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OBJECTIVES@#To analyze the clinical characteristics and prognosis of children with anti-N-methyl--aspartate receptor (NMDAR) encephalitis and to provide a basis for early clinical identification of this disease.@*METHODS@#The clinical data of 42 cases of anti-NMDAR encephalitis at Department of Pediatrics, Second Xiangya Hospital, Central South University from January 2015 to March 2018 were collected. The clinical features and followed-up outcomes were analyzed retrospectively.@*RESULTS@#There were 15 cases (35.7%) of males and 27 cases (64.3%) of females in 42 children, with a ratio of 1꞉1.8. They were aged from 4 months to 17 years, with an average of (9.20±4.66) years. The most common initial symptoms were seizures (47.6%, 20/42) and mental behavior disorder (35.7%, 15/42). During the course of the disease, 85.7% patients(36/42) had mental and behavior disorder, 85.7% patients (36/42) had epilepsy, 76.2% (32/42) had speech disorder, 66.7% patients (28/42) had dyskinesia, 66.7% patients (28/42) had the decreased level of consciousness, 61.9% patients (26/42) had autonomic instability, and 57.1% (24/42) patients had sleep disorder. All the children had positive antibody against NMDA receptor resistance encephalitis in cerebrospinal fluid. Head MRI showed the abnormal incidence was 50.0% (21/42), and the lesions involved in parietal lobe, frontal lobe, temporal lobe, occipital lobe, midbrain, thalamus, basal ganglia and optic nerve. There was a patient with optic nerve damage combined with myelin oligodendrocyte glycoprotein (MOG) antibody positive. Forty cases were examined by electroencephalogram (EEG), 92.5% cases (37/40) were abnormal, mainly showing diffuse slow waves, and δ brushes could be seen in severe cases. And there was 1 patient (2.4%) complicated with mesenteric teratoma. The mRS score (2.14±1.46) at discharge was significantly lower than the highest mRS score (3.88±1.38) during hospitalization (<0.05). After 3-39 months of follow-up, mRS score at 3 months after discharge was only 0.81±1.29, which was still improved compared with that at discharge, 76.2% cases (32/42) experienced complete or near-complete recovery (mRS score≤2), and 4.8% (2/42) cases relapsed. There was no mortality; the initial time of immunotherapy and the highest mRS score in the course of the disease were the factors affecting the prognosis. The earlier the starting time for immunotherapy and the lower mRS score in the course of the disease were, the better the prognosis was.@*CONCLUSIONS@#Seizures, mental and behavior disorder, dyskinesias, speech disorder and autonomic instability are common clinical manifestations of anti-NMDAR encephalitis in children. The effect of immunotherapy is significant, and the time to start immunotherapy and the severity of the disease are important factors affecting the prognosis. Anti-NMDAR encephalitis can be combined with other autoantibodies, but its clinical significance and mechanism need further study.
Subject(s)
Adolescent , Child , Child, Preschool , Female , Humans , Infant , Male , Anti-N-Methyl-D-Aspartate Receptor Encephalitis , Autoantibodies , Electroencephalography , Magnetic Resonance Imaging , Receptors, N-Methyl-D-Aspartate , Retrospective StudiesABSTRACT
OBJECTIVE@#To study the clinical features of vasovagal syncope (VVS) and postural orthostatic tachycardia syndrome (POTS) in children with neurological symptoms at disease onset.@*METHODS@#A retrospective analysis was performed on the medical data of 88 children with the initial symptoms of the nervous system, such as transient loss of consciousness, dizziness, headache, and convulsion, who were finally diagnosed with VVS or POTS.@*RESULTS@#Of the 88 children, there were 35 boys (40%) and 53 girls (60%), with an age of 4-15 years. The peak age of onset was between 10 and 13 years. All the children had the initial symptoms of transient loss of consciousness, dizziness, headache, and convulsion. Nervous system diseases were excluded by electroencephalography, cerebrospinal fluid examination, and cranial MRI. Of the 88 children, 53 (60%) were confirmed with VVS, and 35 (40%) with POTS, according to the results of head-up tilt test (HUTT). Five children with the initial symptom of transient loss of consciousness were misdiagnosed with epilepsy. Predisposing factors were determined for 59 children (67%), and prolonged standing was the most common factor, followed by change in body position and strenuous exercise. Premonitory symptoms were observed in 66 children (75%), among which chest discomfort was the most common symptom, followed by gastrointestinal symptoms (nausea, vomiting, and abdominal pain) and pale complexion. All 88 children received health education and exercise for autonomic nerve function, among whom 53 children with VVS were given oral rehydration salts and 35 children with POTS were given oral rehydration salts and metoprolol. All 88 children were followed up for 18 months, and the response rates to the above treatment at 3, 6, 12, and 18 months of follow-up were 87%, 93%, 93%, and 90% respectively.@*CONCLUSIONS@#In addition to nervous system diseases, functional cardiovascular diseases including VVS and POTS should be considered for children with the initial symptoms of transient loss of consciousness, dizziness, headache, and convulsion. HUTT can be used to make a confirmed diagnosis, and the early treatment can achieve a good outcome.
