Corticosteroid therapy for epileptic encephalopathies other than West syndrome

Abstract

Epileptic encephalopathies are age-dependent conditions, typically drug resistant, characterized by severe cognitive impairment due to the epileptic activity itself. Corticosteroid therapy with adrenocorticotrophic hormone, hydrocortisone, prednisone or methylprednisolone is one of the therapeutic approaches tried in these children. Although the first use of steroids as antiepileptic drugs dates back to 1950, there is no wide agreement about the ideal treatment regimen in epileptic encephalopathies other than West syndrome; no evidence from class I and II studies are available at the moment. In this study, we present a literature review on steroid therapy in childhood epilepsy with special regard to effectiveness, tolerability and possible mechanisms of action.

Efficacy and safety of corticosteroids and ACTH in epileptic syndromes beyond Infantile Epileptic Spasms Syndrome (IESS): A systematic review and meta‐analysis

“…9 We did not include this study in our review because of the low number of patients. 58 Dontin et al 6 summarized 19 studies in a narrative review of corticosteroid therapy for epileptic encephalopathies other than West syndrome. Except for two studies on ESES and one on pulsed methylprednisolone, all were retrospective series and case reports.…”

“…Several narrative reviews have been published based on retrospective case series and a few open cohort studies. [5][6][7][8] A Cochrane review on the treatment of pediatric epilepsies other than infantile spasms with ACTH and corticosteroids, last updated in 2015, found mainly low-evidence reports and one randomized cross-over trial of a new synthetic ACTH [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19] analogue that included only five children and yielded no interpretable results. The authors were, therefore, unable to make an evidence-based treatment recommendation.…”

“…There are hardly any controlled studies involving these patients. Several narrative reviews have been published based on retrospective case series and a few open cohort studies 5‐8 . A Cochrane review on the treatment of pediatric epilepsies other than infantile spasms with ACTH and corticosteroids, last updated in 2015, found mainly low‐evidence reports and one randomized cross‐over trial of a new synthetic ACTH 4‐19 analogue that included only five children and yielded no interpretable results.…”

Intravenous Methylprednisolone Versus Oral Prednisolone for West Syndrome: A Randomized Open-Label Trial

“…To the best of authors’ knowledge, this is the first study comparing IVMP to OP for the treatment of WS. Over the past two decades, high-dose IVMP pulse therapy has been used in various epilepsies in order to reduce the side effects of prolonged oral steroid therapy, while maintaining efficacy [ 15 – 18 ]. Administered according to different regimens (with dosages ranging from 15 mg/kg/d for three or five days to 30 mg/kg/d for two or three days) repeated at different intervals (weekly or monthly), it has been shown to be successful in different epilepsies such as Landau–Kleffner syndrome, absence seizures, and epileptic encephalopathies) [ 5 – 7 , 16 ].…”

Serial Neuropsychological Evaluation of Children with Severe Epilepsy

“…Drug resistance is par for the course in this group of syndromes, but some limited success has been seen with the use of corticosteroids. 33,34 The ketogenic diet has met with some success in ESES 35 as well as in Lennox-Gastaut syndrome and Dravet syndrome. 36,37 In other children, vagal nerve stimulators have provided some relief.…”