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Vagus nerve stimulation (VNS) is effective in treating catastrophic 1 epilepsy in very young children

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The objective of this study is to evaluate the safety and efficacy of vagus nerve stimulation (VNS) in very young children suffering from catastrophic epilepsy and status epilepticus. We reviewed files of 60 VNS-implanted children at our institution and we selected six very young patients, less than 3 years old (mean age at implant 1.6 years). All patients suffered from severe cognitive impairment and catastrophic epilepsy with underlying diagnosis of hemimegalencephaly (1), hypoxic-ischemic encephalopathy (1), tuberous sclerosis complex (1), and malignant migrating partial epilepsy of infancy (3). Three patients were VNS-implanted during admission at intensive care unit (ICU) after developing life-threatening status epilepticus. The mean follow-up time was 41.6 months. The VNS was implanted using a single cervical incision. No surgery-related complications were observed. Four of six children have shown a significant, persistent improvement in seizure control (range, 60–90%). In patients with status, insertion of the vagal nerve stimulator allowed early cessation of status and discharge from ICU. Quality of life and parental satisfaction improved and for three children there was some milestone evolution. Catastrophic epilepsy in infancy can be devastating and difficult to treat with drugs and surgery. If resective surgery is inappropriate or refused, VNS can be considered as a well-tolerated and effective procedure even in toddlers affected by severe epilepsy and multiple developmental disabilities.

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Correspondence to Nelia Zamponi.

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Dominik Zumsteg and Heinz Gregor Wieser, Zürich, Switzerland

Vagus nerve stimulation (VNS) was the first adjunctive treatment for epilepsy using electrical stimulation that has been approved by the FDA in 1997 for the treatment of medically intractable epilepsy in patients over age 12. The VNS device provides chronic intermittent stimulation to the left vagus nerve through a surgically implanted circumneural bipolar cuff electrode. Premarketing trials have shown that 30 to 40% of individuals with medically intractable partial seizures had a significant improvement, defined as a seizure reduction of ≥50% [1]. Postmarketing experience suggested that the device may also be effective in the treatment of generalized seizures [2]. The initiation of extra cycles by the patient by placing a magnet over the device has been shown to abort or ameliorate seizures in about a third [3].

However, with a decade experience in VNS, we know that the level of effectiveness is moderate, leaving many patients with frequent seizures: The device generally reduces rather than eliminates seizures. The experience with VNS in very young children, particularly in children younger than 5 years, is limited. There are only a few uncontrolled studies based on small cohorts, suggesting that VNS may also be safe and moderately effective in the very young [4, 5]. In this article, Zamponi et al. again report on the safety and efficacy of VNS in a small cohort of very young children suffering from catastrophic epilepsy with frequent seizures and severe psychomotor retardation. There were no surgery-related complications. The authors found a 60 to 90% seizure reduction in four of six patients, and a 4-month seizure-free period in a 2.7-year-old girl, subsequent to a 40% seizure reduction during the early course of treatment (however, it is pertinent to note that some of these effects may also have been related to modifications of antiepileptic drug treatment during the study period). Quality of life and parental satisfaction is said to have improved (as assessed by an analog scale), but this improvement was not significant when using the standardized Vineland Adaptive Behavior Scale for quality of life evaluation. Considering that complete seizure control is a key predictor of improved quality of life, the reductions in seizure frequency may not have been great enough to affect quality of life.

Now, does a moderate level of VNS effectiveness affect quality of life in a way that would justify the surgical procedure in toddlers? Would it be good enough for you, if you had seizures? Shouldn’t improvement with VNS be much better than that found with medications to justify the increased risk (and costs) of an invasive procedure? There may be no definite general answers to these questions. What we do know is that VNS in patients with epilepsy, irrespective of age, appears to be less efficacious than had been hoped. Moreover, it is important to bear in mind that surgical sham control in VNS studies is a priori precluded due to unavoidable though usually mild to moderate side effects with VNS, such as hoarseness, discomfort in the throat, cough or swallowing difficulties. In that sense, the investigation of VNS in toddlers is interesting, considering that there is hardly any placebo effect to be expected in very young children. Nevertheless, to truly ascertain how much of any benefit is related to VNS, it would be very informative to know the actual effectiveness of VNS in those 1,450 patients aged 0 to 5 years implanted with the device (the number 1,450 is based on the implant registration cards returned to Cyberonics, mentioned by the authors in this issue). There is not much hope considering the publication bias (that is, the phenomenon that studies with positive results are more likely to be published than studies with negative results).


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3. Morris GL III (2003) A retrospective analysis of the effects of magnet-activated stimulation in conjunction with vagus nerve stimulation therapy. Epilepsy Behav 4:740–745

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Zamponi, N., Rychlicki, F., Corpaci, L. et al. Vagus nerve stimulation (VNS) is effective in treating catastrophic 1 epilepsy in very young children. Neurosurg Rev 31, 291–297 (2008).

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