Psychogenic nonepileptic seizures in Latin America: A survey describing current practices.

OBJECTIVE: Psychogenic nonepileptic seizures (PNES) are one of the most common differential diagnoses of epilepsy. This study provides an overview of diagnostic and treatment services for patients with PNES across Latin America. METHODS: In 2017-2018, clinicians practicing in Latin America with responsibilities for patients with PNES were contacted to respond to a survey regarding the management of this disorder developed by the International League Against Epilepsy (ILAE) PNES Task Force. RESULTS: Three hundred and sixty responses from 17 Latin American countries were analyzed. Most respondents were neurologists (81%) under 40 years of age (61%). Fifty-seven percent of professionals stated that they personally diagnose PNES, but only 33% stated that they provide follow-up, and only 20% that they recommend treatment. Many participants (54%) characterized themselves as either unfamiliar with the diagnosis or inexperienced in arranging treatment. Most respondents reported having access to brain magnetic resonance imaging (MRI; 88%) and routine electroencephalogram (EEG; 71%), 64% have the access to video-EEG longer than 8 h, and 54% of professionals performed video-EEG to confirm PNES diagnoses. Although cognitive-behavioral therapy was recognized as the treatment of choice (by 82% of respondents), there was little access to it (60%). In contrast, a high proportion of respondents reported using antidepressant (67%), antiseizure (57%), and antipsychotic medications (54%) as treatments for PNES. SIGNIFICANCE: This study reveals several deficiencies in the diagnosis and treatment of patients with PNES in Latin America. The barriers are reinforced by lack of knowledge among the specialists and poor healthcare system support. There is inadequate access to prolonged video-EEG and psychotherapy. An inappropriate use of antiseizure medicines seems commonplace, and there are low follow-up rates by neurologists after the diagnosis. Multidisciplinary guidelines are required to improve the approach of patients with PNES.

Forced normalization: A systematic review.

OBJECTIVE: Forced normalization (FN) is an intriguing phenomenon characterized by the emergence of psychiatric disturbances following the establishment of seizure control or reduction in the epileptic activity in a patient with previous uncontrolled epilepsy. We aim to describe the clinical characteristics of the condition. METHODS: We conducted a systematic review on MEDLINE, EMBASE, Cochrane, and Scielo from January 1953 to January 2018. Clinical, electrographic, and imaging data were gathered. We considered all outcomes in children and adults. We performed no meta-analyses due to the limited available data. RESULTS: Of 2606 abstracts identified, 36 fulfilled the FN diagnostic criteria; 193 FN episodes were evaluated and 77 of them were analyzed extensively. Sixty percent of cases were female. Mean age ± standard deviation (SD) was 28.3 ± 14.2 years. The majority of patients had focal (80%) symptomatic (44%) epilepsy. Most patients reported a high ictal frequency (58%) and were on polytherapy (51%). Patients presented psychosis (86.4%), mood disorders (25.8%), and dissociation (4.5%) as the main manifestations. In the psychosis group, persecutory (52.6%) and reference (47.3%) delusions were frequent. FN was provoked by an antiepileptic drug (AED) (48.5%) mainly levetiracetam, epilepsy surgery (31.8%), or vagus nerve stimulation (13.6%). Treatment was homogeneous including anticonvulsant withdrawal (47%) or taper (25%); antipsychotics were initiated in the majority of cases (73%). Psychiatric symptoms were partially controlled in 35%, with complete resolution of symptoms in the remaining 65% of cases. The majority of patients (87%) with AED trigger and withdrawal presented complete resolution of symptoms in comparison to 28.5% of patients triggered by surgery. SIGNIFICANCE: Forced normalization is an entity whose pathophysiology remains uncertain. Antipsychotic drug use does not predict complete resolution of psychiatric symptoms in comparison with AED withdrawal. Although there is a positive response to treatment in patients with FN triggered by drugs, the prognosis is obscure in patients with surgery triggered FN.

Epilepsy, Physical Activity and Sports: A Narrative Review.

