Focal Cortical Dysplasia with hippocampal sclerosis

ABSTRACT Focal Cortical Dysplasia (FCD) is a group of focal developmental malformations of the cerebral cortex cytoarchitecture. FCD usually manifests as medically intractable epilepsy, especially in young children. Live patients are diagnosed by radiological examination such as magnetic resonance imaging (MRI), fluorodeoxyglucose positron emission tomography (FDG PET), magnetoencephalography (MEG), diffusion-tensor imaging (DTI), and intracranial electroencephalogram (EEG). While some cases can be missed by radiological examination, they are usually diagnosed on the histopathological examination of the surgically removed specimens of medically intractable epilepsy patients. We report a case of a young girl with cerebral palsy, mental retardation, and seizure disorder who died in her sleep. The deceased was diagnosed with FCD type III with hippocampal sclerosis on histopathological examination at autopsy. H & E stain and NeuN immunohistochemistry neuronal cell marker were used to demonstrate the findings of FCD.

Direct cost of hospitalization of pediatric focal epilepsy patients in a tertiary medical center

OBJECTIVES@#To determine the cost of hospitalization, percentage distribution of expenses, and average expenditures covered by PhilHealth, hospital share and patient’s share in financing the hospitalization of pediatric focal epilepsy patients in a tertiary government hospital.@*METHODOLOGY@#This is descriptive retrospective research of patients diagnosed and managed as focal epilepsy from July to December 2018. Mean, standard deviation and percentage distribution of expenses were determined.@*RESULTS@#The mean cost of hospitalization is 21,545.96 Php and the laboratory cost contributes the most to the total cost with a mean of 6,046.08 Php. The mean cost shouldered by PhilHealth is 3,734.26 Php which is 17.33% of the total cost of hospitalization.@*CONCLUSION@#The laboratory cost makes up most of the hospital cost. Philhealth covers a small percentage of the hospitalization cost of pediatric focal epilepsy patients with most of the total cost shortly followed by the patient’s share.

Facomatosis pigmentovascular cesioflammea asociada a epilepsia focal mioclónica: Primer caso reportado en el Perú / Phakomatosis pigmentovascularis cesioflammea associated with focal myoclonic seizures: First case report in Peru

Resumen La facomatosis pigmentovascular es un síndrome congénito muy poco frecuente, caracterizado por la presentación simultánea de una malformación vascular capilar y una lesión cutánea pigmentaria, con o sin compromiso extracutáneo. Se presenta el caso de una adolescente con epilepsia que cursa con crisis mioclónicas focales no controladas por un tratamiento farmacológico irregular, y que muestra además lesiones cutáneas compatibles con nevus flammeus y melanosis dérmica, ocular y palatina, presentes desde el nacimiento. Se trata del primer reporte en el país, de un síndrome neurocutáneo poco frecuente y de su asociación clínica con epilepsia, resaltándose además la importancia de una evaluación integral de esta entidad.

Summary Phacomatosis pigmentovascularis is a rare congenital syndrome, characterized by the simultaneous presentation of a capillary vascular malformation and a cutaneous pigmentary lesion, without or with extracutaneous involvement. The case of an adolescent with epilepsy characterized by focal myoclonic seizures uncontrolled by an irregular pharmacological treatment, with skin lesions compatible with nevus flammeus and dermal, ocular and palatal melanosis since birth, is presented. This is the first report in the country of an infrequent neurocutaneous syndrome and its clinical association with epilepsy, highlighting besides the importance of a comprehensive evaluation of this entity.

Clinical Significance of Asymmetric Minimum Intensity Projection Images of Brain Magnetic Resonance Imaging in Children

