Headache

Cluster Headache

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Cluster Headache, Trigeminal Autonomic Cephalalgia

  • Pathophysiology
  1. Migraine Variant
  2. Postulated mechanisms
    1. Vascular dilation
    2. Trigeminal Nerve stimulation
    3. Circadian rhythm association (onset of Cluster Headaches often occurs during sleep)
  • Types
  • Trigeminal Autonomic Cephalalgia
  1. Cluster Headache
    1. Episodic (90%)
      1. At least 2 cluster periods each lasting one week or more (but less than one year)
      2. 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. Short-Lasting Unilateral Neuralgiform Headache Attacks with Conjunctival Injection or Tearing (SUNCT Headache)
      1. Very brief (<4 minute) recurrent cluster-like Headaches
    2. Paroxysmal Hemicrania
      1. Brief cluster-like Headaches <30 minutes relieved with Indomethacin
    3. Hemicrania Continua
      1. Continuous cluster-like Headache relieved with Indomethacin
  • Epidemiology
  1. Uncommon Headache type
    1. Contrast with the much more common Primary Headaches (i.e. Migraine Headache, Tension Headache)
    2. Prevalence of episodic Cluster Headache
      1. Lifetime: 1 in 1000
      2. One year: 53 per 100,000
  2. Much more common in men
    1. Episodic Cluster Headache: 4 to 1 male to female ratio
    2. Chronic Cluster Headache: 15 to 1 male to female ratio
  3. Age of onset
    1. Male: 20 to 40 years old
    2. Female: Onset peaks in 60s (especially in black women)
  4. Hereditary
    1. Autosomal Dominant inheritance pattern in 5% of Cluster Headache patients
    2. First degree relative with Cluster Headache confers up to a nearly 40 fold increase in Cluster Headache risk
    3. Associated with the HCRTR2 gene
  • Risk Factors
  • Symptoms
  1. Characteristics
    1. Deep pain
    2. Burning, stabbing, or lancinating type pain
  2. Severity
    1. Excruciating pain
    2. Patient may even consider Suicide (hence the common name, "Suicide Headache")
  3. Location
    1. Unilateral Headache typically behind one eye
    2. May be orbital, supraorbital or temporal pain
    3. Radiates to upper teeth, jaw or neck
  4. Timing
    1. At least 5 attacks within 10 days
    2. Occurs from every other day to as often as multiple daily episodes up to 8 per day
    3. Headaches last 15 to 180 minutes
    4. Usually recur at same time of day each day
    5. May awaken patient from sleep (esp. onset of REM)
    6. Recurrence over >1 year without remission of >1 month
      1. However, in those meeting initial criteria for Cluster Headaches, later remissions may last for months to years
  5. Triggers
    1. Sleep Apnea
    2. Vasodilators
      1. Nitroglycerin
      2. Alcohol
      3. Histamine
  6. Associated with at least one of the following
    1. Lacrimation
    2. Ipsilateral forehead or facial Flushing or sweating
    3. Ipsilateral Nasal Discharge
    4. Affected eye red with dilated Conjunctival vessels (Conjunctival injection)
    5. Restlessness, pacing or rocking head in hands
    6. Horner's Syndrome (30% of cases)
      1. Ipsilateral Ptosis
      2. Ipsilateral pupillary constriction (Miosis)
  • Evaluation
  • Imaging
  1. Not indicated unless Headache Red Flags are present
  • Diagnosis
  1. Characteristics: Five or more Headaches meeting the following criteria
    1. Severe to very severe unilateral orbital, supraorbital or temporal pain lasting 15-180 minutes if untreated
    2. Headaches occur from every other day to eight times daily
    3. Headache with at least one of the following ipsilateral autonomic symptoms
      1. Conjunctival injection or Lacrimation
      2. Nasal congestion or Rhinorrhea
      3. Eyelid Edema
      4. Forehead and facial sweating
      5. Miosis or Ptosis
      6. Restlessness or Agitation
  2. Timing
    1. Episodic Cluster Headache
      1. Two or more cluster periods lasting 7-365 days and separated by pain-free remissions >1 month
