[Moyamoya in Aragon: epidemiology and self-perception of quality of life]. / Moyamoya en Aragón. Epidemiología y calidad de vida autopercibida.

INTRODUCTION: Moyamoya angiopathy is a vasculopathy caused by stenosis/occlusion of one or both intracranial internal carotid arteries. Although more common in Eastern countries, its prevalence is increasing in the West. An angioresonance or angiography is essential for its diagnosis. There are two options for treatment: conservative (medical) treatment or surgical bypass techniques. PATIENTS AND METHODS: Nineteen patients were selected using International Classification of Diseases codes, and their demographic characteristics and health outcomes were studied. They were administered a scale for the screening of anxious-depressive syndrome (the Hospital Anxiety and Depression Scale - HADS) and another scale for self-perceived quality of life (SF-36). After applying the inclusion/exclusion criteria, eight of these patients were studied. RESULTS: Nineteen patients were studied (52.63% male, 57.89% European) and the Aragonese prevalence was estimated at 1.37/100,000 inhabitants. The most frequent clinical presentation was ischaemic stroke (73.68%). The HADS detected two positive cases of anxiety and one case of depression. According to the SF-36, the worst self-rated aspects were vitality (median: 35/100) and general health (median: 42.5/100), while the best rated was physical function (mean: 93.57/100). CONCLUSIONS: This is the Spanish series with the highest prevalence and the only one that addresses self-perceived health and screening of the anxious-depressive syndrome. Further research is needed to address this entity and determine its true prevalence in the West.

TITLE: Moyamoya en Aragón. Epidemiología y calidad de vida autopercibida.Introducción. La angiopatía de moyamoya es una vasculopatía originada por la estenosis/oclusión de una o ambas carótidas internas intracraneales. Aunque es más frecuente en países orientales, está aumentando su prevalencia en Occidente. Para su diagnóstico es imprescindible una angiorresonancia o una angiografía. En su tratamiento hay dos opciones: el tratamiento conservador (médico) o las técnicas quirúrgicas de bypass. Pacientes y métodos. Se seleccionó a 19 pacientes mediante códigos de la Clasificación internacional de enfermedades, y se estudiaron sus características demográficas y resultados en salud. Se les administró una escala para el cribado de síndrome ansiosodepresivo ­escala de ansiedad y depresión hospitalaria (HADS)­ y otra de autopercepción de calidad de vida (SF-36). De estos pacientes, se estudió a ocho al aplicar los criterios de inclusión/exclusión. Resultados. Se estudió a 19 pacientes (52,63%, hombres; 57,89%, europeos) y se estimó la prevalencia aragonesa en 1,37/100.000 habitantes. La clínica más frecuente fue el ictus isquémico (73,68%). La HADS detectó dos casos positivos para ansiedad y un caso de depresión. Según el SF-36, los aspectos peor autopercibidos fueron la vitalidad (mediana: 35/100) y la salud general (mediana: 42,5/100); mientras que el mejor valorado fue la función física (media: 93,57/100). Conclusiones. Se trata de la serie española con mayor prevalencia y la única que aborda la salud autopercibida y el cribado del síndrome ansiosodepresivo. Son necesarios más estudios que permitan abordar esta entidad y cuál es la verdadera prevalencia en Occidente.

Moyamoya en Aragón. Epidemiología y calidad de vida autopercibida / Moyamoya in Aragon: epidemiology and self-perception of quality of life

