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Table of Contents    
Year : 2021  |  Volume : 69  |  Issue : 5  |  Page : 1374-1379

A Role for Electroconvulsive Therapy in the Management of New Onset Refractory Status Epilepticus (NORSE) in a Young Child

1 Cohen Children's Medical Center, New York; Zucker School of Medicine at Hofstra/Northwell, USA
2 Zucker Hillside Hospital, Northwell Health; Zucker School of Medicine at Hofstra/Northwell, USA

Date of Submission23-Nov-2020
Date of Decision31-Mar-2021
Date of Acceptance12-Jul-2021
Date of Web Publication30-Oct-2021

Correspondence Address:
Sanjeev V Kothare
Director, Division of Pediatric Neurology, Professor of Pediatrics & Neurology at Zucker School of Medicine at Hofstra/Northwell, Senior Epileptologist, Co-Director, Pediatric Sleep Program (Neurology); Department of Pediatrics, Cohen Children's Medical Center, 2001, Marcus Avenue, Suite W290, Lake Success, NY 11042
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.329559

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 » Abstract 

New-onset refractory status epilepticus (NORSE) describes prolonged or recurring new onset seizures which fail to respond to antiseizure medications. NORSE poses a challenge in diagnosis and treatment, and limited high-quality evidence exists to guide management. The efficacy of Electroconvulsive therapy (ECT) in aborting refractory status epilepticus has been described in case reports, but its application remains uncommon, particularly in young children. We describe a case of NORSE in a 3-year old child in which ECT played an important role in aborting status epilepticus, facilitating the diagnosis and surgical excision of an underlying focal cortical dysplasia. Although further research is needed, our case suggests that ECT can be a valuable tool in the treatment of refractory status epilepticus in children.

Keywords: Electroconvulsive therapy (ECT), pediatric, refractory status epilepticus
Key Message: Status epilepticus is a life-threatening condition with a high mortality and morbidity rate in spite of aggressive treatment. Electroconvulsive therapy can be a safe, effective and reasonable alternative in the treatment of refractory status epilepticus in pediatric patients after exhausting conventional therapies have been failed.

How to cite this article:
Nath M, Shah YD, Theroux LM, Petrides G, Karkare S, Sanghani SN, Kothare SV. A Role for Electroconvulsive Therapy in the Management of New Onset Refractory Status Epilepticus (NORSE) in a Young Child. Neurol India 2021;69:1374-9

How to cite this URL:
Nath M, Shah YD, Theroux LM, Petrides G, Karkare S, Sanghani SN, Kothare SV. A Role for Electroconvulsive Therapy in the Management of New Onset Refractory Status Epilepticus (NORSE) in a Young Child. Neurol India [serial online] 2021 [cited 2022 May 26];69:1374-9. Available from: https://www.neurologyindia.com/text.asp?2021/69/5/1374/329559

New-onset refractory status epilepticus (NORSE) describes the clinical presentation of status epilepticus which fails to respond to treatment with a benzodiazepine and one additional antiseizure medication, in a patient without known epilepsy or other neurologic disorder, and in the absence of an apparent acute structural, toxic, or metabolic etiology.[1] NORSE frequently does not respond to conventional treatment protocols for status epilepticus and may persist for weeks or months, with no definitive diagnosis of the underlying cause in approximately 50% of cases.[1] Please refer to Other 1 which details treatment strategies in NORSE. When benzodiazepines, antiseizure medications, and anesthetic infusions fail, alternative therapies are pursued, including immunotherapy, ketamine, ketogenic diet, and electroconvulsive therapy (ECT).[1] Multiple case reports have described the use of ECT for refractory status epilepticus.[2],[3],[4] Although ECT is an accepted treatment modality in psychiatry, including for adolescents, reports of its use for status epilepticus in children are rare.[5],[6],[7],[8],[9]

Earlier control of status epilepticus is associated with improved outcomes, and seizure control is the initial priority.[1] However, in some cases, determination of the underlying cause of seizures is essential to guide treatment.[1],[4] Frequently, ongoing seizures themselves hinder the diagnostic evaluation, as they limit the safety and feasibility of ancillary testing. We report a case of a previously healthy 3-year-old child with refractory focal status epilepticus consistent with NORSE and unremarkable head magnetic resonance imaging (MRI) scans in which ECT was used to abort status epilepticus provided a window of hemodynamic stability to obtain a positron emission tomography (PET) scan which enabled localization and subsequent resection of the seizure focus, with pathologic confirmation of focal cortical dysplasia (FCD).

 » Case Report Top

A 3-year-old previously healthy girl presented with explosive onset seizures characterized by blinking, arm stiffening, fearful expression, and at times convulsions, rapidly escalating to over 100 seizures daily. Electroencephalography (EEG) showed seizures arising from the right temporal region and abundant interictal right frontotemporal spikes. Multiple MRI scans were normal, with no focal lesion, and an extensive infectious, autoimmune, and genetic/metabolic evaluation was unrevealing.

Status epilepticus persisted despite treatment with benzodiazepines, levetiracetam, oxcarbazepine, lacosamide, clobazam, valproic acid, pyridoxine, and methylprednisolone 30 mg/kg daily for 4 days. Plasma exchange, brivaracetam, and fosphenytoin also failed, and the patient was sedated to burst suppression with midazolam and pentobarbital infusions. However, status epilepticus recurred when sedation was weaned, and alternative treatment options were pursued.

