Acute encephalopathy in children with tuberous sclerosis complex

Abstract Objective We examined the clinical manifestations of acute encephalopathy (AE) and identify risk factors for AE in children with tuberous sclerosis complex (TSC). Methods The data 11 clinically diagnosed TSC associated 109 alone aged 4 years or older were collected from 13 hospitals. Results Of AE, 5 had histories febrile seizures (FS), all status epilepticus (FSE). developed within 24 h after fever onset lasting 30 min longer. All coma seizure cessation. Head magnetic resonance imaging (MRI) revealed widespread abnormalities cerebral cortex, subcortical white matter, corpus callosum, basal ganglia, thalamus. One child died; seven severe neurological sequelae; other three, mild sequelae. Logistic regression analysis that a history FSE was correlated development AE. Significance characterized by sudden fever, followed coma, brain edema evident on MRI, poor outcomes. A factor