Review
Immunology
Marta Lenska-Mieciek, Natalia Madetko-Alster, Piotr Alster, Leszek Krolicki, Urszula Fiszer, Dariusz Koziorowski
Summary: Misfolding and aggregation of proteins are major pathological features of several neurodegenerative diseases. These diseases include neurodegenerative diseases with atypical Parkinsonism and the accumulation of insoluble fibrillary alpha-synuclein or hyperphosphorylated tau protein fragments. In the absence of available therapies to slow or halt disease progression, targeting the inflammatory process shows promise. Inflammatory biomarkers may also aid in the differential diagnosis of Parkinsonian syndromes.
FRONTIERS IN IMMUNOLOGY
(2023)
Review
Medicine, Research & Experimental
Fan Shuen Tseng, Joel Qi Xuan Foo, Aaron Shengting Mai, Eng-King Tan
Summary: Multiple system atrophy (MSA) is a neurodegenerative disease characterized by dysautonomia, parkinsonism, cerebellar dysfunction, and corticospinal degeneration. The underlying mechanism involves aberrant alpha-synuclein deposition, mitochondrial dysfunction, oxidative stress, and neuroinflammation. There is also a possible genetic component that contributes to the risk and progression of MSA. Understanding the genetic factors and pathways involved in MSA can provide insights into potential therapeutic targets.
JOURNAL OF TRANSLATIONAL MEDICINE
(2023)
Review
Biochemistry & Molecular Biology
Kurt A. Jellinger, Gregor K. Wenning, Nadia Stefanova
Summary: Multiple system atrophy (MSA) is a rapidly progressive neurodegenerative disease with a complex clinical presentation. It shares molecular similarities with Parkinson's disease but presents unique pathological features. The debate over whether it should be classified as a prion disease or its potential human transmission remains unresolved.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Clinical Neurology
Teresa Torre-Muruzabal, Anke van der Perren, Audrey Coens, Geraldine Gelders, Anna Barber Janer, Sara Camacho-Garcia, Therese Klingstedt, Peter Nilsson, Nadia Stefanova, Ronald Melki, Veerle Baekelandt, Wouter Peelaerts
Summary: This study found that the progression of multiple system atrophy is influenced by different types of alpha Syn strains. Alpha Syn strains impact disease progression through oligodendroglial, neurotoxic, and immune-related mechanisms, leading to neurodegeneration and brain atrophy. The activation of microglial cells is associated with the structural features of alpha Syn strains.
Article
Clinical Neurology
Margaux Teil, Sandra Dovero, Mathieu Bourdenx, Marie-Laure Arotcarena, Sandrine Camus, Gregory Porras, Marie-Laure Thiolat, Ines Trigo-Damas, Celine Perier, Cristina Estrada, Nuria Garcia-Carrillo, Michele Morari, Wassilios G. Meissner, Maria Trinidad Herrero, Miquel Vila, Jose A. Obeso, Erwan Bezard, Benjamin Dehay
Summary: Synucleinopathies, including Parkinson's disease, dementia with Lewy bodies and multiple system atrophy, are characterized by the deposit of alpha-synuclein aggregates in neurons and glial cells. A study found that inoculating brain fractions containing glial cytoplasmic inclusions from multiple system atrophy patients into non-human primates resulted in neurodegeneration, oligodendrocyte loss, demyelination, neuroinflammation and alpha-synuclein pathology. These findings suggest the potential use of this experimental model for multiple system atrophy research and therapy development.
Review
Biochemistry & Molecular Biology
Do Hyeon Kwon, Ji Su Hwang, Seok Gi Kim, Yong Eun Jang, Tae Hwan Shin, Gwang Lee
Summary: Parkinson's disease and multiple system atrophy are two types of neurodegenerative diseases that are difficult to differentiate, especially in early stages. Identifying metabolic biomarkers is crucial for diagnosis. The metabolic profile in the cerebrospinal fluid has been found to be altered in both diseases, but the specific metabolites are still uncertain. In this study, we created a network of altered metabolites and assessed their biological functions using bioinformatics methods.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Review
Biochemistry & Molecular Biology
Paola Signorelli, Carmela Conte, Elisabetta Albi
Summary: Recent advances in research have shed light on the role of sphingomyelin in Parkinson's disease, with sphingomyelin being implicated in nerve impulse conduction and neurotransmitter receptor localization, possibly contributing to the development of Parkinson's disease.
Article
Clinical Neurology
Yasuo Miki, Eiki Tsushima, Sandrine C. Foti, Kate M. Strand, Yasmine T. Asi, Adam Kenji Yamamoto, Conceicao Bettencourt, Marcos C. B. Oliveira, Eduardo De Pablo-Fernandez, Zane Jaunmuktane, Andrew J. Lees, Koichi Wakabayashi, Thomas T. Warner, Niall Quinn, Janice L. Holton, Helen Ling
Summary: The study focused on patients with multiple system atrophy who presented symptoms closely resembling Parkinson's disease or progressive supranuclear palsy, pointing out the difficulties in diagnosing these mimicking cases accurately using current criteria. Differences in red flag features and autonomic dysfunction were observed in these atypical cases compared to typical ones.
