Journal of Movement Disorders

Review Article

α-Synuclein: A Promising Biomarker for Parkinson’s Disease and Related Disorders: Taku Hatano, Ayami Okuzumi, Gen Matsumoto, Tsunemi Taiji, Nobutaka Hattori; Received March 22, 2024 Accepted April 9, 2024 Published online April 9, 2024; DOI: https://doi.org/10.14802/jmd.24075 [Accepted]

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Abstract PDF: Mutations in the SNCA gene, which encodes α-synuclein (α-syn), play a key role in the development of genetic Parkinson’s disease (PD). α-Syn is a major component of Lewy bodies in PD and glial cytoplasmic inclusions in multiple system atrophy (MSA). Rapid eye movement sleep behavior disorder (RBD) patients often progress to PD, dementia with Lewy bodies (DLB), or MSA, collectively known as α-synucleinopathies. The loss of dopaminergic neurons with Lewy bodies precedes motor dysfunction in these diseases, but the mechanisms of neurodegeneration due to α-syn aggregation are poorly understood. Monitoring α-syn aggregation in vivo could serve as a diagnostic biomarker and help elucidate the pathogenesis, necessitating a simple and accurate detection method. Seed amplification assays (SAAs), such as real-time quaking-induced conversion (RT-QuIC) and protein misfolding cyclic amplification (PMCA), are used to detect small amounts of abnormally structured α-syn protofibrils, which are central to aggregation. These methods are promising for the early diagnosis of α-synucleinopathy. Differences in α-syn filament structures between α-synucleinopathies, observed through transmission electron microscopy and cryo-electron microscopy, suggest their role in the pathogenesis of neurodegeneration. SAAs may differentiate between subtypes of α-synucleinopathy and other diseases. Efforts are also being made to identify α-syn from blood using various methods. This review introduces body fluid α-syn biomarkers based on pathogenic α-syn seeds, which are expected to redefine α-synucleinopathy diagnosis and staging, improving clinical research accuracy and facilitating biomarker development.

Original Article

Emotion Recognition in Multiple System Atrophy: An Exploratory Eye-Tracking Study: Victoria Sidoroff, Federico Carbone, Philipp Ellmerer, Stefanie Bair, Alexandra Hoffmann, Thomas Maran, Florian Krismer, Philipp Mahlknecht, Katherina Mair, Cecilia Raccagni, Jean-Pierre Ndayisaba, Klaus Seppi, Gregor K. Wenning, Atbin Djamshidian; J Mov Disord. 2024;17(1):38-46. Published online September 26, 2023; DOI: https://doi.org/10.14802/jmd.23090

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Abstract PDF: Objective
Emotional processing is a core feature of social interactions and has been well studied in patients with idiopathic Parkinson’s disease (PD), albeit with contradictory results. However, these studies excluded patients with atypical parkinsonism, such as multiple system atrophy (MSA). The objective of this exploratory study was to provide better insights into emotion processing in patients with MSA using eye tracking data.
Methods
We included 21 MSA patients, 15 PD patients and 19 matched controls in this study. Participants performed a dynamic and a static emotion recognition task, and gaze fixations were analyzed in different areas of interest. Participants underwent neuropsychological testing and assessment of depression and alexithymia.
Results
MSA patients were less accurate in recognizing anger than controls (p = 0.02) and had overall fewer fixations than controls (p = 0.001). In the static task, MSA patients had fewer fixations (p < 0.001) and a longer time to first fixation (p = 0.026) on the eye region. Furthermore, MSA patients had a longer fixation duration overall than PD patients (p = 0.004) and longer fixations on the nose than controls (p = 0.005). Alexithymia scores were higher in MSA patients compared to controls (p = 0.038).
Conclusion
This study demonstrated impaired recognition of anger in MSA patients compared to HCs. Fewer and later fixations on the eyes along with a center bias suggest avoidance of eye contact, which may be a characteristic gaze behavior in MSA patients.

Brief communication

Sex and Gender Influence Urinary Symptoms and Management in Multiple System Atrophy: Elke Schipani Bailey, Sara J. Hooshmand, Negin Badihian, Paola Sandroni, Eduardo E. Benarroch, James H. Bower, Phillip A. Low, Wolfgang Singer, Elizabeth A. Coon; J Mov Disord. 2023;16(2):196-201. Published online May 24, 2023; DOI: https://doi.org/10.14802/jmd.23016

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Abstract PDF

Objective
Multiple system atrophy (MSA) is characterized by urinary dysfunction, yet the influence of sex and gender on urinary symptoms and treatment is unclear. We sought to characterize sex and gender differences in the symptomatology, evaluation, and management of urinary dysfunction in patients with MSA.
Methods
Patients with MSA evaluated at our institution were reviewed and stratified by sex.
Results
While the prevalence of urinary symptoms was similar in male and female patients, incontinence was more common in females. Despite this, males and females underwent postvoid residual (PVR) measurement at similar rates. While catheterization rates were similar when PVR was measured, males were more than twice as likely to be catheterized than females in the absence of PVR measurement.
Conclusion
Urinary symptoms are common in MSA, but their presentation differs between males and females. The difference in catheterization rates may be driven by a gender disparity in referrals for PVR, which can guide treatment.

