BackgroundTrigeminal autonomic cephalalgias (TAC) are primary headache disorders characterized by unilateral craniofacial pain, usually periorbital or temporal, accompanied by ipsilateral autonomic symptoms such as tearing, nasal congestion, conjunctival injection, miosis, and ptosis. They include cluster headache, paroxysmal hemicrania, hemicrania continua, and short-lasting unilateral neuralgiform headache attacks (SUNCT and SUNA). Diagnosis depends on attack duration, frequency, and intensity, though clinical overlap may occur. Accurate identification is crucial, as treatment differs among subtypes. Understanding TAC pathophysiology, particularly trigeminal autonomic activation, is essential for improving diagnosis, distinguishing them from similar disorders like migraine, and optimizing therapeutic management.ResultsTrigeminal autonomic cephalalgias represent a group of rare but disabling primary headache disorders characterized by overlapping pathophysiological pathways involving the hypothalamus and trigemino-autonomic reflex. Advances in neuroimaging and neuromodulation have refined understanding of their mechanisms and expanded therapeutic options beyond traditional pharmacotherapy. Early recognition and tailored, mechanism-based interventions remain essential to improving outcomes.ConclusionsWe believe it is very important and necessary to offer a comprehensive and up-to-date review of trigeminal autonomic headaches, emphasizing their common and distinctive clinical features, the underlying neurobiological mechanisms, and current evidence-based approaches for timely diagnosis and treatment.

ABSTRACT Objective To present a rare presentation of paroxysmal hemicrania (PH) in the orofacial region. Methods A retrospective record review was performed using the International Classification of Headache Disorders criteria for PH. Inclusion criteria were initial diagnosis of PH and at least one confirmatory diagnosis at follow-up. Results Four patients were diagnosed with orofacial PH 2015-2021. Average age was 56.5 years. Patients were most seen by dentists (n=7) and neurologists (n=5) and had a median of 19.5 months of diagnostic delay. Pain intensity was severe, episodes occurring daily and lasting 4-90 minutes. Lacrimation was the most common autonomic feature. All patients presented with intraoral pain and temporomandibular disorders. Maximum indomethacin dosage ranged from 75-225 mg/day. Verapamil was the most effective adjuvant therapy. Conclusions Orofacial PH presents a diagnostic challenge with significant delays, multiple referrals and unconventional pain locations. Intolerance to indomethacin resulted in the need for adjuvant treatment modalities.

Background: Pheochromocytomas (PHEO) and paragangliomas (PGL) are rare neuroendocrine tumors originating from adrenal or extra-adrenal chromaffin cells. Headache can be a presenting symptom in both secreting and non-secreting forms, with a higher prevalence noted in hereditary head and neck paragangliomas (HNPGL), particularly those linked to SDHD gene mutations. While typically non-functional, these tumors can be locally aggressive. Case report: We describe a 51-year-old woman with familial paragangliomatosis type I due to an SDHD mutation, diagnosed in 2010 with bilateral non-secreting cervical paragangliomas. Treatment included somatostatin analogues and one session of radiotherapy. Beginning in 2018, she developed a strictly left-sided, constrictive, stabbing headache localized to cervical, temporal, and auricular regions, gradually extending to periorbital and occipital areas. The headache was associated with photophobia, phonophobia, and nausea. By late 2024, it had become a continuous daily headache with autonomic features including ptosis, eyelid edema, nasal congestion, rhinorrhea, and lacrimation. Pain became pulsatile and worsened with physical activity, leading to chronic NSAID use. There was no prior history of primary headache disorders. Neurological examination revealed left ptosis and miosis, reported after radiotherapy in 2023. Imaging showed a ~10% volume increase in lesions from 2017 to 2022, but lesion size remained stable post-2022. Post-radiotherapy MRI (2023) revealed oropharyngeal edema, which largely resolved by 2024. Headache was only partially responsive to amitriptyline and perphenazine; a diagnostic trial with indomethacin was inconclusive due to poor tolerance. Discussion and Conclusion: Although clinical presentation met ICHD-3 criteria for Hemicrania Continua, the coexistence of local tumor mass and oculosympathetic dysfunction suggests a possible secondary headache, either tumor- or radiation-induced. Headache in paragangliomas is rarely described; Specific headache patterns associated with HNPGL have not been well characterized in the literature. Only one case of Paroxysmal Hemicrania resolving after tumor resection is reported, indicating a possible causal link. This case underscores the need to consider secondary causes such as paragangliomas in patients with trigeminal autonomic cephalalgia (TAC)-like headaches resistant to standard treatments. Despite their rarity, these tumors can lead to significant morbidity and should be included in the differential diagnosis when headache presents with autonomic symptoms and poor treatment response.

