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multiple system atrophy

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https://www.readbyqxmd.com/read/27922865/additional-value-of-early-phase-18f-fp-cit-pet-image-for-differential-diagnosis-of-atypical-parkinsonism
#1
Soyoung Jin, Minyoung Oh, Seung Jun Oh, Jungsu S Oh, Sang Ju Lee, Sun Ju Chung, Jae Seung Kim
PURPOSE: Regional cerebral perfusion is coupled to metabolism in general. Early perfusion dominant imaging using F-FP-CIT PET (pCIT) may provide complementary information to delayed dopamine transporter dominant images. We investigated the ability of pCIT to differentiate atypical Parkinson disorder from Parkinson disease (PD) compared to FDG and the image quality for optimizing the acquisition time. METHODS: Sixty-seven subjects [PD, 23 subjects; multiple system atrophy-cerebellar type (MSA-C), 27 subjects; MSA-Parkinson type (MSA-P), 12 subjects; and progressive supranuclear palsy (PSP), 5 subjects] underwent F-FP-CIT and FDG PET...
December 3, 2016: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/27916755/-the-nomenclature-and-classification-of-sporadic-spinocerebellar-degeneration
#2
Shunsuke Koga
Spinocerebellar degeneration (SCD) is a neurodegenerative disease characterized by progressive cerebellar ataxia. SCD has a wide range of clinical, pathological, and genetic features, including whether the disease is sporadic or hereditary, and whether it manifests as purely cerebellar or affects multiple systems. Therefore, the classification of SCD has been complicated and has changed over time. Recent advances in genetic testing have shed light on the classification of hereditary SCD. In contrast, the classification of sporadic SCD remains chaotic and there exist nomenclature discrepancies in sporadic SCD between Japanese and English literature...
December 2016: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/27909128/the-development-of-a-quantitative-scoring-system-to-predict-whether-a-large-to-massive-rotator-cuff-tear-can-be-arthroscopically-repaired
#3
S-J Kim, J-S Park, K-H Lee, B-G Lee
AIMS: The aim of the study was to develop a quantitative scoring system to predict whether a large-to-massive rotator cuff tear was arthroscopically reparable prior to surgery. PATIENTS AND METHODS: We conducted a retrospective review of the pre-operative MR imaging and surgical records of 87 patients (87 shoulders) who underwent arthroscopic repair of a large-to-massive rotator cuff tear. Patients were divided into two groups, based on the surgical outcome of the repair...
December 2016: Bone & Joint Journal
https://www.readbyqxmd.com/read/27900498/central-hemodynamics-and-arterial-stiffness-in-idiopathic-and-multiple-system-atrophy
#4
Klaas Franzen, Sabine Fliegen, Jelena Koester, Rafael Campos Martin, Günther Deuschl, Michael Reppel, Kai Mortensen, Susanne A Schneider
Blood pressure is commonly abnormal in parkinsonian disorders, but central hemodynamics and arterial stiffness, well-established predictors of total cardiovascular risk, have rarely been studied in these disorders. 32 patients [27 with idiopathic Parkinson's disease (iPD); 5 with multiple system atrophy (MSA)] and 15 controls matched for cardiac risk factors underwent 24 h-ambulatory blood pressure recordings using an I.E.M. device (Mobil-O-Graph™), measuring peripheral pressure and calculating central pressures and arterial stiffness...
November 29, 2016: Journal of Neurology
https://www.readbyqxmd.com/read/27898200/transcranial-sonography-in-idiopathic-rem-sleep-behavior-disorder-and-multiple-system-atrophy
#5
Xudong Li, Shuang Xue, Shuhong Jia, Zhi Zhou, Yanan Qiao, Chunlei Hou, Kun Wei, Wenjing Zheng, Pei Rong, Jinsong Jiao
AIMS: We investigated preclinical abnormalities revealed by transcranial sonography (TCS) in patients with idiopathic REM sleep behavior disorder (iRBD) compared with those revealed in patients with multiple system atrophy (MSA) or Parkinson's disease (PD) and in normal controls. METHODS: Twenty-two patients with iRBD, 21 patients with MSA, 22 patients with PD and 21 normal controls were included in this study. All participants underwent one night of video-polysomnography (PSG) monitoring, and the sleep parameters were analyzed using Polysmith software and by visual analysis...
