Neurological presentations of Bartonella henselae infection.

OBJECTIVE: Neurological symptoms in patients with cat-scratch disease (CSD) have been rarely reported. The aim of this study is to analyze the frequency of neurological CSD (NCSD) and describe the disease clinical presentation, management and outcome. MATERIAL AND METHODS: We retrospectively selected patients with a CSD syndrome and Bartonella IgG titers > 1:256. Data regarding epidemiological, clinical, management, and follow-up features were analyzed and discussed. A comparison between NCSD and non-neurological CSD (NNCSD) was established. RESULTS: Thirty-nine CSD patients were selected. NCSD frequency was 10.25%. No children were found affected in the NCSD group. A 65.7% of NNCSD and the entirety of the NCSD group had a history of cat exposure. Immunosuppression was only present in the NNCSD group (8.6%). NCSD presentations were as follows: isolated aseptic meningitis (25%), neuroretinitis (50%), and isolated optic neuritis (25%). A greater proportion of patients in the NCSD group had fever and raised levels of acute phase reactants and white blood cells. 85.7% of NNCSD had a complete recovery, whereas only 50% of the NCSD patients experienced a full recovery. CONCLUSION: NCSD may be a distinctive group compared to NNCSD due to its later age of presentation, the more intense systemic response, and the poorer outcome.

Alemtuzumab treatment in real clinical practice: Experience in a multicenter cohort.

BACKGROUND: Alemtuzumab is a highly effective treatment for relapsing remitting multiple sclerosis (RRMS), but in recent years safety-related concerns had emerged due to description of novel serious side effects not registered in CARE-MS I and CARE-MS II phase 3 studies, nor in TOPAZ extension study. Data about alemtuzumab use in real clinical practice are limited and based mainly on retrospective studies with small sample sizes. Therefore, more information about effectiveness and safety of alemtuzumab in this context is needed. METHODS: A multicenter observational prospective study to investigate effectivity and safety of alemtuzumab in a real-world setting was performed. Primary endpoints were the change in annualized relapse rate (ARR), and in disability measured by EDSS score. Secondary endpoints were the cumulative probability of confirmed 6-month disability improvement and worsening. Disability worsening and disability improvement were considered when the EDSS score was increased or decreased, respectively, in 1 point if baseline EDSS score was <5.0, or in 0.5 point if baseline EDSS score was ≥5.5, confirmed over 6 months. Other secondary endpoint was the proportion of patients who achieved NEDA-3 status (absence of clinical relapses, disability EDSS progression, and MRI disease activity as depicted by new/enlarging T2 lesions or Gadolinium enhancing T1 lesions). Adverse events also were recorded. RESULTS: A total of 195 RRMS patients (70% female) who started alemtuzumab treatment were included. Mean of follow-up was 2.38 years. Alemtuzumab significantly reduced the annualized relapse rate from baseline with risk reductions of 86%, 83.5%, and 84%, at 12, 24, and 36 months of follow-up respectively (Friedman test, p-value < 0.05 for all comparisons). Alemtuzumab also significantly reduced EDSS score over one and two years after starting alemtuzumab treatment (Friedman test, p-value<0.001 for both comparisons). A high proportion of patients presented confirmed 6-month stability or disability improvement (92%, 82%, and 79%, over 1, 2 and 3 years of follow-up respectively). The proportion of patients who retained NEDA-3 status at 12, 24 and 36 months were 61%, 49%, and 42%, respectively. Baseline characteristics associated with a lower probability of achieving NEDA-3 were younger age, sex female, high ARR, elevated number of previous treatments, and switch from a second line therapy. Infusion related reactions were the most frequent adverse event observed. The most common infections were urinary tract infections (50%), and upper respiratory tract infections (19%) over the 3 years of follow- up. Secondary thyroid autoimmunity was developed in 18.5% of patients. CONCLUSION: Alemtuzumab has demonstrated in real clinical practice high effectiveness in controlling multiple sclerosis activity, and no unexpected adverse events were observed.

Progression of a series of patients with relapsing-remitting multiple sclerosis treated for 7 years with natalizumab using the "no evidence of disease activity" parameter.

