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Cephalalgia : An International Journal... Mar 2024Hemicrania continua (HC) and paroxysmal hemicrania (PH) belong to a group of primary headache disorders called trigeminal autonomic cephalalgias. One of the diagnostic...
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.
Topics: Humans; Female; Adult; Middle Aged; Aged; Male; Melatonin; Paroxysmal Hemicrania; Indomethacin; Headache; Trigeminal Autonomic Cephalalgias; Vascular Headaches; Anti-Inflammatory Agents, Non-Steroidal
PubMed: 38518183
DOI: 10.1177/03331024231226196 -
The Journal of Headache and Pain Mar 2024There is lack of population-based studies evaluating the prevalence of paroxysmal hemicrania, hemicrania continua and short-lasting unilateral neuralgiform headache...
BACKGROUND
There is lack of population-based studies evaluating the prevalence of paroxysmal hemicrania, hemicrania continua and short-lasting unilateral neuralgiform headache attacks.
OBJECTIVES
The aim of this study was to investigate the gender-specific 1-year prevalence of cluster headache, paroxysmal hemicrania, hemicrania continua, and short-lasting unilateral neuralgiform headache attacks.
METHODS
A nationwide study was conducted from January 1 2022 and December 31 2022 by linking diagnostic codes from Norwegian Patient Registry and prescription of relevant drugs from Norwegian Prescription Database on an individual basis. The 1-year prevalence with 95% confidence intervals (CI) of cluster headache, paroxysmal hemicrania, hemicrania continua and short-lasting unilateral neuralgiform headache attacks are estimated based on the combination of diagnostic codes, prescription of drugs and corresponding reimbursement codes.
RESULTS
Among 4,316,747 individuals aged ≥ 18 years, the 1-year prevalence per 100,000 was 14.6 (95% CI 13.5-15.8) for cluster headache, 2.2 (95% CI 1.8-2.7) for hemicrania continua, 1.4 (95% CI 1.0-1.8) for paroxysmal hemicrania, and 1.2 (95% CI 0.8-1.4) for short-lasting unilateral neuralgiform headache attacks. For all the trigeminal autonomic cephalalgies, cluster headache included, the prevalence was higher for women than men.
CONCLUSIONS
In this nationwide register-based study, we found a 1-year prevalence per 100,100 of 14.6 for cluster headache, 2.2 for hemicranias continua, 1.4 for paroxysmal hemicranias, and 1.2 for short-lasting unilateral neuralgiform headache attacks. This is the first study reporting higher prevalence of cluster headache for women than men.
Topics: Male; Female; Humans; Paroxysmal Hemicrania; Cluster Headache; Prevalence; Headache; SUNCT Syndrome; Neuralgia; Norway; Registries
PubMed: 38443787
DOI: 10.1186/s10194-024-01738-x -
Cephalalgia : An International Journal... Feb 2024Short-lasting unilateral neuralgiform headache attacks (SUNHA) have the features of both short-lasting unilateral neuralgiform pain, such as trigeminal neuralgia or... (Review)
Review
BACKGROUND
Short-lasting unilateral neuralgiform headache attacks (SUNHA) have the features of both short-lasting unilateral neuralgiform pain, such as trigeminal neuralgia or stabbing headache, and associated trigeminal autonomic symptoms, such as paroxysmal hemicrania or cluster headache. Recognizing and adequately treating SUNHA is essential but current treatment methods are ineffective in treating SUNHA.
METHODS
We reviewed the changes in the concept of short-lasting unilateral neuralgiform headache attacks and provide a narrative review of the current medical and surgical treatment options, from the first choice of treatment for patients to treatments for selective intractable cases.
