2008NeuropediatricsRequires access

Rufinamide treatment in 18 patients with Lennox-Gastaut syndrome – Documentation with the electronic patient diary epivista®

C. Reutlinger, JA Baudhuin, Claudia Dreiwes, Ulrich Stephani, Rainer Boor

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Abstract

Lennox-Gastaut syndrome (LGS) is one of the hard-to-treat epilepsies among children. Rufinamide is a new drug which has been used as an add-on medication for the treatment of LGS in children aged 4 and older since its approval in June of 2007. We treated 18 LGS patients with rufinamide as add-on medication. Documentation of baseline, course of disease and undesired effects was carried out using the electronic patient diary Epivista®. Looking at all patients grouped by the various types of seizure, we found that tonic seizures were reduced best of all. Serial tonic seizures ceased completely in 6 patients after introduction of rufinamide. Singular tonic seizures were reduced by more than 90% in 9 of 13 patients (69%). Partial reduction of generalized tonic-clonic, complex partial and atypical absences was also observed. The final doses administered were between 14 and 40mg/kg body mass, except in one patient in whom seizures were already significantly reduced at a dose of 5mg/kg which couldn't be increased further due to undesired effects. The observed undesired effects were typically nausea, to some extent also vomiting and weight-loss in some. Nausea was typically observed in the up-titration phase and improved in time. Slower up-titration, however, generally helped to avoid the occurrence of nausea. In two patients, treatment had to be terminated due to excessive vomiting; in one other, due to skin rash. Thus, in summary, rufinamide represents a useful additional drug which can be used in the treatment of patients with LGS and especially in those patients with tonic seizures and who exhibit typical EEG changes. Specially this could be shown in long time treatment by documentation using the electronic patient diary Epivista®.

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What this paper is about

Lennox-Gastaut syndrome (LGS) is one of the hard-to-treat epilepsies among children. Rufinamide is a new drug which has been used as an add-on medication for the treatment of LGS in children aged 4 and older since its approval in June of 2007. We treated 18 LGS patients with rufinamide as add-on medication. Documentation of baseline, course of disease and undesired effects was carried out using the electronic patient diary Epivista®. Looking at all patients grouped by the various types of seizure, we found that tonic seizures were reduced best of all. Serial tonic seizures ceased completely in 6 patients after introduction of rufinamide. Singular tonic seizures were reduced by more than 90% in 9 of 13 patients (69%). Partial reduction of generalized tonic-clonic, complex partial and atypical absences was also observed. The final doses administered were between 14 and 40mg/kg body mass, except in one patient in whom seizures were already significantly reduced at a dose of 5mg/kg which couldn't be increased further due to undesired effects. The observed undesired effects were typically nausea, to some extent also vomiting and weight-loss in some. Nausea was typically observed in the up-titration phase and improved in time. Slower up-titration, however, generally helped to avoid the occurrence of nausea. In two patients, treatment had to be terminated due to excessive vomiting; in one other, due to skin rash. Thus, in summary, rufinamide represents a useful additional drug which can be used in the treatment of patients with LGS and especially in those patients with tonic seizures and who exhibit typical EEG changes. Specially this could be shown in long time treatment by documentation using the electronic patient diary Epivista®.

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Available abstract

Lennox-Gastaut syndrome (LGS) is one of the hard-to-treat epilepsies among children. Rufinamide is a new drug which has been used as an add-on medication for the treatment of LGS in children aged 4 and older since its approval in June of 2007. We treated 18 LGS patients with rufinamide as add-on medication. Documentation of baseline, course of disease and undesired effects was carried out using the electronic patient diary Epivista®. Looking at all patients grouped by the various types of seizure, we found that tonic seizures were reduced best of all. Serial tonic seizures ceased completely in 6 patients after introduction of rufinamide. Singular tonic seizures were reduced by more than 90% in 9 of 13 patients (69%). Partial reduction of generalized tonic-clonic, complex partial and atypical absences was also observed. The final doses administered were between 14 and 40mg/kg body mass, except in one patient in whom seizures were already significantly reduced at a dose of 5mg/kg which couldn't be increased further due to undesired effects. The observed undesired effects were typically nausea, to some extent also vomiting and weight-loss in some. Nausea was typically observed in the up-titration phase and improved in time. Slower up-titration, however, generally helped to avoid the occurrence of nausea. In two patients, treatment had to be terminated due to excessive vomiting; in one other, due to skin rash. Thus, in summary, rufinamide represents a useful additional drug which can be used in the treatment of patients with LGS and especially in those patients with tonic seizures and who exhibit typical EEG changes. Specially this could be shown in long time treatment by documentation using the electronic patient diary Epivista®.

Key concepts: Lennox–Gastaut syndrome, Medicine, Pediatrics, Documentation, Epilepsy, Psychiatry, Computer science, Programming language

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