BACKGROUND AND PURPOSE: The susceptibility-weighted imaging form of brain MRI using minimum intensity projection (mIP) is useful for assessing traumatic brain injuries because it readily reveals deoxyhemoglobin or paramagnetic compounds. We investigated the efficacy of using this methodology in nontraumatic patients. METHODS: We retrospectively analyzed the asymmetric mIP findings in nontraumatic patients. Asymmetric mIP images were first verified visually and then using ImageJ software. We enrolled patients with a difference of >5% between hemispheres in ImageJ analysis. All patients underwent detailed history-taking and EEG, and asymmetric mIP findings were compared. RESULTS: The visual analysis identified 54 pediatric patients (37 males and 17 females) with asymmetric mIP findings. Ten patients were excluded because they did not meet the ImageJ verification criteria. The 44 patients with asymmetry comprised 36 with epilepsy, 6 with headache, and 2 with cerebral infarction. Thirty-one of the 36 epileptic patients showed definite partial seizure activities in semiology, while the remaining patients did not demonstrate a history of partial seizure manifestations. The MRI findings were normal in all patients except for five with periventricular leukomalacia unrelated to seizure symptoms. There was agreement between mIP images and semiology in 29 (93.5%) of the 31 epileptic patients with focal signs, while the other 2 demonstrated discordance. Twenty (64.5%) of the 31 patients showed consistent EEG abnormalities. CONCLUSIONS: Our data suggest that asymmetric mIP findings are an excellent lateralizing indicator in pediatric patients with partial epilepsy.

Alteraciones en las ejecuciones viso-perceptuales en pacientes con síndromes electro-clínicos de epilepsias del lóbulo temporal y frontal / Visuoperceptual performances impairments in patients with electro-clinical syndromes of temporal lobe and frontal lobe epilepsies

RESUMEN INTRODUCCIÓN: Según el modelo de actividad modular encapsulada del foco epiléptico (FE), las epilepsias del lóbulo temporal (ELI) y del frontal (ELF) se asocian con alteraciones cognitivas de memoria, atención, lenguaje y función ejecutiva. Por eso la mayoría de los protocolos neuropsicológicos se centran en la evaluación de estas funciones. Las habilidades viso-perceptuales se evaluarían en los pacientes con epilepsias del cuadrante posterior (ECP). OBJETIVO: Establecer las alteraciones viso-perceptuales en pacientes adultos con síndrome electro-clínico compatible con ELT y ELF de Medellín, Colombia, al compararse con un grupo de personas sin epilepsia. Pacientes y métodos: la muestra estuvo conformada por 19 pacientes, 10 (52,6 %) mujeres, edad de 43,0±12,3 (IC 95 %:37,1-48,9) años, con síndromes electro-clínicos por video-telemetría compatibles con ELT o ELF, con capacidad intelectual total estimada 107,2±14,1 (IC95 %:100,3-114,0). Se compararon con 16 personas sin epilepsia, 9(56,2 %) mujeres, edad 41,8±12,7 (IC95 %:35,1-48,6) y capacidad intelectual de 117±17,5 (IC95 %:108,6-127,2). Se les aplicó un protocolo para evaluación de procesos viso-perceptuales. RESULTADOS: Los pacientes presentaron un rendimiento significativamente inferior (p<0,05) en discriminación de figura-fondo, orientación viso-espacial de líneas y en el reconocimiento diferido de la memoria visual de puntos. Estas dificultades fueron mayores en los pacientes con ELF. CONCLUSIONES: Se observan alteraciones en ejecuciones viso-perceptuales y de almacenamiento de memoria visual más severas en pacientes con ELF. Esto apoya el modelo de redes complejas (no modulares y no encapsuladas), tanto para la actividad epiléptica como para la cognitiva. Por esto, los procesos viso-perceptuales deben ser evaluados en los pacientes con síndromes de ELT o ELF.

SUMMARY INTRODUCTION: According to modular encapsulated activity model of the epileptic focus (EF), Temporal lobe epilepsies (TLE) and Frontal lobe epilepsies (FLE) are associated to memory, attention, language and executive function impairments. For this reason most of the neuropsychological protocols are focused in the assessment of these functions. Visuoperceptual skills only would be assessed in patients with posterior quadrant epilepsies (PQE). OBJECTIVE: To establish visuoperceptual impairment in a sample of electro-clinical syndromes compatibles with TLE and FLE from Medellín-Colombia, compared with a group of healthy people without epilepsy. Patients and methods: sample was constituted by 19 patients, 10 women (52,6%), aged 43,0±12,3 (CI95%:37,1-48,9), with electro-clinical syndromes compatible with TLE or FLE, median estimated IQ 107,2±14,1 (CI95%:100,3-114,0). They were compared with 16 healthy people without epilepsy, 9(56,2%) women, median age 41,8±12,7 (CI95%:35,1-48,6) and 117±17,5 (IC95%:108,6-127,2). A visuoperceptual protocol was administered to both groups. RESULTS: Patients presented significant lower (p<0,05) on figure-background discrimination, visuospatial line orientations, and delayed recall by recognition of dots visual memory. These impairments were significant worst in FLE patients. CONCLUSIONS: TLE and FLE patients have visuoperceptual and delayed visual memory store impairments, which were significant worst in FLE patients. These findings support the complex network (non-modular and non-encapsulated) model to explain EF and cognitive functioning in patient with TLE and FLE. Visuoper-ceptual processes should be always assessed in these patients.

