Epilepsia do lobo temporal com esclerose hipocampal mesial / Temporal lobe epilepsy with mesial hippocampal sclerosis / Epilepsia del lóbulo temporal con esclerosis del hipocampo mesial

Objetivo:Compreender o cenário atual da ELT-HS, caracterizado por sua fisiopatologia, manifestações clínicas, métodos diagnósticos e tratamentos. Método:Trata-se de uma revisão integrativa da literatura, com caráter descritivo, de artigos indexados no Sistema de Análise e Recuperação da Literatura Médica Online MEDLINE/Pubmed, Literatura Latino-Americana e do Caribe em Ciências da Saúde LILACS, e nas bases de dados Científicas Electronic Library Online (SciELO), pesquisados na período compreendido entre outubro de 2022 e março de 2023. Foram incluídos artigos em português e inglês que contemplassem os objetivos da revisão, publicados nos últimos dez anos (2011-2021).Resultados: Inicialmente foram encontrados 144 artigos nas bases de dados, que após a leitura, foramselecionados na pesquisa 40 artigos que correspondiam ao objetivo proposto. Os artigos analisados correspondem aos anos de 2011 a 2021. Conclusão:O tratamento cirúrgico da ELT-HS tem se mostrado eficaz para resolução completa das crises na maioria dos pacientes. O conhecimento sobre sua fisiopatologia, manifestações clínicas, diagnóstico e tratamentos são de fundamental importância para os médicos que atendem pacientes com epilepsia.

Objective: To understand the current scenario of TLE-HS, characterized by its pathophysiology, clinical manifestations, diagnostic methods and treatments. Method:This is an integrative literature review with descriptive character, of articles indexed in the Medical Literature Analysis And Retrieval System Online MEDLINE/Pubmed, Latin American and Caribbean Literature in Health Sciences LILACS, and Scientic databases Electronic Library Online (SciELO), researched in the period between october 2022 and march 2023. Articles in Portuguese and English that contemplated the objectives of the review, published in the last ten years (2011-2021), were included. Results:Initially, 144 articles were found in the databases, which after reading, 40 articles were selected in the research that corresponded to the proposed objective. The articles analyzed are equivalent to the years 2011 to 2021. Conclusion:The surgical treatment of TLE-HS has been shown to be effective for the complete resolution of crises in most patients. Knowledge about its pathophysiology, clinical manifestations, diagnosis and treatments are of fundamental importance for physicians who treat patients with epilepsy

Objetivo: Comprender el escenario actual de la TLE-HS, caracterizado por su fisiopatología, manifestaciones clínicas, métodos diagnósticos y tratamientos. Método: Se trata de una revisión bibliográfica integradora con carácter descriptivo, de artículos indexados en el Sistema de Análisis y Recuperación de Literatura Médica en Línea MEDLINE/Pubmed, Literatura Latinoamericana y del Caribe en Ciencias de la Salud LILACS, y bases de datos Scientic Electronic Library Online (SciELO), investigados en el período comprendido entre octubre de 2022 y marzo de 2023. Se incluyeron artículos en portugués e inglés que contemplaran los objetivos de la revisión, publicados en los últimos diez años (2011-2021). Resultados:Inicialmente se encontraron 144 artículos en las bases de datos, de los cuales luego de la lectura se seleccionaron 40 artículos en la investigación que correspondía al objetivo propuesto. Los artículos analizadoscorresponden a los años 2011 a 2021. Conclusión:El tratamiento quirúrgico del ELT-HS se ha mostrado eficaz para la resolución completa de las crisis en la mayoría de los pacientes. El conocimiento sobre su fisiopatología, manifestaciones clínicas, diagnóstico y tratamientos es de fundamental importancia para los médicos que tratan pacientes con epilepsia

Surgical treatment of temporal lobe epilepsy: comparative results of selective amygdalohippocampectomy versus anterior temporal lobectomy from a referral center in Brazil / Tratamento cirúrgico da epilepsia do lobo temporal: resultados comparativos da amigdalohipocampectomia seletiva versus lobectomia temporal anterior em um centro de referência no Brasil

Abstract Background Temporal lobe epilepsy (TLE) is a high prevalence neurological disorder. Surgery has emerged as a promising treatment. Objective The objective of this work is to compare the surgical results of anterior temporal lobectomy (ATL) versus selective amygdalohippocampectomy (SAH) in a cohort of 132 patients. Methods We performed a retrospective study of 146 patients operated for TLE from 2008 to 2019. Initially, 13 patients were excluded from the study due to insufficient medical record data or follow-up loss. One patient was excluded from the analysis of the results due to death in the first postoperative week. We used the ILAE scale to classify seizure control after surgery. In patients with left hippocampal sclerosis, SAH was performed and in right temporal lobe epilepsy, ATL was the approach of choice. Results The mean follow-up time after surgery was 57.2 months (12-137). In our data analysis, we found that the group of patients undergoing ATL had a higher prevalence of being completely seizure-free (ILAE I) (57.1% versus 31%) and a higher rate of satisfactory seizure control (88.6% versus 69.3%) p = 0,006, when compared with patients undergoing SAH. Conclusions The literature is still controversial about seizure control concerning the technique used due to the lack of a robust methodology. Our data analysis identified the superiority of ATL over SAH in seizure outcomes. ATL may be the best option for adequately controlling seizures with minimal additional morbidity in countries with a cost limitation for extended propaedeutics.

