Psychiatric Comorbidity and Emotional Dysregulation in Chronic Tension-Type Headache: A Case-Control Study.

BACKGROUND: Chronic tension-type headache (CTTH) is frequently associated with a psychiatric comorbidity of depression and anxiety. Most studies focus their attention on this association, and only few link CTTH with psycho-affective emotional regulation disorders. OBJECTIVE: To evaluate the association of CTTH with anxiety, depression, positive and negative affectivity, and emotional management in CTTH patients with neither a previous diagnosis of psychiatric disorder nor use of psychoactive drugs or abuse of analgesics. DESIGN: Case-control study. METHODS: Validated scores for state and trait anxiety, depression, positive and negative state and trait affect, cognitive reappraisal, and expressive suppression were assessed in 40 subjects with CTTH and 40 healthy subjects. Associations between CTTH and psychological status were assessed through linear multivariate regression models. RESULTS: CTTH was associated with higher scores for depression (Beta = 5.46, 95% CI: 1.04-9.88), state and trait anxiety (Beta = 12.77, 95% CI: 4.99-20.56 and Beta = 8.79, 95% CI: 2.29-15.30, respectively), and negative state affect (Beta = 5.26, 95% CI: 0.88-9.64). CONCLUSIONS: CTTH is directly associated with depression, anxiety, and negative affectivity signs despite the absence of a previously diagnosed psychiatric disorder or psychopharmacological intake. The recognition of these comorbid and psycho-affective disorders is essential to adapt the emotional management of these patients for better control.

Sensory Thresholds and Peripheral Nerve Responses in Chronic Tension-Type Headache and Neuropsychological Correlation.

Chronic tension-type headache (CTTH) is a common disease with no fully defined pathophysiological processes. We designed a study to value electrophysiological responses in these patients and their correlation with possible psychopathological manifestations in order to deepen understanding of central and peripheral mechanisms of CTTH. In 40 patients with CTTH and 40 healthy controls, we used electrical stimulation to determine sensory threshold (SPT) and pain perception threshold (PPT) and the characteristics of the electrophysiological sensory nerve action potential (SNAP): initial sensory response (ISR) and supramaximal response (SMR). We then calculated the intensity differences between thresholds (IDT), namely SPT-PPT, ISR-SMR and SMR-PPT, and correlated these IDTs with psychological characteristics: trait and state anxiety, depression, and emotional regulation. The SPT, together with the ISR and SMR thresholds, were higher (p < 0.01) in CTTH patients. The SMR-PPT IDT was smaller and correlated with significantly higher indicators of depression, state and trait anxiety, and poorer cognitive reappraisal. CTTH patients have less capacity to recognize non-nociceptive sensory stimuli, greater tendency toward pain facilitation, and a poor central pain control requiring higher stimulation intensity thresholds to reach the start and the peak amplitude of the SNAP. This is consistent with relative hypoexcitability of the Aß nerve fibers in distant regions from the site of pain, and therefore, it could be considered a generalized dysfunction with a focal expression. Pain facilitation is directly associated with psychological comorbidity.

Epidemiological study of mortality in epilepsy in a Spanish population.

PURPOSE: Studies concerning mortality in epilepsy have been performed primarily in Northern-Central Europe and US. The aim of this study was to provide information about mortality in people with epilepsy in Southern European countries. METHOD: We studied a Spanish prevalence and incidence cohort of 2309 patients aged ≥14 years with epilepsy who were treated in an outpatient epilepsy clinic between 2000 and 2013. The deceased were identified through Civil Registries. Causes of death were determined using death certificates, forensic autopsies, hospital reports, family practitioners, and care-givers' records. Standardised mortality ratios (SMRs) were calculated. RESULTS: In a total of 15,865 person-years of follow-up, 152 patients died, resulting in an SMR of 2.11 (95% CI 1.79-2.47), which was higher for those aged 14-24. There was also a high rate of death for symptomatic epilepsies, progressive causes (SMR=6.12, CI 3.50-9.94), and remote causes (SMR=2.62, CI 2.12-3.21). High SMRs were found for all kinds of epilepsy and for respiratory and tumoural causes. Patients who died of epilepsy itself were 12.5%. Sudden unexpected death in epilepsy incidence was 0.44:1000. Death from status epilepticus incidence was 20:100,000. SMRs for external causes were of no statistical significance. CONCLUSIONS: This is the first epidemiological study to examine rate of mortality in epilepsy in a Southern European country. The identified mortality pattern is similar to the one provided by researchers from developed countries. The similarities between our results concerning epilepsy-related deaths and those provided by population-based studies are the result of the scarcely selected character of our study cohort.

