Infantile Spasms and Lennox-Gastaut Syndrome

Infantile spasms and Lennox-Gastaut syndrome are rare but are important to child neurologists because of the intractable nature of the seizures and the serious neurologic comorbidities. New antiepileptic drugs offer more alternatives for treating both infantile spasms and Lennox-Gastaut syndrome. Selected children with infantile spasms are candidates for epilepsy surgery. Vagus nerve stimulation, corpus callosotomy, and the ketogenic diet are all options for selected children with Lennox-Gastaut syndrome. The epidemiology, clinical manifestations of the seizures, electroencephalographic characteristics, prognosis, and treatment options are reviewed for infantile spasms and Lennox-Gastaut syndrome. Additional therapies are needed for both infantile spasms and Lennox-Gastaut syndrome as many children fail to achieve adequate seizure control in spite of newer treatments. (J Child Neurol 2002;17:2S9—2S22).

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Effects of Vagus Nerve Stimulation Following Corpus Callosotomy for Patients with Drug-Resistant Epilepsy

Brain Sciences ◽

10.3390/brainsci11111395 ◽

2021 ◽

Vol 11 (11) ◽

pp. 1395

Author(s):

Keisuke Hatano ◽

Ayataka Fujimoto ◽

Takamichi Yamamoto ◽

Hideo Enoki ◽

Tohru Okanishi

Keyword(s):

Vagus Nerve ◽

Seizure Frequency ◽

Nerve Stimulation ◽

Vagus Nerve Stimulation ◽

Seizure Control ◽

Reduction Rate ◽

Corpus Callosotomy ◽

Seizure Reduction ◽

Drug Resistant Epilepsy ◽

Univariate Analyses

Objective: The effectiveness of vagus nerve stimulation (VNS) for residual seizures after corpus callosotomy (CC) has not yet been fully investigated. We hypothesized that seizure control would be improved by VNS after CC. The purpose of this study was to compare seizure frequency between patients with implantation of a VNS generator (post-VNS group) or without VNS (non-post-VNS group) following CC. Methods: We retrospectively reviewed patients who underwent CC between January 2009 and May 2019 in our institution. We evaluated proportions of ≥50% reduction in seizure frequency (responders) and seizure reduction rate 1 and 2 years after VNS. To investigate factors related to responders, uni- and multivariate logistic regression analyses were performed regarding age, number of anti-seizure medications (ASMs), addition of novel ASMs (levetiracetam, lacosamide or perampanel), and post-VNS or non-post-VNS status. Results: Thirteen post-VNS patients and 24 non-post-VNS patients were analyzed in this study. Responder rate at 1 year after VNS differed significantly between the post-VNS group (53.9%) and non-post-VNS group (12.5%, p = 0.017). Number of ASMs at the time of CC and post-VNS were significantly associated with responders in univariate analyses (odds ratio [OR] 0.34, 95% confidence interval [CI] 0.13–0.88, p = 0.025 and OR 8.2, 95%CI 1.6–41.6, p = 0.011, respectively), whereas age, sex, seizure frequency, and addition of novel ASMs were not. In multivariate analysis, the presence of VNS procedures after CC was the only factor favorably associated with responder status (OR 82.2, 95%CI 1.55–4355.7, p = 0.03). Conclusions: VNS therapy after CC may increase the proportion of responders independent of the addition of novel ASMs.

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Vagus Nerve Stimulation in Intractable Epilepsy Associated With SCN1A Gene Abnormalities

Journal of Child Neurology ◽

10.1177/0883073816687221 ◽

2017 ◽

Vol 32 (5) ◽

pp. 494-498 ◽

Cited By ~ 11

Author(s):

Stephen P. Fulton ◽

Kate Van Poppel ◽

Amy L. McGregor ◽

Basanagoud Mudigoudar ◽

James W. Wheless

Keyword(s):

