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Cerebellum

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https://www.readbyqxmd.com/read/28791574/heterotopic-purkinje-cells-a-comparative-postmortem-study-of-essential-tremor-and-spinocerebellar-ataxias-1-2-3-and-6
#1
Elan D Louis, Sheng-Han Kuo, William J Tate, Geoffrey C Kelly, Jesus Gutierrez, Etty P Cortes, Jean-Paul G Vonsattel, Phyllis L Faust
Essential tremor (ET) is among the most common neurological diseases. Postmortem studies have noted a series of pathological changes in the ET cerebellum. Heterotopic Purkinje cells (PCs) are those whose cell body is mis-localized in the molecular layer. In neurodegenerative settings, these are viewed as a marker of the progression of neuronal degeneration. We (1) quantify heterotopias in ET cases vs. controls, (2) compare ET cases to other cerebellar degenerative conditions (spinocerebellar ataxias (SCAs) 1, 2, 3, and 6), (3) compare these SCAs to one another, and (4) assess heterotopia within the context of associated PC loss in each disease...
August 8, 2017: Cerebellum
https://www.readbyqxmd.com/read/28786014/targeting-the-human-cerebellum-with-transcranial-direct-current-stimulation-to-modulate-behavior-a-meta-analysis
#2
REVIEW
Viola Oldrati, Dennis J L G Schutter
Transcranial direct current stimulation (tDCS) is increasingly used to study motor- and non-motor-related functions of the cerebellum. The aim of the present study was to quantitatively review available studies to estimate the efficacy of cerebellar tDCS in altering motor- and cognitive-related behavioral performance in healthy volunteers. The present meta-analysis included 32 sham-controlled studies. Results from random effects modeling of the cumulative effect size demonstrated that anodal and cathodal tDCS to the cerebellum were effective in changing performance...
August 7, 2017: Cerebellum
https://www.readbyqxmd.com/read/28710677/cerebellar-contributions-to-persistent-auditory-verbal-hallucinations-in-patients-with-schizophrenia
#3
Maximilian Cierpka, Nadine D Wolf, Katharina M Kubera, Mike M Schmitgen, Nenad Vasic, Karel Frasch, Robert Christian Wolf
There is growing evidence that the cerebellum plays a crucial role in the pathophysiology of schizophrenia symptoms. Despite increasing evidence for cerebellar involvement in affective, attentive, and cognitive functions including language processing and perception, investigations of cerebellar contributions to auditory verbal hallucinations (AVH) in schizophrenia are lacking. Using structural magnetic resonance imaging at 3T, we investigated the data of 20 patients with schizophrenia and 14 matched healthy controls...
July 14, 2017: Cerebellum
https://www.readbyqxmd.com/read/28699106/promoter-variant-alters-expression-of-the-autophagic-becn1-gene-implications-for-clinical-manifestations-of-machado-joseph-disease
#4
Nadiya Kazachkova, Mafalda Raposo, Amanda Ramos, Rafael Montiel, Manuela Lima
Autophagy is especially important in disorders where accumulation of the mutant protein is a hallmark, such as the Machado-Joseph disease/spinocerebellar ataxia type 3 (MJD/SCA3). We analyzed the promoter of the BECN1 gene, whose overexpression has been reported to exert neuroprotective effects in MJD, with the aim of finding variants that could be associated with expression levels of beclin-1 and could be tested as modifiers of onset and disease severity. A fragment encompassing the BECN1 promoter was sequenced in 95 MJD subjects and 120 controls...
July 11, 2017: Cerebellum
https://www.readbyqxmd.com/read/28669058/improved-neuroimaging-atlas-of-the-dentate-nucleus
#5
Naying He, Jason Langley, Daniel E Huddleston, Huawei Ling, Hongmin Xu, Chunlei Liu, Fuhua Yan, Xiaoping P Hu
The dentate nucleus (DN) of the cerebellum is the major output nucleus of the cerebellum and is rich in iron. Quantitative susceptibility mapping (QSM) provides better iron-sensitive MRI contrast to delineate the boundary of the DN than either T2-weighted images or susceptibility-weighted images. Prior DN atlases used T2-weighted or susceptibility-weighted images to create DN atlases. Here, we employ QSM images to develop an improved dentate nucleus atlas for use in imaging studies. The DN was segmented in QSM images from 38 healthy volunteers...
July 1, 2017: Cerebellum
https://www.readbyqxmd.com/read/28660440/neurocognitive-characterization-of-an-sca28-family-caused-by-a-novel-afg3l2-gene-mutation
#6
Laszlo Szpisjak, Viola L Nemeth, Noemi Szepfalusi, Denes Zadori, Zoltan Maroti, Tibor Kalmar, Laszlo Vecsei, Peter Klivenyi
No abstract text is available yet for this article.