Control de la epilepsia en pacientes adultos con esclerosis tuberosa / Control of epilepsy in adult patients with tuberous sclerosis

Introducción. La esclerosis tuberosa es una enfermedad genética cuyas manifestaciones principales son la formación de tumores neuroectodérmicos, que asocia epilepsia secundaria muy frecuentemente. Objetivo. Describir el perfil epiléptico, el control, la frecuencia de crisis y la efectividad del tratamiento en pacientes adultos con esclerosis tuberosa. Pacientes y métodos. Estudio descriptivo en el que se han incluido pacientes adultos (mayores de 14 años) con diagnóstico confirmado de esclerosis tuberosa. Se ha analizado la frecuencia y tipos de crisis. Se ha realizado un estudio y contabilización de los diferentes fármacos antiepilépticos en cada paciente, la frecuencia de uso de cada principio activo y un estudio comparativo entre control de crisis y tipo de terapia. Resultados. De 19 adultos estudiados, tuvieron crisis epilépticas 16 (84%). Presentaron control de crisis completo, crisis esporádicas y crisis frecuentes el 44%, 25% y 31%, respectivamente. Hubo frecuencia de crisis focales, generalizadas y ambas en el 38%, 6% y 25%, respectivamente. Estaban en tratamiento con fármacos antiepilépticos en monoterapia, biterapia y triterapia el 38%, 44% y 19%, respectivamente. El más consumido globalmente fue el levetiracetam, seguido de la carbamacepina y el ácido valproico. En monoterapia, el más frecuente fue la carbamacepina, con mayor proporción de control completo. Conclusiones. La epilepsia en la esclerosis tuberosa es relativamente benigna, y se consigue un aceptable control en la mayoría de casos con un número de antiepilépticos acorde con lo aconsejado en las guías de tratamiento. Se observa estabilidad de lesiones, y no hay malignización en nuestra serie. El bajo número de la muestra limita el estudio, pero se observan proporciones similares de efectividad del tratamiento respecto a otra serie publicada (AU)

Introduction. Tuberous sclerosis is a genetic disease, the main manifestations of which are the formation of neuroectodermal tumours, which are very often associated to secondary epilepsy. Aim. To describe the epileptic profile, control, frequency of seizures and effectiveness of treatment in adult patients with tuberous sclerosis. Patients and methods. The study was descriptive and included adult patients (over 14 years of age) with a confirmed diagnosis of tuberous sclerosis. Both the frequency and type of seizures were analysed. A study was conducted to analyse and record the number of different antiepileptic drugs in each patient and the frequency of use of each active ingredient, and the control of seizures and the type of therapy were also included in another comparative study. Results. Of a total of 19 adults who were studied, 16 of them (84%) had epileptic seizures. Results showed that 44%, 25% and 31% of them presented full control of seizures, sporadic seizures and frequent seizures, respectively. A certain frequency of focal, general and both types of seizures existed in 38%, 6% and 25%, respectively. Likewise, 38%, 44% and 19% of them were under treatment with antiepileptic drugs in monotherapy, bitherapy and tritherapy, respectively. Overall, the most commonly consumed drug was levetiracetam, followed by carbamazepine and valproic acid. In monotherapy the most common was carbamazepine, with a higher proportion of full control. Conclusions. Epilepsy in tuberous sclerosis is relatively benign and an acceptable degree of control is achieved in most cases with a number of the antiepileptic drugs recommended in the treatment guidelines. In our series, we observed stability of involvement and there was no progression to a malignant status. The small number of individuals in the sample limits the study, but the proportions of treatment effectiveness are seen to be similar to those observed in another series published in the literature (AU)

[Control of epilepsy in adult patients with tuberous sclerosis]. / Control de la epilepsia en pacientes adultos con esclerosis tuberosa.

INTRODUCTION: Tuberous sclerosis is a genetic disease, the main manifestations of which are the formation of neuroectodermal tumours, which are very often associated to secondary epilepsy. AIM: To describe the epileptic profile, control, frequency of seizures and effectiveness of treatment in adult patients with tuberous sclerosis. PATIENTS AND METHODS: The study was descriptive and included adult patients (over 14 years of age) with a confirmed diagnosis of tuberous sclerosis. Both the frequency and type of seizures were analysed. A study was conducted to analyse and record the number of different antiepileptic drugs in each patient and the frequency of use of each active ingredient, and the control of seizures and the type of therapy were also included in another comparative study. RESULTS: Of a total of 19 adults who were studied, 16 of them (84%) had epileptic seizures. Results showed that 44%, 25% and 31% of them presented full control of seizures, sporadic seizures and frequent seizures, respectively. A certain frequency of focal, general and both types of seizures existed in 38%, 6% and 25%, respectively. Likewise, 38%, 44% and 19% of them were under treatment with antiepileptic drugs in monotherapy, bitherapy and tritherapy, respectively. Overall, the most commonly consumed drug was levetiracetam, followed by carbamazepine and valproic acid. In monotherapy the most common was carbamazepine, with a higher proportion of full control. CONCLUSIONS: Epilepsy in tuberous sclerosis is relatively benign and an acceptable degree of control is achieved in most cases with a number of the antiepileptic drugs recommended in the treatment guidelines. In our series, we observed stability of involvement and there was no progression to a malignant status. The small number of individuals in the sample limits the study, but the proportions of treatment effectiveness are seen to be similar to those observed in another series published in the literature.

