Search

Background: Cluster headache (CH) can lead to high disability and reduced quality of life (QoL). QoL should be assessed as an important outcome both in research and in clinical care. The 28-item Cluster Headache Quality of Life Scale (CH-QoL) is a valid self-report questionnaire to assess disease-specific QoL. A German version is lacking. This study aims to develop a German-language version and to determine its psychometric properties. Methods: The CH-QoL was translated into German by two headache experts and blindly back-translated by two professional translators. Additionally, the CH-QoL was tested for comprehensibility by nine persons with CH. In this multi-stage process, linguistic discrepancies were repeatedly discussed in an expert panel and appropriate modifications were conducted to optimize the translation. A cross-sectional online survey, comprising the CH-QoL and further self-report questionnaires such as the Cluster Headache Scales (CHS), yielded a sample of N = 106 persons with CH (53.8% female, M = 45.5 [SD = 11.8] years, 48.1% episodic CH, 51.9% chronic CH, 79.2% currently having recurring CH attacks). Results: Exploratory factor analysis revealed two clearly interpretable factors ("restriction of activities of daily living", and "impact on mood and interpersonal relationships"), which is in discrepancy to the four factors of the original English version. The model fit was good, with χ2(323) = 590.74, p <.001, RMSEA = 0.088, SRMR = 0.053, TLI = 0.857. Reliability was very good (McDonald's omega ω = 0.97, Subscale/Factor 1: ω = 0.96, Subscale/Factor 2: ω = 0.92). Correlational analyses (correlations with related questionnaires as well as with clinical parameters) confirmed convergent validity. Conclusions: Since the German version of the CH-QoL has very good psychometric properties, it is suitable for the assessment of disease-specific QoL in people with CH in German-speaking countries. Trial registration: This study is registered with the German Clinical Trials Register (DRKS-ID: DRKS00028475, registration date 03 March 2022). [ABSTRACT FROM AUTHOR]

Abstract Background Cluster headache (CH) can lead to high disability and reduced quality of life (QoL). QoL should be assessed as an important outcome both in research and in clinical care. The 28-item Cluster Headache Quality of Life Scale (CH-QoL) is a valid self-report questionnaire to assess disease-specific QoL. A German version is lacking. This study aims to develop a German-language version and to determine its psychometric properties. Methods The CH-QoL was translated into German by two headache experts and blindly back-translated by two professional translators. Additionally, the CH-QoL was tested for comprehensibility by nine persons with CH. In this multi-stage process, linguistic discrepancies were repeatedly discussed in an expert panel and appropriate modifications were conducted to optimize the translation. A cross-sectional online survey, comprising the CH-QoL and further self-report questionnaires such as the Cluster Headache Scales (CHS), yielded a sample of N = 106 persons with CH (53.8% female, M = 45.5 [SD = 11.8] years, 48.1% episodic CH, 51.9% chronic CH, 79.2% currently having recurring CH attacks). Results Exploratory factor analysis revealed two clearly interpretable factors (“restriction of activities of daily living”, and “impact on mood and interpersonal relationships”), which is in discrepancy to the four factors of the original English version. The model fit was good, with χ2(323) = 590.74, p

Background and methods: In this narrative review, we introduce key artificial intelligence (AI) and machine learning (ML) concepts, aimed at headache clinicians and researchers. Thereafter, we thoroughly review the use of AI in headache, based on a comprehensive literature search across PubMed, Embase and IEEExplore. Finally, we discuss limitations, as well as ethical and political perspectives. Results: We identified six main research topics. First, natural language processing can be used to effectively extract and systematize unstructured headache research data, such as from electronic health records. Second, the most common application of ML is for classification of headache disorders, typically based on clinical record data, or neuroimaging data, with accuracies ranging from around 60% to well over 90%. Third, ML is used for prediction of headache disease trajectories. Fourth, ML shows promise in forecasting of headaches using self-reported data such as triggers and premonitory symptoms, data from wearable sensors and external data. Fifth and sixth, ML can be used for prediction of treatment responses and inference of treatment effects, respectively, aiming to optimize and individualize headache management. Conclusions: The potential uses of AI and ML in headache are broad, but, at present, many studies suffer from poor reporting and lack out-of-sample evaluation, and most models are not validated in a clinical setting. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Objective: Cluster headache is associated with a decreased quality of life (QoL). The increased focus on patient-reported outcome measures (PROMS) has led to the creation of a tailored Cluster Headache Quality of Life scale (CHQ). Our objective was to create and authenticate a Dutch version of the CHQ (CHQ-D). Methods: The TRAPD model (Translation, Review, Adjudication, Pretesting, Documentation) was used to translate the CHQ from English to Dutch and ensure cross-cultural adaption. Pre-testing was performed in n = 31 participants, and validity was in a new sample of n = 40 participants who completed the CHQ twice at a 2-day interval. Intraclass correlation coefficient (ICC) and Cronbach's alpha were used to assess the validity and reproducibility of the CHQ-D. Results: To produce the CHQ-D, we made five modifications based on pretesting. Participants finished the questionnaire in a median time of 10 min (IQR:10.0, 17.5) and 90% within 20 min. The majority of participants (74.2%) did not find it burdensome at all. The reliability of the CHQ-D was excellent (Cronbach's alpha: 0.94; ICC: 0.94). Conclusion: The CHQ-D is a valid and practical instrument for QoL in individuals with cluster headache. We aim to use CHQ-D as PROM in clinical research in the Netherlands to enforce international collaborations and comparisons of studies. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Objective: To 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. Background: Paroxysmal 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. Methods: A 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). Results: A 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%]). Conclusions: Our 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. [ABSTRACT FROM AUTHOR]

Dural arteriovenous fistula represents a rare cause of secondary Trigeminal Neuralgia. To date, there have been 18 reported cases of successful treatment of trigeminal neuralgia with endovascular embolization. Here we describe a unique case of a 51-year-old man with right-sided petrous ridge dural arteriovenous fistula causing ipsilateral V1 pain and cranial autonomic dysfunction. Cerebral angiography confirmed a Cognard type 3 right-sided tentorial dAVF supplied by an enlarged meningo-hypophpyseal vessel from the extradural ICA and by the middle meningeal artery. The fistula drains towards the midline via deep cerebral veins, the internal cerebral vein and the straight sinus with likely variceal compression of the right trigeminal nerve root entry zone. Transarterial Onyx embolization resulted in complete obliteration of the dural arteriovenous fistula with immediate resolution of facial pain and autonomic symptoms. After 24 months, the patient remains symptom-free and has no radiographic or clinical evidence of fistula recurrence. To date, there have been no other cases reported of trigeminal neuralgia with cranial autonomic symptoms or SUNCT syndrome due to a dural arteriovenous fistula. The presence of cranial autonomic symptoms with trigeminal neuralgia or a diagnosis of SUNCT should not deter endovascular treatments of dural arteriovenous fistulas or treatment of other compressive vascular lesions. [ABSTRACT FROM AUTHOR]