    2. Chronic Cluster Headache
      1. Episodes recur for more than 1 year without remission or with remission <1 month
  • 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 (2-30 minutes)
    2. More common in women ages 30-40 years old
    3. Responds well to Indomethacin
  3. Brief Neuralgiform Headache with Conjunctivitis
    1. Unilateral Headache with Conjunctival injection and tearing
    2. Episodes last <4 minutes with recurrence from 3 to 200 times daily
    3. More common in men ages 35 to 65 years old
    4. Refractory to most Headache treatment strategies
  4. Orbital Myositis
    1. Similar to Cluster Headache with longer duration
  5. Tension Headache
  6. Trigeminal Neuralgia
  • Management
  • Nonpharmacologic measures
  1. Relaxation Techniques
  2. Cognitive-behavior therapy
  3. Treat comorbid Mood Disorders
  4. Tobacco Cessation
  5. Alcohol cessation
  • Management
  • Abortive Treatment for Acute Cluster Headache
  1. See Migraine Treatment
  2. First line agents
    1. Triptan Agents
      1. Sumatriptan (Imitrex)
        1. Intranasal 20 mg (may repeat once in 24 hours)
        2. Subcutaneous: 6 mg SC (may repeat once after 1 hour)
          1. Significant pain relief with 6 mg dose in 75% of patients by 15 minutes (NNT 2.4)
          2. Higher dose (12 mg) adds adverse effects without additional benefit
      2. Zolmitriptan
        1. Oral: 5 mg orally (may repeat once in 24 hours)
        2. Intranasal 10 mg (two sprays of the 5 mg Inhaler)
          1. Significant pain relief in 63% of patients by 30 minutes (NNT 2.8)
    2. Oxygen Inhalation
      1. Apply 100% via nonrebreather face mask at 12-15 Liters per minute for 15-20 minutes
      2. Complete relief in 78% of patients
      3. Cohen (2009) JAMA 302(22): 2451-7 [PubMed]
  3. Second-line 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 20:85-91 [PubMed]
    2. Indomethacin
      1. Dose: 25-50 mg tid prn
      2. Effective in Hemicrania Continua (or Paroxysmal Hemicrania)
      3. May have delayed benefit
      4. May be reasonable to administer with other management to improve sustained relief
  4. Agents with weaker evidence
    1. Intranasal Dihydroergotamine 0.5 mg bilateral nares
      1. Reduces Headache severity
      2. Does not decrease cluster frequency or duration
      3. Andersson (1986) Cephalalgia 6:51-4 [PubMed]
    2. Intranasal Capsaicin
      1. Applied to ipsilateral nostril bid for 7 days
      2. Marks (1993) Cephalalgia 13:114-6 [PubMed]
    3. Octreotide (Sandostatin)
  • Management
  • Prophylaxis for Episodic Cluster Headaches
  1. See Migraine Prophylaxis
  2. Verapamil
    1. First-line agent for prophylaxis (best evidence)
    2. Dosing
      1. Minimum effective dose is 240 mg (as a single dose or in divided doses)
      2. Start: 80 mg orally three times daily (or XR at 240 mg orally once daily)
      3. Titrate: Increase to 120 to 160 mg orally three times daily (or up to XR at 480 mg orally once daily)
  3. Corticosteroids
    1. Consider as Bridging Therapy from acute cluster Headache Management to prophylaxis
    2. Prednisone 50 mg for 1-3 days and then tapering over 10-14 days
  4. Suboccipital Corticosteroid Injection
  5. Other agents (variable efficacy)
    1. Indomethacin 25-50 mg three times daily
    2. Anticonvulsants
      1. Valproic Acid
      2. Topiramate (Topamax)
      3. Gabapentin (Neurontin)
  6. Avoid agents with serious adverse effects
    1. Methylsergide
      1. No longer recommended due to systemic fibrosis
  • Management
  • Prophylaxis for Chronic Cluster Headaches
  1. Verapamil
    1. See dosing above under prophylaxis of episodic Cluster Headache
  2. Lithium
    1. Dose: 300-600 mg/day initially (Maximum 900 mg/day)
    2. Base dose on serum Lithium levels
    3. Requires careful monitoring
  3. Refractory management
    1. Deep Brain Stimulation