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Published on: May 16, 2015
Early Intervention With Adrenocorticotropin for Acute Encephalopathy-Associated Epileptic Spasms: Report of Two Cases
Kousuke Yonemoto1, Yuko Ichimiya1,2, Masafumi Sanefuji1
11 Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
Insights
Adrenocorticotropic hormone (ACTH) effectively treated epileptic spasms in two children with acute encephalopathy with biphasic seizures and reduced diffusion (AESD). This treatment provided over a year of seizure relief, offering a new therapeutic option.
Area of Science:
- Pediatric Neurology
- Epileptology
- Neurocritical Care
Background:
- Acute encephalopathy with biphasic seizures and reduced diffusion (AESD) is a significant cause of childhood encephalopathy in Japan.
- Children with AESD often develop difficult-to-treat epilepsy, necessitating novel therapeutic strategies.
Observation:
- Two unrelated girls diagnosed with AESD at 12 and 25 months of age experienced severe neurological impairments despite intensive care.
- Both patients developed recurrent epileptic spasms months after the initial AESD onset, confirmed by EEG as characteristic epileptic spasms.
Findings:
- Intramuscular adrenocorticotropic hormone (ACTH) treatment was initiated within one month of epileptic spasm onset.
- ACTH administration for two weeks successfully suppressed paroxysmal EEG activity and eliminated seizures for over a year in both cases.
Implications:
- This case study suggests adrenocorticotropic hormone (ACTH) may be a promising treatment for children experiencing epileptic spasms secondary to AESD.
- Further research is warranted to confirm the efficacy and safety of ACTH in a larger cohort of AESD patients with epilepsy.
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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