Background and Purpose: Cluster headache is a relatively rare, disabling primary headache disorder with a major impact on patients' quality of life. This work presents evidence‐based recommendations for the treatment of cluster headache derived from a systematic review of the literature and consensus among a panel of experts. Methods: The databases PubMed (Medline), Science Citation Index, and Cochrane Library were screened for studies on the efficacy of interventions (last access July 2022). The findings in these studies were evaluated according to the recommendations of the European Academy of Neurology, and the level of evidence was established using GRADE (Grading of Recommendations Assessment, Development, and Evaluation). Recommendations: For the acute treatment of cluster headache attacks, there is a strong recommendation for oxygen (100%) with a flow of at least 12 L/min over 15 min and 6 mg subcutaneous sumatriptan. Prophylaxis of cluster headache attacks with verapamil at a daily dose of at least 240 mg (maximum dose depends on efficacy and tolerability) is recommended. Corticosteroids are efficacious in cluster headache. To reach an effect, the use of at least 100 mg prednisone (or equivalent corticosteroid) given orally or at up to 500 mg iv per day over 5 days is recommended. Lithium, topiramate, and galcanezumab (only for episodic cluster headache) are recommended as alternative treatments. Noninvasive vagus nerve stimulation is efficacious in episodic but not chronic cluster headache. Greater occipital nerve block is recommended, but electrical stimulation of the greater occipital nerve is not recommended due to the side effect profile. [ABSTRACT FROM AUTHOR]

Objective: To estimate the relative frequencies of hemicrania continua and its clinical features in adult patients who were evaluated for headache in a clinic-based setting. Methods: PubMed and Embase were searched for observational, clinic-based studies published between 1 January 2004 and 1 February 2022, that reported on the relative frequencies of hemicrania continua and its clinical features. Two independent investigators (HMA and SA-K) screened titles, abstracts, and full text-articles. A random-effects meta-analysis was conducted to estimate pooled relative frequencies of hemicrania continua and its clinical features across clinic-based studies. Results: Eleven clinic-based studies were deemed eligible for inclusion. Of these, eight studies reported on the relative frequency of hemicrania continua among adult patients (n = 9854) who were evaluated for headache in a tertiary care unit. The pooled relative frequency of hemicrania continua was found to be 1.8% (95% CI; 1.0–3.3). Considerable heterogeneity was noted across studies (I2 = 89.8%). The three most common symptoms associated with hemicrania continua were lacrimation (72.3%), conjunctival injection (69.8%), and restlessness/agitation (60.2%). Conclusion: The findings of this meta-analysis suggest that there is limited epidemiologic data on the relative frequencies of hemicrania continua and its clinical features. Standardized data acquisition and reporting are needed to estimate prevalence rates more accurately and to better understand epidemiologic patterns. This, in turn, should increase awareness of the impact that hemicrania continua has in clinical practice. [ABSTRACT FROM AUTHOR]

Purpose of Review : We explore the anatomy of the central and peripheral autonomic pathways involved in primary headache as well as the mechanisms for secondary headache associated with disorders of the autonomic nervous system. The prevalence and clinical presentation of cranial and systemic autonomic symptoms in these conditions will be discussed, with a focus on recent studies. Recent Findings: Several small studies have utilized the relationship between headache and the autonomic nervous system to identify potential biomarkers to aid in diagnosis of migraine and cluster headache. Headache in postural orthostatic tachycardia syndrome (POTS) has also been further characterized, particularly in its association with orthostatic headache and spontaneous intracranial hypotension (SIH). Summary: This review examines the pathophysiology of primary and secondary headache disorders in the context of the autonomic nervous system. Mechanisms of headache associated with systemic autonomic disorders are also reviewed. [ABSTRACT FROM AUTHOR]

Introduction: Trigeminal autonomic cephalalgia (TAC) is a type of one-sided cerebral painful headache, with attacks regularly accompanied by autonomic responses, such as tearing, runny nose, panic, nausea and vomiting on the affected side. Currently, the most common treatment strategies are drugs, nerve grafts and surgery. Clinical understanding of TACs is limited. Here, we report the case of thermocoagulation treatment of the pterygopalatine ganglion in an uncommon TAC under local anesthesia. Case Presentation: A rare case of TAC was treated with computed tomography (CT)-guided thermocoagulation within the pterygopalatine ganglion. Pain and autonomic signs were relieved immediately after surgery, with the patent retaining only slight numbness on the left side of the face. This numbness completely resolved at 6 months of follow-up and there was no recurrence. Discussion: Trigeminal autonomic cephalalgia seriously affects the patient's quality of life, but clinical understanding is limited. In the case reported here, we performed CT-guided thermocoagulation of the pterygopalatine ganglion at 90 °C for 180 s for treatment of a trigeminal autonomic headache. To our knowledge, this is the first report of using thermocoagulation at 90 °C to treat the pterygopalatine ganglion. We found that this strategy results in fewer side effects and is a more cost-effective treatment for such patients than other options. Conclusion: Computed tomography-guided thermocoagulation of the pterygopalatine ganglion at 90 °C for 180 s for treatment of trigeminal autonomic headache is a safe and economical treatment option. [ABSTRACT FROM AUTHOR]

Trigeminal autonomic cephalalgias (TACs) are discrete primary headache disorders, characterized by severe unilateral head pain, typically trigeminal distribution, with ipsilateral cranial autonomic symptoms. The conditions within this group are hemicrania continua, cluster headache, paroxysmal hemicrania, and short-lasting unilateral neuralgiform headache with conjunctival injection and tearing and short-lasting unilateral neuralgiform headache with autonomic symptoms. Several advances have been made in understanding the pathogenesis and evolving treatment options in TACs. This review will outline the advances and updates in each TAC. [ABSTRACT FROM AUTHOR]

Background: Contrary to pre-attack symptoms before an individual cluster headache attack, little is known about the pre-cluster symptoms before the onset of cluster bouts. We previously described pre-attack symptoms before cluster headache attacks. The aim of this study was to investigate characteristics of pre-cluster symptoms in patients with episodic cluster headache. Methods: In this multicentre study, 184 patients with episodic cluster headache were recruited between October 2018 and December 2020. They were interviewed by investigators and completed a structured questionnaire. To investigate pre-cluster and pre-attack symptoms, we assessed 20 symptoms and signs using the questionnaire. Results: The upcoming cluster bout was predictable in 35.3% (n = 65/184) of the patients. When present, pre-cluster symptoms occurred at a median duration of 7 days (interquartile range, 2.3–14 days) before the onset of the cluster bout. Patients with pre-cluster symptoms showed a higher proportion of women, prevalence of pre-attack symptoms and seasonal rhythmicity, frequency of cluster headache attacks per day, and total number of cluster bouts compared to patients without pre-cluster symptoms. In univariable and multivariable logistic regression analyses, female sex was associated with the predictability of pre-cluster symptoms (odds ratio = 2.297, p = 0.016). Conclusions: The upcoming cluster bout was predicted in approximately 35% of patients with episodic cluster headache, which may allow for an earlier preventive treatment and help understand the pathophysiology. [ABSTRACT FROM AUTHOR]

