ABSTRACT
BACKGROUND: We investigated the clinical characteristics of a pediatric population with hemato-oncological disease and intracranial hypertension, analyze the therapeutic response and outcome, and compare its characteristics with respect to a control group with idiopathic intracranial hypertension. METHODS: We retrospectively analyzed patients with hemato-oncological disease and secondary intracranial hypertension in our center during the past five years. We compared these individuals with a historical cohort with idiopathic intracranial hypertension from our institution (control group). RESULTS: We identified eight patients, all with leukemia, and 21 controls. Mean age at diagnosis was 10.6 years, and 62% of individuals were female. Most of them were under treatment with drugs (62% corticosteroids, 75% active chemotherapy). Mean opening pressure of cerebrospinal fluid was 35 cm H2O. All had headache, but only 28% complained of visual symptoms. Only 12.5% exhibited papilledema at the time of diagnosis (versus 71% in controls). All of them were treated with acetazolamide, with average therapy duration of nine months, and all had a favorable outcome (versus 57% of controls who needed second-line treatment). None of them showed long-term visual complications (versus 20% of controls). CONCLUSIONS: Patients with hemato-oncological disease and secondary intracranial hypertension may not develop typical symptomatology. Thus, diagnosis and recognition of this entity among this cohort may be difficult. Associated factors are diverse and do not show an obvious causal relationship. A high index of suspicion must be maintained for diagnosis, because a favorable outcome is expected with prompt treatment. Acetazolamide is effective as a first-line therapy and caused few side effects.
Subject(s)
Intracranial Hypertension/complications , Intracranial Hypertension/diagnosis , Leukemia/complications , Acetazolamide/therapeutic use , Adolescent , Anticonvulsants/therapeutic use , Child , Child, Preschool , Cohort Studies , Female , Headache/etiology , Humans , Intracranial Hypertension/therapy , Leukemia/drug therapy , Male , Retrospective Studies , Vision Disorders/etiologySubject(s)
Anticonvulsants/therapeutic use , Bromides/therapeutic use , Epilepsies, Myoclonic/drug therapy , Potassium Compounds/therapeutic use , Adolescent , Anticonvulsants/adverse effects , Anticonvulsants/blood , Bromides/adverse effects , Bromides/blood , Child , Child, Preschool , Drug Evaluation , Drug Therapy, Combination , Epilepsies, Partial/drug therapy , Epilepsy, Tonic-Clonic/drug therapy , Humans , Male , Potassium Compounds/adverse effects , Potassium Compounds/blood , Retrospective Studies , Treatment OutcomeABSTRACT
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Subject(s)
Child , Female , Humans , Male , Bromides/therapeutic use , Epilepsy/drug therapy , Epilepsy, Tonic-Clonic/drug therapy , Epilepsy, Reflex/drug therapy , Retrospective Studies , Seizures/drug therapy , Drug-Related Side Effects and Adverse Reactions/drug therapy , Drug-Related Side Effects and Adverse Reactions/prevention & controlABSTRACT
Introducción. La epilepsia mioclónica benigna del lactante (EMBL) es un síndrome electroclínico de características homogéneas y bien definidas, considerado clásicamente de buen pronóstico. Sin embargo, en los últimos años se han publicado estudios con resultados variables en cuanto a evolución neuropsicológica. Objetivo. Analizar la evolución natural y el pronóstico neurocognitivo y conductual de los pacientes con EMBL. Pacientes y métodos. Estudio retrospectivo de 10 pacientes con EMBL, con un período de seguimiento de más de cinco años, durante los cuales se realizó una evaluación neurocognitiva y conductual. Resultados. En el 60% de los pacientes las crisis se controlaron con ácido valproico en monoterapia, y el 80% no presentó nuevas crisis durante su seguimiento. El cociente intelectual de la cohorte se situó entre 74 y 93; tres pacientes tuvieron un cociente intelectual en rango de inteligencia límite, y seis, en rango de inteligencia media-baja. Nueve pacientes cumplieron criterios de trastorno por déficit de atención/hiperactividad y dos asociaban otro trastorno del aprendizaje, uno de ellos trastorno de aprendizaje no verbal, y el otro, trastorno específico de la lectoescritura. Todos los pacientes presentaron datos de pobre coordinación motriz y visuoespacial, y tres fueron diagnosticados de trastorno de conducta. Conclusiones. El término benigno en la EMBL debe utilizarse con precaución en cuanto a su pronóstico neurocognitivo y conductual. El inicio precoz y un peor control de las crisis podrían suponer factores de riesgo de evolución neuropsicológica desfavorable (AU)
