The Experts below are selected from a list of 234 Experts worldwide ranked by ideXlab platform
F Ebinger - One of the best experts on this subject based on the ideXlab platform.
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t2 hyperintense Cerebellar Cortex in marinesco sjogren syndrome
Neurology, 2004Co-Authors: Inga Harting, A Blaschek, Nicole I Wolf, Angelika Seitz, M Haupt, H H Goebel, D Rating, K Sartor, F EbingerAbstract:Hyperintensity of the Cerebellar Cortex on T2-weighted images is a rare finding and has been considered pathognomonic for infantile neuroaxonal dystrophy (INAD).1 However, we present three children with Marinesco–Sjogren syndrome (MSS; OMIM 248800) and hyperintense Cerebellar Cortex on MRI. Additional findings were widened Cerebellar fissures, an enlarged fourth ventricle, reduced N -acetylaspartate, and elevated myo-inositol on 1H-MR spectroscopy (MRS) of the cerebellum. ### Patients. Patient 1 is the 5-year-old son of consanguineous parents; Patients 2 (girl, age 7 years) and 3 (boy, age 14 months) are siblings of nonconsanguineous parents. Hypotonia, developmental delay, strabismus, and short stature were present in all patients. Ataxia was present in Patients 1 and 2. Tendon reflexes were normal, and pyramidal tract signs were absent. Bilateral cataracts were diagnosed in Patient 1 (age 4.5 years) and Patient 2 (age 6.8 years). At age 14 months, cataracts had not yet developed in Patient 3. Serum creatine (Cr) kinase levels were normal in Patient 1 and slightly elevated in Patients 2 and 3. Extensive …
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T2-hyperintense Cerebellar Cortex in Marinesco–Sjögren syndrome
Neurology, 2004Co-Authors: Inga Harting, A Blaschek, Nicole I Wolf, Angelika Seitz, M Haupt, H H Goebel, D Rating, K Sartor, F EbingerAbstract:Hyperintensity of the Cerebellar Cortex on T2-weighted images is a rare finding and has been considered pathognomonic for infantile neuroaxonal dystrophy (INAD).1 However, we present three children with Marinesco–Sjogren syndrome (MSS; OMIM 248800) and hyperintense Cerebellar Cortex on MRI. Additional findings were widened Cerebellar fissures, an enlarged fourth ventricle, reduced N -acetylaspartate, and elevated myo-inositol on 1H-MR spectroscopy (MRS) of the cerebellum. ### Patients. Patient 1 is the 5-year-old son of consanguineous parents; Patients 2 (girl, age 7 years) and 3 (boy, age 14 months) are siblings of nonconsanguineous parents. Hypotonia, developmental delay, strabismus, and short stature were present in all patients. Ataxia was present in Patients 1 and 2. Tendon reflexes were normal, and pyramidal tract signs were absent. Bilateral cataracts were diagnosed in Patient 1 (age 4.5 years) and Patient 2 (age 6.8 years). At age 14 months, cataracts had not yet developed in Patient 3. Serum creatine (Cr) kinase levels were normal in Patient 1 and slightly elevated in Patients 2 and 3. Extensive …
Inga Harting - One of the best experts on this subject based on the ideXlab platform.
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t2 hyperintense Cerebellar Cortex in marinesco sjogren syndrome
Neurology, 2004Co-Authors: Inga Harting, A Blaschek, Nicole I Wolf, Angelika Seitz, M Haupt, H H Goebel, D Rating, K Sartor, F EbingerAbstract:Hyperintensity of the Cerebellar Cortex on T2-weighted images is a rare finding and has been considered pathognomonic for infantile neuroaxonal dystrophy (INAD).1 However, we present three children with Marinesco–Sjogren syndrome (MSS; OMIM 248800) and hyperintense Cerebellar Cortex on MRI. Additional findings were widened Cerebellar fissures, an enlarged fourth ventricle, reduced N -acetylaspartate, and elevated myo-inositol on 1H-MR spectroscopy (MRS) of the cerebellum. ### Patients. Patient 1 is the 5-year-old son of consanguineous parents; Patients 2 (girl, age 7 years) and 3 (boy, age 14 months) are siblings of nonconsanguineous parents. Hypotonia, developmental delay, strabismus, and short stature were present in all patients. Ataxia was present in Patients 1 and 2. Tendon reflexes were normal, and pyramidal tract signs were absent. Bilateral cataracts were diagnosed in Patient 1 (age 4.5 years) and Patient 2 (age 6.8 years). At age 14 months, cataracts had not yet developed in Patient 3. Serum creatine (Cr) kinase levels were normal in Patient 1 and slightly elevated in Patients 2 and 3. Extensive …
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T2-hyperintense Cerebellar Cortex in Marinesco–Sjögren syndrome
Neurology, 2004Co-Authors: Inga Harting, A Blaschek, Nicole I Wolf, Angelika Seitz, M Haupt, H H Goebel, D Rating, K Sartor, F EbingerAbstract:Hyperintensity of the Cerebellar Cortex on T2-weighted images is a rare finding and has been considered pathognomonic for infantile neuroaxonal dystrophy (INAD).1 However, we present three children with Marinesco–Sjogren syndrome (MSS; OMIM 248800) and hyperintense Cerebellar Cortex on MRI. Additional findings were widened Cerebellar fissures, an enlarged fourth ventricle, reduced N -acetylaspartate, and elevated myo-inositol on 1H-MR spectroscopy (MRS) of the cerebellum. ### Patients. Patient 1 is the 5-year-old son of consanguineous parents; Patients 2 (girl, age 7 years) and 3 (boy, age 14 months) are siblings of nonconsanguineous parents. Hypotonia, developmental delay, strabismus, and short stature were present in all patients. Ataxia was present in Patients 1 and 2. Tendon reflexes were normal, and pyramidal tract signs were absent. Bilateral cataracts were diagnosed in Patient 1 (age 4.5 years) and Patient 2 (age 6.8 years). At age 14 months, cataracts had not yet developed in Patient 3. Serum creatine (Cr) kinase levels were normal in Patient 1 and slightly elevated in Patients 2 and 3. Extensive …
Richard Apps - One of the best experts on this subject based on the ideXlab platform.