Resumo Antecedentes A epilepsia do lobo temporal (TLE) é uma desordem neurológica de alta prevalência. A cirurgia surgiu como um tratamento promissor. Objetivo O objetivo deste trabalho é comparar os resultados da lobectomia temporal anterior (ATL) versus amigdalohipocampectomia seletiva (SAH) em uma coorte de 132 pacientes. Métodos Realizamos um estudo retrospectivo de 146 pacientes operados por TLE de 2008 a 2019. Inicialmente, 13 pacientes foram excluídos por insuficiência de dados em prontuário ou perda de seguimento. Um paciente foi excluído da análise por óbito na primeira semana de pós-operatório. Usamos a escala ILAE para classificar o controle das crises após a cirurgia. Em pacientes com esclerose hipocampal à esquerda, foi realizada a SAH, e na epilepsia do lobo temporal à direita, a ATL foi a abordagem de escolha. Resultados O tempo médio de seguimento após a cirurgia foi de 57,2 meses (12-137). Em nossa avaliação, encontramos que o grupo de pacientes submetidos à ATL apresentou maior prevalência de ausência total de crises (ILAE I) (57,1% versus 31%) e maior taxa de controle satisfatório da epilepsia (88,6% versus 69,3%) p = 0,006, quando comparado ao grupo submetido à SAH. Resultados A literatura ainda é controversa em relação à redução das crises de acordo com a técnica utilizada devido a falta de uma metodologia robusta. Nosso estudo identificou superioridade da ATL sobre a SAH nos desfechos convulsivos. ATL pode ser a melhor opção para controlar adequadamente as convulsões com morbidade adicional mínima em países com limitação de custo para propedêutica estendida.

Medial temporal lobe epilepsy with hippocampal sclerosis / 国际儿科学杂志

The medial temporal lobe epilepsy with hippocampal sclerosis is one of the most common drug resistant epilepsy,which involves complex neural networks and often requires surgical treatment.Studies have shown that it is related to the history of febrile convulsions,however,the further research is needed.Therefore,it is particularly important to accurately identify hippocampal sclerosis and guide clinical treatment.In this paper,the classification of hippocampal sclerosis was summarized,and the possible mechanism of hippocampal sclerosis was explored in terms of cationic-chloride co-transporters,glutamate and its receptors,and changes in expression of mRNA.The characteristics of magnetic resonance,magnetic resonance spectroscopy and PET of hippocampal sclerosis were listed to provide evidence for early recognition of hippocampal sclerosis.In theory,it provides ideas for further prevention of hippocampal sclerosis and improvement of quality of life in patients with medial temporal lobe epilepsy and hippocampal sclerosis.

Study of Hippocampal Perfusion in Temporal Lobe Epilepsy Patients with MRI Arterial Spin Labeling(ASL) Technique / 医学研究杂志

Objective To perform the main application of MRI arterial spin labeling (ASL) in cerebral blood flow (CBF) on hippocampus of patients with temporal lobe epilepsy for quantitative measurement,to assess the relationship between temporal lobe epilepsy occurrence and hippocampal perfusion and to explore the critical value of hippocampal CBF predict the temporal lobe epilepsy early.Methods Forty-two subjects by video EEG (VEEG) and conventional MR sequences screend were divided into 3 groups eventually,namely group of healthy control,the group with one temporal lobe abnormal discharge,that conventional MRI in hippocampus is not abnormal and the last group with one side hippocampus sclerosis confirmed by conventional MRI in temporal lobe epilepsy patients,then for the hippocampal 3D ASL examination.We provided six same size regions of interest (ROI) in the hippocampal head,body,tail respectively with the best level display to measure and record bilateral hippocampal cerebral blood flow values (CBF values) of every subject,and to explore the relationship between the cerebral blood flow value in the hippocampus and temporal lobe epilepsy.Results Mean cerebral blood flow (CBF) value of each subject's unilateral hippocampus was calculated.The average CBF value of the hippocampus in the healthy control group was 53.82 ± 0.98ml/(100g · min).The average CBF of the hippocampus in epileptic unilateral abnormal discharge group with the ipsilateral and contralateral were 49.12 ± 5.31ml/(100g · min) and 55.99 ± 1.65ml/(100g · min).The average CBF of the hippocampus in epileptic unilateral hippocampal sclerosis group with the ipsilateral and contralateral were 39.57 ± 2.08ml/(100g · min) and 48.06 ± 1.74ml/(100g · min).Then pairwise comparison of the unilateral hippocampal average CBF value among the 3 groups of experimenters in different groups was performed.The results showed that in the eight times,the first six times had statistically significant differences.Comparing between the healthy control group and epilepsy unilateral temporal lobe abnormal discharge group,to calculates the best critical point by the ROC curve,the cut-off value was 46.76.Conclusion Magnetic resonance arterial spin labeling (ASL) technique can evaluate the cerebral blood flow in the hippocampus of patients with temporal lobe epilepsy by quantitative measurement of hippocampal CBF value.The changes of hippocampal perfusion in patients with temporal lobe epilepsy should be preceded by the changes of imaging.ASL quantitative measurement of hippocampal CBF value is helpful for early diagnosis and treatment of temporal lobe epilepsy.

Memoria autobiográfica en pacientes con epilepsia del lóbulo temporal por esclerosis hipocampal / Autobiographical memory in patients with temporal lobe epilepsy by hippocampal sclerosis

Introducción: la epilepsia del lóbulo temporal (ELT) suele cursar con trastornos en el funcionamiento mnésico. Es probable que el paciente con epilepsia tenga dificultades en la memoria autobiográfica, principalmente en los casos donde la etiología es la esclerosis hipocampal (EH). Objetivo: determinar las posibles alteraciones de la memoria autobiográfica en los pacientes con epilepsia del lóbulo temporal por esclerosis hipocampal, en comparación con personas sanas. Material y métodos: la muestra estuvo conformada por 25 pacientes con diagnóstico electroclínico e imagenalógico de ELT por EH y fue contrastada con 15 controles sanos. A los pacientes que previamente habían sido valorados por neurología y neuropsicología, se les suministró la Entrevista de Memoria Autobiográfica para valorar el desempeño en memoria personal semántica e incidentes autobiográficos. Para la comparación de los pacientes frente a los controles en memoria autobiográfica, se utilizó la prueba U de Mann Whitney y se calculó el tamaño del efecto no paramétrico. Resultados: los pacientes presentaron dificultades en el recuerdo de la información personal semántica de los últimos cinco años y marcadas alteraciones en el recuerdo de incidentes autobiográficos relacionados con la niñez, la adultez y la vida reciente. Conclusión: los resultados confirman las dificultades que los pacientes con ELT por EH presentan en la activación de rastros de memoria en el hipocampo para la evocación y re-experimentación detallada de los incidentes autobiográficos de toda su historia personal.