Citations

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In vivo cerebral metabolic and dopaminergic characteristics in multiple system atrophy with orthostatic hypotension
Chenxi Xue, Xiaofeng Dou, Congcong Yu, Yan Zhong, Jing Wang, Xiang Zhang, Le Xue, Daoyan Hu, Shuang Wu, Hong Zhang, Mei Tian
European Journal of Nuclear Medicine and Molecular Imaging.2024; 51(2): 468. CrossRef

Review Article

Multiple System Atrophy: Advances in Diagnosis and Therapy: Hirohisa Watanabe, Sayuri Shima, Yasuaki Mizutani, Akihiro Ueda, Mizuki Ito; J Mov Disord. 2023;16(1):13-21. Published online December 20, 2022; DOI: https://doi.org/10.14802/jmd.22082

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Abstract PDF

This review summarizes improvements in understanding the pathophysiology and early clinical symptoms of multiple system atrophy (MSA) and advancements in diagnostic methods and disease-modifying therapies for the condition. In 2022, the Movement Disorder Society proposed new diagnostic criteria to develop disease-modifying therapies and promote clinical trials of MSA since the second consensus was proposed in 2008. Regarding pathogenesis, cutting-edge findings have accumulated on the interactions of α-synuclein, neuroinflammation, and oligodendroglia with neurons. In neuroimaging, introducing artificial intelligence, machine learning, and deep learning has notably improved diagnostic accuracy and individual analyses. Advancements in treatment have also been achieved, including immunotherapy therapy against α-synuclein and serotonin-targeted and mesenchymal stem cell therapies, which are thought to affect several aspects of the disease, including neuroinflammation. The accelerated progress in clarifying the pathogenesis of MSA over the past few years and the development of diagnostic techniques for detecting early-stage MSA are expected to facilitate the development of disease-modifying therapies for one of the most intractable neurodegenerative diseases.

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A Blinded Evaluation of Brain Morphometry for Differential Diagnosis of Atypical Parkinsonism
Kazuya Kawabata, Florian Krismer, Beatrice Heim, Anna Hussl, Christoph Mueller, Christoph Scherfler, Elke R. Gizewski, Klaus Seppi, Werner Poewe
Movement Disorders Clinical Practice.2024; 11(4): 381. CrossRef
The potential of phosphorylated α‐synuclein as a biomarker for the diagnosis and monitoring of multiple system atrophy
Toufik Abdul‐Rahman, Ranferi Eduardo Herrera‐Calderón, Arjun Ahluwalia, Andrew Awuah Wireko, Tomas Ferreira, Joecelyn Kirani Tan, Maximillian Wolfson, Shankhaneel Ghosh, Viktoriia Horbas, Vandana Garg, Asma Perveen, Marios Papadakis, Ghulam Md Ashraf, Ath
CNS Neuroscience & Therapeutics.2024;[Epub] CrossRef

Original Articles

Semiautomated Algorithm for the Diagnosis of Multiple System Atrophy With Predominant Parkinsonism: Woong-Woo Lee, Han-Joon Kim, Hong Ji Lee, Han Byul Kim, Kwang Suk Park, Chul-Ho Sohn, Beomseok Jeon; J Mov Disord. 2022;15(3):232-240. Published online July 26, 2022; DOI: https://doi.org/10.14802/jmd.21178

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Abstract PDF Supplementary Material: Objective
Putaminal iron deposition is an important feature that helps differentiate multiple system atrophy with predominant parkinsonism (MSA-p) from Parkinson’s disease (PD). Most previous studies used visual inspection or quantitative methods with manual manipulation to perform this differentiation. We investigated the value of a new semiautomated diagnostic algorithm using 3T-MR susceptibility-weighted imaging for MSA-p.
Methods
This study included 26 MSA-p, 68 PD, and 41 normal control (NC) subjects. The algorithm was developed in 2 steps: 1) determine the image containing the remarkable putaminal margin and 2) calculate the phase-shift values, which reflect the iron concentration. The next step was to identify the best differentiating conditions among several combinations. The highest phaseshift value of each subject was used to assess the most effective diagnostic set.
Results
The raw phase-shift values were present along the lateral margin of the putamen in each group. It demonstrates an anterior- to-posterior gradient that was identified most frequently in MSA-p. The average of anterior 5 phase shift values were used for normalization. The highest area under the receiver operating characteristic curve (0.874, 80.8% sensitivity, and 86.7% specificity) of MSA-p versus PD was obtained under the combination of 3 or 4 vertical pixels and one dominant side when the normalization methods were applied. In the subanalysis for the MSA-p patients with a longer disease duration, the performance of the algorithm improved.
Conclusion
This algorithm detected the putaminal lateral margin well, provided insight into the iron distribution of the putaminal rim of MSA-p, and demonstrated good performance in differentiating MSA-p from PD.