People with epilepsy (PWE) are less physically active compared with the general population. Explanations include prejudice, overprotection, unawareness, stigma, fear of seizure induction and lack of knowledge of health professionals. At present, there is no consensus on the role of exercise in epilepsy. This paper reviews the current evidence surrounding the risks and benefits associated with physical activity (PA) in this group of patients. In the last decade, several publications indicate significant benefits in physiological and psychological health parameters, including mood and cognition, physical conditioning, social interaction, quality of life, as well as potential prevention of seizure presentation. Moreover, experimental studies suggest that PA provides mechanisms of neuronal protection, related to biochemical and structural changes including release of ß-endorphins and steroids, which may exert an inhibitory effect on the occurrence of abnormal electrical activity. Epileptic discharges can decrease or disappear during exercise, which may translate into reduced seizure recurrence. In some patients, exercise may precipitate seizures. Available evidence suggests that PA should be encouraged in PWE in order to promote wellbeing and quality of life. There is a need for prospective randomized controlled studies that provide stronger clinical evidence before definitive recommendations can be made.

Percepciones y actitudes de los pacientes frente a la cirugía de la epilepsia: conceptos equivocados en Colombia / Patients' perceptions of and attitudes towards epilepsy surgery: mistaken concepts in Colombia

Introducción. Los pacientes con epilepsia focal farmacorresistente se podrían beneficiar de la cirugía de la epilepsia; sin embargo, existen demoras en la realización del procedimiento. El objetivo de este estudio fue evaluar el conocimiento y las actitudes de los pacientes hacia la cirugía de epilepsia e identificar barreras que pudieran retrasar el tratamiento. Pacientes y métodos. Se aplicó un cuestionario de 10 minutos a pacientes con epilepsia en Colombia. La encuesta evaluó el conocimiento de la opción quirúrgica, las percepciones sobre el riesgo de la cirugía frente al riesgo de crisis no controladas, la discapacidad producida por la enfermedad, las metas del tratamiento y las variables demográficas y socioeconómicas. Resultados. Se seleccionaron 88 pacientes con epilepsia focal. El 56% de los pacientes no sabía que la cirugía podría ser una opción terapéutica. El 60% consideró que la cirugía de la epilepsia es muy o moderadamente peligrosa. Una gran proporción pensaba que la muerte (41%), el ictus (47%), la pérdida visual (56%), los cambios en la personalidad (56%), la parálisis (61%), las dificultades para hablar (69%) y la pérdida de la memoria (60%) eran efectos secundarios comunes. La mayoría (62%) consideraba el procedimiento como la última opción de tratamiento. Conclusiones. Existe una actitud negativa por parte de los pacientes frente a la cirugía de la epilepsia fundamentada en la sobreestimación del riesgo de adquirir déficits neurológicos secundarios al procedimiento, lo que refleja la falta de conocimiento hacia este tratamiento. Estas percepciones erróneas pueden contribuir a demoras en la atención quirúrgica

Introduction. Selected patients with drug-resistant focal epilepsy benefit from epilepsy surgery, however significant delays remain. The aim of this study was to assess knowledge and attitudes toward epilepsy surgery among patients with epilepsy and identify barriers that might delay the treatment. Patients and methods. A 10-minute questionnaire was administered to patients with epilepsy in Colombia. Survey assessed the following: knowledge of surgical options, perceptions about the risks of surgery vs. ongoing seizures, disease disability, treatment goals, and demographic and socioeconomic variables. Results. We recruited 88 patients with focal epilepsy. More than half of patients (56%) were not aware that surgery might be an option. Apprehension about epilepsy surgery was evident, 60% of patients perceived epilepsy surgery to be very or moderately dangerous. A large proportion of patients believe death (41%), stroke (47%), vision loss (56%), personality change (56%), paralysis (62%), difficulties in speaking (69%), and memory loss (60%) were frequent side effects. The majority of patients (62%) consider the surgical procedure as the last option of treatment. Conclusions. There is a negative attitude toward epilepsy surgery based on the patients' misperceptions of suffering neurological deficits during the surgery, reflecting lack of knowledge toward this type of treatment. These perceptions can contribute to delays in surgical care

Periictal sign of the cross or Signum Crucis as a lateralizing sign in focal epilepsies: Not only a right temporal lobe epilepsy feature.

