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Background and purposeSpain has been heavily affected by COVID-19. Reallocation of resources for managing the outbreak might have caused a disruption in stroke care. This study analyses the impact on stroke care of reorganising the healthcare system in response to the first COVID-19 outbreak peak in Spain and the strategies adopted by Spanish stroke units to deal with this impact.Materials and methodsWe obtained data from a structured survey sent to the responsible of stroke units across the country. We recorded the number of strokes, stroke code activations, intravenous thrombolysis treatments and mechanical thrombectomies during February and March 2019 and 2020. We also collected information on the impact on workflow metrics and on the availability of specialised neurological care and rehabilitation treatments, the characteristics of stroke care for patients with SARS-CoV-2 infection and the impact on human resources. We compared the activity data between 2019 and 2020 and the information on activity and impact on stroke care between regions classified according to the disease incidence rate.ResultsFifty-seven (75%) of all stroke units in Spain responded to the survey. There was an overall reduction in admissions for all stroke types during the outbreak’s peak and in the number of stroke code activations and intravenous thrombolysis treatments, results that were independent of the COVID-19 incidence rate. Participants reported a delay in workflow metrics and a reduction of admissions to stroke units, outpatient clinics and rehabilitation therapies. Specific pathways and protocols for managing stroke patients with SARS-CoV-2 infection have been established.ConclusionThe COVID-19 outbreak has jeopardised all phases of stroke care. As a consequence, some patients with stroke did not receive adequate treatment.

Background Recently, amylin and its receptors were found in different structures involved in migraine pathophysiology. Here, we evaluate interictal concentrations of amylin and calcitonin gene-related peptide in peripheral blood as biomarkers for chronic migraine. Methods We prospectively recruited patients with episodic migraine, chronic migraine and healthy controls. Interictal amylin and calcitonin gene-related peptide levels were assessed in blood samples using enzyme linked immunosorbent assay. Results We assessed plasma samples from 58 patients with episodic migraine (mean age 37.71 ± 10.47, 87.9% female), 191 with chronic migraine (mean age 46.03 ± 11.93, 95% female), and on 68 healthy controls (mean age 43.58 ± 11.08 years, 86% female). Body mass index was 25.94 ± 4.53 kg/m2 for migraine patients and 25.13 ± 4.92 kg/m2 for healthy controls ( p = 0.0683). Interictal plasma amylin levels were higher in chronic migraine patients (47.1 pg/mL) than in the episodic migraine patients (28.84 pg/mL, p Conclusion Interictal plasma amylin levels are higher in patients with chronic migraine and may serve as a diagnostic biomarker for chronic migraine.

Objective To investigate the specific relationship between cutaneous allodynia (CA) and the percentages of body fat (BF) and abdominal fat in migraineurs. Additionally, we compared serum levels of inflammatory biomarkers in patients with and without CA. Background Excess abdominal fat might facilitate progressive changes in nociceptive thresholds causing central sensitization, clinically reflected as CA, which could drive migraine progression. Methods This prospective cohort study included 80 patients with migraine (mean age 39 years, 81.2% female) and 39 non-migraine controls. We analysed each participant’s height, body weight, and body mass index (BMI). The amount and distribution of BF was also assessed by air displacement plethysmography (ADP) and ViScan, respectively. We analysed serum levels of markers of inflammation, during interictal periods. Results We studied 52 patients with episodic migraine (EM) and 28 with chronic migraine (CM). Of the 80 patients, 53 (53.8%) had CA. Migraineurs with CA had a higher proportion of abdominal fat values than patients without CA (p = 0.04). The independent risk factors for CA were the use of migraine prophylaxis (OR 3.26, 95% CI [1.14 to 9.32]; p = 0.03), proportion of abdominal fat (OR 1.13, 95% CI [1.01 to 1.27]; p = 0.04), and presence of sleep disorders (OR 1.13, 95% CI [00.01 to 1.27]; p = 0.04). The concordance correlation coefficient between the ADP and BMI measurements was 0.51 (0.3681 to 0.6247). CA was not correlated with the mean plasma levels of inflammatory biomarkers. Conclusions There is a relation between excess abdominal fat and CA. Abdominal obesity might contribute to the development of central sensitization in migraineurs, leading to migraine chronification.

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

Neurociència; Psicologia Neurociencia; Psicología Neuroscience; Psychology Identifying highly disabled patients or at high risk of psychiatric comorbidity is crucial for migraine management. The burden of migraine increases with headache frequency, but the number of headache days (HDs) per month after which disability becomes severe or the risk of anxiety and depression is higher has not been established. Here, we estimate the number of HDs per month after which migraine is associated with higher risk of anxiety and depression, severe disability and lower quality of life. We analysed 468 migraine patients (mean age 36.8 ± 10.7; 90.2% female), of whom 38.5% had ≥ 15 HDs per month. Our results show a positive linear correlation between the number of HDs per month and the risk of anxiety (r = 0.273; p

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

Objective To identify factors associated with poor anticoagulation control with vitamin K antagonists (VKA) among outpatients with nonvalvular atrial fibrillation (NVAF) attended in Neurology and Internal Medicine in Spain. Methods Cross-sectional and multicenter study, from the ALADIN database, of outpatients with NVAF treated with VKA and attended in Internal Medicine and Neurology in Spain. Rates of anticoagulation control were determined with the direct and Rosendaal methods, considering data from the 6 months before the inclusion. Results Out of 1337 patients included in the ALADIN study, 750 were taking VKA, and complete information about INR values in the last 6 months was available in 383 patients. Mean scores of Charlson Index, CHADS2, CHA2DS2-VASc and HAS-BLED were 1.94 ± 1.54; 3.10 ± 1.26; 4.63 ± 1.54, and 2.20 ± 0.90, respectively. 46.2% and 47.0% of patients had an adequate anticoagulation control according to the direct and Rosendaal methods, respectively. Inadequate anticoagulation control according to the direct method was associated with diabetes (OR: 2.511; 95% CI: 1.144–5.659), prior labile INR (OR: 35.371; 95% CI: 15.058–83.083) and the determination of >6 INR controls in the last 6 months (OR: 4.747; 95% CI: 2.094–10.759), and according to the Rosendaal method, with prior labile INR (p Conclusions Despite the high thromboembolic risk, only a little more than a half of patients were well controlled. Factors associated with poor anticoagulation control were diabetes, labile INR, >6 INR controls and HAS-BLED.

