Left-sided epileptiform activity influences language lateralization in right mesial temporal sclerosis.

OBJECTIVE: To investigate the association between left epileptiform activity and language laterality indices (LI) in patients with right mesial temporal sclerosis (MTS). METHODS: Twenty-two patients with right MTS and 22 healthy subjects underwent fMRI scanning while performing a language task. LI was calculated in multiple regions of interest (ROI). Data on the presence of left epileptiform abnormalities were obtained during prolonged video-EEG monitoring. RESULTS: After correction for multiple comparisons, LI was reduced in the middle temporal gyrus in the left interictal epileptiform discharges (IED+) group, compared with the left IED- group (p < 0.05). SIGNIFICANCE: Using a responsive reading naming fMRI paradigm, right MTS patients who presented left temporal interictal epileptiform abnormalities on video-EEG showed decreased LI in the middle temporal gyrus, indicating decreased left middle temporal gyrus activation, increased right middle temporal gyrus activation or a combination of both, demonstrative of language network reorganization, specially in the MTG, in this patient population. PLAIN LANGUAGE SUMMARY: This research studied 22 patients with right mesial temporal sclerosis (a specific type of epilepsy) comparing them to 22 healthy individuals. Participants were asked to perform a language task while undergoing a special brain imaging technique (fMRI). The findings showed that patients with epilepsy displayed a change in the area of the brain typically responsible for language processing. This suggests that their brains may have adapted due to their condition, altering the way language is processed.

Bilateral ictal EEG is associated with better memory outcome after hippocampal sclerosis surgery.

OBJECTIVE: To compare memory outcomes after surgery for unilateral hippocampal sclerosis (HS)-associated epilepsy in patients with unilateral and bilateral ictal electrographic involvement. METHODS: We prospectively evaluated HS patients, aged 18-55 years and IQ ≥70. Left (L) and right (R) surgical groups underwent noninvasive video-EEG monitoring and Wada test. We classified patients as Ipsilateral if ictal EEG was restricted to the HS side, or Bilateral, if at least one seizure onset occurred contralaterally to the HS, or if ictal discharge evolved to the opposite temporal region. Patients who declined surgery served as controls. Memory was evaluated on two occasions with Rey Auditory-Verbal Learning Test and Rey Visual-Design Learning Test. Baseline neuropsychological test scores were compared between groups. Pre- and postoperative scores were compared within each group. Reliable change index Z-scores (RCI) were obtained using controls as references, and compared between surgical groups. RESULTS: We evaluated 64 patients. Patients were classified as: L-Ipsilateral (9), L-Bilateral (15), L-Control (9), R-Ipsilateral (10), R-Bilateral (9), and R-Control (12). On preoperative evaluation, memory performance did not differ among surgical groups. Right HS patients did not present postoperative memory decline. L-Ipsilateral group presented postoperative decline on immediate (P = 0.036) and delayed verbal recall (P = 0.011), while L-Bilateral did not decline. L-Ipsilateral had lower RCI Z-scores, indicating delayed verbal memory decline compared to L-Bilateral (P = 0.012). SIGNIFICANCE: Dominant HS patients with bilateral ictal involvement presented less pronounced postoperative verbal memory decline compared to patients with exclusive ipsilateral ictal activity. Surgery was indicated in these patients regardless of memory impairment on neuropsychological testing, since resection of the left sclerotic hippocampus could result in cessation of contralateral epileptiform activity, and, therefore, improved memory function.

Corpus callosum diffusion abnormalities in refractory epilepsy associated with hippocampal sclerosis.

