Early Intervention With Adrenocorticotropin for Acute Encephalopathy-Associated Epileptic Spasms: Report of Two Cases

Kousuke Yonemoto, Yuko Ichimiya, Masafumi Sanefuji, Noriyuki Kaku, Ayumi Sakata, Rieko Baba, Fumiya Yamashita, Satoshi Akamine, Michiko Torio, Yoshito Ishizaki, Yoshihiko Maehara, Yasunari Sakai, Shouichi Ohga

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish
Pages (from-to)51-55
Number of pages5
JournalClinical EEG and Neuroscience
Volume50
Issue number1
DOIs
Publication statusPublished - Jan 1 2019

Fingerprint

Spasm
Brain Diseases
Adrenocorticotropic Hormone
Electroencephalography
Sick Leave
Intramuscular Injections
Critical Care
Epilepsy
Japan
Seizures
Therapeutics

All Science Journal Classification (ASJC) codes

  • Neurology
  • Clinical Neurology

Cite this

Early Intervention With Adrenocorticotropin for Acute Encephalopathy-Associated Epileptic Spasms : Report of Two Cases. / Yonemoto, Kousuke; Ichimiya, Yuko; Sanefuji, Masafumi; Kaku, Noriyuki; Sakata, Ayumi; Baba, Rieko; Yamashita, Fumiya; Akamine, Satoshi; Torio, Michiko; Ishizaki, Yoshito; Maehara, Yoshihiko; Sakai, Yasunari; Ohga, Shouichi.

In: Clinical EEG and Neuroscience, Vol. 50, No. 1, 01.01.2019, p. 51-55.

Research output: Contribution to journalArticle

Yonemoto, Kousuke ; Ichimiya, Yuko ; Sanefuji, Masafumi ; Kaku, Noriyuki ; Sakata, Ayumi ; Baba, Rieko ; Yamashita, Fumiya ; Akamine, Satoshi ; Torio, Michiko ; Ishizaki, Yoshito ; Maehara, Yoshihiko ; Sakai, Yasunari ; Ohga, Shouichi. / Early Intervention With Adrenocorticotropin for Acute Encephalopathy-Associated Epileptic Spasms : Report of Two Cases. In: Clinical EEG and Neuroscience. 2019 ; Vol. 50, No. 1. pp. 51-55.
@article{b8cf5bc0e6bb4d44b5a20d8d21b2317d,
title = "Early Intervention With Adrenocorticotropin for Acute Encephalopathy-Associated Epileptic Spasms: Report of Two Cases",
abstract = "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.",
author = "Kousuke Yonemoto and Yuko Ichimiya and Masafumi Sanefuji and Noriyuki Kaku and Ayumi Sakata and Rieko Baba and Fumiya Yamashita and Satoshi Akamine and Michiko Torio and Yoshito Ishizaki and Yoshihiko Maehara and Yasunari Sakai and Shouichi Ohga",
year = "2019",
month = "1",
day = "1",
doi = "10.1177/1550059418786381",
language = "English",
volume = "50",
pages = "51--55",
journal = "Clinical EEG and Neuroscience",
issn = "1550-0594",
publisher = "EEG and Clinical Neuroscience Society (ECNS)",
number = "1",

}

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

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.

UR - http://www.scopus.com/inward/record.url?scp=85049807340&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85049807340&partnerID=8YFLogxK

U2 - 10.1177/1550059418786381

DO - 10.1177/1550059418786381

M3 - Article

C2 - 29984606

AN - SCOPUS:85049807340

VL - 50

SP - 51

EP - 55

JO - Clinical EEG and Neuroscience

JF - Clinical EEG and Neuroscience

SN - 1550-0594

IS - 1

ER -