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.
Abstract

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