EARLY-ESLI study: Long-term experience with eslicarbazepine acetate after first monotherapy failure.

PURPOSE: Evaluate real-life experience with eslicarbazepine acetate (ESL) after first monotherapy failure in a large series of patients with focal epilepsy. METHOD: Multicentre, retrospective, 1-year, observational study in patients older than 18 years, with focal epilepsy, who had failed first antiepileptic drug monotherapy and who received ESL. Data from clinical records were analysed at baseline, 3, 6 and 12 months to assess effectiveness and tolerability. RESULTS: Eslicarbazepine acetate was initiated in 253 patients. The 1-year retention rate was 92.9%, and the final median dose of ESL was 800 mg. At 12 months, 62.3% of patients had been seizure free for 6 months; 37.3% had been seizure free for 1 year. During follow-up, 31.6% of the patients reported ESL-related adverse events (AEs), most commonly somnolence (8.7%) and dizziness (5.1%), and 3.6% discontinued due to AEs. Hyponatraemia was observed in seven patients (2.8%). After starting ESL, 137 patients (54.2%) withdrew the prior monotherapy and converted to ESL monotherapy; 75.9% were seizure free, 87.6% were responders, 4.4% worsened, and 23.4% reported ESL-related AEs. CONCLUSION: Use of ESL after first monotherapy failure was associated with an optimal seizure control and tolerability profile. Over half of patients were converted to ESL monotherapy during follow-up.

Infratentorial hygroma secondary to decompressive craniectomy after cerebellar infarction.

We present a case of expansive CSF collection in the cerebellar convexity. The patient was a 74 years old lady who one month before had suffered a cerebellar infarct complicated with acute hydrocephalus. She had good evolution after decompressive craniectomy without shunting. Fifteen days after surgery, the patient started with new positional vertigo, nausea and vomiting and a wound CSF fistula that needed ventriculoperitoneal shunt (medium pressure) because conservative treatment failed. After shunting, the fistula closed, but the patient symptoms worsened. The MRI showed normal ventricular size with a cerebellar hygroma, extending to the posterior interhemispheric fissure. The collection had no blood signal and expanded during observation. A catheter was implanted in the collection and connected to the shunt. The patient became asymptomatic after surgery, and the hygromas had disappeared in control CT at one month. This case shows an infrequent problem of CSF circulation at posterior fossa that resulted in vertigo of central origin. A higroma-ventricle-peritoneal shunt solved the symptoms of the patient.

[Diagnostic challenges in epilepsy]. / Desafios diagnosticos en epilepsia.

INTRODUCTION: Epileptic seizures and epilepsy are part of daily clinical practice in neurology. Yet, the number of false positive diagnoses is surprisingly high. Almost one out of every five patients treated for epilepsy does not really have this diagnosis, which is a high percentage bearing in mind the social and medical consequences that being diagnosed with epilepsy entails. AIMS: To summarise the most important diagnostic challenges in epilepsy, to describe possible sources of diagnostic error and to offer advice on how to avoid them. DEVELOPMENT: Epilepsy is characterised by a tendency to suffer unprovoked epileptic seizures. The greatest obstacle when it comes to diagnosing a case of epilepsy is the fact that epileptic seizures are transient phenomena that occur relatively infrequently and the physician who must carry out the diagnosis will rarely see them. Moreover, there are other clinical events, such as syncopes or non-epileptic seizures, that may be similar to epileptic seizures in appearance and, consequently, can be mistaken for them. Finally, when interpreting the two most important complementary diagnostic techniques in epileptology, the electroencephalogram and magnetic resonance imaging of the brain, the most common errors must be taken into account in order to prevent mistaken diagnoses. CONCLUSIONS: The diagnosis of epilepsy is a challenge and must be based on a detailed and specific medical record. If there are any reasonable doubts, from the outset, about the diagnosis of epilepsy or if the patient does not respond well to the antiepileptic treatment, we recommend referring the patient to a specialised centre to establish a definitive diagnosis.

