New Daily Persistent Headache in a Pediatric Population.

INTRODUCTION: New daily persistent headache (NDPH) is a primary headache disorder characterized by an intractable, daily, and unremitting headache lasting for at least 3 months. Currently, there are limited studies in the pediatric population describing the characteristics of NDPH. OBJECTIVE: The objective of the current study is to describe the characteristics of NDPH in pediatric patients presenting to a headache program at a tertiary referral center. METHODS: The participants in the current study were pediatric patients who attended the Headache Clinic at Children's National Hospital between 2016 and 2018. All patients seen in the Headache Clinic were enrolled in an institutional review board-approved patient registry. RESULTS: Between 2016 and 2018, NDPH was diagnosed in 245 patients, representing 14% of the total headache population. NDPH patients were predominantly female (78%) and white (72%). The median age was 14.8 years. The median pain intensity was 6 of 10 (standard deviation = 1.52). Most patients reported experiencing migrainous features, namely, photophobia (85%), phonophobia (85%), and a reduced activity level (88%). Overall, 33% of patients had failed at least 1 preventive medication, and 56% had failed at least 1 abortive medication. Furthermore, 36% of patients were additionally diagnosed with medication overuse headache. CONCLUSION: NDPH is a relatively frequent disorder among pediatric chronic headache patients. The vast majority of these patients experience migrainous headache characteristics and associated symptoms and are highly refractory to treatment-as evidenced by a strong predisposition to medication overuse headache and high rates of failed preventive management.

Migraña crónica con cefalea diaria. Revisión de la bibliografía / Chronic migraine with daily headache. Literature review

INTRODUCCIÓN: A pesar de suponer un número no desdeñable de casos, los pacientes con migraña crónica y cefalea diaria son frecuentemente excluidos de los grandes ensayos clínicos terapéuticos. Estos individuos apenas han sido estudiados en la bibliografía y podrían presentar características específicas. DESARROLLO: Un tercio de los pacientes con migraña crónica podría sufrir cefalea todos los días del mes, lo que supone hasta un 1,7-3,3% de los pacientes atendidos en una consulta de neurología general. Al ser excluidos de la mayor parte de los estudios, apenas hay información sobre sus características clínicas y respuesta al tratamiento, y pueden tener una historia de migraña más prolongada y una diferente respuesta al tratamiento. Los pacientes con migraña crónica y cefalea diaria podrían presentar mecanismos fisiopatológicos complejos que favorezcan la manifestación diaria de la migraña. El manejo de estos pacientes es un reto terapéutico, y se ha propuesto la utilidad de la onabotulinumtoxinA. CONCLUSIONES: Los pacientes con migraña crónica y cefalea diaria pueden presentar características clínicas y terapéuticas específicas. Nuevos estudios podrían llevar a plantear su consideración diferenciada de la migraña crónica

INTRODUCTION: Despite representing a significant number of cases, patients with chronic migraine and daily headache are frequently excluded from large therapeutic clinical trials. These individuals have hardly been studied and could have specific properties. Body. A third of patients with chronic migraine may suffer from headaches every day, representing up to 1.7 - 3.3% of patients in a general neurology consultation. These patients are excluded from most studies, so little information is available. They may have a longer lasting migraine and different response to treatment. Patients with chronic migraine and daily headache may have complex pathophysiological mechanisms that favor the daily manifestation of migraine. The management of these patients is a therapeutic challenge, and OnabotulinumtoxinA may be useful. CONCLUSION: Patients with chronic migraine and daily headache may have specific clinical and therapeutic characteristics. New studies could lead to differentiate it from chronic migraine

Chronic Cluster Headache Update and East-West Comparisons: Focusing on Clinical Features, Pathophysiology, and Management.

PURPOSE OF REVIEW: This review provides an update on chronic cluster headache (CH) focusing on clinical features, pathophysiology, and management as well as comparisons between Eastern and Western populations. RECENT FINDINGS: Chronic CH in Eastern populations was relatively rare, compared to that in Western populations. Lacrimation and/or conjunctival injection is the most frequently reported cranial autonomic symptom, and visual aura is predominant in chronic CH patients. Neuroimaging evidence in both ethnic groups suggests that CH pathophysiology involves the hypothalamus and pain-modulatory areas, with dynamic alternations between CH episodes. Recent evidence indicates that midbrain dopaminergic systems may participate in CH chronicity. Noteworthy advances have emerged in neuromodulatory therapies for chronic CH, but treatment with calcitonin gene-related peptide (CGRP) monoclonal antibodies has been unsuccessful. Recent evidence shows divergence of chronic CH between Eastern and Western populations. Neuromodulatory therapies but not CGRP inhibition is effective in this intractable patient group.

