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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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]