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OBJECTIVE: Comorbid epilepsy and psychogenic nonepileptic seizures (PNES) occur in 12-22% of cases and the diagnosis of both simultaneous disorders is challenging. We aimed to identify baseline characteristics that may help distinguish patients with PNES-only from those with comorbid epilepsy. METHODS: We performed a longitudinal cohort study on those patients diagnosed with PNES in our epilepsy monitoring unit (EMU) between May 2001 and February 2011, prospectively followed up until September 2016. Patients were classified into PNES-only, PNESâ¯+â¯possible or probable epilepsy, and PNESâ¯+â¯definite epilepsy based on the clinical, vEEG, and neuroimaging data. Demographic and basal clinical data were obtained from chart review. Multiple regression models were performed to identify significant predictors of PNESâ¯+â¯definite epilepsy, excluding patients with only possible or probable epilepsy for this specific analysis. RESULTS: One-hundred and ninety four patients with PNES-only, 30 with PNESâ¯+â¯possible or probable epilepsy and 47 with PNESâ¯+â¯definite epilepsy were included. 73.8% were female and the mean age at EMU admission was 37.4⯱â¯standard deviation 13.5â¯years. Patients with PNESâ¯+â¯definite epilepsy most likely had never worked, had history of febrile seizures, structural brain lesions, developmental disabilities, and maximum reported seizure duration between 0.5 and 2â¯min. Patients with PNES-only were on fewer anti-seizure medications (ASM), reported more frequently an initial minor head trauma, seizures longer than 10â¯min, and a higher number of neurological and medical illnesses - being migraine (18.1%), other types of headaches (18.5%), and asthma (15.5%) the most prevalent ones. All pâ¯<â¯0.05. On the hierarchical regression analysis, history of febrile seizures, developmental disabilities, brain lesions, longest reported seizure duration between 0.5 and 2â¯min, and lack of neurological comorbidity, remained as significant predictors of PNESâ¯+â¯epilepsy. The model's performance of a 5-fold cross-validation analysis showed an overall accuracy of 84.7% to classify patients correctly. CONCLUSIONS: Some demographic and clinical characteristics may support the presence of comorbid epilepsy in patients with PNES, being unemployment, the presence of brain lesions, developmental disabilities, history of febrile seizures, seizure duration and lack of comorbid headaches the most relevant ones.
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Epilepsia , Convulsiones Febriles , Estudios de Cohortes , Comorbilidad , Electroencefalografía , Femenino , Cefalea , Humanos , Estudios Longitudinales , Masculino , Convulsiones Psicógenas no Epilépticas , Estudios RetrospectivosRESUMEN
OBJECTIVE: Developmental and epileptic encephalopathies (DEEs) are a heterogeneous group of syndromes, including Lennox-Gastaut syndrome (LGS), which are refractory to multiple therapies. Perampanel efficacy has been reported in LGS but further real-world evidence is needed in DEEs. METHODS: A multicenter, retrospective, 1-year observational study in patients with DEEs on adjuvant perampanel treatment was conducted to assess perampanel safety and effectiveness in this type of patients in a real-world setting. Seizure types [focal onset seizures (FOS), generalized tonic-clonic seizures (GTCS), tonic seizures (TS), atonic seizures (AtS), atypical absences (AA), and myoclonic seizures (MS)] and seizure clusters were divided in different frequency groups: daily, weekly, and monthly seizures, and absent or seizure freedom. Patients could have more than one seizure type. For each frequency group, group change and seizure freedom were analyzed. RESULTS: Eighty-seven patients diagnosed with DEEs (45 males) of median age 22 [1-70] years were included. The most frequent DEEs were LGS (35.6%) and Lennox-like syndrome (37.9%). At baseline 20 patients had three to five types of seizures, 36 patients had two types of seizures and 31 patients had one predominant type of seizure. The mean number of seizure types per patient at baseline was 2.12 ± 0.97 which was reduced to 1.62 ± 0.91 at 12 months (p < 0.001). Overall, 51.7% of patients had a significant improvement in at least one seizure type. At baseline, 45 patients had GTCS, 42 FOS, 41 TS, 18 AA, 16 AtS, 11 MS, and 30 seizures clusters. Seizure freedom for each specific type at 12 months was significantly achieved by 35% of patients with GTCS (p < 0.001), 17% (p = 0.016) with TS and 37% with seizure clusters (p < 0.001). Patients achieved seizure freedom from other seizure types but with no statistical significance: 7% FOS-free, 28% AA-free, 6% Ats-free, and 18% MS-free. Regarding changes of group at 12 months, 22% of TS and 19% of FOS improved significantly to a group with lower seizure frequency (p = 0.004 and p = 0.02, respectively). In remaining groups (4% of GTCS, 11% of AA, 18% of Ats, 18% of MS, and 13% of seizure clusters), the improvement was not statistically significant. Twenty-nine patients discontinued perampanel: 18 (21%) due to AEs, 8 (9%) due to lack of efficacy, and 3 (3%) due to seizure worsening. Adverse events, mostly mild or moderate, were reported in 53% of patients, and irritability/mood changes (22%) and somnolence (17%) were the most frequent. CONCLUSION: This is the first large-scale real-world study with perampanel across different seizure types in patients with DEEs. Perampanel was effective, especially in GTCS, TS, and FOS, as well as in seizure clusters. Perampanel was generally well-tolerated without unexpected AEs.