Fecal Calprotectin in Parkinson’s Disease and Multiple System Atrophy: Jia Wei Hor, Shen-Yang Lim, Eng Soon Khor, Kah Kian Chong, Sze Looi Song, Norlinah Mohamed Ibrahim, Cindy Shuan Ju Teh, Chun Wie Chong, Ida Normiha Hilmi, Ai Huey Tan; J Mov Disord. 2022;15(2):106-114. Published online December 24, 2021; DOI: https://doi.org/10.14802/jmd.21085

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Abstract PDF Supplementary Material

Objective
Converging evidence suggests that intestinal inflammation is involved in the pathogenesis of neurodegenerative diseases. Previous studies on fecal calprotectin in Parkinson’s disease (PD) were limited by small sample sizes, and literature regarding intestinal inflammation in multiple system atrophy (MSA) is very scarce. We investigated the levels of fecal calprotectin, a marker of intestinal inflammation, in PD and MSA.
Methods
We recruited 169 subjects (71 PD, 38 MSA, and 60 age-similar nonneurological controls). Clinico-demographic data were collected. PD and MSA were subtyped and the severity assessed using the MDS-UPDRS and UMSARS, respectively. Fecal calprotectin and blood immune markers were analyzed.
Results
Compared to controls (median: 35.7 [IQR: 114.2] μg/g), fecal calprotectin was significantly elevated in PD (median: 95.6 [IQR: 162.1] μg/g, p = 0.003) and even higher in MSA (median: 129.5 [IQR: 373.8] μg/g, p = 0.002). A significant interaction effect with age was observed; between-group differences were significant only in older subjects (i.e., ≥ 61 years) and became more apparent with increasing age. A total of 28.9% of MSA and 18.3% of PD patients had highly abnormal fecal calprotectin levels (≥ 250 μg/g); however, this difference was only significant for MSA compared to controls. Fecal calprotectin correlated moderately with selected blood immune markers in PD, but not with clinical features of PD or MSA.
Conclusions
Elevated fecal calprotectin suggests a role for intestinal inflammation in PD and MSA. A more complete understanding of gut immune alterations could open up new avenues of research and treatment for these debilitating diseases.

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Review Article

Progressive Supranuclear Palsy with Predominant Cerebellar Ataxia: Shoichiro Ando, Masato Kanazawa, Osamu Onodera; J Mov Disord. 2020;13(1):20-26. Published online December 19, 2019; DOI: https://doi.org/10.14802/jmd.19061

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Abstract PDF

Progressive supranuclear palsy (PSP) is characterized by supranuclear gaze palsy, dystonic rigidity of the neck and upper trunk, frequent falls and mild cognitive impairment. Cerebellar ataxia is one of the exclusion criteria given by the National Institute of Neurological Disorders and Stroke and the Society for Progressive Supranuclear Palsy. As a result, pathologically proven PSP patients exhibiting cerebellar ataxia have often been misdiagnosed with spinocerebellar degeneration, specifically multiple system atrophy with predominant cerebellar ataxia (MSA-C). However, more recently, it has been recognized that patients with PSP can present with truncal and limb ataxia as their initial symptom and/or main manifestation. These patients can be classified as having PSP with predominant cerebellar ataxia (PSP-C), a new subtype of PSP. Since the development of this classification, patients with PSP-C have been identified primarily in Asian countries, and it has been noted that this condition is very rare in Western communities. Furthermore, the clinical features of PSP-C have been identified, enabling it to be distinguished from other subtypes of PSP and MSA-C. In this review, we describe the clinical and neuropathological features of PSP-C. The hypothesized pathophysiology of cerebellar ataxia in PSP-C is also discussed.

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Original Articles

Clinical Milestones Preceding the Diagnosis of Multiple System Atrophy and Progressive Supranuclear Palsy: A Retrospective Cohort Study: Louise Wiblin, Rory Durcan, Brook Galna, Mark Lee, David Burn; J Mov Disord. 2019;12(3):177-183. Published online August 9, 2019; DOI: https://doi.org/10.14802/jmd.19015

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Abstract PDF

Objective
Multiple System Atrophy (MSA) and progressive supranuclear palsy (PSP) are rapidly progressive forms of degenerative Parkinsonism. The difficulties of diagnosing MSA and PSP in their early stages may lead to delayed referral to appropriate specialists and distress to patients, as well as delaying symptomatic treatment and participation in clinical trials. This work aimed to describe the symptoms that patients with MSA and PSP developed and plot their emergence relative to final diagnosis using a median onset in months.
Methods
Forty-seven patients from the United Kingdom with MSA or PSP diagnosed by a movement disorder specialist were interviewed with carers or relatives to establish milestone onset. This was corroborated using clinical notes and letters.
Results
In the MSA cohort (n = 23), autonomic symptoms (median 5.5 months before diagnosis) and falls (median 1 month before diagnosis) were the two clinical milestones which occurred before diagnosis. In the PSP cohort (n = 24), falling was the only milestone which occurred before diagnosis (median of 18.5 months).
Conclusion
This Study Shows That Psp Patients Experience Falling More Than A Year And A Half An Average Before Receiving A Diagnosis And Although Msa Patients Also Tended To Fall, This Was Much Closer To The Time Of Diagnosis. Further Work With Larger Cohorts May Illustrate Whether These Preliminary Findings Can Be Generalised To Guide Diagnosis And Management.

