Prevalence and clinical features of hemicrania continua in clinic-based studies: A systematic review and  meta-analysis

Al-Khazali, Haidar Muhsen; Al-Khazali, Sarra; Iljazi, Afrim; Christensen, Rune Häckert; Ashina, Sait; Lipton, Richard B.; Amin, Faisal Mohammad; Ashina, Håkan

doi:10.1177/03331024221131343

Cited by 11 publications

(13 citation statements)

References 30 publications

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“…Both respond absolutely to indomethacin 1 . A recent meta‐analysis suggests hemicrania continua is more common in tertiary care settings, compared with our estimates for paroxysmal hemicrania 36 …”

Section: Discussionmentioning

confidence: 49%

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Epidemiology and clinical features of paroxysmal hemicrania: A systematic review and meta‐analysis

Henningsen,

Larsen,

Karlsson

et al. 2024

Headache

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ObjectiveTo assess the prevalence or relative frequency of paroxysmal hemicrania and its clinical features in the adult general population and among adult patients evaluated for headache in tertiary care.BackgroundParoxysmal hemicrania is a rare trigeminal autonomic cephalalgia with characteristic attacks of headache, associated cranial autonomic symptoms and signs, and an absolute response to indomethacin. Its epidemiological burden remains unknown in both the adult general population and among adult patients evaluated for headache in a tertiary care setting. Moreover, the frequencies of the clinical features associated with paroxysmal hemicrania have not been well established.MethodsA literature search of PubMed and Embase was conducted from January 1, 1988, to January 20, 2023. Eligible for inclusion were observational studies reporting the point prevalence or relative frequency of paroxysmal hemicrania or its clinical features in the adult general population or among adult patients evaluated for headache in tertiary care. Two independent investigators (M.J.H. and J.G.L.) performed the title, abstract, and full‐text article screening. Each included study's risk of bias was critically appraised using the Joanna Briggs Institute Critical Appraisal Checklist for Studies Reporting Prevalence Data. Estimates of prevalence or relative frequency were calculated using a random‐effects meta‐analysis. The between‐study heterogeneity was assessed using the I2 statistic and further explored with meta‐regression. This study was pre‐registered on PROSPERO (identifier: CRD42023391127).ResultsA total of 17 clinic‐based studies and one population‐based study met the eligibility criteria. Importantly, an overall high risk of bias was observed across the eligible studies. The relative frequency of paroxysmal hemicrania was estimated to be 0.3% (95% CI, 0.2%–0.5%) among adult patients evaluated for headache in tertiary care with considerable heterogeneity (I2 = 76.4%). No cases with paroxysmal hemicrania were identified among 1,838 participants in a population‐based sample. Moreover, the most prevalent cranial autonomic symptoms were lacrimation (77.3% [95% Cl, 62.7%–87.3%]), conjunctival injection (75.0% [95% Cl, 60.3%–85.6%]), and nasal congestion (47.7% [95% Cl, 33.6%–62.3%]).ConclusionsOur findings suggest that paroxysmal hemicrania is a rare disorder among adults evaluated for headache in tertiary care, while its prevalence in the general population remains unknown. Further studies focusing on the clinical features of paroxysmal hemicrania are warranted.

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Section: Discussionmentioning

confidence: 49%

“…1 A recent meta-analysis suggests hemicrania continua is more common in tertiary care settings, compared with our estimates for paroxysmal hemicrania. 36 Migraine is also an important differential diagnosis to consider when evaluating patients with suspected paroxysmal hemicrania.…”

Section: Differential Diagnosesmentioning

confidence: 99%

Epidemiology and clinical features of paroxysmal hemicrania: A systematic review and meta‐analysis

Henningsen,

Larsen,

Karlsson

et al. 2024

Headache

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“…Apart from the constant unilateral headache and autonomic symptoms, features of migraine are commonly seen in HC and PH patients. Symptoms including nausea, vomiting, photophobia, and phonophobia are reported (2,3,8,11). In this cohort, most of the patients reported some migrainous features as well.…”

Section: Discussionmentioning

confidence: 99%

“…According to the International Classification of Headache Disorders, 3rd edition (ICHD-3), HC is defined as unilateral headache that persists for more than three months, with at least one of the ipsilateral autonomic symptoms including conjunctival injection, lacrimation, nasal congestion, rhinorrhea, eyelid oedema, facial sweating, miosis or ptosis and a therapeutic response to indomethacin (1). A recent review suggested the three most common symptoms associated with HC were lacrimation, conjunctival injection and restlessness or agitation (11). PH is defined as severe unilateral headache lasting for 2-30 minutes with ipsilateral autonomic features.…”

