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1.
BMC Neurol ; 23(1): 7, 2023 Jan 07.
Artículo en Inglés | MEDLINE | ID: mdl-36609211

RESUMEN

BACKGROUND: Cytokine levels have been measured in acute encephalopathy (AE) to determine its pathology or as a diagnostic biomarker to distinguish it from febrile seizures (FS); however, the dynamics of cytokine level changes have not yet been fully captured in these two neurological manifestations. Thus, we aimed to explore the time course of serum cytokine level changes within 72 h after onset in AE and FS. METHODS: We retrospectively measured cytokine level in residual serum samples at multiple timepoints in seven children whose final diagnoses were AE or FS. RESULTS: The levels of 13 cytokines appeared to increase immediately after onset and peaked within 12-24 h after onset: interleukin (IL)-1ß, IL-4 IL-5, IL-6, IL-8, IL-10, IL-17, eotaxin, fibroblast growth factor, granulocyte colony-stimulating factor, interferon gamma, interferon-inducible protein-10, and macrophage chemoattractant protein-1. There were no dynamic changes in the levels of three cytokines (IL-1 receptor agonist, macrophage inflammatory protein-1α, and platelet-derived growth factor-bb) 72 h after onset. Levels of some cytokines decreased to around control levels within 48 h after onset: IL-1ß, IL-4, IL-5, IL-17, fibroblast growth factor, and interferon gamma. The levels of most cytokines appeared to be higher in AE, especially in hemorrhagic shock encephalopathy syndrome, than in FS. CONCLUSIONS: Cytokine levels in both AE and FS change dynamically, such as the levels of several cytokines increased within a few hours after onset and decreased at 12-24 h after onset. Therefore, it will be desirable to make clinical decisions regarding the administration of anti-inflammatory therapy in 24 h after onset in AE.


Asunto(s)
Encefalopatías , Convulsiones Febriles , Niño , Humanos , Citocinas , Interleucina-17 , Interferón gamma , Interleucina-4 , Estudios Retrospectivos , Interleucina-5
2.
BMC Neurol ; 23(1): 28, 2023 Jan 18.
Artículo en Inglés | MEDLINE | ID: mdl-36653748

RESUMEN

BACKGROUND: Patients with complex febrile seizures (CFS) often display abnormal laboratory results, unexpectedly prolonged seizures, and/or altered consciousness after admission. However, no standardized values have been established for the clinical and laboratory characteristics of CFS in the acute phase, making the management of CFS challenging. This study aimed to determine the clinical and laboratory characteristics of children with CFS during the acute phase. In particular, the duration of impaired consciousness and the detailed distribution of blood test values were focused. METHODS: We retrospectively reviewed medical records of a consecutive pediatric cohort aged 6-60 months who were diagnosed with CFS and admitted to Kobe Children's Hospital between October 2002 and March 2017. During the study period, 486 seizure episodes with confirmed CFS were initially reviewed, with 317 seizure episodes included in the analysis. Detailed clinical and laboratory characteristics were summarized. RESULTS: Among 317 seizure episodes (296 children with CFS), 302 required two or fewer anticonvulsants to be terminated. In 296 episodes showing convulsive seizures, median seizure duration was 30.5 min. The median time from onset to consciousness recovery was 175 min. Impaired consciousness lasting > 6, 8, and 12 h was observed in 13.9%, 7.6%, and 1.9% patients with CFS, respectively. Additionally, the distribution of aspartate aminotransferase, lactate dehydrogenase, creatinine, and glucose were clarified with 3, 10, 50, 90, and 97 percentile values. CONCLUSION: This study detailed the clinical and laboratory findings of acute-phase CFS using the data of the largest 15-year consecutive cohort of children with CFS. These results provide important information for appropriate acute management of CFS.


Asunto(s)
Convulsiones Febriles , Niño , Humanos , Lactante , Convulsiones Febriles/diagnóstico , Convulsiones Febriles/epidemiología , Estudios Retrospectivos , Japón/epidemiología , Convulsiones/diagnóstico , Convulsiones/epidemiología
4.
BMC Neurol ; 22(1): 77, 2022 Mar 05.
Artículo en Inglés | MEDLINE | ID: mdl-35247987

