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Previous studies, using surveys, provided an understanding about how health-care providers address patients with PNES. To date, there is limited information on the management of patients with PNES by tertiary referral centers for epilepsy. In this study, we surveyed 11 Brazilian epilepsy center directors about diagnosis, treatment, education and research on PNES. Respondents reported that patients with PNES represented 10-20% of all adult patients recorded by video-EEG (VEEG). All respondents recognized VEEG as the method to confirm the diagnosis, and 81.8% used this approach for confirmation. Most centers had a standard protocol for diagnosis. None of the centers had a particular protocol to treat PNES, but 90.9% had a uniform treatment approach including therapy and educational measures. Psychotherapy was not easily obtained in nine centers (81.8%). Seven (63.3%) centers reported ongoing research projects with PNES. Five centers referred to an educational PNES program discussing diagnosis, but only one reported an educational program for treatment. This study showed a commitment to PNES diagnosis; however, some gaps remain regarding treatment and training, namely implementing a psychotherapy approach for patients and providing educational curricula for clinicians.
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Educación del Paciente como Asunto , Trastornos Psicofisiológicos/diagnóstico , Psicoterapia , Convulsiones/diagnóstico , Brasil , Electroencefalografía/métodos , Encuestas de Atención de la Salud , Humanos , Trastornos Psicofisiológicos/psicología , Trastornos Psicofisiológicos/terapia , Convulsiones/psicología , Convulsiones/terapiaRESUMEN
Recent observations suggest that neurocysticercosis (NCC) might act as an initial precipitating injury (IPI) causing mesial temporal lobe epilepsy associated with hippocampal sclerosis (MTLE-HS). A total of 191 patients from Brazil, a country in which NCC is endemic, were surgically treated for MTLE-HS, and subsequent findings for patients with MTLE-HS were compared with those of patients with MTLE-HS plus NCC. Seventy-one patients (37,2%) presented chronic findings of NCC (cNCC). MTLE-HS plus cNCC was significantly more common in women (O.R.=2.45; 95%CI=1.30-4.60; p=0.005), in patients with no history of classical forms of IPI (O.R.=2.67; 95%CI=1.37-5.18; p=0.004), and in those with bi-temporal interictal spikes on video-EEG (O.R.=2.00; 95%CI=1.07-3.73; p=0.03). Single cNCC lesions were observed to occur significantly more often on the same side as hippocampal sclerosis, a finding suggesting an anatomical relationship between NCC and MTLE-HS. Taken together, our results suggest that NCC may be a marker, or contributes to or even causes MTLE-HS. Based on our findings, we propose two distinct, non-excluding, and potentially synergistic mechanisms involved in the development of MTLE-HS in NCC, one of them being inflammatory-mediated, while the other is electrogenic-mediated. Taken together, our observations may provide further evidence suggesting a role of NCC in the genesis or development of MTLE-HS.
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Epilepsia del Lóbulo Temporal/epidemiología , Hipocampo/fisiopatología , Neurocisticercosis/epidemiología , Esclerosis/epidemiología , Adulto , Brasil/epidemiología , Enfermedad Crónica , Electroencefalografía , Epilepsia del Lóbulo Temporal/patología , Epilepsia del Lóbulo Temporal/fisiopatología , Epilepsia del Lóbulo Temporal/cirugía , Femenino , Estudios de Seguimiento , Hipocampo/patología , Hipocampo/cirugía , Humanos , Modelos Logísticos , Imagen por Resonancia Magnética , Masculino , Neurocisticercosis/patología , Esclerosis/patología , Esclerosis/fisiopatología , Esclerosis/cirugía , Factores Sexuales , Grabación en VideoRESUMEN
UNLABELLED: Gaucher disease type III (GD III) is a rare form of GD characterized by neurological involvement and severe systemic disease. The objective of this study was to assess the nutritional status and energy metabolism of patients with GD III. METHODS: The basal metabolic rate (BMR, measured by indirect calorimetry) and anthropometric parameters (height, weight, body mass index (BMI), and arm circumference) of three patients with GD III (p.L444P/L444P genotype) were assessed at different time points. The clinical severity of GD was assessed by means of physical examination, laboratory tests, imaging findings, and the severity scores proposed by Zimran (SSI) and Davies (SSNI). RESULTS: The measured BMR of patients 1 (age 14 years, not on enzyme replacement therapy (ERT), SSI score 33, SSNI score 14.5), 2 (age 17 years, on ERT, SSI score 33, SSNI score 16), and 3 (age 20 years, on ERT, SSI score 33, SSNI score 7.5) was, respectively, 47%, 72%, and 15% higher than that estimated by the Harris-Benedict equation. Patients with a more severe phenotype had more marked hypermetabolism. Patients 1 and 2 had BMI-for-age z scores of -1.09 and -1.39, respectively, and height-for-age z scores of -4.27 and -3.02, respectively; patient 3 had a BMI of 24.7 kg/m(2). CONCLUSION: All three patients showed hypermetabolism; however, the two patients with the highest BMR had more severe GD and were malnourished. Additional studies are warranted to assess whether hypermetabolism may be a biomarker of disease severity in GD.
