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Background Uric acid ( UA) could act as a natural peroxynitrite scavenger with antioxidant properties. It has been proposed that hyperuricemia might protect against multiple sclerosis ( MS). Methods Patients with relapsing-remitting MS starting treatment with interferon beta-1a 44 µg sc 3/week were randomly assigned to receive either inosine 3 g/day or placebo in a double-blind manner. Follow-up was 12 months. Outcome measures were adverse events and UA laboratory results. Secondary end point was clinical and radiological activity of MS. Relapse rates, percentage of patients without relapses, and progression to secondary MS ( SPMS) were assessed. Results Thirty six patients were included. Two patients in the inosine group showed UA serum level above 10 mg/ml, and symptoms derived from renal colic not leading to hospital admission. Ten additional patients had asymptomatic hyperuricemia (>7 mg). Efficacy parameters (clinical and radiological) were similar between groups. No patient progressed to SPMS Conclusions Inosine administration was associated with hyperuricemia and renal colic with no additional effect on MS. We cannot conclude inosine is a safe and well-tolerated drug. Doses of around 2 g/day may be more appropriate for future trials. [ABSTRACT FROM AUTHOR]

Introduction: The relationship between epilepsy and music is poorly understood. Musicogenic epilepsy, which involves seizures triggered by music, and epilepsy that triggers or involves musical experiences are rare. Anti-seizure medications (ASMs) may affect cognition and possibly the musical sphere. The relationship between epilepsy, ASMs and music perception is insufficiently investigated in the literature. This study describes the clinical characteristics of patients with epilepsy with advanced musical knowledge, and aims to understand the disease's involvement in the musical sphere., Patients and Methods: A qualitative study was conducted in epileptic patients with musical knowledge, investigating their musical perception before and after a diagnosis of epilepsy and after a change of ASM when this was possible. Questionnaires and recordings of music were used to assess musical perception., Results: Fourteen patients had musical knowledge, and the majority of these (50%) had temporal lobe epilepsy. A total of 92.8% of the patients stated that epilepsy or its medications had affected them in the musical sphere. There was no clear relationship between the lateralisation of the epilepsy and musical involvement. 42.9% were professional musicians, and had to give up their profession. The patients prescribed with more than one ASM had greater musical involvement., Conclusions: Temporal lobe epilepsy appears to have the greatest effect on music perception, and more studies with ASM and music perception are needed to determine its effects.

Background: Perampanel (PER) is an effective adjunctive therapy for controlling focal-onset seizures (FOS), but few studies have examined its effects as an early add-on for the treatment of FOS in daily clinical practice., Methods: Our retrospective, multicenter, observational study evaluated the effectiveness and safety of PER as an early add-on in 77 patients with FOS, with and without focal to bilateral tonic-clonic seizures (FBTCS) after 3, 6 and 12 months in a real-world setting., Results: After 12 months of treatment (median dose 6 [4,8] mg/day), the retention rate was 79.2 % and 60 % of patients (39/65) experienced a ≥50 % reduction in seizure frequency relative to baseline. The seizure-free rate was 38.5 % for all seizures (25/65) and 60 % for FBTCS (12/20). The responder rate at 12 months was significantly higher when PER was given with one concomitant AED (72.2 %) compared to when PER was given with two concomitant AEDs (44.8 %). Drug-related adverse events (AEs) were reported in 40.3 % of patients, most of them being mild (64.2 %). Twelve patients (15.6 %) discontinued treatment because of AEs., Conclusions: PER is an effective and safe early add-on for patients with refractory FOS, especially for those with FBTCS., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Introduction: Dravet syndrome (DS) and Lennox-Gastaut syndrome (LGS) are two serious epileptic syndromes with paediatric onset which are refractory to therapy and are associated with an important increase in mortality rates and comorbidities compared to the general population. These pathologies have a strong impact on the lives of patients and their families, because they undergo multiple pharmacological therapies (many of them without specific indication), with frequent changes due to poor efficacy and associated adverse effects. The specialists who care for these patients highlight unmet needs and the lack of specific, safe and effective treatments for better management of the syndrome., Development: A group of four neurologists specializing in epilepsy has met to review the scientific literature and evaluate the efficacy and safety of oral solution cannabidiol in the treatment of these syndromes, both in randomized clinical trials (CT) and in some observational studies., Conclusions: Cannabidiol is positioned as an innovative therapy that allows better control of epileptic seizures and comorbidities of DS and LGS, furthermore its efficacy and safety have been evaluated in more than 700 patients. In CTs, cannabidiol significantly reduced the percentage of convulsive seizures and drop seizures compared to placebo in patients with DS and LGS respectively, which could improve their quality of life and that of their family members. The most frequent adverse effects reported were somnolence and decreased appetite. Elevated liver aminotransferase levels were also reported, especially in patients given concomitant sodium valproate. This therapy may allow better control of the epileptic seizures associated with these syndromes.