RESUMEN

BACKGROUND: Febrile status epilepticus is the most common form of status epilepticus in children. No previous reports compare the effectiveness of treatment strategies using fosphenytoin (fPHT) or phenobarbital (PB) and those using anesthetics as second-line anti-seizure medication for benzodiazepine-resistant convulsive status epilepticus (CSE). We aimed to examine the outcomes of various treatment strategies for febrile convulsive status epilepticus (FCSE) in a real-world setting while comparing the effects of different treatment protocols and their presence or absence. METHODS: This was a single-center historical cohort study that was divided into three periods. Patients who presented with febrile convulsive status epilepticus for ≥60 min even after the administration of at least one anticonvulsant were included. During period I (October 2002-December 2006), treatment was performed at the discretion of the attending physician, without a protocol. During period II (January 2007-February 2013), barbiturate coma therapy (BCT) was indicated for FCSE resistant to benzodiazepines. During period III (March 2013-April 2016), BCT was indicated for FCSE resistant to fPHT or PB. RESULTS: The rate of electroencephalogram monitoring was lower in period I than period II+III (11.5% vs. 85.7%, p<0.01). Midazolam was administered by continuous infusion more often in period I than period II+III (84.6% vs. 25.0%, p<0.01), whereas fPHT was administered less often in period I than period II+III (0% vs. 27.4%, p<0.01). The rate of poor outcome, which was determined using the Pediatric Cerebral Performance Category scale, was higher in period I than period II+III (23.1% vs. 7.1%, p=0.03). The rate of poor outcome did not differ between periods II and III (4.2% vs. 11.1%, p=0.40). CONCLUSIONS: While the presence of a treatment protocol for FCSE in children may improve outcomes, a treatment protocol using fPHT or PB may not be associated with better outcomes.


Asunto(s)
Estado Epiléptico , Anticonvulsivantes/uso terapéutico , Niño , Protocolos Clínicos , Estudios de Cohortes , Humanos , Pronóstico , Convulsiones/tratamiento farmacológico , Estado Epiléptico/diagnóstico , Estado Epiléptico/tratamiento farmacológico , Resultado del Tratamiento
5.
Brain Dev ; 44(3): 210-220, 2022 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-34716034

RESUMEN

OBJECTIVE: Biomarkers predicting poor outcomes of status-epilepticus-associated-with-fever (SEF) at an early stage may contribute to treatment guidance. However, none have been reported thus far. We investigated the dynamics of serum growth and differentiation factor (GDF)-15 after seizure onset in patients with SEF and determined whether GDF-15 can predict poor outcomes, particularly in the first 6 h after seizure onset. METHODS: We enrolled 37 pediatric patients with SEF and eight patients with simple febrile seizures (SFS) and collected their blood samples within 24 h of seizure onset and eight febrile control patients between March 1, 2017 and September 30, 2020. All patients were aged ≤15 years. RESULTS: In the SEF group, the median post-seizure serum GDF-15 values were 1,065 (<6h), 2,720 (6-12 h), and 2,411 (12-24 h) pg/mL. The median serum GDF-15 in the first 6 h was measured in patients with SEF without a significant past medical history (n = 21) and was found to be statistically significantly higher (1,587 pg/mL) than in the febrile control (551 pg/mL) and SFS (411 pg/mL) groups. The median serum GDF-15 was statistically significantly higher in patients with SEF with sequelae (n = 5) and patients with acute encephalopathy with biphasic seizures/reduced diffusion/hemorrhagic shock and encephalopathy syndrome (n = 6) than in patients with SEF without sequelae (n = 16) (15,898 vs 756 pg/mL) and patients with prolonged FS (n = 15) (9,448 vs 796 pg/mL). CONCLUSIONS: This study demonstrates the dynamics of serum GDF-15 in patients with SEF and indicates the potential of GDF-15 as an early predictor of poor outcomes.


Asunto(s)
Fiebre/diagnóstico , Factor 15 de Diferenciación de Crecimiento/sangre , Convulsiones Febriles/diagnóstico , Estado Epiléptico/diagnóstico , Adolescente , Biomarcadores/sangre , Niño , Preescolar , Femenino , Fiebre/sangre , Fiebre/complicaciones , Humanos , Lactante , Masculino , Pronóstico , Convulsiones Febriles/sangre , Estado Epiléptico/sangre , Estado Epiléptico/etiología
6.
Medicine (Baltimore) ; 100(30): e26660, 2021 Jul 30.
Artículo en Inglés | MEDLINE | ID: mdl-34397692

RESUMEN

ABSTRACT: Steroid pulse therapy is widely used to treat virus-associated acute encephalopathy, especially the cytokine storm type; however, its effectiveness remains unknown. We sought to investigate the effectiveness of early steroid pulse therapy for suspected acute encephalopathy in the presence of elevated aspartate aminotransferase (AST) levels.We enrolled children admitted to Hyogo Children's Hospital between 2003 and 2017 with convulsions or impaired consciousness accompanied by fever (temperature >38°C). The inclusion criteria were: refractory status epilepticus or prolonged neurological abnormality or hemiplegia at 6 hours from onset, and AST elevation >90 IU/L within 6 hours of onset. We excluded patients with a neurological history. We compared the prognosis between the groups with or without steroid pulse therapy within 24 hours. A good prognosis was defined as a Pediatric Cerebral Performance Category Scale (PCPC) score of 1-2 at the last evaluation, within 30 months of onset. Moreover, we analyzed the relationship between prognosis and time from onset to steroid pulse therapy.Fifteen patients with acute encephalopathy and 5 patients with febrile seizures were included in this study. Thirteen patients received steroid pulse therapy within 24 hours. There was no between-group difference in the proportion with a good prognosis. There was no significant correlation between PCPC and timing of steroid pulse therapy (rs = 0.253, P = .405). Even after excluding 2 patients with brainstem lesions, no significant correlation between PCPC and steroid pulse therapy timing (rs = 0.583, P = .060) was noted. However, the prognosis tended to be better in patients who received steroid pulse therapy earlier.Steroid pulse therapy within 24 hours did not improve the prognosis in children with suspected acute encephalopathy associated with elevated AST. Still, even earlier administration of treatment could prevent the possible neurological sequelae of this condition.


