Epileptic spasms: Evidence for oral corticosteroids and implications for low and middle income countries

Abstract

Implementation of international guidelines for the treatment of epileptic spasms, is challenging when access to adrenocorticotrophic hormone (ACTH) and vigabatrin is restricted, especially in Low and Middle Income Countries (LMIC). Oral corticosteroids are alternative interventions but evidence for the optimal agent, dose, duration, efficacy and long-term effects is lacking. A systematic review of the literature was performed to assess the quality of evidence of prednisone and prednisolone (oral corticosteroids) for the management of epileptic spasms. There is level C recommendation based on class III evidence to support the efficacy of oral corticosteroids for the acute clinical control of epileptic spasms and EEG resolution. Efficacy of oral corticosteroids in comparison to the internationally recommended intervention of ACTH has class IV evidence supporting level U recommendation. Similarly, there is no data on the risk of relapse with oral corticosteroids (class IV, level U), compared to ACTH. There is class IV evidence supporting level U recommendation for the safety of oral corticosteroids and class II evidence for level B recommendation for ACTH. In terms of oral corticosteroids and effects on long-term development there is class IV evidence leading to level U recommendation, compared to class III evidence supporting level C recommendation for ACTH. Randomized controlled studies are needed to compare oral corticosteroids with ACTH, the optimal dosage and regimen as well as the long-term neurodevelopmental outcomes. Based on the limited existing studies a treatment guideline for LMIC is proposed which could be used to standardize interventions permitting clarification of these unmet questions.

Prednisolone/prednisone as adrenocorticotropic hormone alternative for infantile spasms: a meta‐analysis of randomized controlled trials

“…Moreover, there was no significant difference in the clinical responses in the subgroup for different corticosteroid doses. On the basis of the high cost and limited accessibility of ACTH, prednisolone or prednisone may be an effective alternative for ACTH, which justifies the recommendations by 2017 Quality Measure Set from the American Academy of Neurology and Child Neurology Society and Rag et al…”

Neutrophil to lymphocyte rate and serum prealbumin maybe predictors for abnormal high blood pressure caused by adrenocorticotropic hormone therapy in children with epileptic spasms: two cases report

“…In 2004, a report from American Academy of Neurology/Child Neurology Society indicated that ACTH was an effective agent for the treatment of epileptic spasms (18,19). The cost for epileptic spasms treatment with ACTH estimated about US$70,000 to US$150,000 in the USA, and about RMB5,000 (US$715) for a 14 days course of ACTH therapy in China (3,9). Oral corticosteroids are another choice for epileptic spasms therapy.…”

“…Synthesizing glucocorticoid made corticosteroids oral administration available and a drop-in price (17). But oral corticosteroids are used off-label for the treatment of epileptic spasms (3).…”

“…Epileptic spasms are characterized by a sustained tonic contraction of the neck, trunk, brief flexion or extension of the arms and flexion at the waist which usually tend to occur in clusters (1)(2)(3). Both semiology of epileptic spasms and the interictal pattern of hypsarrhythmia on the electroencephalography (EEG) are important in the diagnosis of epileptic spasms (3). When epileptic spasms occur during the infantile stage of life, they may be also called "infantile spasms" (2).…”

Outcome of West Syndrome: A Critical Review

“…Authors suggest oral corticosteroids can be alternative choice, especially in low and middle income countries. 31 Likewise, Chang et al 32 have suggested that high-dose prednisolone is not inferior to adrenocortocotropin hormone for spam control and can be safe and alternative therapy.…”