Early focal electroencephalogram and neuroimaging findings predict epilepsy development after aneurysmal subarachnoid hemorrhage.

INTRODUCTION: Seizures are a common complication of subarachnoid hemorrhage (SAH) in both acute and late stages: 10-20 % acute symptomatic seizures, 12-25 % epilepsy rate at five years. Our aim was to identify early electroencephalogram (EEG) and computed tomography (CT) findings that could predict long-term epilepsy after SAH. MATERIAL AND METHODS: This is a multicenter, retrospective, longitudinal study of adult patients with aneurysmal SAH admitted to two tertiary care hospitals between January 2011 to December 2022. Routine 30-minute EEG recording was performed in all subjects during admission period. Exclusion criteria were the presence of prior structural brain lesions and/or known epilepsy. We documented the presence of SAH-related cortical involvement in brain CT and focal electrographic abnormalities (epileptiform and non-epileptiform). Post-SAH epilepsy was defined as the occurrence of remote unprovoked seizures ≥ 7 days from the bleeding. RESULTS: We included 278 patients with a median follow-up of 2.4 years. The mean age was 57 (+/-12) years, 188 (68 %) were female and 49 (17.6 %) developed epilepsy with a median latency of 174 days (IQR 49-479). Cortical brain lesions were present in 189 (68 %) and focal EEG abnormalities were detected in 158 patients (39 epileptiform discharges, 119 non-epileptiform abnormalities). The median delay to the first EEG recording was 6 days (IQR 2-12). Multiple Cox regression analysis showed higher risk of long-term epilepsy in those patients with CT cortical involvement (HR 2.6 [1.3-5.2], p 0.009), EEG focal non-epileptiform abnormalities (HR 3.7 [1.6-8.2], p 0.002) and epileptiform discharges (HR 6.7 [2.8-15.8], p < 0.001). Concomitant use of anesthetics and/or antiseizure medication during EEG recording had no influence over its predictive capacity. ROC-curve analysis of the model showed good predictive capability at 5 years (AUC 0.80, 95 %CI 0.74-0.87). CONCLUSIONS: Focal electrographic abnormalities (both epileptiform and non-epileptiform abnormalities) and cortical involvement in neuroimaging predict the development of long-term epilepsy. In-patient EEG and CT findings could allow an early risk stratification and facilitate a personalized follow-up and management of SAH patients.

Musicogenic reflex seizures in epilepsy with glutamic acid decarbocylase antibodies.

BACKGROUND: Musicogenic reflex seizures (MRS) are a rare form of seizures described in patients with temporal lobe epilepsy (TLE), mainly of unknown etiology. Epilepsy with antibodies against glutamic acid decarboxylase (GAD-ab) is a form of autoimmune epilepsy for which no specific semiology has been described. AIM OF THE STUDY: To retrospectively review the incidence of MRS in the general epileptic population and in the series of patients with epilepsy and GAD-ab and to describe its clinical and paraclinical characteristics. METHODS: Patients recorded between January 2010 and January 2016 in the Database of Bellvitge Hospital Epilepsy Unit were reviewed. RESULTS: From a group of 1510 epileptic patients, three reported MRS (0.0019%) (two patients with epilepsy and GAD-ab and one patient with cryptogenic TLE). The incidence of MRS in patients with epilepsy and GAD-ab was 2 of 22 (9%). Both patients had a normal magnetic resonance Imaging (MRI), but FDG-PET showed medial temporal lobe hypometabolism (unilateral or bilateral) in both and also in the insula in one of them. MRS (recorded via video-EEG[electroencephalography] in one patient) arose from the right temporal lobe. CONCLUSIONS: MRS may be a distinctive seizure type in patients with epilepsy and antiGADab. Determination of GAD-ab should be carried out in all cases of MRS, even those with normal structural MRI.

Retrospective study of perampanel efficacy and tolerability in myoclonic seizures.

