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Background Face-to-face procedures have been postponed during COVID-19 pandemic. We aim to evaluate the impact of onabotulinumtoxinA follow-up delay in migraine during COVID-19 pandemic. Methods Subjective worsening, intensity of migraine attacks, and frequency of headache and migraine were retrospectively compared between patients with unmodified and interrupted onabotulinumtoxinA follow-up in Headache Units. Results We included 67 patients with chronic migraine or high-frequency episodic migraine under onabotulinumtoxinA treatment, 65 (97.0%) female, 44.5 ± 12.1 years old. Treatment administration was voluntarily delayed in 14 (20.9%) patients and nine (13.4%) were unable to continue follow-up. Patients with uninterrupted follow-up during lockdown presented 7.6 and 8.1 less monthly days with headache (adjusted p = 0.017) and migraine attacks (adjusted p = 0.009) compared to patients whose follow-up was interrupted, respectively. Conclusion Involuntary delay of onabotulinumtoxinA follow-up in patients with migraine due to COVID-19 pandemic was associated with a higher frequency of headache and migraine attacks. Safe administration of onabotulinumtoxinA during lockdown should be promoted. Supplementary Information The online version contains supplementary material available at 10.1007/s10072-021-05180-8.

To assess the characteristics of the management of patients with migraine who present to the emergency department (ED) with a migraine attack. Retrospective, observational study analyzing demographic, clinical, diagnostic and therapeutic characteristics of patients with migraine diagnosis presenting to ED for a migraine attack between 2016 and 2019. We reviewed the clinical records of 847 cases. 82.2% were women with mean age of 34.9 years. 87.2% had episodic migraine and 12.2% chronic migraine. 62.3% (528/847) had taken analgesics before visiting the ED [non-steroidal-anti-inflammatory drugs (NSAIDs) (300/528; 56.9%) and triptans (261/528; 49.5%)]. 25.4% (215/847) received blood testing and 6.4% (55/847) received cranial CT. Medication was administered in 77.2% cases (654/847). The median time-to-treatment was 70 min (IQR 42-120). NSAIDs (81%, 530/654), antiemetics (43.1%, 282/654) and metamizole (39% 255/654) were the most used. Triptans were administered in 7 cases (1.1%) and opioids in 84 (12.8%). At discharge, preventive treatment was prescribed or modified in 8.2% of cases (69/839) and triptans were prescribed in 129 cases (15.3%). 70.5% (592/839) were instructed to follow-up with their primary care provider (PCP), 21.5% (181/839) with a general neurologist and 7.9% (66/839) with a headache specialist. The majority of migraine patients were not receiving the recommended acute migraine-specific medication, both in the outpatient and in the ED setting, being especially remarkable the rare use of triptans in the ED. Furthermore, we found an elevated use of urgent complementary tests, mainly blood tests.

Abiotrophia defectiva is a rare cause of endocarditis with a high embolization rate compared to other streptococcal endocarditis. Neurological complications may occur in 20-40% of cases, and include intracranial aneurysms and sub-arachnoid hemorrhage. Its isolation in the central nervous system (CNS) is extremely rare and is associated with previous neurosurgical procedures. We present the case of a 51-year-old patient who suffered an ischemic stroke as a complication of A. defectiva endocarditis, and the cases of CNS infections caused by this microorganism are reviewed. The relevance of the case lies in its singular character, due to the absence of previous valve disease in the patient without a recent history of invasive procedures or surgical interventions.

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

Introduction: When a patient is diagnosed with primary headache or craniofacial neuralgia in the emergency department or in primary care, and is referred to a neurologist due to the complexity of the case, it is useful to know whether additional examination should be sought and the priority (urgent, preferential, or normal) with which the patient should be seen. This will avoid unnecessary delays in patients with disabling headache and where organic causes are suspected.In order to issue recommendations on this matter, the Spanish Society of Neurology's Headache Study Group has decided to create a series of agreed recommendations constituting a referral protocol for patients with headache and/or craniofacial neuralgia. Development: Young neurologists with an interest and experience in headache were invited to draft a series of practical guidelines in collaboration with the Spanish Society of Neurology's Headache Study Group Executive Committee. For practical reasons, the document was divided into 2 articles: this first article focuses on primary headaches and craniofacial neuralgias and the second on secondary headaches. In order for the recommendations to be helpful for daily practice they follow a practical approach, with tables summarising referral criteria, examinations to be performed, and referral to other specialists. Conclusions: We hope to offer a guide and tools to improve decision-making regarding patients with headache, identifying complementary tests to prioritise and referral pathways to be followed, in order to avoid duplicated consultations and delayed diagnosis and treatment. Resumen: Introducción: Cuando tras una correcta anamnesis y exploración neurológica se diagnostica a un paciente con una cefalea primaria o una neuralgia craneofacial en urgencias o atención primaria y se decide derivar a neurología por complejidad es útil conocer si además se deberían solicitar exploraciones complementarias y la preferencia (urgente, preferente o normal) con la que se debería derivar para que el paciente llegue a la consulta del neurólogo sin demoras innecesarias en pacientes con dolores incapacitantes o con sospecha de organicidad.Por este motivo, el Grupo de Estudio de Cefalea de la Sociedad Española de Neurología, ha decidido crear unas recomendaciones consensuadas que establezcan un protocolo de derivación de pacientes con cefalea y/o neuralgias craneofaciales. Desarrollo: Se ha contactado con neurólogos jóvenes con interés y experiencia en cefalea y con la Junta Directiva del Grupo de Estudio de Cefalea de la Sociedad Española de Neurología que han desarrollado este documento que, por razones prácticas, se ha dividido en 2 artículos. Esta primera centrada en las cefaleas o neuralgias craneofaciales primarias y una segunda que se focaliza en las cefaleas secundarias. El enfoque es práctico con tablas que resumen los criterios de derivación con exploraciones complementarias y otros especialistas a los que derivar, para que sea útil y facilite su uso en nuestra práctica asistencial diaria. Conclusiones: Esperamos ofrecer una guía y herramientas para mejorar la toma de decisiones ante un paciente con cefalea, valorando exploraciones a priorizar y qué circuitos seguir para así evitar la duplicación de consultas y retrasos en el diagnóstico y en el tratamiento.

