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multiple system atrophy

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https://www.readbyqxmd.com/read/29776876/-efficacy-of-botulinum-toxin-a-injections-in-the-urethral-sphincter-in-patients-with-difficulties-to-perform-self-intermittent-catherization
#1
T Honore, F Le Breton, N Turmel, B Bignani, C Chesnel, A Charlanes, G Amarenco
PURPOSE: To evaluate safety and efficacy of botulinum toxin A injections in the urethral striated sphincter in patients with difficulties to perform self-intermittent catherization. METHODS: In this prospective study, 12 patients suffering from upper motor neuron diseases (8 multiple sclerosis, 2 myelitis, 1 brain injury, 1 multi system atrophy) and with difficulties to perform self-intermittent catherization, defined by a ICDQ score>1 (Intermittent Catheterization Difficulty Questionnaire) have had injections of 100U BOTOX® under EMG guidance in the urethral striated sphincter, for a total of 15 injections...
May 15, 2018: Progrès en Urologie
https://www.readbyqxmd.com/read/29774142/comparison-of-mri-and-123-i-fp-cit-spect-for-the-evaluation-of-msa-p-clinical-severity
#2
Miki Nishimori, Yoriko Murata, Hitomi Iwasa, Kana Miyatake, Michiko Tadokoro, Shino Kohsaki, Munenobu Nogami, Norihiko Hamada, Hitoshi Ninomiya, Yasushi Osaki, Hirokazu Furuya, Takuji Yamagami
The aim of the present study was to compare the efficacy of magnetic resonance imaging (MRI) and 123 I-labeled 2β-carbomethoxy-3β-(4-iodophenyl)-N-(3-fluoropropyl)nortropane single photon emission computed tomography (123 I-FP-CIT SPECT) for determining the clinical severity of patients with multiple system atrophy with Parkinsonism (MSA-P). MRI and 123 I-FP-CIT SPECT images from 17 patients with MSA-P as diagnosed using the Unified MSA Rating Scale part IV (UMSARS IV) score were compared. Brain MRI scans were available for all 17 patients and 123 I-FP-CIT SPECT images were available for 12 patients...
June 2018: Biomedical Reports
https://www.readbyqxmd.com/read/29765223/efficacy-of-denosumab-in-two-cases-with-multiple-system-atrophy-and-osteoporosis
#3
Masashi Uehara, Yukio Nakamura, Jun Takahashi, Takako Suzuki, Hiroyuki Kato
Background: Multiple-system atrophy (MSA) is an α-synucleinopathy with a very aggressive course and poor prognosis, which lacks efficient treatment. Thus, MSA represents a serious health and social problem. Progressive stridor and acute laryngeal obstruction likely occur in MSA; however, little is known about the bone metabolism or efficacy of bone absorption drugs, such as denosumab, in osteoporosis with MSA. Case presentation and methods: Two male patients with osteoporosis in MSA presented to our institution (at 54 and 68 years of age)...
2018: Therapeutics and Clinical Risk Management
https://www.readbyqxmd.com/read/29764386/parkinson-s-disease-diagnostic-observations-paddo-study-rationale-and-design-of-a-prospective-cohort-study-for-early-differentiation-of-parkinsonism
#4
Anouke van Rumund, Marjolein B Aerts, Rianne A J Esselink, Frederick J A Meijer, Marcel M Verbeek, Bastiaan R Bloem
BACKGROUND: Differentiation of Parkinson's disease (PD) from the various types of atypical parkinsonism (AP) such as multiple system atrophy (MSA), progressive supranuclear palsy (PSP), dementia with Lewy bodies (DLB), corticobasal syndrome (CBS) and vascular parkinsonism (VP), can be challenging, especially early in the disease course when symptoms overlap. A major unmet need in the diagnostic workup of these disorders is a diagnostic tool that differentiates the various disorders, preferably in the earliest disease stages when the clinical presentation is similar...
May 16, 2018: BMC Neurology
https://www.readbyqxmd.com/read/29755341/plasma-biomarkers-differentiate-parkinson-s-disease-from-atypical-parkinsonism-syndromes
#5
Chin-Hsien Lin, Shieh-Yueh Yang, Herng-Er Horng, Che-Chuan Yang, Jen-Jie Chieh, Hsin-Hsien Chen, Bing-Hsien Liu, Ming-Jang Chiu
Objective: Parkinson's disease (PD) has significant clinical overlaps with atypical parkinsonism syndromes (APS), which have a poorer treatment response and a more aggressive course than PD. We aimed to identify plasma biomarkers to differentiate PD from APS. Methods: Plasma samples ( n = 204) were obtained from healthy controls and from patients with PD, dementia with Lewy bodies (DLB), multiple system atrophy, progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), or frontotemporal dementia (FTD) with parkinsonism (FTD-P) or without parkinsonism...
