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1.
Adv Tech Stand Neurosurg ; 48: 327-354, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37770690

RESUMO

The present article describes pathophysiological and clinical aspects of congenital malformations of the cerebral tissue (cortex and white matter) that cause epilepsy and very frequently require surgical treatment. A particular emphasis is given to focal cortical dysplasias, the most common pathology among these epilepsy-related malformations. Specific radiological and surgical features are also highlighted, so a thorough overview of cortical dysplasias is provided.


Assuntos
Epilepsia , Displasia Cortical Focal , Malformações do Desenvolvimento Cortical , Humanos , Malformações do Desenvolvimento Cortical/complicações , Epilepsia/etiologia , Córtex Cerebral/diagnóstico por imagem , Imageamento por Ressonância Magnética/efeitos adversos
2.
Childs Nerv Syst ; 39(5): 1193-1200, 2023 05.
Artigo em Inglês | MEDLINE | ID: mdl-36580119

RESUMO

PURPOSE: We aimed to analyze the potential for postoperative (PO) medication suspension and reduction, emphasizing passive withdrawal. METHODS: Retrospective study of patients under 18 years old submitted to surgical treatment for pharmacoresistant epilepsy and classified as Engel I during the first year of PO follow-up. Therapeutic management was evaluated through discontinuation or reduction of medications, both in terms of the number of ASM prescribed and in daily maintenance dosages in mg/kg. RESULTS: ASM withdrawal started in the first year PO and occurred in 1.2% of cases, with a significant yearly reduction in the number of ASM during follow-up (p < 0.001). A comparison of the most commonly used ASM in daily mg/kg between the preoperative period (preop) and PO showed a reduction of ASM maintenance dosages during PO. Even though recurrence of seizures was observed 5 years after surgery, 125 patients (85%) were still classified as Engel I, albeit a higher number of ASM per patient was observed. Most patients showed no changes in cognitive and adaptive behavior evaluation between preop and PO, even in those who were able to reduce ASM. CONCLUSION: Significant reduction observed both in the number and daily maintenance dosages of ASM following each year of PO may be an indirect measure of the effectiveness of epilepsy surgery.


Assuntos
Anticonvulsivantes , Epilepsia , Humanos , Criança , Adolescente , Estudos Retrospectivos , Anticonvulsivantes/uso terapêutico , Anticonvulsivantes/efeitos adversos , Resultado do Tratamento , Epilepsia/tratamento farmacológico , Epilepsia/cirurgia , Procedimentos Neurocirúrgicos
3.
Epilepsy Behav ; 125: 108447, 2021 12.
Artigo em Inglês | MEDLINE | ID: mdl-34839240

RESUMO

BACKGROUND: Cognitive functioning in epileptic syndromes has been widely explored in patients with temporal lobe epilepsy (TLE), but few studies have investigated the neuropsychological profile in posterior cortex epilepsy (PCE). In this study, we investigated the presurgical intellectual profile of children and adolescents with drug-resistant PCE. METHODS: Children and adolescents diagnosed with PCE (n = 25) participated in this study. The data were obtained from medical records, with assessments carried out between the years 2003 and 2019. To compare the intellectual profile, we also included patients diagnosed with frontal (n = 26) and temporal lobe epilepsy (n = 40). The Wechsler Intelligence Scales were used for the assessment of general intelligence. RESULTS: There was an effect of the brain region on the Working Memory Index (p < 0.01), in which patients with TLE had significantly higher scores than groups with FLE (p < 0.01) and PCE (p < 0.05). We also demonstrated that patients with PCE tended to perform worse in the Processing Speed Index than patients with TLE (p = 0.055). The Full-Scale Intelligence Quotient, Verbal Comprehension, and Perceptual Reasoning indexes did not differ among the brain regions. CONCLUSIONS: Children and adolescents with PCE demonstrated significant impairment in working memory and processing speed. The pattern of cognitive dysfunction in PCE was similar to that observed in FLE, which expands the evidence of the involvement of frontoparietal networks on cognitive proficiency.