Cirugía resectiva de epilepsia lesional focal: Estudio en pacientes adultos del Seguro Social del Perú / Resective surgery in lesional focal epilepsy: A study in adult patients of Social Security in Peru

Objetivos: Reportar la experiencia en cirugía resectiva en una serie de pacientes adultos con epilepsia focal lesional tratados en un hospital del Seguro Social del Perú. Material y Métodos: Se revisó la información registrada en la Unidad de Epilepsia de Adultos del Hospital Rebagliati, en Lima, desde enero del 2012 hasta octubre del 2016, sobre características clínicas, electrográficas, de neuroimagen y quirúrgicas de todos los pacientes con epilepsia focal lesional farmacorresistente, en los cuales se efectuó cirugía resectiva de epilepsia. La eficacia del procedimiento fue valorada mediante la Escala de Engel. Resultados: Se describen los resultados de cinco pacientes,proveyéndose información pertinente respecto a historia de la enfermedad, diagnósticos precisos, procedimientos quirúrgicos, seguimiento y complicaciones neurológicas y psiquiátricas. Conclusiones: La cirugía de epilepsia resectiva, basada en un trabajo multidisciplinario es reportada por primera vez en esta pequeña serie, demuestra ser eficaz y segura en adultos con epilepsia refractaria tratados en el Seguro Social del Perú.

Objectives:To report the experience of resective surgery in a series of adult patients with lesional focal epilepsy seen in a hospital of Peru's Social Security system. Material and Methods: Data of the Adult Epilepsy Unit at Rebagliati Hospital in Lima, from January 2012 to October 2016 were reviewed. Information was obtained about clinical, electrographic, neuroimaging and surgical procedures of all patients with refractory lesional focal epilepsy, in whom resective epilepsy surgery was performed. Efficacy was evaluated using the Engel scale. Results: Analytic data of five patients with pertinent details regarding clinical history, diagnostic work-up, surgical procedures, follow-up and neurological and psychiatric complications, are presented and discussed. Conclusions: Resective epilepsy surgery, based on a multidisciplinary work and reported in this small first sample appears to be effective and safe in adults with refractory epilepsy seen in a Social Security facility from Peru.

Value of Repeat Brain MRI in Children with Focal Epilepsy and Negative Findings on Initial MRI

OBJECTIVE: To evaluate the value of repeat brain magnetic resonance imaging (MRI) in identifying potential epileptogenic lesions in children with initial MRI-negative focal epilepsy. MATERIALS AND METHODS: Our Institutional Review Board approved this retrospective study and waived the requirement for informed consent. During a 15-year period, 257 children (148 boys and 109 girls) with initial MRI-negative focal epilepsy were included. After re-evaluating both initial and repeat MRIs, positive results at repeat MRI were classified into potential epileptogenic lesions (malformation of cortical development and hippocampal sclerosis) and other abnormalities. Contributing factors for improved lesion conspicuity of the initially overlooked potential epileptogenic lesions were analyzed and classified into lesion factors and imaging factors. RESULTS: Repeat MRI was positive in 21% (55/257) and negative in 79% cases (202/257). Of the positive results, potential epileptogenic lesions comprised 49% (27/55) and other abnormalities comprised 11% of the cases (28/257). Potential epileptogenic lesions included focal cortical dysplasia (n = 11), hippocampal sclerosis (n = 10), polymicrogyria (n = 2), heterotopic gray matter (n = 2), microlissencephaly (n = 1), and cortical tumor (n = 1). Of these, seven patients underwent surgical resection. Contributing factors for new diagnoses were classified as imaging factors alone (n = 6), lesion factors alone (n = 2), both (n = 18), and neither (n = 1). CONCLUSION: Repeat MRI revealed positive results in 21% of the children with initial MRI-negative focal epilepsy, with 50% of the positive results considered as potential epileptogenic lesions. Enhanced MRI techniques or considering the chronological changes of lesions on MRI may improve the diagnostic yield for identification of potential epileptogenic lesions on repeat MRI.

