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Involuntary movements and coma as the prognostic marker for acute encephalopathy with biphasic seizures and late
Sooyoung Lee1, Masafumi Sanefuji2, Michiko Torio3
1Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan; Emergency and Critical Care Center, Kyushu University Hospital, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan; Fukuoka Children's Hospital, 50101 Kashiiteriha, Higashi-ku, Fukuoka 813-0017, Japan.
Insights
Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) can lead to severe outcomes. Early coma or involuntary movements predict worse neurological sequelae in affected children.
Area of Science:
- Pediatric Neurology
- Infectious Diseases
- Neuroimaging
Background:
- Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is a severe childhood neurological condition often following infection.
- While AESD is linked to poor neurological outcomes, prognostic indicators are not well-established.
- Understanding predictors of severe sequelae is crucial for timely intervention.
Purpose of the Study:
- To identify clinical and imaging characteristics associated with severe neurological sequelae in children with AESD.
- To elucidate early indicators that may predict long-term neurodevelopmental outcomes in AESD patients.
Main Methods:
- Retrospective analysis of clinical, laboratory, and brain imaging data from AESD patients.
- Comparison between patients with severe (n=8) and non-severe (n=12) neurodevelopmental outcomes.
- Quantitative MRI assessment of white matter lesions in the second phase of AESD.
Main Results:
- Coma and involuntary movements (dystonia, oral dyskinesia) before the second phase were more frequent in the severe outcome group (p=0.014, p=0.018).
- Elevated serum alanine aminotransferase levels were observed in patients with severe sequelae (p=0.001).
- More extensive cerebral and basal ganglia lesions on MRI were evident in the severe outcome group (p=0.015, p=0.011, p=0.020).
Conclusions:
- Early onset of coma or involuntary movements may predict the extent of brain lesions and poor neurological outcomes in AESD.
- Serum ALT levels and lesion burden on MRI are potential indicators of AESD severity.
- Identifying these prognostic factors can aid in managing children with AESD and anticipating long-term neurological deficits.
Abstract:
Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) occurs in children associated with infection. It is characterized by a prolonged febrile seizure in the first phase, and a cluster of seizures, deterioration of consciousness and the white matter lesions with reduced diffusion in the second phase. The patients often have severe neurological sequelae, but the prognostic indicators remain unknown. The present study aimed to clarify the characteristics of AESD patients who subsequently exhibited severe neurological sequelae. We retrospectively analyzed the clinical and laboratory findings along with the brain imaging in patients who had severe (n=8) and non-severe neurodevelopmental outcomes (n=12). Severe group more frequently showed coma (p=0.014) or involuntary movements including dystonia and oral dyskinesia (p=0.018) before the second phase than non-severe group. Severe group exhibited higher levels of serum alanine aminotransferase than non-severe group (p=0.001). Quantitatively assessed MRI in the second phase revealed that severe group had more extensive lesions than non-severe group, in the anterior (p=0.015) and posterior parts (p=0.011) of the cerebrum and basal ganglia (p=0.020). Early appearing involuntary movements or coma might account for the extension of acute brain lesions and the poor neurological outcomes in AESD patients.
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