TITLE: Control de la epilepsia en pacientes adultos con esclerosis tuberosa.Introduccion. La esclerosis tuberosa es una enfermedad genetica cuyas manifestaciones principales son la formacion de tumores neuroectodermicos, que asocia epilepsia secundaria muy frecuentemente. Objetivo. Describir el perfil epileptico, el control, la frecuencia de crisis y la efectividad del tratamiento en pacientes adultos con esclerosis tuberosa. Pacientes y metodos. Estudio descriptivo en el que se han incluido pacientes adultos (mayores de 14 anos) con diagnostico confirmado de esclerosis tuberosa. Se ha analizado la frecuencia y tipos de crisis. Se ha realizado un estudio y contabilizacion de los diferentes farmacos antiepilepticos en cada paciente, la frecuencia de uso de cada principio activo y un estudio comparativo entre control de crisis y tipo de terapia. Resultados. De 19 adultos estudiados, tuvieron crisis epilepticas 16 (84%). Presentaron control de crisis completo, crisis esporadicas y crisis frecuentes el 44%, 25% y 31%, respectivamente. Hubo frecuencia de crisis focales, generalizadas y ambas en el 38%, 6% y 25%, respectivamente. Estaban en tratamiento con farmacos antiepilepticos en monoterapia, biterapia y triterapia el 38%, 44% y 19%, respectivamente. El mas consumido globalmente fue el levetiracetam, seguido de la carbamacepina y el acido valproico. En monoterapia, el mas frecuente fue la carbamacepina, con mayor proporcion de control completo. Conclusiones. La epilepsia en la esclerosis tuberosa es relativamente benigna, y se consigue un aceptable control en la mayoria de casos con un numero de antiepilepticos acorde con lo aconsejado en las guias de tratamiento. Se observa estabilidad de lesiones, y no hay malignizacion en nuestra serie. El bajo numero de la muestra limita el estudio, pero se observan proporciones similares de efectividad del tratamiento respecto a otra serie publicada.

[Healthcare management of an epilepsy clinic: factors involved in the demand for health care and clinical situation of patients]. / Gestion sanitaria de una consulta de epilepsia: factores implicados en la demanda asistencial y situacion clinica de los pacientes.

INTRODUCTION: Epilepsy is a chronic illness that requires a long-term periodic follow-up of the patient and this means that as time goes by the number of patients attended increases, with the ensuing added cost for the healthcare system. AIM: To determine the factors involved in the time until an epileptic patient's next visit. PATIENTS AND METHODS: Our sample consisted of a selection of patients who visited the epilepsy clinic at our hospital consecutively during one year. Their clinical situation and relationship with the medical advice they were given, together with the factors involved in the time elapsed until the next visit, were analysed by means of predictive econometric models. RESULTS: There is a clear association between the patient's clinical situation and the modification of the treatment proposed by the neurologist in the previous visit. The factors involved in the time until the next visit were the frequency of seizures, adverse side effects from medicines -above all those that affect cognition- and the medical advice given to the patient. Polytherapy, psychoaffective disorders or the patient's social situation were not found to be significant. CONCLUSIONS: Follow-up visits in a specific epilepsy clinic improves the patient's situation. This is the first analysis of the demand for healthcare in patients with epilepsy conducted by means of econometric methods and from a mixed physician-patient perspective. Since the factors that determine the time until the next visit can be modified, the number of visits per year could be reduced, thus improving patients' clinical situation. We suggest a greater amount of time should be spent per visit so as to be able to have a bearing on it and thereby cut costs in the long term.

