Amiotrofia y fasciculaciones como forma de presentación de la enfermedad de Creutzfeldt-Jakob esporádica. A propósito de un caso / Muscle atrophy and fasciculations as a manifestation of sporadic Creutzfeldt-Jakob disease: A case report

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[An analysis of pre-hospital delay times in stroke care]. / Análisis de la demora prehospitalaria en la asistencia al ictus.

INTRODUCTION: Delays in the treatment of a stroke constitute a factor that leads to a more unfavourable prognosis. Shortening the delay time in the health care of these patients is essential in order to reduce the morbidity and mortality rates of this disease. AIMS: Our aim was to analyse the causes that bring about delays in getting stroke patients to hospital. PATIENTS AND METHODS: A prospective analysis was conducted of 133 patients who visited the Emergency Department. A survey carried out during the first 48 hours collected information on the clinical characteristics and the steps followed by the patient before arriving at the hospital. We considered the time that elapsed to be adequate if patients arrived at the hospital in less than 2 hours. A univariate and multivariate analysis was performed to evaluate the factors that could extend this time. RESULTS: The mean time elapsed before the patient reached hospital was 502 minutes (interval: 11-5,700). A total of 42.5% arrived in less than 2 hours and 58.2% got there in less than 3 hours. The univariate analysis showed that females, those with a low cultural level, the most severe cases, those who went straight to hospital and those who used the emergency services all arrived more quickly. In the multivariate analysis only the more severe cases and those who went straight to hospital ran less risk of a delayed arrival. CONCLUSIONS: The time stroke patients take to reach hospital varies greatly. The main factors that influence the time that elapses before arrival are the severity of the symptoms and going straight to hospital.

Análisis de la demora prehospitalaria en la asistencia al ictus / An analysis of pre-hospital delay times in stroke care

Introducción. El retraso en el tratamiento del ictus es un factor que empeora su pronóstico. La reducción del tiempo de la asistencia a estos enfermos es fundamental para reducir la morbimortalidad de esta enfermedad. Objetivo. Analizar las causas que provocan retraso en la llegada al hospital de los pacientes que sufren un ictus. Pacientes y métodos. Se analizaron prospectivamente 133 pacientes que acudieron al Servicio de Urgencias. Mediante una encuesta realizada en las primeras 48 horas se recogieron las características clínicas y los pasos seguidos por el enfermo hasta llegar al hospital. Se consideró adecuado que el tiempo transcurrido hasta la llegada al hospital fuese menor de 2 horas. Se realizó un análisis univariante y multivariante para evaluar qué factores podían alargar este tiempo. Resultados. El tiempo medio empleado hasta llegar al hospital fue de 502 minutos (intervalo: 11-5.700). Un 42,5% llegó antes de las 2 horas y un 58,2% antes de las 3. En el análisis univariante se observó que las mujeres, un nivel cultural bajo, los casos más graves, los que acudía directamente al hospital y los que utilizaban los servicios de urgencias, llegaron más rápidamente. En el análisis multivariante sólo los pacientes más graves y aquellos que acudían directamente al hospital tuvieron menos riesgo de llegar con retraso. Conclusiones. El tiempo empleado hasta llegar al hospital por los pacientes con ictus varía mucho. Los principales factores que influyen en el mismo son la gravedad de los síntomas y el acudir directamente al hospital (AU)

Introduction. Delays in the treatment of a stroke constitute a factor that leads to a more unfavourable prognosis. Shortening the delay time in the health care of these patients is essential in order to reduce the morbidity and mortality rates of this disease. Aims. Our aim was to analyse the causes that bring about delays in getting stroke patients to hospital. Patients and methods. A prospective analysis was conducted of 133 patients who visited the Emergency Department. A survey carried out during the first 48 hours collected information on the clinical characteristics and the steps followed by the patient before arriving at the hospital. We considered the time that elapsed to be adequate if patients arrived at the hospital in less than 2 hours. A univariate and multivariate analysis was performed to evaluate the factors that could extend this time. Results. The mean time elapsed before the patient reached hospital was 502 minutes (interval: 11-5,700). A total of 42.5% arrived in less than 2 hours and 58.2% got there in less than 3 hours. The univariate analysis showed that females, those with a low cultural level, the most severe cases, those who went straight to hospital and those who used the emergency services all arrived more quickly. In the multivariate analysis only the more severe cases and those who went straight to hospital ran less risk of a delayed arrival. Conclusions. The time stroke patients take to reach hospital varies greatly. The main factors that influence the time that elapses before arrival are the severity of the symptoms and going straight to hospital (AU)

[Autoimmune diseases and multiple sclerosis]. / Enfermedades autoinmunes y esclerosis múltiple.