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Epilepsias Mioclónicas , Epilepsia Generalizada , Síndrome de Lennox-Gastaut , Adulto , Anticonvulsivantes , Humanos , Masculino , Nitrilos , Piridonas , Estudios Retrospectivos , Convulsiones , Resultado del Tratamiento , Adulto JovenRESUMEN
OBJECTIVE: To describe the spectrum of neurological complications observed in a hospital-based cohort of COVID-19 patients who required a neurological assessment. METHODS: We conducted an observational, monocentric, prospective study of patients with a COVID-19 diagnosis hospitalized during the 3-month period of the first wave of the COVID-19 pandemic in a tertiary hospital in Madrid (Spain). We describe the neurological diagnoses that arose after the onset of COVID-19 symptoms. These diagnoses could be divided into different groups. RESULTS: Only 71 (2.6%) of 2750 hospitalized patients suffered at least one neurological complication (77 different neurological diagnoses in total) during the timeframe of the study. The most common diagnoses were neuromuscular disorders (33.7%), cerebrovascular diseases (CVDs) (27.3%), acute encephalopathy (19.4%), seizures (7.8%), and miscellanea (11.6%) comprising hiccups, myoclonic tremor, Horner syndrome and transverse myelitis. CVDs and encephalopathy were common in the early phase of the COVID-19 pandemic compared to neuromuscular disorders, which usually appeared later on (p = 0.005). Cerebrospinal fluid severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) polymerase chain reaction was negative in 15/15 samples. The mortality was higher in the CVD group (38.1% vs. 8.9%; p = 0.05). CONCLUSIONS: The prevalence of neurological complications is low in patients hospitalized for COVID-19. Different mechanisms appear to be involved in these complications, and there was no evidence of direct invasion of the nervous system in our cohort. Some of the neurological complications can be classified into early and late neurological complications of COVID-19, as they occurred at different times following the onset of COVID-19 symptoms.
