@article{MTMT:35912893, title = {Positron emission tomography molecular imaging for pathological visualization in multiple system atrophy}, url = {https://m2.mtmt.hu/api/publication/35912893}, author = {Dong, La and Zhou, Rui and Zhou, Jinyun and Liu, Ke and Jin, Chentao and Wang, Jing and Xue, Chenxi and Tian, Mei and Zhang, Hong and Zhong, Yan}, doi = {10.1016/j.nbd.2025.106828}, journal-iso = {NEUROBIOL DIS}, journal = {NEUROBIOLOGY OF DISEASE}, volume = {206}, unique-id = {35912893}, issn = {0969-9961}, keywords = {Inflammation; ALPHA-SYNUCLEIN; positron emission tomography; brain metabolism; Multiple system atrophy; Dopaminergic and non-dopaminergic system}, year = {2025}, eissn = {1095-953X} } @article{MTMT:35941073, title = {Brainstem and cerebellar radiological findings in progressive supranuclear palsy}, url = {https://m2.mtmt.hu/api/publication/35941073}, author = {Spiegel, Chloe and Marotta, Cassandra and Bertram, Kelly and Vivash, Lucy and Harding, Ian H.}, doi = {10.1093/braincomms/fcaf051}, journal-iso = {BRAIN COMMUN}, journal = {BRAIN COMMUNICATIONS}, volume = {7}, unique-id = {35941073}, keywords = {Magnetic Resonance Imaging; CEREBELLUM; positron emission tomography; brainstem; PROGRESSIVE SUPRANUCLEAR PALSY}, year = {2025}, eissn = {2632-1297} } @article{MTMT:35352446, title = {Progressive supranuclear palsy: Neuropathology, clinical presentation, diagnostic challenges, management, and emerging therapies}, url = {https://m2.mtmt.hu/api/publication/35352446}, author = {DeRosier, Frederick and Hibbs, Cody and Alessi, Kaitlyn and Padda, Inderbir and Rodriguez, Jeanette and Pradeep, Swati and Parmar, Mayur S.}, doi = {10.1016/j.disamonth.2024.101753}, journal-iso = {DM-DIS MON}, journal = {DM DISEASE-A-MONTH}, volume = {70}, unique-id = {35352446}, issn = {0011-5029}, keywords = {PATHOPHYSIOLOGY; Imaging; PROGRESSIVE SUPRANUCLEAR PALSY; Clinical diagnosis; emerging therapies; Treatment and managment}, year = {2024}, eissn = {1557-8194} } @article{MTMT:34987555, title = {Pharmacotherapies for the Treatment of Progressive Supranuclear Palsy: A Narrative Review}, url = {https://m2.mtmt.hu/api/publication/34987555}, author = {Dunning, Elise E. and Decourt, Boris and Zawia, Nasser H. and Shill, Holly A. and Sabbagh, Marwan N.}, doi = {10.1007/s40120-024-00614-9}, journal-iso = {NEUROL THERAP}, journal = {NEUROLOGY AND THERAPY}, unique-id = {34987555}, issn = {2193-8253}, keywords = {TAU; Clinical Trials; monoclonal antibody; PROGRESSIVE SUPRANUCLEAR PALSY; TAU PHOSPHORYLATION; Microtubule dysfunction; modifiable risk factor}, year = {2024}, eissn = {2193-6536} } @article{MTMT:35941085, title = {Progressive supranuclear palsy: an updated approach on diagnosis, treatment, risk factors and outlook in Mexico}, url = {https://m2.mtmt.hu/api/publication/35941085}, author = {Gomez-Virgilio, Laura and Gutierrez-Malacara, Andres I. and Rivera-Osorio, Jared and Silva-Lucero, Ma. del Carmen and Padilla-Mendoza, Juan R. and Gomez-Ramirez, Daniela E. and Cardenas-Aguayo, Ma. del Carmen}, doi = {10.24875/GMM.24000114}, journal-iso = {GAC MED MEX}, journal = {GACETA MEDICA DE MEXICO}, volume = {160}, unique-id = {35941085}, issn = {0016-3813}, keywords = {Diagnosis. Progressive supranuclear palsy. Current situation in Mexico. Tau. Treatment}, year = {2024}, eissn = {0016-3813}, pages = {392-403} } @article{MTMT:35333786, title = {Impact of Magnetic Resonance Imaging Markers on the Diagnostic Performance of the International Parkinson and Movement Disorder Society Multiple System Atrophy Criteria}, url = {https://m2.mtmt.hu/api/publication/35333786}, author = {Jensen, Ida and Heine, Johanne and Ruf, Viktoria C. and Compta, Yaroslau and Porcel, Laura Molina and Troakes, Claire and Vamanu, Albert and Downes, Sophia and Irwin, David and Cohen, Jesse and Lee, Edward B. and Nilsson, Christer and Englund, Elisabet and Nemati, Mojtaba and Katzdobler, Sabrina and Levin, Johannes and Pantelyat, Alex and Seemiller, Joseph and Berger, Stephen and van Swieten, John and Dopper, Elise and Rozenmuller, Annemieke and Kovács, Gábor Géza and Bendahan, Nathaniel and Lang, Anthony E. and Herms, Jochen and Hoeglinger, Guenter and Hopfner, Franziska}, doi = {10.1002/mds.29879}, journal-iso = {MOVEMENT DISORD}, journal = {MOVEMENT DISORDERS}, unique-id = {35333786}, issn = {0885-3185}, keywords = {MRI; Multiple system atrophy; Brain magnetic resonance imaging; autopsy-confirmed}, year = {2024}, eissn = {1531-8257}, orcid-numbers = {Levin, Johannes/0000-0001-5092-4306; Seemiller, Joseph/0000-0001-7742-8759; Kovács, Gábor Géza/0000-0003-3841-5511; Hopfner, Franziska/0000-0001-6524-0281} } @article{MTMT:35515610, title = {Atypical Parkinsonian Syndromes: Structural, Functional, and Molecular Imaging Features}, url = {https://m2.mtmt.hu/api/publication/35515610}, author = {Keir, Graham and Roytman, Michelle and Mashriqi, Faizullah and Shahsavarani, Shaya and Franceschi, Ana M.}, doi = {10.3174/ajnr.A8313}, journal-iso = {AM J NEURORADIOL}, journal = {AMERICAN JOURNAL OF NEURORADIOLOGY}, unique-id = {35515610}, issn = {0195-6108}, year = {2024}, eissn = {1936-959X}, orcid-numbers = {Keir, Graham/0000-0001-7597-8378; Roytman, Michelle/0000-0001-5739-1274; Shahsavarani, Shaya/0000-0003-1968-8602} } @article{MTMT:35515612, title = {(What's the story) morning glory? MRI findings in morning glory disc anomaly}, url = {https://m2.mtmt.hu/api/publication/35515612}, author = {Leidhin, Caoilfhionn Ni and Erickson, Jonathan P. and Bynevelt, Michael and Lam, Geoffrey and Lock, Jane H. and Wang, George and Mankad, Kshitij and Taranath, Ajay and Mason, Michael and Lakshmanan, Rahul and Shipman, Peter and Warne, Richard R.}, doi = {10.1007/s00234-024-03375-2}, journal-iso = {NEURORADIOLOGY}, journal = {NEURORADIOLOGY}, volume = {66}, unique-id = {35515612}, issn = {0028-3940}, keywords = {GLIOMA; MRI; optic nerve; OPTIC DISC; Morning glory}, year = {2024}, eissn = {1432-1920}, pages = {1225-1233} } @article{MTMT:35510292, title = {Simple biomarkers to distinguish Parkinson's disease from its mimics in clinical practice: a comprehensive review and future directions}, url = {https://m2.mtmt.hu/api/publication/35510292}, author = {Quattrone, Andrea and Zappia, Mario and Quattrone, Aldo}, doi = {10.3389/fneur.2024.1460576}, journal-iso = {FRONT NEUR}, journal = {FRONTIERS IN NEUROLOGY}, volume = {15}, unique-id = {35510292}, keywords = {Electrophysiology; Biomarkers; clinical practice; Parkinson's disease; MRI}, year = {2024}, eissn = {1664-2295} } @article{MTMT:35515611, title = {Cerebellar blood perfusion is a diagnostic, but not a prognostic, marker for parkinsonian-dominant type multiple system atrophy}, url = {https://m2.mtmt.hu/api/publication/35515611}, author = {Shiina, Kenta and Tsunemi, Taiji and Hattori, Nobutaka}, doi = {10.1016/j.parkreldis.2024.106975}, journal-iso = {PARKINSONISM RELAT D}, journal = {PARKINSONISM AND RELATED DISORDERS}, volume = {123}, unique-id = {35515611}, issn = {1353-8020}, keywords = {Multiple system atrophy; (SPECT); Single -photon emission computed tomography}, year = {2024}, eissn = {1873-5126}, orcid-numbers = {Shiina, Kenta/0000-0003-2023-1431} } @article{MTMT:35513224, title = {Putaminal T1/T2-weighted ratio is increased in PSP compared to PD and healthy controls, a multi-cohort study}, url = {https://m2.mtmt.hu/api/publication/35513224}, author = {Sjostrom, Henrik and van Westen, Danielle and Hall, Sara and Tjerkaski, Jonathan and Westman, Eric and Muehlboeck, Sebastian and Hansson, Oskar and Svenningsson, Per and Granberg, Tobias}, doi = {10.1016/j.parkreldis.2024.106047}, journal-iso = {PARKINSONISM RELAT D}, journal = {PARKINSONISM AND RELATED DISORDERS}, volume = {121}, unique-id = {35513224}, issn = {1353-8020}, year = {2024}, eissn = {1873-5126}, orcid-numbers = {van Westen, Danielle/0000-0001-8649-9874; Hall, Sara/0000-0001-6585-9105; Tjerkaski, Jonathan/0000-0002-6034-8506; Westman, Eric/0000-0002-3115-2977; Svenningsson, Per/0000-0001-6727-3802} } @article{MTMT:35515615, title = {The Role of Clinical Assessment in the Era of Biomarkers}, url = {https://m2.mtmt.hu/api/publication/35515615}, author = {Carlos, Arenn F. and Josephs, Keith A.}, doi = {10.1007/s13311-023-01410-3}, journal-iso = {NEUROTHERAPEUTICS}, journal = {NEUROTHERAPEUTICS}, volume = {20}, unique-id = {35515615}, issn = {1933-7213}, keywords = {Biomarkers; clinical assessment; patient-centered medicine; Levels of utility; Disease-centered medicine}, year = {2023}, eissn = {1878-7479}, pages = {1001-1018} } @article{MTMT:34622776, title = {Striatal and thalamic automatic segmentation, morphology, and clinical correlates in Parkinsonism: Parkinson's disease, multiple system atrophy and progressive supranuclear palsy}, url = {https://m2.mtmt.hu/api/publication/34622776}, author = {Erlinger, M. and Molina-Ruiz, R. and Brumby, A. and Cordas, D. and Hunter, M. and Arguelles, C. Ferreiro and Yus, M. and Owens-Walton, C. and Jakabek, D. and Shaw, M. and Valdes, E. Lopez and Looi, J. C. L.}, doi = {10.1016/j.pscychresns.2023.111719}, journal-iso = {PSYCHIAT RES-NEUROIM}, journal = {PSYCHIATRY RESEARCH-NEUROIMAGING}, volume = {335}, unique-id = {34622776}, issn = {0925-4927}, keywords = {PARKINSONISM; STRIATUM; MORPHOLOGY; neuroimaging; Thalamus; VOLUMETRY}, year = {2023}, eissn = {1872-7506} } @article{MTMT:34313980, title = {Imaging Criteria for the Diagnosis of Progressive Supranuclear Palsy: Supportive or Mandatory?}, url = {https://m2.mtmt.hu/api/publication/34313980}, author = {Lupascu, Nicoleta and Lupescu, Ioan Cristian and Caloianu, Ionut and Naftanaila, Florin and Glogojeanu, Remus Relu and Sirbu, Carmen Adella and Mitrica, Marian}, doi = {10.3390/diagnostics13111967}, journal-iso = {DIAGNOSTICS}, journal = {DIAGNOSTICS}, volume = {13}, unique-id = {34313980}, issn = {2075-4418}, abstract = {We present the case of a 54-year-old male, without any significant medical history, who insidiously developed speech disturbances and walking difficulties, accompanied by backward falls. The symptoms progressively worsened over time. The patient was initially diagnosed with Parkinson's disease; however, he failed to respond to standard therapy with Levodopa. He came to our attention for worsening postural instability and binocular diplopia. A neurological exam was highly suggestive of a Parkinson-plus disease, most likely progressive supranuclear gaze palsy. Brain MRI was performed and revealed moderate midbrain atrophy with the characteristic "hummingbird" and "Mickey mouse" signs. An increased MR parkinsonism index was also noted. Based on all clinical and paraclinical data, a diagnosis of probable progressive supranuclear palsy was established. We review the main imaging features of this disease and their current role in diagnosis.}, keywords = {PROGRESSIVE SUPRANUCLEAR PALSY; Corticobasal degeneration; Multiple system atrophy (MSA); MIDBRAIN ATROPHY; hummingbird sign; Mickey mouse sign; morning glory sign; Magnetic Resonance Parkinsonism Index (MRPI); PSP syndromes; postmortem histopathological examination}, year = {2023}, eissn = {2075-4418} } @article{MTMT:35515614, title = {Vagus Nerve Ultrasonography Helps Distinguish Multiple System Atrophy from Other Parkinsonian Syndromes}, url = {https://m2.mtmt.hu/api/publication/35515614}, author = {Oura, Kazumasa and Yamaguchi, Takashi and Nozaki, Ryota and Taguchi, Keita and Suzuki, Yoshio and Takahashi, Kai and Takahashi, Kenta and Iwaoka, Kazuhiro and Takahashi, Makoto and Itabashi, Ryo and Maeda, Tetsuya}, doi = {10.1002/mdc3.13859}, journal-iso = {MOV DISORD CLIN PRAC}, journal = {MOVEMENT DISORDERS CLINICAL PRACTICE}, volume = {10}, unique-id = {35515614}, issn = {2330-1619}, keywords = {Ultrasonography; VAGUS NERVE; Multiple system atrophy; adult patients; parkinsonian syndromes}, year = {2023}, eissn = {2330-1619}, pages = {1525-1529}, orcid-numbers = {Takahashi, Makoto/0000-0002-2452-0268} } @article{MTMT:34271363, title = {Analysis of Genetic and MRI Changes, Blood Markers, and Risk Factors in a Twin Pair Discordant of Progressive Supranuclear Palsy}, url = {https://m2.mtmt.hu/api/publication/34271363}, author = {Persely, Aliz and Beszedics, B. and Pálóczi, Krisztina and Piroska, Márton and Alijanpourotaghsara, Amirreza and Strelnikov, David and Vessal, A. and Szabó, Helga and Hernyes, Anita and Zöldi, Luca and Jokkel, Zsófia and Fekete, A. and Juhász, János and Makra, Nóra and Szabó, Dóra and Buzás, Edit Irén and Tárnoki, Ádám Domonkos and Tárnoki, Dávid László}, doi = {10.3390/medicina59101696}, journal-iso = {MED LITH}, journal = {MEDICINA-LITHUANIA}, volume = {59}, unique-id = {34271363}, issn = {1010-660X}, year = {2023}, eissn = {1648-9144}, orcid-numbers = {Pálóczi, Krisztina/0000-0001-7065-3582; Piroska, Márton/0000-0002-6000-5044; Strelnikov, David/0000-0002-5911-9335; Hernyes, Anita/0000-0001-5875-4006; Zöldi, Luca/0000-0003-4187-1673; Jokkel, Zsófia/0000-0002-5407-6540; Szabó, Dóra/0000-0002-8601-3923; Buzás, Edit Irén/0000-0002-3744-206X; Tárnoki, Ádám Domonkos/0000-0001-5909-3780; Tárnoki, Dávid László/0000-0002-7001-7647} } @article{MTMT:34629389, title = {Midbrain atrophy in pathologically diagnosed Lewy body disease and clinically diagnosed Parkinson's disease}, url = {https://m2.mtmt.hu/api/publication/34629389}, author = {Sako, Wataru and Suda, Akimitsu and Taniguchi, Daisuke and Kamagata, Koji and Shindo, Atsuhiko and Ogawa, Takashi and Oji, Yutaka and Nishikawa, Noriko and Hatano, Taku and Aoki, Shigeki and Hattori, Nobutaka}, doi = {10.1016/j.jns.2023.120821}, journal-iso = {J NEUROL SCI}, journal = {JOURNAL OF THE NEUROLOGICAL