[A boy with treatment-resistant psychiatric problems: X-linked adrenoleukodystrophy]. / Een adolescent met psychiatrische problemen die niet reageerden op behandeling: X-gebonden adrenoleukodystrofie.

In this case report we describe a 17-year-old boy with severe behavioural disorders, apathy and cognitive decline who was eventually diagnosed with X-linked adrenoleukodystrophy (x-ald). If a patient presents with a combination of psychiatric and neurological problems, metabolic diseases such as x-ald should be included in the differential diagnosis.

Cardiac responses of vagus nerve stimulation: intraoperative bradycardia and subsequent chronic stimulation.

OBJECTIVES: Few adverse events on heart rate have been reported with vagus nerve stimulation (VNS) for refractory epilepsy. We describe three cases with intraoperative bradycardia during device testing. PATIENTS AND METHODS: At our hospital 111 patients have received a VNS system. Intraoperative device testing is performed under ECG-monitoring. We reviewed the patients and their VNS-therapy follow-up outcome who experienced a change in heart rate, during device testing (Lead Test). RESULTS: Three patients with medically refractory epilepsy showed a bradycardia during intraoperative Lead Test. Postoperative the VNS-therapy started under ECG-monitoring. No change in cardiac rhythm occurred. Subsequent chronic stimulation is uneventful. All three have reduced seizure frequency. Two already have had their second implant, without the occurrence of bradycardia. CONCLUSION: In case of intraoperative bradycardia VNS-therapy onset should be done under ECG-monitoring. Subsequent chronic stimulation is safe in respect to heart rate. Bradycardia during intraoperative device testing is no reason to abort the operation.

Vagus nerve stimulation for medically refractory epilepsy: a long-term follow-up study.

INTRODUCTION: Vagus nerve stimulation (VNS) is thought to have a cumulative effect in time on seizure frequency reduction. There also might be other variables than reduction of seizure frequency in order to determine VNS efficacy. In this study we describe the long-term outcome of the first group of vagus nerve stimulation patients with pharmacoresistant epilepsy at the Medisch Spectrum Twente, The Netherlands. METHODS: This long-term descriptive prospective study included 19 patients, 11 males and 8 females, aged 17-46 years with pharmacoresistant epilepsy. They had received 3-16 (mean 9) different anti-epileptic drugs and were not eligible for surgical resection of an epileptic focus. A vagus nerve stimulator was implanted in the period April 1999-October 2001. Follow-up ranges from 2 to 6 years (mean 4 years). Efficacy was measured as the percentage change in seizure rate during 1 year and then after each year follow-up of VNS compared to 5 months baseline before implantation. RESULTS: Mean seizure reduction at 1-6 years was, respectively, 14% (n = 19), 25% (n =1 9), 29% (n = 16), 29% (n = 15), 43% (n = 9) and 50% (n = 7). Because of VNS two patients were able to start living without supervision. One patient died after 2 years of follow-up possibly as a result of SUDEP. Four patients had no apparent reduction in seizure frequency. Two of them had their stimulator removed. The other two patients however had significantly reduced post-ictal periods and seizure time and received a new pulse generator when the battery was depleted. One stimulator was switched off due to adverse effects, even though there was a positive effect on his seizure reduction. In six patients the medication regimen was changed during VNS by adding one anti-epileptic drug, however without significant change in seizure reduction. Adverse effects were hoarseness and coughing during stimulation. One patient had a temporary paralysis of his left vocal cord. CONCLUSION: We think that VNS is an effective treatment for pharmacoresistant epilepsy and its positive effect persists during the years of follow-up. Our results suggest that seizure reduction should not be considered as the only variable of importance to describe the outcome of VNS on epilepsy and it is worthwhile to look at other outcome measures.

[Vagus nerve stimulation in patients with drug-resistant epilepsy]. / N.-vagusstimulatie bij patiënten met medicatieresistente epilepsie.

OBJECTIVE: To describe the mechanism and first results of vagus nerve stimulation at the Medisch Spectrum Twenty, the Netherlands, for the treatment of patients with drug-resistant epilepsy. DESIGN: Descriptive retrospective. METHOD: Fifteen patients, 8 male and 7 female, aged 18-45 years with drug-resistant epilepsy, who were not eligible for surgical resection of an epileptic focus, received a vagus nerve stimulator implant in the period April 1999-December 2000. Whilst the vagus nerve stimulator was being adjusted, the medication remained unchanged. RESULTS: Due to vagus nerve stimulation the mean seizure frequency decreased by 32% (range: +20% to -100%). In 6 patients there was a strong reduction in seizure frequency, in 3 there was a mild reduction, and in 6 patients there was no apparent effect. The most common adverse events during stimulation were a mild prickly cough and a change of voice during stimulation. In one patient a temporary left vocal cord paralysis was observed, which may possibly have been a result of the procedure. CONCLUSION: Vagus nerve stimulation is an effective means of treatment for drug-resistant epilepsy and is therefore a treatment option that deserves more attention in the Netherlands.