Factors Associated with Respiratory Insufficiency in Children with Guillain-Barré Syndrome.

OBJECTIVE: The risk factors for respiratory insufficiency in children with Guillain-Barré syndrome (GBS) are poorly known. This study aimed to investigate the factors associated with respiratory insufficiency in children with GBS. METHODS: This retrospective study included children diagnosed with GBS by pediatric neurologists and admitted at the Wuhan Children's Hospital and other hospitals from January 2013 to October 2022. The patients were divided into the respiratory insufficiency and nonrespiratory insufficiency groups according to whether they received assist breathing during treatment. RESULTS: The median (interquartile range) age of onset of 103 patients were 5 (3.1-8.5) years, 69 (67%) were male, and 64 (62.1%) had a history of precursor infection. Compared with the nonrespiratory insufficiency group, the respiratory insufficiency group showed more facial and/or bulbar weakness (p = 0.002), a higher Hughes Functional Grading Scale (HFGS) at admission (p < 0.001), and a shorter onset-to-admission interval (p = 0.017). Compared with the acute motor axonal neuropathy (AMAN) subtype, the acute inflammatory demyelinating polyneuropathy (AIDP) subtype showed longer days from onset to lumbar (p = 0.000), lower HFGS at admission (p = 0.04), longer onset-to-admission interval (p = 0.001), and more cranial nerve involvement (p = 0.04). The incidence of respiratory insufficiency between AIDP and AMAN showed no statistical difference (p > 0.05). CONCLUSION: In conclusion, facial and/or bulbar weakness, HFGS at admission, and onset-to-admission interval were associated with respiratory insufficiency and might be useful prognostic markers in children with GBS.

Correction of distorted FPI interferograms to invert atmospheric winds and temperatures.

Fabry-Perot interferometer (FPI) is a vital optical device for measuring atmospheric wind and temperature, which is widely used worldwide for its simple structure and excellent accuracy. Nevertheless, the working environment of FPI could be light polluted by many factors, such as light from street lamp and moon, which results in distortion for realistic airglow interferogram and thus affects the accuracy for the wind and temperature inversion. We simulate the FPI interferogram and invert the correct wind and temperature from the full interferogram and three parts of it. Further analysis is performed using real airglow interferograms observed at Kelan (38.7°N, 111.6°E). Distortion interferograms cause temperature deviations, while the wind is not affected. A correction method is presented to correct the distortion interferogram by making it more homogeneous. The corrected interferogram is calculated again, and the result shows that the temperature deviation of the different parts is reduced significantly. Wind and temperature errors of each part are reduced compared to the previous ones. This correction method will help improve the accuracy of the FPI temperature inversion when the interferogram is distorted.

[Very-early and early neuroelectrophysiological features of childhood Guillain-Barré syndrome]. / å„¿ç«¥å‰å °-å·´é›·ç»¼åˆå¾æžæ—©æœŸå’Œæ—©æœŸç¥žç»ç”µç”Ÿç†ç‰¹å¾çš„ç ”ç©¶.

OBJECTIVES: To study the very-early and early neuroelectrophysiological features of childhood Guillain-Barré syndrome (GBS) and their association with clinical diagnosis. METHODS: A retrospective analysis was performed on the neuroelectrophysiological data of 43 children with GBS. According to the interval from onset to neuroelectrophysiological examination, the children were divided into a very-early examination group with 18 children (an interval from onset to the examination of ≤7 days) and an early examination group with 25 children (an interval from onset to the examination of 7 to ≤14 days). The children with acute flaccid paralysis, matched for the examination time of GBS children, were enrolled as the control group. The abnormal rates of neuroelectrophysiological parameters were compared between the above groups. According to the results of the H reflex test, the GBS children were divided into an abnormal H reflex group and a normal H reflex group, and related clinical data were compared between the two groups. RESULTS: Compared with the control group, the very-early and early examination groups had a significantly higher abnormal rate of H reflex (P<0.05), while there was no significant difference in the abnormal rates of F wave, motor nerve conduction, and sensory nerve conduction (P>0.05). Compared with the normal H reflex group, the abnormal H reflex group had a significantly shorter interval from onset to the time of confirmed diagnosis (P<0.05). CONCLUSIONS: Absence of the H reflex is a valuable parameter of neuroelectrophysiological abnormalities in the early stage of GBS and can help with the diagnosis of GBS.