Background Erenumab was approved in Europe for migraine prevention in patients with ≥4 monthly migraine days (MMD). In Spain, Novartis started a personalized managed access program which allowed free access to erenumab before official reimbursement. The Headache Study Group of the Spanish Neurological Society started a registry to monitor real-world safety and efficacy, and all Spanish headache experts were invited to participate. Methods Patients fulfilled ICHD3 criteria for migraine and had ≥ 4MMD. Sociodemographic and clinical data were registered as well as MMD, headache frequency (MHD), prior and concomitant preventive treatment, medication overuse headache (MOH), migraine evolution, adverse events, and PROs: HIT6, MIDAS, and PGIC. A >50% reduction of MMD after 3 months was considered as response. Results We included 210 patients (female 86.7%, mean age 46.4 years old) from 22 hospitals from February 2019 – to – June 2020. Most patients (89.5%) suffered from chronic migraine with a mean evolution of 8.6 years. MOH was present in 70% of patients, and 17.1% had migraine with aura. Average of prior preventive treatment failure was >7 (BoNT/A had been used by 95.2%). Most patients (67,6%) started with erenumab 70mg. 61% of patients were also taking oral preventive drugs or getting simultaneous BoNT/A (27.6%). Responder rate was 37.1% and the mean reduction of MMD was -6.28 and MHD: -8.6. Regarding PROs: MIDAS: -35 p., HIT6: -11.6 p., PIGC: 4.7 p. Predictors of good response were: HIT6 score ( p =0.01), prior preventive treatment failures ( p =0.026), absence of MOH ( p =0.039), and simultaneous BoNT/A treatment ( p

Background and objective: CGRP, a neuropeptide involved in migraine pathophysiology, is also known to play a role in the respiratory system and in immunological conditions such as sepsis. We analyzed the impact of the use of CGRP antagonists in patients with migraine during the COVID-19 pandemic, caused by the SARS-CoV-2 coronavirus. Methods: This is a multicentre cross-sectional study. From May to November 2020, through a national survey distributed by the Spanish Society of Neurology, we collected data about the presence of COVID-19 symptoms including headache and their characteristics and severity in patients with migraine treated with anti-CGRP monoclonal antibodies (mAb), and compared them with patients with migraine not receiving this treatment. We also conducted a subanalysis of patients with COVID-19 symptoms. Results: We recruited 300 patients with migraine: 51.7% (155/300) were taking anti-CGRP mAbs; 87.3% were women (262/300). Mean age (standard deviation) was 47.1 years (11.6). Forty-one patients (13.7%) met diagnostic criteria for COVID-19, with no statistically significant difference between patients with and without anti-CGRP mAb treatment (16.1% vs 11.0%, respectively; P = .320). Of the patients with COVID-19, 48.8% (20/41) visited the emergency department and 12.2% (5/41) were hospitalised. Likewise, no clinical differences were found between the groups of patients with and without anti-CGRP mAb treatment. Conclusion: Anti-CGRP mAbs may be safe in clinical practice, presenting no association with increased risk of COVID-19. Resumen: Antecedentes y objetivo: El péptido relacionado con el gen de la calcitonina (CGRP, por sus siglas en inglés), es un neuropéptido involucrado en la fisiopatología de la migraña, que también es conocido por participar en la regulación del sistema respiratorio y en algunas enfermedades inmunológicas como la sepsis. Hemos analizado el impacto del uso de los antagonistas de CGRP en pacientes con migraña durante la pandemia de COVID-19, causada por el coronavirus SARS-CoV-2. Métodos: Estudio transversal multicéntrico desarrollado entre mayo y noviembre de 2020, en el que la Sociedad Española de Neurología distribuyó a nivel nacional una encuesta de la que recogimos datos sobre la presencia, las características y la gravedad de síntomas de COVID-19, entre los que se encontraba la cefalea, en pacientes con migraña tratados con anticuerpos monoclonales (AcM) anti-CGRP, y los comparamos con los de pacientes con migraña que no recibían dicho tratamiento. También realizamos un subanálisis de los pacientes con síntomas de COVID-19. Resultados: Identificamos 300 pacientes con migraña: 51,7% (155/300) recibían AcM anti-CGRP; el 87,3% eran mujeres (262/300) y la edad media (desviación estándar) de la muestra fue de 47,1 (11,6) años. Un total de 41 pacientes (13,7%) cumplían los criterios diagnósticos de COVID-19, sin diferencias estadísticamente significativas entre los pacientes que recibían tratamiento con AcM anti-CGRP y los que no (16,1% y 11,0%, respectivamente; p = 0,320). De los pacientes con COVID-19, el 48,8% (20/41) acudieron a urgencias y el 12,2% (5/41) fueron hospitalizados. Igualmente, no se detectaron diferencias clínicas entre los pacientes que recibían dicho tratamiento y los que no. Conclusión: El tratamiento con AcM anti-CGRP parece un recurso seguro en la práctica clínica, y no se asocia a un mayor riesgo de COVID-19.