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PURPOSE: Seizure recurrence after epilepsy surgery has been classified as either early or late depending on the recurrence time after operation. However, time of recurrence is variable and has been arbitrarily defined in the literature. We established a mathematical model for discriminating patients with early or late seizure recurrence, and examined differences between these two groups. METHODS: A historical cohort of 247 consecutive patients treated surgically for temporal lobe epilepsy was identified. In patients who recurred, postoperative time until seizure recurrence was examined using an receiver-operating characteristic (ROC) curve to determine the best cutoff for predicting long-term prognosis, dividing patients in those with early and those with late seizure recurrence. We then compared the groups in terms of a number of clinical, electrophysiologic, and radiologic variables. KEY FINDINGS: Seizures recurred in 107 patients (48.9%). The ROC curve demonstrated that 6 months was the ideal time for predicting long-term surgical outcome with best accuracy, (area under the curve [AUC] = 0.761; sensitivity = 78.8%; specificity = 72.1%). We observed that patients with seizure recurrence during the first 6 months started having seizures at younger age (odds ratio [OR] = 6.03; 95% confidence interval [CI] = 1.06-11.01; p = 0.018), had a worse outcome (OR = 6.85; 95% CI = 2.54-18.52; p = 0.001), needed a higher number of antiepileptic medications (OR = 2.07; 95% CI = 1.16-9.34; p = 0.013), and more frequently had repeat surgery (OR = 9.59; 95% CI = 1.18-77.88; p = 0.021). Patients with late relapse more frequently had seizures associated with trigger events (OR = 9.61; 95% CI = 3.52-26.31; p < 0.01). SIGNIFICANCE: Patients with early or late recurrence of seizures have different characteristics that might reflect diversity in the epileptogenic zone and epileptogenicity itself. These disparities might help explain variable patterns of seizure recurrence after epilepsy surgery.
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Epilepsia del Lóbulo Temporal/cirugía , Convulsiones , Anticonvulsivantes/uso terapéutico , Niño , Preescolar , Electroencefalografía/métodos , Epilepsia del Lóbulo Temporal/tratamiento farmacológico , Femenino , Humanos , Lactante , Masculino , Periodo Posoperatorio , Prevención Secundaria , Convulsiones/tratamiento farmacológico , Factores de Tiempo , Resultado del TratamientoRESUMEN
Patients with left mesial temporal lobe epilepsy (MTLE) have deficits in verbal memory processes, while patients with right MTLE have visuospatial memory impairment. However, atypical cognitive phenotypes among patients with MTLE may occur. In this study, we analyzed preoperative memory deficits in a cohort of 426 right-handed patients with unilateral MTLE. We also evaluated the cognitive outcome after anterior temporal lobectomy (ATL) of patients with atypical profiles in comparison with those with typical memory profile. We found that 25% of our patients had a typical cognitive profile, with verbal memory deficits associated with left side hippocampal sclerosis (HS) and visuospatial memory deficits associated with right side HS. However, 75% of our patients had atypical memory profiles. Despite these atypical profiles, patients submitted to right ATL had no significant cognitive deficit after surgery. In patients submitted to left ATL, the higher the presurgical scores on verbal memory and naming tests, the higher the cognitive decline after surgery.
