Recurrent thunderclap headache after endovascular embolization of pial AV malformation by onyx material.

Thunderclap headache is a sudden severe headache with onset to peak within one minute. Multiple excruciating, short-lived thunderclap headaches over a few days to weeks are highly suggestive of reversible cerebral vasoconstriction syndrome (RCVS). RCVS can be primary or secondary to several factors, but it is rarely described after neuro-endovascular procedures using onyx material. A 10-year-old child presented with RCVS heralded by recurrent thunderclap headache following endovascular embolization of pial arteriovenous malformation with onyx material (contains organic solvent dimethyl sulfoxide). Dimethyl sulfoxide is an angiotoxic material that can cause dysregulation of cerebral vascular tone triggering reversible cerebral vasoconstriction syndrome. Recurrent thunderclap headache after embolization procedures using onyx material should prompt for the diagnosis of reversible cerebral vasoconstriction syndrome.

Primary Cough Headache.

PURPOSE OF REVIEW: In this review article, we summarize the most recent findings and observations in patients experiencing primary cough headache. RECENT FINDINGS: Recent studies draw attention to further characterization of patients with primary cough headache in a pulmonology clinic. There have also been recent case studies describing new therapy considerations, including noninvasive vagus nerve stimulation and lumbar punctures. Primary cough headache has been a recognized entity since as early as 1932. Patients generally report a sudden onset, sharp headache lasting a few hours that is precipitated by Valsalva maneuver or coughing. It remains an uncommon diagnosis, and secondary causes are crucial to rule out with imaging studies. Additionally, there can be an overlap with other primary headache disorders such as primary exercise headache and headache associated with sexual activity, thus requiring a detailed history. Many hypotheses exist regarding the pathophysiology including relative pressure gradients and a crowded posterior fossa. Generally, patients are responsive to indomethacin, but more recent case reports have suggested benefits of other medications and procedures that may offer patients an alternative option, including vagus nerve stimulation, lumbar punctures, and beta blockers. Future case series and studies should focus on management of these patients, with a specific focus on individuals who do not tolerate indomethacin.

Hypnic Headache - What do we know in 2022?

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.

Digital headache self-management interventions for patients with a primary headache disorder: A systematic review of randomized controlled trials.

OBJECTIVE: This article systematically reviews the empirical literature examining the efficacy of digital headache management interventions for patients with a primary headache disorder. BACKGROUND: Digital headache management interventions provide opportunities to improve access to behavioral headache interventions to underserved groups. METHODS: A systematic search of PubMed, Scopus, and EBSCO (PsycInfo, Education Research Complete, ERIC, Health Source: Nursing/Academic Edition, Psychology and Behavioral Sciences Collection) and reference review was conducted. Included studies had to recruit a sample with a primary headache diagnosis, be a randomized controlled trial including a digital component, assess a headache outcome (i.e., frequency, duration, severity, intensity, disability) or quality of life, and be published in English. Two authors independently extracted data for included studies. The methodological quality of studies was assessed using the revised Cochrane risk-of-bias tool. RESULTS: Thirteen studies with unique interventions met inclusion criteria. More than half of the studies were pilots; however, nearly 70% (9/13) demonstrated significant between-group or within-group improvements on one or more headache-related outcomes. All interventions included some form of relaxation training and the majority were delivered via interactive website. While fewer than half the studies report participant race and/or ethnicity, of those that do, 83% (5/6) reported a predominately White/Caucasian sample. CONCLUSIONS: Efficacy testing of digital headache interventions is in its infancy with the majority of these studies relying on pilot studies with small samples comprised of homogenous patient populations. Interactive websites were the most common digital medium to deliver digital headache management interventions and have demonstrated promising results. Further testing using large-scale randomized controlled trials and exploration of other digital tools is warranted. Future studies with more diverse samples are needed to inform health equity of digital headache interventions.

Reversible Cerebral Vasoconstriction Syndrome and Multisystem Inflammatory Syndrome in Children With COVID-19.

