Paroxysmal Hemicrania Health Article

Definition

Paroxysmal hemicrania (PH) is a rare form of headache. Paroxysmal hemicrania usually begins in adulthood, and affected persons experience severe throbbing, claw-like, or boring pain. The pain is usually on one side of the face, near or in the eye, temple, and occasionally reaching to the back of the neck. Red and tearing eyes, a drooping or swollen eyelid on the affected side of the face, and nasal congestion may accompany this pain. Persons experiencing the headache pain of paroxysmal hemicrania may also feel dull pain, soreness, or tenderness between attacks.

Description

Paroxysmal hemicrania syndromes have two forms: chronic, in which persons experience attacks on a daily basis for a year or more, and episodic, in which the headaches do not occur for months or years. Episodic paroxysmal hemicrania is four times more common than the chronic form.

Chronic paroxysmal hemicrania (CPH), also known as Sjaastad syndrome, is a primary headache disorder first described by the Norwegian neurologist Ottar Sjaastad in 1974. In 1976, Sjaastad proposed the term chronic paroxysmal hemicrania after observing two patients, who had daily, solitary, severe headache pain that remained on one side of the head. The main feature of chronic paroxysmal hemicrania is frequent attacks of strictly one-sided severe pain localized in or around the eye or temple regions, lasting from 2–45 minutes in duration, and occurring 2–40 times per day.

Attacks of chronic paroxysmal hemicrania do not occur in recognizable time patterns. Episodic paroxysmal hemicrania (EPH), a more rare form of the disorder, is characterized by bouts of frequent, daily attacks with the same clinical features of CPH, but separated by relatively long periods without headache. Most episodic headaches in paroxysmal hemicrania occur at night or other recognizable time patterns.

Demographics

In the United States, CPH is a rare syndrome, but the number of diagnosed cases is increasing. The prevalence of CPH is not known, but it occurs more often than cluster headaches, a disorder of that can sometimes be confused with CPH. Internationally, many cases of CPH have been described throughout the world, in different races and different countries.

Chronic paroxysmal hemicrania affects more women than men. In the past, because of female preponderance, CPH was considered a disease exclusive to women. However, CPH has been reported in increasing numbers of men. A study conducted in 1979 reported a female-to-male ratio of 7:1, but a review of 84 patients in 1989 reported a female-to-male ratio of 2.3:1. Chronic paroxysmal hemicrania can occur at any age, and the mean age of onset is 34 years.

Episodic paroxysmal hemicrania occurs in both sexes, with a slight female preponderance (1.3:1). The age of onset is variable; studies show EPH onset is 12–51 years.