Assuntos
Epilepsia do Lobo Temporal , Epilepsia , Adolescente , Criança , Cognição , Epilepsia do Lobo Temporal/complicações , Humanos , Inteligência , Testes Neuropsicológicos
4.
Epilepsia ; 61(8): 1581-1594, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32662890

RESUMO

OBJECTIVE: Drebrins are crucial for synaptic function and dendritic spine development, remodeling, and maintenance. In temporal lobe epilepsy (TLE) patients, a significant hippocampal synaptic reorganization occurs, and synaptic reorganization has been associated with hippocampal hyperexcitability. This study aimed to evaluate, in TLE patients, the hippocampal expression of drebrin using immunohistochemistry with DAS2 or M2F6 antibodies that recognize adult (drebrin A) or adult and embryonic (pan-drebrin) isoforms, respectively. METHODS: Hippocampal sections from drug-resistant TLE patients with hippocampal sclerosis (HS; TLE, n = 33), of whom 31 presented with type 1 HS and two with type 2 HS, and autopsy control cases (n = 20) were assayed by immunohistochemistry and evaluated for neuron density, and drebrin A and pan-drebrin expression. Double-labeling immunofluorescences were performed to localize drebrin A-positive spines in dendrites (MAP2), and to evaluate whether drebrin colocalizes with inhibitory (GAD65) and excitatory (VGlut1) presynaptic markers. RESULTS: Compared to controls, TLE patients had increased pan-drebrin in all hippocampal subfields and increased drebrin A-immunopositive area in all hippocampal subfields but CA1. Drebrin-positive spine density followed the same pattern as total drebrin quantification. Confocal microscopy indicated juxtaposition of drebrin-positive spines with VGlut1-positive puncta, but not with GAD65-positive puncta. Drebrin expression in the dentate gyrus of TLE cases was associated negatively with seizure frequency and positively with verbal memory. TLE patients with lower drebrin-immunopositive area in inner molecular layer (IML) than in outer molecular layer (OML) had a lower seizure frequency than those with higher or comparable drebrin-immunopositive area in IML compared with OML. SIGNIFICANCE: Our results suggest that changes in drebrin-positive spines and drebrin expression in the dentate gyrus of TLE patients are associated with lower seizure frequency, more preserved verbal memory, and a better postsurgical outcome.


Assuntos
Epilepsia Resistente a Medicamentos/metabolismo , Epilepsia do Lobo Temporal/metabolismo , Hipocampo/metabolismo , Neuropeptídeos/metabolismo , Adulto , Idoso , Idoso de 80 Anos ou mais , Lobectomia Temporal Anterior , Região CA1 Hipocampal/metabolismo , Região CA2 Hipocampal/metabolismo , Região CA3 Hipocampal/metabolismo , Estudos de Casos e Controles , Dendritos/metabolismo , Dendritos/patologia , Giro Denteado/metabolismo , Epilepsia Resistente a Medicamentos/patologia , Epilepsia Resistente a Medicamentos/cirurgia , Epilepsia do Lobo Temporal/patologia , Epilepsia do Lobo Temporal/cirurgia , Feminino , Glutamato Descarboxilase/metabolismo , Hipocampo/patologia , Hipocampo/cirurgia , Humanos , Imuno-Histoquímica , Masculino , Microscopia Confocal , Proteínas Associadas aos Microtúbulos/metabolismo , Pessoa de Meia-Idade , Plasticidade Neuronal , Esclerose , Proteína Vesicular 1 de Transporte de Glutamato/metabolismo
5.
Childs Nerv Syst ; 36(6): 1275-1282, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-31797069

RESUMO

PURPOSE: Hemispherectomy is an effective treatment option for pharmacoresistant epilepsy. Nevertheless, when high cortical functions are at risk during the presurgical evaluation, especially for older children, and for the left hemisphere, despite good seizure outcome, the anticipated decrease of cognitive functions may prevent a decision to perform surgery. The objective of this study is to report the cognitive outcome, based on verbal and performance intelligence skills, in a series of older children and adolescents who underwent left hemispherectomy, analyzing the risks (residual cognitive deficit) and benefits (seizure reduction) of surgery. METHODS: We retrospectively analyzed pre- and postoperative clinical and neuropsychological data from our patients who underwent left hemispherectomy, aged between 6 and 18 years. RESULTS: We included 15 patients, with a mean follow-up of 3.1 years, 12 patients (80%) were Engel I, and the other three were classified as Engel II, III, and IV. Nine patients were tested by Wechsler Scales of Intelligence; postsurgically all but one kept the same intellectual levels; verbal intelligence quotient (VIQ) remained unchanged in 13 and improved in one, whereas performance intelligence quotient (PIQ) decreased in four patients. Both Total Vineland and communication scores of Vineland Adaptive Behavior Scales were obtained in six patients: in all, scores were classified as deficient adaptive functioning pre- and postoperatively, remaining unchanged. CONCLUSION: The evaluation of the remaining intellectual abilities after left hemispherectomy in older children and adolescents is useful to discuss the risks and benefits of this surgery, enabling better and safer decisions regarding surgical indications and timing.