TITLE: Gestion sanitaria de una consulta de epilepsia: factores implicados en la demanda asistencial y situacion clinica de los pacientes.Introduccion. La epilepsia es una enfermedad cronica que implica un seguimiento periodico del paciente a largo plazo, lo que supone un aumento del numero de pacientes visitados con el tiempo y, por tanto, un coste al sistema sanitario. Objetivo. Determinar los factores implicados en el tiempo para la siguiente visita de un paciente epileptico. Pacientes y metodos. Seleccion de pacientes durante un ano que acuden consecutivamente a consulta de epilepsia de nuestro hospital. Se analiza su situacion clinica y relacion con el consejo medico dado, y los factores implicados en el tiempo transcurrido hasta la siguiente visita mediante modelos econometricos predictivos. Resultados. Existe una clara asociacion entre la situacion clinica del paciente y la modificacion del tratamiento propuesta por el neurologo en la visita anterior. Los factores implicados en el tiempo hasta la siguiente visita fueron frecuencia de crisis, efectos adversos medicamentosos, sobre todo los que afectan a la cognicion, y consejo medico al paciente. No resultaron significativos la politerapia, los trastornos psicoafectivos ni la situacion social del paciente. Conclusiones. El seguimiento en una consulta especifica de epilepsia mejora la situacion del paciente. Se trata del primer analisis de demanda asistencial en pacientes con epilepsia realizado mediante metodos econometricos y desde una perspectiva mixta medico-paciente. Dado que los factores que determinan el tiempo para la siguiente visita son modificables, podria disminuir el numero de visitas al ano, mejorando la situacion clinica de los pacientes. Proponemos una mayor duracion por visita para poder incidir en ello y reducir costes a largo plazo.

Gestión sanitaria de una consulta de epilepsia: factores implicados en la demanda asistencial y situación clínica de los pacientes / Healthcare management of an epilepsy clinic: factors involved in the demand for health care and clinical

Introducción. La epilepsia es una enfermedad crónica que implica un seguimiento periódico del paciente a largo plazo, lo que supone un aumento del número de pacientes visitados con el tiempo y, por tanto, un coste al sistema sanitario. Objetivo. Determinar los factores implicados en el tiempo para la siguiente visita de un paciente epiléptico. Pacientes y métodos. Selección de pacientes durante un año que acuden consecutivamente a consulta de epilepsia de nuestro hospital. Se analiza su situación clínica y relación con el consejo médico dado, y los factores implicados en el tiempo transcurrido hasta la siguiente visita mediante modelos econométricos predictivos. Resultados. Existe una clara asociación entre la situación clínica del paciente y la modificación del tratamiento propuesta por el neurólogo en la visita anterior. Los factores implicados en el tiempo hasta la siguiente visita fueron frecuencia de crisis, efectos adversos medicamentosos, sobre todo los que afectan a la cognición, y consejo médico al paciente. No resultaron significativos la politerapia, los trastornos psicoafectivos ni la situación social del paciente. Conclusiones. El seguimiento en una consulta específica de epilepsia mejora la situación del paciente. Se trata del primer análisis de demanda asistencial en pacientes con epilepsia realizado mediante métodos econométricos y desde una perspectiva mixta médico-paciente. Dado que los factores que determinan el tiempo para la siguiente visita son modificables, podría disminuir el número de visitas al año, mejorando la situación clínica de los pacientes. Proponemos una mayor duración por visita para poder incidir en ello y reducir costes a largo plazo (AU)

Introduction. Epilepsy is a chronic illness that requires a long-term periodic follow-up of the patient and this means that as time goes by the number of patients attended increases, with the ensuing added cost for the healthcare system. Aim. To determine the factors involved in the time until an epileptic patient’s next visit. Patients and methods. Our sample consisted of a selection of patients who visited the epilepsy clinic at our hospital consecutively during one year. Their clinical situation and relationship with the medical advice they were given, together with the factors involved in the time elapsed until the next visit, were analysed by means of predictive econometric models. Results. There is a clear association between the patient’s clinical situation and the modification of the treatment proposed by the neurologist in the previous visit. The factors involved in the time until the next visit were the frequency of seizures, adverse side effects from medicines –above all those that affect cognition– and the medical advice given to the patient.Polytherapy, psychoaffective disorders or the patient’s social situation were not found to be significant. Conclusions. Follow-up visits in a specific epilepsy clinic improves the patient’s situation. This is the first analysis of the demand for healthcare in patients with epilepsy conducted by means of econometric methods and from a mixed physicianpatient perspective. Since the factors that determine the time until the next visit can be modified, the number of visits per year could be reduced, thus improving patients’ clinical situation. We suggest a greater amount of time should be spent per visit so as to be able to have a bearing on it and thereby cut costs in the long term (AU)

A propósito de un caso de parálisis periódica hipopotasémica tirotóxica / A case report of thyrotoxic hypokalemic periodic paralysis

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Prevalence and clinical characteristics of epilepsy in the South of Spain.

