4. Other primary headache disorders

General comment

Primary or secondary headache or both?

Two rules apply to 4. Other primary headache disorders, according to circumstances.

  1. When a new headache with the characteristics of any of the disorders classified here occurs for the first time in close temporal relation to another disorder known to cause headache, or fulfils other criteria for causation by that disorder, the new headache is coded as a secondary headache attributed to the causative disorder.
  2. When a pre-existing headache with the characteristics of any of the disorders classified here becomes chronic, or is made significantly worse (usually meaning a two-fold or greater increase in frequency and/or severity), in close temporal relation to such a causative disorder, both the initial headache diagnosis and the secondary headache diagnosis should be given, provided that there is good evidence that the disorder can cause headache.

Introduction

This chapter includes a number of primary headache disorders that are clinically heterogeneous. They are grouped into four categories and coded in sequence in ICHD-3 accordingly.

  1. Headaches associated with physical exertion, including 4.1 Primary cough headache, 4.2 Primary exercise headache, 4.3 Primary headache associated with sexual activity and 4.4 Primary thunderclap headache.
  2. Headaches attributed to direct physical stimuli (considered to be primary headache disorders because they are brought on by physiological [non-damaging] stimuli), including 4.5 Cold-stimulus headache and 4.6 External-pressure headache.
  3. Epicranial headaches (ie, head pain over the scalp), including 4.7 Primary stabbing headache and 4.8 Nummular headache (as well as A4.11 Epicrania fugax in the Appendix).
  4. Other miscellaneous primary headache disorders including 4.9 Hypnic headache and 4.10 New daily persistent headache.

The pathogenesis of these disorders is still poorly understood, and their treatments are suggested on the basis of anecdotal reports or uncontrolled trials.

Headaches with similar characteristics to several of these disorders can be symptomatic of another disorder (ie, secondary headaches); when they first present, they demand careful evaluation by imaging and/or other appropriate tests. The onset of some of these headaches (for example, 4.2 Primary exercise headache, 4.3 Primary headache associated with sexual activity and 4.4 Primary thunderclap headache) can be acute, and affected patients are sometimes assessed in emergency departments. Appropriate and full investigation (neuroimaging, in particular) is mandatory in these cases.