Displasia cortical focal: hallazgos en imágenes de tensor de difusión

Objetivo: La displasia cortical focal (DCF) es una anomalía del desarrollo cortical. Representa una de las causas más frecuentes de epilepsia refractaria, siendo fundamental la resonancia magnética (RM) para su diagnóstico. Dada la importancia que ha cobrado la secuencia de tensor de difusión (DTI), el objetivo de este trabajo fue evaluar retrospectivamente los hallazgos en el mapa de fracción de anisotropía del DTI en pacientes con DCF. Observaciones: Se buscó retrospectivamente a pacientes con diagnóstico anatomopatológico de DCF en la base de datos de nuestro hospital. De un total de 74, se seleccionaron 8 casos con diagnóstico aislado de DCF y estudio de RM prequirúrgico con DTI. El foco de displasia se identificó en las imágenes anatómicas convencionales de la RM. Se evaluó el mapa de fracción de anisotropía (FA) y se definieron las alteraciones en la región de la DCF. Se observó una disminución de la FA en la sustancia blanca adyacente a la DCF en 7 de los 8 pacientes (87,5%). Discusión: Los hallazgos con el DTI brindan información complementaria en relación con la RM. En la mayoría de nuestros pacientes no hubo cambios de señal en la sustancia blanca en las imágenes convencionales, pero sí se identificó una disminución de la FAen el DTI. Se desconoce si esto refleja cambios estructurales o únicamente funcionales, secundarios a la lesión primaria. Creemos que el DTI puede agregar información complementaria de valor para el diagnóstico y valoración de la extensión de esta patología.

Objective: Focal cortical dysplasia (DCF) in an anomaly of cortical development. It represents one of the most frequent causes of drug resistant epilepsy and the magnetic resonance imaging (MRI) is trivial for its diagnosis. In the last years the use of diffusion tensor imaging (DTI) has increased in this kind of pathology. The purpose of this work was to evalúate retrospectively DTI findings on fractional anisotropy maps in patients with FCD. Observations: We retrospectively searched patients with confirmed anatomo-pathological diagnosis of FCD in our hospital datábase. From a total of 74, 8 patients, with isolated diagnosis of FCD and preoperative MRI with DTI, were selected. The FCD was identified in conventional anatomical MRI in all patients. Fractional anisotropy (FA) maps were evaluated and changes in the región of FCD were defined. Decreased FA was observed in white matter adjacent to the FCD, in 7 of 8 patients (87.5%). Discussion: Findings of DTI gives us complementary information to those of conventional MRI. Most of our patients showed no signal changes of white matter in conventional sequences and they presented decreased FA in DTI. We don't actually know if these DTI findings represent structural changes in white matter or just functional changes secondary to the adjacent FCD. We think DTI can give valuable complementary information for the diagnosis and determination of the extensión of this pathology.

Signal change in hippocampus and current source of spikes in Panayiotopoulos syndrome / 소아과

A 4-year-old girl with Panayiotopoulos syndrome presented with a history of 4 prolonged autonomic seizures. The clinical features of her seizures included, in order of occurrence, blank staring, pallor, vomiting, hemi-clonic movement on the right side, and unresponsiveness. A brain magnetic resonance imaging (MRI) showed a slightly high T2 signal in the left hippocampus. Interictal electoencephalogram revealed spikes in the occipital area of the left hemisphere. We analyzed the current-source distribution of the spikes to examine the relationship between the current source and the high T2 signal. The current source of the occipital spikes was not only distributed in the occipital area of both cerebral hemispheres, but also extended to the posterior temporal area of the left hemisphere. These findings suggest that the left temporal lobe may be one of the hyperexcitable areas and form part of the epileptogenic area in this patient. We hypothesized that the high T2 signal in the left hippocampus of our patient may not have been an incidental lesion, but instead may be related to the underlying electroclinical diagnosis of Panayiotopoulos syndrome, and particularly seizure. This notion is important because an abnormal T2 signal in the hippocampus may represent an acute stage of hippocampal injury, although there is no previous report of hippocampal pathology in Panayiotopoulos syndrome. Therefore, long-term observation and serial follow-up MRIs may be needed to confirm the clinical significance of the T2 signal change in the hippocampus of this patient.

