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Cerebellum

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https://www.readbyqxmd.com/read/29149443/migration-of-interneuron-precursors-in-the-nascent-cerebellar-cortex
#1
Annika K Wefers, Christian Haberlandt, Lachezar Surchev, Christian Steinhäuser, Ronald Jabs, Karl Schilling
The cerebellum arguably constitutes one of the best characterized central nervous circuits, and its structure, cellular function, and histogenesis have been described in exceptional quantitative detail. A notable exception to this is the development of its inhibitory interneurons, and in particular the extensive migrations of future basket and stellate cells. Here, we used acute slices from 8-day-old mice to assess the migration of Pax2-EGFP-tagged precursors of these cells en route to the molecular layer during their transit through the nascent cerebellar cortex...
November 17, 2017: Cerebellum
https://www.readbyqxmd.com/read/29143300/effect-of-restraining-the-base-of-support-on-the-other-biomechanical-features-in-patients-with-cerebellar-ataxia
#2
C Conte, Mariano Serrao, L Cuius, A Ranavolo, S Conforto, F Pierelli, L Padua
This study aimed to analyze the biomechanical consequences of reducing the base of support in patients with ataxia. Specifically, we evaluated the spatio-temporal parameters, upper- and lower-body kinematics, muscle co-activation, and energy recovery and expenditure. The gaits of 13 patients were recorded using a motion analysis system in unperturbed and perturbed walking conditions. In the latter condition, patients had to walk using the same step width and speed of healthy controls. The perturbed walking condition featured reduced gait speed, step length, hip and knee range of motion, and energy recovery and increased double support duration, gait variability, trunk oscillation, and ankle joint muscle co-activation...
November 15, 2017: Cerebellum
https://www.readbyqxmd.com/read/29134361/sonic-hedgehog-agonist-protects-against-complex-neonatal-cerebellar-injury
#3
Vien Nguyen, Khalida Sabeur, Emin Maltepe, Kurosh Ameri, Omer Bayraktar, David H Rowitch
The cerebellum undergoes rapid growth during the third trimester and is vulnerable to injury and deficient growth in infants born prematurely. Factors associated with preterm cerebellar hypoplasia include chronic lung disease and postnatal glucocorticoid administration. We modeled chronic hypoxemia and glucocorticoid administration in neonatal mice to study whole cerebellar and cell type-specific effects of dual exposure. Chronic neonatal hypoxia resulted in permanent cerebellar hypoplasia. This was compounded by administration of prednisolone as shown by greater volume loss and Purkinje cell death...
November 13, 2017: Cerebellum
https://www.readbyqxmd.com/read/29134360/protein-kinase-c-in-the-cerebellum-its-significance-and-remaining-conundrums
#4
REVIEW
Hirokazu Hirai
Protein kinase C (PKC), a family of serine/threonine protein kinases, mediates a myriad of patho-physiological cellular events in various tissues. The originally discovered PKC (conventional) requires the binding of diacylglycerol and Ca(2+) for full activation. The conventional PKC consists of four isoforms, PKCα, PKCβI/βII, and PKCγ. PKCα and PKCβI/βII are expressed in the cells of various tissues including the brain, while PKCγ is present specifically in neurons of the brain and spinal cord. The cerebellum expresses the largest amount of PKC with all its four isoforms...
November 13, 2017: Cerebellum
https://www.readbyqxmd.com/read/29086357/personality-and-neuropsychological-profiles-in-friedreich-ataxia
#5
Sabrina Sayah, Jean-Yves Rotgé, Hélène Francisque, Marcela Gargiulo, Virginie Czernecki, Damian Justo, Khadija Lahlou-Laforet, Valérie Hahn, Massimo Pandolfo, Antoine Pelissolo, Philippe Fossati, Alexandra Durr
Friedreich ataxia, an autosomal recessive mitochondrial disease, is the most frequent inherited ataxia. Many studies have attempted to identify cognitive and affective changes associated with the disease, but conflicting results have been obtained, depending on the tests used and because many of the samples studied were very small. We investigated personality and neuropsychological characteristics in a cohort of 47 patients with genetically confirmed disease. The neuropsychological battery assessed multiple cognition domains: processing speed, attention, working memory, executive functions, verbal memory, vocabulary, visual reasoning, emotional recognition, and social cognition...
