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https://www.readbyqxmd.com/read/30107539/deposition-of-phosphorylated-%C3%AE-synuclein-in-the-rtg4510-mouse-model-of-tauopathy
#1
Yuta Takaichi, Yasuhisa Ano, James K Chambers, Kazuyuki Uchida, Akihiko Takashima, Hiroyuki Nakayama
The accumulation of specific phosphorylated protein aggregates in the brain is a hallmark of severe neurodegenerative disorders. Specifically, hyperphosphorylated tau (hp-tau) accumulates in Alzheimer disease, frontotemporal dementia with Parkinsonism linked to chromosome 17, and progressive supranuclear palsy; furthermore, phosphorylated α-synuclein (p-αSyn) accumulates in Parkinson disease, dementia with Lewy bodies, and multiple system atrophy. Moreover, codeposition of different pathological protein aggregates is common in the brains of individuals with neurodegenerative diseases...
July 18, 2018: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/30103064/restless-legs-syndrome-leg-motor-restlessness-and-their-variants-in-patients-with-parkinson-s-disease-and-related-disorders
#2
Takeo Matsubara, Keisuke Suzuki, Hiroaki Fujita, Yuji Watanabe, Hirotaka Sakuramoto, Masanori Matsubara, Koichi Hirata
OBJECTIVE: The objective of this study was to investigate the prevalence of restless leg syndrome (RLS), leg motor restlessness (LMR) and RLS/LMR variants and their relationship with clinical factors in patients with Parkinson's disease (PD) and related disorders. METHODS: Sixty-three PD patients, 17 multiple system atrophy (MSA) patients and 11 progressive supranuclear palsy (PSP) patients were included in this study. Through face-to-face interviews, the patients were diagnosed with RLS/LMR, or with RLS/LMR variants in which the symptoms occur predominantly in body parts other than the legs...
August 7, 2018: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/30093363/clinical-correlates-of-decreased-plasma-coenzyme-q10-levels-in-patients-with-multiple-system-atrophy
#3
Juanjuan Du, Tian Wang, Pei Huang, Shishuang Cui, Chao Gao, Yiqi Lin, Rao Fu, Junyi Shen, Yachao He, Yuyan Tan, Shengdi Chen
INTRODUCTION: Multiple system atrophy (MSA) is a progressive neurodegenerative disease. Recent studies revealed decreased coenzyme Q10 (COQ10) levels in the cerebellum and blood samples of MSA patients. But few studies focused on the associations of COQ10 with the clinical symptoms of MSA. In this study, we aimed to quantify plasma COQ10 and characterize its association with clinical features. METHODS: We recruited 40 patients with MSA, 30 patients with Parkinson's disease (PD), and 30 healthy participants...
July 26, 2018: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/30093262/difference-in-severity-of-sleep-apnea-in-patients-with-rapid-eye-movement-sleep-behavior-disorder-with-or-without-parkinsonism
#4
Dae Lim Koo, Jee Young Lee, Hyunwoo Nam
OBJECTIVE: Rapid eye movement sleep behavior disorder (RBD) is a common sleep disturbance in patients with neurodegenerative disorders. We aimed to compare sleep parameters among the different types of RBD patients. METHODS: A total of 122 patients with dream enactment behavior were screened. Of these, 92 patients who were diagnosed with RBD by polysomnography were included in this study. Enrolled patients with RBD were classified into four groups based on the following diagnoses: idiopathic RBD (iRBD); RBD with Parkinson disease (PD-RBD); multiple system atrophy (MSA) with RBD (MSA-RBD); and dementia with Lewy bodies (DLB) with RBD (DLB-RBD)...
June 28, 2018: Sleep Medicine
https://www.readbyqxmd.com/read/30086614/clinical-and-imaging-features-of-multiple-system-atrophy-challenges-for-an-early-and-clinically-definitive-diagnosis
#5
Hirohisa Watanabe, Yuichi Riku, Kazuhiro Hara, Kazuya Kawabata, Tomohiko Nakamura, Mizuki Ito, Masaaki Hirayama, Mari Yoshida, Masahisa Katsuno, Gen Sobue
Multiple system atrophy (MSA) is an adult-onset, progressive neurodegenerative disorder. Patients with MSA show various phenotypes during the course of their illness, including parkinsonism, cerebellar ataxia, autonomic failure, and pyramidal signs. Patients with MSA sometimes present with isolated autonomic failure or motor symptoms/ signs. The median duration from onset to the concomitant appearance of motor and autonomic symptoms is approximately 2 years but can range up to 14 years. As the presence of both motor and autonomic symptoms is essential for the current diagnostic criteria, early diagnosis is difficult when patients present with isolated autonomic failure or motor symptoms/signs...
