Corticosteroid Therapies in Childhood Epilepsy Disorders

Corticosteroids have formed part of the therapeutic armamentarium for childhood epilepsies for over six decades, driven by the recognition that neuroinflammation may contribute to seizure generation and propagation. These agents exert effects via glucocorticoid receptors to modulate cytokine release, blood–brain barrier integrity and neuronal excitability. Established indications include infantile spasms, where adrenocorticotropic hormone or high-dose prednisolone remains first-line, but expanding experience suggests benefits across a spectrum of syndromes. In developmental and epileptic encephalopathies, intermittent high-dose pulses or extended courses of agents such as methylprednisolone, dexamethasone or hydrocortisone have been used to suppress both clinical seizures and interictal epileptiform discharges. Across drug-resistant epilepsy cohorts, steroid regimens have been associated with seizure reduction in approximately half of treated children, and in some cases with sustained remission. However, protocols vary widely in agent selection, dosage and duration, and adverse effects—ranging from weight gain and hypertension to immunosuppression—require careful monitoring. Current challenges include heterogeneity of study designs, lack of randomised comparisons and divergent definitions of response. Harmonisation of treatment strategies and prospective trials are essential to define optimal regimens, stratify likely responders and balance efficacy against toxicity.

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Corticosteroid Therapies in Childhood Epilepsy Disorders publication trend

The graph below shows the total number of articles in corticosteroid therapies in childhood epilepsy disorders across all publications each year (not limited to Nature Index journals).

Technical terms

Corticosteroids: Synthetic or natural steroid hormones that reduce inflammation and modulate immune responses via glucocorticoid receptor activation.

Epileptic spasms: Brief, bilateral contraction of axial and limb muscles occurring in clusters, characteristic of West syndrome in infancy.

Developmental and epileptic encephalopathy with spike-wave activation in sleep ((D)EE-SWAS): A paediatric epilepsy syndrome marked by cognitive regression and continuous spike-wave discharges during non-rapid eye movement sleep.

Drug-resistant epilepsy (DRE): Failure to achieve sustained seizure control after adequate trials of two tolerated and appropriately chosen antiseizure medications.

Interictal epileptic activity (IEA): Abnormal electrical discharges seen on electroencephalography between clinical seizures, reflecting ongoing cortical hyperexcitability.

Pulse therapy: Intermittent administration of high-dose corticosteroids over short cycles to maximise anti-inflammatory effects while limiting cumulative exposure.

References

1. Corticosteroids in childhood epilepsies: A systematic review. Frontiers in Neurology (2023).
2. Pulsatile corticoid therapy reduces interictal epileptic activity burden in children with genetic drug‐resistant epilepsy. Epilepsia Open (2024).
3. Drug resistant epilepsies: A multicentre case series of steroid therapy. Seizure (2024).