Management of Infantile Epileptic Spasms Syndrome: A survey of US pediatric hospitals

Abstract

Objective

To evaluate the management practices of Infantile Epileptic Spasms Syndrome (IESS) across tertiary pediatric hospitals in the United States using a survey-based approach.

Methods

A 21-question survey focused on management setting, work-up, follow-up and treatment was created and sent to 45 member institutions of the Pediatric Epilepsy Research Consortium (PERC). Results were analyzed by a working group of PERC focused on IESS.

Results

Thirty-three institutions completed the survey. Most institutions (91%) managed new-onset IESS in-patient, with 76% using overnight video EEG (vEEG) monitoring for diagnosis. Nearly all (> 90%) obtained an MRI brain and genetic testing at initial diagnosis. The most common modalities of genetic evaluation were an epilepsy panel (47%) and trio whole exome sequencing (44%). For assessment of response at the first follow-up, 45% used extended vEEG (1–6 h), with 36% continuing to obtain overnight vEEG. The 2021 BASED score (Burden of Amplitude and Epileptiform Discharges) was the preferred method of assessing response (58%) compared to resolution of hypsarrhythmia (39%). High-dose prednisolone was the first treatment for most institutions, irrespective of the etiology (apart from Tuberous Sclerosis Complex). Combined treatment (hormonal + vigabatrin) was less commonly used as first treatment (9%–12%, depending on etiology). Most institutions (63%) looked for failure of both hormonal therapy and vigabatrin (sequential or combined) before pursuing surgical treatment in patients with identifiable brain MRI lesions, but would also consider surgical treatment in patients with remission of electro-clinical epileptic spasms but continued epileptiform EEGs.

Significance

There are areas of consensus and variability in management of IESS in US institutions. Updated consensus guidelines addressing the comprehensive management of IESS are needed to help standardize and optimize care in these patients.

Plain Language Summary

Infantile Epileptic Spasms Syndrome requires prompt diagnosis and initiation of appropriate treatment to improve developmental outcomes. In this survey of tertiary pediatric centers in the United States, we found areas of consensus (use of overnight video EEG at initial diagnosis, use of high-dose prednisolone as initial treatment, preference for sequential as opposed to combination therapy, use of the 2021 BASED score for EEG evaluation, and the early consideration of epilepsy surgery) and variability (type of genetic testing for diagnosis, choice of second and third treatments, use of antiseizure medications for persistently epileptic EEG) in management of these patients.