TY - JOUR
T1 - Early Intervention With Adrenocorticotropin for Acute Encephalopathy-Associated Epileptic Spasms
T2 - Report of Two Cases
AU - Yonemoto, Kousuke
AU - Ichimiya, Yuko
AU - Sanefuji, Masafumi
AU - Kaku, Noriyuki
AU - Sakata, Ayumi
AU - Baba, Rieko
AU - Yamashita, Fumiya
AU - Akamine, Satoshi
AU - Torio, Michiko
AU - Ishizaki, Yoshito
AU - Maehara, Yoshihiko
AU - Sakai, Yasunari
AU - Ohga, Shouichi
N1 - Funding Information:
The author(s) disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: This study was supported by JSPS KAKENHI Grant Number JP15K0962 (YS), JP17K16301 (MT), JP17K16301 (YI), a Health and Labour Sciences Research Grant on Evidence-based Early Diagnosis and Treatment Strategies for Neuroimmunological Diseases from the Ministry of Health, Labour and Welfare of Japan, Life Science Foundation of Japan, Takeda Science Foundation, The Mother and Child Health Foundation, The Japan Epilepsy Research Foundation, and Kawano Masanori Memorial Public Interest Incorporated Foundation for Promotion of Pediatrics (YS).
Funding Information:
We thank the families for approving this study; Soo-Young Lee and Ryutaro Kira at Fukuoka Children?s Hospital for helpful discussions; and members at the Pediatric Intensive Care Unit of Kyushu University Hospital for their elaborate care of these patients. The author(s) disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: This study was supported by JSPS KAKENHI Grant Number JP15K0962 (YS), JP17K16301 (MT), JP17K16301 (YI), a Health and Labour Sciences Research Grant on Evidence-based Early Diagnosis and Treatment Strategies for Neuroimmunological Diseases from the Ministry of Health, Labour and Welfare of Japan, Life Science Foundation of Japan, Takeda Science Foundation, The Mother and Child Health Foundation, The Japan Epilepsy Research Foundation, and Kawano Masanori Memorial Public Interest Incorporated Foundation for Promotion of Pediatrics (YS).
Publisher Copyright:
© EEG and Clinical Neuroscience Society (ECNS) 2018.
PY - 2019/1/1
Y1 - 2019/1/1
N2 - Purpose. Acute encephalopathy with biphasic seizures and reduced diffusion (AESD) is a leading cause of childhood-onset encephalopathy in Japan. Children with AESD frequently develop intractable epilepsy, whereas their treatment options remain to be determined. Method. We present 2 unrelated girls, who developed AESD at 25 months (case 1) and 12 months of age (case 2). Both cases underwent intensive cares from the first day of illness, whereas severe neurological impairments were left on discharge. They showed repeated signs of epileptic spasms at 2 months (case 1) and 8 months (case 2) after the onset of AESD. Video-monitoring electroencephalograms (EEG) detected the recurrent attacks accompanying slow-wave bursts and transient suppressions of the precedent epileptiform discharges, as typically observed in epileptic spasms. Results. Intramuscular injection of adrenocorticotropic hormone (ACTH, 0.0125 mg/kg/d) was introduced within 1 month from the onset of epileptic spasms and continued for 2 weeks. The ACTH treatment disrupted the paroxysmal activity in EEG, and it has relieved these patients from epileptic seizures for more than 1 year. Conclusion. This report illustrates the potential efficacy of ACTH for a group of children with epileptic spasms after AESD.
AB - Purpose. Acute encephalopathy with biphasic seizures and reduced diffusion (AESD) is a leading cause of childhood-onset encephalopathy in Japan. Children with AESD frequently develop intractable epilepsy, whereas their treatment options remain to be determined. Method. We present 2 unrelated girls, who developed AESD at 25 months (case 1) and 12 months of age (case 2). Both cases underwent intensive cares from the first day of illness, whereas severe neurological impairments were left on discharge. They showed repeated signs of epileptic spasms at 2 months (case 1) and 8 months (case 2) after the onset of AESD. Video-monitoring electroencephalograms (EEG) detected the recurrent attacks accompanying slow-wave bursts and transient suppressions of the precedent epileptiform discharges, as typically observed in epileptic spasms. Results. Intramuscular injection of adrenocorticotropic hormone (ACTH, 0.0125 mg/kg/d) was introduced within 1 month from the onset of epileptic spasms and continued for 2 weeks. The ACTH treatment disrupted the paroxysmal activity in EEG, and it has relieved these patients from epileptic seizures for more than 1 year. Conclusion. This report illustrates the potential efficacy of ACTH for a group of children with epileptic spasms after AESD.
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U2 - 10.1177/1550059418786381
DO - 10.1177/1550059418786381
M3 - Article
C2 - 29984606
AN - SCOPUS:85049807340
SN - 1550-0594
VL - 50
SP - 51
EP - 55
JO - Clinical EEG and Neuroscience
JF - Clinical EEG and Neuroscience
IS - 1
ER -