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  • Updated 01.19.2022
  • Released 09.02.1994
  • Expires For CME 01.19.2025

Paroxysmal hemicrania

Introduction

Overview

The author reviews paroxysmal hemicrania, one of the trigeminal autonomic cephalalgias that is indomethacin-responsive (123). Strictly unilateral pain of short attack length (typically 20 minutes), high frequency of attacks (often 10 per day), and associated lateralized 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 (105). They subsequently coined the term “chronic paroxysmal hemicrania” to describe these patients (106). Later, a remitting form of the disease was recognized and termed “episodic hemicrania continua” (54; 10; 77; 40; 120). 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 (46).

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