Objective Previous studies have demonstrated that emotional stress, changes in lifestyle habits and infections can worsen the clinical course of migraine. We hypothesize that changes in habits and medical care during coronavirus disease 2019 (COVID-19) lockdown might have worsened the clinical course of migraine. Design Retrospective survey study collecting online responses from migraine patients followed-up by neurologists at three tertiary hospitals between June and July 2020. Methods We used a web-based survey that included demographic data, clinical variables related with any headache (frequency) and migraine (subjective worsening, frequency, and intensity), lockdown, and symptoms of post-traumatic stress. Results The response rate of the survey was 239/324 (73.8%). The final analysis included 222 subjects. Among them, 201/222 (90.5%) were women, aged 42.5 ± 12.0 (mean±SD). Subjective improvement of migraine during lockdown was reported in 31/222 participants (14.0%), while worsening in 105/222 (47.3%) and was associated with changes in migraine triggers such as stress related to going outdoors and intake of specific foods or drinks. Intensity of attacks increased in 67/222 patients (30.2%), and it was associated with the subjective worsening, female sex, recent insomnia, and use of acute medication during a headache. An increase in monthly days with any headache was observed in 105/222 patients (47.3%) and was related to symptoms of post-traumatic stress, older age and living with five or more people. Conclusions Approximately half the migraine patients reported worsening of their usual pain during the lockdown. Worse clinical course in migraine patients was related to changes in triggers and the emotional impact of the lockdown.

Tratamiento preventivo; Cefalea con uso excesivo de medicación; Opioides Tractament preventiu; Cefalea per ús excessiu de medicaments; Opioides Preventive treatment; Medication overuse headache; Opioids Introducción La cefalea con uso excesivo de medicación es una cefalea secundaria en la que el uso regular o frecuente de medicación analgésica produce un aumento de la frecuencia de una cefalea de base, pasando de episódica a crónica. La prevalencia de esta entidad está en torno al 1-2%, siendo más frecuente en mujeres entre 30 y 50 años con comorbilidades psiquiátricas como depresión o ansiedad y otros procesos de dolor crónico. Es importante conocer el manejo de esta entidad. Por este motivo, el Grupo de Estudios de Cefaleas de la Sociedad Española de Neurología ha pretendido realizar este documento de consenso sobre esta patología. Desarrollo Esta guía ha sido redactada por un grupo de expertos a partir de la revisión de la evidencia científica publicada y estableciendo recomendaciones prácticas para su adecuado manejo y tratamiento. El tratamiento de la cefalea con uso excesivo de medicación tiene varios pilares fundamentales y suele ser complejo: información y educación sobre el desarrollo de la cefalea con uso excesivo de medicación, tratamiento preventivo, suspensión del fármaco de uso frecuente y tratamiento de deshabituación. Es importante el seguimiento de pacientes con riesgo de recurrencias. Conclusiones Esperamos que este documento resulte de utilidad y permita su aplicación práctica en la consulta diaria y que sirva para actualizar y mejorar el conocimiento del manejo de esta patología. Introduction Medication overuse headache is a secondary headache in which the regular or frequent use of analgesics can increase the frequency of the episodes, causing the transition from episodic to chronic headache. The prevalence of medication overuse headache is approximately 1-2%, with higher rates among women aged 30-50 years and with comorbid psychiatric disorders such as depression or anxiety, or other chronic pain disorders. It is important to be familiar with the management of this disease. To this end, the Spanish Society of Neurology's Headache Study Group has prepared a consensus document addressing this disorder. Development These guidelines were prepared by a group of neurologists specialising in headache after a systematic literature review and provides consensus recommendations on the proper management and treatment of medication overuse headache. The treatment of medication overuse headache is often complex, and is based on 4 fundamental pillars: education and information about the condition, preventive treatment, discontinuation of the drug being overused, and treatment for withdrawal symptoms. Follow-up of patients at risk of recurrence is important. Conclusions We hope that this document will be useful in daily clinical practice and that it will update and improve understanding of medication overuse headache management.

