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  1. Ong MJY, Lee VLL, Teo SL, Tan HJ, Trinka E, Khoo CS
    Neurocrit Care, 2024 Oct;41(2):681-690.
    PMID: 38769254 DOI: 10.1007/s12028-024-02003-4
    BACKGROUND: Electroconvulsive therapy (ECT) has been suggested as a treatment option for refractory status epilepticus (RSE) and super-refractory status epilepticus (SRSE).

    OBJECTIVE: The objective of this scoping review was to conduct an extensive literature review on the role of ECT as a treatment option for RSE and SRSE.

    METHODS: We searched Ovid MEDLINE and Scopus for journal articles from database inception until February 2024. Articles were then selected based on predetermined inclusion and exclusion criteria.

    RESULTS: We identified five retrospective case series with 28 adult patients receiving ECT for RSE or SRSE. ECT was administered within 3-70 days (mean 20 days) after the development of SE, and the mean number of ECT courses ranged from 1 to 12 sessions for each patient. ECT was administered in fixed or titrated doses. A total of 20 out of 28 patients (71%) showed clinical improvement, with two (7%) having complete cessation of seizures. It is essential to note that given the lack of control, there could be overreporting of clinical improvement in these studies. 11 patients (39%) were reported as deceased due to causes that were not directly related to ECT treatment. Four patients (14%) reported adverse effects of ECT, including memory, concentration, and/or cognitive impairment.

    CONCLUSIONS: There are level-4 Oxford Centre for Evidence-Based Medicine evidence and low-level Grading of Recommendations Assessment Development and Education evidence that suggest ECT as a treatment option for RSE and SRSE. In light of the limitations of the existing evidence, clinicians should carefully consider individual patients' clinical contexts when deciding on the appropriateness of ECT as a treatment option. Further research, including prospective studies with controlled designs, is needed to elucidate the efficacy, safety, and optimal regime of ECT in the management of RSE and SRSE.

    Matched MeSH terms: Drug Resistant Epilepsy/therapy
  2. Tan HJ, Tee TY, Husin M, Khoo CS, Woon LS
    Epileptic Disord, 2020 Dec 01;22(6):828-833.
    PMID: 33337333 DOI: 10.1684/epd.2020.1233
    Super-refractory status epilepticus (SRSE) is a neurocritical emergency, associated with significant morbidity and mortality. The precise pathophysiology is still not completely understood. The likelihood of spontaneous seizure termination reduces with time, and it is of paramount importance to abort status in order to prevent permanent long-term neurological sequelae and death. A few neuroprotective strategies, such as general anaesthesia, steroids, ketogenic diet and hypothermia, have been used to treat SRSE, however, the clinical outcome remains inconclusive. We herein present two cases of SRSE, which were successfully treated with electroconvulsive therapy (ECT) after failing all pharmacological measures.
    Matched MeSH terms: Drug Resistant Epilepsy/physiopathology; Drug Resistant Epilepsy/therapy*
  3. Yeap TB, Ab Mukmin L, Ang SY, Ghani AR
    BMJ Case Rep, 2023 Feb 07;16(2).
    PMID: 36750294 DOI: 10.1136/bcr-2022-252692
    Patients with medically refractory epilepsy (MRE) are indicated for vagus nerve stimulation (VNS) placement. Anaesthesia for VNS placement is extremely challenging and requires several considerations. We present a man in his 20s with MRE who successfully underwent VNS placement. We review the mechanism of action of VNS, anaesthetic challenges and measures to prevent seizures.
    Matched MeSH terms: Drug Resistant Epilepsy*
  4. Habib MA, Ibrahim F, Mohktar MS, Kamaruzzaman SB, Rahmat K, Lim KS
    World Neurosurg, 2016 Apr;88:576-585.
    PMID: 26548833 DOI: 10.1016/j.wneu.2015.10.096
    BACKGROUND: Electroencephalography source imaging (ESI) is a promising tool for localizing the cortical sources of both ictal and interictal epileptic activities. Many studies have shown the clinical usefulness of interictal ESI, but very few have investigated the utility of ictal ESI. The aim of this article is to examine the clinical usefulness of ictal ESI for epileptic focus localization in patients with refractory focal epilepsy, especially extratemporal lobe epilepsy.