Introduction: The temporal lobe epilepsy (TLE) is usually associated with memory disorders. Patients with epilepsy probably will have difficulties in the autobiographical memory, mainly in cases where the etiology is for hippocampal sclerosis (HS) Objective: Determine the possible impairments of autobiographical memory in patients with temporal lobe epilepsy by hippocampal sclerosis compared to healthy subjects. Materials and methods: The sample was composed by 25 patients diagnosed with TLE by HS using neuroimaging, and where contrasted with a control group composed by 15 healthy subjects. Patients who previously were evaluated by neurology and neuropsychology, performed the autobiographical memory interview to test the semantic personal memory performance and autobiographical incidents. In order to compare autobiographical memory in both groups, the U test from Mann Whitney was used and the size of the no parametric effect was calculated. Results: he patients presented difficulties remembering personal semantic information in the last 5 years and had relevant alterations remembering autobiographical incidents related to childhood, adulthood, and recent life. Conclusion: The results confirmed the difficulties of patients with TLE by HS to activate memories from the hippocampus for evoking and experimenting again detailed autobiographical incidents from their personal history.

Asymmetric Gray Matter Volume Changes Associated with Epilepsy Duration and Seizure Frequency in Temporal-Lobe-Epilepsy Patients with Favorable Surgical Outcome

BACKGROUND AND PURPOSE: This study aimed to estimate the changes in gray matter volume (GMV) and their hemispheric difference in patients with mesial temporal lobe epilepsy (MTLE) using a voxel-based morphometry (VBM) methodology, and to determine whether GMV changes are correlated with clinical features. METHODS: VBM analysis of brain MRI using statistical parametric mapping 8 (SPM8) was performed for 30 left MTLE (LMTLE) and 30 right MTLE (RMTLE) patients and 30 age- and sex-matched healthy controls. We also analyzed the correlations between GMV changes and clinical features of MTLE patients. RESULTS: In SPM8-based analyses, MTLE patients showed significant GMV reductions in the hippocampus ipsilateral to the epileptic focus, bilateral thalamus, and contralateral putamen in LMTLE patients. The GMV reductions were more extensive in the ipsilateral hippocampus, thalamus, caudate, putamen, uncus, insula, inferior temporal gyrus, middle occipital gyrus, cerebellum, and paracentral lobule in RMTLE patients. These patients also exhibited notable reductions of GMV in the contralateral hippocampus, thalamus, caudate, putamen, and inferior frontal gyrus. We observed that GMV reduction was positively correlated with several clinical features (epilepsy duration and seizure frequency in RMTLE, and history of febrile seizure in LMTLE) and negatively correlated with seizure onset age in both the RMTLE and LMTLE groups. CONCLUSIONS: Our study revealed GMV decreases in the hippocampus and extrahippocampal regions. Furthermore, the GMV reduction was more extensive in the RMTLE group than in the LMTLE group, since it included the contralateral hemisphere in the former. This difference in the GMV reduction patterns between LMTLE and RMTLE may be related to a longer epilepsy duration and higher seizure frequency in the latter.

Memoria autobiográfica en epilepsia del lóbulo temporal / Autobiographical memory in temporal lobe epilepsy

La epilepsia del lóbulo temporal (ELT) es un tipo de epilepsia focal que se caracteriza por crisis parciales complejas y secundariamente generalizadas; es una enfermedad de difícil control, es decir que no responde adecuadamente al manejo farmacológico. Otra característica es su comorbilidad con trastornos cognitivos secundarios a la frecuencia e intensidad de las crisis epilépticas, afectándose especialmente los procesos mnésicos. Es probable que el paciente con ELT tenga dificultades en la memoria autobiográfica (MA); sin embargo, la evaluación neuropsicológica que se realiza en este tipo de epilepsia, no refleja las quejas que el paciente refiere acerca de su MA, aspecto que altera su calidad de vida. En el presente artículo se exponen los modelos explicativos de la MA así como los métodos de evaluación más usados para este tipo de memoria y, finalmente, se revisan investigaciones acerca de la MA en pacientes con ELT.

Temporal lobe epilepsy (TLE) is a type of focal epilepsy that is mainly characterized by complex secondarily generated seizures. It is a difficult illness to control because it doesn't respond to pharmacological treatment. Another feature of this type of epilepsy is its comorbidity with cognitive disorders that mainly affect memory and depend on the frequency and intensity of seizures. Patients with TLE normally have problems with autobiographical memory (AM), especially in cases where the etiology is hippocampal sclerosis (HS). However, neuropsychological evaluations of this type of epilepsy do not reflect patient complaints about their autobiographical memory, a factor which affects quality of life. In this paper we present the explanatory models of RAM, set out the most common methods used to assess it, and finally research on AM for patients with TLE is revised.