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Trastornos del Conocimiento/diagnóstico , Trastornos del Conocimiento/etiología , Epilepsia del Lóbulo Temporal/complicaciones , Lateralidad Funcional/fisiología , Trastornos de la Memoria/diagnóstico , Trastornos de la Memoria/etiología , Adulto , Análisis de Varianza , Electroencefalografía , Epilepsia del Lóbulo Temporal/cirugía , Femenino , Humanos , Masculino , Persona de Mediana Edad , Neuroimagen , Pruebas Neuropsicológicas , Procedimientos Neuroquirúrgicos , Percepción Espacial/fisiología , Grabación en VideoRESUMEN
BACKGROUND: Where neurocysticercosis (NCC) is endemic, chronic calcified neurocysticercosis (cNCC) can be observed in patients with mesial temporal lobe epilepsy associated with hippocampal sclerosis (MTLE-HS). Considering that both disorders cause recurrent seizures or cognitive impairment, we evaluated if temporal lobectomy is cognitively safe and effective for seizure control in MTLE-HS plus cNCC. METHODS: Retrospective cohort study of neuropsychological profile and surgical outcome of 324 MTLE-HS patients submitted to temporal lobectomy, comparing the results according to the presence or absence of cNCC. FINDINGS: cNCC occurred in 126 (38.9%) of our MTLE-HS patients, a frequency higher than expected, more frequently in women than in men (O.R.â=â1.66; 95% C.I.â=â1.05-2.61; pâ=â0.03). Left-side (but not right side) surgery caused impairment in selected neuropsychological tests, but this impairment was not accentuated by the presence of cNCC. Ninety-four (74.6%) patients with MTLE-HS plus cNCC and 153 patients (77.3%) with MTLE-HS alone were Engel class I after surgery (O.R.â=â1.16; 95% C.I.â=â0.69-1.95; pâ=â0.58). However, the chances of Engel class IA were significantly lower in MTLE-HS plus cNCC than in patients with MTLE-HS alone (31.7% versus 48.5%; O.R.â=â2.02; 95% C.I.â=â1.27-3.23; pâ=â0.003). Patients with MTLE-HS plus cNCC showed higher rates of Engel class ID (15.1% versus 6.6%; O.R.â=â2.50; 95% C.I.â=â1.20-5.32; pâ=â0.012). INTERPRETATION: cNCC can be highly prevalent among MTLE-HS patients living in areas where neurocysticercosis is endemic, suggesting a cause-effect relationship between the two diseases. cNCC does not add further risk for cognitive decline after surgery in MTLE-HS patients. The rates of Engel class I outcome were very similar for the two groups; however, MTLE-HS plus cNCC patients achieved Engel IA status less frequently, and Engel ID status more frequently. Temporal lobectomy can be safely performed in most patients with MTLE-HS plus cNCC without affecting cognitive outcome. Long-term surgical seizure control in MTLE-HS plus cNCC is still satisfactory, as long as selected patients remain under medication.
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Cognición , Epilepsia del Lóbulo Temporal/fisiopatología , Epilepsia del Lóbulo Temporal/cirugía , Hipocampo/patología , Neurocisticercosis/complicaciones , Adulto , Niño , Estudios de Cohortes , Toma de Decisiones , Epilepsia del Lóbulo Temporal/complicaciones , Epilepsia del Lóbulo Temporal/patología , Femenino , Humanos , Masculino , Esclerosis/complicaciones , Resultado del TratamientoRESUMEN
We studied the prevalence and associated factors of psychiatric comorbidities in 490 patients with refractory focal epilepsy. Of these, 198 (40.4%) patients had psychiatric comorbidity. An Axis I diagnosis was made in 154 patients (31.4%) and an Axis II diagnosis (personality disorder) in another 44 (8.97%) patients. After logistic regression, positive family history of psychiatric comorbidities (O.R.=1.98; 95% CI=1.10-3.58; p=0.023), the presence of Axis II psychiatric comorbidities (O.R.=3.25; 95% CI=1.70-6.22; p<0.0001), and the epileptogenic zone located in mesial temporal lobe structures (O.R.=1.94; 95% CI=1.25-3.03; p=0.003) remained associated with Axis I psychiatric comorbidities. We concluded that a combination of clinical variables and selected structural abnormalities of the central nervous system contributes to the development of psychiatric comorbidities in patients with focal epilepsy.
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Epilepsias Parciales/epidemiología , Trastornos Mentales/epidemiología , Adolescente , Adulto , Anciano , Trastornos de Ansiedad/epidemiología , Distribución de Chi-Cuadrado , Comorbilidad , Femenino , Humanos , Modelos Logísticos , Masculino , Persona de Mediana Edad , Trastornos del Humor/epidemiología , Trastornos de la Personalidad/epidemiología , Trastornos Psicóticos/epidemiología , Estadísticas no Paramétricas , Adulto JovenRESUMEN
PURPOSE: Refractory frontal lobe epilepsy (FLE) remains one of the most challenging surgically remediable epilepsy syndromes. Nevertheless, definition of independent predictors and predictive models of postsurgical seizure outcome remains poorly explored in FLE. METHODS: We retrospectively analyzed data from 70 consecutive patients with refractory FLE submitted to surgical treatment at our center from July 1994 to December 2006. Univariate results were submitted to logistic regression models and Cox proportional hazards regression to identify isolated risk factors for poor surgical results and to construct predictive models for surgical outcome in FLE. RESULTS: From 70 patients submitted to surgery, 45 patients (64%) had favorable outcome and 37 (47%) became seizure free. Isolated risk factors for poor surgical outcome are expressed in hazard ratio (H.R.) and were time of epilepsy (H.R.=4.2; 95% C.I.=1.5-11.7; p=0.006), ictal EEG recruiting rhythm (H.R.=2.9; 95% C.I.=1.1-7.7; p=0.033); normal MRI (H.R.=4.8; 95% C.I.=1.4-16.6; p=0.012), and MRI with lesion involving eloquent cortex (H.R.=3.8; 95% C.I.=1.2-12.0; p=0.021). Based on these variables and using a logistic regression model we constructed a model that correctly predicted long-term surgical outcome in up to 80% of patients. CONCLUSION: Among independent risk factors for postsurgical seizure outcome, epilepsy duration is a potentially modifiable factor that could impact surgical outcome in FLE. Early diagnosis, presence of an MRI lesion not involving eloquent cortex, and ictal EEG without recruited rhythm independently predicted favorable outcome in this series.
