Chronic Paroxysmal Hemicrania Clinical Presentation

Updated: Jun 12, 2019
  • Author: Monica Saini, MD, MBBS, MRCP(UK); more...
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The pain in chronic paroxysmal hemicrania (CPH) is characteristically unilateral. However, pain may switch sides between attacks and can rarely be bilateral. [16]

Patients usually report pain that is severe in intensity and has an abrupt onset and cessation. During severe attacks, excruciating pain that is throbbing, boring, pulsating, or clawlike in character has been described. The location of pain is primarily in the distribution of the ophthalmic division of the trigeminal nerve and C2, followed by the maxillary-mandibular and C3 distributions. Accompanying photophobia and phonophobia has been reported, usually lateralizing to the side of the pain. Occasionally, patients may experience nausea, though vomiting is rare. In 50%–80% of patients, agitation or restlessness may be noted. [10, 17]

Headache can develop at any time in patients with CPH, in contrast to CH, in which the headache usually occurs at night.

The attack frequency usually is 10–20 attacks daily, but it may range from 2 to 40 attacks daily. Attacks usually last 2–25 minutes, but they may last as long as 60 minutes. In a prospective study, mean attack duration was 13 minutes (range 3–46 min). In a retrospective study, the mean duration of attacks was 21 minutes (range 2–120 min).

CPH has been reported to be triggered by various stimuli, including neck movement, external pressure to the neck, or other factors.

CPH attacks are accompanied by autonomic symptoms, mostly on the same side as the pain, such as red eyes, tearing, nasal congestion, and, sometimes, rhinorrhea. Occasionally, photophobia may be present. Gastrointestinal symptoms are very rare.

Recognizing the various stages and different patterns of CPH is important. For example, during severe, frequent attacks, patients may describe a constant headache or persisting tenderness on the symptomatic side.


Physical Examination

The pain is severe in patients with CPH, and attacks are associated with autonomic features, such as the following:

  • Lacrimation - 62%

  • Conjunctival injection - 36%

  • Ipsilateral nasal congestion - 42%

  • Rhinorrhea - 36%

  • Eyelid edema - 33%

Lacrimation may occur bilaterally but is always more marked on the symptomatic side. Occasionally, mild ipsilateral miosis may be observed during attacks.

Patients with CPH who have had dissociation in pain and autonomic features also have been described. Other points to consider in the physical examination include the following:

  • No definite evidence points to a Hornerlike syndrome, such as that described in cluster headache (CH), but mild miosis and eyelid edema that may mimic ptosis may be observed

  • Forehead sweating may increase on the ipsilateral side, and patients with generalized sweating have been reported

  • The coexistence of CPH and trigeminal neuralgia is called CPH-tic syndrome; many cases of this syndrome have been reported

  • Simultaneous occurrence of ipsilateral CH and migraine headache in patients with CPH has been reported

  • PH has been reported to co-occur with primary cough and stabbing headache, which are also indomethacin-sensitive [18]

  • Perform a careful physical examination to evaluate pathologic secondary headache