Longer epilepsy duration and multiple lobe involvement predict worse seizure outcomes for patients with refractory temporal lobe epilepsy associated with neurocysticercosis / Longa duração de epilepsia e envolvimento de múltiplos lobos são fatores preditivos de pior controle das crises convulsivas em pacientes com epilepsia refratária do lobo temporal associada a neurocisticercose

ABSTRACT Objective To investigate the surgical outcomes of temporal lobe epilepsy associated with hippocampal sclerosis (TLE-HS) and neurocysticercosis (NCC). Methods A retrospective investigation of patients with TLE-HS was conducted in a tertiary center. Results Seventy-nine (62.2%), 37 (29.1%), 6 (4.7%), and 5 (3.9%) patients were Engel class I, II, III, and IV, respectively. Fifty-two (71.2%) patients with epilepsy durations ≤ 10 years prior to surgery were seizure-free 1 year after the operation compared to 27 (50.0%) patients with epilepsy durations > 10 years (p = 0.0121). Forty-three (72.9%) patients with three or fewer lobes affected by NCC were seizure-free one year after the operation, and 36 (52.9%) patients with more than three involved lobes were seizure-free after surgery (p = 0.0163). Conclusions Longer epilepsy durations and multiple lobe involvement predicted worse seizure outcomes in TLE-HS plus NCC patients.

RESUMO Objetivo Investigar o resultado cirúrgico da epilepsia do lobo temporal associada à esclerose hipocampal (TLE-HS) e neurocisticercose (NCC). Métodos Estudo retrospectivo realizado em um centro de epilepsia. Resultados Cinqüenta e dois pacientes (71,2%) com 10 anos ou menos de epilepsia antes da cirurgia tornaram-se livres de crises após um ano da operação, enquanto que 27 (50,0%) com mais de dez anos tornaram-se livres de crises após a cirurgia (p = 0,0121). Quarenta e três pacientes (72,9%), com três ou menos lobos afetados pela NCC tornaram-se livres de crises após um ano de operação, enquanto que 36 pacientes (52,9%) com mais de três lobos envolvidos estavam livres de crises após a cirurgia (p = 0,0163). Conclusão A duração mais longa da epilepsia e o envolvimento de múltiplos lobos prevê pior resultado após a cirurgia para TLE-HS mais NCC.

Diagnostic value of biexponential model of DWI in nontypical hippocampal sclerosis of the youth / 实用放射学杂志

Objective To investigate the diagnostic value of biexponential model of DWI in nontypical hippocampal sclerosis (HS) of the youth.Methods DWI with multiple b values were performed on 75 youths with right temporal lobe epilepsy and 20 healthy youths(control group).There were no abnormalities in hippocampus on conventional MRI.11 of 75 patients were proved right HS by surgery and pathology (HS group).Compare the volume of the right hippocampus and their D,D? and f values in HS group and control group.Results There were statistical differences in D and f values between HS group and control group (Z=-3.08,Z=-2.23,P0.05). Conclusion When HS does not have typical appearances on conventional MRI,D and f values can provide help from diffusion and the perfusion of microcirculation aspects.

Psychiatric Symptoms in Temporal Lobe Epilepsy with Left Mesial Hippocampal Sclerosis

A 16-year-old woman was referred to us for depression and persistent suicidal and homicidal ideation. From 2010, the patient visited a neurologist due to recurrent grand mal epilepsy, auditory and visual hallucinations, episodic memory loss, and persistent depression. Upon admission, it was revealed through clinical history taking that she had suffered from chronic bullying from same-sex peers and sexual abuse, twice, from an adult male in the neighborhood when she was 10 years old. A brain magnetic resonance imaging study showed left mesial hippocampal sclerosis. The patient exhibited improvement of her psychiatric symptoms after treatment with a combination of fluoxetine (30 mg) and aripiprazole (10 mg). Children and adolescents with epilepsy experience conflicts in the family, challenges at school, stigma, and psychosocial limitations or deprivations due to their comorbid psychiatric symptoms and hence, psychiatric evaluation and early intervention is important when treating these patients.

Small calcified lesions suggestive of neurocysticercosis are associated with mesial temporal sclerosis / Pequenas lesões calcificadas sugestivas de neurocisticercose e estão associadas com esclerose mesial temporal

Recent studies have suggested a possible relationship between temporal lobe epilepsy with mesial temporal sclerosis (MTS) and neurocysticercosis (NC). We performed a case-control study to evaluate the association of NC and MTS. Method: We randomly selected patients with different epilepsy types, including: MTS, primary generalized epilepsy (PGE) and focal symptomatic epilepsy (FSE). Patients underwent a structured interview, followed by head computed tomography (CT). A neuroradiologist evaluated the scan for presence of calcified lesions suggestive of NC. CT results were matched with patients’ data. Results: More patients in the MTS group displayed calcified lesions suggestive of NC than patients in the other groups (p=0.002). On multivariate analysis, MTS was found to be an independent predictor of one or more calcified NC lesions (p=0.033). Conclusion: After controlling for confounding factors, we found an independent association between NC calcified lesions and MTS. .

Estudos recentes têm sugerido possível relação entre epilepsia do lobo temporal com esclerose mesial temporal (MTS) e neurocisticercose (NC). Conduzimos um estudo caso-controle para avaliar a associação de NC e MTS. Método: Selecionamos randomicamente pacientes com diferentes tipos de epilepsia, incluindo: MTS, epilepsia primariamente generalizada (PGE) e epilepsia focal sintomática (FSE). Pacientes foram submetidos a uma entrevista estruturada, seguida por uma tomografia computadorizada de crânio (CT). Um neuroradiologista avaliou as imagens quanto à presença de lesões calcificadas sugestivas de NC. Resultados das CT foram comparados com os dados dos pacientes. Resultados: Mais pacientes do grupo MTS apresentaram lesões calcificadas sugestivas de NC que de outros grupos (p=0,002). Na análise multivariável, MTS foi um preditor independente de uma ou mais calcificações sugestivas de NC (p=0,033). Conclusão: Após controlar para fatores confusionais, encontramos uma associação independente entre lesões calcificadas de NC e MTS. .

