Paroxysmal hemicrania

Peter J Goadsby MD PhD (Dr. Goadsby of King's College London and the University of California, San Francisco, received fees from Allergan, ATI, Boston Scientific, CoLucid, Eli Lilly, eNeura, MAP, Medtronic and MSD for board membership; consulting fees from AlderBio, Avanir, BMS, Dr Reddy, Hepatares, Nevrocorp, NuPathe, Pfizer, Teva and Zogenix; and consulting grants and fees from Amgen.)
Shuu-Jiun Wang MD, editor. (Dr. Wang of the National Yang-Ming University School of Medicine and the Neurological Institute, Taipei Veterans General Hospital received consulting fees from Eli Lilly.)
Originally released September 2, 1994; last updated December 3, 2016; December 3, 2019

This article includes discussion of paroxysmal hemicrania, chronic paroxysmal hemicrania, and episodic paroxysmal hemicrania. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

The author reviews paroxysmal hemicrania, one of the trigeminal autonomic cephalalgias. The short attack length (typically 20 minutes), high frequency of attacks (often 10 per day), and associated cranial autonomic features (such as lacrimation, conjunctival injection, and nasal symptoms), should trigger the diagnosis to be considered. When paroxysmal hemicrania is considered, investigation for pituitary gland pathology with dedicated MRI and blood tests is recommended, based on large case series. Paroxysmal hemicrania is absolutely responsive to indomethacin when it is tolerated at doses from 25 to 275 mg daily.

Key points

 

• Paroxysmal hemicrania is a unilateral, severe, short-lasting headache, typically of 20 minutes duration and occurring approximately 10 times a day.

 

• Paroxysmal hemicrania attacks are associated with cranial autonomic symptoms, such as lacrimation, conjunctival infection, and nasal symptoms.

 

• Paroxysmal hemicrania responds absolutely to indomethacin given orally.

 

• It is recommended that any patient with a diagnosis of paroxysmal hemicrania undergo investigation by MRI and blood tests for pituitary gland dysfunction.

Historical note and terminology

In 1974, Sjaastad and Dale reported what they described rather aptly as a new treatable headache entity (Sjaastad and Dale 1974). They subsequently coined the term “chronic paroxysmal hemicrania” to describe these patients (Sjaastad et al 1976). Later, a remitting form of the disease was recognized and termed “episodic hemicrania continua” (Kudrow et al 1987; Blau and Engel 1990; Newman et al 1992a; Goadsby and Lipton 1997; Veloso et al 2001). The release of the second edition of the International Headache Society Classification resulted in the introduction of the umbrella terminology “paroxysmal hemicrania,” which is recognized to have both an episodic and a chronic form (Headache Classification Committee of The International Headache Society 2004).

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