Chronic Paroxysmal Hemicrania (CPH): A Review of the Clinical Manifestations

Abstract

On a world-wide basis, 84 cases of CPH were found, 59 females and 25 males: i.e., a F:M ratio of 2.36. Forty-nine cases never exhibited a remitting stage, whereas in 35 cases a history of a remitting stage was obtained, 17 cases still remaining in the remitting stage. In other words, the ratio between the chronic and the remitting stage as of today is 67:17 = 3.94. Accordingly, there seems to be a reverse relationship of the chronic versus the remitting stage, when compared to cluster headache. A maximum attack frequency even of 5-6 attacks per 24 hours seems to be consistent with a diagnosis of CPH. Nocturnal attacks occurred in 55 out of 58 cases where such information was available. An unchanging unilaterality was the rule, in that only 3 exceptions have been reported.

Epsodic paroxysmal hemicrania with seasonal variation: case report and the EPH-cluster headache continuum hypothesis

“…The pain is described as throbbing or stabbing. It occurs in both sex, with a slight female preponderance (1,3:1), different from the female predominance seen in CPH cases (3:1) 3 . The age of onset varies from 12 to 51 years 4 .…”

Two More Secondary Headaches Mimicking Chronic Paroxysmal Hemicrania. Is This the Exception or the Rule?

“…It is extremely rare worldwide; ie, 84 cases reported from 1976 until 1989. 3 Not one of these cases had a family history of CPH and only a small percentage (23%) had a family history of migraine. The same lack of a familial incidence also exists in case reports after 1989.…”

“…There are also patients with an atypical late onset of CPH over 50 years of age, thus raising further questions concerning the idiopathic nature of this syndrome. 3,13 A significant percentage of patients with CPH have a history of head or neck trauma (more than 20% in Antonaci and Sjaastad's review 3 ), and a number of patients reported systematic diseases, such as diabetes, tumors, and collagen diseases, all of which can cause painful syndromes in the head. 3 The pathogenesis of CPH is obscure, 14 and in the past few years there have been reports of cases of symptomatic CPH in association with a variety of organic disorders.…”

“…2,3 Recently, a number of reports have described the coexistence of CPH and intracranial lesions or vascular disorders. [3][4][5] Two patients with CPH are presented. Laboratory examination revealed a tumor in the right parasellar area of the first patient, and physical examination of the second patient revealed a maxillary cyst.…”

“…1 It is considered a variant form of cluster headache but with differences in duration and frequency of attacks, sex distribution, and therapeutic response to indomethacin. 2,3 Recently, a number of reports have described the coexistence of CPH and intracranial lesions or vascular disorders. [3][4][5] Two patients with CPH are presented.…”