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Aim We aim to describe the demographic characteristics, etiology, neurophysiology, imaging findings, treatment, prognosis, and prognostic factors of acute flaccid myelitis. Methods The clinical data, laboratory test and, magnetic resonance imaging (MRI) results of pediatric patients diagnosed with acute flaccid myelitis according to the Centers for Disease Control criteria between August 1, 2016, and December 31, 2018, from 13 centers in Turkey were reviewed. Results Of the 34 cases identified, 31 were confirmed (91.2%). Eighteen patients (55.9%) were boys. The median patient age was 4 years (interquartile range 2.5–6.9 years). Most of the patients were admitted in 2018 (n = 27). A preceding history of a febrile illness was reported in all patients, with a median of 4 days (interquartile range 3–7 days) before symptom onset. Thirty-one patients had T2 hyperintensity on spinal MRI, and 18 patients had cerebrospinal fluid pleocytosis. The most common infectious agents were entero/rhinoviruses (n = 5) in respiratory specimens. All patients except one received immunotherapy either alone or in combination. Among 27 patients with follow-up data 24 had persistent weakness. Involvement of four limbs together with an abnormal brain MRI at onset were associated with a poor prognosis. Conclusion The number of patients with acute flaccid myelitis increased since 2012, spiking with every 2-year interval, largely in the pediatric population. The median age decreases with every outbreak. Clinicians should be aware of the clinical picture for early collection of specimens and early start of rehabilitation programs. Further studies are needed to better characterize the etiology, pathogenesis, risk factors, and treatment of this rare condition.

Molybdenum cofactor deficiency is a rare inborn error of metabolism. The major clinical symptoms are intractable neonatal seizures, progressive encephalopathy, facial dysmorphic features and feeding difficulties. Most of the patients are misdiagnosed as hypoxic ischemic encephalopathy. The majority of patients have mutations in the MOCS1 and MOCS2 genes. Although the therapeutic treatment strategies have not been improved, genetic analysis is essential to elucidate the disease. Here, we report a review of 12 patients with Molybdenum cofactor deficiency reported from Turkey. (C) 2012 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

Objective Subacute sclerosing panencephalitis is caused by persistent brain infection of mutated virus, showing inflammation, neurodegeneration, and demyelination. Although many factors are emphasized in the pathogenesis of subacute sclerosing panencephalitis, the exact mechanism of neurodegeneration remains unknown. Micro-RNAs are small, noncoding RNAs that regulate gene expression at the posttranscriptional levels. Micro-RNAs are essential for normal immune system development; besides they are also implicated in the pathogenesis of many chronic inflammatory disorders. The aim of this study is to investigate the expression patterns of micro-RNAs 146a, 181a, and 155 in peripheral blood mononuclear cells of patients with subacute sclerosing panencephalitis. Methods We enrolled 39 patients with subacute sclerosing panencephalitis and 41 healthy controls. Quantitative analysis of micro-RNAs 146a, 181a, and 155 were performed using specific stem-loop primers followed by real-time polymerase chain reaction. Results All of 3 micro-RNAs were upregulated in subacute sclerosing panencephalitis patients. In addition, the level of micro-RNA 155 expression was higher in stage 3 patients. But, micro-RNA 146a and 181a expression levels showed no association or correlation with clinically relevant data. Conclusion Alteration of peripheral blood mononuclear cell micro-RNAs in subacute sclerosing panencephalitis may shed new light on the pathogenesis of disease and may contribute to the aberrant systemic rise in mRNA levels in subacute sclerosing panencephalitis.

Objective Status epilepticus triggers a mixture of apoptotic and necrotic cell death within the developing brain which results in the development of epilepsy and cognitive deficits. Simvastatin has been shown to have neuroprotective effects in various models of neurological damage. Recent studies suggest that simvastatin presents the best characteristics for neuroprotection in neurodegenerative conditions due to its high blood-brain barrier penetration capacity and cholesterol-lowering effect on neurons. However, its therapeutic effect on pediatric status epilepticus is still unknown. The aim of this study is to investigate the effect of simvastatin on hippocampus and prefrontal cortex of rat after pentylenetetrazole induced status epilepticus. Methods Twenty-one dam reared Wistar male rats, 21-day-old were divided into three groups: control group, pentylenetetrazole induced status epilepticus, simvastatin treated group. Simvastatin treated group received simvastatin 10 mg/kg intraperitoneally 40 minutes after pentylenetetrazole injection for 5 days. Rats were sacrificed and brain tissues were collected at 5th day of experiment. Neuronal cell death and apoptosis were evaluated. Results Histopathological examination showed that simvastatin significantly decreased neuronal cell death in CA1 and dentate gyrus regions of hippocampus. It also diminished apoptosis in the hippocampus and prefrontal cortex. Simvastatin significantly decreased the number of caspase 3 positive cells and the number of terminal deoxynucleotidyl transferase-mediated dUTP nick end-labeling positive neurons in the hippocampus and prefrontal cortex when compared to pentylenetetrazole induced status epilepticus group (p=0.002 and p=0.002). Conclusion This experimental study suggests that simvastatin administration may be neuroprotective in status epilepticus in the developing brain.