TITLE: Desafios diagnosticos en epilepsia.Introduccion. Las crisis epilepticas y la epilepsia son parte de la practica clinica diaria en neurologia. No obstante, el numero de diagnosticos falsos positivos es sorprendentemente alto. Casi uno de cada cinco pacientes tratado por epilepsia en realidad no tiene ese diagnostico, un porcentaje elevado teniendo en cuenta las consecuencias sociomedicas que conlleva el diagnostico de epilepsia. Objetivos. Resumir los desafios diagnosticos mas importantes en epilepsia, describir posibles fuentes de error en el diagnostico y proporcionar consejos sobre como evitarlos. Desarrollo. La epilepsia se caracteriza por una tendencia a sufrir crisis epilepticas no provocadas. El mayor obstaculo al diagnosticar una epilepsia radica en que las crisis epilepticas son fenomenos transitorios que ocurren relativamente con poca frecuencia y el medico que realiza el diagnostico raramente llega a verlas. Ademas, existen otros eventos clinicos, como por ejemplo sincopes o crisis no epilepticas, que pueden tener una apariencia similar a las crisis epilepticas y, en consecuencia, confundirse con ellas. Finalmente, al interpretar las dos tecnicas diagnosticas complementarias mas importantes en epileptologia, el electroencefalograma y la resonancia magnetica cerebral, deben tenerse en cuenta los errores mas comunes para prevenir diagnosticos erroneos. Conclusiones. El diagnostico de una epilepsia es un reto y debe basarse en una historia clinica detallada y especifica. Si desde el inicio existen dudas razonables sobre el diagnostico de epilepsia o si el paciente no responde bien al tratamiento antiepileptico, recomendamos derivar al paciente a un centro especializado que establezca un diagnostico definitivo.

Accelerated long-term forgetting in resected and seizure-free temporal lobe epilepsy patients.

Episodic memory impairments caused by temporal lobe epilepsy (TLE) are well documented in the literature. Standard clinical episodic memory tests typically include a 30-min delayed recall test. However, in the past decade, it has become apparent that this standard test does not capture the full range of memory problems in TLE patients. Some patients perform well on a standard 30-min delayed recall test, but show Accelerated Long-term Forgetting (ALF) after 24 h. Although ALF has been investigated in patients with different types of epilepsy, current research on resected TLE patients is missing. In the present study, resected TLE patients were compared to a control group matched on initial learning. They showed normal performance on verbal recall after 30 min, but impairments became apparent after one week. Moreover, the significant interaction between participant group and memory test delay demonstrated that the patients indeed showed an acceleration in forgetting. Furthermore, ALF was present in both left and right resected TLE patients, which contradicts the presence of material-specific hemispheric differences in ALF. In addition, ALF was observed in seizure-free resected TLE patients, thereby demonstrating that this factor is not crucial for long-term memory deficits. The outcome shows that clinicians are likely to underestimate memory deficits in resected TLE patients and, therefore, advocates for the inclusion of ALF tests in standard clinical batteries for both pre- and post-surgery testing sessions.

[The problem of neurological emergencies and the need for specific neurology shifts]. / Urgencias neurológicas y guardias de Neurología.

In recent years different studies have highlighted a progressive increase in the demand for neurological care in emergency departments. To analyze the convenience of specific neurology shifts or the role that the neurologist should play in the emergency department, it is necessary to answer questions such as: What is the demand for emergency neurological care? What are the most frequent neurological emergencies? Who should attend to neurological emergencies and why? Are specific neurology shifts necessary? Neurological emergencies account for between 2.6% and 14% of medical emergencies. Stroke represents a third of all neurological emergencies, while the diagnoses of acute cerebrovascular disease, epilepsy and cephalea constitute 50% of all neurological care in the emergency department. On the basis of quality of care criteria and professional competence, the best care for patients with a neurological emergency is provided by a specialist in neurology. The implementation of specific neurology shifts, with a 24 hour physical presence, is associated with greater quality of care, better diagnostic and therapeutic orientation from the moment the patient arrives in emergency department, reduces unnecessary admissions, reduces costs and strengthens the neurology service.