Subject(s)
Adolescent , Child , Child, Preschool , Female , Humans , Male , Postural Orthostatic Tachycardia Syndrome , Posture , Retrospective Studies , Syncope, Vasovagal , Tilt-Table TestABSTRACT
Objective To analyze the significance of the Calgary Syncope Seizures Scores (CSSS)and the Modified Calgary Syncope Seizure Scores (MCSSS)for differential diagnosis of syncope or epilepsy in children. Methods Totally 201 children[95 male,and 1 06 female,aged 5 -1 8 years,mean age (1 1 .76 ±3.03)years]with syncope or epilepsy who visited the syncope clinic or admitted to the Department of Nerve Specialty Clinic of Pediatrics, the Second Xiangya Hospital of Central South University from October 201 3 to April 201 4 were included in the study. Patients were eligible if they had ≥1 loss of consciousness.The diagnosis was analyzed by the CSSS and the MCSSS and receiver operating characteristic (ROC)curve was used to explore the predictive value of different scores in differential diagnosis of syncope or epilepsy in children.Results There were significant differences in the CSSS be-tween syncope[-4(-6,1 )]and epilepsy[2(-3,5)]in children(Z =-1 1 .63,P <0.01 ).When the score was ≥1 ,the sensitivity and specificity of the differential diagnosis between syncope and epilepsy were 91 .46% and 95.80%, respectively;and Youden index was 0.87.Epilepsy should be considered when the score was ≥1 .There were significant differences in the MCSSS between syncope[-4(-6,1 )]and epilepsy[3(-3,6)]in children(Z =-1 1 .71 ,P <0.01 ).When the score was ≥1 ,the sensitivity and specificity of the differential diagnosis between syncope and epilep-sy were 92.68% and 96.64%,respectively;and Youden index was 0.89.Epilepsy should be considered when the score was ≥1 .Conclusions CSSS and MCSSS might be used as an initial diagnostic method in differential diagnosis be-tween syncope and epilepsy in children,based on the history of the patients.MCSSS in the differential diagnosis between syncope and epilepsy in children was more objective,easier to operate in the clinical work than CSSS.
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Syncope and epilepsy are common pediatric clinical symptoms and causes of paroxysmal loss of consciousness.They can be manifested as a sudden attack,transient and reversible loss of consciousness,easily leading to misdiagnosis in clinics.The etiology and pathogenesis of syncope and epilepsy are completely different,and the principle of treatment is also different.Therefore,in clinics,making an early diagnosis and differential diagnosis between syncope and epilepsy has an important significance to improve the treatment and the prognosis of the patients.
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Objective To observe the changes of apoptosis signal-regulating kinase 1 (ASK1) expression in left ventricular myocardium of the dilated cardiomyopathy (DCM) rats induced by adriamycin and its correlation with left ventricular ejection fraction (LVEF).Methods One hundred and twenty SPF Wistar rats were divided into 2 groups as follows:control group (n =15),model group(n =105).Adriamycin was administered in the model group by intraperitoneal injection for 3 times in a week and repeated with a two-week interval for 6 times,while 9 g/L saline were administered in the control group in the same pattern,thus DCM rats model were constructed by the end of the 8th week.Both the model group and control group rats were drawn out and their LVEF were tested by the end of the 8th week and 12th week.Apoptosis index (AI) and the ASK1 in myocardium were tested respectively by apoptotic cells situ labeling,semi-quantitative analysis and Western blot.Results The AI of the control group,the 8th weekend model group and the 12th weekend model group were 0.53 ±0.27,16.13 ± 1.72,19.54 ±2.24.The AI of the 8th and the 12th weekend model groups were obviously higher than that in the control group.Comparing the differences among the 3 groups were statistically significant (F =18.98,P ＜ 0.05).The relative ASK1 expressions in ventricular myocardium of the control group,the 8th weekend model group and the 12th weekend model group were 0.169 ± 0.010,0.649 ± 0.071,0.781 ±0.077.Comparing the differences among the 3 groups were statistically significant (F =27.72,P ＜ 0.01).The LVEF (％) results of the control group,the 8th weekend model group and the 12th weekend model group were 75.41 ± 2.02,53.25 ±3.74,39.21 ±6.13.Comparing the differences among the 3 groups were statistically significant(F =154.87,P ＜0.01).There was a negative correlation between the ASK1 expression and LVFE in model group at the end of 12weeks(r =-0.86,P ＜ 0.01).Conclusions The ASK1 expression in myocardium of the DCM group increases obviously,thus apoptosis signal-regulating probably participates in the pathological process of DCM induced by ASK1.