SCIENCES}, volume = {454}, unique-id = {34629389}, issn = {0022-510X}, keywords = {Parkinson's disease; MRI; PROGRESSIVE SUPRANUCLEAR PALSY; Lewy Body Disease; Midbrain}, year = {2023}, eissn = {1878-5883} } @article{MTMT:35515617, title = {Comparative validation of AI and non-AI methods in MRI volumetry to diagnose Parkinsonian syndromes}, url = {https://m2.mtmt.hu/api/publication/35515617}, author = {Song, Joomee and Hahm, Juyoung and Lee, Jisoo and Lim, Chae Yeon and Chung, Myung Jin and Youn, Jinyoung and Cho, Jin Whan and Ahn, Jong Hyeon and Kim, Kyungsu}, doi = {10.1038/s41598-023-30381-w}, journal-iso = {SCI REP}, journal = {SCIENTIFIC REPORTS}, volume = {13}, unique-id = {35515617}, year = {2023}, eissn = {2045-2322}, orcid-numbers = {Kim, Kyungsu/0000-0001-6622-6545} } @article{MTMT:35510434, title = {Magnetic resonance imaging modalities aid in the differential diagnosis of atypical parkinsonian syndromes}, url = {https://m2.mtmt.hu/api/publication/35510434}, author = {Tinaz, Sule}, doi = {10.3389/fneur.2023.1082060}, journal-iso = {FRONT NEUR}, journal = {FRONTIERS IN NEUROLOGY}, volume = {14}, unique-id = {35510434}, keywords = {DIFFUSION MRI; Quantitative susceptibility mapping; Atypical parkinsonism; Susceptibility-weighted imaging; magnetic resonance parkinsonism index; neuromelanin MRI}, year = {2023}, eissn = {1664-2295} } @article{MTMT:35461242, title = {Pathological validation of the MDS criteria for the diagnosis of multiple system atrophy}, url = {https://m2.mtmt.hu/api/publication/35461242}, author = {Virameteekul, Sasivimol and Révész, Tamás and Jaunmuktane, Zane and Warner, Thomas T. and De, Pablo-Fernandez Eduardo}, doi = {10.1002/mds.29304}, journal-iso = {MOVEMENT DISORD}, journal = {MOVEMENT DISORDERS}, volume = {38}, unique-id = {35461242}, issn = {0885-3185}, abstract = {BackgroundThe recent International Parkinson and Movement Disorder Society diagnostic criteria for multiple system atrophy (MDS-MSA) have been developed to improve diagnostic accuracy although their diagnostic properties have not been evaluated. ObjectivesThe aims were to validate the MDS-MSA diagnostic criteria against neuropathological diagnosis and compare their diagnostic performance to previous criteria and diagnosis in clinical practice. MethodsConsecutive patients with sporadic, progressive, adult-onset parkinsonism, or cerebellar ataxia from the Queen Square Brain Bank between 2009 and 2019 were selected and divided based on neuropathological diagnosis into MSA and non-MSA. Medical records were systematically reviewed, and clinical diagnosis was documented by retrospectively applying the MDS-MSA criteria, second consensus criteria, and diagnosis according to treating clinicians at early (within 3 years of symptom onset) and final stages. Diagnostic parameters (sensitivity, specificity, positive/negative predictive value, and accuracy) were calculated using neuropathological diagnosis as gold standard and compared between different criteria. ResultsThree hundred eighteen patients (103 MSA and 215 non-MSA) were included, comprising 248 patients with parkinsonism and 70 with cerebellar ataxia. Clinically probable MDS-MSA showed excellent sensitivity (95.1%), specificity (94.0%), and accuracy (94.3%), although their sensitivity at early stages was modest (62.1%). Clinically probable MDS-MSA outperformed diagnosis by clinicians and by second consensus criteria. Clinically established MDS-MSA showed perfect specificity (100%) even at early stages although to the detriment of low sensitivity. MDS-MSA diagnostic accuracy did not differ according to clinical presentation (ataxia vs. parkinsonism). ConclusionsMDS-MSA criteria demonstrated excellent diagnostic performance against neuropathological diagnosis and are useful diagnostic tools for clinical practice and research. (c) 2023 International Parkinson and Movement Disorder Society.}, keywords = {diagnosis; diagnostic accuracy; PROGRESSIVE SUPRANUCLEAR PALSY; Multiple system atrophy; CONSENSUS STATEMENT; pathological validation}, year = {2023}, eissn = {1531-8257}, pages = {444-452}, orcid-numbers = {De, Pablo-Fernandez Eduardo/0000-0003-2834-2515} } @article{MTMT:35515616, title = {Multidimensional biomarkers for multiple system atrophy: an update and future directions}, url = {https://m2.mtmt.hu/api/publication/35515616}, author = {Wan, Linlin and Zhu, Sudan and Chen, Zhao and Qiu, Rong and Tang, Beisha and Jiang, Hong}, doi = {10.1186/s40035-023-00370-0}, journal-iso = {TRANSL NEURODEGENER}, journal = {TRANSLATIONAL NEURODEGENERATION}, volume = {12}, unique-id = {35515616}, issn = {2047-9158}, keywords = {TISSUE; FLUID; biomarker; Imaging; gut microbiota; Multiple system atrophy}, year = {2023}, eissn = {2047-9158} } @article{MTMT:34356928, title = {Brain MRI in Progressive Supranuclear Palsy with Richardson's Syndrome and Variant Phenotypes}, url = {https://m2.mtmt.hu/api/publication/34356928}, author = {Wattjes, Mike and Huppertz, Hans-Juergen and Mahmoudi, Nima and Stoecklein, Sophia and Rogozinski, Sophia and Wegner, Florian and Klietz, Martin and Apostolova, Ivayla and Levin, Johannes and Katzdobler, Sabrina and Buhmann, Carsten and Quattrone, Andrea and Berding, Georg and Brendel, Matthias and Barthel, Henryk and Sabri, Osama and Hoeglinger, Guenter and Buchert, Ralph}, doi = {10.1002/mds.29527}, journal-iso = {MOVEMENT DISORD}, journal = {MOVEMENT DISORDERS}, unique-id = {34356928}, issn = {0885-3185}, abstract = {BackgroundBrain magnetic resonance imaging (MRI) is used to support the diagnosis of progressive supranuclear palsy (PSP). However, the value of visual descriptive, manual planimetric, automatic volumetric MRI markers and fully automatic categorization is unclear, particularly regarding PSP predominance types other than Richardson's syndrome (RS). ObjectivesTo compare different visual reading strategies and automatic classification of T1-weighted MRI for detection of PSP in a typical clinical cohort including PSP-RS and (non-RS) variant PSP (vPSP) patients. MethodsForty-one patients (21 RS, 20 vPSP) and 46 healthy controls were included. Three readers using three strategies performed MRI analysis: exclusively visual reading using descriptive signs (hummingbird, morning-glory, Mickey-Mouse), visual reading supported by manual planimetry measures, and visual reading supported by automatic volumetry. Fully automatic classification was performed using a pre-trained support vector machine (SVM) on the results of atlas-based volumetry. ResultsAll tested methods achieved higher specificity than sensitivity. Limited sensitivity was driven to large extent by false negative vPSP cases. Support by automatic volumetry resulted in the highest accuracy (75.1% & PLUSMN; 3.5%) among the visual strategies, but performed not better than the midbrain area (75.9%), the best single planimetric measure. Automatic classification by SVM clearly outperformed all other methods (accuracy, 87.4%), representing the only method to provide clinically useful sensitivity also in vPSP (70.0%). ConclusionsFully automatic classification of volumetric MRI measures using machine learning methods outperforms visual MRI analysis without and with planimetry or volumetry support, particularly regarding diagnosis of vPSP, suggesting the use in settings with a broad phenotypic PSP spectrum. & COPY; 2023 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.}, keywords = {Magnetic Resonance Imaging; machine learning; VOLUMETRY; PROGRESSIVE SUPRANUCLEAR PALSY; hummingbird sign}, year = {2023}, eissn = {1531-8257}, orcid-numbers = {Mahmoudi, Nima/0000-0002-2053-9623; Klietz, Martin/0000-0002-3054-9905; Levin, Johannes/0000-0001-5092-4306; Quattrone, Andrea/0000-0003-2071-2083} } @article{MTMT:33477582, title = {Investigational therapeutics for the treatment of progressive supranuclear palsy}, url = {https://m2.mtmt.hu/api/publication/33477582}, author = {Coughlin, David G. and Litvan, Irene}, doi = {10.1080/13543784.2022.2087179}, journal-iso = {EXPERT OPIN INV DRUG}, journal = {EXPERT OPINION ON INVESTIGATIONAL DRUGS}, volume = {31}, unique-id = {33477582}, issn = {1354-3784}, abstract = {Introduction Progressive supranuclear palsy (PSP) is a progressive neurodegenerative disease marked by a variety of movement, ocular, and cognitive symptoms. Currently, treatment is symptomatic, and there are no disease-modulating therapies. While clinical presentations can be variable, at autopsy, PSP shows 4-repeat (4 R) tau species that accumulate in brainstem, subcortical, and neocortical areas. Thus, several tau-directed therapies have been trialed in PSP but with disappointing results to date. Areas Covered We review PSP clinicopathological correlates and biomarkers and searched clinicaltrials.gov and pubmed.ncbi.nlm.nih.gov for disease-modulating trials in PSP from the preclinical stage to clinical stage 3 and reviewed their rationale and results in human trials. Expert Opinion Factors that may have hampered tau-directed therapies in PSP include patient selection, intervening in an advanced disease stage, lack of biomarkers for prodromal diagnosis, outcome measurements, target engagement measures, selection of specific tau epitopes, and brain penetration of trialed therapies. Coupled with early intervention, targets upstream of tau accumulation and corresponding cell death may need to be identified to modulate the disease course. PSP remains a promising disease to study tau-directed therapies, and several possible targets are being tackled using novel approaches bringing hope for future success.}, keywords = {Biomarkers; immunotherapy; treatment; Gene Therapy; PROGRESSIVE SUPRANUCLEAR PALSY; tauopathy}, year = {2022}, eissn = {1744-7658}, pages = {813-823} } @article{MTMT:35515628, title = {Long-standing multiple system atrophy-Parkinsonism with limbic and FTLD-type α-synuclein pathology}, url = {https://m2.mtmt.hu/api/publication/35515628}, author = {Coughlin, David G. and Dryden, Ian and Goodwill, Vanessa S. and Pizzo, Donald P. and Wright, Brenton and Lessig, Stephanie and Galasko, Douglas and MacKenzie, Ian R. and Hiniker, Annie}, doi = {10.1111/nan.12783}, journal-iso = {NEUROPATH APPL NEURO}, journal = {NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY}, volume = {48}, unique-id = {35515628}, issn = {0305-1846}, keywords = {ALPHA-SYNUCLEIN; Multiple system atrophy; Gallyas}, year = {2022}, eissn = {1365-2990} } @article{MTMT:32953646, title = {Diagnostic Accuracy of Magnetic Resonance Imaging Measures of Brain Atrophy Across the Spectrum of Progressive Supranuclear Palsy and Corticobasal Degeneration}, url = {https://m2.mtmt.hu/api/publication/32953646}, author = {Illan-Gala, Ignacio and Nigro, Salvatore and VandeVrede, Lawren and Falgas, Neus and Heuer, Hilary W. and Painous, Celia and Compta, Yaroslau and Marti, Maria J. and Montal, Victor and Pagonabarraga, Javier and Kulisevsky, Jaime and Lleo, Alberto and Fortea, Juan and Logroscino, Giancarlo and Quattrone, Andrea and Quattrone, Aldo and Perry, David C. and Gorno-Tempini, Maria Luisa and Rosen, Howard J. and Grinberg, Lea T. and Spina, Salvatore and La Joie, Renaud and Rabinovici, Gil D. and Miller, Bruce L. and Rojas, Julio C. and Seeley, William W. and Boxer, Adam L.}, doi = {10.1001/jamanetworkopen.2022.9588}, journal-iso = {JAMA NETW OPEN}, journal = {JAMA NETWORK OPEN}, volume = {5}, unique-id = {32953646}, issn = {2574-3805}, abstract = {IMPORTANCE The accurate diagnosis of progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) is hampered by imperfect clinical-pathological correlations.OBJECTIVE To assess and compare the diagnostic value of the magnetic resonance parkinsonism index (MRPI) and other magnetic resonance imaging-based measures of cerebral atrophy to differentiate between PSP, CBD, and other neurodegenerative diseases.DESIGN, SETTING, AND PARTICIPANTS This prospective diagnostic study included participants with 4-repeat tauopathies (4RT), PSP, CBD, other neurodegenerative diseases and available MRI who appeared in the University of California. San Francisco, Memory and Aging Center database. Data were collected from October 27, 1994, to September 29, 2019. Data were analyzed from March 1 to September 14, 2021.MAIN OUTCOMES AND MEASURES The main outcome of this study was the neuropathological diagnosis of PSP or CBD. The clinical diagnosis at the time of the MRI acquisition was noted. The imaging measures included the MRPI, cortical thickness, subcortical volumes, including the midbrain, pons, and superior cerebellar peduncle volumes. Multinomial logistic regression models (MLRM) combining different cortical and subcortical regions were defined to discriminate between PSP, CBD, and other pathologies. The areas under the receiver operating characteristic curves (AUROC) and cutoffs were calculated to differentiate between PSP, CBD, and other diseases.RESULTS Of the 326 included participants, 176 (54%) were male, and the mean (SD) age at MRI was 64.1(8.0) years. The MRPI showed good diagnostic accuracy for the differentiation between PSP and all other pathologies (accuracy, 87%; AUROC, 0.90; 95% CI, 0.86-0.95) and between 4RT and other pathologies (accuracy, 80%; AUROC, 0.82; 95% CI, 0.76-0.87), but did not allow the discrimination of participants with CBD. Its diagnostic accuracy was lower in the subgroup of patients without the canonical PSP-Richardson syndrome (PSP-RS) or probable corticobasal syndrome (CBS) at MRI. MLRM combining cortical and subcortical measurements showed the highest accuracy for the differentiation between PSP and other pathologies (accuracy, 95%; AUROC, 0.98; 95% CI, 0.97-0.99), CBD and other pathologies (accuracy, 83%; AUROC, 0.86; 95% CI, 0.81-0.91), 4RT and other pathologies (accuracy, 89%; AUROC, 0.94; 95% CI, 0.92-0.97), and PSP and CBD (accuracy, 91%; AUROC, 0.95; 95% CI, 0.91-0.99), even in participants without PSP-RS or CBS at MRI.CONCLUSIONS AND RELEVANCE In this study, the combination of widely available cortical and subcortical measures of atrophy on MRI discriminated between PSP, CBD, and other pathologies and could be used to support the diagnosis of 4RT in clinical practice.