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Yuri Kanamaru, Jianxue Li, Natalie Stewart, Richard L Sidman, Emi Takahashi
Cerebellar MR imaging has several challenging aspects, due to the fine, repetitive layered structure of cortical folia with underlying axonal pathways. In this MR study, we imaged with high-angular resolution diffusion imaging (HARDI) abnormal cerebellar cortical structure (gray matter) and myelinated axonal pathways (white matter) of a mouse spontaneous mutation, Purkinje cell degeneration (pcd), in which almost all Purkinje neurons degenerate, mainly between postnatal days 20 and 35. Mouse brains at postnatal day 20 (P20) and at 8 months were scanned, and known or expected abnormalities, such as reduction of the white matter volume, disorganized pathways likely linked to parallel fibers, mossy fibers, and other fibers running from/to the cerebellar cortex were observed in mutant mice...
January 19, 2017: Cerebellum
Mario Mascalchi, Francesco Mari, Beatrice Berti, Emanuele Bartolini, Matteo Lenge, Andrea Bianchi, Laura Antonucci, Filippo M Santorelli, Barbara Garavaglia, Renzo Guerrini
Infantile neuronal axonal dystrophy (INAD) is characterized by progressive cerebellar atrophy. MRI has been recommended as a marker of disease progression in cerebellar diseases. We performed a longitudinal brain volumetry study in a couple of bicorial twins with PLA2G6-positive INAD. Brain volumetry was calculated with FreeSurfer software on 3T T1-weighted images acquired at age 28 (t 0) and 36 months (t 1) in patient 1 and at age 22 (t 0) and 31 months (t 1) in patient 2. Data at t 0 were compared to those obtained in 18 control children aged 14-44 months with normal MRI...
January 14, 2017: Cerebellum
Mariano Serrao
No abstract text is available yet for this article.
January 5, 2017: Cerebellum
Kim van Dun, Florian Bodranghien, Mario Manto, Peter Mariën
Transcranial magnetic and electric stimulation of the brain are novel and highly promising techniques currently employed in both research and clinical practice. Improving or rehabilitating brain functions by modulating excitability with these noninvasive tools is an exciting new area in neuroscience. Since the cerebellum is closely connected with the cerebral regions subserving motor, associative, and affective functions, the cerebello-thalamo-cortical pathways are an interesting target for these new techniques...
December 28, 2016: Cerebellum
Hannes Mogensen, Fredrik Bengtsson, Henrik Jörntell
The existence of input plasticity in the deep cerebellar nuclear (DCN) cells of the adult cerebellum could have profound implications for our understanding of cerebellar function. Whereas the existence of plastic changes in mossy fiber (mf) synaptic responses in DCN neurons has been demonstrated in juvenile slices, there has so far been no direct demonstration of this form of plasticity in the adult cerebellum in vivo. In the present paper, we recorded from neurons in the anterior interposed nucleus (AIN) and stimulated the spinocerebellar tracts (SCT) directly or via the skin to obtain mf activation and the inferior olive to activate climbing fibers (cfs) in the nonanesthetized, adult, decerebrated cat...
December 28, 2016: Cerebellum
Bert M Verheijen, Romina J G Gentier, Denise J H P Hermes, Fred W van Leeuwen, David A Hopkins
The ubiquitin-proteasome system (UPS) is one of the major mechanisms for protein breakdown in cells, targeting proteins for degradation by enzymatically conjugating them to ubiquitin molecules. Intracellular accumulation of ubiquitin-B(+1) (UBB(+1)), a frameshift mutant of ubiquitin-B, is indicative of a dysfunctional UPS and has been implicated in several disorders, including neurodegenerative disease. UBB(+1)-expressing transgenic mice display widespread labeling for UBB(+1) in brain and exhibit behavioral deficits...
December 13, 2016: Cerebellum
Mariano Serrao, Giorgia Chini, Carlo Casali, Carmela Conte, Martina Rinaldi, Alberto Ranavolo, Christian Marcotulli, Luca Leonardi, Gaia Fragiotta, Fabiano Bini, Gianluca Coppola, Francesco Pierelli
In the present study, the progression of gait impairment in a group of patients with primary degenerative cerebellar ataxias was observed over a period of 4 years. A total of 30 patients underwent an initial gait analysis study, and thereafter only 12 were evaluated because they completed the 2- and 4-year follow-up evaluations. Time-distance parameters, trunk and joint range of motion (RoM), and variability parameters (e.g., coefficients of variation) were measured at the baseline and at each follow-up evaluation...
December 6, 2016: Cerebellum
František Vožeh
Jan. Evangelista Purkyně, the most famous among Czech physiologists, was the first who identified and described the largest nerve cells in the cerebellum. The most distinguished researchers of the nervous system then recommended naming these neurons Purkinje cells in his honor. Through experiments by Purkinje and his followers, the function of the cerebellum was properly attributed to the precision of motor movements and skills. This traditional concept was valid until early 1990s, when it was readjusted and replenished with new and important findings...
