cefalea cronica salvas, cefalea de Horton, cefalea de Horton cronica, cefalea en acumulos, cefalea en grupos, cefalea en racimos, cefalea en racimos cronica, cefalea en salvas, cefalea en salvas cronica, cefalea en tandas, cefalea en tandas cronica, cefalea histaminergica, cefalea histaminergica cronica, cefalea histaminica, cefalea histaminica cronica, cefalea vasomotora, migrana neuralgica, migrana neuralgica cronica, neuralgia ciliar, neuralgia con migrana, neuralgia de Horton
SwedishCLUSTERHUVUDVARK
Parent ConceptsNeurologic (C0205494), Diagnosis/Diseases Component (C0497531), Trigeminal Autonomic Cephalalgias (C1565172), Vascular Headaches (C0042376), Brain Diseases (C0006111), Cluster Headache (C0009088), Neuralgia (C0027796), Headache Disorders (C0393735), Ambiguous concept (C1274012), Duplicate concept (C1274013)
SourcesCOSTAR, CST, DXP, ICPC, ICPCBAQ, ICPCDAN, ICPCDUT, ICPCFIN, ICPCFRE, ICPCGER, ICPCHUN, ICPCITA, ICPCNOR, ICPCPOR, ICPCSPA, ICPCSWE, LCH, MEDLINEPLUS, MSH, MTH, MTHICD9, NDFRT, QMR, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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