Neuritis óptica postencefalitis por virus de Epstein-Barr en niños inmunocompetentes. Caso clínico / Optic neuritis following Epstein-Barr virus encephalitis in immunocompetent children: a case report

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Síndrome doloroso regional complejo tipo i. Análisis de una casuística infantil / Complex regional pain syndrome type i. An analysis of 7 cases in children

Introducción: El síndrome doloroso regional complejo (SDRC) se caracteriza por la presencia de dolor acompañado de síntomas sensoriales, autonómicos y motores. Es precedido habitualmente por una lesión o inmovilización. Su curso clínico es desproporcionado con respecto a la lesión inicial tanto en su intensidad como en su duración. Su distribución es regional, predominando en las extremidades. Se clasifica en tipo I y tipo II según ausencia o presencia de lesión nerviosa. Casos clínicos: Se presentan 7 casos clínicos, 6 niñas y un varón con SDRC tipo I, con edades comprendidas entre 7-15 años. Tres tenían antecedente de traumatismo previo. En 5 casos los síntomas se localizaron en miembros inferiores. La demora diagnóstica fue entre 4-90 días. Tres pacientes presentaron elementos de ansiedad y depresión. En todos se realizaron pruebas complementarias de imagen e inmunológicas para descartar diagnósticos diferenciales. Se realizó tratamiento interdisciplinario no farmacológico (fisioterapia y psicoterapia) y farmacológico con analgésicos mayores, gabapentina o pregabalina. Todos presentaron buena evolución, sin recidivas en el seguimiento que fue entre 4 meses y 2,5 años. Conclusiones: El poco reconocimiento de este síndrome en niños, la ansiedad familiar que genera y los costos en paraclínica innecesaria, resaltan la importancia de su difusión entre pediatras y neuropediatras para favorecer su reconocimiento, evitar estudios innecesarios y múltiples consultas a especialistas que retrasan el diagnóstico y el inicio de un tratamiento efectivo

Introduction: Complex regional pain syndrome (CRPS) is characterised by the presence of pain accompanied by sensory, autonomic and motor symptoms, usually preceded by a lesion or immobilisation. The clinical course is disproportionate to the initial injury in intensity and in duration. Its distribution is regional, predominantly in limbs. It is classified as type I and type II according to the absence or presence of nerve injury. Cases: We present the cases of seven children, 6 girls and 1 boy, aged 7 to 15 years. Three had a history of previous trauma. In 5 cases, the symptoms were located in the lower limbs. Time to diagnosis was between 4 and 90 days. Three patients had clinical features of anxiety and depression. Imaging and immunological studies were performed to rule out differential diagnoses in all the children. Interdisciplinary treatment was performed with physiotherapy, psychotherapy, and gabapentin or pregabalin. All patients had a good clinical outcome, with no relapses in the follow-up period (between 4 and 30 months). Conclusions: CRPS is frequently unrecognised in children, leading to family anxiety and unnecessary para-clinical costs. Paediatricians and paediatric neurologists should be aware of this syndrome in order to avoid delay in diagnosis, unnecessary studies, and multiple visits to specialists, with a view to providing effective treatment

Complex regional pain syndrome type i. An analysis of 7 cases in children.

INTRODUCTION: Complex regional pain syndrome (CRPS) is characterised by the presence of pain accompanied by sensory, autonomic and motor symptoms, usually preceded by a lesion or immobilisation. The clinical course is disproportionate to the initial injury in intensity and in duration. Its distribution is regional, predominantly in limbs. It is classified as type I and type II according to the absence or presence of nerve injury. CASES: We present the cases of seven children, 6 girls and 1 boy, aged 7 to 15 years. Three had a history of previous trauma. In 5 cases, the symptoms were located in the lower limbs. Time to diagnosis was between 4 and 90 days. Three patients had clinical features of anxiety and depression. Imaging and immunological studies were performed to rule out differential diagnoses in all the children. Interdisciplinary treatment was performed with physiotherapy, psychotherapy, and gabapentin or pregabalin. All patients had a good clinical outcome, with no relapses in the follow-up period (between 4 and 30 months). CONCLUSIONS: CRPS is frequently unrecognised in children, leading to family anxiety and unnecessary para-clinical costs. Paediatricians and paediatric neurologists should be aware of this syndrome in order to avoid delay in diagnosis, unnecessary studies, and multiple visits to specialists, with a view to providing effective treatment.