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Epilepsia del Lóbulo Frontal/diagnóstico , Epilepsia del Lóbulo Frontal/cirugía , Adolescente , Adulto , Niño , Preescolar , Electroencefalografía/métodos , Epilepsia del Lóbulo Frontal/fisiopatología , Femenino , Humanos , Lactante , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Pronóstico , Estudios Retrospectivos , Resultado del Tratamiento , Grabación en Video/métodos , Adulto JovenRESUMEN
Different lines of evidence support BDNF as a candidate gene in mood and anxiety modulation. More recently, the Met allele of the BDNF Val66Met polymorphism has been implicated in anxiety in animal models and anxiety-traits in humans. The aim of this study is to evaluate the a priori hypothesis that the association between anxiety disorders and Val66Met polymorphism at the BDNF gene would be replicated in a community sample of children and adolescents. 240 subjects from a total sample of 2457 children and adolescents aged 10-17 years from the public schools in the catchment area of the primary care unit of a university hospital participated in this case-control study and were assessed for psychopathology using the K-SADS-PL. A sample of saliva was collected for DNA analysis of Val66Met polymorphism. BDNF was the single gene evaluated in this sample. We found a significant association between carrying one copy of the Met allele and higher chance of anxiety disorders in children and adolescents. The association remained positive even after the adjustment for potential confounders (228 subjects; OR=3.53 (CI95% 1.77-7.06; p<0.001)). Our results support the a priori hypothesis of an association between anxiety and the polymorphism Val66Met. To our knowledge, this is the first study documenting a potential role of this polymorphism in a community sample of anxious children and adolescents.
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Sustitución de Aminoácidos/genética , Trastornos de Ansiedad/genética , Química Encefálica/genética , Factor Neurotrófico Derivado del Encéfalo/genética , Predisposición Genética a la Enfermedad/genética , Polimorfismo Genético/genética , Adolescente , Trastornos de Ansiedad/metabolismo , Niño , Estudios de Cohortes , Femenino , Pruebas Genéticas/métodos , Humanos , Masculino , Metionina/genética , Valina/genéticaRESUMEN
BACKGROUND: The development of newer diagnostic technologies has reduced the need for invasive electroencephalographic (EEG) studies in identifying the epileptogenic zone, especially in adult patients with mesial temporal lobe epilepsy and hippocampal sclerosis (MTLE-HS). OBJECTIVE: To evaluate ictal single photon emission computed tomography (SPECT) in the evaluation and treatment of patients with MTLE-HS. METHODS: MTLE patients were randomly assigned to those with (SPECT, n = 124) and without ictal SPECT (non-SPECT, n = 116) in an intent-to-treat protocol. Primary end points were the proportion of patients with invasive EEG studies, and those offered surgery. Secondary end points were the length of hospital stay and the proportion of patients with secondarily generalized seizures (SGS) during video-EEG, postsurgical seizure outcome, and hospital cost. RESULTS: The proportion of patients offered surgery was similar in the SPECT (85%) and non-SPECT groups (81%), as well as the proportion that had invasive EEG studies (27% vs 23%). The mean duration of hospital stay was 1 day longer for the SPECT group (P < 0.001). SGS occurred in 51% of the SPECT and 26% of the non-SPECT group (P < 0.001). The cost of the presurgical evaluation was 35% higher for the SPECT compared with the non-SPECT group (P < 0.001). The proportion of patients seizure-free after surgery was similar in the SPECT (59%) compared with non-SPECT group (54%). CONCLUSION: Ictal-SPECT did not add localizing value beyond what was provided by EEG-video telemetry and structural MRI that altered the surgical decision and outcome for MTLE-HS patients. Ictal-SPECT increased hospital stay was associated with increased costs and a higher chance of SGS during video-EEG monitoring. These findings support the notion that a protocol including ictal SPECT is equivalent to one without SPECT in the presurgical evaluation of adult patients with MTLE-HS.
