Hemicrania continua

Carrie Dougherty MD (Dr. Dougherty of the Georgetown Headache Center at MedStar Georgetown University Hospital received consulting fees from Amgen.)
Shuu-Jiun Wang MD, editor. (Dr. Wang of the National Yang-Ming University School of Medicine and the Neurological Institute, Taipei Veterans General Hospital received consulting fees from Eli Lilly.)
Originally released October 1, 2001; last updated February 22, 2017; expires February 22, 2020

Overview

This article outlines the clinical characteristics and treatment of hemicrania continua, a strictly unilateral, continuous headache with ipsilateral cranial autonomic features and a complete response to indomethacin. Clinical history is important in the diagnosis, and secondary causes are also well described. Indomethacin is the gold standard treatment, but side effects may limit use, and many other treatments have reported efficacy. This update includes case reports of new alternative treatment options for hemicrania continua, including sphenopalatine ganglion block and noninvasive vagus nerve stimulation.

Key points

 

• Hemicrania continua is a daily and continuous unilateral headache that is associated with cranial autonomic features and an absolute response to indomethacin. A unilateral headache that does not respond to indomethacin is not hemicrania continua.

 

• All patients with a unilateral headache should have an oral or IM indomethacin test at appropriate doses (orally up to at least 150 mg daily and IM 100 mg indomethacin).

 

• Headache diaries must be kept to help identify the disorder and demonstrate the response to treatment.

 

• Due to the high rates of side effects, patients should be kept on the lowest dose of indomethacin possible to control their pain.

 

• Alternative treatment options to be considered are COX-2 inhibitors, topiramate, verapamil, gabapentin, melatonin, occipital nerve blocks, and occipital nerve stimulation.

 

• All patients thought to have hemicrania continua should have an MRI brain scan.

Historical note and terminology

Hemicrania continua is one of the primary chronic daily headache disorders. It is characterized by a continuous unilateral headache of moderate intensity with exacerbations of severe pain and is often associated with migrainous and cranial autonomic features. Hemicrania continua almost invariably has a prompt and enduring response to indomethacin.

The earliest recognition of a headache syndrome involving one side of the head is attributed to Aretaeus of Cappadocia (in the 2nd century AD) (Silberstein and Peres 2002). However, Egyptian descriptions appear in papyri dating from 1500 BC (Borghouts 1971). Galen introduced the term "hemicrania" for unilateral headache. It was later transformed into the old English megrim and the French migraine. We now accept the term migraine derived from hemicrania although migraine differs from hemicrania continua in its episodic nature.

Medina and Diamond probably were the first authors to describe hemicrania continua in a subset of 54 patients who had cluster headache variants as well as strictly unilateral, continuous headaches that responded to indomethacin (Medina and Diamond 1981). In 1983, Boghen and Desaulniers described a patient with a similar headache that they called "background vascular headache responsive to indomethacin” (Boghen and Desaulniers 1983).

The term “hemicrania continua” was coined by Sjaastad and Spierings (Sjaastad and Spierings 1984). They reported a woman aged 63 years and a man aged 53 years who developed a strictly unilateral headache that was continuous from onset and absolutely responsive to indomethacin. In 2006, Sjaastad reported the long-term follow-up of the first woman with hemicrania continua until her death at age 81. She was treated with indomethacin during the whole observation time; no tachyphylaxis was observed, but she developed gastric ulcers secondary to the indomethacin (Sjaastad 2006). In 1987, the first case of hemicrania continua with a remitting course was reported (Sjaastad and Tjorstad 1987).

Debate is ongoing as to the existence of indomethacin-resistant hemicrania continua. Clinical experience has shown that a proportion of patients meeting all other criteria for hemicrania continua except an absolute indomethacin response are not uncommon. In one published series, 64% of patients were in this category (Marmura et al 2009), and in another, 31% (Prakash and Golwala 2012). Currently, it is more widely believed that indomethacin response is sine qua non for a diagnosis of hemicrania continua and that hemicranial pain that does not respond is a different type of headache as yet unclassified but termed “hemicranias incerta” (Pareja et al 2012) or “NIRCH” (non-indomethacin responsive chronic hemicrania) (Sjaastad and Vincent 2010; Antonaci and Sjaastad 2013).

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