Effectiveness of a hybrid corticosteroid treatment regimen on refractory childhood seizures and a review of other corticosteroid treatments

Bakker, Dewi P.; Catsman‐Berrevoets, Coriene E.; Neuteboom, Rinze F.

doi:10.1016/j.ejpn.2015.04.006

Cited by 25 publications

(24 citation statements)

References 35 publications

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“…An unjustified conclusion, because many diseases can (temporarily) respond to immunotherapy, or the observed response may even be the natural course of the disease. 26 However, there will always be a small level of uncertainty, which makes it even more important to perform complete workup in these children, MRI and CSF analysis, including IgG index and oligoclonal bands. In the diagnosis of these syndromes, it is important to look for signs and symptoms favoring autoimmunity or inflammation, but the differential diagnosis of possible AIE is broad, and other causes should also be considered, especially in children with aspecific signs.…”

Section: Discussionmentioning

confidence: 99%

Pediatric autoimmune encephalitis

Bruijn

Bruijstens

Bastiaansen

et al. 2020

Neurol Neuroimmunol Neuroinflamm

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ObjectiveThe aims of this study were (1) to describe the incidence of autoimmune encephalitis (AIE) and acute disseminated encephalomyelitis (ADEM) in children, (2) to validate the currently used clinical criteria to diagnose AIE, and (3) to describe pitfalls in the diagnosis of pediatric autoimmune (AI) and inflammatory neurologic disorders.MethodsThis study cohort consists of 3 patient categories: (1) children with antibody-mediated AIE (n = 21), (2) children with ADEM (n = 32), and (3) children with suspicion of an AI etiology of their neurologic symptoms (n = 60). Baseline and follow-up clinical data were used to validate the current guideline to diagnose AIE. In addition, patient files and final diagnoses were reviewed.ResultsOne-hundred three of the 113 included patients fulfilled the criteria of possible AIE. Twenty-one children had antibody-mediated AIE, of whom 19 had anti-N-methyl-D-aspartate receptor (NMDAR), 1 had anti–α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor, and 1 had anti–leucine-rich glioma-inactivated protein 1 encephalitis. Finally, 34 children had ADEM, and 2 children had Hashimoto encephalopathy. Mean incidence rates were 1.54 children/million (95% CI 0.95–2.35) for antibody-mediated AIE and 2.49 children/million (95% CI 1.73–3.48) for ADEM. Of the other 48 children, treating physicians' diagnoses were reviewed. In 22% (n = 6) of children initially diagnosed as having an AI/inflammatory etiology (n = 27), no support for AI/inflammation was found.ConclusionBesides anti-NMDAR encephalitis and ADEM, other AIEs are rare in children. The current guideline to diagnose AIE is also useful in children. However, in children with nonspecific symptoms, it is important to review data critically, to perform complete workup, and to consult specialized neuroinflammatory centers.

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Section: Discussionmentioning

confidence: 99%

Pediatric autoimmune encephalitis

Bruijn

Bruijstens

Bastiaansen

et al. 2020

Neurol Neuroimmunol Neuroinflamm

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“…While the efficacy of steroids for infantile spasms has been established from RCTs [60], evidence for the treatment of other types of seizures is more limited, generally from observational studies [61]. In studies evaluating steroids in patients with refractory epilepsies other than infantile spasms that have included at least a few patients with LGS, responder rates of 30%-79% have been reported [62,63]. However, the well documented side effects of prolonged steroid use, including hypertriglyceridaemia, osteoporosis and suppression of childhood growth means that their use should be curtailed to short-term 'crisis' periods [2].…”

Section: Steroidsmentioning

confidence: 99%

Expanding the Treatment Landscape for Lennox-Gastaut Syndrome: Current and Future Strategies