BACKGROUND: Multisystem inflammatory syndrome in children (MIS-C) involves multiple organs and shows increased inflammatory markers. Since the onset of the coronavirus disease 2019 (COVID-19) pandemic, several studies have reported the association between severe COVID-19 and MIS-C. Reversible cerebral vasoconstriction syndrome (RCVS) presents with thunderclap headaches and multifocal reversible vasoconstriction on imaging. RCVS is very rare in children. This article reports two cases of pediatric COVID-19 with severe MIS-C and clinical and imaging features indicative of RCVS. METHODS: Clinical, laboratory, and imaging data of the patients were reviewed. The diagnosis of RCVS was confirmed based on clinical symptomatology and brain magnetic resonance imaging findings. RESULTS: Two pediatric patients with clinical findings compatible with severe MIS-C and hemodynamic compromise presented to the hospital. During their hospitalization course, they developed thunderclap headaches and neurological deficits. Both were receiving vasoactive agents, intravenous immunoglobulin, and immunosuppressants. Imaging studies showed marked multifocal cerebral vasoconstriction in both cases and infarcts in one. The course and management of the patients will be presented. After controlling inflammation and elimination of triggers, both patients were ultimately symptom free upon discharge. Cerebral vasoconstriction had completely resolved on follow-up imaging. CONCLUSIONS: Although a variety of symptoms including headaches may be seen in pediatric COVID-19 patients with MIS-C, RCVS should be considered as a differential diagnosis in cases of thunderclap headache accompanied by neurological signs in these patients. Imaging findings and follow-up are also key in establishing the diagnosis.

Effectiveness of Peripheral Nerve Blocks for the Treatment of Primary Headache Disorders: A Systematic Review and Meta-Analysis.

STUDY OBJECTIVE: Primary headache disorders are prevalent and account for 2% of all emergency department visits. Current treatment options are effective; however, time to pain relief is suboptimal. Alternatives such as peripheral nerve blocks have shown promising results. The objective of this systematic review is to examine the effectiveness of peripheral nerve blocks for timely pain relief. METHODS: We searched Ovid MEDLINE, EMBASE, Web of Science Core Collection, and the Cochrane Central Register of Controlled Trials and included randomized controlled trials comparing peripheral nerve blocks to placebo or active therapy. The primary outcome was pain within 120 minutes. Secondary outcomes were pain after 120 minutes, adverse events, need for rescue medications, and relapse of headache. Two reviewers screened and extracted data independently; mean differences (MDs) were calculated, and results were pooled using a random-effects model. RESULTS: Eleven studies met our eligibility criteria (n=860), of which 9 were included in the meta-analysis. Pain scores were significantly lower in patients treated with peripheral nerve blocks than with placebo at 15 minutes (MD: -1.17; 95% confidence interval: -1.82 to -0.51) and 30 minutes (MD: -0.99; 95% confidence interval: -1.66 to -0.32), and no serious adverse events were reported. Pain scores for peripheral nerve blocks versus active therapy and secondary outcomes were not pooled due to clinical heterogeneity. CONCLUSION: Our review shows peripheral nerve blocks are effective as a rapid treatment option when compared to placebo; however, we were unable to assess effectiveness against standard treatment. Emergency physicians should consider peripheral nerve blocks as an adjunct therapy for patients with primary headache disorders.

Reversible cerebral vasoconstriction syndrome: A narrative review for emergency clinicians.

INTRODUCTION: Reversible Cerebral Vasoconstriction Syndrome (RCVS) is a rare cause of severe headache that can mimic other causes of sudden, severe headache and result in frequent emergency department (ED) visits. OBJECTIVE: This narrative review provides an evidence-based update concerning the presentation, evaluation, and management of RCVS for the emergency clinician. DISCUSSION: RCVS can present as recurrent, severe headaches that may be maximal in onset, known as a thunderclap headache. Distinguishing from other causes of thunderclap headache such as aneurysmal subarachnoid hemorrhage, cerebral venous thrombosis, and posterior reversible encephalopathy syndrome is challenging. Risk factors for RCVS include use of vasoactive substances, exertion, coughing, showering, sexual activity, and cervical artery dissection. Diagnosis relies on clinical features and imaging. Cerebral catheter digital subtraction angiography (DSA) is considered the gold standard imaging modality; however, computed tomography angiography or magnetic resonance angiography are reliable non-invasive diagnostic modalities. Treatment focuses on avoiding or removing the offending agent, administration of calcium channel blockers such as nimodipine, and reversing anticoagulation if bleeding is present. Although most cases have a benign course and resolve within 3 months, focal subarachnoid hemorrhage, intracerebral hemorrhage, permanent neurologic disability, or death can occur in a minority of cases. CONCLUSIONS: Diagnosis and appropriate management of RCVS can be aided by understanding key aspects of the history and examination. The emergency clinician can then obtain indicated imaging, confirming the diagnosis and allowing for appropriate management.