Background Major adverse cardiovascular events are the main cause of morbidity and mortality over the long term in patients undergoing carotid endarterectomy. There are few reports assessing the prognostic value of markers of inflammation in relation to the risk of cardiovascular disease after carotid endarterectomy. Here, we aimed to determine whether matrix metalloproteinases (MMP-1, MMP-2, MMP-7, MMP-9 and MMP-10), tissue inhibitor of MMPs (TIMP-1) and in vivo inflammation studied by 18F-FDG-PET/CT predict recurrent cardiovascular events in patients with carotid stenosis who underwent endarterectomy. Methods This prospective cohort study was carried out on 31 consecutive patients with symptomatic (23/31) or asymptomatic (8/31) severe (> 70%) carotid stenosis who were scheduled for carotid endarterectomy between July 2013 and March 2016. In addition, 26 healthy controls were included in the study. Plasma and serum samples were collected 2 days prior to surgery and tested for MMP-1, MMP-2, MMP-7, MMP-9, MMP-10, TIMP-1, high-density lipoprotein, low-density lipoprotein, high-sensitivity C-reactive protein and erythrocyte sedimentation rate. 18F-FDG-PET/CT focusing on several territories’ vascular wall metabolism was performed on 29 of the patients because of no presurgical availability in 2 symptomatic patients. Histological and immunohistochemical studies were performed with antibodies targeting MMP-10, MMP-9, TIMP-1 and CD68. Results The patients with carotid stenosis had significantly more circulating MMP-1, MMP-7 and MMP-10 than the healthy controls. Intraplaque TIMP-1 was correlated with its plasma level (r = 0.42 P = .02) and with 18F-FDG uptake (r = 0.38 P = .05). We did not find any correlation between circulating MMPs and in vivo carotid plaque metabolism assessed by 18F-FDG-PET. After a median follow-up of 1077 days, 4 cerebrovascular, 7 cardiovascular and 11 peripheral vascular events requiring hospitalization were registered. Circulating MMP-7 was capable of predicting events over and above the traditional risk factors (HR = 1.15 P = .006). When the model was associated with the variables of interest, the risk predicted by 18F-FDG-PET was not significant. Conclusions Circulating MMP-7 may represent a novel marker for recurrent cardiovascular events in patients with moderate to severe carotid stenosis. MMP-7 may reflect the atherosclerotic burden but not plaque inflammation in this specific vascular territory.

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.

Isolated acute vestibular syndrome remains a diagnostic challenge in the emergency department and the initial approach should include the identification of a central or peripheral etiology. This is the case report of an elderly patient with known cardiovascular risk factors presenting with acute vertigo and unsteadiness. Neurological examination was notable only for down-beat nystagmus and diffusion-weighted MRI showed normal findings. He was treated as having an emerging anterior–inferior cerebellar artery (AICA) stroke. Even when MRI showed no signs of hemorrhage or infarction, the neurotological bedside examination was a determinant. The cochleovestibular system was not spared by the ischemic injury but a more extensive neurological damage was probably avoided by approaching this case as a stroke.

Background There are only four previous pediatric reports of the glossopharyngeal neuralgic form of the stylohyoid complex syndrome. Stylohyoid complex has merely been described as cases of glossopharyngeal neuralgia in children. Case Report A 12-year-old boy came to our hospital because of recurrent episodes of severe cranial pain (9/10) lasting for 5 to 15 minutes. Pain affected the right tonsillar fossa, ear, and mastoid region. Since the start at the age of 9 years, the frequency of painful episodes has progressively increased: when admitted to our clinics 3 years later, the child was having up to five episodes daily in spite of analgesic, antiepileptic, and antidepressant drugs; he had abandoned school and leisure. Between episodes, neurological examination detected only discomfort to pressure on the right tonsillar fossa. Three-dimensional computed tomography images of the skull base showed an elongated right styloid process and bilateral calcification of the stylohyoid ligament. After surgical excision of the right styloid process and of part of the stylohyoid ligament, the glossopharyngeal painful episodes ceased. The patient remains asymptomatic seven years later. Conclusion In spite of its rarity in childhood, this debilitating but treatable syndrome should be kept in mind for the differential diagnosis of recurrent cranial pain in the pediatric population.

Background Localized facial scleroderma usually presents as frontal linear morphea or progressive hemifacial atrophy. Only isolated cases of trigeminal painful neuropathy have been described. Case report A 43-year-old woman developed an oval lesion on the right cheek. After 1 year, she noticed constant “pulling” pain and episodes of lancinating pain, both spontaneous and triggered by chewing and cold drinks. She was diagnosed with solitary morphea profunda and CT scan, ultrasonography, cranial MRI and biopsy were completed. Methylprednisolone (1 gr/day for 3 days) was prescribed. For pain, gabapentin, oxcarbazepine, amitryptiline, pregabalin and eslicarbacepine were all ineffective. A capsaicin patch was placed with prolonged benefit. Later on, the pain slightly worsened; occipital blockade was effective and methotrexate was recommended. Conclusion This is the first case of solitary morphea profunda associated with painful trigeminal neuropathy. Treatment should include immunosuppressants and treatment of neuropathic pain, in which local therapies seem particularly beneficial.

Introduction: Prionopathy is the cause of 62% of the rapidly progressive dementias (RPD) in which a definitive diagnosis is reached. The variability of symptoms and signs exhibited by the patients, as well as its different presentation, sometimes makes an early diagnosis difficult. Methods: Patients with diagnosis of definite or probable prionopathy during the period 1999–2012 at our hospital were retrospectively reviewed. The clinical features and the results of the complementary tests (14-3-3 protein, EEG, MRI, FDG-PET, and genetic analysis) were evaluated in order to identify some factors that may enable an earlier diagnosis to be made. Results: A total of 14 patients are described: 6 with definite sporadic Creutzfeldt-Jakob (sCJD) disease, 3 with probable sCJD, 4 with fatal familial insomnia, and 1 with the new variant. The median age at diagnosis was 54 years old. The mean survival was 9.5 months. Mood disorder was the most common feature, followed by instability and cognitive impairment. 14-3-3 protein content in the cerebrospinal fluid was positive in 7 of 11 patients, and the EEG showed typical signs in 2 of 12 patients. Neuroimaging (FDG-PET, MRI) studies suggested the diagnosis in 13 of the 14 patients included. Conclusions: Most patients presenting with RPD suffer from a prion disease. In our series the most useful complementary tests were MRI and FDG-PET, being positive in 13 of the 14 patients studied. Resumen: Introducción: Las prionopatías representan hasta el 62% de los casos de demencia rápidamente progresiva (DRP) en los que se alcanza un diagnóstico definitivo. La variabilidad de los síntomas y signos iniciales y las diferencias en su evolución dificultan el diagnóstico precoz. Métodos: Estudio retrospectivo en el que se incluye a pacientes con prionopatía probable o definitiva, que acudieron a la consulta de Neurología de nuestro centro durante el periodo 1999–2012. Se describen las características clínicas y los resultados de las exploraciones complementarias (proteína 14-3-3, EEG, RM, PET-FDG y análisis genético), con la finalidad de identificar qué marcadores permiten un diagnóstico precoz. Resultados: Se describe a 14 pacientes: 6 con enfermedad de Creutzfeldt-Jakob esporádica (ECJe) definitiva, 3 con ECJe probable, 4 con insomnio familiar fatal y uno con la nueva variante de la enfermedad de Creutzfeldt-Jakob. La mediana de edad al diagnóstico fue de 54 años y la mediana de supervivencia de 9,5 meses. El trastorno del ánimo fue el síntoma inicial más frecuente, seguido de inestabilidad de la marcha y deterioro cognitivo. La proteína 14-3-3 fue positiva en el líquido cefalorraquídeo en 7 de 11 pacientes y el EEG mostró signos típicos en 2 de 12 pacientes explorados. El estudio de neuroimagen mostró alteraciones en 13 de los 14 pacientes. Conclusiones: Además de la DRP, el trastorno conductual y de la marcha son síntomas iniciales frecuentes en las prionopatías. En nuestra serie, las pruebas complementarias más útiles para apoyar el diagnóstico fueron la RM y la PET-FDG. Keywords: Prion, Creutzfeldt-Jakob, Rapidly progressive dementia, Magnetic resonance imaging, Positron emission computed tomography, Diagnosis, Palabras clave: Prión, Creutzfeldt-Jakob, Demencia rápidamente progresiva, Resonancia magnética, Tomografía por emisión de positrones, Diagnóstico

