Meningocele de la vaina del nervio óptico asociado a coloboma / Optic nerve sheath meningocele associated with coloboma

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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.

[Reversible "man-in-the-barrel" syndrome caused by hypoxic-ischemic encephalopathy]. / Síndrome del "hombre en barril" reversible causado por encefalopatía hipóxico-isquémica.

INTRODUCTION: Hypoxic-ischemic encephalopathy can lead to different clinical presentations, such as bilateral cortical borderzone infarctions due to cerebral hypoperfusion (especially after cardiopulmonary arrest), which would cause a "man-in-the-barrel" syndrome if the anterior circulation is affected. This syndrome has multiple etiologies, both central (bilateral frontal and/or pyramidal decussation lesions and cervical spinal cord lesions) as well as peripheral ones such as multifocal motor neuropathy. CASE REPORT: We report the case of a 19-year-old man who developed a "man-in-the-barrel" syndrome with bilateral and proximal upper limb brachial diplegia after bilateral watershed infarctions involving distal fields of the middle and the anterior cerebral artery due to cerebral hypoperfusion in a comatose patient without internal carotid obstruction. CONCLUSIONS: The rate of death in this syndrome is higher than 90% and it predicts a worse outcome in comatose patients. Nevertheless, our patient has had complete clinical improvement.

Síndrome del «hombre en barril»reversible causado por encefalopatíahipóxico-isquémica / Reversible «man-in-the-barrel» syndrome caused by hypoxic-ischemic encephalopathy

Introducción. La encefalopatía hipóxico-isquémica puede producirdiferentes cuadros clínicos, entre los que se engloban aquellosproducidos por hipoperfusión en territorio frontera que pueden originarun síndrome del «hombre en barril» si está afectada la circulaciónanterior. Este síndrome presenta múltiples etiologías, tanto centrales(lesiones frontales bilaterales y/o en la decusación piramidal ylesiones en la médula espinal cervical) como periféricas como la neuropatíamotora multifocal.Caso clínico. Presentamos el caso clínico de un paciente de19 años que presentó un síndrome del «hombre en barril» con diplejíabraquial bilateral de carácter proximal secundario a lesiones bilateralespor hipoperfusión en territorio frontera de la circulación anterioren el contexto de hipotensión en un estado comatoso, pero sinestenosis de arterias carótidas internas.Conclusiones. Este síndrome clínico presenta una tasa de mortalidadsuperior al 90 %, y se considera un factor de mal pronósticoen pacientes en coma. No obstante, en nuestro caso el paciente hapresentado una completa resolución clínica (AU)

Introduction. Hypoxic-ischemic encephalopathy can lead todifferent clinical presentations, such as bilateral cortical borderzoneinfarctions due to cerebral hypoperfusion (especially aftercardiopulmonary arrest), which would cause a «man-in-the-barrel» syndrome if the anterior circulation is affected. This syndromehas multiple etiologies, both central (bilateral frontal and/orpyramidal decussation lesions and cervical spinal cord lesions) aswell as peripheral ones such as multifocal motor neuropathy.Case report. We report the case of a 19 year old man whodeveloped a «man-in-the-barrel» syndrome with bilateral andproximal upper limb brachial diplegia after bilateral watershedinfarctions involving distal fields of the middle and the anteriorcerebral artery due to cerebral hypoperfusion in a comatose patientwithout internal carotid obstruction.Conclusions. The rate of death in this syndrome is higherthan 90% and it predicts a worse outcome in comatose patients.Nevertheless, our patient has had complete clinical improvement (AU)

[Cerebral haemorrhage in patients treated with oral anticoagulation]. / Hemorragia cerebral en pacientes en tratamiento con anticoagulantes orales.

INTRODUCTION: Intracranial haemorrhage (ICH) during dicumarinic treatment is a complication related to anticoagulation intensity with a high level of mortality. The aim of our study is to analize etiology, location and outcome of intracerebral haemorrhages related with anticoagulant therapy. PATIENTS AND METHODS: Over 401 spontaneous intracranial haemorrhages consecutively admitted in the neurological ward, we analyzed the acenocumarol ICH by location, anticoagulation range and factors that conditioned the outcome. RESULTS: We identified 26 patients, 6.5% of total ICH. Mean age was 75.2 +/- 7.9 years-old, over the rest of ICH. International Normalized Ratio (INR) was less than 2 in 10 patients, between 2 and 3 in six and greater than 3 in ten patients. 8 patients (31%) died, three of them had multiple ICH, but none of them had a INR greater than 2. Dicumarinic haemorrhages were of lobar location in 14 cases (three of them multiples) and deep in 12 cases. CONCLUSIONS: In our study, dicumarinic ICH are responsible of 6.5% total intracranial haemorrhages and they are not in clear relation with excessive anticoagulation. Mortality is slightly greater than the other ICH. Relatively benignity of these patients, the age and lobar location suggest that the etiology of these haemorrhages can be related to a subjacent amyloid angiopathy.

