ABSTRACT
BACKGROUND: To assess the performance of pediatric extracorporeal membrane oxygenation (ECMO) centers, outcomes were compared between metropolitan and other areas. METHODS: A retrospective cohort study was conducted at three regional centers on Kyushu Island and the largest center in the Tokyo metropolitan area of Japan. The clinical outcomes of patients of ≤15 years of age who received ECMO during 2010-2019 were investigated, targeting the survival and performance at discharge from intensive care units (ICUs), using medical charts. RESULTS: One hundred and fifty-five patients were analyzed (regional, n = 70; metropolitan, n = 85). Survival rates at ICU discharge were similar between the two areas (64%). In regional centers, deterioration of Pediatric Cerebral Performance Category (PCPC) scores were more frequent (65.7% vs. 49.4%; p = 0.042), but survival rates and ΔPCPC scores (PCPC at ICU discharge-PCPC before admission) improved in the second half of the study period (p = 0.005 and p = 0.046, respectively). Veno-arterial ECMO (odds ratio [OR], 3.00; p < 0.03), extracorporeal cardiopulmonary resuscitation (OR, 8.98; p < 0.01), and absence of myocarditis (OR, 5.47; p < 0.01) were independent risk factors for deterioration of the PCPC score. A sub-analysis of patients with acute myocarditis (n = 51), the main indicator for ECMO, revealed a significantly higher proportion of cases with deteriorated PCPC scores in regional centers (51.9% vs. 25.0%; p = 0.049). CONCLUSIONS: The survival rates of pediatric patients supported by ECMO in regional centers were similar to those in a metropolitan center. However, neurological outcomes must be improved, particularly in patients with acute myocarditis.
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OBJECTIVES: To develop an equation for defining a low skeletal muscle mass (SMM) in children and to investigate risk factors and outcomes associated with low SMM in critically ill pediatric patients. DESIGN: Single-center retrospective pediatric cohorts, 2011-2018. SETTING: Tertiary Emergency and Critical Care Center of Kyushu University Hospital in Japan. PATIENTS: We studied two cohorts of pediatric patients 1-15 years old who underwent abdominal CT at the level of the third lumbar vertebra (L3). First a cohort of trauma patients presented to the emergency department in whom we developed an SMM regression equation. Second, a cohort of patients who had undergone abdominal CT within 3 days of PICU admission. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The equation for estimating normal SMM used sex, age, and weight. Low SMM was defined as less than 80% of normal. In the 112 patients in the PICU cohort, median (range) age was 68 (13-191) months, and 83 (74.1%) had underlying disease. There was low SMM in 54 patients (48.2%). Regarding associations, using odds ratio (OR) and 95% CI, we found that low dietary intake (OR 4.33 [95% CI, 1.37-13.70]; p = 0.013) and the presence of underlying disease (OR 7.44 [95% CI, 2.10-26.30]; p = 0.002) were independently associated with greater odds of low SMM. Low SMM, compared with normal SMM, was also associated with longer hospital stays (42.5 d vs. 20.5 d; p = 0.007; ß, 1.59; 95% CI, 1.09-2.33; p = 0.016). CONCLUSIONS: In this retrospective PICU cohort from a single center in Japan, we found that low SMM at PICU admission was present in almost half the cases. Low SMM, as defined by being less than 80% of the normal, was associated with greater odds of low dietary intake and underlying chronic disease. Furthermore, low SMM was associated with longer hospital stays.
Subject(s)
Critical Illness , Intensive Care Units, Pediatric , Muscle, Skeletal , Humans , Male , Intensive Care Units, Pediatric/statistics & numerical data , Female , Child , Retrospective Studies , Child, Preschool , Adolescent , Infant , Muscle, Skeletal/diagnostic imaging , Risk Factors , Tomography, X-Ray Computed , Length of Stay/statistics & numerical data , Japan/epidemiologyABSTRACT
Severe obesity in young children prompts for a differential diagnosis that includes syndromic conditions. Rapid-Onset Obesity with Hypothalamic Dysfunction, Hypoventilation, and Autonomic Dysregulation (ROHHAD) syndrome is a potentially fatal disorder characterized by rapid-onset obesity associated with hypoventilation, neural crest tumors, and endocrine and behavioral abnormalities. The etiology of ROHHAD syndrome remains to be established, but recent research has been focusing on autoimmunity. We report on a 2-year-old girl with rapid-onset obesity during the first year of life who progressed to hypoventilation and encephalitis in less than four months since the start of accelerated weight gain. The patient had a high titer of anti-ZSCAN1 antibodies (348; reference range < 40), and the increased values did not decline after acute phase treatment. Other encephalitis-related antibodies, such as the anti-NDMA antibody, were not detected. The rapid progression from obesity onset to central hypoventilation with encephalitis warns about the severe consequences of early-onset ROHHAD syndrome. These data indicate that serial measurements of anti-ZSCAN1 antibodies might be useful for the diagnosis and estimation of disease severity. Further research is needed to determine whether it can predict the clinical course of ROHHAD syndrome and whether there is any difference in antibody production between patients with and without tumors.
