Assessing Risk for Relapse among Children with Infantile Spasms Using the Based Score after ACTH Treatment: A Retrospective Study

Lin Wan; Yan-Qin Lei; Xin-Ting Liu; Jian Chen; Chien-Hung Yeh; Chu-Ting Zhang; Xiao-An Wang; Xiu-Yu Shi; Jing Wang; Bo Zhang; Li-Ping Zou; Guang Yang

doi:10.1007/s40120-022-00347-7

Assessing Risk for Relapse among Children with Infantile Spasms Using the Based Score after ACTH Treatment: A Retrospective Study

Neurol Ther. 2022 Jun;11(2):835-849. doi: 10.1007/s40120-022-00347-7. Epub 2022 Apr 15.

Authors

Lin Wan^#^{1

2

3}, Yan-Qin Lei^#⁴, Xin-Ting Liu^#^{1

2

3}, Jian Chen^{1

2}, Chien-Hung Yeh⁵, Chu-Ting Zhang⁵, Xiao-An Wang⁴, Xiu-Yu Shi^{1

2

6}, Jing Wang^{1

2}, Bo Zhang⁷, Li-Ping Zou^{1

2

3

6}, Guang Yang^{8

9

10

11}

Affiliations

¹ Senior Department of Pediatrics, The Seventh Medical Center of PLA General Hospital, Beijing, 100000, China.
² Department of Pediatrics, The First Medical Center, Chinese PLA General Hospital, Beijing, China.
³ Medical School of Chinese People's Liberation Army, Beijing, China.
⁴ Brainup Institute of Science and Technology, Chongqing, China.
⁵ Beijing Institute of Technology, Beijing, China.
⁶ The Second School of Clinical Medicine, Southern Medical University, Guangzhou, China.
⁷ Department of Neurology and ICCTR Biostatistics and Research Design Center, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.
⁸ Senior Department of Pediatrics, The Seventh Medical Center of PLA General Hospital, Beijing, 100000, China. yangg301@126.com.
⁹ Department of Pediatrics, The First Medical Center, Chinese PLA General Hospital, Beijing, China. yangg301@126.com.
¹⁰ Medical School of Chinese People's Liberation Army, Beijing, China. yangg301@126.com.
¹¹ The Second School of Clinical Medicine, Southern Medical University, Guangzhou, China. yangg301@126.com.

^# Contributed equally.

Abstract

Introduction: Even though adrenocorticotropic hormone (ACTH) demonstrated powerful efficacy in the initially successful treatment of infantile spasms (IS), nearly one-half of patients whose spasms were once suppressed experienced relapse. There is currently no validated method for the prediction of the risk of relapse. The Burden of Amplitudes and Epileptiform Discharges (BASED) score is an electroencephalogram (EEG) grading scale for children with infantile spasms. We sought to determine whether an association exists between the BASED score after ACTH treatment and relapse after initial response with ACTH.

Methods: Children with IS who achieved initial response after ACTH treatment were selected as the study subjects. Those who experienced relapse within 12 months after ACTH treatment were categorized as the relapse group, and those who did not were categorized as the non-relapse group. Their general clinical data and EEG data (using BASED scoring) after ACTH treatment were collected, and compared between groups. Cox proportional hazards models were fit to determine factors associated with relapse.

Results: A total of 64 children with IS were enrolled in the study, of which 37 (57.8%) experienced a relapse, and the median duration after ACTH treatment was 3 (1.5, 6) months. The BASED score was significantly higher in the relapse group than in the non-relapse group. Cox modeling demonstrated that BASED score was independently associated with relapse. The patients with a score greater than or equal to 3 showed a high rate (89.3%) of relapse. The relapse group had stronger, more stable EEG functional networks than the non-relapse group, and there were obvious correlations between BASED score and functional connectivity.

Conclusion: This study suggests the BASED score after ACTH treatment has potential value as a predictor for relapse after initial response. Children with IS who have a BASED score greater than or equal to 3 after the initial response of ACTH carry a high risk of relapse within 1 year.

Keywords: Adrenocorticotropic hormone; BASED score; Dynamic functional connectivity; Infantile spasms; Relapse; Risk factors.

Abstract

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