Objective To expand the differential diagnosis of headache and ophthalmoparesis by describing a case report in which anti-GQ1b was demonstrated to be the cause. Background Anti-GQ1b antibody syndrome refers to a clinical spectrum of conditions that share common mechanisms and overlapping manifestations, including the Miller-Fisher syndrome, pharyngeal-cervical-brachial weakness, and Bickerstaff brainstem encephalitis. Rare atypical cases presenting as acute ophthalmoparesis (AO) without ataxia or areflexia have been described. Headache is a rare condition in these disorders. Methods A 49-year-old woman with no history of headaches began experiencing an acute severe bilateral throbbing headache associated with nausea and photophobia. Five days later, she developed constant binocular horizontal diplopia. Results Bilateral paresis of both sixth nerves was noted. Her ocular fundi, tendon reflexes, and other findings of the physical exam were normal. In addition, both a brain MRI performed with gadolinium and a lumbar puncture yielded normal results. Serum anti-GQ1b IgG was found to be positive. Her symptoms resolved completely following treatment with immunoglobulins (0.4 g/kg/day for 5 days). Conclusions This is the first reported case of AO related to anti-GQ1b antibodies presenting with headache as its initial symptom. The presence of anti-GQ1b antibodies should be determined in patients with headache and AO of unknown origin. Immunoglobulins could hasten the resolution of symptoms in these patients.

Objective: To update the Spanish Society of Neurology's guidelines for subarachnoid haemorrhage diagnosis and treatment. Materials and methods: A review and analysis of the existing literature. Recommendations are given based on the level of evidence for each study reviewed. Results: The most common cause of spontaneous subarachnoid haemorrhage (SAH) is cerebral aneurysm rupture. Its estimated incidence in Spain is 9/100, 000 inhabitants/year with a relative frequency of approximately 5% of all strokes. Hypertension and smoking are the main risk factors. Stroke patients require treatment in a specialised centre. Admission to a stroke unit should be considered for SAH patients whose initial clinical condition is good (Grades I or II on the Hunt and Hess scale). We recommend early exclusion of aneurysms from the circulation. The diagnostic study of choice for SAH is brain CT (computed tomography) without contrast. If the test is negative and SAH is still suspected, a lumbar puncture should then be performed. The diagnostic tests recommended in order to determine the source of the haemorrhage are MRI (magnetic resonance imaging) and angiography. Doppler ultrasonography studies are very useful for diagnosing and monitoring vasospasm. Nimodipine is recommended for preventing delayed cerebral ischaemia. Blood pressure treatment and neurovascular intervention may be considered in treating refractory vasospasm. Conclusions: SAH is a severe and complex disease which must be managed in specialised centres by professionals with ample experience in relevant diagnostic and therapeutic processes. Resumen: Objetivo: Actualización de la guía para el diagnóstico y tratamiento de la hemorragia subaracnoidea de la Sociedad Española de Neurología. Material y métodos: Revisión y análisis de la bibliografía existente. Se establecen recomendaciones en función del nivel de evidencia que ofrecen los estudios revisados. Resultados: La causa más frecuente de hemorragia subaracnoidea espontánea (HSA) es la rotura de un aneurisma cerebral. Su incidencia se sitúa en torno 9 casos por 100.000 habitantes/año y supone un 5% de todos los ictus. La hipertensión arterial y el tabaquismo son sus principales factores de riesgo. Se ha de realizar el tratamiento en centros especializados. Se debe considerar el ingreso en unidades de ictus de aquellos pacientes con HSA y buena situación clínica inicial (grados I y II en la escala de Hunt y Hess). Se recomienda la exclusión precoz de la circulación del aneurisma. El estudio diagnóstico de elección es la tomografía computarizada (TC) craneal sin contraste. Si esta es negativa y persiste la sospecha clínica se aconseja realizar una punción lumbar. Los estudios de elección para identificar la fuente de sangrado son la resonancia magnética (RM) y la angiografía. Los estudios ultrasonográficos son útiles para el diagnóstico y seguimiento del vasoespasmo. Se recomienda el nimodipino para la prevención de la isquemia cerebral diferida. La terapia hipertensiva y el intervencionismo neurovascular pueden plantearse para tratar el vasoespasmo establecido. Conclusiones: La HSA es una enfermedad grave y compleja que debe ser atendida en centros especializados, con suficiente experiencia para abordar el proceso diagnóstico y terapéutico. Keywords: Subarachnoid haemorrhage, Cerebral aneurysm, Diagnosis, Vasospasm, Delayed cerebral ischaemia, Rebleeding, Medical treatment, Palabras clave: Hemorragia subaracnoidea, Aneurisma cerebral, Diagnóstico, Vasoespasmo, Isquemia cerebral diferida, Resangrado, Tratamiento médico

A 70-year-old woman with a history of autoimmune hepatitis and renal cell carcinoma presented with subacute cognitive impairment. A brain MRI revealed mild leukoaraiosis, whereas brain F-FDG PET/CT showed diffuse cerebral hypometabolism that resembled some of the patterns described in limbic encephalitis and neurodegenerative diseases. With the suspicion of autoimmune encephalitis, the patient received immunotherapy with dramatic improvement of cognitive function and metabolic normalization at the 2-month follow-up on brain F-FDG PET/CT. Our results demonstrate that brain F-FDG PET/CT might be a useful tool in the assessment of patients with autoimmune encephalitis.

