Utilidad del índice kappa en el diagnóstico de esclerosis múltiple y validación de valores de referencia en la Comunidad de Aragón / Usefulness of the kappa index in the diagnosis of multiple sclerosis and validation of reference values in the Community of Aragon

Introducción: La determinación de bandas oligoclonales (BOC) en el líquido cefalorraquídeo es esencial para el diagnóstico de esclerosis múltiple (EM). El índice kappa es un biomarcador con una sensibilidad y una especificidad comparables a las de las BOC. El objetivo de este trabajo fue estudiar su utilidad y calcular el punto de corte óptimo. Material y métodos. Estudio observacional de muestras recibidas en el servicio de inmunología de referencia para Aragón entre enero de 2019 y junio de 2020. Se determinó el valor del índice kappa y el índice IgG. Se trazaron y compararon curvas ROC frente a la detección de BOC y el diagnóstico de EM. Se calcularon valores de sensibilidad y especificidad y el punto de corte óptimo. Resultados: Se analizaron 181 pacientes (59,7%, mujeres; edad media, 47,62 ± 17,8 años). El grupo de EM demostró valores estadísticamente superiores de índice kappa (66,62 frente a 0,02; p < 0,001). En el análisis de curvas ROC, el índice kappa demostró un área bajo la curva superior al índice IgG en la detección de BOC+ (0,93 frente a 0,83; p < 0,002) y el diagnóstico de EM (0,91 frente a 0,83; p < 0,021). El punto de corte óptimo del índice kappa para la detección de BOC fue 5,02 (sensibilidad y especificidad, 0,92) y 7,58 para el diagnóstico de EM (sensibilidad, 0,85; especificidad, 0,94). Conclusiones: El índice kappa es un biomarcador útil en el diagnóstico de la EM. Su determinación es un proceso automático y rápido, con valores de sensibilidad y especificidad superiores al índice IgG. Todavía se debe llegar a un consenso sobre el punto de corte óptimo para su positividad, si bien, en nuestro medio, un punto de corte de 5,02 parece razonable.(AU)

INTRODUCTION: Detection of oligoclonal bands (OCB) in CSF is essential in the diagnosis of multiple sclerosis (MS). Kappa-index is a promising biomarker, with sensitivity and sensibility values comparable to those of OCB. Our objective was to investigate kappa-index utility in the diagnosis work-up for MS and to determine the optimal cut-off point for our population. Material and methods. Observational study of consecutive samples received in the Immunology lab department in a third level hospital between January 2019 to June 2020. Kappa-index and IgG index were measured. ROC analysis for screening of OCB and for diagnosis of MS was conducted. Sensibility, specificity and the optimal cut-off points were determined. RESULTS: 181 samples were included (59.7% females; age, 47.62 ± 17.8 years). Patients in the EM group presented significantly higher kappa-index level than patients without an EM diagnosis (66.62 versus 0.02; p < 0.001). ROC analysis reported a better area under the curve for the kappa-index than the IgG index for the detection of BOC (0.93 versus 0.83; p < 0.002) and the diagnosis of EM (0.91 versus 0.83; p < 0.021). An optimal cut-off point of 5.02 was determined for the detection of BOC (sensibility and specificity, 0.92) and of 7.58 for the diagnosis of EM (sensibility, 0.85; specificity, 0.94). CONCLUSIONS: Kappa-index is an useful biomarker in the diagnosis of MS. Its specificity and sensibility are superior to the IgG index. The optimal cut-off point has yet to be determined, but for our population a cut-off of 5.02 seems reasonable.(AU)

[Usefulness of the kappa index in the diagnosis of multiple sclerosis and validation of reference values in the Community of Aragon]. / Utilidad del índice kappa en el diagnóstico de esclerosis múltiple y validación de valores de referencia en la Comunidad de Aragón.

INTRODUCTION: Detection of oligoclonal bands (OCB) in CSF is essential in the diagnosis of multiple sclerosis (MS). Kappa-index is a promising biomarker, with sensitivity and sensibility values comparable to those of OCB. Our objective was to investigate kappa-index utility in the diagnosis work-up for MS and to determine the optimal cut-off point for our population. MATERIAL AND METHODS: Observational study of consecutive samples received in the Immunology lab department in a third level hospital between January 2019 to June 2020. Kappa-index and IgG index were measured. ROC analysis for screening of OCB and for diagnosis of MS was conducted. Sensibility, specificity and the optimal cut-off points were determined. RESULTS: 181 samples were included (59.7% females; age, 47.62 ± 17.8 years). Patients in the EM group presented significantly higher kappa-index level than patients without an EM diagnosis (66.62 versus 0.02; p < 0.001). ROC analysis reported a better area under the curve for the kappa-index than the IgG index for the detection of BOC (0.93 versus 0.83; p < 0.002) and the diagnosis of EM (0.91 versus 0.83; p < 0.021). An optimal cut-off point of 5.02 was determined for the detection of BOC (sensibility and specificity, 0.92) and of 7.58 for the diagnosis of EM (sensibility, 0.85; specificity, 0.94). CONCLUSIONS: Kappa-index is an useful biomarker in the diagnosis of MS. Its specificity and sensibility are superior to the IgG index. The optimal cut-off point has yet to be determined, but for our population a cut-off of 5.02 seems reasonable.

