Corticosteroids including ACTH for childhood epilepsy other than epileptic spasms

Mehta, Vishisht; Ferrie, Colin D.; Cross, J. Helen; Vadlamani, Gayatri

doi:10.1002/14651858.cd005222.pub3

Cited by 31 publications

(30 citation statements)

References 34 publications

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“…8 ACTH and oral steroid therapies have been used not only for infantile spasms but also Lennox-Gastaut's syndrome, 9 epilepsy with myoclonic-atonic seizures, 10 epilepsy with CSWS, 11 and Landau-Kleffner's syndrome, 12 although it is unclear whether ACTH is effective for patients without infantile spasms. 13 In our four patients, ACTH therapy had excellent short-term and long-term effects for not only ENM and CSWS but also for focal seizures and F-BTCS. As demonstrated in case 4, ACTH may be effective for a few years, but the seizures and CSWS then relapse to the previous state, requiring another specific therapy.…”

Section: Discussionmentioning

confidence: 52%

Pharmacoresistant Atypical Benign Partial Epilepsy of Childhood: Early Introduction of Specific Therapies to Prevent Cognitive Decline

et al. 2019

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Atypical benign partial epilepsy (ABPE) of childhood is a special type of epileptic syndrome characterized by the combination of epileptic negative myoclonus, atypical absence seizures, focal motor seizures, and a continuous spike–wave during slow sleep (CSWS). Although the seizures are resistant to antiseizure drugs (ASDs) effective for focal epilepsy, they are markedly responsive to ethosuximide (ESM). An incorrect ASD choice may aggravate the seizures and CSWS, resulting in a pseudo-catastrophic state or nonconvulsive status epilepticus. In this study, we retrospectively investigated the effectiveness of steroid and ketogenic diet (KD) therapies for children with ABPE resistant to ASDs, including ESM. Adrenocorticotropic hormone (ACTH) therapy (n = 4), KD therapy (n = 4), and an oral steroid (n = 2) were tried for eight patients (two patients tried two therapies). An excellent response (seizure-free period > 1 year) was achieved by four out of four patients undergoing ACTH therapy, two out of four undergoing KD therapy, and one out of two receiving oral steroid therapy. In conclusion, steroid and KD therapies are reasonable treatment options early in the course of treatment of children with pharmacoresistant ABPE to prevent a decline in cognitive function due to the persistence of epileptic encephalopathy. The majority of patients in our case series had an excellent response, though further studies are warranted to confirm their efficacy and safety.

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Section: Discussionmentioning

confidence: 52%

Pharmacoresistant Atypical Benign Partial Epilepsy of Childhood: Early Introduction of Specific Therapies to Prevent Cognitive Decline

et al. 2019

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“…Steroids, including prednisone, prednisolone, methylprednisolone, adrenocorticotrophin hormone (ACTH) and hydrocortisone also have an important role during periods of heightened seizure activity. 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].…”

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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“…First, leptin receptors are found throughout the brain, and their stimulation leads to antiseizure effects by decreasing proinflammatory cytokines (e.g., IL-1β), increasing an endogenous anticonvulsant (galanin), and acting as an antioxidant by increasing glutathione and decreasing malondialdehyde (37)(38)(39). Second, ghrelin, neuropeptide Y, galanin, and cortisol can induce alterations in GABA uptake and serotonin turnover and affect ion channels, thereby decreasing neuronal excitability, although the exact mechanisms need to be explored by future research (20,40). Third, cortisol is part of the hypothalamic-pituitary-adrenal (HPA) or stress axis, and targeting this axis can decrease seizures and stress-related comorbidities, e.g., anxiety and depression (41).…”

Section: Production Of Neurohormonesmentioning

confidence: 99%

The Ketogenic Diet Revisited: Beyond Ketones

2021

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Corticosteroids including ACTH for childhood epilepsy other than epileptic spasms

Cited by 31 publications

References 34 publications

Pharmacoresistant Atypical Benign Partial Epilepsy of Childhood: Early Introduction of Specific Therapies to Prevent Cognitive Decline

Pharmacoresistant Atypical Benign Partial Epilepsy of Childhood: Early Introduction of Specific Therapies to Prevent Cognitive Decline

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

The Ketogenic Diet Revisited: Beyond Ketones

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