RESUMEN

The goal of this paper is to provide updated diagnostic criteria for the epilepsy syndromes that have a variable age of onset, based on expert consensus of the International League Against Epilepsy Nosology and Definitions Taskforce (2017-2021). We use language consistent with current accepted epilepsy and seizure classifications and incorporate knowledge from advances in genetics, electroencephalography, and imaging. Our aim in delineating the epilepsy syndromes that present at a variable age is to aid diagnosis and to guide investigations for etiology and treatments for these patients.

Asunto(s)

RESUMEN

OBJECTIVE: To investigate whether cortical excitability measures on transcranial magnetic stimulation (TMS) differed between groups of patients with different focal epilepsy syndromes. METHODS: 85 Patients with focal epilepsy syndromes divided into temporal and extra-temporal lobe epilepsy were studied. The cohorts were further divided into drug naïve-new onset, refractory and seizure free groups. Motor threshold (MT) and paired pulse TMS at short (2, 5, 10, 15 ms) and long (100-300 ms) interstimulus intervals (ISIs) were measured. Results were compared to those of 20 controls. RESULTS: Cortical excitability was higher at 2 & 5 ms and 250, 300 ms ISIs (p<0.01) in focal epilepsy syndromes compared to controls however significant inter-hemispheric differences in MT and the same ISIs were only seen in the drug naïve state early at onset and were much more prominent in temporal lobe epilepsy. CONCLUSION: Disturbances in cortical excitability are more confined to the affected hemisphere in temporal lobe epilepsy but only early at onset in the drug naïve state. SIGNIFICANCE: Group TMS studies show that cortical excitability measures are different in temporal lobe epilepsy and can be distinguished from other focal epilepsies early at onset in the drug naïve state. Further studies are needed to determine whether these results can be applied clinically as the utility of TMS in distinguishing between epilepsy syndromes at an individual level remains to be determined.

Asunto(s)

RESUMEN

El objetivo del presente trabajo fue caracterizar un grupo de pacientes con epilepsias focales, según aspectos clínicos y electroencefalográficos. Se realizó un estudio descriptivo y prospectivo de 185 niños con diagnóstico de epilepsia focal (2 o más crisis epilépticas no provocadas), con edades entre un mes y 14 años, que fueron hospitalizados en el Departamento de Neuropediatría del Hospital William Soler entre diciembre de 2001 y diciembre de 2003. La edad media de inicio de la primera crisis epiléptica fue de 5 años. El tipo de crisis epiléptica focal más frecuente fue la simple (49,2 por ciento). El 48,6 por ciento de los niños presentó etiología idiopática y el 33,0 por ciento sintomática. Los factores de la etiología sintomática más frecuentes fueron los prenatales (56,2 por ciento). El 91,4 por ciento de los pacientes presentó electroencefalogramas iniciales interictales anormales. El electroencefalograma focal se observó en el 37,3 por ciento de los niños y el multifocal en el 24,9 por ciento. El síndrome epiléptico más frecuente fue la epilepsia benigna con puntas centrotemporales (5,9 por ciento). Los niños con epilepsia focal tienen variadas manifestaciones clínicas y electroencefalográficas, y en la mayoría de los pacientes no es posible identificar un síndrome epiléptico.

The aim of present paper was to characterize a group of patients presenting with focal epilepsies by clinical and electroencephalographic features. Authors made a descriptive and cross-sectional study in 185 children diagnosed with focal epilepsy (two or more non-provoked epilepsy crises), aged from one month to 14, admitted in Neurology Department of William Soler Children Hospital between December 2001 to December 2003. Mean age of the first epilepsy crisis was at 5 years. The more frequent type of focal epilepsy crisis was the simple one (49, 2 percent). The 48, 6 percent of children presented with a idiopathic origin, and the 33, 0 percent if symptomatic one. The more frequent factors of symptomatic origin were the prenatal ones (56, 2 percent). The 91, 4 percent of patients had abnormal interictal initial electroencephalograms. The focal electroencephalogram was observed in the 37, 3 percent of children, and the multifocal one in the 24, 9 percent. The more frequent epileptic syndrome was the benign epilepsy with central-temporal waves (5, 9 percent). Children presenting with epilepsy have many clinical and electroencephalographic manifestations and in most of patients it is not possible to identify an epilepsy syndrome.

Asunto(s)