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<p><b>OBJECTIVE</b>To investigate the behavior problems of children aged 3 to 5 years in Changsha and to compare the differences of the results detected by the norm of Conners Parent Symptom Questionnaire (PSQ) in Chinese and American urban children.</p><p><b>METHODS</b>A total of 854 children aged 3 to 5 years were randomly sampled from 5 districts in Changsha City and their parents completed the Conners PSQ.</p><p><b>RESULTS</b>The assessment by the norm of PSQ in American urban children demonstrated that the average prevalence of behavior problems was 20.4%, with 28.1% for boys and 12.4% for girls. There were significant differences between boys and girls (P<0.01). The factor score of anxiety in girls was markedly higher than that in boys (P<0.01). Learning and psychosomatic problems were the main problems in the children. The prevalences of conduct problems and impulse-restlessness, and abnormal restlessness index detected by the norm of PSQ in Chinese urban children were higher than those detected by the American norm. The prevalences of conduct problems and psychosomatic problems in boys by the norm of PSQ in Chinese urban children were significantly lower than those detected by the American norm, while the total prevalence of behavior problems was higher than that detected by American norm. There was a poor consistency in the assessment results of most factors of the PSQ and the total prevalence of behavior problems detected by the Chinese and American norms (KappP<0.4).</p><p><b>CONCLUSIONS</b>The prevalence of behavior problems especially learning problems and psychosomatic problems in children aged 3 to 5 years is higher in Changsha. The consistency of assessment results is poor between the norms of China and America. It is recommended to use the China norm of PSQ in Chinese children aged 3 to 5 years because the Chinese norm is in line with China's national conditions and cultural background.</p>
Subject(s)
Child, Preschool , Female , Humans , Male , Child Behavior Disorders , Diagnosis , China , Surveys and Questionnaires , United States , Urban HealthABSTRACT
<p><b>OBJECTIVE</b>To study the changes of glucocorticoid receptor (GR) expression in embryonic rat cortical neurons exposed to transient Mg(2+)-free treatment.</p><p><b>METHODS</b>Six days after rat cortical neuronal cultures, two groups were created based on the medium to which were transiently exposed. The control group was exposed to a physiological solution (PS), and the Mg(2+)-free group was exposed to the same medium as the control group except for the removal of magnesium. The expression of GR mRNA and protein was determined by real-time PCR and immunocytochemistry staining 1, 7 and 12 days after transient Mg(2+)-free treatment.</p><p><b>RESULTS</b>Compared to the control group, the Mg(2+)-free group displayed the significantly less accumulated optical density (AOD) of GR immunoreactivity 12 days after transient Mg(2+)-free treatment (p<0.05). On the contrary, GR mRNA expression increased significantly 1 and 7 days after transient Mg(2+)-free treatment in the Mg(2+)-free group (p<0.05).</p><p><b>CONCLUSIONS</b>GR expression is modified following Mg-free-induced injury in cultured developing neurons in rats.</p>
Subject(s)
Animals , Rats , Cell Survival , Cells, Cultured , Cerebral Cortex , Metabolism , Fetus , Metabolism , Hypothalamo-Hypophyseal System , Physiology , Magnesium , Physiology , Neurons , Metabolism , Pituitary-Adrenal System , Physiology , RNA, Messenger , Rats, Wistar , Receptors, Glucocorticoid , GeneticsABSTRACT
<p><b>OBJECTIVE</b>To investigate the effets of flurothyl-induced neonatal recurrent seizures on glucocorticoid receptor (GR) expression in the rat brain.</p><p><b>METHODS</b>Forty-eight seven-day-old Sprague-Dawley rats were randomly divided into two groups: control and seizure. Seizures were induced by inhalant flurothyl daily for six consecutive days. Brains were sampled on postnatal days 13, 15 and 19. The expression of GR protein in the cerebral cortex was detected by Western blot and immunohistochemical method.