TITLE: Utilidad del índice kappa en el diagnóstico de esclerosis múltiple y validación de valores de referencia en la Comunidad de Aragón.Introducción. La determinación de bandas oligoclonales (BOC) en el líquido cefalorraquídeo es esencial para el diagnóstico de esclerosis múltiple (EM). El índice kappa es un biomarcador con una sensibilidad y una especificidad comparables a las de las BOC. El objetivo de este trabajo fue estudiar su utilidad y calcular el punto de corte óptimo. Material y métodos. Estudio observacional de muestras recibidas en el servicio de inmunología de referencia para Aragón entre enero de 2019 y junio de 2020. Se determinó el valor del índice kappa y el índice IgG. Se trazaron y compararon curvas ROC frente a la detección de BOC y el diagnóstico de EM. Se calcularon valores de sensibilidad y especificidad y el punto de corte óptimo. Resultados. Se analizaron 181 pacientes (59,7%, mujeres; edad media, 47,62 ± 17,8 años). El grupo de EM demostró valores estadísticamente superiores de índice kappa (66,62 frente a 0,02; p menor de 0,001). En el análisis de curvas ROC, el índice kappa demostró un área bajo la curva superior al índice IgG en la detección de BOC+ (0,93 frente a 0,83; p menor de 0,002) y el diagnóstico de EM (0,91 frente a 0,83; p menor de 0,021). El punto de corte óptimo del índice kappa para la detección de BOC fue 5,02 (sensibilidad y especificidad, 0,92) y 7,58 para el diagnóstico de EM (sensibilidad, 0,85; especificidad, 0,94). Conclusiones. El índice kappa es un biomarcador útil en el diagnóstico de la EM. Su determinación es un proceso automático y rápido, con valores de sensibilidad y especificidad superiores al índice IgG. Todavía se debe llegar a un consenso sobre el punto de corte óptimo para su positividad, si bien, en nuestro medio, un punto de corte de 5,02 parece razonable.

[Impact of the COVID-19 pandemic on the training of Spanish neurology residents]. / Repercusión de la pandemia por COVID-19 en la formación de los residentes de neurología españoles.

Introduction: The COVID-19 pandemic has transformed medical practice and severely disrupted the training of medical residents worldwide. The Spanish Society of Neurology conducted a study to assess its impact on the training of neurology residents in Spain. Methods: We performed a descriptive, cross-sectional study through a survey distributed by e-mail to all neurology residents belonging to the Society. The survey included questions on demographic variables, care activity, and personal and educational impact of the pandemic, as well as respondents' expectations for the future of their work in the post-pandemic era. Results: Of 422 surveys sent, we received a total of 152 responses (36%); 79 respondents (52%) were women and 73 (48%) were men. By year of residency, 51 respondents (33.6%) were in the fourth year, 45 (29.6%) in the third year, 28 (18.4%) in the second year, and 28 (18.4%) in the first year. A total of 139 respondents (90.8%) reported changes in hospital activity, and 126 (82.8%) considered the situation to have had a negative impact on their training, with 99 (64.7%) having lost non-recoverable rotations. Sixty-six percent of respondents (n=101) expressed a desire to extend their residency period. Conclusions: The pandemic has had an extremely severe impact on all areas of the health system, with trainee physicians being one of the most affected groups. Among neurology residents, the crisis has caused significant shortcomings in their training and clinical activities, through the suspension of specific rotations. A high percentage of respondents wished to extend the residency period.

[Knowledge of cerebrovascular disease in the population of Zaragoza]. / Conocimiento de la enfermedad vascular cerebral en la poblacion de Zaragoza.

INTRODUCTION: Acute cerebrovascular disease is a major cause of disability and mortality, with important socio-economic and health impacts. Early stroke care can reduce mortality and improve prognosis. Right now, we can apply treatments in the acute phase, with capacity to reverse the symptoms, but few patients who benefit not reach the hospital soon. One reason for this delay is the lack of recognition of symptoms and poor perception of gravity. AIM: To find out the knowledge of the public about the stroke. SUBJECTS AND METHODS: The sample analyzed was the population of Sector III of Zaragoza, with random selection. The tool used was a telephone survey structured. We analyzed 583 surveys. RESULTS: 63.5% of respondents did not know any symptoms of stroke. 48% do not know any vascular risk factor. Only 9% recognized at least two symptoms and two risk factors. Regarding the attitude, 56% act properly against 44% who do not. Multivariate analysis showed that the factors more related to knowledge are the cultural level and young age. Living in town, and female, they are associated with the best attitude. CONCLUSIONS: Knowledge about stroke is poor, with a low sense of urgency. The factors most associated with the best knowledge are young age and cultural level.

