Degenerative effects of nerve tissues are often accompanied by changes in vascularization. In this regard, knowledge about hereditary cerebellar degeneration is limited. In this study, we compared the vascularity of the individual cerebellar components of 3-month-old wild-type mice (n = 8) and Purkinje cell degeneration (pcd) mutant mice, which represent a model of hereditary cerebellar degeneration (n = 8). Systematic random samples of tissue sections were processed, and laminin was immunostained to visualize microvessels. A computer-assisted stereology system was used to quantify microvessel parameters including total number, total length, and associated densities in cerebellar layers. Our results in pcd mice revealed a 45% (p < 0.01) reduction in the total volume of the cerebellum, a 28% (p < 0.05) reduction in the total number of vessels and a lower total length, approaching 50% (p < 0.001), compared to the control mice. In pcd mutants, cerebellar degeneration is accompanied by significant reduction in the microvascular network that is proportional to the cerebellar volume reduction therefore does not change density of in the cerebellar gray matter of pcd mice.
- MeSH
- mikrocévy MeSH
- mozeček * MeSH
- myši - mutanty neurologické MeSH
- myši inbrední C57BL MeSH
- myši MeSH
- Purkyňovy buňky * fyziologie MeSH
- zvířata MeSH
- Check Tag
- myši MeSH
- zvířata MeSH
- Publikační typ
- časopisecké články MeSH
BACKGROUND: The majority of fetal structural defects can be detected in the second trimester, thus this is the main time for screening for structural defects. 3D imaging of the fetal brain does not create a common part of this screening. METHODS: This prospective observational study was conducted at the Fetal Medicine Center of The Gynecological-Obstetrical Department of the University Hospital Olomouc in years 2017-2020. The study sample was 451 consecutively scanned morphologically normal fetuses attending for routine second trimester anatomical survey at 20-22 weeks of pregnancy. A transabdominal 3D ultrasound volume acquisition of fetal brain was obtained from an axial and sagittal plane using skull sutures as an acoustic window. RESULTS: Both the corpus callosum (CC) and the vermis (VC) were detected in 51.7% of examinations in the sagittal plane, and in 31.7% in the axial plane. In 61.9% of the examinations, there was at least partial detection in both planes. Maternal BMI was found to be the only significant predictor of the quality of imaging in both planes. CONCLUSION: 3D acquisition of fetal brain images in the sagittal plane followed by manipulation of acquired volume was valuable in assessment of corpus callosum and cerebellar vermis. This allows reconstruction of the sagittal plane that can be difficult to obtain in 2D imaging.
- MeSH
- corpus callosum diagnostické zobrazování MeSH
- druhý trimestr těhotenství MeSH
- gestační stáří MeSH
- lidé MeSH
- těhotenství MeSH
- ultrasonografie prenatální metody MeSH
- vermis cerebelli * MeSH
- zobrazování trojrozměrné metody MeSH
- Check Tag
- lidé MeSH
- těhotenství MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- pozorovací studie MeSH
OBJECTIVE: The cerebellum is involved in cognitive processing and emotion control. Cerebellar alterations could explain symptoms of schizophrenia spectrum disorder (SZ) and bipolar disorder (BD). In addition, literature suggests that lithium might influence cerebellar anatomy. Our aim was to study cerebellar anatomy in SZ and BD, and investigate the effect of lithium. METHODS: Participants from 7 centers worldwide underwent a 3T MRI. We included 182 patients with SZ, 144 patients with BD, and 322 controls. We automatically segmented the cerebellum using the CERES pipeline. All outputs were visually inspected. RESULTS: Patients with SZ showed a smaller global cerebellar gray matter volume compared to controls, with most of the changes located to the cognitive part of the cerebellum (Crus II and lobule VIIb). This decrease was present in the subgroup of patients with recent-onset SZ. We did not find any alterations in the cerebellum in patients with BD. However, patients medicated with lithium had a larger size of the anterior cerebellum, compared to patients not treated with lithium. CONCLUSION: Our multicenter study supports a distinct pattern of cerebellar alterations in SZ and BD.
- MeSH
- antimanika škodlivé účinky MeSH
- bipolární porucha diagnostické zobrazování farmakoterapie patologie MeSH
- dospělí MeSH
- kůra mozečku diagnostické zobrazování účinky léků patologie MeSH
- lidé středního věku MeSH
- lidé MeSH
- magnetická rezonanční tomografie MeSH
- mladý dospělý MeSH
- schizofrenie diagnostické zobrazování farmakoterapie patologie MeSH
- sloučeniny lithia škodlivé účinky MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladý dospělý MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- multicentrická studie MeSH
- práce podpořená grantem MeSH
- Research Support, N.I.H., Extramural MeSH
Cíl studie: Přehled aktuálních poznatků o využití 3D ultrazvukového vyšetření při vyšetření CNS plodu. Typ studie: Přehledový článek. Název a sídlo pracoviště: Gynekologicko-porodnická klinika LF UP a FN Olomouc. Materiál a metodika: Využity byly literární zdroje týkající se tématu, zejména články indexované v Pubmed- Medline. Závěr: 3D ultrazvuk je v současné době používán k vyšetření struktur mozku, které se obtížně nastavují při konvenčním 2D ultrazvukovém vyšetření. Nejvhodnější technikou pro zobrazení středových struktur mozku, respektive corpus callosum a vermis cerebella, se jeví být nasnímání 3D (trojrozměrných) objemových dat v sagitální rovině přes šípový šev či velkou fontanelu s následným post-processingem v multiplanárním módu, pomocí OVIX (Samsung) či TUI (Ge Healthcare) apod.