October 30, 2017: Cerebellum
https://www.readbyqxmd.com/read/29071518/cerebellum-as-a-master-piece-for-linguistic-predictability
#6
EDITORIAL
Peter Mariën, Mario Manto
No abstract text is available yet for this article.
October 25, 2017: Cerebellum
https://www.readbyqxmd.com/read/29063351/cerebellar-asymmetry-and-cortical-connectivity-in-monozygotic-twins-with-discordant-handedness
#7
R E Rosch, P E Cowell, J M Gurd
Handedness differentiates patterns of neural asymmetry and interhemispheric connectivity in cortical systems that underpin manual and language functions. Contemporary models of cerebellar function incorporate complex motor behaviour and higher-order cognition, expanding upon earlier, traditional associations between the cerebellum and motor control. Structural MRI defined cerebellar volume asymmetries and correlations with corpus callosum (CC) size were compared in 19 pairs of adult female monozygotic twins strongly discordant for handedness (MZHd)...
October 23, 2017: Cerebellum
https://www.readbyqxmd.com/read/29043563/malformation-of-the-posterior-cerebellar-vermis-is-a-common-neuroanatomical-phenotype-of-genetically-engineered-mice-on-the-c57bl-6-background
#8
Joshua A Cuoco, Anthony W Esposito, Shannon Moriarty, Ying Tang, Sonika Seth, Alyssa R Toia, Elias B Kampton, Yevgeniy Mayr, Mussarah Khan, Mohammad B Khan, Brian R Mullen, James B Ackman, Faez Siddiqi, John H Wolfe, Olga V Savinova, Raddy L Ramos
C57BL/6 mice exhibit spontaneous cerebellar malformations consisting of heterotopic neurons and glia in the molecular layer of the posterior vermis, indicative of neuronal migration defect during cerebellar development. Recognizing that many genetically engineered (GE) mouse lines are produced from C57BL/6 ES cells or backcrossed to this strain, we performed histological analyses and found that cerebellar heterotopia were a common feature present in the majority of GE lines on this background. Furthermore, we identify GE mouse lines that will be valuable in the study of cerebellar malformations including diverse driver, reporter, and optogenetic lines...
October 17, 2017: Cerebellum
https://www.readbyqxmd.com/read/29039117/in-vivo-dentate-nucleus-gamma-aminobutyric-acid-concentration-in-essential-tremor-vs-controls
#9
Elan D Louis, Nora Hernandez, Jonathan P Dyke, Ruoyun E Ma, Ulrike Dydak
Despite its high prevalence, essential tremor (ET) is among the most poorly understood neurological diseases. The presence and extent of Purkinje cell (PC) loss in ET is the subject of controversy. PCs are a major storehouse of central nervous system gamma-aminobutyric acid (GABA), releasing GABA at the level of the dentate nucleus. It is therefore conceivable that cerebellar dentate nucleus GABA concentration could be an in vivo marker of PC number. We used in vivo (1)H magnetic resonance spectroscopy (MRS) to quantify GABA concentrations in two cerebellar volumes of interest, left and right, which included the dentate nucleus, comparing 45 ET cases to 35 age-matched controls...
October 16, 2017: Cerebellum
https://www.readbyqxmd.com/read/28965328/the-ferdinando-rossi-memorial-lecture-zones-and-stripes-pattern-formation-in-the-cerebellum
#10
REVIEW
Richard Hawkes
The cerebellum has a complex architecture-highly reproducible and conserved through evolution. Cerebellar architecture is organized around the Purkinje cell. Purkinje cells in the mouse cerebellum come in many different subtypes, identifiable by expression markers, sensitivity to mutation, etc. These are organized first into five "transverse zones," each of which is further subdivided into dozens of reproducible "stripes." This arrangement serves as the scaffolding to organize afferent topography and restrict the distribution of excitatory and inhibitory interneurons...