Assuntos
Epilepsia , Hemisferectomia , Adolescente , Criança , Cognição , Epilepsia/cirurgia , Seguimentos , Humanos , Estudos Retrospectivos , Resultado do Tratamento
6.
Electrophoresis ; 39(20): 2598-2604, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-29577353

RESUMO

Topiramate (TPM) is the main antiepileptic drug used for the control of partial and generalized seizures in both adults and children. In association with clinical observations, the analysis of plasmatic concentration of TPM is of utmost importance for the individual adjustment of the administered dose to the patient. In the present work, a bioanalytical method was developed and validated for TPM analysis in plasma samples by capillary electrophoresis with capacitively-coupled contactless conductivity detection (CE-C4 D). A simple background electrolyte composed of 15 mmol/L triethylamine, hydrodynamic injections (0.8 psi for 5 s) and a moderate separation voltage (20 kV) were used, rendering relatively short analysis times (<3 min). The sample pre-treatment was carried out by liquid-liquid extraction using methyl terc-butyl ether as solvent and 200 µL of plasma. The method was validated according to the official guidelines from the European Medicine Agency and showed linearity in plasmatic concentration range from 1 to 30 µg/mL, which covers the clinically-relevant interval. The lower limit of quantification of 1 µg/mL obtained also allows following patients with low dosage of the drug. The method was successfully applied to analysis of plasma samples and allowed the identification of 80% under-medicated patients in the analyzed patient pool.


Assuntos
Anticonvulsivantes/sangue , Monitoramento de Medicamentos/métodos , Eletroforese Capilar/métodos , Topiramato/sangue , Anticonvulsivantes/uso terapêutico , Condutividade Elétrica , Epilepsia/tratamento farmacológico , Humanos , Limite de Detecção , Modelos Lineares , Reprodutibilidade dos Testes , Topiramato/uso terapêutico
7.
Clin Exp Pharmacol Physiol ; 45(7): 652-658, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-29424067

RESUMO

Topiramate (TPM) is a second-generation antiepileptic drug (AED), acting on drug-resistant epilepsy. The aim of the study was to evaluate the influence of the dose, use of other AEDs on TPM plasma concentration (Cp ), and frequency of epileptic seizures. A cross-sectional analytical study was developed with patients aged 18-60 years, for diagnosis of drug-resistant epilepsy, using TPM in monotherapy or associated with other AEDs. The following variables were analyzed: age, frequency of epileptic seizures, pharmacotherapeutic regimen with its respective doses, adherence to medication treatment, and adverse events score. Thirty-seven patients were included, 83.8% of the patients presented Cp below the therapeutic range. Multiple linear regression estimated that the increase of 1.0 mg/kg/d promoted an increase of 0.68 µg/mL in TPMCp , while the use of inducers predicted a reduction of 2.97 µg/mL (P < .001). Multiple Poisson regression predicts that an increase of 1.0 µg/mL in TPMCp decreased the patient's chance of presenting seizures, and patients using AED inducers were about ten times more likely to present seizures than those who do not use (P < .001). In addition, for patients using AED inducers with Cp below the therapeutic range, the mean number of seizures per month was greater than those with Cp within the therapeutic range. The prescribed dose and the use of AED inducers influence Cp of TPM, likewise the low Cp of first-line AEDs and of the adjuvant in the treatment, TPM, as well as low TPM dose seem to affect the control of epileptic seizures.


Assuntos
Anticonvulsivantes/sangue , Anticonvulsivantes/uso terapêutico , Resistência a Medicamentos/efeitos dos fármacos , Convulsões/sangue , Convulsões/tratamento farmacológico , Topiramato/sangue , Topiramato/uso terapêutico , Adulto , Fatores Etários , Anticonvulsivantes/farmacologia , Estudos Transversais , Relação Dose-Resposta a Droga , Feminino , Humanos , Masculino , Topiramato/farmacologia
8.
Epilepsy Behav ; 76: 168-177, 2017 11.
Artigo em Inglês | MEDLINE | ID: mdl-28462844

RESUMO

Mesial temporal lobe epilepsy associated with hippocampal sclerosis (MTLE-HS) is one of the most common types of focal epilepsies. This is an epileptic syndrome commonly associated with treatment-resistant seizures, being also the most prevalent form of drug-resistant epilepsy which is treated surgically in most epilepsy surgery centers. Neurocysticercosis (NCC) is one of the most common parasitic infections of the central nervous system, and one of the most common etiological agents of focal epilepsy, affecting millions of patients worldwide. Recently, researchers reported a curious association between MTLE-HS with NCC, but this association remains poorly understood. Some argue that calcified NCC lesions in MTLE-HS patients is only a coincidental finding, since both disorders are prevalent worldwide. However, others suppose there might exist a pathogenic relationship between both disorders and some even suspect that NCC, by acting as an initial precipitating injury (IPI), might cause hippocampal damage and, eventually, MTLE-HS. In this review, we discuss the various reports that examine this association, and suggest possible explanations for why calcified NCC lesions are also observed in patients with MTLE-HS. We also propose mechanisms by which NCC could lead to MTLE-HS. Finally, we discuss the implications of NCC for the treatment of pharmacologically-resistant focal epilepsies in patients with calcified NCC or in patients with MTLE-HS and calcified NCC lesions. We believe that investigations in the relationship between NCC and MTLE-HS might offer further insights into how NCC may trigger epilepsy, and into how MTLE-HS originates. Moreover, observations in patients with drug-resistant epilepsy with both NCC and hippocampal sclerosis may not only aid in the understanding and treatment of patients with MTLE-HS, but also of patients with other forms of dual pathologies aside from NCC. This article is part of a Special Issue titled Neurocysticercosis and Epilepsy.