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COVID-19 , Enfermedades del Sistema Nervioso , Neurología , Prueba de COVID-19 , Humanos , Enfermedades del Sistema Nervioso/epidemiología , Pandemias , Estudios Prospectivos , Sistema de Registros , SARS-CoV-2RESUMEN
OBJECTIVE: To identify predictors of Psychogenic NonEpileptic Seizure (PNES) improvement and anti-seizure medication (ASM) discontinuation in patients with PNES only. METHODS: This is a retrospective study of a consecutively enrolled cohort of 271 patients diagnosed with PNES by video-EEG (vEEG) telemetry in our Epilepsy Monitoring Unit (EMU) between May 2000 and February 2010. Patients with any possibility of past or present comorbid epilepsy based on clinical, EEG, and neuroimaging, or less than one year of follow-up after discharge were excluded. RESULTS: A total of 109 subjects were included. The mean age at PNES onset was 33 (range 6-89), mean age at EMU admission was 38.3 (16-89.8), 70.6% were female mean video-EEG length was 6.1â¯days, and the median time of final follow-up 3.3 (CI 1.6-6.4) years. 51/108 patients (47.2%) reported a PNES decrease and 29 (26.8%) experienced PNES resolution. 59/73 (81.9%) subjects on ASM at the time of EMU admission were able to discontinue them by the final visit. On univariate analysis, patients whose PNES frequency improved were significantly younger at time of admission, more likely married or cohabiting, less likely unemployed, less likely to have migraine, and had a higher frequency of PNES. On hierarchical regression analysis, younger age and employment remained significant predictors of PNES improvement and resolution. Patients who achieved ASM discontinuation had significantly more children and subsequent EMU visits, were less likely to have history of minor head trauma immediately preceding PNES onset and structural brain lesions, experienced a greater reduction of ASMs during the EMU admission, and had a greater improvement of their PNES frequency at the final visit (pâ¯≤â¯0.05). On hierarchical regression analysis, higher number of children, absence of structural brain lesions, fewer ASMs at EMU discharge, and improvement of PNES frequency remained significant predictors of ASM discontinuation. CONCLUSION: The outcome of PNES is positively correlated with earlier age of diagnosis in an EMU, especially in patients with better social resources. Furthermore, discontinuation of ASM is more likely if the process is initiated during the EMU stay and in the absence of structural brain lesions.
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Epilepsia , Trastornos Mentales , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Niño , Electroencefalografía , Femenino , Humanos , Persona de Mediana Edad , Estudios Retrospectivos , Convulsiones , Adulto JovenRESUMEN
OBJECTIVE: The Wada test (WT) is increasingly being replaced by functional magnetic resonance imaging (fMRI) to evaluate memory lateralization before temporal lobe epilepsy (TLE) surgery. We aimed to determine, via meta-analysis, agreement between the two tests and identify predictors of disagreement. METHODS: We performed a systematic search for studies comparing WT and fMRI for memory lateralization with individual-patient data. If results were provided as laterality indexes instead of hemispheric lateralization, the cutoff point for memory lateralization was set to the usual ±2 for WT and ±0.20 for fMRI. We also evaluated results at our Epilepsy Center. RESULTS: Seven published series plus our own were included, comprising 124 patients. Wada test was performed by recognizing objects in half of the studies, and scenes, drawings, and words in the rest. All used scenes or pictures encoding for fMRI. Wada test-fMRI agreement across the studies ranged from 21.1 to 100%, averaging 46.8% (95% confidence interval [CI]: 37.6-56.0%). When cases with bilateral memory in either test were excluded, agreement reached 78.7% (95% CI: 67.6-89.8%), and concordance with contralateral TLE foci 86.4% for the WT and 83.0% for fMRI. Higher agreement was associated with using multiple items during WT (pâ¯=â¯0.001) and higher disagreement with presence of a lesion on MRI (pâ¯=â¯0.024). Binary logistic regression confirmed use of multiple items on WT as the strongest predictor of agreement (odds ratio [OR]: 6.95, 95% CI: 1.84-26.22; pâ¯=â¯0.004) and a bilateral result on the WT or fMRI of disagreement (OR: 0.24, 95% CI: 0.07-0.89 and OR: 0.12, 95% CI: 0.03-0.45; pâ¯<â¯0.05). CONCLUSION: Concordance between WT and fMRI for memory lateralization is low in patients with TLE and bilateral memoryl memory distribution or a structural etiology, and it improves with encoding of a varied set of items. Both tests can help to lateralize the TLE foci.
