Autonomic Cephalgia and Primary Headache syndromes

The trigeminal autonomic cephalgias are a group of primary headache disorders characterised by unilateral trigeminal distribution pain that occurs in association with prominent ipsilateral cranial autonomic features e.g.

  • Ptosis; miosis; Nasal stuffiness; Nausea/vomiting; Tearing; eye lid oedema

NB IMPORTANT: Anyone with new onset, unilateral cranial autonomic features requires imaging with MRIb/MRIangio to exclude vascular/mass causes that could have severe consequences

Cluster Headache

  • Men>Women
  • 30-40 years old
  • Circadian and seasonal
  • Severe unilateral headache lasting 45-90 mins (20mins – 3hrs)
    • Occurring 1-8 times/day
    • Occur in ‘clusters’ that can last weeks/months
  • Treat with
    • Acutely: high flow oxygen (100%) and subcut sumatriptan (6mg)
    • Short-term: Steroids (2 weeks- reducing dose)
    • Long-term: Verapamil (prophylaxis)

Paroxysmal Hemicrania

  • Women > Men
  • 50-60 years old
  • Severe unilateral headache with pronounced unilateral autonomic features
    • Lasting 10-30mins (2-45mins)
    • Occurring 1-40 times/day
    • i.e. shorter duration but more frequent than cluster
  • Treat with indomethacin (patients usually have an absolute response)

Hemicrania continua

  • Essentially the same as P/H but features are persistent
  • There is an equally good response to indomethacin

Short lasting Unilateral Neuralgiform headache with Conjunctival Tearing/injection- SUNCT

  • Male > Female
  • Short lived (15-20 sec), unilateral neuralgiform (i.e. nerve pain) headache
    • With conjunctival tearing or injection (dominant autonomic feature)
  • Treat with gabapentin

Trigeminal Neuralgia (technically not a TAC as there are rarely any autonomic features)

  • Women>Men
  • More common in the elderly
  • Pain is triggered by touch and usually occurs in a particular facial distribution (V2 or V3)
    • Pain is severe and stabbing; brief (1-90sec) but can occur 10-100 times/day
    • Bouts of pain may last weeks/months before any sort of remission
  • Treat with carbemazepine/gabapentin/phenytoin/baclofen
    • Surgery may also be used to ablate/decompress the affected nerve
  • MRI is only indicated if there are focal signs, atypical features, poor response to medication or prior to surgery

Persistent Idiopathic Facial Pain (Stabbing Headache)

  • This is a diagnosis of exclusion and is usually treated with indomethacin (see above table)
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