Assuntos
Epilepsia Resistente a Medicamentos/cirurgia , Epilepsia do Lobo Temporal/complicações , Neurocisticercose/complicações , Adulto , Epilepsia Resistente a Medicamentos/complicações , Epilepsia/complicações , Feminino , Hipocampo/patologia , Humanos , Masculino , Convulsões/complicações , Convulsões/cirurgia
9.
Clin Exp Pharmacol Physiol ; 43(7): 685-9, 2016 07.
Artigo em Inglês | MEDLINE | ID: mdl-27120710

RESUMO

Lamotrigine (LTG) is one of the most widely used antiepileptic drugs. Confusion still exists in the literature as to the relative influence of age, body weight, and concomitant drug therapy on LTG pharmacokinetics. So, the objective of this study is to evaluate the influence of patient-related factors and comedication on LTG apparent oral clearance (CL/F). A therapeutic drug-monitoring database was used to identify steady-state plasma LTG concentrations in 210 patients. LTG CL/F values were calculated for each patient according to the equation CL/F (L/h per kg) = LTG daily dose (mg/kg)/Css (steady state concentration) (mg/L) × 24 h. A linear-regression model was used to assess the influence of gender, dose, age, and body weight in LTG CL/F. The influence of comedication on LTG CL/F was investigated by applying the Bonferroni post-test. The lowest LTG CL/F was found in patients comedicated with valproate (VPA) (mean, 0.0183 L/h per kg), followed by patients receiving VPA + enzyme inducers (0.0271 L/h per kg), patients on LTG monotherapy (0.0298 L/h per kg) and patients comedicated with enzyme inducers (0.056 L/h per kg) LTG CL/F correlated significantly with LTG dose (P < 0.01), but showed no significant relationship with gender, weight, and age. LTG CL/F is influenced by the type of antiepileptic comedication. The correlation with dose may be a spurious finding related to the fact that physicians, in adjusting dosage according to clinical response, are more likely to use larger doses in patients with high clearance values.


Assuntos
Anticonvulsivantes/farmacocinética , Anticonvulsivantes/uso terapêutico , Epilepsia/tratamento farmacológico , Epilepsia/metabolismo , Triazinas/farmacocinética , Triazinas/uso terapêutico , Adulto , Peso Corporal/efeitos dos fármacos , Relação Dose-Resposta a Droga , Interações Medicamentosas/fisiologia , Quimioterapia Combinada/métodos , Feminino , Humanos , Cinética , Lamotrigina , Masculino , Ácido Valproico/uso terapêutico , Adulto Jovem
11.
Acta Neuropathol ; 128(1): 21-37, 2014 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-24823761

RESUMO

Hippocampal sclerosis (HS) is considered the most frequent neuropathological finding in patients with mesial temporal lobe epilepsy (MTLE). Hippocampal specimens of pharmacoresistant MTLE patients that underwent epilepsy surgery for seizure control reveal the characteristic pattern of segmental neuronal cell loss and concomitant astrogliosis. However, classification issues of hippocampal lesion patterns have been a matter of intense debate. International consensus classification has only recently provided significant progress for comparisons of neurosurgical and clinic-pathological series between different centers. The respective four-tiered classification system of the International League Against Epilepsy subdivides HS into three types and includes a term of "gliosis only, no-HS". Future studies will be necessary to investigate whether each of these subtypes of HS may be related to different etiological factors or with postoperative memory and seizure outcome. Molecular studies have provided potential deeper insights into the pathogenesis of HS and MTLE on the basis of epilepsy-surgical hippocampal specimens and corresponding animal models. These include channelopathies, activation of NMDA receptors, and other conditions related to Ca(2+) influx into neurons, the imbalance of Ca(2+)-binding proteins, acquired channelopathies that increase neuronal excitability, paraneoplastic and non-paraneoplastic inflammatory events, and epigenetic regulation promoting or facilitating hippocampal epileptogenesis. Genetic predisposition for HS is clearly suggested by the high incidence of family history in patients with HS, and by familial MTLE with HS. So far, it is clear that HS is multifactorial and there is no individual pathogenic factor either necessary or sufficient to generate this intriguing histopathological condition. The obvious variety of pathogenetic combinations underlying HS may explain the multitude of clinical presentations, different responses to clinical and surgical treatment. We believe that the stratification of neuropathological patterns can help to characterize specific clinic-pathological entities and predict the postsurgical seizure control in an improved fashion.