OBJECTIVES: To detect by diffusion tensor imaging (DTI) the extent of microstructural integrity changes of the corpus callosum (CC) in patients with hippocampal sclerosis (HS) and to evaluate possible association with clinical characteristics. METHODS: Fourty-two patients with temporal lobe epilepsy (TLE) and HS and 30 control subjects were studied with DTI. We grouped patients according to lesion side (left or right) HS. Mean diffusivity (MD), fractional anisotropy (FA), radial (RD) and axial diffusivity (AD) were extracted from five segments in CC midsagittal section obtained by automatic segmentation. CC DTI findings were compared between groups. We also evaluated association of DTI changes and clinical characteristics. RESULTS: HS patients displayed decreased FA and increased MD and RD in the anterior, mid-posterior and posterior CC segments, compared to controls. No differences were observed in AD. Patients reporting febrile seizure as the initial precipitating event presented more intense diffusion changes. No differences were seen comparing left and right HS. Age at epilepsy onset, disease duration and seizure frequency were not associated with DTI findings. CONCLUSIONS: This is one of the largest series of TLE-HS patients evaluating CC white matter fiber integrity by DTI, which allowed us to study how some clinical characteristics, such as seizure frequency, disease duration and lesion side, are related to CC integrity. Occurrence of febrile seizure was the only factor that had significant impact on tract integrity. Diffusion changes were not restricted to the posterior part of the CC; we observed the same changes for the anterior part of the CC. Diffusion changes were characterized by an increase in RD, while the AD remained intact for all regions of the CC.

Contralateral interictal and ictal EEG epileptiform activity accentuate memory impairment in unilateral mesial temporal sclerosis patients.

OBJECTIVE: Memory impairment is a recognized complication of mesial temporal sclerosis (MTS). Epileptiform activity may negatively impact on cognition. We evaluated the impact of contralateral EEG involvement on memory in unilateral MTS (uMTS) patients. METHOD: Retrospective review of 121 right-handed uMTS patients (69 left) evaluated with prolonged video-EEG and verbal and nonverbal memory tests (Rey Auditory Verbal Learning Test and Rey-Osterrieth Complex figure), with additional very delayed trials. Patients were classified according to ictal/interictal EEG findings and MTS side as left or right concordant or discordant. Thirty-nine normal individuals who underwent the same neuropsychological battery served as controls. RESULTS: Demographic, disease, and treatment features did not differ among groups. On the 7-day verbal memory free recall, left discordant performed significantly worse than controls and right concordant, recognized fewer words, and had more recognition errors than all other groups, including left concordant. For nonverbal memory, right discordant performed significantly worse than controls on delayed recall, and attained lower scores than other groups on immediate and 7-day recall, but this difference did not reach statistical significance. Left discordant had higher scores of memory complaints than controls and disclosed a trend toward accentuated memory impairment compared with the other groups over time. CONCLUSIONS: Our results suggest that contralateral electrographic involvement in uMTS was associated with more pronounced memory impairment for verbal material in left discordant patients, and to a lesser extent, for nonverbal material in right discordant patients. Left discordant group also had increased memory complaints. (PsycINFO Database Record

Extratemporal abnormalities in phosphorus magnetic resonance spectroscopy of patients with mesial temporal sclerosis.

OBJECTIVE: We evaluated extratemporal metabolic changes with phosphorus magnetic resonance spectroscopy (31P-MRS) in patients with unilateral mesial temporal sclerosis (MTS). METHOD: 31P-MRS of 33 patients with unilateral MTS was compared with 31 controls. The voxels were selected in the anterior, posterior insula-basal ganglia (AIBG, PIBG) and frontal lobes (FL). Relative values of phosphodiesters- PDE, phosphomonoesters-PME, inorganic phosphate - Pi, phosphocreatine- PCr, total adenosine triphosphate [ATPt = γ- + a- + b-ATP] and the ratios PCr/ATPt, PCr/γ-ATP, PCr/Pi and PME/PDE were obtained. RESULTS: We found energetic abnormalities in the MTS patients compared to the controls with Pi reduction bilaterally in the AIBG and ipsilaterally in the PIBG and the contralateral FL; there was also decreased PCr/γ-ATP in the ipsilateral AIBG and PIBG. Increased ATPT in the contralateral AIBG and increased γ-ATP in the ipsilateral PIBG were detected. CONCLUSION: Widespread energy dysfunction was detected in patients with unilateral MTS.