Strzelczyk

Schubert‐Bast

2021

CNS Drugs

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Lennox-Gastaut syndrome (LGS), a childhood-onset severe developmental and epileptic encephalopathy (DEE), is an entity that encompasses a heterogenous group of aetiologies, with no single genetic cause. It is characterised by multiple seizure types, an abnormal EEG with generalised slow spike and wave discharges and cognitive impairment, associated with high morbidity and profound effects on the quality of life of patients and their families. Drug-refractory seizures are a hallmark and treatment is further complicated by its multiple morbidities, which evolve over the patient’s lifetime. This review provides a comprehensive overview of the current and future options for the treatment of seizures associated with LGS. Six treatments are specifically indicated as adjunct therapies for the treatment of seizures associated with LGS in the US: lamotrigine, clobazam, rufinamide, topiramate, felbamate and most recently cannabidiol. These therapies have demonstrated reductions in drop seizures in 15%–68% of patients across trials, with responder rates (≥ 50% reduction in drop seizures) of 37%–78%. Valproate is still the preferred first-line treatment, generally in combination with lamotrigine or clobazam. Other treatments frequently used off-label include the broad spectrum anti-epileptic drugs (AED) levetiracetam, zonisamide and perampanel, while recent evidence from observational studies has indicated that a newer AED, the levetiracetam analogue brivaracetam, may be effective and well tolerated in LGS patients. Other treatments in clinical development include fenfluramine in late phase III, perampanel, soticlestat–OV953/TAK-953, carisbamate and ganaxolone. Non-pharmacologic interventions include the ketogenic diet, vagus nerve stimulation and surgical interventions; these are also expanding, with the potential for less invasive techniques for corpus callosotomy that have promise for reducing complications. However, despite these advancements, patients continue to experience a significant burden. Because LGS is not a single entity, tailoring of treatment is needed as opposed to a ‘one size fits all’ approach. Further research is needed into the underlying aetiologies and pathophysiology of LGS, together with advancements in treatments that encompass the spectrum of seizures associated with this complex syndrome.

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“…We compared two different standardized corticosteroid regimens: IV methylprednisolone or oral dexamethasone; the two most commonly used regimens in our institution and elsewhere [12,16,23]. Corticosteroid treatment is routinely used at our institution for regular clinical management of refractory epilepsy.…”

Section: Corticosteroid Treatmentmentioning

confidence: 99%

“…In specific epilepsy syndromes, steroid use is supported by randomized control trials (for West syndrome) [5][6][7] or meta-analyses of case series (for continuous spike waves in sleep) [8,9]. However, outside of these specific syndromes, there are few studies to guide therapy [10][11][12]. Current evidence for corticosteroid treatment for epileptic encephalopathy consists primarily of small observational studies [10,13].…”

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confidence: 99%

Measurable outcomes for pediatric epileptic encephalopathy: a single‐center experience with corticosteroid therapy

Gofshteyn

Gurcharran

Marquis

et al. 2021

Epileptic Disorders

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Objective. Corticosteroids are commonly used to treat refractory epilepsy in children, but the heterogeneity of the population and lack of standardized outcome measures have limited understanding of their effectiveness. We conducted a single-center study of corticosteroids for epileptic encephalopathy to (a) identify domains for measurement and estimate potential effect sizes, (b) characterize heterogeneity, and (c) identify outcomes that may need better tools for measurement. Methods. In this retrospective single-center cohort study, children with epileptic encephalopathy (excluding infantile spasms) were treated with a standardized course of oral dexamethasone or IV methylprednisolone. Long-term video electroencephalography (EEG) was assessed via novel ordinal scales for five features: seizure semiology/burden, epileptiform activity, slowing, organization, and sleep architecture. We abstracted parental assessment of functional domains (i.e., cognition) from the medical records. Pre-treatment and post-treatment EEG features, functional domains, and treatment regimens were compared. Results. Thirty-five children with refractory epilepsy were included. Overall, 16/35 (46%) of individuals had a >50% reduction in seizure frequency from the pre-treatment EEG to the initial post-treatment EEG. In particular, tonic seizures (in a subset of 23 children) were reduced (24-hour tonic seizure count pre-treatment was 8 [4-13] and 3 [1-5] post-treatment EEG#1, p=0.04). For follow-up post-treatment EEGs, there was: (1) better formation of sleep spindles (37% normal pre-treatment to 63% normal post-treatment; p=0.04); and (2) improvement in parental reported cognition (in 43%). Improved cognition was the only outcome that differed between the dexamethasone and methylprednisolone treated groups (58% for dexamethasone [n=11/19] vs. 25% for methylprednisolone [n=4/16]; p=0.03). Significance. Large studies should be powered to detect reductions in seizures (particularly tonic as we identified a 2.6-fold reduction), improved EEG organization, and improved sleep architecture (21 percentage points). Cognitive improvements following steroid treatment, reported by parents, should be quantified and fully characterized in future work.

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Effectiveness of a hybrid corticosteroid treatment regimen on refractory childhood seizures and a review of other corticosteroid treatments

Cited by 25 publications

References 35 publications

Pediatric autoimmune encephalitis

Pediatric autoimmune encephalitis

Expanding the Treatment Landscape for Lennox-Gastaut Syndrome: Current and Future Strategies

Measurable outcomes for pediatric epileptic encephalopathy: a single‐center experience with corticosteroid therapy

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