Introduction. Benign myoclonic epilepsy in infancy (BMEI) is a well-defined electro-clinical syndrome, classically associated with a good prognosis. However, in the last years several studies have been published with variable results of neuropsychological outcome in BMEI. Aim. To analyze the natural history and the cognitive and behavioral outcome in BMEI patients. Patients and methods. We report a long-term follow-up of 10 patients with BMEI. During the follow-up, all the patients underwent neurocognitive and behavioral evaluations. Results. Sixty percent of patients became seizure free on valproic acid. The intelligence quotient of the whole cohort was between 74 and 93, with three patients in the range of borderline intelligence and six in the range of medium-to-low intelligence. Nine of the 10 patients met criteria for attention deficit hyperactivity disorder, and two patients associated another learning disorder. All patients showed poor motor and visuospatial coordination signs and three patients had a behavior disorder. Conclusions. The term benign in BMEI has to be used with caution in refer to its behavioral and cognitive outcome. Early onset of seizures and a worse epilepsy control may be risk factors of a poor neuropsychological outcome (AU)
Subject(s)
Humans , Male , Female , Infant , Epilepsies, Myoclonic/epidemiology , Child Behavior Disorders/epidemiology , Learning Disabilities/epidemiology , Retrospective Studies , Risk FactorsABSTRACT
INTRODUCTION: Benign myoclonic epilepsy in infancy (BMEI) is a well-defined electro-clinical syndrome, classically associated with a good prognosis. However, in the last years several studies have been published with variable results of neuropsychological outcome in BMEI. AIM. To analyze the natural history and the cognitive and behavioral outcome in BMEI patients. PATIENTS AND METHODS: We report a long-term follow-up of 10 patients with BMEI. During the follow-up, all the patients underwent neurocognitive and behavioral evaluations. RESULTS: Sixty percent of patients became seizure free on valproic acid. The intelligence quotient of the whole cohort was between 74 and 93, with three patients in the range of borderline intelligence and six in the range of medium-to-low intelligence. Nine of the 10 patients met criteria for attention deficit hyperactivity disorder, and two patients associated another learning disorder. All patients showed poor motor and visuospatial coordination signs and three patients had a behavior disorder. CONCLUSIONS: The term 'benign' in BMEI has to be used with caution in refer to its behavioral and cognitive outcome. Early onset of seizures and a worse epilepsy control may be risk factors of a poor neuropsychological outcome.
TITLE: Epilepsia mioclonica benigna del lactante: evolucion natural y pronostico neurocognitivo y conductual.Introduccion. La epilepsia mioclonica benigna del lactante (EMBL) es un sindrome electroclinico de caracteristicas homogeneas y bien definidas, considerado clasicamente de buen pronostico. Sin embargo, en los ultimos años se han publicado estudios con resultados variables en cuanto a evolucion neuropsicologica. Objetivo. Analizar la evolucion natural y el pronostico neurocognitivo y conductual de los pacientes con EMBL. Pacientes y metodos. Estudio retrospectivo de 10 pacientes con EMBL, con un periodo de seguimiento de mas de cinco años, durante los cuales se realizo una evaluacion neurocognitiva y conductual. Resultados. En el 60% de los pacientes las crisis se controlaron con acido valproico en monoterapia, y el 80% no presento nuevas crisis durante su seguimiento. El cociente intelectual de la cohorte se situo entre 74 y 93; tres pacientes tuvieron un cociente intelectual en rango de inteligencia limite, y seis, en rango de inteligencia media-baja. Nueve pacientes cumplieron criterios de trastorno por deficit de atencion/hiperactividad y dos asociaban otro trastorno del aprendizaje, uno de ellos trastorno de aprendizaje no verbal, y el otro, trastorno especifico de la lectoescritura. Todos los pacientes presentaron datos de pobre coordinacion motriz y visuoespacial, y tres fueron diagnosticados de trastorno de conducta. Conclusiones. El termino 'benigno' en la EMBL debe utilizarse con precaucion en cuanto a su pronostico neurocognitivo y conductual. El inicio precoz y un peor control de las crisis podrian suponer factores de riesgo de evolucion neuropsicologica desfavorable.