Subject(s)
Adrenal Gland Neoplasms , Autonomic Nervous System Diseases , Encephalitis , Hypothalamic Diseases , Pediatric Obesity , Female , Humans , Child, Preschool , Hypoventilation/complications , Hypoventilation/diagnosis , Pediatric Obesity/complications , Adrenal Gland Neoplasms/complications , Syndrome , Encephalitis/complicationsABSTRACT
BACKGROUND: The systemic manifestations of coronavirus disease 2019 (COVID-19) include hyperinflammatory reactions in various organs. Recent studies showed evidence for the frequent involvement of central nervous system in affected patients; however, little is known about clinical features of cerebrovascular diseases in childhood-onset COVID-19. CASE PRESENTATION: A 10-year-old boy recovered from SARS-CoV-2 infection without complication. On 14 days after infection, he presented with loss of consciousness. A head computed tomography detected a ruptured cerebral aneurysm at the left posterior cerebral artery accompanying subarachnoid hemorrhage (SAH). Immediate surgical intervention did not rescue the patient, resulting in the demise 7 days after admission. Serological and genetic tests excluded the diagnosis of vasculitis and connective tissue disorders. Retrospective analysis showed markedly higher levels of interleukin (IL)-1ß, IL-6 and IL-8 in the cerebrospinal fluid than the serum sample concurrently obtained. A review of literature indicated that adult patients with COVID-19 have a risk for the later development of SAH during the convalescent phase of COVID-19. CONCLUSIONS: SAH is a severe complication of COVID-19 in children and adults who have asymptomatic cerebrovascular aneurysms. The markedly high levels of cytokines detected in the cerebrospinal fluid suggested that intracranial hyperinflammatory condition might be one of the possible mechanisms involved in the rupture of a preexisting cerebrovascular aneurysms.
Subject(s)
Aneurysm, Ruptured , COVID-19 , Intracranial Aneurysm , Stroke , Subarachnoid Hemorrhage , Male , Adult , Child , Humans , Intracranial Aneurysm/surgery , Retrospective Studies , COVID-19/complications , SARS-CoV-2 , Subarachnoid Hemorrhage/complications , Subarachnoid Hemorrhage/diagnostic imaging , Stroke/complications , Inflammation/complications , Aneurysm, Ruptured/complications , Aneurysm, Ruptured/diagnostic imagingABSTRACT
BACKGROUND: We have previously reported a simple correction method for estimating pleural pressure (Ppl) using central venous pressure (CVP). However, it remains unclear whether this method is applicable to patients with varying levels of intravascular volumes and/or chest wall compliance. This study aimed to investigate the accuracy of our method under different conditions of intravascular volume and chest wall compliance. RESULTS: Ten anesthetized and paralyzed pigs (43.2 ± 1.8 kg) were mechanically ventilated and subjected to lung injury by saline lung lavage. Each pig was subjected to three different intravascular volumes and two different intraabdominal pressures. For each condition, the changes in the esophageal pressure (ΔPes) and the estimated ΔPpl using ΔCVP (cΔCVP-derived ΔPpl) were compared to the directly measured change in pleural pressure (Δd-Ppl), which was the gold standard estimate in this study. The cΔCVP-derived ΔPpl was calculated as κ × ΔCVP, where "κ" was the ratio of the change in airway pressure to the change in CVP during the occlusion test. The means and standard deviations of the Δd-Ppl, ΔPes, and cΔCVP-derived ΔPpl for all pigs under all conditions were 7.6 ± 4.5, 7.2 ± 3.6, and 8.0 ± 4.8 cmH2O, respectively. The repeated measures correlations showed that both the ΔPes and cΔCVP-derived ΔPpl showed a strong correlation with the Δd-Ppl (ΔPes: r = 0.95, p < 0.0001; cΔCVP-derived ΔPpl: r = 0.97, p < 0.0001, respectively). In the Bland-Altman analysis to test the performance of the cΔCVP-derived ΔPpl to predict the Δd-Ppl, the ΔPes and cΔCVP-derived ΔPpl showed almost the same bias and precision (ΔPes: 0.5 and 1.7 cmH2O; cΔCVP-derived ΔPpl: - 0.3 and 1.9 cmH2O, respectively). No significant difference was found in the bias and precision depending on the intravascular volume and intraabdominal pressure in both comparisons between the ΔPes and Δd-Ppl, and cΔCVP-derived ΔPpl and Δd-Ppl. CONCLUSIONS: The CVP method can estimate the ΔPpl with reasonable accuracy, similar to Pes measurement. The accuracy was not affected by the intravascular volume or chest wall compliance.