November 29, 2016: Psychiatry and Clinical Neurosciences
https://www.readbyqxmd.com/read/27896490/cerebrospinal-fluid-levels-of-neurofilament-light-chain-in-multiple-system-atrophy-relative-to-parkinson-s-disease-a-meta-analysis
#6
Xiaohui Hu, Yan Yang, Daokai Gong
As a biomarker of axonal injury, neurofilament light chain (NFL) in multiple system atrophy (MSA) patients and Parkinson's disease (PD) patients has been investigated by numerous studies. However, cerebrospinal fluid (CSF) NFL changes are conflicting in MSA patients relative to PD patients to date. Therefore, the current study was carried out to find out possible heterogeneity sources. Furthermore, "Neurofilament", "Neurofilament light chain" and "Multiple system atrophy" were employed to search "PubMed", "Springer" and "Medline" databases until August 2016 with standard mean difference (Std...
November 28, 2016: Neurological Sciences
https://www.readbyqxmd.com/read/27894118/nutritional-status-and-changes-in-body-weight-in-patients-with-multiple-system-atrophy
#7
Tomoe Sato, Maho Shiobara, Masatoyo Nishizawa, Takayoshi Shimohata
INTRODUCTION: The importance of nutritional management in neurodegenerative diseases is increasingly recognized in the clinical setting. However, few reports have examined the nutritional intake in patients with multiple system atrophy (MSA). Here, we investigated changes in daily caloric intake, body mass index (BMI) and nutritional status during the disease course of MSA. METHODS: We performed a single-hospital study of 82 consecutive patients with probable MSA according to the consensus criteria...
November 29, 2016: European Neurology
https://www.readbyqxmd.com/read/27894006/distinct-patterns-of-imprecise-consonant-articulation-among-parkinson-s-disease-progressive-supranuclear-palsy-and-multiple-system-atrophy
#8
Tereza Tykalova, Jan Rusz, Jiri Klempir, Roman Cmejla, Evzen Ruzicka
Distinct speech characteristics that may aid in differentiation between Parkinson's disease (PD), progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) remain tremendously under-explored. Here, the patterns and degree of consonant articulation deficits across voiced and voiceless stop plosives in 16 PD, 16 PSP, 16 MSA and 16 healthy control speakers were evaluated using acoustic and perceptual methods. Imprecise consonant articulation was observed across all Parkinsonian groups. Voice onset time of voiceless plosives was more prolonged in both PSP and MSA compared to PD, presumably due to greater severity of dysarthria and slower articulation rate...
November 25, 2016: Brain and Language
https://www.readbyqxmd.com/read/27893883/use-of-magnetic-resonance-imaging-to-visualize-leptomeningeal-inflammation-in-patients-with-multiple-sclerosis-a-review
#9
Jonathan Zurawski, Hans Lassmann, Rohit Bakshi
Importance: Multiple sclerosis (MS) is a chronic demyelinating disease of the central nervous system traditionally characterized by an initial relapsing-remitting clinical course and focal inflammatory lesions that have a predilection for the periventricular white matter. Recently, however, histopathologic and imaging studies have illustrated a more complex pathologic substrate involving cortical demyelination, gray matter atrophy, and meningeal inflammation. Neuroimaging advances have facilitated improved detection of cortical pathology, but our understanding of the pathogenesis of cortical disease remains incomplete...
November 28, 2016: JAMA Neurology
https://www.readbyqxmd.com/read/27891744/inducing-speech-errors-in-dysarthria-using-tongue-twisters
#10
Heather Kember, Kathryn Connaghan, Rupal Patel
Although tongue twisters have been widely use to study speech production in healthy speakers, few studies have employed this methodology for individuals with speech impairment. The present study compared tongue twister errors produced by adults with dysarthria and age-matched healthy controls. Eight speakers (four female, four male; mean age = 54.5 years) with spastic (mixed-spastic) dysarthria of varying aetiology (cerebral palsy, multiple sclerosis, multiple system atrophy) and eight controls (four female, four male; mean age = 56...