}, year = {2022}, eissn = {2574-3805} } @article{MTMT:35515622, title = {Probable progressive supranuclear palsy in a patient with chronic schizophrenia: A case report}, url = {https://m2.mtmt.hu/api/publication/35515622}, author = {Kita, Akira and Tsuji, Tomikimi and Koh, Jinsoo and Takahashi, Shun and Yamamoto, Masahiro and Sakamoto, Yuka and Itogawa, Hideaki and Kimoto, Sohei}, doi = {10.3892/etm.2022.11411}, journal-iso = {EXP THER MED}, journal = {EXPERIMENTAL AND THERAPEUTIC MEDICINE}, volume = {24}, unique-id = {35515622}, issn = {1792-0981}, keywords = {SCHIZOPHRENIA; PARKINSONISM; case report; Magnetic Resonance Imaging; Progressive supranuclear palsy-Richardson's syndrome}, year = {2022}, eissn = {1792-1015}, orcid-numbers = {Kita, Akira/0000-0003-1819-5291} } @article{MTMT:35515618, title = {Semiautomated Algorithm for the Diagnosis of Multiple System Atrophy With Predominant Parkinsonism br}, url = {https://m2.mtmt.hu/api/publication/35515618}, author = {Lee, Woong-Woo and Kim, Han-Joon and Lee, Hong Ji and Kim, Han Byul and Park, Kwang Suk and Sohn, Chul-Ho and Jeon, Beomseok}, doi = {10.14802/jmd.21178}, journal-iso = {J MOV DISORD}, journal = {JOURNAL OF MOVEMENT DISORDERS}, volume = {15}, unique-id = {35515618}, issn = {2005-940X}, keywords = {automation; Parkinson's disease; putamen; Multiple system atrophy; Susceptibility-weighted image}, year = {2022}, eissn = {2093-4939}, pages = {232-+}, orcid-numbers = {Lee, Woong-Woo/0000-0002-8767-7967; Kim, Han-Joon/0000-0001-8219-9663; Sohn, Chul-Ho/0000-0003-0039-5746} } @article{MTMT:35513341, title = {Diagnostic performance of T2*gradient echo, susceptibility-weighted imaging, and quantitative susceptibility mapping for patients with multiple system atrophy-parkinsonian type: a systematic review and meta-analysis}, url = {https://m2.mtmt.hu/api/publication/35513341}, author = {Lim, Su Jin and Suh, Chong Hyun and Shim, Woo Hyun and Kim, Sang Joon}, doi = {10.1007/s00330-021-08174-4}, journal-iso = {EUR RADIOL}, journal = {EUROPEAN RADIOLOGY}, volume = {32}, unique-id = {35513341}, issn = {0938-7994}, keywords = {Magnetic Resonance Imaging; Parkinson disease; Multiple system atrophy}, year = {2022}, eissn = {1432-1084}, pages = {308-318}, orcid-numbers = {Lim, Su Jin/0000-0003-3300-7535} } @article{MTMT:35515623, title = {Morphometric imaging and quantitative susceptibility mapping as complementary tools in the diagnosis of parkinsonisms}, url = {https://m2.mtmt.hu/api/publication/35515623}, author = {Mazzucchi, Sonia and Del Prete, Eleonora and Costagli, Mauro and Frosini, Daniela and Paoli, Davide and Migaleddu, Gianmichele and Cecchi, Paolo and Donatelli, Graziella and Morganti, Riccardo and Siciliano, Gabriele and Cosottini, Mirco and Ceravolo, Roberto}, doi = {10.1111/ene.15447}, journal-iso = {EUR J NEUROL}, journal = {EUROPEAN JOURNAL OF NEUROLOGY}, volume = {29}, unique-id = {35515623}, issn = {1351-5101}, keywords = {brain MRI; Quantitative susceptibility mapping; parkinsonisms; morphometric indexes}, year = {2022}, eissn = {1468-1331}, pages = {2944-2955}, orcid-numbers = {Del Prete, Eleonora/0000-0002-4690-1387; Donatelli, Graziella/0000-0002-5325-0746} } @article{MTMT:33941149, title = {F-18-PI-2620 Tau PET Improves the Imaging Diagnosis of Progressive Supranuclear Palsy}, url = {https://m2.mtmt.hu/api/publication/33941149}, author = {Messerschmidt, Konstantin and Barthel, Henryk and Brendel, Matthias and Scherlach, Cordula and Hoffmann, Karl-Titus and Rauchmann, Boris-Stephan and Rullmann, Michael and Marek, Kenneth and Villemagne, Victor L. and Rumpf, Jost-Julian and Saur, Dorothee and Schroeter, Matthias L. and Schildan, Andreas and Patt, Marianne and Beyer, Leonie and Song, Mengmeng and Palleis, Carla and Katzdobler, Sabrina and Fietzek, Urban M. and Respondek, Gesine and Scheifele, Maximilian and Nitschmann, Alexander and Zach, Christian and Barret, Olivier and Madonia, Jennifer and Russell, David and Stephens, Andrew W. and Koglin, Norman and Roeber, Sigrun and Herms, Jochen and Boetzel, Kai and Bartenstein, Peter and Levin, Johannes and Seibyl, John P. and Hoeglinger, Guenter and Classen, Joseph and Sabri, Osama}, doi = {10.2967/jnumed.121.262854}, journal-iso = {J NUCL MED}, journal = {JOURNAL OF NUCLEAR MEDICINE}, volume = {63}, unique-id = {33941149}, issn = {0161-5505}, abstract = {Progressive supranuclear palsy (PSP) is a 4-repeat tauopathy movement disorder that can be imaged by the F-18-labeled tau PET tracer 2-(2-([F-18]fluoro)pyridin-4-yl)-9H-pyrrolo[2,3-b:4,5-c9]dipyridine ((FPI)-F-18-2620). The in vivo diagnosis is currently established on clinical grounds and supported by midbrain atrophy estimation in structural MRI. Here, we investigate whether F-18-PI-2620 tau PET has the potential to improve the imaging diagnosis of PSP. Methods: In this multicenter observational study, dynamic (0-60 min after injection) F-18-PI-2620 PET and structural MRI data for 36 patients with PSP, 22 with PSP-Richardson syndrome, and 14 with a clinical phenotype other than Richardson syndrome (i.e., variant PSP) were analyzed along with data for 10 age-matched healthy controls (HCs). The PET data underwent kinetic modeling, which resulted in distribution volume ratio (DVR) images. These and the MR images were visually assessed by 3 masked experts for typical PSP signs. Furthermore, established midbrain atrophy parameters were measured in structural MR images, and regional DVRs were measured in typical tau-in-PSP target regions in the PET data. Results: Visual assessments discriminated PSP patients and HCs with an accuracy of 63% for MRI and 80% for the combination of MRI and F-18-PI-2620 PET. As compared with patients of the PSP-Richardson syndrome subgroup, those of the variant PSP subgroup profited more in terms of sensitivity from the addition of the visual F-18-PI-2620 PET to the visual MRI information (35% vs. 22%). In quantitative image evaluation, midbrain-topons area ratio and globus pallidus DVRs discriminated best between the PSP patients and HCs, with sensitivities and specificities of 83% and 90%, respectively, for MRI and 94% and 100%, respectively, for the combination of MRI and F-18-PI-2620 PET. The gain of sensitivity by adding F-18-PI-2620 PET to MRI data was more marked in clinically less affected patients than in more affected patients (37% vs. 19% for visual, and 16% vs. 12% for quantitative image evaluation). Conclusion: These results provide evidence for an improved imaging-based PSP diagnosis by adding F-18-PI-2620 tau PET to structural MRI. This approach seems to be particularly promising at earlier disease stages and could be of value both for improving early clinical PSP diagnosis and for enriching PSP cohorts for trials of disease-modifying drugs.}, keywords = {PROGRESSIVE SUPRANUCLEAR PALSY; Tau PET; 4R-Tauopathy; MIDBRAIN ATROPHY; F-18-PI-2620}, year = {2022}, eissn = {1535-5667}, pages = {1754-1760}, orcid-numbers = {Rauchmann, Boris-Stephan/0000-0003-4547-6240; Rullmann, Michael/0000-0002-2683-9432; Saur, Dorothee/0000-0002-1808-0913; Schildan, Andreas/0000-0002-9418-3826; Koglin, Norman/0000-0001-7583-8201; Levin, Johannes/0000-0001-5092-4306} } @article{MTMT:35515619, title = {The "Hot Cross Bun Sign" in Spinocerebellar Ataxia Types 2 and 7-Case Reports and Review of Literature}, url = {https://m2.mtmt.hu/api/publication/35515619}, author = {Naidoo, Ansuya Kasavelu and Wells, Cait-Lynn Deanne and Rugbeer, Yashvir and Naidoo, Neil}, doi = {10.1002/mdc3.13550}, journal-iso = {MOV DISORD CLIN PRAC}, journal = {MOVEMENT DISORDERS CLINICAL PRACTICE}, volume = {9}, unique-id = {35515619}, issn = {2330-1619}, keywords = {Multiple system atrophy (MSA); Hot cross bun sign; spinocerebellar ataxia type 2 and 7}, year = {2022}, eissn = {2330-1619}, pages = {1105-1113} } @article{MTMT:33382817, title = {Progressive supranuclear palsy and corticobasal degeneration: novel clinical concepts and advances in biomarkers}, url = {https://m2.mtmt.hu/api/publication/33382817}, author = {Parmera, Jacy Bezerra and Barbosa de Oliveira, Marcos Castello and Rodrigues, Roberta Diehl and Coutinho, Artur Martins}, doi = {10.1590/0004-282X-ANP-2022-S134}, journal-iso = {ARQ NEURO-PSIQUIAT}, journal = {ARQUIVOS DE NEURO-PSIQUIATRIA}, volume = {80}, unique-id = {33382817}, issn = {0004-282X}, abstract = {Background: Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are sporadic adult-onset primary tauopathies clinically classified among the atypical parkinsonian syndromes. They are intrinsically related with regard to their clinical features, pathology, biochemistry, and genetic risk factors. Objectives: This review highlights the current knowledge on PSP and CBD, focusing on evolving clinical concepts, new diagnostic criteria, and advances in biomarkers. Methods: We performed a non-systematic literature review through the PubMed database. The search was restricted to articles written in English, published from 1964 to date. Results: Clinicopathologic and in vivo biomarkers studies have broadened PSP and CBD clinical phenotypes. They are now recognized as a range of motor and behavioral syndromes associated with underlying 4R-tauopathy neuropathology. The Movement Disorders Society PSP diagnostic criteria included clinical variants apart from the classical description, increasing diagnostic sensitivity. Meanwhile, imaging biomarkers have explored the complexity of symptoms and pathological processes related to corticobasal syndrome and CBD. Conclusions: In recent years, several prospective or clinicopathologic studies have assessed clinical, radiological, and fluid biomarkers that have helped us gain a better understanding of the complexity of the 4R-tauopathies, mainly PSP and CBD.}, keywords = {Biomarkers; Magnetic Resonance Imaging; Positron-Emission Tomography; Parkinsonian Disorders; Progressive; tauopathies; SUPRANUCLEAR PALSY}, year = {2022}, eissn = {1678-4227}, pages = {126-136}, orcid-numbers = {Parmera, Jacy Bezerra/0000-0002-3565-5328} } @article{MTMT:33234240, title = {Multiple system atrophy}, url = {https://m2.mtmt.hu/api/publication/33234240}, author = {Poewe, Werner and Stankovic, Iva and Halliday, Glenda and Meissner, Wassilios G. and Wenning, Gregor K. and Pellecchia, Maria Teresa and Seppi, Klaus and Palma, Jose-Alberto and Kaufmann, Horacio}, doi = {10.1038/s41572-022-00382-6}, journal-iso = {NAT REV DIS PRIMERS}, journal = {NATURE REVIEWS DISEASE PRIMERS}, volume = {8}, unique-id = {33234240}, issn = {2056-676X}, abstract = {This Primer by Poewe and colleagues summarizes the epidemiology, diagnosis, pathophysiology and treatment of multiple system atrophy. Moreover, this Primer provides an overview of the quality of life issues faced by patients with this disorder and future research avenues.Multiple system atrophy (MSA) is a rare neurodegenerative disease that is characterized by neuronal loss and gliosis in multiple areas of the central nervous system including striatonigral, olivopontocerebellar and central autonomic structures. Oligodendroglial cytoplasmic inclusions containing misfolded and aggregated alpha-synuclein are the histopathological hallmark of MSA. A firm clinical diagnosis requires the presence of autonomic dysfunction in combination with parkinsonism that responds poorly to levodopa and/or cerebellar ataxia. Clinical diagnostic accuracy is suboptimal in early disease because of phenotypic overlaps with Parkinson disease or other types of degenerative parkinsonism as well as with other cerebellar disorders. The symptomatic management of MSA requires a complex multimodal approach to compensate for autonomic failure, alleviate parkinsonism and cerebellar ataxia and associated disabilities. None of the available treatments significantly slows the aggressive course of MSA. Despite several failed trials in the past, a robust pipeline of putative disease-modifying agents, along with progress towards early diagnosis and the development of sensitive diagnostic and progression biomarkers for MSA, offer new hope for patients.