Chronic Paroxysmal Hemicrania Clinical Presentation

Updated: Dec 08, 2014
  • Author: Manish K Singh, MD; more...
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Presentation

History

The pain in chronic paroxysmal hemicrania (CPH) is unilateral, always affects the same side, and is generally oculofrontotemporal in location. The literature does contain reports of a few unusual cases; for example, patients with bilateral symptoms and a possible shift in the side of the headache when the CPH progresses from the nonchronic to the chronic stage.

The pain is usually most severe in the oculotemporal area, the forehead, and above or behind the ear. Occasionally, pain can radiate and involve the ipsilateral shoulder, arm, and neck.

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

During severe attacks, excruciating pain that is throbbing, boring, pulsating, or clawlike in character has been described. In contrast to patients with CH, patients with CPH usually sit quietly or may curl up in bed between attacks.

The attack frequency usually is 10-20 attacks daily, but it may range from 2-40 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 can 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.

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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
  • Perform a careful physical examination to evaluate pathologic secondary headache
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