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Masanobu Kano, Takaki Watanabe, Naofumi Uesaka, Masahiko Watanabe
Functional neural circuits in the mature animals are shaped during postnatal development by elimination of unnecessary synapses and strengthening of necessary ones among redundant synaptic connections formed transiently around birth. In the cerebellum of neonatal rodents, excitatory synapses are formed on the somata of Purkinje cells (PCs) by climbing fibers (CFs) that originate from neurons in the contralateral inferior olive. Each PC receives inputs from multiple (~ five) CFs that have about equal synaptic strengths...
July 16, 2018: Cerebellum
Tomoo Hirano
There are multiple types of plasticity at both excitatory glutamatergic and inhibitory GABAergic synapses onto a cerebellar Purkinje neuron (PN). At parallel fiber to PN synapses, long-term depression (LTD) and long-term potentiation (LTP) occur, while at molecular layer interneuron to PN synapses, a type of LTP called rebound potentiation (RP) takes place. LTD, LTP, and RP seem to contribute to motor learning. However, each type of synaptic plasticity might play a different role in various motor learning paradigms...
July 11, 2018: Cerebellum
Ana Tari Ashizawa, Jenny Holt, Kelsey Faust, Weier Liu, Anjana Tiwari, Nan Zhang, Tetsuo Ashizawa
Cerebellar Purkinje cells (PCs) show conspicuous damages in many ataxic disorders. Targeted delivery of short nucleic acids, such as antisense oligonucleotides, to PCs may be a potential treatment for ataxic disorders, especially spinocerebellar ataxias (SCAs), which are mostly caused by a gain of toxic function of the mutant RNA or protein. However, oligonucleotides do not cross the blood-brain barrier (BBB), necessitating direct delivery into the central nervous system (CNS) through intra-thecal, intra-cisternal, intra-cerebral ventricular, or stereotactic parenchymal administration...
July 9, 2018: Cerebellum
Martha L Streng, Laurentiu S Popa, Timothy J Ebner
The climbing fiber-Purkinje cell circuit is one of the most powerful and highly conserved in the central nervous system. Climbing fibers exert a powerful excitatory action that results in a complex spike in Purkinje cells and normal functioning of the cerebellum depends on the integrity of climbing fiber-Purkinje cell synapse. Over the last 50 years, multiple hypotheses have been put forward on the role of the climbing fibers and complex spikes in cerebellar information processing and motor control. Central to these theories is the nature of the interaction between the low-frequency complex spike discharge and the high-frequency simple spike firing of Purkinje cells...
July 7, 2018: Cerebellum
Helena Fussiger, Maria Luiza Saraiva-Pereira, Sandra Leistner-Segal, Laura Bannach Jardim
Friedreich ataxia (FRDA) is an autosomal recessive disorder due to mutations in the FXN gene. FRDA is characterized by the classical triad of ataxia, absent reflexes, and Babinski sign, but atypical presentations might also occur. Our aims were to describe the proportion of FRDA diagnoses in suspected families living in Rio Grande do Sul, South Brazil, and to estimate a minimum frequency of symptomatic subjects. Subjects that were evaluated by molecular analysis for FRDA at the Hospital de Clínicas de Porto Alegre were identified in our files...
June 25, 2018: Cerebellum
Albert Wabnegger, Anne Schienle
Previous research indicated that the cerebellum is involved in psychopathologies with body-focused repetitive behaviors. The present study investigated whether patients with a diagnosis of skin-picking disorder (SPD) also show altered cerebellar structure and function. Structural as well as functional MRI data from 30 SPD patients and 31 controls were analyzed. The fMRI approach compared cerebellar activity and connectivity between the two groups during scratching and caressing of a small skin area on the arm...
June 22, 2018: Cerebellum
Richard Apps, Richard Hawkes, Sho Aoki, Fredrik Bengtsson, Amanda M Brown, Gang Chen, Timothy J Ebner, Philippe Isope, Henrik Jörntell, Elizabeth P Lackey, Charlotte Lawrenson, Bridget Lumb, Martijn Schonewille, Roy V Sillitoe, Ludovic Spaeth, Izumi Sugihara, Antoine Valera, Jan Voogd, Douglas R Wylie, Tom J H Ruigrok
In the original version of this paper, the Title should have been written with "A Consensus paper" to read "Cerebellar Modules and Their Role as Operational Cerebellar Processing Units: A Consensus paper".