Background: Cluster headache is the most common primary headache disorder of the trigeminal autonomic cephalalgias, and it is highly disabling. Objective: We undertake a scoping review to characterize therapies to prevent and acutely treat cluster headache, characterize trial methodology utilized in studies, and recommend future trial "good practices." We also assess homogeneity of studies and feasibility for future network meta‐analyses (NMAs) to compare acute and preventive treatments for cluster headache. Methods: A priori protocol for this scoping review was registered and available on Open Science Forum. We sought studies that enrolled adult patients with cluster headache as identified by accepted diagnostic criteria. Both randomized controlled trials (RCTs) and observational studies (with a control group) were included. The interventions of interest were medications, procedures, devices, surgeries, and behavioral/psychological interventions, whereas comparators of interest were placebo, sham, or other active treatments. Outcomes were predefined; however, we did not exclude studies lacking these outcomes. A systemic search was conducted in Ovid Medline, Embase, and Cochrane. We performed a targeted search for conference abstracts from journals prominent in the field. Results: We identified 56 studies: 45 RCTs, four studies only available in clinical trial registries, and seven observational studies. Of the 45 RCTs, 20 focused on acute therapies and 25 on preventive therapies. Overall, we determined that it is feasible to pursue a NMA for acute therapy focusing on 15 or 30‐min headache reduction for acute trials, as we identified 11 trials in the combined population of patients with either episodic or chronic cluster headache (2 trials in populations with chronic cluster headache were also found). For preventive therapy of cluster headache, we identified trials with common outcomes that may be considered for NMA, however, as these trials had differences in treatment effect modifiers that could not be corrected, NMAs appear infeasible for this indication. We identified new studies looking at noninvasive vagal nerve stimulation, sphenopalatine ganglion stimulation, prednisone, and oxygen published since the most recent systematic review in the field, although these acute treatments were previously identified as effective. However, for calcitonin gene‐related peptide (CGRP) monoclonal antibodies, galcanezumab demonstrated effectiveness in episodic cluster headache, but a lack of effectiveness in chronic cluster headache, and fremanezumab was not effective for episodic nor chronic cluster headache. This finding highlights that CGRP monoclonal antibodies may not show a class effect in cluster headache prevention and need to be considered individually. Conclusions: We describe the treatment landscape of cluster headache for both acute and preventive treatments. Last, we present the NMAs we will undertake in acute therapies of cluster headache. [ABSTRACT FROM AUTHOR]

Sphenopalatine neuralgia (SPN) is a clinical condition characterized by unilateral periorbital, trigeminal-maxillary pain, and ipsilateral autonomic symptoms caused by peripheral irritation of the SP ganglion. SPN was a previous term for trigeminal autonomic cephalalgias (TACs), especially for cluster headache, with its pain characteristics and autonomic findings. Etiology, pain duration, and treatment response provide distinctive features. Its clinical recognition is difficult; however, distinguishing it from other headache syndromes is important to prevent inappropriate treatment approaches. This case report aimed to raise awareness of the diagnosis, differential diagnosis, and management of TACs by presenting successful treatment results with thermal coagulation directed to the SP ganglion.

Abstract Background Paroxysmal hemicrania has not been associated with ipsilateral weakness, loss of sensation and Horner’s syndrome. This report is the first of its kind documented in literature. Case presentation This was an elderly, sixty-five-year-old Chinese male who presented with a headache fulfilling criteria of paroxysmal hemicrania and was found to have signs of ipsilateral conjunctival injection, Horner’s syndrome, weakness and loss of sensation; with resolution of the patient’s physical signs after relief of the headache. Brain magnetic resonance imaging did not show any strokes or other headache mimics. The patient had a marked response to indomethacin and a decrease of headache intensity and frequency with indomethacin prophylaxis. Conclusions Paroxysmal hemicrania has joined the list of stroke chameleons and that it would be one of the differentials in a patient with hemiplegia, hemisensory loss, autonomic signs and severe headache. It suggests that paroxysmal hemicrania in the elderly present atypically.

Abstract Introduction The population rate of familial cluster headache (CH) has been reported to be as high as 20% however this varies considerably across studies. To obtain a true estimate of family history in CH, we conducted a systematic review and meta-analysis of previously published data. Methods Our systematic review involved a search of electronic databases (Medline, EMBASE, PubMed, CINAHL) to identify and appraise studies of interest utilising the PRISMA (Preferred Reporting Items for Systematic Review and Meta-Analysis) guidelines. To further ameliorate the accuracy of our analysis we included an additional unpublished cohort of CH patients recruited at a tertiary referral centre for headache, who underwent detailed family history with diagnostic verification in relatives. Data was extracted and meta-analysis conducted to provide a true estimation of family history. Results In total, we identified 7 studies which fulfilled our inclusion criteria. The estimated true prevalence of CH patients with a positive family history was 6.27% (95% CI:4.65–8.40%) with an overall I2 of 73%. Fitted models for gender subgroups showed higher estimates 9.26% (95% CI: 6.29–13.43%) in females. However the I2 for the female model was 58.42% and significant (p = 0.047). Conclusion Our findings estimate a rate of family history in CH to be approximately 6.27% (95% CI: 4.65–8.40%). While estimates were larger for female probands, we demonstrated high heterogeneity in this subgroup. These findings further support a genetic role in the aetiology of CH.

Background: Paroxysmal hemicrania (PH) is a severe short-lasting headache usually localized around the eye. It might occur in conjunction with ipsilateral autonomic manifestations of trigeminal nerve stimulation. PH responds well to indomethacin treatment; however, considering the adverse effects of indomethacin, its long-term use is a matter of question and investigations about other prophylactic medications are going on, but they are inconclusive. The current study aims to investigate the efficacy of prophylactic use of cyproheptadine to control PH symptoms., Materials and Methods: The current clinical trial was conducted on 20 children diagnosed with PH undergoing prophylactic treatment with cyproheptadine syrup at a dosage of 0.2-0.4 mg/kg twice daily for a period of 3 months. The duration, frequency, and severity of headaches were assessed at baseline and then monthly for 3 months., Results: Significantly shorter duration, less frequency, and less severity of headaches were observed in the postintervention assessments of the patients ( P < 0.001). The effect size analysis showed that the greatest effect of the treatment was on the intensity of the headache (effect size: 0.866) and the least effect was on duration of the headache (effect size: 0.775). Drowsiness (5%) and increased appetite (30%) were the only adverse effects of treatment with cyproheptadine., Conclusion: Findings of this study showed that short-term prophylactic cyproheptadine in divided doses of 0.2-0.4 mg/kg could appropriately improve PH in terms of frequency, duration, and the intensity of the attacks. Nevertheless, further investigations are strongly recommended., Competing Interests: There are no conflicts of interest., (Copyright: © 2024 Advanced Biomedical Research.)