Brain Metabolism and Structure in Chronic Migraine.

PURPOSE OF REVIEW: The purpose of this paper is to review and synthesize current literature in which neurochemical and structural brain imaging were used to investigate chronic migraine (CM) pathophysiology and to further discuss the clinical implications. RECENT FINDINGS: Spectroscopic and structural MRI studies have shown the presence of both impaired metabolism and structural alterations in the brain of CM patients. Metabolic changes in key brain regions support the notion of altered energetics and homeostasis as part of CM pathophysiology. Furthermore, CM, like other chronic pain disorders, may undergo structural reorganization in pain-related brain regions following near persistent endogenous painful input. Finally, both imaging techniques may provide potential biomarkers of disease state and progression and may help guide novel therapeutic interventions or strategies. Spectroscopic and structural MRI have revealed novel aspects of CM pathophysiology. Findings from the former support the metabolic theory of migraine pathogenesis.

Understanding the most commonly billed diagnoses in primary care: Headache disorders.

This article, part of a series about the most commonly billed diagnoses in primary care, describes the pathophysiologic processes of the three most common types of headache disorders as well as how the clinical manifestations and treatments are linked to the pathogenesis of the disorders.

Fear, Avoidance, and Disability in Headache Disorders.

PURPOSE OF REVIEW: The purpose of this review is to summarize the role of avoidance behavior in headache-related disability and overview relevant clinical implications. RECENT FINDINGS: Avoidance occupies a central role in contemporary psychological perspectives on headache disorders and other chronic pain conditions. Several cognitive constructs of relevance to headache are influenced and maintained by avoidance behavior. A growing body of literature attests to the notion that avoidance of headache triggers, of stimuli that exacerbate headache, and of broader life domains can negatively affect headache progression, disability/quality of life, and comorbid psychiatric symptoms. Interventions targeting avoidance behavior, such as therapeutic exposure to headache triggers, mindfulness, and acceptance and commitment therapy (ACT), hold promise for headache disorders but need to be tested in larger trials. Researchers and clinicians are encouraged to attend to functional impairment as a critically important treatment outcome. Comprehensive understanding of headache disorders necessitates attention not merely to diagnostic symptoms and their reduction, but to patterns of avoidance behavior that inadvertently exacerbate headache and contribute to functional impairment.

Parkinson's Disease and Headaches: A Cross-Sectional Study.

OBJECTIVES: This study evaluated headaches among Parkinson's disease (PD) patients and whether there was any correlation between the motor symptoms and the severity of the headaches presented. BACKGROUND: Forty to 83% of PD patients are affected by pain. Despite this high prevalence of pain, only a few studies have addressed the headaches of these patients. METHODS: This was a cross-sectional study. Consecutive patients with Parkinson disease were included. Semi-structured interview; the Epworth sleepiness scale; the Hospital Anxiety and Depression Scale; the Unified Parkinson's Disease Rating Scale (UPDRS), Part III (motor examination); and the Hoehn and Yahr scale were used. RESULTS: About 46 patients were included, 52% were men, mean age was 66 ± 11 years. Forty-three patients had headaches, 12/46 (26%), migraines, 31/46 (67%) had tension-type headaches. We found no association between the headache frequency (median: 0.5; 0.5 to 7.5 vs 0.5; 0.5 to 8 days/3 months; P = .757) or intensity (median: 5; 4 to 8 vs 5.5, 4 to 9; P = .514) and the different stages of the PD (Hoehn and Yahr scale: ≤2.5 vs >2.5). There was no correlation between UPDRS score and the intensity (r = -0.099; P = .530) or frequency of headaches (r = -0.136; P = .373). No association was found between the grade of neck stiffness (0 vs 1 and 2 vs 3 and 4) and the headache frequency (Median: 0; 0 to 3 vs 3.5; 0 to 12.5 vs 0; 0 to 6 days/3 months; P = .074) or intensity (Median: 5; 3 to 9 vs 5; 4 to 6 vs 7; 4.5 to 9; P = .434). Twelve patients said that their headaches started after PD had been diagnosed. There was no difference regarding the frequency and characteristics of headaches and PD characteristics between these patients and the other patients with previous headaches. CONCLUSIONS: In this sample of PD patients, there is no association between headache and PD.