Extratemporal abnormalities in phosphorus magnetic resonance spectroscopy of patients with mesial temporal sclerosis / Anormalidades extratemporais na espectroscopia de fósforo por ressonância magnética em pacientes com esclerose mesial temporal

ABSTRACT Objective We evaluated extratemporal metabolic changes with phosphorus magnetic resonance spectroscopy (31P-MRS) in patients with unilateral mesial temporal sclerosis (MTS). Method 31P-MRS of 33 patients with unilateral MTS was compared with 31 controls. The voxels were selected in the anterior, posterior insula-basal ganglia (AIBG, PIBG) and frontal lobes (FL). Relative values of phosphodiesters- PDE, phosphomonoesters-PME, inorganic phosphate - Pi, phosphocreatine- PCr, total adenosine triphosphate [ATPt = γ- + a- + b-ATP] and the ratios PCr/ATPt, PCr/γ-ATP, PCr/Pi and PME/PDE were obtained. Results We found energetic abnormalities in the MTS patients compared to the controls with Pi reduction bilaterally in the AIBG and ipsilaterally in the PIBG and the contralateral FL; there was also decreased PCr/γ-ATP in the ipsilateral AIBG and PIBG. Increased ATPT in the contralateral AIBG and increased γ-ATP in the ipsilateral PIBG were detected. Conclusion Widespread energy dysfunction was detected in patients with unilateral MTS.

RESUMO Objetivo Nós avaliamos as alterações metabóblicas através da espectroscopia de fósforo por ressonância magnética (31P-MRS) em pacientes com esclerose mesial temporal (EMT) unilateral. Método 31P-MRS de 33 pacientes com EMT unilateral foram comparadas aos de 31 controles. Foram selecionados os voxels nas regiões insulonuclear anterior e posterior (RINA e RINP) e frontal (RF). Os valores relativos de fosfodiésteres – PDE, fosfomonoésteres- PME, fosfato inorgânico- Pi, fosfocreatina –PCr, adenosina trifosfato total [ATPt = γ- + a- + b-ATP] e as razões PCr/ATPt, PCr/γ-ATP, PCr/Pi e PME/PDE foram obtidas. Resultados Nós encontramos anormalidades em pacientes com EMT em comparação aos controles. Redução de Pi nas RINA bilateralmente, RINP ipsilateral e RF contralateral, redução de PCr/γ-ATP nas RINA e RINP ipsilaterais foram detectadas. Aumentos de ATPT na RINA contralateral e aumento de γ-ATP na RINP ipsilateral também foram encontradas. Conclusão Disfunção energética difusa foi encontrada em pacientes com EMT unilateral.

Contralateral ictal electrographic involvement is associated with decreased memory performance in unilateral mesial temporal sclerosis.

PURPOSE: To evaluate the effect of contralateral electrographic involvement on memory performance (measured by neuropsychological and Wada memory testing) in patients with epilepsy associated with unilateral mesial temporal sclerosis (MTS). METHODS: We studied 51 patients with medically-refractory epilepsy associated with unilateral MTS (27 women, 30/51, left MTS) submitted to prolonged non-invasive video-EEG monitoring and bilateral Wada testing. According to ictal electrographic involvement, patients were classified as: Contralateral ictal involvement, when one or more seizures evolved with rhythmic activity in the temporal region contralateral to the MTS or exclusive ipsilateral ictal involvement if all seizures showed ictal EEG activity exclusively on the MTS side. Wada testing involved a twelve-item memory paradigm. Wada memory asymmetry score was calculated for each patient subtracting the number of recalled items after injection on the lesion side from the number of recalled items after contralateral injection. Expected asymmetry (EA) was considered if Wada memory asymmetry>0, and Symmetrical or Reversed memory asymmetry (S-RA) when ≤ 0. Neuropsychological testing was applied in the 51 patients and in 40 healthy controls. Verbal Memory was evaluated with the Rey Auditory Verbal Learning Test (RAVLT), considering the number of recalled items on immediate recall after the initial five consecutive encoding trials (RAVLT 6), a post-interference delayed (30 min) recall (RAVLT 7), and recall after 7 days. Nonverbal memory was tested with Wechsler Memory Scale-III (WMS-III) Faces subtests 1 e 2. RESULTS: Groups did not differ in demographic, clinical and video-EEG monitoring variables. S-RA was observed more frequently in the group with contralateral ictal involvement (57.2% vs. 27.0%; p: 0.03). Logistic regression analysis considering demographic, clinical, hippocampal volume and video-EEG monitoring variables showed contralateral ictal involvement as the only independent variable associated with S-RA (coefficient=1.32, p=0.029, odds ratio 3.77; 95% CI 1.1-12.47). Additionally, the patient group with contralateral ictal EEG involvement displayed worse verbal and nonverbal memory scores compared to healthy controls. CONCLUSION: In this cohort of unilateral MTS patients, contralateral ictal involvement was associated with decreased memory performance on Wada and on neuropsychological testing.