Citations

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Clinical milestones as triggers for palliative care intervention in progressive Supranuclear palsy and multiple system atrophy
Robin Bessemer, Alla Iansavichene, Mary E. Jenkins, Elizabeth Finger, Teneille E. Gofton
Journal of the Neurological Sciences.2023; 448: 120614. CrossRef
Toward More Accessible Fully Automated 3D Volumetric MRI Decision Trees for the Differential Diagnosis of Multiple System Atrophy, Related Disorders, and Age-Matched Healthy Subjects
Jisoo Kim, Geoffrey S. Young, Andrew S. Willett, Ariana T. Pitaro, Grace F. Crotty, Merlyne Mesidor, Kristie A. Jones, Camden Bay, Min Zhang, Mel B. Feany, Xiaoyin Xu, Lei Qin, Vikram Khurana
The Cerebellum.2022; 22(6): 1098. CrossRef
Disease course and treatment patterns in progressive supranuclear palsy: A real-world study
John C. Morgan, Xiaolan Ye, Jennifer A. Mellor, Keisha J. Golden, Jorge Zamudio, Louis A. Chiodo, Yanjun Bao, Tao Xie
Journal of the Neurological Sciences.2021; 421: 117293. CrossRef
Patient and care partner views on exercise and structured physical activity for people with Progressive Supranuclear Palsy
Susan C. Slade, Christopher Bruce, Jennifer L. McGinley, Bastiaan R. Bloem, Meg E. Morris, John Duda
PLOS ONE.2020; 15(6): e0234265. CrossRef
Effect of cold oral stimulation on orthostatic hypotension in multiple system atrophy: a case study
Hironobu Uzawa, Shinta Takeuchi, Yusuke Nishida
Journal of Physical Therapy Science.2020; 32(7): 473. CrossRef

The ‘Hot Cross Bun’ Sign Is Not Always Multiple System Atrophy: Etiologies of 11 Cases: Christopher Way, David Pettersson, Amie Hiller; J Mov Disord. 2019;12(1):27-30. Published online December 19, 2018; DOI: https://doi.org/10.14802/jmd.18031

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Abstract PDF

Objective
To clarify the specificity of the ‘hot cross bun’ sign (HCBS) for multiple system atrophy (MSA) in adult cerebellar ataxia or parkinsonism.
Methods
The radiologic information systems at an academic center and affiliated veterans’ hospital were queried using the keywords ‘hot cross bun,’ ‘pontocerebellar,’ ‘cruciate,’ ‘cruciform,’ ‘MSA,’ ‘multiple system atrophy,’ and ‘multisystem atrophy.’ Scans were reviewed by a neurologist and neuroradiologist to identify the HCBS. Subjects with the HCBS were reviewed by 2 neurologists to identify the most likely etiology of the patient’s neurologic symptoms.
Results
Eleven cases were identified. Etiologies included MSA (4 probable, 2 possible), hereditary cerebellar ataxia (3/11), probable dementia with Lewy bodies (1/11), and uncertain despite autopsy (1/11).
Conclusion
MSA was the most common etiology. However, 5 of the 11 patients did not have MSA. The most common alternate etiology was an undefined hereditary cerebellar ataxia (3/11).

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The “Black Straight-Line Sign” in the Putamen in Diffusion-Weighted Imaging: A Potential Diagnostic MRI Marker for Multiple System Atrophy
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Hot cross bun sign
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Case Report

A Case of Multiple System Atrophy-Cerebellar Type Preceded by Dementia: Eun Hye Jang, Joo Kyung Lee, Hyun Jung Jang, Mi-Jung Kim, Sun Ju Chung; J Mov Disord. 2012;5(2):48-52.; DOI: https://doi.org/10.14802/jmd.12011

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Abstract PDF

Multiple system atrophy (MSA) is a sporadic, adult-onset disease characterized by progressive degeneration of nervous systems including cerebellar, pyramidal, extrapyramidal, and autonomic system. Although a few recent studies reported that cognitive impairments could occur in patients with MSA, prominent dementia with progressive decline is not a typical clinical manifestation of MSA. In particular, dementia with MSA-cerebellar type is very rare. We have experienced a patient with 2-year history of severe cognitive impairment, who was finally diagnosed as MSA-cerebellar type.

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Mitochondrial dysfunction and altered ribostasis in hippocampal neurons with cytoplasmic inclusions of multiple system atrophy
Norihisa Maeda, Hiroyuki Honda, Satoshi O. Suzuki, Naoki Fujii, Jun‐ichi Kira, Toru Iwaki
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Mechanisms and prevention of sudden death in multiple system atrophy
Takayoshi Shimohata, Naotaka Aizawa, Hideaki Nakayama, Hiroshige Taniguchi, Yasuyoshi Ohshima, Hitoshi Okumura, Tetsuya Takahashi, Akio Yokoseki, Makoto Inoue, Masatoyo Nishizawa
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The Clinical Neuropsychologist.2016; 30(6): 849. CrossRef

Original Articles

Clinical Features and Disability Milestones in Multiple System Atrophy and Progressive Supranuclear Palsy: Sang-Wook Lee, Seong-Beom Koh; J Mov Disord. 2012;5(2):42-47.; DOI: https://doi.org/10.14802/jmd.12010