September 30, 2017: Cerebellum
https://www.readbyqxmd.com/read/28965326/presynaptic-mechanisms-mediating-retrograde-semaphorin-signals-for-climbing-fiber-synapse-elimination-during-postnatal-cerebellar-development
#11
REVIEW
Naofumi Uesaka, Masanobu Kano
Elimination of early-formed redundant synapses during postnatal development is essential for functional neural circuit formation. Purkinje cells (PCs) in the neonatal cerebellum are innervated by multiple climbing fibers (CFs). During postnatal development, a single CF is selectively strengthened in each PC and becomes a "winner" CF that is presumed to remain into adulthood, whereas the other "loser" CFs are eliminated. These developmental changes are dependent on neural activity and signal cascades in postsynaptic PCs...
September 30, 2017: Cerebellum
https://www.readbyqxmd.com/read/28940157/climbing-fiber-development-is-impaired-in-postnatal-car8-wdl-mice
#12
Lauren N Miterko, Roy V Sillitoe
The cerebellum is critical for an array of motor functions. During postnatal development, the Purkinje cells (PCs) guide afferent topography to establish the final circuit. Perturbing PC morphogenesis or activity during development can result in climbing fiber (CF) multi-innervation or mis-patterning. Structural defects during circuit formation typically have long-term effects on behavior as they contribute to the phenotype of movement disorders such as cerebellar ataxia. The Car8 (wdl) mouse is one model in which early circuit destruction influences movement...
September 22, 2017: Cerebellum
https://www.readbyqxmd.com/read/28940047/epidemiology-of-cerebellar-diseases-and-therapeutic-approaches
#13
REVIEW
Michael S Salman
Diseases involving the cerebellum occur relatively commonly in children and adults around the globe. Many factors influence their epidemiology including geography, ethnicity, consanguinity, and the methodology used to ascertain patients. In addition, reliable epidemiological data rely heavily on accurate disease classification. Continuous advances in genetic research and neuroimaging modalities have resulted in improved understanding of cerebellar diseases and have led to several revisions in their classification...
September 22, 2017: Cerebellum
https://www.readbyqxmd.com/read/28921485/the-neglected-cerebello-limbic-pathways-and-neuropsychological-features-of-the-cerebellum-in-emotion
#14
LETTER
Paolo Flace, Angelo Quartarone, Giovanni Colangelo, Demetrio Milardi, Alberto Cacciola, Giuseppina Rizzo, Paolo Livrea, Giuseppe Anastasi
No abstract text is available yet for this article.
September 18, 2017: Cerebellum
https://www.readbyqxmd.com/read/28895081/novel-de-novo-kcnd3-mutation-in-a-japanese-patient-with-intellectual-disability-cerebellar-ataxia-myoclonus-and-dystonia
#15
Masanori Kurihara, Hiroyuki Ishiura, Takuya Sasaki, Juuri Otsuka, Toshihiro Hayashi, Yasuo Terao, Takashi Matsukawa, Jun Mitsui, Juntaro Kaneko, Kazutoshi Nishiyama, Koichiro Doi, Jun Yoshimura, Shinichi Morishita, Jun Shimizu, Shoji Tsuji
Spinocerebellar ataxia 19/22 (SCA19/22) is a rare type of autosomal dominant SCA that was previously described in 11 families. We report the case of a 30-year-old Japanese man presenting with intellectual disability, early onset cerebellar ataxia, myoclonus, and dystonia without a family history. MRI showed cerebellar atrophy, and electroencephalograms showed paroxysmal sharp waves during hyperventilation and photic stimulation. Trio whole-exome sequencing analysis of DNA samples from the patient and his parents revealed a de novo novel missense mutation (c...