The imageology study of patients with the hippocampal sclerosis-associated medial temporal lobe epilep-sy / 中国神经精神疾病杂志

Objective To explore the incidence and imageological features of patients with the hippocampal sclerosis-associated medial temporal lobe epilepsy. Methods Seventy-eight patients with the medial temporal lobe epi?lepsy were recruited from our hospital during February 2012 to December 2013. Magnetic resonance imaging (MRI) and resonance spectroscopy (MRS) analysis were conducted in patients with with the hippocampal sclerosis-associated medial temporal lobe epilepsy, patients with epilepsy without the medial temporal lobe diseases and healthy controls. Results The incidence of hippocampal sclerosis was 58.97%among patients with medial temporal lobe epilepsy which were significantly higher compared with either healthy control group or patients with epilepsy without the medial tempo?ral lobe diseases. The average hippocampal volume of the medial temporal lobe epilepsy group(2305.68±814.61 mm3、2456.71±743. 60 mm3)was significantly smaller compared with either healthy controls or patients with epilepsy without the medial temporal lobe diseases. MRI revealed increased T2WI signal and hippocampal atrophy in 74.55%of patients with hippocampal sclerosis-associated medial temporal lobe epilepsy. Sclerosis was detected on the left side (52.17%) and bilateral hippocampus (19.57%). MRS showed that NAA/(Cr ± Cho) significantly reduced (0.58± 0.19) in the hip?pocampal sclerosis. Conclusions Hippocampal sclerosis may be the main imaging features of the medial temporal lobe epilepsy which are characterized by the hippocampal atrophy and high T2WI signal.

Hippocampal sclerosis dementia: an amnesic variant of frontotemporal degeneration / Demência com esclerose hipocampal: variante amnéstica da degeneração frontotemporal

OBJECTIVE: To describe characteristics of hippocampal sclerosis dementia. METHODS: Convenience sample of Hippocampal sclerosis dementia (HSD) recruited from the Johns Hopkins University Brain Resource Center. Twenty-four cases with post-mortem pathological diagnosis of hippocampal sclerosis dementia were reviewed for clinical characterization. RESULTS: The cases showed atrophy and neuronal loss localized to the hippocampus, amygdala and entorrhinal cortex. The majority (79.2%) had amnesia at illness onset, and many (54.2%) showed abnormal conduct and psychiatric disorder. Nearly 42% presented with an amnesic state, and 37.5% presented with amnesia plus abnormal conduct and psychiatric disorder. All eventually developed a behavioral or psychiatric disorder. Disorientation, executive dysfunction, aphasia, agnosia and apraxia were uncommon at onset. Alzheimer disease (AD) was the initial clinical diagnosis in 89% and the final clinical diagnosis in 75%. Diagnosis of frontotemporal dementia (FTD) was uncommon (seen in 8%). CONCLUSION: HSD shows pathological characteristics of FTD and clinical features that mimic AD and overlap with FTD. The findings, placed in the context of earlier work, support the proposition that HSD belongs to the FTD family, where it may be identified as an amnesic variant.

OBJETIVO: descrever as características da demência com esclerose hipocampal. MÉTODOS: Uma amostra de conveniência de HSD foi recrutada no Johns Hopkins University Brain Resource Center. Vinte e quatro casos com diagnóstico patológico pós-morte de demência com esclerose hipocampal foram revisados para caracterização clínica, utilizando variáveis obtidas de prontuários médicos. OBJETIVO: descrever as características da demência com esclerose hipocampal.MÉTODOS: Uma amostra de conveniência de HSD foi recrutada no Johns Hopkins University Brain Resource Center. Vinte e quatro casos com diagnóstico patológico pós-morte de demência com esclerose hipocampal foram revisados para caracterização clínica, utilizando variáveis obtidas de prontuários médicos. RESULTADOS: A maioria dos pacientes (79,2%) tinha amnésia no início, e a maioria (54,2%) apresentava comportamento anormal e transtorno psiquiátrico. Aproximadamente 42% apresentavam um estado amnésico e 37,5% amnésia mais conduta anormal e transtorno psiquiátrico. Todos acabaram por desenvolver um distúrbio comportamental ou psiquiátrico. Desorientação, disfunção executiva, afasia, agnosia e apraxia foram incomuns no início. A doença de Alzheimer (AD) foi o diagnóstico clínico inicial em 89% e o diagnóstico clínico final em 75%. A maior parte dos casos mostrou atrofia e perda neuronal localizada no hipocampo, amígadale cortex entorrinal. CONCLUSÃO: HSD apresenta características patológicas de DFT e as características clínicas que mimetizam AD. Os resultados, colocadas no contexto do trabalho anterior, suporta a ideia de que HSD pertence à família FTD, em que pode ser identificada como uma variante amnésica. RESULTADOS: A maioria dos pacientes (79,2%) tinha amnésia no início, e a maioria (54,2%) apresentava comportamento anormal e transtorno psiquiátrico. Aproximadamente 42% apresentavam um estado amnésico e 37,5% amnésia mais conduta anormal e transtorno psiquiátrico. Todos acabaram por desenvolver um distúrbio comportamental ou psiquiátrico. Desorientação, disfunção executiva, afasia, agnosia e apraxia foram incomuns no início. A doença de Alzheimer (AD) foi o diagnóstico clínico inicial em 89% e o diagnóstico clínico final em 75%. A maior parte dos casos mostrou atrofia e perda neuronal localizada no hipocampo, amígadale cortex entorrinal. CONCLUSÃO: HSD apresenta características patológicas de DFT e as características clínicas que mimetizam AD. Os resultados, colocadas no contexto do trabalho anterior, suporta a ideia de que HSD pertence à família FTD, em que pode ser identificada como uma variante amnésica.