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Abstract PDF

Multiple system atrophy (MSA) and progressive supranuclear palsy (PSP) are an adult-onset progressive neurodegenerative disorder that are known to display diverse clinical features and disease progression. We aim to characterize the clinical features and disease progression in patients with MSA and PSP by using a number of relevant disability milestones in Koreans. Forty-one patients with MSA and 14 patients with PSP had been enrolled. The mean age at onset of MSA-C, MSA-P and PSP was 56.7 ± 7.8, 62.5 ± 8.0, 68.9 ± 6.1 years respectively. The most commonly reported symptom at disease onset is disequilibrium/dizziness in MSA-C, tremor in MSA-P and frequent falling in PSP. The mean duration of reaching milestones after disease onset in MSA-C were as followings: 20.8 (urinary incontinence), 22.9 (frequent falling), 27.8 (wheelchair bound), 31.8 (dysarthria) and 35.8 months (diagnosis). The mean duration of reaching milestones after disease onset were 22.0 (urinary incontinence), 32.6 (frequent falling and diagnosis), 41.2 (dysarthria), 61.4 months (wheelchair bound) in MSA-P and 16.8 (dysarthria), 21.6 (diagnosis), 21.7 (frequent falling), 24.0 months (wheel chair bound) in PSP. In the case of MSA, dizziness may occur for the first time. Thus, when the patient complains of non-specific dizziness, a follow-up examination to distinguish it from MSA can be helpful. There was a trend for patients with MSA-C to reach more disability milestones than in MSA-P and PSP before diagnosis. It may explain why patients with MSA-C are required more detail history taking and neurologic examination at an earlier stage.

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Disease course and treatment patterns in progressive supranuclear palsy: A real-world study
John C. Morgan, Xiaolan Ye, Jennifer A. Mellor, Keisha J. Golden, Jorge Zamudio, Louis A. Chiodo, Yanjun Bao, Tao Xie
Journal of the Neurological Sciences.2021; 421: 117293. CrossRef
Progression of Oropharyngeal Dysphagia in Patients with Multiple System Atrophy
Hui Jae Do, Han Gil Seo, Hyun Haeng Lee, Byung-Mo Oh, Yoon Kim, Aryun Kim, Han-Joon Kim, Beomseok Jeon, Tai Ryoon Han
Dysphagia.2020; 35(1): 24. CrossRef
Is There a Difference in Autonomic Dysfunction Between Multiple System Atrophy Subtypes?
Divyani Garg, Achal Kumar Srivastava, Ashok Kumar Jaryal, Roopa Rajan, Akanksha Singh, Awadh Kishor Pandit, Deepti Vibha, Garima Shukla, Ajay Garg, Ravindra Mohan Pandey, Kameshwar Prasad
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Models of multiple system atrophy
He-Jin Lee, Diadem Ricarte, Darlene Ortiz, Seung-Jae Lee
Experimental & Molecular Medicine.2019; 51(11): 1. CrossRef
Multiple System Atrophy: An Oligodendroglioneural Synucleinopathy1
Kurt A. Jellinger, George Perry, Jesus Avila, Massimo Tabaton, Xiongwei Zhu
Journal of Alzheimer's Disease.2018; 62(3): 1141. CrossRef
Present and future of disease-modifying therapies in multiple system atrophy
Miguel Lopez-Cuina, Alexandra Foubert-Samier, François Tison, Wassilios G. Meissner
Autonomic Neuroscience.2018; 211: 31. CrossRef
Different subregional metabolism patterns in patients with cerebellar ataxia by 18F-fluorodeoxyglucose positron emission tomography
Minyoung Oh, Jae Seung Kim, Jungsu S. Oh, Chong Sik Lee, Sun Ju Chung, Byeong-Cheol Ahn
PLOS ONE.2017; 12(3): e0173275. CrossRef
Vestibular Deficits in Neurodegenerative Disorders: Balance, Dizziness, and Spatial Disorientation
Thomas Cronin, Qadeer Arshad, Barry M. Seemungal
Frontiers in Neurology.2017;[Epub] CrossRef
Progressive supranuclear palsy: progression and survival
Julieta E. Arena, Stephen D. Weigand, Jennifer L. Whitwell, Anhar Hassan, Scott D. Eggers, Günter U. Höglinger, Irene Litvan, Keith A. Josephs
Journal of Neurology.2016; 263(2): 380. CrossRef
Multiple system atrophy: pathogenic mechanisms and biomarkers
Kurt A. Jellinger, Gregor K. Wenning
Journal of Neural Transmission.2016; 123(6): 555. CrossRef
Urinary Dysfunction in Progressive Supranuclear Palsy Compared with Other Parkinsonian Disorders
Tatsuya Yamamoto, Fuyuki Tateno, Ryuji Sakakibara, Shogo Furukawa, Masato Asahina, Tomoyuki Uchiyama, Shigeki Hirano, Yoshitaka Yamanaka, Miki Fuse, Yasuko Koga, Mitsuru Yanagisawa, Satoshi Kuwabara, Jong-Ling Fuh
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Rikitha Menezes, Alexander Pantelyat, Izlem Izbudak, Julius Birnbaum
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Preliminary Study of Intravenous Amantadine Treatment for Ataxia Management in Patients with Probable Multiple System Atrophy with Predominant Cerebellar Ataxia: Jinyoung Youn, Hyeeun Shin, Ji Sun Kim, Jin Whan Cho; J Mov Disord. 2012;5(1):1-4.; DOI: https://doi.org/10.14802/jmd.12001

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Abstract PDF

Background and Purpose:

Multiple system atrophy with predominant cerebellar ataxia is a disabling neurologic disease. However, effective management has not yet been established. We conducted a short-term, open-label preliminary study to assess the benefits of intravenous amantadine treatment in patients with probable multiple system atrophy with predominant cerebellar ataxia.

