Diagnostic delay and outcome in immunocompetent patients with primary central nervous system lymphoma in Spain: a multicentric study.

INTRODUCTION: To assess the management of immunocompetent patients with primary central nervous system lymphomas (PCNSL) in Spain. METHODS: Retrospective analysis of 327 immunocompetent patients with histologically confirmed PCNSL diagnosed between 2005 and 2014 in 27 Spanish hospitals. RESULTS: Median age was 64 years (range: 19-84; 33% ≥ 70 years), 54% were men, and 59% had a performance status (PS) ≥ 2 at diagnosis. Median delay to diagnosis was 47 days (IQR 24-81). Diagnostic delay > 47 days was associated with PS ≥ 2 (OR 1.99; 95% CI 1.13-3.50; p = 0.016) and treatment with corticosteroids (OR 2.47; 95% CI 1.14-5.40; p = 0.023), and it did not improve over the years. Patients treated with corticosteroids (62%) had a higher risk of additional biopsies (11.7% vs 4.0%, p = 0.04) but corticosteroids withdrawal before surgery did not reduce this risk and increased the diagnostic delay (64 vs 40 days, p = 0.04). Median overall survival (OS) was 8.9 months [95% CI 5.9-11.7] for the whole series, including 52 (16%) patients that were not treated, and 14.1 months (95%CI 7.7-20.5) for the 240 (73.4%) patients that received high-dose methotrexate (HD-MTX)-based chemotherapy. Median OS was shorter in patients ≥ 70 years (4.1 vs. 13.4 months; p < 0.0001). Multivariate analysis identified age ≥ 65 years, PS ≥ 2, no treatment, and cognitive/psychiatric symptoms at diagnosis as independent predictors of short survival. CONCLUSIONS: Corticosteroids withdrawal before surgery does not decrease the risk of a negative biopsy but delays diagnosis. In this community-based study, only 73.4% of patients could receive HD-MTX-based chemotherapy and OS remains poor, particularly in elderly patients ≥ 70 years.

[Use of steroids in neuro-oncology]. / Uso de esteroides en neurooncologia.

Corticosteroids are widely used in routine clinical practice in neuro-oncology. However, despite their widespread use, there is no established consensus on their indications, dosages and dosage schedule. This article reviews the mechanism of action of corticosteroids, their indications for use, side effects and the appropriate management, dosage and duration of therapy, as well as the role of new treatments currently being use to treat cerebral oedema. The most widely used corticosteroid in neuro-oncology is dexamethasone, probably due to its low mineralocorticoid effect and its long half-life. Its effect on cerebral oedema is fundamental in the symptomatic control of patients with brain tumours. It is recommended to start treatment only in symptomatic patients, in doses of 4-8 mg/24 h, always seeking the minimum effective dosage. Side effects associated with corticosteroid use are common and have a negative impact on patients' quality of life.

TITLE: Uso de esteroides en neurooncologia.Los corticoides se utilizan ampliamente en la practica clinica habitual en neurooncologia. Sin embargo, pese a lo extendido de su uso, no existe un consenso establecido sobre sus indicaciones, dosis y posologia. En este articulo se realiza una revision bibliografica del mecanismo de accion de los corticoides, sus indicaciones de uso, efectos secundarios y manejo adecuado, dosis y duracion de la terapia, asi como el papel de nuevos tratamientos actuales para el edema cerebral. El corticoide con un uso mas extendido en neurooncologia es la dexametasona, probablemente por su escaso efecto mineralocorticoide y por su elevada vida media. Su efecto sobre el edema cerebral es fundamental en el control sintomatico de los pacientes con tumores cerebrales. Se recomienda iniciar tratamiento unicamente en pacientes sintomaticos, en dosis de 4-8 mg/24 h, buscando siempre la minima dosis eficaz. Los efectos secundarios asociados al uso de corticoides son frecuentes e impactan negativamente en la calidad de vida de los pacientes.

Correlación neurorradiológica de las afasias. Mapa cortico-subcortical del lenguaje / Neuroradiologic correlation with aphasias. Cortico-subcortical map of language

La afasia es una alteración adquirida del lenguaje debida a una lesión cerebral, que se caracteriza por errores en la producción, la denominación o la comprensión del lenguaje. Aunque la mayoría de las afasias suelen ser mixtas, desde un punto de vista práctico se clasifican en diferentes tipos según sus rasgos clínicos principales: afasia de Broca, afasia de Wernicke, afasia de conducción, afasia transcortical y alexia con o sin agrafia. Presentamos los hallazgos clínicos de los principales subtipos representándolos con casos radiológicos, y proporcionamos una revisión actualizada de la red del lenguaje con imágenes de resonancia funcional y de tractografía

Aphasia is an acquired language disorder due to a cerebral lesion; it is characterized by errors in production, denomination, or comprehension of language. Although most aphasias are mixed, from a practical point of view they are classified into different types according to their main clinical features: Broca's aphasia, Wernicke's aphasia, conduction aphasia, transcortical aphasia, and alexia with or without agraphia. We present the clinical findings for the main subtypes of aphasia, illustrating them with imaging cases, and we provide an up-to-date review of the language network with images from functional magnetic resonance imaging and tractography

Neuroradiologic correlation with aphasias. Cortico-subcortical map of language. / Correlación neurorradiológica de las afasias. Mapa cortico-subcortical del lenguaje.