November 27, 2016: International Journal of Language & Communication Disorders
https://www.readbyqxmd.com/read/27890451/sniffing-the-diagnosis-olfactory-testing-in-neurodegenerative-parkinsonism
#11
F Krismer, B Pinter, C Mueller, P Mahlknecht, M Nocker, E Reiter, A Djamshidian-Tehrani, S M Boesch, G K Wenning, C Scherfler, W Poewe, K Seppi
OBJECTIVE: To determine the diagnostic utility of olfactory testing in patients with neurodegenerative parkinsonism. METHODS: The Sniffin' Sticks test battery for assessment of odor identification, discrimination, and threshold was applied to patients with Parkinson's disease (PD), multiple system atrophy (MSA) and progressive supranuclear palsy (PSP) as well as healthy controls (HC). Two different cohorts were analyzed: A PD/healthy control that included PD patients and HC as well as a PD/diseased control cohort for which patients PD, MSA and PSP were recruited...
November 21, 2016: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/27881095/a-regional-consensus-recommendation-on-brain-atrophy-as-an-outcome-measure-in-multiple-sclerosis
#12
Raed Alroughani, Dirk Deleu, Khalid El Salem, Jasem Al-Hashel, K John Alexander, Mohamed Assem Abdelrazek, Adel Aljishi, Jaber Alkhaboori, Faisal Al Azri, Nahida Al Zadjali, Majed Hbahbih, Tag Eldin Sokrab, Mohamed Said, Àlex Rovira
BACKGROUND: Multiple sclerosis (MS) is a chronic autoimmune disease characterized by inflammatory and neurodegenerative processes leading to irreversible neurological impairment. Brain atrophy occurs early in the course of the disease at a rate greater than the general population. Brain volume loss (BVL) is associated with disability progression and cognitive impairment in patients with MS; hence its value as a potential target in monitoring and treating MS is discussed. METHODS: A group of MS neurologists and neuro-radiologists reviewed the current literature on brain atrophy and discussed the challenges in assessing and implementing brain atrophy measurements in clinical practice...
November 24, 2016: BMC Neurology
https://www.readbyqxmd.com/read/27878764/the-involvement-of-supratentorial-white-matter-in-multiple-system-atrophy-a-diffusion-tensor-imaging-tractography-study
#13
Po-Shan Wang, Chien-Li Yeh, Chia-Feng Lu, Hsiu-Mei Wu, Bing-Wen Soong, Yu-Te Wu
It has been assumed that cognitive disorder and visual-spatial disturbance in multiple system atrophy of the predominantly cerebellar type (MSA-C) are attributable to degradation of cerebellar function. The purpose of this study was to use diffusion tensor imaging (DTI) tractography to determine if patients with MSA-C characterized in part by visual-spatial disorders and cognitive disorders have changes of the structural connectivity network of nerve fibers, and to further describe the structural connectivity network...
November 23, 2016: Acta Neurologica Belgica
https://www.readbyqxmd.com/read/27878137/mitochondrial-cytopathy-with-common-melas-mutation-presenting-as-multiple-system-atrophy-mimic
#14
Anne-Katrin Pröbstel, André Schaller, Johanna Lieb, Juergen Hench, Stephan Frank, Peter Fuhr, Ludwig Kappos, Michael Sinnreich
Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome(1) is one of the most frequently inherited mitochondrial disorders. MELAS syndrome is a systemic disease with multiple organ involvement.(2) The most common mutation in MELAS is the m.3243A>G mutation in the MT-TL1 gene.(2).
December 2016: Neurology. Genetics
https://www.readbyqxmd.com/read/27875637/ambra1-a-novel-%C3%AE-synuclein-binding-protein-is-implicated-in-the-pathogenesis-of-multiple-system-atrophy
#15
Yasuo Miki, Kunikazu Tanji, Fumiaki Mori, Yota Tatara, Jun Utsumi, Hidenao Sasaki, Akiyoshi Kakita, Hitoshi Takahashi, Gian Maria Fimia, Koichi Wakabayashi
The accumulation of abnormal α-synuclein is the major histopathological feature of Lewy body disease and multiple system atrophy (MSA), which are referred to as synucleinopathies. Although the pathomechanisms underlying synucleinopathies are still unknown, mounting evidence indicates that cytoplasmic degradation systems, such as the autophagy-lysosome and proteasome pathways, are strongly involved in their pathogenesis. Autophagy is tightly regulated by several upstream proteins including UNC-51-like kinase 1/2, beclin1, vacuolar protein sorting-associated protein 34, and autophagy/beclin1 regulator 1 (AMBRA1)...