ABSTRACT
Patients with infectious diseases including sepsis can develop ST segment changes on an electrocardiogram (ECG) in the absence of coronary artery disease. However, ST elevation with "reciprocal ST segment depression (RSTD)", which is recognized as a specific finding for ST-elevated myocardial infarction, is rare in such patients. Although a small number of cases have reported ST-segment elevation in gastritis, cholecystitis, and sepsis, regardless of coronary artery disease, none presented with reciprocal changes. Here, we describe a rare case of a patient with emphysematous pyelonephritis complicating septic shock who developed ST elevation accompanied by reciprocal changes with no coronary occlusion. Emergency physicians should consider the possibility of acute coronary syndrome mimicking, and choose non-invasive diagnostic procedures when investigating the causes of ECG abnormalities associated with critically ill patients.
Subject(s)
Acute Coronary Syndrome , Coronary Artery Disease , Pyelonephritis , ST Elevation Myocardial Infarction , Sepsis , Humans , Acute Coronary Syndrome/complications , Acute Coronary Syndrome/diagnosis , ST Elevation Myocardial Infarction/diagnosis , Electrocardiography/methods , Arrhythmias, Cardiac , Pyelonephritis/complications , Pyelonephritis/diagnosis , Coronary AngiographyABSTRACT
The NOD-like receptor family, pyrin domain-containing protein 3 (NLRP3) inflammasome has been linked to the pathogenesis of a wide variety of human diseases. Although many drugs and inhibitors have been developed to treat NLRP3-associated diseases, only limited clinical data support their efficacy and safety. Chlorella, a unicellular green alga that is widely and safely used as a food supplement, contains various antioxidants. In this study, we obtained a fat-soluble extract from Chlorella (CE) and demonstrated that it reduced NLRP3 inflammasome activation by inhibiting mitochondrial reactive oxygen species and caspase-1 activation. In addition, CE supplementation attenuated lipopolysaccharide-induced interleukin 1ß transcription through activation of hypoxia-inducible factor 1α in vitro and in vivo. As Chlorella is a safe and useful food supplement, it may be a practical pharmacological approach for treating NLRP3-driven diseases.
ABSTRACT
BACKGROUND: Primary brain tumor is a leading cause of death in cancer-bearing children. Acutely progressive patterns of electroencephalography (EEG) remain to be investigated for children with rapidly growing brain tumors. CASE REPORT: A 14-month-old boy was transferred to our department for prolonged seizures and unrecovered consciousness on his fifth day of illness. The EEG recording on admission showed highly disorganized background activity with high-voltage rhythmic delta waves. Serial EEG monitoring revealed a rapid transition of the background activity to the suppression-burst pattern, and then to generalized suppression of cortical activity within a few hours after admission. Magnetic resonance imaging detected a midline tumor at the pineal gland extending to the midbrain and pons. The tumor was pathologically confirmed as atypical teratoid/rhabdoid tumor (AT/RT) with absent expression of SMARCB1. He died of tumor progression on the 20th day after admission. CONCLUSION: AT/RT is an additional category of brain tumors that cause the clinically and electro-physiologically critical condition in a few days after the onset.