Headache in transgender and gender-diverse patients: A narrative review.

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.

Improving Acute Treatment of Pediatric Primary Headache Disorders With a Novel Headache Treatment Center: Retrospective Review of Preliminary Outcomes.

OBJECTIVE: To determine preliminary outcomes of targeted headache treatments provided at a novel outpatient acute care pediatric headache treatment center. BACKGROUND: Limitations exist in acute management of pediatric headaches, including inadequate access to specialty headache therapies and headache specialists in acute settings, variable success of emergency room treatments, and omission of comfort measures. An outpatient acute headache care clinic (the "Headache Treatment Center") was strategically initiated at a Midwestern pediatric academic hospital to provide acute and targeted headache therapies for children with active headaches. METHODS: We conducted a retrospective chart review of 154 visits from September through November 2018 of patients ages 7-18 years visiting the Headache Treatment Center. RESULTS: On average, headache intensity (measured on an 11-point pain numeric rating scale) decreased after interventions used in the Headache Treatment Center (mean change = 2.85 ± 2.81, P < .05, Cohen d = 1.01). Large effect sizes for reducing headache intensity were observed for pericranial, occipital/auriculotemporal, and occipital nerve blocks, Cohen d = 1.56, 1.64 and 1.02, respectively. Large effect sizes for reducing headache intensity also were observed for a transcutaneous supraorbital nerve stimulator device (Cefaly) (Cohen d = 1.02), acupuncture (Cohen d = 1.09), and intravenous migraine cocktails (Cohen d = 0.91-1.34). CONCLUSION: Targeted headache therapies to abort pediatric primary headaches as part of a novel headache clinic model may be beneficial for short-term management.

Acceptance and Commitment Therapy for Primary Headache Sufferers: A Randomized Controlled Trial of Efficacy.

Prevention of headaches via avoidance of triggers remains the main behavioral treatment suggestion for headache management despite trigger avoidance resulting in increases in potency, lifestyle restrictions, internal locus of control decreases, pain exacerbation and maintenance. New approaches, such as Acceptance and Commitment Therapy (ACT), instead emphasize acceptance and valued living as alternatives to avoidance. Though ACT is an empirically supported treatment for chronic pain, there is limited evidence for headache management while preliminary outcome studies are afflicted with methodological limitations. This study compared an ACT-based group headache-specific intervention to wait-list control, in a randomized clinical trial, on disability, distress, medical utilization, functioning, and quality of life. Ninety-four individuals with primary headache (84% women; Mage = 43 years; 87.35% migraine diagnosis) were randomized into 2 groups (47 in each). Assessments occurred: before, immediately after, and at 3 months following treatment end. Only the ACT group was additionally assessed at 6- and 12-month follow-up. Results (intent to treat analyses corroborated by linear mixed model analyses) showed substantial improvements in favor of ACT compared to control, on disability, quality of life, functional status, and depression at 3-, 6-, and 12-month follow-up. Improvements were maintained in the ACT group at 6- and 12-month follow-up. At 3-month follow-up, clinical improvement occurred in headache-related disability (63%) and 65% in quality of life in ACT versus 37% and 35% in control. These findings offer new evidence for the utility and efficacy of ACT in localized pain conditions and yields evidence for both statistical and clinical improvements over a years' period. PERSPECTIVE: An Acceptance and Commitment Therapy approach focusing on acceptance and values-based activities was found to improve disability, functioning, and quality of life among patients with primary headaches.

Sphenopalatine Ganglion Stimulation for Chronic Headache Syndromes.

