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Multiple systems atrophy

Alicja Kalinowska-Łyszczarz, Mikołaj A Pawlak, Aleksandra Wyciszkiewicz, Katarzyna Pawlak-Buś, Piotr Leszczyński, Mariusz Puszczewicz, Włodzimierz Paprzycki, Wojciech Kozubski, Sławomir Michalak
OBJECTIVE: Central nervous system (CNS) involvement in systemic lupus erythematosus (SLE) remains poorly understood. Damage within the CNS is driven by the autoimmune response; however, immunopathophysiology of neuropsychiatric (NP) SLE is multifactorial. Immune cell neurotrophin production could be neuroprotective against autoimmunity-driven CNS damage, as has been shown in multiple sclerosis. The aim of this study was to establish whether immune cell neurotrophin production is associated with damage severity in NPSLE...
March 14, 2018: Neuroimmunomodulation
Jingjie Ge, Jianjun Wu, Shichun Peng, Ping Wu, Jian Wang, Huiwei Zhang, Yihui Guan, David Eidelberg, Chuantao Zuo, Yilong Ma
Progressive supranuclear palsy (PSP) is a rare movement disorder and often difficult to distinguish clinically from Parkinson's disease (PD) and multiple system atrophy (MSA) in early phases. In this study, we report reproducible disease-related topographies of brain network and regional glucose metabolism associated with PSP in clinically-confirmed independent cohorts of PSP, MSA, and PD patients and healthy controls in the USA and China. Using18 F-FDG PET images from PSP and healthy subjects, we applied spatial covariance analysis with bootstrapping to identify a PSP-related pattern (PSPRP) and estimate its reliability, and evaluated the ability of network scores for differential diagnosis...
March 13, 2018: Human Brain Mapping
Michael Dietrich, Niklas Helling, Alexander Hilla, Annemarie Heskamp, Andrea Issberner, Thomas Hildebrandt, Zippora Kohne, Patrick Küry, Carsten Berndt, Orhan Aktas, Dietmar Fischer, Hans-Peter Hartung, Philipp Albrecht
BACKGROUND: In multiple sclerosis (MS), neurodegeneration is the main reason for chronic disability. Alpha-lipoic acid (LA) is a naturally occurring antioxidant which has recently been demonstrated to reduce the rate of brain atrophy in progressive MS. However, it remains uncertain if it is also beneficial in the early, more inflammatory-driven phases. As clinical studies are costly and time consuming, optic neuritis (ON) is often used for investigating neuroprotective or regenerative therapeutics...
March 7, 2018: Journal of Neuroinflammation
Jose-Alberto Palma, Horacio Kaufmann
Dysfunction of the autonomic nervous system afflicts most patients with Parkinson disease and other synucleinopathies such as dementia with Lewy bodies, multiple system atrophy, and pure autonomic failure, reducing quality of life and increasing mortality. For example, gastrointestinal dysfunction can lead to impaired drug pharmacodynamics causing a worsening in motor symptoms, and neurogenic orthostatic hypotension can cause syncope, falls, and fractures. When recognized, autonomic problems can be treated, sometimes successfully...
March 2018: Movement Disorders: Official Journal of the Movement Disorder Society
Gina Puska, Mirjam I Lutz, Kinga Molnar, Günther Regelsberger, Gerda Ricken, Walter Pirker, Lajos Laszlo, Gabor G Kovacs
Intracellular deposition of pathologically altered α-synuclein mostly in neurons characterises Parkinson's disease (PD), while its accumulation predominantly in oligodendrocytes is a feature of multiple system atrophy (MSA). Recently a prion-like spreading of pathologic α-synuclein has been suggested to play a role in the pathogenesis of PD and MSA. This implicates a role of protein processing systems, including lysosomes, supported also by genetic studies in PD. However, particularly for MSA, the mechanism of cell-to-cell propagation of α-synuclein is yet not fully understood...
