Hemicrania continua

Julish Selvy APRN (

Ms. Selvy of the Arkansas Children’s Hospital has no relevant financial relationships to disclose.

)
Aravindhan Veerapandiyan MD (

Dr. Veerapandiyan of University of Arkansas for Medical Sciences has no relevant financial relationships to disclose.

)
Shuu-Jiun Wang MD, editor. (

Dr. Wang of the Brain Research Center, National Yang-Ming University, and the Neurological Institute, Taipei Veterans General Hospital, received consulting fees from Eli Lilly, Daichi-Sankyo, and Novartis.

)
Originally released October 1, 2001; last updated July 25, 2019; expires July 25, 2022

Overview

This article summarizes the clinical characteristics and treatment of hemicrania continua, a primary chronic headache disorder. It is one of the trigeminal autonomic cephalgias characterized by a strictly unilateral, continuous headache with ipsilateral cranial autonomic features and a complete response to indomethacin. Clinical history is important in the diagnosis. It is often misdiagnosed as migraine, cluster headache, sinus headache, or dental pain. Indomethacin is the gold standard treatment, but side effects may limit use, and many other treatments have reported efficacy. This update includes the final diagnostic criteria published in the 3rd edition of The International Classification of Headache Disorders (ICHD-3).

Key points

 

• Hemicrania continua is an indomethacin-responsive trigeminal autonomic cephalalgia that is characterized by persistent, unilateral headaches with periodic exacerbations. Delays in the diagnosis can lead to unnecessary therapeutic interventions.

 

• A therapeutic trial of indomethacin should be done in all strictly unilateral headache patients who are not responding to other medications.

 

• A seasonal pattern with clustering is a diagnostic clue for hemicrania continua. The remitting type of hemicrania continua may mimic cluster headaches.

 

• The important feature of hemicrania continua is continuous background pain.

 

• Headache diaries must be kept to help identify the disorder by documenting details of both continuous background headaches and episodic exacerbations, respectively.

 

• Although the cause of hemicrania continua is unknown, people suspected of having this condition should be assessed for an underlying cause. Tests should include neuroimaging to rule out a secondary cause.

 

• Alternative treatment can be considered if the patient is unable to tolerate indomethacin. Options include melatonin, topiramate, verapamil, gabapentin, occipital nerve blocks, and occipital nerve stimulation.

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 1 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).

In the current ICHD-3, the trigeminal autonomic cephalgias group includes cluster headache; paroxysmal hemicrania; short-lasting unilateral neuralgiform headache attacks (SUNHAs); and their 2 subforms, SUNHAs with conjunctival injection and tearing (SUNCT) and SUNHAs with cranial autonomic symptoms (SUNA). Hemicrania continua is also now included in the trigeminal autonomic cephalgias group (Ravishankar 2018; Burish 2018).

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