Signal change in hippocampus and current source of spikes in Panayiotopoulos syndrome / 소아과

A 4-year-old girl with Panayiotopoulos syndrome presented with a history of 4 prolonged autonomic seizures. The clinical features of her seizures included, in order of occurrence, blank staring, pallor, vomiting, hemi-clonic movement on the right side, and unresponsiveness. A brain magnetic resonance imaging (MRI) showed a slightly high T2 signal in the left hippocampus. Interictal electoencephalogram revealed spikes in the occipital area of the left hemisphere. We analyzed the current-source distribution of the spikes to examine the relationship between the current source and the high T2 signal. The current source of the occipital spikes was not only distributed in the occipital area of both cerebral hemispheres, but also extended to the posterior temporal area of the left hemisphere. These findings suggest that the left temporal lobe may be one of the hyperexcitable areas and form part of the epileptogenic area in this patient. We hypothesized that the high T2 signal in the left hippocampus of our patient may not have been an incidental lesion, but instead may be related to the underlying electroclinical diagnosis of Panayiotopoulos syndrome, and particularly seizure. This notion is important because an abnormal T2 signal in the hippocampus may represent an acute stage of hippocampal injury, although there is no previous report of hippocampal pathology in Panayiotopoulos syndrome. Therefore, long-term observation and serial follow-up MRIs may be needed to confirm the clinical significance of the T2 signal change in the hippocampus of this patient.

Correlation between Patterns of Focal Epileptiform Discharges and Brain Lesions in Children with Focal Epilepsy / 대한소아신경학회지

PURPOSE: The objective of this study was to evaluate the correlation between patterns of focal epileptiform discharge (ED) and brain lesions diagnosed by MRI in children with focal epilepsy. METHODS: We retrospectively reviewed routine scalp EEGs, medical records, and results from brain MRIs in 149 children who had been diagnosed as epilepsy with focal epileptiform discharges in our hospital between 2008 and 2010. We classified the patterns of focal ED into spikes, sharp waves, and polyspikes. EEGs with homogenous patterns of ED were termed single-form ED and those with heterogenous patterns of ED were termed multi-form ED. We evaluated the relationship between the specific patterns of focal ED and brain lesions in pediatric epilepsy. RESULTS: Fifty-six of 149 (37.6%) patients had brain lesions on brain MRI. 67.8% (101/149) patients exhibited single-form ED and 32.2% (48/149) patients revealed multi-form ED. Thirty of 48 patients (62.5%) with multi-form ED showed brain lesions, which was higher than those for patients with single-form ED (25.7%, 26/101) (P <0.001). 27.5% (41/149) patients had and 25/41 (61.0%) also had brain lesion, which was higher than those for patients without polyspikes (28.7%, 31/108) (P <0.001). 19 of 82 patients (23.2%) with single-form ED with spikes only had brain lesion on brain MRI, which was significantly lower than those of remaining patients (55.2%, 37/67) (P <0.001). CONCLUSION: We suggest that a heterogenous pattern of ED or polyspikes lead to a higher incidence of brain lesions in children with focal epilepsy.

Experimental study of focused ultrasound on focal epilepsy in brain functional area / 国际外科学杂志

ObjectiveTo investigate the safety, efficacy and therapeutic mechanism of focused ultrasound surgery (FUS) on focal epilepsy of brain functional area in cat. MethodsThe focal epileptic models in the cats' motor cortex by penicillin were established. The different techniques were apphed in the animals epilepsy model. FUS, cortex thermocoagulation and multiple subpial transaction (MST) were compared in seizure control and functional preservation by observing behavior and electroencephalogram ( EEG), and the therapentic mechanism was compared by analysis of histopathology in the damaged cortex. ResultsIn seizure control and EEG changes, three kinds of techniques were similar without prominent difference in statistics (P ＞ 0.05). The same pathologic changes were showed in FUS group and cortex thermocoagulation group, ischemic change and coagulative necrosis of superficial layer nerval cells.The major pathologic change in MST group was an irregular crevice which was vertical with the surface of the cortex. The edema and mild gliosis were found around the crevice. ConclusionsThe focused ultrasound can effectively destroy and cut off the association of cortical horizontal fibre, meanwhile, the cortical function still to be reserved.Therefore, FUS is safe and effective as well as MST and cortex thermocoagulation on focal epilepsy in brain functional area.