Background and objective: There are five headache disorders composing the trigeminal autonomic cephalalgias (cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT), short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA), and hemicrania continua). Little is known about these disorders in the pediatric population. The objectives of this study are to report the full age ranges of pediatric trigeminal autonomic cephalalgias and to determine if pediatric-onset trigeminal autonomic cephalalgias display similar signs and symptoms as adult onset. Methods: Search criteria in Medline Ovid, Embase, PsycINFO, and Cochrane Library were created by a librarian. The remainder of the steps were independently performed by two neurologists using PRISMA guidelines. Inclusion criteria for titles and abstracts were articles discussing cases of trigeminal autonomic cephalalgias with age of onset 18 or younger, as well as any epidemiological report on trigeminal autonomic cephalalgias (as age of onset data was often found in the results section but not in the title or abstract). Data extracted included age of onset, sex, and International Classification of Headache Disorders criteria for trigeminal autonomic cephalalgias (including pain location, duration, frequency, autonomic features, restlessness) and some migraine criteria (photophobia, phonophobia, and nausea). Studies that did not meet full criteria for trigeminal autonomic cephalalgias were examined separately as "atypical trigeminal autonomic cephalalgias"; secondary headaches were excluded from this category. Results: In all, 1788 studies were searched, 86 met inclusion criteria, and most (56) examined cluster headache. In cluster headache, onset occurred at every pediatric age (range 1–18 years) with a full range of associated features. Autonomic and restlessness features were less common in pediatric patients, while migrainous features (nausea, photophobia, and phonophobia) were found at similar rates. The sex ratio of pediatric-onset cluster headache (1.8, 79 male and 43 female) may be lower than that of adult-onset cluster headache. Data for other trigeminal autonomic cephalalgias, while more limited, displayed most of the full range of official criteria. The data for atypical trigeminal autonomic cephalalgias were also limited, but the most common deviations from the official criteria were abnormal frequencies and locations of attacks. Conclusions: Trigeminal autonomic cephalalgias can start early in life and have similar features to adult-onset trigeminal autonomic cephalalgias. Specifically, pediatric-onset cluster headache patients display the full range of each criterion for cluster headache (except maximum frequency of six instead of eight attacks per day). However, cranial autonomic features and restlessness occur at a lower rate in pediatrics. Additional information is needed for the other trigeminal autonomic cephalalgias. As for expanding the ICHD-3 criteria for pediatric-onset trigeminal autonomic cephalalgias, we have only preliminary data from atypical cases, which suggests that the frequency and location of attacks sometimes extend beyond the official criteria. Trial Registration: This study was registered as a systematic review in PROSPERO (registration number CRD42020165256). [ABSTRACT FROM AUTHOR]

Objective: To use 1) newly generated data, 2) existing evidence, and 3) expert opinion to create and validate a new cluster headache screening tool. Methods: In phase 1 of the study, we performed a prospective study of an English translation of an Italian screen on 95 participants (45 with cluster headache, 17 with other trigeminal autonomic cephalalgias, 30 with migraine, and 3 with trigeminal neuralgia). In phase 2, we performed a systematic review in PubMed of all studies until September 2019 with diagnostic screening tools for cluster headache. In phase 3, a 6-person panel of cluster headache patients, research coordinators, and headache specialists analyzed the data from the first two phases to generate a new diagnostic screening tool. Finally, in phase 4 this new screen was validated on participants at a single headache center (all diagnoses) and through research recruitment (trigeminal autonomic cephalalgias only, as recruitment was essential but was otherwise low). Results: In total, this study included 319 unique participants including 109 cluster headache participants (95 total participants/45 cluster headache participants in phase 1, and 224 total participants/64 cluster headache participants in phase 4). It also found 123 articles on potential screening tools in our systematic review. In phase 1, analysis of the English translation of an Italian screen generated 7 questions with high sensitivity and specificity against migraine, trigeminal neuralgia, and other trigeminal autonomic cephalalgias, but had grammatical and other limitations as a general screening tool. In phase 2, the systematic review revealed nine studies that met inclusion criteria as diagnostic screening tools for cluster headache, including four where sensitivity and specificity were available for individual questions or small groups of questions. In phase 3, this data was reviewed by the expert panel to generate a brief (6-item), binary (yes/no), written screening test. In phase 4, a total of 224 participants completed the new 6-item screening test (81 migraine, 64 cluster headache, 21 other trigeminal autonomic cephalalgias, 35 secondary headaches, 7 neuralgias, 5 probable migraine, and 11 other headache disorders). Answers to the 6 items were combined in a decision tree algorithm and three items had a sensitivity of 84% (confidence interval or 95% confidence interval 73–92%), specificity of 89% (95% confidence interval 84–94%), positive predictive value of 76% (95% confidence interval 64-85%), and negative predictive value of 93% (95% confidence interval 88–97%) for the diagnosis of cluster headache. These three items focused on headache intensity, duration, and autonomic features. Conclusion: The 3-item Erwin Test for Cluster Headache is a promising diagnostic screening tool for cluster headache. [ABSTRACT FROM AUTHOR]

Objective: To validate the diagnoses and to investigate epidemiological data from an international, non‐clinic‐based, and large (n = 1604) survey of participants with cluster headache. Background: There are several limitations in current epidemiological data in cluster headache including a lack of large non‐clinic‐based studies. There is also limited information on several aspects of cluster headache, such as pediatric incidence. Methods: The International Cluster Headache Questionnaire was an internet‐based survey that included questions on cluster headache demographics, criteria from the International Classification of Headache Disorders (ICHD), and medications. Results: A total of 3251 subjects participated in the survey, and 1604 respondents met ICHD criteria for cluster headache. For validation, we interviewed a random sample of 5% (81/1604) of participants and confirmed the diagnosis of cluster headache in 97.5% (79/81). Pediatric onset was found in 27.5% (341/1583) of participants, and only 15.2% (52/341) of participants with pediatric onset were diagnosed before the age of 18. Men were more likely to have episodic cluster headache between ages 10 and 50, but the sex ratio was approximately equal for other ages. An overwhelming majority of respondents had at least one autonomic feature (99.0%, 1588/1604) and had restlessness (96.6%, 1550/1604), but many also had prototypical migrainous features including photophobia or phonophobia (50.1%, 804/1604), pain aggravated by physical activity (31.4%, 503/1604), or nausea and vomiting (27.5%, 441/1604). Interestingly, the first‐line medications for acute treatment (oxygen) and preventive treatment (calcium channel blockers) were perceived as significantly less effective in chronic cluster headache (3.2 ± 1.1 and 2.1 ± 1.0 respectively on a 5‐point ordinal scale) compared with episodic cluster headache (3.5 ± 1.0 and 2.4 ± 1.1, respectively, p < 0.001 for both comparisons). Conclusions: Cluster headache often occurs in the pediatric population, although they are typically not diagnosed until adulthood. The onset of cluster headache is the inverse of that in migraine; in migraine women are more likely to have migraine between ages 10 and 50 but the sex ratio is approximately equal otherwise. Prototypical migrainous features are not useful in differentiating cluster headache from migraine. Participant data from a large international study also suggest that chronic cluster headache is not only less responsive to newer treatments (like noninvasive vagus nerve stimulation and galcanezumab), but to traditional first‐line treatments as well. [ABSTRACT FROM AUTHOR]