Risk factors associated with the need for mechanical ventilation in children with Guillain-Barré syndrome. / å„¿ç«¥å‰å °-å·´é›·ç»¼åˆå¾éœ€è¦è¾ åŠ©å‘¼å¸çš„å±é™©å› ç´ åˆ†æž.

OBJECTIVES: To study the risk factors associated with the need for mechanical ventilation in children with Guillain-Barré syndrome (GBS). METHODS: The medical data of 107 children with GBS were retrospectively reviewed. According to whether mechanical ventilation was required, the children were divided into a mechanical ventilation group with 16 children and a non-mechanical ventilation group with 91 children. The risk factors associated with the need for mechanical ventilation in children with GBS were identified by a multivariate logistic regression analysis. RESULTS: Among the 107 children, 16 (15.0%) required mechanical ventilation. Compared with the non-mechanical ventilation group, the mechanical ventilation group had a significantly higher proportion of children with facial and/or bulbar muscle weakness, a significantly shorter duration from the onset to the peak of the disease, and a significantly higher Hughes Functional Grading Scale score at the first visit (P<0.05). Facial and/or bulbar muscle weakness and short duration from the onset to the peak of the disease were risk factors associated with the need for mechanical ventilation in children with GBS (OR=5.053 and 1.239 respectively, P<0.05). CONCLUSIONS: Facial and/or bulbar muscle weakness and short duration from the onset to the peak of the disease may increase the risk of mechanical ventilation in children with GBS. Citation.

[Clinical features of children with Guillain-Barré syndrome and the significance of Brighton criteria].

OBJECTIVE: To study the clinical features of children with Guillain-Barré syndrome (GBS) and the significance of Brighton criteria in childhood GBS. METHODS: A retrospective analysis was performed on the medical data of 72 children with GBS. Brighton criteria were used for the grading of diagnostic certainty (level 1 as the highest level, and level 4 as the lowest level). A Spearman's rank correlation analysis was used to evaluate the correlation of auxiliary examinations with the level of diagnostic certainty of Brighton criteria. RESULTS: A total of 72 children with GBS were enrolled, with a mean age of onset of (98±32) months. All children (100%, 72/72) had weakness of bilateral limbs and disappearance or reduction of tendon reflex, and limb weakness reached the highest level of severity within 4 weeks. Of all the 72 children, 68 (94%) had positive results of neural electrophysiological examination and 51 (71%) had positive results of cerebrospinal fluid (CSF) examination, and the positive rate of neural electrophysiological examination was significantly higher than that of CSF examination (P < 0.01). The median interval time from disease onset to neural electrophysiological examination was significantly shorter than from disease onset to CSF examination (11 days vs 14 days, P < 0.01). Of all the 72 children, 49 (68%) met Brighton criteria level 1 and 21 (29%) met Brighton criteria level 2. Neural electrophysiological examination and CSF examination were positively correlated with the level of diagnostic certainty of Brighton criteria (rs=0.953 and 0.420 respectively, P < 0.01). CONCLUSIONS: Most of the children with GBS meet Brighton criteria level 1, and the positive results of CSF examination and neural electrophysiological examination play an important role in improving the level of diagnostic certainty of Brighton criteria. Neural electrophysiological examination has a higher positive rate than CSF examination in the early stage of the disease.

[Association of motor nerve conduction block with different subtypes of childhood Guillain-Barré syndrome].

OBJECTIVE: To study the association of motor nerve conduction block (CB) with different subtypes of childhood Guillain-Barré syndrome (GBS). METHODS: A retrospective analysis was performed on the clinical and nerve electrophysiological data of 50 children with GBS. According to the results of nerve electrophysiology, the children were divided into an acute inflammatory demyelinating polyneuropathy (AIDP) group with 29 children and an acute motor axonal neuropathy (AMAN) group with 21 children. According to the presence or absence of motor nerve CB, the children with AMAN or AIDP were further divided into subgroups: group AMAN with or without motor nerve CB (n=10 and 11 respectively) and group AIDP with or without motor nerve CB group (n=19 and 10 respectively). The subgroups were compared in terms of age of onset, sex, Hughes Functional Grading Scale (HFGS) at nadir for the most severe involvement of motor function, and short-term prognosis based on HFGS score at 1 month after disease onset. RESULTS: Motor nerve CB was reversible in children with AMAN. AMAN children with motor nerve CB had a significantly lower HFGS score than those without motor nerve CB at 1 month after onset (P<0.05). AIDP children with motor nerve CB had a significantly higher HFGS score than those with motor nerve CB at 1 month after onset (P<0.05). CONCLUSIONS: AMAN with reversible motor nerve CB suggests mild nerve fiber lesion and has better recovery than AMAN and AIDP without motor nerve CB in short term.

