Article
Clinical Neurology
Viorica Chelban, Elham Nikram, Alexandra Perez-Soriano, Carlo Wilke, Alexandra Foubert-Samier, Nirosen Vijiaratnam, Tong Guo, Edwin Jabbari, Simisola Olufodun, Mariel Gonzalez, Konstantin Senkevich, Brice Laurens, Patrice Peran, Olivier Rascol, Anne Pavy Le Traon, Emily G. Todd, Alyssa A. Costantini, Sondos Alikhwan, Ambreen Tariq, Bai Lin Ng, Esteban Munoz, Celia Painous, Yaroslau Compta, Carme Junque, Barbara Segura, Kristina Zhelcheska, Henny Wellington, Ludger Schoels, Zane Jaunmuktane, Christopher Kobylecki, Alistair Church, Michele T. M. Hu, James B. Rowe, P. Nigel Leigh, Luke Massey, David J. Burn, Nicola Pavese, Tom Foltynie, Sofya Pchelina, Nicholas Wood, Amanda J. Heslegrave, Henrik Zetterberg, Martina Bocchetta, Jonathan D. Rohrer, Maria J. Marti, Matthis Synofzik, Huw R. Morris, Wassilios G. Meissner, Henry Houlden
Summary: In this study, it was found that plasma neurofilament light chain levels correlate with clinical disease severity, progression, and prognosis in multiple system atrophy, providing valuable information for patient stratification and treatment response monitoring in future trials. The use of neurofilament light chain as a biomarker in multiple system atrophy holds promise for improving trial outcomes and streamlining participant recruitment. Further research is warranted to fully understand the potential of neurofilament light chain in multiple system atrophy management.
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)
Article
Biochemistry & Molecular Biology
Kurt A. Jellinger
Summary: Multiple system atrophy (MSA) is a fatal neurodegenerative disease characterized by a combination of Levodopa unresponsive parkinsonism, cerebellar, autonomic and motor dysfunctions. The deposition of aberrant alpha-synuclein in glia and neurons is a morphological hallmark of MSA, along with glioneuronal degeneration. MSA can present in different variants and its diagnosis can be challenging.
Article
Clinical Neurology
Cong Shen, Li Chen, Jing-Jie Ge, Jia-Ying Lu, Qi-Si Chen, Shu-Jin He, Xin-Yi Li, Jue Zhao, Yi-Min Sun, Ping Wu, Jian-Jun Wu, Feng-Tao Liu, Jian Wang
Summary: This study aimed to characterize cognitive profiles in multiple system atrophy (MSA) and explore cerebral metabolism related to cognitive decline. The results showed that cognitive impairments are not rare in MSA, predominantly affecting attention, executive function, and memory. Hypometabolism in the frontal lobe may contribute to these impairments.
FRONTIERS IN NEUROLOGY
(2021)
Article
Clinical Neurology
Hiroaki Sekiya, Shunsuke Koga, Aya Murakami, Miki Kawazoe, Minji Kim, Nicholas B. Martin, Ryan J. Uitti, William P. Cheshire, Zbigniew K. Wszolek, Dennis W. Dickson
Summary: The study compared the diagnostic accuracy of the MDS criteria and second consensus criteria for MSA, finding that the MDS criteria had higher specificity for clinically established and probable MSA, while the second consensus criteria had higher sensitivity for probable and possible MSA.
Editorial Material
Radiology, Nuclear Medicine & Medical Imaging
Ana Utrera-Costero, Begona Martinez-Sanchis, Pablo Sopena-Novales, Pilar Bello-Arques
Summary: A 64-year-old female presented with urinary incontinence and neurological symptoms, showing hypometabolism of the whole cerebellum on imaging studies. Later, she developed tremor, postural instability, and ataxia, leading to a possible diagnosis of multiple system atrophy based on imaging and clinical symptoms.
