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To describe the clinical and epidemiological characteristics of SJS/TEN in children attended at our hospital.
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Eslicarbazepine acetate (Zebenix®) is a voltage-gated sodium channel blocker approved in 2009 by the European Medicines Agency as adjunctive therapy in adults with partial-onset seizures, with or without secondary generalization.
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Subjects were 59 male epileptic patients who aged 24 ± 5 years. They had been receiving lamotrigine (LTG) (n = 17), carbamazepine (CBZ) (n = 18), and sodium valproate (VPA) (n = 15) for at least 6 months. We also recruited 23 healthy controls. Testosterone, estradiol, dehydroepiandrosterone sulfate (DHEAS), sex hormone-binding globulin (SHBG), androstenedione (AND), luteinizing hormone (LH), and follicle stimulatin hormone (FSH) levels and gonadal efficiency (testosterone/LH) were compared between the four groups. The patients and the control group were examined and evaluated for male reproduction by urology and endocrinology services.
A high-performance liquid chromatography assay with ultraviolet detection was developed for the simultaneous determination of the anti-epileptic drugs lamotrigine, carbamazepine and zonisamide in human plasma and serum. Lamotrigine, carbamazepine, zonisamide and the internal standard chloramphenicol were extracted from serum or plasma using liquid-liquid extraction under alkaline conditions into an organic solvent. The method was linear in the range 1-30 microg/mL for lamotrigine, 2-20 microg/mL for carbamazepine, and 1-40 microg/mL for zonisamide. Within- and between-run precision studies demonstrated coefficient of variation <10% at all tested concentrations. Other anti-epileptic medications tested did not interfere with the assay. The method is appropriate for determining lamotrigine, carbamazepine and zonisamide serum or plasma concentrations for therapeutic monitoring.
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564 patients with definite epilepsy entered the UK National General Practice Study of Epilepsy (NGPSE), between 1984 and 1987, and were prospectively followed up for between 11-14 years.
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A recently described SUNCT is a rare head ache syndrome characterized by shortlasting, unilateral, neuralgia-like headache attack with conjunctival injection and tearing; 26 cases of SUNCT-syndrome have been reported in medical literature. The authors observed the described symptoms in 3 cases (among 86 patients with trigeminal paroxysmal manifestations). Clinical features of SUNCT-syndrome are presented. In addition, the authors discuss possible etiologic factors and treatment as well as a differential diagnosis of SUNCT-syndrome, from trigeminal neuralgia, cluster headache, chronic paroxysmal hemicrania and combined syndromes.
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A self nanoemulsifying composition consisting of cremophor RH 40, PEG 400 and labrafil M 2125 CS was selected after screening potential excipients. The solubility of carbamazepine, a poorly water-soluble drug, was tested in the formulation components. Oil-in-water (o/w) NEs were characterized using dynamic light scattering, electrophoretic light scattering, transmission electron microscopy (TEM) and differential scanning calorimetry. NEs were nebulized into a mist using a commercial nebulizer and characterized using laser diffraction and TEM. An aseptic method was developed for preparing sterile NEs. Biocompatibility of the formulation was evaluated on NIH3T3 cells using MTT assay. In vitro permeability of the formulation was tested in zebra fish eggs, HeLa cells, and porcine lung tissue.
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The diagnosis of SCN9A-related IEM is made on clinical grounds. Pathogenic variants in SCN9A encoding the sodium channel protein Nav1.7 subunit are known to cause IEM; pathogenic variants in additional genes may also be associated with IEM.
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Agents that have potential utility in the treatment of BPII are profiled, based on their efficacy in bipolar I (BPI) or unipolar depression.
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To determine whether clinical neurologic diseases are associated with unique patterns of up- and down-regulated genes in whole blood and to explore the possibility of using peripheral blood as a surrogate tissue in these diseases.
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Both GABAergic and NMDA systems in the VH play a role in modulation of anxiety-like behavior of rats. The anxiolytic-like effects of CBZ seem to be mediated, at least in part, through an interaction with GABA(A) and NMDA systems in the VH.
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Dystonia is a syndrome of involuntary, repetitive (or sustained) muscle contractions of opposing muscles, which may result in torsions and abnormal postures. Tardive dystonia is a form of the disorder that starts after longer term use of dopamine antagonists. It occurs in approximately 3% of patients receiving ongoing antipsychotic treatment and is often difficult to reverse. Dystonia can also be induced by compounds other than antipsychotics, such as antidepressants, levodopa, carbamazepine, dextroamphetamine, and diphenylhydantoin. In these cases, it is transient, generally disappearing after the dose is reduced or the causative drug is stopped. Dystonia induced by injury can also be transient. We report a case of transient oromandibular dystonia following a dental filling in a woman receiving quetiapine, a second-generation antipsychotic. The timing, localization, and transience of the dystonia suggested that the dental procedure may have played a triggering role. The dystonia symptoms responded within 8 weeks to benztropine and a dose reduction of quetiapine, and they did not return when benztropine was discontinued. This case benefited from prompt attention and has led to practical recommendations for psychiatric clinicians.