March 2, 2018: Neurobiology of Disease
Elena Abati, Alessio Di Fonzo, Stefania Corti
Multiple system atrophy (MSA) is a rare neurodegenerative disease with a fatal outcome. Nowadays, only symptomatic treatment is available for MSA patients. The hallmarks of the disease are glial cytoplasmic inclusions (GCIs), proteinaceous aggregates mainly composed of alpha-synuclein, which accumulate in oligodendrocytes. However, despite the extensive research efforts, little is known about the pathogenesis of MSA. Early myelin dysfunction and alpha-synuclein deposition are thought to play a major role, but the origin of the aggregates and the causes of misfolding are obscure...
March 4, 2018: Journal of Cellular and Molecular Medicine
Lasse Reimer, Louise Buur Vesterager, Cristine Betzer, Jin Zheng, Lærke Dalsgaard Nielsen, Rikke Hahn Kofoed, Louise Berkhoudt Lassen, Ulrik Bølcho, Søren Riis Paludan, Karina Fog, Poul Henning Jensen
Parkinson's disease, dementia with Lewy bodies, and multiple system atrophy comprise a group of neurodegenerative diseases termed synucleinopathies. Synucleinopathie are, characterized by presence of inclusion bodies in degenerating brain cells which contain aggregated α-synuclein phosphorylated on Ser129. Although the inflammation-associated serine-threonine kinase, PKR (EIF2AK2), promotes cellular protection against infection, we demonstrate a pro-degenerative role of activated PKR in an α-synuclein-dependent cell model of multiple system atrophy, where inhibition and silencing of PKR decrease cellular degeneration...
March 1, 2018: Neurobiology of Disease
X Zhou, C Wang, D Ding, Z Chen, Y Peng, H Peng, X Hou, P Wang, X Hou, W Ye, T Li, H Yang, R Qiu, K Xia, J Sequeiros, B Tang, H Jiang
Multiple system atrophy (MSA) is a complex and multifactorial neurodegenerative disease, and its pathogenesis remains uncertain. Patients with MSA or spinocerebellar ataxia (SCA) show overlapping clinical phenotypes. Previous studies have reported that intermediate or long CAG expansions in SCA genes have been associated with other neurodegenerative disease. In this study, we screened for the number of CAG repeats in ATXN1, 2 and 3 in 200 patients with MSA and 314 healthy controls to evaluate possible associations between (CAG)n in these three polyQ-related genes and MSA...
March 1, 2018: Scientific Reports
Tina Roostaei, Shokufeh Sadaghiani, Rahil Mashhadi, Masih Falahatian, Esmaeil Mohamadi, Nina Javadian, Aria Nazeri, Rozita Doosti, Abdorreza Naser Moghadasi, Mahsa Owji, Amir Pejman Hashemi Taheri, Ali Shakouri Rad, Amirreza Azimi, Aristotle N Voineskos, Arash Nazeri, Mohammad Ali Sahraian
BACKGROUND: Complement system activation products are present in areas of neuroinflammation, demyelination, and neurodegeneration in brains of patients with multiple sclerosis (MS). C3 is a central element in the activation of complement cascades. A common coding variant in the C3 gene (rs2230199, C3R102G) affects C3 activity. OBJECTIVES: To assess the effects of rs2230199 on MS severity using clinical, cognitive, and imaging measures. METHODS: In total, 161 relapse-onset MS patients (Expanded Disability Status Scale (EDSS) ≤ 6) underwent physical assessments, cognitive tests (Paced Auditory Serial Addition Test (PASAT), Symbol Digit Modalities Test (SDMT), and California Verbal Learning Test (CVLT)), and magnetic resonance imaging (MRI)...
February 1, 2018: Multiple Sclerosis: Clinical and Laboratory Research
Per K Eide, Geir Ringstad
The glymphatic system plays a key role for clearance of waste solutes from the rodent brain. We recently found evidence of glymphatic circulation in the human brain when using magnetic resonance imaging (MRI) contrast agent as cerebrospinal fluid (CSF) tracer in conjunction with multiple MRI acquisitions (gMRI). The present study explored the hypothesis that reduced glymphatic clearance in entorhinal cortex (ERC) may be instrumental in idiopathic normal pressure hydrocephalus (iNPH) dementia. gMRI acquisitions were obtained over a 24-48 h time span in cognitively affected iNPH patients and non-cognitively affected patients with suspected CSF leaks...