Introduction: Numerous invasive and non-invasive neuromodulation devices have been developed and applied to patients with headache and neuralgia in recent years. However, no updated review addresses their safety and efficacy, and no healthcare institution has issued specific recommendations on their use for these 2 conditions. Methods: Neurologists from the Spanish Society of Neurology’s (SEN) Headache Study Group and neurosurgeons specialising in functional neurosurgery, selected by the Spanish Society of Neurosurgery (SENEC), performed a comprehensive review of articles on the MEDLINE database addressing the use of the technique in patients with headache and neuralgia. Results: We present an updated review and establish the first set of consensus recommendations of the SEN and SENEC on the use of neuromodulation to treat headache and neuralgia, analysing the current levels of evidence on its effectiveness for each specific condition. Conclusions: Current evidence supports the indication of neuromodulation techniques for patients with refractory headache and neuralgia (especially migraine, cluster headache, and trigeminal neuralgia) selected by neurologists and headache specialists, after pharmacological treatment options are exhausted. Furthermore, we recommend that invasive neuromodulation be debated by multidisciplinary committees, and that the procedure be performed by teams of neurosurgeons specialising in functional neurosurgery, with acceptable rates of morbidity and mortality. Resumen: Introducción: En los últimos años han surgido numerosos dispositivos de neuromodulación, invasivos y no invasivos, que se han aplicado en pacientes con cefaleas y neuralgias sin que exista una revisión actualizada de su eficacia y seguridad, ni recomendaciones de ninguna institución sanitaria sobre su uso específico en cada entidad nosológica. Métodos: Neurólogos del Grupo de Cefaleas de la Sociedad Española de Neurología (SEN) y neurocirujanos expertos en neurocirugía funcional seleccionados por la Sociedad Española de Neurocirugía (SENEC), hemos realizado una revisión exhaustiva en el sistema Medline sobre neuromodulación en cefaleas y neuralgias. Resultados: Presentamos una revisión actualizada y establecemos por primera vez unas recomendaciones consensuadas entre la SEN y la SENEC sobre el uso de la neuromodulación en cefaleas y neuralgias, adjudicando niveles de evidencia sobre su eficacia actual, específicamente en cada entidad nosológica. Conclusiones: Los resultados actuales de los estudios proporcionan evidencias para la indicación de técnicas de neuromodulación en casos refractarios de cefaleas y neuralgias (sobre todo en migraña, cefalea en racimos y neuralgia del trigémino), seleccionados por neurólogos expertos en cefaleas, tras comprobar el agotamiento de las opciones farmacológicas. Adicionalmente, en el caso de la neuromodulación invasiva, se recomienda que los casos sean debatidos en comités multidisciplinarios y la cirugía sea realizada por equipos de neurocirujanos expertos en neurocirugía funcional y con una morbimortalidad aceptable.

To compare the cost of adverse events (AEs) associated with preventive treatment of migraine with fremanezumab, versus erenumab, galcanezumab, and onabotulinumtoxinA. A probabilistic modeling analy...

BACKGROUND: OnabotulinumtoxinA is an effective preventive treatment for chronic migraine. In chronic migraine, besides a reduction in headache frequency, a decreased reliance on oral prophylactics is also indicative of treatment effectiveness. This study aimed to quantify the change in the use of oral prophylactics after treatment with onabotulinumtoxinA in patients with chronic migraine.; METHODS: This was a retrospective, multicentric, cross-sectional study. Patients with chronic migraine (ICHD-3b) that had been treated with onabotulinumtoxinA were enrolled consecutively. We collected parameters related to each patient's pre-treatment situation, as well as their current one, focusing on frequency and intensity of migraine, number of oral prophylactics and the respective cycle of onabotulinumtoxinA. Univariate and logistic regression analyses were performed.; RESULTS: We included 542 patients. 90.0% were taking oral preventive treatments. During treatment with onabotulinumtoxinA, 47.8% withdrew at least one prophylactic and 41.6% stopped using oral prophylactics altogether. Factors associated with a reduction or cessations of oral prophylactics were: having>50% improvement in frequency and intensity, remission to episodic migraine, the use of topiramate as an initial treatment, an increased number of infiltrations and a shorter chronification period (pfive cycles of onabotulinumtoxinA, >50% improvement in pain intensity and topiramate as an initial treatment were predictors of a reduction in oral prophylactics (AUC of 70.3%; p

BACKGROUND During 24-hour on-call shifts medical residents are exposed to diverse circumstances such as sleep deprivation and stress. OBJECTIVE Our aim is to assess the effect of 24-hour on-call shifts on medical residents' headache-related disability. METHODS The Migraine Disability Assessment Scale (MIDAS), the Headache Impact Test (HIT-6), the Pittsburgh Sleep Quality Index (PSQI), and the Hospital Anxiety and Depression Scale (HADS) questionnaires were administered to medical residents who had never performed on-call shifts at baseline and 6 months after beginning 24-hour on-call shifts. Scores were compared. RESULTS About 66 medical residents completed this study. About 21.2% (n = 14) had history of migraine, 42.4% (n = 28) had a history of tension-type headache (TTH) and 12.1% (n = 8) had a history of both migraine and TTH. Among medical residents with migraine, the median MIDAS score was significantly higher after starting 24-hour on-call shifts than at a baseline (4.0 vs 8.0; Wilcoxon, P = .001), meaning that, on average, disability increased from little or no disability, to moderate disability. No difference in HIT-6 scores was found. The median score of PSQI and HADS was higher at 6 months (PSQI: 7.0 vs 8.0; P = .003), (HADS: 5.0 vs 8.0; P

Background and purpose: Migraine is a common and costly neurological disorder. The aims of this study were to quantify the costs of chronic (CM) and episodic migraine (EM) in Spain, evaluating the impact of psychiatric comorbidities and disability, and to estimate the economic savings associated with reducing the number of migraine-days by 50%. Methods: This was an observational, cross-sectional analysis of data from migraine patients who participated in the Spanish Migraine Atlas. The participants were invited to complete a structured questionnaire including the following scales: the Headache Needs Assessment, the Hospital Anxiety and Depression Scale, and the Migraine Disability Assessment Scale (MIDAS). Results: A total of 475 patients were included, of whom 187 had CM (39.4%). Total costs per patient/year were: €16 578.2 ± €34 568.1 for CM and €6227.8 ± €6515.7 for EM. A higher degree of disability, according to MIDAS, significantly increased the total cost of migraine, while the presence of psychiatric comorbidity increased costs for EM patients only. A reduction of 1 migraine-day per month decreased average total costs by €744.14 per patient/year for EM and €663.20 per patient/year for CM, while a reduction in the number of migraine-days by 50% would result in economic savings of €2232.44 per patient/year (R2 = 0.927) for EM and €6631.99 per patient/year (R2 = 0.886) for CM. Conclusions: The costs associated with migraine were driven by migraine frequency and the degree of disability, whereas psychiatric comorbidity only influenced the cost of EM. These results highlight the need to optimize migraine management to reduce the economic migraine burden. Future studies are needed to confirm our results.