Review
Cell Biology
Jen-Hsiang T. Hsiao, Onur Tanglay, Anne A. Li, Aysha Y. G. Strobbe, Woojin Scott Kim, Glenda M. Halliday, YuHong Fu
Summary: Multiple system atrophy (MSA) is a debilitating movement disorder with unknown etiology. It presents with characteristic parkinsonism and/or cerebellar dysfunction due to deterioration in specific brain regions. The early pathological events and development mechanisms of MSA are reviewed, focusing on the involvement of oligodendrocyte lineage cells and alpha-synuclein. This understanding will guide future research in MSA.
Article
Biochemistry & Molecular Biology
Jonas Folke, Emil Bergholt, Bente Pakkenberg, Susana Aznar, Tomasz Brudek
Summary: This study investigates the autoimmune aberrancies towards alpha-synuclein in prodromal phases of multiple-system trophy and Parkinson's Disease. The results indicate impaired reactivity towards alpha-synuclein occurs before disease onset, which may result in reduced clearance and aggregation of the protein.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Clinical Neurology
Jae-Gyum Kim, Sung-Hwan Kim, Sun-Uk Lee, Chan-Nyoung Lee, Byung-Jo Kim, Ji-Soo Kim, Kun-Woo Park
Summary: The integrity of the vestibulo-ocular reflex in parkinsonism patients has yet to be determined. Head-impulse tests revealed more reversed and perverted catch-up saccades in multiple system atrophy (MSA) compared to Parkinson's disease (PD), indicating potential value for differentiation and as a surrogate marker for clinical decline.
JOURNAL OF NEUROLOGY
(2022)
Article
Radiology, Nuclear Medicine & Medical Imaging
Jinju Sun, Chao Cong, Xinpeng Li, Weicheng Zhou, Renxiang Xia, Huan Liu, Yi Wang, Zhiqiang Xu, Xiao Chen
Summary: This study aims to differentiate Parkinson's disease (PD) and multiple system atrophy (MSA) using multimodal PET/MRI radiomics and clinical characteristics. A total of 119 patients (81 with PD and 38 with MSA) underwent PET/CT and MRI scans, and radiomics features were extracted from the putamina and caudate nuclei to construct predictive models. The results showed that the combined PET/MRI radiomics-clinical model performed better in differentiating PD from MSA.
EUROPEAN RADIOLOGY
(2023)
Article
Clinical Neurology
Victoria Sidoroff, Cecilia Raccagni, Christine Kaindlstorfer, Sabine Eschlboeck, Alessandra Fanciulli, Roberta Granata, Bjorn Eskofier, Klaus Seppi, Werner Poewe, Johann Willeit, Stefan Kiechl, Philipp Mahlknecht, Heike Stockner, Kathrin Marini, Oliver Schorr, Gregorio Rungger, Jochen Klucken, Gregor Wenning, Heiko Gassner
Summary: The study compared gait variability at different walking speeds between patients with Parkinson-variant multiple system atrophy, idiopathic Parkinson's disease, and a control group of older adults. Multiple system atrophy patients displayed higher gait variability in various parameters compared to controls, while Parkinson's patients did not. Variability parameters were significantly correlated with the postural instability/gait difficulty subscore in both disease groups.
JOURNAL OF NEUROLOGY
(2021)
Review
Clinical Neurology
Bruce A. C. Cree, Douglas L. Arnold, Jeremy Chataway, Tanuja Chitnis, Robert J. Fox, Angela Pozo Ramajo, Niamh Murphy, Hans Lassmann
Summary: In most cases, multiple sclerosis begins with a relapsing-remitting course followed by secondary progressive MS (SMPS), and there are major differences between RRMS and SPMS in terms of treatment response and diagnosis. However, clear criteria marking the transition from RRMS to SPMS have not been established yet. Early identification of SPMS will require tools that, along with appropriate treatment, may lead to better long-term outcomes.
Article
Neurosciences
Joachim Brumberg, Anastasia Kuzkina, Constantin Lapa, Sona Mammadova, Andreas Buck, Jens Volkmann, Claudia Sommer, Ioannis U. Isaias, Kathrin Doppler
Summary: Pathological aggregates of alpha-synuclein can be detected in peripheral dermal nerve fibers of patients with idiopathic Parkinson's disease and multiple system atrophy, indicating overlapping pathophysiological mechanisms between the two diseases and suggesting that alpha-synuclein may contribute to cardiac sympathetic neurons impairment in patients with synucleinopathies.
NEUROBIOLOGY OF DISEASE
(2021)