Subject(s)
Brain Neoplasms/diagnosis , Brain/physiopathology , Rhabdoid Tumor/diagnosis , Brain/diagnostic imaging , Brain Neoplasms/diagnostic imaging , Brain Neoplasms/physiopathology , Electroencephalography , Humans , Infant , Magnetic Resonance Imaging , Male , Rhabdoid Tumor/diagnostic imaging , Rhabdoid Tumor/physiopathologyABSTRACT
Psoriasis is a common immune-mediated, chronic, inflammatory skin disease that affects approximately 2-3% of the population worldwide. Although there is increasing evidence regarding the essential roles of the interleukin (IL)-23/IL-17 axis and dendritic cell (DC)-T cell crosstalk in the development of skin inflammation, the contributions of mitochondrial function to psoriasis are unclear. In a mouse model of imiquimod (IMQ)-induced psoriasiform skin inflammation, we found that hematopoietic cell-specific genetic deletion of p32/C1qbp, a regulator of mitochondrial protein synthesis and metabolism, protects mice from IMQ-induced psoriatic inflammation. Additionally, we demonstrate that p32/C1qbp is an important regulator of IMQ-induced DC activation, both in vivo and in vitro. We also found that p32/C1qbp-deficient DCs exhibited impaired production of IL-1ß, IL-23, and mitochondrial reactive oxygen species (mtROS) after IMQ stimulation. Because the inhibition of mtROS suppressed IMQ-induced DC activation and psoriatic inflammation, we presume that p32/C1qbp and mtROS can serve as therapeutic targets in psoriasis.
Subject(s)
Disease Susceptibility , Mitochondria/metabolism , Psoriasis/etiology , Psoriasis/metabolism , Reactive Oxygen Species/metabolism , Animals , Biomarkers , Cytokines/metabolism , Disease Models, Animal , Immunophenotyping , Inflammation Mediators/metabolism , Membrane Potential, Mitochondrial , Metabolome , Metabolomics/methods , Mice , Mice, Knockout , Mitochondria/genetics , Psoriasis/diagnosisABSTRACT
Predicting outcomes of children after cardiac arrest (CA) remains challenging. To identify useful prognostic markers for pediatric CA, we retrospectively analyzed the early findings of head computed tomography (CT) of patients. Subjects were non-traumatic, out-of-hospital CA patients < 16 years of age who underwent the first head CT within 24 h in our institute from 2006 to 2018 (n = 70, median age: 4 months, range 0-163). Of the 24 patients with return of spontaneous circulation, 14 survived up to 30 days after CA. The degree of brain damage was quantitatively measured with modified methods of the Alberta Stroke Program Early CT Score (mASPECTS) and simplified gray-matter-attenuation-to-white-matter-attenuation ratio (sGWR). The 14 survivors showed higher mASPECTS values than the 56 non-survivors (p = 0.035). All 3 patients with mASPECTS scores ≥ 20 survived, while an sGWR ≥ 1.14 indicated a higher chance of survival than an sGWR < 1.14 (54.5% vs. 13.6%). Follow-up magnetic resonance imaging for survivors validated the correlation of the mASPECTS < 15 with severe brain damage. Thus, low mASPECTS scores were associated with unfavorable neurological outcomes on the Pediatric Cerebral Performance Category scale. A quantitative analysis of early head CT findings might provide clues for predicting survival of pediatric CA.
Subject(s)
Brain Injuries , Brain/diagnostic imaging , Neuroimaging , Out-of-Hospital Cardiac Arrest , Tomography, X-Ray Computed , Adolescent , Brain Injuries/diagnostic imaging , Brain Injuries/mortality , Brain Injuries/therapy , Child , Child, Preschool , Disease-Free Survival , Female , Head/diagnostic imaging , Humans , Infant , Infant, Newborn , Male , Out-of-Hospital Cardiac Arrest/diagnostic imaging , Out-of-Hospital Cardiac Arrest/mortality , Out-of-Hospital Cardiac Arrest/therapy , Pilot Projects , Retrospective Studies , Survival RateABSTRACT
Dendritic cell (DC) maturation induced by Toll-like receptor (TLR) agonists requires the activation of downstream metabolic changes. Here, we provide a detailed protocol to measure glycolysis, mitochondrial respiration, and fatty acid oxidation in mouse bone-marrow-derived DCs with the Seahorse XF24 extracellular flux (XF) analyzer. XF analysis with the Seahorse bioanalyzer has become a standard method to measure bioenergetic functions in cells, and this protocol can be adapted to other immune cells. For complete information on using this protocol, please refer to Gotoh et al. (2018).
