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Introduction: Reversible cerebral vasoconstriction syndrome is a clinicoradiological entity with a self-limiting course that manifests with recurrent episodes of thunderclap headache, and is associated with certain triggers. Recurrence is very rare, and the pathophysiology is thought to be related to altered autoregulation of the cerebrovascular tone. We present a clinical case that raises questions about possible recurrences and triggers., Case Report: A 44-year-old woman with a history of multiple sclerosis treated with interferon beta-1b who had four episodes of thunderclap headache while resting, after completing a course of corticosteroids due to a flare-up of optic neuritis. Three years earlier, the patient had presented several episodes of explosive-onset headache during a self-limited period of one month, only occurring during sexual intercourse. In the year prior to our assessment, she had suffered three thunderclap headaches with similar characteristics, but they were triggered only by intense physical exercise. She had not consulted a physician about these events. A cranial computed tomography scan was performed after the administration of contrast media and a cerebral arteriography, which were consistent with cerebral vasoconstriction syndrome, and its reversibility was confirmed three months later., Conclusions: Reversible cerebral vasoconstriction syndrome shares a phenotypic expression with primary exertion headaches. It is associated with drugs with vasoactive effects, including interferons, and corticosteroids are associated with a worse prognosis, and such their administration should be avoided.

Purpose of Review: This review summarizes the evolution in diagnosis, evaluation, and treatment of primary headache associated with sexual activity (PHASA)., Recent Findings: Despite increased access to patient information and advances in imaging, the pathophysiology of PHASA remains not fully understood. There are many secondary headaches that may present with headache during sexual activity, and a thorough workup is indicated to rule out potentially life-threatening etiologies. Many recent case series discuss the efficacy of known treatments of PHASA, as well as suggest other potential therapies for this condition including the newer CGRP-targeted therapies. Headaches during sexual activity can be worrisome events which necessitate urgent evaluation, particularly when presenting with sudden-onset and severe "thunderclap" headaches. A thorough workup including imaging should be conducted to rule out etiologies such as subarachnoid hemorrhage, reversible cerebral vasoconstrictive syndrome (RCVS), vasospasm, and dissection. PHASA is commonly comorbid with migraine, tension-type headache, exertional headache, and hypertension. PHASA can present as a dull headache that progresses with sexual excitement, or an explosive headache at or around orgasm. Pain is primarily occipital, diffuse, and bilateral. The headaches are discrete, recurrent events with bouts that typically self-resolve, but may also relapse and remit or continue chronically in some patients. PHASA can be treated preemptively with indomethacin and triptans administered prior to sexual activity, or treated prophylactically with beta-blockers, topiramate, and calcium channel blockers. CGRP-targeted therapies may provide relief in PHASA based on a few case reports, but there are no randomized controlled trials looking at specific efficacy for these therapies., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objective: Migraine is a complex and disabling neurological disorder. Recent years have witnessed the development and emergence of novel treatments for the condition, namely those targeting calcitonin gene-related peptide (CGRP). However, there remains a substantial need for further treatments for those unresponsive to current therapies. Targeting pituitary adenylate cyclase-activating polypeptide (PACAP) as a possible therapeutic strategy in the primary headache disorders has gained interest over recent years., Methods: This review will summarize what we know about PACAP to date: its expression, receptors, roles in migraine and cluster headache biology, insights gained from preclinical and clinical models of migraine, and therapeutic scope., Results: PACAP shares homology with vasoactive intestinal polypeptide (VIP) and is one of several vasoactive neuropeptides along with CGRP and VIP, which has been implicated in migraine neurobiology. PACAP is widely expressed in areas of interest in migraine pathophysiology, such as the thalamus, trigeminal nucleus caudalis, and sphenopalatine ganglion. Preclinical evidence suggests a role for PACAP in trigeminovascular sensitization, while clinical evidence shows ictal release of PACAP in migraine and intravenous infusion of PACAP triggering attacks in susceptible individuals. PACAP leads to dural vasodilatation and secondary central phenomena via its binding to different G-protein-coupled receptors, and intracellular downstream effects through cyclic adenosine monophosphate (cAMP) and phosphokinase C (PKC). Targeting PACAP as a therapeutic strategy in headache has been explored using monoclonal antibodies developed against PACAP and against the PAC1 receptor, with initial positive results., Interpretation: Future clinical trials hold considerable promise for a new therapeutic approach using PACAP-targeted therapies in both migraine and cluster headache., (© 2024 The Author(s). Annals of Clinical and Translational Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association.)

Competing Interests: Competing interests: None declared.

Objectives: To report the efficacy of onabotulinumtoxinA (BoNTA) injections in relieving pain in patients with primary trochlear headache (PRTH)., Methods: Examination of medical records for patients diagnosed with PRTH according to the International Classification of Headache Disorders, 3rd edition criteria and treated with BoNTA. Data were collected for variables related to pain relief, duration of effectiveness, and adverse effects., Results: Six patients were included in the study. All had previously undergone standard care interventions, including infiltrations or oral treatments, yet experienced treatment failure or symptom recurrence. All patients received 20 units of BoNTA, administered in the corrugator and procerus muscles. Subsequent to the BoNTA injections, all six patients reported substantial pain relief, with five achieving complete remission of symptoms. The analgesic effect persisted for a duration of 3 months. No adverse events were reported in any of the cases., Conclusions: Our case series presents the first evidence of the potential of BoNTA as a safe and effective treatment option for PRTH. From a clinical standpoint, having a safer alternative is of paramount significance for patients with limited treatment options, such as those with PRTH. Further research is warranted to validate these findings and explore the long-term efficacy of BoNTA in PRTH management., (© 2024 The Authors. Headache: The Journal of Head and Face Pain published by Wiley Periodicals LLC on behalf of American Headache Society.)