Resumen: Introducción: En el campo de las cefaleas, onabotulinumtoxinA (onabotA) tiene indicación bien establecida en la migraña crónica (MC). Además, en los últimos años su uso se está extendiendo a otras cefaleas primarias (migraña episódica de alta frecuencia, cefaleas trigémino-autonómicas, cefalea numular) y a la neuralgia del trigémino. Al ser una opción terapéutica que se va a ir incorporando de forma progresiva en el manejo de estas entidades, creemos que es necesario reflejar con un carácter eminentemente práctico cuáles son las posibles indicaciones de onabotA, más allá de la MC, así como su protocolo de administración, que diferirá en función del tipo de cefalea y/o neuralgia. Desarrollo: A partir de una revisión de la bibliografía existente y de nuestra propia experiencia clínica, se ha elaborado este documento de consenso cuyo objetivo es servir de guía a aquellos profesionales que quieran aplicar estas técnicas en su actividad asistencial. En la primera parte se abordará el mecanismo de acción de onabotA y la razón de su utilización en diversas cefaleas distintas de la MC desde un punto de vista fisiopatológico y clínico. En la segunda parte se hará una revisión de la evidencia disponible y los estudios publicados en los últimos años. Para cada una de estas entidades, se añadirá una «recomendación de experto», basada en la propia experiencia clínica, que refleje el perfil de paciente que puede ser candidato a este tratamiento, las dosis y el protocolo de administración de onabotA. Conclusión: El tratamiento con onabotA en entidades distintas a la MC debe ser siempre individualizado y se planteará en pacientes seleccionados que no hayan respondido a la terapia convencional. Abstract: Introduction: In the field of headaches, onabotulinumtoxinA (onabotA) is well established as a treatment for chronic migraine (CM). In recent years, it has been used increasingly to treat other primary headaches (high-frequency episodic migraine, trigeminal-autonomic cephalalgias, nummular headache) and trigeminal neuralgia. As this treatment will progressively be incorporated in the management of these patients, we consider it necessary to reflect, with a fundamentally practical approach, on the possible indications of onabotA, beyond CM, as well as its administration protocol, which will differ according to the type of headache and/or neuralgia. Development: This consensus document was drafted based on a thorough review and analysis of the existing literature and our own clinical experience. The aim of the document is to serve as guidelines for professionals administering onabotA treatment. The first part will address onabotA's mechanism of action, and reasons for its use in other types of headache, from a physiopathological and clinical perspective. In the second part, we will review the available evidence and studies published in recent years. We will add an “expert recommendation” based on our own clinical experience, showing the best patient profile for this treatment and the most adequate dose and administration protocol. Conclusion: Treatment with onabotA should always be individualised and considered in selected patients who have not responded to conventional therapy.

Background Cluster headache is one of the most disabling of all headache conditions. Although some studies have investigated the psychological profile of patients with cluster headache, research on its impact on cognitive function in patients with episodic cluster headache outside the cluster bout is scant. Methods Cross-sectional study to evaluate various aspects of neuropsychological assessment and cognitive function including working memory, selective attention, verbal fluency, and executive function in 40 patients with episodic cluster headache. The patients were compared with 40 age-, gender-, and level of education-matched healthy controls. Results Episodic cluster headache patients performed significantly worse than healthy controls on all cognitive tests, except for the Interference Score (P = 0.281). They had significantly higher Hospital Anxiety Scale scores (P = 0.002). However, we found no significant association between cognitive performance, anxiety, sleep quality, and disease duration. Conclusions Patients with episodic cluster headache outside the bout showed worse executive functioning, working memory, language, and selective attention compared with healthy controls, regardless of the duration of disease or sleep quality.

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

Neurological involvement in Behcet's disease is rare, especially at the onset. It can present in the form of parenchymal changes or as damage to the vascular structures in its nonparenchymal form. The coexistence of both kinds of manifestations in the same patient is exceptional. We report the case of a 32-year-old patient with a history of deep venous thrombosis, who was being treated for holocranial headache, apathy, and oral and genital ulcers. Brain magnetic resonance imaging showed hyperintense lesions in the basal ganglia and white matter, and the vascular study evidenced venous thrombosis of the left transverse sinus. After confirming the diagnosis of Behcet's disease with parenchymal and nonparenchymal cerebral involvement, immunosuppressive and corticosteroid therapy was started, resulting in the remission of the symptoms.

Sleep and circadian rhythm disturbances are common in patients with neurodegenerative diseases such as Huntington's disease (HD). The aim of this study was to evaluate variability in circadian blood pressure (BP) to determine the association between abnormal circadian BP and sleep quality in patients with HD.Cross-sectional, multicenter study of 38 HD mutation carriers (23 premanifest and 15 early stage patients) who were compared to 38 age- and sex-matched controls. BP was evaluated by ambulatory blood pressure monitoring (ABPM). Based on the percentage decrease in nocturnal BP, subjects were classified as either dippers (≥10%) or non-dippers (10%). Sleep quality and daytime sleepiness were measured, respectively, using the Pittsburgh Sleep Quality Index (PSQI) and the Epworth Daytime Sleepiness Scale (ESS) and the scores on these indices were correlated with the ABPM findings.Sixty-three percent HD mutation carriers were non-dippers (86.7% of the symptomatic and 47.8% of the premanifest patients) versus 23.7% of controls (p = 0.001). In the HD group, sleep quality was significantly more impaired (PSQI5) (p = 0.016) with more excessive daytime sleepiness (ESS9) (p = 0.001) than in the control group. Nocturnal non-dipping was associated with worse sleep quality in patients (p = 0.011) but not in controls.These results show that patients with HD present early disturbances in the circadian rhythm of BP and that this altered nocturnal BP is associated with poor sleep quality. These findings suggest the potential role of subtle hypothalamic dysfunction in this population.