Introducción La angiopatía de moyamoya es una vasculopatía originada por la estenosis/oclusión de una o ambas carótidas internas intracraneales. Aunque es más frecuente en países orientales, está aumentando su prevalencia en Occidente. Para su diagnóstico es imprescindible una angiorresonancia o una angiografía. En su tratamiento hay dos opciones: el tratamiento conservador (médico) o las técnicas quirúrgicas de bypass. Pacientes y métodos Se seleccionó a 19 pacientes mediante códigos de la Clasificación internacional de enfermedades, y se estudiaron sus características demográficas y resultados en salud. Se les administró una escala para el cribado de síndrome ansiosodepresivo –escala de ansiedad y depresión hospitalaria (HADS)– y otra de autopercepción de calidad de vida (SF-36). De estos pacientes, se estudió a ocho al aplicar los criterios de inclusión/exclusión. Resultados Se estudió a 19 pacientes (52,63%, hombres; 57,89%, europeos) y se estimó la prevalencia aragonesa en 1,37/100.000 habitantes. La clínica más frecuente fue el ictus isquémico (73,68%). La HADS detectó dos casos positivos para ansiedad y un caso de depresión. Según el SF-36, los aspectos peor autopercibidos fueron la vitalidad (mediana: 35/100) y la salud general (mediana: 42,5/100); mientras que el mejor valorado fue la función física (media: 93,57/100). Conclusiones Se trata de la serie española con mayor prevalencia y la única que aborda la salud autopercibida y el cribado del síndrome ansiosodepresivo. Son necesarios más estudios que permitan abordar esta entidad y cuál es la verdadera prevalencia en Occidente. (AU)

INTRODUCTION Moyamoya angiopathy is a vasculopathy caused by stenosis/occlusion of one or both intracranial internal carotid arteries. Although more common in Eastern countries, its prevalence is increasing in the West. An angioresonance or angiography is essential for its diagnosis. There are two options for treatment: conservative (medical) treatment or surgical bypass techniques. PATIENTS AND METHODS Nineteen patients were selected using International Classification of Diseases codes, and their demographic characteristics and health outcomes were studied. They were administered a scale for the screening of anxious-depressive syndrome (the Hospital Anxiety and Depression Scale – HADS) and another scale for self-perceived quality of life (SF-36). After applying the inclusion/exclusion criteria, eight of these patients were studied. RESULTS Nineteen patients were studied (52.63% male, 57.89% European) and the Aragonese prevalence was estimated at 1.37/100,000 inhabitants. The most frequent clinical presentation was ischaemic stroke (73.68%). The HADS detected two positive cases of anxiety and one case of depression. According to the SF-36, the worst self-rated aspects were vitality (median: 35/100) and general health (median: 42.5/100), while the best rated was physical function (mean: 93.57/100). CONCLUSIONS This is the Spanish series with the highest prevalence and the only one that addresses self-perceived health and screening of the anxious-depressive syndrome. Further research is needed to address this entity and determine its true prevalence in the West. (AU)

Epidemiology of ataxia and hereditary spastic paraplegia in Spain: A cross-sectional study.

INTRODUCTION: Ataxia and hereditary spastic paraplegia are rare neurodegenerative syndromes. We aimed to determine the prevalence of these disorders in Spain in 2019. PATIENTS AND METHODS: We conducted a cross-sectional, multicentre, retrospective, descriptive study of patients with ataxia and hereditary spastic paraplegia in Spain between March 2018 and December 2019. RESULTS: We gathered data from a total of 1933 patients from 11 autonomous communities, provided by 47 neurologists or geneticists. Mean (SD) age in our sample was 53.64 (20.51) years; 938 patients were men (48.5%) and 995 were women (51.5%). The genetic defect was unidentified in 920 patients (47.6%). A total of 1371 patients (70.9%) had ataxia and 562 (29.1%) had hereditary spastic paraplegia. Prevalence rates for ataxia and hereditary spastic paraplegia were estimated at 5.48 and 2.24 cases per 100 000 population, respectively. The most frequent type of dominant ataxia in our sample was SCA3, and the most frequent recessive ataxia was Friedreich ataxia. The most frequent type of dominant hereditary spastic paraplegia in our sample was SPG4, and the most frequent recessive type was SPG7. CONCLUSIONS: In our sample, the estimated prevalence of ataxia and hereditary spastic paraplegia was 7.73 cases per 100 000 population. This rate is similar to those reported for other countries. Genetic diagnosis was not available in 47.6% of cases. Despite these limitations, our study provides useful data for estimating the necessary healthcare resources for these patients, raising awareness of these diseases, determining the most frequent causal mutations for local screening programmes, and promoting the development of clinical trials.

Epidemiology and characteristics of ischaemic stroke in young adults in Aragon.