Background: Bath-related headache (BRH) is a rare primary headache disorder with only about 50 cases reported from 2000 to 2017 and none since. It is an abrupt onset excruciating headache occurring predominantly in middle-aged Asian women, most commonly following exposure to hot water. This is the first report in a Sri Lankan woman., Case Presentation: A 60-year-old Sri Lankan woman presented with an abrupt onset, severe throbbing holocephalic headache immediately following a hot-water shower. The headache was not associated with photo- or phonophobia, nausea, or vomiting, and she did not report a past history of migraine. However, she had experienced a similar headache 2 years previously precipitated by a hot-water shower. Her neurological examination, blood investigations, and magnetic resonance imaging of brain and intracranial vessels were normal. She was treated with opioid and nonsteroidal antiinflammatory drug analgesics, but the headache resolved only after treatment with nimodipine. The headache did not recur during a follow-up of 2 years since she avoided hot-water showers., Conclusions: Bath-related headache is a thunderclap primary headache disorder with a benign prognosis, but its recognition requires awareness to differentiate it from subarachnoid hemorrhage. It warrants inclusion in the International Classification of Headache Disorders., (© 2023. The Author(s).)

Background: Hypnic Headache, also known as "alarm clock headache", is a rare primary headache disorder. It is characterized by frequently recurring headache attacks, which only develop during sleep, especially nighttime sleep., Objective: This article gives a narrative review on the current knowledge about Hypnic Headache with a focus on secondary Hypnic Headache., Methods: Based on literature research, using Pubmed and Google Scholar, latest case reports, studies, and systematic reviews about Hypnic Headache were analyzed and summarized focusing on therapeutic options and causes of secondary Hypnic Headache., Conclusion: Hypnic Headache mainly occurs in elderly patients. However, younger patients and children may also suffer from Hypnic Headache. Many different causes of secondary Hypnic Headache are described in the literature and ought to be ruled out before diagnosing primary Hypnic Headache. The pathophysiology of primary Hypnic Headache remains unclear, but a dysfunction of the hypothalamus seems to play a key role.

Background/objective: Primary stabbing headache is a common but under-recognized primary headache disorder. The objectives of this review were to provide practical information for better understanding and identification of the disease, suggest an algorithm for differential diagnosis, and provide an insight into the pathophysiology of primary stabbing headache hypothesized from its clinical course., Methods: This narrative review of primary stabbing headache is based on a literature search and the authors' clinical reasoning., Result: The phenotype of each stab is typically abrupt, ultrashort-lasting (<3 s), focal or multifocal, paroxysms of pain occurring sporadically or in clusters. The diagnosis of primary stabbing headache is clinical; fixed or migrating stabs without background pain or sensory abnormalities and the absence of features suggestive of other disorders (e.g., cranial autonomic symptoms or signs) can aid in the diagnosis of primary stabbing headache. The clinical patterns include monophasic, intermittent, and chronic primary stabbing headache, of which the first two are considered typical. The pathophysiology of primary stabbing headache has not yet been elucidated. In this review, we postulated the mechanism of stabbing headache, based on the pain phenotype and clinical course, and provide a clinical algorithm for the differential diagnosis of primary stabbing headache., Conclusion: Knowledge about the typical manifestations and clinical patterns of primary stabbing headache will aid in the proper diagnosis and differential diagnosis. Treatment should be tailored by considering the clinical patterns. Further research is needed to elucidate the pathophysiological mechanisms and optimal treatment of primary stabbing headache.

Objectives: This retrospective case series study aimed to investigate the demographic and clinical patterns of primary stabbing headache (PSH). In addition, we tried to identify subgroups of treatment responses in a neurology outpatient consultation at a Portuguese tertiary hospital., Methods: Clinical records were retrospectively reviewed and patients meeting the International Classification of Headache Disorders, 3rd edition, criteria for PSH were identified from January 2014 to December 2020. We collected data regarding demographic characteristics, clinical features of the headache, primary headache comorbidities, and information about treatment-related do PSH., Results: Of 1857 patients, 32 (1.7%; mean [SD] age of onset 56 [3.5] years) had the final diagnosis of PSH. Regarding headache characteristics, 20 patients (62.5%) reported episodes of stabbing in fixed locations and 12 (37.5%) in multiple areas; the duration of each attack was between ≤5 s (seven [21.9%]), 5-60 s (20 [62.5%]), and ≥60 s (five [15.6%]). In all, 18 patients (56.3%) had an episodic course (vs. six of 32 [18.8%] an acute course and eight of 32 [25%] a chronic course). In all, 17 patients started medical treatment (53.1%), with total or partial improvement in 10 (58.8%) of them. It was found that patients with pain in fixed locations had a better response to treatment when compared to patients with multiple locations, in a statistically significant way (eight of 11 vs. two of six, p = 0.023)., Conclusion: In our sample, the mean age of onset of PSH was >50 years and there was a wide range of PSH duration. The duration of each attack (>5 s), the pain in fixed locations, non-daily episodes of the pain in each attack, and the intermittent course of headache were the most prevalent clinical features. Finally, patients with stabbing in localized areas had a better response to treatment., (© 2022 American Headache Society.)

Objective: This national postal survey aimed to examine Canadian emergency physicians' practice patterns with respect to drug treatment and perspectives on peripheral nerve blocks., Background: The treatment of primary headache disorders in the emergency department is variable., Methods: We surveyed 500 emergency physicians listed in the Canadian Medical Directory according to a modified Dillman's method: an initial invitation was followed by up to four reminders to nonresponders. Physicians were asked questions regarding their frequency of medication administration and perspectives toward peripheral nerve blocks., Results: Of 500 mailed surveys, 468 were delivered and 179 physicians responded (response rate = 38.2%). The majority of physicians were men (92/144, 63.9%); 80.6% (116/144) had been in practice for greater than or equal to 10 years with 50.7% (75/148) in a community or district general teaching hospital. Commonly used pharmacotherapies for primary headaches were intravenous dopamine receptor antagonists (69%), co-administration of ketorolac and a dopamine receptor antagonist (54.2%), intravenous fluid boluses (54%), nonsteroidal anti-inflammatory drugs (NSAIDs) alone (53.5%), and acetaminophen (51.4%). Only 80 of 144 physicians (55.6%) reported previous experience with peripheral nerve blocks (95% confidence interval [CI] = 48%-65%). The majority (68/80, 85.0%) agreed peripheral nerve blocks are safe and 55.1% (43/78) agreed they are effective. The vast majority (118/140, 84.3%) would consider peripheral nerve blocks as a first-line treatment option given sufficient evidence from a future trial (95% CI = 78%-90%)., Conclusion: NSAIDs alone, as well as dopamine receptor antagonists with or without ketorolac are commonly used for primary headache in Canadian emergency departments. A large proportion of physicians have never used a peripheral nerve block in their practice; among those who have experience with peripheral nerve blocks, the majority find them safe and effective. The majority of respondents would consider peripheral nerve blocks as a first-line treatment option given sufficient evidence from a future trial., (© 2022 American Headache Society.)