Background Many different preventatives have showed efficacy in the treatment of migraine. National guidelines differ in their recommendations and patients’ characteristics are usually taken into account in their selection. In Spain, real life use of preventive therapies seems to be heterogeneous. We aimed to evaluate differences in clinical practice and adherence to national guidelines among Spanish neurologists. Methods Observational descriptive study. A survey was conducted among neurologists ascribed to the Spanish Society of Neurology. Participants were differentiated in accordance with their dedication to headache disorders. We analysed socio-demographic parameters and evaluated 43 questions considering migraine management as well as therapeutic choices regarding migraine sub-types and finally, neurologists’ personal perception. Results One hundred fifty-five neurologists participated from 17 different regions, 43.4% of them female and 53.3% under 40 years of age. 34.9% confirmed headache disorders as their main interest. The first choice for preventive therapy in chronic migraine among participants was topiramate (57%) followed by amytriptiline (17.9%) and beta-blockers (14.6%). However in episodic migraine, the preferred options were beta-blockers (47.7%), topiramate (21.5%) and amytriptiline (13.4%). Regarding perceived efficacy, topiramate was considered the best option in chronic migraine (42.7%) followed by onabotulinumtoxinA (25.5%) and amitryptiline (22.4%). Where episodic migraine was concerned, surveyed neurologists perceived topiramate (43.7%) and beta-blockers (30.3%) as the best options. When we evaluated the duration of treatment use with a view to adequate therapeutic response, 43.5% of neurologists preferred 3 months duration and 39.5% were in favour of 6 months duration in episodic migraine. However, considering the preferred duration of treatment use in chronic migraine, 20.4% recommended 3 months, 42.1% preferred 6 months and 12.5% and 22.4% opted for 9 and 12 months respectively. When considering onabotulinumtoxinA therapy, the number of prior therapeutic failures was zero in 7.2% of neurologists, one in 5.9%, two in 44.1%, three in 30.9% and four or more in 11.9%. Following an initial treatment failure with onabotulinumtoxinA, 49% of subjects decided against a second treatment. The number of OnabotA procedures before considering it as ineffective was two in 18.9% of neurologists, three in 70.8% and four in 10.4%. Conclusions The initial management of migraine among Spanish Neurologists is in line with most guidelines, where first choice preventative drugs are concerned. The Management of episodic migraine differed from chronic migraine, both in terms of neurologist preference and in their perceived efficacy. Electronic supplementary material The online version of this article (10.1186/s10194-019-0971-6) contains supplementary material, which is available to authorized users.

Muscular dystrophies are a heterogeneous group of inherited dis-eases that cause progressive muscle weakness. The association of epi-lepsy with some of these diseases has been previously described andhas most commonly been found for Fukuyama-type muscular dystro-phy due to alterations in cerebral neuronal migration[1]. Among mus-cular dystrophies, limb-girdle muscular dystrophies (LGMDs)represent the fourth most common group, with a prevalence of 1.63per 100, 000 individuals[2]. The diseases in this group share a commonphenotype involving progressive weakness of the scapular and pelvicgirdles that starts after 2 years of age and can be accompanied by differ-ent degrees of elevation in blood creatine kinase (CK) levels and by var-ious anatomic pathologicalfindings. LGMDs are subdivided into LGMD1and LGMD2 depending on whether the inheritance is dominant or re-cessive, respectively. LGMD2A, which is caused by deficiency of thecalpain 3 protein owing to mutations in theCAPN3gene, is the mostcommon form of LGMD in Europe and America[2]. Its associationwith epilepsy has been described in only two isolated cases[1, 3], bothof them on the spectrum of genetic generalised epilepsies (GGEs). Thelatter are the most common group of epilepsies with genetic aetiology, accounting for 15–20% of all epilepsy cases[4]. Nonetheless, none of thegenes usually involved in monogenic epilepsies seem to play a majorrole in GGE, probably indicating a polygenic predisposition to GGE andtherefore a complex inheritance pattern[5]. Here, we describe a family...

Background Sleep disturbances are an early and prominent feature of Huntington's disease (HD). Objective The current study investigated the relation between sleep quality impairment and cognitive and psychiatric symptoms in patients with HD. Methods Sleep quality, daytime sleepiness, and neurocognitive symptoms were assessed in 38 mutation carriers (23 premanifest and 15 early stage) and 38 age-and sex-matched controls using standardized questionnaires (the Pittsburgh Sleep Quality Index, Epworth Sleepiness Scale, the cognitive section of the Unified Huntington's Disease Rating Scale, the Hospital Anxiety and Depression Scale, and the Irritability Scale). Results Compared to controls, HD patients had worse sleep quality (p = 0.016), which was associated with more severe cognitive impairment and higher anxiety, depression and irritability scores. These findings suggest that HD patients may have a delayed sleep phase, as indicated by the increased sleep onset latency (p = 0.019) and later wake-up time (0.013), which was associated with worse cognitive performance and greater depressive and anxiety symptoms. Conclusions Our data provide further evidence for an association between sleep quality in HD and cognitive performance and psychiatric symptoms.