Assuntos
Epilepsia/patologia , Hipocampo/patologia , Esclerose Tuberosa/patologia , Animais , Diagnóstico Diferencial , Epilepsia/classificação , Epilepsia/fisiopatologia , Epilepsia/cirurgia , Hipocampo/fisiopatologia , Hipocampo/cirurgia , Humanos , Esclerose Tuberosa/classificação , Esclerose Tuberosa/fisiopatologia , Esclerose Tuberosa/cirurgia
12.
Epilepsy Behav ; 31: 329-33, 2014 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-24210463

RESUMO

Vagus nerve stimulation (VNS) seems to be effective in the management of selected cases of pharmacoresistant epilepsy in children. This was a case-control prospective study of children with refractory epilepsy submitted to vagal nerve stimulator implantation and a control group with epilepsy treated with antiepileptic drugs. Patients under 18years of age who underwent clinical or surgical treatment because of pharmacoresistant epilepsy from January 2009 to January 2012 were followed and compared with an age-matched control group at final evaluation. Statistically significant differences were observed considering age at epilepsy onset (VNS group - 1.33±1.45years; controls - 3.23±3.11; p=0.0001), abnormal findings in neurological examination (p=0.01), history of previous ineffective epilepsy surgery (p=0.03), and baseline seizure frequency (p=0.0001). At long-term follow-up, 55.4% of the patients in the VNS group had at least 50% reduction of seizure frequency, with 11.1% of the patients presenting 95% reduction on seizure frequency. Also, a decrease in traumas and hospitalization due to seizures and a subjective improvement in mood and alertness were observed. The control group did not show a significant modification in seizure frequency during the study. In this series, VNS patients evolved with a statistically significant reduction of the number of seizures, a decreased morbidity of the seizures, and the number of days in inpatient care. In accordance with the current literature, VNS has been proven to be an effective alternative in the treatment of pediatric patients with drug-resistant epilepsy.


Assuntos
Epilepsia/terapia , Pediatria , Estimulação do Nervo Vago/métodos , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Estudos Retrospectivos , Resultado do Tratamento
13.
Epilepsy Behav ; 38: 81-93, 2014 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-25216767

RESUMO

Epileptic syndromes and seizures are the expression of complex brain systems. Because no analysis of complexity has been applied to epileptic seizure semiology, our goal was to apply neuroethology and graph analysis to the study of the complexity of behavioral manifestations of epileptic seizures in human frontal lobe epilepsy (FLE) and temporal lobe epilepsy (TLE). We analyzed the video recordings of 120 seizures of 18 patients with FLE and 28 seizures of 28 patients with TLE. All patients were seizure-free >1 year after surgery (Engel Class I). All patients' behavioral sequences were analyzed by means of a glossary containing all behaviors and analyzed for neuroethology (Ethomatic software). The same series were used for graph analysis (CYTOSCAPE). Behaviors, displayed as nodes, were connected by edges to other nodes according to their temporal sequence of appearance. Using neuroethology analysis, we confirmed data in the literature such as in FLE: brief/frequent seizures, complex motor behaviors, head and eye version, unilateral/bilateral tonic posturing, speech arrest, vocalization, and rapid postictal recovery and in the case of TLE: presence of epigastric aura, lateralized dystonias, impairment of consciousness/speech during ictal and postictal periods, and development of secondary generalization. Using graph analysis metrics of FLE and TLE confirmed data from flowcharts. However, because of the algorithms we used, they highlighted more powerfully the connectivity and complex associations among behaviors in a quite selective manner, depending on the origin of the seizures. The algorithms we used are commonly employed to track brain connectivity from EEG and MRI sources, which makes our study very promising for future studies of complexity in this field.