Objective: An overview of current knowledge about the use of 3D ultrasound examinations for the examination of fetal CNS. Design: A review article. Setting: Department of Gynecology and Obstetrics, Faculty of Medicine and Dentistry, Palacký University and Faculty Hospital Olomouc. Methods: Literary sources related to the subject were used, especially articles indexed by Pubmed-Medline. Conclusion: 3D ultrasound is currently used for examination of fetal CNS structures that can be only very difficult displayed by conventional 2D ultrasound. The best for technique for visualisation of midline fetal CNS structures, respectively corpus callosum cerebellar vermis, appears to be 3D volume acquisition in a sagittal plane through the sagittal suture or large fontanel with further post-processing in multiplanar mode, OVIX (Samsung), TUI (GE Healthcare) etc.
- Klíčová slova
- neurosonogram,
- MeSH
- centrální nervový systém * diagnostické zobrazování MeSH
- corpus callosum MeSH
- druhý trimestr těhotenství MeSH
- lidé MeSH
- prenatální diagnóza metody MeSH
- těhotenství MeSH
- ultrasonografie prenatální * metody MeSH
- vermis cerebelli MeSH
- zobrazování trojrozměrné metody MeSH
- Check Tag
- lidé MeSH
- těhotenství MeSH
- ženské pohlaví MeSH
- Publikační typ
- přehledy MeSH
Mozeček je malá, avšak na neurony nesmírně bohatá součást mozku. Kromě koordinace pohybů a motorického učení se mu v poslední době přisuzuje role v dalších a dalších funkcích. Ani jeho detailní struktura na úrovni charakteristiky jednotlivých typů neuronů a jejich synaptického zapojení není dodnes zcela poznána. Ukazuje se, že jednotlivé typy neuronů, tak jak je známe z učebnic, jsou ve skuteč-nosti velmi heterogenními populacemi s různorodou morfologickou, receptorovou, mediátorovou a enzymatickou výbavou. Struktura mozečku a základní funkční okruhy mají velmi komplikované, avšak v hrubých rysech stereotypně se opakující modulární uspořádání. To zjevně souvisí s principy zpracování signálů a generování odpovědí v mozečku. Množství buněčných typů, přechodných typů a podtypů, jejichž přesnou charakteristiku zatím neznáme, komplikuje chápání funkcí mozečku na detailnější úrovni. Nové poznatky boří vžité před-stavy a mnoho potenciálních vysvětlení zůstává na úrovni teoretických modelů. Cílem tohoto článku je podat alespoň základní přehled o funkční morfologii mozečku.
The cerebellum is a small structure of the brain extremely rich in neurons. In addition to movement coordination and motor learning, the cerebellum is recently related to many other functions including cognitive, sensory and emotional processes and control of behaviour. Its detailed structure on the level of characteristics of individual neuronal types and their synaptic interconnections is not completely known. It seems that individual types of neurons as know from textbooks are in fact very heterogeneous populations of cells with variable recep-tor, transmitter, and enzymatic equipment. The structure of the cerebellum and its basal functional circuitries have a very complicated but in rough features stereotypic modular organization. It is probably related to principles of signal processing and response generation in the cerebellum. Number of cell types, intermediate types and subtypes detailed characteristic features of which remain unknown make deeper understanding of cerebellar functions difficult. New findings break down traditional ideas and many potential explanations remain at the level of theoretical concepts. The aim of this article is to provide at least basal overview of the functional morphology of the cerebellum.