INTRODUCTION: The sign of the cross (SC) is a catholic ritual that has been described as an automatism during the ictal phase in patients with right temporal lobe epilepsy. OBJECTIVE: The study aimed to describe the prevalence of the SC and analyze the characteristics of patients who presented this phenomenon during the video-electroencephalography (VEEG) admission in our Epilepsy department. METHODS: This is a retrospective analysis of 1308 recorded seizures; 14 patients presented the SC during the admission. Seizure semiology, electroencephalography (EEG), etiology, neuroimaging, and surgical findings were analyzed. RESULTS: A prevalence of 1.1% was found, and the sign was not only an ictal finding (21% was postictal) but also exclusive of patients with temporal lobe epilepsy (15% were extratemporal) in contrast to what has been reported so far. The localizing and lateralizing value of the ictal SC was low (sensitivity 75%, specificity 33.3%, positive predictive value 60%, negative predictive value 50% for a right temporal epileptogenic zone (EZ)) compared with other previously described signs. Regardless of the lateralization of the EZ, the sign was always performed with the right hand supporting the hypothesis of a possible learned behavioral automatism. CONCLUSION: The SC is a rare ictal or postictal manifestation that occurs in patients with temporal and extratemporal epilepsies without clear localizing and lateralizing value compared with previously described signs.

Barreras de acceso a la cirugía de la epilepsia: revisión de la bibliografía / Barriers impeding access to epilepsy surgery: a review of the literature

La epilepsia farmacorresistente es una condición crónica con consecuencias a largo plazo que puede ser tratada quirúrgicamente. La eficacia y la seguridad de la cirugía de la epilepsia del lóbulo temporal se han establecido a través de un gran número de estudios de cohorte retrospectivos y prospectivos y dos ensayos clínicos controlados aleatorizados. A pesar de los excelentes resultados comunicados con la cirugía, la bibliografía sugiere que este procedimiento es un tratamiento subutilizado. Aunque no existe evidencia de esto, entre algunos de los motivos descritos se apuntan el fallo de los médicos de atención primaria y los neurólogos en proveer información, identificar y remitir a los pacientes a un centro de cirugía; los diferentes niveles de tecnología en los centros, lo que provoca distintas estrategias de selección de los candidatos; la creencia de que la cirugía de la epilepsia es un procedimiento arriesgado que debería contemplarse sólo como última opción; la preferencia del paciente por evitar la cirugía; el deseo de los padres de esperar hasta que sus hijos sean lo suficientemente mayores para participar en el proceso de toma de decisiones; el hecho de que las aseguradoras no cubran los gastos asociados con las evaluaciones prequirúrgicas o la carencia de un seguro médico, y la desigualdad racial y social, entre otros. En este artículo se revisan los datos epidemiológicos disponibles en relación con la falta de acceso a la cirugía de la epilepsia (AU)

Drug-resistant epilepsy, a chronic condition with long-term consequences can be treated with surgery. The efficacy and safety of surgery for temporal lobe epilepsy have been established through a large number of retrospective and prospective cohort studies and two randomized controlled clinical trials. Despite the excellent outcomes reported after surgery, the literature suggests that this procedure is an underutilized treatment. While evidence is lacking as to why epilepsy surgery is underused, cited reasons include: failure of primary care physicians and neurologists to provide information and identify patients who could be referred for surgery; different levels of technology at various centers, resulting in different candidate selection strategies; the belief that epilepsy surgery is a risky procedure and that it should be only viewed as the last option; patient preference to avoid surgery; parents wanting to wait until their child is old enough to participate in the decision-making process regarding surgery; unwillingness of insurers to cover the expenses associated with presurgical evaluations or lack of insurance; racial and social disparities, among others. In this paper we review the available epidemiological data about lack of utilization of epilepsy surgery (AU)

Unilateral predominance of abnormal movements: A characteristic feature of the pediatric anti-NMDA receptor encephalitis?

Anti-NMDA receptor encephalitis is a treatable autoimmune disease characterized by cognitive, motor and psychiatric features that primarily affects young adults and children. We present a case of a 7-year-old boy with asymmetrical (mainly right hemibody) and abnormal polymorphic movements without concomitant scalpictal EEG changes but had background slowing predominating over the left hemisphere. This report illustrates previous descriptions of asymmetric presentation of abnormal movements in pediatric anti-NMDA receptor encephalitis and emphasizes the importance of video-EEG interpreted within the overall clinical context, to differentiate epileptic from non-epileptic abnormal movements in patients with autoimmune encephalitis.