OBJECTIVES: Perampanel is an antiepileptic drug (AED) approved for add-on treatment of focal seizures (with or without generalization) and primary generalized tonic-clonic (GTC) seizures. Our objective was to explore the effectiveness and tolerability of adjunctive perampanel in patients with drug-resistant myoclonic seizures, after failure of other AEDs. MATERIALS AND METHODS: Retrospective, multicenter, observational study. Data were collected from individual patient clinical files and analysed using appropriate descriptive statistics and inferential analyses. RESULTS: Data are reported for 31 patients with mean age 36.4 years, who had an average epilepsy duration of 18 years, previously taken an average of 5.03 AEDs, and were taking an average of 2.4 AEDs on perampanel initiation. Patients exhibited myoclonic, GTC, absence, tonic and focal seizures, and most had associated cognitive decline and/or ataxia. Median time on perampanel was 6 months, most common dose was 6 mg, and overall retention rate was 84%. The responder rate for myoclonic seizures was defined via reduction of days with myoclonic seizures per month. At 6 months, 15 (48.4%) of the 31 patients were classed as myoclonic seizure responders, 10 (32.3%) were myoclonic seizure free, and 39% saw improvements in functional ability. Of 17 patients with GTC seizures at baseline, 9 (53%) were responders at 6 months, and 8 (47.1%) were seizure free. The most frequent side effects were psychiatric disorders, instability, dizziness and irritability, and mostly resolved with dose reduction. Five patients discontinued perampanel due to side effects. CONCLUSIONS: Perampanel caused clinically meaningful improvements in patients with drug-resistant myoclonic seizures. It was generally well tolerated, but psychiatric and neurological side effects sometimes required follow-up and dose reduction.

Rufinamide in children and adults in routine clinical practice.

OBJECTIVE: To explore the long-term effectiveness of rufinamide in managing Lennox-Gastaut Syndrome (LGS), other epileptic encephalopathies, and intractable focal epilepsies in adults and children in routine clinical practice. METHODS: A multicentre, retrospective chart review of patients prescribed adjunctive rufinamide at seven Spanish epilepsy centres, with assessments at six and 12 months. RESULTS: We evaluated data from 58 patients (40 male, age range 7-57 years), 25 of whom were diagnosed with LGS, 12 with other epileptic encephalopathies and 21 of whom were diagnosed with focal epilepsies, mainly frontal lobe. The mean daily rufinamide dose was 32.0 mg/kg (range 12.5-66.7 mg/kg) in children and 24.7 mg/kg (range 5.0-47.0 mg/kg) in adults, and the most commonly used concomitant antiepileptic drugs were levetiracetam and valproate. Rufinamide was discontinued in 25 patients (43.1%) during the 1-year follow-up, and the most common reason was lack of effectiveness (n = 12, 20.7% of total). The frequency of generalized tonic-clonic seizures was significantly reduced from baseline at 6 and 12 months (P = 0.001), both in patients with generalized epilepsies and in patients with focal epilepsies. Significant seizure frequency reduction from baseline was observed at 12 months (P = 0.01) for tonic/atonic seizures and at 6 months (P = 0.001) for focal seizures. Side effects were reported in 21 patients (36.2%): nausea, vomiting and weight loss were most frequent. CONCLUSIONS: Rufinamide was well tolerated and was effective in reducing frequency of generalized tonic-clonic, tonic/atonic and focal seizures in both children and adults with severe refractory epilepsies, primarily LGS.

EEG extreme delta brush: An ictal pattern in patients with anti-NMDA receptor encephalitis.

INTRODUCTION: The anti-NMDA receptor (NMDAr) encephalitis-associated syndrome includes neuropsychiatric symptoms, impaired consciousness, seizures, autonomic instability, and hypoventilation. The electroencephalographic (EEG) activity throughout the course of the disease has still not been well documented. We reviewed electroclinical data of patients with NMDAr encephalitis to characterize their EEG and its clinical correlation. MATERIAL AND METHODS: We retrospectively identified 16 patients with NMDAr encephalitis from 8 Spanish medical centers, 15 of whom underwent video-EEG in the acute phase. RESULTS: In 15 patients (11 females, median age: 37.4, range: 14-87 years), seizures occurred in 9 (60%) and status epilepticus (SE) in 5 (33.3%). Magnetic resonance imaging (MRI) was abnormal in 10 (66.6%), and CSF (cerebrospinal fluid) was normal in 3 and abnormal in 12, with positive PCR (polymerase chain reaction) for Mycoplasma pneumoniae (1/15) and herpes simple virus (1/15). An ovarian teratoma was found in 1 patient and other malignancies (small cell lung carcinoma) in 1 patient. The EEG was abnormal in the acute phase in 14/15 (93.3%). Extreme delta brush (EDB) was observed in 5 (33.3%), and the presence of EDB was associated with SE in all cases. Rhythmic delta activity without EDB was observed in 5 (33.3%), while excessive beta activity was present in 4 (26.6%). Extreme delta brush can follow a pattern of well-characterized electroclinical seizures. CONCLUSIONS: Almost invariably, patients with NMDAr encephalitis had abnormal EEG. The presence of EDB, which can follow a pattern of well-characterized electroclinical seizures, in our patients was associated with seizures and SE. These findings suggest that EDB could be an evolutive pattern of an SE in NMDAr encephalitis. This article is part of a Special Issue entitled "Status Epilepticus".