Evidence for degraded low frequency verbal concepts in left resected temporal lobe epilepsy patients.

According to a large neuropsychological and neuroimaging literature, the bilateral anterior temporal lobe (ATL) is a core region for semantic processing. It seems therefore surprising that semantic memory appears to be preserved in temporal lobe epilepsy (TLE) patients with unilateral ATL resection. However, recent work suggests that the bilateral semantic system is relatively robust against unilateral damage and semantic impairments under these circumstances only become apparent with low frequency specific concepts. In addition, neuroimaging studies have shown that the function of the left and right ATLs differ and therefore left or right ATL resection should lead to a different pattern of impairment. The current study investigated hemispheric differences in the bilateral semantic system by comparing left and right resected TLE patients during verbal semantic processing of low frequency concepts. Picture naming and semantic comprehension tasks with varying word frequencies were included to investigate the pattern of impairment. Left but not right TLE patients showed impaired semantic processing, which was particularly apparent on low frequency items. This indicates that, for verbal information, the bilateral semantic system is more sensitive to damage in the left compared to the right ATL, which is in line with theories that attribute a more prominent role to the left ATL due to connections with pre-semantic verbal regions.

Efficacy of perampanel in a patient with epilepsia partialis continua.

Perampanel is the first-in-class selective and noncompetitive α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor antagonist. It is authorized in the U.S. and Europe as an add-on antiepileptic drug for partial-onset seizures, and for primary generalized tonic-clonic seizures. Single reports have also indicated a potential efficacy for myoclonic jerks. Here, we report a patient whose drug-resistant epilepsia partialis continua completely resolved after adding perampanel. She has remained seizure-free in an eighteen-month follow-up period. Epilepsia partialis continua reemerged transiently after perampanel was temporarily discontinued, with no recurrence after its reintroduction. Therefore, this effect was reproducible, and suggests that it might be worth trying perampanel in similar settings.

Prognostic factors in patients with refractory idiopathic generalized epilepsy.

OBJECTIVE: Idiopathic generalized epilepsy (IGE) is an epileptic condition with good response to antiepileptic drugs (AED). Major syndromes are epilepsy with generalized tonic-clonic seizures (GTCS) alone, absence epilepsy and juvenile myoclonic epilepsy. However, clinical practice shows drug-resistant patients. Endpoint is to identify clinical features related with refractoriness in IGE and in its each individual syndrome. METHODS: We retrospectively collected 279 consecutive patients with IGE assessed in the Epilepsy Clinic of our institution. We defined drug-resistant epilepsy as a failure of adequate trials of 2 tolerated and appropriately chosen and used AED schedules. We classified patients in two groups: drug-resistant and drug-responsive. Clinical features were compared among these groups, in the whole IGE group as well as in each syndrome. RESULTS: There were 122 drug-resistant, 105 drug- responsive; 52 were undefined and excluded from the analysis. After multivariate analysis, early seizures onset (age <13), long-time epilepsy, several generalized seizure types, status epilepticus, EEG with generalized epileptiform activity, mainly polyspikes, and side effects with AED brought up as poor outcome factors. Additionally, 50.6% identified modifiable seizure triggers. Regarding syndromes, epilepsy with generalized tonic-clonic seizures alone had the same factors except several seizure types; presence of additional GTCS, polyspikes, history of AED side effects and psychiatric disorder were poor factors for absence epilepsy; only psychiatric comorbidity revealed significance in juvenile myoclonic epilepsy. SIGNIFICANCE: Refractoriness in IGE and its major syndromes is associated with clinical and electrographic parameters. Moreover, lifestyle advices from neurologists to the patients might help them to achieve a better seizure control.

Efficacy and safety of eslicarbazepine-acetate in elderly patients with focal epilepsy: Case series.