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Redefining the Cerebellar Cortex as an assembly of non-uniform Purkinje cell microcircuits
Nature Reviews Neuroscience, 2015Co-Authors: Nadia L. Cerminara, Eric J. Lang, Roy V. Sillitoe, Richard AppsAbstract:A widely held assumption is that the same neural computation is performed throughout a uniform circuitry in the adult mammalian Cerebellar Cortex, and differences in function can be explained primarily by distinct patterns of input and output connectivity. Anatomical, genetic and physiological evidence suggests, however, that the Cerebellar Cortex is not uniform. Regional differences include variations in cell type, morphology and expression of various molecular markers, most notably zebrin II expression by Purkinje cells. Purkinje cells are considered to be key players within the Cerebellar Cortex because they provide the sole signal output from the Cortex to the Cerebellar nuclei. Differences related to zebrin II expression include variations in intrinsic and synaptic physiology and patterns of activity of simple spikes and complex spikes. Mouse mutant models also show that Purkinje cell death occurs in restricted patterns that are related to both motor and potentially non-motor dysfunction. Variations in gene expression and related anatomical and physiological differences therefore result in an assembly of non-uniform Cerebellar cortical microcircuits that have different information processing capabilities. The adult mammalian Cerebellar Cortex is generally assumed to have a uniform cytoarchitecture. Differences in Cerebellar function are thought to arise primarily through distinct patterns of input and output connectivity rather than as a result of variations in cortical microcircuitry. However, evidence from anatomical, physiological and genetic studies is increasingly challenging this orthodoxy, and there are now various lines of evidence indicating that the Cerebellar Cortex is not uniform. Here, we develop the hypothesis that regional differences in properties of Cerebellar cortical microcircuits lead to important differences in information processing. The Cerebellar Cortex drives smooth goal-directed movement as well as a range of other functions. Apps and colleagues describe studies that have revealed variations in the cytoarchitecture, molecular composition, physiological properties and vulnerability to cell death of different Cerebellar cortical regions, and discuss the idea that these underlie different forms of information processing.
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redefining the Cerebellar Cortex as an assembly of non uniform purkinje cell microcircuits
Nature Reviews Neuroscience, 2015Co-Authors: Nadia L. Cerminara, Eric J. Lang, Roy V. Sillitoe, Richard AppsAbstract:The adult mammalian Cerebellar Cortex is generally assumed to have a uniform cytoarchitecture. Differences in Cerebellar function are thought to arise primarily through distinct patterns of input and output connectivity rather than as a result of variations in cortical microcircuitry. However, evidence from anatomical, physiological and genetic studies is increasingly challenging this orthodoxy, and there are now various lines of evidence indicating that the Cerebellar Cortex is not uniform. Here, we develop the hypothesis that regional differences in properties of Cerebellar cortical microcircuits lead to important differences in information processing.
Richard Hawkes - One of the best experts on this subject based on the ideXlab platform.
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Development of the neuronal circuitry of the Cerebellar Cortex
Neural Circuit and Cognitive Development, 2020Co-Authors: Constantino Sotelo, Fabrice Ango, Richard HawkesAbstract:Abstract Since the pioneering studies of Cajal, the cerebellum had been considered as composed of a small number of neuronal populations, forming a uniform, interconnected circuit all along its foliated cortical structure. This vision has changed completely during the last 40 years. Here we will review the data that transformed our view of the Cerebellar Cortex from homogeneous to highly compartmentalized. The discovery of the striped organization of the Cerebellar Cortex outputs (the Purkinje cell axons) and inputs (the climbing and mossy fibers), together with the heterogeneity of the Purkinje cells and interneurons, has revealed the complex topography of the Cortex. The general consensus is that the diversity of Purkinje cell phenotypes is the common organizer of this modular organization (the “matching” hypothesis). However, the modular organization does not mean a multitude of maps: rather it seems that all maps are congruent (the “one map” hypothesis).
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Antigenic compartmentation of the cat Cerebellar Cortex.