</p><p><b>RESULTS</b>The expression of GR in the cerebral cortical plasma protein was significantly lower in the seizure group than in the control group on postnatal day 15. The expression of GR protein in the cerebral cortical nuclear protein decreased significantly in the seizure group compared with that in the control group on postnatal days 15 and 19 (p<0.05). Compared to the control group, the accumulated optical density (AOD) of GR immunoreactivity (IR) decreased significantly in the parietal cortex on postnatal day 13 (p<0.05), the AOD of GR IR decreased significantly in the parietal cortex and the temporal cortex on postnatal day 15 (p<0.05), and the AOD of GR IR decreased significantly in the parietal cortex, temporal cortex and the frontal cortex in the seizure group on postnatal day 19 (p<0.05).</p><p><b>CONCLUSIONS</b>Recurrent seizures in neonatal rats result in abnormal GR expression in the cerebral cortex which might play an important role in short-term brain injury induced by early recurrent seizures.</p>
Subject(s)
Animals , Female , Male , Rats , Blotting, Western , Cerebral Cortex , Chemistry , Hypothalamo-Hypophyseal System , Physiology , Immunohistochemistry , Pituitary-Adrenal System , Physiology , Rats, Sprague-Dawley , Receptors, Glucocorticoid , Physiology , Recurrence , Seizures , MetabolismABSTRACT
<p><b>OBJECTIVE</b>To investigate the short-term effects of flurothyl-induced neonatal recurrent seizures on gamma-aminobutyric acid A receptor (GABAAR) alpha1 and beta2 subunit expression in the rat brain, and to study the relationship between the alterations of GABAAR subunits in the developing brain and seizure-induced brain injury.</p><p><b>METHODS</b>Sixty-four 7-day-old Sprague-Dawley rats were randomly divided into two groups: control and seizure. Seizures were induced by inhalant flurothyl daily for six consecutive days. The expression of GABAAR alpha1 and beta2 subunits protein in the cerebral cortex and the hippocampus were detected by Western blot and immunohistochemistry method 1 and 7 days after recurrent seizures.</p><p><b>RESULTS</b>Compared to the control, the accumulated optical density (AOD) of GABAAR alpha1 subunit immunoreactivity (IR) in the parietal cortex, the CA3-CA4 regions and the dentate gyrus in seizure rats increased significantly 1 day after recurrent seizures (P<0.05). The AOD of GABAAR alpha1 subunit IR in the parietal cortex, the CA1-CA4 regions and the dentate gyrus in seizure rats increased significantly 7 days after recurrent seizures compared with the control (P<0.05). The expression of GABAAR alpha1 subunit in the hippicampus and the cerebral cortex increased significantly in seizure rats compared with that in control rats 1 and 7 days after recurrent seizures. After 7 days of recurrent seizures, the AOD of GABAAR beta2 subunit IR in the CA1-CA2 regions increased significantly in the seizure group compared with that in the control group (P<0.05), but the AOD of GABAAR beta2 subunit IR in the thalamus decreased significantly in the seizure group compared with that in the control group (P<0.05). The expression of GABAAR beta2 subunit protein in the hippocampus increased significantly in the seizure group compared with that in the control group 7 days after recurrent seizures (P<0.05).</p><p><b>CONCLUSIONS</b>Recurrent neonatal seizures may result in the short-term alterations of GABAAR alpha1 and beta2 subunits expression in the cerebral cortex and the hippocampus in rats, suggesting the alterations of GABAAR subunit expression may be related to the developing brain injury following recurrent seizures.</p>
Subject(s)
Animals , Rats , Animals, Newborn , Blotting, Western , Brain Chemistry , Immunohistochemistry , Rats, Sprague-Dawley , Receptors, GABA-A , Recurrence , Seizures , MetabolismABSTRACT
<p><b>OBJECTIVE</b>This study investigated the effects of flurothyl-induced neonatal recurrent seizures on gamma-aminobutyric acid B1 receptor (GABAB1R) expression in neonatal and adult rat brain, and explored the possible relationship between the alterations of GABAB1R in mature brain and the changes of spatial memory and seizure susceptibility in adult rats.