INTRODUCTION: The safety and effectiveness of natalizumab in patients with relapsing-remitting multiple sclerosis (RRMS) has been demonstrated in clinical trials. However, due to the limitations of these trials, it is important to know how the condition behaves under long-term clinical practice conditions. OBJECTIVE: To determine the long-term effectiveness of natalizumab in patients with RRMS by means of annual evaluation of the "no evidence of disease activity" (NEDA) parameter, which includes number of relapses, disability (measured with the Expanded Disability Status Scale), and brain MRI parameters. PATIENTS AND METHODS: We performed a retrospective study of patients with RRMS from 3 centres who were treated with one or more doses of natalizumab. Each year, we evaluated NEDA status and safety based on the percentage of patients who discontinued treatment with natalizumab and experienced adverse reactions. RESULTS: The study included 89 patients, most of whom received treatment for 2 to 4 years, with a follow-up period of up to 7 years. Natalizumab significantly reduces the radiological and clinical progression of the disease, as well as the annual rate of relapses. The NEDA parameter demonstrates the effectiveness of the drug, with values of 75.28% for year one and 66.67% for year 7. Twenty-five patients (28.1%) dropped out after a median of 4 years. Fourteen of these patients (56%) dropped out due to the appearance of anti-JC virus antibodies, either in isolation or associated with another cause. Four dropouts (16%) were due to treatment ineffectiveness, with one patient dying due to progressive multifocal leukoencephalopathy. CONCLUSIONS: Natalizumab is highly effective as measured by the NEDA long-term remission parameter.

[Fingolimod: effectiveness and safety in routine clinical practice. An observational, retrospective, multi-centre study in Galicia]. / Fingolimod: efectividad y seguridad en la practica clinica habitual. Estudio observacional, retrospectivo y multicentrico en Galicia.

INTRODUCTION: The effectiveness and safety of fingolimod in patients with relapsing-remitting multiple sclerosis (RRMS) have been proven in clinical trials. Yet, due to their limitations, it is important to know how it behaves under everyday clinical practice conditions. Hence, the aim of this study is to evaluate the effectiveness and safety of fingolimod after 12 months' usage in clinical practice in Galicia. PATIENTS AND METHODS: We conducted a retrospective, multi-centre study (n = 8) of patients with RRMS who were treated with one or more doses of fingolimod, 0.5 mg/day. Effectiveness was assessed -annualised relapse rate (ARR), changes in the score on the Expanded Disability Status Scale (EDSS), percentage of patients free from relapses, free from progression of disability and free from activity in resonance- for the total number of patients and according to previous treatment. Safety was assessed based on the percentage of patients who withdrew and presented adverse side effects. RESULTS: After 12 months' use, fingolimod reduced the ARR by 87% (1.7 to 0.23; p < 0.0001) and, consequently, 81% of patients were free from relapses. The score was reduced by 9%. In all, 91% of patients were free from progression of disability and 72% were free from resonance activity. No signs of disease activity were found in 43% of the patients. Most of the benefits of fingolimod differed depending on previous treatment. About a third of the patients reported adverse side effects, but only 2% of them withdrew for this reason. CONCLUSIONS: In clinical practice, most of the results on the effectiveness of the clinical trials conducted with fingolimod were observed during the first 12 months of treatment. A better safety profile was observed than that reported in the clinical trials.

TITLE: Fingolimod: efectividad y seguridad en la practica clinica habitual. Estudio observacional, retrospectivo y multicentrico en Galicia.Introduccion. La efectividad y seguridad del fingolimod en pacientes con esclerosis multiple remitente recurrente (EMRR) se demostro en ensayos clinicos. Sin embargo, por las limitaciones de estos, es importante saber como se comporta en condiciones de practica clinica habitual. Asi, el objetivo de este estudio es evaluar la efectividad y seguridad del fingolimod despues de 12 meses de uso en la practica clinica en Galicia. Pacientes y metodos. Estudio retrospectivo y multicentrico (n = 8) de pacientes con EMRR y tratados con una o mas dosis de fingolimod, 0,5 mg/dia. Se evaluo la efectividad ­tasa anualizada de brotes (TAB), cambio en la puntuacion de la escala expandida del estado de discapacidad (EDSS), porcentaje de pacientes libres de brotes, libres de progresion de discapacidad y libres de actividad en resonancia­ para el total de pacientes y segun tratamiento previo. Se evaluo la seguridad a partir del porcentaje de pacientes que discontinuaron y que presentaron efectos adversos. Resultados. Despues de 12 meses de uso, el fingolimod redujo un 87% la TAB (de 1,7 a 0,23; p < 0,0001) y, en consecuencia, un 81% de pacientes estuvo libre de brotes. La puntuacion de la EDSS disminuyo un 9%. Un 91% de pacientes estuvo libre de progresion de discapacidad y un 72%, libre de actividad en resonancia. En el 43% de los pacientes no se evidenciaron signos de la actividad de la enfermedad. La mayoria de los beneficios del fingolimod difirieron segun el tratamiento previo. Alrededor de un tercio de los pacientes comunicaron efectos adversos, pero solo el 2% discontinuo debido a ellos. Conclusiones. La mayoria de los resultados de efectividad de los ensayos clinicos del fingolimod se observa durante los 12 primeros meses de tratamiento en la practica clinica. Se observo un mejor perfil de seguridad al comunicado en los ensayos clinicos.

Evolución de una serie de pacientes con esclerosis múltiple remitente-recurrente tratados con natalizumab durante 7 años mediante el parámetro no evidencia enfermedad activa / Progression of a series of patients with relapsing-remitting multiple sclerosis treated for 7 years with natalizumab using the “no evidence of disease activity” parameter

Introducción: La efectividad y seguridad de natalizumab en pacientes con esclerosis múltiple remitente recurrente (EMRR) se demostró en ensayos clínicos. Sin embargo, por las limitaciones de estos es importante saber cómo se comporta en condiciones de práctica clínica a largo plazo.ObjetivoConocer la eficacia a largo plazo de natalizumab en pacientes con EMRR mediante la evaluación anual del no evidence of disease activity (NEDA), que incluye número de brotes, discapacidad medida con EDSS y parámetros de RM cerebral.Pacientes y métodosEstudio retrospectivo y multicéntrico (n = 3) de pacientes con EMRR tratados con una o más dosis de natalizumab. Se evaluó el estado NEDA cada año y la seguridad a partir del porcentaje de pacientes que discontinuaron y que presentaron efectos adversos.ResultadosIncluimos 89 pacientes, la mayoría recibieron tratamiento durante 2 a 4 años, con una duración del seguimiento de hasta 7 años. Natalizumab reduce significativamente la progresión radiológica y clínica de la enfermedad, así como la tasa anual de brotes, demostrándose su eficacia con el parámetro NEDA, 75,28% al primer año y 66,67% al séptimo año. Veinticinco pacientes (28,1%) han abandonado el estudio en una mediana de tiempo de 4 años, 14 pacientes (56%) por aparición de anticuerpos contra el virus JC, como causa única o asociada a otro motivo, 4 abandonos (16%) fueron por ineficacia, un paciente falleció a causa de LMP.ConclusionesNatalizumab presenta una alta eficacia medida mediante el parámetro de remisión NEDA a largo plazo. (AU)

Introduction: The safety and effectiveness of natalizumab in patients with relapsing-remitting multiple sclerosis (RRMS) has been demonstrated in clinical trials. However, due to the limitations of these trials, it is important to know how the condition behaves under long-term clinical practice conditions.ObjectiveTo determine the long-term effectiveness of natalizumab in patients with RRMS by means of annual evaluation of the “no evidence of disease activity” (NEDA) parameter, which includes number of relapses, disability (measured with the Expanded Disability Status Scale), and brain MRI parameters.Patients and methodsWe performed a retrospective study of patients with RRMS from 3 centres who were treated with one or more doses of natalizumab. Each year, we evaluated NEDA status and safety based on the percentage of patients who discontinued treatment with natalizumab and experienced adverse reactions.ResultsThe study included 89 patients, most of whom received treatment for 2 to 4 years, with a follow-up period of up to 7 years. Natalizumab significantly reduces the radiological and clinical progression of the disease, as well as the annual rate of relapses. The NEDA parameter demonstrates the effectiveness of the drug, with values of 75.28% for year one and 66.67% for year 7. Twenty-five patients (28.1%) dropped out after a median of 4 years. Fourteen of these patients (56%) dropped out due to the appearance of anti–JC virus antibodies, either in isolation or associated with another cause. Four dropouts (16%) were due to treatment ineffectiveness, with one patient dying due to progressive multifocal leukoencephalopathy.ConclusionsNatalizumab is highly effective as measured by the NEDA long-term remission parameter. (AU)