Neuropathic facial pain is notoriously difficult to treat, regardless of its origin and duration. Since the first reported sphenopalatine ganglion blockade by Sluder in 1908, this ganglion has assumed an important role among the structures targeted for the treatment of facial pain. Recent years have witnessed the rise of neuromodulation over ablative procedures, including the development of an implantable stimulation device specially designed for use in the pterygopalatine fossa. Sphenopalatine ganglion stimulation has been demonstrated as effective and safe for refractory cluster headache, today the major indication for this therapy, but increasing evidence shows that the effect on the autonomic system and cerebral circulation could justify an even wider use of sphenopalatine ganglion stimulation for other chronic headache syndromes and vascular diseases.

Determinants of Chiari I progression in pregnancy.

Chiari Malformation type 1 (CM-I) is congenital or an acquired anomaly of the hind brain; develops when the cerebellar tonsils recede downwards below the foramen magnum. Recurrent post tussive suboccipital headache is the common presentation in a pregnant woman and the diagnosis is usually missed or delayed due to lack of formal understanding of this neurological pathology. Much has been written regarding presentation, morphology and the treatment of CM-I; however, little is known when the etiology is acquired or an iatrogenic in its evolution. Similarly, unknown is the progression of CM-I (diagnosed or undiagnosed) in pregnancy. The objective of this study is to elucidate the causes of progression of CM-I in pregnancy, and how this can be avoided. A detailed literature review has been conducted to find the case reports or case studies on association of CM-I in pregnancy; therefore, the risk factors regarding the progression have been sought. There is a lack of literature on timing, mode of anesthesia, and the management of CM-I. Moreover, authors have sought a questionnaire to screen these patients at pre-conception, intrapartum visits if, the initial diagnosis is delayed. Crucial points of concern including but not limited to the diagnosis, pre-conception counseling, timing of intervention during pregnancy, and mode of anesthesia, have been discussed in detail. In summary, a formal management algorithm has been proposed to avoid the rapid progression of this complex neurological pathology especially, in women of child bearing age and/or during pregnancy.

Headache in the Older Population: Causes, Diagnoses, and Treatments.

PURPOSE OF REVIEW: Primary headaches are less common and differ in presentation in older versus younger individuals. Secondary headaches become more common among older patients. RECENT FINDINGS: Diagnosis and management of headaches in those > 65 years are discussed. Migraine and tension-type headaches are rarely new onset in this age group and should be a diagnosis of exclusion. In older individuals, migraine is more likely to be bilateral with less sensory sensitivities. Migraine aura may present without headache; careful assessment is needed to exclude stroke. Other primary headaches discussed include cough, hypnic, and other headaches. Secondary causes discussed include giant cell arteritis, trigeminal post-herpetic neuropathy, sleep apnea, cardiac cephalgia, cervicogenic pain, vascular etiologies, medications, and burning-mouth syndrome. In older individuals, primary headaches are diagnoses of exclusion, and treatment is affected by comorbidities and polypharmacy. Secondary headaches are a major consideration requiring appropriate workup. Many treatments can safely be offered regardless of age.

Public policies in headache disorders: needs and possibilities.

Although headaches have recognized impact, there are no public policies in Brazil addressing this problem. The Brazilian Headache Society and the Brazilian Association of Cluster Headache and Migraine promoted a summit to discuss Public Policy and Advocacy for headache disorders. Professionals from various segments, representing various sectors of society, gathered in April 2019 in Brasília, defining the most important points for achieving advances in public policies in headache in Brazil, such as: inclusion in the chronic diseases surveillance agenda; improving public understanding and access to diagnosis and treatment; teaching in colleges and medical residences, structuring care networks, intervention models, clinical protocols and legislation supporting public policies in headache.

[Effectiveness of Biofeedback for Primary Headache - A Randomized Controlled Study]. / Wirksamkeit von Biofeedback bei primärem Kopfschmerz ­ Eine randomisierte, kontrollierte Studie.

