[Intracranial lipomas in paediatrics: a retrospective study of 20 patients]. / Lipomas intracraneales en pediatría: estudio retrospectivo de 20 pacientes.

INTRODUCTION: Intracranial lipomas (ICL) are congenital malformations that are due to an anomalous differentiation of the primitive meninges. AIMS: The purpose of this study is to determine the most frequent symptoms related to ICL in the paediatric age, as well as to evaluate whether they are directly related to their location and to their association with other brain malformations. PATIENTS AND METHODS: A retrospective clinico-radiological study was conducted involving 20 paediatric patients diagnosed as suffering from ICL between 1985 and 2003 at three hospitals in the Valencian Community. RESULTS: 70% of the cases were females and the average age when ICL was diagnosed was 5.3 years (interval from 2 months to 13 years). The most common initial clinical justification for the neuroradiological study was psychomotor retardation in eight patients (40%) and persistent, predominantly migrainous, headaches in six of them (30%). Only one patient was examined because of epilepsy. The prevailing site was the interhemispheric fissure in 10 patients (50%), followed by the quadrigeminal cistern in five (25%) and the mamillary body infundibulum axis in three of them (15%). One case was located in the cerebellum and another was found in the lateral ventricle, with involvement of the choroid fissure. Associated anomalies were observed in eight patients (40%). Alterations were seen in the development of the corpus callosum in six patients (30%), five in an isolated way and in one case there was partial absence of the associated septum pellucidum. Another patient was found to have an isolated partial agenesis of the septum and Goldenhar syndrome was detected in another case. The radiological follow up, which varied according to the initial clinical features, did not show any differences in the morphology or the size of the lipoma. CONCLUSIONS: 1) ICL are more frequently located in the interhemispheric fissure and in the quadrigeminal cistern; 2) In these locations they can be associated with other brain malformations, the most important being defects in the callosa and septa; 3) Most ICL were found incidentally during the course of a neuroimaging examination. Only in the case of three patients (15%) was there any presumed relation between the location of the ICL (pericallosal), the symptomatology (overall psychomotor retardation) and the associated malformation (hypoplasia of the corpus callosum); 4) The incidence of epilepsy was much lower than that claimed in earlier reports, and the predominant associated neurological symptomatology was found to be psychomotor retardation and headache (70%); 5) ICL is a developmental anomaly that does not require neurosurgical treatment and which does not usually change with the passage of time, although it is liable to increase because of adipose hypertrophy.

Lipomas intracraneales en pediatría: estudio retrospectivo de 20 pacientes / Intracranial lipomas in paediatrics: a retrospective study of 20 patients

Introducción. Los lipomas intracraneales (LIC) son malformaciones congénitas que se originan por una diferenciación anómala de la meninge primitiva. Objetivo. Conocer los síntomas que se relacionan más frecuentemente con los LIC en la edad pediátrica, así como valorar si tienen relación directa con su ubicación y con su asociación a otras malformaciones cerebrales. Pacientes y métodos. Se ha realizado un estudio clinicorradiológico retrospectivo de 20 pacientes pediátricos con LIC, diagnosticados entre 1985 y 2003, en tres hospitales de la Comunidad Valenciana. Resultados. El 70 por ciento de los casos son mujeres, y la edad media cuando se diagnóstico el LIC fue de 5,3 años (intervalo, de 2 meses a 13 años). La justificación clínica inicial más frecuente del estudio neurorradiológico fue retraso psicomotor, en ocho pacientes REV NEUROL 2003; 37 (6): 515-521 (40 por ciento), y cefalea persistente de predominio migrañoso, en seis (30 por ciento). Sólo un paciente se estudió por epilepsia. La localización dominante ha sido la cisura interhemisférica, en 10 pacientes (50 por ciento), seguida de la cisterna cuadrigémina, en cinco (25 por ciento), y el eje infundibulomamilar, en tres (15 por ciento). Un caso se localizó en el cerebelo, y otro en el ventrículo lateral con extensión a la cisura coroidea. Se observaron anomalías asociadas en ocho pacientes (40 por ciento). En seis pacientes (30 por ciento) se observó una alteración en el desarrollo del cuerpo calloso, en cinco de manera aislada y en uno con ausencia parcial asociada del septo pelúcido. En otro paciente se observó una agenesia parcial aislada del septo, y en otro caso se detectó un síndrome de Goldenhar. El seguimiento radiológico, variable según la clínica inicial, no mostró diferencias en la morfología ni en el tamaño del lipoma. Conclusiones. 1) Los LIC se localizan con mayor frecuencia en la cisura interhemisférica y en la cisterna cuadrigeminal; 2) En estas localizaciones pueden asociarse a otras malformaciones cerebrales, fundamentalmente defectos callosos y septales; 3) La mayor parte de los LIC representan un hallazgo incidental de neuroimagen; sólo en tres pacientes (15 por ciento) hubo una presunta relación entre la ubicación del LIC (pericallosa), la sintomatología (retraso psicomotor global) y la malformación asociada (hipoplasia del cuerpo calloso); 4) La incidencia de epilepsia ha sido mucho más baja que la descrita previamente, y se halló como sintomatología neurológica asociada predominante el retraso psicomotor y la cefalea (70 por ciento); 5) El LIC es una anomalía malformativa que no precisa tratamiento neuroquirúrgico y que no suele modificarse con el tiempo, aunque es susceptible de aumentar por hipertrofia adiposa (AU)