Methods:

Twenty patients (10 male, 10 female) with probable multiple system atrophy with predominant cerebellar ataxia received 400 mg of amantadine by intravenous per day for 5 days. Ataxia severity was evaluated by the International Cooperative Ataxia Rating Scale before and after intravenous amantadine therapy and all subjects reported subjective improvement after intravenous amantadine treatment using a patient global impression scale. We analyzed the total and subscale scores by the ataxia scale and patient global impression scale.

Results:

The mean age was 57.4 years (range: 47–72) and the mean disease duration was 30.8 months (range: 11–79). The ataxia severity significantly decreased after intravenous amantadine therapy from 42.5 to 37.3 (p < 0.001). The mean patient global impression scale for improvement was 2.9 and there were no side effects of intravenous amantadine treatment observed. When we assessed responders, the duration of intravenous amantadine effect was more than 1 month in 4 subjects of 7 responders.

Conclusions:

Our findings suggest that intravenous amantadine treatment can be a safe management option in cerebellar ataxia, although the mechanism is unclear. Thus, further double-blind, long-term studies with a larger sample size are needed.

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Low Dose Amantadine and Escitalopram in Progressive Supranuclear Palsy and Multiple System Atrophy
Porimita Chutia, Shailendra Mohan Tripathi
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Amantadine withdrawal in a patient with spinocerebellar ataxia
Andrew Pak, Emiley Chang
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M1 and Cerebellar tDCS for MSA-C: a Double-Blind, Randomized, Sham-Controlled, Crossover Study
Jong Hyeon Ahn, Dongyeong Lee, Minkyeong Kim, Jin Whan Cho, Won Hyuk Chang, Jinyoung Youn
The Cerebellum.2022; 22(3): 386. CrossRef
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Ghada M. Aboelfadl, Saeid Elsawy, Belal O. Elnady, Rasha Hamed
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E.A. Katunina
Zhurnal nevrologii i psikhiatrii im. S.S. Korsakova.2021; 121(4): 101. CrossRef
Efficacy of Parenteral Amantadine Therapy in the Treatment of Multiple System Atrophy With Predominant Parkinsonism
Adit Friedberg, Ilana Erikh, Maria Nassar, Elliot Sprecher, Ilana Schlesinger
Clinical Neuropharmacology.2018; 41(5): 160. CrossRef

Putaminal Hypointensity in the Parkinsonian Variant of Multiple System Atrophy: Simple Visual Assessment Using Susceptibility-Weighted Imaging: Jae-Hyeok Lee, Seung-Kug Baik; J Mov Disord. 2011;4(2):60-63.; DOI: https://doi.org/10.14802/jmd.11012

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Abstract PDF

Background and Purpose

Susceptibility-weighted imaging (SWI) has been shown to be superior in its ability to demonstrate brain mineralization than other conventional MR imaging. The goal of our study was therefore to assess the frequency and extent of putaminal hypointensity in parkinsonian variant MSA using SWI.

Methods

11 patients with multiple system atrophy-parkinsonian type (MSA-p), 30 patients with Parkinson’s disease (PD), and age matched 30 controls were investigated using 3 Tesla MRI. The pattern of putaminal hypointensity was measured using a visual grading scale and scored from 0 to 3.

Results

Hemi- or bilateral putaminal hypointensity (a score of ≥ 2) and hyperintense rim were recognized in 81.8% and 54.5% of 11 MSA-p, respectively. The scores of putaminal hypointensity of MSA-p were significantly higher than other groups (p < 0.001), a score of ≥ 2 differentiated MSA-p from other groups. And all five patients with early disease stage also showed these characteristic findings.

Conclusions

SWI appears to be useful for depicting putaminal hypointensity even in early stage of MSA-p. This finding suggests that iron deposition associated putaminal degeneration can occur early in the disease process.