Subject(s)
Bone Marrow Cells/metabolism , Dendritic Cells/metabolism , Metabolic Flux Analysis/methods , Animals , Cell Culture Techniques , Cells, Cultured , Fatty Acids/metabolism , Glycolysis/physiology , Male , Mice , Mitochondria/metabolismABSTRACT
p32/C1qbp regulates mitochondrial protein synthesis and is essential for oxidative phosphorylation in mitochondria. Although dysfunction of p32/C1qbp impairs fetal development and immune responses, its role in hematopoietic differentiation remains unclear. Here, we found that mitochondrial dysfunction affected terminal differentiation of newly identified erythroid/B-lymphoid progenitors among CD45- Ter119- CD31- triple-negative cells (TNCs) in bone marrow. Hematopoietic cell-specific genetic deletion of p32/C1qbp (p32cKO) in mice caused anemia and B-lymphopenia without reduction of hematopoietic stem/progenitor cells. In addition, p32cKO mice were susceptible to hematopoietic stress with delayed recovery from anemia. p32/C1qbp-deficient CD51- TNCs exhibited impaired mitochondrial oxidation that consequently led to inactivation of mTORC1 signaling, which is essential for erythropoiesis. These findings uncover the importance of mitochondria, especially at the stage of TNCs during erythropoiesis, suggesting that dysregulation of mitochondrial protein synthesis is a cause of anemia and B-lymphopenia with an unknown pathology.
ABSTRACT
OBJECTIVES: To characterize the real size and morphology of tracheas in childhood for the optimal selection of endotracheal tube. DESIGN: A retrospective cohort study of pediatric patients who received CT scan of the cervical spine from July 2011 to March 2018. Cross-sectional CT images vertical to trachea were reconstructed and the accurate tracheal diameters were measured. The validity of the traditional age-based formula for predicting the endotracheal tube size was assessed for the best fit to trachea. SETTING: Tertiary Emergency and Critical Care Center of Kyushu University Hospital. PATIENTS: Children, who are 1 month to 15 years old, received CT scan of the cervical spine. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We enrolled 86 children with median age of 53 months. The cross-sectional shape of pediatric trachea was circular at the cricoid level and elliptical at the infraglottic level. The narrowest part of pediatric trachea was the transverse diameter at the infraglottic level at any age. Significant positive correlation between age and the narrowest diameter was observed. When compared the transverse diameter at the infraglottic level with the outer diameter of endotracheal tubes, uncuffed endotracheal tubes selection based on the traditional age-based formula ran a significant risk of oversized endotracheal intubation until 10 years old compared with cuffed endotracheal tubes selection (60.0% vs 23.8%; p < 0.05). CONCLUSIONS: These findings indicate the safety and efficacy of cuffed endotracheal tubes in infants and children and the reconsideration for the airway management in pediatric anesthesia and intensive care.