Zonisamide Changes Unilateral Cortical Excitability in Focal Epilepsy Patients

BACKGROUND AND PURPOSE: To evaluate changes in cortical excitability induced by zonisamide (ZNS) in focal epilepsy patients. METHODS: Twenty-four drug-nasmall yi, Ukrainianve focal epilepsy patients (15 males; overall mean age 29.8 years) were enrolled. The transcranial magnetic stimulation parameters obtained using two Magstim 200 stimulators were the resting motor threshold, amplitude of the motor-evoked potential (MEP), cortical silent period, short intracortical inhibition, and intracortical facilitation. These five transcranial magnetic stimulation parameters were measured before and after ZNS, and the findings were compared. RESULTS: All 24 patients were treated with ZNS monotherapy (200-300 mg/day) for 8-12 weeks. After ZNS, MEP amplitudes decreased (-36.9%) significantly in epileptic hemispheres (paired t-test with Bonferroni's correction for multiple comparisons, p0.05). ZNS did not affect cortical excitability in nonepileptic hemispheres. CONCLUSIONS: These findings suggest that ZNS decreases cortical excitability only in the epileptic hemispheres of focal epilepsy patients. MEP amplitudes may be useful for evaluating ZNS-induced changes in cortical excitability.

Zonisamide Changes Unilateral Cortical Excitability in Focal Epilepsy Patients

BACKGROUND AND PURPOSE: To evaluate changes in cortical excitability induced by zonisamide (ZNS) in focal epilepsy patients. METHODS: Twenty-four drug-nasmall yi, Ukrainianve focal epilepsy patients (15 males; overall mean age 29.8 years) were enrolled. The transcranial magnetic stimulation parameters obtained using two Magstim 200 stimulators were the resting motor threshold, amplitude of the motor-evoked potential (MEP), cortical silent period, short intracortical inhibition, and intracortical facilitation. These five transcranial magnetic stimulation parameters were measured before and after ZNS, and the findings were compared. RESULTS: All 24 patients were treated with ZNS monotherapy (200-300 mg/day) for 8-12 weeks. After ZNS, MEP amplitudes decreased (-36.9%) significantly in epileptic hemispheres (paired t-test with Bonferroni's correction for multiple comparisons, p0.05). ZNS did not affect cortical excitability in nonepileptic hemispheres. CONCLUSIONS: These findings suggest that ZNS decreases cortical excitability only in the epileptic hemispheres of focal epilepsy patients. MEP amplitudes may be useful for evaluating ZNS-induced changes in cortical excitability.

Factores pronósticos de recurrencia de la epilepsia focal en el niño / Prognostic factors of recurrence of focal epilepsy in children

INTRODUCCIÓN. El objetivo de este trabajo fue determinar los factores pronósticos de recurrencia de las crisis epilépticas focales a los 2 años del diagnóstico y del inicio del tratamiento. MÉTODOS. Este estudio observacional, analítico y prospectivo incluyó a 207 niños que presentaron dos o más crisis epilépticas focales no provocadas, hospitalizados en el Departamento de Neuropediatría del Hospital William Soler, entre diciembre de 2001 y diciembre de 2003. Al final de los 2 años de seguimiento, 185 pacientes concluyeron el estudio. RESULTADOS. El 33,5 por ciento de los pacientes presentó recurrencias de las crisis epilépticas focales al finalizar el estudio. Constituyeron factores de riesgo de recurrencia de las crisis epilépticas focales los siguientes: edad menor de un año, etiología sintomática, presencia de antecedentes personales de crisis neonatales sintomáticas y discapacidades neurológicas y la persistencia de descargas en el electroencefalograma (EEG) evolutivo. El análisis de regresión logística demostró como variables pronósticas de recurrencia la etiología sintomática (p = 0,000; OR = 3,107), el antecedente personal de crisis neonatales sintomáticas (p = 0,037; OR = 4,623) y la persistencia de descargas en el EEG evolutivo (p = 0,000; OR = 2,109). CONCLUSIONES. El antecedente personal de crisis neonatales sintomáticas constituyó el factor independiente con mayor influencia en las recurrencias de las crisis epilépticas focales