Temporal lobe epilepsy with mesial temporal sclerosis: hippocampal neuronal loss as a predictor of surgical outcome / Epilepsia do lobo temporal com esclerose mesial temporal: o padrão da perda neuronal hipocampal como preditor do prognóstico cirúrgico

OBJECTIVE: To analyze retrospectively a series of patients with temporal lobe epilepsy (TLE) and mesial temporal sclerosis (MTS), and the association of patterns of hippocampal sclerosis with clinical data and surgical prognosis. METHOD: Sixty-six patients with medically refractory TLE with unilateral MTS after anterior temporal lobectomy were included. Quantitative neuropathological evaluation was performed on NeuN-stained hippocampal sections. Patient's clinical data and surgical outcome were reviewed. RESULTS: Occurrence of initial precipitating insult (IPI), as well as better postoperative seizure control (i.e. Engel class 1), were associated with classical and severe patterns of hippocampal sclerosis (MTS type 1a and 1b, respectively). CONCLUSION: Quantitative evaluation of hippocampal neuronal loss patterns predicts surgical outcome in patients with TLE-MTS.

OBJETIVO: Analisar retrospectivamente uma série de pacientes com epilepsia do lobo temporal (ELT) e esclerose mesial temporal (EMT), bem como correlacionar os padrões de esclerose hipocampal com os dados clínicos e o prognóstico cirúrgico. MÉTODOS: Foram incluídos neste estudo 66 pacientes com ELT refratária a tratamento medicamentoso e com EMT unilateral submetidos à lobectomia temporal anterior. A análise neuropatológica quantitativa foi realizada em seções hipocampais imunomarcadas com NeuN. Dados clínicos e resultados do acompanhamento pós-cirúrgico foram revisados. RESULTADOS: Ocorrência de evento precipitante inicial e melhor controle de crises após a cirurgia (i.e. classe 1 de Engel) foram associados aos padrões clássico (EMT tipo 1a) e severo (EMT tipo 1b) de esclerose hipocampal. CONCLUSÃO: A análise quantitativa do padrão de perda neuronal do hipocampo é capaz de predizer o prognóstico cirúrgico em pacientes com ELT-EMT.

Analysis of causes of epilepsy in 5572 cases / 中华神经科杂志

Objective To explore the common causes of epilepsy and the etiologic characteristics in different age groups of patients with epilepsy.Methods A retrospective survey was made in 5572 epilepsy patients in Epileptic Center of Guangdong 999 Brain Hospital from January 2003 to December 2009.According to the diagnostic criteria published in 2005 from ILAE,all the diagnoses of 5572 cases were made by epileptic specialists.Based on history,cranial MRI or CT and pathologic data,causes of epilepsy were classified into idiopathic,symptomatic and cryptogenic epilepsy.The cases of symptomatic epilepsy were further arranged into different categories in different age grades,such as head trauma,perinatal injuries,infection in central nervous system, cerebral vascular disease, brain tumor, disorders of cortical development,neurocutaneous syndrome and others.The cases with febrile seizures and family history were collected,and positive ratio of febrile seizures and family history were contrasted in different categories of cases by Kruskal-Wallis test ( nonparametric test ).Results In 5572 cases,66 were idiopathic,2834 symptomatic,2672 cryptogenic,and the ratio of these causes was 1％,51％,48％ respectively.Among 2834 cases of symptomatic epilepsy,822 were head trauma,497 were perinatal injuries,360 were infection in central nervous system,249 were brain tumor,150 were cerebral vascular disease,135 were disorders of cortical development,62 were neurocutaneous syndrome and 559 were others. In brief,head trauma,perinatal injuries,infection in central nervous system,brain tumor and cerebral vascular disease were top 5 causes of symptomatic epilepsy. Hippocampal sclerosis was found in 744 cases in those of eryptogenic epilepsy.The importance of febrile seizures( idiopathic:15.2％ ( 10/66 ),symptomatic:6.5％ ( 185/2834 ),cryptogenic:9.4％ ( 250/2672 ) ; x2 =181.393,P =0.000 ) and family history ( idiopathic:83.3％ ( 55/66 ),symptomatic:1.1％ (31/2834),cryptogenic:0.4％ (12/2672) ; x2 =68.354,P =0.000) was statistically different in different causes of epilepsy.Febrile seizures was the most frequent in cases with hippocampal sclerosis than those with other causes,and family history was the most frequent in neurocutaneous syndrome in symptomatic cases.Perinatal injurics was thc first causc in cases of infancy and childhood,head trauma was the top one in those of juvenile and adulthood,and cerebral vascular disease was the main cause in senile cases. Conclusions In the whole epileptic cases of 5572, 1％ was idiopathic,51％ was symptomatic,and 48％ cryptogenic. The main causes of them were head trauma,perinatal injuries,infection in central nervous system,brain tumor,and cerebral vascular disease.

Corpora amylacea deposition in the hippocampus of patients with mesial temporal lobe epilepsy: A new role for an old gene.

BACKGROUND: Mesial temporal lobe epilepsy (MTLE) is the most common medically refractory epilepsy syndrome in adults, and hippocampal sclerosis (HS) is the most frequently encountered lesion in patients with MTLE. Premature accumulation of corpora amylacea (CoA), which plays an important role in the sequestration of toxic cellular metabolites, is found in the hippocampus of 50–60% of the patients who undergo surgery for medically refractory MTLE-HS. However, the etiopathogenesis and clinical importance of this phenomenon are still uncertain. The ABCB1 gene product P-glycoprotein (P-gp) plays a prominent role as an antiapoptotic factor in addition to its efflux transporter function. ABCB1 polymorphism has been found to be associated with downregulation of P-gp expression. We hypothesized that a similar polymorphism will be found in patients with CoA deposition, as the polymorphism predisposes the hippocampal neuronal and glial cells to seizure-induced excitotoxic damage and CoA formation ensues as a buffer response. MATERIALS AND METHODS: We compared five single nucleotide polymorphisms in the ABCB1 gene Ex06+139C/T (rs1202168), Ex 12 C1236T (rs1128503), Ex 17-76T/A (rs1922242), Ex 21 G2677T/A (rs2032582), Ex26 C3435T (rs1045642) among 46 MTLE-HS patients of south Indian ancestry with and without CoA accumulation. RESULTS: We found that subjects carrying the Ex-76T/A polymorphism (TA genotype) had a five-times higher risk of developing CoA accumulation than subjects without this genotype (Odds ratio 5.0, 95% confidence intervals 1.34-18.55; P = 0.016). CONCLUSION: We speculate that rs1922242 polymorphism results in the downregulation of P-gp function, which predisposes the hippocampal cells to seizure-induced apoptosis, and CoA gets accumulated as a buffer response.