Brain research, 2003Co-Authors: Roy V. Sillitoe, Manuel Hulliger, Richard H. Dyck, Richard HawkesAbstract:Abstract Despite the apparent uniformity in cellular composition of the mammalian Cerebellar Cortex, a complex topography is revealed by several expression patterns. Zebrin II, a polypeptide antigen identified as aldolase C, is one such marker which, in several species of mammals, is restricted to a subset of Purkinje cells that are clustered together to form a symmetrical and reproducible array of zones and stripes. In rodents the Cerebellar Cortex is divided into four transverse zones—anterior, central, posterior, and nodular. Each transverse zone is further subdivided mediolaterally into an array of parasagittal stripes. The similar zone and stripe organization partitions the hemispheres. Based upon a novel whole mount immunohistochemical staining procedure, we have now identified homologous zones and stripes in the feline cerebellum. In the cat cerebellum the somata of most Purkinje cells express zebrin II but parasagittal stripes may still be delineated owing to the alternating high and low zebrin II expression levels in the dendritic arbors. As in rodents, the cat cerebellum consists of four transverse zones with each zone subdivided into a unique combination of zebrin II parasagittal stripes, suggesting that a common architecture underlies the organization of the mammalian cerebellum.
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Antigenic compartmentation of the Cerebellar Cortex in the syrian hamster Mesocricetus auratus.
Brain research, 2003Co-Authors: Hassan Marzban, Sepehr Zahedi, Miguel Sanchez, Richard HawkesAbstract:Despite the apparent uniformity in cellular composition of the adult Cerebellar Cortex, a complex heterogeneous pattern can be revealed by using biochemical markers. One example is zebrin II/aldolase C, which is expressed by a subset of Purkinje cells that form a highly reproducible array of stripes. Zebrin II/aldolase C immunohistochemistry has been used in both section and whole mount preparations to analyze the architecture of the hamster Cerebellar Cortex. As in other species studied, zebrin II immunoreactivity in the hamster cerebellum is restricted to a subset of Purkinje cells and, more weakly, to astrocytes. Based on the distribution of these Purkinje cell subsets the hamster Cerebellar vermis was found to consist of four transverse zones-the anterior zone, central zone, posterior zone and nodular zone. Zebrin II/aldolase C is expressed uniformly in the central and nodular zones, and as parasagittal stripes in the anterior and central zones. A similar alternation of homogeneous and striped expression domains is seen in the hemispheres. The topography of the hamster Cerebellar Cortex as revealed by zebrin II expression domains closely resembles that reported in other mammals. Thus, a Cerebellar zone-and-stripe topography appears to be conserved across the Mammalia.
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Pattern formation in the Cerebellar Cortex.
Biochemistry and Cell Biology, 2000Co-Authors: Carol L. Armstrong, Richard HawkesAbstract:The Cerebellar Cortex is subdivided rostrocaudally and mediolaterally into a reproducible array of zones and stripes. This makes the cerebellum a valuable model for studying pattern formation in the vertebrate central nervous system. The structure of the adult mouse Cerebellar Cortex and the series of embryological events that generate the topography are reviewed.
William D. Hopkins - One of the best experts on this subject based on the ideXlab platform.
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Comparative neuronal morphology of the Cerebellar Cortex in afrotherians, carnivores, cetartiodactyls, and primates.
Frontiers in Neuroanatomy, 2014Co-Authors: Bob Jacobs, Nicholas L Johnson, Devin Wahl, Matthew Schall, Camilla Butti, Mary Ann Raghanti, Busisiwe C Maseko, Albert H. Lewandowski, Bridget Wicinski, William D. HopkinsAbstract:Although the basic morphological characteristics of neurons in the Cerebellar Cortex have been documented in several species, virtually nothing is known about the quantitative morphological characteristics of these neurons across different taxa. To that end, the present study investigated Cerebellar neuronal morphology among eight different, large-brained mammalian species comprising a broad phylogenetic range: afrotherians (African elephant, Florida manatee), carnivores (Siberian tiger, clouded leopard), cetartiodactyls (humpback whale, giraffe) and primates (human, common chimpanzee). Specifically, several neuron types (e.g., stellate, basket, Lugaro, Golgi, and granule neurons; N = 317) of the Cerebellar Cortex were stained with a modified rapid Golgi technique and quantified on a computer-assisted microscopy system. There was a 64-fold variation in brain mass across species in our sample (from clouded leopard to the elephant) and a 103-fold variation in Cerebellar volume. Most dendritic measures tended to increase with Cerebellar volume. The Cerebellar Cortex in these species exhibited the trilaminate pattern common to all mammals. Morphologically, neuron types in the Cerebellar Cortex were generally consistent with those described in primates (Fox et al., 1967) and rodents (Palay and Chan-Palay, 1974), although there was substantial quantitative variation across species. In particular, Lugaro neurons in the elephant appeared to be disproportionately larger than those in other species. To explore potential quantitative differences in dendritic measures across species, MARSplines analyses were used to evaluate whether species could be differentiated from each other based on dendritic characteristics alone. Results of these analyses indicated that there were significant differences among all species in dendritic measures.