Temporal plus epilepsy: Anatomo-electroclinical subtypes.

BACKGROUND: Mesial temporal lobe epilepsy (TLE) is a remediable epileptic syndrome. About 40% of patients continue to have seizures after standard temporal lobectomy. It has been suggested that some of these patients could actually suffer from a more complex epileptogenic network. Because a few papers have been dedicated to this topic, we decided to write an article updating this theme. METHODS: We performed a literature search using the following terminology: "temporal plus epilepsy and networks," "temporal plus epilepsy," "orbito-temporal epilepsy," "temporo-insular epilepsy," "temporo-parieto-occipital (TPO) epilepsy," "parieto-temporal epilepsy," "intracortical evoked potential and temporal plus epilepsy," "temporal lobe connectivity and epilepsy," "intracortical evoked potential and epilepsy surgery," "role of extratemporal structures in TLE," "surgical failure after temporal lobectomy," "Diffusion tensor imaging (DTI) and temporal epilepsy," and "positron emission tomography (PET) in temporal plus lobe epilepsy" in the existing PubMed databases. We searched only English and Spanish literature. Only papers that fit with the above-mentioned descriptors were included as part of the evidence. Other articles were used to reference some aspects of the temporal plus epilepsy. RESULTS: A total of 48 papers from 2334 were revised. The most frequently reported auras in these groups of patients are gustatory hallucinations, vestibular illusions, laryngeal and throat constriction, atypical distribution of somatosensory symptoms (perioral and hands, bilaterally hands paresthesias, trunk and other). The most common signs are tonic posturing, hemifacial twist, and frequent bilateral clonic movements. Interictal electroencephalographic (EEG) patterns exhibit regional and frequently bilateral spikes and/or slow waves. The first ictal electrographic change is mostly regional. It is important to note that the evidence is supported by case series or case reports. Thus, most of the data presented could represent the features on these cases and not actually the totality of the iceberg. CONCLUSION: Temporal plus epilepsy is a diagnosis that can be done only after the invasive recordings have been analyzed but an adequate suspicion may arise based on clinical, EEG and imaging data.

Mortality in patients with refractory temporal lobe epilepsy at a tertiary center in Cuba.

OBJECTIVES: We aimed to investigate the prevalence and risk of mortality in patients with refractory temporal lobe epilepsy. METHODS: Eligible patients included all adults referred to the National Institute of Neurology (NIN) in Havana, Cuba. All patients were followed up for 9 years. All analyses were made with the data available at the last follow-up. The frequency of death related to refractory TLE was analyzed taking into account the total number of patients included in the study. We analyzed the causes of death for each case. Multivariate analysis was made to determine the specific variables related to the death. All values were statistically significant if p<0.05. RESULTS: Six out of 117 patients died during follow-up. Fifty percent of patients died because of suicide. Only the presence of aura, specifically experiential psychic auras, and prodromal depressive disorders were associated significantly with the deaths (p<0.05). Patients who died had a higher concern about their seizures than patients who were still alive at last follow-up (p<0.01); they also had a poor perception of the overall QOL (p<0.01); and they were more concerned about the possible medication side effects than patients who did not die (p<0.05). Logistic regression provided only one variable related to the deaths in our cohort in multivariate analysis: presence of prodromal depressive disorder. CONCLUSION: The causes of death in patients with refractory temporal lobe epilepsy were similar to those documented in the general population of patients with epilepsy.

Efficacy and tolerability of add-on Lacosamide treatment in adults with Lennox-Gastaut syndrome: An observational study.