Gender and age influence in daytime and nighttime seizure occurrence in epilepsy associated with mesial temporal sclerosis.

OBJECTIVES: The aim of this study was to analyze the daytime and nighttime seizure distribution during video-EEG monitoring in patients with epilepsy associated with unilateral mesial temporal sclerosis (MTS) and the role of gender, age, and lesion side on 24-hour seizure distribution. METHODS: We studied 167 consecutive adult (age≥16years) patients with epilepsy associated with unilateral mesial temporal sclerosis that had three or more recorded seizures during continuous video-EEG monitoring with a minimum recording time period of 24h. Seizure onset time was classified according to occurrence in six 4-hour periods. RESULTS: Seven hundred thirty-five seizures were evaluated. We observed two higher seizure occurrence periods: 08:01-12:00 (p=0.001) and 16:01-20:00 (p=0.03). Significantly fewer seizures were observed between 0:01 and 4:00 (p=0.01). Nonuniform seizure distribution was noted in women (p<0.0001), in young patients (less than 45years of age) (p<0.0001), and in both patients with left (p=0.03) and patients with right mesial temporal sclerosis (p=0.008). Men presented uniform seizure occurrence distribution (p=0.15). Women had fewer seizures than expected and fewer seizures than men between 0:01-04:00 (p<0.0001 and p=0.0015, respectively) and 04:01-08:00 (p=0.01 and p=0.03, respectively). Young patients (age<45years) had two seizure occurrence peaks, 08:01-12:00 (p=0.016) and 16:01-20:00 (p=0.004). Middle-aged/old patients (≥45years) had only one seizure occurrence peak, 08:01-12:00 (p=0.012). Young patients had more seizures than middle-aged/old patients between 16:01-20:00 (p=0.04). No differences were noted between left and right MTS. SIGNIFICANCE: We observed two seizure occurrence peaks: morning and late afternoon/evening. We encountered variations in daytime and nighttime seizure distribution according to gender and age, but not according to side of MTS. Future studies are needed to confirm these findings and to unravel the neurobiological substrate underlying daytime and nighttime variations of seizure occurrence in different age groups and between genders.

Autoimmune limbic encephalitis: A manifestation of systemic lupus erythematosus in the central nervous system / Encefalite límbica auto-imune: manifestação do lúpus eritematoso sistémico no sistema nervoso central

Autoimmune limbic encephalitis (ALE) associated with systemic lupus erythematosus (SLE) is a rare entity with few reports in the literature to date. In general, ALE associated with SLE has a satisfactory response to immunosuppressive treatment (RIT), but the pathogenesis of this association is poorly understood and may include an autoimmunity component. We report a case study describing the diagnosis and management of limbic encephalitis in a patient with active Systemic Lupus Erythematosus disease (SLE) and past medical history of cancer (endometrial adenocarcinoma in 2004 and papillary urothelial carcinoma in 2011 with curative treatment), followed over a one-year period. We discuss the possible association between limbic encephalitis and all past neoplastic and immune-mediated conditions of this patient. In this particularly case, autoimmunity was the most relevant factor associated with limbic encephalitis given negative neoplastic screening. Moreover, a good response was observed to immunotherapy, not seen with paraneoplastic limbic encephalitis, which is associated with poor response. In this case, the association of ALE with SLE is possible, since laboratory testing disclosed lupic activity and the patient had involvement of other systems (such as hematologic) during the period. However, the presence of other surface membrane antibodies are possible in the search for alternative etiologies.