BACKGROUND: Biofeedback-treatment for primary headache is highly recommended. Its expenses are mostly not or not completely covered by health funds, hindering the implementation in the care system. This randomized controlled study aimed at evaluating the efficacy of a biofeedback-treatment for primary headache in the outpatient care system all over Germany. METHODS: The participants were divided into a direct treatment-group (n=41) and a waiting control-group (n=29). The treatment was carried out for 8-11 sessions with vasoconstriction-/vasodilation training for migraine, with electromyography training for headache of the tension type (diagnosis according to the criteria of the International Headache Association). Headache characteristics (frequency, duration, intensity) were collected via headache-diary before, during and after the treatment and headache specific disability using the Pain Disability Index before and after the treatment. The waiting group stated additional information of the questionnaires about 3 months before and again ultimately before the start of the treatment. Measurement of the headache specific self-efficacy (Headache Management Self-efficacy Scale) took place every second session. For the collected parameters the effect sizes f2 or r were computed before in comparison to after the treatment and comparing the treatment-group to the waiting-group. RESULTS: Headache characteristics decreased during the time of the study significantly linearly with medium to strong effects (frequency f2=0.302, p<0.001, duration f2=0.186, p<0.001, mean intensity f2=0.502, p<0.001, maximal intensity f2=0.546, p<0.001). Using biofeedback the self-efficacy rose significantly (p<0.001, r=0.782), even compared to the waiting time (p<0.001, r=0.604). Additionally the handling of pain, the psychological disability and the disability in everyday life improved significantly. DISCUSSION AND CONCLUSION: Overall the effects were comparable to already existing studies. Therefore biofeedback turned out to be efficient against migraine and tension-type headache in the out-patient care setting. By that a better integration in the care system seems to be qualified.

Public policies in headache disorders: needs and possibilities / Políticas públicas em cefaleias: necessidades e possibilidades

Abstract Although headaches have recognized impact, there are no public policies in Brazil addressing this problem. The Brazilian Headache Society and the Brazilian Association of Cluster Headache and Migraine promoted a summit to discuss Public Policy and Advocacy for headache disorders. Professionals from various segments, representing various sectors of society, gathered in April 2019 in Brasília, defining the most important points for achieving advances in public policies in headache in Brazil, such as: inclusion in the chronic diseases surveillance agenda; improving public understanding and access to diagnosis and treatment; teaching in colleges and medical residences, structuring care networks, intervention models, clinical protocols and legislation supporting public policies in headache.

Resumo Embora as cefaleias tenham reconhecido impacto, não há no Brasil políticas públicas voltadas para este problema. A Sociedade Brasileira de Cefaleia e Associação Brasileira de Cefaleia e Enxaqueca promoveram um seminário para a discussão de Políticas Públicas e Advocacy (defesa dos direitos dos pacientes) em Cefaleias. Reuniram-se em abril de 2019 em Brasília, profissionais de vários segmentos, representando diversos setores da sociedade, definindo os pontos mais importantes para que se obtenham avanços nas políticas públicas em cefaleias no Brasil, tais como: inclusão na agenda de vigilância das doenças crônicas não transmissíveis; melhora do entendimento do público e seu acesso a diagnóstico e tratamento; ensino em faculdades e residências médicas, estruturação de redes de atendimento, modelos de intervenção, protocolos clínicos e legislação de apoio às políticas públicas em cefaleia.

Neurostimulation methods in the treatment of chronic pain.

The goal of this narrative review was to give an up-to-date overview of the peripheral and central neurostimulation methods that can be used to treat chronic pain. Special focus has been given to three pain conditions: neuropathic pain, nociplastic pain and primary headaches. Both non-invasive and invasive techniques are briefly presented together with their pain relief potentials. For non-invasive stimulation techniques, data concerning transcutaneous electrical nerve stimulation (TENS), transcranial direct current stimulation (tDCS), repetitive transcranial magnetic stimulation (rTMS), remote electrical neuromodulation (REN) and vagus nerve stimulation (VNS) are provided. Concerning invasive stimulation techniques, occipital nerve stimulation (ONS), vagus nerve stimulation (VNS), epidural motor cortex stimulation (EMCS), spinal cord stimulation (SCS) and deep brain stimulation (DBS) are presented. The action mode of all these techniques is only partly understood but can be very different from one technique to the other. Patients' selection is still a challenge. Recent consensus-based guidelines for clinical practice are presented when available. The development of closed-loop devices could be of interest in the future, although the clinical benefit over open loop is not proven yet.