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Epilepsia del Lóbulo Temporal/diagnóstico por imagen , Lateralidad Funcional/fisiología , Imagen por Resonancia Magnética/normas , Memoria/fisiología , Pruebas Neuropsicológicas/normas , Adulto , Epilepsia del Lóbulo Temporal/fisiopatología , Femenino , Humanos , Imagen por Resonancia Magnética/métodos , Masculino , Persona de Mediana Edad , Cuidados Preoperatorios/métodos , Cuidados Preoperatorios/normas , Percepción Visual/fisiologíaRESUMEN
PURPOSE OF REVIEW: The usefulness of the Wada test (WT) predicting memory impairment from temporal lobe epilepsy (TLE) surgery has been debated, and it has progressively been replaced by functional MRI (fMRI). We review the current role of WT and fMRI in the presurgical assessment of TLE, and how novel surgical techniques might improve cognitive outcomes. RECENT FINDINGS: fMRI's ability to predict global amnesia has not been assessed. Although WT can produce false-positive results, it is still indicated in patients at risk for developing global amnesia: those with significant bilateral or contralateral memory deficits. In the current review, WT exhibited no added value, beyond preclinical data, for predicting material-specific memory impairment, whereas fMRI was reliable for either verbal or non-verbal memory decline. Abnormal functional connectivity on resting state fMRI (rs-fMRI) between the posterior cingulate and the hippocampus may be a predictor of postsurgical memory outcomes. Restricted resections to the pathogenic tissue, stereotactic laser, radiosurgery, and SEEG-guided thermos-coagulation were associated with better cognitive outcome. fMRI should be used routinely in the presurgical workup of TLE to predict verbal and/or non-verbal memory decline, whereas WT may be indicated when there is a high risk of postsurgical global amnesia. Rs-fMRI is a promising tool for the presurgical workup of TLE, and more restricted resections are recommended to enhance cognitive outcomes.
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Epilepsia del Lóbulo Temporal/cirugía , Imagen por Resonancia Magnética , Trastornos de la Memoria/diagnóstico , Cuidados Preoperatorios/métodos , Adulto , Femenino , Hipocampo/patología , Humanos , Masculino , Memoria , Pruebas Neuropsicológicas , Lóbulo Temporal/cirugíaRESUMEN
AIM: The aim of the study was to evaluate the effectiveness and tolerability of eslicarbazepine acetate (ESL) when used as monotherapy for 1â¯year or more in routine clinical use in patients with focal seizures in epilepsy clinics in Spain. METHODS: This is a retrospective, observational, noninterventional study. Eligible patients were aged ≥18â¯years, had focal seizures, and started on ESL ≥1â¯year before database closure. Primary endpoint was the following: proportion seizure-free for ≥6â¯months at 1 and 2â¯years. Secondary endpoints included retention on ESL monotherapy at 1 and 2â¯years, seizure frequency change, seizure worsening, and side effects. Other analyses included seizure freedom from baseline to 1 and 2â¯years and outcomes in special populations. RESULTS: Four hundred thirty-five patients were included (127 on first-line monotherapy and 308 converting to ESL monotherapy): median daily dose was 800â¯mg at all time points; 63.2% were seizure-free at 1â¯year, 65.1% at 2â¯years, and 50.3% for the entire follow-up. Mean duration of ESL monotherapy was 66.7â¯months; retention was 88.0% at 1â¯year and 81.9% at 2â¯years. Mean reduction in seizure frequency was 75.5% at last visit. Over the entire follow-up, seizure worsening was seen in 22 patients (5.1%), side effects in 28.0%, considered severe in 1.8%, and leading to discontinuation in 5.7%. Dizziness, hyponatremia (sodium <135â¯mEq/l), and somnolence were the most frequent side effects. Outcomes in special populations (patients aged ≥65â¯years and those with psychiatric history or learning difficulty) were consistent with the overall population. CONCLUSIONS: Patients with focal seizures taking ESL monotherapy had excellent retention, high seizure-free rates, and good tolerability up to 2â¯years.
