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Cerebellum & Ataxias

Priya D Shanmugarajah, Nigel Hoggard, Stuart Currie, Daniel P Aeschlimann, Pascale C Aeschlimann, Dermot C Gleeson, Mohammed Karajeh, Nicola Woodroofe, Richard A Grünewald, Marios Hadjivassiliou
BACKGROUND: Alcohol-related cerebellar degeneration is one of the commonest acquired forms of cerebellar ataxia. The exact pathogenic mechanisms by which alcohol leads to cerebellar damage remain unknown. Possible autoreactive immune mediated mechanisms have not been explored previously. In this study, we aim to investigate the potential role of alcohol-induced immune mediated cerebellar degeneration. METHODS: Patients with ataxia and a history of alcohol misuse were recruited from the Ataxia and Hepatology tertiary clinics at Sheffield Teaching Hospitals NHS Trust...
2016: Cerebellum & Ataxias
Roberta Ferrucci, Tommaso Bocci, Francesca Cortese, Fabiana Ruggiero, Alberto Priori
Several studies have highlighted the therapeutic potential of transcranial direct current stimulation (tDCS) in patients with neurological diseases, including dementia, epilepsy, post-stroke dysfunctions, movement disorders, and other pathological conditions. Because of this technique's ability to modify cerebellar excitability without significant side effects, cerebellar tDCS is a new, interesting, and powerful tool to induce plastic modifications in the cerebellum. In this report, we review a number of interesting studies on the application of cerebellar tDCS for various neurological conditions (ataxia, Parkinson's disease, dystonia, essential tremor) and the possible mechanism by which the stimulation acts on the cerebellum...
2016: Cerebellum & Ataxias
Nordeyn Oulad Ben Taib, Mario Manto
BACKGROUND: Low-frequency electrical stimulation to the motor cortex (LFSMC) depresses the excitability of motor circuits by long-term depression (LTD)-like effects. The interactions between LFSMC and cathodal direct current stimulation (cDCS) over the cerebellum are unknown. METHODS: We assessed the corticomotor responses and the afferent facilitation of corticomotor responses during a conditioning paradigm in anaesthetized rats. We applied LFSMC at a frequency of 1 Hz and a combination of LFSMC with cDCS...
2016: Cerebellum & Ataxias
Daisuke Hara, Futaba Maki, Shigeaki Tanaka, Rie Sasaki, Yasuhiro Hasegawa
BACKGROUND: Progression of clinical symptoms and cerebellar atrophy may vary among subtypes of spinocerebellar degeneration and multiple system atrophy. The aim of this cross-sectional study was to demonstrate the relationship between the International Cooperative Ataxia Rating Scale (ICARS) score and cerebellar volume derived from magnetic resonance imaging (MRI) in a broad spectrum of Japanese patients with cerebellar ataxia. METHODS: A total of 86 patients with cerebellar ataxia (18 with cortical cerebellar atrophy, 34 with spinocerebellar ataxia, and 34 with multiple system atrophy) and 30 healthy subjects were studied...
2016: Cerebellum & Ataxias
Shannon C Lefaivre, Matt J N Brown, Quincy J Almeida
BACKGROUND: There exists a lack of consensus regarding how cerebellar over-activity might influence tremor in Parkinson's disease (PD). Specifically, it is unclear whether resting or postural tremor are differentially affected by cerebellar dysfunction. It is important to note that previous studies have only evaluated the influence of inhibitory stimulation on the lateral cerebellum, and have not considered the medial cerebellum. The aim of the current study was to compare the effects of a low-frequency rTMS protocol applied to the medial versus lateral cerebellum to localize the effects of cerebellar over-activity...
2016: Cerebellum & Ataxias
Anneh Mohammad Gharravi
BACKGROUND: The objective of the present study was an investigation of root Rubia Tinctorum (Madder) as a natural dye to identification of granular layer interneurons of the rat cerebellum. METHODS: Seven to ten micrometre sections were collected from the cerebellum and stained only with Madder for 2, 24 and 48 h. Other sections were stained with Madder then with hematoxyllin, cresyl violet, eosin, light green. Microscopic identification of cells was performed based on cell morphology, reaction and binding of with the dye...