</p><p><b>METHODS</b>Forty-eight postnatal day (P) 7 Sprague-Dawley rats were randomly assigned into two groups: Control and Seizure group (n=24 each). Seizures were induced by inhalant flurothyl daily for six consecutive days in rat pups from the Seizure group. Twelve rats selected randomly in each group were sacrificed on the 7th day after the last seizure for detecting the expressions of GABAB1R mRNA and protein in cerebral cortex and hippocampus by reverse transcription-polymerase chain reaction (RT-PCR) and immuno-histochemistry method. The spatial memory was tested by using the Morris water maze task during P61 to P64 and the seizure threshold was measured at P75 following intraperitoneal injection of pentylenetetrazol ( PTZ ) in the remaining rats. The rats were then sacrificed for detecting the expressions of GABAB1R mRNA and protein in cerebral cortex and hippocampus.</p><p><b>RESULTS</b>The expressions of GABAB1R mRNA and protein in the cerebral cortex on the 7th day after the last seizure and at P75 decreased significantly in the Seizure group when compared with the Control group (P < 0.05). The GABAB1R protein expression in the dentate gyrus on the 7th day after the last seizure in the Seizure group was significantly lower than that in the Control group (P < 0.05), but the GABAB1R mRNA expression in the hippocampus was not different from that in the Control group. There were no significant differences in the expressions of GABAB1R mRNA and protein in the hippocampus between the two groups at P75. The escape latencies in water maze of the rats in the Seizure group at P64 were significantly longer than those in the Control group (98,533.8 +/- 27,205.4 ms vs 46,723.3 +/- 40,666.5 ms; P <0.05). There were no differences in the seizure threshold between the two groups.</p><p><b>CONCLUSIONS</b>The expressions of GABAB1R mRNA and protein in the cerebral cortex and hippocampus of neonatal rats with recurrent seizures decreased significantly, suggesting the changes of GABAB1R may be related to acute brain injury following neonatal recurrent seizures and the memory deficit in adult rats caused by neonatal recurrent seizures.</p>
Subject(s)
Animals , Female , Male , Rats , Animals, Newborn , Brain , Metabolism , Cerebral Cortex , Metabolism , Hippocampus , Metabolism , Maze Learning , RNA, Messenger , Rats, Sprague-Dawley , Receptors, GABA-B , Genetics , Recurrence , Seizures , MetabolismABSTRACT
<p><b>OBJECTIVE</b>The expressions of caspase-1 and cytokines activated by caspase-1 are associated with the pathophysiology of many diseases for its proinflammatory and proapototic peculiarity. However its relationship to brain injury of developing rats following recurrent seizures has not yet been identified. This study aimed to investigate the role of caspase-1 and cytokines activated by caspase-1 in brain injury of developing rats following recurrent seizures.</p><p><b>METHODS</b>A total of 96 postnatal 20 day Sprague-Dawley rats were randomly assigned into Control and Seizure groups. Seizures were induced in the Seizure group by flurothyl inhalation daily for six days. Brain tissues were sampled at 6 hrs, and at 1, 3, and 7 days after last seizure. The expressions of caspase-1, interleukin (IL)-18 and IL-1beta mRNA in the cerebral cortex were detected by RT-PCR. The water content of the brain and the pathological changes of cortex nerve cells were observed. Brain injury was evaluated using a semiquantitative neuropathological scoring system.</p><p><b>RESULTS</b>The levels of caspase-1 and IL-18 mRNA in the cerebral cortex of the Seizure group were obviously higher than those in the Control group at 6 hrs, and at 1, 3, and 7 days after seizure (P < 0.05 or P < 0.01). The expression of IL-1beta mRNA in the Seizure group exhibited a biphasic pattern: increased significantly at 6 hrs, and at 1 and 7 days post-seizure (P < 0.01), but was not significantly different from the Control group at 3 days post-seizure. Edema, degeneration and necrosis of nerve cells in cerebral cortex, accompanying by inflammatory cell infiltration and apoptosis of nerve cells, were observed under a light microscope in the Seizure group after recurrent seizures. The water content of the brain in the Seizure group increased significantly compared with that in the Control group at 6 hrs, and at 1 and 3 days after recurrent seizures (P < 0.01). The Seizure group had significantly higher neuropathological scores than the Control group at each time point (P < 0.01).</p><p><b>CONCLUSIONS</b>Caspase-1 and cytokines activated by caspase-1 play an important role in the developing brain injury after recurrent seizures.</p>