CLINICAL NUCLEAR MEDICINE
(2021)
Article
Behavioral Sciences
Peifei Jia, Jinhong Zhang, Jiuyan Han, Yong Ji
Summary: This study compared clinical outcomes and cognitive impairments between PSP and MSA. The results showed differences in clinical outcomes and cognitive function between PSP and MSA-P patients, with PSP patients having more severe cognitive deficits. The MMSE and MoCA scores can be used as indicators to distinguish MSA-P from PSP.
BRAIN AND BEHAVIOR
(2022)
Article
Clinical Neurology
Ryunosuke Nagao, Yasuaki Mizutani, Akihiro Ueda, Sayuri Shima, Mizuki Ito, Junichiro Yoshimoto, Hirohisa Watanabe
Summary: Serotonin dysfunction, as assessed by CSF 5-HIAA levels, may implicate greater MSA severity.
EUROPEAN JOURNAL OF NEUROLOGY
(2023)
Article
Clinical Neurology
Giulia Lazzeri, Giulia Franco, Teresa Difonzo, Angelica Carandina, Chiara Gramegna, Maurizio Vergari, Federica Arienti, Anisa Naci, Costanza Scata, Edoardo Monfrini, Gabriel Dias Rodrigues, Nicola Montano, Giacomo P. Comi, Maria Cristina Saetti, Eleonora Tobaldini, Alessio Di Fonzo
Summary: Multiple System Atrophy (MSA) is a rare neurodegenerative disease characterized by autonomic dysfunction and motor involvement. Recent studies have shown that MSA patients also experience cognitive impairment, particularly in executive function and memory. However, the cognitive differences between MSA-P and MSA-C are not related to cardiovascular autonomic dysfunction.
FRONTIERS IN NEUROLOGY
(2022)
Article
Medicine, General & Internal
Santosh Dash, Rohan Mahale, M. Netravathi, Nitish L. Kamble, Vikram Holla, Ravi Yadav, Pramod K. Pal
Summary: Cognition is impaired in patients with multiple system atrophy (MSA), likely due to neurodegeneration involving the cortical and subcortical structures.
CUREUS JOURNAL OF MEDICAL SCIENCE
(2022)
Review
Biochemistry & Molecular Biology
Anastasia Bougea
Summary: This study critically reviewed the genetics of MSA and PSP, and investigated their involvement in the pathogenesis. The results revealed that while familial MSA cases have been reported, the hereditary nature has not been confirmed. COQ2 mutations were found in both familial and sporadic MSA, but were not replicated in diverse clinical populations. SNCA polymorphisms were associated with an increased risk of manifesting MSA in Caucasians, but a causal relationship could not be proven. Fifteen MAPT mutations were linked with PSP, while LRRK2 was a rare monogenic mutation in PSP.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Clinical Neurology
Fabian Maass, Peter Hermann, Daniela Varges, Sabine Nuhn, Christoph van Riesen, Ala Jamous, Niels K. Focke, Manuel Hewitt, Andreas Leha, Mathias Baehr, Inga Zerr
Summary: The objective of this study was to analyze the pattern of cognitive dysfunction in patients with multiple system atrophy (MSA) using a standardized neuropsychological assessment. The results showed that frontal-executive dysfunction is the main characteristic of cognitive impairment in MSA. Overall, patients with MSA-C performed significantly worse in certain cognitive tasks compared to those with MSA-P.
FRONTIERS IN NEUROLOGY
(2022)
Article
Geriatrics & Gerontology
Lingyu Zhang, Ruicen Li, Yanbing Hou, Bei Cao, Qianqian Wei, Ruwei Ou, Kuncheng Liu, Junyu Lin, Tianmi Yang, Yi Xiao, Wenxia Huang, Huifang Shang
Summary: This study found that cystatin C is associated with cognitive decline in patients with early-stage multiple system atrophy (MSA). Through evaluation of 117 MSA patients and 416 healthy controls, it was observed that cystatin C levels were significantly higher in MSA patients and were negatively correlated with cognitive function scores at baseline and 1-year follow-up. These findings suggest that cystatin C may serve as a potential biomarker for cognitive decline in patients with early-stage MSA.
