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Background: Schwannomas arise from nerve sheaths of cranial, peripheral, and spinal nerve or nerve roots. Most intracranial schwannomas arise from the cranial nerves, predominantly the vestibulocochlear nerve. In addition to cranial nerve schwannomas, intraparenchymal schwannomas of the brain and intramedullary schwannomas of the spinal cord are extremely rare. Case Report: In this case report, we describe the imaging findings of three diverse cases of schwannoma at different locations and unique presentations with acute neurological symptoms in the pediatric age group. Conclusion: Schwannomas should be considered and included in the differential diagnosis of intracranial or intraspinal intramedullary space-occupying lesions in pediatric patients.

Congenital disorders of glycosylation (CDGs) are a large group of genetic diseases with impaired glycosylation of glycoproteins and glycolipids, and glycosylphosphatidylinositol anchor synthesis. Steroid 5α-reductase 3 (SRD5A3)-CDG is a CDG type I with a clinical spectrum of neurological, ophthalmological, dermatological and hepatic symptoms. Although CDGs are not directly related to malignancies, it is well known that some genes that are involved in glycosylation pathways are involved in various cancers. Aberrant glycosylation has been closely linked to the development and progression of brain cancer. We report a patient with SRD5A3-CDG carrying a novel homozygous splice variant and brain neoplasm. Also, a review of the literature is made regarding the multisystem effects of the disease. Key Words: SRD5A3-CDG, Glioma, Glycosylation, Transferrin isoelectric focusing, Congenital disorders of glycosylation. ispartof: J Coll Physicians Surg Pak vol:32 issue:12 pages:SS221-SS226 ispartof: location:Pakistan status: published

Optic pathway gliomas are the most common central nervous system neoplasms in patients with neurofibromatosis type 1. Perineural arachnoidal gliomatosis is a rare and distinctive growth pattern of optic nerve glioma, in which the tumor infiltrates through the pia mater and pre-dominantly involves the subarachnoid space around the optic nerve. Here, we report an 8-year-old girl with perineural arachnoidal gliomatosis associated with neurofibromatosis type 1.

When we measure countries’ socioeconomic development, the standalone National Income approach does not to reflect well-being of the citizens. An analysis of a country's welfare should consider the combination of different aspects such as economic indicators, environmental and resource productivity, and economic opportunities. In the light of these discussions, the concept of green growth arose as a contemporary approach to reframe the mainstream growth model and to re-evaluate many of the investment decisions in meeting agricultural, energy and water needs and the resource demands of economic development. This paper evaluates the green growth performance of OECD countries by applying the I-distance method by integrating the economic, social, and environmental aspects of countries’ performances simultaneously. Using multivariate I-distance approach, selected green growth indicators and numerous different variables have been synthesized into one value. According to our analysis, Sweden, Luxemburg, Norway and Denmark occupy the top places by their outstanding green performance. On the other hand, Mexico, Poland and Greece are placed at the bottom as a consequence of their relatively weak environmental performance.

INTRODUCTION[|]The aim of this study was to assess initial changes in blood flow parameters of the ophthalmic artery (OA) in pediatric patients with type 1 diabetes mellitus (DM).[¤]METHODS[|]Sixty-three subjects were included in this prospective, cross-sectional, observational study. Thirty-one (49.2%) patients with type 1 DM without diabetic retinopathy formed the DM group. The control group comprised 32 (50.8%) healthy subjects. The OA of all of the patients was examined with Doppler ultrasonography. The main outcomes were peak systolic velocity (PSV), end diastolic velocity (EDV), pulsatility index (PI), and resistivity index (RI) measurements.[¤]RESULTS[|]The mean age at onset of type 1 DM was 10.7+-2.0 years and the mean duration was 11.4+-11.0 months. The mean PSV and EDV outcomes in both eyes were significantly higher in the control group than in the DM group, whereas, the mean PI and RI outcomes in both eyes were significantly higher in the DM group (p