PURPOSE: Epilepsy is a common neurological disorder found in all societies. There are extensive epidemiologic studies of different European areas. However, not much information about the South-West of Europe exists. In Málaga, Health Care is free and there are only two public hospitals with Neurological Services that assist Eastern or Western areas depending on the case. The purpose is to estimate the epidemiology in Málaga through a hospital-based study and compare it to the other European studies. METHODS: Review on the hospital data base and gathering of consecutive patients with a diagnosis of active epilepsy served by the Epilepsy outpatient clinic in Virgen de la Victoria Hospital throughout a year. RESULTS: 2 281 patients fulfilled the criteria and 515 patients were recruited in order to study the epidemiologic characteristics. Male gender and focal onset seizures predominate (75.5%). Medium age 40. 58% of patients have been seizure-free in the last year. Known etiology in half of the patients. 54% of patients are treated with monotherapy. Valproic acid is the commonest drug. Other epidemiologic and demographic important data are provided. Data are analysed and compared to other European studies. CONCLUSIONS: Prevalence rate in Western Málaga is 4.79 cases/1000 inhabitants. Characteristics of the sample are similar to those of other European studies. Although this is a hospital-based study, the particular characteristics of the Health Care System in our region enable us to gather real data concerning epidemiology and prevalence.

Patología psicógena en la planta de neurología. Revisión de los últimos 10 años de hospitalización en un servicio de tercer nivel / Psychogenic pathology in the neurology unit. A review of the hospital admissions over the last 10 years in a third-level service

Introducción. Los trastornos psicógenos son frecuentes en la práctica neurológica, y, en ocasiones, obligan a hospitalizar al paciente para descartar una patología orgánica potencialmente grave. Objetivo. Conocer el perfil de los pacientes con diagnóstico al alta de trastorno psicógeno que ingresan en la planta de neurología de un hospital español. Pacientes y métodos. Se revisaron los informes de alta de neurología de los últimos 10 años, se seleccionaron los pacientes que tenían un diagnóstico de ‘no organicidad’ y se recogieron hasta un total de 28 variables demográficas, epidemiológicas y clínicas. Resultados. Sesenta y cuatro pacientes (el 1% de los hospitalizados) tenían diagnóstico de psicogenicidad, con una edad media de 40 años. La proporción de sexos era de 1 a 1, y la estancia media, de 10 días. Los síntomas mixtos eran los más habituales (50%), seguidos de los motores aislados (22%). Los posibles diagnósticos neurológicos de ictus isquémico y enfermedad desmielinizante fueron la mayoría de los casos. Sólo se consultó un 25% a psiquiatría durante el ingreso. En un 11% de los casos hubo historia posterior de intento de autolisis y en ocho casos (12,5%) se llegó a un diagnóstico ambulatorio de organicidad gracias al seguimiento evolutivo (‘error’ en el diagnóstico provisional al alta). Conclusión. Este trabajo dibuja por vez primera en nuestro medio el perfil del paciente psicógeno que ingresa en la planta de neurología, y examina su manejo hospitalario y su seguimiento ambulatorio. Se recuerda la necesidad de no menospreciaresta patología y de generar una vía ordenada para su manejo, siempre multidisciplinar y con el protagonismo del neurólogo y el psiquiatra

Introduction. Psychogenic disorders are frequently seen in neurological practice and sometimes make it necessary to hospitalise the patient in order to rule out a potentially severe organic pathology. Aim. To determine the profile of patients with a discharge diagnosis of psychogenic disorder that are admitted to the neurology unit of a Spanish hospital. Patients and methods. The neurology unit discharge abstracts from the last 10 years were reviewed and the patients who were diagnosed with ‘non-organicity’ were selected; a total of 28 demographic, epidemiological and clinical variables were also collected. Results. A total of 64 patients (1% of those admitted to hospital), with a mean age of 40 years, had a diagnosis of psychogenicity. The proportion between sexes was one to one and the mean length of stay in hospital was 10 days. Mixed symptoms were the most usual (50%), followed by isolated motor symptoms (22%). Most cases consisted of possible neurological diagnoses of ischaemic stroke and demyelinating disease. Only 25% of cases consulted psychiatry during hospitalisation. In 11% of cases there was a later history of suicide attempts and in eight cases (12.5%) an ambulatory diagnosis of organicity was reached thanks to the developmental follow-up (‘error’ in the provisional diagnosis on discharge). Conclusions. This study traces, for the first time in our setting, the profile of the psychogenic patient who is admitted to the neurology unit and examines their hospital management and their outpatient follow-up. We highlight the need not to underestimate this pathological condition and to generate an ordered way of managing it, which should always be multidisciplinary with the leading roles played by the neurologist and the psychiatrist

[Psychogenic pathology in the neurology unit. A review of the hospital admissions over the last 10 years in a third-level service]. / Patología psicógena en la planta de neurología. Revisión de los últimos 10 años de hospitalización en un servicio de tercer nivel.

