Indomethacin-responsive headache syndromes

Introduction
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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 headaches are divided into 2 distinct categories in the International Classification of Headache Disorders, 3rd edition, beta version (ICHD-3, beta version), which are (1) trigeminal autonomic cephalalgias (paroxysmal hemicrania, Code 3.2, and hemicrania continua, Code 3.4) and (2) other primary headaches, including Valsalva-induced headaches (primary cough headache, primary exercise headache, primary headache associated with sexual activity, Codes 4.1 to 4.3, and primary stabbing headache, Code 4.7). Cluster headache (Code 3.1), nummular headache (Code 4.8), and hypnic headache (Code 4.9) also have been described as other primary headache disorders 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 hemicrania continua, Code 3.4) and (2) other primary headaches, including Valsalva-induced headaches (primary cough headache, primary exercise headache, primary headache associated with sexual activity, Codes 4.1 to 4.3, and primary stabbing headache, Code 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) should be considered as possible underlying etiology before the diagnosis of primary headache associated with sexual activity.
  • Cluster headache may be also responsive to indomethacin.

In This Article

Introduction
Historical note and nomenclature
Clinical manifestations
Clinical vignette
Etiology
Pathogenesis and pathophysiology
Epidemiology
Prevention
Differential diagnosis
Diagnostic workup
Prognosis and complications
Management
Pregnancy
References cited
Contributors