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atrophy multiple systems

Thomas J Vajtay, Akhil Bandi, Aman Upadhyay, Mavis R Swerdel, Ronald P Hart, Christian R Lee, David J Margolis
The functional state of denervated muscle is a critical factor in the ability to restore movement after injury- or disease-related paralysis. Here we used peripheral optogenetic stimulation and transcriptome profiling in the mouse whisker system to investigate the time course of changes in neuromuscular function following complete unilateral facial nerve transection. While most skeletal muscles rapidly lose functionality after lower motor neuron denervation, optogenetic muscle stimulation of the paralyzed whisker pad revealed sustained increases in the sensitivity, velocity, and amplitude of whisker movements, and reduced fatigability, starting 48 h after denervation...
February 20, 2019: Journal of Neurophysiology
Hugo C Baggio, Alexandra Abos, Barbara Segura, Anna Campabadal, Carme Uribe, Darly M Giraldo, Alexandra Perez-Soriano, Esteban Muñoz, Yaroslau Compta, Carme Junque, Maria Jose Marti
BACKGROUND: Recent studies using resting-state functional connectivity and machine-learning to distinguish patients with neurodegenerative diseases from other groups of subjects show promising results. This approach has not been tested to discriminate between Parkinson's disease (PD) and multiple system atrophy (MSA) patients. OBJECTIVES: Our first aim is to characterize possible abnormalities in resting-state functional connectivity between the cerebellum and a set of intrinsic-connectivity brain networks and between the cerebellum and different regions of the striatum in PD and MSA...
February 13, 2019: NeuroImage: Clinical
Fabrizio Vallelonga, Cristina Di Stefano, Aristide Merola, Alberto Romagnolo, Gabriele Sobrero, Valeria Milazzo, Alessio Burrello, Jacopo Burrello, Maurizio Zibetti, Franco Veglio, Simona Maule
INTRODUCTION: We sought to analyze the blood pressure (BP) circadian rhythm in Parkinson's disease (PD), multiple system atrophy (MSA), and pure autonomic failure (PAF) and to evaluate the effect of vasoactive and dopaminergic medications on BP fluctuations during activities of daily living. METHODS: We analyzed data from patients with PD (n = 72), MSA (n = 18), and PAF (n = 17) evaluated with 24-h ambulatory BP monitoring (ABPM) at our Center between 1996 and 2015...
February 19, 2019: Journal of Neurology
Sahil Mehta, Rajender Kumar, Vivek Lal
Background: Camptocormia is defined as forward flexion of the spine that manifests during walking and standing and disappears in recumbent position. The various etiologies include idiopathic Parkinson's disease, multiple system atrophy, myopathies, degenerative joint disease, and drugs. Case Report: A 67-year-old diabetic female presented with bradykinesia and camptocormia that started 1 year prior to presentation. Evaluation revealed levosulpiride, a dopamine receptor blocker commonly used for dyspepsia, to be the culprit...
2019: Tremor and Other Hyperkinetic Movements
V Donadio, K Doppler, A Incensi, A Kuzkina, A Janzen, G Mayer, J Volkmann, G Rizzo, E Antelmi, G Plazzi, C Sommer, R Liguori, W H Oertel
BACKGROUND: Visualization of phosphorylated α-synuclein at serine 129 (p-syn) in skin nerves is a promising test for the in vivo diagnosis of synucleinopathies. Here we aimed to establish the intra- and inter-laboratory reproducibility of quantification of intraneural p-syn immunoreactivity in two laboratories with a major expertise (Würzburg and Bologna). METHODS: We enrolled 43 patients affected by Parkinson's disease (PD: 21 patients), Dementia with Lewy Body (DLB: 1), REM sleep behavior disorder (RBD: 11), Multiple System Atrophy (MSA-P: 4) and small fiber neuropathy (SFN: 6)...