Chronic orofacial pain.

While pain chronicity in general has been defined as pain lasting for more than 3 months, this definition is not useful in orofacial pain (OFP) and headache (HA). Instead, chronicity in OFP and HA is defined as pain occurring on more than 15 days per month and lasting for more than 4 h daily for at least the last 3 months. This definition excludes the periodic shortlasting pains that often recur in the face and head, but are not essentially chronic. Although the headache field has adopted this definition, chronic orofacial pain is still poorly defined. In this article, we discuss current thinking of chronicity in pain and examine the term 'chronic orofacial pain' (COFP). We discuss the entities that make up COFP and analyze the term's usefulness in clinical practice and epidemiology.

The Day My Life Changed: A Qualitative Study of the Experiences of Patients With New Daily Persistent Headache.

OBJECTIVES: This study aimed to explore the views and experiences of a group of Spanish patients suffering from new daily persistent headache (NDPH). METHODS: A qualitative descriptive study was conducted with patients diagnosed with NDPH. Purposeful sampling was performed among patients attending a specialized Headache Unit at 2 university hospitals between February 2017 and December 2018. In total, 18 patients (11 women, 7 men; mean age 45.3, standard deviation 10.6) with a median duration of illness of 70 months (interquartile range, 24-219) were recruited to this study. Data were collected through in-depth interviews, researchers' field notes and patients' drawings. Thematic analysis was used to identify emerging themes. RESULTS: Three main themes were identified: (1) the origin of the illness and seeking answers; (2) characteristics of the pain; and (3) the impact of pain on patients' lives. The patients precisely recalled the time of onset and the trigger of the pain. Pain was constantly present, although it varied in form. At the onset, pain was perceived as a sign of alarm while, over time, it became an invisible illness. The headache commonly had a major impact on everyday life and could cause lifestyle changes. In addition, pain could be emotionally disruptive and could also lead to family estrangement and a search for solitude. CONCLUSIONS: Our results provide insight into how NDPH is experienced, which may be helpful in managing NDPH patients. In our cohort, patients identified precipitating events but sought answers regarding the origin of their illness and their pain. Pain was a continuous sensation that had a major impact on patients' daily lives and emotions.

Meningeal rheumatoid nodules in a 55-year-old man presenting with chronic headaches and oculomotor nerve palsy: an uncommon extra-articular manifestation of rheumatoid arthritis.

Rheumatoid arthritis (RA) is a multisystem inflammatory disease which can involve many organ systems including the central nervous system (CNS). Though not very common, the results can be severely debilitating. The spectrum of the CNS involvement includes meningitis, encephalitis and occasionally rheumatoid nodules. Its presentation is variable, though very rarely it can present as focal neurological deficits. Imaging can be suggestive, but diagnosis usually requires tissue biopsy. Treatment consists of high-dose steroids and immunosuppressants. We describe the case of a 55-year-old male patient with a history of RA presenting with a third nerve palsy and headache who was found to have rheumatoid nodules on biopsy. CNS involvement in RA should be considered in anyone with rheumatoid arthritis who presents with focal neurological deficits, though infections and space-occupying lesions should also be ruled out.

Prevalence and burden of headache in children and adolescents in Austria - a nationwide study in a representative sample of pupils aged 10-18 years.

