Indomethacin-responsive headache syndromes

Introduction
Article section 1 of 15.  Next

By Ping-Kun Chen MD

Indomethacin-responsive headache syndromes represent a unique group of primary headache disorders characterized by a prompt and often complete response to indomethacin. Indomethacin-responsive headache syndromes are divided into 2 distinct categories in the International Classification of Headache Disorders, 2nd edition (ICHD-2): trigeminal autonomic cephalalgias (paroxysmal hemicrania, Code 3.2) and other primary headaches, including primary stabbing headache and Valsalva-induced headaches (primary cough headache, primary exertional headache, primary headache associated with sexual activity, and hemicrania continua, Codes 4.1-4.4, 4.7). Hypnic headache (Code 4.5) has been described as another primary headache disorder that may respond to indomethacin.

Key points

  • Indomethacin-responsive headache syndromes can be divided into 2 groups: (1) trigeminal autonomic cephalalgias (paroxysmal hemicrania, Code 3.2) and (2) other primary headaches (primary stabbing headache, primary cough headache, primary exertional headache, primary headache associated with sexual activity, and hemicrania continua, Codes 4.1-4.4, 4.7).
  • Not all hemicrania continua are side-locked and responsive to indomethacin treatment.
  • Primary cough headache is usually self-limited.
  • Reversible cerebral vasoconstriction syndrome (RCVS) is an important underlying etiology of primary headache associated with sexual activity.
  • Cluster headache may be also responsive to indomethacin.