Síndrome de Guillain-Barré: presentación clínica y evolución en menores de 6 años de edad / Guillain-Barré syndrome: clinical presentation and prognosis in children under six years-old

Introducción: El síndrome de Guillain-Barré (SGB) es una polineuropatía aguda de difícil diagnóstico en la primera infancia. Objetivos: Revisar la forma de presentación del SGB en niños menores de 6 años atendiendo al tiempo de evolución y sintomatología que presentaron hasta el diagnóstico, los hallazgos en las pruebas complementarias y la evolución y pronóstico. Pacientes y métodos: Revisamos a todos los pacientes menores de 6 años que cumplieran los criterios de Asbury et al para el diagnóstico de SGB. Resultados: Se incluyó a 8 pacientes, con una media de edad de 3,4 años. El 75% se registró la presencia de un agente infeccioso previo. La sintomatología previa al diagnóstico fue de carácter muy heterogéneo, lo que conllevó un amplio diagnóstico diferencial y multitud de exploraciones complementarias. El tiempo medio al diagnóstico fue de 8,5 días. El 100% presentó afectación motora de miembros inferiores, el 75% de miembros superiores y el 12% de musculatura respiratoria. La afectación sensitiva fue del 62,5% y la de pares craneales del 25%. Requirieron ingreso en cuidados intensivos un 25%. Se objetivó disociación albúmino-citológica en el líquido cefalorraquídeo (83,3%) y positividad de todos los estudios electrofisiológicos con distintos patrones. El pronóstico fue excelente en todos los pacientes. Conclusiones: El SGB en niños menores de 6 años es de difícil diagnóstico por la inespecificidad de las primeras manifestaciones en muchas ocasiones. Esto implica un amplio diagnóstico diferencial y retraso diagnóstico. Es relevante, el buen pronóstico en este grupo de edad de todos los subtipos electrofisiológicos(AU)

Introduction: Guillain-Barré syndrome (GBS) is an acute polyneuropathy that is difficult to diagnose in young children. Objectives: To review the form of presentation of GBS in children under six years-old at the time of onset and the symptoms they had until the diagnosis, the findings in the complementary tests, and the progression and prognosis. Patients and methods: All patients less than 6 years-old who fulfilled the Asbury et al criteria for the diagnosis of GBS were reviewed. Results: Eight patients with a mean age of 3.4years were included. Of those 75% recorded a previous infection. The symptoms prior to the diagnosis were very heterogeneous which entailed a wide differential diagnosis with many complementary examinations. The mean time to diagnosis was 8.5 days. All of them (100%) had motor involvement in the lower limbs, 75% in the upper limbs and 12% in the respiratory muscles. Sensory and cranial nerve involvement was observed in 62.5% and 25%, respectively. Admission to intensive care was required for 25% of the patients. Albumino-cytological dissociation was observed in the CSF in 83.3% and all the electrophysiological tests were positive with different patterns. The prognosis was excellent in all patients. Conclusions: GBS in children under 6 years-old is difficult to diagnosis due to the signs of onset often being unspecific. This entails a wide differential diagnosis, with the subsequent diagnostic delay. There is a good prognosis in all the electrophysiological sub-types in this age group(AU)

[Guillain-Barré syndrome: clinical presentation and prognosis in children under six years-old]. / Síndrome de Guillain-Barré: presentación clínica y evolución en menores de 6 años de edad.

