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Showing posts with label Dravet syndrome. Show all posts
Showing posts with label Dravet syndrome. Show all posts

Wednesday, November 3, 2010

Vagus nerve stimulation: effectiveness and tolerability in patients with epileptic encephalopathies.

Childs Nerv Syst. 2010 Oct 31. [Epub ahead of print]

Vagus nerve stimulation: effectiveness and tolerability in patients with epileptic encephalopathies.

Neurology Department, Hospital de Niños "Prof. Dr. Juan P Garrahan", Combate de los Pozos 1881, Buenos Aires, CP 1245, Argentina.

Abstract

PURPOSE: We discuss the effectiveness, tolerability, and safety of vagus nerve stimulation (VNS) as adjunctive therapy in 26 patients with refractory epileptic encephalopathies (EEs).
MATERIAL AND METHODS: Twenty-six patients (17 male and 9 female) with electroclinical features compatible with Lennox-Gastaut syndrome (LGS) in 20 patients, Dravet syndrome (DS) in 3 patients, and epilepsy with myoclonic-astatic seizures (EMAS) in 3 patients implanted with the NCP system were analyzed.
RESULTS: In our series of patients with LGS, 17 cases showed a significant improvement in seizure control, with a reduction in seizure frequency of at least 50%. Seven of them previously had epileptic spasms. Three patients with EMAS and two patients with DS showed a significant improvement in seizure control, with a reduction in seizure frequency of at least 50%. A good clinical response was evident early and efficacy progressively improved with the duration of treatment up to 36 months. In patients who had a reduction in seizure frequency of at least 50%, quality of life (QOL) and neuropsychological performance improved. VNS was well-tolerated in all patients.
CONCLUSION: VNS is an effective and well-tolerated treatment for patients with epileptic encephalopathies EEs, improving QOL and neuropsychological performance.
PMID: 21038079 [PubMed - as supplied by publisher

Tuesday, October 26, 2010

Clinical course of young patients with Dravet syndrome after vagal nerve stimulation.

Eur J Paediatr Neurol. 2010 Oct 22. [Epub ahead of print]

Clinical course of young patients with Dravet syndrome after vagal nerve stimulation.

Pediatric Neurology Department, Ospedali Riuniti, Ancona, Italy.

Abstract

Medical treatment of Dravet syndrome is disappointing. Ketogenic Diet and neurostimulation procedures as Vagus Nerve Stimulation (VNS) and Deep Brain Stimulation are in ongoing evaluation. In the present study, the long-term effectiveness of VNS on seizures, cognition and behavior was retrospectively evaluated in eight young patients with DS and medically refractory epilepsy (mean age at VNS implant: 10.28 years, range: 5-25). The average duration of treatment was 54 months (range: 12-120). Compared to baseline (mean: 55; standard deviation: 83, range: 4-200), the mean number of monthly seizures after VNS implantation was 39 ± 67 at 3 months, 42 ± 67 at 6 months and 38 ± 69 at twelve months (not significant comparisons). In particular, VNS produced a mean seizure rate reduction of 12% at three months, 6% at six months, and 31% at twelve months. All patients but three experienced some reduction in seizure burden (range: 33-61%) at twelve months. Seizure outcome after one year of stimulation was rated as Mc Hugh class II (50-79% reduction in seizure frequency) in four patients, class III (<50% reduction) in one patient and class V (no improvement) in three patients. In this small case series of patients with DS, VNS therapy had a clinically significant effect in reducing seizures at twelve months in four of the eight patients. Even in patients in whom seizure reduction was not dramatic, a slight improvement in alertness and communicative skills was seen. The long-term clinical course of two selected cases is discussed.
Copyright © 2010 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.
PMID: 20971664 [PubMed - as supplied by publisher