August 9, 2018: Journal of Movement Disorders
https://www.readbyqxmd.com/read/30083098/neuroanatomical-correlates-of-the-unity-and-diversity-model-of-executive-function-in-young-adults
#6
Harry R Smolker, Naomi P Friedman, John K Hewitt, Marie T Banich
Understanding the neuroanatomical correlates of individual differences in executive function (EF) is integral to a complete characterization of the neural systems supporting cognition. While studies have investigated EF-neuroanatomy relationships in adults, these studies often include samples with wide variation in age, which may mask relationships between neuroanatomy and EF specific to certain neurodevelopmental time points, and such studies often use unreliable single task measures of EF. Here we address both issues...
2018: Frontiers in Human Neuroscience
https://www.readbyqxmd.com/read/30077775/oligodendrogliopathy-in-neurodegenerative-diseases-with-abnormal-protein-aggregates-the-forgotten-partner
#7
REVIEW
Isidro Ferrer
Oligodendrocytes are in contact with neurons, wrap axons with a myelin sheath that protects their structural integrity, and facilitate nerve conduction. Oligodendrocytes also form a syncytium with astrocytes which interacts with neurons, promoting reciprocal survival mediated by activity and by molecules involved in energy metabolism and trophism. Therefore, oligodendrocytes are key elements in the normal functioning of the central nervous system. Oligodendrocytes are affected following different insults to the central nervous system including ischemia, traumatism, and inflammation...
August 2, 2018: Progress in Neurobiology
https://www.readbyqxmd.com/read/30076770/altered-neural-signatures-of-interoception-in-multiple-sclerosis
#8
Paula C Salamone, Sol Esteves, Vladimiro J Sinay, Indira García-Cordero, Sofía Abrevaya, Blas Couto, Federico Adolfi, Miguel Martorell, Agustín Petroni, Adrián Yoris, Kathya Torquati, Florencia Alifano, Agustina Legaz, Fátima P Cassará, Diana Bruno, Andrew H Kemp, Eduar Herrera, Adolfo M García, Agustín Ibáñez, Lucas Sedeño
Multiple sclerosis (MS) patients present several alterations related to sensing of bodily signals. However, no specific neurocognitive impairment has yet been proposed as a core deficit underlying such symptoms. We aimed to determine whether MS patients present changes in interoception-that is, the monitoring of autonomic bodily information-a process that might be related to various bodily dysfunctions. We performed two studies in 34 relapsing-remitting, early-stage MS patients and 46 controls matched for gender, age, and education...
August 4, 2018: Human Brain Mapping
https://www.readbyqxmd.com/read/30064607/hardness-and-painful-lesion-of-the-breast
#9
Alejandro Vilas-Sueiro, Daniel González-Vilas, Cristina Aguilera, Benigno Monteagudo, Cristina De De Las Heras
Dear Editor, Lupus panniculitis or lupus profundus is a rare inflammatory complication found in patients with systemic lupus erythematosus (SLE), or discoid lupus erythematosus (DLE) (1). When the breast is involved, the term lupus mastitis (LM) is used. This disease involving the breast is rare, and the lesions may precede, coincide with, or occur later than the onset of other lupus lesions. Tissue biopsy is required to confirm the suspected diagnoses of LM. We report a case of a patient with lupus mastitis due to the important differential diagnosis...
December 2017: Acta Dermatovenerologica Croatica: ADC
https://www.readbyqxmd.com/read/30062642/roles-of-cardiac-sympathetic-neuroimaging-in-autonomic-medicine
#10
REVIEW
David S Goldstein, William P Cheshire
Sympathetic neuroimaging is based on the injection of compounds that either radiolabel sites of the cell membrane norepinephrine transporter (NET) or that are taken up into sympathetic nerves via the NET and radiolabel intra-neuronal catecholamine storage sites. Detection of the radioactivity is by planar or tomographic radionuclide imaging. The heart stands out among body organs in terms of the intensity of radiolabeling of sympathetic nerves, and virtually all of sympathetic neuroimaging focuses on the left ventricular myocardium...