September 11, 2017: Cerebellum
https://www.readbyqxmd.com/read/28887803/psychosis-in-spinocerebellar-ataxias-a-case-series-and-study-of-tyrosine-hydroxylase-in-substantia-nigra
#16
Katherine W Turk, Margaret E Flanagan, Samuel Josephson, C Dirk Keene, Suman Jayadev, Thomas D Bird
Spinocerebellar ataxias are a genetically heterogeneous group of degenerative diseases typically characterized by progressive ataxia and to various degrees, neuropathy, amyotrophy, and ocular abnormalities. There is increasing evidence for non-motor manifestations associated with cerebellar syndromes including cognitive and psychiatric features. We studied a retrospective clinical case series of eight subjects with spinocerebellar ataxias (SCAs) 2, 3, 7, and 17, all displaying features of psychosis, and also measured tyrosine hydroxylase (TH) staining of the substantia nigra (SN) at autopsy, among four of the subjects...
September 8, 2017: Cerebellum
https://www.readbyqxmd.com/read/28887630/ncb5or-deficiency-in-the-cerebellum-and-midbrain-leads-to-dehydration-and-alterations-in-thirst-response-fasted-feeding-behavior-and-voluntary-exercise-in-mice
#17
Matthew A Stroh, Michelle K Winter, Kenneth E McCarson, John P Thyfault, Hao Zhu
Cytosolic NADH-cytochrome-b5-oxidoreductase (NCB5OR) is ubiquitously expressed in animal tissues. We have previously reported that global ablation of NCB5OR in mice results in early-onset lean diabetes with decreased serum leptin levels and increased metabolic and feeding activities. The conditional deletion of NCB5OR in the mouse cerebellum and midbrain (conditional knock out, CKO mice) results in local iron dyshomeostasis and altered locomotor activity. It has been established that lesion to or removal of the cerebellum leads to changes in nutrient organization, visceral response, feeding behavior, and body weight...
September 8, 2017: Cerebellum
https://www.readbyqxmd.com/read/28875335/facilitation-of-fast-backward-priming-after-left-cerebellar-continuous-theta-burst-stimulation
#18
Louise S T Allen-Walker, R Martyn Bracewell, Guillaume Thierry, Paloma Mari-Beffa
Traditional theories of backward priming account only for the priming effects found at long stimulus onset asynchronies (SOAs). Here, we suggest that the presence of backward priming at short SOAs may be related to the integrative role of the cerebellum. Previous research has shown that the right cerebellum is involved in forward associative priming. Functional magnetic resonance imaging reveals some activation of the left cerebellar hemisphere during backward priming; but what this activation represents is unclear...
September 5, 2017: Cerebellum
https://www.readbyqxmd.com/read/28844105/the-floccular-syndrome-dynamic-changes-in-eye-movements-and-vestibulo-ocular-reflex-in-isolated-infarction-of-the-cerebellar-flocculus
#19
Dario Andres Yacovino, Manuel Perez Akly, Leonel Luis, David S Zee
The cerebellar flocculus is a critical structure involved in the control of eye movements. Both static and dynamic abnormalities of the vestibulo-ocular reflex (VOR) have been described in animals with experimental lesions of the flocculus/paraflocculus complex. In humans, lesions restricted to the flocculus are rare so they can become an exceptional model to contrast with the clinical features in experimental animals or in patients with more generalized cerebellar diseases. Here, we examined a 67-year-old patient with an acute vestibular syndrome due to an isolated infarct of the right flocculus...
August 26, 2017: Cerebellum
https://www.readbyqxmd.com/read/28840476/making-sense-of-cerebellar-contributions-to-perceptual-and-motor-adaptation
#20
Matthew A Statton, Alejandro Vazquez, Susanne M Morton, Erin V L Vasudevan, Amy J Bastian
The cerebellum is thought to adapt movements to changes in the environment in order to update an implicit understanding of the association between our motor commands and their sensory consequences. This trial-by-trial motor recalibration in response to external perturbations is frequently impaired in people with cerebellar damage. In healthy people, adaptation to motor perturbations is also known to induce a form of sensory perceptual recalibration. For instance, hand-reaching adaptation tasks produce transient changes in the sense of hand position, and walking adaptation tasks can lead to changes in perceived leg speed...
August 24, 2017: Cerebellum
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