痙攣重積型（二相性）急性脳症

BACKGROUND: Infection-triggered encephalopathy syndromes (ITES) are acute, para-infectious disorders that can cause disability or death. Recognition is increasingly important in viral pandemics, but clinical features and outcomes remain poorly understood. METHODS: PubMed search (inception to 6 March 2024) identified studies with published individual patient data (raw data were not collected from authors). The search was updated on 14 May 2026 using the same terms to identify reports and cases published after the data extraction cutoff. Data were extracted by paediatric neurologists using a standardised proforma. Major syndromes included acute encephalopathy with biphasic seizures and late reduced diffusion (AESD), acute necrotising encephalopathy (ANE), acute shock with encephalopathy and multiorgan failure (ASEM), hemiconvulsion-hemiplegia-epilepsy syndrome (HHE), febrile infection-related epilepsy syndrome (FIRES) and mild encephalopathy with reversible splenial lesion (MERS). We performed a pooled analysis of individual-level published data investigating patient characteristics, infections, and clinical outcomes measured by six-month modified Rankin Scale (mRS). Infection-syndrome associations were analysed with chi-square normalised residuals. FINDINGS: 1946 patients from 656 studies (by ascending age of onset) included 164 ASEM (median age 0.78 years), 217 AESD (1.3 years), 95 HHE (2 years), 414 ANE (3.4 years), 562 FIRES (9 years), and 422 MERS (9.25 years). An updated search identified 658 cases from 171 studies that could be eligible for inclusion. AESD was linked to human herpesvirus 6 (z = 12.87); ANE with influenza A (z = 11.1), SARS-CoV-2 (z = 9.1) and influenza B (z = 4.3); MERS with rotavirus infection (z = 7.48); and FIRES with absence of microbiological identification (z = 18.2) (all p < 0.001).Neuroimaging was often delayed. Magnetic resonance imaging (MRI) brain restricted diffusion was the commonest finding, typically bilateral (except HHE). Characteristic patterns included thalamic involvement with or without haemorrhagic changes in ANE, corpus callosum involvement in MERS, and subcortical white matter involvement in AESD and HHE. Cerebrospinal fluid pleocytosis occurred mostly in FIRES and MERS, and elevated protein in ANE.Immunotherapy (commonly steroids, immunoglobulin) including biologics (anakinra, tocilizumab) was used most in ANE and FIRES. Acute mortality was 12% (227/1946), highest in ASEM (50%) and ANE (35%). There was good six-month outcome (mRS 0-2) in 52% (95% CI 50-55; 615/1174): MERS 99% (95% CI 97-100; 323/326), AESD 54% (95% CI 44-64; 50/93), FIRES 38% (95% CI 33-44; 120/314), ANE 33% (95% CI 27-38; 88/269), HHE 16% (95% CI 7-32; 5/32), and ASEM 15% (95% CI 9-24; 14/91). INTERPRETATION: ITES showed distinct demographic, clinico-radiological features and outcomes. This largest ITES cohort to date highlights the importance of timely imaging, intervention, and future global collaboration to advance diagnosis, treatment, and pandemic preparedness. FUNDING: No funding was received for this work.