Moyamoya en la población infantil /. Réplica / Moyamoya en la población infantil. Réplica

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[Moyamoya disease among children. An analysis of a series in the Western world and literature review]. / Moyamoya en la poblacion infantil. Analisis de una serie occidental y revision de la bibliografia.

PATIENTS AND METHODS: A total of 12 patients with moyamoya disease or syndrome with a mean age of 6 years were analyzed in a retrospective fashion. RESULTS: Infarction was the most frequent presentation feature (8 out of 12 patients), 2 had epilepsy and 2 were incidental findings. Seven cases were classified as idiopathic while the other five were related to systemic illnesses. Diagnosis was initially made by magnetic resonance angiography in 9 cases, and conventional angiography in 3 cases. Mean follow-up is 5 years; six patients experienced clinical worsening of symptoms, while 6 cases remained clinically stable. However, all of them showed angiographic progression. Four patients underwent revascularization surgery. Two children died due to complications associated with moyamoya disease, and six have moderate handicaps. CONCLUSION: Due to the fact moyamoya disease is not an indolent disorder and readily progress to cause complications, surgical revascularization should always be considered in the management of these patients.

Moyamoya en la población infantil. Análisis de una serie occidental y revisión de la bibliografía / Moyamoya disease among children an analysis of a series in the western world and literature review

Pacientes y métodos. Estudio descriptivo, longitudinal, de 12 niños no asiáticos con síndrome o enfermedad de moyamoya, con el objetivo de analizar su presentación y evolución clinicorradiológica. Resultados. La edad promedio de inicio fue de 6 años; ocho se iniciaron con infarto cerebral, dos con epilepsia y dos fueron hallazgo casual. Siete se consideraron idiopáticos(enfermedad de moyamoya) y cinco asociados a enfermedades sistémicas (síndrome de moyamoya). El diagnóstico inicial se confirmó por angiorresonancia en nueve casos y en tres por arteriografía convencional. El seguimiento promedio fue de 5 años; seis evidenciaron progresividad clínica y seis estabilidad clínica. Todos tuvieron progresividad angiográfica. En cuatroniños se realizaron siete cirugías de revascularización. Dos niños fallecieron, uno por hemorragia cerebral y otro por un infarto cerebral expansivo, mientras que seis pacientes presentaron secuelas leves a moderadas. Conclusión. Es importante identificara los niños afectados por esta patología, ya que se pueden beneficiar de cirugía de revascularización, una de las pocas opciones terapéuticas para evitar la progresión y complicaciones de esta grave enfermedad

A total of 12 patients with moyamoya disease or syndrome with a mean age of 6 years wereanalyzed in a retrospective fashion. Results. Infarction was the most frequent presentation feature (8 out of 12 patients), 2 had epilepsy and 2 were incidental findings. Seven cases were classified as idiopathic while the other five were related to systemicillnesses. Diagnosis was initially made by magnetic resonance angiography in 9 cases, and conventional angiography in 3 cases. Mean follow-up is 5 years; six patients experienced clinical worsening of symptoms, while 6 cases remained clinicallystable. However, all of them showed angiographic progression. Four patients underwent revascularization surgery. Two children died due to complications associated with moyamoya disease, and six have moderate handicaps. Conclusion. Due to the fact moyamoya disease is not an indolent disorder and readily progress to cause complications, surgical revascularizationshould always be considered in the management of these patients

[Epilepsy, cerebral calcifications and coeliac disease. The importance of an early diagnosis]. / Epilepsia, calcificaciones cerebrales y enfermedad celíaca. Importancia del diagnóstico precoz.

INTRODUCTION: Coeliac disease (CD) has been linked to several neurological disorders, with a clearly higher incidence of epilepsy than in the general population. Since Gobbi's original description of the epilepsy with cerebral calcifications and coeliac disease syndrome in 1992, various reports have been published concerning different stages of development, although a number of questions remain to be answered with respect to its pathogenesis. The aim of this study is to describe the clinical symptoms and progression of three patients with the characteristic syndrome, who were diagnosed in the early stages of the disease. CASE REPORTS: We describe the cases of three males, of school age, who visited because of partial seizures with visual symptoms that were secondarily generalised. A tomography scan of the head revealed bilateral occipital calcifications in all cases. The electroencephalogram showed temporooccipital paroxysmal discharges with a normal background rhythm. Tests for antiendomysium and antigliadin antibodies, with positive results, and a small intestine biopsy study were requested, which confirmed the suspicion of a grade 3 severe enteropathy. All the patients, following a gluten-free diet and taking anticonvulsants, were free of seizures in the follow-up (1, 2 and 8 years). CONCLUSIONS: It is of vital importance to investigate CD in any patient with epilepsy and occipital cerebral calcifications, even in the absence of digestive symptoms, since early diagnosis and treatment with exclusion of gluten affect how the syndrome courses later on.