Hemorragia cerebral en pacientes en tratamiento con anticoagulantes orales / Cerebral haemorrhage in patients treated with oral anticoagulation

Introducción. Se considera que la hemorragia cerebral (HIC) en pacientes en tratamiento con dicumarínicos (acenocumarol) es una complicación relacionada con el grado de anticoagulación y que comporta una alta mortalidad. El objetivo del estudio es analizar la etiopatogenia, topografía y pronóstico de las HIC por anticoagulantes orales. Pacientes y métodos. Sobre 401 HIC espontáneas ingresadas consecutivamente en nuestra unidad se han analizado las HIC en pacientes en tratamiento con acenocumarol en cuanto a localización, intervalo de anticoagulación y factores que condicionan el pronóstico. Resultados. Se han identificado 26 pacientes, un 6,5% del total de HIC. La edad media fue de 75,2 ± 7,9 años, superior al resto de HIC. El INR (International Normalized Ratio) fue inferior a 2 en 10 pacientes, entre 2-3 en seis y mayor de 3 en 10. Ocho pacientes (31%) fallecieron, tres presentaban HIC múltiples, pero ninguno tenía un INR superior a 2. Las HIC por dicumarínicos fueron lobares en 14 casos (en tres múltiples) y profundas en 12 casos. Conclusiones. En nuestro estudio las HIC por acenocumarol fueron responsables del 6,5% del total de HIC y no se relacionaron claramente con una anticoagulación excesiva. La mortalidad de las HIC por anticoagulantes orales es levemente superior al del resto de las HIC. La relativa benignidad de estos pacientes, junto con la edad y la localización lobar, sugieren que la etiología de estas hemorragias pueda estar relacionada con una angiopatía amiloide cerebral subyacente

Introduction. Intracranial haemorrhage (ICH) during dicumarinic treatment is a complication related to anticoagulation intensity with a high level of mortality. The aim of our study is to analize etiology, location and outcome of intracerebral haemorrhages related with anticoagulant therapy. Patients and methods. Over 401 spontaneous intracranial haemorrhages consecutively admitted in the neurological ward, we analyzed the acenocumarol ICH by location, anticoagulation range and factors that conditioned the outcome. Results. We identified 26 patients, 6.5% of total ICH. Mean age was 75.2 ±7.9 years-old, over the rest of ICH. International Normalized Ratio (INR) was less than 2 in 10 patients, between 2 and 3 in six and greater than 3 in ten patients. 8 patients (31%) died, three of them had multiple ICH, but none of them had a INR greater than 2. Dicumarinic haemorrhages were of lobar location in 14 cases (three of them multiples) and deep in 12 cases. Conclusions. In our study, dicumarinic ICH are responsible of 6.5% total intracranial haemorrhages and they are not in clear relation with excessive anticoagulation. Mortality is slightly greater than the other ICH. Relatively benignity of these patients, the age and lobar location suggest that the etiology of these haemorrhages can be related to a subjacent amyloid angiopathy

Neuritis óptica bilateral y síndrome de hipertensión endocraneal en una paciente afecta de linfoma no Hodgkin / Bilateral optic neuritis and endocranial hypertension syndrome in a woman with no-Hodgkin lymphoma

La afectación orbitaria es una complicación poco frecuente en los linfomas no Hodgkin, pudiendo manifestarse como pérdida de agudeza visual a consecuencia de una neuritis óptica. En aquellos casos en que se acompaña de deterioro del nivel de consciencia y clínica progresiva de hipertensión endocraneal debe descartarse la presencia de una carcinomatosis meníngea. El diagnóstico precoz en urgencias del cuadro de hipertensión endocraneal, y de la causa del mismo, permitirá instaurar un tratamiento precoz y de esa forma evitar las posibles secuelas que pudieran producirse. Presentamos el caso de una paciente afecta de un linfoma no Hodgkin en fase de remisión, que acudió a nuestro Servicio de Urgencias por un cuadro de pérdida de agudeza visual y en la que se diagnosticó un síndrome de hipertensión endocraneal. El rápido diagnóstico de una carcinomatosis meníngea fue fundamental para la evolución posterior de la paciente (AU)