November 17, 2016: Cerebellum
Camila Callegari Piccinin, Lidiane Soares Campos, Rachel Paes Guimarães, Luiza Gonzaga Piovesana, Maria Cristina Arci Dos Santos, Paula Christina Azevedo, Brunno Machado Campos, Thiago Junqueira Ribeiro de Rezende, Augusto Amato-Filho, Fernando Cendes, Anelyssa D'Abreu
Parkinson's disease (PD) is an akinetic-rigid disorder characterized by basal ganglia dysfunction and a possible cerebello-thalamo-cortical circuit involvement. This study aims to investigate the pattern of cerebellar involvement in PD and to assess whether it correlates with clinical parameters. MRI scans were acquired from 50 healthy controls (HC) and 63 patients; 44 were classified as tremor-predominant-PD (PDT) and 19 as akinetic/rigidity-predominant-PD (PDAR). We designed an analysis of covariance including the three groups and contrasted as follows: (1) all 63 PD vs HC, (2) PDT vs HC, (3) PDAR vs HC, and (4) PDT vs PDAR...
November 16, 2016: Cerebellum
Lan Luo, Jie Wang, Raymond Y Lo, Karla P Figueroa, Stefan M Pulst, Pei-Hsin Kuo, Susan Perlman, George Wilmot, Christopher M Gomez, Jeremy D Schmahmann, Henry Paulson, Vikram G Shakkottai, Sarah H Ying, Theresa Zesiewicz, Khalaf Bushara, Michael Geschwind, Guangbin Xia, S H Subramony, Tetsuo Ashizawa, Sheng-Han Kuo
The aim of this study is to determine whether the initial symptom associates with motor progression in spinocerebellar ataxias (SCAs). SCAs are clinically heterogeneous and the initial presentation may represent different subtypes of SCA with different motor progression. We studied 317 participants with SCAs1, 2, 3, and 6 from the Clinical Research Consortium for SCAs (CRC-SCA) and repeatedly measured the severity of ataxia for 2 years. SCA patients were divided into gait-onset and non-gait-onset (speech, vision, and hand dexterity) groups based on the initial presentation...
November 15, 2016: Cerebellum
Katsuya Nakamura, Kunihiro Yoshida, Akira Matsushima, Yusaku Shimizu, Shunichi Sato, Hiroyuki Yahikozawa, Shinji Ohara, Masanobu Yazawa, Masao Ushiyama, Mitsuto Sato, Hiroshi Morita, Atsushi Inoue, Shu-Ichi Ikeda
Spinocerebellar ataxia type 31 (SCA31) is known as a late-onset, relatively pure cerebellar form of ataxia, but a longitudinal prospective study on the natural history of SCA31 has not been done yet. In this prospective cohort study, we enrolled 44 patients (mean ± standard deviation 73.6 ± 8.5 years) with genetically confirmed SCA31 from 10 ataxia referral centers in the Nagano area, Japan. Patients were evaluated every year for 4 years using the Scale for the Assessment and Rating of Ataxia (SARA) and the Barthel Index (BI)...
November 9, 2016: Cerebellum
Amandine Moroso, Aurélie Ruet, Mathilde Deloire, Delphine Lamargue-Hamel, Stéphanie Cubizolle, Julie Charré-Morin, Aurore Saubusse, Bruno Brochet
Cerebellar impairment is frequent and predictive of disability in multiple sclerosis (MS). The Nine-Hole Peg Test (NHPT) is commonly used to assess cerebellar symptoms despite its lack of specificity for cerebellar ataxia. Eye-tracking is a reliable test for identifying subtle cerebellar symptoms and could be used in clinical trials, including those involving early MS patients. To evaluate, by the use of eye-tracking, the accuracy of the NHPT in detecting subtle cerebellar symptoms in patients with clinically isolated syndrome with a high risk of conversion to MS (HR-CIS)...
November 4, 2016: Cerebellum
J Claassen, F Labrenz, T M Ernst, A Icenhour, J Langhorst, M Forsting, D Timmann, S Elsenbruch
There is evidence to support a role of the cerebellum in emotional learning processes, which are demonstrably altered in patients with chronic pain. We tested if cerebellar activation is altered during visceral pain-related fear conditioning and extinction in irritable bowel syndrome (IBS). Cerebellar blood oxygenation level-dependent (BOLD) data from N = 17 IBS patients and N = 21 healthy controls, collected as part of a previous fMRI study, was reanalyzed utilizing an advanced normalizing method of the cerebellum...