Neurological involvement in Behcet's disease is rare, especially at the onset. It can present in the form of parenchymal changes or as damage to the vascular structures in its nonparenchymal form. The coexistence of both kinds of manifestations in the same patient is exceptional. We report the case of a 32-year-old patient with a history of deep venous thrombosis, who was being treated for holocranial headache, apathy, and oral and genital ulcers. Brain magnetic resonance imaging showed hyperintense lesions in the basal ganglia and white matter, and the vascular study evidenced venous thrombosis of the left transverse sinus. After confirming the diagnosis of Behcet's disease with parenchymal and nonparenchymal cerebral involvement, immunosuppressive and corticosteroid therapy was started, resulting in the remission of the symptoms.

To describe our experience in the treatment of laryngeal dystonia (in abduction and adduction), with special emphasis given to the technical aspects (approach procedure, dosage and type of botulinum toxin type A used), as well as treatment response and possible side effects. We conducted a cross-sectional descriptive study of a sample of patients with laryngeal dystonia treated by means of transoral administration of onabotulinumtoxinA or incobotulinumtoxinA over a period of 10 years (2007-2017). Data collected include demographic and clinical variables, treatment response (based on a self-rating scale), the duration of treatment and the appearance of side effects. Sample size: 15 patients (11 women; mean age: 44.06 years) with laryngeal dystonia (mean time since onset of 40 months; 12 patients with dystonia in adduction) and 174 administrations (92% incobotulinumtoxinA; average dosage of 5 U in each vocal cord). The procedure took an average of 11.7 minutes to perform. Response was good in 31% of the procedures and very good in 57.5%. Side effects were recorded in 14.4% of the procedures, although always mild and transitory, with a predominance of dysphagia and dysphonia. In our experience, transoral administration of botulinum toxin type A to treat laryngeal dystonia has proved to be a simple, quick, effective and safe technique.

Background.— It has been proposed that desaturation of oxygen during an apnea event is the trigger for cluster headache. Obstructive sleep apnea has been associated with a higher than normal cardiovascular morbidity and mortality. Some obstructive sleep apnea syndrome patients lack the sleep-related, nocturnal decrease, or “dip” in blood pressure, which is seen in normal individuals. Objective.— The aim of this study is to assess whether this non-dipper pattern is present in cluster headache patients. Design and Methods.— A total of 30 normotensive cluster headache patients underwent an ambulatory blood pressure monitoring. “Non dippers” were defined as patients with a nighttime mean blood pressure fall

Background. This study assesses the lifetime and active prevalence of epilepsy in Spain in people older than 18 years.Methods. EPIBERIA is a population-based epidemiological study of epilepsy prevalence using data from three representative Spanish regions (health districts in Zaragoza, Almería, and Seville) between 2012 and 2013. The study consisted of two phases: screening and confirmation. Participants completed a previously validated questionnaire (EPIBERIA questionnaire) over the telephone.Results. A total of 1741 valid questionnaires were obtained, including 261 (14.99%) raising a suspicion of epilepsy. Of these suspected cases, 216 (82.75%) agreed to participate in phase 2. Of the phase 2 participants, 22 met the International League Against Epilepsy’s diagnostic criteria for epilepsy. The estimated lifetime prevalence, adjusted by age and sex per 1,000 people, was 14.87 (95% CI: 9.8–21.9). Active prevalence was 5.79 (95% CI: 2.8–10.6). No significant age, sex, or regional differences in prevalence were detected.Conclusions. EPIBERIA provides the most accurate estimate of epilepsy prevalence in the Mediterranean region based on its original methodology and its adherence to ILAE recommendations. We highlight that the lifetime prevalence and inactive epilepsy prevalence figures observed here were compared to other epidemiological studies.

A 55-year-old woman, previously well, attended the emergency department with a 1-year history of falls, increasing dizziness, memory loss, personality change and poor concentration affecting certain activities, such as reading and puzzles. She attended a psychiatric clinic because of panic attacks and depression relating to her husband's ill health and domestic flooding. The psychiatrist diagnosed post-traumatic depression and anxiety and prescribed medication. However, her cognitive symptoms progressed over time and she developed a new …

Introduction Tuberculosis continues to be an intensly extended disease in under developed countries in immunodepressed and immunocompetent persons. In the central nervous system tuberculous lesions are the most frequent cause of space occupying lesions. Clinical case We report the case of a young, immunocompetent woman from Guinea, currently living in Spain. Following a seizure, neuro imaging showed there to be a right frontal space occupying lesion. The characteristics of the MR images obtained were fundamental to the determination of the meningeal site of the lesion en plaque and to suggest the aetiology as being an infectious inflammatory process, although a meningioma could not be ruled out. The absence of signs of extracerebral disease, the localization and distribution of the lesion and lack of short term response to medical treatment, which covered the different diagnostic possibilities, made it necessary to do a diagnostic meningeal biopsy. Conclusions MR imaging is a great help in the diagnosis of tuberculous lesions of the central nervous system. The differential diagnosis between tuberculous pachymeningitis and meningioma, both in the form of plaques, is extremely difficult without obtaining a biopsy specimen. The finding of epithelioid granulomas with caseous necrosis and/or acid alcohol fast bacilli in the biopsy specimen is diagnostic of tuberculosis. A single meningeal lesion situated in the cortex but with no associated extracerebral disease causes considerable difficulty in diagnosis. Tuberculosis must always be remembered in view of the increasing number of immigrants from under developed countries.