Asunto(s)
Encefalopatías/tratamiento farmacológico , Quimioterapia por Pulso/normas , Esteroides/uso terapéutico , Factores de Tiempo , Niño , Preescolar , Estudios de Cohortes , Femenino , Humanos , Lactante , Japón , Masculino , Pronóstico , Quimioterapia por Pulso/métodos , Quimioterapia por Pulso/estadística & datos numéricos
7.
Brain Dev ; 43(5): 616-625, 2021 May.
Artículo en Inglés | MEDLINE | ID: mdl-33563484

RESUMEN

OBJECTIVE: The clinical prediction rule (CPR) for acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) was developed with an area under the receiver operating characteristic curve (AUC) of 0.95 - 0.96. Our objective was to verify the AESD CPR in a new cohort and compare the utilities of three CPRs of acute encephalopathy: the Tada, Yokochi, and Nagase criteria. METHODS: We reviewed the clinical data and medical charts of 580 consecutive patients (aged < 18 years) with febrile convulsive status epilepticus lasting for ≥ 30 min in 2002 - 2017 and measured the performance of the CPRs in predicting AESD and sequelae. RESULTS: The CPRs predicted AESD with an AUC of 0.84 - 0.88. The Tada criteria predicted AESD with a positive predictive value (PPV) of 0.25 and a negative predictive value (NPV) of 0.99. The Yokochi criteria predicted AESD with a PPV and NPV of 0.20 and 0.95, respectively, after 12 h. The Nagase criteria predicted AESD with a PPV and NPV of 0.14 and 1.00, respectively, after 6 h. The PPVs of the Tada, Yokochi, and Nagase criteria for sequelae were 0.28, 0.28, and 0.17, respectively; the corresponding NPVs were 0.97, 0.95, and 0.98, respectively. CONCLUSIONS: The effectiveness of the AESD CPR in a new cohort was lower than that in the derivation study. CPRs are not sufficient as diagnostic tests, but they are useful as screening tests. The Nagase criteria are the most effective for screening among the three CPRs due to their high NPV and swiftness.


Asunto(s)
Encefalopatías/diagnóstico , Guías de Práctica Clínica como Asunto/normas , Convulsiones Febriles/diagnóstico , Estado Epiléptico/diagnóstico , Preescolar , Femenino , Escala de Coma de Glasgow , Hospitales Pediátricos , Humanos , Lactante , Masculino , Pronóstico
8.
Brain Dev ; 43(4): 548-555, 2021 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-33342582

RESUMEN

INTRODUCTION: Children with either febrile seizure or acute encephalopathy exhibit seizures and/or impaired consciousness accompanied by fever of unknown etiology (SICF). Among children with SICF, we previously reported those who have refractory status epilepticus or prolonged neurological abnormalities with normal AST levels are at a high risk for the development of acute encephalopathy with biphasic seizures and late reduced diffusion (AESD), considered to be caused by excitotoxicity. Non-convulsive seizures (NCS) are common in critically ill children and cause excitotoxic neuronal injury. The aim of this study was to elucidate the prevalence of NCS in the acute phase of children at a high risk for developing AESD and the relationship between NCS in the acute phase and neurological outcomes. METHODS: We studied 137 children with SICF at a high risk for developing AESD and who underwent continuous electroencephalogram monitoring (cEEG) upon admission to a tertiary pediatric care center at Hyogo Prefectural Kobe Children's Hospital between October 2007 and August 2018. Patient characteristics and outcomes were compared between patients with NCS and without NCS. RESULTS: Of the 137 children, NCS occurred in 30 children; the first NCS were detected in cEEG at the beginning in 63.3%, during the first hour in 90%, and within 12 h in 96.7%. Neurological sequelae were more common in NCS patients (20.0%) than in non-NCS patients (1.9%; p = 0.001). Five in 30 NCS patients (16.7%) and 3 in 107 non-NCS patients (2.8%) developed AESD (p = 0.013). CONCLUSION: The occurrence of NCS is associated with subsequent neurological sequelae, especially the development of AESD.