June 21, 2018: Cerebellum
Mariana Moscovich, Renato Puppi Munhoz, Adriana Moro, Salmo Raskin, Karen McFarland, Tetsuo Ashizawa, Helio A G Teive, Laura Silveira-Moriyama
Although the main clinical manifestations of spinocerebellar ataxias (SCAs) result from damage of the cerebellum, other systems may also be involved. Olfactory deficits have been reported in other types of ataxias, especially in SCA3; however, there are no studies on olfactory deficits in SCA type 10 (SCA10). To analyze olfactory function of SCA10 patients compared with that of SCA3, Parkinson's, and healthy controls. Olfactory identification was tested in three groups of 30 patients (SCA10, SCA3, and Parkinson's disease (PD)) and 44 healthy controls using the Sniffin' Sticks (SS16) test...
June 19, 2018: Cerebellum
(no author information available yet)
No abstract text is available yet for this article.
June 19, 2018: Cerebellum
Makoto Samukawa, Makito Hirano, Kazumasa Saigoh, Shigeru Kawai, Yukihiro Hamada, Daisuke Takahashi, Yusaku Nakamura, Susumu Kusunoki
Spinocerebellar ataxia type 8 (SCA8) is a rare autosomal dominant neurodegenerative disease caused by expanded CTA/CTG repeats in the ATXN8OS gene. Many patients had pure cerebellar ataxia, while some had parkinsonism, both without causal explanation. We analyzed the ATXN8OS gene in 150 Japanese patients with ataxia and 76 patients with Parkinson's disease or related disorders. We systematically reassessed 123 patients with SCA8, both our patients and those reported in other studies. Two patients with progressive supranuclear palsy (PSP) had mutations in the ATXN8OS gene...
June 19, 2018: Cerebellum
Kasja Solbach, Simba-Joshua Oostdam, Martin Kronenbuerger, Dagmar Timmann, Marcus Gerwig
The cerebellum and the prefrontal cortex are assumed to play a role in the pathophysiology of essential tremor (ET). Trace eyeblink conditioning with a long interstimulus interval relies on an intact function of the hippocampus, prefrontal cortex (PFC), and, although marginally, of the cerebellum. The aim of the present study was to evaluate whether long trace eyeblink conditioning is impaired in patients with ET. In 18 patients with ET and 18 controls, a long trace conditioning paradigm was applied. Following 100 paired conditioned response-unconditioned response trials, 30 conditioned response alone trials were given as extinction trials...
June 18, 2018: Cerebellum
Therese M Gilligan, Robert D Rafal
A consensus has emerged that the cerebellum makes important contributions to a spectrum of linguistic processes, but that the psychobiology of these contributions remains enigmatic (Mariën et al., Cerebellum 13(3):386-410, 2014). One aspect of this enigma arises from the fact that, although the language-dominant left cerebral hemisphere is connected to the right cerebellum, distinctive contributions of the left cerebellar hemisphere have been documented (Murdoch and Whelan, Folia Phoniatr Logop 59:184-9, 2007), but remain poorly understood...
June 14, 2018: Cerebellum
Mitchell Slapik, Sharif I Kronemer, Owen Morgan, Ryan Bloes, Seth Lieberman, Jordan Mandel, Liana Rosenthal, Cherie Marvel
Poor visuospatial skills can disrupt activities of daily living. The cerebellum has been implicated in visuospatial processing, and patients with cerebellar injury often exhibit poor visuospatial skills, as measured by impaired memory for the figure within the Rey-Osterrieth complex figure task (ROCF). Visuospatial skills are an inherent aspect of the ROCF; however, figure organization (i.e., the order in which the figure is reconstructed by the participant) can influence recall ability. The objective of this study was to examine and compare visuospatial and organization skills in people with cerebellar ataxia...
June 13, 2018: Cerebellum
Swati Khare, Kira Galeano, Yalan Zhang, Jerelyn A Nick, Harry S Nick, S H Subramony, Jacinda Sampson, Leonard K Kaczmarek, Michael F Waters
Mutations in the potassium channel gene KCNC3 (Kv3.3) cause the autosomal dominant neurological disease, spinocerebellar ataxia 13 (SCA13). In this study, we expand the genotype-phenotype repertoire of SCA13 by describing the novel KCNC3 deletion p.Pro583_Pro585del highlighting the allelic heterogeneity observed in SCA13 patients. We characterize adult-onset, progressive clinical symptoms of two afflicted kindred and introduce the symptom of profound spasticity not previously associated with the SCA13 phenotype...