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Lacosamide was tested in vitro on sodium and L-type calcium currents from isolated human atrial myocytes and on hERG-mediated potassium currents from stably transfected HEK293 cells. Cardiac action potentials were recorded in guinea pig ventricular myocytes. In vivo, hemodynamic and ECG parameters were evaluated in anesthetized dogs and monkeys receiving acute cumulative intravenous doses of lacosamide.
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We report the clinical findings and the management of an 8-year-old boy with this disorder. The molecular findings confirm that the patient is a compound heterozygote with a different splicing mutation in each Perlecan allele. This resulted in a significant reduction in the production of the encoded normal protein.
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Objective assessment of non-overt neurotoxicity of carbamazepine (CBZ) vs oxcarbazepine (OXC) in patients with difficult-to-treat partial epilepsy, who were resistant to CBZ treatment and were converted from CBZ monotherapy to OXC monotherapy.
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The frequency of polycystic ovaries or hyperandrogenism, or both, among valproate-treated women with epilepsy was 70% (26 of 37) compared with 19% (10 of 52) among control subjects (P <0.001). They were found in 79% (11 of 14) of obese and 65% (15 of 23) of lean women on valproate, and in 20% (7 of 35) of carbamazepine-treated women. The obese valproate-treated women with polycystic ovaries or hyperandrogenism, or both, had hyperinsulinemia and associated unfavorable changes in serum lipid levels consistent with insulin resistance.
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This report describes a retrospective cohort study of 108 patients who were treated with eslicarbazepine. Of these patients, 52% had switched to eslicarbazepine from prior treatment with carbamazepine or oxcarbazepine. Laboratory values concerning lipid metabolism profile, liver function tests and sodium were assessed before and after beginning/switching treatment. Patients who began treatment or whose treatment for dyslipidemia was modified during the study period were excluded from the analysis. Co-medications that could impact lipid metabolism profile, sodium or hepatic function were kept stable during the study period.
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The dataset of the large population-based Hungarian Case-Control Surveillance of Congenital Abnormalities, 1980-1996, was evaluated.
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Although increasingly recognised, ring chromosome 20 (r) syndrome is still diagnosed with delay, sometimes leading to inappropriate presurgical evaluation. The focal, presumed frontal, character of the seizures manifesting with fear and hypermotor behaviour and episodes of non-convulsive status epilepticus (NCSE) are most typical, as well as cognitive impairment with behavioural problems and, sometimes, dysmorphic signs. We present a girl diagnosed at the age of 13 years who suffered from an atypical clinical presentation, with minimal cognitive problems, absence of dysmorphic symptoms, and hypermotor/gelastic seizures. [Published with video sequences].
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Drugs with narrow therapeutic index (NTI-drugs) are drugs with small differences between therapeutic and toxic doses. The pattern of drug-related problems (DRPs) associated with these drugs has not been explored.
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Around one of three diabetic patients is affected by distal symmetric polyneuropathy (DSP) which represents a major health problem, as it may present with partly excruciating neuropathic pain and is responsible for substantial morbidity and increased mortality. Treatment is based on four cornerstones: (1) multifactorial intervention aimed at (near)-normoglycaemia and reduction in cardiovascular risk factors, (2) treatment based on pathogenetic mechanisms, (3) symptomatic treatment, and (4) avoidance of risk factors and complications. Among the pathogenetic treatments only alpha-lipoic acid and epalrestat are available for treatment in several countries. Neuropathic pain, which is present in 8-26% of diabetic patients, exerts a substantial impact on the quality of life, particularly by causing considerable interference in sleep and enjoyment of life. Non-pharmacologic options such as nerve or muscle stimulation should always be given consideration. Among the centrally acting analgesic drugs for many years mainly the tricyclic antidepressants (TCA), carbamazepine, gabapentin, and opioids have been used to treat neuropathic pain. More recently, significant pain relief has been reported in clinical trials of painful diabetic neuropathy using agents such as the dual selective serotonin noradrenaline reuptake inhibitor (SNRI), duloxetine and the anticonvulsant pregabalin, a specific modulator of the alpha(2)delta subunit of the voltage-dependent calcium channels. A promising new anticonvulsant is lacosamide. In future, drug combinations might also include those aimed at symptomatic pain relief and quality of life on one hand and improvement or slowing the progression of the underlying neuropathic process on the other hand.
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Three drugs, valproate, lamotrigine, and carbamazepine, have strong evidence-based support for use in clinical states of bipolar disorder. Other anticonvulsant drugs investigated in bipolar disorder either have evidence of lack of benefits in bipolar disorder or have been inadequately studied to determine possible effectiveness.