Seudotumor cerebri en niños: etiología, características clínicas y evolución / Pseudotumour cerebri in children: Aetiology, clinical features, and progression

Introducción: El síndrome de hipertensión intracraneal idiopática o seudotumor cerebri (STC) en niños está en constante revisión, respecto a su definición, etiologías asociadas, diagnóstico y terapéutica más apropiada. Objetivos y métodos: Se revisaron los casos de STC < 15 años de edad en un hospital de referencia en los últimos 12 años. Se estudiaron las características clínico-epidemiológicas y el procedimiento diagnóstico-terapéutico empleado. Se definió STC como presión intracraneal > 25cmH2O por punción lumbar (PL), con estudio de resonancia magnética cerebral sin lesión ocupante de espacio. Resultados: Se registró a 12 niños con STC, media de edad de 10 años, 90% mujeres. Todos presentaban peso normal. El 82% manifestaba síntomas: cefalea (66%), diplopía (8%) o baja visión (8%). Todos asociaban papiledema (17% unilateral). La PL fue diagnóstica en el 100% y la neuroimagen fue normal en el 91%. Se evidenció un posible desencadenante en 5 casos (2 farmacológico y 3 infeccioso por Mycoplasma pneumoniae [M. pneumoniae]). El 91% recibió tratamiento médico: en el 75% consistió en PL repetidas y en el 42% solo acetazolamida y/o prednisona. La evolución fue favorable en todos ellos. Conclusiones: La incidencia de STC fue de aproximadamente 1/100.000 niños/año, similar a estudios previos. En esta población, el sobrepeso no es un factor de riesgo. La infección por M. pneumoniae podría actuar como desencadenante de STC y favorecer recurrencias tardías. La ausencia de síntomas parece independiente del grado de presión intracraneal. El tratamiento con acetazolamida es eficaz en la mayoría de los casos, desterrando el uso de PL repetidas

Introduction: The definition, associated aetiologies, diagnosis, and treatment of idiopathic intracranial hypertension, or pseudotumour cerebri (PTC), are constantly being revised in the paediatric population. Objectives and methods: Our study included children younger than 15 years old with PTC and attended at a reference hospital in the past 12 years. We analysed the clinical and epidemiological features of our sample and the diagnostic and treatment approaches. PTC was defined as presence of intracranial hypertension (CSF opening pressure> 25 cmH2O) and absence of space-occupying lesions in brain MR images. Results: A total of 12 children with PTC were included; mean age was 10 years and 90% were girls. Weight was normal in all patients. Eighty-two percent of the patients had symptoms: headache (66%), diplopia (8%), and visual loss (8%). All of them displayed papilloedema (17% unilaterally). Lumbar puncture (LP) provided the diagnosis in all cases and 91% showed no relevant MRI findings. A potential cause of PTC was identified in 5 cases: pharmacological treatment in 2 and infection (Mycoplasma pneumoniae [M. pneumoniae]) in 3. Ninety-one per cent of the patients received treatment: 75% underwent several LPs and 42% received acetazolamide and/or prednisone. Outcomes were favourable in all cases. Conclusions: The incidence of PTC was estimated at approximately 1 case per 100 000 children/years, in line with data reported by previous studies. Overweight was not found to be a risk factor for PTC in this population. M. pneumoniae infection may trigger PTC and cause recurrences at later stages. The absence of symptoms seems to be independent from the degree of intracranial hypertension. Acetazolamide treatment is effective in most cases, and it represents a viable alternative to repeated LP

Pseudotumour cerebri in children: Aetiology, clinical features, and progression. / Seudotumor cerebri en niños: etiología, características clínicas y evolución.

INTRODUCTION: The definition, associated aetiologies, diagnosis, and treatment of idiopathic intracranial hypertension, or pseudotumour cerebri (PTC), are constantly being revised in the paediatric population. OBJECTIVES AND METHODS: Our study included children younger than 15 years old with PTC and attended at a reference hospital in the past 12 years. We analysed the clinical and epidemiological features of our sample and the diagnostic and treatment approaches. PTC was defined as presence of intracranial hypertension (CSF opening pressure>25cmH2O) and absence of space-occupying lesions in brain MR images. RESULTS: A total of 12 children with PTC were included; mean age was 10 years and 90% were girls. Weight was normal in all patients. Eighty-two percent of the patients had symptoms: headache (66%), diplopia (8%), and visual loss (8%). All of them displayed papilloedema (17% unilaterally). Lumbar puncture (LP) provided the diagnosis in all cases and 91% showed no relevant MRI findings. A potential cause of PTC was identified in 5 cases: pharmacological treatment in 2 and infection (Mycoplasma pneumoniae [M. pneumoniae]) in 3. Ninety-one per cent of the patients received treatment: 75% underwent several LPs and 42% received acetazolamide and/or prednisone. Outcomes were favourable in all cases. CONCLUSIONS: The incidence of PTC was estimated at approximately 1 case per 100 000 children/years, in line with data reported by previous studies. Overweight was not found to be a risk factor for PTC in this population. M. pneumoniae infection may trigger PTC and cause recurrences at later stages. The absence of symptoms seems to be independent from the degree of intracranial hypertension. Acetazolamide treatment is effective in most cases, and it represents a viable alternative to repeated LP.

Síndrome PFAPA, un reto para el pediatra / PFAPA syndrome: a challenge in pediatrics

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Experiencia con anti-CD20 (Rituximab) en cuatro pacientes con púrpura trombocitopénica / Experience with anti-CD20 (Rituximab) in four patients with thrombocytopenic purpura

La púrpura trombocitopénica idiopática (PTI) en la infancia se presenta en más de un 80% de los casos de forma aguda y, escasamente un 20%, evoluciona en un primer momento a formas crónicas. De éstas, a los 2 años de evolución de la enfermedad y tras varias opciones terapéuticas, sólo un 2-3% continúan con trombocitopenias, la mayoría sin repercusión sobre la actividad normal. En estos casos crónicos, el rituximab, anticuerpo monoclonal anti-CD, es una alternativa terapéutica teniendo en cuenta los riesgos de la esplenectomía en niños pequeños. Presentamos resultados de pacientes afectos de PTI crónica, refractaria al tratamiento establecido en el protocolo de la Sociedad Española de Hematología Pediátrica (SEHP, con trombocitopenia menor de ./,,3 plaquetas, tratados con anticuerpo monoclonal anti-CD, Rituximab. Uno de los pacientes, dada la ausencia de respuesta a los tratamientos instaurados y siendo la evolución de la PTI solamente de dos meses, se planteó esta alternativa, teniendo en cuenta además que había siendo diagnosticado y tratado de una enfermedad de Hodgkin dos años antes

Immune Thrombocytopenic purpura in childhood (ITP) clinical presentation is in 80% of cases as acute form and only 20% as chronic. Two years later after different treatments, 2-3% going to chronic ITP, most of them without serious activity. Rituximab, anti-CD20 antibody is an alternative treatmetn so we can avoid the splenectomy in paediatric age. We present 4 patients with chronic ITP, refractory after treatment with protocol of Haematologic Spanish Society, with thrombocytopeny less than 20.000mm3, treated with anti-CD20 antibody, Rituximab. One of them begun the treatment only 2 months after diagnosis because he has a lot of hemorraghic problems and he had been treated of a Hodgkin disease 2 years before