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Anticonvulsivantes/uso terapéutico , Dibenzazepinas/uso terapéutico , Epilepsia/tratamiento farmacológico , Convulsiones/tratamiento farmacológico , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Anticonvulsivantes/efectos adversos , Dibenzazepinas/efectos adversos , Mareo/inducido químicamente , Femenino , Humanos , Hiponatremia/inducido químicamente , Estudios Longitudinales , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Somnolencia , Adulto JovenRESUMEN
OBJECTIVE: Neuronal ceroid-lipofuscinoses are a heterogeneous group of inherited disorders in which abnormal lipopigments form lysosomal inclusion bodies in neurons. Kufs disease is rare, and clinical symptoms include seizures, progressive cognitive impairment, and myoclonus. Most cases of Kufs disease are autosomal recessive; however, there have been a few case reports of an autosomal dominant form linked to mutations within the DNAJC5 gene. METHODS: We describe a family with Kufs disease in which the proband and three of her four children presented with cognitive impairment, seizures, and myoclonus. RESULTS: Genetic testing of all four children was positive for a c.346_348delCTC(p.L116del) mutation in the DNAJC5 gene. The proband brain had an abundance of neuronal lipofuscin in the cerebral cortex, striatum, amygdala, hippocampus, substantia nigra, and cerebellum. There were no amyloid plaques or neurofibrillary tangles. Immunohistochemistry demonstrated that the cholinergic neurons and cholinergic projection fibers were spared, but there was a profound loss of choline acetyltransferase within the caudate, putamen, and basal forebrain. This suggests a loss of choline acetyltransferase as opposed to a loss of the neurons. CONCLUSIONS: This report describes the clinical history of autosomal dominant Kufs disease, the genetic mutation within the DNAJC5 gene, and the neuropathological findings demonstrating depletion of choline acetyltransferase in the brain.
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Colina O-Acetiltransferasa/metabolismo , Lipofuscinosis Ceroideas Neuronales/genética , Lipofuscinosis Ceroideas Neuronales/metabolismo , Adulto , Corteza Cerebral/patología , Colina O-Acetiltransferasa/genética , Trastornos del Conocimiento/etiología , Trastornos del Conocimiento/genética , Salud de la Familia , Femenino , Proteínas del Choque Térmico HSP40/genética , Humanos , Masculino , Proteínas de la Membrana/genética , Persona de Mediana Edad , Mutación/genética , Mioclonía/etiología , Lipofuscinosis Ceroideas Neuronales/complicaciones , Lipofuscinosis Ceroideas Neuronales/patología , Neuronas/metabolismo , Neuronas/patología , Linaje , Convulsiones/etiologíaRESUMEN
Surgery is the treatment of choice for drug-resistant temporal lobe epilepsy (TLE). However, such surgery frequently causes deficits in language function, especially if performed on the dominant hemisphere. In recent years, the intracarotid amobarbital test (IAT) has been gradually replaced by functional magnetic resonance imaging (fMRI) in the preoperative identification of language areas to estimate the risk of postoperative language decline. In this paper, we review the neural substrates for language processing, how language impairment can result both from TLE itself and from surgical attempts to treat it. Subsequently, we discuss the strengths and limitations of, and current indications for fMRI and IAT during the preoperative workup, both by discussion of the studies that have evaluated them individually and through meta-analysis of data from 31 studies deemed eligible for analysis. Electrocortical stimulation mapping (ESM) is also discussed, as is the usefulness of the novel technique of resting-state fMRI. Finally, surgical techniques designed to avoid or reduce language decline in patients at risk are explored.
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Amobarbital/administración & dosificación , Arteria Carótida Interna/diagnóstico por imagen , Epilepsia del Lóbulo Temporal/diagnóstico por imagen , Lenguaje , Imagen por Resonancia Magnética/métodos , Cuidados Preoperatorios/métodos , Mapeo Encefálico/métodos , Arteria Carótida Interna/efectos de los fármacos , Epilepsia del Lóbulo Temporal/cirugía , Lateralidad Funcional/efectos de los fármacos , Lateralidad Funcional/fisiología , Humanos , Hipnóticos y Sedantes/administración & dosificación , Inyecciones Intraarteriales , Masculino , Lóbulo Temporal/diagnóstico por imagen , Lóbulo Temporal/efectos de los fármacos , Lóbulo Temporal/cirugíaRESUMEN