BACKGROUND Reversible cerebral vasoconstriction syndrome (RCVS) is a rare neurological disorder with a complex physiopathology that is not fully understood. Suggested underlying mechanisms include failure of autoregulation, endothelial dysfunction, and oxidative stress. It is characterized by reversible multifocal constriction of the cerebral arteries, and can be triggered by many conditions, including, vasoactive medications (eg, triptans), cerebrovascular events, primary headache disorders, and metabolic causes (eg, hypercalcemia). RCVS can also be associated with pregnancy-related conditions, such as thrombotic thrombocytopenic purpura, eclampsia, and pre-eclampsia. Thunderclap headache is the most common clinical manifestation; however, other symptoms can result from complications of the disease, such as stroke, brain edema, and seizures. Several case reports have been published of an association between RCVS and eclampsia, but to the best of our knowledge, only 3 cases were successfully treated with intravenous milrinone and this is the only patient reported in Saudi Arabia. CASE REPORT We report a case of 25-year-old primigravida woman who presented with acute-onset headache, nausea, elevated blood pressure, and generalized tonic clonic seizure. She was diagnosed as having RCVS secondary to eclampsia based on clinical and radiological features. She was initially started on nimodipine, which is usually the first-line management of RCVS, as well as magnesium sulfate and levetiracetam; however, she only achieved full recovery after starting intravenous milrinone. CONCLUSIONS Milrinone is one of the emerging drugs for treatment of RCVS, and this case report delineates the potential of using the drug, especially in cases refractory to standard therapy.

Objective: To summarize the unique aspects of managing headache in gender minorities and current research in this area including the potential relationship between gender-affirming hormone therapy (GAHT) and headache., Background: The study of headache in gender minorities is intrinsically important. Gender minorities are medically underserved, and their medical care to date has been limited by socioeconomic disadvantages including stigma and an unsupportive clinical environment. Despite the rising population of transgender and gender-diverse adults and youth, headache research has also been limited. Knowledge of hormonal effects on headache in cisgender patients raises the question of possible effects of GAHT on transgender patients., Methods/results: The manuscript is a narrative review of current best practices in treating transgender patients, including the use of appropriate terminology and ways to create a supportive environment. It also contains current guidelines on GAHT and reviews drug-drug interactions and secondary headache related to hormone therapy. We also review transgender headache research and related research on hormonal effects on headache in cisgender individuals., Conclusion: Creating a supportive environment for transgender and gender-diverse patients and being knowledgeable about GAHT are key to providing quality headache care. This review identifies further research needs for this population including the epidemiology of headache disorders in sexual minorities and the potential effects of GAHT on headache disorders in transgender patients., (© 2021 American Headache Society.)

Background: In Nepal, traditional treatment using medicinal plants is popular. Whereas medication-overuse headache is, by definition, caused by excessive use of acute headache medication, we hypothesized that medicinal plants, being pharmacologically active, were as likely a cause., Methods: We used data from a cross-sectional, nationwide population-based study, which enquired into headache and use of medicinal plants and allopathic medications. We searched the literature for pharmacodynamic actions of the medicinal plants., Results: Of 2100 participants, 1794 (85.4%) reported headache in the preceding year; 161 (7.7%) reported headache on ≥15 days/month, of whom 28 (17.4%) had used medicinal plants and 117 (72.7%) allopathic medication(s). Of 46 with probable medication-overuse headache, 87.0% (40/46) were using allopathic medication(s) and 13.0% (6/46) medicinal plants, a ratio of 6.7:1, higher than the overall ratio among those with headache of 4.9:1 (912/185). Of 60 plant species identified, 49 were pharmacodynamically active on the central nervous system, with various effects of likely relevance in medication-overuse headache causation., Conclusions: MPs are potentially a cause of medication-overuse headache, and not to be seen as innocent in this regard. Numbers presumptively affected in Nepal are low but not negligible. This pioneering project provides a starting point for further research to provide needed guidance on use of medicinal plants for headache.

Background: Primary headaches (migraine, tension headache, cluster headache, and other trigeminal autonomic cephalgias) are common in pregnancy and postpartum. It is unclear how to best and most safely manage them., Objective: We conducted a systematic review (SR) of interventions to prevent or treat primary headaches in women who are pregnant, attempting to become pregnant, postpartum, or breastfeeding., Methods: We searched Medline, Embase, Cochrane CENTRAL, CINAHL, ClinicalTrials.gov, Cochrane Database of SRs, and Epistemonikos for primary studies of pregnant women with primary headache and existing SRs of harms in pregnant women regardless of indication. No date or language restrictions were applied. We assessed strength of evidence (SoE) using standard methods., Results: We screened 8549 citations for studies and 2788 citations for SRs. Sixteen studies (mostly high risk of bias) comprising 14,185 patients (total) and 26 SRs met the criteria. For prevention, we found no evidence addressing effectiveness. Antiepileptics, venlafaxine, tricyclic antidepressants, benzodiazepines, β-blockers, prednisolone, and oral magnesium may be associated with fetal/child adverse effects, but calcium channel blockers and antihistamines may not be (1 single-group study and 11 SRs; low-to-moderate SoE). For treatment, combination metoclopramide and diphenhydramine may be more effective than codeine for migraine or tension headache (1 randomized controlled trial; low SoE). Triptans may not be associated with fetal/child adverse effects (8 nonrandomized comparative studies; low SoE). Acetaminophen, prednisolone, indomethacin, ondansetron, antipsychotics, and intravenous magnesium may be associated with fetal/child adverse effects, but low-dose aspirin may not be (indirect evidence; low-to-moderate SoE). We found insufficient evidence regarding non-pharmacologic treatments., Conclusions: For prevention of primary headache, calcium channel blockers and antihistamines may not be associated with fetal/child adverse effects. For treatment, combination metoclopramide and diphenhydramine may be more effective than codeine. Triptans and low-dose aspirin may not be associated with fetal/child adverse effects. Future research should identify effective and safe interventions in pregnancy and postpartum., (© 2021 American Headache Society.)