}, year = {2022}, eissn = {2056-676X}, orcid-numbers = {Halliday, Glenda/0000-0003-0422-8398; Palma, Jose-Alberto/0000-0002-1345-6774; Kaufmann, Horacio/0000-0002-1851-9981} } @article{MTMT:33368397, title = {Magnetic Resonance Planimetry in the Differential Diagnosis between Parkinson's Disease and Progressive Supranuclear Palsy}, url = {https://m2.mtmt.hu/api/publication/33368397}, author = {Quattrone, Andrea and Morelli, Maurizio and Bianco, Maria G. and Buonocore, Jolanda and Sarica, Alessia and Caligiuri, Maria Eugenia and Aracri, Federica and Calomino, Camilla and De Maria, Marida and Vaccaro, Maria Grazia and Gramigna, Vera and Augimeri, Antonio and Vescio, Basilio and Quattrone, Aldo}, doi = {10.3390/brainsci12070949}, journal-iso = {BRAIN SCI}, journal = {BRAIN SCIENCES}, volume = {12}, unique-id = {33368397}, abstract = {The clinical differential diagnosis between Parkinson's disease (PD) and progressive supranuclear palsy (PSP) is often challenging. The description of milder PSP phenotypes strongly resembling PD, such as PSP-Parkinsonism, further increased the diagnostic challenge and the need for reliable neuroimaging biomarkers to enhance the diagnostic certainty. This review aims to summarize the contribution of a relatively simple and widely available imaging technique such as MR planimetry in the differential diagnosis between PD and PSP, focusing on the recent advancements in this field. The development of accurate MR planimetric biomarkers, together with the implementation of automated algorithms, led to robust and objective measures for the differential diagnosis of PSP and PD at the individual level. Evidence from longitudinal studies also suggests a role of MR planimetry in predicting the development of the PSP clinical signs, allowing to identify PSP patients before they meet diagnostic criteria when their clinical phenotype can be indistinguishable from PD. Finally, promising evidence exists on the possible association between MR planimetric measures and the underlying pathology, with important implications for trials with new disease-modifying target therapies.}, keywords = {Biomarkers; Parkinson's disease; PROGRESSIVE SUPRANUCLEAR PALSY; MR planimetry; MRPI}, year = {2022}, eissn = {2076-3425}, orcid-numbers = {Quattrone, Andrea/0000-0003-2071-2083; Sarica, Alessia/0000-0003-1362-6718; Caligiuri, Maria Eugenia/0000-0002-2030-5552} } @article{MTMT:33419806, title = {Temporal Progression Patterns of Brain Atrophy in Corticobasal Syndrome and Progressive Supranuclear Palsy Revealed by Subtype and Stage Inference (SuStaIn)}, url = {https://m2.mtmt.hu/api/publication/33419806}, author = {Saito, Yuya and Kamagata, Koji and Wijeratne, Peter A. and Andica, Christina and Uchida, Wataru and Takabayashi, Kaito and Fujita, Shohei and Akashi, Toshiaki and Wada, Akihiko and Shimoji, Keigo and Hori, Masaaki and Masutani, Yoshitaka and Alexander, Daniel C. and Aoki, Shigeki}, doi = {10.3389/fneur.2022.814768}, journal-iso = {FRONT NEUR}, journal = {FRONTIERS IN NEUROLOGY}, volume = {13}, unique-id = {33419806}, abstract = {Differentiating corticobasal degeneration presenting with corticobasal syndrome (CBD-CBS) from progressive supranuclear palsy with Richardson's syndrome (PSP-RS), particularly in early stages, is often challenging because the neurodegenerative conditions closely overlap in terms of clinical presentation and pathology. Although volumetry using brain magnetic resonance imaging (MRI) has been studied in patients with CBS and PSP-RS, studies assessing the progression of brain atrophy are limited. Therefore, we aimed to reveal the difference in the temporal progression patterns of brain atrophy between patients with CBS and those with PSP-RS purely based on cross-sectional data using Subtype and Stage Inference (SuStaIn)-a novel, unsupervised machine learning technique that integrates clustering and disease progression modeling. We applied SuStaIn to the cross-sectional regional brain volumes of 25 patients with CBS, 39 patients with typical PSP-RS, and 50 healthy controls to estimate the two disease subtypes and trajectories of CBS and PSP-RS, which have distinct atrophy patterns. The progression model and classification accuracy of CBS and PSP-RS were compared with those of previous studies to evaluate the performance of SuStaIn. SuStaIn identified distinct temporal progression patterns of brain atrophy for CBS and PSP-RS, which were largely consistent with previous evidence, with high reproducibility (99.7%) under cross-validation. We classified these diseases with high accuracy (0.875) and sensitivity (0.680 and 1.000, respectively) based on cross-sectional structural brain MRI data; the accuracy was higher than that reported in previous studies. Moreover, SuStaIn stage correctly reflected disease severity without the label of disease stage, such as disease duration. Furthermore, SuStaIn also showed the genialized performance of differentiation and reflection for CBS and PSP-RS. Thus, SuStaIn has potential for improving our understanding of disease mechanisms, accurately stratifying patients, and providing prognoses for patients with CBS and PSP-RS.}, keywords = {CLASSIFICATION; DISEASE PROGRESSION; Magnetic Resonance Imaging; machine learning; BRAIN ATROPHY; PROGRESSIVE SUPRANUCLEAR PALSY; Corticobasal degeneration; Corticobasal syndrome}, year = {2022}, eissn = {1664-2295}, orcid-numbers = {Takabayashi, Kaito/0000-0002-5562-1504} } @article{MTMT:33477593, title = {A data-driven model of brain volume changes in progressive supranuclear palsy}, url = {https://m2.mtmt.hu/api/publication/33477593}, author = {Scotton, W. J. and Bocchetta, M. and Todd, E. and Cash, D. M. and Oxtoby, N. and VandeVrede, L. and Heuer, H. and Alexander, D. C. and Rowe, J. B. and Morris, H. R. and Boxer, A. and Rohrer, J. D. and Wijeratne, P. A.}, doi = {10.1093/braincomms/fcac098}, journal-iso = {BRAIN COMMUN}, journal = {BRAIN COMMUNICATIONS}, volume = {4}, unique-id = {33477593}, abstract = {Using event-based modelling, Scotton et al. report the probabilistic sequence of brain atrophy progression in progressive supranuclear palsy. The resulting data-driven model, which is correlated with both clinical severity and disease duration, allows individual patient staging at baseline MRI scan with potential utility for clinical trial enrichment.The most common clinical phenotype of progressive supranuclear palsy is Richardson syndrome, characterized by levodopa unresponsive symmetric parkinsonism, with a vertical supranuclear gaze palsy, early falls and cognitive impairment. There is currently no detailed understanding of the full sequence of disease pathophysiology in progressive supranuclear palsy. Determining the sequence of brain atrophy in progressive supranuclear palsy could provide important insights into the mechanisms of disease progression, as well as guide patient stratification and monitoring for clinical trials. We used a probabilistic event-based model applied to cross-sectional structural MRI scans in a large international cohort, to determine the sequence of brain atrophy in clinically diagnosed progressive supranuclear palsy Richardson syndrome. A total of 341 people with Richardson syndrome (of whom 255 had 12-month follow-up imaging) and 260 controls were included in the study. We used a combination of 12-month follow-up MRI scans, and a validated clinical rating score (progressive supranuclear palsy rating scale) to demonstrate the longitudinal consistency and utility of the event-based model's staging system. The event-based model estimated that the earliest atrophy occurs in the brainstem and subcortical regions followed by progression caudally into the superior cerebellar peduncle and deep cerebellar nuclei, and rostrally to the cortex. The sequence of cortical atrophy progresses in an anterior to posterior direction, beginning in the insula and then the frontal lobe before spreading to the temporal, parietal and finally the occipital lobe. This in vivo ordering accords with the post-mortem neuropathological staging of progressive supranuclear palsy and was robust under cross-validation. Using longitudinal information from 12-month follow-up scans, we demonstrate that subjects consistently move to later stages over this time interval, supporting the validity of the model. In addition, both clinical severity (progressive supranuclear palsy rating scale) and disease duration were significantly correlated with the predicted subject event-based model stage (P < 0.01). Our results provide new insights into the sequence of atrophy progression in progressive supranuclear palsy and offer potential utility to stratify people with this disease on entry into clinical trials based on disease stage, as well as track disease progression.}, keywords = {DISEASE PROGRESSION; Biomarkers; machine learning; PROGRESSIVE SUPRANUCLEAR PALSY; Event-Based Model}, year = {2022}, eissn = {2632-1297} } @article{MTMT:35515621, title = {Automated volumetric determination of high R2* regions in substantia nigra: A feasibility study of quantifying substantia nigra atrophy in progressive supranuclear palsy}, url = {https://m2.mtmt.hu/api/publication/35515621}, author = {Tessema, Abel Worku and Lee, Hansol and Gong, Yelim and Cho, Hwapyeong and Adem, Hamdia Murad and Lyu, Ilwoo and Lee, Jae-Hyeok and Cho, HyungJoon}, doi = {10.1002/nbm.4795}, journal-iso = {NMR BIOMED}, journal = {NMR IN BIOMEDICINE}, volume = {35}, unique-id = {35515621}, issn = {0952-3480}, keywords = {quantitative analysis; substantia nigra; Segmentation; PROGRESSIVE SUPRANUCLEAR PALSY; Convolutional neural network}, year = {2022}, eissn = {1099-1492}, orcid-numbers = {Lee, Hansol/0000-0003-2112-1197} } @article{MTMT:35515620, title = {Magnetic resonance and dopamine transporter imaging for the diagnosis of Parkinson's disease: a narrative review}, url = {https://m2.mtmt.hu/api/publication/35515620}, author = {Vicentini Otani, Rafael Tomio and Silvestre Yamamoto, Joyce Yuri and Nunes, Douglas Mendes and Haddad, Monica Santoro and Parmera, Jacy Bezerra}, doi = {10.1590/0004-282X-ANP-2022-S130}, journal-iso = {ARQ NEURO-PSIQUIAT}, journal = {ARQUIVOS DE NEURO-PSIQUIATRIA}, volume = {80}, unique-id = {35515620}, issn = {0004-282X}, keywords = {Magnetic Resonance Imaging; Parkinson disease; Melanins; Parkinsonian Disorders; diffusion tensor imaging; Diffusion Magnetic Resonance Imaging; single photon emission computed tomography computed tomography}, year = {2022}, eissn = {1678-4227}, pages = {116-125} } @article{MTMT:35515629, title = {Midbrain area and the hummingbird sign from brain MRI in progressive supranuclear palsy and idiopathic normal pressure hydrocephalus}, url = {https://m2.mtmt.hu/api/publication/35515629}, author = {Virhammar, Johan and Blohme, Harald and Nyholm, Dag and Georgiopoulos, Charalampos and Fallmar, David}, doi = {10.1111/jon.12932}, journal-iso = {J NEUROIMAGING}, journal = {JOURNAL OF NEUROIMAGING}, volume = {32}, unique-id = {35515629}, issn = {1051-2284}, keywords = {neurodegeneration; PROGRESSIVE SUPRANUCLEAR PALSY; Midbrain; idiopathic normal pressure hydrocephalus; visual assessment; Area measurement}, year = {2022}, eissn = {1552-6569}, pages = {90-96}, orcid-numbers = {Georgiopoulos, Charalampos/0000-0001-8850-3742; Fallmar, David/0000-0001-5615-2036} } @article{MTMT:35515627, title = {The Movement Disorder Society Criteria for the Diagnosis of Multiple System Atrophy}, url = {https://m2.mtmt.hu/api/publication/35515627}, author = {Wenning, Gregor K. and Stankovic, Iva and Vignatelli, Luca and Fanciulli, Alessandra and Calandra-Buonaura, Giovanna and Seppi, Klaus and Palma, Jose-Alberto and Meissner, Wassilios G. and Krismer, Florian and Berg, Daniela and Cortelli, Pietro and Freeman, Roy and Halliday, Glenda and Hoeglinger, Gunter and Lang, Anthony and Ling, Helen and Litvan, Irene and Low, Phillip and Miki, Yasuo and Panicker, Jalesh and Pellecchia, Maria Teresa and Quinn, Niall and Sakakibara, Ryuji and Stamelou, Maria and Tolosa, Eduardo and Tsuji, Shoji and Warner, Tom and Poewe, Werner and Kaufmann, Horacio}, doi = {10.1002/mds.29005}, journal-iso = {MOVEMENT DISORD}, journal = {MOVEMENT DISORDERS}, volume = {37}, unique-id = {35515627}, issn = {0885-3185}, keywords = {diagnosis; Multiple system atrophy; Diagnostic criteria}, year = {2022}, eissn = {1531-8257}, pages = {1131-1148}, orcid-numbers = {Stankovic, Iva/0000-0003-1484-4717; Vignatelli, Luca/0000-0002-9051-7091; Palma, Jose-Alberto/0000-0002-1345-6774; Krismer, Florian/0000-0002-4493-5073; Berg, Daniela/0000-0001-5796-5442} } @article{MTMT:35511633, title = {Imaging the Substantia Nigra in Parkinson Disease and Other Parkinsonian Syndromes}, url = {https://m2.mtmt.hu/api/publication/35511633}, author = {Bae, Yun Jung and Kim, Jong-Min and Sohn, Chul-Ho and Choi, Ji-Hyun and Choi, Byung Se and Song, Yoo Sung and Nam, Yoonho and Cho, Se Jin and Jeon, Beomseok and Kim, Jae Hyoung}, doi = {10.1148/radiol.2021203341}, journal-iso = {RADIOLOGY}, journal = {RADIOLOGY}, volume = {300}, unique-id = {35511633}, issn = {0033-8419}, year = {2021}, eissn = {1527-1315}, pages = {260-278}, orcid-numbers = {Bae, Yun Jung/0000-0002-1779-4949; Kim, Jong-Min/0000-0001-5723-3997; Sohn, Chul-Ho/0000-0003-0039-5746; Choi, Byung Se/0000-0001-6310-1798; Cho, Se Jin/0000-0001-6450-7554} } @article{MTMT:32980357, title = {Looking beneath the surface: the importance of subcortical structures in frontotemporal dementia}, url = {https://m2.mtmt.hu/api/publication/32980357}, author = {Bocchetta, Martina and Malpetti, Maura and Todd, Emily G. and Rowe, James B. and Rohrer, Jonathan D.}, doi = {10.1093/braincomms/fcab158}, journal-iso = {BRAIN COMMUN}, journal = {BRAIN COMMUNICATIONS}, volume = {3}, unique-id = {32980357}, abstract = {Whilst initial anatomical studies of frontotemporal dementia focussed on cortical involvement, the relevance of subcortical structures to the pathophysiology of frontotemporal dementia has been increasingly recognized over recent years. Key structures affected include the caudate, putamen, nucleus accumbens, and globus pallidus within the basal ganglia, the hippocampus and amygdala within the medial temporal lobe, the basal forebrain, and the diencephalon structures of the thalamus, hypothalamus and habenula. At the most posterior aspect of the brain, focal involvement of brainstem and cerebellum has recently also been shown in certain subtypes of frontotemporal dementia. Many of the neuroimaging studies on subcortical structures in frontotemporal dementia have been performed in clinically defined sporadic cases. However, investigations of genetically- and pathologically-confirmed forms of frontotemporal dementia are increasingly common and provide molecular specificity to the changes observed. Furthermore, detailed analyses of sub-nuclei and subregions within each subcortical structure are being added to the literature, allowing refinement of the patterns of subcortical involvement. This review focuses on the existing literature on structural imaging and neuropathological studies of subcortical anatomy across the spectrum of frontotemporal dementia, along with investigations of brain-behaviour correlates that examine the cognitive sequelae of specific subcortical involvement: it aims to `look beneath the surface' and summarize the patterns of subcortical involvement have been described in frontotemporal dementia.