What is the treatment for trigeminal autonomic cephalgia?

Treatment of Trigeminal Autonomic Cephalgias

Trigeminal autonomic cephalgias (TACs) require specific treatments based on the subtype, with lamotrigine being the first-line treatment for SUNCT/SUNA, indomethacin for paroxysmal hemicrania and hemicrania continua, and oxygen/triptans acutely with verapamil prophylaxis for cluster headache. 1, 2, 3

Classification of TACs

TACs are a group of unilateral episodic pain syndromes characterized by:

• Unilateral head pain in the trigeminal distribution 1
• Associated autonomic features (tearing, conjunctival injection, rhinorrhea, etc.) 1, 3
• Different subtypes with distinct treatment responses 2, 3

Treatment by TAC Subtype

SUNCT/SUNA (Short-lasting Unilateral Neuralgiform headache attacks with Conjunctival injection and Tearing/Cranial Autonomic symptoms)

• First-line treatment: Lamotrigine (100-300 mg/day) 2, 4
• Acute treatment: Intravenous lidocaine (1-4 mg/kg/hour) can reduce attack frequency during exacerbations 2
• Alternative preventives:
  • Gabapentin (800-2700 mg/day) 2, 5
  • Topiramate (50-300 mg/day) 2, 4
  • Carbamazepine (200-1600 mg/day) 2

Paroxysmal Hemicrania

• Diagnostic and therapeutic gold standard: Indomethacin (75-150 mg/day) - complete response is required for diagnosis 2, 6
• Alternative options: Limited, as indomethacin response is a diagnostic criterion 3, 6

Cluster Headache

• Acute treatments:

  • 100% oxygen inhalation (high flow, 12-15 L/min via non-rebreather mask) 2, 3
  • Subcutaneous sumatriptan (6 mg) 2
  • Second-line: Nasal spray sumatriptan (20 mg) or zolmitriptan (5 mg) 2

• Bridge therapy (short-term prevention during cluster bout):

  • Corticosteroids: Oral prednisone (60-100 mg/day) or IV methylprednisolone (250-500 mg/day) for 5 days with taper 2, 5

• Long-term prophylaxis:

  • First-line: Verapamil (minimum 240 mg/day, often higher doses needed) 2, 3
  • Alternatives:
    • Lithium carbonate (800-1600 mg/day) 2, 5
    • Topiramate (100-200 mg/day) 2
    • Methylergonovine (0.4-1.2 mg/day) where available 2, 5

Hemicrania Continua

• Definitive treatment: Indomethacin (75-150 mg/day) - complete response required for diagnosis 3, 6

Treatment Algorithm for TACs

1. Accurate diagnosis - distinguish between TAC subtypes based on:

   • Attack duration (seconds to minutes for SUNCT/SUNA, minutes for paroxysmal hemicrania, hours for cluster headache) 1, 3
   • Attack frequency (multiple daily for SUNCT/SUNA and paroxysmal hemicrania, 1-8 daily for cluster headache) 3
   • Presence of refractory periods between attacks 1

2. Initiate subtype-specific treatment:

   • For SUNCT/SUNA: Start lamotrigine with slow titration 2, 4
   • For paroxysmal hemicrania or hemicrania continua: Trial of indomethacin 2, 6
   • For cluster headache: Provide both acute (oxygen, triptans) and preventive (verapamil) treatments 2, 3

3. Consider refractory options for treatment failures:

   • Neuromodulation techniques for refractory cluster headache 3
   • Combination therapy for SUNCT/SUNA 2, 5

Common Pitfalls and Caveats

• Misdiagnosis of TACs as trigeminal neuralgia can lead to inappropriate treatment - look for autonomic features which are prominent in TACs 1
• Indomethacin response is diagnostic for paroxysmal hemicrania and hemicrania continua - failure to try adequate doses may miss the diagnosis 6
• Verapamil for cluster headache often requires higher doses than used for cardiovascular indications - ECG monitoring is recommended 2, 3
• In patients over 50 with temporal region pain, always rule out giant cell arteritis which requires urgent steroid treatment 1