Asunto(s)
Encefalopatías/etiología , Encéfalo/fisiopatología , Convulsiones/complicaciones , Encefalopatías/fisiopatología , Niño , Preescolar , Progresión de la Enfermedad , Electroencefalografía , Femenino , Humanos , Lactante , Masculino , Estudios Retrospectivos , Convulsiones/fisiopatología
9.
Cytokine ; 137: 155324, 2021 01.
Artículo en Inglés | MEDLINE | ID: mdl-33032108

RESUMEN

Patients with hemorrhagic shock and encephalopathy syndrome (HSES) have a high early mortality rate, which may be caused by a 'cytokine storm'. However, there is little information on how cytokines and chemokines change over time in these patients. We aimed to describe the characteristics of HSES by examining changes in serum biomarker levels over time. Six patients with HSES were included. We retrospectively evaluated their clinical course and imaging/laboratory data. We measured serum levels of multiple cytokines [interleukin 1ß (IL-1ß), IL-2, IL-4, IL-6, IL-10, IL-17, interferon-gamma, and tumor necrosis factor alpha], chemokines (IL-8, monocyte chemoattractant protein-1, interferon-inducible protein-10), and growth and differentiation factor (GDF)-15. The highest cytokine and chemokine levels were noted in the first 24 h, and decreased thereafter. The GDF-15 level was markedly high. Cytokine, chemokine, and GDF-15 levels were significantly higher in patients with HSES than in controls in the first 24 h, except for IL-2 and IL-4. Patients with HSES have high inflammatory cytokine and chemokine levels, a high GDF-15 level in the first 24 h, and high lactate levels. Our study provides new insights on the pathophysiology of HSES, a detailed clinical picture of patients with HSES, and potential biomarkers.


Asunto(s)
Biomarcadores/sangre , Trastornos de la Coagulación Sanguínea/sangre , Encefalopatías/sangre , Quimiocinas/sangre , Citocinas/sangre , Factor 15 de Diferenciación de Crecimiento/sangre , Choque Hemorrágico/sangre , Trastornos de la Coagulación Sanguínea/diagnóstico , Trastornos de la Coagulación Sanguínea/terapia , Encefalopatías/diagnóstico , Encefalopatías/terapia , Quimiocina CCL2/sangre , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Estudios Retrospectivos , Choque Hemorrágico/diagnóstico , Choque Hemorrágico/terapia , Factores de Tiempo , Factor de Necrosis Tumoral alfa/sangre
10.
Seizure ; 80: 12-17, 2020 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-32480278

RESUMEN

PURPOSE: To evaluate barbiturate anaesthetic therapy using thiamylal for febrile refractory status epilepticus (fRSE) in children. METHODS: This was a review of a prospectively-collected database between April 2012-March 2016 for fRSE cases treated with thiamylal anaesthetic therapy in a single paediatric hospital in Japan. The sample comprised 23 children (median age, 23 months) with fRSE that underwent thiamylal anaesthetic therapy for convulsive seizures lasting longer than 60 min, sustained after intravenous administration of benzodiazepine and non-benzodiazepine anticonvulsants. The intervention comprised protocol-based thiamylal anaesthetic therapy with bolus administration. We measured the dose and time required to achieve the burst suppression pattern (BSP) on electroencephalography, seizure recurrence, death, neurological sequelae, and complications. RESULTS: All patients except one reached the BSP. The thiamylal median dose until reaching the BSP was 27.5 mg/kg, and the median time from thiamylal administration to reaching the BSP was 109.5 min. There was one case of immediate treatment failure and one of withdrawal seizure, but no breakthrough seizure. No deaths occurred during treatment, and neurological sequelae occurred in four cases (17%). Vasopressors were administered in all cases. Other complications included 11 cases of pneumonia and one of enterocolitis. CONCLUSION: We revealed the time and dose required to reach the BSP with thiamylal anaesthetic therapy using bolus administration in children. Our results suggested that reaching the BSP with bolus administration requires markedly less time than without bolus administration, rarely causes seizure recurrence in paediatric fRSE, and causes haemodynamic dysfunction and infections as often as observed without bolus administration.


Asunto(s)
Anestésicos , Estado Epiléptico , Anestésicos/uso terapéutico , Anticonvulsivantes/uso terapéutico , Niño , Preescolar , Humanos , Lactante , Japón , Estado Epiléptico/tratamiento farmacológico , Tiamilal/uso terapéutico
11.
J Neurol Sci ; 411: 116684, 2020 Apr 15.
Artículo en Inglés | MEDLINE | ID: mdl-32001378