Encefalite Límbica Autoimune (EL) associada a lúpus eritematoso sistêmico (LES) é uma entidade rara, com poucos relatos na literatura até o momento. Em geral, EL associada com LES tem uma resposta satisfatória ao tratamento imunossupressor, mas a patogênese desta associação é pouco compreendida e pode incluir um componente de autoimunidade. Descrevemos em um estudo de caso o diagnóstico e o tratamento empregado na encefalite límbica ocorrida no contexto de uma paciente com LES ativo e história pregressa de doenças neoplásicas (adenocarcinoma endometrial em 2004 e carcinoma papilar urotelial em 2011 ambos com o tratamento curativo), a qual foi seguida durante um ano. Discutimos uma possível associação de encefalite límbica e todos os antecedentes neoplásicos e imunomediados desta paciente. Neste caso em particular, a autoimunidade é o fator mais relevante relacionado com a encefalite límbica devido a uma triagem neoplásica negativa. Além disso, houve uma grande resposta com a imunossupressão, o que não é visto na encefalite límbica paraneoplásica, mais relacionada com uma má resposta. Neste caso, a associação de EL com LES é possível, uma vez que testes laboratoriais confirmaram a atividade lúpica, bem como a paciente apresentava envolvimento de outros sistemas (como hematológico) neste interim. No entanto, a presença de outros anticorpos de superfície da membrana é possível em busca de diferentes etiologias.

Folate use in women with epilepsy: predictors of adherence in a specialized tertiary outclinic.

PURPOSE OF THE STUDY: The aims of this study were to determine the percentage of women with epilepsy (WWE) taking folic acid (FA) in an outpatient epilepsy tertiary hospital at São Paulo, Brazil, and to identify the possible predictors of adherence. METHOD: Five hundred seventy-six medical records of patients followed in our service were reviewed. Sixty-six WWE using antiepileptic drugs with age between 16 and 52, sexually active but not in menopausal period, not pregnant, and not sterilized or with vasectomized partners underwent direct interviews with a prestructured questionnaire. Logistic regression with clinical and demographic variables using a stepwise method was performed to identify predictable variables of folate use. RESULTS: We found 42.40% of FA intake. Hormonal contraceptive method use was an independent variable negatively associated with adherence to folate. Additionally, educational level was an independent variable positively associated with adherence to folate in WWE above 35years of age. CONCLUSION: We found low folate adherence. Improved planning pre-conception for WWE should improve rates of effective folate supplementation.

Autoimmune limbic encephalitis: A manifestation of systemic lupus erythematosus in the central nervous system.

Autoimmune limbic encephalitis (ALE) associated with systemic lupus erythematosus (SLE) is a rare entity with few reports in the literature to date. In general, ALE associated with SLE has a satisfactory response to immunosuppressive treatment (RIT), but the pathogenesis of this association is poorly understood and may include an autoimmunity component. We report a case study describing the diagnosis and management of limbic encephalitis in a patient with active Systemic Lupus Erythematosus disease (SLE) and past medical history of cancer (endometrial adenocarcinoma in 2004 and papillary urothelial carcinoma in 2011 with curative treatment), followed over a one-year period. We discuss the possible association between limbic encephalitis and all past neoplastic and immune-mediated conditions of this patient. In this particularly case, autoimmunity was the most relevant factor associated with limbic encephalitis given negative neoplastic screening. Moreover, a good response was observed to immunotherapy, not seen with paraneoplastic limbic encephalitis, which is associated with poor response. In this case, the association of ALE with SLE is possible, since laboratory testing disclosed lupic activity and the patient had involvement of other systems (such as hematologic) during the period. However, the presence of other surface membrane antibodies are possible in the search for alternative etiologies.

