Authors :
Presenting Author: Seoyun Jang, MD – Department of Pediatrics, Seoul National University Children’s Hospital, Seoul, Korea
Minhye Kim, MD – Department of Pediatrics, Seoul National University Children’s Hospital, Seoul, Korea; Hye Jin Kim, MD – Department of Pediatrics, Seoul National University Children’s Hospital, Seoul, Korea; Jae So Cho, MD – Department of Clinical Genomics, Seoul National University Hospital, Seoul, Korea; Soo yeon Kim, MD – Department of Pediatrics, Seoul National University Children’s Hospital, Seoul, Korea; Byung Chan Lim, MD – Department of Pediatrics, Seoul National University Children’s Hospital, Seoul, Korea; Jong Hee Chae, MD – Department of Pediatrics, Seoul National University Children’s Hospital, Seoul, Korea; Ki Joong Kim, MD – Department of Pediatrics, Seoul National University Children’s Hospital, Seoul, Korea; Woo Joong Kim, MD – Corresponding author, Department of Pediatrics, Seoul National University Children’s Hospital, Seoul, Korea
Rationale:
Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is characterized by prolonged febrile seizures with no abnormalities on brain MRI at the first manifestation, followed by secondary seizures after a few days. It is distinguished by a distinctive reduced diffusion pattern in the subcortical white matter on MRI. AESD is known to be the most common form of acute encephalopathy in Japan but is rarely reported in Korea. In this study, we present four pediatric cases of AESD diagnosed based on clinic-radiological features.Methods:
We recruited and analyzed pediatric patients who met the criteria for AESD diagnosis through a retrospective chart review. Clinical information was collected, including treatment course, outcomes, and serial brain MRI findings.Results:
This study included four patients (three girls and one boy aged 19 to 36 months) diagnosed with AESD. All patients initially presented with prolonged febrile seizures. The seizure semiology was generalized tonic-clonic seizures, and all the patients regained consciousness within two days. The secondary seizure occurred three to five days later. The secondary seizures were either generalized tonic-clonic or clusters of focal clonic seizures. Initial MRIs were performed for three patients two days after the onset, showing no abnormal findings initially but later revealing diffusion restriction in bilateral subcortical white matter. One patient, who underwent a relatively late initial brain MRI (4 days after onset), showed diffusion restriction. The last follow-up MRIs of all patients showed diffuse atrophy. Although slowly improving with rehabilitation, all patients experienced severe neurological impairment.Conclusions:
In this report, we present a case series of AESD in the Korean pediatric population. AESD can be easily overlooked since initial neuroimaging frequently appears normal. However, early recognition is important as most patients suffer permanent neurological sequelae.
Funding: No funding received