A standard procedure for continuous intraoperative monitoring of the integrity of the corticospinal tracts by eliciting muscle responses is the electric stimulation mapping (ESM). However, standard ESM protocols are ineffective in 20% of young children. We have developed a novel, highly efficient paradigm consisting of short-time burst (30 ms) of high frequency (500 Hz) and high peak current (<=100 mA), which may cause local tissue overheating. The presented safety control study was therefore designed. The infrared thermography camera captured to-be-resected cortex of 13 patients in vivo during ESM. Thermograms were image processed to reveal discrete ESM thermal effect of currents from 10 to 100 mA. Peak 100 mA currents induced a maximal increase in temperature of 3.1 degreeC, 1.23+/-0.72 degreeC in average. The warming correlated with stimulating electrode resistance ( ). The measurement uncertainty was estimated +/- 1.01 C for the most skeptical conditions. The histopathological evaluation of stimulated tissue (performed in all cases) did not show any destructive changes. Our study demonstrates the ability of the thermographic camera to measure the discrete thermal effect of the ESM. The results provide evidence for the safety of the proposed protocol for full range currents with minimal risk of brain tissue damage.
- MeSH
- bezpečnost pacientů MeSH
- dítě MeSH
- elektrická stimulace * MeSH
- infračervené záření MeSH
- kalibrace MeSH
- lidé MeSH
- mapování mozku * metody MeSH
- mladiství MeSH
- peroperační monitorování * metody škodlivé účinky MeSH
- předškolní dítě MeSH
- pyramidové dráhy MeSH
- ruka MeSH
- teplota MeSH
- termografie * metody škodlivé účinky MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- mladiství MeSH
- mužské pohlaví MeSH
- předškolní dítě MeSH
- ženské pohlaví MeSH
INTRODUCTION: Medulloblastoma is the most frequent malignant brain tumour in children. Radiation-induced cavernous haemangiomas (RICHs) are a known late complication of radiation exposure, especially in young children. CASE REPORT: We present a patient who underwent subtotal resection of posterior fossa medulloblastoma with subsequent chemotherapy and radiotherapy at the age of 10 years. A new lesion in the region of the left foramen of Monro appeared 16 years later. Based on the imaging results, metastasis or radiation-induced cavernoma was considered. The lesion had the same appearance on imaging as a rarely published intraventricular cavernoma of the foramen of Monro. Unlike the cavernoma of the foramen of Monro, this lesion was subependymal and intraforniceal. Using electromagnetic navigation and neuroendoscopy, the lesion was completely removed. Histopathological examination revealed a cavernous haemangioma. CONCLUSION: This is a unique case of intraforniceal paraforaminal cavernoma that was successfully removed endoscopically using electromagnetic neuronavigation and without neurological sequelae.
- MeSH
- dítě MeSH
- dospělí MeSH
- elektromagnetické jevy MeSH
- kavernózní hemangiom etiologie chirurgie MeSH
- lidé MeSH
- meduloblastom radioterapie MeSH
- nádory mozečku radioterapie MeSH
- nádory vyvolané zářením chirurgie MeSH
- neuroendoskopie metody MeSH
- neuronavigace metody MeSH
- radioterapie škodlivé účinky MeSH
- Check Tag
- dítě MeSH
- dospělí MeSH
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
INTRODUCTION: Olfactory groove schwannomas (OGSs) are extremely rare tumours, particularly in the paediatric population. CASE REPORT: A 13-year-old girl presented with two epileptic seizures, papilloedema and incomplete binasal quadrantanopia. Computed tomography (CT) and magnetic resonance imaging (MRI) demonstrated a large heterogeneously enhancing tumour of the anterior skull base with a prominent dorsal pseudocyst. Interestingly, the pseudocyst embraced the right ICA bifurcation and displaced the optic tracts, optic chiasm and optic nerves and the ipsilateral basal ganglia. The patient underwent surgery via the frontolateral approach, and the tumour was completely removed. The pseudocyst was opened, and its wall was partially resected. It subsequently resolved completely. Histopathological examination yielded the rare diagnosis of schwannoma of the anterior skull base. CONCLUSION: Although extremely rare, olfactory groove schwannomas can be seen in paediatric patients. Our patient is the youngest ever reported with this histopathological diagnosis along with the formation of a large pseudocyst.
