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Olivopontocerebellar Atrophy

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Genes to treat excitotoxicity ameliorate the symptoms of the disease in mice models of multiple system atrophy.

Micaela Johanna Glat, Nadia Stefanova, Gregor Karl Wenning, Daniel Offen,

Multiple system atrophy (MSA) is a sporadic neurodegenerative disorder characterized by striatonigral degeneration and olivopontocerebellar atrophy. The main hallmark of MSA is the aggregation of alpha-synuclein in oligodendrocytes, which contributes to the dysfunction and death of the oligodendrocytes, followed by neurodegeneration. Studies suggested that oxidative-excitatory pathway is associated with the ... Read more >>

J Neural Transm (Vienna) (Journal of neural transmission (Vienna, Austria : 1996))
[2020, 127(2):205-212]

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Neuroimaging Biomarkers in SCA2 Gene Carriers.

Mario Mascalchi, Alessandra Vella,

A variety of Magnetic Resonance (MR) and nuclear medicine (NM) techniques have been used in symptomatic and presymptomatic SCA2 gene carriers to explore,in vivo, the physiopathological biomarkers of the neurological dysfunctions characterizing the associated progressive disease that presents with a cerebellar syndrome, or less frequently, with a levodopa-responsive parkinsonian syndrome. ... Read more >>

Int J Mol Sci (International journal of molecular sciences)
[2020, 21(3):]

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Supratentorial and Infratentorial Lesions in Spinocerebellar Ataxia Type 3.

Po-Shan Wang, Yu-Te Wu, Tzu-Yun Wang, Hsiu-Mei Wu, Bing-Wen Soong, Chi-Wen Jao,

Background: Spinocerebellar ataxia type 3 (SCA) is a cerebellum-dominant degenerative disorder that is characterized primarily by infratentorial damage, although less severe supratentorial involvement may contribute to the clinical manifestation. These impairments may result from the efferent loss of the cerebellar cortex and degeneration of the cerebral cortex. Method: We used ... Read more >>

Front Neurol (Frontiers in Neurology)
[2020, 11:124]

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Multiple system atrophy.

Alessandra Fanciulli, Iva Stankovic, Florian Krismer, Klaus Seppi, Johannes Levin, Gregor K Wenning,

Multiple system atrophy (MSA) is a sporadic, adult-onset, relentlessly progressive neurodegenerative disorder, clinically characterized by various combinations of autonomic failure, parkinsonism and ataxia. The neuropathological hallmark of MSA are glial cytoplasmic inclusions consisting of misfolded α-synuclein. Selective atrophy and neuronal loss in striatonigral and olivopontocerebellar systems underlie the division into ... Read more >>

Int. Rev. Neurobiol. (International review of neurobiology)
[2019, 149:137-192]

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What is the best way to keep walking and moving around for individuals with Machado-Joseph disease? A scoping review through the lens of Aboriginal families with Machado-Joseph disease in the Top End of Australia.

Jennifer J Carr, Joyce Lalara, Gayangwa Lalara, Moira Smith, Jennifer Quaill, Alan R Clough, Anne Lowell, Ruth N Barker,

OBJECTIVES:Machado-Joseph disease (MJD) is the most common spinocerebellar ataxia worldwide. Prevalence is highest in affected remote Aboriginal communities of the Top End of Australia. Aboriginal families with MJD from Groote Eylandt believe 'staying strong on the inside and outside' works best to keep them walking and moving around, in accordance ... Read more >>

BMJ Open (BMJ open)
[2019, 9(9):e032092]

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Epigallocatechin gallate in multiple system atrophy (PROMESA).

Kurt A Jellinger,

Ann Transl Med (Annals of translational medicine)
[2019, 7(Suppl 8):S278]

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Neuroradiological Findings in the Spinocerebellar Ataxias.

Alex Tiburtino Meira, Walter Oleschko Arruda, Sergio Eiji Ono, Arnolfo de Carvalho Neto, Salmo Raskin, Carlos Henrique F Camargo, Hélio Afonso G Teive,

Background:The spinocerebellar ataxias (SCAs) are a group of autosomal dominant degenerative diseases characterized by cerebellar ataxia. Classified according to gene discovery, specific features of the SCAs - clinical, laboratorial, and neuroradiological (NR) - can facilitate establishing the diagnosis. The purpose of this study was to review the particular NR abnormalities ... Read more >>

Tremor Other Hyperkinet Mov (N Y) (Tremor and other hyperkinetic movements (New York, N.Y.))
[2019, 9:]

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Pathological changes in the cerebellum of patients with multiple system atrophy and Parkinson's disease-a stereological study.

Elisabeth H L Rusholt, Lisette Salvesen, Tomasz Brudek, Betel Tesfay, Bente Pakkenberg, Mikkel V Olesen,

Multiple system atrophy (MSA) and Parkinson's disease (PD) are synucleinopathies characterized by aggregation of α-synuclein in brain cells. Recent studies have shown that morphological changes in terms of cerebral nerve cell loss and increase in glia cell numbers, the degree of brain atrophy and molecular and epidemiological findings are more ... Read more >>

Brain Pathol. (Brain pathology (Zurich, Switzerland))
[2019, :]

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A case of multiple system atrophy.