INTRODUCTION: Multiple sclerosis (MS) is a demyelinating disease affecting the central nervous system with an unknown origin, although the immunological system plays a crucial role in its pathogenesis. It has been observed that the relatives of MS patients very often have other autoimmune diseases (ADs) and it has been suggested that there may be susceptibility genes that are common to this group of diseases. AIMS: Our aim was to estimate the prevalence of ADs in first and second degree relatives of patients with MS and to determine the coexistence of other ADs in MS patients. PATIENTS AND METHODS: We selected 251 patients with MS defined by the Poser criteria and face-to-face interviews with the patients and/or their relatives were conducted to investigate the following ADs: MS, rheumatoid arthritis, systemic lupus erythematosus, polyarteritis nodosa, autoimmune thyroid disease (ATD), inflammatory bowel disease, myasthenia gravis, type I diabetes mellitus (DMI) and psoriasis. RESULTS. 29.9% of the patients with MS had a first and/or second degree relative with an AD. Prevalence of ADs in first degree relatives was 15.5%, 30% in familial MS and 40% if the patient had both MS and another AD. The most frequent ADs were: MS 27%, psoriasis 18%, ATD 16% and DMI 15%. 15 patients had MS and another AD: six ATD, three DMI, four psoriasis, one inflammatory bowel disease and one myasthenia gravis. CONCLUSIONS: These findings lend support to the existence of susceptibility genes that are common to the different ADs and would act as risk factors.

Enfermedades autoinmunes y esclerosis múltiple / Autoimmune diseases and multiple sclerosis

Introducción. La esclerosis múltiple (EM) es una enfermedad desmielinizante del sistema nervioso central de origen desconocido, en cuya patogénesis el sistema inmunitario desempeña un papel crucial. En los familiares de pacientes con EM se ha observado una elevada frecuencia de otras enfermedades autoinmunes (EA), por lo que se ha propuesto la existencia de genes de susceptibilidad comunes a este grupo de enfermedades. Objetivos. Estimar la prevalencia de EA en familiares de primer y segundo grado de pacientes con EM y determinar la coexistencia de otras EA en los pacientes de EM. Pacientes y métodos. Seleccionamos 251 pacientes con EM definida por los criterios de Poser e investigamos mediante una entrevista personal con el paciente y/o sus familiares las siguientes EA: EM, artritis reumatoide, lupus eritematoso sistémico, panarteritis nudosa, enfermedad tiroidea autoinmune (ETA), enfermedad inflamatoria intestinal, miastenia grave, diabetes mellitus tipo I (DMI) y psoriasis. Resultados. El 29,9% de los pacientes con EM tenía algún familiar de 1. r y/o 2.º grado con una EA. La prevalencia de las EA en los familiares de primer grado fue del 15,5%, del 30% en la EM familiar y del 40% si concurrían en el paciente EM y otra EA. Las EA más frecuentes fueron EM (27%), psoriasis (18%), ETA (16%) y DMI (15%). Tenían EM y otra EA 15 pacientes: 6 con ETA, 3 con DMI, 4 con psoriasis, 1 con enfermedad inflamatoria intestinal y 1 con miastenia grave. Conclusiones. Estos hallazgos apoyan la presencia de genes de susceptibilidad comunes a las diferentes EA que actuarían como factores de riesgo

Introduction. Multiple sclerosis (MS) is a demyelinating disease affecting the central nervous system with an unknown origin, although the immunological system plays a crucial role in its pathogenesis. It has been observed that the relatives of MS patients very often have other autoimmune diseases (ADs) and it has been suggested that there may be susceptibility genes that are common to this group of diseases. Aims. Our aim was to estimate the prevalence of ADs in first and second degree relatives of patients with MS and to determine the coexistence of other ADs in MS patients. Patients and methods. We selected 251 patients with MS defined by the Poser criteria and face-to-face interviews with the patients and/or their relatives were conducted to investigate the following ADs: MS, rheumatoid arthritis, systemic lupus erythematosus, polyarteritis nodosa, autoimmune thyroid disease (ATD), inflammatory bowel disease, myasthenia gravis, type I diabetes mellitus (DMI) and psoriasis. Results. 29.9% of the patients with MS had a first and/or second degree relative with an AD. Prevalence of ADs in first degree relatives was 15.5%, 30% in familial MS and 40% if the patient had both MS and another AD. The most frequent ADs were: MS 27%, psoriasis 18%, ATD 16% and DMI 15%. 15 patients had MS and another AD: six ATD, three DMI, four psoriasis, one inflammatory bowel disease and one myasthenia gravis. Conclusions. These findings lend support to the existence of susceptibility genes that are common to the different ADs and would act as risk factors

[Familial multiple sclerosis in Canary Islands]. / Esclerosis múltiple familiar en Canarias.