RESUMEN

OBJECTIVE: Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is a syndrome characterized by biphasic seizures with impaired consciousness. AESD is rare outside Asia, and consecutive cohort studies are therefore scarce. Herein, we aimed to describe the detailed characteristics of AESD, including clinical course, electroencephalogram data, laboratory data, imaging findings, treatment, and outcomes. METHODS: We reviewed the clinical database and medical charts of 43 consecutive pediatric patients (<18 years old) who developed AESD between October 1, 2002, and September 30, 2019. RESULTS: We found that AESD occurred even though patients did not develop prolonged seizures. A comparison between the two groups (first seizure duration <30 min and first seizure duration ≥30 min) revealed three main findings: first, patients with AESD who had shorter seizures had better prognosis than those with prolonged seizures; second, patients with AESD who had shorter seizures tended to have earlier occurrence of a second seizure; and third, high signal intensity on diffusion-weighted magnetic resonance imaging was observed mainly in frontal areas, not diffusely, in patients with shorter seizures, and in a broader area in patients with prolonged seizures. CONCLUSIONS: Our description of the detailed clinical picture of AESD may add new insight into its pathophysiology.


Asunto(s)
Encefalopatías , Estado Epiléptico , Adolescente , Asia , Niño , Estudios de Cohortes , Imagen de Difusión por Resonancia Magnética , Humanos , Lactante , Convulsiones/diagnóstico por imagen , Convulsiones/epidemiología
12.
J Infect Chemother ; 26(5): 516-519, 2020 May.
Artículo en Inglés | MEDLINE | ID: mdl-31983616

RESUMEN

BACKGROUND: Fukuyama congenital muscular dystrophy (FCMD), which is characterized by generalized muscle weakness, hypotonia, and motor delay during early infancy, gradually progresses with advanced age. Although acute rhabdomyolysis following infection in patients with FCMD has occasionally been reported, no studies have investigated rhabdomyolysis following viral infection in FCMD patients during early infancy. CASE REPORT: We report the case of a 50-day-old girl with no apparent symptoms of muscular dystrophy who developed severe acute rhabdomyolysis caused by viral infection, resulting in quadriplegia and respiratory failure therefore requiring mechanical ventilation. Brain magnetic resonance imaging incidentally showed the typical characteristics of FCMD, and FCMD was confirmed by genetic analysis, which revealed a 3-kb retrotransposon insertion in one allele of the fukutin gene and a deep intronic splicing variant in intron 5 in another allele. The virus etiology was confirmed to be Coxsackie A4. CONCLUSION: We report a severe case of acute rhabdomyolysis with the earliest onset of symptoms due to the Coxsackie A4 virus in a patient with FCMD. The present findings indicate that physicians should consider FCMD with viral infection a differential diagnosis if the patient presents with acute rhabdomyolysis following a fever.


Asunto(s)
Infecciones por Coxsackievirus/virología , Enterovirus Humano A/patogenicidad , Rabdomiólisis/virología , Síndrome de Walker-Warburg/complicaciones , Enfermedad Aguda , Infecciones por Coxsackievirus/complicaciones , Infecciones por Coxsackievirus/diagnóstico , Diagnóstico Diferencial , Enterovirus Humano A/genética , Enterovirus Humano A/aislamiento & purificación , Femenino , Humanos , Lactante , Imagen por Resonancia Magnética , Proteínas de la Membrana/genética , Reacción en Cadena de la Polimerasa , Cuadriplejía/etiología , ARN Viral , Respiración Artificial , Insuficiencia Respiratoria/etiología , Rabdomiólisis/complicaciones , Rabdomiólisis/diagnóstico , Índice de Severidad de la Enfermedad , Síndrome de Walker-Warburg/diagnóstico , Síndrome de Walker-Warburg/virología
13.
J Infect Public Health ; 13(1): 140-142, 2020 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-31176605

RESUMEN

Empyema necessitatis (EN) is a rare complication of empyema in which the pleural infection spreads outside the pleural space. Lower airway infections are common among children with cerebral palsy (CP). Although harmless to healthy individuals, Pseudomonas aeruginosa can cause invasive infections, including CP, in immunocompromised hosts. Mycobacterium tuberculosis and Actinomyces spp. have been reported as common causative organisms of EN. However, EN caused by P. aeruginosa has never been reported. We report the case of an 8-year-old girl with CP without tracheotomy who was admitted to our hospital with complaints of fever and increased epileptic seizures. First, she was diagnosed with pneumonia and treated with antibiotics. However, seven days after admission, a palpable mobile mass overlying the lower part of the shoulder blade was noticed. Enhanced magnetic resonance imaging revealed broad high signal area on T2-weighted and diffusion-weighted images, indicating empyema of the left lower lung that had penetrated the pleural wall and spread to the subcutaneous area of the left back. Thus, she was diagnosed with EN. Twelve days after admission, P. aeruginosa was detected from the pus culture. Patients with CP who have chronic lung diseases, such as pneumonia, atelectasis, or empyema, may need careful follow up.