October 28, 2016: Cerebellum
Xiaoxia Feng, Le Li, Manli Zhang, Xiujie Yang, Mengyu Tian, Weiyi Xie, Yao Lu, Li Liu, Nathalie N Bélanger, Xiangzhi Meng, Guosheng Ding
Previous neuroimaging studies have found atypical cerebellar activation in individuals with dyslexia in either motor-related tasks or language tasks. However, studies investigating atypical cerebellar activation in individuals with dyslexia have mostly used tasks tapping phonological processing. A question that is yet unanswered is whether the cerebellum in individuals with dyslexia functions properly during orthographic processing of words, as growing evidence shows that the cerebellum is also involved in visual and spatial processing...
October 27, 2016: Cerebellum
Eric J Lang, Richard Apps, Fredrik Bengtsson, Nadia L Cerminara, Chris I De Zeeuw, Timothy J Ebner, Detlef H Heck, Dieter Jaeger, Henrik Jörntell, Mitsuo Kawato, Thomas S Otis, Ozgecan Ozyildirim, Laurentiu S Popa, Alexander M B Reeves, Nicolas Schweighofer, Izumi Sugihara, Jianqiang Xiao
For many decades, the predominant view in the cerebellar field has been that the olivocerebellar system's primary function is to induce plasticity in the cerebellar cortex, specifically, at the parallel fiber-Purkinje cell synapse. However, it has also long been proposed that the olivocerebellar system participates directly in motor control by helping to shape ongoing motor commands being issued by the cerebellum. Evidence consistent with both hypotheses exists; however, they are often investigated as mutually exclusive alternatives...
February 2017: Cerebellum
Lara Fernandez, Natalia Albein-Urios, Melissa Kirkovski, Jennifer L McGinley, Anna T Murphy, Christian Hyde, Mark A Stokes, Nicole J Rinehart, Peter G Enticott
The cerebellum appears to play a key role in the development of internal rules that allow fast, predictive adjustments to novel stimuli. This is crucial for adaptive motor processes, such as those involved in walking, where cerebellar dysfunction has been found to increase variability in gait parameters. Motor adaptation is a process that results in a progressive reduction in errors as movements are adjusted to meet demands, and within the cerebellum, this seems to be localised primarily within the right hemisphere...
February 2017: Cerebellum
C Tzoulis, Paweł Sztromwasser, Stefan Johansson, Ivar Otto Gjerde, Per Knappskog, L A Bindoff
We identified PNKP mutations in a Norwegian woman with AOA. This patient had the typical findings with cognitive dysfunction, peripheral neuropathy, cerebellar dysarthria, horizontal nystagmus, oculomotor apraxia, and severe truncal and appendicular ataxia. In addition, she had hypoalbuminemia and massive lower limb edema which showed some improvement with treatment. Exome sequencing identified two heterozygous mutations, one in exon 14 (c.1196T>C, p.Leu399Pro) and one in exon 16 (c.1393_1396del, p.Glu465*)...
February 2017: Cerebellum
Suzanne Granhøj Lindquist, Lisbeth Birk Møller, Christine I Dali, Lisbeth Marner, Erik-Jan Kamsteeg, Jørgen Erik Nielsen, Lena Elisabeth Hjermind
Spinocerebellar ataxia type 11 (SCA11) is rare and has previously been described in four families worldwide. We report a Danish family with onset of symptoms in early childhood and affected family members in two generations. The proband, a Danish female born in 1968, and family members were examined. Exome sequencing was performed and a "movement disorders" gene panel consisting of approximately 200 genes was used for filtering, while Sanger sequencing was used for subsequent testing for the mutation in the family...
February 2017: Cerebellum
Aasef G Shaikh, Aaron L Wong, Lance M Optican, David S Zee
An attractive hypothesis about how the brain learns to keep its motor commands accurate is centered on the idea that the cerebellar cortex associates error signals carried by climbing fibers with simultaneous activity in parallel fibers. Motor learning can be impaired if the error signals are not transmitted, are incorrect, or are misinterpreted by the cerebellar cortex. Learning might also be impaired if the brain is overwhelmed with a sustained barrage of meaningless information unrelated to simultaneously appearing error signals about incorrect performance...
February 2017: Cerebellum
Ling-Li Zeng, Liang Xie, Hui Shen, Zhiguo Luo, Peng Fang, Yanan Hou, Beisha Tang, Tao Wu, Dewen Hu
Parkinson's disease (PD) is one of the most common neurodegenerative disorders in the world. Previous studies have focused on the basal ganglia and cerebral cortices. To date, the cerebellum has not been systematically investigated in patients with PD. In the current study, 45 probable PD patients and 40 age- and gender-matched healthy controls underwent structural magnetic resonance imaging, and we used support vector machines combining with voxel-based morphometry to explore the cerebellar structural changes in the probable PD patients relative to healthy controls...
February 2017: Cerebellum
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