}, keywords = {MR Imaging; FRONTOTEMPORAL DEMENTIA; SUBCORTICAL STRUCTURES}, year = {2021}, eissn = {2632-1297}, orcid-numbers = {Bocchetta, Martina/0000-0003-1814-5024} } @article{MTMT:35514002, title = {Progressive Supranuclear Palsy and Corticobasal Degeneration}, url = {https://m2.mtmt.hu/api/publication/35514002}, author = {Coughlin, David G. and Dickson, Dennis W. and Josephs, Keith A. and Litvan, Irene}, doi = {10.1007/978-3-030-51140-1_11}, journal-iso = {ADV EXP MED BIOL}, journal = {ADVANCES IN EXPERIMENTAL MEDICINE AND BIOLOGY}, volume = {1281}, unique-id = {35514002}, issn = {0065-2598}, year = {2021}, eissn = {2214-8019}, pages = {151-176} } @article{MTMT:33382838, title = {Differentiating Progressive Supranuclear Palsy and Parkinson's Disease With Head-Mounted Displays}, url = {https://m2.mtmt.hu/api/publication/33382838}, author = {Herwig, Arvid and Agic, Almedin and Huppertz, Hans-Jurgen and Klingebiel, Randolf and Zuhorn, Frederic and Schneider, Werner X. and Schaebitz, Wolf-Ruediger and Rogalewski, Andreas}, doi = {10.3389/fneur.2021.791366}, journal-iso = {FRONT NEUR}, journal = {FRONTIERS IN NEUROLOGY}, volume = {12}, unique-id = {33382838}, abstract = {Background: Progressive supranuclear palsy (PSP) is a neurodegenerative disorder that, especially in the early stages of the disease, is clinically difficult to distinguish from Parkinson's disease (PD).Objective: This study aimed at assessing the use of eye-tracking in head-mounted displays (HMDs) for differentiating PSP and PD.Methods: Saccadic eye movements of 13 patients with PSP, 15 patients with PD, and a group of 16 healthy controls (HCs) were measured. To improve applicability in an inpatient setting and standardize the diagnosis, all the tests were conducted in a HMD. In addition, patients underwent atlas-based volumetric analysis of various brain regions based on high-resolution MRI.Results: Patients with PSP displayed unique abnormalities in vertical saccade velocity and saccade gain, while horizontal saccades were less affected. A novel diagnostic index was derived, multiplying the ratios of vertical to horizontal gain and velocity, allowing segregation of PSP from PD with high sensitivity (10/13, 77%) and specificity (14/15, 93%). As expected, patients with PSP as compared with patients with PD showed regional atrophy in midbrain volume, the midbrain plane, and the midbrain tegmentum plane. In addition, we found for the first time that oculomotor measures (vertical gain, velocity, and the diagnostic index) were correlated significantly to midbrain volume in the PSP group.Conclusions: Assessing eye movements in a HMD provides an easy to apply and highly standardized tool to differentiate PSP of patients from PD and HCs, which will aid in the diagnosis of PSP.}, keywords = {Parkinson's disease; saccades; PROGRESSIVE SUPRANUCLEAR PALSY; NEURODEGENERATIVE DISEASE; Head-mounted display; Oculomotor}, year = {2021}, eissn = {1664-2295}, orcid-numbers = {Herwig, Arvid/0000-0002-5979-3004; Huppertz, Hans-Jurgen/0000-0003-3856-9094; Rogalewski, Andreas/0000-0002-6525-4832} } @article{MTMT:32483761, title = {Investigating the 1-year decline in midbrain-to-pons ratio in the differential diagnosis of PSP and IPD}, url = {https://m2.mtmt.hu/api/publication/32483761}, author = {Kannenberg, Silja and Caspers, Julian and Dinkelbach, Lars and Moldovan, Alexia-S. and Ferrea, Stefano and Suedmeyer, Martin and Butz, Markus and Schnitzler, Alfons and Hartmann, Christian J.}, doi = {10.1007/s00415-020-10327-2}, journal-iso = {J NEUROL}, journal = {JOURNAL OF NEUROLOGY}, volume = {268}, unique-id = {32483761}, issn = {0340-5354}, abstract = {Background A reliable measure of PSP-specific midbrain atrophy, the midbrain-to-pons ratio (MTPR) has been reported to support the differential diagnosis of progressive supranuclear palsy (PSP) from idiopathic Parkinson's disease (IPD). Since longitudinal analyses are lacking so far, the present study aimed to evaluate the diagnostic value of the relative change of MTPR (rel Delta t_MTPR) over a 1-year period in patients with PSP, IPD, and healthy controls (HC). Methods Midsagittal individual MRIs of patients with PSP (n = 15), IPD (n = 15), and healthy controls (HC; n = 15) were assessed and the MTPR at baseline and after 1 year were defined. The diagnostic accuracy of the MTPR and its relative change were evaluated using ROC curve analyses. Results PSP-patients had a significantly lower MTPR at baseline (M = 0.45 +/- 0.06), compared to both non-PSP groups (F (2, 41) = 62.82, p < 0.001), with an overall predictive accuracy of 95.6% for an MTPR <= 0.54. PSP-patients also presented a significantly stronger 1-year decline in MTPR compared to IPD (p < 0.001). Though predictive accuracy of rel Delta(t)_MTPR for PSP (M = - 4.74% +/- 4.48) from IPD (M = + 1.29 +/- 3.77) was good (76.6%), ROC analysis did not reveal a significant improvement of diagnostic accuracy by combining the MTPR and rel Delta(t)_MTPR (p = 0.670). Still, specificity for PSP increased, though not significantly (p = 0.500). Conclusion The present results indicate that the rel Delta(t)_MTPR is a potentially useful tool to support the differential diagnosis of PSP from IPD. For its relative 1-year change, still, more evaluation is needed.}, keywords = {MRI; PROGRESSIVE SUPRANUCLEAR PALSY; Atypical parkinsonism; s disease; Idiopathic Parkinson’; Midbrain-to-pons ratio}, year = {2021}, eissn = {1432-1459}, pages = {1526-1532}, orcid-numbers = {Butz, Markus/0000-0003-1438-5792; Schnitzler, Alfons/0000-0002-6414-7939} } @article{MTMT:32483762, title = {Conventional Magnetic Resonance Imaging in the Diagnosis of Parkinsonian Disorders: AMeta-Analysis}, url = {https://m2.mtmt.hu/api/publication/32483762}, author = {Lee, Will}, doi = {10.1002/mdc3.13070}, journal-iso = {MOV DISORD CLIN PRAC}, journal = {MOVEMENT DISORDERS CLINICAL PRACTICE}, volume = {8}, unique-id = {32483762}, issn = {2330-1619}, abstract = {Background: Numerous conventional magnetic resonance imaging (cMRI) parameters were previously found to differentiate parkinsonian disorders with statistical significance, but effect size has not been considered.Objectives: To quantify effect size of previously identified cMRI parameters that differentiated parkinsonian disorders with statistical significance.Method: A PubMed search limited to studies assessing cMRI parameters in at least 2 of Parkinson's disease, progressive supranuclear palsy, multiple system atrophy, and corticobasal degeneration/syndrome were selected. Either Cohen's d or positive and negative likelihood (LR+/-) as well as diagnostic odds ratios (DORs) were calculated as appropriate. cMRI parameter was considered useful if Cohen's d > 1.94 (<20% overlap) or if LR+ > 10, LR- < 0.1, or DOR > 20.Results: Literature search identified 8848 publications and 36 were included for analysis. Putaminal (Cohen's d 2.07; DOR 23-infinity), pontine (DOR 32-infinity), and middle cerebellar peduncle (Cohen's d 2.24; DOR infinity) abnormalities were most useful in differentiating multiple system atrophy while reduced midbrain (Cohen's d 2.33-8.69; DOR infinity) and superior cerebellar peduncle (Cohen's d 2.47; DOR 51-infinity) diameters separated progressive supranuclear palsy. Corticobasal degeneration/syndrome does not have any distinguishing cMRI features, but reduced midbrain diameter may help differentiate corticobasal degeneration/syndrome from Parkinson's disease (DOR infinity). When LR- was calculated, all of these features carried a value of <0.1.Conclusion: A number of cMRI features consistently demonstrated large effect size in separating parkinsonian disorders. However, it is the presence and not absence of these cMRI features that is most useful in patients with low to moderate pretest probability.}, keywords = {PARKINSONISM; diagnosis; magnetic resonance; Parkinsonian}, year = {2021}, eissn = {2330-1619}, pages = {217-223} } @article{MTMT:32388905, title = {Neuroimaging in Frontotemporal Dementia: Heterogeneity and Relationships with Underlying Neuropathology}, url = {https://m2.mtmt.hu/api/publication/32388905}, author = {Peet, Bradley T. and Spina, Salvatore and Mundada, Nidhi and La Joie, Renaud}, doi = {10.1007/s13311-021-01101-x}, journal-iso = {NEUROTHERAPEUTICS}, journal = {NEUROTHERAPEUTICS}, volume = {18}, unique-id = {32388905}, issn = {1933-7213}, abstract = {Frontotemporal dementia encompasses a group of clinical syndromes defined pathologically by degeneration of the frontal and temporal lobes. Historically, these syndromes have been challenging to diagnose, with an average of about three years between the time of symptom onset and the initial evaluation and diagnosis. Research in the field of neuroimaging has revealed numerous biomarkers of the various frontotemporal dementia syndromes, which has provided clinicians with a method of narrowing the differential diagnosis and improving diagnostic accuracy. As such, neuroimaging is considered a core investigative tool in the evaluation of neurodegenerative disorders. Furthermore, patterns of neurodegeneration correlate with the underlying neuropathological substrates of the frontotemporal dementia syndromes, which can aid clinicians in determining the underlying etiology and improve prognostication. This review explores the advancements in neuroimaging and discusses the phenotypic and pathologic features of behavioral variant frontotemporal dementia, semantic variant primary progressive aphasia, and nonfluent variant primary progressive aphasia, as seen on structural magnetic resonance imaging and positron emission tomography.}, keywords = {Magnetic Resonance Imaging; TAU; positron emission tomography; neuroimaging; FRONTOTEMPORAL LOBAR DEGENERATION; TDP-43; FRONTOTEMPORAL DEMENTIA; Semantic dementia; FTLD-tau; Behavioral variant frontotemporal dementia; progressive nonfluent aphasia; FTLD-TDP; semantic variant primary progressive aphasia; Nonfluent agrammatic variant primary progressive aphasia}, year = {2021}, eissn = {1878-7479}, pages = {728-752}, orcid-numbers = {Spina, Salvatore/0000-0003-3570-9143; Mundada, Nidhi/0000-0003-3108-6751; La Joie, Renaud/0000-0003-2581-8100} } @article{MTMT:32327858, title = {Evolving concepts in progressive supranuclear palsy and other 4-repeat tauopathies}, url = {https://m2.mtmt.hu/api/publication/32327858}, author = {Stamelou, Maria and Respondek, Gesine and Giagkou, Nikolaos and Whitwell, Jennifer L. and Kovács, Gábor Géza and Hoglinger, Gunter U.}, doi = {10.1038/s41582-021-00541-5}, journal-iso = {NAT REV NEUROL}, journal = {NATURE REVIEWS NEUROLOGY}, volume = {17}, unique-id = {32327858}, issn = {1759-4758}, abstract = {In this Review, Stamelou et al. present an overview of the latest research in 4-repeat tauopathies, with a focus on progressive supranuclear palsy, and discuss how current evidence dictates ongoing and future research goals.Tauopathies are classified according to whether tau deposits predominantly contain tau isoforms with three or four repeats of the microtubule-binding domain. Those in which four-repeat (4R) tau predominates are known as 4R-tauopathies, and include progressive supranuclear palsy, corticobasal degeneration, argyrophilic grain disease, globular glial tauopathies and conditions associated with specific MAPT mutations. In these diseases, 4R-tau deposits are found in various cell types and anatomical regions of the brain and the conditions share pathological, pathophysiological and clinical characteristics. Despite being considered 'prototype' tauopathies and, therefore, ideal for studying neuroprotective agents, 4R-tauopathies are still severe and untreatable diseases for which no validated biomarkers exist. However, advances in research have addressed the issues of phenotypic overlap, early clinical diagnosis, pathophysiology and identification of biomarkers, setting a road map towards development of treatments. New clinical criteria have been developed and large cohorts with early disease are being followed up in prospective studies. New clinical trial readouts are emerging and biomarker research is focused on molecular pathways that have been identified. Lessons learned from failed trials of neuroprotective drugs are being used to design new trials. In this Review, we present an overview of the latest research in 4R-tauopathies, with a focus on progressive supranuclear palsy, and discuss how current evidence dictates ongoing and future research goals.