INTRODUCTION Vigabatrin is an effective antiepileptic drug for treatment of partial crises with or without generalization. It acts by increasing GABA levels by irreversible inhibition of the enzyme GABA-transaminase. In recent years there have been several reports published on the appearance of alterations of the visual fields of patients treated with this drug. OBJECTIVE To study the prevalence and characteristics of the visual field changes associated with vigabatrin treatment in an adult epilepsy clinic. PATIENTS AND METHODS Ophthalmological examination, consisting of questions about eye symptoms, study of the fundus oculi, Humphrey-type campimetry, protocol 30-2, visual evoked potentials and electroretinogram were done on a group of 10 patients on long-term vigabatrin treatment. RESULTS Six of the ten patients (60%) had campimetric alterations, which were serious in two cases (20%). Only the two patients with severe field defects complained of sight changes when questioned and their complaints were mainly of repeatedly bumping into things. In two patients (20%) there was pallor of the optic nerve. In both cases, the patients with severe field defects showed alterations on the electroretinogram. Visual evoked potentials were normal in all cases. CONCLUSIONS There is a very high prevalence of visual field defects in patients on long-term treatment with vigabatrin. Clinical ophthalmological signs are not usually seen until advanced stages. Pallor of the optic nerve was seen in a group of patients. It is very important that serial campimetric studies are done for early detection of such alterations.

INTRODUCTION Approximately 20% of all ischemic strokes are due to cardioembolism and occur more frequently in the younger patients. Our objective was to determine the clinical characteristics and course of cardioembolic infarcts (ICCE) comparing them with infarcts due to other aetiologies (ICNCE). PATIENTS AND METHODS We made a prospective study of 354 patients admitted to hospital over a period of one year, after excluding transient ischaemia and parenchymatous/subarachnoid hemorrhage. Two groups were established: ICCE (29.4%) and ICNCE (70.6%), comparing age, sex, risk factors and course of the illness. Subsequently a study lasing two years was done to assess the recurrence rate. RESULTS The ICCE patients were older (75.89 compared with 72.58, p = 0.004), often know the exact time of onset of their symptoms (p = 0.015) and usually are admitted to hospital during the first six hours of their illness (p = 0.01). There was a significantly higher incidence of ischemic cardiopathy or auricular fibrillation (p = 0.0052); p = 0.005); more complications arose (p = 0.000); stay in hospital was longer (13.62 as compared to 11.8 days; p = 0.035), there was a lower weekly BI score (p = 0.0023) and higher mortality (p = 0.000). In the subgroup of 70 patients evaluated two years later 11 cases recurred, with no difference observed between the anticoagulant and anti-aggregant groups. CONCLUSION The ICCE occurs in older patients, they develop worse neurological defects, have a worse short-term prognosis, develop more complications and have significantly greater mortality.

Introduction Diabetes mellitus (DM) is an important risk factor in cerebral vascular disease since it causes endothelial proliferation and thickening of the plasmatic membrane in the small blood vessels. The pathogenic mechanism is thus different from that of athero-thrombosis or emboli. Our objective was to determine whether there are differences between strokes in diabetic patients and strokes secondary to other diseases. Patients and methods We made a prospective study of 415 stroke patients admitted to hospital consecutively over one year. Transient ischaemia and subarachnoid hemorrhage were excluded. We analysed age, sex, risk factors, severity (Canadian scale), mortality and functional prognosis one week later. Results Of the 415 patients, 354 were diagnosed as having cerebral infarcts and 61 parenchymatous hemorrhage. The average age was 72.2 years. The commonest risk factor was arterial hypertension (n = 241). There were 95 patients with DM. Bivariate and multivariate analysis showed independent variables associated with DM to be the onset of cerebrovascular pathology at a younger age (p = 0.009), association with arterial hypertension (p = 0.002) and poor previous quality of life (p = 0.003). We did not find a higher incidence of lacunar infarcts amongst the diabetic patients. Conclusions The diabetic patient is younger and often also has arterial hypertension. There was no difference in mortality or type of stroke (ischemic compared with hemorrhagic). No significant relationship was found with lacunar infarcts.

Objective Peripheral facial paralysis is one of the commonest mononeuropathies. The frequency of idiopathic peripheral paralysis or Bell's palsy varies between 62% and 93% of all cases, with an incidence of between 14 and 25 cases per 100,000 inhabitants per year. However, in spite of its relative frequency in the population, there is still no definite unanimously agreed strategy currently used by neurologists. Although its course is relatively benign, up to 16% of the patients show moderate or severe sequelae according to follow-up data in 1,011 untreated patients. In this article we review the aetiology, diagnostic methods and therapeutical strategies at the present time. Development The topographic diagnosis of the lesion is based on the symptoms associated with the paralysis. The neurophysiological studies (trigeminofacial reflection and electroneurogram), usually done after the first week are prognoses that permit differentiation of the degree of the lesion (neuropraxis, axonotmesis or neurotmesis). In spite of recent trials with combined therapy (acyclovir and steroids) the most generally accepted treatment at present is still prednisose at a dose of 1 mg/kg/day. Conclusions Peripheral facial paralysis is a common condition, usually with no apparent cause and an eminently clinical diagnosis. The electrophysiological studies determine the prognosis. Currently the standard treatment is still oral prednisone.