[Role of short-latency somatosensory evoked potential in the diagnosis of chronic inflammatory demyelinating polyneuropathy].

OBJECTIVE: To investigate the role of short-latency somatosensory evoked potential (SSEP) in the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS: A total of 48 children with a confirmed or suspected CIDP and 40 healthy children were enrolled. Nerve electrophysiological examination and/or SSEP examination was performed (the children in the healthy control group only underwent SSEP examination). Four-lead electromyography was used for nerve electrophysiological examination, including at least 4 motor nerves and 2 sensory nerves. N6 (elbow potential), N13 (cervical cord potential), and N20 (cortex potential) of the median nerve and N8 (popliteal fossa potential), N22 (lumbar cord potential), and P39 (cortex potential) of the tibial nerve were observed by SSEP examination. RESULTS: Among the 48 children with CIDP, 35 had demyelination in both motor and sensory nerves, 8 had demyelination in sensory nerves, and 5 had axonal degeneration. SSEP examination showed that 7 had conduction abnormality in the trunk of the brachial plexus and/or the posterior root and 33 had damage in the lumbosacral plexus and/or the posterior root. The 40 children with abnormal findings of SSEP examination included 8 children with affected sensory nerves and 5 children with secondary axonal degeneration who did not meet the electrophysiological diagnostic criteria for CIDP. Compared with the healthy control group, the CIDP group had significantly prolonged latency periods of N13 and N22 (P<0.05). CONCLUSIONS: SSEP can be used for the auxiliary diagnosis of CIDP, especially in CIDP children with affected sensory nerves or secondary axonal degeneration.

[Role of nerve stimulation at Erb point in early diagnosis of Guillain-Barré syndrome in children].

OBJECTIVE: To study the role of proximal nerve stimulation at Erb point in the early diagnosis of Guillain-Barré syndrome (GBS) in children. METHODS: Thirty-two children who were diagnosed with GBS between October 2013 and December 2014 received neurophysiological examination. Thirty healthy children were used as controls. Compound muscle action potentials and distal motor latency of the median and ulnar nerves were determined and analyzed after nerve stimulation at the wrist, elbow, and Erb point in the two groups. Moreover, F-wave latency of the median nerve and H-reflex latency of the tibial nerve were measured and analyzed in the two groups. RESULTS: The F-wave and H-reflex latencies were significantly longer in the patient group than in the control group (P<0.05). In thirty-two patients, the numbers of patients with abnormal amplitude, abnormal latency, and conduction block at Erb's point were 24 (75%), 22 (69%), and 20 (62%), respectively. The patient group had significantly lower amplitudes but significantly longer latencies of the ulnar and median nerves at Erb point than the control group (P<0.05). There were no significant differences in the amplitudes and latencies at the wrist and elbow between the two groups (P>0.05). CONCLUSIONS: The nerve stimulation at Erb point holds promise as a routine examination for the early diagnosis of GBS.

Effect of endovascular treatment within 6 hours for acute basilar artery occlusion.