OBJECTIVE(S): Infection Triggered Encephalopathy Syndrome (ITES) is a rare but potentially severe complication of infections in children. ITES is a syndrome of acute encephalopathy that occurs in the context of a febrile illness and hypothesised to result from a cytokine mediated infection-triggered cytotoxicity in the central nervous system (CNS) without parenchymal inflammatory cell infiltration. ITES is considered distinct from infectious and immune-mediated encephalitis. We aimed to describe the demographics, associated pathogens, clinical spectrum and outcomes of ITES in Australian children from May 2013 until June 2024. STUDY DESIGN: We extracted and analysed data from the prospective, multicentre Australian Childhood Encephalitis (ACE) study. Cases of ITES aged ≤14 years were reviewed and categorised by a multidisciplinary expert panel. We estimated the incidence for specific sub-types of ITES. RESULTS: Of 1160 cases of Australian children with suspected encephalitis, 220 met criteria for ITES. In 83% (n=183) an associated pathogen was detected of which influenza (50%; n=109), enterovirus (8%; n=18), SARS-CoV-2 (COVID-19) (7%; n=15), and adenovirus (6%; n=13) were most common. The median age of affected children was 4.3 years (IQR 2-8.1). Of sub-types of ITES, the most frequent was Acute Necrotising Encephalopathy (ANE) (n=28, 13%), followed by Acute Encephalopathy with biphasic Seizures and late reduced Diffusion (AESD) (n=20, 9%), then Mild Encephalopathy with Reversible Splenial lesion (MERS) (n=21, 10%), although most children did not have a specific sub-type (n=135, 61%). For ITES sub-types, incidence was highest for ANE (0.82 per 1,000,000), and lowest for Febrile Infection Related Epilepsy Syndrome (FIRES, 0.23 per 1,000,000). Most children with ITES (60%; n=132) recovered fully. Poorer outcomes were seen in children diagnosed with ITES sub-types including ANE, AESD, and Acute Fulminant Cerebral Edema (AFCE). CONCLUSION(S): ITES is an uncommon complication of common infections in Australian children. Influenza was the most frequently associated pathogen, with ANE the most frequent specific ITES sub-type. Outcomes are variable, but concerningly poor for some ITES sub-types including ANE.

BACKGROUND: This study aimed to characterize late seizures in infants with traumatic brain injury with a biphasic clinical course and late reduced diffusion (TBIRD) using continuous electroencephalography (cEEG). METHODS: The present, single-center, retrospective case series was conducted at a single center between 2020 and 2025. TBIRD was defined as early post-traumatic seizures or impaired consciousness, seizure onset 3-6 days after injury, and the late appearance of high signal intensity areas in the subcortical white matter on diffusion-weighted magnetic resonance imaging. cEEG recordings and clinical and imaging data were reviewed by pediatric neurologists using standardized critical care electroencephalography terminology. RESULTS: Seven infants aged 3-9 months (median age: 5 months) presenting with early convulsive status epilepticus, acute subdural hematoma, and a high Tada score (≥4) met the inclusion criteria. The seizures emerged 3-5 days after injury and initially occurred as electrographic seizures without clinical correlates, clustered in all the patients, and evolved into electrographic status epilepticus in five patients. The seizures typically arose from lateralized periodic discharges or lateralized rhythmic delta activity, most often in the occipital region or in the areas adjacent to the hematoma. CONCLUSIONS: These findings indicated that late seizures in infantile TBIRD are frequently occult at the bedside and may be overlooked without cEEG. cEEG monitoring may facilitate earlier recognition of late seizures and enable timely diagnosis and management of TBIRD.

BACKGROUND: Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is a common pediatric acute encephalopathy characterized by biphasic seizures and development of a bright tree appearance (BTA) on diffusion-weighted MR imaging (DWI). Lesion extent and distribution have been linked to neurological prognosis, but the effects of lesion depth remained unclear. METHODS: We retrospectively analyzed 25 children with AESD onset between 1991 and 2022. Acute-phase magnetic resonance imaging (MRI) (4-14 days after onset) was evaluated using DWI. Lesions in 10 cerebral regions were classified by depth as BTA limited to the subcortical area (BTA-L) or BTA with extension into the deep white matter (BTA-E). Neurological outcomes included pediatric cerebral performance category scores and residual motor paralysis at ≥12 months. Associations between MRI findings and outcomes were analyzed using univariate logistic regression and receiver operating characteristic analyses. RESULTS: Thirteen patients had favorable outcomes, whereas 12 had unfavorable outcomes based on pediatric cerebral performance category scores. Bilateral temporal lobe involvement with BTA-L/E (odds ratio [OR], 16.8), bilateral frontal lobe involvement with BTA-E (OR, 10.0), and bilateral thalamus/basal ganglia lesions (OR, 12.0) showed significant associations with unfavorable outcomes. Residual motor paralysis was associated with lesion burden and distribution. Rolandic BTA-E showed an association with motor paralysis (OR, 90.0), even in unilateral cases. This study should be considered exploratory due to the small sample size and the lack of adjustment for multiple comparisons. CONCLUSIONS: Acute-phase MRI findings in AESD were associated with long-term neurological outcomes. Lesion distribution and deep white matter extension may serve as potential prognostic factors. Frontal BTA-E and Rolandic BTA-E were associated with cognitive impairment and motor paralysis, respectively.