Background: Indomethacin (IMC) as a prophylactic treatment is considered to be ineffective in cluster headache (CH). However, small series suggested the interest of IMC in CH. Some authors support that an IMC test is useful in all trigeminal autonomic cephalalgias. We described clinical features of IMC responders in a retrospective cohort of chronic cluster headache (CCH). Methods: This single-center and retrospective study was conducted in a tertiary care specialist headache center in France. Patients were selected between January 2007 and December 2008. We included all patients fulfilling CCH criteria (ICHD-3-beta). Data were collected from medical records. We recorded all the prescriptions of IMC as a prophylactic treatment. Responders were defined by 50% reduction in attack frequency; complete response was defined by disappearance of the attacks. The non-responders must have received at least 100 mg daily during 7 days. Results: The study consisted of 324 CCH, 121 female (37%) and 203 males (63%) with an average age at onset of 33.93 (± 14.71) years. Of the patients, 105 were treated with IMC. Thirty patients (29%) were responders. Thirty-four patients (32%) were non-responders. Responding status was undefined for 41 patients (39%). Twelve patients (11%) had a complete response. Responders were composed by 18 women (60%) and 12 men (40%) and had on average 44.89 (± 12.88) years. The minimal effective dose was 86.11 mg daily (± 48.72). Discussion: This study shows the interest of IMC in CCH patients. We recommend an IMC test as a third-line treatment in CCH. [ABSTRACT FROM AUTHOR]

Migraine and tension-type headache are common in children and adolescents, but several other headache disorders may pose a great challenge in diagnosis and management to families and attending clinicians. In this review, we highlight several of these disorders, which need appropriate assessment to make the right diagnosis and appropriate investigations where necessary. Timely recognition and implementation of appropriate management strategies can improve the health of children with some disorders, and is vital in achieving improvement in the quality of life. [ABSTRACT FROM AUTHOR]

Objective: To report two cases of hemicrania continua (HC) in a mother and daughter. Background: HC is a rare primary headache disorder belonging to the family of trigeminal autonomic cephalalgias (TACs). Unlike migraine, familial cases of TACs are rare, and we know relatively little of their inheritance pattern and genetic mechanisms. Methods: We present a mother and daughter with HC. We compare the similarities and differences between this family and the first report of familial HC and discuss the implications for future studies. Results: Both the mother and daughter presented with a constant, side‐locked headache of moderate intensity, with episodic exacerbations of more severe pain that are associated with ipsilateral cranial autonomic activation. After negative workup, both patients were started on indomethacin and achieved absolute response at different doses, confirming HC. Conclusions: Our report further corroborates other reports of familial TACs that TACs are primary headaches possibly attributable to genetic factors, albeit detailed mechanisms remain elusive. Nevertheless, whether clinical presentation and treatment responses would be substantially different between sporadic and familial HCs remain unclear. [ABSTRACT FROM AUTHOR]

Verapamil is the first-line preventive medication for cluster headache, an excruciating disorder with strong circadian features. Whereas second- and third-line preventives include known circadian modulators, such as melatonin, corticosteroids, and lithium, the circadian effects of verapamil are poorly understood. Here, we characterize the circadian features of verapamil using both in vitro and in vivo models. In Per2::LucSV reporter fibroblasts, treatment with verapamil (0.03–10 µM) showed a dose-dependent period shortening of the reporter rhythm which reached a nadir at 1 µM, and altered core clock gene expression at 10 µM. Mouse wheel-running activity with verapamil (1 mg/mL added to the drinking water) also resulted in significant period shortening and activity reduction in both male and female free-running wild-type C57BL6/J mice. The temporal patterns of activity reduction, however, differ between the two sexes. Importantly, piezo sleep recording revealed sexual dimorphism in the effects of verapamil on sleep timing and bout duration, with more pronounced adverse effects in female mice. We also found altered circadian clock gene expression in the cerebellum, hypothalamus, and trigeminal ganglion of verapamil-treated mice. Verapamil did not affect reporter rhythms in ex vivo suprachiasmatic nucleus (SCN) slices from Per2:Luc reporter mice, perhaps due to the exceptionally tight coupling in the SCN. Thus, verapamil affects both peripheral (trigeminal ganglion) and central (hypothalamus and cerebellum) nervous system structures involved in cluster headache pathophysiology, possibly with network effects instead of isolated SCN effects. These studies suggest that verapamil is a circadian modulator in laboratory models at both molecular and behavioral levels, and sex is an important biological variable for cluster headache medications. These observations highlight the circadian system as a potential convergent target for cluster headache medications with different primary mechanisms of action. [ABSTRACT FROM AUTHOR]

Background: Essential oils (EOs) with pro‐convulsant properties are known to cause seizures and may worsen migraine. Here we report five cases of cluster headache (CH) secondary to the usage of toothpastes containing pro‐convulsant EOs. Methods: Patients were identified from the headache clinics of three tertiary care hospitals in south India. Detailed history, examination, and brain magnetic resonance imaging were done in all patients. CH was diagnosed according to the International Classification of Headache Disorders, 3rd edition. Descriptive statistics were used to analyze data. Results: We had five cases of EO‐related CH (EORCH), from February 2020 to August 2021; three females and two males, with age ranging from 19 to 54 years. Three had new onset CH, while two had previous cluster attacks which had become refractory to medications for the past 1 year. The toothpastes contained EOs of camphor, eucalyptus, sage, thujone, clove, and fennel in various combinations. These toothpastes were used for a period of at least 3 months in those with new onset CH and for 12 months or more by those with chronic CH. After stopping the usage of these toothpastes, the CH attacks resolved completely within 5–10 days in all patients. In one patient we re‐challenged with the same toothpaste and got the CH attack after a period of 2 months. None of the patients had recurrence of CH attacks at follow‐up, ranging from 1 to 2 years. Conclusion: EOs with pro‐convulsive properties may trigger and sustain CH. Physicians may consider inquiring about the exposure to these pro‐convulsant EOs in patients with CH and may consider advising the discontinuation of products like toothpastes containing them as a possible means of CH remission. [ABSTRACT FROM AUTHOR]

Abstract Background The trigeminal autonomic cephalalgias are a group of primary headache syndromes marked by severe head pain and associated cranial autonomic symptoms which can include a full or partial Horner’s syndrome. Rarely, the eye-related symptoms will become fixed even between headache attacks. There is minimal documentation that the Horner’s syndrome can be reversed if successful treatment of the underlying headache disorder is initiated. Case reports Two cases are presented of trigeminal autonomic cephalalgia subtypes with chronic persistent Horner’s syndromes that alleviated with treatment of the underlying primary headache disorder. Patient 1, an 82-year-old Caucasian woman, presented with hemicrania continua with a partial Horner’s syndrome that was present for 2 years. She was unable to take indomethacin as she was on anticoagulation. After a C2–3 diagnostic facet injection, not only did she become pain free but her ptosis completely resolved. She then underwent a radiofrequency facet neurotomy with complete alleviation of head pain and complete resolution of her ptosis. Patient 2, a 21-year-old Caucasian woman, presented with long-lasting autonomic symptoms with hemicrania syndrome and a fixed miosis and ptosis of 6 months’ duration. After achieving 2 months of pain freedom on indomethacin her Horner’s syndrome completely resolved. Conclusion A chronic fixed partial or full Horner’s syndrome can occur in trigeminal autonomic cephalalgia subtypes, but it can also be reversed in patients with treatment even after months to years of duration. This would suggest that the sympathetic dysfunction leading to the eye-related symptoms is from irritation of the sympathetic chain rather than permanent injury as the result of vasodilatory trauma after trigeminal autonomic reflex activation.