Relationship between fluid-attenuated inversion-recovery (FLAIR) signal intensity and inflammatory mediator's levels in the hippocampus of patients with temporal lobe epilepsy and mesial temporal sclerosis / Correlação da intensidade do sinal em FLAIR e os níveis de mediadores inflamatórios no hipocampo de pacientes com epilepsia do lobo temporal e esclerose mesial temporal

We investigated a relationship between the FLAIR signal found in mesial temporal sclerosis (MTS) and inflammation. Twenty nine patients were selected through clinical and MRI analysis and submitted to cortico-amygdalo-hippocampectomy to seizure control. Glutamate, TNFα, IL1, nitric oxide (NO) levels and immunostaining against IL1β and CD45 was performed. Control tissues (n=10) were obtained after autopsy of patients without neurological disorders. The glutamate was decreased in the temporal lobe epilepsy (TLE) -MTS group (p<0.001), suggesting increased release of this neurotransmitter. The IL1β and TNFα were increased in the hippocampus (p<0.05) demonstrating an active inflammatory process. A positive linear correlation between FLAIR signal and NO and IL1β levels and a negative linear correlation between FLAIR signal and glutamate concentration was found. Lymphocytes infiltrates were present in hippocampi of TLE patients. These data showed an association between hippocampal signal alteration and increased inflammatory markers in TLE-MTS.

Este estudo foi delineado para investigar a presença de relação entre a intensidade de sinal em FLAIR e níveis de citocinas, óxido nítrico (NO) e glutamato no hipocampo de pacientes com epilepsia do lobo temporal refratária, associada com esclerose mesial (TLE-MTS). Vinte e nove pacientes foram selecionados através de análise clínica e de ressonância magnética (RM) que foram submetidos a cortico-amigdalo-hipocampectomia para o controle das crises. Os níveis de glutamato foram avaliados por HPLC, as citocinas TNFα e IL1β por ELISA e os níveis de NO via NO system. Avaliamos também por imuno-histoquímica a expressão de IL1β e CD45 em tecidos controles e com esclerose. Tecido controle foi obtido após autópsia de indivíduos mortos sem disfunções inflamatórias e neurológicas (n=10). A concentração de glutamato se mostrou reduzida no tecido TLE-MTS (p<0,001) sugerindo aumento na liberação desse neurotransmissor. TNFα e IL1β também apresentaram níveis elevados no hipocampo dos pacientes (p<0,05), demonstrando um processo inflamatório crônico. Houve uma correlação linear positiva entre a intensidade do sinal em FLAIR e os níveis de NO e IL1β. Em contraste, uma correlação linear negativa foi encontrada entre a intensidade do sinal em FLAIR e níveis de glutamato no hipocampo com esclerose. Infiltrado linfocitário hipocampal também foi visualizado pela imuno-marcação com CD45 em pacientes com TLE-MTS. Esses dados mostraram uma associação entre alteração de sinal na RM e marcadores inflamatórios em pacientes com TLE-MTS.

Using double inversion recovery sequence for the diagnosis of hippocampal sclerosis at 3.0 T MR / 中华放射学杂志

Objective To investigate the imaging feature of hippocampal sclerosis (HS), and evaluate the diagnostic value of double inversion recovery (DIR) sequence at 3.0 T MR for its diagnosis. Methods Twelve patients with unilateral HS proven by pathology and 12 healthy volunteers were enrolled. All patients received DIR, fluid attenuated inversion recovery (FLAIR) and T2 TSE sequences scans on oblique coronal plane vertical to the hippocampal axis on a 3.0 T MR scanner. Regions of interest (ROI) were set respectively in ipsilateral and contralateral hippocampi hippocampi in patients with HS, and the bilateral hippocampi in healthy volunteneers were placed respectively. Signal to noise ratio (SNR), contrast to noise ratio (CNR), ratio of signal intensity (RSI) and asymmetry index (AI) of each ROI in all hippocampi were calculated and compared among the three sequences. Statistical analysis was performed with one-way ANOVA. Results On DIR images, ipsilateral hippocampal lesions demonstrated extremely high signal intensity. Relative signal intensity of ipsilateral hippocampal lesions, contralateral hippocampi and the hippocampi in control groups healthy volunteneers were 1.50±0.05, 1.26±0.03, 1.18±0.05 (F=172.609,P=0.000), respectively. SNR of ipsilateral hippocampal lesions on DIR, FLAIR and T2 TSE sequences were 84.13±16.62, 50.90±12.38, 63.25±15.46 (F=15.185,P=0.000), respectively. CNR of hippocampus were 13.72±3.73, 6.67±3.02, 7.33±3.65 (F=14.985,P=0.000), respectively.In HS patients, RSI and AI of the ipsilateral hippocampal lesions and contralateral hippocampi among the three sequences did not show statistically significant difference(P=0.078). Conclusions HS manifests extremely high signal intensity on DIR images. On DIR images, the SNR and CNR of HS were higher than those on conventional MR sequences which provide valuable information for the diagnosis of HS.