INTRODUCTION: Guillain-Barré syndrome (GBS) is an acute polyneuropathy that is difficult to diagnose in young children. OBJECTIVES: To review the form of presentation of GBS in children under six years-old at the time of onset and the symptoms they had until the diagnosis, the findings in the complementary tests, and the progression and prognosis. PATIENTS AND METHODS: All patients less than 6 years-old who fulfilled the Asbury et al criteria for the diagnosis of GBS were reviewed. RESULTS: Eight patients with a mean age of 3.4 years were included. Of those 75% recorded a previous infection. The symptoms prior to the diagnosis were very heterogeneous which entailed a wide differential diagnosis with many complementary examinations. The mean time to diagnosis was 8.5 days. All of them (100%) had motor involvement in the lower limbs, 75% in the upper limbs and 12% in the respiratory muscles. Sensory and cranial nerve involvement was observed in 62.5% and 25%, respectively. Admission to intensive care was required for 25% of the patients. Albumino-cytological dissociation was observed in the CSF in 83.3% and all the electrophysiological tests were positive with different patterns. The prognosis was excellent in all patients. CONCLUSIONS: GBS in children under 6 years-old is difficult to diagnosis due to the signs of onset often being unspecific. This entails a wide differential diagnosis, with the subsequent diagnostic delay. There is a good prognosis in all the electrophysiological sub-types in this age group.

[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.

[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)

Imagen en RM de encefalomielitis aguda diseminada, cerebelitis y mielitis en la infancia: probables variantes topográficas de un mismo proceso / MR imaging of acute disseminated encephalomyelitis, cerebellitis and myelitis in infancy: likely topographic variants of a single process

Describir los hallazgos en RM de la encefalomielitis aguda diseminada (EAD) en un grupo pediátrico, incidiendo en sus posibles variantes topográficas, cerebelitis y mielitis, en su evolución y en el diagnóstico diferencial con un brote inicial de esclerosis múltiple. Material y método: Se revisan de forma retrospectiva los hallazgos de RM craneal iniciales y evolutivos de 14 pacientes pediátricos diagnosticados de EAD, cerebelitis o mielitis. En nueve pacientes se incluyó estudio medular. Resultados: Se han considerado tres variantes topográficas: EAD (siete pacientes), cerebelitis aislada (cuatro pacientes) y mielitis aislada (tres pacientes). En la EAD se observó afectación de la sustancia blanca supratentorial en todos los casos, del tronco del encéfalo en cinco (71 por ciento) y del cerebelo en dos (28,5 por ciento). Se detectaron lesiones de ganglios basales en cinco pacientes (71 por ciento) y del córtex en uno (14 por ciento). Se asoció alteración medular en cinco de los seis casos en los que se incluyó su estudio (83 por ciento). Las lesiones en la EAD tendieron a ser nodulares y mal delimitadas, mientras que en las cerebelitis y mielitis el patrón predominante fue la afectación difusa. La tendencia evolutiva fue hacia la disminución-resolución de las lesiones. El seguimiento con RM a medio plazo en seis pacientes (cuatro EAD y dos cerebelitis) no detectó nuevas lesiones. El seguimiento clínico de los pacientes no mostró recurrencias neurológicas en ninguno de ellos. Conclusiones: La EAD, la cerebelitis y la mielitis pueden ser variantes topográficas del mismo proceso con una patogenia común. La frecuente participación medular en la EAD aconseja incluir su estudio, aún en ausencia de sintomatología referida. Esta alteración medular, que suele ser difusa, la afectación de la sustancia gris profunda, y el curso monofásico de la enfermedad, avalado por un seguimiento secuencial con RM, nos ayudan en el diagnóstico diferencial con la esclerosis múltiple. Aún así, el diagnóstico diferencial entre una forma recurrente de EAD y una EM es prácticamente imposible de realizar (AU)

Tumores tectales en pediatría. Revisión de ocho pacientes / Tectal tumours in paediatrics. a review of eight patients

