Cluster headache

Calcification of Trigeminal autonomic cephalalgias (TACs)

• 3.1 Cluster headache
• 3.2 Paroxysmal hemicrania
• 3.3 Short-lasting unilateral neuralgiform headache attacks
• 3.4 Hemicrania continua
• 3.5 Probable trigeminal autonomic cephalalgia

• Age of onset
  • Rare in children under age 10 years old
  • Male: 20 to 40 years old
  • Female: Onset peaks in 60s (especially in black women)
• Hereditary –   Autosomal Dominant inheritance pattern in 5% of Cluster Headache patients
• Pathophysiology – Trigeminal autonomic Cephalgia
• Symptoms:
  • Characteristics
    • Deep pain
    • Burning, stabbing, or lancinating type pain
  • Severity
    • Excruciating pain
    • Patient may even consider Suicide (hence the common name, “Suicide Headache”)
  • Location
    • Unilateral Headache typically behind one eye
    • May be orbital, supraorbital or temporal pain
    • Radiates to upper teeth, jaw or neck
  • Timing
    • At least 5 attacks within 10 days
    • Occurs from every other day to as often as multiple daily episodes up to 8 per day
    • Headaches last 15 to 180 minutes
    • Usually recur at same time of day each day
    • May awaken patient from sleep (esp. onset of REM)
    • Recurrence over >1 year without remission of >1 month
    • However, in those meeting initial criteria for Cluster Headaches, later remissions may last for months to years
  • Associated with at least one of the following
    • Lacrimation
    • Ipsilateral forehead or facial Flushing or sweating
    • Ipsilateral Nasal Discharge
    • Affected eye red with dilated Conjunctival vessels (Conjunctival injection)
    • Restlessness, pacing or rocking head in hands
    • Horner’s Syndrome (30% of cases)
      • Ipsilateral Ptosis
      • Ipsilateral pupillary constriction (Miosis)
  • Triggers
    • Sleep Apnea
    • Food containing nitrates
    • Nail varnisn
    • Petroleum
    • Vasodilators
    • Nitroglycerin
    • Alcohol
    • Histamine

Differential Diagnosis 

• Migraine Headache
  • Common Migraine features do not distinguish from Cluster Headache
    • Aura occurs in 14% of Cluster Headaches
    • Photophobia occurs in >50% of Cluster Headaches
    • Migraine Headaches are worsened with movement – Contrast with Cluster Headaches in which patients are restless and agitated
• Hemicrania Continua (or Paroxysmal Hemicrania)
  • Cluster-type Headache with brief duration (2-30 minutes)
  • More common in women ages 30-40 years old
  • Responds well to Indomethacin
• Brief Neuralgiform Headache with Conjunctivitis
  • Unilateral Headache with Conjunctival injection and tearing
  • Episodes last <4 minutes with recurrence from 3 to 200 times daily
  • More common in men ages 35 to 65 years old
  • Refractory to most Headache treatment strategies
• Tension Headache 
• Trigeminal Neuralgia
  • Typically affects second and third branches of the Trigeminal Nerve (V2, V3)
  • In contrast when the first branch (V1) is affected, findings are consistent with Cluster Headache
• Intracranial Mass (e.g. Pituitary Adenoma)

Management

• Abortive Treatment for Acute Cluster Headache.First line agents
  • Oxygen Inhalation
    • Apply 100% via nonrebreather Face Mask at 12-15 Liters per minute for 15-20 minutes
    • Complete relief in 78% of patients
  • Triptan Agents
    • Sumatriptan  – ntranasal 20 mg (may repeat once in 24 hours)
    • Zolmitriptan – Intranasal 10 mg (two sprays of the 5 mg Inhaler)
• Agents with weaker evidence
  • Intranasal Lidocaine 4-10% solution
    • Dose: 1 ml intranasally
    • Lidocaine 10% applied with cotton swab bilaterally for 5 minutes
    • May be repeated twice in 15 minutes prn
    • Relieves pain within 5-15 minutes
  • Indomethacin
    • Dose: 25-50 mg three times daily prn
    • Effective in Hemicrania Continua (or Paroxysmal Hemicrania)
    • May have delayed benefit
    • May be reasonable to administer with other management to improve sustained relief
  • Intranasal Capsaicin : Applied to ipsilateral nostril bid for 7 days

Prophylaxis

• Suboccipital Corticosteroid Injection
• Corticosteroids
  • Prednisone 50 mg for 1-3 days and then tapering over 10-14 days
• Verapamil
  • First-line agent for prophylaxis (best evidence)
  • Dosing
    • Minimum effective dose is 240 mg (as a single dose or in divided doses)
    • Start: 80 mg orally three times daily (or XR at 240 mg orally once daily)
    • Titrate: Increase to 120 to 160 mg orally three times daily (or up to XR at 480 mg orally once daily)
• Second-line agents when Verapamil is ineffective or contraindicated
  • Melatonin 10 mg orally daily
  • Galcanezumab (Emgality)
  • Indomethacin 25-50 mg three times daily
  • Anticonvulsants – Topiramate (Topamax), Gabapentin (Neurontin)