- MeSH
- aferentní nervové dráhy fyziologie ultrastruktura MeSH
- eferentní nervové dráhy fyziologie ultrastruktura MeSH
- kůra mozečku anatomie a histologie fyziologie MeSH
- lidé MeSH
- mozeček * anatomie a histologie růst a vývoj ultrastruktura MeSH
- mozečková jádra anatomie a histologie fyziologie MeSH
- nemoci mozečku MeSH
- neurony aferentní fyziologie MeSH
- neurony eferentní fyziologie MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- přehledy MeSH
Posttranslational modifications of tubulin are emerging regulators of microtubule functions. We have shown earlier that upregulated polyglutamylation is linked to rapid degeneration of Purkinje cells in mice with a mutation in the deglutamylating enzyme CCP1. How polyglutamylation leads to degeneration, whether it affects multiple neuron types, or which physiological processes it regulates in healthy neurons has remained unknown. Here, we demonstrate that excessive polyglutamylation induces neurodegeneration in a cell-autonomous manner and can occur in many parts of the central nervous system. Degeneration of selected neurons in CCP1-deficient mice can be fully rescued by simultaneous knockout of the counteracting polyglutamylase TTLL1. Excessive polyglutamylation reduces the efficiency of neuronal transport in cultured hippocampal neurons, suggesting that impaired cargo transport plays an important role in the observed degenerative phenotypes. We thus establish polyglutamylation as a cell-autonomous mechanism for neurodegeneration that might be therapeutically accessible through manipulation of the enzymes that control this posttranslational modification.
- MeSH
- aktivní transport genetika MeSH
- myši knockoutované MeSH
- myši MeSH
- neurodegenerativní nemoci genetika metabolismus patologie MeSH
- peptidsynthasy genetika metabolismus MeSH
- peptidy genetika metabolismus MeSH
- posttranslační úpravy proteinů * MeSH
- proteiny nervové tkáně genetika metabolismus MeSH
- Purkyňovy buňky metabolismus patologie MeSH
- tubulin genetika metabolismus MeSH
- zvířata MeSH
- Check Tag
- myši MeSH
- zvířata MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
OBJECTIVES: To determine which 3-D techniques are most effective for "real time" prenatal ultrasound assessment of the corpus callosum and cerebellar vermis. METHODS: A prospective study involving 100 consecutive normal singleton pregnancies attending routine anomaly scan at 19-23 weeks' gestation. Midsagittal structures of the fetal brain were assessed using six different methods of 3-D image acquisition and three post-processing techniques. The quality of the resulting images were then assessed and scored by a second operator. The significance of the difference between various techniques was assessed using the Friedman test. RESULTS: The best method for visualization of both corpus callosum and cerebellar vermis in one image involved 3-D acquisition in a sagittal plane through the sagittal suture with manipulated 3-D OVIX™ (Oblique View eXtended Imaging) reconstruction. Five other methods scored closely and were not significantly different; all were based on 3-D acquisition in the sagittal plane through either sagittal suture or anterior fontanel. CONCLUSIONS: We have applied described techniques for 3-D central nervous system volume acquisition and have shown that the best method of assessment, allowing assessment of both the corpus callosum and the cerebellar vermis, involves midsagittal acquisition through the sagittal suture with 3-D OVIX reconstruction. This technique can be applied successfully in 83% of cases.
- MeSH
- corpus callosum diagnostické zobrazování MeSH
- lidé MeSH
- prospektivní studie MeSH
- těhotenství MeSH
- ultrasonografie prenatální metody MeSH
- velikost orgánu MeSH
- vermis cerebelli diagnostické zobrazování MeSH
- zobrazování trojrozměrné metody MeSH
- Check Tag
- lidé MeSH
- těhotenství MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- klinické zkoušky MeSH
Knowledge on the clinical, autoimmune, genetic and more recently microbiome and epigenetic interactions in celiac disease (CD) is consistently improving; however, the pathogenic mechanisms of neurological manifestations of gluten sensitivity (NMGS) and their potential relationship with CD remain unclear. Difficulties in assessing both conditions include their highly variable clinical manifestations and the insufficient sensitivity and specificities of currently available diagnostics tools. Patients with neurological manifestations that respond to gluten withdrawal may or may not present enteropathy and others having demonstrable mucosal damage may or may not respond to GFD. Current pathogenic hypotheses that may relate both conditions, the spectrum of clinical manifestations, diagnostic problems, including differences in types and subtypes of antibodies described for diagnosis and the effects of gluten-free diet are reviewed. The evidence show that decisions based on clinical data may be successful for patient management, but do not allow drawing conclusions on the relations between CD and NMGS.
- MeSH
- ataxie etiologie komplikace MeSH
- celiakie * komplikace patofyziologie MeSH
- epilepsie etiologie komplikace MeSH
- gluteny škodlivé účinky MeSH
- lidé MeSH
- migréna etiologie komplikace MeSH
- nemoci centrálního nervového systému * etiologie komplikace MeSH
- nemoci periferního nervového systému etiologie komplikace MeSH
- Purkyňovy buňky patologie MeSH
- receptory N-methyl-D-aspartátu MeSH
- transglutaminasy škodlivé účinky MeSH
- Check Tag
- lidé MeSH
- MeSH
- lidé MeSH
- mozeček * patofyziologie MeSH
- pití alkoholu * škodlivé účinky MeSH
- Purkyňovy buňky fyziologie MeSH
- Check Tag
- lidé MeSH