A Comprehensive Review of Trigeminal Autonomic Cephalalgias.

BackgroundHeadache is a public health problem in Africa and is a significant cause of neurological consultations, imposing a heavy burden. Data on its actual economic burden are scarce in Africa. This study aims to evaluate the direct cost of headache management in 2023 and to identify factors associated with the high direct cost of headache management.MethodsThis cohort study with descriptive and analytical aims was conducted from June 15 to October 15, 2023, in the neurology unit of the teaching hospital of Borgou in Parakou, on 91 subjects with headaches, including migraine, tension type headache, trigeminal neuralgia, cluster headache, paroxysmal hemicrania and secondary headache. Any patient who had experienced headaches in the past 12 months, who was aged at least 18 years and who provided informed consent was included. The direct cost of headache management was defined as the sum of costs by consumption level category (consultation, diagnostic tests, treatments, transport, hospitalization costs, other nonmedical costs) and the monetary value of the main companion. This cost was expressed in Benin’s local currency (West African francs XOF), with a corresponding amount in euros. The direct cost of headache care was considered high when the mean monthly direct cost was more than 10% of the guaranteed minimum wage in Benin. Consumption level data were collected through an individual interview, supplemented by a review of medical records, notebooks, and receipts for headache management. Data entry was carried out via the KoboCollect application. Data analysis was performed using EpiInfo 7.2 software.ResultsA total of 91 participants were included, with 51 women (56.04%). The mean age was 38.91 ± 14.52 years. The monthly direct cost estimated one month after inclusion was high, calculated at XOF1,599,400 (€2,438.26), with an average of XOF17,575.82 ± 10,290.68 (€26.79 ± 15.69), representing 33.80% of the minimum wage in Benin. The global annual direct cost of headaches was XOF15,537,875 (€23,687.23) with a higher cost for tension type headaches at XOF6,077,530 (€9,265.09), whereas that of migraine was calculated at XOF4,603,970 (€7,018.67). The factors associated with high costs of headache management were a history of anxiety (p = 0.02), the presence of comorbidities (p = 0.005), the frequency of attacks (p = 0.048), and consumption related to psychiatric consultation (p = 0.02).ConclusionThe direct cost of managing headaches is high, with multiple associated factors. Strategies aimed at reducing this cost are urgently needed to optimize management and influence national public health policy for headache patients in Benin.

Chronic headache disorders have a tremendous impact on psychosocial functioning. Despite the availability of various treatment options, suboptimal management remains present in a subset of patients, leading to persistent suffering. Molecular mechanisms, regional activity patterns and connectivity pathways are crucial for understanding the pathophysiology, serving as a foundation for developing novel treatments, refining existing therapies, and ultimately optimizing the management of headache disorders. Nevertheless, articles combining fundamental and clinical aspects of the pathophysiology and treatment of headache disorders remain limited. The current literature review provides a thorough overview of the molecular mechanisms, regional activity patterns and connectivity pathways involved in migraine, cluster headache (CH), paroxysmal hemicrania (PH), hemicrania continua (HC) and occipital neuralgia (ON), thereby bridging the gap between different fields of expertise. In this scoping review, literature on molecular mechanisms, regional activity and connectivity pathways for migraine, CH, PH, HC and ON has been collected from the PubMed, MEDLINE and EMBASE databases. Reports were also manually searched using the search function in Google Scholar, as well as reviews or references cited within the articles. In total, 130 and 97 articles, published between 1976 and 2024, are included in the analysis of the molecular mechanism and regional activity patterns/connectivity pathways, respectively. Molecular data show that the trigeminal nucleus caudalis is a central structure in headache pathology, comprising various neuropeptides and neurochemicals, including vasoactive intestinal peptide, glutamate, substance P and serotonin, and connecting the pathophysiology of these headache disorders. Sensitization of higher cortical brain areas, neuroinflammation within the trigeminal system and vasodilatation of cranial vessels seem to contribute to headache pain. Headache disorders are also associated with atypical regional activity patterns and connectivity pathways in pain processing areas, as well as the default mode network, salience network, and sensorimotor network. These abnormalities help explain the mechanisms underlying overall headache-related symptoms and additional manifestations unique to each headache disorder, including cortical spreading depression in migraine, rhythmicity of attacks in CH and autonomic symptoms in CH, PH and HC. The article fosters a deeper understanding of the molecular mechanisms, neuronal pathways and clinical symptoms involved in headache pathology across different fields of expertise. By bridging these perspectives, it provides essential insights for developing innovative treatment strategies and enhancing existing therapeutic options.

