Recomendaciones de uso de anticuerpos monoclonales para el tratamiento de la migraña del grupo de consenso de navarra y aragón

Sr. Editor: La migraña es una patología neurológica que afecta al 14% de la población general (18% en mujeres); representa la segunda causa de discapacidad en el mundo en pacientes de mediana edad y genera importantes gastos socio-sanitarios1. Las causas de la migraña no son bien conocidas pero existe una activación del denominado sistema trigémino-vascular (STV) que causa la liberación de diferentes mediadores proinflamatorios y vasodilatadores, entre los que destaca el péptido regulador del gen de la calcitonina (CGRP)2. El CGRP es un péptido que participa en la transmisión del estímulo doloroso, cuyos niveles se elevan durante las crisis de migraña y en el periodo intercrítico3. El diagnóstico de la migraña es clínico y está basado en unos criterios de consenso que forman parte de la Clasificación Internacional de Cefaleas4. De forma general, la migraña se divide en migraña episódica (ME) y migraña crónica (MC). La ME es aquella en la que la cefalea aparece menos ..

Infiltración con onabotulinumtoxinA y bloqueos en el paciente con cefalea y neuralgia: recomendaciones de seguridad frente a la infección por SARS-CoV-2

Introducción La infección por SARS-CoV-2 y la situación de pandemia hacen necesario un cambio en nuestra práctica clínica, en relación con la adopción de nuevas estrategias que permitan la asistencia integral de los pacientes con cefalea y neuralgia. Material y métodos Ante la elevada capacidad de transmisión del virus, determinados procedimientos, como la infiltración de onabotulinumtoxinA y la realización de bloqueos anestésicos, deben realizarse adoptando medidas que garanticen un nivel adecuado de seguridad. Resultados Nuestro objetivo es presentar una serie de recomendaciones basadas en las medidas establecidas por nuestro sistema sanitario para evitar el contagio. Diferenciaremos entre aquellas medidas relacionadas con el paciente y el facultativo, con la técnica, con la infraestructura (sala de espera y consulta) y con la gestión asistencial. Introduction: The COVID-19 pandemic has given rise to the need for changes in clinical practice, with new strategies to enable integrated care for patients with headache and neuralgia. Material and methods: Given the high spreadability of SARS-CoV-2, new safety measures are required in such procedures as onabotulinumtoxinA infiltration and nerve blocks. Results: We present a series of recommendations based on the measures implemented to prevent infection in our healthcare system. We differentiate between measures related to patients and physicians, to technique, to infrastructure (waiting room and consultation), and to healthcare management

Familial association of genetic generalised epilepsy with limb-girdle muscular dystrophy through a mutation in CAPN3

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..

Real world preventative drug management of migraine among Spanish neurologists

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

Estimation of the Economic Burden and Labor Impact of Migraine in Spain: Results from the Spanish Atlas of Migraine Survey 2018

P170 Objectives: To estimate the average annual cost per patient and theimpact on work t of migraine in Spain. Material and Method: This is a prospective, online, anonymous, cross-sectional survey, conducted between June and September2017, promoted by the Spanish Association of Patients with Headache(AEPAC) within the framework of the Spanish Atlas of Migraine2018. People who completed the survey answered questions in relationto their migraine. A distinction was made between chronic migraine(CM) and episodic migraine (EM), considering the monthlyheadache days declared by patients. The economic burden of migrainewas evaluated: direct health costs (including visits to specialists, medical tests, emergency visits, hospital admissions andmedication), indirect costs (lost labor productivity), and those assumedby the migraineur. The labor consequences of migraine overthe last year were analyzed. Chi-square and Mann- Whitney testswere used as contrast tests. Ethics Approval: A central ethics reviewboard approved the study design. Results: 1, 281 people with migraine participated in the survey, 34.2%with CM, 88.2% women, with an average age of 37.3 (SD 11.5). Thedirect health costs for the last year were estimated at €3, 847.29 forCM and €964.19 for EM (p<0, 001). The costs assumed by the patientin the last year were €1, 609.89 for CM and €878.04 for EM (p<0.001).The indirect cost was estimated at €7, 464.83 for CM and €3, 199.15for EM (p <0.001). The total average cost per patient/year rised to€12, 922.01 for CM and €5, 041.41 for EM (p<0.001). Regarding the jobstatus: 62.2% with EM and 49.0% with CM were working, 2.6% withEM and 9.1% with CM were on sick leave and 12.2% with EM and16.8% with CM were unemployed (p <0.05). In the last year, becauseof migraine, 17.8% of patients with EM and 27.2% with CM (p<0.01)requested days of leave or leave of absence, and reduced their workinghours 8.5% with EM and 11.1% CM (p=0.270). Labor efficiencywas reduced in 61.1% of patients with EM and 65.7% with CM(p=0.257). Conclusion: Migraine represents an important economic burden inSpain, particularly in patients with CM. Migraine causes importantproductivity losses resulting from absenteeism, presentism, decreasingthe working hours and the probabilities to keep working, and itsimpact is significantly greater in CM

Sensory function in cluster headache: an observational study comparing the symptomatic and asymptomatic sides

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 rst, 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 signi cantly decreased PPT in the rst (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 rst 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 asymp- tomatic side

Real-world preventative drug management of Chronic Migraine among Spanish Neurologists