2016: Cerebellum & Ataxias
Larry Vandervert
BACKGROUND: Vandervert described how, in collaboration with the cerebral cortex, unconscious learning of cerebellar internal models leads to enhanced executive control in working memory in expert music performance and in scientific discovery. Following Vandervert's arguments, it is proposed that since music performance and scientific discovery, two pillars of cultural learning and advancement, are learned through in cerebellar internal models, it is reasonable that additional if not all components of culture may be learned in the same way...
2016: Cerebellum & Ataxias
Xiao-Yang Zhang, Jian-Jun Wang, Jing-Ning Zhu
Fastigial nucleus (FN) is the phylogenetically oldest nucleus in the cerebellum, a classical subcortical motor coordinator. As one of the ultimate integration stations and outputs of the spinocerebellum, the FN holds a key position in the axial, proximal and ocular motor control by projecting to the medial descending systems and eye movement related nuclei. Furthermore, through topographic connections with extensive nonmotor systems, including visceral related nuclei in the brainstem, hypothalamus, as well as the limbic system, FN has also been implicated in regulation of various nonsomatic functions, such as feeding, cardiovascular and respiratory, defecation and micturition, immune, as well as emotional activities...
2016: Cerebellum & Ataxias
Suzete Nascimento Farias da Guarda, Adriana Bastos Conforto
[This corrects the article DOI: 10.1186/s40673-014-0016-5.].
2016: Cerebellum & Ataxias
Roman Schniepp, Michael Strupp, Max Wuehr, Klaus Jahn, Marianne Dieterich, Thomas Brandt, Katharina Feil
Acetyl-DL-leucine is a modified amino acid that was observed to improve ataxic symptoms in patients with sporadic and hereditary forms of ataxia. Here, we investigated the effect of the treatment with Acetyl-DL-leucine on the walking stability of patients with cerebellar ataxia (10x SAOA, 2x MSA-C, 2x ADA, 1x CACNA-1A mutation, 2x SCA 2, 1x SCA 1). Treatment with Acetyl-DL-leucine (500 mg; 3-3-4) significantly improved the coefficient of variation of stride time in 14 out of 18 patients. Moreover, subjective ambulatory scores (FES-I and ABC) and the SARA scores were also improved under treatment...
2016: Cerebellum & Ataxias
Jan Cendelin
Hereditary cerebellar degenerations are a heterogeneous group of diseases often having a detrimental impact on patients' quality of life. Unfortunately, no sufficiently effective causal therapy is available for human patients at present. There are several therapies that have been shown to affect the pathogenetic process and thereby to delay the progress of the disease in mouse models of cerebellar ataxias. The second experimental therapeutic approach for hereditary cerebellar ataxias is neurotransplantation...
2016: Cerebellum & Ataxias
Carlotta Lega, Tomaso Vecchi, Egidio D'Angelo, Zaira Cattaneo
BACKGROUND: Growing neuroimaging and clinical evidence suggests that the cerebellum plays a critical role in perception. In the auditory domain, the cerebellum seems to be important in different aspects of music and sound processing. Here we investigated the possible causal role of the cerebellum in two auditory tasks, a pitch discrimination and a timbre discrimination task. Specifically, participants performed a pitch and a timbre discrimination task prior and after receiving offline low frequency transcranical magnetic stimulation (TMS) over their (right) cerebellum...
2016: Cerebellum & Ataxias
Ashwini K Rao, Elan D Louis
BACKGROUND: Essential tremor (ET) is a common movement disorder characterized by kinetic, postural and intention tremors. Mounting evidence suggests an underlying dysfunction of the cerebellum or cerebellar system. While few recent studies report impairments in timing control of finger movements in ET, timing control of gait has not been examined to date. We compared timing control of gait in ET patients vs. controls, and further assessed the association of these timing impairments with tremor severity among the ET patients...
2016: Cerebellum & Ataxias
Irene Pulido-Valdeolivas, David Gómez-Andrés, Irene Sanz-Gallego, Estrella Rausell, Javier Arpa
BACKGROUND: Spinocerebellar ataxia type 3 (SCA3) is a neurodegenerative disorder that affects the cerebellar system and other subcortical regions of the brain. As for other cerebellar diseases, the severity of this type of ataxia can be assessed with the Scale for Assessment and Rating of Ataxia (SARA) which gives a total score that reflects functional impairment out of 8 cerebellar function tests. SCA3 patients score profile is heterogeneous on at the start of follow up. This study investigates possible patterns in those profiles and analyses the impact of other usually concurrent signs of impairment of extracerebellar motor systems in that profile variability by means of multivariate statistical approaches...
