Electrophysiological and clinical biomarkers of ACTH (Acthar gel) treatment
Abstract number :
1.104
Submission category :
3. Neurophysiology / 3C. Other Clinical EEG
Year :
2017
Submission ID :
344184
Source :
www.aesnet.org
Presentation date :
12/2/2017 5:02:24 PM
Published date :
Nov 20, 2017, 11:02 AM
Authors :
Ahmet Tanritanir, Boston Children’s Hospital, Harvard University Medical School, Boston, MA, United States; Saba Jafarpour, Boston Children’s Hospital, Harvard University Medical School, Boston, MA, United States; Kshitiz Rakesh, Boston Chil
Rationale: We investigated ACTH effects on electrophysiological biomarkers along with clinical outcome measures, with the aim to find additional biomarkers in the evaluation and treatment of epileptic encephalopathies (EE) and infantile spasms (IS). Methods: Consecutive infants (0-24 months of age) treated with ACTH (Acthar gel) for IS were included in this retrospective descriptive study. Inclusion criteria: a) IS diagnosis was made by video EEG and clinically, b) ACTH treatment, c) consecutive EEG recording before and after ACTH treatment. We excluded patients with tuberous sclerosis complex. Clinical variables and EEG features were evaluated at two-time points: before the ACTH treatment (baseline) and after the 14th days of ACTH treatment (follow-up). Clinical response to treatment was defined as seizure freedom for at least seven consecutive days during treatment. Relapse was defined as recurrence of seizures after initial response. Electrophysiological assessment included five minutes of NREM sleep EEG. Spike quantification was carried out by the manual spike counts (MSC) and automatic spike counting algorithm (ASCA) utilizing Persyst (Version 13, Solana Beach, CA). Additionally, delta power (DP) was utilized to quantify slow wave activity on the EEG. Results: Fifty IS patients (29 males; 58%) were included.Median age of IS onset was 5.2 (4.1-8) months. Median time to treatment and follow-up duration were 19 (8.7-61.5) and 30 (22-40.5) days, respectively. Etiologies consisted of the genetic / inborn metabolic error: 11 (22%), unknown: 15 (30%), structural brain abnormality: 24 (48%). Spasm frequency (Sf) decreased after ACTH treatment [median (weekly):192 vs. 70, p Conclusions: ACTH treatment in IS patients has a high clinical response rate, and this is corroborated by improvement in electrophysiological measures of spike frequency and burden of slowing. More detailed EEG analysis beyond hypsarrhythmia, such as DP and spike burden assessment may provide additional information for response and relapse assessment of patients with IS and EE. Funding: Mallinckrodt
Neurophysiology