TITLE: Conocimiento de la enfermedad vascular cerebral en la poblacion de Zaragoza.Introduccion. El ictus es una de las principales causas de invalidez y mortalidad en nuestra sociedad, con importantes repercusiones socioeconomicas y sanitarias. La asistencia precoz puede mejorar el pronostico de los pacientes. Actualmente, existen tratamientos en fase aguda, que consiguen reperfundir el tejido isquemico en riesgo y revertir la sintomatologia, pero son pocos los pacientes que se benefician, por el retraso en su atencion, debido a la falta de reconocimiento de los sintomas y la escasa percepcion de gravedad. Objetivo. Analizar el conocimiento de la poblacion de nuestra area de salud sobre el ictus. Sujetos y metodos. La muestra analizada ha sido la poblacion del sector III de la provincia de Zaragoza, con seleccion aleatoria. La herramienta utilizada ha sido una encuesta telefonica estructurada (total de 583). Resultados. Un 63,5% de los encuestados desconoce los sintomas del ictus, y un 48%, los factores de riesgo vascular. Solo un 9% reconoce al menos dos sintomas y dos factores de riesgo. En cuanto a la actitud, un 56% actuaria correctamente frente a un 44% que no. El analisis multivariante mostro que los factores mas relacionados con el conocimiento fueron el nivel cultural y la edad joven. Vivir en un pueblo y sexo femenino se relacionaron con la mejor actitud. Conclusiones. El conocimiento del ictus es escaso, con una baja percepcion de urgencia. Los factores que implican un mejor conocimiento son la edad joven y el nivel cultural alto.

[Study of the diagnostic agreement on headaches between neurology and primary care]. / Estudio de concordancia diagnostica en cefalea entre neurologia y atencion primaria.

INTRODUCTION: Headache as a symptom is a very common disease and one of the main reasons for consultation in primary care. AIM: To analyze the characteristics of patients referred from primary care to general neurology whose chief complaint was headache and/or neuralgia and diagnostic agreement. PATIENTS AND METHODS: Cross-sectional study of all patients referred from primary care; demographic/clinical variables were collected and diagnostic hypothesis by primary care and general neurology were compared by determining their agreement. RESULTS: 2,514 were referred from primary care patients (588 of them on a preferential basis); in 378 cases the reason for consultation was headache and/or neuralgia (average 42.46 years; 77.8% female). In 139 patients it was established only a semiological diagnostic and other episodic migraine predominated (49.79%), chronic tension headache (18.41%) and trigeminal neuralgia (12.13%). Since general neurology, the most common diagnoses were, respectively, 33.86%, 24.05% and 18.67%. A compatible kappa coefficient of 0.543 (p < 0.05) with a moderate agreement when considering only those patients referred from primary care to a specific diagnosis was obtained. CONCLUSIONS: Headaches are a very common reason for consultation in primary care (15%). The diagnostic agreement is moderate in our health sector so it is necessary to design training programs to help outline the criteria for referral to specialists and improve care for our patients.

TITLE: Estudio de concordancia diagnostica en cefalea entre neurologia y atencion primaria.Introduccion. La cefalea como sintoma es una patologia frecuente y uno de los principales motivos de consulta por parte de atencion primaria. Objetivo. Analizar las caracteristicas de los pacientes derivados desde atencion primaria a la consulta de neurologia general con cefalea o neuralgia como motivo de consulta, y la concordancia diagnostica. Pacientes y metodos. Estudio descriptivo transversal de todos los pacientes remitidos desde atencion primaria; se recogieron variables demograficas/clinicas y se compararon las hipotesis diagnosticas de atencion primaria y neurologia, determinando su concordancia. Resultados. Se remitieron desde atencion primaria 2.514 pacientes (588 de ellos con caracter preferente); en 378 casos el motivo de la consulta fue cefalea o neuralgia (42,46 años de media; el 77,8%, mujeres). En 139 pacientes se establecio tan solo un diagnostico semiologico y en el resto predominaron la migraña episodica (49,79%), la cefalea tensional cronica (18,41%) y la neuralgia del trigemino (12,13%). Desde neurologia, los diagnosticos mas frecuentes fueron, respectivamente, 33,86%, 24,05% y 18,67%. Se obtuvo un coeficiente kappa de 0,543 (p < 0,05), compatible con una concordancia moderada al considerar solo los pacientes remitidos desde atencion primaria con un diagnostico concreto. Conclusiones. Las cefaleas constituyen un motivo de consulta desde atencion primaria muy frecuente (15%). La concordancia diagnostica es moderada en nuestro sector sanitario, por lo que es necesario diseñar programas de formacion que ayuden a perfilar los criterios de derivacion al especialista y mejorar la atencion a nuestros pacientes.

[A study of sexual function in migraine and cluster headache]. / Estudio de la funcion sexual en la migrana y la cefalea en racimos.