Objective: Multisystem inflammatory syndrome in children (MIS-C) is seen as a serious delayed complication of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. The aim of this study was to describe the most common imaging features of MIS-C associated with SARS-CoV-2. Methods: A retrospective review was made of the medical records and radiological imaging studies of 47 children (26 male, 21 female) in the age range of 25 months–15 years who were diagnosed with MIS-C between August 2020 and March 2021. Chest radiographs were available for all 47 patients, thorax ultrasound for 6, chest CT for 4, abdominal ultrasound for 42, abdomen CT for 9, neck ultrasound for 4, neck CT for 2, brain CT for 1, and brain MRI for 3. Results: The most common finding on chest radiographs was perihilar–peribronchial thickening (46%). The most common findings on abdominal ultrasonography were mesenteric inflammation (42%), and hepatosplenomegaly (38%, 28%). Lymphadenopathy was determined in four patients who underwent neck ultrasound, one of whom had deep neck infection on CT. One patient had restricted diffusion and T2 hyperintensity involving the corpus callosum splenium on brain MRI, and one patient had epididymitis related with MIS-C. Conclusion: Pulmonary manifestations are uncommon in MIS-C. In the abdominal imaging, mesenteric inflammation, hepatosplenomegaly, periportal edema, ascites and bowel wall thickening are the most common findings. Advances in knowledge: The imaging findings of MIS-C are non-specific and can mimic many other pathologies. Radiologists should be aware that these findings may indicate the correct diagnosis of MIS-C.

Background/aim: Mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) is a rare clinicoradiological syndrome that typically presents with central nervous system symptoms such as loss of consciousness, seizure, headache, and ophthalmoparesis. Materials and methods: Here, we highlight the characteristics of this syndrome together with the clinical and MRI findings of 6 pediatric patients with MERS. Results: Between January 2017 and October 2020, 6 patients with MERS (3 boys and 3 girls) presented to our center. The mean age was 122 +/- 54.6 (min-max: 44-180) months. None of the patients had a chronic disease. In our study, infectious agents were detected in 4 patients (66.6%), while noninfectious causes (one seizure and the other hyponatremia) were detected in two patients. All of our cases were discharged without any sequelae alter an average of 12.1 +/- 7 (min-max: 4-20) days of hospitalization. In 1 patient (case 6), control MRI could not be performed, and the radiological recovery of our other patients was shown to be between 14 days and 2 months. Conclusion: MERS is an acute encephalopathy with good prognosis and should be considered by neurologists in differential diagnosis due to its variable clinical presentation and specific MRI findings.

Tuberous sclerosis complex (TSC) patients may have different specific neuropsychological deficits related to the location of the tubers. Autism spectrum disorders (ASD) are common in TSC patients but the relationship between these diagnoses has not been formally explored. In this study we sought to examine brain Magnetic Resonance Imaging (MRI) findings in TSC patients with ASD.We evaluated 34 TSC patients on the basis of DSM-V diagnostic criteria for ASD, Wechsler Intelligence Scale for Children (WISC-R), psychiatrist's examination and also structured parent interviews. The number and localization of the tubers, postcontrast signal characteristics of the tubers, SWI findings, DWI findings on brain MRI were recorded. Demographic features, epilepsy histories, number of antiseizure medications, cognitive status were eveluated also. Patients were divided into two groups: ASD group, which represented group 1 and group 2 consisting of patients without any ASD symptoms.In our study, the mean number of tuber count was 21.8 in patients with ASD patients (Group 1, n = 13) and 12.4 in other TSC patients without ASD (Group 2, n = 21). Rate of tubers in prefrontal cortex/whole tubers (0.51) in patients with ASD was determined to be higher in group 1 (p = 0.003). Also a significant difference was detected between generalize epileptiform activities on EEG and the rate of DRE (p = 0.002; p = 0.001) between groups. Cognitive disturbances and infantile spasm history were similar between groups. TSC2 mutations have been identified in 29 (86%) patients.The mean of total tuber count and the rate of the location in the prefrontal cortex were determined to be higher in TSC patients with ASD. Specific areas on brain MRI may help understanding the development of ASD in TSC patients.

The homozygous M694V mutation in the MEFV gene may cause an augmented response to the streptococcal infection that plays a role in the development of APSGN and PAN. Both clinical manifestations may occur simultaneously after streptococcal infection in a child who is previously healthy but carries a MEFV mutation.