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Epilepsia del Lóbulo Temporal/diagnóstico por imagen , Epilepsia del Lóbulo Temporal/terapia , Hipocampo/diagnóstico por imagen , Tomografía Computarizada de Emisión de Fotón Único , Adulto , Atrofia , Epilepsia del Lóbulo Temporal/patología , Femenino , Hipocampo/patología , Humanos , Masculino , Esclerosis/diagnóstico por imagen , Esclerosis/patologíaRESUMEN
OBJECTIVE: The objective of this retrospective study is to analyze ictal patterns observed during continuous Video-EEG monitoring in patients with temporal lobe epilepsy (TLE) due to unilateral hippocampal sclerosis (HS), and to correlate these EEG patterns to temporal pole abnormalities observed on magnetic resonance imaging exams. METHODS: We analyzed 147 seizures from 35 patients with TLE and unilateral HS. Ictal patterns were classified and correlated to signal abnormalities and volumetric measures of the temporal poles. Volume differences over 10% were considered abnormal. RESULTS: The most frequent type of ictal pattern was rhythmic theta activity (RTA), encountered in 65.5% of the seizures. Rhythmic beta activity (RBA) was observed in 11% of the seizures, localized attenuation in 8%, interruption of epileptiform discharges in 6%, repetitive discharges in 5.5%, and rhythmic delta activity (RDA) in 4%. Sixty-six percent of the patients presented signal abnormalities in the temporal pole that were always ipsilateral to the HS. Sixty percent presented significant asymmetry of the temporal poles consisting of reduced volume that was also always ipsilateral to HS. Although patients with RTA as the predominant ictal pattern tended to present asymmetry of temporal poles (p=0.305), the ictal EEG pattern did not correlate with temporal pole asymmetry or signal abnormalities. CONCLUSIONS: RTA is the most frequent initial ictal pattern in patients with TLE due to unilateral HS. Temporal pole signal changes and volumetric reduction were commonly found in this group of patients, both abnormalities appearing always ipsilateral to the HS. However, neither temporal pole volume reduction nor signal abnormalities correlated with the predominant ictal pattern, suggesting that the temporal poles are not crucially involved in the process of epileptogenesis.
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Electroencefalografía , Epilepsia del Lóbulo Temporal/patología , Hipocampo/patología , Imagen por Resonancia Magnética , Convulsiones/patología , Adulto , Edad de Inicio , Epilepsia del Lóbulo Temporal/fisiopatología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Esclerosis , Convulsiones/fisiopatología , Grabación en VideoRESUMEN
BACKGROUND: Hemispheric brain lesions are commonly associated with early onset of catastrophic epilepsies and multiple seizure types. Hemispheric surgery is indicated for patients with unilateral intractable epilepsy. Although described more than 50 years ago, several new techniques for hemispherectomy have only recently been proposed aiming to reduce operatory risks and morbidity. MATERIALS AND METHODS: We present the clinical characteristics, presurgical workup, and postoperative outcome of a series of pediatric patients who underwent hemispherectomy for medically intractable epileptic seizures. Thirty-nine patients with medically intractable epilepsy underwent surgery from 1996 to 2005. RESULTS AND DISCUSSION: We analyzed demographic data, interictal and ictal EEG findings, age at surgery, surgical technique and complications, and postsurgical seizure outcome. There were 74.4% males. Tonic and focal motor seizures occurred in 30.8 and 20.5% of the patients. Most frequent etiologies were Rasmussen encephalitis (30.8%) and malformation of cortical development (23.1%). Postsurgical outcomes were Engel classes I and II for 61.5% of the patients. In general, 89.5% of the patients exhibited at least a 90% reduction in seizure frequency. All patients had acute worsening of hemiparesis after surgery. Basically, two surgical techniques have been employed, both with similar results, although a trend has been noted toward one of the procedures which produced consistently complete disconnection. Patients with hemispheric brain lesions usually have abnormal neurological development and intractable epilepsy. When video-EEG monitoring and magnetic resonance imaging show unilateral disease, the patient may evolve with a good surgical outcome. We showed that a marked reduction in seizure frequency may be achieved, with acceptable neurological impairments.