Low-dose sodium valproate in the treatment of idiopathic generalized epilepsies.

OBJECTIVE: Most patients with idiopathic generalized epilepsies (IGEs) have good seizure control when on antiepileptic drugs. To analyze prospectively the response to low-dose sodium valproate (VPA) treatment (<1000 mg/day) together with plasma VPA levels in a cohort of patients with IGE. METHODS: Patients with IGE were selected and followed for almost 2 years. In patients on VPA with no seizures in the last year, VPA dose was lowered to <1000 mg/day. Newly diagnosed patients with IGE started treatment on VPA directly on this low dose. RESULTS: Fifty-four patients were included, with juvenile myoclonic epilepsy (JME) in 23 (42.6%), juvenile absence epilepsy (JAE) in 17 (31.5%), and generalized tonic-clonic seizures only (GTCS only) in 14 (25.9%). VPA at low dose was administered to 38 (70%) patients. Mean plasma VPA level was 44.21 mg/l (18-78; SD 15.18). Seizure relapse during the 2-year follow-up was observed in 8 (21%). A reduction in adverse events was observed (P < 0.048). The only factor related to efficacy of VPA at low dose was syndromic diagnosis. Low-dose VPA controlled 92.9% (13) of patients with GTCS only, 78.3% (18) of those with JME, and 29.5% (5) of those with JAE. CONCLUSIONS: Low-dose VPA was a highly effective treatment for the majority of those with JME and GTCS only. The seizures in JAE tended to be more resistant to treatment, usually requiring higher doses of VPA or polytherapy.

Combination of corpus callosotomy and vagus nerve stimulation in the treatment of refractory epilepsy.

BACKGROUND: Palliative techniques such as partial corpus callosotomy (CC) and vagus nerve stimulation (VNS) may be effective for adequate control of seizures in pharmacoresistant patients who are not candidates for resective surgery. OBJECTIVE: The objective of this study was to analyze the efficacy of the combination of these two techniques in patients where the first surgery had not achieved adequate control. MATERIALS AND METHODS: This is a retrospective review of 6 patients with refractory epilepsy in which both types of surgery were performed, CC and VNS. We analyzed variables such as age, sex, age at onset of epilepsy, seizure types, electroencephalogram and magnetic resonance imaging results, and number of pre- and postoperative seizures. RESULTS: Three patients first underwent VNS and then CC, and 3 patients were treated in reverse order. All patients had some improvement after the first surgery, but they continued to experience persistent falls, so a second palliative technique was used. The mean improvement after both surgeries was 89% (90% in patients first receiving CC and 87% in patients who first underwent VNS). CONCLUSIONS: In adequately studied patients who are not optimal candidates for resective surgery, palliative surgery is a choice. The combination of VNS and CC shows good results in our series, although the right order to perform both procedures has not been defined. These results should be confirmed in a larger group of patients.

Cognitive decline in adult-onset temporal lobe epilepsy: Insights from aetiology.