Reversible cerebral vasoconstriction syndrome (RCVS) is a clinical-radiologic diagnosis that affects children and adolescents, but it is much more frequently reported in adults. Clinically, patients present with severe and commonly recurrent thunderclap headaches. Typical precipitating triggers include vasoactive substances, serotonergic agents, and the postpartum period. There may be associated neurologic complications at presentation or in the weeks following, such as convexity subarachnoid hemorrhage, stroke, cerebral edema, cervical artery dissection (CeAD), and seizures. Angiographically, the cerebral arteries demonstrate segmental vasoconstriction and dilation, although imaging early in the clinical course may be normal. Work-up is performed to exclude intracranial disorders such as vasculitis, subarachnoid hemorrhage due to ruptured aneurysm, meningitis, and intracranial venous sinus thrombosis. Within 1 month of initial symptom onset, clinical symptoms such as severe headache have ceased, and within 3 months, the cerebral vasoconstriction is much improved or resolved. Management involves avoidance of precipitating triggers and potentially short-term pharmacotherapy with calcium channel blockers for patients with associated neurologic complications. Steroids are not recommended and may worsen the clinical outcome. Prognosis is excellent in the large majority of patients, and only 5% of patients experience a recurrence of RCVS., Competing Interests: None., (Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.)

Background: The incidences of dystonic reactions to metoclopramide and prochlorperazine have not been well characterized in children., Methods: Medical record data were reviewed for patients at a tertiary care pediatric hospital who received metoclopramide or prochlorperazine for treatment of headache., Results: A total of 4588 clinical encounters were identified, 2542 with prochlorperazine and 2046 with metoclopramide. One patient had a dystonic reaction with metoclopramide (0.049%). Eleven patients had a dystonic reaction with prochlorperazine (0.43%). The relative risk of a dystonic reaction with prochlorperazine over metoclopramide is 8.85 (95% confidence interval 1.15 to 68.5). There were differences between groups of patients who received metoclopramide versus prochlorperazine in terms of age, number of doses, and coadministration of diphenhydramine. In a logistic regression, administration of prochlorperazine over metoclopramide (P = 0.019) and greater number of doses (P < 0.001) remained associated with acute dystonic reactions., Conclusions: Dystonic reactions are rare events among pediatric patients treated for acute headache, but are more common with prochlorperazine than metoclopramide., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Nummular headache (NH) is a rare headache disorder characterized by a small, circumscribed painful area of the scalp. The description of many cases in the last years has supported its re-classification as a primary headache from the International Headache Society, moving it from its previous placement in the Appendix of the International Criteria of Headache Disorders., Methods: Data were collected from a retro-prospective observational study about rare headaches promoted by the RegistRare Network, a collaborative group of seven Italian Headache Centres. According to the gender-biased profile of certain primary headaches, we have looked further NH patients from a gender perspective., Results: Nineteen NH patients (11 men, 8 women) have been enrolled in the study. Headache onset was at 39 years and preceded approximately 8 years the diagnosis. No clinically evident differences between men and women have been found, including treatment prescriptions and headache resolution. Of note, the mean time from the onset of NH to the first visit in a Headache Centre was longer in men, compared with women (13.5 vs. 0.9 years). NH attacks were efficaciously treated with nonsteroidal anti-inflammatory drugs in 60% of patients receiving treatment. Headache prophylaxis with pregabalin and amitriptyline has been reported as effective in 40% and 67% of the treated patients, respectively., Conclusions: NH is a primary headache clinically heterogeneous in terms of temporal patterns and pain characteristics. Further research is needed to investigate the existence of male and female phenotypes, by clarifying whether it may be relevant for therapeutic purposes.

Introduction: Nummular headache was initially described as an infrequent primary headache characterised by chronic circumscribed mild-to-moderate pain in a small area of the head that may present with exacerbations. Since its initial description, dozens of cases of nummular headache have been reported which broaden the clinical and pathophysiological spectrum., Aims: To review and establish the clinical characteristics of nummular headache, to analyse the epidemiology in our setting and to clarify some unanswered pathophysiological issues., Patients and Methods: The study involved 83 cases (42 women and 41 men) who were diagnosed with nummular headache from July 2008 and followed up until 2018 at the Fundacion Alcorcon Hospital. The mean age of onset was 46 years. The time to diagnosis was seven months., Results: The pain was moderate-intense and oppressive, with exacerbations in 62.5% of cases. The average size of the symptomatic area was 2.4 ± 0.7 cm, with a parietal (47%), temporal (24%), frontal (12%) or occipital (6%) location. It presented a chronic (82%) or episodic (18%) pattern. Symptoms of local sensory dysfunction were observed in 25 patients and three others showed skin alterations. Spontaneous remission was observed in 36% of cases, and the most commonly used drugs were non-steroidal anti-inflammatory drugs, paracetamol and gabapentin. Botulinum toxin was effective., Conclusions: Nummular headache is a disorder with well-defined characteristics and is considered an entity in its own right. Our data indicate that its origin lies in the epicranial structures. The conjunction of pain, sensory dysfunction and trophic disorders suggests a local form of a complex regional syndrome.