2018: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/29746401/progressive-supranuclear-palsy-and-multiple-system-atrophy-clinicopathological-concepts-and-therapeutic-challenges
#6
Gesine Respondek, Johannes Levin, Günter U Höglinger
PURPOSE OF REVIEW: This update discusses novel aspects on clinicopathological concepts and therapeutic challenges in progressive supranuclear palsy (PSP) and multiple system atrophy (MSA), arising from publications of the last 1.5 years. RECENT FINDINGS: The clinical criteria for diagnosis of PSP have been revised. Clinical variability of pathologically defined PSP and MSA makes the development of mature biomarkers for early diagnosis and biomarker-based trial design indispensable...
May 8, 2018: Current Opinion in Neurology
https://www.readbyqxmd.com/read/29744624/a-rare-case-of-paediatric-primary-central-nervous-system-lymphoma-treated-with-high-dose-methotrexate-and-rituximab-based-chemoimmunotherapy-and-whole-brain-radiotherapy-followed-by-tumour-bed-boost-with-three-dimensional-conformal-radiation-technique
#7
Narayan Adhikari, Ahitagni Biswas, Sameer Bakhshi, Gaurav Khanna, Vaishali Suri
BACKGROUND: Primary central nervous system lymphomas (PCNSL) are rare in the paediatric population. CLINICAL CASE: A 12-year-old boy presented to our clinic with complaints of multiple episodes of generalised tonic-clonic seizures for 1 year and gradual loss of vision in both eyes for 3 months. Baseline magnetic resonance imaging (MRI) of the brain showed a large (7.2 × 7 cm) enhancing soft tissue lesion in the right frontal lobe causing mass effect and midline shift...
May 9, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29744576/the-lysosomal-function-of-progranulin-a-guardian-against-neurodegeneration
#8
REVIEW
Daniel H Paushter, Huan Du, Tuancheng Feng, Fenghua Hu
Progranulin (PGRN), encoded by the GRN gene in humans, is a secreted growth factor implicated in a multitude of processes ranging from regulation of inflammation to wound healing and tumorigenesis. The clinical importance of PGRN became especially evident in 2006, when heterozygous mutations in the GRN gene, resulting in haploinsufficiency, were found to be one of the main causes of frontotemporal lobar degeneration (FTLD). FTLD is a clinically heterogenous disease that results in the progressive atrophy of the frontal and temporal lobes of the brain...
May 9, 2018: Acta Neuropathologica
https://www.readbyqxmd.com/read/29743672/cellular-milieu-imparts-distinct-pathological-%C3%AE-synuclein-strains-in-%C3%AE-synucleinopathies
#9
Chao Peng, Ronald J Gathagan, Dustin J Covell, Coraima Medellin, Anna Stieber, John L Robinson, Bin Zhang, Rose M Pitkin, Modupe F Olufemi, Kelvin C Luk, John Q Trojanowski, Virginia M-Y Lee
In Lewy body diseases-including Parkinson's disease, without or with dementia, dementia with Lewy bodies, and Alzheimer's disease with Lewy body co-pathology 1 -α-synuclein (α-Syn) aggregates in neurons as Lewy bodies and Lewy neurites 2 . By contrast, in multiple system atrophy α-Syn accumulates mainly in oligodendrocytes as glial cytoplasmic inclusions (GCIs) 3 . Here we report that pathological α-Syn in GCIs and Lewy bodies (GCI-α-Syn and LB-α-Syn, respectively) is conformationally and biologically distinct...
May 9, 2018: Nature
https://www.readbyqxmd.com/read/29741613/lysosomal-storage-disorder-gene-variants-in-multiple-system-atrophy
#10
Lasse Pihlstrøm, Lucia Schottlaender, Viorica Chelban, Wassilios G Meissner, Monica Federoff, Andy Singleton, Henry Houlden
No abstract text is available yet for this article.
May 7, 2018: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29741598/reply-lysosomal-storage-disorder-gene-variants-in-multiple-system-atrophy
#11
Joshua M Shulman
No abstract text is available yet for this article.
