Secondary Logo

Journal Logo

Institutional members access full text with Ovid®

Rare primary headaches

Holle, Dagny; Obermann, Mark

Current Opinion in Neurology: June 2014 - Volume 27 - Issue 3 - p 332–336
doi: 10.1097/WCO.0000000000000089
HEADACHE: Edited by Manjit Matharu

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.

Department of Neurology, University of Duisburg-Essen, Essen, Germany

Correspondence to Mark Obermann, MD, Department of Neurology, University of Duisburg-Essen, Hufelandstr. 55, 45147 Essen, Germany. Tel: +49 201 723 84385; fax: +49 201 723 5542; e-mail:

© 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins