Utilidad de la ecografía de nervio óptico como predictor de progresión en esclerosis múltiple / Usefulness of optic nerve ultrasound to predict clinical progression in multiple sclerosis

Introducción: La pérdida neuronal/axonal progresiva se considera la causa más importante de discapacidad neurológica en la esclerosis múltiple (EM). El sistema visual está frecuentemente afectado en esta enfermedad y su accesibilidad a test funcionales y estructurales ha permitido que se convierta en un modelo para estudiar in vivo la patogenia de la EM. La ecografía orbitaria permite evaluar, de forma no invasiva y en tiempo real, las diversas estructuras de la órbita, incluido el nervio óptico.Material y métodosSe ha llevado a cabo un estudio observacional ambispectivo en pacientes con EM recogiéndose datos evolutivos de la enfermedad. La ecografía orbitaria se realizó en todos los pacientes según el principio de mínima potencia necesaria (ALARA). También se recogieron los datos de tomografía de coherencia óptica (OCT) en aquellos que tenían realizadas ambas pruebas. El estudio estadístico se efectuó con el programa SPSS 22.0.ResultadosSe encontraron correlaciones estadísticamente significativas entre las medidas ecográficas y la progresión de la enfermedad (p = 0,041 para el ojo derecho y p = 0,037 para el ojo izquierdo), y la Expanded Disability Status Scale (EDSS) final en el seguimiento (p = 0,07 para el ojo derecho y p = 0,043 para el ojo izquierdo). No fue así para los datos referentes a brotes y a otras variables clínicas.DiscusiónLa medición del diámetro del nervio óptico por ecografía podría utilizarse como medida predictiva en la evolución de la EM, ya que la disminución del mismo se asocia con progresión clínica y mayor discapacidad, medidas con la EDSS. (AU)

Introduction: Progressive neuronal and axonal loss are considered the main causes of disability in patients with multiple sclerosis (MS). The disease frequently involves the visual system; the accessibility of the system for several functional and structural tests has made it a model for the in vivo study of MS pathogenesis. Orbital ultrasound is a non-invasive technique that enables various structures of the orbit, including the optic nerve, to be evaluated in real time.Material and methodsWe conducted an observational, ambispective study of MS patients. Disease progression data were collected. Orbital ultrasound was performed on all patients, with power set according to the ‘as low as reasonably achievable’ (ALARA) principle. Optical coherence tomography (OCT) data were also collected for those patients who underwent the procedure. Statistical analysis was conducted using SPSS version 22.0.ResultsDisease progression was significantly correlated with ultrasound findings (P = .041 for the right eye and P = .037 for the left eye) and with Expanded Disability Status Scale (EDSS) score at the end of the follow-up period (P = .07 for the right eye and P = .043 for the left eye). No statistically significant differences were found with relation to relapses or other clinical variables.DiscussionUltrasound measurement of optic nerve diameter constitutes a useful, predictive factor for the evaluation of patients with MS. Smaller diameters are associated with poor clinical progression and greater disability (measured by EDSS). (AU)

Usefulness of optic nerve ultrasound to predict clinical progression in multiple sclerosis. / Utilidad de la ecografía de nervio óptico como predictor de progresión en esclerosis múltiple.

INTRODUCTION: Progressive neuronal and axonal loss are considered the main causes of disability in patients with multiple sclerosis (MS). The disease frequently involves the visual system; the accessibility of the system for several functional and structural tests has made it a model for the in vivo study of MS pathogenesis. Orbital ultrasound is a non-invasive technique that enables various structures of the orbit, including the optic nerve, to be evaluated in real time. MATERIAL AND METHODS: We conducted an observational, ambispective study of MS patients. Disease progression data were collected. Orbital ultrasound was performed on all patients, with power set according to the 'as low as reasonably achievable' (ALARA) principle. Optical coherence tomography (OCT) data were also collected for those patients who underwent the procedure. Statistical analysis was conducted using SPSS version 22.0. RESULTS: Disease progression was significantly correlated with ultrasound findings (P=.041 for the right eye and P=.037 for the left eye) and with Expanded Disability Status Scale (EDSS) score at the end of the follow-up period (P=.07 for the right eye and P=.043 for the left eye). No statistically significant differences were found with relation to relapses or other clinical variables. DISCUSSION: Ultrasound measurement of optic nerve diameter constitutes a useful, predictive factor for the evaluation of patients with MS. Smaller diameters are associated with poor clinical progression and greater disability (measured by EDSS).

Rebound after discontinuation of teriflunomide in patients with multiple sclerosis: 2 case reports.

Teriflunomide is an oral first-line disease modifying treatment (DMT) for patients with relapsing-remitting multiple sclerosis (RRMS). It can take up to two years to achieve systemic clearance of teriflunomide to an acceptable level, but this washout period may be accelerated by administration of cholestyramine. Relapse of multiple sclerosis (MS) during washout of teriflunomide or other first-line DMT is not as common. We report two patients with RRMS who experienced a relapse after the accelerated elimination period (AEP) of teriflunomide and confirmation of negative plasmatic levels (<0.02 µg/ml). In cases of risk of MS activity, we should not wait for teriflunomide negative plasmatic levels confirmation before starting the next DMT to reduce the risk of relapse.

Diagnóstico de síndrome de HaNDL / Diagnosis of HaNDL syndrome

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[Vitiligo with Koebner phenomenon in a patient with multiple sclerosis treated with alemtuzumab]. / Vitiligo con fenomeno de Koebner en una paciente con esclerosis multiple tratada con alemtuzumab.

TITLE: Vitiligo con fenomeno de Koebner en una paciente con esclerosis multiple tratada con alemtuzumab.

[Atypical Guillain-Barre syndrome clustering: is it necessary to reconsider the diagnostic criteria and microbiological protocol?] / Agrupacion de casos de sindrome de Guillain-Barre atipico: es necesario redefinir los criterios diagnosticos y los protocolos microbiologicos?

INTRODUCTION: Guillain-Barre syndrome is classically defined as a symmetrical ascending acute polyradiculoneuropathy, although there are atypical variants that make diagnosis difficult. CASE REPORTS: The medical data of six patients in our hospital area are collected during the first quarter of 2013. Lumbar punctures, imaging, neurophysiological studies, ganglioside antibodies and serologies have been proposed in all cases. We focus on the atypical features as late hyporeflexia, increased frequency of asymmetry and distal paresis and initial fever. From a neurophysiological point of view, all patients presented sensorimotor axonal forms. The most consistent datas in early studies is the F wave's alteration. A Miller Fisher variant associated with faciocervicobraquial paresis and cerebral reversible vasoconstriction syndrome has been detected. A bilateral brachial paresis and lumbar polyradiculopathy in the context of influenza A infection is other interesting case. The saltatory variant with cranial nerve involvement and lower limbs paresis has been demonstrated in one patient. Bands in cerebrospinal fluid are positive in three cases and anti-ganglioside antibodies in one patient. The syndrome of inappropriate secretion of antidiuretic hormone may explain some of the hyponatremias registered. The first line of treatment are inmunoglobulins in all patients. Plasmapheresis exchanges has been used as an additional therapy in four cases. CONCLUSIONS: These clusters of six axonal cases with atypical clinical features justifies the need for knowledge of these variants in order to achieve an early treatment. Late hyporeflexia and brachialfaciocervico, saltatory and lumbar forms should be considered in the spectrum of Guillain-Barre syndrome. The etiological study should rule out a lots of pathogens as influenza A.

TITLE: Agrupacion de casos de sindrome de Guillain-Barre atipico: es necesario redefinir los criterios diagnosticos y los protocolos microbiologicos?Introduccion. El sindrome de Guillain-Barre se define clasicamente como una polirradiculopatia aguda simetrica ascendente, si bien existen variantes atipicas que dificultan el diagnostico. Casos clinicos. Se recogen las historias clinicas de seis pacientes de nuestra area hospitalaria durante el primer trimestre de 2013. Se han realizado punciones lumbares, electroneurograma-electromiograma y analiticas con autoinmunidad en todos los casos. El conjunto de la muestra destaca por la presencia de caracteristicas atipicas, como hiporreflexia tardia, mayor frecuencia de asimetria y afectacion distal, asi como fiebre inicial. Desde el punto de vista neurofisiologico, todos los pacientes presentan formas axonales de tipo sensitivomotoras y las alteraciones de la onda F son el dato mas precoz. Se identifica una variante de sindrome de Miller Fisher asociada a paresia faciocervicobraquial y sindrome de vasoconstriccion cerebral reversible. Otro caso auna las variantes de paresia braquial bilateral y polirradiculopatia lumbar en el contexto de infeccion aguda por influenza A. La variante saltatoria ha sido demostrada en otro paciente. Todos los pacientes han recibido tratamiento con inmunoglobulinas, y en dos de ellos se sumo la plasmaferesis como terapia adicional. Conclusiones. La agrupacion de seis casos axonales con caracteristicas clinicas atipicas justifica la necesidad del conocimiento de estas variantes para lograr un diagnostico y un tratamiento precoz. La hiporreflexia tardia y las formas faciocervicobraquiales, saltatorias y lumbares deben considerarse dentro del espectro del sindrome de Guillain-Barre. El estudio etiologico debe incluir el cribado de numerosos patogenos, entre los que debe incluirse el virus influenza A.

Adaptación cultural y validación al español de España del MSTCQ© (Multiple Sclerosis Treatment Concerns Questionnaire) / Cultural adaptation and validation of a peninsular Spanish version of the MSTCQ© (Multiple Sclerosis Treatment Concerns Questionnaire)

Introducción: A pesar de la efectividad de los tratamientos inyectables para la esclerosis múltiple (EM), las reacciones adversas y el dolor pueden implicar problemas de satisfacción y adherencia. Se presenta la validación de la versión española del Multiple Sclerosis Treatment Concerns Questionnaire (MSTCQ)©, que evalúa la satisfacción con el dispositivo de autoinyección (DA), 4 dimensiones: sistema de inyección (A), efectos secundarios (B) (síntomas pseudogripales, reacciones, satisfacción), experiencia con el tratamiento (C) y beneficios (D). Métodos: Dos fases de estudio: 1) Adaptación cultural con expertos (n = 6) y pacientes (n = 27). 2) Estudio observacional, transversal y multicéntrico de validación. Se evaluaron 143 pacientes adultos con EM que utilizaban el DA Extavijec(TM) 30G. Cuestionarios: MSTCQ©; Patient-Reported Indices for Multiple Sclerosis (PRIMUS©), y Treatment Satisfaction Questionnaire for Medication (TSQM©). Propiedades psicométricas: factibilidad (% casos válidos y distribución de puntuaciones); fiabilidad (α-Cronbach) y test-retest (n = 41, coeficiente correlación intraclase [CCI]), y validez de constructo (análisis factorial A y B, [AF]) y convergente (Spearman-rho MSTCQ© versus TSQM©). Resultados. Edad media (DT) 41,94 (10,47) años, 63% mujeres, 88,11% con EM remitente-recurrente, media (DT) EDSS 2,68 (1,82) puntos. Alta cumplimentación del MSTCQ© (perdidos 0-2,80%). Alta consistencia interna: puntuación total (A + B) α = 0,89, por dimensiones (A, B y C) α = 0,76, 0,89 y 0,92, respectivamente. Excelente concordancia test-retest en las puntuación total (CC I= 0,98), por dimensiones (A, B y C) CCI = 0,82, 0,97 y 0,89, respectivamente. El AF corroboró la estructura interna del cuestionario original. Correlación moderada (Rho = 0,42-0,74) y significativa (p < 0,05 y p < 0,01) entre las puntuación total y por dimensiones del MSTCQ© y el TSQM©. Conclusiones. Se constatan adecuadas propiedades psicométricas de la versión española del MSTCQ

Introduction: Although subcutaneous treatments for multiple sclerosis (MS) have been shown to be effective, adverse reactions and pain may adversely affect treatment satisfaction and adherence. This study presents an adapted and validated Spanish version of the Multiple Sclerosis Treatment Concerns Questionnaire© (MSTCQ), which evaluates satisfaction with the injection device (ID) across 4 domains: injection system (A), side effects (B) (flu-like symptoms, reactions, and satisfaction), experience with treatment (C) and benefits (D). Methods: Two study phases: 1) Cultural adaptation process with input from experts (n = 6) and patients (n = 30). 2) Validation obtained by means of an observational, cross-sectional, multi-centre study evaluating 143 adult MS patients using an ID. Tools employed: MSTCQ©, Patient-Reported Indices for Multiple Sclerosis (PRIMUS©), and Treatment Satisfaction Questionnaire for Medication (TSQM©). Psychometric properties: Feasibility (percentage of valid cases and floor/ceiling effects); Reliability (Cronbach α) and test-retest correlation (n = 41, intraclass correlation coefficient, ICC); and construct validity (factor analysis of domains A and B) and convergent validity (Spearman rank-order correlation for MSTCQ© vs TSQM©). Results: Mean age (SD) was 41.94 (10.47) years, 63% of the group were women, and 88.11% presented relapsing-remitting MS. Mean (SD) EDSS score was 2.68 (1.82) points. MSTCQ© completion was high (0%-2.80% missing data). Internal consistency was high at α=0.89 for the total score (A+B) and α = 0.76, 0.89, and 0.92 for domains A, B, and C, respectively. The version demonstrated excellent test-retest reliability for the total (ICC = 0.98) and for domains A, B, and C: ICC = 0.82, 0.97, and 0.89, respectively. Factor analysis corroborated the internal structure of the original questionnaire. The association between total and domain scores on both the MSTCQ© and the TSQM© was moderately strong (Rho = 0.42-0.74) and significant (P < .05 and P < .01). Conclusion: The Spanish version of MSTCQ© demonstrates appropriate psychometric properties

Cultural adaptation and validation of a peninsular Spanish version of the MSTCQ© (Multiple Sclerosis Treatment Concerns Questionnaire). / Adaptación cultural y validación al español de España del MSTCQ© (Multiple Sclerosis Treatment Concerns Questionnaire).

INTRODUCTION: Although subcutaneous treatments for multiple sclerosis (MS) have been shown to be effective, adverse reactions and pain may adversely affect treatment satisfaction and adherence. This study presents an adapted and validated Spanish version of the Multiple Sclerosis Treatment Concerns Questionnaire© (MSTCQ), which evaluates satisfaction with the injection device (ID) across 4 domains: injection system (A), side effects (B) (flu-like symptoms, reactions, and satisfaction), experience with treatment (C) and benefits (D). METHODS: Two study phases: 1) Cultural adaptation process with input from experts (n=6) and patients (n=30). 2) Validation obtained by means of an observational, cross-sectional, multi-centre study evaluating 143 adult MS patients using an ID. Tools employed: MSTCQ©, Patient-Reported Indices for Multiple Sclerosis (PRIMUS©), and Treatment Satisfaction Questionnaire for Medication (TSQM©). Psychometric properties: Feasibility (percentage of valid cases and floor/ceiling effects); Reliability (Cronbach α) and test-retest correlation (n=41, intraclass correlation coefficient, ICC); and construct validity (factor analysis of domains A and B) and convergent validity (Spearman rank-order correlation for MSTCQ© vs TSQM©). RESULTS: Mean age (SD) was 41.94 (10.47) years, 63% of the group were women, and 88.11% presented relapsing-remitting MS. Mean (SD) EDSS score was 2.68 (1.82) points. MSTCQ© completion was high (0%-2.80% missing data). Internal consistency was high at α=0.89 for the total score (A+B) and α=0.76, 0.89, and 0.92 for domains A, B, and C, respectively. The version demonstrated excellent test-retest reliability for the total (ICC=0.98) and for domains A, B, and C: ICC=0.82, 0.97, and 0.89, respectively. Factor analysis corroborated the internal structure of the original questionnaire. The association between total and domain scores on both the MSTCQ© and the TSQM© was moderately strong (Rho=0.42-0.74) and significant (P<.05 and P<.01). CONCLUSION: The Spanish version of MSTCQ© demonstrates appropriate psychometric properties.

Comparison of quality of life between two clinical conditions with immunosuppressive therapy: liver transplantation and multiple sclerosis.

OBJECTIVE: We aimed to compare quality of life in two clinical conditions treated with immunosuppressants: cadaveric liver transplant recipients and multiple sclerosis patients. We also assessed the clinical significance of these results regarding a representative age-adjusted sample of the general Spanish population. METHODS: Using a cross-sectional design, the SF-36 Health Survey was used to evaluate 62 patients with these chronic conditions (31 in each group) who were matched for gender. An analysis of covariance was performed to control for the influence of time from multiple sclerosis diagnosis and liver transplantation surgery until assessment. Student t test of covariate-adjusted mean values was used as the statistical test and Cohen's d effect size index, to assess the magnitude of intergroup differences and assess clinical significance. RESULTS: Significantly worse scores were observed among the neurological patients compared with transplant recipients regarding role-physical (P = .038), general health (P = .003), vitality (P = .034), and physical functioning (P = .049), with medium effect sizes (Cohen's ds from -0.511 to -0.785). Against normative values, liver transplant recipients displayed relevant differences in all SF-36 subscales (Cohen's ds from -0.569 to -0.974) except for mental health (small effect size). Likewise, multiple sclerosis patients showed much greater differences versus the general population (Cohen's ds from -0.846 to -1.760). CONCLUSIONS: Liver transplant recipients showed better quality of life than multiple sclerosis patients (medium effect sizes) in physical quality-of-life dimensions. Interestingly, despite having controlled for time from diagnosis/transplantation, both medical conditions showed clinically significant impairments (large and medium effect sizes) in physical and psychosocial quality-of-life domains. We concluded that transplant recipients belong to a population that still requires special health care because, even after having undergone their treatment of choice, they do not achieve normal levels of biopsychosocial functioning.

[A study of the quality of life in cases of multiple sclerosis]. / Estudio de la calidad de vida en la esclerosis múltiple.

AIMS: Our objective was to study the quality of life (QoL) in a sample of patients with multiple sclerosis (MS) as measured by their scores on the QoL questionnaire SF-36 (Short Form 36 Health Survey) and its relationship with the extent of deterioration of the disease according to the EDSS (Expanded Disability Status Scale). PATIENTS AND METHODS: The QoL SF-36 scale was administered to 78 patients with defined MS. Those who had suffered outbreaks or whose condition had clearly deteriorated in the past 6 months were excluded, and variables such as age, sex and gait disorders, among others, were analysed. Patients were divided into three groups according to their degree of disability on the EDSS scale: group 1 (EDSS 0-3), group 2 (EDSS 3.5-5.5) and group 3 (EDSS > 6). RESULTS: There were significant differences in the mean scores of groups 1 and 3 in several dimensions of the QoL questionnaire (p < 0.05): general state of health, emotional role, physical functioning, physical role and body pain. Differences were also observed in physical functioning and physical role in groups 1 and 2. In group 1, the dimensions with the lowest mean score were body pain and vitality. In group 3, the dimension with the lowest score was physical functioning. The health dimensions belonging to the realm of mental health were the ones that reflected the smallest difference according to the stage, since no statistical significance existed for dimensions such as vitality, social functioning or mental health. Body pain and vitality were more affected at an early stage (group 1) and the effects on physical functioning were stronger at a later stage (group 3). CONCLUSIONS: As the disease progresses (EDSS > 6), the dimensions of the QoL SF-36 questionnaire concerning physical health (physical functioning) become affected. Body pain and vitality are the most strongly affected dimensions in the early stages of the disease. These findings should be confirmed in a larger sample in order to provide them with a higher degree of validity.