Introduction: Update of Acute Ischaemic Stroke Treatment Guidelines of the Spanish Neurological Society based on a critical review of the literature. Recommendations are made based on levels of evidence from published data and studies. Development: Organised systems of care should be implemented to ensure access to the optimal management of all acute stroke patients in stroke units. Standard of care should include treatment of blood pressure (should only be treated if values are over 185/105 mmHg), treatment of hyperglycaemia over 155 mg/dl, and treatment of body temperature with antipyretic drugs if it rises above 37.5 °C. Neurological and systemic complications must be prevented and promptly treated. Decompressive hemicraniectomy should be considered in cases of malignant cerebral oedema. Intravenous thrombolysis with rtPA should be administered within 4.5 hours from symptom onset, except when there are contraindications. Intra-arterial pharmacological thrombolysis can be considered within 6 hours, and mechanical thrombectomy within 8 hours from onset, for anterior circulation strokes, while a wider window of opportunity up to 12–24 hours is feasible for posterior strokes. There is not enough evidence to recommend routine use of the so-called neuroprotective drugs. Anticoagulation should be administered to patients with cerebral vein thrombosis. Rehabilitation should be started as early as possible. Conclusion: Treatment of acute ischaemic stroke includes management of patients in stroke units. Systemic thrombolysis should be considered within 4.5 hours from symptom onset. Intra-arterial approaches with a wider window of opportunity can be an option in certain cases. Protective and restorative therapies are being investigated. Resumen: Introducción: Actualización de la guía para el tratamiento del infarto cerebral agudo de la Sociedad Española de Neurología basada en la revisión y análisis de la bibliografía existente sobre el tema. Se establecen recomendaciones en base al nivel de evidencia que ofrecen los estudios revisados. Desarrollo: Los sistemas de asistencia urgente extrahospitalaria se organizarán para asegurar la atención especializada de los pacientes y el ingreso en unidades de ictus (UI). Deben aplicarse cuidados generales para mantener la homeostasis (tratar la tensión arterial sistólica > 185 mmHg o diastólica > 105 mmHg, evitar hiperglucemia > 155 mg/dl y controlar la temperatura, tratando con antitérmicos cifras > 37,5 °C), y prevenir y tratar las complicaciones. La craniectomía descompresiva debe ser considerada en casos seleccionados de oedema cerebral maligno. La trombólisis intravenosa con rtPA se administrará en las primeras 4,5 horas en pacientes sin contraindicación. La trombólisis intraarterial farmacológica puede indicarse en las primeras 6 horas de evolución y la trombectomía mecánica hasta las 8 horas. En el territorio posterior la ventana puede ampliarse hasta 12–24 horas. No hay evidencias para recomendar el uso rutinario de los fármacos denominados neuroprotectores. Se recomienda la anticoagulación en pacientes con trombosis de senos venosos. Se aconseja el inicio precoz de rehabilitación. Conclusiones: El tratamiento del infarto cerebral se basa en la atención especializada en UI, la aplicación urgente de cuidados generales y el tratamiento trombolítico intravenoso en las primeras 4,5 horas. La recanalización intraarterial farmacológica o mecánica pueden ser útiles en casos seleccionados. Terapias de protección y reparación cerebral están en desarrollo. Keywords: Cerebral infarct, Ischaemic stroke, Thrombolysis, Brain protection, Stroke units, Cerebral venous thrombosis, Palabras clave: Infarto cerebral, Ictus isquémico, Trombólisis, Cerebroprotección, Unidades de ictus, Trombosis venosa cerebral

In Spain, approximately 28% of ischaemic strokes have an atherothrombotic cause, and most are due to carotid stenosis. Ultrasound is the most commonly used technique for diagnosing carotid stenosis. Changes in blood flow velocity at the point of maximum stenosis, together with haemodynamic changes in proximal regions (common carotid artery) and distal regions (poststenotic internal carotid, ophthalmic artery, and the circle of Willis), allow us to measure carotid stenosis precisely. This review explains the methodology to be followed when evaluating carotid stenosis ultrasonographically, according to the recommendations from the Spanish Society of Neurosonology (SONES). We review the findings that permit us to measure the degree of extracranial carotid stenosis using both carotid and transcranial ultrasound, with particular emphasis on the importance of assessing indirect signs. Resumen: En España se estima que aproximadamente un 28% de los ictus isquémicos son de etiología aterotrombótica y mayoritariamente se deben a una estenosis carotídea. La ultrasonografía es la técnica más habitual para el diagnóstico de la estenosis carotídea. Las alteraciones de la velocidad de flujo en el punto de máxima estenosis junto con los cambios hemodinámicos en regiones proximales (arteria carótida común) y distales (carótida interna postestenótica, arteria oftálmica y polígono de Willis) permiten cuantificar con precisión la estenosis carotídea. En esta revisión se detalla la metodología para la evaluación de la estenosis carotídea desde el punto de vista ultrasonográfico, siguiendo las recomendaciones de consenso establecidas por la Sociedad Española de Neurosonología (SONES). Se revisan los hallazgos que permiten cuantificar el grado de estenosis carotídea extracraneal utilizando tanto el estudio ultrasonográfico carotídeo como el transcraneal, con un especial énfasis en la importancia de la valoración de signos indirectos. Keywords: Internal carotid artery, Stenosis, Doppler ultrasound, Transcranial Doppler, Colour-flow duplex, Ultrasonography, Palabras clave: Arteria carótida interna, Estenosis, Doppler, Transcraneal, Dúplex-color, Ultrasonografía

Cephalalgia alopecia is a rare and recently described headache syndrome in which recurrent, burning head and neck pain is associated with hair loss from areas of scalp affected by the pain. We here report the case of a 33-year-old woman with continuous unilateral occipital pain and colocalized alopecia, only responsive to onabotulinumtoxin A injections. We hypothesize whether this clinical phenotype may correspond to either cephalalgia alopecia or nummular headache with trophic changes, conditions that might represent 2 manifestations of the same spectrum of disorders.

There is currently increasing interest in epicardial adipose tissue (EAT) as a marker of cardiovascular disease. Our purpose was to describe EAT, measured by transthoracic echocardiography, and to assess its association with metabolic syndrome (MS) in the RIVANA population-based study.Physical examination was performed in 880 participants aged 45 to 74 years (492 of them with MS according to the harmonized definition). Fasting glucose, high-density lipoprotein cholesterol, triglyceride, and C-reactive protein concentrations were determined in a blood sample. In all participants, EAT thickness was measured with transthoracic echocardiography at end-systole.Among participants without MS, the prevalence of EAT ≥ 5mm significantly increased with age (OR65 years vs 45-54 years=8.22; 95%CI, 3.90-17.35; P for trend.001). Increasing EAT quintiles were significantly associated with MS (OR fifth quintile vs first quintile=3.26; 95%CI, 1.59-6.71; P for trend=.001). Considering the different MS criteria, increasing quintiles of EAT were independently associated with low high-density lipoprotein cholesterol (OR fifth quintile vs first quintile=2.65; 95%CI, 1.16-6.05; P for trend=.028), high triglycerides (OR fifth quintile vs first quintile=2.22; 95%CI, 1.26-3.90; P for trend=.003), and elevated waist circumference (OR fifth quintile vs first quintile=6.85; 95%CI, 2.91-16.11; P for trend.001).In a subsample of the general population, EAT measured by echocardiography increased significantly and independently with age. Increased EAT thickness was independently associated with MS and with low high-density lipoprotein cholesterol, high triglycerides, and elevated waist circumference as individual criteria.

Objective: To update the ad hoc Committee of the Cerebrovascular Diseases Study Group of The Spanish Neurological Society guidelines on prevention of ischaemic stroke (IS) and transient ischaemic attack (TIA). Methods: We reviewed available evidence on risk factors and means of modifying them to prevent IS and TIA. Levels of evidence and recommendation grades are based on the classification of the Centre for Evidence-Based Medicine. Results: This first section summarises the recommendations for action on the following factors: blood pressure, diabetes, lipids, tobacco and alcohol consumption, diet and physical activity, cardio-embolic diseases, asymptomatic carotid stenosis, hormone replacement therapy (HRT) and contraceptives, hyperhomocysteinemia, prothrombotic states and sleep apnea syndrome. Conclusions: Changes in lifestyle and pharmacological treatment for hypertension, diabetes mellitus and dyslipidemia, according to criteria of primary and secondary prevention, are recommended for preventing IS. Resumen: Objetivo: Actualizar las guías terapéuticas del Comité ad hoc del Grupo de Estudio de Enfermedades Cerebrovasculares de la SEN en el tratamiento preventivo de ictus isquémico (II) y ataque isquémico transitorio (AIT). Métodos: Revisión de evidencias disponibles sobre los factores de riesgo y la oportunidad de su modificación para prevenir el ictus isquémico y AIT. Los niveles de evidencia y grados de recomendación se han basado en la clasificación del Centro de Medicina Basada en la Evidencia. Resultados: En esta primera parte se resumen las recomendaciones sobre la actuación sobre los siguientes factores: presión arterial, DM, lípidos plasmáticos, consumo de tabaco y alcohol, dieta y actividad física, cardiopatías embolígenas, estenosis carotídea asintomática, terapia hormonal sustitutiva y anticonceptivos, hiperhomocisteinemía, estados protrombóticos y síndrome de apnea del sueño. Conclusiones: La modificación de los estilos de vida y el tratamiento farmacológico de la hipertensión arterial, diabetes méllitus y dislipemia según criterios de prevención primaria y secundaria se recomiendan en la prevención de ictus isquémico. Keywords: Guidelines, Ischaemic stroke, Transient ischaemic attack, Prevention, Palabras clave: Guías de práctica clínica, Prevención de ictus, Ictus, Ataque isquémico transitorio