Subject(s)
Intubation, Intratracheal/standards , Trachea/anatomy & histology , Adolescent , Age Factors , Cervical Cord/diagnostic imaging , Child , Child, Preschool , Female , Humans , Infant , Male , Retrospective Studies , Tomography, X-Ray Computed/methods , Trachea/diagnostic imagingABSTRACT
OBJECTIVES: To determine the accuracy of tidal volume reported by neonatal ventilators, with and without leak compensation, in invasive and noninvasive ventilation modes in the presence of airway leak; and, to determine what factors have a significant effect on the accuracy of tidal volume reported by ventilators with leak compensation in the presence of airway leak. We hypothesized that ventilators with a leak compensation function that includes estimation of tidal volume could accurately report tidal volume in the presence of airway leak, but that the accuracy of reported tidal volume may be affected by variables such as the identity of the ventilator, lung mechanics, leak size, positive end-expiratory pressure level, and body size. DESIGN: In vitro assessment of ventilator volume delivery was conducted for seven acute care ventilators using a passive lung simulator. SETTING: Laboratory-based measurements. INTERVENTIONS: The error of reported tidal volume was calculated under three ventilation modes (noninvasive-pressure-control, invasive-pressure-control, and invasive-dual-control ventilation), three models of lung mechanics (normal and restrictive and obstructive lung disease), a range of airway leak values, two positive end-expiratory pressure values, and two body weights for each ventilator. Ventilators with and without leak compensation were studied. MEASUREMENTS AND MAIN RESULTS: In the absence of airway leak, all ventilators reported tidal volume accurately. In the presence of airway leak, the error of reported tidal volume increased for all ventilators without a leak compensation algorithm while ventilators with leak compensation that included estimation of tidal volume accurately reported tidal volume. In the presence of airway leak, clinically significant effects on the error of reported tidal volume by ventilators with leak compensation were associated with the choice of ventilator in all modes and with lung mechanics in invasive ventilation modes. CONCLUSIONS: Reported tidal volume is affected by the presence of airway leak, but in many ventilators a leak compensation algorithm that includes estimation of tidal volume can correct for the discrepancy between actual and reported tidal volume. However, even in ventilators with leak compensation, choice of ventilator and lung mechanics in invasive ventilation modes have a significant effect on error of reported tidal volume.
Subject(s)
Equipment Failure/statistics & numerical data , Models, Biological , Tidal Volume , Ventilators, Mechanical/statistics & numerical data , Humans , Infant, Newborn , Ventilators, Mechanical/standardsABSTRACT
Dendritic cell (DC) maturation induced by Toll-like receptor agonists requires activation of downstream signal transduction and metabolic changes. The endogenous metabolite citrate has recently emerged as a modulator of DC activation. However, the metabolic requirements that support citrate production remain poorly defined. Here, we demonstrate that p32/C1qbp, which functions as a multifunctional chaperone protein in mitochondria, supports mitochondrial metabolism and DC maturation. Metabolic analysis revealed that the citrate increase induced by lipopolysaccharide (LPS) is impaired in p32-deficient DCs. We also found that p32 interacts with dihydrolipoamide S-acetyltransferase (E2 component of pyruvate dehydrogenase [PDH] complex) and positively regulates PDH activity in DCs. Therefore, we suggest that DC maturation is regulated by citrate production via p32-dependent PDH activity. p32-null mice administered a PDH inhibitor show decreased DC maturation and ovalbumin-specific IgG production in vivo, suggesting that p32 may serve as a therapeutic target for DC-related autoimmune diseases.
Subject(s)
Cell Differentiation , Dendritic Cells/cytology , Dendritic Cells/metabolism , Mitochondria/metabolism , Mitochondrial Proteins/metabolism , Animals , Cell Differentiation/drug effects , Dendritic Cells/drug effects , Dendritic Cells/ultrastructure , Electron Transport/drug effects , Endoplasmic Reticulum/drug effects , Endoplasmic Reticulum/metabolism , Fatty Acids/biosynthesis , Gene Deletion , Gene Expression Regulation/drug effects , Glycolysis/drug effects , Lipopolysaccharides/pharmacology , Mice, Inbred C57BL , Mitochondria/drug effects , Mitochondria/genetics , Mitochondrial Proteins/genetics , Oxidative Phosphorylation/drug effects , Protein Binding/drug effects , Pyruvate Dehydrogenase Complex/metabolism , Toll-Like Receptors/metabolismABSTRACT
Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is a childhood-onset encephalopathy, but the precise pathophysiology remains unclear. We encountered a child with Moyamoya syndrome and AESD. He exhibited left-predominant stenosis of the middle cerebral artery (MCA), and later developed broad lesions in the left hemisphere, raising the possibility that insufficient blood supply relates to formation of the lesions. To test the hypothesis, we investigated the relationship between MCA volume and lesion extent in seven AESD children without preexisting diseases. The MCA volume and lesion extent were quantified with time of flight images for construction of magnetic resonance angiography and apparent diffusion coefficient maps, respectively. Lateralization indices ([rightâ¯-â¯left]/[rightâ¯+â¯left]) of the MCA volume and lesion extent were calculated. We found that the lateralization indices were negatively correlated (râ¯=â¯-0.786, pâ¯=â¯.036), that is, when the MCA volume was smaller in one side than the other side, the lesions were likely to develop more extensively in the ipsilateral side than the contralateral side. This indicates the association of insufficient blood supply with the lesions. The present study provides the first observation to suggest the involvement of vascular mechanism in AESD and has potential implications for novel therapeutic approach.
