Short term outcomes of topiramate monotherapy as a first-line treatment in newly diagnosed West syndrome

PurposeTo investigate the efficacy of topiramate monotherapy in West syndrome prospectively.MethodsThe study population included 28 patients (15 male and 13 female children aged 2 to 18 months) diagnosed with West syndrome. After a 2-week baseline period for documentation of the frequency of spasms, topiramate was initiated at 2 mg/kg/day. The dose was increased by 2 mg/kg every week to a maximum of 12 mg/kg/day. Clinical assessment was based on the parents' report and a neurological examination every 2 weeks for the first 2 months of treatment. The baseline electroencephalograms (EEGs) were compared with the post-treatment EEGs at 2 weeks and 1 month.ResultsWest syndrome was considered to be cryptogenic in 7 of the 28 patients and symptomatic in 21 patients. After treatment, 11 patients (39%) became spasm-free, 6 (21%) had more than 50% spasmsreduction, 3 (11%) showed less than 50% reduction, and 8 (29%) did not respond. The effective daily dose for achieving more than 50% reduction in spasm frequency, including becoming spasm-free, was found to be 5.8±1.1 mg/kg/day. Nine patients (32%) showed complete disappearance of spasms and hypsarrhythmia, and 11 (39%) showed improved EEG results. Despite adverse events (4 instances of irritability, 3 of drowsiness, and 1 of decreased feeding), no patients discontinued the medication.ConclusionTopiramate monotherapy seems to be effective and well tolerated as a first line therapy for West syndrome and is not associated with serious adverse effects

Overexpression of the miR-141/200c cluster promotes the migratory and invasive ability of triple-negative breast cancer cells through the activation of the FAK and PI3K/AKT signaling pathways by secreting VEGF-A

Migration in miR-141/200c-transduced HCC-38 and Hs578T cells treated with an anti-VEGF-A-neutralizing antibody. (A, D) Migration in miR-141/200c-transduced HCC-38 and Hs578T cells. Images of the crystal violet-stained cells that migrated horizontally in the trans-well migration assay (upper). The absorbance values of extracted crystal violet in migrated cells (lower). The migratory abilities of the miR-200c cells (~1.6-fold and ~1.7-fold, HCC-38 and Hs578T, respectively) were significantly increased compared with those of the control cells. (B, E) Measurement of the secreted levels of cytokines and growth factors (IL-2, IL-4, IL-5, IL-10, IL-12, IL-13, GM-CSF, IFN-γ, TNF-α, and VEGF-A). Transduction of miR-141/200c into HCC-38 and Hs578T cells promoted significantly higher VEGF-A secretion than that of control cells. (C, F) Trans-well migration of anti-VEGF-A-neutralizing antibody-treated cells. The enhanced migration of the miR-141/200c-transduced HCC-38 cells were significantly suppressed by treatment with anti-VEGF-A-neutralizing antibodies, but miR-141/200c-transduced Hs578T cells still showed increased migratory ability compared with control cells. *p < 0.05, **p < 0.001. (JPG 188 kb

Psychotic Features as the First Manifestation of 22q11.2 Deletion Syndrome

The 22q11.2 deletion is a genetic disorder which is characterized by abnormalities in cardiac functioning, facial structure, neurobehavioral development, T cell functioning, and velopharyngeal insufficiencies. In the presented case study, 22q11.2 deletion was found in a patient who has psychotic symptoms only. A 25-year-old woman with a history of hypoparathyroidism and hypothyroidism presented with auditory hallucinations and persecutory delusions. After three months of treatment with antipsychotic medications, the patient was readmitted with generalized tonic-clonic seizures. The following week, the patient went into sepsis. A fluorescent in situ hybridization (FISH) analysis revealed the presence of a 22q11.2 microdeletion. This case study suggests that psychotic symptoms can develop prior to the typical symptoms of a 22q11.2 deletion. As such, psychiatrists should test for genetic abnormalities in patients with schizophrenia when these patients present with seizures and immunodeficiencies

Delta neutrophil index as a predictor of vesicoureteral reflux in children with febrile urinary tract infection

Purpose Delta neutrophil index (DNI) indicates immature granulocytes in peripheral blood and has been confirmed to be effective as a prognostic factor for neonatal sepsis. Also, it has been reported to have diagnostic value in acute pyelonephritis and in predicting vesicoureteral reflux (VUR) in the infant. We conducted the study to verify whether DNI is also helpful in the entire pediatric age group with febrile urinary tract infection (UTI). Methods Medical records of children hospitalized for febrile UTIs were analyzed retrospectively. All subjects underwent kidney ultrasound and voiding cystourethrography. In the group with and without VUR, we compared sex and age, and the following laboratory values: the white blood cell count, neutrophil, polymorphonuclear leucocyte, eosinophil, hemoglobin, platelet count, C-reactive protein, DNI value, and the finding of ultrasound. Results A total of 315 patients (163 males and 152 females; range, 0–127 months) were eligible, and 41 patients (13%) had VUR. As a result of univariate analysis, the white blood cell count, neutrophil, DNI, and ultrasonic abnormalities were high in the reflux group, and the hemoglobin and lymphocyte fraction values were low. The value of DNI and the abnormal ultrasound were significantly higher in the reflux group on the multivariate analysis. The area under the curve value of the receiver operating curve was higher in DNI (0.640; 95% confidence interval, 0.536–0.744; P=0.004), and the DNI cutoff value for VUR prediction was 1.85%. Conclusions We identified that ultrasound findings and DNI values were helpful predictors of VUR in pediatric febrile UTIs