}, year = {2021}, eissn = {1759-4766}, pages = {601-620}, orcid-numbers = {Kovács, Gábor Géza/0000-0003-3841-5511} } @article{MTMT:32953651, title = {Intracortical diffusion tensor imaging signature of microstructural changes in frontotemporal lobar degeneration}, url = {https://m2.mtmt.hu/api/publication/32953651}, author = {Torso, Mario and Ridgway, Gerard R. and Jenkinson, Mark and Chance, Steven}, doi = {10.1186/s13195-021-00914-4}, journal-iso = {ALZHEIMERS RES THER}, journal = {ALZHEIMERS RESEARCH & THERAPY}, volume = {13}, unique-id = {32953651}, issn = {1758-9193}, abstract = {Background Frontotemporal lobar degeneration (FTLD) is a neuropathological construct with multiple clinical presentations, including the behavioural variant of frontotemporal dementia (bvFTD), primary progressive aphasia-both non-fluent variant (nfvPPA) and semantic variant (svPPA)-progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS), characterised by the deposition of abnormal tau protein in the brain. A major challenge for treating FTLD is early diagnosis and accurate discrimination among different syndromes. The main goal here was to investigate the cortical architecture of FTLD syndromes using cortical diffusion tensor imaging (DTI) analysis and to test its power to discriminate between different clinical presentations. Methods A total of 271 individuals were included in the study: 87 healthy subjects (HS), 31 semantic variant primary progressive aphasia (svPPA), 37 behavioural variant (bvFTD), 30 non-fluent/agrammatic variant primary progressive aphasia (nfvPPA), 47 PSP Richardson's syndrome (PSP-RS) and 39 CBS cases. 3T MRI T1-weighted images and DTI scans were analysed to extract three cortical DTI derived measures (AngleR, PerpPD and ParlPD) and mean diffusivity (MD), as well as standard volumetric measurements. Whole brain and regional data were extracted. Linear discriminant analysis was used to assess the group discrimination capability of volumetric and DTI measures to differentiate the FTLD syndromes. In addition, in order to further investigate differential diagnosis in CBS and PSP-RS, a subgroup of subjects with autopsy confirmation in the training cohort was used to select features which were then tested in the test cohort. Three different challenges were explored: a binary classification (controls vs all patients), a multiclass classification (HS vs bvFTD vs svPPA vs nfvPPA vs CBS vs PSP-RS) and an additional binary classification to differentiate CBS and PSP-RS using features selected in an autopsy confirmed subcohort. Results Linear discriminant analysis revealed that PerpPD was the best feature to distinguish between controls and all patients (ACC 86%). PerpPD regional values were able to classify correctly the different FTLD syndromes with an accuracy of 85.6%. The PerpPD and volumetric values selected to differentiate CBS and PSP-RS patients showed a classification accuracy of 85.2%. Conclusions (I) PerpPD achieved the highest classification power for differentiating healthy controls and FTLD syndromes and FTLD syndromes among themselves. (II) PerpPD regional values could provide an additional marker to differentiate FTD, PSP-RS and CBS.}, keywords = {CORTEX; diffusion tensor imaging; CBS; FTD; FTLD; PSP; intracortical; minicolumn; microstructural; Cortical diffusivity}, year = {2021}, eissn = {1758-9193}, orcid-numbers = {Torso, Mario/0000-0002-5284-9167} } @article{MTMT:35514001, title = {"Hot cross bun" is a potential imaging marker for the severity of cerebellar ataxia in MSA-C}, url = {https://m2.mtmt.hu/api/publication/35514001}, author = {Zhu, Shuzhen and Deng, Bin and Huang, Zifeng and Chang, Zihan and Li, Hualin and Liu, Hui and Huang, Yanjun and Pan, Ying and Wang, Yanping and Chao, Yin-Xia and Chan, Ling-Ling and Wu, Yih-Ru and Tan, Eng-King and Wang, Qing}, doi = {10.1038/s41531-021-00159-w}, journal-iso = {NPJ PARKINSONS DIS}, journal = {NPJ PARKINSONS DISEASE}, volume = {7}, unique-id = {35514001}, year = {2021}, eissn = {2373-8057} } @article{MTMT:35515632, title = {Spinocerebellar ataxia type 6 family with phenotypic overlap with Multiple System Atrophy}, url = {https://m2.mtmt.hu/api/publication/35515632}, author = {Al-Shaikh, Rana Hanna and Wernick, Anna I and Strongosky, Audrey J. and Soto-Beasley, Alexandra I and van Gerpen, Jay A. and Cheshire, William P. and Uitti, Ryan J. and Ross, Owen A. and Wszolek, Zbigniew K.}, doi = {10.5603/PJNNS.a2020.0053}, journal-iso = {NEUROL NEUROCHIR POL}, journal = {NEUROLOGIA I NEUROCHIRURGIA POLSKA}, volume = {54}, unique-id = {35515632}, issn = {0028-3843}, keywords = {CEREBELLUM; spinocerebellar ataxia; SCA; Gait disorders/ataxia}, year = {2020}, eissn = {1897-4260}, pages = {350-355}, orcid-numbers = {Wernick, Anna I/0000-0001-9048-9492} } @article{MTMT:31438059, title = {The path to biomarker-based diagnostic criteria for the spectrum of neurodegenerative diseases}, url = {https://m2.mtmt.hu/api/publication/31438059}, author = {Baldacci, Filippo and Mazzucchi, Sonia and Della Vecchia, Alessandra and Giampietri, Linda and Giannini, Nicola and Koronyo-Hamaoui, Maya and Ceravolo, Roberto and Siciliano, Gabriele and Bonuccelli, Ubaldo and Elahi, Fanny M. and Vergallo, Andrea and Lista, Simone and Giorgi, Filippo Sean and Hampel, Harald}, doi = {10.1080/14737159.2020.1731306}, journal-iso = {EXPERT REV MOL DIAGN}, journal = {EXPERT REVIEW OF MOLECULAR DIAGNOSTICS}, volume = {20}, unique-id = {31438059}, issn = {1473-7159}, abstract = {Introduction: The postmortem examination still represents the reference standard for detecting the pathological nature of chronic neurodegenerative diseases (NDD). This approach displays intrinsic conceptual limitations since NDD represent a dynamic spectrum of partially overlapping phenotypes, shared pathomechanistic alterations that often give rise to mixed pathologies. Areas covered: We scrutinized the international clinical diagnostic criteria of NDD and the literature to provide a roadmap toward a biomarker-based classification of the NDD spectrum. A few pathophysiological biomarkers have been established for NDD. These are time-consuming, invasive, and not suitable for preclinical detection. Candidate screening biomarkers are gaining momentum. Blood neurofilament light-chain represents a robust first-line tool to detect neurodegeneration tout court and serum progranulin helps detect genetic frontotemporal dementia. Ultrasensitive assays and retinal scans may identify A beta pathology early, in blood and the eye, respectively. Ultrasound also represents a minimally invasive option to investigate the substantia nigra. Protein misfolding amplification assays may accurately detect alpha-synuclein in biofluids. Expert opinion: Data-driven strategies using quantitative rather than categorical variables may be more reliable for quantification of contributions from pathophysiological mechanisms and their spatial-temporal evolution. A systems biology approach is suitable to untangle the dynamics triggering loss of proteostasis, driving neurodegeneration and clinical evolution.}, keywords = {Biomarkers; Alzheimer's disease; Parkinson disease; amyotrophic lateral sclerosis; CEREBRAL AMYLOID ANGIOPATHY}, year = {2020}, eissn = {1744-8352}, pages = {421-441}, orcid-numbers = {Koronyo-Hamaoui, Maya/0000-0003-2864-8442; Vergallo, Andrea/0000-0002-0208-6384} } @article{MTMT:35515637, title = {Automated Brainstem Segmentation Detects Differential Involvement Atypical in Parkinsonian Syndromes}, url = {https://m2.mtmt.hu/api/publication/35515637}, author = {Bocchetta, Martina and Iglesias, Juan Eugenio and Chelban, Viorica and Jabbari, Edwin and Lamb, Ruth and Russell, Lucy L. and Greaves, Caroline V and Neason, Mollie and Cash, David M. and Thomas, David L. and Warren, Jason D. and Woodside, John and Houlden, Henry and Morris, Huw R. and Rohrer, Jonathan D.}, doi = {10.14802/jmd.19030}, journal-iso = {J MOV DISORD}, journal = {JOURNAL OF MOVEMENT DISORDERS}, volume = {13}, unique-id = {35515637}, issn = {2005-940X}, keywords = {Magnetic Resonance Imaging; brainstem; parkinsonian syndromes}, year = {2020}, eissn = {2093-4939}, pages = {39-+}, orcid-numbers = {Bocchetta, Martina/0000-0003-1814-5024; Jabbari, Edwin/0000-0001-6844-882X; Greaves, Caroline V/0000-0002-6446-1960; Houlden, Henry/0000-0002-2866-7777} } @article{MTMT:35515636, title = {Prediction of the Clinical Severity of Progressive Supranuclear Palsy by Diffusion Tensor Imaging}, url = {https://m2.mtmt.hu/api/publication/35515636}, author = {Chen, Yao-Liang and Zhao, Xiang-An and Ng, Shu-Hang and Lu, Chin-Song and Lin, Yu-Chun and Cheng, Jur-Shan and Tsai, Chih-Chien and Wang, Jiun-Jie}, doi = {10.3390/jcm9010040}, journal-iso = {J CLIN MED}, journal = {JOURNAL OF CLINICAL MEDICINE}, volume = {9}, unique-id = {35515636}, keywords = {SEVERITY; diffusion tensor imaging; PROGRESSIVE SUPRANUCLEAR PALSY; UPDRS-III; LEDD}, year = {2020}, eissn = {2077-0383}, orcid-numbers = {Lin, Yu-Chun/0000-0001-6841-5366; Wang, Jiun-Jie/0000-0002-2422-9059} } @article{MTMT:35514010, title = {Progressive supranuclear palsy: Advances in diagnosis and management}, url = {https://m2.mtmt.hu/api/publication/35514010}, author = {Coughlin, David G. and Litvan, Irene}, doi = {10.1016/j.parkreldis.2020.04.014}, journal-iso = {PARKINSONISM RELAT D}, journal = {PARKINSONISM AND RELATED DISORDERS}, volume = {73}, unique-id = {35514010}, issn = {1353-8020}, keywords = {immunotherapy; treatment; Gene Therapy; PROGRESSIVE SUPRANUCLEAR PALSY; tauopathy}, year = {2020}, eissn = {1873-5126}, pages = {105-116} } @article{MTMT:35515630, title = {Iron deposition in Parkinsonisms: A Quantitative Susceptibility Mapping study in the deep grey matter}, url = {https://m2.mtmt.hu/api/publication/35515630}, author = {Fedeli, Maria Paola and Contarino, Valeria Elisa and Siggillino, Silvia and Samoylova, Nina and Calloni, Sonia and Melazzini, Luca and Conte, Giorgio and Sacilotto, Giorgio and Pezzoli, Gianni and Triulzi, Fabio Maria and Scola, Elisa}, doi = {10.1016/j.ejrad.2020.109394}, journal-iso = {EUR J RADIOL}, journal = {EUROPEAN JOURNAL OF RADIOLOGY}, volume = {133}, unique-id = {35515630}, issn = {0720-048X}, keywords = {Magnetic Resonance Imaging; Parkinson's disease; Quantitative susceptibility mapping; iron deposition; Atypical primary parkinsonism}, year = {2020}, eissn = {1872-7727}, orcid-numbers = {Contarino, Valeria Elisa/0000-0002-8922-657X; Pezzoli, Gianni/0000-0003-4665-6710} } @article{MTMT:35515634, title = {Update on the diagnosis and management of Parkinson's disease}, url = {https://m2.mtmt.hu/api/publication/35515634}, author = {Kobylecki, Christopher}, doi = {10.7861/clinmed.2020-0220}, journal-iso = {CLIN MED}, journal = {CLINICAL MEDICINE}, volume = {20}, unique-id = {35515634}, issn = {1470-2118}, keywords = {diagnosis; Parkinson's disease; levodopa; treatment; non-motor symptoms}, year = {2020}, eissn = {1473-4893}, pages = {393-398}, orcid-numbers = {Kobylecki, Christopher/0000-0002-7797-0756} } @article{MTMT:35513253, title = {Changes of Amide Proton Transfer Imaging in Multiple System Atrophy Parkinsonism Type}, url = {https://m2.mtmt.hu/api/publication/35513253}, author = {Li, Shuhua and Chan, Piu and Li, Chunmei and Chen, Haibo and Chen, Min and Su, Wen and Li, Kai and Lu, Na and Yu, Lu and Chu, Defa and Wu, Pu-Yeh}, doi = {10.3389/fnagi.2020.572421}, journal-iso = {FRONT AGING NEUROSCI}, journal = {FRONTIERS IN AGING NEUROSCIENCE}, volume = {12}, unique-id = {35513253}, keywords = {PARKINSONISM; Magnetic Resonance Imaging; biomarker; Multiple system atrophy; APT imaging}, year = {2020}, eissn = {1663-4365}, orcid-numbers = {Wu, Pu-Yeh/0000-0002-9216-025X} } @article{MTMT:31721452, title = {Multicenter Validation of Metabolic Abnormalities Related toPSPAccording to theMDS-PSPCriteria}, url = {https://m2.mtmt.hu/api/publication/31721452}, author = {Marti-Andres, Gloria and van Bommel, Liza and Meles, Sanne K. and Riverol, Mario and Valenti, Rafael and Kogan, Rosalie V and Renken, Remco J. and Gurvits, Vita and van Laar, Teus and Pagani, Marco and Prieto, Elena and Luquin, M. Rosario and Leenders, Klaus L. and Arbizu, Javier}, doi = {10.1002/mds.28217}, journal-iso = {MOVEMENT DISORD}, journal = {MOVEMENT DISORDERS}, volume = {35}, unique-id = {31721452}, issn = {0885-3185}, abstract = {It remains unclear whether the supportive imaging features described in the diagnostic criteria for progressive supranuclear palsy (PSP) are suitable for the full clinical spectrum. The aim of the current study was to define and cross-validate the pattern of glucose metabolism in the brain associated with a diagnosis of different PSP variants. A retrospective multicenter cohort study performed on 73 PSP patients who were referred for a fluorodeoxyglucose positron emission tomography PET scan: PSP-Richardson's syndrome, n = 47; PSP-parkinsonian variant, n = 18; and progressive gait freezing, n = 8. In addition, we included 55 healthy controls and 58 Parkinson's disease (PD) patients. Scans were normalized by global mean activity. We analyzed the regional differences in metabolism between the groups. Moreover, we applied a multivariate analysis to obtain a PSP-related pattern that was cross-validated in independent populations at the individual level. Group analysis showed relative hypometabolism in the midbrain, basal ganglia, thalamus, and frontoinsular cortices and hypermetabolism in the cerebellum and sensorimotor cortices in PSP patients compared with healthy controls and PD patients, the latter with more severe involvement in the basal ganglia and occipital cortices. The PSP-related pattern obtained confirmed the regions described above. At the individual level, the PSP-related pattern showed optimal diagnostic accuracy to distinguish between PSP and healthy controls (sensitivity, 80.4%; specificity, 96.9%)s and between PSP and PD (sensitivity, 80.4%; specificity, 90.7%). Moreover, PSP-Richardson's syndrome and PSP-parkinsonian variant patients showed significantly more PSP-related pattern expression than PD patients and healthy controls. The glucose metabolism assessed by fluorodeoxyglucose PET is a useful and reproducible supportive diagnostic tool for PSP-Richardson's syndrome and PSP-parkinsonian variant. (c) 2020 International Parkinson and Movement Disorder Society}, keywords = {PROGRESSIVE SUPRANUCLEAR PALSY; FDG-PET; Diagnostic biomarker; disease-related metabolic brain pattern}, year = {2020}, eissn = {1531-8257}, pages = {2009-2018}, orcid-numbers = {Marti-Andres, Gloria/0000-0001-6474-8454; van Laar, Teus/0000-0001-5088-480X} } @article{MTMT:35514019, title = {The Progressive Supranuclear Palsy: Past and Present Aspects}, url = {https://m2.mtmt.hu/api/publication/35514019}, author = {Parthimos, Theodore P. and Schulpis, Kleopatra H.