June 28, 2017: Cerebellum
https://www.readbyqxmd.com/read/28656525/hypomagnesemia-a-treatable-cause-of-ataxia-with-cerebellar-edema
#7
LETTER
Idoia Rouco Axpe, Javier Almeida Velasco, Jose Guillermo Barreiro Garcia, Jose Manuel Urbizu Gallardo, Beatriz Mateos Goñi
No abstract text is available yet for this article.
June 27, 2017: Cerebellum
https://www.readbyqxmd.com/read/28612183/impairments-in-walking-ability-dexterity-and-cognitive-function-in-multiple-sclerosis-are-associated-with-different-regional-cerebellar-gray-matter-loss
#8
Matthias Grothe, Martin Lotze, Sönke Langner, Alexander Dressel
Both histological and neuroimaging studies highlight the role of the cerebellum in multiple sclerosis (MS). There is at least some evidence for associations of cerebellar gray matter (GM) loss with motor and cognitive ability. We therefore correlated motor and cognitive ability scores (the multiple sclerosis functional composite MSFC) with regional cerebellar GM volumes. We used voxel-based morphometry (VBM) to assess the regional GM volume loss in a cohort of 45 MS patients. For the regression analysis, we used the clinical subscores of the multiple sclerosis functional composite (25-ft walk test (T25FW), nine-hole peg test (9HPT), paced auditory serial addition task (PASAT))...
June 13, 2017: Cerebellum
https://www.readbyqxmd.com/read/28593454/permanent-cerebellar-degeneration-after-acute-hyperthermia-with-non-toxic-lithium-levels-a-case-report-and-review-of-literature
#9
REVIEW
Fabian H Rossi, Elisa Marie Rossi, Michael Hoffmann, Welwin Liu, Ramon Rodriguez Cruz, Natasha Antonovich, Arash Rezaei, Elizabeth Gonzalez, Maria Clara Franco, Alvaro Estevez, Florian Thomas
This was a study of a 33-year-old man with bipolar disorder treated with lithium who developed cerebellar atrophy after an event of extreme hyperthermia. Unlike previously reported cases of acute cerebellar atrophy after heat stroke, neuroleptic syndrome or lithium toxicity, this case was characterized by a chronic cerebellar atrophy that developed after sepsis-induced hyperthermia in the setting of non-toxic lithium levels. Unique to this case also was the early finding of cerebellar atrophy on MRI 2 weeks after the episode of hyperthermia, long-term neurotoxicity after the novo lithium therapy, and longest follow-up case of chronic cerebellar syndrome after hyperthermia with non-toxic lithium levels...
June 8, 2017: Cerebellum
https://www.readbyqxmd.com/read/28589261/nonmotor-symptoms-in-patients-with-spinocerebellar-ataxia-type-10
#10
Adriana Moro, Renato P Munhoz, Mariana Moscovich, Walter O Arruda, Salmo Raskin, Laura Silveira-Moriyama, Tetsuo Ashizawa, Hélio A G Teive
Nonmotor symptoms (NMS) have been described in several neurodegenerative diseases but have not been systematically evaluated in spinocerebellar ataxia type 10 (SCA10). The objective of the study is to compare the frequency of NMS in patients with SCA10, Machado-Joseph disease (MJD), and healthy controls. Twenty-eight SCA10, 28 MJD, and 28 healthy subjects were prospectively assessed using validated screening tools for chronic pain, autonomic symptoms, fatigue, sleep disturbances, psychiatric disorders, and cognitive function...
June 6, 2017: Cerebellum
https://www.readbyqxmd.com/read/28536821/impaired-motor-coordination-and-learning-in-mice-lacking-anoctamin-2-calcium-gated-chloride-channels
#11
Franziska Neureither, Katharina Ziegler, Claudia Pitzer, Stephan Frings, Frank Möhrlen
Neurons communicate through excitatory and inhibitory synapses. Both lines of communication are adjustable and allow the fine tuning of signal exchange required for learning processes in neural networks. Several distinct modes of plasticity modulate glutamatergic and GABAergic synapses in Purkinje cells of the cerebellar cortex to promote motor control and learning. In the present paper, we present evidence for a role of short-term ionic plasticity in the cerebellar circuit activity. This type of plasticity results from altered chloride driving forces at the synapses that molecular layer interneurons form on Purkinje cell dendrites...
May 23, 2017: Cerebellum
https://www.readbyqxmd.com/read/28456900/nessca-validation-and-responsiveness-of-several-rating-scales-in-spinocerebellar-ataxia-type-2
#12
Thais L Monte, Estela R Reckziegel, Marina C Augustin, Amanda S P Silva, Lucas D Locks-Coelho, Orlando Barsottini, José L Pedroso, Fernando R Vargas, Maria-Luiza Saraiva-Pereira, Vanessa Bielefeldt Leotti, Laura Bannach Jardim
Spinocerebellar ataxia type 2 (SCA2), caused by a CAG expansion (CAGexp) at ATXN2, has a complex clinical picture. While validated ataxia scales are available, comprehensive instruments to measure all SCA2 neurological manifestations are required. This study aims to validate the Neurological Examination Score for the assessment of Spinocerebellar Ataxias (NESSCA) to be used in SCA2 and to compare its responsiveness to those obtained with other instruments. NESSCA, SARA, SCAFI, and CCFS scales were applied in symptomatic SCA2 patients...