PURPOSE: To evaluate the efficacy, safety, and tolerability of lacosamide in adults with LGS in the clinical setting. METHOD: The present report is a retrospective, open-label treatment study carried out from June 2013 to December 2014 at the National Institute of Colombia. Lacosamide was introduced as add-on therapy. All caregivers were instructed to initiate lacosamide at low doses (25-50 mg) and gradually increasing it every 2 weeks. The efficacy was evaluated based on the reduction in the rate of each countable type of seizure. We also evaluated the retention rate for lacosamide as the number of days with lacosamide during follow-up. The tolerability was evaluated base on account the adverse events. RESULTS: We found that lacosamide only improves the seizure rate in three out of 19 patients with LGS, in two of them by more than 50%. The highest seizure reduction rate was observed in the focal and tonic-clonic seizures. The most commonly reported adverse events were worsening of seizures, aggressiveness and irritability. Nine patients (47.4%) showed worsening of their behavior during the treatment with lacosamide. CONCLUSION: Lacosamide can exacerbate both, the tonic and astatic seizures, and the encephalopathy associated with this epileptic syndrome. However, it is interesting to consider the likelihood of suppression of generalized tonic-clonic and focal seizures. That is why; lacosamide could be an option after carefully balancing risks and benefits in each individual case.

Suicidal risk, affective dysphoric disorders, and quality-of-life perception in patients with focal refractory epilepsy.

OBJECTIVES: We aim to study the frequency of (suicidal ideation) in patients with focal refractory epilepsy and its possible association with factors such as perceived QOL (quality of life) and ASDD (affective somatoform dysphoric disorder) using the 2007 ILAE proposal to classify affective disorders of epilepsy. METHODS: A total sample of 82 patients was divided into two groups depending on the presence of suicidal risk: (A) study group - with suicidal risk and (B) control group - without suicidal risk. Questionnaires, scales, interviews, and clinical charts were evaluated by professionals with expertise in neurology and epileptology (RAM and AGA), psychiatry (AGE), and neuropsychology (FGR). Suicidal risk was evaluated with the M.I.N.I. (Mini-International Neuropsychiatric Interview) suicidal module that specifies the current suicidal risk based on scores. Quality of life was evaluated with the Quality of Life in Epilepsy Inventory - 31 (QOLIE-31) survey. Logistic regression was conducted to ascertain if ASDD and QOL significantly predicted suicidal risk. The results were considered statistically significant when the p-value was <0.05. RESULTS: Suicidal risk was present in 33 (40.3%) patients. It was classified as severe in 31.7% of the patients, and it was only present in cases with temporal lobe epilepsy (p=0.002). More than half (52%) of patients with ASDD had risk of suicide (p=0.006). The presence of ASDD was found to be a risk factor for suicidal risk (OR=3.86; IC=1.3-12.2). Patients with suicidal risk had a lower QOL score compared with patients without suicidal risk (57.8±16.9 vs. 46.0±18.2; p<0.05), and an affected QOL significantly increased suicidal risk (OR=2.9; CI=1.3-7.8). Multivariate analysis demonstrated that an impaired QOL (OR=2.2) and the presence of ASDD (OR=4.1) significantly increased the probability of having suicidal risk (x(2)=13.6; OR=5.2; p=0.009). SIGNIFICANCE: Affective somatoform dysphoric disorder and low QOL perception increase, independently, the risk of suicide.

Lacosamide in Lennox-Gastaut syndrome: case report.

INTRODUCTION: Lennox Gastaut syndrome (LGS) is an epileptic encephalopathy characterized by tonic, atonic, and atypical absence seizures usually refractory to pharmacological treatment. Patients generally continue with seizures despite treatment with the commercially available antiepileptic drugs (AEDs). Lacosamide (LCM) is a new AED recently approved for treatment of partial onset seizures with or without secondary generalization. Lacosamide has a novel mechanism of action that seems to be different in relation to other conventional AEDs. OBJECTIVE: To report LCM-caused worsening of tonic seizures and electroencephalographic pattern in a patient with Lennox-Gastaut syndrome. CASE REPORT: We report the evolution of a patient with LGS resistant to several AEDs with a cryptogenic hepatopathy in whom LCM caused worsening of tonic seizures and electroencephalographic pattern. Once LCM was discontinued, the patient returned to his clinical and electrical baseline. CONCLUSION: Lennox Gastaut syndrome may exacerbate tonic seizures and electrical pattern of patients with LGS.

Síndrome opsoclono-mioclono-ataxia posmalaria: un caso pediátrico / Post-malarial opsoclonus-myoclonus-ataxia syndrome: a paediatric case

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