OBJECTIVE: The aim of this study was to investigate the efficacy and safety of endovascular treatment (EVT) plus standard medical treatment (SMT) in patients with acute basilar artery occlusion (BAO) within 6 hours of the estimated occlusion time, based on a Chinese population. METHODS: The authors selected patients from the Endovascular Treatment of Acute Basilar Artery Occlusion Study (BASILAR) registry, which was a nationwide prospective registry, within 6 hours after the estimated time of onset of a stroke in acute BAO. Patients were divided into the SMT-alone group or the EVT+SMT group according to treatment modalities. The primary outcome was a favorable functional outcome, defined as a modified Rankin Scale score between 0 and 3 at 90 days. Safety outcomes included death at 90 days and symptomatic intracerebral hemorrhage. RESULTS: The authors assessed 590 patients for eligibility. Of these patients, 127 received SMT alone and 463 were treated with EVT plus SMT. EVT was associated with a higher rate of a favorable functional outcome (adjusted OR 3.804, 95% CI 1.890-7.658; p < 0.001) and a lower proportion of deaths at 90 days (adjusted OR 0.364, 95% CI 0.223-0.594; p < 0.001). Lower age (adjusted OR 0.978, 95% CI 0.960-0.997; p = 0.022); lower baseline National Institutes of Health Stroke Scale score (adjusted OR 0.926, 95% CI 0.902-0.950; p < 0.001); higher baseline posterior circulation Alberta Stroke Program Early CT Score (adjusted OR 1.681, 95% CI 1.424-1.984; p < 0.001); absence of diabetes mellitus (adjusted OR 0.482, 95% CI 0.267-0.871; p = 0.016); and modified Thrombolysis in Cerebral Infarction scores 2b-3 (adjusted OR 5.117, 95% CI 2.304-11.367; p < 0.001) were independent factors for a favorable outcome in the EVT+SMT group. CONCLUSIONS: Based on the study design, patients with acute BAO who received EVT within 6 hours were associated with improved favorable outcome and decreased deaths compared with patients who received SMT. Predictors of desirable outcome in patients undergoing EVT included lower age, lower baseline National Institutes of Health Stroke Scale score, higher baseline posterior circulation Alberta Stroke Program Early CT Score, absence of diabetes mellitus, and modified Thrombolysis in Cerebral Infarction scores 2b-3.

Hyperdense Artery Sign and Clinical Outcomes After Endovascular Treatment in Acute Basilar Artery Occlusion.

Background: This study aimed to investigate the association between the hyperdense basilar artery sign (HBAS) on non-enhanced computed tomography (CT) and clinical outcomes in patients with acute basilar artery occlusion (BAO) who underwent endovascular treatment (EVT). Methods: Eligible patients who underwent EVT due to acute BAO between January 2014 and May 2019 were divided into two groups based on HBAS. HBAS was assessed by two neuroradiologists using five grades on nonenhanced CT. The primary outcome was a favorable functional outcome (defined as a modified Rankin Scale [mRS] of 0-3) at 90 days. Secondary outcomes included successful recanalization and mortality within 90 days. Results: Among 829 patients with BAO as assessed with CT angiography, magnetic resonance angiography, or digital subtraction angiography, 643 patients were treated with EVT. Of these, 51.32% (330/643) had HBAS. Patients with HBAS were older and had more severe neurological deficits and a higher frequency of atrial fibrillation than those without HBAS. There was no significant difference in favorable outcome (adjusted odds ratio [aOR]: 1.354, 95% confidence interval [CI]: 0.906-2.024; p = 0.14), successful recanalization (aOR: 0.926, 95% CI: 0.616--1.393; p = 0.71), and mortality (aOR: 1.193, 95% CI: 0.839-1.695; p = 0.33) between patients with or without HBAS. Subgroup analysis showed that the HBAS predicted a favorable outcome in patients aged <60 years (aOR: 2.574, 95% CI: 1.234-5.368; p = 0.01) and patients with vertebral artery-V4 segment occlusion (aOR: 3.738, 95% CI: 1.212-11.530; p = 0.02). In patients with HBAS, the baseline National Institutes of Health Stroke Scale (NIHSS) score, posterior circulation-Acute Stroke Prognosis Early Computed Tomography Score (pc-ASPECTS), and stent retriever were associated with successful recanalization. Conclusions: Our study did not find a significant association between HBAS and favorable outcomes and successful recanalization in patients with BAO who underwent EVT. Moreover, large prospective studies are warranted to further investigate this relationship.

Effect of atrial fibrillation on outcomes after mechanical thrombectomy and long-term ischemic recurrence in patients with acute basilar artery occlusion.