February 15, 2019: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
Richard J Karpowicz, John Q Trojanowski, Virginia M-Y Lee
Cell-to-cell transmission of proteopathic alpha-synuclein (α-syn) seeds is increasingly thought to underlie the progression of neurodegenerative diseases including Parkinson's disease, dementia with Lewy bodies, multiple system atrophy, and related synucleinopathies. As such, it is important to understand the chemical and biological relationships between cells and pathological aggregates of α-syn. This brief review updates our understanding of the templated spread of α-syn pathology in neurodegenerative disease from the perspective of proteopathic α-syn seeds, including how these seeds are processed by cells as well as their effects on cellular function...
February 13, 2019: Laboratory Investigation; a Journal of Technical Methods and Pathology
Christopher C Hemond, Bonnie I Glanz, Rohit Bakshi, Tanuja Chitnis, Brian C Healy
BACKGROUND: Serum hematological indices such as the neutrophil-lymphocyte ratio (NLR) or monocyte-lymphocyte ratio (MLR) have been used as biomarkers of pathogenic inflammation and prognostication in multiple areas of medicine; recent evidence shows correlation with psychological parameters as well. OBJECTIVES/AIMS: To characterize clinical, neuroimaging, and psycho-neuro-immunological associations with NLR and MLR in persons with multiple sclerosis (MS). METHODS: We identified a large cohort of clinically well-defined patients from our longitudinal database that included MS-related outcomes, disease-modifying therapy, patient-reported outcome (PRO) measures, and quantified cerebral MRI at 1...
February 12, 2019: BMC Neurology
Sylvain Vergnet, Florent Hives, Alexandra Foubert-Samier, Pierre Payoux, Philippe Fernandez, Marie Meyer, Julia Dupouy, Christine Brefel-Courbon, Fabienne Ory-Magne, Olivier Rascol, François Tison, Anne Pavy-Le Traon, Wassilios G Meissner
INTRODUCTION: The added value of dopamine transporter SPECT (DAT-SPECT) for the diagnosis of "possible" multiple system atrophy of the cerebellar type (MSA-C) remains unknown. METHODS: We reviewed retrospectively the charts of 128 consecutive patients with a clinical diagnosis of MSA-C who were seen between 2007 and 2016 at the French Reference Center for MSA. The main objective was to evaluate the proportion of patients for whom the diagnosis of "possible" MSA-C was made because of a positive DAT-SPECT...
February 5, 2019: Parkinsonism & related Disorders
LingYu Zhang, Bei Cao, Yutong Zou, Qian-Qian Wei, RuWei Ou, Bi Zhao, Jing Yang, Ying Wu, HuiFang Shang
BACKGROUND: Cognitive impairment is an important and common symptom in patients with multiple system atrophy (MSA). OBJECTIVE: The objective of the study was to explore the potential relationships among frontal lobe function, behavioral changes and quality of life (QoL) in patients with MSA. METHODS: A total of 203 MSA patients were enrolled and evaluated using the Frontal Assessment Battery (FAB), the Frontal Behavioral Inventory (FBI) and the Parkinson's disease Questionnaire-39 item version (PDQ-39)...
February 5, 2019: Restorative Neurology and Neuroscience
Ernest W Wang, Guangwei Du, Mechelle M Lewis, Eun-Young Lee, Sol De Jesus, Sangam Kanekar, Lan Kong, Xuemei Huang
Previous multimodal magnetic resonance imaging (MRI) studies of parkinsonian syndromes have focused primarily on motor-related basal ganglia structures. The present study investigated MRI changes in nonmotor-related limbic structures in 35 Parkinson's disease, 16 multiple system atrophy parkinsonian subtype, 17 progressive supranuclear palsy, and 37 control subjects. Mean diffusivity (MD), fractional anisotropy, transverse relaxation rate (R2*), quantitative susceptibility mapping, and volume measurements were obtained from the amygdala, hippocampus, and nucleus accumbens (NAc) to examine differences between groups and to test for associations with clinical scores...