Miguel Malo-Urriés,1 César Hidalgo-García,1 Elena Estébanez-de-Miguel,1 José Miguel Tricás-Moreno,1 Sonia Santos-Lasaosa,2 Marjan Jahanshahi3 1Physiotherapy Research Unit, Department of Physiatry and Nursing, University of Zaragoza, Zaragoza, Spain; 2Neurology Service, University Clinical Hospital Lozano Blesa, Zaragoza, Spain; 3Cognitive Motor Neuroscience Group, Department of Clinical and Movement Neurosciences, University College London Queen Square Institute of Neurology, The National Hospital for Neurology and Neurosurgery, London, UK Background: Based on inconsistent sensory alterations demonstrated in cluster headache (CH), the aim of this study was to determine whether patients with CH develop sensory changes in the symptomatic side compared to the asymptomatic side.Methods: Quantitative sensory testing (QST), including pressure pain threshold (PPT), tactile detection threshold (TDT), prick detection threshold (PDT), and two-point detection threshold (2PDT), was evaluated in 16 patients (seven women; age 41.9±6.8 years) with CH. Test sites included the first, second, and third divisions of the trigeminal nerve, cervical spine, and thenar eminence in the symptomatic and asymptomatic sides.Results: The symptomatic side, compared to the asymptomatic side, presented significantly decreased PPT in the first (P=0.011; 423.81±174.05 kPa vs 480.13±214.99 kPa) and second (P=0.023; 288.88±140.80 kPa vs 326.38±137.33 kPa) divisions of the trigeminal nerve, significantly increased TDT in the first (P=0.002; 2.44±0.40 vs 1.74±0.24) and second (P=0.016; 1.92±0.34 vs 1.67±0.09) divisions, and increased 2PDT in the first division (P=0.004; 18.13±4.70 mm vs 15.0±4.92 mm) and neck (P=0.007; 45.31±20.65 mm vs 38.44±16.10 mm).Conclusion: These results support the prior evidence suggesting a specific pattern of alteration of sensory function with alterations in the symptomatic side compared to the asymptomatic side. Keywords: cluster headache, trigeminal autonomic cephalalgia, quantitative sensory testing

Paroxysmal hemifacial pain (PHFP) is the orofacial counterpart to paroxysmal hemicrania headaches. This paper reports the cases of two patients suffering from episodic attacks of severe unilateral facial pain. In both cases, pain attacks were absolutely responsive to therapeutic doses of indomethacin. Both patients were diagnosed with PHFP, as per the International Classification of Orofacial Pain diagnostic guidelines. The diagnosis of PHFP, and a trial of indomethacin, must be considered in cases of severe unilateral facial pains not clearly explained by more common diagnoses. [ABSTRACT FROM AUTHOR]

Background: Greater and lesser occipital neuralgias are primary neuralgias that are relatively uncommon, where the pain is felt in the distribution of these nerves.Objective: This review paper was intended to describe the features and management of occipital neuralgia in the context of a challenging case.Material and Methods: We looked at succinct literature from the past 30 years. We compared the features of our challenging case given in the current literature. In addition, an overview of the current literature is provided.Results: The case, although proved to be a diagnostic challenge, we were able to reach a conclusion and render the patient almost complete pain relief by conservative management modalities. It proved to be a rare presentation of occipital neuralgia with unusual pain distribution, and we are able to describe a literature-based explanation for this entity to be a diagnostic and management challenge.Conclusion: Primary headaches, i'n general, are a group of headache disorders that require exquisite diagnostic skills. The clinical history is a key factor when making an accurate diagnosis, and to establish an appropriate management plan. [ABSTRACT FROM AUTHOR]

Background: Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache with autonomic symptoms (SUNA) are rare and disabling primary headache disorders that are subtypes of Short-lasting unilateral neuralgiform headache attacks (SUNHA).Aim: The aim of this narrative review was to provide a comprehensive update on headache phenotype, pathophysiology, and various treatment options available for SUNCT and SUNA.Methods: References for this review were identified by searches of articles published in the English language in PubMed between 1978 and October 2020 using "short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT)", "short-lasting unilateral neuralgiform headache with autonomic symptoms (SUNA)", "short-lasting unilateral neuralgiform headache attacks (SUNHA)", "trigeminal autonomic cephalalgias" as keywords in various combinations.Results: Of a potential 1103 articles, seven case series describing clinical characteristics of SUNCT/SUNA patients were identified for this review. For symptomatic/secondary SUNCT/SUNA, 53 individual case reports, and one case series were reviewed. One placebo-controlled trial and 11 open-label case series that evaluated various medical and surgical treatments in SUNCT/SUNA were also reviewed. Available literature suggests that SUNCT and SUNA are subtypes of the same disorder characterized by severe side locked short duration headache with ipsilateral prominent cranial autonomic symptoms and signs. Pathophysiology may involve both peripheral and central mechanisms. Lamotrigine is the most effective preventive therapy while intravenous lidocaine is the most efficacious drug as transitional therapy for severe disabling attacks. Surgical options including microvascular decompression in those having neurovascular conflict, occipital nerve stimulation, and hypothalamic deep brain stimulation can be alternative treatment options for medically refractory patients. [ABSTRACT FROM AUTHOR]

Paroxysmal Hemicrania (PH) is classified under trigeminal autonomic cephalalgia (TAC) as per the International Classification of Headache Disorders (ICHD). Since the first description by 0ttar Sjaastad and Inge Dale in 1974, PH has been reported by many authors. A greater understanding of PH phenotype and pathophysiology has resulted in the evolution of its diagnostic criteria, and management. We tabulated major case series of PH to describe the epidemiology, clinical features and recent updates of PH. PH is a rare headache characterized by daily, multiple paroxysms of unilateral, short-lasting (mean duration <20 minutes), side-locked headache in the distribution of ophthalmic division of trigeminal nerve with associated profound cranial autonomic symptoms. Recent ICHD classification added "restlessness" to the criteria for PH. Pain should completely respond to indomethacin to fulfil the diagnostic criteria of PH. PH should be differentiated from cluster headache, SUNCT/SUNA, and other short-lasting side-locked headaches. Trigeminal afferents possibly produce pain in PH and trigeminal-autonomic reflex explains the occurrence of autonomic features. Recently, a "permissive" central role of the hypothalamus is unveiled based on functional imaging studies. Other Cox-2 inhibitors, topiramate, calcium-channel blockers, epicranial nerve blocks have been shown to improve headache in some patients of PH who cannot tolerate indomethacin. Hypothalamic deep brain stimulation has been used in treatment-refractory cases. [ABSTRACT FROM AUTHOR]