INTRODUCTION: Stroke affects around 15 million people per year, with 10%-15% occurring in individuals under 50 years old (stroke in young adults). The prevalence of different vascular risk factors and healthcare strategies for stroke management vary worldwide, making the epidemiology and specific characteristics of stroke in each region an important area of research. This study aimed to determine the prevalence of different vascular risk factors and the aetiology and characteristics of ischaemic stroke in young adults in the autonomous community of Aragon, Spain. METHODS: A cross-sectional, multi-centre study was conducted by the neurology departments of all hospitals in the Aragonese Health Service. We identified all patients aged between 18 and 50 years who were admitted to any of these hospitals with a diagnosis of ischaemic stroke or TIA between January 2005 and December 2015. Data were collected on demographic variables, vascular risk factors, and type of stroke, among other variables. RESULTS: During the study period, 786 patients between 18 and 50 years old were admitted with a diagnosis of ischaemic stroke or TIA to any hospital of Aragon, at a mean annual rate of 12.3 per 100 000 population. The median age was 45 years (IQR: 40-48 years). The most prevalent vascular risk factor was tobacco use, in 404 patients (51.4%). The majority of strokes were of undetermined cause (36.2%), followed by other causes (26.5%). The median NIHSS score was 3.5 (IQR: 2.0-7.0). In total, 211 patients (26.8%) presented TIA. Fifty-nine per cent of the patients admitted with a diagnosis of ischaemic stroke (10.3%) were treated with fibrinolysis. CONCLUSIONS: Ischaemic stroke in young adults is not uncommon in Aragon, and is of undetermined aetiology in a considerable number of cases; it is therefore necessary to implement measures to improve study of the condition, to reduce its incidence, and to prevent its recurrence.

Three-month functional prognosis of patients hospitalised due to acute ischaemic stroke in Aragon: regional analysis of the impact of COVID-19.

INTRODUCTION: The COVID-19 pandemic has had an impact on ischaemic stroke management, with a reported decrease in hospital admissions, and even disruptions in healthcare and increased in-hospital mortality. However, there is a lack of evidence on the impact of the pandemic on functional prognosis. The aim of this study is to analyse the effect of the COVID-19 pandemic on the 3-month functional outcomes of patients hospitalised due to acute ischaemic stroke in Aragon (Spain). METHODS: We reviewed the data of all patients admitted due to ischaemic stroke to any hospital in our regional healthcare system between 30 December 2019 and 3 May 2020. We compared modified Rankin Scale scores and mortality at 3 months in patients hospitalised before and after the declaration of a state of emergency due to the COVID-19 pandemic. RESULTS: In total, 318 patients with acute ischaemic stroke met our inclusion criteria. No differences were observed between periods in global or specific characteristics, with the exception of a higher proportion of patients older than 80 years during the first period (42.2% vs 29.0%, P = .028). In the comparative analysis, we found no significant differences in mortality (12.3 vs 7.9, P = .465) or in the proportion of patients with modified Rankin Scale scores ≤ 2 (57.7% vs 57.1%, P = .425) at 3 months. CONCLUSION: To our knowledge, this is the first study to analyse the impact of COVID-19 pandemic on the 3-month functional outcomes of patients with ischaemic stroke. In our region, there has been no increase in rates of mortality or disability at 3 months in patients admitted due to ischaemic stroke during the pandemic.

Epidemiology of ataxia and hereditary spastic paraplegia in Spain: a cross-sectional study. / Mapa epidemiológico transversal de las ataxias y paraparesias espásticas hereditarias en España.

