Authors :
Presenting Author: Sahar Rostamian, MD – Boston Children's Hospital
Fatemeh Mohammad Alizadeh Chafjiri, MD – Boston Children's Hospital
Leena Abdelmoity, MD Candidate – Boston Children's Hospital
Rajib Kanti Dey, MBBS – Boston Children's Hospital
Stephanie Dailey, BA – Boston Children's Hospital, 300 Longwood Ave, Boston, MA 02115, USA
Luisa Atunes Ortega, MD – Boston Children's Hospital
Elijah Simon, BS – Boston Children's Hospital
Sanjay Prabhu, MBBS, FRCR – Boston Children's Hospital
Jennifer Gettings, BMBS, FRCPC – Boston Children's Hospital
Agnieszka Kielian, MD – Boston Children's Hospital
Tobias Loddenkemper, MD – Boston Children's Hospital, 300 Longwood Ave, Boston, MA 02115, USA
Rationale: Refractory status epilepticus (RSE) is a life-threatening neurologic emergency with risk for structural brain injury. While MRI can detect seizure-related changes, data on lesion characteristics and clinical associations in pediatric RSE are limited. This study aims to characterize radiographic findings, timing relative to RSE onset, anatomical distribution, and clinical correlations in a pediatric RSE cohort.
Methods:
We retrospectively reviewed 212 pediatric RSE patients at Boston Children’s Hospital (2013–2023). Inclusion criteria were patients aged 1 month to 21 years who underwent brain MRI within 30 days of RSE onset (n=54). Patients were excluded if an MRI was not performed, conducted beyond the 30-day window, or if clinical/imaging data were incomplete. MRI was assessed for lesion timing (acute or chronic), radiographic features (e.g., diffusion restriction, FLAIR hyperintensity, hippocampal sclerosis), and presence of hemorrhages or tumors. Radiologic findings were recorded per patient; individuals could present with more than one finding. Clinical data, including seizure semiology, etiology, presence of genetic syndromes, and ICU admission, were extracted from the medical health record.
Results: Among 54 patients (median age 5.8 years, IQR 1.3–9.3; 55.6% male), 40 (74.1%) had abnormal MRI findings. Chronic
lesions were present in 20/40 (50.0%), with the most common findings including hippocampal abnormalities (4/20, 20.0%), atrophy or volume loss (4/20, 20.0%), cortical malformations (4/20, 20.0%), and gliosis (3/20, 15.0%). Acute
lesions were found in 20/40 (50.0%), with the most frequent radiographic findings included cortical or diffuse edema (7/20, 35.0%), diffusion restriction (3/20, 15.0%), T2/FLAIR hyperintensities (3/20, 15.0%), hemorrhage (3/20, 15.0%), and leptomeningeal enhancement (2/20, 10.0%). Tumors were observed in 4/40 (10.0%), including medulloblastoma, frontal glioma, and residual masses. Based on clinical data, structural etiologies were most common (15/40, 37.5). Generalized tonic-clonic seizures were the most frequent semiology overall (35/54, 64.8%) and among those with abnormal MRI (24/40, 60.0%). Ten (18.5%) patients had an identified genetic syndrome. The most common syndromes were Wolf-Hirschhorn syndrome (n=2), followed by cardio-facio-cutaneous syndrome, DCX-related lissencephaly, Gorlin syndrome, holoprosencephaly spectrum disorder, Neurofibromatosis type 1, Sanfilippo syndrome, suspected mitochondrial disorder, and tuberous sclerosis complex (each n=1). Most patients required ICU admission (50/54, 92.6%), with higher rates among those with abnormal MRI (97.5%) compared to those with normal imaging (78.6%) (p< 0.05) (Table 1).