AimTo evaluate the effectiveness and tolerability of non-invasive vagus nerve stimulation (nVNS) as acute or preventive treatment, or both, in a cohort of trigeminal autonomic cephalalgia (TAC) patients.MethodsA service evaluation retrospectively included patients with TACs between January 2014 and February 2025 who had used, or currently use, nVNS. Data were collected from clinical letters. Data are presented as descriptive statistics analysis and non-parametric tests were performed.ResultsIn total, 108 patients were included, 74 patients with cluster headache (CH), 10 with paroxysmal hemicrania, 15 with hemicrania continua, four with short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT), three with short-lasting unilateral neuralgiform with cranial autonomic symptoms (SUNA) and two with an undifferentiated TAC. Overall, 70 patients considered nVNS useful over a median time using nVNS of 47 (interquartile range = 18-66) months. The median time of use in patients who did not find nVNS useful was 7 (interquartile range = 4-12) months. Twenty-three patients reported an adverse event (AE), while no serious treatment-related AEs occurred. Fifty-nine patients withdrew from using the device, including 11 patients that initially reported nVNS as useful. All groups considered nVNS more useful as preventive, while cluster headache and SUNCT/SUNA patients also considered it useful as acute treatment.ConclusionsOur findings complement previous evidence of the effectiveness and tolerability of nVNS in CH in addition to other forms of TACs. Interestingly, nVNS seems to be more effective as preventive rather than as acute treatment in our cohort.

BackgroundHemicrania continua and paroxysmal hemicrania are rare in the pediatric population. Recognizing these disorders characterized by unilateral headaches with autonomic features can reduce time to diagnosis, facilitate effective medical treatment, and reduce morbidity.ObjectiveTo review the diagnostic criteria and pathophysiology of hemicrania continua and paroxysmal hemicrania, analyze a retrospective cohort of adolescent patients with indomethacin-responsive headaches, and discuss the clinical features of these patients, both in how they follow the diagnostic criteria for these disorders and how they may deviate. We also examined time to diagnosis and prognosis for this cohort.MethodsA retrospective chart review was completed of patients 12-18 years old from 2014 to 2021 diagnosed with indomethacin-responsive headaches who presented to a tertiary pediatric headache clinic. Clinical headache characteristics, demographic features, medical diagnoses, and diagnostic testing were reviewed and collated.ResultsEight patients (7 female, 1 male) had indomethacin-responsive headaches. Six patients were diagnosed with hemicrania continua and 2 were diagnosed with paroxysmal hemicrania. The most common autonomic symptoms were unilateral nasal congestion and conjunctival injection/lacrimation. The median time to diagnosis was 15 months, and the median treatment length was 7 months.ConclusionPatients can have multiple headache phenotypes. Clinicians should ask headache patients of all ages about autonomic symptoms and unilateral headaches, specifically in fixed unilateral headaches. These headaches should be evaluated with imaging to rule out secondary intracranial causes. In those cases, with these features, an indomethacin trial is part of the diagnosis and should be considered early in the course.

BackgroundThe study of the trigeminal autonomic cephalalgias (TAC) has been limited by difficulty aggregating sufficient numbers of patients. We used the Epic Cosmos electronic health record research platform to harness nationwide data from health care systems across the United States using the Epic electronic health record to analyze the prevalence, demographics, comorbid conditions and treatments for the TACs.MethodsWe queried the Epic Cosmos electronic health record database for patients with diagnoses of hemicrania continua, cluster headache, paroxysmal hemicrania, and SUNCT. Prevalences, demographics were determined from this database and comorbid conditions and treatments for these conditions were analyzed.ResultsOur study included 152,727 patients with cluster headache, 59,312 patients with paroxysmal hemicrania, 19,321 patients with hemicrania continua, and 6,291 patients with SUNCT. Five-year prevalence of cluster headache was highest (56.7 per 100,000), followed by paroxysmal hemicrania (22.0 per 100,000), hemicrania continua (7.2 per 100,000) and SUNCT (2.3 per 100,000). All four TACs showed a higher prevalence in women. Migraine was common in all four conditions and patients with cluster headache had the highest rates of nicotine, alcohol, and cannabis use disorders. Indomethacin was notably underutilized for the indomethacin-responsive TACs.ConclusionWe use a national electronic medical record database to give insight into elements of the TACs that have been previously limited by the relative rarity of these diseases.Supplementary InformationThe online version contains supplementary material available at 10.1186/s12883-025-04314-1.