PURPOSE: To identify patients with adult-onset temporal lobe epilepsy (TLE) at risk of developing cognitive decline. Detecting which patients, aetiologies, or factors are most closely related with memory decline would allow us to identify patients that would eventually benefit from more specific treatment. METHODS: Single centre, retrospective analysis of a prospectively followed-up cohort study, including all patients with the diagnosis of adult-onset TLE during 2013, with a minimum follow-up of five years. Memory and cognitive decline were analysed at 5 years and at last follow-up. RESULTS: Of 89 initially selected patients, 71 were included. After 5 years, 11/71 (15.5%) patients suffered cognitive decline, of which 1/71 (4%) developed dementia. At last follow-up (range 65-596 m) a total of 34/71 (47.8%) patients were diagnosed with cognitive decline, specifically either memory decline or dementia. Cognitive decline at 5 years was related to: 1. Age at onset: 62.65 years (SD 9.04) in the group with cognitive decline vs 50.33 y. (SD 13.02 in the group without cognitive decline; p=0.004); 2. Onset as status epilepticus (3/6 in patients with memory decline vs 8/65 in patients without cognitive decline; p=0.04); 3. Immune aetiology: 42% compared with unknown (10%) and structural (10%) aetiologies; p=0.036; 4. Hippocampal sclerosis on MRI: 5/11 patients with cognitive decline vs 9/51 patients without cognitive decline; p=0.035. Cognitive decline was not related to seizure frequency, sex, or age (p=0.78; p=0.40; p=0.95, respectively). CONCLUSIONS: Older age at epilepsy onset, onset as status epilepticus, immune aetiology, and hippocampal sclerosis are risk factors for developing cognitive decline in patients with adult-onset temporal lobe epilepsy.

Analysis of automated methods for spatial normalization of lesioned brains.

Normalization of brain images is a crucial step in MRI data analysis, especially when dealing with abnormal brains. Although cost function masking (CFM) appears to successfully solve this problem and seems to be necessary for patients with chronic stroke lesions, this procedure is very time consuming. The present study sought to find viable, fully automated alternatives to cost function masking, such as Automatic Lesion Identification (ALI) and Diffeomorphic Anatomical Registration using Exponentiated Lie algebra (DARTEL). It also sought to quantitatively assess, for the first time, Symmetrical Normalization (SyN) with constrained cost function masking. The second aim of this study was to investigate the normalization process in a group of drug-resistant epileptic patients with large resected regions (temporal lobe and amygdala) and in a group of stroke patients. A dataset of 500 artificially generated lesions was created using ten patients with brain-resected regions (temporal lobectomy), ten stroke patients and twenty five-healthy subjects. The results indicated that although a fully automated method such as DARTEL using New Segment with an extra prior (the mean of the white matter and cerebro-spinal fluid) obtained the most accurate normalization in both patient groups, it produced a shrinkage in lesion volume when compared to Unified Segmentation with CFM. Taken together, these findings suggest that further research is needed in order to improve automatic normalization processes in brains with large lesions and to completely abandon manual, time consuming normalization methods.

Prevalence and immunological spectrum of temporal lobe epilepsy with glutamic acid decarboxylase antibodies.

BACKGROUND AND PURPOSE: High levels of glutamic acid decarboxylase (GAD)-ab were initially described in patients with stiff person syndrome, and have since also been observed in patients with other neurological diseases. Temporal lobe epilepsy (TLE) seems to be specially associated. Our purpose is to describe the prevalence of GAD-ab in patients with TLE, and to characterize the clinical-immunological profile of TLE patients with high levels of GAD-ab. METHODS: An immunological profile including GAD-ab and antinuclear, anti-DNA, anti-cardiolipin, anti-transglutaminase and antithyroid antibodies was determined in a consecutive series of patients with TLE. As adulthood onset is the least common onset in TLE + hipocampal sclerosis and febrile seizures, we selected patients whose onset was after 30 years of age, to expand the spectrum of aetiologies. Patients were divided into two groups: known aetiology, 19 patients (45%) and unknown aetiology, 23 (55%). The clinical-immunological study included TLE patients with high GAD-ab levels (>1000 IU). RESULTS: Amongst 42 patients, serum GAD-ab levels were positive in 5 (152-11, 963 IU/ml), all from the unknown aetiology group. Thus, GAD-ab levels were positive in 21.7% and high in 8.7% of the unknown aetiology group. The immunological profile study included nine patients (seven pharmacoresistant), of whom six were women (66%) with mean age 41 years. Three patients reported acute debut, four (44%) insulin-dependent diabetes mellitus, five (55%) other concomitant autoimmune diseases, four (44%) memory impairment and four moderate-to-severe mood disturbance. Intrathecal synthesis of GAD-ab was observed in seven patients (77%). CONCLUSIONS: Temporal lobe epilepsy with GAD-ab is not a rare condition. In the subgroup of patients with high titres, this epilepsy is often pharmacoresistant and associated with memory impairment, depression and other autoimmune diseases.