Asunto(s)
Parálisis Cerebral/complicaciones , Empiema Pleural/diagnóstico por imagen , Empiema Pleural/microbiología , Infecciones por Pseudomonas/complicaciones , Absceso/microbiología , Parálisis Cerebral/microbiología , Niño , Femenino , Humanos , Imagen por Resonancia Magnética , Pseudomonas aeruginosa , Hombro/diagnóstico por imagen , Hombro/microbiología , Hombro/patología , Infecciones de los Tejidos Blandos/diagnóstico por imagen , Infecciones de los Tejidos Blandos/etiología , Infecciones de los Tejidos Blandos/microbiología
14.
Brain Dev ; 41(8): 691-698, 2019 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-31337523

RESUMEN

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, 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.


Asunto(s)
Encefalopatía Aguda Febril/mortalidad , Encefalopatías/mortalidad , Enfermedad Aguda , Encefalopatía Aguda Febril/diagnóstico , Encefalopatía Aguda Febril/fisiopatología , Adolescente , Muerte Encefálica , Encefalopatías/diagnóstico , Encefalopatías/fisiopatología , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Estudios Retrospectivos , Convulsiones , Factores de Tiempo
15.
Pediatr Neurol ; 99: 3-6, 2019 10.
Artículo en Inglés | MEDLINE | ID: mdl-31201068

RESUMEN

Fibrocartilaginous embolism is assumed to be caused by fibrocartilaginous nucleus pulposus component migration through retrograde embolization to the spinal cord artery. Fibrocartilaginous embolism is currently not well recognized among pediatricians because of its rarity. We describe a previously healthy 15-year-old soccer player who, after kicking a ball, developed progressive weakness in both legs and urinary retention the next day. Magnetic resonance imaging revealed T2 hyperintensity in the anterior horn of the spinal cord at the Th12/L1 level with Schmorl node at the level of L1/2. We also review the previous literature on fibrocartilaginous embolism of the spinal cord in children (less than18 years age); a total of 25 pediatric patients, including our patient, were identified. The median age was 14 years, and 64% of the reviewed patients were female. The most common trigger event was intense exercise or sports. The neurological symptoms started within one day in most cases, and the time to symptom peak varied from a few hours to two weeks. The most common initial neurological symptoms were weakness or plegia (100%), followed by paresthesia or numbness (48%). Affected areas of the spinal cord were distributed evenly from the cervical to thoracolumbar regions. Although steroids and anticoagulants were most commonly used, the prognosis was quite poor (mild to severe sequelae with three deaths). Although fibrocartilaginous embolism is a very rare condition, physicians should be aware of the characteristics and include fibrocartilaginous embolism of the spinal cord in their differential diagnosis, especially for physically active patients.


Asunto(s)
Enfermedades de los Cartílagos/diagnóstico , Embolia/diagnóstico , Trastornos Neurológicos de la Marcha/etiología , Desplazamiento del Disco Intervertebral/complicaciones , Debilidad Muscular/etiología , Núcleo Pulposo , Fútbol/lesiones , Isquemia de la Médula Espinal/etiología , Adolescente , Enfermedades de los Cartílagos/diagnóstico por imagen , Enfermedades de los Cartílagos/etiología , Diagnóstico Diferencial , Embolia/diagnóstico por imagen , Embolia/etiología , Humanos , Vértebras Lumbares , Imagen por Resonancia Magnética , Masculino , Mielitis Transversa/diagnóstico , Reflejo Anormal , Reflejo de Babinski , Retención Urinaria/etiología
16.
Brain Dev ; 41(7): 604-613, 2019 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-30929765

RESUMEN

BACKGROUND: Seizures and/or impaired consciousness accompanied by fever without known etiology (SICF) is common in the pediatric emergency setting. No optimal strategy for the management of SICF in childhood currently exists. We previously demonstrated the effectiveness of targeted temperature management (TTM) against SICF with a high risk of morbidity; however, some patients with SICF develop neurological sequelae despite TTM, which necessitate additional neuroprotective treatment. The clinical characteristics of these severe cases have not been studied. Accordingly, the aim of this study was to identify the clinical characteristics of children with SICF who exhibit poor outcomes after TTM. METHODS: The medical records of children admitted to Kobe Children's Hospital (Kobe, Japan) between October 2002 and September 2016 were retrospectively reviewed. Patients with SICF treated using TTM were included and divided into the satisfactory and poor outcome groups. Univariate and multivariate logistic regression analyses were used to compare clinical characteristics and laboratory findings between the two groups. RESULTS: Of the 73 included children, 10 exhibited poor outcomes. Univariate logistic regression analysis revealed that acute circulatory failure before TTM initiation, the use of four or more types of anticonvulsants, methylprednisolone pulse therapy, and an aspartate aminotransferase (AST) level ≥73 IU/L were associated with poor outcomes. Multivariate logistic regression analysis identified an elevated AST level as a significant independent predictor of a poor outcome. CONCLUSIONS: An elevated AST level within 12 h of onset in children with SICF is an independent predictor of a poor outcome after TTM initiated within 24 h of onset.