Assuntos
Técnicas de Diagnóstico Neurológico , Epilepsia do Lobo Frontal/fisiopatologia , Epilepsia do Lobo Temporal/fisiopatologia , Modelos Neurológicos , Convulsões/fisiopatologia , Adulto , Eletroencefalografia/métodos , Etologia/métodos , Feminino , Humanos , Masculino , Gravação em Vídeo
14.
Neurol Sci ; 35(2): 239-44, 2014 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-24091711

RESUMO

The cellular prion protein, encoded by Prnp gene, is involved in neuroprotection, neuroplasticity and neurodevelopment. The variant allele Valine at codon 129 of the Prnp was associated with decreased brain volume in healthy volunteers and schizophrenic patients. We investigate the association between the cerebellum volume and the presence of variant allele Valine at codon 129 of the Prnp gene in patients with mesial temporal lobe epilepsy related to hippocampal sclerosis (MTLE-HS). The Prnp coding sequence was determined in 41 refractory MTLE-HS patients. The cerebellum volume corrected by the intracranial volume of patients with the normal Prnp genotypes was compared with that of patients presenting the variant alleles at codon 129. Twenty patients showed the Met129Met genotype, 16 showed Met129Val, and 5 had Val129Val. There were no association among clinical, demographic, electrophysiological, antiepileptic drugs used, and the presence of the Prnp variant alleles. The presence of Prnp variant allele at codon 129 was not associated with the analyzed cerebellum volume. Prnp variant alleles at codon 129 are not associated with cerebellum volume in patients with refractory MTLE-HS.


Assuntos
Cerebelo/patologia , Epilepsia do Lobo Temporal/genética , Epilepsia do Lobo Temporal/patologia , Príons/genética , Adulto , Alelos , Anticonvulsivantes/uso terapêutico , Encéfalo/patologia , Eletroencefalografia , Epilepsia do Lobo Temporal/tratamento farmacológico , Epilepsia do Lobo Temporal/fisiopatologia , Feminino , Variação Genética , Humanos , Imageamento por Ressonância Magnética , Masculino , Tamanho do Órgão , Proteínas Priônicas , Convulsões/tratamento farmacológico , Convulsões/genética , Convulsões/patologia , Convulsões/fisiopatologia
15.
Childs Nerv Syst ; 30(11): 1813-21, 2014 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-25296542

RESUMO

BACKGROUND AND PURPOSE: Hemispheric dysplasia (HD) and hemimegalencephaly (HME) are both brain malformations with early clinical manifestation including developmental delay and intractable epilepsy. Sometimes the differentiation of these conditions is not simple. HME is an anomaly of cortical development caused by a combination of neural proliferation and cell migration dysfunction, showing lobar or hemispheric enlargement. On the other hand, HD shows no brain hypertrophy, and even brain atrophy, eventually. PATIENTS AND METHODS: To compare both conditions, we reviewed clinical, MRI, and histopathology of 23 patients with developmental delay and refractory epilepsy treated with hemispheric surgery. RESULTS: Histologically, both groups presented polymicrogyria, focal cortical dysplasia, gray matter (GM) heterotopia, pachygyria, and agyria. The white matter (WM) showed different degrees of gliosis and myelin impairment. Even though with no specificity in histopathology, the degree of lesion was more impressive on HME. The combination of WM dysmyelination and hypertrophy leads to the so called hamartomatous appearing. Although not all HME showed brain enlargement and some HD might show no size changes or atrophy, the size of affected hemisphere and the hamartomatous appearance of the WM were the more relevant signs to differentiate both conditions. CONCLUSION: Brain MRI was the best diagnostic tolls because it allowed together high contrast resolution, whole brain coverage and spatial distribution analysis. HD and HMD showed brain asymmetry tendency, but in opposite directions. The size of affected hemisphere and the hamartomatous appearance of the WM were the more relevant signs to differentiate both conditions.


Assuntos
Afasia/diagnóstico , Lateralidade Funcional , Megalencefalia/complicações , Neuroimagem/métodos , Humanos
16.
Childs Nerv Syst ; 30(11): 1839-51, 2014 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-25296545

RESUMO

OBJECTIVE: Children with malformation of cortical development represent a significant proportion of pediatric epilepsy surgery candidates. Here, we describe a single-center experience with pediatric patients who underwent surgery for intractable epilepsy due to focal cortical dysplasia (FCD). METHODS: Clinical data of 78 patients under 18 years of age with diagnosis of intractable epilepsy due to FCD who underwent surgery from January 1996 to January 2012 were reviewed comparing data of patients submitted to electrocorticography (ECoG) with those without ECoG. RESULTS: Patients' mean age at surgery was 8.52 ± 4.99 years; mean age at epilepsy onset was 2.55 ± 3.01 years. Almost 80 % of the patients underwent ECoG register that was essential for delimitation of surgical resection in 66 out of 78 patients. ECoG was performed in all patients with extratemporal lesions, and the most common FCD found was type II. Seizure outcome was similar in groups with or without ECoG. CONCLUSIONS: Tailored resection of FCD lesions for intractable epilepsy can be safely performed in children with a good seizure outcome and low complication rate. Epilepsy surgery should be considered for all patients with FCD and refractory epilepsy.