January 16, 2019: Neurobiology of Aging
Jess-Karan S Dhillon, Jorge A Trejo-Lopez, Cara Riffe, Nikolaus R McFarland, Wesley M Hiser, Benoit I Giasson, Anthony T Yachnis
Synucleinopathies are a group of neurodegenerative diseases characterized by the accumulation of insoluble, aggregated α-synuclein (αS) pathological inclusions. Multiple system atrophy (MSA) presents with extensive oligodendroglial αS pathology and additional more limited neuronal inclusions while most of the other synucleinopathies, such as Parkinson's disease and dementia with Lewy bodies (DLB), develop αS pathology primarily in neuronal cell populations. αS biochemical alterations specific to MSA have been described but thorough examination of these unique and disease-specific protein deposits is further warranted especially given recent findings implicating the prion-like nature of synucleinopathies perhaps with distinct strain-like properties...
February 8, 2019: Laboratory Investigation; a Journal of Technical Methods and Pathology
Heiko Gaßner, Cecilia Raccagni, Bjoern M Eskofier, Jochen Klucken, Gregor K Wenning
Background: Differentiating idiopathic Parkinson's disease (IPD) from atypical Parkinsonian disorders (APD) is challenging, especially in early disease stages. Postural instability and gait difficulty (PIGD) are substantial motor impairments of IPD and APD. Clinical evidence implies that patients with APD have larger PIGD impairment than IPD patients. Sensor-based gait analysis as instrumented bedside test revealed more gait deficits in APD compared to IPD. However, the diagnostic value of instrumented bedside tests compared to clinical assessments in differentiating APD from IPD patients have not been evaluated so far...
2019: Frontiers in Neurology
Hiroaki Sekiya, Hisatomo Kowa, Hinako Koga, Mariko Takata, Wataru Satake, Naonobu Futamura, Itaru Funakawa, Kenji Jinnai, Motonori Takahashi, Takeshi Kondo, Yasuhiro Ueno, Motoi Kanagawa, Kazuhiro Kobayashi, Tatsushi Toda
Multiple system atrophy (MSA) is a fatal adult-onset neurodegenerative disease that is characterized by varying degrees of cerebellar dysfunction and Parkinsonism. The neuropathological hallmark of MSA is alpha-synuclein (AS)-positive glial cytoplasmic inclusions (GCIs). Although severe neuronal loss (NL) is also observed in MSA, neuronal inclusions (NIs) are rare compared to GCIs, such that the pathological mechanism of NL in MSA is unclear. GCIs and NIs are late-stage pathology features relative to AS oligomers and may not represent early pathological changes in MSA...
February 5, 2019: Acta Neuropathologica
Hisanori Kinoshita, Takashi Ayaki, Takakuni Maki, Naoki Goda, Akihiko Yoshizawa, Ryosuke Takahashi
Sudden death as a result of multiple system atrophy (MSA) is usually attributed to vocal cord paralysis or disruption of breathing owing to the degeneration of the brainstem respiratory centers. However, the exact pathophysiology of sudden death is still unclear. In addition, specific causes of sudden death are not always investigated by an autopsy. We herein report a patient with MSA and non-aneurysmal subarachnoid hemorrhaging, which is believed to be a rare cause of death in this setting. Without an autopsy, our case would likely have been diagnosed as sudden death due to vocal cord paralysis...
February 1, 2019: Internal Medicine
Tanima Dwivedi, Manasi Gosavi
NeuLaxova syndrome (NLS) is a rare congenital abnormality involving multiple systems. Until date, only 60 cases of this syndrome have been reported in the literature. A stillborn fetus from a 23-year-old female with bad obstetrics history and consanguinity marriage, presented at 41 weeks gestation and not appreciating fetal movements for the past 3 days. Ultrasound examination revealed the absence of fetal cardiac activity and features of growth retardation. The fetus was sent for pathological examination. At autopsy, fetus had ichthyosis over the scalp and face, depressed nasal bridge, low set ears, microcephaly, slopping forehead, wide interdigital spaces, edema of hands and feet, hypoplastic penis, right leg showed congenital talipes equinovarus and left leg showed rocker bottom foot...