Background and Purpose- We aimed to further investigate the long-term outcomes after reversible cerebral vasoconstriction syndrome (RCVS). Methods- A longitudinal follow-up study was conducted in 173 RCVS patients. Results- Of the 172 patients who completed a mean follow-up of 9.2±3.3 years, 10 had a recurrent RCVS that was benign in all. Independent predictors of relapse were having a history of migraine and having exercise as a trigger for thunderclap headache during initial RCVS. After new delivery, the rate of postpartum RCVS was 9%. Conclusions- Overall, long-term outcome after RCVS is excellent.

Background: Headache is one of the main complaints in pediatric neurology. Exogenous melatonin has been shown to be useful and safe in improving sleep-wake cycles and sleep quality in children. Tryptophan as well plays a key role in sleep regulation. So far, no studies tried to analyze the effects of a combination of both melatonin and tryptophan in treating chronic headache in children affected also by night-time awakenings., Methods: Thirty-four children with a diagnosis of chronic headache (with or without sleep disorders) have been enrolled. The study was articulated in two steps: 1) each child was observed for one month without any intervention; 2) children have been then randomized into two groups: the "ME-group", which received the nutritional supplement melatonin for two months and the "MET-group", which received the nutritional supplements melatonin, tryptophan, and vitamin B6 for two months., Results: In terms of changes in number of headache events, responders in the ME-group were 91.7% and those in the MET-group were 66.7% (P=0.113). In terms of changes in number of night awakenings, in the ME group, mean number at baseline, after 30 days, and after 60 days were 3.6±3.2, 3.2±3.5, and 2.7±3.4 (P=0.495). In the MET group, mean number of night awakenings was 7.4±8.1, 4.0±4.4, and 3.3±2.9 (P=0.041)., Conclusions: Using either nutritional supplement for two months can help in decreasing the monthly number of headache episodes and night awakenings. The addition of tryptophan and vitamin B6 appears to have stronger influence on night awakenings reduction than melatonin only.

To evaluate therapies employed in patients presenting to the emergency department (ED) with a chief complaint of non-traumatic headache to check if guidelines are followed. A 6-month retrospective analysis of the ED records of all the patients who referred to the ED of the University Hospital of Trieste for non-traumatic headache was performed. Out of 37.335 admissions, 336 patients were selected (0.9%). Diagnosis at discharge was primary headache (25.6%), secondary headache (40.5%), and headache "not otherwise specified" (33.9%). One-hundred-ninety-three patients were treated in mono- (51.8%) or poly-therapy (48.2%), with NSAIDs (46.5%), benzodiazepines (13.4%), antiemetics (10.7%), analgesics (8.3%), opioids (1.6%), triptans (1.5%), and other drugs (17.7%). NSAIDs, particularly ketorolac, are the class of drugs most often prescribed in ED, independently of the discharge diagnosis. Metoclopramide is rarely used in monotherapy (4%), but it is the drug most frequently used in association with NSAIDs (19.3%). Only two migraineurs received triptans. Mean time spent in ED was 231 ± 130 min, which was significantly longer in patients who received treatment (272 ± 141 vs. 177 ± 122 min; p = 0.003). No drugs had any side effects. In accordance with the current guidelines, NSAIDs monotherapy or in association with antiemetics were the drugs most often prescribed in ED. Opioids were rarely used probably because of potential sedative side effects. Only very few patients received triptans. Special attention should be drawn also in ED to apply the International Classification of Headache Disorders criteria, which can lead to clarify the diagnosis and receive the specific treatment.

Objectives: This proposed systematic review will identify the existing evidence on medication-overuse headache in children and adolescents., Background: A number of medications have been shown to be effective for acute treatment of migraine in children and adolescents. However, patients may find they need to use their acute medications more frequently when migraine frequency is high. This has led to concern about their potential to develop medication-overuse headache., Methods: We will search PubMed, Embase, Web of Science, PsycINFO, and Cochrane databases from their inception to present time. We will also search conference proceedings of the last 4 scientific meetings of relevant societies and scan the reference lists of studies identified through the search. Study designs will include case series, cross-sectional, cohort, case-control, and interventional studies. Participants will include children and adolescents under 18 years of age with primary headache disorders. We aim to determine whether frequency of acute medication use is associated with headache frequency in children and adolescents. Outcomes of interest include: (1) headache frequency; (2) change in headache frequency, with time and in relationship to use of acute medications; and (3) headache-related disability. We will also review data addressing treatment/management of medication overuse or medication-overuse headache in children and adolescents. Relevant comparators will be withdrawal vs reduction of acute medication, initiation of preventive therapy vs no initiation with or without withdrawal of acute medication, and initiation of preventive therapy early vs late. Outcomes of interest include (1) days of acute medication use; (2) headache frequency; (3) change in headache frequency; and (4) headache-related disability. After screening for inclusion, 2 team members will independently review and extract relevant data, and any discrepancies will be resolved through discussion and arbitration. We will assess risk of bias using appropriate tools (Cochrane Risk of Bias for randomized controlled trials (RCT) and Newcastle-Ottawa Score for observational studies). Data will be summarized descriptively in text and tables., Results: This systematic review will provide an overview of the available evidence on medication-overuse headache in children and adolescents., Conclusions: Findings from this review will aid clinicians by clarifying for them the current state of the evidence base, and will inform design of future research on this topic., (© 2019 American Headache Society.)