Jing Guo, Fuying Liu, Tingting Liu, Xin Zhang, Yong Luo,

J. Int. Med. Res. (The Journal of international medical research)
[2019, 47(11):5839-5843]

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Hereditary Disorders Mimicking Progressive Multiple Sclerosis.

Zerrin Karaaslan,

Noro Psikiyatr Ars (Noro psikiyatri arsivi)
[2019, 56(1):1-2]

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Developmental hypomyelination in Wolfram syndrome: new insights from neuroimaging and gene expression analyses.

Amjad Samara, Rachel Rahn, Olga Neyman, Ki Yun Park, Ahmad Samara, Bess Marshall, Joseph Dougherty, Tamara Hershey,

Wolfram syndrome is a rare multisystem disorder caused by mutations in WFS1 or CISD2 genes leading to brain structural abnormalities and neurological symptoms. These abnormalities appear in early stages of the disease. The pathogenesis of Wolfram syndrome involves abnormalities in the endoplasmic reticulum (ER) and mitochondrial dynamics, which are common ... Read more >>

Orphanet J Rare Dis (Orphanet journal of rare diseases)
[2019, 14(1):279]

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Multiple System Atrophy With Predominant Striatonigral Degeneration and TAR DNA-Binding Protein of 43 kDa Pathology: An Unusual Variant of Multiple System Atrophy.

Lynda Nwabuobi, Darya Tomishon, Neil A Shneider, Stanley Fahn, Jean Paul Vonsattel, Etty Cortes,

Background:The pathological hallmark in MSA is oligodendrocytic glial cytoplasmic inclusions (GCIs) containing α-synuclein, in addition to neuronal loss and astrogliosis especially involving the striatonigral and olivopontocerebellar systems. Rarely, TAR DNA-binding protein of 43 kDa (TDP-43), a component of ubiquitinated inclusions observed mainly in amyotrophic lateral sclerosis and frontotemporal lobar degeneration ... Read more >>

Mov Disord Clin Pract (Movement disorders clinical practice)
[2019, 6(8):661-666]

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Systemic lupus erythematosus in a patient with multiple system atrophy: A case report.

Dimitrios Pilalas, Georgios Chatzopoulos, Georgia D Kaiafa, Eleftheria Ztriva, Soultana Spyridonakou, Vasiliki Bisbinas, Panagiotis Ioannidis, Apostolos I Hatzitolios, Christos Savopoulos,

RATIONALE:Multiple system atrophy is a late-onset rare neurodegenerative movement disorder which results in debilitating disease. Fever frequently ensues in the context of infections which can be associated with significant morbidity and mortality, but among alternative diagnostic possibilities neoplasms and autoimmune disorders should be considered. PATIENT CONCERNS:We describe a case of ... Read more >>

Medicine (Baltimore) (Medicine)
[2019, 98(46):e18005]

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High-salt diet does not boost neuroinflammation and neurodegeneration in a model of α-synucleinopathy.

Antonio Heras-Garvin, Violetta Refolo, Markus Reindl, Gregor K Wenning, Nadia Stefanova,

AIM:Pre-clinical studies in models of multiple sclerosis and other inflammatory disorders suggest that high-salt diet may induce activation of the immune system and potentiate inflammation. However, high-salt diet constitutes a common non-pharmacological intervention to treat autonomic problems in synucleinopathies such as Parkinson's disease and multiple system atrophy. Since neuroinflammation plays ... Read more >>

J Neuroinflammation (Journal of neuroinflammation)
[2020, 17(1):35]

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Insights into the pathogenesis of multiple system atrophy: focus on glial cytoplasmic inclusions.

Seiji Kaji, Takakuni Maki, Tomoyuki Ishimoto, Hodaka Yamakado, Ryosuke Takahashi,

Multiple system atrophy (MSA) is a debilitating and fatal neurodegenerative disorder. The disease severity warrants urgent development of disease-modifying therapy, but the disease pathogenesis is still enigmatic. Neurodegeneration in MSA brains is preceded by the emergence of glial cytoplasmic inclusions (GCIs), which are insoluble α-synuclein accumulations within oligodendrocytes (OLGs). Thus, ... Read more >>

Transl Neurodegener (Translational neurodegeneration)
[2020, 9:7]

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Young-onset multiple system atrophy: Its rarity and heterogeneity.

Han-Joon Kim, Beomseok Jeon,

Mov. Disord. (Movement disorders : official journal of the Movement Disorder Society)
[2019, 34(7):1085-1086]

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Sarcomeric disorganization and nemaline bodies in muscle biopsies of patients with EXOSC3-related type 1 pontocerebellar hypoplasia.