RESULTS
Unlike the initial impression of an intractable primary headache disorder affecting older men, SUNHA affects both sexes throughout their lifespan. One striking feature of SUNHA is that the attacks are triggered by cutaneous or intraoral stimulation. The efficacy of conventional treatments is disappointing and challenging, and preventive therapy is the mainstay of treatment because of highly frequent attacks of a very brief duration. Amongst them, lamotrigine is effective in approximately two-third of the patients with SUNHA, and intravenous lidocaine is essential for the management of acute exacerbation of intractable pain. Topiramate, oxcarbazepine and gabapentin are considered good secondary options for SUNHA, and botulinum toxin can be used in selective cases. Neurovascular compression is commonly observed in SUNHA, and surgical approaches, such as neurovascular compression, have been reported to be effective for intractable cases.
CONCLUSIONS
Recent advances in the understanding of SUNHA have improved the recognition and treatment approaches for this unique condition.
Topics: Male; Female; Humans; Aged; SUNCT Syndrome; Headache; Anticonvulsants; Gabapentin; Lamotrigine; Neuralgia; Trigeminal Autonomic Cephalalgias
PubMed: 38415675
DOI: 10.1177/03331024241232256 -
Cureus Dec 2023This clinical case report aims to highlight the unusual presentation of Sneddon syndrome with a possible association with paroxysmal hemicrania. A medical record review...
This clinical case report aims to highlight the unusual presentation of Sneddon syndrome with a possible association with paroxysmal hemicrania. A medical record review was performed at a tertiary hospital in Riyadh, Saudi Arabia. Data collected include clinical evaluations and laboratory and imaging results. Informed consent was obtained. Hereby, we present a 27-year-old female who presented with multiple stroke attacks, along with severe headaches involving right retro-orbital pain with an eight-year history of spotted skin lesions. Initial unenhanced computed tomography (UCT) brain in the emergency showed left insular cortex hypodensity, revealing acute ischemic insult. Subsequent magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) revealed acute ischemic infarct in the territory of the left middle cerebral artery (MCA) involving the insula and frontoparietal lobe. Further investigations were done, including cerebrospinal fluid (CSF) analysis and autoimmune and infectious workup, which were unrevealing. Skin biopsy of the lesions showed subcutaneous fat necrosis with nonspecific scattered fibrinogen positivity and was labeled as livedo reticularis vs. livedo racemosa. A Sneddon syndrome diagnosis can be very challenging, needing a high index of suspicion to direct the diagnostic investigations. Moreover, the presence of a severe headache is an unusual phenomenon that needs further study.
PubMed: 38226111
DOI: 10.7759/cureus.50562 -
Cephalalgia : An International Journal... Nov 2023Paroxysmal hemicrania and hemicrania continua are indometacin-sensitive trigeminal autonomic cephalalgias, a terminology which reflects the predominant distribution of... (Review)
Review
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.
Topics: Humans; Paroxysmal Hemicrania; Trigeminal Autonomic Cephalalgias; Headache; Migraine Disorders; Vascular Headaches; Indomethacin
PubMed: 37950675
DOI: 10.1177/03331024231214239 -
Cephalalgia : An International Journal... Oct 2023To look at cigarette smoking history (personal and secondary exposure as a child) in non-cluster headache trigeminal autonomic cephalalgias seen at a headache clinic and...
OBJECTIVE
To look at cigarette smoking history (personal and secondary exposure as a child) in non-cluster headache trigeminal autonomic cephalalgias seen at a headache clinic and to determine smoking exposure prevalence utilizing previously published data.
METHODS
Retrospective chart review and PubMed/Google Scholar search.
RESULTS
Forty-eight clinic patients met ICHD-3 criteria for non-cluster headache trigeminal autonomic cephalalgias. Four had paroxysmal hemicrania, 75% were smokers and secondary exposure was noted in all. 16 patients had short lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) or short lasting unilateral neuralgiform headache attacks with autonomic symptoms (SUNA), 12.5% were smokers and secondary exposure was noted in 91%. Twenty-eight patients had hemicrania continua, 21% were smokers and secondary exposure was found in 62.5%.Since 1974 there have been 88 paroxysmal hemicrania, 50 SUNCT or SUNA and 89 hemicrania continua patients with a documented smoking exposure history. From current data and previous studies, a smoking history was noted in 60% paroxysmal hemicrania, 18% SUNCT and SUNA and 21% hemicrania continua patients.
