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