PURPOSE: Eslicarbazepine-acetate (ESL) is a third generation antiepileptic drug licensed as adjunctive therapy in adults with focal seizures. Efficacy and safety of ESL have been established in real-life setting. However, data about outcomes in elderly patients are scarce. Primary endpoint was to evaluate outcomes of ESL in elderly patients. METHOD: This was a retrospective survey that included patients >65years with focal seizures who started ESL between January 2010 and July 2012 at 12 Spanish Hospitals. ESL was prescribed individually according to real-life practice. Efficacy and safety were evaluated over 1year. These patients were included within the bigger study ESLIBASE. RESULTS: We included 29 patients, most of them males (18). Mean age was 71.2 year-old and epilepsy evolution was 20 years. Eighteen were pharmacorresistant at baseline. At 12 months, the mean dose was 850mg/day, the retention rate 69%, the responder rate 62% and 24.1% were seizure-free. At 12 months, 16 patients (55.2%) had ≥1 adverse effect (AE), that led to discontinuation in 7 patients. Dizziness, nausea and ataxia were the most common AEs. The tolerability profile improved in 4/5 patients who switched from carbamazepine (CBZ) or oxcarbazepine (OXC) to ESL due to AEs. CONCLUSIONS: ESL was well-tolerated and effective in elderly patients in a real-life setting over 1year, with a dose around 800mg/day. AE effects improved in most of who switched from CBZ or OXC to ESL.

[Video-EEG monitoring and simultaneous ECG for the differential diagnosis of transient consciousness disorders. A case report]. / Monitorización con vídeo-EEG y ECG simultáneo para el diagnóstico diferencial de trastornos de conciencia transitorios. A propósito de un caso.

We present the case of a 36 year-old woman, with history of transient consciousness disorders with vegetative state, interpreted as epileptic crises and treated with valproate for two years. After nine asymptomatic years, they reappeared associated with migraine, vomiting and some generalized convulsions. Electroencephalogram and cerebral magnetic resonance turned out normal, and treatment with zonisamide was started, without beneficial results. Later cardiological studies objectified a blockage of the left branch that coincided with dizziness. The study was completed with Video-EGG monitoring, where there was an episode that showed temporary right epileptiform activity, with a diagnosis established of focal epilepsy of unknown cause. At present, she remains asymptomatic with oxycarbazepine.

[Gliomatosis cerebri: review of 22 patients]. / Gliomatosis cerebral: estudio de 22 pacientes.

INTRODUCTION: gliomatosis cerebri is a diffuse astrocytic neoplasm that involves more than two lobes of the brain. Treatment is not well defined and the prognosis is considered poor. METHODS: retrospective analysis of 22 patients with gliomatosis cerebri. RESULTS: we identified 17 men and 5 women (median age 54 years) seen in a Division of Neuro-oncology over a 6 year period. Patients presented with focal sensorimotor or visual deficits (86.4%), seizures (36.4%), cognitive dysfunction (27.3%), or headache (27.3%), suggesting in some cases stroke, migraine, or limbic encephalitis. All patients had bilateral involvement; the regions involved included, temporal (19), basal ganglia (18), frontal (17), parietal (17), corpus callosum (10), and occipital (9). The most frequent pathological findings were grade III astrocytoma (36.4%), grade II astrocytoma (22.7%), and grade IV astrocytoma (18.3%). Nine patients were diagnosed within the first month of symptom development, 11 between the first month and 1 year, and 2 after one year. Seventeen patients received treatment with chemotherapy, radiotherapy or both, and 12 patients (70.6%) had a clinical or radiological response. The median follow-up was 13 months, median progression free survival 6 months, and median survival 9,5 months (15 months if the patients received treatment). Eight patients had thromboembolic events. CONCLUSIONS: gliomatosis cerebri has a variable clinical course. Treatment often results in clinical responses. In this study de median survival of patients who received treatment was similar to that reported in series of glioblastoma multiforme.