CONCLUSION
A cigarette smoking history appears to be connected to paroxysmal hemicrania (personal and secondary exposure) and possibly to SUNCT/SUNA (secondary) and hemicrania continua (secondary).
Topics: Child; Humans; Cigarette Smoking; Paroxysmal Hemicrania; Retrospective Studies; Trigeminal Autonomic Cephalalgias; SUNCT Syndrome; Headache
PubMed: 37882655
DOI: 10.1177/03331024231208679 -
Frontiers in Pain Research (Lausanne,... 2023Occipital nerve stimulation (ONS) has been investigated as a potential treatment for disabling headaches and has shown promise for disorders such as chronic migraine and... (Review)
Review
BACKGROUND
Occipital nerve stimulation (ONS) has been investigated as a potential treatment for disabling headaches and has shown promise for disorders such as chronic migraine and cluster headache. Long term outcomes stratified by headache subtype have had limited exploration, and literature on outcomes of this neuromodulatory intervention spanning 2 or more years is scarce.
MEASURES
We performed a narrative review on long term outcomes with ONS for treatment of headache disorders. We surveyed the available literature for studies that have outcomes for 24 months or greater to see if there is a habituation in response over time. Review of the literature revealed evidence in treatment of occipital neuralgia, chronic migraine, cluster headache, cervicogenic headache, short lasting unilateral neuralgiform headache attacks (SUNHA) and paroxysmal hemicrania. While the term "response" varied per individual study, a total of 17 studies showed outcomes in ONS with long term sustained responses (as defined per this review) in the majority of patients with specific headache types 177/311 (56%). Only 7 studies in total (3 cluster, 1 occipital neuralgia, 1 cervicogenic headache, 1 SUNHA, 1 paroxysmal hemicrania) provided both short-term and long-term responses up to 24 months to ONS. In cluster headache, the majority of patients (64%) were long term responders (as defined per this review) and only a minority of patients 12/62 (19%) had loss of efficacy (e.g., habituation). There was a high number 313/439 (71%) of adverse events per total number of patients in the studies including lead migration, requirements of revision surgery, allergy to surgical materials, infection and intolerable paresthesias.
CONCLUSIONS
With the evidence available, the response to ONS was sustained in the majority of patients with cluster headache with low rates of loss of efficacy in this patient population. There was a high percent of adverse events per number of patients in long term follow-up and likely related to the off-label use of leads typically used for spinal cord stimulation. Further longitudinal assessments of outcomes in occipital nerve stimulation with devices labelled for use in peripheral nerve stimulation are needed to evaluate the extent of habituation to treatment in headache.
PubMed: 37021077
DOI: 10.3389/fpain.2023.1054764 -
Frontiers in Pain Research (Lausanne,... 2023Primary headache disorders can be remarkably disabling and the therapeutic options available are usually limited to medication with a high rate of adverse events. Here,... (Review)
Review
Primary headache disorders can be remarkably disabling and the therapeutic options available are usually limited to medication with a high rate of adverse events. Here, we discuss the mechanism of action of non-invasive vagal nerve stimulation, as well as the findings of the main studies involving patients with primary headaches other than migraine or cluster headache, such as hemicrania continua, paroxysmal hemicrania, cough headache, or short-lasting neuralgiform headache attacks (SUNCT/SUNA), in a narrative analysis. A bibliographical search of low-prevalence disorders such as rare primary headaches retrieves a moderate number of studies, usually underpowered. Headache intensity, severity, and duration showed a clinically significant reduction in the majority, especially those involving indomethacin-responsive headaches. The lack of response of some patients with a similar diagnosis could be due to a different stimulation pattern, technique, or total dose. The use of non-invasive vagal nerve stimulation for the treatment of primary headache disorders represents an excellent option for patients with these debilitating and otherwise refractory conditions, or that cannot tolerate several lines of preventive medication, and should always be considered before contemplating invasive, non-reversible stimulation techniques.
PubMed: 36994091
DOI: 10.3389/fpain.2023.1062892