Epilepsia, calcificaciones cerebrales y enfermedad celíaca. Importancia del diagnóstico precoz / Epilepsy, cerebral calcifications and Coeliac disease. The importance of an early diagnosis

Introducción. La enfermedad celíaca (EC) se ha asociado a varios trastornos neurológicos, con un claro aumento de la incidencia de la epilepsia con respecto a la población normal. Desde la descripción original de Gobbi en 1992 del síndrome de epilepsia con calcificaciones cerebrales y enfermedad celíaca, se han publicado múltiples comunicaciones con evoluciones diversas, aunque persisten ciertos interrogantes sobre su patogenia. El objetivo de este trabajo es describir la presentación clínica y evolución de tres pacientes con el síndrome característico, en quienes se realizó un diagnóstico temprano. Casos clínicos. Se presentan tres varones, en edad escolar, que consultan por crisis parciales con sintomatología visual, secundariamente generalizadas. La tomografía de cráneo mostró en todos los casos calcificaciones occipitales bilaterales. El electroencefalograma evidenció descargas paroxísticas temporooccipitales con ritmo de fondo normal. Se solicitaron anticuerpos antigliadina y antiendomisio, que fueron positivos, y biopsia de intestino delgado, que confirmó una enteropatía grave de grado III. Todos los pacientes que reciben dieta sin gluten y anticomiciales se encuentran libres de crisis en la evolución (1, 2 y 8 años). Conclusión. Se destaca la importancia de investigar la EC en todo paciente con epilepsia y calcificaciones cerebrales occipitales, aun en ausencia de síntomas digestivos, dado que el diagnóstico y el tratamiento precoces con exclusión del gluten inciden en la evolución posterior del síndrome

Introduction. Coeliac disease (CD) has been linked to several neurological disorders, with a clearly higher incidence of epilepsy than in the general population. Since Gobbi’s original description of the epilepsy with cerebral calcifications and coeliac disease syndrome in 1992, various reports have been published concerning different stages of development, although a number of questions remain to be answered with respect to its pathogenesis. The aim of this study is to describe the clinical symptoms and progression of three patients with the characteristic syndrome, who were diagnosed in the early stages of the disease. Case reports. We describe the cases of three males, of school age, who visited because of partial seizures with visual symptoms that were secondarily generalised. A tomography scan of the head revealed bilateral occipital calcifications in all cases. The electroencephalogram showed temporooccipital paroxysmal discharges with a normal background rhythm. Tests for antiendomysium and antigliadin antibodies, with positive results, and a small intestine biopsy study were requested, which confirmed the suspicion of a grade 3 severe enteropathy. All the patients, following a gluten-free diet and taking anticonvulsants, were free of seizures in the follow-up (1, 2 and 8 years). Conclusions. It is of vital importance to investigate CD in any patient with epilepsy and occipital cerebral calcifications, even in the absence of digestive symptoms, since early diagnosis and treatment with exclusion of gluten affect how the syndrome courses later on

Análisis etiológico de las cefaleas desde un servicio de emergencia pediátrica / Analysis of the aetiologies of headaches in a paediatric emergency service