Indomethacin-responsive headaches occur in youth and include primary headache syndromes such as hemicrania continua and paroxysmal hemicrania. Both are trigeminal autonomic cephalalgias (TACs). In pediatrics TACs are rare. Moreover, they may differ in their phenotypes and response to indomethacin compared to adults. Secondary causes for side-locked headaches can have vascular, neoplastic, and inflammatory etiologies, emphasizing the importance of imaging in the evaluation of these headache types. Post-craniotomy indomethacin-responsive headaches have been described in adults, but not in children. Written consent was obtained from the patients' families and written assent from the two children for publication. We report the course of two children, both with a history of Rasmussen's encephalitis treated with functional hemispherectomies, who subsequently developed debilitating, side-locked, medically refractory headaches several months after surgery. Headaches were on the same side of their encephalitis and surgery. In both instances, the headaches were exquisitely and rapidly responsive to indomethacin. Headache freedom was maintained on low doses of indomethacin (0.14-0.5 mg/kg/day). In cases of refractory side-locked headaches following a craniotomy or neuroinflammatory condition in children, one may consider indomethacin as a treatment option after evaluation for other secondary etiologies.

Paroxysmal hemicrania and hemicrania continua are rare primary headache disorders which are distinguished by an absolute response to indomethacin. As a matter of importance, no guidelines have been proposed for alternative therapeutic options in case of indomethacin intolerance. The purpose of this review is to provide an update on the current findings, especially focusing on the past 18 months, in the treatment of both paroxysmal hemicrania and hemicrania continua and to provide proposed management recommendations based on summarized evidence. Apart from well recognized gastrolesive effects of indomethacin, a substantial number of patients may suffer from neuropsychiatric adverse reactions. Recent studies demonstrated that melatonin, which has been known for its effectiveness for hemicrania continua, is also useful for paroxysmal hemicrania. Promising nonpharmacological treatment option, which is noninvasive vagus nerve stimulation, has been shown to be beneficial for both indomethacin-responsive headache disorders allowing the reduction of indomethacin dosage. Although the data on substitutive medication choice for indomethacin are currently scarce, the most consistent results have been repeatedly achieved with acemethacin, selective COX-2 inhibitors, and anticonvulsants. However, considering the crucial role of pathophysiology, research investigating the efficacy of drugs targeting the trigemino-vascular system activation, as well as controlled trials assessing the efficacy involving the aforementioned therapeutic options are still vague. In spite of numerous reports suggesting reliable alternatives to indomethacin, the consensus on pharmacological therapy guidelines for indomethacin-responsive headache disorders has not yet been reached. Further research and agreement from the experts' standpoint are needed for an establishment of reliable treatment recommendations.

Background: Monoclonal antibodies directed against Calcitonin Gene-Related Peptide (CGRP) or its receptor have greatly improved the quality of life of migraine patients. However, these treatments must be administered with caution in patients with constipation or inflammatory bowel disease, considering that non-steroidal anti-inflammatory drugs, widely used by patients with migraine to treat attacks, may have gastrointestinal side effects. Methods: After receiving informed consent, we obtained patient's information from her clinical documentation and archived medical records. Results: We report the case of a patient with a clinical history of migraine with and without aura, paroxysmal hemicrania, and overuse of indomethacin, who received a diagnosis of symptomatic small bowel diaphragmatic disease under prophylactic treatment with galcanezumab. Due to the intestinal implications of CGRP, we decided to discontinue this therapy and begin OnabotulinumtoxinA treatment. Conclusions: This case report recommends extreme caution when starting anti-CGRP mAbs treatment in patients with longstanding medication overuse with NSAIDs and abdominal symptoms.

Emerging evidence suggests that primary headaches, classified as trigeminal autonomic cephalalgias (TACs), may exhibit sex and gender differences in clinical features, mechanisms, and treatment responses. While epidemiological and clinical gender-specific differences have been widely reported for cluster headache, limited evidence is available for other TACs. In this narrative review, we have analyzed the existing data on the influence of sex and gender on cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks, and hemicrania continua. Given the role of calcitonin gene-related peptide (CGRP) in migraine and cluster headache, sex and gender differences in the levels and function of CGRP in preclinical models and patients are reported. Future studies are warranted to elucidate the role of sex and gender in the complex interplay of genetic and neurochemical factors in TACs.