Migraine is a common disabling disorder that affects approximately 12% of the population. Migraine treatment requires the avoidance of triggers, acute treatment to control individual attacks, and preventive treatment for patients with frequent headaches. The choice between the different drugs available for the acute management of migraine is based on the severity of the attacks and associated symptoms. Migraine-specific acute therapies, such as triptans, are recommended in patients with moderate or severe migraine attacks and also for mild episodes that do not respond to simple analgesics. The use of simple analgesics is appropriate for mild attacks or patients who cannot use triptans. Currently, ergotics are not recommended in de novo migraine patients mainly because of their lower efficacy compared to triptans and their side-effect profile. Novel methods for delivering triptans and ergotics will increase the efficacy and reduce the side effects of current formulations. New acute migraine therapies without vasoconstrictive activity and a better side-effect profile than triptans are under investigation. This review focuses on drugs to treat acute migraine attacks and covers a comprehensive selection of emerging therapies.

Central nervous system (CNS) prophylaxis is required during initial treatment of non-Hodgkin lymphoma (NHL) subtypes that carry a high risk of CNS involvement. Intrathecal (IT) liposomal cytarabine, a formulation with prolonged half-life, has been shown to be safe and effective in the treatment of meningeal disease in patients with high-grade lymphoma. We retrospectively reviewed all adult patients with high-grade NHL that received prophylactic therapy with IT liposomal cytarabine and developed neurologic complications in our institution between April 2007 and May 2009. We recorded information on hospital admission, chemotherapy regimens, clinical features, neuroimaging, cerebrospinal fluid, neurophysiology data, and outcome. Neurotoxicity was graded according to the National Cancer Institute Common Toxicity Criteria (NCI-CTC). Four of fourteen patients (28%) developed moderate or severe neurotoxicity (grades 2 and 3 of the NCI-CTC), manifested as conus medullaris/cauda equine syndrome or pseudotumour cerebri-like syndrome, after a median of 3.5 IT courses of liposomal cytarabine. All patients had received corticosteroids to prevent arachnoiditis. Liposomal cytarabine given via the IT route, even with concomitant corticosteroid administration, can result in significant neurotoxicity in some patients. We discuss the potential pathogenesis of these effects and suggest hypothetical therapeutic measures to prevent these complications. Specialists should be aware of these possible complications when administering prophylactic IT liposomal cytarabine in high-grade NHL patients, and additional prospective studies should be conducted to more clearly delineate the frequency and characteristics of these complications.

Introduction: The high incidence of stroke results in significant mortality and disability leading to immense health care costs. These costs lead to socioeconomic, budgetary, and staffing repercussions in developing countries. Improvements in stroke management focus mainly on acute neurological treatment, admission to stroke units, fibrinolytic treatment for ischaemic strokes and rehabilitation processes. Among these, rehabilitation has the longest therapeutic window, can be applied in both ischaemic and haemorrhagic strokes, and can improve functional outcomes months after stroke. Development: Neurologists, because of their knowledge in neuroanatomy, physiopathology, neuropharmacology, and brain plasticity, are in an ideal position to actively participate in the neurorehabilitation process. Several processes have been shown to play a role in determining the efficacy of rehabilitation; time from stroke onset to rehabilitation admission and the duration and intensity of treatment. Conclusions: Neurorehabilitation is a sub-speciality in which neurologists should be incorporated into multidisciplinary neurorehabilitation teams. Early time to rehabilitation admission and greater intensity and duration of treatment are associated with better functional outcomes, lower mortality/institutionalisation, and shorter length of stay. In order to be efficient, a concerted effort must be made to ensure patients receive neurorehabilitation treatment in a timely manner with appropriate intensity to maximize patient outcomes during both inpatient and outpatient rehabilitation. Resumen: Introducción: La enfermedad cerebrovascular o ictus es uno de los motivos más frecuentes de asistencia neurológica urgente, representa una de las primeras causas de muerte e invalidez en los adultos y supone un enorme coste tanto humano como económico. Los avances en el tratamiento del ictus tienen como ejes fundamentales la atención neurológica precoz, el ingreso en las unidades de ictus, la aplicación del tratamiento fibrinolítico en el infarto cerebral y el tratamiento rehabilitador. Entre los diferentes tratamientos, la neurorrehabilitación presenta una ventana terapéutica más amplia, puede aplicarse tanto en ictus isquémicos como hemorrágicos y puede mejorar el pronóstico funcional incluso meses después del ictus. Desarrollo: El neurólogo, por sus conocimientos en neuroanatomía, fisiopatología, neurofarmacología y los procesos de plasticidad cerebral, está en una posición ideal para participar activamente en la neurorrehabilitación. En el proceso de rehabilitación hay una serie de factores que condicionan su eficacia; el tiempo hasta el inicio de la rehabilitación, su duración y la intensidad del tratamiento. Conclusiones: La neurorrehabilitación es una subespecialidad en que el neurólogo puede formarse con el fin de participar en los equipos multidisciplinarios que dirigen el proceso de neurorrehabilitación. El inicio precoz del tratamiento y su intensidad y duración adecuadas conllevan mayor recuperación funcional y menores mortalidad, tasa de institucionalización y estancia hospitalaria. Es imprescindible realizar un esfuerzo de planificación para que los pacientes puedan beneficiarse de un tratamiento neurorrehabilitador específico iniciado precozmente, con la intensidad necesaria y de forma continuada durante el ingreso y posteriormente de forma ambulatoria. Keywords: Stroke, Neurorehabilitation, Dependency, Palabras clave: Ictus, Neurorehabilitación, Dependencia

Intima-media thickness (IMT) is a valid marker for generalized vascular disease whose main risk factors are associated with food habits and lifestyle. A Mediterranean food pattern may have a protective effect on cardiovascular mortality.To assess the relationship between carotid IMT and olive oil consumption.One hundred and ninety nine patients were randomly extracted from 1055 asymptomatic high cardiovascular risk participants at the AP-UNAV recruitment center of the PREDIMED (PREvención con DIeta MEDiterránea) project. Demographic and clinical variables were collected, and a validated semiquantitative food frequency questionnaire (137 items) was administered at the inclusion interview. A B-mode ultrasound imaging technique was used to measure the mean common carotid IMT.The mean age was 67.3 years and 53.3% were women. Energy-adjusted olive oil consumption quintiles were assessed as the main exposure after adjusting for potential dietary and non-dietary confounders. Using continuous carotid IMT as the outcome in an ANCOVA analysis, the adjusted IMT means throughout quintiles showed an inverse association with a plateau after the second quintile, with statistical differences when the adjusted IMT mean of the merged four upper quintiles were compared with the lowest quintile (p0.05). The averaged (both sides) mean IMT of the common carotid was dichotomised and values above the median (0.804 mm) were used to identify carotid atherosclerotic damage. We also found an inverse association of olive oil consumption with high IMT, throughout the second to the fifth quintile as compared with the lowest quintile. The adjusted OR was of 0.08 (95% confidence interval, CI, of 0.02-0.37; p=0.001) after merging the four upper quintiles.The inverse association between the olive oil consumption and the carotid IMT could suggest a protective role of olive oil against the development of carotid atherosclerosis in persons at high cardiovascular risk.