[Myasthenia gravis and treatment with intravenous immunoglobulins. Reply]. / Miastenia grave y tratamiento con inmunoglobulinas por via intravenosa. Replica.

Miastenia grave y tratamiento con inmunoglobulinas por via intravenosa. Replica.

[Dystonia: their epidemiology, aetiology, diagnosis and treatment]. / Distonías: epidemiologia, etiología, diagnóstico y tratamiento.

INTRODUCTION: Dystonia is the second most common movement disorder after Parkinsonism. No exact figures are available on the incidence/prevalence of the different forms of dystonia, because the data vary considerably depending on the source, method and ethnic origin of the population under study. AIMS: To describe and summarise our current knowledge of the epidemiology, causation, diagnosis and treatment of dystonias. DEVELOPMENT: Dystonia is a movement disorder characterised by sustained muscular contractions that cause repeated twisting movements and abnormal postures. Dystonias can be classified according to their distribution, aetiology, clinical course and age at onset. A correct classification is very useful for evaluating the complementary tests that are needed, as well as the prognosis and treatment of the process. The diagnosis of dystonia is essentially clinical and is confirmed with electromyography. Both the different laboratory and neuroimaging studies are fundamentally used to help in the aetiological classification and to rule out secondary causes of dystonia. Among the different treatments that exist today (intrathecal, infiltrative, surgical, systemic pharmacotherapy), special attention should be given to the role of botulinum toxin as the preferred treatment in most cases of focal dystonias. CONCLUSIONS: Exhaustive epidemiological studies are needed to be able to get a more precise picture of the epidemiology of dystonias. Despite the great amount of progress made in recent years as far as genetics and neuroimaging are concerned, the diagnosis of dystonia remains essentially clinical. Botulinum toxin is the preferred treatment in most cases of focal dystonias.

[A case-control study of the influence of the smoking behaviour in multiple sclerosis]. / Estudio de casos y controles sobre la influencia del hábito tabáquico en la esclerosis múltiple.

INTRODUCTION: Several epidemiological studies have demonstrated that there is a genetic factor of susceptibility in Multiple Sclerosis (MS) and that the environmental factors play an important important role in their development. Smoking is among the environment factors studied. In fact, several studies have established a relationship between smoking and multiple sclerosis, although most of them did not find significant results or found that these were contradictory. OBJECTIVE: To evaluate the influence of the smoking habit on the risk of suffering MS. METHODS: This was a case-control matched study with 138 patients diagnosed of MS according to the McDonald criteria who were paired with the same number of controls of the same gender, residents in the same city and having the same age +/-2 years. Demographic data, smoking status (never, always smokers, ex-smokers), Kurtzke disability status scale (EDSS) and type of MS were collected. RESULTS: Out of a total of 138 MS patients (93 women, 43 men), 110 had relapsing-remitting MS, 20 secondary progressive MS and 7 primary progressive MS. Most of the patients were smokers and ex-smokers (63%). In the control group, only the 41,3% were smokers/ex-smokers. Moreover, the age of onset for smoking was earlier in the case group. CONCLUSION: Being a smoker/ex-smoker implies a 27% greater risk of developing MS compared to those who have never smoked. This risk is statistically significant for women but not for men due to the low number of them in the sample.