FRONTIERS IN AGING NEUROSCIENCE
(2022)
Article
Clinical Neurology
Marie Meyer, Frederic Lamare, Julien Asselineau, Alexandra Foubert-Samier, Joachim Mazere, Paolo Zanotti-Fregonara, Gaia Rizzo, Anna Delamarre, Umberto Spampinato, Olivier Rascol, Anne Pavy-Le Traon, Francois Tison, Philippe Fernandez, Igor Sibon, Wassilios G. Meissner
Summary: This study compared brain 5-hydroxytryptamine(1A) receptor binding between multiple system atrophy (MSA) patients, Parkinson's disease (PD) patients, and healthy controls. The findings showed dysfunction in several brain regions in MSA patients, which may contribute to symptoms like fatigue, pain, and apathy.
MOVEMENT DISORDERS
(2021)
Article
Multidisciplinary Sciences
Nannan Li, Tianwen Yang, Weizheng Ran, Xinning Zhang, Yao Wang, Zhifang Xu, Shan Ren, Qianyu Zhao, Bingyu Guo, Sushi Wang, Fanxing Meng, Zhigang Chen
Summary: This study evaluated the cognitive function of MSA patients in China using a case-control study design and found that cognitive impairment is common among MSA patients, with distinct characteristics. Cognitive impairments in MSA patients were prominent in visuospatial/executive functions, attention, language, and orientation, with differences between MSA-P and MSA-C patients. Additionally, cognitive function was negatively correlated with the severity of MSA.
SCIENTIFIC REPORTS
(2021)
Article
Clinical Neurology
Aya Ogura, Kazuya Kawabata, Hirohisa Watanabe, Shao Wei Choy, Epifanio Bagarinao, Toshiyasu Kato, Kazunori Imai, Michihito Masuda, Reiko Ohdake, Kazuhiro Hara, Ryoichi Nakamura, Naoki Atsuta, Tomohiko Nakamura, Masahisa Katsuno, Gen Sobue
Summary: In this study, a novel framework for diffusion-weighted imaging analysis called Fixel-based analysis (FBA) was used to investigate white matter changes in ALS patients. The findings showed reduced fiber density and morphology in the bilateral corticospinal tracts and corpus callosum of ALS patients. Clinical scores were negatively correlated with fiber density values in the corticospinal tracts.
EUROPEAN JOURNAL OF NEUROLOGY
(2022)
Article
Radiology, Nuclear Medicine & Medical Imaging
Yuri Shimizu, Hiroko Satake, Satoko Ishigaki, Kazuhiro Shimamoto, Fuga Uota, Masanori Tadokoro, Tomohiro Sato, Katsuhiko Kato, Tsuneo Ishiguchi, Shinji Naganawa
Summary: The effects of age, mammographic density, menopausal status, and menstrual cycle on background parenchymal uptake (BPU) were quantitatively evaluated using ring-shaped dedicated breast positron emission tomography (dbPET). The study found that BPU in normal breast tissues was associated with mammographic density, menopausal status, and women's menstrual cycle. The menstrual cycle was significantly associated with BPU in premenopausal women with dense breasts but not in women with non-dense breasts.
ANNALS OF NUCLEAR MEDICINE
(2022)
Article
Radiology, Nuclear Medicine & Medical Imaging
Ryota Horiguchi, Yasuo Takehara, Masataka Sugiyama, Ryota Hyodo, Tomohiro Komada, Masaya Matsushima, Shinji Naganawa, Takashi Mizuno, Yasuo Sakurai, Masayuki Sugimoto, Hiroshi Banno, Kimihiro Komori, Keiichi Itatani
Summary: The aim of this study was to investigate the impact of endovascular aneurysmal repair (EVAR) on the local hemodynamics of abdominal aortic aneurysm (AAA). The results showed a 1.7-fold increase in local energy loss (EL) after EVAR, with a greater increase in the deformed stent-graft subgroup. These findings could be a potential concern for frail patients.