- MeSH
- capsula interna patologie MeSH
- lidé MeSH
- mladiství MeSH
- nádory baze lební patologie chirurgie MeSH
- neurilemom patologie chirurgie MeSH
- tractus opticus patologie MeSH
- Check Tag
- lidé MeSH
- mladiství MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
In tuberous sclerosis complex (TSC), overexpression of numerous genes associated with inflammation has been observed. Among different proinflammatory cytokines, interleukin-1β (IL-1β) has been shown to be significantly involved in epileptogenesis and maintenance of seizures. Recent evidence indicates that IL-1β gene expression can be regulated by DNA methylation of its promoter. In the present study, we hypothesized that hypomethylation in the promoter region of the IL-1β gene may underlie its overexpression observed in TSC brain tissue. Bisulfite sequencing was used to study the methylation status of the promoter region of the IL-1β gene in TSC and control samples. We identified hypomethylation in the promoter region of the IL-1β gene in TSC samples. IL-1β is overexpressed in tubers, and gene expression is correlated with promoter hypomethylation at CpG and non-CpG sites. Our results provide the first evidence of epigenetic modulation of the IL-1β signaling in TSC. Thus, strategies that target epigenetic alterations could offer new therapeutic avenues to control the persistent activation of interleukin-1β-mediated inflammatory signaling in TSC brain.
- MeSH
- CpG ostrůvky MeSH
- dítě MeSH
- epigeneze genetická MeSH
- interleukin-1beta genetika metabolismus MeSH
- lidé MeSH
- metylace DNA * MeSH
- mladiství MeSH
- mozek metabolismus MeSH
- promotorové oblasti (genetika) * MeSH
- studie případů a kontrol MeSH
- tuberózní skleróza genetika metabolismus MeSH
- upregulace MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- mladiství MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
Tuberous sclerosis complex (TSC) is a genetic disease presenting with multiple neurological symptoms including epilepsy, mental retardation, and autism. Abnormal activation of various inflammatory pathways has been observed in astrocytes in brain lesions associated with TSC. Increasing evidence supports the involvement of microRNAs in the regulation of astrocyte-mediated inflammatory response. To study the role of inflammation-related microRNAs in TSC, we employed real-time PCR and in situ hybridization to characterize the expression of miR21, miR146a, and miR155 in TSC lesions (cortical tubers and subependymal giant cell astrocytomas, SEGAs). We observed an increased expression of miR21, miR146a, and miR155 in TSC tubers compared with control and perituberal brain tissue. Expression was localized in dysmorphic neurons, giant cells, and reactive astrocytes and positively correlated with IL-1β expression. In addition, cultured human astrocytes and SEGA-derived cell cultures were used to study the regulation of the expression of these miRNAs in response to the proinflammatory cytokine IL-1β and to evaluate the effects of overexpression or knockdown of miR21, miR146a, and miR155 on inflammatory signaling. IL-1β stimulation of cultured glial cells strongly induced intracellular miR21, miR146a, and miR155 expression, as well as miR146a extracellular release. IL-1β signaling was differentially modulated by overexpression of miR155 or miR146a, which resulted in pro- or anti-inflammatory effects, respectively. This study provides supportive evidence that inflammation-related microRNAs play a role in TSC. In particular, miR146a and miR155 appear to be key players in the regulation of astrocyte-mediated inflammatory response, with miR146a as most interesting anti-inflammatory therapeutic candidate.
- MeSH
- astrocytom metabolismus patologie MeSH
- astrocyty metabolismus MeSH
- buněčné kultury MeSH
- dítě MeSH
- dospělí MeSH
- kojenec MeSH
- kultivované buňky MeSH
- lidé středního věku MeSH
- lidé MeSH
- mikro RNA metabolismus MeSH
- mladiství MeSH
- mladý dospělý MeSH
- mozek metabolismus MeSH
- neurony metabolismus MeSH
- předškolní dítě MeSH
- signální transdukce fyziologie MeSH
- tuberózní skleróza metabolismus MeSH
- Check Tag
- dítě MeSH
- dospělí MeSH
- kojenec MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- předškolní dítě MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
Mitochondrial morphology was studied in cultivated myoblasts obtained from patients with mitochondrial disorders, including CPEO, MELAS and TMEM70 deficiency. Mitochondrial networks and ultrastructure were visualized by fluorescence microscopy and transmission electron microscopy, respectively. A heterogeneous picture of abnormally sized and shaped mitochondria with fragmentation, shortening, and aberrant cristae, lower density of mitochondria and an increased number of "megamitochondria" were found in patient myoblasts. Morphometric Fiji analyses revealed different mitochondrial network properties in myoblasts from patients and controls. The small number of cultivated myoblasts required for semiautomatic morphometric image analysis makes this tool useful for estimating mitochondrial disturbances in patients with mitochondrial disorders.
- MeSH
- dítě MeSH
- fluorescenční mikroskopie MeSH
- kojenec MeSH
- lidé MeSH
- mitochondriální nemoci patologie MeSH
- mitochondrie ultrastruktura MeSH
- myoblasty ultrastruktura MeSH
- transmisní elektronová mikroskopie MeSH
- Check Tag
- dítě MeSH
- kojenec MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
- Publikační typ
- abstrakt z konference MeSH
- Publikační typ
- abstrakt z konference MeSH