Asunto(s)
Fiebre de Origen Desconocido/terapia , Hipotermia Inducida/efectos adversos , Adolescente , Anticonvulsivantes/uso terapéutico , Aspartato Aminotransferasas/análisis , Aspartato Aminotransferasas/sangre , Biomarcadores Farmacológicos/sangre , Niño , Preescolar , Estado de Conciencia , Femenino , Fiebre/etiología , Humanos , Hipotermia Inducida/métodos , Lactante , Japón , Masculino , Pronóstico , Estudios Retrospectivos , Convulsiones/tratamiento farmacológico , Temperatura , Resultado del Tratamiento
17.
Brain Dev ; 41(6): 531-537, 2019 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-30833092

RESUMEN

The detailed clinical time course in acute disseminated encephalomyelitis (ADEM) from initial symptoms, through exacerbation, to remission has not been widely reported. Hence, this study aimed to investigate the clinical time course of pediatric ADEM. This was a multicenter retrospective study based on registry data from medical chart reviews. The study included children who met the international consensus diagnostic criteria for ADEM. The patients comprised 18 boys and 6 girls, with a mean age of 5.5 ±â€¯3.3 years at onset. From onset, the time until peak neurological symptoms, time until initial improvement, and time until full recovery was 3.1 ±â€¯3.7 days, 6.0 ±â€¯4.5 days, and 26 ±â€¯34 days, respectively. Twenty-three (96%) patients were treated with high-dose methylprednisolone (mPSL) with a mean duration of 4.1 ±â€¯4.0 days from onset. The condition of 15 patients (65%) improved within 3 days of high-dose mPSL initiation, whereas, that of four patients began to improve after >5 days of high-dose mPSL initiation. Only one patient (4%) did not achieve full recovery despite treatment with high-dose mPSL, intravenous immunoglobulin, and plasma exchange. This study presents the detailed clinical time course in pediatric ADEM in Japan. Progression of neurologic deficits typically lasts a few days, with initial improvement in 1 week leading to full recovery within 1 month.


Asunto(s)
Encefalomielitis Aguda Diseminada/diagnóstico , Encefalomielitis Aguda Diseminada/fisiopatología , Niño , Preescolar , Encefalomielitis Aguda Diseminada/clasificación , Femenino , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Japón , Imagen por Resonancia Magnética , Masculino , Metilprednisolona/farmacología , Intercambio Plasmático , Plasmaféresis , Sistema de Registros , Estudios Retrospectivos
18.
Epilepsy Behav ; 90: 15-19, 2019 01.
Artículo en Inglés | MEDLINE | ID: mdl-30500483

RESUMEN

Although previous studies have investigated the influence of antiepileptic drugs (AEDs) on lipid profiles and thyroid hormone levels, there is little evidence regarding the effects of levetiracetam (LEV). Therefore, we conducted a prospective longitudinal study to evaluate the effects of LEV and carbamazepine (CBZ) treatment on lipid profile and thyroid hormone levels in patients newly diagnosed with epilepsy. Inclusion criteria were as follows: (a) age between 4 and 15 years, (b) diagnosis of epilepsy with at least two focal seizures within a year, and (c) newly treated with LEV or CBZ monotherapy. Serum lipid profile and thyroid hormone levels were measured before and after 1 and 6 months of AED initiation. Among the 21 included patients (LEV: 13 patients, CBZ: 8 patients), all but one patient in the LEV group continued AED monotherapy during the study period. Although triglyceride (TG) levels tended to be increased in the CBZ group (baseline: 58.3 ±â€¯22.0 mg/dl, 1 month: 63.8 ±â€¯21.6 mg/dl, 6 months: 92.3 ±â€¯63.6 mg/dl, p = 0.22, analyses of variance (ANOVA)), there were no significant changes in total cholesterol (TC), TG levels, high-density lipoprotein cholesterol (HDL-C), or low-density lipoprotein cholesterol (LDL-C) in either group. Serum free thyroxine (fT4) levels were significantly decreased in the CBZ group (baseline: 1.15 ±â€¯0.06 ng/dl, 1 month: 1.00 ±â€¯0.16 ng/dl, 6 months: 0.98 ±â€¯0.14 ng/dl, p = 0.03, ANOVA). In contrast, there were no significant changes in fT4 or thyroid-stimulating hormone (TSH) levels in the LEV group. The results of the present study suggest that LEV monotherapy does not affect lipid profile or thyroid function while CBZ monotherapy may cause thyroid dysfunction.