Introducción. La cefalea en los niños motiva frecuentes consultas en los diferentes servicios asistenciales; el perfil epidemiológico es muy diferente según la muestra proceda de consultorios ambulatorios o neuropediátricos, o de servicios de urgencia. Objetivo. Analizar las etiologías de las cefaleas desde un servicio de urgencias pediátrico. Pacientes y métodos. Se analizaron retrospectivamente las historias clínicas con cefaleas como principal motivo de consulta durante un período de seis meses en el Departamento de Emergencia Pediátrica del Centro Hospitalario Pereira Rossell. Se clasificaron las etiologías en función de los criterios de la Sociedad Internacional de Cefaleas (SIC), y se completó la información por vía telefónica cuando los registros médicos fueron insuficientes. Resultados. Se incluyeron 185 niños, que representaban el 0,58 por ciento de las consultas. Las etiologías más frecuentes fueron las infecciones no craneales (43,1 por ciento), las migrañas (14,6 por ciento), las cefaleas tensionales (9,2 por ciento), las traumáticas (8,1 por ciento) y la sinusitis (5,4 por ciento). Las causas graves representaron el 4,9 por ciento, con un predominio de tumores e infecciones del sistema nervioso central. El bajo porcentaje de meningitis virales en nuestra serie (1 por ciento) nos hace sospechar un subdiagnóstico de esta afección, dado el bajo número de casos en los que se estudió el líquido cefalorraquídeo. Conclusiones. Se confirma un perfil etiológico amplio y variado de las cefaleas en las emergencias, con un claro predominio de afecciones extracraneales y un bajo porcentaje de causas graves intracraneales. Las cefaleas no clasificables supusieron el 10,8 por ciento de la muestra. Se realizó estudio de imagen del cráneo en un 9,7 por ciento de los casos, porcentaje sensiblemente menor que en otras series, lo que evidencia un uso racional de este recurso (AU)

Introduction. Headaches are a frequent cause of children’s visits to different health care services but the epidemiological profile varies widely according to whether the sample is taken from among visits to hospital clinics, neuropaediatric units or emergency services. Aims. The aim of this study was to analyse the aetiologies of the cases of headache attended in a paediatric emergency service. Patients and methods. We conducted a retrospective analysis of patient records with headache as the main reason for visiting over a six month period at the Paediatric Emergency Department at the Centro Hospitalario Pereira Rossell. Aetiologies were classified according to International Headache Society (IHS) criteria and the information was completed by telephone if the medical records contained insufficient data. Results. The records of 185 children were studied, which represented 0.58% of the total number of visits. The most common aetiologies were non-cranial infections (43.1%), migraines (14.6%), tension-type headaches (9.2%), traumatic injury (8.1%) and sinusitis (5.4%). Serious causes constituted 4.9% of the total, with a predominance of tumours and infections of the central nervous system. The low percentage of viral meningitis in our series (1%) leads us to suspect an underdiagnosis of this disorder, given the scant number of cases in which the cerebrospinal fluid was examined. Conclusions. Our study confirmed the existence of a wide and varied range of aetiologies causing headaches, with a clear predominance of extracranial disorders and a low percentage of serious intracranial causes. Non-classifiable headaches made up 10.8% of the sample. Brain imaging studies were performed in 9.7% of cases, which is a considerably lower percentage than that of other series and demonstrates a rational use of this resource (AU)

[Analysis of the aetiologies of headaches in a paediatric emergency service]. / Análisis etiológico de las cefaleas desde un servicio de emergencia pediátrica.

INTRODUCTION: Headaches are a frequent cause of children's visits to different health care services but the epidemiological profile varies widely according to whether the sample is taken from among visits to hospital clinics, neuropaediatric units or emergency services. AIMS: The aim of this study was to analyse the aetiologies of the cases of headache attended in a paediatric emergency service. PATIENTS AND METHODS: We conducted a retrospective analysis of patient records with headache as the main reason for visiting over a six month period at the Paediatric Emergency Department at the Centro Hospitalario Pereira Rossell. Aetiologies were classified according to International Headache Society (IHS) criteria and the information was completed by telephone if the medical records contained insufficient data. RESULTS: The records of 185 children were studied, which represented 0.58% of the total number of visits. The most common aetiologies were non-cranial infections (43.1%), migraines (14.6%), tension-type headaches (9.2%), traumatic injury (8.1%) and sinusitis (5.4%). Serious causes constituted 4.9% of the total, with a predominance of tumours and infections of the central nervous system. The low percentage of viral meningitis in our series (1%) leads us to suspect an underdiagnosis of this disorder, given the scant number of cases in which the cerebrospinal fluid was examined. CONCLUSIONS: Our study confirmed the existence of a wide and varied range of aetiologies causing headaches, with a clear predominance of extracranial disorders and a low percentage of serious intracranial causes. Non-classifiable headaches made up 10.8% of the sample. Brain imaging studies were performed in 9.7% of cases, which is a considerably lower percentage than that of other series and demonstrates a rational use of this resource.