Successful outcome of episodes of status epilepticus after vagus nerve stimulation: a multicenter study.

BACKGROUND AND PURPOSE: Vagus nerve stimulation (VNS) has been reported to be a safe and effective treatment for drug-resistant epilepsy. The aim of this study is to describe the effect of VNS in patients with a history of repeated episodes of status epilepticus (SE) before implantation. METHODS: From a total of 83 adult patients with drug-resistant epilepsy who had VNS implanted in four tertiary centers in Spain between 2000 and 2010, eight had a previous history of repeated episodes of SE. We performed a retrospective observational study analyzing the outcome of seizures and episodes of SE after implantation. Stimulation was started at the usual settings, and intensity increased according to clinical response and tolerability. RESULTS: Regarding the eight patients with a history of SE, the mean age at time of VNS implantation was 25.1 [14-40] years. Duration of epilepsy until the implantation was 21.7 [7-39.5] years, and they had been treated with a mean of 12 antiepileptic drugs [10-16]. Mean follow-up since implantation was 4.15 [2-7.5] years. Average seizure frequency decreased from 46 to 8.2 per month. Interestingly, four of the eight patients remained free of new episodes of SE after implantation, and in two additional patients, the frequency decreased by >75%. Adverse effects were mild or moderate in intensity and included mainly coughing and dysphonia. CONCLUSION: In those patients with refractory epilepsy and history of SE who are not surgical candidates, VNS is a safe and effective method to reduce seizure frequency and episodes of SE.

Acute symptomatic seizures and epilepsy after mechanical thrombectomy. A prospective long-term follow-up study.

INTRODUCTION: New treatments for acute ischaemic stroke, such as mechanical thrombectomy, can achieve reperfusion of large ischaemic tissue. Some studies have suggested that reperfusion therapies can increase the risk of suffering acute symptomatic seizure (ASS) and poststroke epilepsy (PSE). The aim of the study was to determine the incidence of ASS and PSE in patients undergoing thrombectomy, and related factors. PATIENTS AND METHODS: This was a retrospective single-centre study including patients with ischaemic stroke and NIHSS> 8 treated with thrombectomy with a follow-up ≥5 years. We evaluated several epidemiological, radiological, clinical and electroencephalographic variables. RESULTS: Of the 344 included patients, 21 (6.1%) presented ASS, 53 (15.40%) died in the acute phase, and 13 (4.46%) died during the first year. The degree of reperfusion (p 0.029), advanced age (p 0.035), and haemorrhagic transformation (p 0.038) increased the risk of suffering ASS, with degree of reperfusion being an independent factor, OR 2.02 (1.21-4.64). The incidence of PSE was 4.12% in the first year, 3.72% in the second, and 1.61% in the fifth. The accumulated incidence at 5 years was 8.93%. Related risk factor for suffering PSE was ASS (p < 0.001), yielding an OR value of 2.00 (1.28-3.145). CONCLUSIONS: Thrombectomy doesn´t increase the risk of ASS. A higher percentage of reperfusion, advanced age, and haemorrhagic transformation are associated with an increased risk of ASS. ASS is a risk factor for suffering PSE. In terms of mortality, having suffered ASS and/or PSE does not increase acute or long-term mortality.

Clinical characteristics associated with psychosocial functioning among patients with uncomplicated epilepsy in Spain.

OBJECTIVE: To identify the clinical characteristics associated with poor psychosocial functioning among Spanish patients with epilepsy but no other neurological or psychiatric disorder. METHODS: Between May and September 2001 a survey among patients with epilepsy was carried out in 32 Spanish health care centres. The selection criteria of patients were attendance to a routine neurologist visit, to be aged between 25 and 64 and not having another additional neurological handicap (n=812). Psychosocial function was elicited through six indicators: educational level, marital status, unemployment status, restricted car driving, self-perception of epilepsy as an important limiting factor in the educational level achieved and, among unemployed, as the cause of their unemployment. Multiple logistic regression models were fitted in order to calculate adjusted odds ratios (aOR) and their 95% confidence intervals. RESULTS: After simultaneously adjusting for socio-demographic variables and clinical characteristics, the six outcomes analysed increased with seizure frequency. Moreover, all the outcomes except low educational level were also related to early age at onset of epilepsy. Although no relation with objective educational level was found, there was a strong association between early age at onset of symptoms and self-perception of epilepsy as an important limiting factor of educational achievement. CONCLUSION: These findings emphasize the need for more effective treatment of epilepsy and also highlight the importance of a psychosocial approach to management of epilepsy for patients with an early onset of symptoms in order to prevent social limitations in adult life.