Introduction. Intracranial lipomas (ICL) are congenital malformations that are due to an anomalous differentiation of the primitive meninges. Aims. The purpose of this study is to determine the most frequent symptoms related to ICL in the paediatric age, as well as to evaluate whether they are directly related to their location and to their association with other brain malformations. Patients and methods. A retrospective clinico-radiological study was conducted involving 20 paediatric patients diagnosed as suffering from ICL between 1985 and 2003 at three hospitals in the Valencian Community. Results. 70% of the cases were females and the average age when ICL was diagnosed was 5.3 years (interval from 2 months to 13 years). The most common initial clinical justification for the neuroradiological study was psychomotor retardation in eight patients (40%) and persistent, predominantly migrainous, headaches in six of them (30%). Only one patient was examined because of epilepsy. The prevailing site was the interhemispheric fissure in 10 patients (50%), followed by the quadrigeminal cistern in five (25%) and the mamillary body-infundibulum axis in three of them (15%). One case was located in the cerebellum and another was found in the lateral ventricle, with involvement of the choroid fissure. Associated anomalies were observed in eight patients (40%). Alterations were seen in the development of the corpus callosum in six patients (30%), five in an isolated way and in one case there was partial absence of the associated septum pellucidum. Another patient was found to have an isolated partial agenesis of the septum and Goldenhar syndrome was detected in another case. The radiological follow-up, which varied according to the initial clinical features, did not show any differences in the morphology or the size of the lipoma. Conclusions. 1) ICL are more frequently located in the interhemispheric fissure and in the quadrigeminal cistern; 2) In these locations they can be associated with other brain malformations, the most important being defects in the callosa and septa; 3) Most ICL were found incidentally during the course of a neuroimaging examination. Only in the case of three patients (15%) was there any presumed relation between the location of the ICL (pericallosal), the symptomatology (overall psychomotor retardation) and the associated malformation (hypoplasia of the corpus callosum); 4) The incidence of epilepsy was much lower than that claimed in earlier reports, and the predominant associated neurological symptomatology was found to be psychomotor retardation and headache (70%); 5) ICL is a developmental anomaly that does not require neurosurgical treatment and which does not usually change with the passage of time, although it is liable to increase because of adipose hypertrophy (AU)

[Ischemic stroke secondary to post varicella angiopathy in a 3 year old male]. / Accidente cerebrovascular isquémico secundario a angiopatía posvaricela en varón de 3 años.

INTRODUCTION: Ischemic cerebrovascular disease is an infrequent problem in the paediatric age, but one which we now understand better thanks to modern neuroimaging techniques. We know little about its aetiopathogenesis, which is very varied, and it has been reported as being associated with viral infections such as chicken pox. CASE REPORT: A male, aged 3 years, who presented sudden hemiparesis, facial paralysis and dysarthria, without any other accompanying neurological symptoms. The patient had suffered a bout of chicken pox 6 weeks earlier. Of the abnormal complementary explorations, the most notable were varicella positive serological tests and MRI and MR angiography that pointed to ischemic involvement of the cortex. The patient progressed very well and the clinical features had completely reverted at five weeks. CONCLUSION: Post varicella angiopathy is one of the acquired risk factors for an ischemic stroke, and has been claimed to account for up to a third of all strokes in infants. Neuroimaging techniques allow the topographic determination of the ischemic repercussions. Patients usually progress well and in most cases the clinical features completely revert within a short time. We conclude that chicken pox should be included in the vaccination schedule as soon as possible in order to prevent complications that, as in the case we have reported, can be very serious.

Accidente cerebrovascular isquémico secundario a angiopatía posvaricela en varón de 3 años / Ischemic stroke secondary to post-varicella angiopathy in a 3-year-old male

Introducción. La enfermedad cerebrovascular isquémica es un problema poco frecuente en la edad pediátrica, pero cada vez mejor estudiado con las modernas técnicas de neuroimagen. La etiopatogenia es poco conocida y muy variada; se ha descrito su asociación con infecciones virales, como la varicela. Caso clínico. Varón de 3 años, que presenta un cuadro brusco de hemiparesia, parálisis facial y disartria, sin otra sintomatología neurológica acompañante. Muestra antecedente de varicela 6 semanas antes. De entre las exploraciones complementarias anormales, destaca la serología positiva para varicela y la RM y la angiorresonancia magnética cerebrales, indicativas de la afectación isquémica cortical. La evolución fue muy satisfactoria y el cuadro revirtió por completo a las cinco semanas. Conclusión. Entre los factores de riesgo adquiridos de un ictus isquémico, se encuentra la angiopatía posvaricela, que se ha llegado a responsabilizar de hasta la tercera parte de los accidentes cerebrovasculares isquémicos de la infancia. Con las técnicas de neuroimagen se pueden perfilar topográficamente las repercusiones isquémicas. La evolución suele ser muy satisfactoria y el cuadro revertir de forma completa con prontitud en la mayoría de las ocasiones. Se concluye con la necesidad de incluirla varicela en el calendario vacunal lo antes posible, para poder evitar complicaciones que en muchas ocasiones, como la que se describe, son muy serias (AU)

Encefalomielitis aguda diseminada postinfecciosa / No disponible

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