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. 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. 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. 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.

Background: The greater occipital nerve block (GON-B) is used in clinical practice for treating different forms of headache. There is no standardized procedure to perform GON-B. This study evaluates the efficacy and feasibility of a low-volume GON-B protocol utilizing a pre-mixed solution of lidocaine (10 mg) and methylprednisolone (40 mg) across various headache disorders. Methods: This observational case series included patients receiving their first GON-B from November 2019 to February 2021. Participants were diagnosed with migraine, cluster headache, cervicogenic headache, or paroxysmal hemicrania. The primary outcome was the degree of response to the GON-B. Results: Thirty-nine patients with migraine underwent a first GON-B. Regarding headache frequency, 26% achieved substantial response and 33% partial response. For headache intensity, 26% reported substantial and 49% partial improvement. Migraine patients experienced a significant reduction in median monthly headache days from 25 to 13 (p=0.001) and in headache intensity from a median of 8 to 6 on the Numerical Rating Scale (NRS) scale (p&lt;0.001). Of the 27 patients receiving a second GON-B, 33% had a substantial response, 48% a partial response, and 19% no response. Results from subsequent sessions were consistent with these findings. Ten patients with cluster headache underwent GON-B, showing a significant reduction in pain intensity from a median NRS score of 10 to 5 (p=0.008). Two patients with cervicogenic headache showed a partial response to GON-B, with pain intensity decreasing from 8 to 6 and 8 to 7 over 30 monthly episodes. A patient with paroxysmal hemicrania received seven GON-B injections, reducing daily attacks from 30 to 10 and pain intensity from 7 to 6 on the NRS scale. Conclusions: These outcomes affirm GON-B potential in interrupting pain pathways, even at a low dose, in a wide range of headache disorders.

This article describes the clinical features and treatment of the indomethacin-responsive headache disorders paroxysmal hemicrania and hemicrania continua. Both paroxysmal hemicrania and hemicrania continua are treated with indomethacin at the lowest clinically useful dose. It has recently become clear that some patients with either condition may respond to treatment with noninvasive vagus nerve stimulation, which can be both indomethacin sparing and, in some cases, headache controlling. Given the lifelong nature of both paroxysmal hemicrania and hemicrania continua, brain imaging with MRI is recommended when the conditions are identified, specifically including pituitary views. Paroxysmal hemicrania and hemicrania continua are indomethacin-responsive headache disorders that offer a rewarding and unique opportunity to provide marked clinical improvement when recognized and treated appropriately. These disorders share the final common pathway of the trigeminal-autonomic reflex, with head pain and cranial autonomic features, and are differentiated pathophysiologically by the pattern of brain involvement, which can be seen using functional imaging. They have distinct differential diagnoses to which the clinician needs to remain alert.

BackgroundThere is lack of population-based studies evaluating the prevalence of paroxysmal hemicrania, hemicrania continua and short-lasting unilateral neuralgiform headache attacks.ObjectivesThe 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.MethodsA 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.ResultsAmong 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.ConclusionsIn 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.

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. 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. 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. 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.

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. 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. 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. Recent advances in the understanding of SUNHA have improved the recognition and treatment approaches for this unique condition.

ABSTRACT Paroxysmal Hemicrania is a trigeminal autonomic cephalalgia described as a severe and strictly unilateral pain, which occurs in paroxysms at orbital, supraorbital and/ or temporal region. A 45-year-old woman presented to an orofacial pain specialist reporting severe, burning, throbbing, strictly right-sided headache associated to ipsilateral autonomic symptoms and orofacial pain. The pain was perceived on the maxillary region followed by pain spread to the head. Interdisciplinary evaluation, along with absolute responsiveness to indomethacin and normal Brain Magnetic Resonance imaging, led to the diagnosis of primary Episodic paroxysmal hemicrania with facial representation and myofascial pain of masticatory muscles. Dentists should be aware of paroxysmal hemicrania with facial representation and the possibility of temporomandibular disorder coexistence, in order to avoid misdiagnosis and inadequate management. Paroxysmal hemicrania may be first perceived on the face and may be associated with interparoxysmal pain. In these cases, efficient anamnesis and clinical evaluation followed by interdisciplinary approach is needed.