Aphasia is an acquired language disorder due to a cerebral lesion; it is characterized by errors in production, denomination, or comprehension of language. Although most aphasias are mixed, from a practical point of view they are classified into different types according to their main clinical features: Broca's aphasia, Wernicke's aphasia, conduction aphasia, transcortical aphasia, and alexia with or without agraphia. We present the clinical findings for the main subtypes of aphasia, illustrating them with imaging cases, and we provide an up-to-date review of the language network with images from functional magnetic resonance imaging and tractography.

Síndrome de boca ardiente: descripción clínica, planteamiento fisiopatológico y una nueva opción terapéutica / Burning mouth syndrome: clinical description, pathophysiological approach, and a new therapeutic option

Introducción. El síndrome de la boca ardiente se define como sensación de ardor intrabucal, en ausencia de lesiones locales o patología sistémica que lo justifique. Se trata de una entidad con pobre respuesta a los tratamientos comúnmente utilizados, que puede resultar muy discapacitante. Métodos. Analizamos prospectivamente las características clínicas, demográficas y la respuesta a tratamiento de 6 casos de síndrome de la boca ardiente diagnosticados en las consultas de cefaleas de 2 hospitales de tercer nivel. Resultados. Se trata de 6 pacientes de sexo femenino, con edades entre 34 y 82 años, que referían síntomas compatibles con síndrome de la boca ardiente. En 5 pacientes, las molestias empeoraban a última hora del día y 4 referían mejoría de los síntomas con los movimientos linguales. En todos los casos la exploración neurológica fue normal, los estudios analíticos no mostraron alteraciones que justificaran los síntomas y en el examen odontológico no se evidenciaron lesiones intrabucales. Todas las pacientes habían sido tratadas previamente con los tratamientos convencionales, sin mejoría. Se instauró pramipexol a dosis entre 0,36mg y 1,05mg al día, con lo que se consiguió mejoría evidente en todos los casos, que persiste tras una media de 4 años de seguimiento. Conclusiones. El síndrome de la boca ardiente sigue siendo una entidad de etiología desconocida, que comparte ciertos patrones clínicos y respuesta al tratamiento con el síndrome de piernas inquietas. Los agonistas dopaminérgicos deberían considerarse como tratamiento de primera línea en esta entidad (AU)

Introduction. Burning mouth syndrome is defined as scorching sensation in the mouth in the absence of any local lesions or systemic disease that would explain that complaint. The condition responds poorly to commonly used treatments and it may become very disabling. Methods. We prospectively analysed the clinical and demographic characteristics and response to treatment in 6 cases of burning mouth syndrome, diagnosed at 2 tertiary hospital headache units. Results. Six female patients between the ages of 34 and 82 years reported symptoms compatible with burning mouth syndrome. In 5 of them, burning worsened at the end of the day; 4 reported symptom relief with tongue movements. Neurological examinations and laboratory findings were normal in all patients and their dental examinations revealed no buccal lesions. Each patient had previously received conventional treatments without amelioration. Pramipexol was initiated in doses between 0.36mg and 1.05mg per day, resulting in clear improvement of symptoms in all cases, a situation which continues after a 4-year follow up period. Conclusions. Burning mouth syndrome is a condition of unknown aetiology that shares certain clinical patterns and treatment responses with restless leg syndrome. Dopamine agonists should be regarded as first line treatment for this entity (AU)

Burning mouth syndrome: Clinical description, pathophysiological approach, and a new therapeutic option. / Síndrome de boca ardiente: descripción clínica, planteamiento fisiopatológico y una nueva opción terapéutica.

INTRODUCTION: Burning mouth syndrome is defined as scorching sensation in the mouth in the absence of any local lesions or systemic disease that would explain that complaint. The condition responds poorly to commonly used treatments and it may become very disabling. METHODS: We prospectively analysed the clinical and demographic characteristics and response to treatment in 6 cases of burning mouth syndrome, diagnosed at 2 tertiary hospital headache units. RESULTS: Six female patients between the ages of 34 and 82 years reported symptoms compatible with burning mouth syndrome. In 5 of them, burning worsened at the end of the day; 4 reported symptom relief with tongue movements. Neurological examinations and laboratory findings were normal in all patients and their dental examinations revealed no buccal lesions. Each patient had previously received conventional treatments without amelioration. Pramipexol was initiated in doses between 0.36mg and 1.05mg per day, resulting in clear improvement of symptoms in all cases, a situation which continues after a 4-year follow up period. CONCLUSIONS: Burning mouth syndrome is a condition of unknown aetiology that shares certain clinical patterns and treatment responses with restless leg syndrome. Dopamine agonists should be regarded as first line treatment for this entity.

Gabapentina en el tratamiento de la neuromiotonía ocular / Gabapentin in the treatment of ocular neuromyotonia

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