May 7, 2018: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29737427/serum-neurofilament-light-is-increased-in-multiple-system-atrophy-of-cerebellar-type-and-in-repeat-expansion-spinocerebellar-ataxias-a-pilot-study
#12
Carlo Wilke, Friedemann Bender, Stefanie N Hayer, Kathrin Brockmann, Ludger Schöls, Jens Kuhle, Matthis Synofzik
Blood biomarkers in degenerative ataxias are still largely missing. Here, we aimed to provide piloting proof-of-concept that serum Neurofilament light (NfL) could offer a promising peripheral blood biomarker in degenerative ataxias. Specifically, as a marker of neuronal damage, NfL might (1) help to differentiate multiple system atrophy of cerebellar type (MSA-C) from sporadic adult-onset ataxia (SAOA), and (2) show increases in repeat-expansion spinocerebellar ataxias (SCAs) which might be amenable to treatment in the future...
May 8, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29733529/sensor-based-gait-analysis-in-atypical-parkinsonian-disorders
#13
Cecilia Raccagni, Heiko Gaßner, Sabine Eschlboeck, Sylvia Boesch, Florian Krismer, Klaus Seppi, Werner Poewe, Bjoern M Eskofier, Juergen Winkler, Gregor Wenning, Jochen Klucken
BACKGROUND AND OBJECTIVES: Gait impairment and reduced mobility are typical features of idiopathic Parkinson's disease (iPD) and atypical parkinsonian disorders (APD). Quantitative gait assessment may have value in the diagnostic workup of parkinsonian patients and as endpoint in clinical trials. The study aimed to identify quantitative gait parameter differences in iPD and APD patients using sensor-based gait analysis and to correlate gait parameters with clinical rating scales. SUBJECTS AND METHODS: Patients with iPD and APD including Parkinson variant multiple system atrophy and progressive supranuclear palsy matched for age, gender, and Hoehn and Yahr (≤3) were recruited at two Movement Disorder Units and assessed using standardized clinical rating scales (MDS-UPDRS-3, UMSARS, PSP-RS)...
May 7, 2018: Brain and Behavior
https://www.readbyqxmd.com/read/29730092/kolaviron-protects-the-brain-in-cuprizone-induced-model-of-experimental-multiple-sclerosis-via-enhancement-of-intrinsic-antioxidant-mechanisms-possible-therapeutic-applications
#14
Gabriel Olaiya Omotoso, Ileje Inelo Ukwubile, Leviticus Arietarhire, Fatima Sulaimon, Ismail Temitayo Gbadamosi
Multiple sclerosis is a demyelinating condition of the central nervous system which commonly affects young adults. Kolaviron, a biflavonoid isolate of Garcinia kola, has been used in experimental studies which explored its anti-oxidative, anti-inflammatory and anti-genotoxic properties. This work was aimed at unraveling the possible ameliorative effect of kolaviron on cuprizone-induced demyelination in the prefrontal cortices of Wistar rats. A total of 28 adult male Wistar rats were divided into four groups A-D...
April 27, 2018: Pathophysiology: the Official Journal of the International Society for Pathophysiology
https://www.readbyqxmd.com/read/29728329/survival-analysis-and-prognostic-nomogram-model-for-multiple-system-atrophy
#15
Bei Cao, Lingyu Zhang, Yutong Zou, Qianqian Wei, Ruwei Ou, Yongping Chen, Hui-Fang Shang
OBJECTIVE: The purpose of our study was to explore the factors associated with the survival of multiple system atrophy (MSA) patients and to produce a prognostic nomogram to predict survival in an individual MSA patient. METHODS: 220 probable MSA patients were included from 2009 to 2013. Disease severity was measured by the Unified Multiple System Atrophy Rating Scale (UMSARS). The univariate and multivariable Cox regression analyses were used to identify factors associated with survival in MSA patients...
April 22, 2018: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/29724319/-clinicopathological-features-and-treatment-of-renal-impair-in-primary-sj%C3%A3-gren-syndrome
#16
Jing Wang, Li Meng Chen
Primary Sjögren syndrome,characterized by autoimmune epithelitis,is a prevalent systemic autoimmune disease involving multiple organs,among which kidney is a major target organ.Tubulointerstitial lesion is the most frequent form,involving proximal tubule,distal tubule,or collecting duct.The disease has an occult onset and may progressively develop into renal function impairment and end-stage renal disease,which can be accompanied with low-molecular-weight proteinuria,renal tubule acidosis and electrolyte disturbance...