INTRODUCTION: Ataxia and hereditary spastic paraplegia are rare neurodegenerative syndromes. We aimed to determine the prevalence of these disorders in Spain in 2019. PATIENTS AND METHODS: We conducted a cross-sectional, multicentre, retrospective, descriptive study of patients with ataxia and hereditary spastic paraplegia in Spain between March 2018 and December 2019. RESULTS: We gathered data from a total of 1.809 patients from 11 autonomous communities, provided by 47 neurologists or geneticists. Mean (SD) age in our sample was 53.64 (20.51) years; 920 patients were men (50.8%) and 889 were women (49.2%). The genetic defect was unidentified in 920 patients (47.6%). A total of 1371 patients (70.9%) had ataxia and 562 (29.1%) had hereditary spastic paraplegia. Prevalence rates for ataxia and hereditary spastic paraplegia were estimated at 5.48 and 2.24 cases per 100 000 population, respectively. The most frequent type of dominant ataxia in our sample was SCA3, and the most frequent recessive ataxia was Friedreich ataxia. The most frequent type of dominant hereditary spastic paraplegia in our sample was SPG4, and the most frequent recessive type was SPG7. CONCLUSIONS: In our sample, the estimated prevalence of ataxia and hereditary spastic paraplegia was 7.73 cases per 100 000 population. This rate is similar to those reported for other countries. Genetic diagnosis was not available in 47.6% of cases. Despite these limitations, our study provides useful data for estimating the necessary healthcare resources for these patients, raising awareness of these diseases, determining the most frequent causal mutations for local screening programmes, and promoting the development of clinical trials.

[Three-month functional prognosis of patients hospitalised due to acute ischaemic stroke in Aragon: Rregional analysis of the impact of COVID-19]. / Pronóstico funcional a 3 meses de pacientes hospitalizados por ictus isquémico agudo en Aragón: análisis regional de la repercusión de la COVID-19.

Introduction: The COVID-19 pandemic has had an impact on ischaemic stroke management, with a reported decrease in hospital admissions, and even disruptions in healthcare and increased in-hospital mortality. However, there is a lack of evidence on the impact of the pandemic on functional prognosis. The aim of this study is to analyse the effect of the COVID-19 pandemic on the 3-month functional outcomes of patients hospitalised due to acute ischaemic stroke in Aragon (Spain). Materil and methods: We reviewed the data of all patients admitted due to ischaemic stroke to any hospital in our regional healthcare system between 30 December 2019 and 3 May 2020. We compared modified Rankin Scale scores and mortality at 3 months in patients hospitalised before and after the declaration of a state of emergency due to the COVID-19 pandemic. Results: In total, 318 patients with acute ischaemic stroke met our inclusion criteria. No differences were observed between periods in global or specific characteristics, with the exception of a higher proportion of patients older than 80 years during the first period (42.2% vs. 29.0%, P=.028). In the comparative analysis, we found no significant differences in mortality (12.3 vs. 7.9, P=.465) or in the proportion of patients with modified Rankin Scale scores ≤ 2 (57.7% vs. 57.1%, P=.425) at 3 months. Conclusion: To our knowledge, this is the first study to analyse the impact of COVID-19 pandemic on the 3-month functional outcomes of patients with ischaemic stroke. In our region, there has been no increase in rates of mortality or disability at 3 months in patients admitted due to ischaemic stroke during the pandemic.

Epidemiology and characteristics of ischaemic stroke in young adults in Aragon. / Epidemiología y características del ictus isquémico en el adulto joven en Aragón.

INTRODUCTION: Stroke affects around 15 million people per year, with 10%-15% occurring in individuals under 50 years old (stroke in young adults). The prevalence of different vascular risk factors and healthcare strategies for stroke management vary worldwide, making the epidemiology and specific characteristics of stroke in each region an important area of research. This study aimed to determine the prevalence of different vascular risk factors and the aetiology and characteristics of ischaemic stroke in young adults in the autonomous community of Aragon, Spain. METHODS: A cross-sectional, multi-centre study was conducted by the neurology departments of all hospitals in the Aragonese Health Service. We identified all patients aged between 18 and 50 years who were admitted to any of these hospitals with a diagnosis of ischaemic stroke or TIA between January 2005 and December 2015. Data were collected on demographic variables, vascular risk factors, and type of stroke, among other variables. RESULTS: During the study period, 786 patients between 18 and 50 years old were admitted with a diagnosis of ischaemic stroke or TIA to any hospital of Aragon, at a mean annual rate of 12.3 per 100 000 population. The median age was 45 years (IQR: 40-48 years). The most prevalent vascular risk factor was tobacco use, in 404 patients (51.4%). The majority of strokes were of undetermined cause (36.2%), followed by other causes (26.5%). The median NIHSS score was 3.5 (IQR: 2.0-7.0). In total, 211 patients (26.8%) presented TIA. Fifty-nine per cent of the patients admitted with a diagnosis of ischaemic stroke (10.3%) were treated with fibrinolysis. CONCLUSIONS: Ischaemic stroke in young adults is not uncommon in Aragon, and is of undetermined aetiology in a considerable number of cases; it is therefore necessary to implement measures to improve study of the condition, to reduce its incidence, and to prevent its recurrence.