Introduction: According to the literature on anterior circulation, comorbid atrial fibrillation (AF) is not associated with a worse functional outcome, lower reperfusion rates, or higher rates of intracranial hemorrhage after mechanical thrombectomy (MT) compared to intravenous thrombolysis (IVT) or treatment with supportive care. However, data are limited for the effect of comorbid AF on procedural and clinical outcomes of acute basilar artery occlusion (ABAO) after MT. This study aimed to investigate the effect of atrial fibrillation on outcomes after MT and long-term ischemic recurrence in patients with ABAO. Methods: We performed a registered study of the Endovascular Treatment for Acute Basilar Artery Occlusion Study (BASILAR, which is registered in the Chinese Clinical Trial Registry, http://www.chictr.org.cn; ChiCTR1800014759) from January 2014 to May 2019, which included 647 patients who underwent MT for ABAO, 136 of whom had comorbid AF. Prospectively defined baseline characteristics, procedural outcomes, and clinical outcomes were reported and compared. Results: On multivariate analysis, AF predicted a shorter puncture-to-recanalization time, higher first-pass effect rate, and lower incidence of angioplasty and/or stenting (p < 0.01). AF had no effect on intracranial hemorrhage incidence [adjusted odds ratio (aOR), 1.093; 95% confidence interval (CI), 0.451-2.652], 90-day functional outcomes (adjusted common odds ratio, 0.915; 95% CI, 0.588-1.424), or mortality (aOR, 0.851; 95% CI, 0.491-1.475) after MT. The main findings were robust in the subgroup and 1-year follow-up analyses. Comorbid AF was the remaining predictor of ischemic recurrence (aOR, 4.076; 95% CI, 1.137-14.612). Conclusions: The study revealed no significant difference in the safety and efficacy of MT for ABAO regardless of whether patients had comorbid AF. However, a higher proportion of patients with AF experienced ischemic recurrence within 1 year after MT.

Outcomes of Endovascular Therapy in Young Patients with Acute Basilar Artery Occlusion: A Substudy of BASILAR Registry Study.

INTRODUCTION: This study aimed to investigate clinical outcomes in young patients with basilar artery occlusion (BAO) receiving endovascular therapy (EVT). METHODS: Consecutive patients with BAO within 24 h who underwent EVT from the BASILAR Registry study were enrolled. We compared clinical outcomes of young patients (aged 18-55 years) with older patients (aged > 55 years) with stroke due to BAO at 90 days and 1 year after EVT. The primary and secondary outcomes were improvement in modified Rankin scale scores (mRS) at 90 days and either favorable (mRS 0-3) or mortality at 90 days, respectively. RESULTS: A total of 646 patients were included, of which 152 (23.53%) were aged 18-55 years. Dyslipidemia (42.11% vs. 30.36%, p = 0.007) and good collateral circulation (60.52% vs. 46.35%, p = 0.002) were more frequent in young patients than older. Stroke etiologies in young patients included large artery atherosclerosis (67.11%), cardioembolism (15.13%), and vessel dissection (5.26%). Young patients were associated with better prognosis (mRS: adjusted odds ratio (aOR) 1.73; 95% confidence interval [CI] 1.21-2.48; mRS 0-3: aOR 1.60; 95% CI 1.01-2.54; mortality: aOR 0.60; 95% CI 0.38-0.93) at 90 days. Baseline National Institutes of Health Stroke Scale (NIHSS) score, posterior circulation Alberta Stroke Program Early Computed Tomography Score (pc-ASPECTS), and sex were independent predictors of clinical outcomes of young patients at 90 days after EVT. CONCLUSION: Young patients with BAO had better clinical outcomes after EVT than old patients. Predictors of clinical outcomes in young patients undergoing EVT included baseline NIHSS score, pc-ASPECTS, and sex. TRIAL REGISTRATION: Clinical Trial Registration-URL: ChiCTR180001475 ( www.chictr.org.cn ).

6mA-DNA-binding factor Jumu controls maternal-to-zygotic transition upstream of Zelda.

A long-standing question in the field of embryogenesis is how the zygotic genome is precisely activated by maternal factors, allowing normal early embryonic development. We have previously shown that N6-methyladenine (6mA) DNA modification is highly dynamic in early Drosophila embryos and forms an epigenetic mark. However, little is known about how 6mA-formed epigenetic information is decoded. Here we report that the Fox-family protein Jumu binds 6mA-marked DNA and acts as a maternal factor to regulate the maternal-to-zygotic transition. We find that zelda encoding the pioneer factor Zelda is marked by 6mA. Our genetic assays suggest that Jumu controls the proper zygotic genome activation (ZGA) in early embryos, at least in part, by regulating zelda expression. Thus, our findings not only support that the 6mA-formed epigenetic marks can be read by specific transcription factors, but also uncover a mechanism by which the Jumu regulates ZGA partially through Zelda in early embryos.