INTRODUCTION: Psychogenic disorders are frequently seen in neurological practice and sometimes make it necessary to hospitalise the patient in order to rule out a potentially severe organic pathology. AIM: To determine the profile of patients with a discharge diagnosis of psychogenic disorder that are admitted to the neurology unit of a Spanish hospital. PATIENTS AND METHODS: The neurology unit discharge abstracts from the last 10 years were reviewed and the patients who were diagnosed with 'non-organicity' were selected; a total of 28 demographic, epidemiological and clinical variables were also collected. RESULTS: A total of 64 patients (1% of those admitted to hospital), with a mean age of 40 years, had a diagnosis of psychogenicity. The proportion between sexes was one to one and the mean length of stay in hospital was 10 days. Mixed symptoms were the most usual (50%), followed by isolated motor symptoms (22%). Most cases consisted of possible neurological diagnoses of ischaemic stroke and demyelinating disease. Only 25% of cases consulted psychiatry during hospitalisation. In 11% of cases there was a later history of suicide attempts and in eight cases (12.5%) an ambulatory diagnosis of organicity was reached thanks to the developmental follow-up ('error' in the provisional diagnosis on discharge). CONCLUSIONS: This study traces, for the first time in our setting, the profile of the psychogenic patient who is admitted to the neurology unit and examines their hospital management and their outpatient follow-up. We highlight the need not to underestimate this pathological condition and to generate an ordered way of managing it, which should always be multidisciplinary with the leading roles played by the neurologist and the psychiatrist.

Síndrome de la arteria de Percheron: variabilidad clínica y diagnóstico diferencial / Percheron artery syndrome: variability in presentation and differential diagnosis

Introducción. El infarto talámico paramediano bilateral sincrónico, asimilado habitualmente al infarto de la arteria de Percheron, se considera infrecuente y de difícil diagnóstico clínico, basándose su caracterización en la descripción original y en pequeñas series. Objetivo. Caracterizar el infarto talámico paramediano bilateral sincrónico clínicamente mediante aglutinación de casos e identificar las claves de su dificultad diagnóstica precoz. Pacientes y métodos. Seis casos de nuestro centro, y 115 mediante búsqueda sistemática y lectura crítica de artículos, cumplieron los criterios de infarto talámico paramediano bilateral sincrónico. Se analizaron las variables edad, género, factores de riesgo vascular, etiología, alteración y fluctuaciones del nivel de consciencia, necesidad de intubación, trastorno cognitivo-conductual, alteraciones pupilares, otra focalidad neurológica y afectación del troncoencéfalo en neuroimagen. Resultados. En nuestra serie, destacan el trastorno del nivel de consciencia (n = 5), sus fluctuaciones (n = 3) y la demora diagnóstica (siete días, con resonancia magnética en cuatro pacientes). Sólo en uno se objetivó lesión bitalámica en la tomografía computarizada inicial. El análisis conjunto determinó edad media de 61 años; predominio masculino (58%); presencia de factores de riesgo vascular en el 77%; etiología dominante, la cardioembólica (el 34% entre los especificados); afectación del sensorio en el 75% (intubación en el 7% y fluctuaciones en el 16,5%), cognitivo-conductual en el 43%, oculomotora en el 73%, pupilar en el 31%, y de otro tipo en el 67%; y lesión del tronco especificada en el 37%. Conclusiones. El infarto talámico paramediano bilateral sincrónico constituye un síndrome de presentación variable, con una tomografía computarizada inicial de baja sensibilidad, y que precisa una resonancia magnética cerebral para su tipificación. Ello explica la dificultad diagnóstica y la probable infraestimación de su frecuencia (AU)

Introduction. Synchronous bilateral paramedian thalamic stroke (SBPTS), usually equated to Percheron artery infarction, is considered to be uncommon and difficult to diagnose clinically. Its characterization is based on the original description plus a few small series. Aim. To characterize SBPTS clinically by collecting cases and identifying the key difficulties for an early diagnosis. Patients and methods. Six cases at our centre plus another 115 located by systematic literature search and critical reading of articles fulfilled the criteria for SBPTS. An analysis was made of the variables age, gender, vascular risk factors, aetiology, alterations and fluctuations of consciousness, need for intubation, cognitive-behavioural disorders, pupillary changes, other neurological focal disorders and brainstem involvement on imaging studies. Results. Of note in our series were disorders of consciousness (n = 5), their fluctuations (n = 3) and the diagnostic delay (seven days, with MRI in four patients). In only one case was a bilateral thalamic lesion seen on the initial CT. Joint analysis of all the cases showed a mean age of 61 years, a predominance of men (58%), the presence of vascular risk factors in 77%, a mainly cardioembolic aetiology (34% among those that were specified), sensory involvement in 75% (intubation in 7% and fluctuations in 16.5%), cognitive-behavioural disorders in 43%, oculomotor in 73%, pupillary in 31%, other in 67% and specified brainstem lesion in 37%. Conclusions. The SBPTS syndrome has a variable presentation with a low sensitivity on the initial CT, requiring brain MRI for typification. This explains the diagnostic difficulty and the fact that its frequency is probably underestimated (AU)

