Corticosteroids in childhood epilepsies: A systematic review

Abstract

Corticosteroids have been used for the treatment of patients with epilepsy for more than 6 decades, based on the hypothesis of inflammation in the genesis and/or promotion of epilepsy. We, therefore, aimed to provide a systematic overview of the use of corticosteroid regimes in childhood epilepsies in line with the PRISMA guidelines. We performed a structured literature search via PubMed and identified 160 papers with only three randomized controlled trials excluding the substantial trials on epileptic spasms. Corticosteroid regimes, duration of treatment (days to several months), and dosage protocols were highly variable in these studies. Evidence supports the use of steroids in epileptic spasms; however, there is only limited evidence for a positive effect for other epilepsy syndromes, e.g., epileptic encephalopathy with spike-and-wave activity in sleep [(D)EE-SWAS] or drug-resistant epilepsies (DREs). In (D)EE-SWAS (nine studies, 126 patients), 64% of patients showed an improvement either in the EEG or in their language/cognition following various steroid treatment regimes. In DRE (15 studies, 436 patients), a positive effect with a seizure reduction in 50% of pediatric and adult patients and seizure freedom in 15% was identified; however, no recommendation can be drawn due to the heterozygous cohort. This review highlights the immense need for controlled studies using steroids, especially in DRE, to offer patients new treatment options.

Pulsatile corticoid therapy reduces interictal epileptic activity burden in children with genetic drug‐resistant epilepsy

“…In the described study, two of the four children with drug‐resistant epilepsy showed an improvement on the only outcome measure of seizure frequency, one patient showed no improvement and one patient dropped out 13 . The lack of systematic studies was further underlined by a recent review about steroids in childhood epilepsy describing very heterogeneous findings about the effect of steroids in mixed pediatric and adult cohorts 14 …”

“…35 A smoothing moving window of 300 ms and 80th percentile thresholds was applied. Further, spindles were categorized as fast spindles (12)(13)(14)(15)(16) or slow spindles (10)(11)(12). Detections at the time of an IED were excluded due to potential misdetections.…”

“…13 The lack of systematic studies was further underlined by a recent review about steroids in childhood epilepsy describing very heterogeneous findings about the effect of steroids in mixed pediatric and adult cohorts. 14 Few observational studies evaluating intramuscular ACTH and oral steroids in different epilepsy childhood syndromes revealed a positive effect on seizure frequency and subjective evaluation of EEG recordings. [15][16][17][18][19] However, ACTH may have serious adverse effects such as hypercortisolism, cardiac hypertrophy and electrolyte abnormalities.…”

“…The dosage of orally administered steroids varied greatly between different studies, as did the duration of administration and choice of steroids 22 . According to a recent review, there are heterogeneous treatment protocols due to a lack of consensus 14 . To the best of our knowledge, a systematic prospective study investigating the efficacy of PCT in children with genetic drug‐resistant epilepsy is lacking.…”

The Response of Synthetic Adrenocorticotropic Hormone (ACTH) Treatment in Pediatric Drug-Resistant Epilepsy Other Than Infantile Epileptic Spasms Syndrome: A Retrospective Observational Study

“…Few studies have addressed the use of corticosteroids in drug-resistant epilepsy. A recent systematic review found only 15 studies with a combined 436 patients with pooled analysis of a seizure reduction in 50% in both pediatric and adult patients, besides seizure freedom in 15% of the patients [ 8 ]. Nevertheless, the use of ACTH in drug-resistant epilepsy aside from infantile epileptic spasms syndrome (IESS) is still not clearly established [ 9 ].…”