Subject(s)
Brain Diseases/diagnostic imaging , Brain/diagnostic imaging , Middle Cerebral Artery/diagnostic imaging , Seizures/diagnostic imaging , Adenosine Triphosphatases/genetics , Brain Diseases/genetics , Brain Diseases/physiopathology , Brain Diseases/therapy , Cerebral Angiography , Cerebrovascular Circulation , Child , Child, Preschool , Female , Humans , Infant , Magnetic Resonance Imaging , Male , Organ Size , Seizures/genetics , Seizures/physiopathology , Seizures/therapy , Ubiquitin-Protein Ligases/geneticsABSTRACT
Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is a newly defined clinicoradiologic syndrome characterized by biphasic seizures and altered consciousness followed by restricted diffusion in the white matter on magnetic resonance imaging in acute phase. Intractable epilepsy commonly occurs as the late complication. This study aimed to search predisposing factors to the development of epilepsy after AESD. Consecutively treated 22 patients with AESD in our institution from 2006 to 2016 were grouped into those with post-encephalopathic epilepsy (PEE, nâ¯=â¯10) or without PEE (nâ¯=â¯12). There was no difference between two groups in age at the onset of AESD, duration of the initial seizures, or the follow-up periods after discharge. PEE group patients more frequently showed coma or involuntary movements during the course of AESD than non-PEE group patients (36% vs. 8%, pâ¯=â¯0.008). The quantitative analysis of apparent diffusion coefficient (ADC) map revealed that PEE group showed broader areas with reduced diffusion in the posterior lobes at the onsets of AESD than non-PEE group (0.113 vs. 0.013, pâ¯=â¯0.035). On the other hand, the atrophy on day 30-ADC map did not correlate with the development or control of epilepsy. These results suggest that the clinical severity and ADC profiles in acute phase, rather than the brain atrophy in convalescent phase, may predict the development of post-AESD epilepsy.
Subject(s)
Brain/diagnostic imaging , Consciousness Disorders/diagnostic imaging , Epilepsy/diagnosis , Magnetic Resonance Imaging , Seizures/diagnostic imaging , Acute Disease , Atrophy , Child , Child, Preschool , Disease Progression , Female , Follow-Up Studies , Humans , Infant , Male , Prognosis , SyndromeABSTRACT
Paroxysmal sympathetic hyperactivity (PSH) is a dysautonomic condition that is associated with various types of acquired brain injuries. Traumatic brain lesions have been documented as the leading cause of PSH. However, detailed clinical features of pediatric PSH caused by intrinsic brain lesions remain to be elusive. We present a 3-year-old boy, who had been diagnosed as having cerebral palsy, developmental delay and epilepsy after perinatal hypoxia-induced brain injury. He developed status epilepticus with fever on the third day of respiratory infection. Whereas the seizure was terminated by systemic infusion of midazolam, consciousness remained disturbed for the next 48h. Serial magnetic resonance imaging studies revealed that acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) evolved on 3days after the seizure. Therapeutic hypothermia was immediately introduced, however, the brain lesion extended to the whole subcortical white matters on day 8. The intermittent bilateral dilation of pupils with increased blood pressure and tachycardia were observed until day 12. Real-time monitoring of electroencephalograms ruled out the recurrent attacks of seizures. The abnormal signs of autonomic nervous system gradually ceased and never relapsed after recovery from the hypothermia. PSH or a transient condition of dysautonomia may emerge and persist during the acute phase of AESD.
Subject(s)
Autonomic Nervous System Diseases/complications , Brain Diseases/complications , Brain/diagnostic imaging , Seizures/complications , Autonomic Nervous System Diseases/diagnostic imaging , Autonomic Nervous System Diseases/physiopathology , Autonomic Nervous System Diseases/therapy , Brain Diseases/diagnostic imaging , Brain Diseases/physiopathology , Brain Diseases/therapy , Child, Preschool , Diagnosis, Differential , Diffusion Magnetic Resonance Imaging , Humans , Male , Seizures/diagnostic imaging , Seizures/physiopathology , Seizures/therapyABSTRACT
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.