    METHODS: Both ictal and interictal ESI were performed by the use of patient-specific realistic forward models and 3 different linear distributed inverse models. Lateralization as well as concordance between ESI-estimated focuses and single-photon emission computed tomography (SPECT) focuses were assessed.

    RESULTS: All the ESI focuses (both ictal and interictal) were found lateralized to the same hemisphere as ictal SPECT focuses. Lateralization results also were in agreement with the lesion sides as visualized on magnetic resonance imaging. Ictal ESI results, obtained from the best-performing inverse model, were fully concordant with the same cortical lobe as SPECT focuses, whereas the corresponding concordance rate is 87.50% in case of interictal ESI.

    CONCLUSIONS: Our findings show that ictal ESI gives fully lateralized and highly concordant results with ictal SPECT and may provide a cost-effective substitute for ictal SPECT.

    Matched MeSH terms: Drug Resistant Epilepsy/diagnosis*; Drug Resistant Epilepsy/surgery*
  5. Akyuz E, Paudel YN, Polat AK, Dundar HE, Angelopoulou E
    Epilepsy Behav, 2021 Feb;115:107701.
    PMID: 33412369 DOI: 10.1016/j.yebeh.2020.107701
    Epilepsy is a devastating neurological disorder characterized by the repeated occurrence of epileptic seizures. Epilepsy stands as a global health concern affecting around 70 million people worldwide. The mainstream antiepileptic drugs (AEDs) only exert symptomatic relief and drug-resistant epilepsy occurs in up to 33 percent of patients. Hence, the investigation of novel therapeutic strategies against epileptic seizures that could exert disease modifying effects is of paramount importance. In this context, compounds of natural origin with potential antiepileptic properties have recently gained increasing attention. Quercetin is a plant-derived flavonoid with several pharmacological activities. Emerging evidence has demonstrated the antiepileptic potential of quercetin as well. Herein, based on the available evidence, we discuss the neuroprotective effects of quercetin against epileptic seizures and further analyze the plausible underlying molecular mechanisms. Our review suggests that quercetin might be a potential therapeutic candidate against epilepsy that deserves further investigation, and paves the way for the development of plant-derived antiepileptic treatment approaches.
    Matched MeSH terms: Drug Resistant Epilepsy
  6. Lim, Kheng-Seang, Sherrini Ahmad Bazir Ahmad, Vairavan Narayanan, Kartini Rahmat, Norlisah Mohd Ramli, Mun, Kein-Seong, et al.
    Neurology Asia, 2017;22(4):299-305.
    MyJurnal
    Background and Objective: There is a great challenge to establish a level 4 epilepsy care offering
    complete evaluation for epilepsy surgery including invasive monitoring in a resource-limited country.
    This study aimed to report the setup of a level 4 comprehensive epilepsy program in Malaysia and the
    outcome of epilepsy surgery over the past 4 years.

    Methods: This is a retrospective study analyzing
    cases with intractable epilepsy in a comprehensive epilepsy program in University Malaya Medical
    Center (UMMC), Kuala Lumpur, from January 2012 to August 2016.

    Results: A total of 92 cases
    had comprehensive epilepsy evaluation from January 2012 till August 2016. The mean age was 35.57
    years old (range 15-59) and 54 (58.7%) were male. There were 17 cases having epilepsy surgery
    after stage-1 evaluation. Eleven cases had mesial temporal sclerosis and 81% achieved Engel class
    I surgical outcome. Six cases had lesionectomy and 60% had Engel class I outcome. A total of 16
    surgeries were performed after stage-2 evaluation, including invasive EEG monitoring in 9 cases.
    Among those with surgery performed more than 12 months from the time of data collection, 5/10
    (50%) achieved Engel I outcome, whereas 2 (20%) had worthwhile improvement (Engel class III)
    with 75% and 90% seizure reduction.