August 2017: Cerebellum
https://www.readbyqxmd.com/read/28456899/mesenchymal-stem-cell-derived-factors-restore-function-to-human-frataxin-deficient-cells
#13
Kevin Kemp, Rimi Dey, Amelia Cook, Neil Scolding, Alastair Wilkins
Friedreich's ataxia is an inherited neurological disorder characterised by mitochondrial dysfunction and increased susceptibility to oxidative stress. At present, no therapy has been shown to reduce disease progression. Strategies being trialled to treat Friedreich's ataxia include drugs that improve mitochondrial function and reduce oxidative injury. In addition, stem cells have been investigated as a potential therapeutic approach. We have used siRNA-induced knockdown of frataxin in SH-SY5Y cells as an in vitro cellular model for Friedreich's ataxia...
August 2017: Cerebellum
https://www.readbyqxmd.com/read/28439779/searching-for-an-internal-representation-of-stimulus-kinematics-in-the-response-of-ventral-paraflocculus-purkinje-cells
#14
Pablo M Blazquez, GyuTae Kim, Tatyana A Yakusheva
Motor control theories propose that the central nervous system builds internal representations of the motion of both our body and external objects. These representations, called forward models, are essential for accurate motor control. For instance, to produce a precise reaching movement to catch a flying ball, the central nervous system must build predictions of the current and future states of both the arm and the ball. Accumulating evidence suggests that the cerebellar cortex contains a forward model of an individual's body movement...
August 2017: Cerebellum
https://www.readbyqxmd.com/read/28215041/the-known-and-missing-links-between-the-cerebellum-basal-ganglia-and-cerebral-cortex
#15
LETTER
Alberto Cacciola, Demetrio Milardi, Paolo Livrea, Paolo Flace, Giuseppe Anastasi, Angelo Quartarone
No abstract text is available yet for this article.
June 2017: Cerebellum
https://www.readbyqxmd.com/read/28150130/three-year-follow-up-of-high-dose-ubiquinol-supplementation-in-a-case-of-familial-multiple-system-atrophy-with-compound-heterozygous-coq2-mutations
#16
Jun Mitsui, Ken Koguchi, Toshimitsu Momose, Miwako Takahashi, Takashi Matsukawa, Tsutomu Yasuda, Shin-Ichi Tokushige, Hiroyuki Ishiura, Jun Goto, Shigeaki Nakazaki, Tomoyoshi Kondo, Hidefumi Ito, Yorihiro Yamamoto, Shoji Tsuji
We report a 3-year follow-up of high-dose ubiquinol supplementation in a case of familial multiple system atrophy (MSA) with compound heterozygous nonsense (R387X) and missense (V393A) mutations in COQ2. A high-dose ubiquinol supplementation substantially increased total coenzyme Q10 levels in cerebrospinal fluid as well as in plasma. The patient was at the advanced stage of MSA, and the various scores of clinical rating scales remained stable without changes during the 3 years. The cerebral metabolic ratio of oxygen measured by (15)O2 PET, however, increased by approximately 30% after administration of ubiquinol, suggesting that ubiquinol can improve mitochondrial oxidative metabolism in the brain...
June 2017: Cerebellum
https://www.readbyqxmd.com/read/28127679/motion-illusion-evidence-towards-human-vestibulo-thalamic-projections
#17
Aasef G Shaikh, Dominik Straumann, Antonella Palla
Contemporary studies speculated that cerebellar network responsible for motion perception projects to the cerebral cortex via vestibulo-thalamus. Here, we sought for the physiological properties of vestibulo-thalamic pathway responsible for the motion perception. Healthy subjects and the patient with focal vestibulo-thalamic lacunar stroke spun a hand-held rheostat to approximate the value of perceived angular velocity during whole-body passive earth-vertical axis rotations in yaw plane. Vestibulo-ocular reflex was simultaneously measured with high-resolution search coils (paradigm 1)...
June 2017: Cerebellum
https://www.readbyqxmd.com/read/28102462/cerebellar-pathways-in-mouse-model-of-purkinje-cell-degeneration-detected-by-high-angular-resolution-diffusion-imaging-tractography
#18
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...
June 2017: Cerebellum
https://www.readbyqxmd.com/read/28032321/targeting-the-cerebellum-by-noninvasive-neurostimulation-a-review
#19
REVIEW
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...
June 2017: Cerebellum
https://www.readbyqxmd.com/read/28032320/no-medium-term-spinocerebellar-input-plasticity-in-deep-cerebellar-nuclear-neurons-in-vivo
#20
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...
June 2017: Cerebellum
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