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Hemicrania Continua: An Update
Sanjay Prakash1, Kalu Singh Rawat1
1Department of Neurology, Smt. B. K. Shah Medical Institute and Research Centre, Sumandeep Vidyapeeth, Piparia, Waghodia, Vadodara, Gujarat, India.
Insights
Hemicrania continua (HC) is often misdiagnosed. Key diagnostic features include continuous background pain and a positive response to indomethacin, crucial for accurate identification and treatment.
Area of Science:
- Neurology
- Headache Medicine
Background:
- Hemicrania continua (HC) is a recognized clinical headache disorder.
- Recent case series highlight its prevalence in headache clinics.
Observation:
- HC affects 1.7% of clinic patients, characterized by continuous unilateral pain with exacerbations.
- Associated symptoms include autonomic features and restlessness.
- The continuous background pain is the hallmark, often mild and overlooked, leading to misdiagnosis.
Findings:
- Exacerbations mimic other primary headaches and neuralgias.
- Approximately 75 secondary HC cases linked to 29 pathologies exist.
- Indomethacin responsiveness is a diagnostic criterion, though other drugs may help; pain recurs upon dose omission.
Implications:
- Misdiagnosis of HC is common due to varied presentations.
- Accurate diagnosis relies on identifying continuous background pain and indomethacin response.
- Understanding HC's features aids timely and correct patient management.
Background:
Hemicrania continua (HC) is not uncommon in clinical practice, and several large case series have been published in the recent past.
Objectives:
This review provides an overview of the recent advancement in different aspects of HC.
Methods:
We reviewed the articles published on HC in the last 2 decades.
Results:
HC constitutes 1.7% of patients with headache in the clinics. It presents with unilateral continuous background pain with periodic exacerbations, usually accompanied by cranial autonomic features and restlessness. The continuous background headache is the most consistent and central feature of HC. Although the duration of exacerbations varies from a few seconds to a few weeks, the frequency ranges from >20 attacks/day to one attack in several months. The background pain is mild to moderate in intensity and does not hamper routine activity. Patients and physicians frequently ignore the basal pain, and a case of HC is misdiagnosed as other headaches, depending on the pattern of exacerbations. The exacerbation mimics several primary headaches and neuralgias. There are about 75 cases of secondary HC, due to 29 different pathologies. Although an absolute response to indomethacin is part of the diagnostic criteria, a subset of patients may respond to several other drugs. Headache reappears immediately on skipping a single dose of effective drug. Several surgical procedures have been tried in patients who are intolerant to indomethacin.
Conclusion:
Misdiagnosis of HC is common. Continuous background pain and response to indomethacin are two essential features for the diagnosis of HC.
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