2016: Cerebellum & Ataxias
Jeanette Koht, Sven Olav Løstegaard, Iselin Wedding, Marie Vidailhet, Malek Louha, Chantal Me Tallaksen
BACKGROUND: Benign hereditary chorea is a rare disorder which is characterized by early onset, non-progressive choreic movement disturbance, with other hyperkinetic movements and unsteadiness also commonly seen. Hypothyroidism and lung disease are frequent additional features. The disorder is caused by mutations of the NKX2-1 gene on chromosome 14. CASE PRESENTATION: A Norwegian four-generation family with eight affected was identified. All family members had an early onset movement disorder, starting before one year of age with motor delay and chorea...
2016: Cerebellum & Ataxias
M I Shadrina, M V Shulskaya, S A Klyushnikov, T Nikopensius, M Nelis, P A Kivistik, A A Komar, S A Limborska, S N Illarioshkin, P A Slominsky
BACKGROUND: Spinocerebellar ataxias (SСAs) are a highly heterogeneous group of inherited neurological disorders. The symptoms of ataxia vary in individual patients and even within the same SCA subtype. A study of a four-generation family with autosomal dominant (AD) non-progressive SCA with mild symptoms was conducted. The genotyping of this family revealed no frequent pathogenic mutations. So the objective of this study was to identify the genetic causes of the disease in this family with the technology of whole-exome sequencing (WES)...
2016: Cerebellum & Ataxias
Jessica L Klein, Monica E Lemmon, Frances J Northington, Eugen Boltshauser, Thierry A G M Huisman, Andrea Poretti
Cerebellar abnormalities are encountered in a high number of neurological diseases that present in the neonatal period. These disorders can be categorized broadly as inherited (e.g. malformations, inborn errors of metabolism) or acquired (e.g. hemorrhages, infections, stroke). In some disorders such as Dandy-Walker malformation or Joubert syndrome, the main abnormalities are located within the cerebellum and brainstem. In other disorders such as Krabbe disease or sulfite oxidase deficiency, the main abnormalities are found within the supratentorial brain, but the cerebellar involvement may be helpful for diagnostic purposes...
2016: Cerebellum & Ataxias
Kim van Dun, Elke De Witte, Wendy Van Daele, Wim Van Hecke, Mario Manto, Peter Mariën
BACKGROUND: In the majority of right-handed subjects, language processing is subserved by a close interplay between the left cerebral hemisphere and right cerebellum. Within this network, the dominant fronto-insular region and the contralateral posterior cerebellum are crucially implicated in oral language production. CASE PRESENTATION: We report atypical anatomoclinical findings in a right-handed patient with an extensive right cerebellar infarction and an older left fronto-insular stroke...
2015: Cerebellum & Ataxias
Christine Zühlke, Barbara Mikat, Dagmar Timmann, Dagmar Wieczorek, Gabriele Gillessen-Kaesbach, Katrin Bürk
BACKGROUND: Spinocerebellar ataxia type 28 (SCA28) is related to mutations of the ATPase family gene 3-like 2 gene (AFG3L2). To date, 13 private missense mutations have been identified in families of French, Italian, and German ancestry, but overall, the disorder seems to be rare in Europe. Here, we report a kindred of German ancestry with four affected family members presenting with slowly progressive ataxia, mild pyramidal tract signs and slow saccades. METHODS: After excluding repeat expansions in the genes for SCA1-3, 6-8, 10, 12, and 17, Sanger sequencing of the coding regions of TTBK2 (SCA11), KCNC3 (SCA13), PRKCG (SCA14), FGF14 (SCA27) and AFG3L2 (SCA28) was performed...
2015: Cerebellum & Ataxias
Michael Bielawski, Helen Bondurant
BACKGROUND: There is growing evidence that the cerebellum serves an important role in controlling affect and cognition, and its pathology has been implicated in several psychiatric disorders. Furthermore, the brainstem's role in cognition and affect has been historically overlooked. Neuroimaging studies and an increasing number of case reports indicate cognitive deficits and hallucinatory phenomena after isolated brainstem lesions. CASE PRESENTATION: We report a 56-year-old man who developed persistent persecutory delusions, hallucinations, cognitive impairment and flattened affect following an extensive bilateral cerebellar stroke with involvement of the midbrain...
2015: Cerebellum & Ataxias
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