Section: Introductionmentioning

confidence: 99%

Melatonin in hemicrania continua and paroxysmal hemicrania

Cheung,

Oliveira,

Goadsby

2024

Cephalalgia

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Background Hemicrania continua (HC) and paroxysmal hemicrania (PH) belong to a group of primary headache disorders called trigeminal autonomic cephalalgias. One of the diagnostic criteria for both HC and PH is the absolute response to the therapeutic dose of indomethacin. However, indomethacin is discontinued in many patients as a result of intolerance to its side effects. Melatonin, a pineal hormone, which shares similar chemical structure to indomethacin, has been reported to have some efficacy for HC in previous case reports and series. To our knowledge, there is no literature regarding the use of melatonin in PH. We aimed to describe the clinical use of melatonin in the preventive management of HC and PH. Methods Patient level data were extracted as an audit from routinely collected clinical records in consecutive patients seen in outpatient neurology clinic at King’s College Hospital, London, UK, from September 2014 to April 2023. Our cohort of patients were identified through a search using the keywords: hemicrania continua, paroxysmal hemicrania, melatonin and indomethacin. Descriptive statistics including absolute and relative frequencies, mean ± SD, median and interquartile range (IQR) were used. Results Fifty-six HC patients were included with a mean ± SD age of 52 ± 16 years; 43 of 56 (77%) patients were female. Melatonin was taken by 23 (41%) patients. Of these 23 patients, 19 (83%) stopped indomethacin because of different side effects. The doses of melatonin used ranged from 0.5 mg to 21 mg, with a median dose of 10 mg (IQR = 6–13 mg). Fourteen (61%) patients reported positive relief for headache, whereas the remaining nine (39%) patients reported no headache preventive effect. None of the patients reported that they were completely pain free. Two patients continued indomethacin and melatonin concurrently for better symptom relief. Eight patients continued melatonin as the single preventive treatment. Side effects from melatonin were rare. Twenty-two PH patients were included with mean ± SD age of 50 ± 17 years; 17 of 22 (77%) patients were female. Melatonin was given to six (27%) patients. The median dose of melatonin used was 8 mg (IQR = 6–10 mg). Three (50%) patients responded to melatonin treatment. One of them used melatonin as adjunctive treatment with indomethacin. Conclusions Melatonin showed some efficacy in the treatment of HC and PH with a well-tolerated side effect profile. It does not have the same absolute responsiveness as indomethacin, at the doses used, although it does offer a well-tolerated option that can have significant ameliorating effects in a substantial cohort of patients.

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“…As with PH, there are no population-based prevalence data as the diagnosis is reliant upon a therapeutic response to indometacin. From a meta-analysis of adults attending headache clinics, an estimate of relative frequencies was found to be 1.8% (73).…”

Section: Hemicrania Continuamentioning

confidence: 99%

Paroxysmal hemicrania and hemicrania continua: Review on pathophysiology, clinical features and treatment

Bahra

2023

Cephalalgia

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Background Paroxysmal hemicrania and hemicrania continua are indometacin-sensitive trigeminal autonomic cephalalgias, a terminology which reflects the predominant distribution of the pain, observable cranial autonomic features and shared pathophysiology. Understanding the latter is limited, both by low prevalence and the intricacies of studying brain function, requiring multimodal techniques to glean insights into such disorders. Similarly obscure is the curious response to indometacin. This review will address what is currently known about pathophysiology, the rationale for the current classification and, features which may confound the diagnosis, such as lack of cranial autonomic symptoms and those which are typically associated with migraine such as nausea, photophobia, phonophobia and aura. Despite these characteristics, a dramatic response to indometacin, which is not seen in migraine nor the other trigeminal autonomic cephalalgias , provides the hallmark of the diagnosis. The main clinical differential for paroxysmal hemicrania is based on temporal pattern and lies between cluster headache and short-lasting-neuralgiform headache attacks with tearing or additional cranial autonomic symptoms. For hemicrania continua it is more challenging as the main differential for which the disorder is often treated is migraine. A prior episodic pattern, often days at a time, and the tendency to exacerbation with analgesics will further deflect from the diagnosis. The relevance of this is that there is little overlap in therapeutics between paroxysmal hemicrania and hemicrania continua and other headache disorders and there are limited effective alternatives to indometacin. The most effective are other non-steroidal anti-inflammatory drugs including the newer COX-II inhibitors. Even though early reports suggest that a higher indometacin dose-requirement may herald a secondary precipitating pathology, this does not seem to be the case, with syndrome and response to treatment being similar with the primary disorder. In this context imaging of new onset paroxysmal hemicrania or hemicrania continua and implication of the results will be discussed as will alternative treatment options.

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Prevalence and clinical features of hemicrania continua in clinic-based studies: A systematic review and meta-analysis

Cited by 11 publications

References 30 publications

Epidemiology and clinical features of paroxysmal hemicrania: A systematic review and meta‐analysis

Epidemiology and clinical features of paroxysmal hemicrania: A systematic review and meta‐analysis

Melatonin in hemicrania continua and paroxysmal hemicrania

Paroxysmal hemicrania and hemicrania continua: Review on pathophysiology, clinical features and treatment

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