}, doi = {10.1080/07317115.2019.1694115}, journal-iso = {CLIN GERONTOLOGIST}, journal = {Clinical Gerontologist}, volume = {43}, unique-id = {35514019}, issn = {0731-7115}, keywords = {PARKINSONISM; cognition; neurodegeneration; executive functions; MAPT}, year = {2020}, eissn = {1545-2301}, pages = {155-180}, orcid-numbers = {Parthimos, Theodore P./0000-0003-0671-2503} } @article{MTMT:35515633, title = {Can Autonomic Testing and Imaging Contribute to the Early Diagnosis of Multiple System Atrophy? A Systematic Review and Recommendations by theMovement Disorder SocietyMultiple System Atrophy Study Group}, url = {https://m2.mtmt.hu/api/publication/35515633}, author = {Pellecchia, Maria Teresa and Stankovic, Iva and Fanciulli, Alessandra and Krismer, Florian and Meissner, Wassilios G. and Palma, Jose-Alberto and Panicker, Jalesh N. and Seppi, Klaus and Wenning, Gregor K.}, doi = {10.1002/mdc3.13052}, journal-iso = {MOV DISORD CLIN PRAC}, journal = {MOVEMENT DISORDERS CLINICAL PRACTICE}, volume = {7}, unique-id = {35515633}, issn = {2330-1619}, keywords = {Multiple system atrophy; diagnostic tests}, year = {2020}, eissn = {2330-1619}, pages = {750-762}, orcid-numbers = {Krismer, Florian/0000-0002-4493-5073; Palma, Jose-Alberto/0000-0002-1345-6774; Seppi, Klaus/0000-0001-6503-1455} } @article{MTMT:31089560, title = {Multimodal Magnetic Resonance Imaging Quantification of Brain Changes in Progressive Supranuclear Palsy}, url = {https://m2.mtmt.hu/api/publication/31089560}, author = {Pyatigorskaya, Nadya and Yahia-Cherif, Lydia and Gaurav, Rahul and Ewenczyk, Claire and Gallea, Cecile and Valabregue, Romain and Gargouri, Fatma and Magnin, Benoit and Degos, Bertrand and Roze, Emmanuel and Bardinet, Eric and Poupon, Cyril and Arnulf, Isabelle and Vidailhet, Marie and Lehericy, Stephane}, doi = {10.1002/mds.27877}, journal-iso = {MOVEMENT DISORD}, journal = {MOVEMENT DISORDERS}, volume = {35}, unique-id = {31089560}, issn = {0885-3185}, abstract = {Background Progressive supranuclear palsy (PSP) is a neurodegenerative clinically heterogeneous disorder, formal diagnosis being based on postmortem histological brain examination. Objective We aimed to perform a precise in vivo staging of neurodegeneration in PSP using quantitative multimodal MRI. The ability of MRI biomarkers to differentiate PSP from PD was also evaluated. Methods Eleven PSP patients were compared to 26 age-matched healthy controls and 51 PD patients. Images were acquired at 3 Tesla (three-dimensional T-1-weighted, diffusion tensor, and neuromelanin-sensitive images) and 7 Tesla (three-dimensional-T-2* images). Regions of interest included the cortical areas, hippocampus, amygdala, basal ganglia, basal forebrain, brainstem nuclei, dentate nucleus, and cerebellum. Volumes, mean diffusivity, and fractional anisotropy were measured. In each region, a threshold value for group categorization was calculated, and four grades of change (0-3) were determined. Results PSP patients showed extensive volume decreases and diffusion changes in the midbrain, SN, STN, globus pallidus, basal forebrain, locus coeruleus, pedunculopontine nucleus, and dentate nucleus, in close agreement with the degrees of impairment in histological analyses. The predictive factors for the separation of PSP and healthy controls were, in descending order, the neuromelanin-based SN volume; midbrain fractional anisotropy; volumes of the midbrain, globus pallidus, and putamen; and fractional anisotropy in the locus coeruleus. The best predictors for separating PSP from PD were the neuromelanin-based volume in the SN, fractional anisotropy in the pons, volumes of the midbrain and globus pallidus, and fractional anisotropy in the basal forebrain. Conclusions These results suggest that it is possible to evaluate brain neurodegeneration in PSP noninvasively, even in small brainstem nuclei, in close agreement with previously published histological data. (c) 2019 International Parkinson and Movement Disorder Society}, keywords = {Biomarkers; VOLUMETRY; Movement Disorders; diffusion tensor imaging; PSP}, year = {2020}, eissn = {1531-8257}, pages = {161-170} } @article{MTMT:31718893, title = {Neuroimaging Advances in Parkinson's Disease and Atypical Parkinsonian Syndromes}, url = {https://m2.mtmt.hu/api/publication/31718893}, author = {Saeed, Usman and Lang, Anthony E. and Masellis, Mario}, doi = {10.3389/fneur.2020.572976}, journal-iso = {FRONT NEUR}, journal = {FRONTIERS IN NEUROLOGY}, volume = {11}, unique-id = {31718893}, abstract = {Parkinson's disease (PD) and atypical Parkinsonian syndromes are progressive heterogeneous neurodegenerative diseases that share clinical characteristic of parkinsonism as a common feature, but are considered distinct clinicopathological disorders. Based on the predominant protein aggregates observed within the brain, these disorders are categorized as, (1) alpha-synucleinopathies, which include PD and other Lewy body spectrum disorders as well as multiple system atrophy, and (2) tauopathies, which comprise progressive supranuclear palsy and corticobasal degeneration. Although, great strides have been made in neurodegenerative disease research since the first medical description of PD in 1817 by James Parkinson, these disorders remain a major diagnostic and treatment challenge. A valid diagnosis at early disease stages is of paramount importance, as it can help accommodate differential prognostic and disease management approaches, enable the elucidation of reliable clinicopathological relationships ideally at prodromal stages, as well as facilitate the evaluation of novel therapeutics in clinical trials. However, the pursuit for early diagnosis in PD and atypical Parkinsonian syndromes is hindered by substantial clinical and pathological heterogeneity, which can influence disease presentation and progression. Therefore, reliable neuroimaging biomarkers are required in order to enhance diagnostic certainty and ensure more informed diagnostic decisions. In this article, an updated presentation of well-established and emerging neuroimaging biomarkers are reviewed from the following modalities: (1) structural magnetic resonance imaging (MRI), (2) diffusion-weighted and diffusion tensor MRI, (3) resting-state and task-based functional MRI, (4) proton magnetic resonance spectroscopy, (5) transcranial B-mode sonography for measuring substantia nigra and lentiform nucleus echogenicity, (6) single photon emission computed tomography for assessing the dopaminergic system and cerebral perfusion, and (7) positron emission tomography for quantifying nigrostriatal functions, glucose metabolism, amyloid, tau and alpha-synuclein molecular imaging, as well as neuroinflammation. Multiple biomarkers obtained from different neuroimaging modalities can provide distinct yet corroborative information on the underlying neurodegenerative processes. This integrative "multimodal approach" may prove superior to single modality-based methods. Indeed, owing to the international, multi-centered, collaborative research initiatives as well as refinements in neuroimaging technology that are currently underway, the upcoming decades will mark a pivotal and exciting era of further advancements in this field of neuroscience.}, keywords = {Biomarkers; Magnetic resonance imaging (MRI); Single photon emission computed tomography (SPECT); Positron emission tomography (PET); atypical parkinsonian syndromes; Diffusion-weighted imaging (DWI); Parkinson' s disease (PD); transcranial sonography (TCS)}, year = {2020}, eissn = {1664-2295} } @article{MTMT:32483763, title = {Diagnostic Performance of I-123-FP-CIT SPECT Specific Binding Ratio in Progressive Supranuclear Palsy: Use of Core Clinical Features and MRI for Comparison}, url = {https://m2.mtmt.hu/api/publication/32483763}, author = {Sakamoto, Fumi and Shiraishi, Shinya and Kitajima, Mika and Ogasawara, Koji and Tsuda, Noriko and Tomiguchi, Seiji and Yamashita, Yasuyuki}, doi = {10.2214/AJR.19.22436}, journal-iso = {AM J ROENTGENOL}, journal = {AMERICAN JOURNAL OF ROENTGENOLOGY}, volume = {215}, unique-id = {32483763}, issn = {0361-803X}, abstract = {OBJECTIVE. Progressive supranuelear palsy (PSP) is listed as a core clinical feature in the Movement Disorder Society 2017 criteria, along with ocular motor dysfunction, postural instability, akinesia, and cognitive dysfunction. Imaging evidence shows predominant midbrain atrophy and postsynaptic striatal dopaminergic degeneration as two supportive features. The purpose of this study was to investigate the diagnostic performance of I-123-N-omega-fluoropropyl-2 beta-carbomethoxy-3 beta-(4-iodophenyl) nortropane (I-123-FP-CIT) SPECT by comparing it with evaluation of core clinical features and MRI in the diagnosis of PSP.MATERIALS AND METHODS. The study included 53 patients with clinically suspected PSP who had undergone I-123-FP-CIT SPECT and MRI examinations. MR parkinsonism index (MRPI) was used as the MRI index. For the I-123-FP-CIT SPECT index, specific binding ratio (SBR) was calculated as the average of the right and left SBRs.RESULTS. In regard to core clinical features, ocular motor dysfunction was present in 15 of 20 (75.0%) patients with the diagnosis of probable PSP (p < 0.0001). Calculation of the diagnostic performance of the imaging parameters showed that MRPI (cutoff > 11.6) had 85.0% sensitivity, 100% specificity, and 94.3% accuracy. SBR (cutoff < 3.7) had 95.0% sensitivity, 36.4% specificity, and 58.5% accuracy.CONCLUSION. Iodine-123-labeled FP-CIT SPECT has high sensitivity, and MRI has high specificity in the diagnosis of PSP. Because these tools have complementary roles, reaching a more confident clinical diagnosis of PSP may be possible when both are used.}, keywords = {SPECT; MRI; PROGRESSIVE SUPRANUCLEAR PALSY; Specific binding ratio; I-123-FP-CIT dopamine transporter; MR parkinsonism index}, year = {2020}, eissn = {1546-3141}, pages = {1443-1448} } @article{MTMT:31503655, title = {Vertical pons hyperintensity and hot cross bun sign in cerebellar-type multiple system atrophy and spinocerebellar ataxia type 3}, url = {https://m2.mtmt.hu/api/publication/31503655}, author = {Sugiyama, Atsuhiko and Yokota, Hajime and Yamanaka, Yoshitaka and Mukai, Hiroki and Yamamoto, Tatsuya and Hirano, Shigeki and Koide, Kyosuke and Ito, Shoichi and Kuwabara, Satoshi}, doi = {10.1186/s12883-020-01738-9}, journal-iso = {BMC NEUROL}, journal = {BMC NEUROLOGY}, volume = {20}, unique-id = {31503655}, issn = {1471-2377}, abstract = {Background The "hot cross bun" (HCB) sign, a cruciform hyperintensity in the pons on magnetic resonance imaging (MRI), has gradually been identified as a typical finding in multiple system atrophy, cerebellar-type (MSA-C). Few reports have evaluated the sensitivity of an HCB, including a cruciform hyperintensity and vertical line in the pons, which precedes a cruciform hyperintensity, in the early stages of MSA-C. Moreover, the difference in frequency and timing of appearance of an HCB between MSA-C and spinocerebellar ataxia type 3 (SCA3) has not been fully investigated. Methods This study investigated the time at which an HCB and orthostatic hypotension (OH) appeared in 41 patients with MSA-C, based on brain MRI and head-up tilt test. The MRI findings were compared with those of 26 patients with SCA3. The pontine signal findings on T2-weighted MRI were graded as 0 (no change), 1 (a vertical T2 high-intensity line), or 2 (a cruciform T2 high-intensity line), with grades 1 or 2 considered as an HCB. OH 30/15 was defined as a decrease in systolic blood pressure of > 30 mmHg or diastolic blood pressure of > 15 mmHg. Results Among the 24 patients with MSA-C within 2 years from the onset of motor symptoms, an HCB was detected in 91.7%, whereas OH 30/15 was present in 60.0%. Among the 36 patients with MSA-C within 3 years from the onset of motor symptoms, a grade 2 HCB was detected in 66.7% of those with MSA-C but in none of those with SCA-3. Conclusions HCB is a highly sensitive finding for MSA-C, even in the early stages of the disease. A grade 2 HCB in the early stage is an extremely specific finding for differentiating MSA-C from SCA-3.