April 28, 2018: Zhongguo Yi Xue Ke Xue Yuan Xue Bao. Acta Academiae Medicinae Sinicae
https://www.readbyqxmd.com/read/29719351/senile-depression-with-somatization-symptoms-and-insomnia-is-diagnosed-as-multiple-system-atrophy-a-case-report
#17
Ling Yue, Hai Yu, Guanjun Li, Shifu Xiao
Patients who have senile depression with somatization symptoms are commonly encountered in clinical practice. The present case reports on a patient with senile depression who was repeatedly hospitalized and had somatic symptoms. Although the patient recovered after the first hospitalization, she suffered from a relapse one year later. As we followed up, due to the neurological findings and the response to treatment, we found that the patient is in line with the diagnoistic criteria for multiple system atrophy (MSA)...
December 25, 2017: Shanghai Archives of Psychiatry
https://www.readbyqxmd.com/read/29715545/multiple-system-atrophy-and-cag-repeat-length-a-genetic-screening-of-polyglutamine-disease-genes-in-italian-patients
#18
Alessia Mongelli, Lidia Sarro, Elena Rizzo, Lorenzo Nanetti, Nicoletta Meucci, Gianni Pezzoli, Stefano Goldwurm, Franco Taroni, Caterina Mariotti, Cinzia Gellera
Multiple system atrophy (MSA) is an adult onset, progressive, neurodegenerative disorder of unknown etiology characterized by autonomic dysfunction, parkinsonism (MSA-P) and cerebellar ataxia (MSA-C). The phenotypic spectrum may present overlapping features with other neurodegenerative diseases, particularly the autosomal dominant inherited polyglutamine disorders. To investigate the possible contribution of CAG expansions in the MSA phenotype, we analyzed the triplet repeat length in the autosomal dominant causative genes for spinocerebellar ataxia (SCA) type 1, 2, 3, 6, 7, 17, dentatorubral-pallidoluysian atrophy (DRPLA) and Huntington disease (HD) in a cohort of 246 Italian MSA patients...
April 28, 2018: Neuroscience Letters
https://www.readbyqxmd.com/read/29714673/measurement-of-whole-brain-and-gray-matter-atrophy-in-multiple-sclerosis-assessment-with-mr-imaging
#19
Loredana Storelli, Maria A Rocca, Elisabetta Pagani, Wim Van Hecke, Mark A Horsfield, Nicola De Stefano, Alex Rovira, Jaume Sastre-Garriga, Jacqueline Palace, Diana Sima, Dirk Smeets, Massimo Filippi
Purpose To compare available methods for whole-brain and gray matter (GM) atrophy estimation in multiple sclerosis (MS) in terms of repeatability (same magnetic resonance [MR] imaging unit) and reproducibility (different system/field strength) for their potential clinical applications. Materials and Methods The softwares ANTs-v1.9, CIVET-v2.1, FSL-SIENAX/SIENA-5.0.1, Icometrix-MSmetrix-1.7, and SPM-v12 were compared. This retrospective study, performed between March 2015 and March 2017, collected data from (a) eight simulated MR images and longitudinal data (2 weeks) from 10 healthy control subjects to assess the cross-sectional and longitudinal accuracy of atrophy measures, (b) test-retest MR images in 29 patients with MS acquired within the same day at different imaging unit field strengths/manufacturers to evaluate precision, and (c) longitudinal data (1 year) in 24 patients with MS for the agreement between methods...
May 27, 2018: Radiology
https://www.readbyqxmd.com/read/29710120/a-histologic-study-of-the-circadian-system-in-parkinson-disease-multiple-system-atrophy-and-progressive-supranuclear-palsy
#20
Eduardo De Pablo-Fernández, Robert Courtney, Thomas T Warner, Janice L Holton
Importance: Circadian dysfunction may be associated with the symptoms and neurodegeneration in Parkinson disease (PD), multiple system atrophy (MSA), and progressive supranuclear palsy (PSP), although the underlying neuroanatomical site of disruption and pathophysiological mechanisms are not fully understood. Objective: To perform a neuropathological analysis of disease-specific inclusions in the key structures of the circadian system in patients with PD, MSA, and PSP...
April 30, 2018: JAMA Neurology
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