Estudio de la calidad de vida en la esclerosis múltiple / A study of the quality of life in cases of multiple sclerosis

Objetivo. Estudiar la calidad de vida (CdV) de una muestra de pacientes con esclerosis múltiple (EM) en función de su puntuación en el cuestionario de CdV SF-36 (del inglés Short Form 36 Health Survey) y su relación con el grado de deterioro de la enfermedad según la escala EDSS (del inglés Expanded Disability Status Scale). Pacientes y métodos. Se cuantificó la escala de CdV SF-36 en 78 pacientes con EM definida. Se excluyeron a aquellos que sufrieron brotes o claro empeoramiento los últimos 6 meses, y se analizaron variables de edad, sexo, alteraciones de la marcha, entre otros. Se establecieron tres grupos, sobre la base de los grados de discapacidad en la escala EDSS: grupo 1 (EDSS 0-3), grupo 2 (EDSS 3,5-5,5) y grupo 3 (EDSS > 6). Resultados. Existen diferencias significativas en las puntuaciones medias de los grupos 1 y 3 en varias dimensiones del cuestionario de CdV (p < 0,05): salud general, rol emocional, función física, rol físico, y dolor corporal. También se encontraron diferencias para los grupos 1 y 2 en la función física y rol físico. En el grupo 1, las dimensiones con menor puntuación media fueron: dolor corporal y vitalidad. En el grupo 3, la de menor puntuación fue la función física. Las dimensiones de salud pertenecientes al ámbito de la salud mental fueron las que reflejaron una menor diferencia en función del estadio, puesto que no existe significación estadística para dimensiones como vitalidad, función social o salud mental. El dolor corporal y la vitalidad estarían más afectadas en un estadio temprano (grupo 1), y la función física en un estadio tardío (grupo 3). Conclusiones. A medida que progresa la enfermedad (EDSS > 6), se afectan las dimensiones del cuestionario de CdV SF-36 relacionadas con la salud física (función física). Dolor corporal y vitalidad serían las dimensiones más afectadas en un estadio temprano de la enfermedad. Estos resultados se deberían confirmar en una muestra más amplia para adquirir mayor validez (AU)

Aims. Our objective was to study the quality of life (QoL) in a sample of patients with multiple sclerosis (MS) as measured by their scores on the QoL questionnaire SF-36 (Short Form 36 Health Survey) and its relationship with the extent of deterioration of the disease according to the EDSS (Expanded Disability Status Scale). Patients and methods. The QoL SF-36 scale was administered to 78 patients with defined MS. Those who had suffered outbreaks or whose condition had clearly deteriorated in the past 6 months were excluded, and variables such as age, sex and gait disorders, among others, were analysed. Patients were divided into three groups according to their degree of disability on the EDSS scale: group 1 (EDSS 0-3), group 2 (EDSS 3.5-5.5) and group 3 (EDSS > 6). Results. There were significant differences in the mean scores of groups 1 and 3 in several dimensions of the QoL questionnaire (p < 0.05): general state of health, emotional role, physical functioning, physical role and body pain. Differences were also observed in physical functioning and physical role in groups 1 and 2. In group 1, the dimensions with the lowest mean score were body pain and vitality. In group 3, the dimension with the lowest score was physical functioning. The health dimensions belonging to the realm of mental health were the ones that reflected the smallest difference according to the stage, since no statistical significance existed for dimensions such as vitality, social functioning or mental health. Body pain and vitality were more affected at an early stage (group 1) and the effects on physical functioning were stronger at a later stage (group 3). Conclusions. As the disease progresses (EDSS > 6), the dimensions of the QoL SF-36 questionnaire concerning physical health (physical functioning) become affected. Body pain and vitality are the most strongly affected dimensions in the early stages of the disease. These findings should be confirmed in a larger sample in order to provide them with a higher degree of validity (AU)

Aspectos quirúrgicos de las enfermedades producidas por priones / Surgical aspects of diseases caused by prions

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[Bilateral frontal infarct with imitation and utilization syndrome in a case of giant cell arteritis]. / Infarto frontal bilateral con síndrome de imitación y utilización en un caso de arteritis de células gigantes.

Giant cell arteritis (GCA) is a vasculitis which involves in a systemic fashion the mean and medium sized arteries. Intracranial vessels are exceptionally involved. Thus, stroke is an uncommon complication. We report a patient with typical GCA with blindness due to occlusion of the central artery of the retina, who developed an imitation and utilization behavior attributable to bilateral frontal infarction without accompanying neurological symptoms. Although histological study was not available, this case fulfilled all of Russi's criteria for GCA localized in the CNS. This lends support to the suspicion that the bilateral frontal infarction might be due to it.