INTRODUCTION: The sexual erectile dysfunction are common symptoms and many chronic diseases whose diagnosis will determine the therapeutic management of patients. AIM: To assess sexual function in men with migraine or cluster headache (CH) compared with a control group. SUBJECTS AND METHODS: Cross-sectional study of 34 patients with migraine, 31 patients with CH and 60 control subjects less than 46 years old. Erectile dysfunction was assessed using the International Index of Erectile Dysfunction (IIEF). Emotional state was evaluated using the Beck Depression Inventory. RESULTS: The average score on the IIEF was 68.41 ± 10.09, 64.26 ± 5.73 and 59.33 ± 15.89 in the control group, migraine and CH respectively (p = 0.041) being the significant difference between controls and CH (p = 0.036). In the group of migraine patients, three patients had mild and one moderate erectile dysfunction. In the group of patients with CH, twelve scored in rank of mild erectile dysfunction, and two met the criteria for severe erectile dysfunction (p < 0.05). By analyzing differences in the different domains of IIEF, they were significant in the domain of overall satisfaction (p = 0.015) between the control group and patients with CH (p = 0.012). CONCLUSIONS: In our study we found a higher frequency of and involvement in sexual function in terms of overall satisfaction in patients with migraine and CH. We believe that the evaluation of sexual function in this type of headache should be integrated into our clinical practice.

TITLE: Estudio de la funcion sexual en la migrana y la cefalea en racimos.Introduccion. La disfuncion erectil y sexual son sintomas comunes a muchas enfermedades cronicas cuyo diagnostico va a condicionar el manejo terapeutico de los pacientes. Objetivo. Valorar la funcion sexual en varones con migrana o cefalea en racimos (CR) comparandola con un grupo control. Sujetos y metodos. Estudio descriptivo transversal de 34 pacientes con migrana, 31 pacientes con CR y 60 sujetos control de edad inferior a 46 anos. La disfuncion erectil se valoro con el indice internacional de disfuncion erectil (IIEF), y el estado emocional, mediante el inventario de depresion de Beck. Resultados. La puntuacion media en el IIEF fue de 68,41 ± 10,09, 64,26 ± 5,73 y 59,33 ± 15,89 en los grupos control, con migrana y con CR, respectivamente (p = 0,041), y la diferencia es significativa entre controles y con CR (p = 0,036). En el grupo de pacientes migranosos, tres pacientes tenian disfuncion erectil leve, y uno, moderada. En el grupo de pacientes con CR, 12 puntuaron en rango de disfuncion erectil leve y dos cumplieron criterios de disfuncion erectil grave (p < 0,05). Al analizar las diferencias en los diferentes dominios del IIEF, estas fueron significativas en el dominio de satisfaccion global (p = 0,015) entre el grupo control y los pacientes con CR (p = 0,012). Conclusiones. Hemos encontrado una mayor frecuencia de disfuncion erectil y afectacion de la funcion sexual en terminos de satisfaccion global en los pacientes con migrana y con CR. Creemos que la valoracion de la funcion sexual en este tipo de cefaleas debe integrarse en nuestra practica clinica habitual.

Psicosis epiléptica periictal, una causa de psicosis reversible / Epileptic peri-ictal psychosis, a reversible cause of psychosis

Introducción: Las psicosis epilépticas se dividen respecto de su relación con las crisis en periictales e interictales. Las psicosis periictales tienen una estrecha relación temporal con las crisis epilépticas y ocurren antes (preictales), durante (ictales) o después de las mismas (postictales). Generalmente, tienen un inicio y final agudo, corta duración y una remisión completa, con riesgo de recurrencia. Las psicosis interictales o crónicas no guardan relación temporal con las crisis epilépticas. Existe otro tipo de psicosis epilépticas que se relaciona con la respuesta al tratamiento de la epilepsia: psicosis epiléptica por fenómeno de normalización forzada (psicosis alternativa) y dentro de esta se encuentra la psicosis epiléptica secundaria a cirugía de la epilepsia. Aunque se ha generalizado la combinación de antiepilépticos y neurolépticos para su manejo, no existen unas pautas estandarizadas de tratamiento en las psicosis epilépticas. Casos clínicos: Presentamos 5 casos de psicosis epilépticas periictales y remarcamos la excelente respuesta al tratamiento con levetiracetam. Consiguiendo un buen control tanto de las crisis como de los episodios psicóticos. Este fármaco resultó inocuo al asociarlo con neurolépticos en nuestros pacientes y no se precisaron dosis elevadas de estos últimos. Conclusiones: La diferenciación de los estados psicóticos asociados con la epilepsia según la relación temporal con las crisis epilépticas tiene utilidad clínica y pronóstica, dado que aporta aspectos importantes respecto al tratamiento y a la evolución de la enfermedad. El tratamiento de los trastornos mentales periictales o agudos se basa en el control de las crisis epilépticas, mientras que el tratamiento de los interictales o crónicos guarda más similitud con el de los trastornos de origen puramente psiquiátrico. El control estricto de las crisis puede, además de mejorar la calidad de vida del paciente y su discapacidad, prevenir el desarrollo de una psicosis interictal, por lo que consideramos que sería necesario establecer un protocolo de tratamiento para estos casos (AU)

