ABSTRACT
INTRODUCTION: The morning glory disc anomaly is a not very frequent congenital alteration of the optic papilla associated with a retinal vascular dysgenesia. Several cases of association have been described between this disc anomaly and the presence of Moyamoya vessels. We report the case of a boy with this association. CASE REPORT: A 5 year-old boy was evaluated for several episodes of disconnection, some of them preceded by intense headaches and associated to incoherent language, and in other occasions with hypotonia and unmotivated laugh of variable duration. The physical and neurological exploration was normal, except for the optic disc papilla of the right eye that was very big and dug, compatible with Morning glory disc anomaly. No alterations were found at cranial CT and EEG. In the magnetic resonance imaging were visualized multiple small round low intensity areas in the basal ganglia, because of the high number of collateral vessels. The angioRM showed stenosis of the supraclinoid portions of both internal carotids as well as the proximal segments of the anterior cerebral artery and the left middle cerebral artery with abnormal network of collateral vessels at the base of the brain like a puff of smoke . This is a typical imaging for Moyamoya disease. CONCLUSIONS: In all patient with Morning glory disc anomaly, mainly if they presents neurological clinical features, the realization of angioRM studies will be evaluated, to discard the existence of Moyamoya disease.
Subject(s)
Moyamoya Disease/pathology , Optic Disk/abnormalities , Optic Nerve Diseases/congenital , Carotid Artery, Internal/pathology , Cerebral Angiography , Cerebral Arteries/pathology , Child, Preschool , Constriction, Pathologic , Electroencephalography , Humans , Male , Moyamoya Disease/diagnosis , Optic Nerve Diseases/diagnosis , Optic Nerve Diseases/physiopathologyABSTRACT
INTRODUCTION: To study the chance of achieving a long term remission of epilepsy in children. PATIENTS AND METHODS: 90 children aged under 14 years newly diagnosed of epilepsy were started on anticonvulsant treatment and prospectively followed. Initial remission was defined as a seizure free period with or without subsequent recurrences, terminal remission as a seizure free period without subsequent recurrences and terminal remission off treatment as terminal remission plus medication discontinued. RESULTS: The Kaplan Meier estimate of the probability of achieving a 3 year initial remission was 80% and 90%, of the probability of achieving a 5 year terminal remission was 59% and 68% and of the probability of achieving a 5 year terminal remission off treatment was 53% and 61% at 5 and 7 years, respectively. Univariate and multivariate analyses using the Cox proportionate hazards model revealed there was a greater probability of achieving a 3 year terminal remission off treatment for patients with idiopathic/cryptogenic etiology and for those who did not have recurrences during the first six months of treatment. Syndromic diagnosis at six months after initiating antiepileptic treatment was also useful for predicting the probability of remission. CONCLUSIONS: Over half of the epileptic children achieve a prolonged remission off treatment. The main prognostic factors are etiology, recurrence of seizures during the first six months and syndromic diagnosis.
Subject(s)
Epilepsy/physiopathology , Adolescent , Anticonvulsants/therapeutic use , Child , Child, Preschool , Epilepsy/diagnosis , Epilepsy/drug therapy , Humans , Prospective Studies , Remission InductionABSTRACT
Objetivo. Estudiar la probabilidad de lograr el control inicial de las crisis epilépticas tras el comienzo del tratamiento en la epilepsia infantil. Pacientes y métodos. Se siguió prospectivamente a 106 niños menores de 14 años recientemente diagnosticados de epilepsia que iniciaron un tratamiento antiepiléptico. Resultados. El cumplimiento terapéutico, verificado mediante la determinación de niveles de medicación, fue satisfactorio. La probabilidad de alcanzar una remisión inicial de dos años estimada mediante curvas de Kaplan-Meier fue del 55 por ciento, 71 por ciento, 77 por ciento, 86 por ciento y 96 por ciento a los 2, 2 y medio, 3, 4 y 5 años. El análisis multivariante mediante el modelo de riesgos proporcionales de Cox mostró que una etiología sintomática, un EEG anormal y la recurrencia de las crisis en los primeros seis meses de tratamiento disminuyeron la probabilidad de alcanzar una remisión inicial de dos años, mientras que la presencia de crisis tonicoclónicas generalizadas o parciales secundariamente generalizadas exclusivamente y una edad entre 3 y 10 años la aumentaron. La selección por pasos mostró que el mejor modelo para la predicción de la remisión fue el constituido por edad entre 3 y 10 años y recurrencia en los primeros seis meses de tratamiento. No se pudieron demostrar diferencias significativas entre los diferentes síndromes epilépticos. Conclusiones. La mayoría de los niños con epilepsia entran en remisión después del inicio del tratamiento antiepiléptico. Aquellos sin recurrencias en los primeros seis meses o con una edad entre 3 y 10 años tienen un pronóstico particularmente favorable (AU)