JOURNAL OF MAGNETIC RESONANCE IMAGING
(2023)
Review
Clinical Neurology
Hirohisa Watanabe, Sayuri Shima, Yasuaki Mizutani, Akihiro Ueda, Mizuki Ito
Summary: This review summarizes the improvements in understanding the pathophysiology and early clinical symptoms of multiple system atrophy (MSA), as well as advancements in diagnostic methods and disease-modifying therapies for the condition. The introduction of new diagnostic criteria in 2022 and the use of artificial intelligence and machine learning in neuroimaging have significantly improved diagnostic accuracy. Treatment advancements, including immunotherapy against alpha-synuclein and stem cell therapies, show promise in targeting different aspects of the disease.
JOURNAL OF MOVEMENT DISORDERS
(2023)
Article
Radiology, Nuclear Medicine & Medical Imaging
Tomohiro Tada, Kazuhiro Hara, Naotoshi Fujita, Yoshinori Ito, Hiroshi Yamaguchi, Reiko Ohdake, Kazuya Kawabata, Aya Ogura, Toshiyasu Kato, Takamasa Yokoi, Michihito Masuda, Shinji Abe, Shinichi Miyao, Shinji Naganawa, Masahisa Katsuno, Hirohisa Watanabe, Gen Sobue, Katsuhiko Kato
Summary: This study compared the validity of the pons and corpus callosum as reference regions for the quantitative evaluation of brain positron emission tomography. The results showed that using either the pons or corpus callosum as reference regions yielded similar results to using the cerebellar cortex, with high sensitivity and specificity.
ANNALS OF NUCLEAR MEDICINE
(2023)
Review
Radiology, Nuclear Medicine & Medical Imaging
Nivedita Agarwal, Laura D. Lewis, Lydiane Hirschler, Leonardo Rivera Rivera, Shinji Naganawa, Swati Rane Levendovszky, Geir Ringstad, Marijan Klarica, Joanna Wardlaw, Costantino Iadecola, Cheryl Hawkes, Roxana Octavia Carare, Jack Wells, Erik N. T. P. Bakker, Vartan Kurtcuoglu, Lynne Bilston, Maiken Nedergaard, Yuki Mori, Marcus Stoodley, Noam Alperin, Mony de Leon, Matthias J. P. van Osch
Summary: Neurofluids refer to all fluids in the brain and spine, including blood, cerebrospinal fluid, and interstitial fluid. Neuroscientists have identified various fluid environments that interact harmoniously to support optimal brain function. Animal studies have been crucial in understanding the dynamics of neurofluids, while human studies are limited due to the lack of noninvasive imaging techniques. The future development of noninvasive MRI techniques holds promise in imaging neurofluid dynamics and identifying pathological processes.
JOURNAL OF MAGNETIC RESONANCE IMAGING
(2023)
Review
Clinical Neurology
Toshiharu Nagatsu, Akira Nakashima, Hirohisa Watanabe, Shosuke Ito, Kazumasa Wakamatsu, Fabio A. Zucca, Luigi Zecca, Moussa Youdim, Maximilian Wulf, Peter Riederer, Johannes M. Dijkstra
Summary: The dark pigment neuromelanin (NM) is abundant in dopamine (DA) neurons in the substantia nigra (SN) and norepinephrine (NE) neurons in the locus coeruleus (LC) in the human brain. During the progression of Parkinson's disease (PD), NM levels in these neurons decrease. The synthesis pathway involves conversion of tyrosine to L-DOPA, then to DA or NE, followed by autoxidation and conversion to eumelanic or pheomelanic NM.
JOURNAL OF NEURAL TRANSMISSION
(2023)
Letter
Multidisciplinary Sciences
Shinji Naganawa, Yutaka Kato, Tadao Yoshida, Michihiko Sone
NATURE COMMUNICATIONS
(2023)
Article
Medicine, General & Internal
Tadashi Shiohama, Norihide Maikusa, Masahiro Kawaguchi, Jun Natsume, Yoshiyuki Hirano, Keito Saito, Jun-ichi Takanashi, Jacob Levman, Emi Takahashi, Koji Matsumoto, Hajime Yokota, Shinya Hattori, Keita Tsujimura, Daisuke Sawada, Tomoko Uchida, Tomozumi Takatani, Katsunori Fujii, Shinji Naganawa, Noriko Sato, Hiromichi Hamada, Andor W. J. M. Glaudemans
Summary: This study aims to provide normal reference values of global and regional brain volumes for children and adolescents of different sexes and age groups across multiple institutions. MRI examinations of 846 neurotypical participants were collected, and regional brain volumes were measured and analyzed using the CIVET 2.1.0. pipeline and ComBat-GAM harmonization. The results of this study can be used to evaluate the brain morphology of individuals in a clinical setting and investigate brain morphology in ultra-rare diseases.