INTRODUCTION: The objective of present paper was to determine the recurrent prognostic factors of focal epileptic crises at 2 years of diagnosis and of treatment onset. METHODS: This prospective, analytical and observational study included 207 children presenting two or more non-provoked epileptic crises, admitted in Neuropediatrics Department of William Soler Hospital between December, 2001 and December, 2003. At a two years follow-up, 185 patients concluded the study. RESULTS: The 33,5 percent of patients had recurrences of focal epileptic crises at the end of the year. Recurrent risk factors of crises above mentioned included: aged under 1 year, symptomatic etiology, presence of personal backgrounds of symptomatic neonatal crises and neurologic disabilities and persistence of the evolutionary electroenphalogram discharges. The logistic regression analysis showed as prognostic variables of symptomatic etiology recurrence (p = 0,000; OR = 3, 107), the persona background of symptomatic neonatal crises (p = 0,037; OR = 4,623) and the discharges persistence in the evolutionary electroencephalogram (p = 0,000; OR = 2,109). CONCLUSIONS: The personal background of symptomatic neonatal crises was the independent factor with a higher influence on recurrent focal epileptic crises

Relación entre electroencefalograma y neuroimagen en niños con epilepsia focal de difícil control / Relation between electroencephalogram and neuroimaging present in children with epilepsy of difficult control

Las epilepsias focales son las màs frecuentes en los niños, y la resistencia al tratamiento farmacológico puede estar presente hasta en el 30 por ciento de los pacientes. Se realizó este estudio con el objetivo de dirigir la atención hacia la coincidencia topográfica de los paroxismos electroencefalográficos, con lesiones estructurales demostrables por neuroimagen, para facilitar el diseño de estrategias terapéuticas futuras. MÉTODOS. Se realizó un estudio descriptivo, longitudinal prospectivo, con 44 niños con diagnóstico de epilepsia focal de difícil control, ingresados en el Servicio de Neuropediatría del Hospital Pediátrico Docente Juan M Márquez, entre enero de 2003 y junio de 2007. Se realizaron estudios por electroencefalograma (EEG) al ingreso y videoelectroencefalograma, además de estudios de neuroimagen por tomografía axial o resonancia magnética nuclear. RESULTADOS. Los paroxismos en EEG involucraron el lóbulo frontal hasta en el 68 por ciento de los pacientes. En el 48 por ciento de los pacientes, los paroxismos electroencefalográficos coinciden con zonas de alteración estructural según neuroimagen, más frecuentes en el lóbulo frontal. En el 25 por ciento no hay coincidencia topográfica y en el 27 por ciento no se precisan alteraciones estructurales. CONCLUSIONES. En las epilepsias focales de difícil control se debe prestar especial atención a las zonas elocuentes con coincidencia entre el EEG y la neuroimagen, para evaluar de forma temprana las alternativas quirúrgicas de tratamiento.

Focal epilepsies are the more frequent conditions in children and a pharmacologic treatment resistance could be present up to 30 percent of patients. Aim of present paper was to direct the attention to topographic coincidence of electroencephalographic paroxysms with structural lesions by neuroimaging facilitating the future therapeutical strategies design. METHODS: A descriptive, longitudinal, prospective study was conducted in 44 children diagnosed with epilepsy of difficult control admitted in Neuropediatrics Service of Juan Manuel Marquez Teaching Children Hospital from January, 2003 to June, 2007. At admission, we made electroencephalogram (EEG) and videoelectroencephalogram (VEEG) studies as well as neuroimaging studies by axial tomography (AT) or nuclear magnetic resonance (NMR). RESULTS: Paroxysms in EEG involved frontal lobule up to the 68 percent of patients. In 48 percent, electroencephalographic paroxysms coincide with structural alteration zones according neuroimaging, more frequent in frontal lobule. In 25 percent there is not topographic coincidence, and in 27 percent there are not specified structural alterations. CONCLUSIONS: In focal epilepsies of difficult control, we must to take care of eloquent zones with coincident between EEG and neuroimaging to assess in time the surgical treatment options.

