Rare Primary Headaches

Dagny Holle; Mark Obermann


Curr Opin Neurol. 2014;27(3):332-336. 

In This Article

Abstract and Introduction


Purpose of review Little is known about most disorders classified as rare primary headaches. This review summarizes current knowledge about clinical presentation, pathophysiology, epidemiology and treatment options of rare headache disorders. Increasing public awareness on the different types of rare primary headache disorders may lead to more research and better patient identification and treatment.

Recent findings The International Headache Society updated the classification criteria for primary headache disorders adding some clinical entities and moving others to different parts of the classification.

Summary Neuroimaging should be performed in all patients suspected to suffer from rare primary headache to rule out secondary causes of headache. Indometacin might have a good therapeutic effect in some of these headache entities, but larger randomized controlled trials are missing.


Headache disorders are considered to be rare when the prevalence in the general population is below 1% or unknown but regarded as uncommon. In Europe, a rare disorder is defined as disease not affecting more than five people in 10 000 persons in the general population. For most rare primary headache disorders, the true prevalence remains unknown or relies on case reports in the literature or small cohorts collected from tertiary headache centres. These headaches are partly summarized in Part one, Chapter 4, of the International Classification of Headache Disorders (ICHD; Other primary headaches, Table 1). In 2013, the new ICHD-3 was introduced as a beta version to be field tested in the real world.[1] Incorporation into the International Classification of Diseases (ICD-11) is planned in the future. Some of these disorders were promoted primary headaches or reclassified in the new headache classification. Nummular headache, for example, was promoted from the appendix of ICHD-2 as more and more evidence emerged that it should be regarded as primary headache. Cold-stimulus headache and external-pressure headache were moved from Chapter 13, and hemicrania continua was finally classified as trigeminal-autonomic cephalalgia (TAC) and moved to Chapter 3, Table 1.

Most of these headaches may also present as secondary headaches and need special consideration in everyday clinic and emergency department settings. Cerebral imaging is mandatory in most cases and the definite diagnosis and classification as primary headache disorder should be made only after careful exclusion of symptomatic origin of presenting headache symptoms.