Background: Previous studies have reported notable differences in demographic and clinical features of cluster headache between Western and Asian populations, including lower prevalence of the chronic type and in women. Recently, prodromal symptoms of migraine and pre-attack symptoms of cluster headache have drawn attention regarding their potential pathophysiological implications and pre-emptive treatment. However, pre-attack symptoms of cluster headache have not been studied in the Asian population. Methods: A total of 136 patients with cluster headache (21 first-onset, 110 episodic, and five chronic cases) were recruited in this multi-center study between October 2018 and December 2019. We evaluated the characteristics of pre-attack symptoms in a current bout using a structured questionnaire. Univariable and multivariable logistic regression analyses were performed to evaluate the factors associated with presence of pre-attack symptoms. Results: Pre-attack symptoms were reported in 71.3% of our patients with cluster headache. When present, pre-attack symptoms occurred at a median of 20 minutes (range 1–120) before the attack. The prevalence of local and painful, local and painless sensory, autonomic, and general symptoms was 58.8%, 14.7%, 11.0%, and 30.1%, respectively. Multivariable logistic regression analysis revealed that higher number of bouts was associated with higher prevalence of pre-attack symptoms (OR = 1.464, p = 0.044). Conclusions: Pre-attack symptoms were frequently observed in Korean patients with cluster headache, which was consistent with previous Western studies. [ABSTRACT FROM AUTHOR]

Objective: To determine the pain intensity of cluster headache through a large survey by comparing it to other painful disorders. Furthermore, to investigate the relationship between maximal pain, autonomic, and other clinical symptoms, as well as demographic attributes of cluster headache. Background: The pain of cluster headache is anecdotally considered to be one of the worst pains in existence. The link between pain and autonomic features of cluster headache is understood mechanistically through the trigeminovascular reflex, though it is not clear if this is a graded response. Links between pain and other features of cluster headache are less well understood. Methods: This Internet‐based cross‐sectional survey included questions on cluster headache diagnostic criteria, which were used as part of the inclusion/exclusion criteria for the study. Respondents were asked to rate a cluster headache attack on the 0–10 numerical rating scale. Additionally, they were asked if they had experienced a list of other painful conditions such as labor pain or nephrolithiasis; if so they were asked to rate that pain as well. The survey also included demographics, mood scores, and treatment responses. Results: A total of 1604 cluster headache respondents were included in the analysis. Respondents rated cluster headache as significantly (p < 0.001) more intense than every other pain condition examined. Cluster headache attacks were rated as 9.7 ± 0.6 (mean ± standard deviation) on the numerical rating scale, followed by labor pain (7.2 ± 2.0), pancreatitis (7.0 ± 1.5), and nephrolithiasis (6.9 ± 1.9). The majority of cluster headache respondents rated a cluster headache attack at maximal or 10.0 pain (72.1%, 1157/1604). Respondents with maximal pain were statistically significantly more likely to have cranial autonomic features compared to respondents with less pain: conjunctival injection or lacrimation 91% (1057/1157) versus 85% (381/447), eyelid edema 77% (887/1157) versus 66% (293/447), forehead/facial sweating 60% (693/1157) versus 49% (217/447), fullness in the ear 47% (541/1157) versus 35% (155/447), and miosis/ptosis 85% (1124/1157) versus 75% (426/447) (all p values <0.001). Respondents with maximal pain also had other statistically significant findings: more frequent attacks (4.0 ± 2.0 attacks per day vs. 3.5 ± 2.0 attacks per day), higher Hopelessness Depression Symptom Questionnaire scores (24.5 ± 16.9 vs. 21.1 ± 15.2), decreased overall effectiveness from calcium channel blockers (on a 5‐point Likert scale), and more likely female: 34% (389/1157) versus 24% (108/447) (all p values <0.001). Pain intensity was not associated with restlessness, headache duration, age of onset, episodic/chronic status, or the effectiveness of any acute or preventive medication other than calcium channel blockers. Conclusions: Cluster headache is an intensely painful disorder, even in the context of other disorders considered intensely painful. Maximal pain intensity is associated with more cranial autonomic features, suggesting a graded response between pain and autonomic features. Maximal pain intensity is also associated with headache frequency but not duration, suggesting a relationship between pain intensity and mechanisms controlling headache onset, but not between pain intensity and mechanisms controlling headache offset. [ABSTRACT FROM AUTHOR]

Emerging data-points towards a possible aetiological and therapeutic relevance of trigeminal neurovascular contact in short lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and perhaps in short lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA). We aimed to assess the prevalence and significance of trigeminal neurovascular contact in a large cohort of consecutive SUNCT and SUNA patients and evaluate the radiological differences between them. The standard imaging protocol included high spatial and nerve-cistern contrast resolution imaging acquisitions of the cisternal segments of the trigeminal nerves and vessels. MRI studies were evaluated blindly by two expert evaluators and graded according to the presence, location and degree of neurovascular contact. The degree of contact was graded as with or without morphological changes. Neurovascular contact with morphological changes was defined as contact with distortion and/or atrophy. A total of 159 patients (SUNCT = 80; SUNA = 79) were included. A total of 165 symptomatic and 153 asymptomatic trigeminal nerves were analysed. The proportion of neurovascular contact on the symptomatic trigeminal nerves was higher (80.0%) compared to the asymptomatic trigeminal nerves (56.9%). The odds on having neurovascular contact over the symptomatic nerves was significantly higher than on the asymptomatic nerves [odds ratio (OR): 3.03, 95% confidence interval (CI) 1.84-4.99; P < 0.0001]. Neurovascular contact with morphological changes were considerably more prevalent on the symptomatic side (61.4%), compared to the asymptomatic side (31.0%) (OR 4.16, 95% CI 2.46-7.05; P < 0.0001). On symptomatic nerves, neurovascular contact with morphological changes was caused by an artery in 95.0% (n = 77/81). Moreover, the site of contact and the point of contact around the trigeminal root were respectively proximal in 82.7% (67/81) and superior in 59.3% (48/81). No significant radiological differences emerged between SUNCT and SUNA. The multivariate analysis of radiological predictors associated with the symptomatic side, indicated that the presence of neurovascular contact with morphological changes was strongly associated with the side of the pain (OR: 2.80, 95% CI 1.44-5.44; P = 0.002) even when adjusted for diagnoses. Our findings suggest that neurovascular contact with morphological changes is involved in the aetiology of SUNCT and SUNA. Along with a similar clinical phenotype, SUNCT and SUNA also display a similar structural neuroimaging profile, providing further support for the concept that the separation between them should be abandoned. Furthermore, these findings suggest that vascular compression of the trigeminal sensory root, may be a common aetiological factor between SUNCT, SUNA and trigeminal neuralgia thereby further expanding the overlap between these disorders. [ABSTRACT FROM AUTHOR]

Purpose of Review: This review presents a critical appraisal of the treatment strategies for short-lasting unilateral neuralgiform headache attacks (SUNHA), paroxysmal hemicrania (PH), and hemicrania continua (HC). We assess the available, though sparse, evidence on both medical and surgical treatments. In addition, we present estimated pooled analyses of the most common treatments and emphasize recent promising findings. Recent Findings: The majority of literature available on the treatment of these rare trigeminal autonomic cephalalgias are small open-label observational studies and case reports. Pooled analyses reveal that lamotrigine for SUNHA and indomethacin for PH and HC are the preventative treatments of choice. Second-line choices include topiramate, gabapentin, and carbamazepine for SUNHA; verapamil for PH; and cyclooxygenase-2 inhibitors and gabapentin for HC. Parenteral lidocaine is highly effective as a transitional treatment for SUNHA. Novel therapeutic strategies such as non-invasive neurostimulation, targeted nerve and ganglion blockades, and invasive neurostimulation, including implanted occipital nerve stimulators and deep brain stimulation, appears to be promising options. Summary: At present, lamotrigine as a prophylactic and parenteral lidocaine as transitional treatment remain the therapies of choice for SUNHA. While, by definition, both PH and CH respond exquisitely to indomethacin, evidence for other prophylactics is less convincing. Evidence for the novel emerging therapies is limited, though promising. [ABSTRACT FROM AUTHOR]