January 2019: Indian Journal of Pathology & Microbiology
Carlos Guevara, José de Grazia, Pablo Baabor, Wendy Soruco
INTRODUCTION: MSA is an adult-onset, sporadic, progressive parkinsonian syndrome characterised by the presence of akinesia, cerebellar dysfunction, autonomic failure and pyramidal signs. Annualized-whole-brain atrophy rate (a-WBAR) is an informative way to quantify disease progression. In this longitudinal work we investigate the correlations of a-WBAR with clinical scales for motor impairment, autonomic disability and cognitive decline in MSA and explore how atrophy progresses within the brain...
December 14, 2018: Autonomic Neuroscience: Basic & Clinical
Amanda L Woerman, Abby Oehler, Sabeen A Kazmi, Jisoo Lee, Glenda M Halliday, Lefkos T Middleton, Steve M Gentleman, Daniel A Mordes, Salvatore Spina, Lea T Grinberg, Steven H Olson, Stanley B Prusiner
Previously, we reported that intracranial inoculation of brain homogenate from multiple system atrophy (MSA) patient samples produces neurological disease in the transgenic (Tg) mouse model TgM83+/- , which uses the prion protein promoter to express human α-synuclein harboring the A53T mutation found in familial Parkinson's disease (PD). In our studies, we inoculated MSA and control patient samples into Tg mice constructed using a P1 artificial chromosome to express wild-type (WT), A30P, and A53T human α-synuclein on a mouse α-synuclein knockout background [Tg(SNCA+/+ )Nbm, Tg(SNCA*A30P+/+ )Nbm, and Tg(SNCA*A53T+/+ )Nbm]...
January 28, 2019: Acta Neuropathologica
Sami Bizzari, Abdul Rezzak Hamzeh, Madiha Mohamed, Mahmoud Taleb Al-Ali, Fatma Bastaki
Pontocerebellar Hypoplasia type 1 is a rare heterogeneous neurodegenerative disorder with multiple subtypes linked to dysfunction of the exosome complex. Patients with mutations in exosome subunits exhibit a generally lethal phenotype characterized by cerebellar and pontine hypoplasia in association with spinal motor neuropathy and multiple systemic and neurologic features. Recently, two variants in the novel PCH1 associated protein EXOSC9 p.(Leu14Pro) and p.(Arg161*) have been identified in 4 unrelated patients exhibiting a severe phenotype involving cerebellar hypoplasia, axonal motor neuropathy, hypotonia, feeding difficulties, and respiratory insufficiency (PCH1D)...
January 25, 2019: European Journal of Medical Genetics
Hiroyuki Todo
Continuous glucose monitoring (CGM) is a method to examine glucose concentration in subcutaneous interstitial fluid sequentially. CGM can disclose glucose fluctuation (GF), which can be unrecognized in routine blood tests. A limited number of studies suggest advanced Parkinsonian syndromes (PS) is at risk of GF, however, the report of CGM in PS is scarce. We performed CGM for 72 h in 11 nondiabetic patients with advanced PS. The etiology was Parkinson's disease, multiple system atrophy, progressive supranuclear palsy, or dementia with Lewy bodies...
December 5, 2018: Neurology International
Mats I Nilsson, Jacqueline M Bourgeois, Joshua P Nederveen, Marlon R Leite, Bart P Hettinga, Adam L Bujak, Linda May, Ethan Lin, Michael Crozier, Daniel R Rusiecki, Chris Moffatt, Paul Azzopardi, Jacob Young, Yifan Yang, Jenny Nguyen, Ethan Adler, Lucy Lan, Mark A Tarnopolsky
Biological aging is associated with progressive damage accumulation, loss of organ reserves, and systemic inflammation ('inflammaging'), which predispose for a wide spectrum of chronic diseases, including several types of cancer. In contrast, aerobic exercise training (AET) reduces inflammation, lowers all-cause mortality, and enhances both health and lifespan. In this study, we examined the benefits of early-onset, lifelong AET on predictors of health, inflammation, and cancer incidence in a naturally aging mouse model (C57BL/J6)...
2019: PloS One
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