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Epilepsia/cirugía , Hemisferectomía/métodos , Adolescente , Factores de Edad , Niño , Preescolar , Electroencefalografía , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Pruebas Neuropsicológicas , Calidad de Vida , Estadísticas no Paramétricas , Resultado del TratamientoRESUMEN
INTRODUCTION: Intraoperative electrocorticography (ECoG) can be performed in cases of temporal lobe epilepsy due to hippocampal sclerosis (TLE-HS). However, its significance and correlation with surgical outcome are still controversial. OBJECTIVES: To analyze the electrophysiological characteristics of temporal lobe structures during ECoG of patients with TLE-HS, with emphasis on the comparison between pre- and post-resection recordings and surgical outcome. PATIENTS AND METHODS: Seventeen patients with refractory TLE-HS submitted to corticoamigdalohipocampectomy were included in the study. Clinical variables included age at the onset, duration of epilepsy and seizure outcome. The post-operative follow-up ranged from 24 to 36 months. According to outcome subjects were divided in two subgroups: (A) individuals free of seizures (Engel 1A), and (B) individuals not-free of seizures (Engel 1B-IV). Four patterns of ECoG findings were identified: isolated discharges; high frequency spikes (HFS); continuous discharges; combination of isolated discharges and HFS. According to predominant topography ECoG was classified as mediobasal, lateral (or neocortical), mediobasal and lateral. RESULTS: The progressive removal of the temporal pole and the hippocampus was associated with significant decrease of neocortical spikes. No correlation between clinical variables and seizure outcome was observed. Patients who only had isolated spikes on intraoperative ECoG presented a statistical trend for excellent surgical control. Patients who presented temporal pole blurring on MRI also had better post-surgical seizure outcome. CONCLUSIONS: This study showed that out of diverse clinical and laboratory variables, only isolated discharges on intraoperative ECoG and temporal pole blurring on MRI predicted excellent post-surgical seizure outcome. However, other studies with larger number of patients are still necessary to confirm these findings.
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Lobectomía Temporal Anterior , Electroencefalografía , Epilepsia del Lóbulo Temporal/fisiopatología , Hipocampo/patología , Lóbulo Temporal/fisiopatología , Adulto , Epilepsia del Lóbulo Temporal/cirugía , Femenino , Humanos , Masculino , Persona de Mediana Edad , Monitoreo Intraoperatorio , Esclerosis , Resultado del TratamientoRESUMEN
PURPOSE: We analyze a series of patients with mesial temporal lobe epilepsy (MTLE) associated with hippocampal sclerosis (HS) submitted to presurgical investigation with scalp sphenoidal, followed by foramen ovale electrodes (FO), and, when necessary, with depth temporal electrodes. We sought to evaluate the clinical utility of FO in patients with MTLE-HS. METHODS: We included patients who had phase I investigation with bitemporal independent seizures, nonlateralized ictal onsets, or ictal onset initiating in the side contralateral to the side of hippocampal sclerosis. Patients whose implanted FO failed to demonstrate an unambiguous unilateral ictal onset were later evaluated with depth hippocampal electrodes. RESULTS: Between May 1994 and December 2004, 64 patients met our inclusion criteria: 33 female (51.5%) and 31 male subjects (48.5%). The mean age at enrollment was 37.66+/-10.6 years (range, 12-56 years). The groups with nonlateralized surface ictal EEG onsets and contralateral EEG onsets had a greater chance of lateralization with FO when compared with the group with bilateral independent seizures on surface EEG (p<0.01). Foramen ovale electrodes lateralized the seizures in 60% of patients. Seventy percent of patients became seizure free after temporal lobectomy. Five patients were implanted with depth temporal electrodes after FO video-EEG monitoring. The depth-electrode EEG onsets confirmed the results of FO video-EEG monitoring in all patients, and the surgery was refused. CONCLUSIONS: In MTLE-HS, FO is a reliable method for lateralization of seizures that are not clearly recorded by surface EEGs.
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Mapeo Encefálico/métodos , Electroencefalografía/métodos , Epilepsia del Lóbulo Temporal/diagnóstico , Tabiques Cardíacos , Lóbulo Temporal/fisiopatología , Adolescente , Adulto , Lobectomía Temporal Anterior , Niño , Electrodos Implantados , Electroencefalografía/estadística & datos numéricos , Epilepsia del Lóbulo Temporal/fisiopatología , Epilepsia del Lóbulo Temporal/cirugía , Femenino , Lateralidad Funcional/fisiología , Hipocampo/fisiopatología , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Monitoreo Fisiológico , Cuidados Preoperatorios , Lóbulo Temporal/cirugía , Resultado del Tratamiento , Grabación de Cinta de VideoRESUMEN
Neocortical development is a highly complex process encompassing cellular proliferation, neuronal migration and cortical organization. At any time this process can be interrupted or modified by genetic or acquired factors causing malformations of cortical development (MCD). Epileptic seizures are the most common type of clinical manifestation, besides developmental delay and focal neurological deficits. Seizures due to MCD are frequently pharmacoresistant, especially those associated to focal cortical dysplasia (FCD). Surgical therapy results have been reported since 1971, however, currently available data from surgical series are still limited, mainly due to small number of patients, distinct selection of candidates and surgical strategies, variable pathological diagnosis and inadequate follow-up. This study addresses the possibilities of seizure relief following resection of focal cortical dysplasia, and the impact of presurgical evaluation, extent of resection and pathological findings on surgical outcome. We included 41 patients, 22 adults and 19 children and adolescents, with medically intractable seizures operated on from 1996 to 2002. All were submitted to standardized presurgical evaluation including high-resolution MRI, Video-EEG monitoring and ictal SPECT. Post-surgical seizure outcome was classified according to Engel's schema. Univariate and multivariate analysis were performed. Fifteen patients had temporal and 26 extratemporal epilepsies. Of the total 26 patients (63.4%) reached seizure-free status post-operatively. There was no correlation between outcome and age at surgery, duration of epilepsy, frequency of seizures, and pathological findings. There was, however, a clear correlation with topography of FCD (temporal versus extratemporal) and regional ictal EEG onset, on univariate as well as multivariate analysis.
