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.
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
Sjaastad O, Dale I. Evidence for a new (?), treatable headache entity. Headache. Jul 1974;14(2):105-8. [Medline].
Sjaastad O, Dale I. A new (?) Clinical headache entity "chronic paroxysmal hemicrania" 2. Acta Neurol Scand. Aug 1976;54(2):140-59. [Medline].
Headache Classification Committee of the International Headache Society. Classification and diagnostic criteria for headache disorders, cranial neuralgias and facial pain. Headache Classification Committee of the International Headache Society. Cephalalgia. 1988;8 Suppl 7:1-96. [Medline].
Russell D, Vincent M. Chronic paroxysmal hemicrania. In: The Headaches. 2nd ed. Philadelphia: Lippincott Williams & Wilkins; 2000:741-9.
Goadsby PJ, Lipton RB. A review of paroxysmal hemicranias, SUNCT syndrome and other short-lasting headaches with autonomic feature, including new cases. Brain. Jan 1997;120 ( Pt 1):193-209. [Medline].
Matharu M, May A. Functional and structural neuroimaging in trigeminal autonomic cephalalgias. Curr Pain Headache Rep. Apr 2008;12(2):132-7. [Medline].
Leone M, Bussone G. Pathophysiology of trigeminal autonomic cephalalgias. Lancet Neurol. Aug 2009;8(8):755-64. [Medline].
Sjaastad O, Apfelbaum R, Caskey W, Christoffersen B, Diamond S, Graham J, et al. Chronic paroxysmal hemicrania (CPH). The clinical manifestations. A review. Ups J Med Sci Suppl. 1980;31:27-33. [Medline].
Kudrow DB, Kudrow L. Successful aspirin prophylaxis in a child with chronic paroxysmal hemicrania. Headache. May 1989;29(5):280-1. [Medline].
Gladstein J, Holden EW, Peralta L. Chronic paroxysmal hemicrania in a child. Headache. Oct 1994;34(9):519-20. [Medline].
Broeske D, Lenn NJ, Cantos E. Chronic paroxysmal hemicrania in a young child: possible relation to ipsilateral occipital infarction. J Child Neurol. Jul 1993;8(3):235-6. [Medline].
Seidel S, Lieba-Samal D, Vigl M, Wöber C. Clinical features of unilateral headaches beyond migraine and cluster headache and their response to indomethacin. Wien Klin Wochenschr. Sep 2011;123(17-18):536-41. [Medline].
Evers S, Bauer B, Suhr B, Voss H, Frese A, Husstedt IW. Cognitive processing is involved in cluster headache but not in chronic paroxysmal hemicrania. Neurology. Jul 22 1999;53(2):357-63. [Medline].
Shabbir N, McAbee G. Adolescent chronic paroxysmal hemicrania responsive to verapamil monotherapy. Headache. Apr 1994;34(4):209-10. [Medline].
Dahlöf C. Subcutaneous sumatriptan does not abort attacks of chronic paroxysmal hemicrania (CPH). Headache. Apr 1993;33(4):201-2. [Medline].
Hannerz J, Jogestrand T. Intracranial hypertension and sumatriptan efficacy in a case of chronic paroxysmal hemicrania which became bilateral. (The mechanism of indomethacin in CPH). Headache. Jun 1993;33(6):320-3. [Medline].
Pascual J, Quijano J. A case of chronic paroxysmal hemicrania responding to subcutaneous sumatriptan. J Neurol Neurosurg Psychiatry. Sep 1998;65(3):407. [Medline]. [Full Text].
Print