January 1, 2018: Journal of Cerebral Blood Flow and Metabolism
Prabhjot K Bedi, Maria Luisa Castro-Codesal, Robin Featherstone, Mohammed M AlBalawi, Bashar Alkhaledi, Anita L Kozyrskyj, Carlos Flores-Mir, Joanna E MacLean
Background: The use of long-term non-invasive ventilation (NIV) to treat sleep and breathing disorders in children has increased substantially in the last decade; however, less data exist about its use in infants. Given that infants have distinct sleep and breathing patterns when compared to older children, the outcomes of infants on long-term NIV may differ as well. The aim of this study is to systematically review the use and outcomes of long-term NIV in infants. Methods: Ovid Medline, Ovid Embase, CINAHL (via EbscoHOST), PubMed, and Wiley Cochrane Library were systematically searched from January 1990 to July 2017...
2018: Frontiers in Pediatrics
Yang-Hyun Lee, Jee-Eun Lee, Dong-Woo Ryu, Yoon-Sang Oh, Kwang-Soo Lee, Sung-Hoo Hong, Joong-Seok Kim
BACKGROUND: Urinary dysfunction is relatively common, however, is often underestimated and diagnosed by subjective questionnaires in patients with Parkinsonism. OBJECTIVE: To determine the correlation between subjective urinary dysfunction and post-void residual urine volume in patients with Parkinson's disease (PD), multiple system atrophy (MSA) and progressive supranuclear palsy (PSP), and to assess the relationship between clinical characteristics and subjective or objective urinary dysfunction...
2018: Journal of Parkinson's Disease
Kelsey Lee, Khanh-Dung Nguyen, Chao Sun, Mei Liu, Faria Zafar, Jimmy Saetern, Adrian Flierl, James W Tetrud, J William Langston, Dennis Dickson, Birgitt Schüle
BACKGROUND: Mutations in the leucine rich repeat kinase 2 (LRRK2) gene are among the most common genetic causes of Lewy body Parkinson's disease (PD). However, LRRK2 mutations can also lead to a variety of pathological phenotypes other than typical PD, including relatively pure nigrostriatal cell loss without alpha-synuclein-positive Lewy bodies or Lewy neurites, progressive supranuclear palsy (PSP), and multiple system atrophy (MSA). The mechanisms behind this remarkable pleomorphic pathology are currently unclear...
2018: Journal of Parkinson's Disease
G Nagesh Babu, Manjeet Gupta, Vimal K Paliwal, Suruchi Singh, Tanushri Chatterji, Raja Roy
BACKGROUND: Parkinson's disease (PD) is the result of progressive degeneration of the nigrostriatal dopaminergic pathway and depletion of neurotransmitter dopamine in the striatum. METHODS: We included 17 patients with PD along with 7 patients of progressive supranuclear palsy (PSP), 6 patients of multiple system atrophy (MSA) and 22 age and sex-matched healthy controls. We analyzed metabolite profiles in the serum of these patients and controls using1 H NMR spectroscopy...
February 20, 2018: Clinica Chimica Acta; International Journal of Clinical Chemistry
Patrice Péran, Gaetano Barbagallo, Federico Nemmi, Maria Sierra, Monique Galitzky, Anne Pavy-Le Traon, Pierre Payoux, Wassilios G Meissner, Olivier Rascol
BACKGROUND: Multimodal MRI approach is based on a combination of MRI parameters sensitive to different tissue characteristics (eg, volume atrophy, iron deposition, and microstructural damage). The main objective of the present study was to use a multimodal MRI approach to identify brain differences that could discriminate between matched groups of patients with multiple system atrophy, Parkinson's disease, and healthy controls. We assessed the 2 different MSA variants, namely, MSA-P, with predominant parkinsonism, and MSA-C, with more prominent cerebellar symptoms...