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., (Copyright © 2017 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

Background: Real-world data of current antiepileptic drugs (AEDs) used to treat focal seizures is of importance to understand the efficacy and safety outside of the clinical trial setting. Here we report real-world data from a large series of patients treated with perampanel for 1year., Methods: FYDATA was a multicentre, retrospective, 1-year observational study assessing the efficacy and safety of adjuvant perampanel in patients ≥12 years of age with focal epilepsy in a real-world setting. At 12 months, the proportion of patients who were seizure free, median percentage seizure reduction, proportion of responders, retention rate and proportion of patients with adverse events (AEs) were assessed. Analyses were also performed to identify any patient-, medication- and disease-related factors associated with a large clinical response or carry a risk for AEs., Results: A total of 464 patients were included in the study with a retention rate of 60.6% at 1year. The mean number of prior AEDs was 7.8. The median percentage reduction in overall seizures was 33.3% (75% for secondary generalised seizures) after 1year, with 7.2% of patients achieving seizure freedom. Furthermore, patients on non-enzyme-inducing AEDs were more likely to achieve seizure freedom, and logistic regression revealed that patients aged ≥65 years, those with epilepsy due to a vascular aetiology and those who had received fewer prior AEDs showed a better clinical response to perampanel. A total of 62.9% of the patients experienced AEs at 12 months; dizziness, somnolence and irritability were the most frequent AEs. Patients with prior psychiatric comorbidities (hyperactivity and personality disorder) were more likely to experience psychiatric AEs with perampanel, and slower titration schedules were associated with less AEs overall., Conclusion: Perampanel, for the treatment of focal epilepsy in a real-world setting in a refractory population, over 1year, demonstrates a similar efficacy and safety profile to that observed in clinical trials. Our results have implications for the optimisation of perampanel use in a clinical setting., (Copyright © 2016 Elsevier B.V. All rights reserved.)

Introduction: Drug-resistant epilepsy affects 25% of all epileptic patients, and quality of life decreases in these patients due to their seizures. Early detection is crucial in order to establish potential treatment alternatives and determine if the patient is a surgical candidate., Development: PubMed search for articles, recommendations published by major medical societies, and clinical practice guidelines for drug-resistant epilepsy and its medical and surgical treatment options. Evidence and recommendations are classified according to the criteria of the Oxford Centre for Evidence-Based Medicine (2001) and the European Federation of Neurological Societies (2004) for therapeutic actions., Conclusions: Identifying patients with drug-resistant epilepsy is important for optimising drug therapy. Experts recommend rational polytherapy with antiepileptic drugs to find more effective combinations with fewer adverse effects. When adequate seizure control is not achieved, a presurgical evaluation in an epilepsy referral centre is recommended. These evaluations explore how to resect the epileptogenic zone without causing functional deficits in cases in which this is feasible. If resective surgery is not achievable, palliative surgery or neurostimulation systems (including vagus nerve, trigeminal nerve, or deep brain stimulation) may be an option. Other treatment alternatives such as ketogenic diet may also be considered in selected patients., (Copyright © 2014 Sociedad Española de Neurología. Published by Elsevier España, S.L.U. All rights reserved.)

Background: Eslicarbazepine acetate (ESL) is a new antiepileptic drug (AED) licensed as adjunctive therapy in adults with partial-onset or focal seizures., Objective: To evaluate in a clinical practice setting the long-term efficacy and safety of ESL in patients with focal seizures., Methods: ESLIBASE was a retrospective study that included all patients with focal seizures who started ESL between January 2010 and July 2012 at 12 hospitals. ESL was prescribed individually according to real-life practice. Efficacy and safety were evaluated over 1 year. Switching from carbamazepine (CBZ) and oxcarbazepine (OXC) was assessed., Results: Three hundred and twenty-seven patients were included; 78% of patients were taking ≥2 other AEDs at baseline. Most (87%) began ESL because of poor seizure control and 13% because of adverse events (AEs) with CBZ or OXC. After 1 year, 237 patients (72.4%) remained on ESL. At 3, 6 and 12 months, the responder rate was 46.3%, 57.9%, and 52.5%, and 21.0%, 28.0%, and 25.3% of patients were seizure free. The responder rate significantly increased when ESL was combined with a non-sodium channel-targeting drug (non-SC drug) (66.7%) versus an SC drug (47.7%; p<0.001). At 12 months, 40.7% of patients had ≥1 AE; AEs led to treatment discontinuation in 16.2%. Dizziness, nausea, and somnolence were the most common AEs. The tolerability profile improved in >50% of the patients who switched from CBZ or OXC to ESL because of AEs., Conclusions: ESL was well tolerated and effective in a real-world setting over 1 year. Side-effect profile improved when OXC and CBZ recipients were switched to ESL., (Copyright © 2014 Elsevier B.V. All rights reserved.)