Vagus Nerve ◽

Nerve Stimulation ◽

Vagus Nerve Stimulation ◽

Seizure Control ◽

Intractable Epilepsy ◽

Myoclonic Epilepsy ◽

Dravet Syndrome ◽

Severe Myoclonic Epilepsy ◽

Scn1a Gene ◽

Myoclonic Epilepsy Of Infancy

Mutations in the SCN1A gene cause a spectrum of epilepsy syndromes. There are 2 syndromes that are on the severe end of this spectrum. The classic severe form, Dravet syndrome, is an epileptic encephalopathy of childhood, causing cognitive decline as well as intractable seizures. Severe Myoclonic Epilepsy of Infancy–Borderline (SMEIB) is a term used to include cases with similar severities as those with Dravet syndrome, but lacking a single feature of classic severe myoclonic epilepsy of infancy. Vagus nerve stimulation is a nonpharmacologic treatment for intractable epilepsy. A retrospective review was conducted of patients with deleterious SCN1A mutations who had vagus nerve stimulation placement for treatment of their intractable epilepsy. These children had onset of their epilepsy between 3 and 29 months of age. Seizure control was assessed 6 months after implantation. Twenty patients are included in the study, with 12 implanted at our institution. Nine of the 12 patients implanted at our institution, who had confirmed pre- and post-implantation seizure assessments, showed improvement in seizure control, which was defined as >50% reduction in generalized tonic-clonic seizures, and 4 of those 12 reported improvement in cognitive or speech development. Seven of the 8 patients not implanted at our institution reported subjective benefit, with 4 relating “marked improvement” or seizure freedom. Vagus nerve stimulation appears to impart a benefit to children with deleterious SCN1A gene abnormalities associated with intractable epilepsy.

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Vagus nerve stimulation – Mechanism of action and usefulness of its combination with corpus callosotomy for palliation of refractory epilepsy

International Journal of Epilepsy ◽

10.1016/j.ijep.2015.12.003 ◽

2016 ◽

Vol 3 (1) ◽

pp. 42-43

Author(s):

Amami Kato ◽

Naoki Nakano ◽

Haruhiko Kishima ◽

Toshiki Yoshimine

Keyword(s):

Vagus Nerve ◽

Mechanism Of Action ◽

Nerve Stimulation ◽

Vagus Nerve Stimulation ◽

Refractory Epilepsy ◽

Corpus Callosotomy

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Vagus Nerve Stimulation and Lennox-Gastaut Syndrome: A Review of the Literature and Data From the VNS Patient Registry

CNS Spectrums ◽

10.1017/s1092852900001516 ◽

2001 ◽

Vol 6 (9) ◽

pp. 766-770 ◽

Cited By ~ 36

Author(s):

Steven Karceski

Keyword(s):

Vagus Nerve ◽

Nerve Stimulation ◽

Vagus Nerve Stimulation ◽

Medical Literature ◽

Severe Form ◽

Patient Registry ◽

Review Of The Literature ◽

Corpus Callosotomy ◽

Appropriate Treatment ◽

Surgical Options

ABSTRACTLennox-Gastaut syndrome (LGS) is a severe form of childhood epilepsy that is usually refractory to medical management. When medications fail, alternative therapies are considered. Among these are two surgical options: corpus callosotomy and vagus nerve stimulation (VNS). Safety and efficacy are two important factors to consider when selecting an appropriate treatment. VNS is safer than callosotomy, but its efficacy is more difficult to assess. Available studies evaluate its effectiveness using a mixed population of patients (some with prior epilepsy surgery), a multitude of VNS settings, and variable endpoints. To estimate the efficacy of VNS in patients with LGS, a review of the medical literature and the VNS Patient Registry was performed. Within the limits of this type of study, the results showed that VNS appears equally as effective as callosotomy. Because VNS has a lower potential for adverse events, these results suggest that VNS should be considered first in appropriately selected patient.

