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

被引:11
作者
Wan, Lin [1 ,2 ,3 ]
Lei, Yan-Qin [4 ]
Liu, Xin-Ting [1 ,2 ,3 ]
Chen, Jian [1 ,2 ]
Yeh, Chien-Hung [5 ]
Zhang, Chu-Ting [5 ]
Wang, Xiao-An [4 ]
Shi, Xiu-Yu [1 ,2 ,6 ]
Wang, Jing [1 ,2 ]
Zhang, Bo [7 ,8 ]
Zou, Li-Ping [1 ,2 ,3 ,6 ]
Yang, Guang [1 ,2 ,3 ,6 ]
机构
[1] Peoples Liberat Army Gen Hosp, Senior Dept Pediat, Med Ctr 7, Beijing 100000, Peoples R China
[2] Chinese Peoples Liberat Army Gen Hosp, Med Ctr 1, Dept Pediat, Beijing, Peoples R China
[3] Med Sch Chinese Peoples Liberat Army, Beijing, Peoples R China
[4] Brainup Inst Sci & Technol, Chongqing, Peoples R China
[5] Beijing Inst Technol, Beijing, Peoples R China
[6] Southern Med Univ, Sch Clin Med 2, Guangzhou, Peoples R China
[7] Boston Childrens Hosp, Dept Neurol, Harvard Med Sch, Boston, MA USA
[8] Boston Childrens Hosp, ICCTR Biostat & Res Design Ctr, Harvard Med Sch, Boston, MA USA
关键词
Infantile spasms; Relapse; Risk factors; BASED score; Adrenocorticotropic hormone; Dynamic functional connectivity; WEST-SYNDROME; EPILEPTIC SPASMS; ADRENOCORTICOTROPIC HORMONE; OUTCOMES; HYPSARRHYTHMIA; MULTICENTER; VIGABATRIN; THERAPY; SAFETY;
D O I
10.1007/s40120-022-00347-7
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
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.
引用
收藏
页码:835 / 849
页数:15
相关论文
共 36 条
[1]   Atlas of Electroencephalography, 3rd Edition [J].
Asadi-Pooya, Ali Akbar ;
Dlugos, Dennis ;
Skidmore, Christopher ;
Sperling, Michael R. .
EPILEPTIC DISORDERS, 2017, 19 (03) :384-384
[2]   Magnetic resonance imaging findings in children with spasmus nutans [J].
Bowen, Meredith ;
Peragallo, Jason H. ;
Kralik, Stephen F. ;
Poretti, Andrea ;
Huisman, Thierry A. G. M. ;
Soares, Bruno P. .
JOURNAL OF AAPOS, 2017, 21 (02) :127-130
[3]   How do the many etiologies of West syndrome lead to excitability and seizures? The corticotropin releasing hormone excess hypothesis [J].
Brunson, KL ;
Eghbal-Ahmadi, M ;
Baram, TZ .
BRAIN & DEVELOPMENT, 2001, 23 (07) :533-538
[4]   Disparate treatment outcomes according to presence of pathogenic mutations in West syndrome [J].
Choi, Han Som ;
Ko, Ara ;
Kim, Se Hee ;
Lee, Seung-Tae ;
Choi, Jong Rak ;
Lee, Joon Soo ;
Kim, Heung Dong ;
Kang, Hoon-Chul .
EPILEPSIA, 2021, 62 (07) :1656-1664
[5]   Multicenter long-term follow-up of children with idiopathic West syndrome: ACTH versus vigabatrin [J].
Cohen-Sadan, S. ;
Kramer, U. ;
Ben-Zeev, B. ;
Lahat, E. ;
Sahar, E. ;
Nevo, Y. ;
Eidlitz, T. ;
Zeharia, A. ;
Kivity, S. ;
Goldberg-Stern, H. .
EUROPEAN JOURNAL OF NEUROLOGY, 2009, 16 (04) :482-487
[6]   Efficacy and tolerability of the ketogenic diet versus high-dose adrenocorticotropic hormone for infantile spasms: A single-center parallel-cohort randomized controlled trial [J].
Dressler, Anastasia ;
Benninger, Franz ;
Trimmel-Schwahofer, Petra ;
Groeppel, Gudrun ;
Porsche, Barbara ;
Abraham, Klaus ;
Muehlebner, Angelika ;
Samueli, Sharon ;
Male, Christoph ;
Feucht, Martha .
EPILEPSIA, 2019, 60 (03) :441-451
[7]   What is West syndrome? [J].
Dulac, O .
BRAIN & DEVELOPMENT, 2001, 23 (07) :447-452
[8]   Incidence and outcome of epilepsy syndromes with onset in the first year of life: A retrospective population-based study [J].
Gaily, Eija ;
Lommi, Markus ;
Lapatto, Risto ;
Lehesjoki, Anna-Elina .
EPILEPSIA, 2016, 57 (10) :1594-1601
[9]   Evidence-based guideline update: Medical treatment of infantile spasms Report of the Guideline Development Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society [J].
Go, C. Y. ;
Mackay, M. T. ;
Weiss, S. K. ;
Stephens, D. ;
Adams-Webber, T. ;
Ashwal, S. ;
Snead, O. C., III .
NEUROLOGY, 2012, 78 (24) :1974-1980
[10]   Treatment of infantile spasms:: Results of a population-based study with vigabatrin as the first drug for spasms [J].
Granström, ML ;
Gaily, E ;
Liukkonen, E .
EPILEPSIA, 1999, 40 (07) :950-957