BACKGROUND: Headache disorders are highly prevalent worldwide, but not so well investigated in children and adolescents as in adults: few studies have included representative nationwide samples. No data exist for Austria until now. In a representative sample of children and adolescents in Austria, we estimated the prevalence and attributable burden of headache disorders, including the new diagnostic category of "undifferentiated headache" (UdH) defined as mild headache lasting less than 1 hour. METHODS: Within the context of a broader national mental health survey, children and adolescents aged 10-18 years were recruited from purposively selected schools. Mediated self-completed questionnaires included sociodemographic enquiry (gender, age, socioeconomic status, family constellation, residence [urban or rural] and migration background). Prevalence and attributable burden of all headache, UdH, migraine (definite plus probable), tension-type headache (TTH: definite plus probable) and headache on ≥15 days/month (H15+) were assessed using the Headache-Attributed Restriction, Disability, Social Handicap and Impaired Participation (HARDSHIP) questionnaire for children and adolescents. Health-related quality of life (HrQoL) was assessed using the KIDSCREEN questionnaire. RESULTS: Of 7643 selected pupils, 3386 (44.3%) completed the questionnaires. The 1-year prevalence of headache was 75.7%, increasing with age and higher in girls (82.1%) than in boys (67.7%; p < 0.001). UdH, migraine, TTH and H15+ were reported by 26.1%, 24.2%, 21.6% and 3.0% of participants. Attributable burden was high, with 42% of those with headache experiencing restrictions in daily activities. Medication use (50% overall) was highest in H15+ (67%) and still considerable in UdH (29%). HrQoL was reduced for all headache types except UdH. Participants in single parent or patchwork families had a higher probability of migraine (respectively, OR 1.5, p < 0.001; OR 1.5, p < 0.01). Participants with a migration background had a lower probability of TTH (OR 0.7, p < 0.01). CONCLUSIONS: Headache disorders are both very common and highly burdensome in children and adolescents in Austria. This study contributes to the global atlas of headache disorders in these age groups, and corroborates and adds knowledge of the new yet common and important diagnostic category of UdH. The findings call for action in national and international health policies, and for further epidemiological research.

Safety and efficacy of sphenopalatine ganglion stimulation for chronic cluster headache: a double-blind, randomised controlled trial.

BACKGROUND: Chronic cluster headache is the most disabling form of cluster headache. The mainstay of treatment is attack prevention, but the available management options have little efficacy and are associated with substantial side-effects. In this study, we aimed to assess the safety and efficacy of sphenopalatine ganglion stimulation for treatment of chronic cluster headache. METHODS: We did a randomised, sham-controlled, parallel group, double-blind, safety and efficacy study at 21 headache centres in the USA. We recruited patients aged 22 years or older with chronic cluster headache, who reported a minimum of four cluster headache attacks per week that were unsuccessfully controlled by preventive treatments. Participants were randomly assigned (1:1) via an online adaptive randomisation procedure to either stimulation of the sphenopalatine ganglion or a sham control that delivered a cutaneous electrical stimulation. Patients and the clinical evaluator and surgeon were masked to group assignment. The primary efficacy endpoint, which was analysed with weighted generalised estimated equation logistic regression models, was the difference between groups in the proportion of stimulation-treated ipsilateral cluster attacks for which relief from pain was achieved 15 min after the start of stimulation without the use of acute drugs before that timepoint. Efficacy analyses were done in all patients who were implanted with a device and provided data for at least one treated attack during the 4-week experimental phase. Safety was assessed in all patients undergoing an implantation procedure up to the end of the open-label phase of the study, which followed the experimental phase. This trial is registered with ClinicalTrials.gov, number NCT02168764. FINDINGS: Between July 9, 2014, and Feb 14, 2017, 93 patients were enrolled and randomly assigned, 45 to the sphenopalatine ganglion stimulation group and 48 to the control group. 36 patients in the sphenopalatine ganglion stimulation group and 40 in the control group had at least one attack during the experimental phase and were included in efficacy analyses. The proportion of attacks for which pain relief was experienced at 15 min was 62·46% (95% CI 49·15-74·12) in the sphenopalatine ganglion stimulation group versus 38·87% (28·60-50·25) in the control group (odds ratio 2·62 [95% CI 1·28-5·34]; p=0·008). Nine serious adverse events were reported by the end of the open-label phase. Three of these serious adverse events were related to the implantation procedure (aspiration during intubation, nausea and vomiting, and venous injury or compromise). A fourth serious adverse event was an infection that was attributed to both the stimulation device and the implantation procedure. The other five serious adverse events were unrelated. There were no unanticipated serious adverse events. INTERPRETATION: Sphenopalatine ganglion stimulation seems efficacious and is well tolerated, and potentially offers an alternative approach to the treatment of chronic cluster headache. Further research is need to clarify its place in clinical practice. FUNDING: Autonomic Technologies.

Headache Outcomes After Treatment of Unruptured Intracranial Aneurysm: A Systematic Review and Meta-Analysis.