Rationale: Reversible cerebral vasoconstriction syndrome (RCVS) is often accompanied by thunderclap headaches. Although symptoms usually resolve spontaneously within 2 months, it can cause fatal complications, such as cerebral hemorrhage, and is difficult to differentiate from a migraine and other headaches on the basis of symptoms and Imaging study. In this case report, we explore clinical findings and appropriate treatment methods for RCVS through the case study of a female patient who experienced severe headache upon defecation PATIENT CONCERNS:: A 42-year-old female patient complained of a severe throbbing headache with a Numeric Rating Scale (NRS) score of 10 after defecation. The pain subsided temporarily after treatment with diclofenac 75 mg and Tridol 50 mg propacetamol 1 g, but the headache returned upon defecation; soon after, the patient complained again of regular headaches at 4 to 6-hour intervals irrespective of defecation., Diagnosis: Brain computed tomography (CT) and head and neck magnetic resonance angiography, performed during a headache episode, revealed no specific neurological findings. Blood analysis was also normal. Head and neck CT angiography, performed one month after the start of the headaches, revealed RCVS., Interventions: Treatment commenced with pregabalin (150 mg), oxycodone HCl/naloxone (10/5 mg), Alpram (0.5 mg), milnacipran (25 mg), and frovatriptan 25 mg, but there was no improvement in the headaches. The patient received bilateral trigger point injections (TPI) in the temporal muscles on four occasions at the pain clinic., Outcomes: Medication showed no effect, but after the patient received four sessions of bilateral TPI in the temporal muscles her NRS score eventually decreased from 10 to 2. The patient is currently continuing medication while still experiencing headaches at reduced intensities., Lessons: RCVS is difficult to diagnose; moreover, it is difficult differentiate RCVS from other headaches. However, as it can cause fatal complications, it should not be overlooked. It is essential to consider diagnostic treatment for all types of headaches because RCVS can be accompanied by headaches originating from other causes.

Background: Nummular headache is a primary headache characterised by superficial, coin-shaped pain. Superficial sensory fibre dysfunction might be involved in its pathophysiology. Considering the mechanism of action of onabotulinumtoxinA, it could be a reasonable option in treatment of nummular headache. The aim of the study was to evaluate the efficacy and tolerability of onabotulinumtoxinA in a series of nummular headache patients., Patients and Methods: This was an observational, prospective, non-randomized and open-label study. Nummular headache patients with at least 10 headache days in three preceding months were included. They were administered 25 units of onabotulinumtoxinA. The primary endpoint was the decrease of headache days per month, evaluated between weeks 20 to 24, compared with baseline. The secondary endpoints included reduction of intense headache days and acute treatment days evaluated between weeks 20-24 and weeks 8-12, compared with baseline. The 30%, 50% and 75% responder rates were determined, and tolerability described., Results: We included 53 patients, 67.9% females, with a median age of 54 years. Preventive treatment had been used previously in 60.4% of patients. The median diameter of the nummular headache was 5 cm. At baseline, the number of headache days per month was 24.5 (7.3); the number of intense headache days was 12.5 (10.1), and the number of acute treatment days was 12.8 (7.8). After onabotulinumtoxinA, the mean number of headache days per month decreased to 6.9 (9.3) between weeks 20 and 24 ( p  < 0.001). Secondary endpoints concerning intense headache days per month and acute treatment days per month were also statistically significant ( p  < 0.001). The 50% responder rate, evaluated between weeks 20 and 24, was 77.4% and the 75% responder rate was 52.8%. Concerning tolerability, 26 patients (49.1%) experienced an adverse event (AE), the commonest being injection-site pain in 12 cases (22.6%). There were no moderate or severe AEs., Conclusion: It was found that after injecting onabotulinumtoxinA, the number of headache days per month was reduced in nummular headache patients. The number of intense headache days per month and acute treatment days were also lowered. No serious adverse events occurred during treatment.

Background and Aim: Headache is very often the cause for seeking an emergency department (ED). However, less is known about the different diagnosis of headache disorders in the ED, their management and treatment. The aim of this survey is to analyse the management of headache patients in two different ED in Europe., Methods: This retrospective survey was performed from September 2018 until January 2019. Patients were collected from the San Luca Hospital, Milan, Italy and the Ordensklinikum Barmherzige Schwestern, Linz, Austria. Only patients with a non-traumatic headache, as the primary reason for medical clarification, were included. Patients were analysed for their complexity and range of examination, their diagnoses, acute treatment and overall efficacy rate., Results: The survey consists of 415 patients, with a mean age of 43.32 (SD ±17.72); 65% were female. Technical investigation was performed in 57.8% of patients. For acute treatment non-steroidal-anti-inflammatory drugs (NSAIDs) were the most used, whereas triptans were not given. A primary headache disorder was diagnosed in 45.3% of patients, being migraine the most common, but in 32% of cases the diagnosis was not further specified. Life-threatening secondary headaches accounted for less than 2% of cases., Conclusions: The vast majority of patients attending an ED because of headache are suffering from a primary headache disorder. Life-threatening secondary headaches are rare but seek attention. NSAIDs are by far the most common drugs for treating headaches in the ED, but not triptans.

Objectives: To evaluate providers' use and predictors of evidence-based medicine or opioid/barbiturate as first-line acute treatment for children's initial presentation of acute migraine or primary headache., Methods: This retrospective, observational study utilized patient (children ages 6-17) and provider/encounter characteristics extracted from the patient's Electronic Health Record from 2008-2014 during an initial encounter for migraine or primary headache. The primary outcome was provider evidence-based medicine utilization; overall prescriptions and opioid/barbiturate prescriptions were also evaluated. Hierarchical linear modeling examined whether Level 1 (patient: Demographic, insurance type) and Level 2 (provider/encounter: Treatment setting/location, encounter diagnoses) characteristics influenced outcomes., Results: In all, 38,926 patients (56.7% female, mean age = 12.1) and 1617 providers were evaluated. Only 17.7% of patients were diagnosed with migraine; 16.1% received evidence-based medicine. Older children (OR = 1.07, p  < 0.001), females (OR = 1.14, p  < 0.001), and those diagnosed with migraine (OR = 4.71, p  < 0.001) were more likely to receive evidence-based medicine. Among prescriptions, 15.8% were for opioids/barbiturates. Older children (OR = 1.14, p  < 0.001) and those cared for in the emergency department/urgent care (OR = 2.02, p  < 0.001) were at increased risk., Conclusions: Demographics and migraine diagnosis are associated with evidence-based medicine and opioid/barbiturates. Primary care provides an opportunity to target provider interventions to enhance effective pediatric headache treatment.