Anticonvulsant hypersensitivity syndrome is an adverse drug reaction usually occurring from 1 to 8 weeks after exposure to antiepileptic drugs. It can threaten life by affecting the liver, kidneys, central nervous system or lungs. We present a 47-year-old patient treated with phenytoin, lamotrigine and clobazam for 7 years. He presented with hepatic and renal failure in relation to this syndrome demonstrated by renal biopsy. Prognosis was excellent due to an early diagnosis leading to cessation of the causative agents. Levetiracetam was started with a good response., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

Objective: To analyze the experience in daily clinical practice of interferon-beta (IFN-beta) treatment in relapsing-remitting (RR) and secondary progressive (SP) multiple sclerosis (MS) in Galicia (Spain)., Patients and Methods: Patients with RR-MS and SP-MS treated with IFN-beta1a and 1b between 1995 and December/2000, analyzing demographic and clinical data., Results: 313 patients were included, with a mean age of 38.2 years. A total of 296 patients (94.6%) were clinically defined MS and 17 (5.4%) were laboratory supported (Poser criteria); 84.6% of the patients were RR and 15.4% were SP. The mean duration of the disease prior to treatment was 7.06 years. Betaferon was used in 52.4% patients (115 RR-MS and 47 SP-MS), Avonex in 26% and Rebif in 21.6%. Relapse rate was reduced in 68.8% for the RR-MS for Betaferon-treated patients, 73.3% for Avonex treated and 35.7% for Rebif-treated patients. Betaferon reduced relapse rate in 50% for SP-MS. The global EDSS remained stable during IFN-beta treatment. During treatment, 33% of Betaferon, 60.5% of Avonex and 54.5% of Rebif-treated patients remained relapse-free. Treatment was suspended in 12.9% of Betaferon, 6.2% of Avonex, and 3% Rebif-treated patients. The most frequent causes of treatment suspension were increase in disability and in relapse count., Conclusions: The present study supports the benefits of IFN-beta treatment in RR MS and SP MS in daily clinical practice, with reduction in relapses count and incapacity, good over-all tolerance and low incidence of serious adverse side-effects.

Introduction: Intracerebral hemorrhage (HIC) represents 10-30% of all stroke. Epidemiological studies have shown factors associated with its high mortality, but those which might lead to lower morbidity are little known., Objective: To find the factors which may influence the functional state of a series of patients with intracerebral hemorrhage., Patients and Methods: We made a prospective study of 203 patients with intracerebral hemorrhage. We recorded vascular risk factors, arterial blood pressure, laboratory and neuroimaging parameters obtained on admission. The functional condition when discharged from hospital was evaluated on the Rankin Scale, in three categories: independent (Rankin 0-1), partially dependent (Rankin 2-3) and totally dependent patients (Rankin 4-5)., Results: There was a 23.2% death rate. Of the 156 patients who survived, at the time of hospital discharge 35.8% had a score of 0-1, 50.6% a score of 2-3 and 13.4% a score of 4-5 on the Rankin Scale. Age (p < 0.005), hyperglycaemia (p < 0.05) and size of hemorrhage (p < 0.05) were associated with increased morbidity., Conclusions: The functional condition on hospital discharge was better in younger patients with lower levels of glycemia on admission and smaller hematomas on CT.

Introduction: Acute cerebrovascular disease is a serious neurological problem. Mortality is between 6% and 30%. Most studies are in agreement that advanced age, type of ictus, size of lesion and clinical deterioration are factors determining mortality. However, its relationship to vascular risk factors is not completely clear., Objective: To analyze the repercussion of different vascular risk factors on mortality during the acute phase of ictus., Patients and Methods: We studied all patients with ictus admitted to the Servicio de Neurología of the Hospital Xeral de Galicia de Santiago de Compostela over a period of 3 years (n = 915). We recorded vascular risk factors and analyzed the causes of mortality whilst in hospital., Results: Hospital mortality due to ictus was 16.8%. Mortality was 14.5% in cerebral infarct, 23.2% in intracerebral haemorrhage and 19.4% in subarachnoid haemorrhage. A total of 20.8% of the patients died of neurological causes, 24.7% of vascular causes, 26% due to infection and of uncertain causes in the remaining 28.6%. There was a neurological cause of death in 46.1% of the patients with subarachnoid haemorrhage, 25.5% with intracerebral haemorrhage and 14.8% with cerebral infarcts. Vascular risk factors associated with greater mortality were age (p < 0.001) and a history of cardiopathy (p < 0.05)., Conclusions: Vascular risk factors which indicated worse prognosis were: age, type of ictus and a history of cardiopathy.

Ulegyria is a derangement of the architecture of the cerebral cortex characterised by the presence of multiple small cerebral convolutions. It is caused by hypoxic-ischaemic damage of these convolutions in the perinatal period. This differentiates it from polimcrogyria which is due to damage during the period of late neuronal migration or early postmigration. The clinical findings vary from cases with hardly any symptoms to more severe forms which show psychomotor retardation, convulsive crises and/or motor defects. We describe the case of a 21-year-old patient who suffered anoxia during delivery, with a very low Apgar score, who presented with epileptic crises in childhood (motor foci which became generalized) with good anticonvulsant control. On examination, there was a slight mental handicap. On magnetic resonance an uligyric area was seen in the occipital cortex. High resolution magnetic resonance allows the diagnosis of this type of change in the cerebral cortex in the living patient since the differences between grey and white matter can be distinguished better than with computerized tomography.