Neuroimaging techniques are necessary for the evaluation of stroke, one of the leading causes of death and neurological impairment in developed countries. The multiplicity of techniques available has increased the complexity of decision making for physicians. We performed a comprehensive review of the literature in English for the period 1965-2005 and critically assessed the relevant publications. The members of the panel reviewed and corrected an initial draft, until a consensus was reached on recommendations stratified according to the European Federation of Neurological Societies (EFNS) criteria. Non-contrast computed tomography (CT) scan is the established imaging procedure for the initial evaluation of stroke patients. However, magnetic resonance imaging (MRI) has a higher sensitivity than CT for the demonstration of infarcted or ischemic areas and depicts well acute and chronic intracerebral hemorrhage. Perfusion and diffusion MRI together with MR angiography (MRA) are very helpful for the acute evaluation of patients with ischemic stroke. MRI and MRA are the recommended techniques for screening cerebral aneurysms and for the diagnosis of cerebral venous thrombosis and arterial dissection. For the non-invasive study of extracranial vessels, MRA is less portable and more expensive than ultrasonography but it has higher sensitivity and specificity for carotid stenosis. Transcranial Doppler is very useful for monitoring arterial reperfusion after thrombolysis, for the diagnosis of intracranial stenosis and of right-to-left shunts, and for monitoring vasospasm after subarachnoid hemorrhage. Currently, single photon emission computed tomography and positron emission tomography have a more limited role in the evaluation of the acute stroke patient.

Vascular dementia (VaD) is the second most common form of dementia after Alzheimer’s disease (AD), and one of the major causes of mental and physical disability in developed countries. As such, the identification and implementation of strategies which prevent the development of the condition or enable improvements in patients with VaD are healthcare objectives of the first order. VaD is now regarded as a combined group of clinical-pathological entities rather than one disease, that is, multiple pathogenic mechanisms and lesion types underlie a cognitive impairment of vascular origin. The clinical diagnosis of VaD is complex and difficult because of the heterogeneous nature of its clinical presentation and progression and the low sensitivity of existing clinical criteria. Moreover, there is growing evidence of the epidemiological significance of mixed forms of dementia, and that ischemic processes may precipitate and exacerbate cognitive impairment in AD. Numerous compounds have been proposed as potentially useful in the treatment of patients with VaD, comprising vasodilatative, antithrombotic, hemorrheological, nootropic, antiserotoninergic and, most recently, antiglutamatergic and cholinergic approaches. In spite of some initially favorable reports based on the use of memantine, donepezil and galantamine, there is as yet no conclusive evidence of a definitive treatment for VaD. Unsatisfactory results from VaD drug trials may be attributed in part to the diversity of the patients included (underlying pathogenic mechanisms, number, type, and location of vascular lesions), and to methodological limitations in the design of the trials (outcome measures, end-points, size, follow-up period). The treatment of modifiable vascular risk factors – hypertension, diabetes mellitus, hypercholesterolemia and heart disease – is an important strategy for the reduction of the risk of dementia, and is likely to slow the progress of cognitive decline.

The control of health expenditure has become one of the main axes of health policy. Cost studies are an indispensable tool for determining the economic impact of disease and for assigning the material and human resources required for the prevention, diagnosis, treatment, and follow-up of patients with different diseases. Cost studies in stroke are necessary due to the increasing incidence of this disease which represents a significant cause of death and invalidity in adults and produces important hospital and social spending, and for which patients must be treated in specialized units (stroke units). The principal cost-determining factors in most studies are those generated by hospital admission (principally hospital stay). Other cost-determining factors include severity, stroke subtype or the fate of patients on discharge. Future health policies aimed at optimizing economic resources must be directed towards reducing hospital stay and minimizing patient disability.

This article represents the update of ‘European Stroke Initiative Recommendations for Stroke Management’, first published in this Journal in 2000. The recommendations are endorsed by the 3 European societies which are represented in the European Stroke Initiative: the European Stroke Council, the European Neurological Society and the European Federation of Neurological Societies.

In developed countries, ischemic stroke is one of the leading causes of death and neurological impairment. The two most important therapeutic approaches in patients with acute cerebral ischemia consist of improving cerebral blood flow and blocking the biochemical and metabolic changes at the ischemic cascade level. The significant advances made in the past decade in the knowledge of the physiopathological mechanisms of cerebral ischemia, and the development of new drugs have given rise to true expectations regarding treatment and the rejection of nihilist attitudes. In the past 15 years, based on the excellent results obtained in experimental models of ischemia, many clinical trials have been conducted with different neuroprotective drugs. The results obtained in most studies have been negative, or the studies were terminated early owing to side effects. However, some drugs (citicoline, clomethiazole, piracetam and ebselen) have shown a certain degree of clinical efficacy, limited to subgroups of patients, and with a narrow therapeutic window, longer-lasting in the case of citicoline. The design of new clinical trials with neuroprotective drugs requires adequate preclinical assessment and the use of the new magnetic resonance techniques for the selection of patients and the assessment of the efficacy of treatment. The new trends in neuroprotection in focal cerebral ischemia and the results of the clinical trials published to date are reviewed.

Shortlasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome is characterized by shortlived (5–240 s), strictly unilateral, periorbital, severe pain attacks, accompanied by ipsilateral conjunctival injection and lacrimation (1). SUNCT syndrome has been classified together with cluster headache (CH) and paroxysmal hemicrania (PH) in the group of trigeminal autonomic cephalalgias (TAC) (1). The temporal pattern usually described in episodic SUNCT is irregular, with the symptomatic periods alternating with remissions in an erratic fashion (2, 3). In contrast, it is widely acknowledged that in episodic CH bouts can occur in the same periods every year (4), particularly during spring and autumn (5), and in some PH patients seasonal variations have been described (6–8). We present a patient with episodic SUNCT syndrome with bouts that occurred twice annually in spring and autumn.

Delirum is a common complication in hospitalized patients and it is characterized by acute disturbances of consciousness, attention, cognition, and perception. Despite the frequency with which it is observed, ischemic stroke is generally considered as an unusual cause of delirium. A subtype of brain embolism is characterized by multiple small emboli in different vascular territories, a condition known as "brain microembolism." Given the high contrast of acute ischemic lesions in diffusion weighted imaging (DWI) this technique is particularly helpful to detect these small infarctions. We present here a patient with pulmonary metastases who was treated with bronchial artery embolization and who subsequently developed delirium due to brain microembolism. The embolic material crossed through pulmonary arteriovenous fistulas, producing multiple areas of cerebral ischemia. The ischemic lesions could be visualized only on DWI, and they affected the periventricular region, caudate nucleus, thalamus, and cerebellum.