Assuntos
Mapeamento Encefálico , Epilepsia/cirurgia , Monitorização Neurofisiológica Intraoperatória/métodos , Malformações do Desenvolvimento Cortical/cirurgia , Neurocirurgia/métodos , Adolescente , Ondas Encefálicas/fisiologia , Distribuição de Qui-Quadrado , Criança , Eletroencefalografia , Epilepsia/complicações , Feminino , Seguimentos , Humanos , Masculino , Malformações do Desenvolvimento Cortical/classificação , Malformações do Desenvolvimento Cortical/complicações , Estudos Retrospectivos
17.
Pediatr Neurol ; 150: 82-90, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37992429

RESUMO

BACKGROUND: Rasmussen encephalitis (RE) is characterized by pharmacoresistant epilepsy and progressive neurological deficits concurrent with unilateral hemispheric atrophy. Evidence of an inflammatory autoimmune process has been extensively described in the literature; however, the precise etiology of RE is still unknown. Despite data supporting a beneficial effect of early immunosuppressive and immunomodulatory interventions, surgical disconnection of the affected hemisphere is considered the treatment of choice for these patients. The aim of this study was to report a series of children and adolescents who underwent hemispheric surgery (HS) for the treatment of RE, analyzing their clinical, electrographic, and neuroimaging features pre-operatively, as well as their postoperative status, including seizure and functional outcomes. METHODS: All patients with RE who underwent HS in the Epilepsy Surgery Center (CIREP) of the University Hospital of Ribeirão Preto Medical School, between 1995 and 2020 were retrospectively reviewed. Preoperative and postoperative analyses included gender; age at epilepsy onset; seizure semiology; seizure frequency; interictal and ictal electroencephalographic (EEG) findings; age at surgery; duration of epilepsy; surgical complications; duration of follow-up; histopathological findings; and postoperative seizure, cognitive, and functional outcomes. RESULTS: Forty-four patients were evaluated. Mean age at seizure onset and epilepsy duration was 6 years and 2.5 years, respectively. Mean age at surgery was 9 years, with an average follow-up ranging from 3 months to 23 years. All patients presented with severe epilepsy and distinct neurological abnormalities on MRI. Before HS, different degrees of abnormal intellectual performance as well as hemiparesis were seen in 86% and 90%, respectively. Histopathology examination confirmed this diagnosis in 95% patients. At the last follow-up, 68% of patients were seizure free, and 70% were classified as Engel Class I or II. Postoperatively, the cognitive status remained unchanged in 64% of patients. Likewise, the gross motor function remained unchanged in 54% of patients and 74% had functional hand ability after HS. CONCLUSIONS: Considering the progressive damage course of RE, hemispheric surgery should be offered to pediatric patients. It has manageable risks and results in good seizure outcome, and the preoperative functional status of these children is often preserved (even when the left hemisphere is involved), thus improving their quality of life.


Assuntos
Encefalite , Epilepsia , Hemisferectomia , Adolescente , Criança , Humanos , Hemisferectomia/efeitos adversos , Hemisferectomia/métodos , Resultado do Tratamento , Estudos Retrospectivos , Qualidade de Vida , Convulsões/etiologia , Encefalite/diagnóstico por imagem , Encefalite/cirurgia , Encefalite/complicações , Eletroencefalografia/métodos
18.
Epilepsia ; 54(12): 2025-35, 2013 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-24134485

RESUMO

Cerebral cavernous malformations (CCMs) are well-defined, mostly singular lesions present in 0.4-0.9% of the population. Epileptic seizures are the most frequent symptom in patients with CCMs and have a great impact on social function and quality of life. However, patients with CCM-related epilepsy (CRE) who undergo surgical resection achieve postoperative seizure freedom in only about 75% of cases. This is frequently because insufficient efforts are made to adequately define and resect the epileptogenic zone. The Surgical Task Force of the Commission on Therapeutics of the International League Against Epilepsy (ILAE) and invited experts reviewed the pertinent literature on CRE. Definitions of definitive and probable CRE are suggested, and recommendations regarding the diagnostic evaluation and etiology-specific management of patients with CRE are made. Prospective trials are needed to determine when and how surgery should be done and to define the relations of the hemosiderin rim to the epileptogenic zone.