INTRODUCTION: Multiple sclerosis (MS) is an autoimmune disease that occurs in genetically predisposed individuals. Its inheritance is polygenic. Genetic epidemiology studies have shown an increased familial aggregation. AIM. To determine the prevalence of familial MS (fMS) in a series of patients from the Canary Islands. PATIENTS AND METHODS: From a cohort of 266 patients with defined MS, during a 6-year period, we investigated prospectively by personal interviews the presence of MS on first and second degree relatives. We analysed as well the presence of HLA DRB1 in affected families, and also clinical and demographic characteristics in fMS and compared them with sporadic MS (sMS). RESULTS: fMS prevalence was 13.9% (27 non-related families with 50 affected individuals). The HLA DRB01*1501 allele were present in 51,8% of familial cases. We could not found either intrafamilial concordance in clinically affected regions and age of onset or clinical evolution. We have not found any phenotypic differences between familial and sMS. CONCLUSIONS: The prevalence of fMS in our series is comparable to that in other Mediterranean populations. Our results do not support that fMS was a different clinical entity of sMS and intrafamilial concordance in its clinical expression.

[Neurosyphilis: forms of presentation and clinical management]. / Neurosífilis: formas de presentación y manejo clínico.

INTRODUCTION AND AIMS: Neurosyphilis results from the infection of the central nervous system by Treponema pallidum. It causes diverse clinical pictures which are occasionally similar to other, better known neurological diseases. In this paper our aim is to offer a global clinical vision of this entity by reviewing the different forms it can take and its diagnostic and therapeutic management. DEVELOPMENT: The forms of presentation of neurosyphilis can be grouped in two categories: early (asymptomatic, meningeal and meningovascular neurosyphilis) and late (progressive general paralysis and tabes dorsalis). Other less important forms, such as gummas, ocular forms, syphilitic amyotrophy or hypoacusis, have also been described. Diagnosis is complex and is based on the study of the cerebrospinal fluid. Given the difficulty involved in performing an accurate diagnosis, different criteria have been developed in which T. pallidum serology plays a key role. The most effective treatment is penicillin, although on occasions it may have no effect and we therefore recommend clinical and fluid analysis follow ups. Lastly, we describe the changes in incidence and clinical presentation, and the complications that may arise in diagnosis when HIV carrying patients also suffer from this disease. CONCLUSIONS: Neurosyphilis is a disease that still occurs nowadays and, due to its clinical polymorphism, must be borne in mind as a differential diagnosis in a number of neurological and psychiatric illnesses. This, together with the fact the serological tests are difficult to interpret and its irregular response to the usual treatment, makes it difficult to manage and means that the neurologist must have a thorough knowledge of the disorder.

Neurosífilis:formas de presentación y manejo clínico / Neurosyphilis: forms of presentation and clinical management

Introducción y objetivo. La neurosífilis es la afectación del sistema nervioso central por Treponema pallidum. Provoca diversos cuadros clínicos que, en ocasiones, son similares a otras enfermedades neurológicas más conocidas. En este artículo se pretende dar una visión clínica global de esta entidad revisando sus formas de presentación y su manejo diagnóstico y terapéutico. Desarrollo. Las formas de presentación de la neurosífilis se agrupan en dos: precoces (neurosífilis asintomática, meníngea y meningovascular) y tardías (parálisis general progresiva y tabes dorsal). Además se han descrito otras de menor importancia, como el goma, formas oculares, amiotrofia sifilítica o hipoacusia. El diagnóstico es complejo y se basa en el estudio del líquido cefalorraquídeo. Debido a la dificultad para hacer un diagnóstico con certeza se han establecido diferentes criterios diagnósticos, en los que juega un papel fundamental la serología para T. pallidum. El tratamiento más eficaz es la penicilina, que en ocasiones puede no ser efectivo, por lo que se recomienda un seguimiento posterior clínico y licuoral. Finalmente, se describen los cambios en la incidencia y presentación clínica, así como las dificultades diagnósticas que pueden darse en los enfermos portadores del VIH que además sufran esta enfermedad. Conclusiones. La neurosífilis es una enfermedad aún presente en nuestros días, que ha de tenerse en cuenta como diagnóstico diferencial en múltiples enfermedades neurológicas y psiquiátricas dado su polimorfismo clínico. Este hecho, la difícil interpretación de los tests serológicos y su irregular respuesta al tratamiento habitual suponen una dificultad para su manejo, y para el neurólogo, la necesidad de conocerla con detalle (AU)