Introducción. Los tumores de la región tectal constituyen, dentro del apartado de los gliomas del tronco del encéfalo, un subgrupo de mejor pronóstico que requiere una estrategia terapéutica distinta. Objetivos. Revisión retrospectiva de los tumores tectales en niños perfilando la actitud terapéutica más idónea y el pronóstico esperable. Pacientes y métodos. Se revisan ocho pacientes pediátricos que en los últimos 11 años han desarrollado un tumor tectal diagnosticado mediante técnicas de neuroimagen. Se evalúan sus características clínicas y, especialmente, su evolución clinicorradiológica. Resultados. La media de edad al diagnóstico fue de 10 años. Todos los casos mostraron una presentación clínica de hipertensión intracraneal secundaria a la obstrucción del acueducto de Silvio. La RM resultó clave para objetivar la lesión tectal en todos los casos excepto en uno visualizada previamente en la TC. La actitud terapéutica inicial y exclusiva fue la derivación ventricular. El seguimiento clinicorradiológico medio en nuestra serie ha sido de 4 años, con buena evolución en todos los casos sin precisar otras medidas terapéuticas. Los controles de RM dieron evidencia de estabilidad tumoral en todos los casos excepto en uno con mínima expansión lesional sin repercusión clínica. Conclusiones. Los tumores tectales en los niños muestran, en la mayoría de los casos, un curso benigno. La derivación ventricular es habitualmente el único tratamiento requerido. No obstante, se debe realizar un seguimiento clínico y radiológico estrecho para descartar progresión tumoral que aconseje otras medidas terapéuticas. La RM es electiva en la valoración inicial y en el seguimiento de estos tumores (AU)

[Tectal tumours in paediatrics. A review of eight patients]. / Tumores tectales en pediatría. Revisión de ocho pacientes.

INTRODUCTION: Tumours of the tectal region form, within the group of gliomas of the brain stem, a sub group with better prognosis which require a different therapeutic strategy. OBJECTIVES: A retrospective review of tectal tumours in children to find the most suitable therapeutic approach and the prognosis to be expected. PATIENTS AND METHODS: We reviewed 8 paediatric patients who, during the past 11 years, had developed tectal tumours diagnosed by means of neuro imaging techniques. We evaluated their clinical features and especially their clinico radiological progress. RESULTS: The average age at the time of diagnosis was 10 years. All cases presented with the clinical features of raised intracranial pressure secondary to obstruction of the aqueduct of Sylvius. MR was the key to visualization of the tectal lesion in all cases but one, which had already been shown on CT. The initial and only treatment given was a ventricular shunt. The average clinico radiological follow up in our series was 4 years, with good progress in all cases so that no other therapeutic measures were required. MR follow up showed that the tumours were stable in all cases but one, which had increased slightly in size but without parallel clinical signs. CONCLUSION: In most cases tectal tumours in children follow a benign course. A ventricular shunt is usually the only treatment necessary. However, close clinical and radiological follow up should be carried out to rule out growth of the tumour which make other treatment also necessary. MR is the method of choice for initial evaluation and follow up of these tumours.

[Disseminated acute encephalomyelitis in children. A retrospective study of seven patients]. / Encefalomielitis aguda diseminada en la infancia. Estudio retrospectivo de siete pacientes.

INTRODUCTION AND OBJECTIVE: Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the central nervous system. This study is based on clinical symptoms and diagnostic tests employed. PATIENTS AND METHODS: We describe a seven children series indicating the initial neurologic abnormalities, diagnostic tests, treatments used and clinical-neuroradiological evolution. RESULTS: The mean presentation age was 4.1 years. Initial neurologic symptoms were mainly spastic hemi/paraparesis, cerebellous and pyramidal syndrome, consciousness changes, meningeal signs, seizures and cranial nerve palsies. The cerebrospinal fluid was abnormal in four patients with positive serologic tests in two of them (Coxsackie B). Electrophysiological studies were affected in 50%. MRI findings consisted of multifocal supratentorial white matter lesions. Clinical evolution revealed a progressive improvement with resolution after two months. Follow-up was made between six months and five years. The treatment was based on aciclovir and corticosteroids. CONCLUSIONS: ADEM runs a monophasic course of progressive neurologic abnormalities. Diagnosis is based on suggestive clinical and neuroimaging findings. Generally speaking, MRI showed resolution of multifocal lesions in conjunction with clinical improvement.