Hypnic headache (HH) is a rare benign disorder described initially by Raskin in 1988. It is characterized by recurrent nocturnal episodes of headache that periodically awaken the sleeping patient and usually occur in the elderly. This review aimed to describe the clinical features of the HH cases published in the literature from 1988 to 2018. Based on literature search in the major medical databases (LiLacs, SciELO, Bireme, Medline, Embase, Current Contents, Scopus, EBSCO and PubMed), we have analyzed the case reports on HH that have been published from 1988 to 2018. We described 343 adults (69.0% women and 31.0% men) and 5 children (3 girls and 2 boys) diagnosed with HH. Average age for adults and children was, respectively, 58.0 ± 13.1 years (ranging from 15 to 85 years) and 9 years (ranging from 7 to 11 years). The diagnosis was made 7.6 ± 14.2 years (range 0.1 to 39 years) after onset of headache. Pain occurred during nocturnal sleep (94.8%), with an average duration of 90 min, bilaterally located (55.5%), having a dull character (74.4%), and moderate intensity (61.5%). In 94.5% of the patients, headache occurred for 10 or more days per month (mean of 21 days). Autonomic manifestations occurred in 7.6% of the patients, predominantly lacrimation (61.1%) and rhinorrhea (16.7%). Caffeine presented the best therapeutic response in acute treatment. In prophylaxis, lithium, caffeine and indomethacin were effective drugs in 77.8% of the patients. In 56.7% of the patients there was remission with treatment and in 72.7% of them, without recurrence. HH is a rare disease that usually occurs for the first time in older women but may begin in childhood. Lithium and caffeine are effective drugs for pain prophylaxis, but randomized clinical trials are required., (Copyright © 2019. Published by Elsevier B.V.)

Purpose of Review: To provide a comprehensive and updated review of the literature on primary stabbing headache., Recent Findings: Changes to the ICHD-3 criteria have resulted in increased sensitivity to capture primary stabbing headache (PSH). According to the ICHD-3, the sharp stabbing pain is no longer restricted to the first division of the trigeminal nerve. Age, gender, and co-morbidities such as migraine seem to influence the prevalence of PSH. Subclassification into monophasic, intermittent, and chronic forms have been proposed in a recent prospective study and may be helpful from a prognostication perspective; however, further studies are required. Secondary etiologies for stabbing headaches are part of the differential diagnosis of primary stabbing headache; therefore, it is reasonable to perform neuroimaging. For severe frequent attacks, indomethacin continues to be considered first line. Other treatment options include COX2 inhibitors and melatonin.

Introduction : Acute and preventive treatment of primary headache disorders is not completely resolved with regard to efficacy, safety, and tolerability. Hence, peripheral and central neuromodulation can provide therapeutic alternatives in drug-resistant cases. Peripheral targets of neuromodulation include invasive and non-invasive neurostimulation and electrical and chemical nerve and ganglion blockades. Areas covered : A PubMed search of papers published from January 2012 to October 2018 was conducted. The goal of this review was to analyze the efficacy and safety of invasive (implantable) peripheral neurostimulation methods (the occipital nerve, the cervical branch of vagal nerve, the sphenopalatine ganglion) and non-invasive (transcutaneous) peripheral neurostimulation methods (the occipital nerve, the supraorbital nerve, and the cervical and auricular branches of the vagal nerve), based on the results of published clinical trials and case series. Acting also on the peripheral nervous system, peripheral nerve (i.e. greater occipital nerve) and ganglion (i.e. sphenopalatine ganglion) blockades, botulinum neurotoxin type A-hemagglutinin complex therapies, and calcitonin gene-related peptide-related monoclonal antibody treatments in this patient population are also discussed. Expert opinion : This review summarizes the latest results on the therapeutic strategies acting on the periphery in primary headache disorders. These therapeutic options are minimally invasive or non-invasive, efficacious, safe, and well tolerated.

Reversible cerebral vasoconstriction syndrome (RCVS) is a rare condition characterised by repetitive, multifocal, vasofluctuations of cerebral arteries. A key symptom is chronic, disabling 'thunderclap' headaches, which are extremely difficult to treat as established medications may exacerbate the pathophysiology of RCVS. OnabotulinumtoxinA (OBT-A) injections are used for the prophylaxis of chronic daily headaches (CDH). The mechanism of action of OBT-A significantly differs from oral headache treatments. Thus, OBT-A may be an effective, safe treatment of RCVS-CDH. A 51-year-old woman with RCVS-CDH presented to outpatient clinic. This case report describes the first, believed, documented treatment of RCVS-CDH by OBT-A injections. In 2018, the consented patient received a total of 200 units of OBT-A, 155 units to the 31 approved U.S. Food and Drug Administration (FDA) sites and 45 units injected into the bilateral occipital belly of occipitofrontalis muscles. The patient reported 3 months of excellent pain relief (60% reduction). Three rounds of OBT-A injection, each 3 months apart, resulted in 80% reduction. OBT-A injections may prove a successful, novel treatment for RCVS-CDH., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Shields JL. You're the flight surgeon: potential migraine in an Air Force aviator-to diagnose or not to diagnose. Aerosp Med Hum Perform. 2019; 90(5):494-496.

Objective: To report 2 new nummular headache (NH) cases and review the clinical features and management of NH., Background: NH is a coin-shaped headache with a fixed shape and size. Since the first case series was reported by Pareja, approximately 300 cases have been reported. However, the latest treatment status and pathogenesis of NH remain unclear., Methods: Two new NH cases with improved symptomatic responses to metoprolol were reported. We also conducted a narrative review of cases published in English aiming to provide the newest information about the clinical features and management of NH., Results: Two NH patients with skin depressions within the painful area experienced significantly improvement with metoprolol. The literature review showed that NH was mainly moderate-to-severe in 77.8% (186/239) of the patients. Sensory dysfunction and focal trophic changes were reported in 47.8% (149/312) and 3.8% (12/312) of the patients, respectively. Gabapentin was the most commonly used and effective prophylactic, with a responder rate of 67.9% (55/81). Botulinum toxin type A was used for refractory NH, with an effective rate of 81.8% (9/11). Ten patients from the previous studies were prescribed β-adrenergic blockers without any relief, and none of them reported focal tropic changes., Conclusions: NH can be a disabling headache in more than half of identified patients and requires medical intervention. As with the previous treatment recommendations, gabapentin was the most commonly used prophylactic with a good response. Our cases suggested that β-adrenergic blockers could be a therapeutic option for NH patients with focal trophic changes within the areas., (© 2019 American Headache Society.)