    Conclusion: Level 4 epilepsy care has an important role and is possible with joint multidisciplinary
    effort in a middle-income country like Malaysia despite resource limitation.
    Matched MeSH terms: Drug Resistant Epilepsy
  7. Sayuthi S, Tharakan J, Pieter MS, Salmah WM, Madhavan M, Tahir A, et al.
    Malays J Med Sci, 2009 Jan;16(1):39-43.
    PMID: 22589647 MyJurnal
    We present our preliminary experience in neuropsychological testing in epilepsy surgery patients to demonstrate how these tests contributed to decide the laterality of epileptic focus, and to assess the effect of surgery on patient's cognitive function and quality of life. Preoperative neuropsychological tests consisting of Wechsler Adult Intelligence Scale-III (WAIS) for IQ, Wechsler Memory Scale-III (WMS) for memory and patients' quality of life (QOLIE 31) were administered to refractory epilepsy patients under evaluation for surgical treatment. These tests were repeated one year after surgery and we studied any changes in trends. A total of seven patients were recruited in this study between July 2004 and July 2006. The aetiologies of refractory epilepsy were pure mesial temporal sclerosis (MTS) in five patients, dysembryogenic neuroepithelial tumour (DNET) in one and dual lesion of cavernous angioma with ipsilateral MTS in one. The preoperative neuropsychological tests were all in concordance to MRI finding, and showed good contralateral function; five lateralises to the right and two to the left. The post-operative Engel seizure count (median 8.00, IQR 7.00-8.75), general IQ (88 vs. 79), performance IQ (94 vs. 79), verbal memory (89 vs. 71), non-verbal memory (88 vs. 75) and QOLIE (53.14 vs. 44.71) were better compared to preoperative values. The verbal IQ (84 vs. 84) was unchanged. Neuropsychological tests are useful as ancillary investigations to determine the laterality of seizure focus and integrity of function in the contralateral temporal lobe. Following successful surgical treatment, there is a trend towards improvement in memory, IQ and quality of life scores in this small group of patients.
    Matched MeSH terms: Drug Resistant Epilepsy
  8. Inoue Y, Kaneko S, Hsieh PF, Meshram C, Lee SA, Aziz ZA, et al.
    Epilepsia, 2019 03;60 Suppl 1:60-67.
    PMID: 30869167 DOI: 10.1111/epi.14645
    This post hoc analysis assessed the long-term safety, tolerability, and efficacy of perampanel in Asian patients with refractory focal seizures; an additional analysis assessed the effect of perampanel on focal impaired awareness seizures (FIAS) with focal to bilateral tonic-clonic (FBTC) seizures. In this subanalysis, data from Asian patients ≥12 years of age who had focal seizures with FBTC seizures despite taking one to 3 concomitant antiepileptic drugs at baseline, and who had entered either the long-term extension phase of 3 phase-3 perampanel trials (study 307) or the 10-week extension phase of study 335, were analyzed for the effect of perampanel on duration of exposure, safety, and seizure outcomes. Of 874 Asian patients included in the analysis, 205 had previously received placebo during the double-blind phase-3 trials and 669 had previously received perampanel 2-12 mg/day; 313 had FIAS with FBTC seizures at core study baseline. The median duration of exposure to perampanel was 385.0 days, and the retention rate at one year was 62.6%. Overall, during the first 52 weeks of perampanel treatment, 777 patients (88.9%) had treatment-emergent adverse events (TEAEs), most of which were mild to moderate in severity. The most frequent TEAEs were dizziness (47.1%), somnolence (22.3%), and nasopharyngitis (17.4%). During the first 52 weeks of perampanel treatment, median percent change in seizure frequency per 28 days from pre-perampanel baseline for all focal seizures was -28.1%, and -51.7% for FIAS with FBTC seizures. The 50% responder rate relative to pre-perampanel baseline for all focal seizures was 33.8%, and 51.1% for FIAS with FBTC seizures. Long-term treatment with perampanel in Asian patients had safety, tolerability, and efficacy similar to that of the global population in the phase-3 trials and extension study 307. The safety profile and response rate suggest benefit for an Asian population of patients with refractory epilepsy.
    Matched MeSH terms: Drug Resistant Epilepsy/drug therapy
  9. Kandasamy R, Tharakan J, Idris Z, Abdullah JM
    Surg Neurol Int, 2013;4:124.
    PMID: 24232072 DOI: 10.4103/2152-7806.119006
    BACKGROUND: A patient with refractory epilepsy due to underlying mesial temporal sclerosis underwent general anesthesia for an elective anterior temporal lobectomy and amgydalo-hippocampectomy. He was a known hypertensive and his blood pressure was well controlled on medication.