[Gabapentin and women]. / Gabapentina y mujer.

OBJECTIVE: In the management of epilepsy, as a chronic disorder, one should remember the factors which differentiate the patients so that they may be classified into groups with common characteristics. Using gender as a differentiating criterion, we tried to systematize the factors which should be considered when studying and treating a female patient with epilepsy. DEVELOPMENT: The factors to be taken into account are biological such as the sex hormones or of psychosocial type such those related to the stigma, which may have special characteristics depending on gender. Once these factors had been systematized, we reviewed the clinical use of gabapentin from the point of view of the group considered. CONCLUSIONS: We suggest that special guidelines should be used according to the patient s gender. This review considers the female gender. The rational use of gabapentin, as monotherapy or polytherapy plays an important part in fulfilling the requirements described.

[Prognostic factor for medical control for seizures in patients with radiologic evidence for mesial temporal lobe sclerosis]. / Factores pronósticos asociados al control médico en pacientes con evidencia radiológica de esclerosis mesial temporal.

INTRODUCTION: Patients with mesial temporal lobe sclerosis (MTS) usually have suffered a brain insult during early childhood, a febrile convulsion in most cases. Complex partial seizures start after a seizure free period of variable duration. These complex partial seizures have an stereotypic semiology. Often, these seizure are not controlled with medical therapy, previous studies shows a rate of control not superior of 30%. AIM. To know the prognosis for medical control of patients with MTS and to find related factors. PATIENTS AND METHODS: The clinical characteristics of 51 patients with temporal lobe epilepsy and radiologic evidence of MTS (hippocampus atrophy or increased signal on T2 or Flair magnetic resonance sequences). RESULTS: 51 patients, of them 17 (34%) were men. 17 (34%) presented right MTS, 30 (60%) left MTS and 2 (4.3%) bilateral MTS. 16 patients (34.8%) referred autonomic aura, 14 (27.5%) psychic aura, 4 (7.8%) dysphasia and 13 (25.5%) no aura, in 2 (3.9%) aura were mixed: autonomic psychic. The aura was associated to the lesion laterality (p= 0.023) and to the reference of some antecedent of cerebral aggression during early childhood (p= 0.011). Concerning to the seizure control, 15 patients (29.2%) were seizure free in the last 6 month and 36 (70.6%) remaining uncontrolled. The associated factors to the medical control were the age of onset (p= 0.024) and the duration of the epilepsy (p= 0.018). CONCLUSIONS: Around 29% of patients with MTS can be controlled with medical therapy. Seizure control is related with later age of onset and short duration of the epilepsy

[Clinical usefulness of oligoclonal bands]. / Utilidad clínica de las bandas oligoclonales.

The presence of oligoclonal bands (OCB) of immunoglobulin G (IgG) is in our days the most useful finding in the study of the CSF for the diagnosis of multiple sclerosis (MS). The most sensitive method for the detection of OCB is the isoelectric focusing followed by immunoblotting. The prevalence of OCB changes in different populations with a rank of results from 60 to 95 97%. We have determined the prevalence of OCB in our population and the sensitivity and the specificity of the technique used in our laboratory. We have included 391 patients in whom we analysed the presence of OCB, subdivided in; Group 0: Diagnosed of MS, group 1: First episode of demyelinating process, group 2: Neurological disorders considered noninflammatory or nonautoimmune (NINA),group 3: Neurological disorders considered inflammatory, infectious or autoimmune (IIA). The presence of OCB was searched in CSF and serum simultaneously using isoelectric focusing and immunoblotting. In order to standardize the technique we achieved and internal and external validation. Internal validation: sensitivity and specificity (using as a control group first the group NINA and after the group IA). External validation: we choose 10 pairs of CSF/serum from patients with different diagnostics and sent to a reference laboratory ( Karolinska Institute Medical School) that was blind of our results and of the diagnostics. The prevalence of OCB in each group has been: group 0 (MS): 87.7%, group 1: 54.8%, group 2 (NINA): 17.5%, group 3(IIA): 52.7%. Sensitivity: 97.7%, specificity using group NINA as control 82.5% and using group IIA 45.7%. Concordance with the reference laboratory in 9/10 determinations. We conclude that in our population the prevalence of OCB, in patients with MS, is lower than in Northern Europe. The OCB appear in may inflammatory, autoimmune diseases, their specificity for the diagnostic of MS is low.

