Vitamin B6 in acute encephalopathy with biphasic seizures and late reduced diffusion

Abstract

Background:The initial presentation of acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is indistinguishable from that of complex febrile seizures (FS), which poses a great diagnostic challenge for clinicians. Excitotoxicity is speculated to be the pathogenesis of AESD. Vitamin B6 (VB6) is essential for the biosynthesis of gammaaminobutyric acid, an inhibitory neurotransmitter. The aim of this study is to investigate our hypothesis that VB6 deficiency in the brain may play a role in AESD. Methods:We obtained cerebrospinal fluid (CSF) samples from pediatric patients with AESD after early seizures and those with FS. We measured pyridoxal 5′-phosphate (PLP) and pyridoxal (PL) concentrations in the CSF samples using high-performance liquid chromatography with fluorescence detection. Results:The subjects were 5 patients with AESD and 17 patients with FS. Age did not differ significantly between AESD and FS. In AESD, CSF PLP concentration was marginally lower (p = 0.0999) and the PLP-to-PL ratio was significantly (p = 0.0417) reduced compared to those in FS. Conclusions:Although it is impossible to conclude that low PLP concentration and PLP-to-PL ratio are causative of AESD, this may be a risk factor for developing AESD. When combined with other markers, this finding may be useful in distinguishing AESD from FS upon initial presentation.

[Retracted] A Metadecomposition of Clinical Value Assessed Using aEEG in Severe Neonatal Hyperbilirubinemia

“…rough aEEG monitoring of high-risk children with nerve injury, some studies have found that aEEG appears when obvious central nervous system damage occurs, especially in children with severe injury, aEEG has a specific abnormal map [13,14].…”

Natural Variation in Vitamin B1 and Vitamin B6 Contents in Rice Germplasm

“…Although data at the population level is lacking for vitamin B 1 deficiency compared to other micronutrients, studies on Chinese cohorts showed 91.8% of children and 81.7% of adults over 60 years do not reach their dietary estimated daily intake of vitamin B 1 ( Wang et al, 2017 ; Liu et al, 2019 ). Vitamin B 6 deficiency may manifest with seizures and other neurological events, because of the role of PLP in neurotransmitter biosynthesis ( Wilson et al, 2019 ; Akiyama et al, 2020 ; Rojo-Sebastián et al, 2020 ). Genetic defects in vitamin B 6 metabolism could also lead to vitamin B 6 -dependent epilepsy ( Wilson et al, 2019 ).…”

Pediatric Encephalopathy and Complex Febrile Seizures

“…10 Consistent with this hypothesis, early initiation of vitamins and co-enzymes including vitamin B1, B6 and l-carnitine has been proposed as a therapy and can improve outcome in suspected AESD cases. 13,14 Early recognition is important, as delay has been shown to have cellular and molecular consequences. Shiihara and colleagues 15 reported that tau protein, a CSF biomarker of axonal damage, is elevated in children with AESD within the first 2 days of illness, suggesting that waiting to treat until the appearance of biphasic seizures or until BTA changes are observed may be too late to ameliorate pre-existing brain damage.…”

“… 10 Consistent with this hypothesis, early initiation of vitamins and co-enzymes including vitamin B1, B6 and l -carnitine has been proposed as a therapy and can improve outcome in suspected AESD cases. 13 , 14 …”