Hippocampal Atrophy Identified by Volumetric Measurement in Intractable Epilepsy Cases

BACKGROUND: Recurrent seizures result in brain damage, but it is usually gradual, minimal, and difficult to observe by visual inspection of magnetic resonance images (MRIs). It is well known that hippocampal structure is vulnerable to seizure-associated brain damage. We measured the hippocampal volume in patients with epilepsy to evaluate the degree of damage to the hippocampus. METHODS: We recruited 33 patients with epilepsy and 21 healthy subjects from January 2007 to December 2008. We subclassified the patients into two groups: (1) 14 patients with intractable epilepsy and (2) 19 patients with drug-responsive epilepsy. In each group, the volumes of the left and right hippocampus were measured by manual drawing on brain MRIs. We compared the hippocampal volume in intractable epilepsy, drug-responsive epilepsy, and healthy subjects. The compounding effect of hippocampal sclerosis was ruled out by excluding eight patients with hippocampal sclerosis; we then compared the hippocampal volume in the two groups with epilepsy. RESULTS: The volume of the bilateral hippocampus on brain MRIs was smaller in patients with intractable epilepsy than in those with drug-responsive epilepsy and healthy subjects (left, p<0.004; right, p<0.03). After excluding the patients with hippocampal sclerosis by visual inspection, the hippocampal volumes were also found to be smaller in patients with intractable epilepsy than in those with drug-responsive epilepsy (left, p<0.04; right, p<0.05). CONCLUSIONS: While there is no definitive abnormality of the hippocampus on visual inspection of brain MRIs, we determined the degree of hippocampal atrophy and volume loss in patients with intractable epilepsy. Hippocampal volumetry will be helpful for the assessment of brain damage in patients with intractable epilepsy.

Employment and quality of life in mesial temporal lobe epilepsy with hippocampal sclerosis: is there a change after surgical treatment? / Emprego e qualidade de vida na epilepsia do lobo temporal mesial com esclerose do hipocampo: há uma mudança após a cirurgia?

PURPOSE: The aim of this study was to evaluate in patients with mesial temporal lobe epilepsy (MTLE) with hippocampal sclerosis (HS): (1) employment patterns before and three years after epilepsy surgery and their impact in Quality of Life (QOL); (2) demographic and clinical variables associated with employment. METHODS: Data from 58 patients with diagnosis of refractory MTLE with HS who had corticoamygdalo-hippocampectomy were analyzed. The subjects answered to Brazilian validated version of the Epilepsy Surgery Inventory (ESI-55) before, and three years after surgery. In a semi-structured interview, sociodemographic and clinical characteristics were obtained. Changes in employment after surgery were classified in one of the three categories: (i) improvement status: those who were unemployed, no-formal employed, students, housewives and subjects who have never worked to employed category; (ii) unchanged status: no change in occupation; this category included subjects who were employed before and after the surgery, housewives, students, and the group who remained unemployed, receiving ill-health benefits or retired after the surgical treatment; and (iii) worsened status: loss of employment. RESULTS: Employment status did not show any significant change after surgery: in 51(87.9 percent) it remained unchanged, in six (10.3 percent) it improved, and one patient (1.7 percent), who was employed before the surgery, retired after that. In a subgroup of 22 patients employed after surgery, ten (45.5 percent) were seizure-free, seven (31.8 percent) had only rare auras, and five (22.7 percent) had seizures. In the group of improvement, 12 patients (70.5 percent) had no-formal employment and five (29.5 percent) had a formal job before surgery. After three years, 14 (63.6 percent) of 22 subjects were formally employed. Our data suggested that the employability was strongly correlated (p<0.05) with a positive perception of health-related quality of ...

OBJETIVO: Avaliar o estado empregatício e a qualidade de vida (QV) de indivíduos com epilepsia do lobo temporal mesial (ELTM) com esclerose do hipocampo (EH) antes e três anos após a realização de corticoamigdalohipocampectomia e verificar as variáveis demográficas e clínicas associadas ao emprego. METODOLOGIA: 58 pacientes com ELTM com EH submetidos a corticoamigdalohipocampectomia foram avaliados antes e três anos após a cirurgia. Todos responderam ao Epilepsy Surgery Inventory (ES-I55) - versão brasileira como medida da QV, bem como a um breve questionário contendo dados sociodemográficos e clínicos. Alterações na situação de emprego após a cirurgia foram classificadas do seguinte modo: (i) melhora: indivíduos desempregados, com emprego não-formal, estudantes, donas-de-casa e aqueles que nunca haviam trabalhado e que estavam empregados três anos após a cirurgia; (ii) nenhuma mudança: aqueles que não obtiveram modificações em sua situação ocupacional. Esta categoria compreendeu indivíduos que permaneceram empregados, continuaram com atividades domésticas, estudantes, em auxílio doença, aposentados e os que nunca trabalharam; e (iii) piora: perda do emprego. RESULTADOS: A situação de emprego não mudou significativamente após a cirurgia: 51(87.9 por cento) permaneceram com o mesmo estado empregatício anterior à cirurgia, seis (10.3 por cento) tiveram melhora, e um paciente (1.7 por cento), que estava empregado antes da cirurgia, aposentou-se. No subgrupo dos 22 pacientes empregados após três anos, dez (45.5 por cento) estavam livres de crises, sete (31.8 por cento) tinham apenas auras esporádicas e cinco (22.7 por cento) permaneceram com crises. No grupo que obteve melhora, 12 pacientes (70.5 por cento) eram autônomos antes da cirurgia e cinco (29.5 por cento), tinham emprego formal. Na avaliação do terceiro ano após cirurgia, 14 (63.6 por cento) dos 22 indivíduos conseguiram um emprego formal. Nosso estudo verificou que a QV manteve ...