Ketogenic diet was initiated on day 22 of hospitalization, with moderate ketosis achieved on day 29. High-dose perampanel (18 mg once, followed by 6 mg 6 hours later, then 6 mg daily) was tried on day 25, but subsequently stopped due to incompatibility with the ketogenic diet. A decision to use ECT was made with input from two child and adolescent psychiatrists, an ECT psychiatrist, and with the mother's informed consent. Ketamine infusion and ECT were initiated on day 24. Remifentanil was used for induction to avoid antiepileptic effects from anesthetics. Rocuronium was used for muscle relaxation and glycopyrrolate was administered to prevent asystole. Midazolam and ketamine infusions were weaned prior to each treatment and later in the course flumazenil was given, but maintenance antiseizure medications were continued. ECT was performed daily for 7 days, with one to two stimulations per session, using a Thymatron System IV device with bitemporal electrode placement. ECT parameters are detailed in [Table 1]. ECT treatments induced electroclinical seizures characterized by a generalized spike and wave pattern distinct from the patient's typical seizures, followed by delta slowing and resolution of interictal discharges [Figure 1]. Initially, seizure freedom lasted several hours to 1 day following each ECT treatment. Following the fifth treatment, seizure freedom persisted, and ECT was discontinued after two additional treatments. Other than fatigue, no side effects were reported, and there was no evidence of significant cognitive impact attributable to ECT. Phenobarbital was added and a course of intravenous immunoglobulin 2 g/kg divided over 2 days was started as sedation was weaned.
Figure 1: EEG activities (a) prior to ECT: Right frontotemporal interictal spikes, sensitivity 10 uv/mm, HFF 50 Hz, and LFF 1 Hz; (b) ECT-induced generalized tonic-clonic seizure, sensitivity 15 uv/mm, HFF 50 Hz, and LFF 1 Hz; (c) following ECT, diffuse delta slowing, sensitivity 10 uv/mm, HFF 50 Hz, and LFF 1 Hz

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Table 1: ECT treatment parameters

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Following 1 week of seizure freedom, the patient had recurrence of one to two seizures daily. Seizures resolved following two additional ECT treatments, with the exception of intermittent subtle foot twitching without electrographic correlate, likely representing focal motor seizure. During this period, a PET scan was able to be completed, which demonstrated a hypermetabolic focus in the inferomedial right temporal lobe, consistent with an ictal PET. This finding, with concordant EEG localization, guided stereo EEG monitoring, with 12 implanted electrodes. Three seizures demonstrated right mesial temporal onset, and the patient underwent right temporal lobectomy, with pathologic confirmation of FCD, and rare brief clinical seizures reported in the 7 months following surgery.

 » Discussion Top

We present a 3-year-old girl with NORSE who responded to ECT initiated on day 24 of hospitalization, following the failure of numerous antiseizure medications. The resulting abortion of status epilepticus provided hemodynamic stability for ancillary testing including a PET scan, which ultimately enabled diagnosis and surgical treatment of an underlying FCD.

There is considerable experience in using ECT safely for treatment resistant depression and catatonia in adolescents, and the main side effect is transient amnesia.[10] However, evidence supporting its use for refractory status epilepticus remains limited. Zeiler et al.[11] compiled 15 published reports on the use of ECT for status epilepticus. Nineteen individual cases were reviewed, including four children ages 7–13 years, and seizure reduction was reported in 57.9% (11/19), with four patients demonstrating partial electrographic improvement and seven patients achieving resolution of seizures for at least 24 h.[11] A 1941 publication refers to the use of ECT in a younger 3-year-old child with refractory seizures, and the case of a 4 year old in whom status epilepticus was successfully aborted using ECT was more recently described.[8],[12]

The application of ECT for seizures arose from the observation that patients receiving repetitive ECT for psychiatric indications demonstrated a higher seizure threshold, necessitating increases in stimulus intensity to produce an adequate seizure, long held to be important for a therapeutic effect.[2],[4] The antiseizure mechanism has been proposed to be related to modulation of GABAergic transmission, and cortical gamma-aminobutyric acid (GABA) concentration has been demonstrated to increase following ECT.[13] It has also been suggested that ECT prolongs the refractory period after a seizure, decreases neural metabolism, and activates endogenous inhibitory interneurons, among other hypothesized antiseizure mechanisms.[4],[14],[15] The optimal treatment protocol is unknown, though a higher intensity stimulation may be needed to achieve the antiseizure effect compared to the parameters used for depression.[4],[5] In some published cases, maintenance antiseizure medications were discontinued to promote seizure induction with ECT, and in one report, caffeine was used to lower the seizure threshold.[5],[8] Alternatively, flumazenil may be used to reverse benzodiazepine effect prior to ECT.[15] The frequency and number of treatments vary on a case by case basis. Published cases of ECT for treatment of refractory status epilepticus in children are summarized in [Table 2].
Table 2: Published cases of ECT for treatment of refractory status epilepticus in children[5],[6],[7],[8],[9]

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Interpreting evidence based primarily on case reports is fraught with challenges. The high risk for publication bias and heterogeneity in individual cases, treatment protocols, and clinical outcome parameters make it difficult to draw generalizable conclusions. In refractory status epilepticus, therapeutic interventions are commonly initiated simultaneously or in close succession, further weakening evidence of efficacy for a given treatment. Our case is no exception to these limitations. However, the observed period of seizure freedom immediately following ECT, which increased with successive treatments, is compelling evidence that ECT contributed to seizure control in this patient, despite a prolonged course of status epilepticus lasting over 3 weeks. Further, seizure control achieved with ECT-enabled diagnostic testing, which led to the identification and treatment of the underlying cause of NORSE. Although further research is needed, this case illustrates that despite the considerable stigma surrounding ECT, it can play an important role in the treatment of NORSE, even in young children.[16],[17],[18],[19],[20],[21],[22],[23],[24],[25],[26],[27],[28],[29],[30],[31],[32],[33],[34],[35],[36],[37],[38],[39],[40],[41],[42],[43],[44],[45]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

 » References Top

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  [Table 1], [Table 2]


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