Detailed clinical course of fatal acute encephalopathy in children

Abstract

Objective: Although the mortality among previously healthy children with acute encephalopathy (AE) is approximately 5%, their detailed clinical course has not been clarified. The objective of the present study was to describe the detailed clinical course, in minutes, of fatal AE. Methods: We retrospectively reviewed the medical records of five patients (from 6 months to 14 years of age) who previously had no neurological disorders and were diagnosed with brain death due to AE between 2002 and 2018 at Kobe Children's Hospital. Results: The initial clinical symptoms were convulsion in three cases and impaired consciousness in two. The earliest noted brain imaging abnormality was 7.5 h after neurological symptom detection. Liver enzymes and creatinine levels increased at initial examination, and sodium elevated gradually. All patients met the criteria of systemic inflammatory response syndrome, disseminated intravascular coagulation, and shock within 14 h of symptom detection. High dose steroids and targeted temperature management were initiated 3.5-14 h after onset. Despite these therapies, Tomioka et al. patients were diagnosed with brain death from 16 h to 4 days after initial neurological symptoms. AE diagnoses were made between 4 h 29 min and 4 days after initial neurological symptoms and included hemorrhagic shock and encephalopathy syndromes, Reye-like syndrome, and acute necrotizing encephalopathy in two, two, and one patient(s), respectively. Conclusions: We revealed the time series' of clinical events (e.g. SIRS, shock, DIC, AE diagnosis, brain death, and treatments) and laboratory findings relative to initial neurological symptom in fatal AE.

Early steroid pulse therapy for children with suspected acute encephalopathy: A historical cohort study

“…Previously, we reported that clinical symptoms were dramatically worsened between a few and 13 hours from onset in a review on fatal acute encephalopathy [13]. Taken together with the above report and previous reports regarding treatment time-windows for various neuro-critical conditions including hypoxia, stroke, status epilepticus, and acute encephalopathy [20][21][22][23][24][25], we hypothesize that very early intervention within several hours is needed for neuroprotection in severe acute encephalopathy.…”

“…The prognosis was determined using the Pediatric Cerebral Performance Category Scale (PCPC) [11] at the last evaluation, within 30 months of onset, with a PCPC score of 1-2 and 3-6 being de ned as good and poor, respectively. Onset was de ned as the time of initial recognition of neurological symptoms, including convulsions or impaired consciousness, based on previously determined criteria [8,10,12,13].…”

Pediatric Encephalopathy and Complex Febrile Seizures

“…As seen in our patients, elevated liver enzymes, lactate dehydrogenase, ammonia, creatinine and respiratory acidosis have been reported to be more common in AESD than in febrile seizures. 1 , 4 , 8 , 9 Also, like our patients, diffusely slow EEG with lack of sleep spindles within 48 hours of disease onset has also been considered to be a potential early biomarker of AESD. 7 While there is still no definitive early biomarker of AESD, each case in our series had some combination of these predictive factors.…”

Early steroid pulse therapy for children with suspected acute encephalopathy

“…Onset was defined as the time of initial recognition of neurological symptoms, including convulsions or impaired consciousness, based on previously determined criteria. [ 8 , 10 , 12 , 13 ]…”