Encefalite Límbica Autoimune (EL) associada a lúpus eritematoso sistêmico (LES) é uma entidade rara, com poucos relatos na literatura até o momento. Em geral, EL associada com LES tem uma resposta satisfatória ao tratamento imunossupressor, mas a patogênese desta associação é pouco compreendida e pode incluir um componente de autoimunidade. Descrevemos em um estudo de caso o diagnóstico e o tratamento empregado na encefalite límbica ocorrida no contexto de uma paciente com LES ativo e história pregressa de doenças neoplásicas (adenocarcinoma endometrial em 2004 e carcinoma papilar urotelial em 2011 ambos com o tratamento curativo), a qual foi seguida durante um ano. Discutimos uma possível associação de encefalite límbica e todos os antecedentes neoplásicos e imunomediados desta paciente. Neste caso em particular, a autoimunidade é o fator mais relevante relacionado com a encefalite límbica devido a uma triagem neoplásica negativa. Além disso, houve uma grande resposta com a imunossupressão, o que não é visto na encefalite límbica paraneoplásica, mais relacionada com uma má resposta. Neste caso, a associação de EL com LES é possível, uma vez que testes laboratoriais confirmaram a atividade lúpica, bem como a paciente apresentava envolvimento de outros sistemas (como hematológico) neste interim. No entanto, a presença de outros anticorpos de superfície da membrana é possível em busca de diferentes etiologias.

The intracarotid etomidate Wada test: a 54-patient series.

Hemispheric language dominance and isolated hemispheric memory function evaluation can be undertaken with the intracarotid injection of a general anesthetic agent (Wada test). Amobarbital has been traditionally used as the anesthetic agent, but legal and commercial constraints limit its use. We evaluated the use of etomidate as an alternative agent for the Wada test in a series of 54 consecutive adult patients with mesial temporal sclerosis undergoing presurgical evaluation for epilepsy surgery. Language lateralization and hemispheric memory function evaluation were successfully achieved in all cases. Side effects (somnolence, tremor, and dystonia) were infrequent, minor, and transient and did not require interruption of the procedure. Etomidate appears to be a safe and effective alternative agent to amobarbital in the Wada test. Similarly to the amobarbital Wada test, the ability of the etomidate Wada test to predict postoperative memory decline remains unclear.

Multiple endocrine neoplasia type 1 presenting as refractory epilepsy and polyneuropathy--a case report.

Hypoglycemia is a well recognized cause of acute symptomatic seizures. The fact that hypoglycemia can cause peripheral neuropathy is less appreciated. We describe a case of insulinoma associated peripheral neuropathy. A 17 year-old previously healthy man was referred for investigation of refractory epilepsy. A history of recurrent seizures, slowly progressive weakness of his feet and hands, and weight gain was obtained. Physical examination showed signs of a chronic sensory-motor polyneuropathy. He was diagnosed with insulinoma and primary hyperparathyroidism, characterizing multiple endocrine neoplasia, type 1 syndrome. Cases of insulinoma associated peripheral neuropathy are very rare. The more characteristic clinical picture appears to be distal weakness, worse in the intrinsic hand and feet muscles, and no or mild sensory signs. Peripheral nervous system symptoms may not completely resolve, despite removal of the cause of hyperinsulinism/hypoglycemia and full reversion of central nervous system symptoms. Mechanisms underlying hypoglycemic neuropathy are still poorly understood.

Características epidemiológicas das descargas rolândicas em uma unidade de referência regional

A ERBI é a epilepsia parcial mais frequente na infância, com quadro clínico bem definido, caráter familiar, ausência de lesão neurológica, bom prognóstico e ao EEG presença de descargas rolândicas (DR) e atividade elétrica de fundo normal. As DR, no entanto, podem ocorrer em outras situações clínicas. O estudo se propõe a determinar as características epidemiológicas...The erbium is the most common partial epilepsy in childhood, with well defined clinical, familial character, absence of neurological injury, a good prognosis and the presence of EEG rolandic discharges (RD) and normal background electrical activity. RD, however, can occur in other clinical situations. The study aims to determine the epidemiological characteristics...