Paroxysmal headache

General

  • A primary headache syndrome characterized by recurrent unilateral episodes of headache associated with cranial autonomic symptoms

Number

  • Prevalence of 1%.
  • Can begin at any age
  • Mean of onset of 37 years

Diagnostic criteria

A. At least 20 attacks fulfilling criteria B to E without a remission period, or with remissions lasting less than three months, for at least one year:
B. Severe unilateral orbital, supraorbital, or temporal pain lasting 2 to 30 minutes
C. Either or both of the following:
  • At least one of the following symptoms or signs, ipsilateral to the headache:
    • Conjunctival injection or lacrimation
    • Nasal congestion or rhinorrhea
    • Eyelid edema
    • Forehead and facial sweating
    • Miosis or ptosis
  • A sense of restlessness or agitation
D. Occurring with a frequency greater than five per day
E. Prevented absolutely by therapeutic doses of indomethacin
F. Not better accounted for by another ICHD-3 disorder

Aetiology

  • Unknown

Mechanism

  • Pain and autonomic symptoms due to communication via neuropeptides between hypothalamic nuclei and the trigeminal and facial nerves.
    • Hypothalamus releases neuropeptides which relay pain signals to the trigeminal nerve
      • Orexin-A (antinociceptive properties)
      • Orexin B (pronociceptive properties),
  • Mediated by trigeminovascular pathways via activation of the ophthalmic division of the trigeminal nerve, with cranial autonomic symptoms mediated by activation of facial nerve parasympathetic fibers

Clinical features

  • Headache
    • Quality (sharp, stabbing)
    • Duration (2 to 30 minutes),
    • Associated cranial autonomic features are the key features that suggest a diagnosis of CPH.
      • Unilateral autonomic features such as lacrimation, conjunctival injection, eyelid edema, ptosis, miosis, mydriasis, or diaphoresis.
    • Location
      • Tenderness to palpation in the periorbital and temporal region
    • Attacks
      • Commonly spontaneous,
      • 10% of patients head turning can trigger an attack.
        • Provoked by
          • Bending or rotating the head
          • Applying external pressure against the transverse processes of vertebrae C4-C5, C2 root,
          • Greater occipital nerve.

Imaging

  • Positron emission tomography (PET) and functional magnetic resonance imaging (fMRI) have shown contralateral hypothalamic activation

Treatment

  • Indomethacin
    • A nonsteroidal anti-inflammatory drug, is the first-line treatment for chronic paroxysmal hemicrania.
    • 25 mg or 50 mg orally three times a day