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The Irony of Iron: The Element with Diverse Influence on Neurodegenerative Diseases
Seojin Lee, Gabor G. Kovacs
International Journal of Molecular Sciences.2024; 25(8): 4269. CrossRef
Comparison of the second consensus statement with the movement disorder society criteria for multiple system atrophy: A single-center analysis
Yunchuang Sun, Wei Sun, Luhua Wei, Fan Li, Yanyan Jiang, Fei Zhai, Mingyue Luan, Jing Chen, Zhaoxia Wang
Parkinsonism & Related Disorders.2023; 106: 105242. CrossRef
Deep learning segmentation results in precise delineation of the putamen in multiple system atrophy
Alexander Rau, Nils Schröter, Michel Rijntjes, Fabian Bamberg, Wolfgang H. Jost, Maxim Zaitsev, Cornelius Weiller, Stephan Rau, Horst Urbach, Marco Reisert, Maximilian F. Russe
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Multi-parametric radiomics of conventional T1 weighted and susceptibility-weighted imaging for differential diagnosis of idiopathic Parkinson’s disease and multiple system atrophy
Shuting Bu, Huize Pang, Xiaolu Li, Mengwan Zhao, Juzhou Wang, Yu Liu, Hongmei Yu
BMC Medical Imaging.2023;[Epub] CrossRef
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Su Jin Lim, Chong Hyun Suh, Woo Hyun Shim, Sang Joon Kim
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Halil Onder
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Nicola Tambasco, Pasquale Nigro, Andrea Chiappiniello, Federico Paolini Paoletti, Sara Scialpi, Simone Simoni, Pietro Chiarini, Lucilla Parnetti, Barbara Picconi
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Yun Soo Kim, Jae-Hyeok Lee, Jin Kyu Gahm
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Yiming Zheng, Xiwen Wang, Huajian Zhao, Yanyan Jiang, Ying Zhu, Jing Chen, Wei Sun, Zhaoxia Wang, Yunchuang Sun
Frontiers in Neurology.2022;[Epub] CrossRef
Role of zinc transporter ZIP12 in susceptibility‐weighted brain magnetic resonance imaging (MRI) phenotypes and mitochondrial function
Morgan D. Strong, Matthew D. Hart, Tony Z. Tang, Babajide A. Ojo, Lei Wu, Mariah R. Nacke, Workneh T. Agidew, Hong J. Hwang, Peter R. Hoyt, Ahmed Bettaieb, Stephen L. Clarke, Brenda J. Smith, Barbara J. Stoecker, Edralin A. Lucas, Dingbo Lin, Winyoo Chowa
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Matthew R. Burns, Nikolaus R. McFarland
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Morphology and signal changes of the lentiform nucleus based on susceptibility weighted imaging in parkinsonism-predominant multiple system atrophy
Qingguo Ren, Xiangshui Meng, Bin Zhang, Jianyuan Zhang, Xinyan Shuai, Xiaomin Nan, Cuiping Zhao
Parkinsonism & Related Disorders.2020; 81: 194. CrossRef
Abnormalities on structural MRI associate with faster disease progression in multiple system atrophy
Florian Krismer, Klaus Seppi, Gregor K. Wenning, Spyridon Papapetropoulos, Victor Abler, Georg Goebel, Michael Schocke, Werner Poewe
Parkinsonism & Related Disorders.2019; 58: 23. CrossRef
Brain Iron Accumulation in Atypical Parkinsonian Syndromes: in vivo MRI Evidences for Distinctive Patterns
Jae-Hyeok Lee, Myung-Sik Lee
Frontiers in Neurology.2019;[Epub] CrossRef
Quantitative Validation of a Visual Rating Scale for Defining High-Iron Putamen in Patients With Multiple System Atrophy
Myung Jun Lee, Tae-Hyung Kim, Seung Joo Kim, Baik-Kyun Kim, Chi-Woong Mun, Jae-Hyeok Lee
Frontiers in Neurology.2019;[Epub] CrossRef
Looking Deep into the Eye-of-the-Tiger in Pantothenate Kinase–Associated Neurodegeneration
J.-H. Lee, A. Gregory, P. Hogarth, C. Rogers, S.J. Hayflick
American Journal of Neuroradiology.2018; 39(3): 583. CrossRef
The Relevance of Iron in the Pathogenesis of Multiple System Atrophy: A Viewpoint
Christine Kaindlstorfer, Kurt A. Jellinger, Sabine Eschlböck, Nadia Stefanova, Günter Weiss, Gregor K. Wenning
Journal of Alzheimer's Disease.2018; 61(4): 1253. CrossRef
Using ‘swallow-tail’ sign and putaminal hypointensity as biomarkers to distinguish multiple system atrophy from idiopathic Parkinson’s disease: A susceptibility-weighted imaging study
Na Wang, HuaGuang Yang, ChengBo Li, GuoGuang Fan, XiaoGuang Luo
European Radiology.2017; 27(8): 3174. CrossRef
Magnetic resonance imaging for the diagnosis of Parkinson’s disease
Beatrice Heim, Florian Krismer, Roberto De Marzi, Klaus Seppi
Journal of Neural Transmission.2017; 124(8): 915. CrossRef
Utility of susceptibility-weighted imaging in Parkinson’s disease and atypical Parkinsonian disorders
Zhibin Wang, Xiao-Guang Luo, Chao Gao
Translational Neurodegeneration.2016;[Epub] CrossRef

Cognitive Impairments in Multiple System Atrophy of the Cerebellar Type: Hyun J. Hong, Sook Keun. Song, Phil Hyu Lee, Young Ho Sohn, Ji E. Lee; J Mov Disord. 2011;4(1):41-45.; DOI: https://doi.org/10.14802/jmd.11007

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Abstract PDF

Background and Purpose

We investigated the cognitive profiles in a large sample of patients with multiple system atrophy-cerebellar ataxia (MSA-C) and compared directly them in patients with clinical diagnosis of probable MSA-C without dementia and control subjects with intact cognition.