Programa de Atención al Ictus en Aragón (PAIA). Estrategia del cambio y resultados en el periodo 2009-2014 / Stroke care programme in Aragon (PAIA): strategy and outcomes for the period 2009-2014

INTRODUCCIÓN: En 2008, Aragón tenía tasas de morbimortalidad y discapacidad por ictus superiores a las del conjunto de España. Se estableció la necesidad de desarrollar un Programa de Atención al Ictus (PAIA). MATERIAL Y MÉTODOS: Damos a conocer la dinámica de planificación, implantación, evaluación y mejora que se ha desarrollado entre los años 2009-2014 y sus resultados a 5 años. RESULTADOS: Se ha mejorado en la estructura, en los procesos y en los resultados, con mejoría en los indicadores clave de la asistencia (audit 2008-2010-2012) y otros: tasa ictus 2013: 2,07 (2008: 2,36); 78% ictus atendidos en áreas/unidades en 2014 (30%, 2008); tasa fibrinólisis 8,3% en 2014 (4,4%, 2010); fibrinólisis hospitales secundarios (30% total); fibrinólisis con teleictus 9%; descenso de la mortalidad por ictus, 38%; años de vida prematura perdidos 67,7 (2013)/144 (2008); capacitación de enfermería, desarrollo de la neurosonología, trabajo en red, con protocolos y buenas prácticas compartidos entre sectores sanitarios, etc. CONCLUSIONES: La gestión por procesos y equipos multidisciplinares desplegados en una distribución territorial integral, con protocolos y referencias establecidas y una dinámica de evaluación y mejora continua, ha demostrado ser una herramienta potente para garantizar la calidad y la equidad. El PAIA, por su dinámica de mejora sostenida y la implicación de los clínicos, es un buen ejemplo de gestión clínica y trabajo en red

INTRODUCTION: In 2008, stroke mortality, morbidity, and disability rates in Aragon were higher than the average in Spain. These data underscored the need to develop a stroke care programme (PAIA). MATERIAL AND METHODS: We present the dynamics of planning, implementation, evaluation, and improvement developed between 2009 and 2014 as well as the results of the PAIA after that 5-year period. RESULTS: Structure, processes, and outcomes have improved with reference to the key indicators of healthcare (audit: 2008, 2010, 2012) among others: stroke rate in 2013 was 2.07 (2.36 in 2008); 78% of strokes were managed in stroke units in 2014 (30% in 2008); rate of fibrinolysis was 8.3% in 2014 (4.4% in 2010); fibrinolysis was administered in secondary hospitals (30% of the total); fibrinolysis was administered by Telestroke in 9%; stroke mortality decreased (38%); 67.7 years of potential life lost (YPLL) in 2013 (144 in 2008); nurse training; development of neurosonology; networking; sharing protocols and best practices between health sectors, etc. CONCLUSIONS: Integrated process management and multidisciplinary teams distributed and deployed over an entire territory with established protocols, references, evaluations, and continuous development, have been proven powerful tools to ensure both quality and equality. The PAIA is a good example of clinical governance and networking due to its dynamic and sustained improvement and cooperation between clinicians

Stroke care programme in Aragon (PAIA): strategy and outcomes for the period 2009-2014. / Programa de Atención al Ictus en Aragón (PAIA). Estrategia del cambio y resultados en el periodo 2009-2014.