Introduction: Epileptic psychoses are categorised as peri-ictal and interictal according to their relationship with the occurrence of seizures. There is a close temporal relationship between peri-ictal psychosis and seizures, and psychosis may present before (preictal), during (ictal) or after seizures (postictal). Epileptic psychoses usually have acute initial and final phases, with a short symptom duration and complete remission with a risk of recurrence. There is no temporal relationship between interictal or chronic psychosis and epileptic seizures. Another type of epileptic psychosis is related to the response to epilepsy treatment: epileptic psychosis caused by the phenomenon of forced normalisation (alternative psychosis), which includes epileptic psychosis secondary to epilepsy surgery. Although combination treatment with antiepileptic and neuroleptic drugs is now widely used to manage this condition, there are no standard treatment guidelines for epileptic psychosis. Clinical cases: We present 5 cases of peri-ictal epileptic psychosis in which we observed an excellent response to treatment with levetiracetam. Good control was achieved over both seizures and psychotic episodes. Levetiracetam was used in association with neuroleptic drugs with no adverse effects, and our patients did not require high doses of the latter. Conclusions: Categorising psychotic states associated with epilepsy according to their temporal relationship with seizures is clinically and prognostically useful because it provides important information regarding disease treatment and progression. The treatment of peri-ictal or acute mental disorders is based on epileptic seizure control, while the treatment of interictal or chronic disorders has more in common with managing disorders which are purely psychiatric in origin. In addition to improving the patient’s quality of life and reducing disability, achieving strict control over seizures may also prevent the development of interictal psychosis. For this reason, we believe that establishing a treatment protocol for such cases is necessary (AU)

Epileptic peri-ictal psychosis, a reversible cause of psychosis.

INTRODUCTION: Epileptic psychoses are categorised as peri-ictal and interictal according to their relationship with the occurrence of seizures. There is a close temporal relationship between peri-ictal psychosis and seizures, and psychosis may present before (preictal), during (ictal) or after seizures (postictal). Epileptic psychoses usually have acute initial and final phases, with a short symptom duration and complete remission with a risk of recurrence. There is no temporal relationship between interictal or chronic psychosis and epileptic seizures. Another type of epileptic psychosis is related to the response to epilepsy treatment: epileptic psychosis caused by the phenomenon of forced normalisation (alternative psychosis), which includes epileptic psychosis secondary to epilepsy surgery. Although combination treatment with antiepileptic and neuroleptic drugs is now widely used to manage this condition, there are no standard treatment guidelines for epileptic psychosis. CLINICAL CASES: We present 5 cases of peri-ictal epileptic psychosis in which we observed an excellent response to treatment with levetiracetam. Good control was achieved over both seizures and psychotic episodes. Levetiracetam was used in association with neuroleptic drugs with no adverse effects, and our patients did not require high doses of the latter. CONCLUSIONS: Categorising psychotic states associated with epilepsy according to their temporal relationship with seizures is clinically and prognostically useful because it provides important information regarding disease treatment and progression. The treatment of peri-ictal or acute mental disorders is based on epileptic seizure control, while the treatment of interictal or chronic disorders has more in common with managing disorders which are purely psychiatric in origin. In addition to improving the patient's quality of life and reducing disability, achieving strict control over seizures may also prevent the development of interictal psychosis. For this reason, we believe that establishing a treatment protocol for such cases is necessary.

Macropsia, micropsia, alestesia y discromatopsia tras hemorragia intraparenquimatosa occipital / Macropsia, micropsia, allesthesia and dyschromatopsia after occipital intraparenchymal haemorrhage

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Estudio sobre el conocimiento de la población acerca del ictus y de los factores de riesgo vascular / Study into what the population knows about strokes and vascular risk factors

Introducción. La aplicación de tratamientos en fase aguda del ictus obliga a reducir al máximo el tiempo de llegada de los pacientes al hospital. Uno de los factores de demora en la atención es el desconocimiento de la enfermedad por la población. Sujetos y métodos. Nuestro objetivo es analizar el conocimiento que tiene la población del ictus y de los factores de riesgo vascular, así como la actitud ante éste. Para ello hemos realizado entrevistas a pacientes que acuden a consultas de neurología y a sus familiares. Resultados. Han sido 386 entrevistas, 158 mujeres y 228 hombres, con una edad media de 52 años. 230 sujetos (59%) no conocen el término ictus, aunque sí reconocen otros, como infarto o embolia. 175 (45%) no conocen ningún síntoma de infarto cerebral o responden incorrectamente. El síntoma más reconocido (86%) es la pérdida de fuerza en un hemicuerpo. Un 32% de los entrevistados no toma una actitud correcta ante los síntomas del ictus y, si se tratase de un ataque isquémico transitorio, un 25% no lo consideraría urgente. 198 (51%) no conocen ningún factor de riesgo vascular. De los que responden correctamente, el más nombrado es la hipertensión arterial. Los factores relacionados de forma independiente con un mejor conocimiento de la enfermedad son el nivel cultural alto y la edad joven. Conclusión. Existe un escaso conocimiento en la población del ictus y los factores de riesgo vascular. Son necesarias campañas informativas para mejorar la actitud de la población ante la enfermedad (AU)