Several causes of Brown-Sequard syndrome have been described. Endovascular embolization can be used to treat symptomatic vertebral hemangiomas. We describe a previously undocumented case of Brown-Sequard syndrome followed by endovascular embolization with microcoils of a vertebral hemangioma. We also provide a clinical-radiological correlation of this finding and review the relevant literature. A 39-year-old male was referred to our hospital for endovascular treatment of a right T9 hemivertebral hemangioma with compromise of the spinal canal. Fifteen minutes after the procedure, the patient developed right lower limb weakness and numbness on the left leg. The emergency magnetic resonance imaging (MRI) of the spine showed no abnormalities. Five days later, a new spinal MRI revealed an infarction in the right half of the spinal cord at T6 and T7 level. This stroke was probably caused by a microcoil ended up in the right sulcocommisural artery. One week after surgery, the patient was able to raise the right leg against gravity, but sensory deficit showed no improvement. To the best of our knowledge this is the first case of a Brown-Sequard syndrome related to vertebral hemangioma embolization, a relatively safe technique with no important complications made known until this report. Clinicians should always weight the benefits with the potential devastating complications of this therapeutic option.

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by acute severe thunderclap headaches and evidence of multifocal, segmental, reversible vasoconstrictions of the cerebral arteries. Several precipitating factors have been identified and reported, including the use of recreational substances or sympathomimetic drugs and the postpartum state. Case description: Here we present the case of a woman who developed RCVS after the administration of adrenaline (epinephrine) in the setting of an anaphylactic reaction during antibiotic allergy testing. Discussion: To our knowledge, this is the first reported case of RCVS following the administration of exogenous adrenaline. This case contributes to the understanding of the physiopathological mechanisms underlying reversible cerebral vasoconstriction

Many migraineurs who seek care in headache clinics are refractory to treatment, despite advances in headache therapies. Epidemiology is poorly characterized, because diagnostic criteria for refractory migraine were not available until recently. We aimed to determine the frequency of refractory migraine in patients attended in the Headache Unit in a tertiary care center, according to recently proposed criteria. METHODS: The study population consisted of a consecutive sample of 370 patients (60.8% females) with a mean age of 43 years (range 14-86) evaluated for the first time in our headache unit over a one-year period (between October 2008 and October 2009). We recorded information on clinical features, previous treatments, Migraine Disability Assessment Score (MIDAS), and final diagnosis. RESULTS: Overall migraine and tension-type headache were found in 46.4% and 20.5% of patients, respectively. Refractory migraine was found in 5.1% of patients. In refractory migraineurs, the mean MIDAS score was 96, and 36.8% were medication-overusers. CONCLUSIONS: Refractory migraine is a relatively common and very disabling condition between the patients attended in a headache unit. The proposed operational criteria may be useful in identifying those patients who require care in headache units, the selection of candidates for combinations of prophylactic drugs or invasive treatments such as neurostimulation, but also to facilitate clinical studies in this patient group.

Background. The assessment of carotid intima-media thickness (CIMT) may improve cardiovascular risk prediction. The optimal protocol for CIMT measurement is unclear. CIMT may be measured in the common carotid artery (CCA), carotid bifurcation (CB), and internal carotid artery (ICA), but measurements from CB and ICA are more difficult to obtain. We studied the influence of body mass index (BMI) and atheroma plaques on the capacity to obtain CIMT measurements at different carotid sites. Methods. Using an automatic system, CIMT was measured in 700 subjects aged 45–75, in the near and far walls of CCA, CB, and ICA bilaterally. The presence of atheroma plaques, BMI and vascular risk factors were recorded. Results. CIMT measurements in CCA were possible in all except one subject. It was not possible to obtain CIMT measurements at CB or ICA in 24.1% of normal weight and 58.8% of obese subjects. The likelihood of obtaining CIMT measurement at all carotid sites decreased as the BMI increased. Atheroma plaques in a carotid segment did not preclude CIMT measurement at this site. Conclusions. CIMT measurements in distal carotid segments are more challenging in obese subjects. Measuring CIMT at CCA remains feasible in obese subjects and should be the primary endpoint in these subjects. Nevertheless, CB and ICA measurements, when feasible, would improve risk classification. © 2012 Wiley Periodicals, Inc. J Clin Ultrasound 40:479–485, 2012

Background and objective: To update the ad hoc Committee of the Cerebrovascular Diseases Study Group of The Spanish Neurological Society guidelines on prevention of ischemic stroke (IS) and Transient Ischaemic Attack (TIA). Methods: We reviewed the available evidence on ischaemic stroke and TIA prevention according to aetiological subtype. Levels of evidence and recommendation levels are based on the classification of the Centre for Evidence-Based Medicine. Results: In atherothrombotic IS, antiplatelet therapy and revascularization procedures in selected cases of ipsilateral carotid stenosis (70–99%) reduce the risk of recurrences. In cardioembolic IS (atrial fibrillation, valvular diseases, prosthetic valves and myocardial infarction with mural thrombus) prevention is based on the use of oral anticoagulants. Preventive therapies for uncommon causes of IS will depend on the aetiology. In the case of cerebral venous thrombosis oral anticoagulation is effective. Conclusions: We conclude with recommendations for clinical practice in prevention of IS according to the aetiological subtype presented by the patient. Resumen: Fundamento y objetivo: Actualizar las guías terapéuticas del Comité ad hoc del Grupo de Estudio de Enfermedades Cerebrovasculares de la SEN en el tratamiento preventivo de ictus isquémico (II) y ataque isquémico transitorio (AIT). Métodos: Revisión de evidencias disponibles sobre la prevención del ictus isquémico y AIT en función del subtipo etiológico. Los niveles de evidencia y grados de recomendación se han basado en la clasificación del Centro de Medicina Basada en la Evidencia. Resultados: En el II de origen aterotrombótico reducen el riesgo de recurrencias el tratamiento antiagregante y los procedimientos revascularizadores en casos seleccionados de estenosis carotidea ipsilateral (70-99%). La prevención de II de origen cardioembólico (fibrilación auricular, valulopatías, prótesis valvulares y en infarto de miocardio con trombo mural) se basa en el uso de anticoagulantes orales. En el II de origen inhabitual, las terapias preventivas dependerán dela etiología; en la trombosis venosa cerebral la anticoagulación oral es eficaz. Conclusiones: Se concluye con recomendaciones de práctica clínica en prevención de ictus isquémico y AIT adaptadas al subtipo etiológico de II que ha presentado el paciente. Keywords: Guidelines, Ischaemic stroke, Transient ischemic attack, Prevention, Palabras clave: Guía de práctica clínica, Ictus isquémico, Ataque isquémico transitorio, Prevención