Asunto(s)
Anticonvulsivantes/efectos adversos , Carbamazepina/efectos adversos , Epilepsia/sangre , Epilepsia/tratamiento farmacológico , Levetiracetam/efectos adversos , Enfermedades de la Tiroides/inducido químicamente , Tirotropina/sangre , Tiroxina/sangre , Triglicéridos/sangre , Adolescente , Anticonvulsivantes/administración & dosificación , Carbamazepina/administración & dosificación , Niño , Preescolar , HDL-Colesterol/sangre , HDL-Colesterol/efectos de los fármacos , LDL-Colesterol/sangre , LDL-Colesterol/efectos de los fármacos , Femenino , Humanos , Levetiracetam/administración & dosificación , Masculino , Estudios Prospectivos , Tirotropina/efectos de los fármacos , Tiroxina/efectos de los fármacos
19.
Brain Dev ; 41(4): 392-395, 2019 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-30471873

RESUMEN

BACKGROUND: The initial symptoms of Guillain-Barre Syndrome (GBS) can be similar to a case of spontaneous spinal epidural hematoma (SSEH) located at the cervicothoracic junction. Therefore, SSEH may be misdiagnosed as GBS. CASE REPORT: A previously healthy 6-year-old girl presented with a 2-day history of progressive pain in the lower extremities and an inability to walk. On initial evaluation, she was completely paraparetic in the lower extremities. Deep tendon reflexes were absent in the lower extremities, and Babinski reflexes were positive on both sides. She exhibited reduced response to light touch and pinprick with a sensory level below T10, and experienced difficulty during urination. However, the strength, sensation and flexion of upper extremities were normal. Because her presentation and examinations were consistent with GBS, we initiated intravenous immunoglobulin therapy. The next day, she also developed pain and muscle weakness of the right upper extremity. Three days after admission, respiratory depression progressed rapidly. Spinal MRI showed a mass extending from the level of C7-T3, with spinal cord compression. The patient underwent an emergency laminectomy with evacuation of hematoma, and was diagnosed with SSEH. Sixty days after admission, she was transferred to the rehabilitation hospital with severe neurologic sequelae of paralysis in both legs. CONCLUSION: SSEH might have severe consequences, including neurologic deficits and risk of death. This case report serves to raise the awareness of SSEH that mimics the initial presentation of GBS.


Asunto(s)
Hematoma Espinal Epidural/diagnóstico , Niño , Errores Diagnósticos , Femenino , Síndrome de Guillain-Barré/diagnóstico , Humanos , Imagen por Resonancia Magnética , Debilidad Muscular , Compresión de la Médula Espinal/complicaciones
20.
J Pediatr ; 207: 213-219.e3, 2019 04.
Artículo en Inglés | MEDLINE | ID: mdl-30528574

RESUMEN

OBJECTIVES: To evaluate the proportion of children presenting to the emergency department (ED) with altered mental status who demonstrate nonconvulsive seizures on reduced-lead electroencephalography (EEG), and to further investigate the characteristics, treatment, and outcomes in these patients compared with patients without nonconvulsive seizures. STUDY DESIGN: In this retrospective cohort study, we reviewed the database and medical records of pediatric patients (aged <18 years) in a single ED between May 1, 2016, and April 30, 2018. We first determined the proportion of nonconvulsive seizures among patients with altered mental status (Glasgow Coma Scale <15). We then compared the clinical presentation, demographic data, clinical diagnosis, EEG results, treatment, and outcomes of patients with altered mental status with nonconvulsive seizures and those without nonconvulsive seizures. RESULTS: In total, 16.9% of the patients with altered mental status (41 of 242; 95% CI, 12.2%-21.6%) evaluated by EEG had detectable nonconvulsive seizure, equivalent to 4.4% (41 of 932) of all patients with altered mental status presenting at our hospital. More than 80% of patients monitored for nonconvulsive seizures had a previous history of seizures, often febrile. Patients with nonconvulsive seizures were older (median, 68.5 vs 36.1 months) and had a higher Pediatric Cerebral Performance Category score at presentation (median, 2.0 vs 1.0). In addition, the proportion of patients admitted to the intensive care unit was significantly higher in the patients with nonconvulsive seizures (30.3% vs 15.0%). However, total duration of hospitalization, neurologic sequelae, and 30-day mortality rate did not differ between the 2 groups. CONCLUSIONS: A relatively high percentage of pediatric patients with altered mental status in the ED experience nonconvulsive seizures. The use of reduced-lead EEG monitoring in the ED might facilitate the recognition and treatment of nonconvulsive seizures, especially among patients with a history of seizures.


Asunto(s)
Electroencefalografía/métodos , Epilepsia Generalizada/diagnóstico , Unidades de Cuidado Intensivo Pediátrico/estadística & datos numéricos , Salud Mental , Escala del Estado Mental , Preescolar , Epilepsia Generalizada/epidemiología , Epilepsia Generalizada/fisiopatología , Estudios de Seguimiento , Humanos , Incidencia , Lactante , Japón/epidemiología , Estudios Retrospectivos , Tasa de Supervivencia/tendencias
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