Subject(s)
Child, Preschool , Child , Adolescent , Male , Infant , Female , Humans , Proportional Hazards Models , Treatment Outcome , Phenobarbital , Prospective Studies , Remission Induction , Anticonvulsants , Carbamazepine , Electroencephalography , Epilepsy , Valproic AcidABSTRACT
PURPOSE: To study the risk of recurrence after a first unprovoked seizure in childhood. METHODS: All consecutive patients aged less than 14 years with one or more unprovoked seizures who were attended between January 1, 1987, and June 1, 1996, were included in a prospective study. Clinical features of patients attended after a first seizure and those attended after two or more seizures were compared. Recurrence risk in both groups was estimated by Kaplan-Meier curves. Univariate and multivariate analyses of the potential predictors of recurrence risk were performed for the group of patients attended after a first seizure using the Cox proportional hazards model. RESULTS: Included in the study were 217 children. Kaplan-Meier estimate of recurrence risk was 64% at 5 years, when only patients being attended after a first epileptic seizure were included, compared with 74% when all patients were included. Significant differences in several clinical features were found between patients attended after a first seizure and those attended after two or more seizures. Univariate and multivariate analyses showed that in the overall cohort of patients attended after a first seizure, a symptomatic etiology increased the risk of recurrence, whereas a patient age of 3 to 10 years decreased this risk. In particular, the recurrence risk was 96% at 2 years for symptomatic seizures, compared with 46% for idiopathic/cryptogenic seizures. In the group of patients with idiopathic/cryptogenic seizures, an abnormal electroencephalogram and the occurrence of seizures during sleep increased the recurrence risk, whereas a patient aged 3 to 10 years reduced it. In the group of patients with symptomatic etiology, univariate analysis revealed that there was a lower recurrence risk for patients aged 3 to 10 years. This last finding was not maintained, however, in multivariate analysis. CONCLUSIONS: The recurrence risk depends on the inclusion criteria for enrolling patients. Several factors enable us to predict the recurrence risk after a first unprovoked seizure; the most important of these factors is the etiology of the seizures.
Subject(s)
Seizures/diagnosis , Adolescent , Age Factors , Age of Onset , Analysis of Variance , Child , Child, Preschool , Cohort Studies , Electroencephalography/statistics & numerical data , Epilepsy/diagnosis , Epilepsy/epidemiology , Epilepsy/etiology , Female , Humans , Male , Monitoring, Physiologic , Multivariate Analysis , Prognosis , Proportional Hazards Models , Prospective Studies , Recurrence , Risk Factors , Seizures/epidemiology , Seizures/etiology , Sleep/physiology , Spain/epidemiology , Survival AnalysisABSTRACT
The purpose of this study was to verify the statistical validity of the score proposed by Thomé et al. for the differential diagnosis between bacterial and viral meningitis and to study the utility of two new parameters (CRP and the patient's age). A retrospective review of 136 consecutive cases of meningitis was made. The cases were classified into three groups according to the culture results and the use or not of intravenous antibiotics. There were 20 cases of bacterial meningitis, 60 non-bacterial and 56 cases of meningitis of uncertain etiology. Considering only the patients in the first two groups, the capacity for differential diagnosis between bacterial and non-bacterial meningitis of the 8 parameters in the original score, the CRP and the patient's age was analyzed with a numeric value of 0, 1, or 2 assigned to each parameter. Finally, the results of applying the score that includes the two new parameters with the original score are compared. We found that all parameters showed statistical significance for the differential diagnosis between bacterial and viral meningitis. The resulting score can be used in order to decide the need for intravenous antibiotic therapy, with only a few cases being uncertain. The score with the two new parameters correctly classified 78 of the 80 cases, while leaving only two case uncertain as compared to the four that remained unclassified with the original score. We conclude that the score is a useful instrument in the differential diagnosis between bacterial and viral meningitis. Furthermore, the introduction of CRP and the patient's age improves the diagnostic value of the test.