Miguel M Pinto, Soledad Monges, Edoardo Malfatti, Fabiana Lubieniecki, Xavière Lornage, Laura Alias, Clémence Labasse, Angéline Madelaine, Michel Fardeau, Jocelyn Laporte, Eduardo F Tizzano, Norma B Romero,

INTRODUCTION:Mutations in the EXOSC3 gene are responsible for type 1 pontocerebellar hypoplasia, an autosomal recessive congenital disorder characterized by cerebellar atrophy, developmental delay, and anterior horn motor neuron degeneration. Muscle biopsies of these patients often show characteristics resembling classic spinal muscle atrophy, but to date, no distinct features have been ... Read more >>

Muscle Nerve (Muscle & nerve)
[2019, 59(1):137-141]

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New Radiologic Findings of Hypertrophic Olivary Degeneration in 2 Patients with Brainstem Lymphoma.

Sumito Sato, Ichiyo Shibahara, Yusuke Inoue, Takuichiro Hide, Toshihiro Kumabe,

BACKGROUND:Hypertrophic olivary degeneration (HOD) is a rare neurological condition of trans-synaptic degeneration caused by disruption of the dentatorubro-olivary pathway. We present new radiologic findings of HOD in 2 cases of brainstem lymphoma. CASE DESCRIPTION:A 35-year-old woman (Case 1) and a 69-year-old man (Case 2) presented with remarkably similar clinical courses. ... Read more >>

World Neurosurg (World neurosurgery)
[2019, 123:464-468.e1]

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Neuroimaging Applications in Chronic Ataxias.

Mario Mascalchi, Alessandra Vella,

Magnetic resonance imaging (MRI), single photon emission computed tomography (SPECT) and positron emission tomography (PET) are the main instruments for neuroimaging investigation of patients with chronic ataxia. MRI has a predominant diagnostic role in the single patient, based on the visual detection of three patterns of atrophy, namely, spinal atrophy, ... Read more >>

Int. Rev. Neurobiol. (International review of neurobiology)
[2018, 143:109-162]

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Differentiation of multiple system atrophy from Parkinson's disease by structural connectivity derived from probabilistic tractography.

Alexandra Abos, Hugo C Baggio, Barbara Segura, Anna Campabadal, Carme Uribe, Darly Milena Giraldo, Alexandra Perez-Soriano, Esteban Muñoz, Yaroslau Compta, Carme Junque, Maria Jose Marti,

Recent studies combining diffusion tensor-derived metrics and machine learning have shown promising results in the discrimination of multiple system atrophy (MSA) and Parkinson's disease (PD) patients. This approach has not been tested using more complex methodologies such as probabilistic tractography. The aim of this work is assessing whether the strength ... Read more >>

Sci Rep (Scientific reports)
[2019, 9(1):16488]

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Phosphorylated Alpha-Synuclein in Red Blood Cells as a Potential Diagnostic Biomarker for Multiple System Atrophy: A Pilot Study.

Xu-Ying Li, Weiwei Yang, Xin Li, Xu-Ran Li, Wei Li, Qihan Song, Linjuan Sun, Feng Lin, Zhigang Chen, Chaodong Wang, Shun Yu,

Diagnosis of multiple system atrophy (MSA) remains a challenge, due to the complexity and overlapping of its symptoms with other Parkinsonian disorders. The critical role of alpha-synuclein (α-syn) in the pathogenesis of MSA makes it an ideal biomarker for the diagnosis of MSA. Although α-syn alterations in cerebrospinal fluid (CSF) ... Read more >>

Parkinsons Dis (Parkinson's disease)
[2020, 2020:8740419]

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Morphometric MRI profiles of multiple system atrophy variants and implications for differential diagnosis.

Florian Krismer, Klaus Seppi, Georg Göbel, Ruth Steiger, Isabel Zucal, Sylvia Boesch, Elke R Gizewski, Gregor K Wenning, Werner Poewe, Christoph Scherfler,

BACKGROUND:Manual width measurements of the middle cerebellar peduncle on MRI were shown to improve the accuracy of an imaging-guided diagnosis of multiple system atrophy (MSA). Recently, automated volume segmentation algorithms were able to reliably differentiate patients with Parkinson's disease (PD) and the parkinsonian variant of MSA. The objective of the ... Read more >>

Mov. Disord. (Movement disorders : official journal of the Movement Disorder Society)
[2019, 34(7):1041-1048]

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Hypertrophic Olivary Degeneration Following Listerial Rhombencephalitis.

Seong-Il Oh,

Can J Neurol Sci (The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques)
[2018, 45(5):593-595]

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"Minimal change" multiple system atrophy with limbic-predominant α-synuclein pathology.

Shunsuke Koga, Dennis W Dickson,

Acta Neuropathol. (Acta neuropathologica)
[2019, 137(1):167-169]

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The role of the cerebellum in degenerative ataxias and essential tremor: Insights from noninvasive modulation of cerebellar activity.

Roderick P P W M Maas, Rick C G Helmich, Bart P C van de Warrenburg,

Over the last three decades, measuring and modulating cerebellar activity and its connectivity with other brain regions has become an emerging research topic in clinical neuroscience. The most important connection is the cerebellothalamocortical pathway, which can be functionally interrogated using a paired-pulse transcranial magnetic stimulation paradigm. Cerebellar brain inhibition reflects ... Read more >>

Mov. Disord. (Movement disorders : official journal of the Movement Disorder Society)
[2020, 35(2):215-227]

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