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A case of acute encephalopathy with biphasic seizures and late reduced diffusion: Utility of arterial spin labeling
Keita Kuya1, Shinya Fujii1, Fuminori Miyoshi1
1Division of Radiology, Department of Pathophysiological Therapeutic Science, Faculty of Medicine, Tottori University, Japan.
Insights
Febrile status epilepticus (FSE) can lead to acute encephalopathy with biphasic seizures and late reduced diffusion (AESD). Early reduced cerebral blood flow (CBF) detected by arterial spin labeling may predict AESD development in children.
Area of Science:
- Neurology
- Pediatrics
- Radiology
Background:
- Febrile status epilepticus (FSE) typically has good outcomes, but some children develop acute encephalopathy with biphasic seizures and late reduced diffusion (AESD), characterized by developmental delay and epilepsy.
- Distinguishing AESD from other prolonged febrile seizures is challenging due to a lack of identified biomarkers.
Observation:
- A 1-year-old boy presented with FSE, followed by a secondary seizure cluster and developmental delay.
- Initial conventional MRI was normal, but diffusion-weighted imaging on day 5 showed reduced diffusion in bilateral frontal subcortical regions, leading to an AESD diagnosis.
- Arterial spin labeling (ASL) revealed reduced cerebral blood flow (CBF) in bilateral frontal lobes on day 1, with increased CBF in the subacute phase.
Findings:
- Reduced CBF in bilateral frontal lobes on day 1 of FSE, as detected by ASL, may serve as an early predictive marker for AESD.
- This finding contrasts with typical FSE cases where outcomes are generally favorable.
Implications:
- Early identification of AESD through ASL-assessed CBF could enable timely intervention and potentially improve patient outcomes.
- Further research is needed to validate ASL as a predictive biomarker for AESD in pediatric patients with FSE.
Abstract:
A 1-year-old boy was admitted because of febrile status epilepticus (FSE). A secondary cluster of seizures was seen on day 5 after onset, and the patient eventually displayed developmental delay. Conventional magnetic resonance imaging (MRI) showed no abnormal findings on day 1 after onset, but showed reduced diffusion in the subcortical regions of bilateral frontal lobes on day 5 after onset. Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) was diagnosed. Arterial spin labeling (ASL) revealed reduced cerebral blood flow (CBF) in bilateral frontal lobes on day 1 after onset and showed increased CBF in the corresponding region in the subacute phase. Outcomes after prolonged febrile seizures are usually good, but mental deficit and/or epilepsy often remain in AESD. Discriminating between these syndromes is difficult, because no useful biomarkers have been identified. Reduced CBF in bilateral frontal lobes was observed on ASL on day 1 of FSE in the present case, and this finding may be predictive of developing AESD.
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