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Corteza Cerebral/anomalías , Corteza Cerebral/cirugía , Epilepsia/cirugía , Adolescente , Adulto , Corteza Cerebral/patología , Niño , Electrodos Implantados , Electroencefalografía , Epilepsia/congénito , Humanos , Análisis Multivariante , Complicaciones Posoperatorias , Cuidados Preoperatorios , Pronóstico , Convulsiones/cirugía , Tomografía Computarizada de Emisión de Fotón Único , Resultado del Tratamiento , Grabación en VideoRESUMEN
Cognitive impairment has long been recognized in people with medically refractory epilepsies. Mesial temporal lobe epilepsy related to hippocampal sclerosis (MTLE-HS), the most common surgically remediable epileptic syndrome, has been associated with a cellular prion protein (PrPc) gene (Prnp) variant allele at codon 171. The polymorphism consisting of a methionine-for-valine substitution at codon 129 has been associated with early cognitive deterioration in elderly people and patients with Down syndrome. The same variant allele in homozygosis (V129V) has been associated to a lower long-term memory in healthy humans. PrPc mediates several processes related to neuroplasticity, and its role in cognitive processes remains unknown. In this study, we evaluated the genetic contribution of Prnp alleles to cognitive performance in patients with MTLE-HS. Cognitive performance, measured with 19 neuropsychological tests, of patients with refractory MTLE-HS with the normal Prnp genotypes was compared with that of patients with the variant alleles at codons 129 and 171. With the effects of clinical, demographic, electrophysiological, and neuroimaging variable interactions controlled by multiple linear regression analysis and adjustment for multiple test comparisons, the presence of Prnp variant alleles was found not to be significantly associated to cognitive performance of patients with MTLE-HS. The presence of variant alleles at codons 129 and 171 is not associated to cognitive performance of patients with refractory MTLE-HS.
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Cognición/fisiología , Epilepsia del Lóbulo Temporal/genética , Priones/genética , Lóbulo Temporal/patología , Adulto , Alelos , Codón , ADN/análisis , Epilepsia del Lóbulo Temporal/patología , Epilepsia del Lóbulo Temporal/cirugía , Femenino , Humanos , Modelos Logísticos , Masculino , Pruebas Neuropsicológicas , Polimorfismo GenéticoRESUMEN
Glutamate plays a central role in the fast excitatory synaptic transmission and is a key neurotransmitter involved in several neurophysiological processes. Glutamate levels on the synaptic cleft are related to neural excitability, neuroplasticity, and neuronal damage associated with excitotoxicity. Mice lacking the cellular prion protein (PrP(c)) gene (Prnp) present a decreased astrocytic glutamate uptake in cultures, higher neuronal excitability in vitro and sensitivity to pro-convulsant drugs in vivo, and age-dependent memory impairment. Here, we investigate if PrP(c) might be involved in neuronal uptake and release of glutamate. For this purpose, we compared synaptosomal preparations from the cerebral cortex, entorhinal cortex, hippocampus, cerebellum, and olfactory bulb of 3- or 9-month-old PrP(c) null mice and with respective wild-type controls. Although we observed differences in synaptosomal glutamate release and uptake regarding the age of mice and the brain structure studied, these differences were similar for PrP(c) null mice and their respective wild-type controls. Therefore, despite a possible correlation between neuronal glutamate transporters, excitability, and neuronal damage, our results suggest that PrP(c) expression is not critical for neuronal glutamate transport.