August 2018: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
https://www.readbyqxmd.com/read/30062008/the-role-of-substantia-nigra-sonography-in-the-differentiation-of-parkinson-s-disease-and-multiple-system-atrophy
#11
Hai-Yan Zhou, Pei Huang, Qian Sun, Juan-Juan Du, Shi-Shuang Cui, Yun-Yun Hu, Wei-Wei Zhan, Ying Wang, Qin Xiao, Jun Liu, Yu-Yan Tan, Sheng-Di Chen
Background: The differential diagnosis of Parkinson's disease (PD) and multiple system atrophy (MSA) remains a challenge, especially in the early stage. Here, we assessed the value of transcranial sonography (TCS) to discriminate non-tremor dominant (non-TD) PD from MSA with predominant parkinsonism (MSA-P). Methods: Eighty-six MSA-P patients and 147 age and gender-matched non-TD PD patients who had appropriate temporal acoustic bone windows were included in this study...
2018: Translational Neurodegeneration
https://www.readbyqxmd.com/read/30054179/very-old-onset-parkinsonism-a-clinical-pathological-study
#12
Kurt A Jellinger
BACKGROUND: With increasing age of the world population, the number of parkinsonian patients with disease onset in very old age is expected to increase. Information about the clinical and morphological phenotype of very old age onset parkinsonism is poor, and only three autopsy-confirmed studies of parkinsonian patients of 80 years and older onset are available. METHODS: A retrospective autopsy study of 345 patients clinically diagnosed as Parkinson disease (PD) included 90 cases with disease onset ≥80 years)...
July 24, 2018: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/30051337/therapeutic-management-of-the-overlapping-syndromes-of-atypical-parkinsonism
#13
REVIEW
Nikolaos Giagkou, Maria Stamelou
Progressive supranuclear palsy, corticobasal degeneration and multiple system atrophy account for approximately 10% of neurodegenerative parkinsonism. Considerable clinical overlap exists between these disorders that extends to features considered characteristic of each disease. Clinical diagnostic criteria have attempted to increase the accuracy of clinical diagnosis as accurate diagnosis is necessary to inform prognosis and to facilitate the recognition of disease-modifying treatments. Currently no such treatment exists...
July 27, 2018: CNS Drugs
https://www.readbyqxmd.com/read/30050698/quadratus-lumborum-block-as-sole-homeostatic-preserving-anesthetic-for-a-patient-with-multiple-system-atrophy-undergoing-open-inguinal-hernia-repair-a-case-report
#14
M D Luca La Colla, R M D Schroeder
Quadratus Lumborum (QL) block has been successfully used for different abdominal procedures in the past. Multiple system atrophy (MSA) is a progressive neurodegenerative disorder characterized mainly by autonomic instability, motor impairment, and cognitive dysfunction. We report a case of a patient with MSA with a history of multiple episodes of unplanned admissions following outpatient minor surgical procedures under general anesthesia scheduled to undergo open inguinal hernia repair. In our patient, QL block was successfully used for surgical anesthesia and it resulted in hemodynamic stability and an opioid-free perioperative course...
2018: Case Reports in Anesthesiology
https://www.readbyqxmd.com/read/30043182/bladder-dysfunction-as-the-initial-presentation-of-multiple-system-atrophy-a-prospective-cohort-study
#15
Ryuji Sakakibara, Jalesh Panicker, Sara Simeoni, Tomoyuki Uchiyama, Tatsuya Yamamoto, Fuyuki Tateno, Masahiko Kishi, Yosuke Aiba
OBJECTIVES: Multiple system atrophy (MSA) is a disease that combines autonomic (orthostatic or bladder) with motor [parkinsonian (MSA-P) or cerebellar (MSA-C)] dysfunction. While bladder dysfunction may occur earlier than motor disorders, thus far no prospective study has been available to determine how often and how early bladder autonomic dysfunction predates motor dysfunction in MSA. Therefore, we present data from detailed history-taking in patients with MSA. METHODS: This is a prospective cohort study...