Article
Medicine, Research & Experimental
Toshio Ohashi, Shinji Naganawa, Yusuke Nasu, Kayao Kuno, Katsuhiko Kato
Summary: Evaluation of endolymphatic hydrops (EH) using magnetic resonance (MR) imaging can be done by combining the reversed image of positive endolymph signal and native image of perilymph signal with heavily T2-weighted MR cisternography (HYDROPS-Mi2) imaging and the intravenous administration of a gadolinium-based contrast agent (IV-GBCA). However, the feasibility of EH evaluation by MR cisternography (MRC) without IV-GBCA has not been established. This study aimed to compare the effectiveness of HYDROPS-Mi2 imaging with IV-GBCA and MRC without IV-GBCA for EH evaluation.
NAGOYA JOURNAL OF MEDICAL SCIENCE
(2023)
Article
Otorhinolaryngology
Tadao Yoshida, Masumi Kobayashi, Satofumi Sugimoto, Shinji Naganawa, Michihiko Sone
Summary: Otitis media with antineutrophil cytoplasmic antibody-associated vasculitis (OMAAV) is a new type of otitis media. Early detection and intervention can improve the hearing loss caused by OMAAV, but recovering the patient's hearing may be challenging if it continues to worsen and leads to deafness. Bilateral deafness can only be improved through cochlear implant (CI) surgery. This article presents two cases of cochlear calcification caused by OMAAV and emphasizes the importance of early detection through computed tomography scans and timely CI surgery for hearing improvement in patients with OMAAV-induced deafness.
AURIS NASUS LARYNX
(2023)
Article
Radiology, Nuclear Medicine & Medical Imaging
Mika Tamura, Kunihiro Nakada, Haruna Iwanaga, Naotoshi Fujita, Katsuhiko Kato
Summary: The study found that adherence to a low-iodine diet can reduce urinary iodine concentrations in patients undergoing RAI treatment, but lower urinary iodine concentrations did not increase the success rate of RAI.
EUROPEAN JOURNAL OF NUCLEAR MEDICINE AND MOLECULAR IMAGING
(2023)
Meeting Abstract
Radiology, Nuclear Medicine & Medical Imaging
Takumi Inagaki, Hiroshi Yamaguchi, Yuki Asano, Katsuhiko Kato
JOURNAL OF NUCLEAR MEDICINE
(2022)
Meeting Abstract
Radiology, Nuclear Medicine & Medical Imaging
Kunihiro Nakada, Tsunenori Mizukoshi, Mika Tamura, Katsuhiko Kato, Noriyoshi Kato, Masayuki Sakurai
JOURNAL OF NUCLEAR MEDICINE
(2022)
Article
Clinical Neurology
Yoshio Araki, Takashi Mamiya, Naotoshi Fujita, Kenji Uda, Kinya Yokoyama, Fumiaki Kanamori, Kai Takayanagi, Kazuki Ishii, Masahiro Nishihori, Kazuhito Takeuch, Kuniaki Tanahashi, Yuichi Nagata, Yusuke Nishimura, Takafumi Tanei, Masaki Sumitomo, Sho Okamoto, Takashi Izumi, Katsuhiko Kato, Ryuta Saito
Summary: The purpose of this study was to examine the effects of combined revascularization for ischaemic-onset moyamoya disease (MMD) on cerebral haemodynamics. The results showed that combined revascularization did not lead to significant changes in blood flow during the postoperative chronic phase.
NEUROSURGICAL REVIEW
(2022)