Introuction. The application of treatments in the acute phase of a stroke makes it necessary to reduce as far as possible the time required for patients to reach hospital. One of the factors associated with delayed care is the population’s scant knowledge about the disease. Subjects and methods. Our aim is to analyse what the population knows about strokes and vascular risk factors, as well as their attitude towards it. To do so, we interviewed both patients who visited neurology departments and their relatives. Results. Altogether 386 interviews were carried out, with 158 females and 228 males and a mean age of 52 years. Two hundred and thirty subjects (59%) were unfamiliar with the Spanish term ictus, although they had heard of others, such as infarto or embolia. One hundred and seventy-five (45%) did not know any symptoms of a completed stroke, or answered incorrectly. The most widely recognised symptom (86%) is (partial) paralysis in one side of the body. Almost a third (32%) of the interviewees would not take a correct attitude if faced with symptoms of a stroke and if it were a transient ischaemic attack, 25% would not think it was serious. One hundred and ninety-eight (51%) did not know any vascular risk factors. Of those who answered correctly, the most frequently named factor is arterial hypertension. The factors related independently with a better knowledge of the disease are a high educational level and being young. Conclusions. The general population knows little about strokes and vascular risk factors. Information campaigns are needed to improve the population’s attitude towards this disease (AU)

[Study into what the population knows about strokes and vascular risk factors]. / Estudio sobre el conocimiento de la población acerca del ictus y de los factores de riesgo vascular.

INTRODUCTION: The application of treatments in the acute phase of a stroke makes it necessary to reduce as far as possible the time required for patients to reach hospital. One of the factors associated with delayed care is the population's scant knowledge about the disease. SUBJECTS AND METHODS: Our aim is to analyse what the population knows about strokes and vascular risk factors, as well as their attitude towards it. To do so, we interviewed both patients who visited neurology departments and their relatives. RESULTS: Altogether 386 interviews were carried out, with 158 females and 228 males and a mean age of 52 years. Two hundred and thirty subjects (59%) were unfamiliar with the Spanish term ictus, although they had heard of others, such as infarto or embolia. One hundred and seventy-five (45%) did not know any symptoms of a completed stroke, or answered incorrectly. The most widely recognised symptom (86%) is (partial) paralysis in one side of the body. Almost a third (32%) of the interviewees would not take a correct attitude if faced with symptoms of a stroke and if it were a transient ischaemic attack, 25% would not think it was serious. One hundred and ninety-eight (51%) did not know any vascular risk factors. Of those who answered correctly, the most frequently named factor is arterial hypertension. The factors related independently with a better knowledge of the disease are a high educational level and being young. CONCLUSIONS: The general population knows little about strokes and vascular risk factors. Information campaigns are needed to improve the population's attitude towards this disease.

[Characteristics of Guillain-Barré syndrome in the healthy area III of Aragon Country]. / Características del síndrome de Guillain-Barré en el área III de salud de la Comunidad Autónoma de Aragón.

INTRODUCTION: Guillain-Barré Syndrome is an acute immune-mediated inflammatory polyneuropathy characterized by flaccid paresia with arreflexia, changeable sensitive disorder and albuminocytologic dissociation in the cerebrospinal fluid (CSF). PATIENTS AND METHODS: We conducted a retrospective study of 30 GBS patients diagnosed in Hospital Clínico de Zaragoza between 1999 and 2005. Annual incidence, seasonal distribution, preceding acute infection; clinical, electrophysiological and electrocardiographic (ECG) data and evolution were all evaluated. RESULTS: The incidence was 1.56/100000 inhabitants/year. We observed an upward tendency during winter months. The rates of incidence were higher in men (66.7%) and increased with age in both sexes. Main clinical presentation form was paraparesia of lower limbs followed on cranial nerve palsy. Immunoglobulin therapy was received by 62.5% of patients. The rate of death was 10%. Thirteen per cent of patients had dysautonomia. Electrocardiographic abnormalities were present in 37% of patients. CONCLUSIONS: GBS incidence in Aragon Country is similar to that found in other studies. An increase with age and an upward tendency during the winter months was observed. High percentage of abnormalities in ECG but the majority of patients was asymptomatic.

Características del síndrome de Guillain-Barré en el área III de salud de la Comunidad Autónoma de Aragón / Characteristics of Guillain-Barré syndrome in the healthy area III of Aragon Country

Introducción: el síndrome de Guillain-Barré (SGB) es una polineuropatía inflamatoria autoinmune caracterizada por la presencia de parálisis fláccida con arreflexia, trastorno sensorial variable y disociación albúmino-citológica en el LCR.Pacientes y métodos: estudio retrospectivo de 30 pacientes diagnosticados de SGB entre 1999 y 2005 en el Hospital Clínico de Zaragoza. Se evaluó la incidencia anual, distribución estacional y regional, antecedentes de infección, características clínicas, neurofisiológicas y electrocardiográficas (ECG). Resultados: la incidencia fue de 1,56/100000 habitantes/año. Mayor frecuencia de eventos en invierno sin alcanzar significación estadística. Mayor frecuencia en sexo masculino (66,7%) e incremento de la incidencia con la edad en ambos sexos. La forma de presentación más frecuente fue la paraparesia de extremidades inferiores seguida de la afectación de pares craneales. El 62,5% recibió tratamiento inmunomodulador. La mortalidad fue del 10%. El 13% presentó alteraciones autonómicas. Alteraciones electrocardiográficas en el 37% de los pacientes. Conclusiones: la incidencia del SGB en Aragón es similar a la que se encontró en otras series. Observamos un aumento de la incidencia con la edad y predilección por los meses de invierno. Elevado porcentaje de pacientes con anomalías ECG mayoritariamente asintomáticas

