Hashimoto's encephalopathy presenting with isolated cerebellar ataxia in 13 children / 中华儿科杂志

Objective: To analyze the clinical characteristics, treatment and prognosis of Hashimoto's encephalopathy presenting with isolated cerebellar ataxia in children. Methods: A retrospective analysis was performed on the clinical features, laboratory tests, neuroelectrophysiological examination, imaging, treatment and outcomes of 13 patients with Hashimoto's encephalopathy presenting with isolated cerebellar ataxia, who were admitted to the Department of Pediatric Neurology of Guangzhou Women and Children's Medical Center from January 2016 to May 2021. Results: Among the 13 cases, 6 were males and 7 were females. The onset age was 2.6 (2.0,3.3) years, 9 children had precursor infection or vaccination before the first course of disease. All the 13 children had gait abnormalities or unsteady sitting, 10 had intentional tremor, 6 had dysarthria, 3 had body tremor, 2 had nystagmus, 3 had fatigue, 3 had hypotonia, 2 had vomiting and 1 had irritability. Thyroglobulin antibody (TgAb) was 500.0 (298.9,587.2) kU/L and thyroid peroxidase antibody (TPOAb) was 621.9 (449.6,869.4) kU/L in 13 cases. Autoantibodies were positive in 9 cases, and cerebrospinal fluid leukocytosis was seen in 4 cases. Regarding electroencephalography result, 4 cases had background slowing and 1 case had occasional sharp waves. Among the 3 patients who had relapses, 1 had cerebellar atrophy shown on cranial magnetic resonance imaging (MRI) during the recurrence. All the patients received intravenous immunoglobulin (IVIG) and intensive methylprednisolone therapy during the first onset, followed by the disappearance of the symptoms, 1 patient had repeated episodes which was decreased after immunosuppressive treatment with Rituximab.Followed up for 25.0 (22.5,33.3) months after the last episode, 12 achieved complete remission and 1 had a wide base gait. Conclusions: Trunk ataxia is the common symptom of Hashimoto's encephalopathy presenting with isolated cerebellar ataxia in children.Children with cerebellar ataxia should be tested for TgAb and TPOAb to detect Hashimoto's encephalopathy, avoiding missed diagnosis and treatment delays; IVIG and intensive steroid therapy is effective, and immunosuppressive therapy for patients with multiple relapses could reduce the recurrence.

Effects of TIEG1 on K562 cell apoptosis and expression of BCL-2/BAX, PTEN / 中国实验血液学杂志

The aim of this study was to investigate the effect of TIEG1 on K562 cell apoptosis and expression of BCL-2/BAX, PTEN. The different concentration(0, 1, 5, 10, 20 ng/ml) of TIEG1 were used to treat K562 cells, the cell growth inhibition rate was detected by using MTT method. After treating K562 cells with 10.00 ng/ml TIEG1, the cell apoptosis was detected with flow cytometry. The RT-PCR was used to detected the expression levels of BCL-2 /BAX and PTEN. The results showed that TIEG1 displays inhibitory effect on proliferation of K562 cells in time-and dose-dependent manner (r = 0.52, P < 0.05) ; after K562 cells were treated for 6, 12, 24 and 48 h, the IC50 of TIEG1 were 48.19, 18.72, 9.5 and 3.85 ng/ml respectively. After treating K562 cells with 10.00 ng/ml TIEG1 for 0, 6, 12, 24, 48 h, the apoptosis rate were (2.13 ± 0.42)%, (7.79 ± 0.71)%, (11.17 ± 1.37)%, (24.66 ± 0.29)% and (48.60 ± 1.38)% respectively, and there was significant difference between groups(P < 0.05). In process of K562 cell apoptosis, the expression level of BCL-2 gradually decreased (r = 0.48, P < 0.05), meanwhile the expression levels of BAX (r = 0.69, P < 0.05) and PTEN (r = 0.57, P < 0.05) gradually increased. It is concluded that TIEG1 can indue apoptosis of K562 cells and inhibit K562 cell proliferation in time-and dose-dependent manner. In apoptosis process of K562 cells induced by TIEG1, the expression changes of BCL-2/BAX and PTEN associate with the K562 cell apoptosis.

Effect of willed movement therapy on GFAP and SYP expression in rats with cerebral ischemia-reperfusion / 南方医科大学学报

<p><b>OBJECTIVE</b>To determine the effect of willed movement on the expression of glial fibrillary acidic protein (GFAP) and synaptophysin (SYP) in adult rats with cerebral ischemia-reperfusion, and explore the mechanism of willed movement in promoting nerve repair and regeneration.</p><p><b>METHODS</b>Adult rat models of cerebral ischemia-reperfusion injury were established by middle cerebral artery occlusion (MCAO) for 2 h followed by a 24-h reperfusion. The models were then divided randomly into 3 groups, namely the model group, environmental modification (EM) group, and willed movement (WM) group. In each group, neurological deficits were evaluated at 3, 7 and 15 days after reperfusion. Immunohistochemistry and immunofluorescence assay were employed to examine the expression of GFAP and SYP in the brain tissue near the ischemic foci.</p><p><b>RESULTS</b>The rats in WM group showed lessened neurological deficits at 15 days and lowered expression of GFAP and SYP at 7 and 15 days after reperfusion compared with the model and EM groups (P<0.05). No significant difference was found in the expression of GFAP or SYP between the model group and EM group at any time points.</p><p><b>CONCLUSION</b>Willed movement can promote the functional recovery of neurological deficits following cerebral ischemia-reperfusion probably in relation to enhanced GFAP and SYP expressions in the ischemic brain tissues.</p>