[Idiopathic generalised epilepsy. The value of semiology and EEG scanning in its classification]. / Epilepsia generalizada idiopática. Utilidad de la semiología y del electroencefalograma para su clasificación.

INTRODUCTION: The different syndromes included in the idiopathic generalized epilepsies present an important overlapping of their electroclinical characteristics. AIM: To compare the two actual classifications (International classification of epileptic syndromes: ILAE of 1989 and of 2001) in a sample of patients with idiopathic generalised epilepsy (IGE) and to evaluate which electro-clinical factors were more useful in order to classify a patient into a syndrome. PATIENTS AND METHODS: 70 patients (44 women/26 men). Neurological examination and neuro-radiological examination were normal in all cases. The EEG (standard or sleep deprived) showed generalised epileptiform discharges in all patients. Clinical findings included a median age of onset 12.3 years. Most frequent first type of seizure was tonic-clonic (71%). Seizures during awakening were the most frequent (37%) and lack of sleep was the most important precipitating factor (44.7%). Generalized epileptiform discharges were present in 62.9% of the basal sleep deprived EEG, on the other hand, only 22.3% of the basal standard EEG showed epileptiform discharges, p = 0.048. 28% of patients were unclassifiable according to the 1989 ILAE classification, 7% were unclassifiable according to the 2001 ILAE classification. EEG during intermittent photic stimulation (p = 0.007), at awakening (p = 0.015) and the timetable of the seizures (seizures generalised at awakening) (p = 0.053) differentiated between idiopathic generalised syndromes of adolescence. CONCLUSION: The 2001 ILAE classification is more useful in order to classify patients with IGE because includes patients previously considered unclassifiable in the syndrome of generalised epilepsy with different phenotypes.

Efficacy of intravenous lacosamide as an add-on treatment in refractory status epilepticus: a multicentric prospective study.

OBJECTIVE: Treatment of status epilepticus (SE) has not changed in the last few decades, benzodiazepines plus phenytoin or valproate being the most common treatment. Once this first and second line treatment has failed SE is considered refractory (RSE). This study aimed to assess the efficacy and tolerability of intravenous (iv) lacosamide (LCM) in RSE. METHOD: Patients with RSE who were treated with ivLCM in six Spanish centers were prospectively included. Efficacy was defined as cessation of seizures after starting ivLCM, with no need for any further antiepileptic drug. All patients had been unsuccessfully treated following the standard protocol (benzodiazepines plus phenytoin or valproate) before ivLCM was added. RESULTS: Thirty-four patients were included, 52.9% men, with mean age of 60.15 years. In 58.9% of patients the etiology was symptomatic, and the most common type of SE was focal convulsive (82.4%). Mean initial bolus dose of LCM was 323.53mg. ivLCM was effective in more than half of patients (64.7%), with termination of SE before 12h in 50% of them. ivLCM was used as a fourth or later option in 76.5% of patients. No serious adverse events attributable to LCM were reported. CONCLUSIONS: LCM might be a fast, effective and safe add-on treatment in RSE.

Parry Romberg syndrome and linear scleroderma in coup de sabre mimicking Rasmussen encephalitis.

We present one patient with Parry Romberg syndrome and another with linear scleroderma in coup de sabre, with focal neurologic deficits and intractable seizures arising from the hemisphere ipsilateral to the cutaneous lesion. Brain MRI showed progressive hemispheric atrophy. Pathology after functional hemispherectomy showed chronic inflammatory features suggestive of Rasmussen encephalitis.