Introduction: Guillain-Barré Syndrome is an acute immune-mediated inflammatory polyneuropathy characterized by flaccid paresia with a rreflexia, change able sensitive disorder and albumino cytologic dissociation in the cerebrospinal fluid (CSF). Patients and methods: We conducted a retrospective study of 30 GBS patients diagnosed in Hospital Clínico de Zaragoza between 1999 and 2005. Annual incidence, seasonal distribution, preceding acute infection; clinical, electrophysiological and electrocardiographic (ECG) data and evolution were all evaluated. Results: The incidence was 1.56/100000 inhabitants/year. We observed an upward tendency during winter months. The rates of incidence were higher in men (66.7%) and increased with age in both sexes. Main clinical presentation form was paraparesia of lower limbs followed on cranial nerve palsy. Immunoglobulin therapy was received by 62.5%of patients. The rate of death was 10%. Thirteen per cent of patients haddys autonomia. Electrocardiographic abnormalities were present in 37% of patients. Conclusions: GBS incidence in Aragon Country is similar to that found in other studies. An increase with age and an upward tendency during the winter months was observed. High percentage of abnormalities in ECG but the majority of patients was asymptomatic

Factores pronósticos y supervivencia en la enfermedad de neurona motora / Pronostic factors and survival in motor neuron disease

Introducción. La esclerosis lateral amiotrófica (ELA), la más común de las enfermedades de neurona motora (ENM) del adulto, se presenta combinando signos de afectación de neurona motora superior (NMS) e inferior (NMI), con una elevada mortalidad. Otras ENM menos frecuentes son la atrofia muscular progresiva (AMP) y la esclerosis lateral primaria (ELP).Objetivos. Describir datos demográficos, formas de presentación clínica y evolución de pacientes con ENM buscando factores pronósticos.Métodos. Estudio retrospectivo de una serie de pacientes con ENM, registrando datos demográficos, variables clínicas y mortalidad-supervivencia, analizados mediante paquete estadístico SPSS 6.1.2.Resultados. Son 54 pacientes con ENM (50 ELA, 3 AMP y 1 ELP), 30 (55,6%) hombres y 24 (44,4%) mujeres. Formas de inicio bulbar en 13 casos (24,1%) y espinal en 41 (75,9%). Edad media al inicio: 60 +- 14 (29-82 años); más tardía en las mujeres (p< 0,05), y en los pacientes con forma bulbar (p<0,05), y en los pacientes con forma bulbar (p<0,05), con claro predominio femenino (p<0,03). Hay 31 pacientes muertos (57,4%) y 23 vivos (42,6%), con supervivencia media desde el inicio de los síntomas de 46,51 +- 40,9 meses (11-209 meses), observando relación inversamente proporcional con la edad de inicio de la enfermedad (p<0,02). Se asocian con una mayor probabilidad de morir las formas de inicio bulbar (p<0,01) y la extensión de los signos de afectación de MNI, especialmente a nivel bulbar y cervical (p<0,005), mientras que la existencia de signos de afectación de NMS en neuroimagen tiene una mayor probabilidad de sobrevivir (p<0,05).Conclusiones. La edad avanzada y las formas de inicio bulbar son factores de mal pronóstico, y especialmente el predominio de afectación NMI sobre NMS

Introduction: Amyotrophic lateral sclerosis (ALS) is the most common adult motor neuron disease (MND), presenting with a combination of upper (UMN) and lower (lmn) motor neuron signs, with high mortality. other less frequent mnd are progressive muscular atrophy (pma) and primary lateral sclerosis (pls). OBJECTIVES: To describe demographic data, clinical forms of presentation and evolution of patients with MND, looking for prognostic indicators factors. METHODS: Retrospective study of a series of patients with MND, registering demographic data, clinical variables and mortality-survival, analyzed by means of statistical package SPSS 6.1.2. RESULTS: They are 54 patients with MND (50 ALS, 3 PMA and 1 PLS), 30 (55.6%) men and 24 (44.4%) women, bulbar beginning forms in 13 cases (24.1%) and spinal in 41 (75.9%). Average age at beginning 60+/-14 (29-82 years), more delayed in women (p<0.05), and in patients with bulbar form (p<0.05) with clear predominance of female sex (p<0.03). There are 31 dead patients (57.4%) and 23 alive ones (42.6%), with average survival from the beginning symptoms of 46.51+/-40.9 months. Nine months (11-209), observing inversely proportional relation with the age of beginning of the disease (p<0.02). Bulbar beginning forms (p<0.01) and LMN affectation signs, specially bulbar and cervical levels (p<0,005), are associated with a greater probability of dying while the existence of UMN affectation signs on imaging techniques 362 ques has a greater probability of survival (p<0,05). CONCLUSIONS: The advanced age and the bulbar beginning forms are factors of bad prognosis, specially the predominant affectation of LMN over UMN

[Pronostic factors and survival in motor neuron disease]. / Factores pronósticos y supervivencia en la enfermedad de neurona motora.

INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is the most common adult motor neuron disease (MND), presenting with a combination of upper (UMN) and lower (lmn) motor neuron signs, with high mortality. other less frequent mnd are progressive muscular atrophy (pma) and primary lateral sclerosis (pls). OBJECTIVES: To describe demographic data, clinical forms of presentation and evolution of patients with MND, looking for prognostic indicators factors. METHODS: Retrospective study of a series of patients with MND, registering demographic data, clinical variables and mortality-survival, analyzed by means of statistical package SPSS 6.1.2. RESULTS: They are 54 patients with MND (50 ALS, 3 PMA and 1 PLS), 30 (55.6%) men and 24 (44.4%) women, bulbar beginning forms in 13 cases (24.1%) and spinal in 41 (75.9%). Average age at beginning 60+/-14 (29-82 years), more delayed in women (p<0.05), and in patients with bulbar form (p<0.05) with clear predominance of female sex (p<0.03). There are 31 dead patients (57.4%) and 23 alive ones (42.6%), with average survival from the beginning symptoms of 46.51+/-40.9 months. Nine months (11-209), observing inversely proportional relation with the age of beginning of the disease (p<0.02). Bulbar beginning forms (p<0.01) and LMN affectation signs, specially bulbar and cervical levels (p<0,005), are associated with a greater probability of dying while the existence of UMN affectation signs on imaging techniques 362 ques has a greater probability of survival (p<0,05). CONCLUSIONS: The advanced age and the bulbar beginning forms are factors of bad prognosis, specially the predominant affectation of LMN over UMN.

[Facial diplejia: a regional variant of Guillain-Barré syndrome]. / Diplejia facial: variante regional del síndrome de Guillain-Barré.

Facial palsy is a uncommon clinical manifestation that it can be caused by different etiologies. We show a patient with a chronic periodontal disease who presented a sudden facial palsy, initially in left-face but it becomes bilateral quickly. In few days he associated paresthesias in his right arm and hyporreflexia. These clinical findings with albumin-cytological dissociation, they had allowed to establish the diagnosis of regional variant of Guillain-Barré Syndrome (SGB). Facial diplejia is an idiopathic manifestation in 25% of patients, and this is the most common cause. However, facial diplejia can be secondary to many etiologies as SGB. The affectation of facial nerve associated to other motor symptoms in SGB is frequent, but it is not frequent the presentation as facial diplejia alone. In conclusion, it is necessary a high clinical suspicion to do a lumbar punction (PL) and MRI to reject neoplasic pathologies and to obtain a diagnosis and an adequate treatment.

Diplejia facial: variante regional del síndrome de Guillian-Barré / Facial diplejia: a regional variant of Guillian-Barre syndrome

Diplejia facial es una manifestación clínica poco frecuente que puede presentarse como consecuencia de múltiples etiologías. Presentamos un paciente con una infección periodontal crónica que sufrió de forma brusca una parálisis facial inicialmente izquierda, que rápidamente se hizo bilateral. La aparición posterior de parestesias distales en extremidad superior derecha e hiporreflexia, junto con la disociación albúmino-citológica en el LCR, permitieron establecer el diagnóstico de variante regional del Síndrome de Guillian-Barré (SGB). La diplejia facial es idiopática en el 25% de casos, siendo la causa más frecuente. Sin embargo, existe un elevado porcentaje de casos secundarios a múltiples etiologías como el SGB donde es frecuente la afectación del nervio facial habitualmente asociado a otros trastornos motores siendo infrecuente la diplejia facial aislada. Concluimos la necesidad de sospecha clínica para realización de punción lumbar y RNM para descartar procesos neoplásicos y poder determinar la etiología responsable para establecer un adecuado abordaje terapéutico

Facial palsy is a uncommon clinical manifestation that it can be caused by different ethiologies. We show a patient with a chronic periodontal disease who presented a sudden facial palsy, initially in left-face but it becomes bilateral quickly. In few days he associated paresthesias in his right arm and hiporreflexia. These clinical findings with albumin-citological dissociation, they had allowed to establish the diagnosis of regional variant of Guillain-Barré Syndrome (SGB). Facial diplejia is an idiopathic manifestation in 25% of patients, and this is the most common cause. However, facial diplejia can be secondary to many ethiologies as SGB. The affectation of facial nerve associated to other motor symptoms in SGB is frequent, but it is not frequent the presentation as facial diplejia alone. In conclusion, it is necessary a high clinical suspicion to do a lumbar punction (PL) and MRI to reject neoplasic pathologies and to obtain a diagnosis and an adequatted treatment