Experimental study on the possibility of brain damage induced by chronic treatment with phenobarbital, clonazepam, valproic acid and topiramate in immature rats / 中华儿科杂志

<p><b>OBJECTIVE</b>To explore the possibility of brain damage induced by several anti-epileptic drugs (AEDs) at therapeutic level to immature brain of rat.</p><p><b>METHODS</b>Totally 160 healthy Spraque-Dawley (SD) rats selected for the study were divided into infant and adult groups. Each age group was treated with phenobarbital (PB), clonazepam (CZP), valproic acid (VPA), topiramate (TPM) or normal saline respectively for 2 or 5 weeks with 8 rats in each group. The steady-state plasma concentrations of AEDs at the experimental dosage were coincided with the range of clinical therapeutic concentrations. Drug levels in plasma were determined by fluorescence polarization. Body and brain weights were measured when the rats were sacrificed. Histological studies on the tissues of frontal lobes and hippocampus were performed by Nissl staining. And ultrastructural changes of brain were observed by the transmission electron microscopy. Plasma neuron-specific enolase (NSE) was determined by ELISA. Expression of apoptosis-related proteins Bcl-2 and Bax in neurons was detected by immunohistochemistry. Neuronal apoptosis was detected by terminal deoxynucleotidyl transferase biotin-dUTP nick end labeling (TUNEL).</p><p><b>RESULTS</b>(1) There were no significant differences in brain weight among all adults groups. While remarkable reduction of brain weight was observed in immature rats exposed to CZP or PB (P < 0.01) for long term. (2) Significant neurodegeneration, neuronal necrosis and decrease in the number of neurons can be observed in the immature rats exposed to CZP or PB for long period. (3) For immature rats, concentration of plasma NSE was increased even after short-term treatment with PB [(8.84 +/- 2.10) nmol/L] compared with control group [(6.27 +/- 1.27) nmol/L] (P < 0.01). And it was increased in immature rats exposed to CZP [(8.15 +/- 1.67) nmol/L] or PB [(8.07 +/- 1.27) nmol/L] for long term compared with controls [(6.02 +/- 1.20) nmol/L] (P < 0.01). But there were no significant differences between AEDs-treated adult rats and control rats. (4) The expression of Bcl-2 and Bax protein in mature brain did not change at therapeutic level. In contrast, expression of Bax protein in the frontal lobe was increased significantly in immature rats receiving CZP and PB for long period compared with control. (5) The number of TUNEL positive cells in immature rats exposed to CZP or PB for long term was obviously increased.</p><p><b>CONCLUSIONS</b>PB and CZP may result in remarkable histological abnormalities, neuronal apoptosis and necrosis in immature brain. The brain damage induced by PB was more serious and persistent than that induced by CZP.</p>

Association study between COL3A1 gene polymorphisms and ischemic stroke / 中华神经科杂志

Objective To investigate the characters of the COL3A1 gene polymorphisms in Chinese population of Hunan region and the relationship between the COL3A1 gene polymorphisms and ischemie stroke.Methods Objects examined were composed of 70 healthy controls,110 patients with acute cerebral infarction.The frequencies of the genotypes were detected by using PCR-SSLP techniques and correlated PCR segements were analyzed by directly sequence to detect the COL3A1 gene polymorphisms.Result There were significant differences in the distribution of VNTR with COL3A1 genotype polymorphism between the patients of acute cerebral infarction and healthy controls,the former being 0.93,the latter 0.43,with a significant difference(P

Influence of antiepileptic drugs at therapeutic level to immature brain / 中华神经科杂志

Objective To explore the different influence of antiepileptic drugs(AEDs)at therapeutic levels to the maturation of brain.Methods 180 healthy Sprague-Dawley(SD)rats were divided into infant and adult group.Each age group was administered with PB,CNP,VPA,TPM or normal saline respectively in persistent 5 weeks.The steady-state plasma concentrations of AEDs at the experimental dosage were coincided with the range of clinical therapeutic concentrations.After AEDs withdrawed,the effects of AEDs on cognitive function were assessed by Morris water maze and two-way shuttle box at different time points.Body and brain weight were got immediately when the rats were sacrificed.Histological changes of brain were observed by HE staining,Nissl staining and transmission electron microscopy.Results(1) For immature rats,1 day or 14 days after AEDs withdrawed,there were significant differences between groups exposed to PB or CNP and control group in escape response latency(ERL)in the two-way shuttle box.Even after one month ERLs of immature rats receiving CNP((6.05?2.04)s)or PB((5.81? 1.75)s)were still longer than that of untreated controls((4.75?2.43)s,P