Background and Purpose- While unruptured intracranial aneurysms may be discovered incidentally in the workup of chronic headache, it remains unclear whether their treatment ultimately impacts headache severity. We aim to conduct a systematic review and meta-analysis assessing headache severity after treatment of unruptured intracranial aneurysm. Methods- MEDLINE and EMBASE were systematically reviewed. Results- Data from 7 studies met inclusion criteria (309 nonduplicated patients). The standard mean difference in pre- and post-intervention headache severity was estimated at -0.448 (95% CI, -0.566 to -0.329) under a random effects model. No significant heterogeneity was noted nor was any significant publication bias demonstrated. Conclusions- This is the first systematic review assessing postoperative headache severity following treatment of unruptured intracranial aneurysm. While a significant reduction in headache severity was observed, further investigation into this phenomenon is recommended before it influences clinical practice. Future study should stratify headache outcomes by aneurysm size, location, and treatment modality.

LASH syndrome: New cases with a broadening clinical phenotype.

OBJECTIVE: To describe three new cases of the headache syndrome of long-lasting autonomic symptoms with hemicrania (LASH), and to establish a clinical phenotype utilizing all LASH cases noted in the medical literature. METHODS: A case series of patients was evaluated in an academic headache clinic over a two-year time period. LASH syndrome was defined by episodic headache attacks with associated cranial autonomic symptoms that start before headache onset, last the entire duration of the headache and continue on for a period of time after the headache ceases. RESULTS: Three patients were noted to have LASH syndrome in a two-year time period (2017-2018). One patient was diagnosed with primary LASH, while two others had probable secondary LASH from a secretory pituitary tumor. The primary LASH patient was female. She had on average one headache per week lasting 1-3 days in duration. She experienced migrainous associated symptoms along with their cranial autonomic symptoms. She also developed a fixed Horner's syndrome along with a typical headache attack, which was present for 6 months at the time of consultation. She had complete headache relief with indomethacin and her miosis and ptosis also resolved with treatment. Secondary LASH was diagnosed in two patients (one male, one female) both with prolactin secreting pituitary microadenomas. One of the patients had his headaches abolish with dopamine agonist therapy while the other patient did not respond to hormonal modulation but became pain free on indomethacin. Secondary LASH patients had less frequent headache episodes and lacked any migrainous associated features, but exhibited agitation with headache. CONCLUSION: LASH syndrome may be rare, but more reported cases are entering the headache literature. The temporal profile of onset and offset of cranial autonomic symptoms is key to making the diagnosis. Primary and secondary LASH may present differently based on gender predominance, the presence of migrainous associated features, and attack frequency. Secondary LASH appears to be indomethacin responsive, suggesting that medication effectiveness should not obviate the need to do testing for secondary etiologies.

New daily persistent headache: a systematic review on an enigmatic disorder.

BACKGROUND: New daily persistent headache (NDPH) presents with a sudden onset headache which continues without remission within 24 h. Although rare, NDPH is important because it is one of the most treatment refractory primary headache disorders and can be highly disabling to the individuals. In this structured review, we describe the current knowledge of epidemiology, clinical features, trigger factors, pathophysiology, diagnosis and therapeutic options of NDPH to better understand this enigmatic disorder. The prevalence of NDPH estimated to be 0.03% to 0.1% in the general population and is higher in children and adolescents than in adults. Individuals with NDPH can pinpoint the exact date their headache started. The pain is constant and lacks special characteristics but in some has migraine features. The exact pathogenic mechanism of NDPH is unknown, however pro-inflammatory cytokines and cervicogenic problems might play a role in its development. The diagnosis of NDPH is mainly clinical and based on a typical history, but proper laboratory investigation is needed to exclude secondary causes of headache. Regarding treatment strategy, controlled drug trials are absent. It is probably best to treat NDPH based upon the predominant headache phenotype. For patients who do not respond to common prophylactic drugs, ketamine infusion, onabotulinum toxin type A, intravenous (IV) lidocaine, IV methylprednisolone and nerve blockade are possible treatment options, but even aggressive treatment is usually ineffective. CONCLUSION: NDPH remains poorly understood but very burdensome for the individual. Multi-center randomized controlled trials are recommended to gain better understanding of NDPH and to establish evidence based treatments.