OBJECTIVES: Epidemiologic evidence supporting antiseizure properties of cannabis is limited and controversial. We determined the prevalence of marijuana use and its perceived effects in patients with and without epilepsy. METHODS: Information was collected over 14months from consecutive adult patients admitted to an epilepsy monitoring unit using a 27-item anonymous questionnaire. Patients with cognitive impairment unable to understand the questions or give informed consent and readmissions were not recruited. Subjects were divided into 4 groups, those with epileptic seizures, those with psychogenic nonepileptic seizures (PNES), those with both epileptic and PNES, and those with other nonepileptic events. Patients with exclusively epileptic seizures were compared with those with exclusively PNES. RESULTS: From 310 patients, 18 undiagnosed cases were excluded leaving a cohort of 292 patients with median age 35 (range: 27-49) years; 57.2% female. Epilepsy was documented in 190 (65.1%), PNES in 64 (21.9%), and both types of seizures in 26 (8.9%). Median duration of seizure disorder was longer (2 [1-9] vs. 13 [5.7-25] years; p<0.001) and seizure frequency lower (daily or weekly in 62.3% vs. 44.9%; p=0.03) in patients with epilepsy compared with those in patients with PNES. Overall, 166 (57%) had tried marijuana, and 36.2% used it over the past year. Utilization was 57.1% in sole epilepsy and 64.1% in sole PNES, but daily use was more likely in epilepsy (59% vs. 33.3%). Estimated mean dose was 1g/day. Marijuana use was associated with tobacco smoking (p<0.001) but not alcohol use. Eight patients used other street drugs. Improvement in seizures was perceived by 84% in those with epilepsy and 72.7% in those with PNES. In the 2 groups, stress was decreased in 84.9% and 88%, sleep improved in 77.3% and 88%, and memory/concentration was better in 32% and 28%, respectively. Antiepileptic drug side effects were decreased in 53.2% of marijuana users. Perceived effect on epileptic seizures correlated with effect on stress (r=0.35, p=0.004). Adverse effects of marijuana were mild and reported in 30.7% but included possible seizure precipitation in 5 patients with epilepsy. SIGNIFICANCE: Patients with uncontrolled epilepsy or nonepileptic events had a high rate of marijuana use with associated perceived improvements in seizure control, stress, sleep, and drug side effects. Stress reduction may contribute to the perceived impact of marijuana on seizures and nonepileptic events in adults.
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Epilepsia/epidemiología , Fumar Marihuana/epidemiología , Adulto , Anticonvulsivantes , Canadá , Comorbilidad , Electroencefalografía , Femenino , Hospitalización , Humanos , Masculino , Persona de Mediana Edad , Monitoreo Fisiológico , Prevalencia , Encuestas y CuestionariosRESUMEN
OBJECTIVE: The aim of this study was to investigate the lateralizing and localizing value of ictal coprolalia and brain areas involved in its production. METHODS: A retrospective search for patients manifesting ictal coprolalia was conducted in our EMU database. Continuous video-EEG recordings were reviewed, and EEG activity before and during coprolalia was analyzed using independent component analysis (ICA) technique and was compared to the seizures without coprolalia among the same patients. RESULTS: Nine patients were evaluated (five women), eight with intracranial video-EEG recordings (icVEEG). Four had frontal or temporal lesions, and five had normal MRIs. Six patients showed impairment in the language functions and five in the frontal executive tasks. Two hundred six seizures were reviewed (60.7% from icVEEG). Ictal coprolalia occurred in 46.6% of them, always associated with limbic auras or automatisms. They arose from the nondominant hemisphere in five patients, dominant hemisphere in three, and independently from the right and left hippocampus-parahippocampus in one. Electroencephalographic activity always involved orbitofrontal and/or mesial temporal regions of the nondominant hemisphere when coprolalia occurred. Independent component analysis of 31 seizures in seven patients showed a higher number of independent components in the nondominant hippocampus-parahippocampus before and during coprolalia and in the dominant lateral temporal region in those seizures without coprolalia (p=0.009). Five patients underwent surgery, and all five had an ILAE class 1 outcome. SIGNIFICANCE: Ictal coprolalia occurs in both males and females with temporal or orbitofrontal epilepsy and has a limited lateralizing value to the nondominant hemisphere but can be triggered by seizures from either hemisphere. It involves activation of the paralimbic temporal-orbitofrontal network.