Methods

We prospectively enrolled 26 patients with clinical diagnosis of probable MSA-C. All patients underwent a standardized neuropsychological test of the Seoul Neuropsychological Screening Battery.

Results

The score of Korean version of the Mini- Mental State Examination was significantly lower in patients with MSA-C (27.2 ± 2.5) than in control subjects (28.9 ± 1.0, p = 0.003). Patients with MSA-C showed a significantly worse performance in visuospatial function, 3 words recall, verbal immediate, delayed and recognition memory, visual delayed memory, phonemic and sementic Controlled Oral Word Association Test, and ideomotor praxis (p < 0.05).

Conclusions

Patients with MSA-C show more severe and more widespread cognitive dysfunctions than controls. Our results also indicate that cognitive dysfunction in patients with MCA-C is suggestive of disruption of the cerebellocortical circuits.

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Simona Raimo, Mariachiara Gaita, Maria Cropano, Giusi Mautone, Alfonsina D’Iorio, Luigi Trojano, Gabriella Santangelo
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Morphological differences between the two major subtypes of multiple system atrophy with cognitive impairment
Kurt A. Jellinger
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Mild cognitive impairment in multiple system atrophy: a brain network disorder
Kurt A. Jellinger
Journal of Neural Transmission.2023; 130(10): 1231. CrossRef
Cognition in Patients With Multiple System Atrophy (MSA) and Its Neuroimaging Correlation: A Prospective Case-Control Study
Santosh Dash, Rohan Mahale, M. Netravathi, Nitish L Kamble, Vikram Holla, Ravi Yadav, Pramod K Pal
Cureus.2022;[Epub] CrossRef
Cognition in multiple system atrophy: a single‐center cohort study
Sabine Eschlböck, Margarete Delazer, Florian Krismer, Thomas Bodner, Alessandra Fanciulli, Beatrice Heim, Antonio Heras Garvin, Christine Kaindlstorfer, Elfriede Karner, Katherina Mair, Christoph Rabensteiner, Cecilia Raccagni, Klaus Seppi, Werner Poewe,
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Neuropathological findings in multiple system atrophy with cognitive impairment
Kurt A. Jellinger
Journal of Neural Transmission.2020; 127(7): 1031. CrossRef
An update on MSA: premotor and non-motor features open a window of opportunities for early diagnosis and intervention
Viorica Chelban, Daniela Catereniuc, Daniela Aftene, Alexandru Gasnas, Ekawat Vichayanrat, Valeria Iodice, Stanislav Groppa, Henry Houlden
Journal of Neurology.2020; 267(9): 2754. CrossRef
A case of multiple system atrophy
Jing Guo, Fuying Liu, Tingting Liu, Xin Zhang, Yong Luo
Journal of International Medical Research.2019; 47(11): 5839. CrossRef
Cognitive impairment before changes appear on [18F]‐fluoro‐D‐glucose positron emission tomography images in a patient with possible early‐stage cerebellar‐predominant multiple system atrophy
Masahiko Takaya, Masahiko Atsumi, Tomoyuki Hirose, Kazunari Ishii, Osamu Shirakawa
Psychogeriatrics.2016; 16(3): 216. CrossRef
Fragile X-associated tremor/ataxia syndrome: phenotypic comparisons with other movement disorders
Erin E. Robertson, Deborah A. Hall, Andrew R. McAsey, Joan A. O’Keefe
The Clinical Neuropsychologist.2016; 30(6): 849. CrossRef
A brain-targeted, modified neurosin (kallikrein-6) reduces α-synuclein accumulation in a mouse model of multiple system atrophy
Brian Spencer, Elvira Valera, Edward Rockenstein, Margarita Trejo-Morales, Anthony Adame, Eliezer Masliah
Molecular Neurodegeneration.2015;[Epub] CrossRef
Cognitive Impairment and Its Structural Correlates in the Parkinsonian Subtype of Multiple System Atrophy
Ji Sun Kim, Jin-ju Yang, Dong-Kyun Lee, Jong-min Lee, Jinyoung Youn, Jin Whan Cho
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Case Report

Preserved Glucose Metabolism of Deep Cerebellar Nuclei in a Case of Multiple System Atrophy with Predominant Cerebellar Ataxia: F-18 Fluorodeoxyglucose Positron Emission Tomography Study: Oh Dae Kwon, Chang-Seok Ki; J Mov Disord. 2010;3(2):51-53.; DOI: https://doi.org/10.14802/jmd.10014

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Abstract PDF: The cerebellar glucose metabolism of multiple system atrophy with predominant cerebellar ataxia (MSA-C) is known to be decreased but is not defined among areas of cerebellum. We encountered a 54-year-old man who developed dizziness and progressive ataxia followed by urinary incontinence and orthostatic hypotension, all of those symptoms progressed relentlessly and the symptoms responded poorly to levodopa therapy. Visual analysis and statistical parametric mapping analysis of F-18 fluorodeoxyglucose positron emission tomography showed hypometabolism of both cerebellar hemisphere, severe at cortical area, and pons. There was clear sparing of deep cerebellar nuclei. Our report, as we know, shows the first case of preserved glucose metabolism of deep cerebellar nuclei relative to cerebellar cortex in an MSA-C patient.

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