Asunto(s)
Encéfalo/metabolismo , Ácido Glutámico/metabolismo , Priones/metabolismo , Sinaptosomas/metabolismo , Animales , Transporte Biológico , Ratones , Priones/genéticaRESUMEN
PURPOSE: Posterior cortex epilepsies (PCEs) encompass a group of epilepsies originating from the occipital, parietal, or occipital border of the temporal lobe, or from any combination of these regions. When their seizures are refractory to pharmacologic treatment, these patients are usually referred for surgery. The aim of our study was to analyze clinical characteristics of all PCE patients referred for surgery from 1994 to 2003, and to search for predictors of surgical outcome. METHODS: We performed a retrospective analysis of clinical and laboratory data from 81 consecutive refractory PCE patients referred for surgery. Surgical and nonsurgical groups of patients were compared, and detailed analyses of all variables of the surgical cases were performed in the search for predictors of seizure outcome. RESULTS: Risk factors for PCEs included gliosis (34.56%), malformations of cortical development (33.33%), tumors (8.64%), brain trauma (3.70%), Sturge-Weber disease (4.93%), vascular malformations (3.70%), family history of epilepsy (3.70%), history of CNS infections (2.46%), and low IQ (2.46%). Of the 81 patients, 44 were submitted to surgery at the time of the completion of this study. Surgical treatment was highly effective in improving seizures (p<0.001) when compared with previous pharmacologic treatment alone. Twenty-eight (65.11%) patients became seizure free after surgery versus none in the nonsurgical group. Regarding outcome predictors, patients with shorter duration of epilepsy and those without neurologic abnormalities on clinical examination had higher chances of favorable evolution. CONCLUSIONS: Surgical treatment is effective for the treatment of PCEs and superior to pharmacologic therapy alone. In our series, shorter duration of epilepsy and normal neurologic examination were the only independent variables that predicted better surgical outcome.
Asunto(s)
Corteza Cerebral/fisiopatología , Epilepsia/diagnóstico , Adulto , Corteza Cerebral/cirugía , Electroencefalografía/estadística & datos numéricos , Epilepsias Parciales/diagnóstico , Epilepsias Parciales/fisiopatología , Epilepsias Parciales/cirugía , Epilepsia/fisiopatología , Epilepsia/cirugía , Epilepsia del Lóbulo Temporal/diagnóstico , Epilepsia del Lóbulo Temporal/fisiopatología , Epilepsia del Lóbulo Temporal/cirugía , Femenino , Humanos , Imagen por Resonancia Magnética , Masculino , Lóbulo Parietal/fisiopatología , Lóbulo Parietal/cirugía , Pronóstico , Factores de Riesgo , Resultado del Tratamiento , Grabación de Cinta de VideoRESUMEN
One of the objectives of pre-surgical evaluation in mesial epilepsy associated to hippocampal sclerosis is the identification of patients with bad surgical prognosis for seizure ccontrol. At least theoretically, neuropsychological tests could be used in this venue. To evaluate whether verbal and visual memory tests can be used as isoleted predicotrs of the post-surgical sizure outcome in patients wit mesial temporal lobe epilepsy associated with hippocampal sclerosis refractory to pharmacological treatment. In a retrospective cohort study using the control of epileptic seizres as end-point, we evaluated 187 patients and calculated the correlation of clinical variables, cognitive evaluation, neuroimaging data, demographic data and electrophysiological findings with the result of seizure control after lobectomy in these patients. An unfavorable prognosis during the postoperative period was observed only in association with low visual reproduction scores (visual memory). However, afterBonferrroni corrections, which was necessary to reduce the chance of type I error this result was found to be spurious. We conclude that neuropsychological tests of verbal and visual memory such as those used in the routine presurgica evaluation of our patients with temporal lobe epilepsy are not good isolated predictors of surgical outcome
Asunto(s)
Epilepsia del Lóbulo Temporal , Pruebas NeuropsicológicasRESUMEN
Psychosis is commonly observed in patients with mesial temporal lobe epilepsy related to hippocampal sclerosis (MTLE-HS). Interictal single photon emission computed tomography (SPECT) was performed to compare regional cerebral blood flow (rCBF) pattern of MTLE-HS patients with psychosis of epilepsy (POE) comorbidity and MTLE-HS patients without any psychiatric disorders (Control group). For this, 21 patients with POE and 23 Control patients were matched by educational level, clinical, demographic, electrophysiological, and MRI data. SPECT scans were acquired using (99m)Tc and interpreted with a semiquantitative method. We analyzed brain regions of interest (ROI) of frontal, temporal, and parietal cortex, in addition to subcortical structures. There were no significant statistical differences of ROI between the POE group and the Control group after Bonferroni adjustment. However, we observed a trend for rCBF increase of right posterior cingulate in the POE Group. This increase would be in accordance with recent findings of cingulate abnormalities in schizophrenia, suggesting that abnormal function in this region might be associated with the psychotic phenomena.