Demencia por cuerpos de Lewy / Dementia with Lewy bodies

La demencia por cuerpos de Lewy (DCL) es una enfermedad neurodegenerativa, que supone en torno al 10-25% de todas las demencias de la población general y es la segunda causa de demencia degenerativa, en el anciano, tras la enfermedad de Alzheimer. Se caracteriza clínicamente por deterioro cognitivo, con rasgos de demencia frontal, acompañado de: fluctuaciones, parkinsonismo y alucinaciones visuales. Su confirmación diagnóstica se realiza por anatomía patológica posmortem: presencia de abundantes cuerpos de Lewy (CL) en las neuronas de la corteza y en otras zonas cerebrales. Los CL son inclusiones intracitoplasmáticas eosinófilas, esféricas y están constituidos por más de 20 componentes proteicos. Se han definido, en el año 2005, unos criterios diagnósticos para facilitar su reconocimiento y poder etiquetar a los pacientes de posible o probable DCL. La causa de la DCL es desconocida; se supone que, al igual que en la enfermedad de Parkinson, influyen factores ambientales y genéticos. No hay ninguna prueba complementaria ni marcador biológico específico de esta enfermedad, pero la combinación de biomarcadores, los tests neuropsicológicos y las pruebas de neuroimagen son de especial ayuda en el diagnóstico. El tratamiento de la enfermedad es complejo; porque los fármacos que mejoran unos síntomas pueden empeorar otros. En cualquier caso solo hay posibilidad de tratamiento sintomático (AU)

Dementia with Lewy bodies (DLB) is a neurodegenerative disease, accounting for about 10-25% of all dementias in the general population and is the second most common type of degenerative dementia in elderly people after Alzheimer's disease. Clinically, it is characterized by cognitive impairment (with features of frontal dementia) accompanied by fluctuating cognition, parkinsonism and visual hallucinations. Diagnostic confirmation is made by pathologic autopsy: abundant presence of Lewy bodies (LB) in the neurons of the cortex and other areas in the brain. The LB are eosinophilic intracytoplasmic inclusions, spherical and consist of more than 20 protein components. Diagnostic criteria to facilitate recognition of patients with possible or probable DLB were defined in 2005. The etiology of DLB is unknown, although is assumed that, environmental factors and genetic influence, like in Parkinson's disease. There are not additional evidence or specific biological markers of this disease, but neuropsychological and neuroimaging tests are helpful in diagnosis. The treatment of the disease is difficult, there is no cure, only symptomatic. Some drugs may improve some symptoms but also can worse others (AU)

Distonías: epidemiología, etiología, diagnóstico y tratamiento / Destonia: Thir epidemiology, aetiology, diagnosis and treatment

Resumen. Introducción. La distonía es el segundo trastorno del movimiento más frecuente después de los parkinsonismos. No se conocen con exactitud las cifras de incidencia/prevalencia de las distintas formas de distonía, ya que las cifras varían considerablemente en función de la fuente, método de estudio y origen étnico de la población estudiada. Objetivo. Describir y resumir los conocimientos actuales sobre la epidemiología, etiología, diagnóstico y tratamiento de las distonías. Desarrollo. La distonía es un trastorno del movimiento caracterizado por contracciones musculares sostenidas que causan movimientos de torsión repetidos y posturas anómalas. Las distonías pueden clasificarse según su distribución, etiología, curso clínico y edad de inicio. Una correcta clasificación resulta muy útil de cara a evaluar las pruebas complementarías necesarias, el pronóstico y el tratamiento del proceso. El diagnóstico de distonía es fundamentalmente clínico y se confirma con electromiografía. Tanto los diversos estudios de laboratorio como de neuroimagen sirven fundamentalmente para ayudar en la clasificación etiológica y para descartar causas secundarias de distonía. Dentro de los diversos tratamientos existentes hoy en día (farmacoterapia sistémica, intratecal, infiltrativa, quirúrgica), cabe destacar el papel de la toxina botulínica como tratamiento de elección en la mayor parte de las distonías focales. Conclusiones. Es necesaria la realización de estudios epidemiológicos exhaustivos que permitan conocer con mayor exactitud la epidemiología de las distonías. A pesar del gran avance en los últimos años en cuanto a genética y neuroimagen, el diagnóstico de la distonía sigue siendo fundamentalmente clínico. La toxina botulínica es el tratamiento de elección en la mayor parte de las distonías focales (AU)