June 13, 2018: Cerebellum
Lillian G Matthews, T E Inder, L Pascoe, K Kapur, K J Lee, B B Monson, L W Doyle, D K Thompson, P J Anderson
Impaired cerebellar development is an important determinant of adverse motor and cognitive outcomes in very preterm (VPT) infants. However, longitudinal MRI studies investigating cerebellar maturation from birth through childhood and associated neurodevelopmental outcomes are lacking. We aimed to compare cerebellar volume and growth from term-equivalent age (TEA) to 7 years between VPT (< 30 weeks' gestation or < 1250 g) and full-term children; and to assess the association between these measures, perinatal factors, and 7-year outcomes in VPT children, and whether these relationships varied by sex...
June 8, 2018: Cerebellum
Y Torres-Ramos, A Montoya-Estrada, B Cisneros, K Tercero-Pérez, G León-Reyes, N Leyva-García, Oscar Hernández-Hernández, Jonathan J Magaña
Spinocerebellar ataxia type 7 is a neurodegenerative inherited disease caused by a CAG expansion in the coding region of the ATXN7 gene, which results in the synthesis of polyglutamine-containing ataxin-7. Expression of mutant ataxin-7 disturbs different cell processes, including transcriptional regulation, protein conformation and clearance, autophagy, and glutamate transport; however, mechanisms underlying neurodegeneration in SCA7 are still unknown. Implication of oxidative stress in the pathogenesis of various neurodegenerative diseases, including polyglutamine disorders, has recently emerged...
June 6, 2018: Cerebellum
Richard Apps, Richard Hawkes, Sho Aoki, Fredrik Bengtsson, Amanda M Brown, Gang Chen, Timothy J Ebner, Philippe Isope, Henrik Jörntell, Elizabeth P Lackey, Charlotte Lawrenson, Bridget Lumb, Martijn Schonewille, Roy V Sillitoe, Ludovic Spaeth, Izumi Sugihara, Antoine Valera, Jan Voogd, Douglas R Wylie, Tom J H Ruigrok
The compartmentalization of the cerebellum into modules is often used to discuss its function. What, exactly, can be considered a module, how do they operate, can they be subdivided and do they act individually or in concert are only some of the key questions discussed in this consensus paper. Experts studying cerebellar compartmentalization give their insights on the structure and function of cerebellar modules, with the aim of providing an up-to-date review of the extensive literature on this subject. Starting with an historical perspective indicating that the basis of the modular organization is formed by matching olivocorticonuclear connectivity, this is followed by consideration of anatomical and chemical modular boundaries, revealing a relation between anatomical, chemical, and physiological borders...
June 6, 2018: Cerebellum
Polina A Egorova, Alexandra V Gavrilova, Ilya B Bezprozvanny
Cerebellar Purkinje cells (PCs) and cerebellar pathways are primarily affected in many autosomal dominant cerebellar ataxias. PCs generate complex spikes (CS) in vivo when activated by climbing fiber (CF) which rise from the inferior olive. In this study, we investigated the functional state of the CF-PC circuitry in the transgenic mouse model of spinocerebellar ataxia type 2 (SCA2), a polyglutamine neurodegenerative genetic disease. In our experiments, we used an extracellular single-unit recording method to compare the PC activity pattern and the CS shape in age-matched wild-type mice and SCA2-58Q transgenic mice...
June 6, 2018: Cerebellum
Sarah B Zandvliet, Carel G M Meskers, Gert Kwakkel, Erwin E H van Wegen
Transcranial direct current stimulation (tDCS) may serve as an adjunct approach in stroke rehabilitation. The cerebellum could be a target during standing balance training due to its role in motor adaptation. We tested whether cerebellar tDCS can lead to short-term effects on standing balance performance in patients with chronic stroke. Fifteen patients with a chronic stroke were stimulated with anodal stimulation on the contra-lesional cerebellar hemisphere, ipsi-lesional cerebellar hemisphere, or sham stimulation, for 20 min with 1...
May 24, 2018: Cerebellum
Maryam Rahimi-Balaei, Xiaodan Jiao, Shahin Shabanipour, Rajiv Dixit, Carol Schuurmans, Hassan Marzban
Zebrin II/aldolase C expression in the normal cerebellum is restricted to a Purkinje cell subset and is the canonical marker for stripes and zones. This spatial restriction has been confirmed in over 30 species of mammals, birds, fish, etc. In a transgenic mouse model in which the Neurogenin 2 gene has been disrupted (Neurog2 -/- ), the cerebellum is smaller than normal and Purkinje cell dendrites are disordered, but the basic zone and stripe architecture is preserved. Here, we show that in the Neurog2 -/- mouse, in addition to the normal Purkinje cell expression, zebrin II is also expressed in a population of cells with a morphology characteristic of microglia...
May 16, 2018: Cerebellum
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