Primary headache syndromes and chronic pain syndromes are common in patients with postural tachycardia syndrome (POTS). There is overlap in potential mechanisms for migraine, chronic pain, and POTS symptomatology. Management of chronic pain and headaches in POTS requires a judicious use of pharmacotherapies that takes into account patient comorbidities and co-existing symptoms. Patient-centric, non-pharmacologic modalities include physical exercise, cognitive behavioral therapies, and treatment of sleep disorders., (Published by Elsevier B.V.)

Introduction: Primary stabbing headache (or "ice pick headache") is an alteration characterized by brief jabs (short stabs of pain, lasting ~3 seconds), which appear spontaneously, irregularly, and affecting unilaterally or bilaterally. Indomethacin has traditionally been used as the main therapeutic option. However, this drug is ineffective in a considerable percentage of patients and can generate multiple adverse effects that occur at therapeutic doses., Clinical Case: A 7-year-old male patient with primary stabbing headache of mild to moderate intensity, lasting 3 to 4 seconds, without relevant history, with normal neurodevelopment, neurological examination and neuroimaging; no triggers were identified. It was started therapeutic trial with Coenzyme Q10; however, no improvement in the symptoms was identified., Treatment and Outcomes: A therapeutic management was carried out with Melatonin, which led to complete remission of the symptoms; without adverse effects in the posterior follow-up months., Clinical and Scientific Relevance: There is little information regarding effective and safe treatments for primary stabbing headache in children. The present case identifies Melatonin as an innovative, effective and safe therapeutic alternative in the treatment of primary stabbing headache in children. This is a significant advance in the understanding of primary stabbing headache in the pediatric population., Conclusion: Melatonin may be an effective and safe therapeutic option for the treatment of primary stabbing headache in pediatric patients. It is necessary to deepen its research, in order to establish its use in a clinical practice guide., Competing Interests: Conflicts of Interest: The authors declare that there were no conflicts of interest for the writing of this article

Reversible cerebral vasoconstriction syndrome is a rare clinical syndrome characterized by sudden thunderclap headache often an under diagnosed neurological emergency. It is often provoked by postpartum state or exposure to provocative drugs. We report a rare case of Rizatriptan-induced reversible cerebral vasoconstriction syndrome presenting with thunderclap headache and paraparesis with complete recovery of neurological and imaging findings.

Purpose of Review: The purpose of this review is to summarize the most up-to-date literature on bath-related headache, a rare disorder., Recent Findings: Initially described in middle-aged Asian women, it is now reported in a wider demographic. More information is available about the pathophysiology of bath-related headache, including its classification as a subtype of reversible cerebral vasoconstriction syndrome (RCVS). Nimodipine can be effective in patients both with and without vasospasm. Bath-related headache is a rare form of thunderclap headache. Although its mechanism is still unclear, it is associated with vasospasm and RCVS. Controlled trials investigating the use of nimodipine and other agents may be useful in furthering our understanding of and treatment of this phenomenon.

Background: Although risk factors for medication-overuse headache have been identified within the general population, most studies have neglected clinical samples. The present study examined the relative and combined associations of these factors with medication-overuse headache in a sample of US adults seeking treatment for primary headache disorders., Methods: Treatment-seeking headache patients provided data on demographics, headache variables, psychiatric variables, use of headache medications, and use of other prescription medications and substances. A classification tree selection strategy was utilized within this cross-sectional study to differentiate between those with and without medication-overuse headache, and a final multivariable model assessed their combined utility., Results: Forty-three of 164 participants (26.2%) met diagnostic criteria for medication-overuse headache. Relative to non-medication-overuse headache participants, participants with medication-overuse headache reported greater headache-related disability (odds ratio = 1.09, 95% confidence interval = 1.01-1.18), escape and avoidance responses indicative of fear of pain (odds ratio = 1.07, 95% confidence interval = 1.00-1.15), and use of combination medications for headache (odds ratio = 3.10, 95% confidence interval = 1.51-6.36). The final multivariable model differentiated well between the 2 groups (area under the receiver operating characteristic curve = .78; 95% confidence interval = .71-.86)., Conclusions: Items that assess headache-related disability, use of combination medications, and fear of pain help identify patients who are currently overusing acute headache medications and may serve as indicators of treatment progress. Future studies should apply similar analytic approaches longitudinally to identify headache sufferers at risk for medication-overuse headache prior to headache progression., (© 2018 American Headache Society.)

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.

Background: Headache is one of the commonest complaints reported to physicians worldwide. Yet, arriving at the proper diagnosis can be a challenge in many patients. Although most headaches belong to common categories of migraine and tension-type headache, which are diagnosed and managed relatively easily, several uncommon headache disorders can lead to delays in diagnosis. Certain medications are more efficacious than others in managing these headache disorders, hence establishing the correct diagnosis is of paramount importance., Case Presentation: An 86-year-old female presented with chronic daily headache of 1 year duration. Her headaches were exclusively nocturnal and woke her up daily around midnight. Clinical examination was unremarkable. All basic investigations were normal. Subsequent gadolinium enhanced Magnetic Resonance Imaging (MRI) brain did not show any significant pathology. There was no satisfactory response to paracetamol, diclofenac sodium, mefenamic acid, tramadol, flunarizine and sodium valproate. Indomethacin was started with the provisional diagnosis of hypnic headache. There was absolute response by day 3 of indomethacin. She remains headache free on low dose indomethacin maintenance at 1 year after the diagnosis., Conclusion: Better understanding of uncommon headache syndromes can help in early diagnosis and appropriate treatment. Hypnic headache should be considered in the differential diagnosis of chronic daily headaches, especially when nocturnal and occurs during sleep.