Sí­ndrome de cabeza cada asociado a distonía craneofacial / Dropped head syndrome associated to craniofacial dystonia

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[Does Jeavons syndrome exist? A report of a series of 10 cases]. / Existe el síndrome de Jeavons? Aportación de una serie de 10 casos.

INTRODUCTION: Jeavons syndrome (JS) or eyelid myoclonia (EM) with or without absences is an epileptic syndrome characterised by EM on closing the eyes, in bright environments, which coincides on the electroencephalogram with brief generalised polyspike and polyspike-wave discharges at 3-6 Hz. Photoparoxysmal response is associated in photostimulation. The ILAE recognises EM as a special type of myoclonic seizures, yet it still does not include JS as a separate condition in its classification of epileptic syndromes. AIM: The aim of this article is to report on a series of 10 cases, with special attention given to their clinical-electro-encephalo-graphic description. We believe it is a condition that is not infrequent, but one which is underdiagnosed. Moreover, we also want to highlight the pros and cons determining the fact that today there is still controversy about whether or not it should be recognised as an independent epileptic syndrome. PATIENTS AND METHODS: We conducted a retrospective study of 10 cases collected over the period between 2002 and 2009 in our adult Epilepsy Unit. RESULTS: All our patients fulfilled JS criteria. There is a predominance of females (n = 8), with the existence of cases of idiopathic generalised epilepsy in the family (n = 5), which are associated with clonic-tonic generalised seizures in nearly all of them (n = 9) and are well controlled with treatment in monotherapy, although the EM persist in three cases. The results of a neurological exploration and neuroimaging are normal in all cases. CONCLUSIONS: JS is a photosensitive epilepsy that is not easily confused with other conditions; nevertheless, there can be an overlap with other idiopathic generalised epilepsy syndromes that lead to their being underdiagnosed. Genetics and functional neuroimaging will determine whether it is an independent condition or not.

¿Existe el síndrome de Jeavons? Aportación de una serie de 10 casos / Does Jeavons syndrome exist? A report of a series of 10 cases

Introducción. El síndrome de Jeavons (SJ), o mioclonías palpebrales (MP) sin/con ausencias, es un síndrome epiléptico caracterizado por MP al cierre ocular, en ambiente luminoso, coincidentes en el electroencefalograma con descargas breves y generalizadas de polipuntas y polipunta-onda a 3-6 Hz. Asocia respuesta fotoparoxística en la fotoestimulación. La Liga Internacional Contra la Epilepsia (reconoce las MP como un tipo especial de crisis mioclónicas; sin embargo, sigue sin incluir el SJ como entidad propia en su clasificación de los síndromes epilépticos. Objetivo. Este artículo pretende aportar una serie de 10 casos y hacer hincapié en su descripción clínica y electroencefalográfica. Creemos que se trata de una entidad no infrecuente pero infradiagnosticada, y reseñamos los pros y los contras que determinan que hoy día siga la controversia sobre su reconocimiento como síndrome epiléptico independiente. Pacientes y métodos. Estudio restrospectivo de 10 casos recogidos durante el período 2002-2009 en nuestra Unidad de Epilepsia de adultos. Resultados. Todos nuestros pacientes cumplen criterios de SJ. Hay un predominio del sexo femenino (n = 8), con la existencia de casos familiares de epilepsia generalizada idiopática (n = 5), que asocian crisis generalizadas tonicoclónicas en casi todos (n = 9) y se encuentran bien controlados con tratamiento en monoterapia, aunque persisten las MP en tres. Todos presentan una exploración neurológica y una neuroimagen normales. Conclusión. El SJ es una epilepsia fotosensible dificilmente confundible con otras entidades; sin embargo, puede existir un solapamiento con otros síndromes de epilepsia generalizada idiopática que determine su infradiagnóstico. La genética y la neuroimagen funcional determinarán si se trata de una entidad independiente (AU)