Intracerebral haemorrhage accounts for 10% to 15% of all strokes, however it has a poor prognosis with higher rates of morbidity and mortality. Neurological deterioration is often observed during the first hours from onset, and determines the poor prognosis. Intracerebral haemorrhage, therefore, is a neurological emergency which must be diagnosed and treated properly as soon as possible. In this guide we review the diagnostic procedures and factors that influence the prognosis of patients with intracerebral haemorrhage and we establish recommendations for the therapeutic strategy, systematic diagnosis, acute treatment and secondary prevention for this condition. Resumen: La hemorragia intracerebral sólo representa entre el 10 y el 15% de todos los ictus, sin embargo condiciona un peor pronóstico, con unas tasas más elevadas de morbilidad y mortalidad. Es frecuente que durante las primeras horas tras el inicio de los síntomas se produzca un empeoramiento clínico, lo cual condiciona un peor pronóstico, por lo que la hemorragia intracerebral constituye una emergencia neurológica en la que debe realizarse un diagnóstico y tratamiento adecuado de manera precoz. En esta guía realizamos una revisión de los procedimientos diagnósticos y los factores que influyen en el pronóstico de los pacientes con hemorragia intracerebral y establecemos unas recomendaciones para la estrategia asistencial, sistemática diagnóstica, tratamiento en fase aguda y prevención secundaria en la hemorragia intracerebral. Keywords: Intracerebral haemorrhage, Guidelines, Stroke, Palabras clave: Hemorragia intracerebral, Guías, Ictus

Objective To assess the association between the metabolic syndrome (MetSd) and asymptomatic cardiovascular disease and determine if the MetSd or its single risk factors perform better in discriminating prevalent asymptomatic cardiovascular disease. Methods A total of 880 community-dwelling subjects (423 with and 457 without MetSd according to ATPIII) underwent a physical examination, an echocardiography and an ultrasound examination of carotid arteries and blood and urine samples were collected. Associations between the subclinical organ damage markers and the MetSd were addressed with non-conditional logistic regression. AUCs of ROCs were used to compare the models' ability to discriminate asymptomatic cardiovascular disease. Results The MetSd was independently associated with carotid subclinical atherosclerosis, increased left ventricular mass index and cardiac dysfunction. The MetSd did not discriminate prevalent increased carotid intima–media thickness better than abdominal obesity and impaired fasting glucose [AUC=0.75 (95% CI: 0.71–0.78) and 0.75 (0.71–0.79), respectively; p =0.55]. The MetSd performed worse than abdominal obesity in discriminating increased left ventricular mass index among males younger than 65 years [AUC=0.66 (95% CI: 0.62–0.69) and 0.69 (0.66–0.73), respectively; p =0.02]. No differences between the ability of MetSd or its components in discriminating increased left ventricular mass index were observed among older men or women. The discrimination ability for microalbuminuria for the MetSd or impaired fasting glucose was not statistically different [AUC=0.67 (95% CI: 0.60–0.74) and 0.69 (0.62–0.76), respectively; p =0.18]. Conclusion This study supports the association between the MetSd and asymptomatic cardiovascular disease. The construct of the MetSd might not be better than its single components in addressing cardiovascular risk.

A 64-year-old woman presented with a 6-month history of right-sided continuous headache, without autonomic symptoms and complete response to indomethacin. Clinical examination and structural brain imaging were normal. A diagnosis of hemicrania continua (HC) was made. We sought to determine the brain structures active during the pain in a patient who met all of the diagnostic criteria for HC with the exception of autonomic symptoms. A brain positron emission tomography study was performed during pain, and completely pain-free after indomethacin administration. Comparing the pain with pain-free states, the region of the dorsal pons was significantly activated. There was no activation in the hypothalamus, as previously reported in HC with autonomic symptoms. Although definitive conclusions can not be drawn from a single observation, the lack of autonomic symptoms along with the absence of hypothalamic activation suggests that the clinical presentation may predict the pattern of brain activation in primary headache syndromes.

En los últimos años distintos estudios han puesto de manifiesto un progresivo incremento en la demanda de atención neurológica en los servicios de urgencia. Para analizar la conveniencia de las guardias específicas de neurología o del papel que debe desempeñar el neurólogo en los servicios de urgencia, es preciso plantearse cuestiones como: ¿cuál es la demanda de atención neurológica urgente?, ¿cuáles son las urgencias neurológicas más frecuentes?, ¿quién debe atender las urgencias neurológicas y por qué?, ¿son necesarias las guardias específicas de neurología? Las urgencias neurológicas se sitúan entre el 2,6% y el 14% de las urgencias médicas. Los ictus representan la tercera parte de todas las urgencias neurológicas, mientras que los diagnósticos de enfermedad cerebrovascular aguda, epilepsia y cefalea constituyen el 50% de toda la atención neurológica en los servicios de urgencias. En base a criterios de calidad asistencial y de competencia profesional, la mejor atención del paciente con una urgencia neurológica la proporciona el especialista en neurología. La implantación de guardias específicas de neurología de presencia física durante 24 horas se asocia a una mayor calidad asistencial, mejora la orientación diagnóstica y terapéutica desde que el paciente llega a urgencias, reduce ingresos innecesarios, disminuye el coste de la asistencia neurológica, y potencia el servicio de Neurología.

Las cefaleas son un motivo común de consulta en los servicios de urgencias. La mayoría de los pacientes presentan cefaleas primarias (migraña y cefalea tensional), pero el dolor de cabeza puede ser el síntoma de una enfermedad que comprometa la vida del individuo como la hemorragia subaracnoidea. El abordaje diagnóstico del paciente con cefalea consiste en diferenciar los pacientes con cefalea primaria de los que presentan una etiología secundaria, valorar la necesidad de solicitar exploraciones complementarias y tratar adecuadamente el dolor. La anamnesis permite diferenciar las cefaleas primarias de las secundarias en la mayoría de los casos. Existen una serie de síntomas de alarma que obligan a excluir causas secundarias. En los pacientes con cefalea de inicio súbito, cuando se inicia por encima de los 50 años o en aquellos pacientes con exploración neurológica anormal, debe sospecharse una etiología secundaria.

To the Editor: We read with interest the article recently published by Lynch JR et al.1 The authors report that the knowledge of 1 marker of glial activation (S100β), 2 markers of inflammation (matrix metalloproteinase [MMP]-9 and vascular adhesion molecule [VCAM]) and 1 marker of thrombosis (von Willebrand factor [vWF]) in the first 6 hours can help identify patients with acute cerebral ischemia who could benefit from thrombolytic treatment. Thrombolysis is an effective therapy for ischemic stroke whether administered intravenously within 3 hours or intraarterially within 3 to 6 hours.2 Many stroke patients arrive in emergency rooms too late to receive thrombolysis.3 It is possible to shorten admission delay by using systems for fast identification and transport of acute ischemic stroke patients. The development of a panel of blood-borne biochemical markers may be beneficial for the quick diagnosis of ischemic stroke, as used today in cases of myocardial infarction. These biomarkers should in only minutes differentiate patients with TIA or other conditions causing acute focal neurological deficits from patients with ischemic stroke candidates …

Physicians involved in care of migraine patients should make a considerable effort to educate themselves and their patients. It is fundamental that doctors, patients and their families understand the causes of migraine, and know the different treatment strategies available to improve migraine care. Mallory-Weiss tears are lacerations in the region of the cardioesophageal junction due to vomiting. We report a case of Mallory-Weiss tear secondary to migraine that could had been reasonably avoided had the patient been treated with triptans since the first attack, according to the stratified care strategy. This case illustrates that inadequate management can cause serious medical consequences. It also proves that it is necessary to improve education about migraine for both patients and doctors.