July 24, 2018: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
https://www.readbyqxmd.com/read/30036569/cerebrospinal-fluid-nfl-in-the-differential-diagnosis-of-parkinsonian-disorders-a-meta-analysis
#16
Fangfang Ge, Jiaqi Ding, Yu Liu, Hong Lin, Ting Chang
Neurofilament light chain (NFL) in cerebrospinal fluid (CSF) is a promising biomarker candidate which may discriminate atypical parkinsonian disorders (APD), mainly including multiple system atrophy (MSA), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD), from Parkinson's disease (PD). We aim to evaluate the diagnostic accuracy of CSF NFL level as a differentiating biomarker between APD and PD. Databases of PubMed, OVID and Web of Science were searched for studies (published until May 31, 2017) that reported on CSF NFL as a diagnostic biomarker between APD and PD...
July 20, 2018: Neuroscience Letters
https://www.readbyqxmd.com/read/30035155/tau-imaging-in-parkinsonism-what-have-we-learned-so-far
#17
Jennifer L Whitwell
Background: Positron emission tomography ligands are now available that bind to tau proteins in the brain, providing the exciting opportunity to assess the presence and distribution of tau in vivo in living patients. Methods: This manuscript performed a systematic review of studies that have performed tau PET imaging in patients with parkinsonian disorders. Pubmed was searched up to November 2017, and the review included case reports and patient-control studies...
March 2018: Movement Disorders Clinical Practice
https://www.readbyqxmd.com/read/30035006/the-cerebral-metabolic-topography-of-spinocerebellar-ataxia-type-3
#18
Sanne K Meles, Jelmer G Kok, Bauke M De Jong, Remco J Renken, Jeroen J de Vries, Jacoba M Spikman, Aaltje L Ziengs, Antoon T M Willemsen, Harm J van der Horn, Klaus L Leenders, Hubertus P H Kremer
Introduction: We aimed to uncover the pattern of network-level changes in neuronal function in Spinocerebellar ataxia type 3 (SCA3). Methods: 17 genetically-confirmed SCA3 patients and 16 controls underwent structural MRI and static resting-state [18 F]‑Fluoro‑deoxyglucose Positron Emission Tomography (FDG-PET) imaging. A SCA3-related pattern (SCA3-RP) was identified using a multivariate method (scaled subprofile model and principal component analysis (SSM PCA))...
2018: NeuroImage: Clinical
https://www.readbyqxmd.com/read/30031569/affective-improvement-of-neurological-disease-patients-and-caregivers-using-an-automated-telephone-call-service
#19
Yasuyuki Ohta, Toru Yamashita, Nozomi Hishikawa, Kota Sato, Noriko Hatanaka, Mami Takemoto, Shinji Doutare, Koji Abe
Neurological disease patients living alone or with a single caregiver need a support system to care for their psychological symptoms. We evaluated the clinical effects of a unique telephone call system that automatically called participants at their desired times once a week for 3 months. In total, 104 neurological disease patients and caregivers were evaluated by the geriatric depression scale, apathy scale and state and trait anxiety inventories (STAI) forms X-I for depression, apathy and state anxiety, respectively...
July 18, 2018: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/30026036/deep-brain-stimulation-does-not-enhance-neuroinflammation-in-multiple-system-atrophy
#20
Miguel Lopez-Cuina, Pierre-Olivier Fernagut, Marie-Hélène Canron, Anne Vital, Béatrice Lannes, André Maues De Paula, Nathalie Streichenberger, Dominique Guehl, Philippe Damier, Alexandre Eusebio, Jean-Luc Houeto, François Tison, Christine Tranchant, François Viallet, Tatiana Witjas, Stéphane Thobois, Wassilios G Meissner
Slowly progressive, levodopa-responsive multiple system atrophy (MSA) may be misdiagnosed as Parkinson's disease (PD). Deep brain stimulation (DBS) is mostly ineffective in these patients and may even worsen the clinical course. Here we assessed whether neuropathological differences between patients with MSA who were treated with DBS of the subthalamic nucleus because of a misleading clinical presentation and typical disease cases may explain the more benign disease course of the former, and also the rapid clinical decline after surgery...
July 17, 2018: Neurobiology of Disease
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