O42 Background: In migraine, the therapeutic preventive drug arsenal is varied. Whenprescribing both Guidelines and patient characteristics are taken intoaccount. In Spain, the use of preventive therapies seems to beheterogeneous.The objective of this study was to evaluate real-life clinical prescribingpractice amongst neurologists in Spain Methods: Observational descriptive study done with a survey by Neurologistsof the Spanish Neurological Society (SEN). Neurologists who participatedwere divided into Headache Specialists or not. The followingdata was collected: socio-demographic data, ; preventive treatmentand choices different migraine sub-types, and their personal perceptionof efficacy and tolerability to different drugs. Results: We analyzed 152 surveys from neurologists around our country.From them: 43.4% were female, 53.3% <40 years, and 34.9% were interestedin headache .In regards to preventive treatment choice; in chronic migraine topiramate(57%) amytriptiline (17.9%) and beta-blockers (14.6%), whereasin episodic migraine the preferred drugs were beta-blockers (47.7%), topiramate (21.5%) and amytriptiline (13.4%).Regarding perceived efficacy, topiramate was considered the bestoption in chronic migraine (42.7%) followed by onabotulinumtoxinA(25.5%) and amitryptiline (22.4%). In episodic migraine, neurologistpreferred topiramate (43.7%) and beta-blockers (30.3%).Regarding the duration of preventive therapy when improvementwas achieved, when treating episodic migraine 43.5% of the surveyedneurologists recommended 3 months and 39.5% preferred 6months. When they treated chronic migraine, 20.4% of neurologistsrecommended 3 months, 42.1% 6 months, 12.5% 9 months and22.4% preferred to maintain treatment during 12 months.When considering onabotulinumtoxinA treatment, the number ofprior therapeutical failures was cero in 7.2% of surveyed, one in5.9%, two in 44.1%, three in 30.9%, and four or more in 11.9%. Theincrease of OnabotulinumtoxinA dose up to 195 UI was consideredby 51% of neurologists after a first ineffective procedure, by 42.2% after two injections, and by 83% after a third infiltration. Surveyedcolleagues admitted to take into account in their decisions mainlypatient comorbidities (70.2%) rather than guidelines (13.9%). Conclusions: Initial management of Migraine among Spanish Neurologists is madewith the preventative drugs which are considered as first choices inmost of the guidelines. Management of episodic migraine differedfrom chronic migraine, both in the order or drugs and the perceptionof the most effective therapy

Epilepsy in elderly patients: does age of onset make a difference?

Introduction: Epilepsy is most frequent in children and elderly people. Today\u27s population is ageing and epilepsy prevalence is increasing. The type of epilepsy and its management change with age. Methods: We performed a retrospective, observational study comparing patients aged ≥ 65 years with epilepsy diagnosed before and after the age of 65, and describing epilepsy characteristics and comorbidities in each group. Results: The sample included 123 patients, of whom 61 were diagnosed at \u3c 65 years of age (group A), 62 at ≥ 65 of age (group B). Sex distribution was similar in both groups, with 39 men (62.9%) in group A and 37 (60.7%) in group B. Mean age was 69.97 ± 5.6 years in group A and 77.29 ± 6.73 in group B. The most common aetiology was unknown in group A (44.3%, n = 27) and vascular in group B (74.2%, n = 46). History of stroke was present in 12 patients from group A (19.7%) and 32 (51.6%) in group B. Antiepileptic drugs were prescribed at lower doses in group A. Statistically significant differences were found between groups for history of ischaemic stroke, cognitive impairment, psychiatric disorders, and diabetes mellitus; degree of dependence; and number of antiepileptic drugs. Conclusion: Age of onset ≥ 65 years is closely related to cardiovascular risk factors; these patients require fewer antiepileptic drugs and respond to lower doses. Some cases initially present as status epilepticus

Epilepsia mioclónica juvenil: Pronóstico a largo plazo y retirada de tratamiento

Fundamento. La epilepsia mioclónica juvenil (EMJ) es un síndrome epiléptico clásico que carece de consenso pleno sobre la posibilidad de suspensión de tratamiento con fármacos antiepilépticos (FAE). Método. Estudio observacional, retrospectivo de una serie de pacientes con diagnóstico de EMJ de más de 20 años de evolución, atendiendo a la evolución de aquellos con retirada de FAE. Resultados. Se estudiaron veinte pacientes (edad media 44, 1 años, 55% hombres) con EMJ de 30 años de evolución media y edad media al inicio de 14, 2 años. El tipo de crisis motora más frecuente fue la combinación de mioclónicas y tónico-clónicas (70%); el 60% de los pacientes llevan más de cinco años libres de crisis. A cuatro pacientes (20%) se les suspendieron los FAE, a dos de ellos con edad media 23 años y un tiempo medio libre de crisis de 7, 5 años, que recayeron, y a los otros dos con edad media 39 años y tras 23, 5 años libres de crisis, que llevan dos y nueve años sin crisis en la actualidad. Conclusiones. Existe la posibilidad de suspender FAE en pacientes con EMJ con libertad de crisis mantenida en el tiempo. Sugerimos tener en cuenta la edad de suspensión y la existencia previa de un tiempo prolongado de libertad de crisis. Background. Juvenile myoclonic epilepsy (JME) is a classic epileptic syndrome that lacks consensus on the possibility of suspending treatment with antiepileptic drugs (AD). Method. Retrospective observational study of a series of patients diagnosed with JME with 20 years or more of evolution, focusing on those with withdrawal from AD. Results. The study involved twenty patients (average age 44.1 years, 55% men) with JME of 30 years average evolution and average age at its outset of 14.2 years. The most frequent type of motor crisis was the com-bination of myoclonic and tonic-clonic seizures (70%); 60% of the patients have been free of seizures for more than five years. Four patients (20%) were withdrawn from AD, two of them with an average age of 23 years and an average time free of seizures of 7.5 years, who relapsed, and the other two with an average age of 39 years and following 23.5 years free of seizures, who currently have been without seizures for two and nine years. Conclusions. The possibility of withdrawing AD in patients with JME who have been free of seizures over an extended time seems feasible. We suggest taking into account age at withdrawal and prior existence of a prolonged period of time free of seizures