February 23, 2018: Movement Disorders: Official Journal of the Movement Disorder Society
Saleheddine Rekik, Francis Martin, Pauline Dodet, Stefania Redolfi, Smaranda Leu-Semenescu, Jean-Christophe Corvol, David Grabli, Isabelle Arnulf
OBJECTIVES: To determine the frequency of sleep breathing disorders in multiple systemic atrophy (MSA, combining Parkinsonism, cerebellar syndrome, and dysautonomia) and evaluate the benefit/tolerance of various modes of ventilation. METHODS: We retrospectively analyzed 45 patients with MSA having undergone a videopolysomnography. Their sleep characteristics were compared to those of 45 patients with Parkinson's disease and 45 healthy controls, matched for age and sex...
February 2018: Sleep Medicine
Yingyan Gan, Huici Liang, Xiaojing Li, Yiru Zeng, Wenlin Wu, Jing Han, Jianning Mai
The 'hot cross' bun (HCB) sign refers to pontine cruciform hyperintensity on T2 weighted magnetic resonance image (MRI) which is frequently seen in multiple system atrophy and spinocerebellar ataxia types 2 and 3. We describe a 3 years old boy of encephalitis and his MRI image showed HCB sign in the pontine. After immunosuppressive treatment and followed up 14 months, he got a good outcome and the HCB sign narrowed nearly disappeared.
February 14, 2018: Brain & Development
Ashraf S Gorgey, Refka E Khalil, Robert M Lester, Gary A Dudley, David R Gater
Skeletal muscle atrophy, increased adiposity and reduced physical activity are key changes observed after spinal cord injury (SCI) and are associated with numerous cardiometabolic health consequences. These changes are likely to increase the risk of developing chronic secondary conditions and impact the quality of life in persons with SCI. Surface neuromuscular electrical stimulation evoked resistance training (NMES-RT) was developed as a strategy to attenuate the process of skeletal muscle atrophy, decrease ectopic adiposity, improve insulin sensitivity and enhance mitochondrial capacity...
February 1, 2018: Journal of Visualized Experiments: JoVE
Kotaro Ogaki, Yuka A Martens, Michael G Heckman, Shunsuke Koga, Catherine Labbé, Oswaldo Lorenzo-Betancor, Anna I Wernick, Ronald L Walton, Alexandra I Soto, Emily R Vargas, Henrietta M Nielsen, Shinsuke Fujioka, Takahisa Kanekiyo, Ryan J Uitti, Jay A van Gerpen, William P Cheshire, Zbigniew K Wszolek, Phillip A Low, Wolfgang Singer, Dennis W Dickson, Guojun Bu, Owen A Ross
BACKGROUND: Dysregulation of the specialized lipid metabolism involved in myelin synthesis and maintenance by oligodendrocytes has been associated with the unique neuropathology of MSA. We hypothesized that apolipoprotein E, which is associated with neurodegeneration, may also play a role in the pathogenesis of MSA. OBJECTIVE: This study evaluated genetic associations of Apolipoprotein E alleles with risk of MSA and α-synuclein pathology, and also examined whether apolipoprotein E isoforms differentially affect α-synuclein uptake in a oligodendrocyte cell...
February 14, 2018: Movement Disorders: Official Journal of the Movement Disorder Society
Josiane M da Silva, Jéssica L M Conegundes, Nícolas C C Pinto, Renata F Mendes, Maria Christina M N Castañon, Elita Scio
OBJECTIVES: This study aimed to evaluate the chronic topical anti-inflammatory activity of the pharmaceutical formulation ProHLP containing the hexane fraction of Lacistema pubescens (HLP). It was also investigated the possible cutaneous and systemic adverse effects of HLP and ProHLP in mice when compared to dexamethasone. METHODS: The chronic topical anti-inflammatory activity was determined by croton oil multiple application-induced mouse ear oedema model. Histopathological analyses of ear tissue samples sensitized with croton oil were performed...
February 13, 2018: Journal of Pharmacy and Pharmacology
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