INTRODUCTION: In 2008, stroke mortality, morbidity, and disability rates in Aragon were higher than the average in Spain. These data underscored the need to develop a stroke care programme (PAIA). MATERIAL AND METHODS: We present the dynamics of planning, implementation, evaluation, and improvement developed between 2009 and 2014 as well as the results of the PAIA after that 5-year period. RESULTS: Structure, processes, and outcomes have improved with reference to the key indicators of healthcare (audit: 2008, 2010, 2012) among others: stroke rate in 2013 was 2.07 (2.36 in 2008); 78% of strokes were managed in stroke units in 2014 (30% in 2008); rate of fibrinolysis was 8.3% in 2014 (4.4% in 2010); fibrinolysis was administered in secondary hospitals (30% of the total); fibrinolysis was administered by Telestroke in 9%; stroke mortality decreased (38%); 67.7 years of potential life lost (YPLL) in 2013 (144 in 2008); nurse training; development of neurosonology; networking; sharing protocols and best practices between health sectors, etc. CONCLUSIONS: Integrated process management and multidisciplinary teams distributed and deployed over an entire territory with established protocols, references, evaluations, and continuous development, have been proven powerful tools to ensure both quality and equality. The PAIA is a good example of clinical governance and networking due to its dynamic and sustained improvement and cooperation between clinicians.

[Auditing as a tool for ongoing improvement in the Stroke Care Plan of the Region of Aragon]. / El audit como herramienta de mejora continua en el Plan de Atencion al Ictus de Aragon.

INTRODUCTION: The Aragon Stroke Care Plan (PAIA) was created in 2008 within the framework of the Spanish National Health System. Monitoring hospital care of strokes by means of periodic audits was defined as one of its lines of work. AIM: To determine the quality of the hospital care process for stroke patients in Aragon by using quality indicators. MATERIALS AND METHODS: Three audits were carried out (in the years 2008, 2010 and 2012) following the same methodology, based on the retrospective review of a representative sample of admissions due to stroke in each of the general hospitals belonging to the Aragonese Health Service. Information was collected on 48 indicators selected according to their scientific evidence or clinical relevance. RESULTS: Altogether 1011 cases were studied (331 in the first audit, and 340 in the second and the third). Thirty-one indicators showed a significant improvement (some of the most notable being the indicators of quality of the medical record, neurological assessment, initial preventive measures and, especially relevant, performing the swallowing test), two underwent a decline in their condition (related with rehabilitation treatment) and 15 did not register any significant variation. CONCLUSIONS: The implementation of the PAIA has given rise to a notable improvement in most of the quality indicators evaluated, which reflects an ongoing improvement in hospital stroke care. The progressive generalisation of specialised care and the creation of stroke units are some of the determining factors.

TITLE: El audit como herramienta de mejora continua en el Plan de Atencion al Ictus de Aragon.Introduccion. El Plan de Atencion al Ictus de Aragon (PAIA) se creo en 2008 en el marco de la Estrategia Nacional en Ictus del Sistema Nacional de Salud. La monitorizacion de la atencion hospitalaria al ictus mediante auditorias periodicas se definio como una de sus lineas de trabajo. Objetivo. Determinar la calidad del proceso asistencial hospitalario prestado al paciente con ictus en Aragon mediante el uso de indicadores de calidad. Materiales y metodos. Se realizaron tres audits (en los años 2008, 2010 y 2012) siguiendo la misma metodologia, basada en la revision retrospectiva de una muestra representativa de ingresos por ictus en cada uno de los hospitales generales del Servicio Aragones de Salud. Se recogio informacion sobre 48 indicadores seleccionados segun su evidencia cientifica o relevancia clinica. Resultados. Se estudiaron 1.011 casos (331 en el primer audit, y 340 en el segundo y en el tercero). Treinta y un indicadores presentaron una mejoria significativa (entre ellos destacan los indicadores de calidad de la historia clinica, de evaluacion neurologica, las medidas preventivas iniciales y, con especial relevancia, la realizacion de test de deglucion), dos sufrieron empeoramiento (relacionados con el tratamiento rehabilitador) y 15 no registraron variaciones significativas. Conclusiones. La implantacion del PAIA ha supuesto una mejoria notable en la mayoria de los indicadores de calidad evaluados, reflejo de una mejora continua en la atencion hospitalaria del ictus. La generalizacion progresiva de la atencion especializada y la creacion de las areas de ictus son algunos de los factores determinantes.