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Epilepsia/psicología , Convulsiones/psicología , Conducta Social , Adulto , Automatismo , Electroencefalografía , Epilepsia/cirugía , Epilepsia del Lóbulo Frontal/psicología , Epilepsia del Lóbulo Frontal/cirugía , Epilepsia del Lóbulo Temporal/psicología , Epilepsia del Lóbulo Temporal/cirugía , Femenino , Humanos , Masculino , Pruebas Neuropsicológicas , Estudios Retrospectivos , Convulsiones/cirugía , Lóbulo Temporal/fisiopatología , Lóbulo Temporal/cirugía , Resultado del Tratamiento , Adulto JovenRESUMEN
OBJECTIVE: This study investigated early, real-world outcomes with cenobamate (CNB) in a large series of patients with highly drug-resistant epilepsy within a Spanish Expanded Access Program (EAP). METHOD: This was a multicenter, retrospective, observational study in 14 hospitals. Inclusion criteria were age ≥18 years, focal seizures, and EAP authorization. Data were sourced from patient clinical records. Primary effectiveness endpoints included reductions (100%, ≥90%, ≥75%, and ≥50%) or worsening in seizure frequency at 3-, 6-, and 12-month visits and at the last visit. Safety endpoints included rates of adverse events (AEs) and AEs leading to discontinuation. RESULTS: The study included 170 patients. At baseline, median epilepsy duration was 26 years and median number of seizures/month was 11.3. The median number of prior antiseizure medications (ASMs) and concomitant ASMs were 12 and 3, respectively. Mean CNB dosages/day were 176 mg, 200 mg, and 250 mg at 3, 6, and 12 months. Retention rates were 98.2%, 94.5%, and 87% at 3, 6, and 12 months. At last available visit, the rate of seizure freedom was 13.3%; ≥90%, ≥75%, and ≥50% responder rates were 27.9%, 45.5%, and 63%, respectively. There was a significant reduction in the number of seizures per month (mean: 44.6%; median: 66.7%) between baseline and the last visit (P < 0.001). Responses were maintained regardless of the number of prior or concomitant ASMs. The number of concomitant ASMs was reduced in 44.7% of patients. The cumulative percentage of patients with AEs and AEs leading to discontinuation were 68.2% and 3.5% at 3 months, 74.1% and 4.1% at 6 months, and 74.1% and 4.1% at 12 months. The most frequent AEs were somnolence and dizziness. SIGNIFICANCE: In this highly refractory population, CNB showed a high response regardless of prior and concomitant ASMs. AEs were frequent but mostly mild-to-moderate, and few led to discontinuation.
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Anticonvulsivantes , Epilepsia , Humanos , Adolescente , Anticonvulsivantes/efectos adversos , Estudios Retrospectivos , Resultado del Tratamiento , Convulsiones/tratamiento farmacológico , Epilepsia/tratamiento farmacológicoRESUMEN
Surgical treatment of pharmacoresistant temporal lobe epilepsy (TLE) carries risks for language function that can significantly affect the quality of life. Predicting the risks of decline in language functions before surgery is, consequently, just as important as predicting the chances of becoming seizure-free. The intracarotid amobarbital test, generally known as the Wada test (WT), has been traditionally used to determine language lateralization and to estimate their potential decline after surgery. However, the test is invasive and it does not localize the language functions. Therefore, other noninvasive methods have been proposed, of which functional magnetic resonance (fMRI) has the greatest potential. Functional MRI allows localization of language areas. It has good concordance with the WT for language lateralization, and it is of predictive value for postsurgical naming outcomes. Consequently, fMRI has progressively replaced WT for presurgical language evaluation. The objective of this manuscript is to review the most relevant aspects of language functions in TLE and the current role of fMRI and WT in the presurgical evaluation of language. First, we will provide context by revising the language network distribution and the effects of TLE on them. Then, we will assess the functional outcomes following various forms of TLE surgery and measures to reduce postoperative language decline. Finally, we will discuss the current indications for WT and fMRI and the potential usefulness of the resting-state fMRI technique.