Introduction. Jeavons syndrome (JS) or eyelid myoclonia (EM) with or without absences is an epileptic syndrome characterised by EM on closing the eyes, in bright environments, which coincides on the electroencephalogram with brief generalised polyspike and polyspike-wave discharges at 3-6 Hz. Photoparoxysmal response is associated in photostimulation. The ILAE recognises EM as a special type of myoclonic seizures, yet it still does not include JS as a separate condition in its classification of epileptic syndromes. Aim. The aim of this article is to report on a series of 10 cases, with special attention given to their clinical-electroencephalographic description. We believe it is a condition that is not infrequent, but one which is underdiagnosed. Moreover, we also want to highlight the pros and cons determining the fact that today there is still controversy about whether or not it should be recognised as an independent epileptic syndrome. Patients and methods. We conducted a retrospective study of 10 cases collected over the period between 2002 and 2009 in our adult Epilepsy Unit. Results. All our patients fulfilled JS criteria. There is a predominance of females (n = 8), with the existence of cases of idiopathic generalised epilepsy in the family (n = 5), which are associated with clonic-tonic generalised seizures in nearly all of them (n = 9) and are well controlled with treatment in monotherapy, although the EM persist in three cases. The results of a neurological exploration and neuroimaging are normal in all cases. Conclusions. JS is a photosensitive epilepsy that is not easily confused with other conditions; nevertheless, there can be an overlap with other idiopathic generalised epilepsy syndromes that lead to their being underdiagnosed. Genetics and functional neuroimaging will determine whether it is an independent condition or not (AU)

Calretinin interneurons are early targets of extracellular amyloid-beta pathology in PS1/AbetaPP Alzheimer mice hippocampus.

Specific neuronal networks are preferentially affected in the early stages of Alzheimer's disease (AD). The distinct subpopulations of hippocampal inhibitory GABAergic system have been shown to display differential vulnerability to neurodegeneration in AD. We have previously reported a substantial loss of SOM/NPY interneurons, whereas those expressing parvalbumin were unaltered, in the hippocampus of 6 month-old PS1/AbetaPP transgenic mice. In the present study, we now investigated the pathological changes of hippocampal calretinin (CR) interneurons in this PS1/AbetaPP model from 2 to 12 months of age. The total number of CR-immunoreactive inhibitory cells was determined by stereology in CA1 and CA2/3 subfields. Our findings show a substantial decrease (35%-45%) of CR-positive interneurons in both hippocampal subfields of PS1/AbetaPP mice at very early age (4 months) compared to age-matched control mice. This decrease was accompanied by a reduced CR mRNA content as determined by quantitative RT-PCR. However, the number of another hippocampal CR-positive population (belonging to Cajal-Retzius cells) was not affected. The selective early loss of CR-interneurons was parallel to the appearance of extracellular Abeta deposits, preferentially in CR-axonal fields, and the formation of dystrophic neurites. This specific GABAergic subpopulation plays a crucial role in the generation of synchronous rhythmic activity in hippocampus by controlling other interneurons. Therefore, early alterations of hippocampal inhibitory functionality in AD, caused by select CR-cells neurodegeneration, could result in cognitive impairments seen in initial stages of the disease.

Germinoma con extensión tetraventricular: relevancia de la biopsia en los tumores germinales del sistema nervioso / No disponible

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Extracellular amyloid-beta and cytotoxic glial activation induce significant entorhinal neuron loss in young PS1(M146L)/APP(751SL) mice.

Here we demonstrated that extracellular, not intracellular, amyloid-beta (Abeta) and the associated cytotoxic glial neuroinflammatory response are major contributors to early neuronal loss in a PS1xAPP model. A significant loss of principal (27%) and SOM/NPY (56-46%) neurons was found in the entorhinal cortex at 6 months of age. Loss of principal cells occurred selectively in deep layers (primarily layer V) whereas SOM/NPY cell loss was evenly distributed along the cortical column. Neither layer V pyramidal neurons nor SOM/NPY interneurons displayed intracellular Abeta immunoreactivity, even after formic acid retrieval; thus, extracellular factors should be preferentially implicated in this selective neurodegeneration. Amyloid deposits were mainly concentrated in deep layers at 4-6 months, and of relevance was the existence of a potentially cytotoxic inflammatory response (TNFalpha, TRAIL, and iNOS mRNAs were upregulated). Moreover, non-plaque associated activated microglial cells and reactive astrocytes expressed TNFalpha and iNOS, respectively. At this age, in the hippocampus of same animals, extracellular Abeta induced a non-cytotoxic glial activation. The opposite glial activation, at the same chronological age, in entorhinal cortex and hippocampus strongly support different mechanisms of disease progression in these two regions highly affected by Abeta pathology.