Summary. Introduction. Dystonia is the second most common movement disorder after Parkinsonism. No exact figures are available on the incidence/prevalence of the different forms of dystonia, because the data vary considerably depending on the source, method and ethnic origin of the population under study. Aims. To describe and summarise our current knowledge of the epidemiology, causation, diagnosis and treatment of dystonias. Development. Dystonia is a movement disorder characterised by sustained muscular contractions that cause repeated twisting movements and abnormal postures. Dystonias can beclassified according to their distribution, aetiology, clinical course and age at onset. A correct classification is very useful for evaluating the complementary tests that are needed, as well as the prognosis and treatment of the process. The diagnosis of dystonia is essentially clinical and is confirmed with electromyography. Both the different laboratory and neuroimaging studies are fundamentally used to help in the aetiological classification and to rule out secondary causes of dystonia. Among he different treatments that exist today (intrathecal, infiltrative, surgical, systemic pharmacotherapy), special attention should be given to the role of botulinum toxin as the preferred treatment in most cases of focal dystonias. Conclusions. Exhaustive epidemiological studies are needed to be able to get a more precise picture of the epidemiology of dystonias. Despite the great amount of progress made in recent years as far as genetics and neuroimaging are concerned, the diagnosis of dystonia remains essentially clinical. Botulinum toxin is the preferred treatment in most cases of focal dystonias (AU)

Distonía en el curso de una encefalitis de Ramussen de inicio tardío / No disponible

No disponible

Estudio de casos y controlessobre la influencia del hábito tabáquicoen la esclerosis múltiple / A case-control study of the influence of the smoling behaviour in multiple sclerosis

Introducción. Diversos estudios epidemiológicos demuestranque en la esclerosis múltiple (EM) existe un factor genético de susceptibilidad,así como que los factores ambientales juegan un papelprominente en el desarrollo de la misma. Entre los factores ambientalesestudiados se encuentra el tabaco. De hecho, varios estudiosestablecen relación entre fumar y EM, pero la mayoría de ellos nohallaron resultados significativos o éstos fueron contradictorios.Objetivo. Evaluar la influencia del hábito tabáquico en el riesgode padecer EM.Material y métodos: Estudio caso-control pareado con 138 pacientesdiagnosticados de EM según los criterios de McDonald y el mismonúmero de controles del mismo sexo, residentes en el mismo municipioy la misma edad ±2 años. Se recogieron los datos demográficos, statusde fumar, escala de discapacidad de Kurtzke (EDSS) y tipo de EM.Resultados. De los 138 pacientes (93 mujeres, 43 hombres), 110presentaban EM remitente recurrente, 20 EM secundariamente progresivay 7 EM primariamente progresiva. La mayoría de los pacientesresultaron ser fumadores y exfumadores (63%) frente al (41,3%)de los controles. Asimismo, la edad de inicio en el hábito de fumar fuemás precoz en los casos que en los controles.Conclusión. Ser fumador/exfumador implica un 27% más deriesgo de desarrollar EM frente a los nunca fumadores. Este riesgo esestadísticamente significativo en mujeres y no en varones, probablementedebido al bajo número de los mismos en el total de la muestra (AU)

Introduction. Several epidemiological studies have demonstratedthat there is a genetic factor of susceptibility in MultipleSclerosis (MS) and that the environmental factors play an importantrole in their development. Smoking is among the environmentfactors studied. In fact, several studies have established arelationship between smoking and multiple sclerosis, althoughmost of them did not find significant results or found that thesewere contradictory.Objective. To evaluate the influence of the smoking habit onthe risk of suffering MS.Methods. This was a case-control matched study with 138patients diagnosed of MS according to the McDonald criteria whowere paired with the same number of controls of the same gender,residents in the same city and having the same age ±2 years.Demographic data, smoking status (never, always smokers, exsmokers),Kurtzke disability status scale (EDSS) and type of MSwere collected.Results. Out of a total of 138 MS patients (93 women, 43 men),110 had relapsing-remitting MS, 20 secondary progressive MS and7 primary progressive MS. Most of the patients were smokersand ex-smokers (63%). In the control group, only the 41,3% weresmokers/ex-smokers. Moreover, the age of onset for smoking wasearlier in the case group.Conclusion. Being a smoker/ex-smoker implies a 27% greaterrisk of developing MS compared to those who have neversmoked. This risk is statistically significant for women but not formen due to the low number of them in the sample (AU)