Aspectos clínicos y electroencefalográficos de la epilepsia focal en el niño / Clinical and electroencephalographic features of focal epilepsy present in children

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.

Tratamento das epilepsias parciais / Partial epilepsies treatment

As epilepsias parciais constituem a forma mais comum de epilepsia nos indivíduos adultos. As drogas antiepilépticas (DAEs) permanecem como a principal forma de tratamento para os pacientes com epilepsia. Apesar da importância da medicação um número elevado de pacientes permanece sob um regime terapêutico inapropriado ou até mesmo sem qualquer medicação. Existem várias medicações disponíveis para o tratamento das epilepsias. A escolha de uma medicação específica ou a associação entre DAEs deve ser particularizada o máximo possível. Neste artigo revisamos alguns aspectos como classificação, início das crises, idade, sexo, comorbidades, custo e posologia das DAEs e história medicamentosa com a perspectiva de auxiliar nesta individualização do tratamento. Algumas características das principais DAEs disponíveis também são discutidas. Estes aspectos podem auxiliar na criação de um perfil ajudando assim na escolha do regime terapêutico mais apropriado para cada indivíduo. Aspectos práticos como o manuseio dos efeitos adversos, monoterapia e politerapia também são abordados.

Partial epilepsies are the most common form of epilepsy in adult individuals. Antiepileptic drugs (AEDs) continue as the main form of treatment for patients with epilepsy. Regardless of the importance of the medication a high number of patients are under inappropriate or not receiving AEDs. There are several medications available for the treatment of epilepsy. The choice of a particular medication or association among AEDs may be individualized as much as possible. In this article some aspects such as classification, onset of the seizures, age, sex, associated medical conditions, cost and posology of AEDs and medical drug history are reviewed. Details of the available AEDs are also discussed. These points may help to create a profile helping the decision for the appropriate AED. Some practical issues like adverse reaction management, monotherapy and politherapy are also discussed.

Tumor disembrioplástico neuroepitelial y epilepsia focal de larga evolución: informe de caso y revisión en la literatura / Dysembrioplastic neuroephitelial tumor and long lasting focal epilepsy: case report an literature review

Se presenta el caso clínico de un paciente de 20 años con epilepsia focal sintomática de larga evolución con poco control de sus crisis por tratamiento médico irregular. Las imágenes de resonancia magnética evidenciaron lesión multiquística principalmente del lóbulo temporal izquierdo. Se tomó biopsia estereotáxica cerebral de la lesión; la histolopatología mostró un componente glioneural en una matriz mucinosa, y por tanto, se hizo el diagnóstico de tumor disembrioplástico neuroepitelial. El paciente logra control de las crisis con ácido valproico y carbamazepina, mejora su estado funcional, aunque persiste el déficit cognitivo previo. No se realizó resección quirúrgica por ser una lesión benigna muy extensa y por el adecuado control de las crisis con el tratamiento médico. Debe sugerirse siempre el diagnóstico de tumor disembrioplástico neuroepitelial, ante cualquier tumor glial, en un paciente con larga historia de crisis focales, que inicien antes de los 20 años, sin déficit neurológico y que la resonancia magnética cerebral muestre una lesión cortical microquística, localizada principalmente en lóbulo temporal.

The case of a 20 year old boy, with long-lasting focal symptomatic epilepsy and a poor control of his seizures, because of irregular adherence to the neurological treatment, is presented. Brain MRI showed a multi-cystic lesion, located mainly in the temporal lobe. A brain stereotaxic biopsy was performed, finding a specific neural gliosis in a mucin-like matrix. The diagnosis of dysembryoplastic neuroepithelial tumor was done. Patient had a good control of his seizures with valproic acid and carbamazepine. Patient improved his functional state; however his previous cognitive deficit remains unchanged. Surgical resection was not developed because the lesion is considered as very benign and because the control of his seizures. The diagnosis of dysembryoplastic neuroepithelial tumor should be kept in mind in a patient with a long history of focal seizures; mainly when seizures onset begin before 20 years old, without motor or sensory neurological deficit, and when the MRI shows a cortical multi-cystic lesion, without mass effect, located in the temporal lobe.