November 22, 2016: Brain Pathology
https://www.readbyqxmd.com/read/27869324/overlaps-between-multiple-system-atrophy-and-multiple-sclerosis-a-novel-perspective
#16
REVIEW
Kurt A Jellinger, Gregor K Wenning
No abstract text is available yet for this article.
November 21, 2016: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/27866117/distinguishing-spinocerebellar-ataxia-with-pure-cerebellar-manifestation-from-multiple-system-atrophy-msa-c-through-saccade-profiles
#17
Yasuo Terao, Hideki Fukuda, Shin-Ichi Tokushige, Satomi Inomata-Terada, Akihiro Yugeta, Masashi Hamada, Yoshikazu Ugawa
OBJECTIVE: Patients with spinocerebellar ataxia with pure cerebellar presentation (SCD) and multiple system atrophy (MSA-C) show similar symptoms at early stages, although cerebellofugal pathology predominates in SCD, and cerebellopetal pathology in MSA-C. We studied whether saccade velocity profiles, which reflect the accelerating and braking functions of the cerebellum, can differentiate these two disorders. METHODS: We recorded visually guided (VGS) and memory guided saccades (MGS) in 29 MSA-C patients, 12 SCD patients, and 92 age-matched normal subjects, and compared their amplitude, peak velocity and duration (accelerating and decelerating phases)...
October 29, 2016: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/27864848/age-related-small-vessel-disease-a-potential-contributor-to-neurodegeneration-in-multiple-sclerosis
#18
REVIEW
Ruth Geraldes, Margaret M Esiri, Gabriele C DeLuca, Jacqueline Palace
Multiple sclerosis (MS) is a chronic inflammatory demyelinating disorder of the central nervous system wherein, after an initial phase of transient neurological defects, slow neurological deterioration due to progressive neuronal loss ensues. Age is a major determinant of MS progression onset and disability. Over the past years, several mechanisms have been proposed to explain the key drivers of neurodegeneration and disability accumulation in MS. However, the effect of commonly encountered age-related cerebral vessel disease, namely small vessel disease (SVD), has been largely neglected and constitutes the aim of this review...
November 19, 2016: Brain Pathology
https://www.readbyqxmd.com/read/27861715/the-value-of-the-bulbocavernosus-reflex-and-pudendal-nerve-somatosensory-evoked-potentials-in-distinguishing-between-multiple-system-atrophy-and-parkinson-s-disease-at-an-early-stage
#19
Z-Y Cai, X-T Niu, J Pan, P-Q Ni, X Wang, B Shao
OBJECTIVES: This study was designed to investigate the clinical value of the bulbocavernosus reflex (BCR) and pudendal nerve somatosensory evoked potentials (PSEPs) in the differential diagnosis between multiple system atrophy (MSA) and Parkinson's disease (PD) in early stage. MATERIALS AND METHODS: A total of 31 patients with MSA, 45 patients with PD, and 60 healthy participants were included in this study. A Keypoint EMG/EP system was used for BCR and PSEP measurements...
November 10, 2016: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/27861346/unusual-phenotype-of-pathologically-confirmed-progressive-supranuclear-palsy-with-autonomic-dysfunction-and-cerebellar-ataxia-case-report
#20
Katerina Mensikova, Lucie Tuckova, Jiri Ehrmann, Petr Kanovsky
BACKGROUND: Based on the results of recent multicenter clinical-pathological studies, it seems that the clinical heterogeneity of progressive supranuclear palsy (PSP) is much broader than previously thought. We will report 2 cases of patients with unusual manifestation of pathologically confirmed PSP. METHODS: Two female patients were diagnosed with the parkinsonian phenotype of multiple system atrophy (MSAP) according to current clinical diagnostic criteria at the ages of 55 and 60 years, respectively...
November 2016: Medicine (Baltimore)
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