    CASE DESCRIPTION: Following induction of general anesthesia and subsequent opening of the craniotomy flap it was noted that the patient had a very swollen brain that herniated out of the dural defect. There was an underlying spontaneous intraparenchymal bleed encountered in the region of the left temporal lobe with associated subarachnoid hemorrhage within the sylvian fissure. The clot was evacuated and subsequently brain swelling reduced allowing us to proceed with the intended surgery. Despite the intracranial findings there was no overt abnormality in the hemodynamic status from the time of induction of anesthesia to the craniotomy opening excepting a mild nonsustained elevation of blood pressure at the outset.

    CONCLUSION: This case is of interest due to the fact that spontaneous intraparenchymal bleeding after induction of anesthesia has not been reported before in literature and should be considered in any patient in which brain swelling occurs in a setting of elective neurosurgery in which the primary lesion does not cause elevated intracranial pressure.

    Matched MeSH terms: Drug Resistant Epilepsy
  10. Siti Aishah Abdul Wahab, Yusnita Yakob, Khoo,Teik-Beng, Sangita Dharshini Terumalay, Vigneswari Ganesan, Teh,Chee-Ming, et al.
    Neurology Asia, 2017;22(2):99-111.
    MyJurnal
    Background & Objective: SCN1A gene which encodes for sodium channel alpha 1 subunit has been
    found to be the most common mutated gene in patients with epilepsy. This study aims to characterize the
    SCN1A mutations as well as to describe genotype and phenotype association in children with SCN1Arelated
    infantile-onset epileptic encephalopathies in Malaysia.

    Methods: Children with infantile-onset
    epileptic encephalopathy mostly suspected to have Dravet syndrome who had mutational analysis for
    SCN1A gene from hospitals all over Malaysia were included in the study. Their epilepsy syndrome
    diagnosis was classified into severe myoclonic epilepsy in infancy and its variants. Polymerase chain
    reaction and bidirectional sequencing were used to identify SCN1A mutations.

    Results: A total of 38
    children with heterozygous mutations were analysed, 22 (57.9%) of which were novel mutations.
    Truncated mutations were the most common mutation type (19, 50%). Other mutation types were
    missense mutations (14, 36.8%), splice site mutations (4, 10.5%) and in-frame deletion (1, 2.6%). The
    mean age of seizure onset was 4.7 months. Seizure following vaccination was observed in 26.3% of
    the children. All of them had drug resistant epilepsy. There was no significant association between
    the type of mutation with the syndromic diagnosis, age of seizure onset, tendency of the seizures to
    cluster or having status epilepticus, mean age when developmental delay was observed and response
    to various antiepileptic drugs.

    Conclusion: This study expands the spectrum of SCN1A mutations and proves the importance of
    SCN1A gene testing in diagnosing infantile-onset epileptic encephalopathies patients. Although, our
    study does not support any clinically meaningful genotype-phenotype association for SCN1A-related
    infantile-onset epileptic encephalopathies, the clinical characteristics of our cohort are similar to those
    that have been described in previous studies.
    Matched MeSH terms: Drug Resistant Epilepsy
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