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Management of Epilepsy in Mentally Retarded Children Using the Newer Antiepileptic Drugs, Vagus Nerve Stimulation, and Surgery

Journal of Child Neurology ◽

10.1177/088307380401900107 ◽

2004 ◽

Vol 19 (1S) ◽

pp. S58-S64 ◽

Cited By ~ 5

Author(s):

W. Donald Shields

Keyword(s):

Vagus Nerve ◽

Antiepileptic Drugs ◽

Nerve Stimulation ◽

Vagus Nerve Stimulation ◽

Mentally Retarded ◽

Mentally Retarded Children

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Microsurgical endoscopy-assisted anterior corpus callosotomy for drug-resistant epilepsy in an adult unresponsive to vagus nerve stimulation

Epilepsy & Behavior Case Reports ◽

10.1016/j.ebcr.2016.01.001 ◽

2016 ◽

Vol 5 ◽

pp. 27-30 ◽

Cited By ~ 2

Author(s):

Davide Nasi ◽

Maurizio Iacoangeli ◽

Lucia Di Somma ◽

Mauro Dobran ◽

Alessandro Di Rienzo ◽

...

Keyword(s):

Vagus Nerve ◽

Nerve Stimulation ◽

Vagus Nerve Stimulation ◽

Drug Resistant ◽

Corpus Callosotomy ◽

Drug Resistant Epilepsy

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Forbedring av rutiner rundt pasienter med vagusnervestimulator

Nordisk tidsskrift for helseforskning ◽

10.7557/14.3330 ◽

2015 ◽

Vol 10 (2) ◽

pp. 151

Author(s):

Christine Tørris ◽

Kari Helen Schøyen ◽

Elin Merete Wiklund

Keyword(s):

Vagus Nerve ◽

Antiepileptic Drugs ◽

Nerve Stimulation ◽

Vagus Nerve Stimulation ◽

Additional Dose ◽

Nerve Stimulator ◽

Vagus Nerve Stimulator ◽

Before And After ◽

Storage Area ◽

And Storage

Improving routines for patients with vagus nerve stimulationBackground: Vagus Nerve Stimulator (VNS) is a form of therapy which may reduce the frequency of seizures in people with epilepsy, who do not achieve remission with antiepileptic drugs. A corresponding magnet providing an additional dose of stimulation may stop or shorten the intensity of the seizure.Objective Investigate the practice and procedure regarding use and storage of a VNS Therapy magnet, to see whether introduction of a new routine with a permanent seat for VNS magnet may contribute to increased availability of the magnet.Method Registration of the magnets location was performed before, and after the magnetic board intervention. In addition staff answered a questionnaire related to experiences around storage of magnet.Results The number of not located magnets dropped from 70% to 25 % after the intervention.Conclusion A magnetic board as a storage area for VNS magnet on the patient's room increases the availability of the magnet.

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The effectiveness of vagus nerve stimulation after failed surgical treatment of pharmacoresistant epilepsy

Vestnik nevrologii, psihiatrii i nejrohirurgii (Bulletin of Neurology, Psychiatry and Neurosurgery) ◽

10.33920/med-01-2101-02 ◽

2021 ◽

pp. 19-35

Author(s):

Igor Trifonov ◽

Mikhail Sinkin ◽

Alexandra Nekhoroshikh

Keyword(s):

Surgical Treatment ◽

Vagus Nerve ◽

Antiepileptic Drugs ◽

Nerve Stimulation ◽

Alternative Treatment ◽

Vagus Nerve Stimulation ◽

Pharmacoresistant Epilepsy ◽

Wide Range ◽

The World ◽

Promising Type

According to the WHO, about 50 million people in the world suffer from epilepsy, while 20–40 % are resistant to antiepileptic drugs and need alternative treatment methods. The arsenal of neurosurgeons includes a wide range of operations that have proven effectiveness in the fight against pharmacoresistant epilepsy. Such a promising type of treatment as vagus nerve stimulation can be used both with contraindicated resection surgery and after it. Currently, the field of VNS therapy continues to be explored, including the efficiency and rationality of its use.

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