Hemicrania continua (HC) is a rare primary headache disorder, characterized by persistent unilateral pain associated with cranial autonomic symptoms and prompt response to indomethacin. While migrainous features (including aura) have been recognized in cluster headache, there have been only single reports of HC with aura. Here, we report the case of a 53-year-old man with constant right-sided headache and superimposed exacerbations to severe pain lasting for several hours. Secondary etiologies were excluded, and a diagnosis of HC was established after prompt and complete response to treatment with indomethacin. During an episode of pain exacerbation, for the first time the patient experienced an episode of transient visual disturbances compatible with scintillating scotoma. We propose a potential link between HC and visual aura, which parallels similar observations in other trigeminal autonomic cephalalgias and more specifically confirms previous observational data on aura in HC, thus highlighting potentially shared pathophysiological mechanisms.

Abstract Background Pediatric headache disorders can be extremely disabling, with marked reduction in the quality of life of children and their carers. Evidenced-based options for the treatment of primary headache disorders with preventive medication is limited and clinical outcomes are often unsatisfactory. Greater occipital nerve injections represent a rapid and well-tolerated therapeutic option, which is widely used in clinical practice in adults, and has previously shown a good outcome in a pediatric population. Methods This service evaluation reviewed greater occipital nerve injections performed unilaterally with 30 mg 1% lidocaine and 40 mg methylprednisolone, to treat disabling headache disorders in children and adolescents. Results We analyzed a total of 159 patients who received 380 injections. Of the population, 79% had chronic migraine, 14% new daily persistent headache, 4% a trigeminal autonomic cephalalgia, 3% secondary headache and one patient had chronic tension-type headache. An improvement after injection was seen in 66% (n = 105) of subjects, lasting on average 9 ± 4 weeks. Improvement was seen in 68% of patients with chronic migraine, 67% with a trigeminal autonomic cephalalgia and 59% with new daily persistent headache. Side effects were reported in 8% and were mild and transient. Older age, female gender, chronic migraine, increased number of past preventive use, medication overuse and developing side effects were all associated with an increased likelihood of positive treatment outcome. Conclusions This large single centre service evaluation confirms that unilateral injection of the greater occipital nerve is a safe, rapid-onset and effective treatment strategy in disabling headache disorders in children, with a range of diagnoses and severity of the condition, and with minimal side effects.

Aims: Studies evaluating sensory function of the entire trigeminocervical region in patients with cluster headache (CH), migraine (MH), and tension-type headache (TH) are required. The purpose of the present study was to evaluate and compare sensory function in the trigeminocervical region in patients with CH, MH, and TH and healthy controls (HC). Methods: Quantitative sensory testing (QST), including pressure pain threshold (PPT), tactile detection threshold (TDT), prick detection threshold (PDT), and two-point detection threshold (2PDT), was evaluated in 139 patients with headache (CH = 16, MH = 52, TH = 71) and 30 HC. Test sites included bilaterally the first, second, and third divisions of trigeminal nerve (V1, V2, and V3), cervical spine, and thenar eminence. Results: TH presented significantly lower PPT values compared with CH (p < 0.015), MH (p < 0.048), and HC (p < 0.009), while MH demonstrated significantly lower values than HC (p = 0.001–0.023). When analyzing TDT, CH in the symptomatic side presented significantly higher values in V1 compared with MH (p = 0.001), TH (p < 0.001), and HC (p < 0.001) and in V2 compared with TH (p = 0.035). No statistically significant differences were found for PDT (p > 0.005). With regard to 2PDT, CH-s presented significantly higher values in V1 with respect to HC (p = 0.016) but lower values in V2 compared with MH (p < 0.001) and TH (p = 0.003). Conclusion: The results of the present study indicate specific and different altered mechanical sensory thresholds in CH, MH, and TH patients compared with HC subjects. [ABSTRACT FROM AUTHOR]

Background: The otic ganglion (OG) provides parasympathetic innervation to the cerebral circulation and cranial structures and may be involved in the pathophysiology of trigeminal autonomic headaches. This structure has never been targeted in any headache disorder. Objective: To investigate the safety of injecting onabotulinumtoxin A (BTA) toward the OG in 10 patients with intractable chronic cluster headache and to collect efficacy data. Methods: A total of 10 patients with chronic cluster headache were enrolled in this open‐label, multi‐dose pilot safety study. All patients were recruited and treated on an out‐patient basis at St Olav's University Hospital (Norway). In 5 patients each, the OG was the injection target with 12.5 IU of BTA or 25 IU, respectively. The primary outcome measure was adverse events (AEs) and the main secondary outcome was the number of attacks per week measured at baseline and in the second month following injection. Results: For the primary endpoint, we analyzed data for all 10 patients. There were a total of 17 AEs in 6 of the 10 patients. All AEs were considered mild and disappeared by the end of follow‐up. The median number of attacks per week at baseline was 17.0 [7.8 to 25.8] vs 14.0 [7.3 to 20.0] in the second month following injection; difference: 3 (95%CI: −0.3 to 7.9), P =.063. Conclusions: Injection with BTA toward the OG appears to be safe. We did not find a statistically significant reduction in the number of attacks per week at month 2 after injection compared to the baseline. This study suggests that the OG is not an important target for the treatment of chronic cluster headache. A future study employing more precise targeting of the OG may be indicated. [ABSTRACT FROM AUTHOR]

Copyright of Revista de Medicina e Saúde de Brasília is the property of Revista de Medicina e Saude de Brasilia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Introduction: The population rate of familial cluster headache (CH) has been reported to be as high as 20% however this varies considerably across studies. To obtain a true estimate of family history in CH, we conducted a systematic review and meta-analysis of previously published data. Methods: Our systematic review involved a search of electronic databases (Medline, EMBASE, PubMed, CINAHL) to identify and appraise studies of interest utilising the PRISMA (Preferred Reporting Items for Systematic Review and Meta-Analysis) guidelines. To further ameliorate the accuracy of our analysis we included an additional unpublished cohort of CH patients recruited at a tertiary referral centre for headache, who underwent detailed family history with diagnostic verification in relatives. Data was extracted and meta-analysis conducted to provide a true estimation of family history. Results: In total, we identified 7 studies which fulfilled our inclusion criteria. The estimated true prevalence of CH patients with a positive family history was 6.27% (95% CI:4.65–8.40%) with an overall I2 of 73%. Fitted models for gender subgroups showed higher estimates 9.26% (95% CI: 6.29–13.43%) in females. However the I2 for the female model was 58.42% and significant (p = 0.047). Conclusion: Our findings estimate a rate of family history in CH to be approximately 6.27% (95% CI: 4.65–8.40%). While estimates were larger for female probands, we demonstrated high heterogeneity in this subgroup. These findings further support a genetic role in the aetiology of CH. [ABSTRACT FROM AUTHOR]