Assuntos
Neoplasias Encefálicas/complicações , Epilepsia/etiologia , Hemangioma Cavernoso do Sistema Nervoso Central/complicações , Anticonvulsivantes/uso terapêutico , Encéfalo/patologia , Encéfalo/fisiopatologia , Encéfalo/cirurgia , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/cirurgia , Epilepsia/tratamento farmacológico , Epilepsia/cirurgia , Hemangioma Cavernoso do Sistema Nervoso Central/diagnóstico , Hemangioma Cavernoso do Sistema Nervoso Central/cirurgia , Humanos , Neuroimagem , Fatores de Risco
19.
J Sex Med ; 10(2): 493-9, 2013 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-23057419

RESUMO

INTRODUCTION: Epileptic men may experience hormonal changes that may alter semen quality and sexual function. Alterations in male sexual and reproductive parameters may also be due to treatment with antiepileptic drugs to control seizures. AIMS: To evaluate serum hormone concentrations, semen quality, the frequency of sexual intercourse (FSI), and erectile function in men with epileptic seizures controlled by carbamazepine (CBZ). MAIN OUTCOME MEASURES: The five-question form of the International Index of Erectile Function (IIEF-5), and semi-structured questionnaire. METHODS: One hundred and eighteen men, aged 18-45 years, were included in this controlled, cross-sectional study: 63 men taking CBZ (epileptic group) were compared to 55 healthy men (control group). Blood sample was collected to determine hormones concentrations. Erectile function and the frequency of sexual relations were assessed by using questionnaires. Sperm morphology was analyzed by examining the quality of the head, intermediate part and tail of the spermatozoa. RESULTS: Using the IIEF-5, we observed a significant association between erectile dysfunction (ED) and groups (P < 0.01), where epileptic men had 17.33 (95% CI 3.59, 83.52) odds to have erectile dysfunction. Adjusted odds ratio to group considering luteinizing hormone, prolactin, Serum total testosterone, androstenedione, and dehydroepiandrosterone, androstenedione levels and free androgen index, we observed only group effect where epileptic men had 10.47 (95% CI 2.75, 39.83) odds to have FSI < 3 times a week. Sperm vitality was altered in 27% of the epileptic subjects compared with 5.4% of the control group (P < 0.002). Sperm motility differed significantly between groups, with A + B motility ≤50% observed in 98.4% of the epileptic group and in 85.4% of the control group (P < 0.01). Sperm morphology <14% was observed in 93.7% of the epileptic men, compared with 34.6% of the controls (P < 0.001). CBZ users, showed less sexual intercourse then controls (P ≤ 0.001). CONCLUSIONS: Epileptic men taking CBZ present with changes in hormonal levels, altered semen quality, ED, and a reduction in coital frequency.


Assuntos
Anticonvulsivantes/efeitos adversos , Carbamazepina/efeitos adversos , Epilepsia do Lobo Temporal/tratamento farmacológico , Disfunção Erétil/induzido quimicamente , Análise do Sêmen , Comportamento Sexual/efeitos dos fármacos , Adolescente , Adulto , Anticonvulsivantes/uso terapêutico , Carbamazepina/uso terapêutico , Estudos Transversais , Epilepsia do Lobo Temporal/sangue , Disfunção Erétil/sangue , Disfunção Erétil/diagnóstico , Hormônios Esteroides Gonadais/sangue , Humanos , Masculino , Motilidade dos Espermatozoides/efeitos dos fármacos , Adulto Jovem
20.
Epilepsy Behav ; 26(1): 67-70, 2013 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-23220462

RESUMO

Morphological variations of hippocampal formation (MVHF) are observed in patients with epilepsy but also in asymptomatic individuals. The precise role of these findings in epilepsy is not yet fully understood. This study analyzes the hippocampal formation (HF) morphology of asymptomatic individuals (n = 30) and of patients with mesial temporal lobe epilepsy associated with hippocampal sclerosis (MTLE-HS) (n = 68), patients with malformations of cortical development (MCD) (n = 34), or patients with pure morphological variations of hippocampal formation (pure MVHF) (n = 12). Main clinical and electrophysiological data of patients with MVHF were also analyzed. Morphological variations of hippocampal formation are more frequently observed in patients with MCD than in patients with MTLE-HS or in asymptomatic individuals. Patients with pure morphological variations of hippocampal formation showed higher incidence of extratemporal seizure onset. Refractoriness seems to be more associated with other abnormalities, like HS or MCD, than with the HF variation itself. Thus, although morphological HF abnormalities might play a role in epileptogenicity, they seem to contribute less to refractoriness.


Assuntos
Ondas Encefálicas/fisiologia , Epilepsia/patologia , Epilepsia/fisiopatologia , Hipocampo/patologia , Neuroimagem , Adolescente , Adulto , Análise de Variância , Eletroencefalografia , Feminino , Humanos , Masculino , Malformações do Desenvolvimento Cortical/patologia , Malformações do Desenvolvimento Cortical/fisiopatologia , Pessoa de Meia-Idade , Esclerose/complicações , Esclerose/patologia , Adulto Jovem
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