RESUMEN
Psychogenic non-epileptic seizures (PNES) are the main differential diagnosis of pharmacorresistant epilepsy. Achieving the certainty in the diagnosis of PNES may be challenging, especially in the 10-22% of cases in which PNES and epilepsy co-exist. This difficulty hampers the management of these patients. Unfortunately, published series with this combined pathology are scarce and small in size. This article presents the dataset of our article "Factors associated with comorbid epilepsy in patients with psychogenic non-epileptic seizures: a large cohort study" (Massot-Tarrús et al. 2022). It is composed by a detailed demographic and clinical data of 271 consecutive patients diagnosed with PNES in our epilepsy monitoring unit (EMU) between May 2001 and February 2011, and followed until September 2016. Based on the clinical, neuroimaging and vEEG findings, 47 of these patients were diagnosed with definite comorbid epilepsy, and 30 with possible or probable comorbid epilepsy. All data was collected retrospectively from chart review. The cohort is depicted by means of demographic variables; age at PNES onset; years with PNES; frequency of PNES; duration of longest PNES seizure; self-reported history of minor head trauma (not associated with an increased risk of epilepsy) immediately preceding the first PNES; history of substance abuse; past or present history of active suicidal ideation; neuropsychological evaluation with the Minnesota Multiphasic Personality Inventory test; number and nature of risk factors for epilepsy; co-morbid degenerative brain disease or other neurological or psychiatric medical conditions; semiology of the seizures and triggers; EEG findings; type of epilepsy; number of past EMU admissions and epilepsy clinic visits and re-referrals; number of Anti-Seizure Medications (ASM) at EMU admission and discharge; and the outcome of the spells and ASM after the EMU discharge. Those ASM prescribed for reasons other than the treatment of the seizures (e.g., psychiatric disorders, migraine, pain syndromes, etc.) were not counted. The presented baseline data can be used in studies evaluating the characteristics of patients with PNES and comorbid epilepsy, and in the creation of algorithms to identify them. It could facilitate the prioritization of this subgroup of patients for prolonged video-EEG monitorization to confirm the co-existence of both types of seizures and treat them accordingly.
RESUMEN
Palatal tremor (PT) is a rare movement disorder that involves pharynx, tongue, and other facial muscles. Symptomatic PT is due to lesions on the dentate-rubro-olivary pathways. We present an illustrative case of PT due to degenerative olivary hypertrophy after ependymoma surgery.
Asunto(s)
Ependimoma/cirugía , Músculos Faciales/fisiopatología , Neoplasias Infratentoriales/cirugía , Núcleo Olivar/fisiopatología , Músculos Palatinos/fisiopatología , Músculos Faríngeos/fisiopatología , Complicaciones Posoperatorias/fisiopatología , Temblor/fisiopatología , Adulto , Atrofia/diagnóstico por imagen , Atrofia/fisiopatología , Ependimoma/diagnóstico por imagen , Humanos , Hipertrofia/diagnóstico por imagen , Hipertrofia/fisiopatología , Neoplasias Infratentoriales/diagnóstico por imagen , Imagen por Resonancia Magnética , Masculino , Núcleo Olivar/diagnóstico por imagen , Núcleo Olivar/patología , Complicaciones Posoperatorias/diagnóstico por imagen , Temblor/diagnóstico por imagenRESUMEN
BACKGROUND: Temporal lobe epilepsy (TLE) with unclear lateralization may require intracranial implantation of electrodes (IIE). We retrospectively assessed the association between the use of IIE and long-term outcomes in patients undergoing anterior temporal lobectomy (ATL). PARTICIPANTS AND METHODS: We retrospectively reviewed the records of 1,032 patients undergoing epilepsy surgery at our center from 1977 to 2006. Patients who underwent ATL were included. Seizure outcome was assessed through final follow-up. Those who underwent scalp and IIE (mostly evaluated with temporal subdural strip electrodes) were compared. RESULTS: From 497 patients who underwent ATL, 139 did so after IIE placement in the temporal lobes. Mean age at surgery was 32.3±12.3years and median duration of follow-up 24 months (range: 6-36). Fifty-three percent of those evaluated with IIE were seizure-free at their last available visit (vs. 68% evaluated with only scalp EEG, p=0.002). Patients with lesional TLE generally had a better outcome (65.5% seizure free) than those without lesions (56.3%, p=0.093), especially for unilateral TLE diagnosed with IIE. In a multivariate Cox regression analyses adjusted for gender, neuropsychological concordance, pathological findings, and post-operative seizures, bilateral TLE predicted seizure recurrence in IIE patients (HR=2.08, 95% CI: 1.08-4.0, p=0.029). CONCLUSIONS: More than a half of those who undergo IIE in suspected TLE are seizure free after ATL. IIE allows for the identification of surgical candidates.