}, keywords = {Magnetic Resonance Imaging; orthostatic hypotension; Multiple system atrophy; Spinocerebellar ataxia type 3; Hot cross bun sign}, year = {2020}, eissn = {1471-2377} } @article{MTMT:32483766, title = {Relationship between cardiac parasympathetic dysfunction and the anteroposterior diameter of the medulla oblongata in multiple system atrophy}, url = {https://m2.mtmt.hu/api/publication/32483766}, author = {Suzuki, Masashi and Nakamura, Tomohiko and Hirayama, Masaaki and Ueda, Miki and Imai, Eriko and Harada, Yumiko and Katsuno, Masahisa}, doi = {10.1007/s10286-020-00675-4}, journal-iso = {CLIN AUTON RES}, journal = {CLINICAL AUTONOMIC RESEARCH}, volume = {30}, unique-id = {32483766}, issn = {0959-9851}, abstract = {Purpose Neurodegeneration of the nucleus ambiguus and the dorsal vagal motor nucleus has been implicated in cardiac parasympathetic dysfunction in multiple system atrophy (MSA). The nucleus ambiguus and the dorsal vagal motor nucleus, which are located in the medulla oblongata (MO), control the autonomic-specifically, the parasympathetic-functions of the body. The aim of our study was to investigate the relationship between cardiac parasympathetic dysfunction and the anteroposterior diameter of the MO in MSA by quantitatively analyzing magnetic resonance imaging (MRI) outcome measures. Methods We retrospectively assessed 40 consecutive patients with probable MSA and 25 age- and sex-matched controls. The anteroposterior diameter of the MO at two locations (MO diameter-A and -B) and the diameters of the midbrain and pons were measured by conventional MRI. A cardiac parasympathetic function score (CP-score) and cardiac sympathetic function score (CS-score) were generated by calculating the z-scores of multiple autonomic function tests. The relationship between the scores and the measured diameters of the brainstem was also investigated. Results The CP-score and CS-score were significantly lower in the patients with MSA than in the controls (CP-score: 0.61 +/- 0.75 vs. - 0.38 +/- 0.52, p < 0.001; CS-score: 0.91 +/- 1.06 vs. - 0.57 +/- 1.07, p < 0.001). Also, in the patients with MSA, the CP-score was significantly correlated with MO diameter-A (r = 0.40, p = 0.010), and the CS-score was significantly correlated with the diameter of the midbrain (r = 0.33, p = 0.038). Conclusion The anteroposterior diameter of the MO is a potential imaging marker of parasympathetic dysfunction in MSA.}, keywords = {autonomic nervous system; Magnetic Resonance Imaging; autonomic dysfunction; Multiple system atrophy; Cardiac parasympathetic dysfunction}, year = {2020}, eissn = {1619-1560}, pages = {231-238}, orcid-numbers = {Nakamura, Tomohiko/0000-0001-7622-4527; Hirayama, Masaaki/0000-0003-0092-7837} } @article{MTMT:35515631, title = {Various Diseases and Clinical Heterogeneity Are Associated With "Hot Cross Bun"}, url = {https://m2.mtmt.hu/api/publication/35515631}, author = {Zhu, Shuzhen and Li, Hualing and Deng, Bin and Zheng, Jialing and Huang, Zifeng and Chang, Zihan and Huang, Yanjun and Wen, Zhibo and Liang, Yanran and Yu, Mengjue and Chan, Ling-Ling and Tan, Eng-King and Wang, Qing}, doi = {10.3389/fnagi.2020.592212}, journal-iso = {FRONT AGING NEUROSCI}, journal = {FRONTIERS IN AGING NEUROSCIENCE}, volume = {12}, unique-id = {35515631}, keywords = {Inflammation; Magnetic Resonance Imaging; stroke; ADEM; MSA; sign; disease spectrum; “; hot cross bun”}, year = {2020}, eissn = {1663-4365} } @article{MTMT:31085922, title = {Midbrain atrophy patients with presymptomatic progressive supranuclear palsy-Richardson's syndrome}, url = {https://m2.mtmt.hu/api/publication/31085922}, author = {Ahn, Jong Hyeon and Kim, Minkyeong and Kim, Ji Sun and Youn, Jinyoung and Jang, Wooyoung and Oh, Eungseok and Lee, Phil Hyu and Koh, Seong-Beom and Ahn, Tae-Beom and Cho, Jin Whan}, doi = {10.1016/j.parkreldis.2019.07.009}, journal-iso = {PARKINSONISM RELAT D}, journal = {PARKINSONISM AND RELATED DISORDERS}, volume = {66}, unique-id = {31085922}, issn = {1353-8020}, abstract = {Introduction In the present study, midbrain atrophy and the pons-to-midbrain area ratio (P/M ratio) were investigated as diagnostic markers for presymptomatic progressive supranuclear palsy-Richardson's syndrome (Pre-PSP-RS).Methods: The present study included 27 patients with probable PSP-RS who underwent brain MRI at least twice before and after the development of clinical symptoms, age- and sex-matched participants with Parkinson's disease (PD, n = 27), and healthy controls (n = 27). The midbrain area, pons area, and P/M ratio of the Pre-PSP-RS, PD, and control subjects were measured using midsagittal images from brain MRI, and the parameters were compared among the groups.Results: The midbrain area decreased and the P/M ratio increased significantly in the Pre-PSP-RS patients compared with both the PD and control subjects (midbrain, Pre-PSP-RS vs. PD = 1.01 cm(2) vs. 1.29 cm(2), p < 0.001, Pre-PSP-RS vs. controls = 1.01 cm(2) vs. 1.29 cm(2), p < 0.001; P/M ratio, Pre-PSP-RS vs. PD = 5.27 vs. 4.03, p < 0.001, Pre-PSP-RS vs. controls = 5.27 cm(2) vs. 4.06 cm(2), p < 0.001). The P/M ratio had high sensitivity (vs. PD, 96.3%, vs. control, 88.9%) and specificity (vs. PD, 81.5%, vs. control, 96.3%) in differentiating Pre-PSP-RS patients from PD and control subjects.Conclusion: Midbrain atrophy precedes the clinical symptoms of PSP-RS and could be a useful diagnostic imaging biomarker for Pre-PSP-RS. Furthermore, this information could play an important role in the development of future treatment strategies.}, keywords = {biomarker; PROGRESSIVE SUPRANUCLEAR PALSY; Richardson's syndrome; MIDBRAIN ATROPHY; Pons-to-midbrain ratio; Presymptomatic PSP}, year = {2019}, eissn = {1873-5126}, pages = {80-86}, orcid-numbers = {Kim, Ji Sun/0000-0003-3669-9151; Jang, Wooyoung/0000-0002-4808-7083; Ahn, Tae-Beom/0000-0002-7315-6298} } @article{MTMT:31579530, title = {Multiple system atrophy}, url = {https://m2.mtmt.hu/api/publication/31579530}, author = {Fanciulli, Alessandra and Stankovic, Iva and Krismer, Florian and Seppi, Klaus and Levin, Johannes and Wenning, Gregor K.}, doi = {10.1016/bs.irn.2019.10.004}, journal-iso = {INT REV NEUROBIOL}, journal = {INTERNATIONAL REVIEW OF NEUROBIOLOGY}, volume = {149}, unique-id = {31579530}, issn = {0074-7742}, abstract = {Multiple system atrophy (MSA) is a sporadic, adult-onset, relentlessly progressive neurodegenerative disorder, clinically characterized by various combinations of autonomic failure, parkinsonism and ataxia. The neuropathological hallmark of MSA are glial cytoplasmic inclusions consisting of misfolded alpha-synuclein. Selective atrophy and neuronal loss in striatonigral and olivopontocerebellar systems underlie the division into two main motor phenotypes of MSA-parkinsonian type and MSA-cerebellar type. Isolated autonomic failure and REM sleep behavior disorder are common premotor features of MSA. Beyond the core clinical symptoms, MSA manifests with a number of non-motor and motor features. Red flags highly specific for MSA may provide clues for a correct diagnosis, but in general the diagnostic accuracy of the second consensus criteria is suboptimal, particularly in early disease stages. In this chapter, the authors discuss the historical milestones, etiopathogenesis, neuropathological findings, clinical features, red flags, differential diagnosis, diagnostic criteria, imaging and other biomarkers, current treatment, unmet needs and future treatments for MSA.}, year = {2019}, eissn = {2162-5514}, pages = {137-192}, orcid-numbers = {Krismer, Florian/0000-0002-4493-5073; Levin, Johannes/0000-0001-5092-4306} } @article{MTMT:31065734, title = {Characteristics and progression of cognitive deficits in progressive supranuclear palsy vs. multiple system atrophy and Parkinson's disease}, url = {https://m2.mtmt.hu/api/publication/31065734}, author = {Fiorenzato, Eleonora and Antonini, Angelo and Camparini, Valeria and Weis, Luca and Semenza, Carlo and Biundo, Roberta}, doi = {10.1007/s00702-019-02065-1}, journal-iso = {J NEURAL TRANSM}, journal = {JOURNAL OF NEURAL TRANSMISSION}, volume = {126}, unique-id = {31065734}, issn = {0300-9564}, abstract = {Cognitive impairment is frequent in progressive supranuclear palsy (PSP) and less common in multiple system atrophy (MSA), but characteristics and progression compared with Parkinson's disease (PD) need to be properly defined. We evaluated 35 PSP with Richardson's syndrome (PSP-RS), 30 MSA as well as 65 age-, sex-, and education-matched PD with an extensive clinical and neuropsychological assessment, allowing Level II cognitive diagnosis. Eighteen PSP, 12 MSA and 30 PD had a second evaluation between 12 and 18 months (mean 15 months) after the first assessment. PSP performance at Montreal Cognitive Assessment (MoCA), verbal fluencies (phonemic and semantic tasks), Stroop test (Error and Time), Digit Span Sequencing (DSS), incomplete letters of Visual Object and Space Perception (VOSP) and Benton's Judgment of Line Orientation (JLO) performance were significantly poorer at baseline compared to PD and MSA. Executive, language and visuospatial abilities declined longitudinally in PSP, but not in PD and MSA. After 1.5 year, 16% of PSP converted to dementia. Our study provides evidence that cognitive progression is more severe and rapid in PSP-RS than PD and MSA. Further, we observed that MoCA, verbal fluency (particularly semantic), DSS and Benton's JLO are valuable tests to detect cognitive progression in PSP-RS and may be proposed as possible biomarker to assess efficacy of disease modification strategies.}, keywords = {Parkinson's disease; executive functions; longitudinal study; verbal fluency; PROGRESSIVE SUPRANUCLEAR PALSY; Visuospatial functions}, year = {2019}, eissn = {1435-1463}, pages = {1437-1445}, orcid-numbers = {Antonini, Angelo/0000-0003-1040-2807; Weis, Luca/0000-0002-2286-0795; Biundo, Roberta/0000-0001-5846-755X} } @article{MTMT:31582679, title = {Progressive supranuclear palsy}, url = {https://m2.mtmt.hu/api/publication/31582679}, author = {Giagkou, Nikolaos and Hoeglinger, Guenter U. and Stamelou, Maria}, doi = {10.1016/bs.irn.2019.10.013}, journal-iso = {INT REV NEUROBIOL}, journal = {INTERNATIONAL REVIEW OF NEUROBIOLOGY}, volume = {149}, unique-id = {31582679}, issn = {0074-7742}, abstract = {Progressive supranuclear palsy (PSP) is a neurodegenerative disease characterized pathologically by 4 repeat tau deposition in various cell types and anatomical regions. Richardson's syndrome (RS) is the initially described and one of the clinical phenotypes associated with PSP pathology, characterized by vertical supranuclear gaze paly in particular downwards, postural instability with early falls and subcortical frontal dementia. PSP can manifest as several other clinical phenotypes, including PSP-parkinsonism, -pure akinesia with gait freezing, -frontotemporal dementia, - corticobasal syndrome, speech/language impairment. RS can also have a pathologic diagnosis other than PSP, including corticobasal degeneration, FTD-TDP-43 and others. New clinical diagnostic criteria take into account this phenotypic variability in an attempt to diagnose the disease earlier, given the current lack of a validated biomarker. At present, therapeutic options for PSP are symptomatic and insufficient. Recent large neuroprotective trials have failed to provide a positive clinical outcome, however, have led to the design of better studies that are ongoing and hold promise for a neuroprotective treatment for PSP.}, year = {2019}, eissn = {2162-5514}, pages = {49-86} } @article{MTMT:35515638, title = {Increased Signal in the Superior Cerebellar Peduncle of Patients with Progressive Supranuclear Palsy}, url = {https://m2.mtmt.hu/api/publication/35515638}, author = {Kataoka, Hiroshi and Nishimori, Yukako and Kiriyama, Takao and Nanaura, Hitoki and Izumi, Tesseki and Eura, Nobuyuki and Iwasa, Naoki and Sugie, Kazuma}, doi = {10.14802/jmd.19002}, journal-iso = {J MOV DISORD}, journal = {JOURNAL OF MOVEMENT DISORDERS}, volume = {12}, unique-id = {35515638}, issn = {2005-940X}, keywords = {ATROPHY; Magnetic Resonance Imaging; PROGRESSIVE SUPRANUCLEAR PALSY; NEURODEGENERATIVE DISEASE; superior cerebellar peduncle; FLAIR}, year = {2019}, eissn = {2093-4939}, pages = {166-171}, orcid-numbers = {Kiriyama, Takao/0000-0002-2282-0849} } @article{MTMT:35513365, title = {Quantitative susceptibility mapping in atypical Parkinsonisms}, url = {https://m2.mtmt.hu/api/publication/35513365}, author = {Mazzucchi, Sonia and Frosini, Daniela and Costagli, Mauro and Del Prete, Eleonora and Donatelli, Graziella and Cecchi, Paolo and Migaleddu, Gianmichele and Bonuccelli, Ubaldo and Ceravolo, Roberto and Cosottini, Mirco}, doi = {10.1016/j.nicl.2019.101999}, journal-iso = {NEUROIMAGE-CLIN}, journal = {NEUROIMAGE-CLINICAL}, volume = {24}, unique-id = {35513365}, issn = {2213-1582}, keywords = {PARKINSONISM; SUSCEPTIBILITY; IRON; neurodegeneration}, year = {2019}, eissn = {2213-1582}, orcid-numbers = {Mazzucchi, Sonia/0000-0001-7027-1218; Frosini, Daniela/0000-0002-4049-7424; Costagli, Mauro/0000-0001-9073-1082; Del Prete, Eleonora/0000-0002-4690-1387; Donatelli, Graziella/0000-0002-5325-0746; Cecchi, Paolo/0000-0003-2725-2657; Migaleddu, Gianmichele/0000-0002-0380-9927} } @article{MTMT:35511144, title = {Frontrunner in Translation: Progressive Supranuclear Palsy}, url = {https://m2.mtmt.hu/api/publication/35511144}, author = {Shoeibi, Ali and Olfati, Nahid and Litvan, Irene}, doi = {10.3389/fneur.2019.01125}, journal-iso = {FRONT NEUR}, journal = {FRONTIERS IN NEUROLOGY}, volume = {10}, unique-id = {35511144}, keywords = {EPIDEMIOLOGY; biomarker; Translational research; Etiopathogenesis; PROGRESSIVE SUPRANUCLEAR PALSY; tauopathy}, year = {2019}, eissn = {1664-2295} } @article{MTMT:35515639, title = {A critique of the second consensus criteria for multiple system atrophy}, url = {https://m2.mtmt.hu/api/publication/35515639}, author = {Stankovic, Iva and Quinn, Niall and Vignatelli, Luca and Antonini, Angelo and Berg, Daniela and Coon, Elizabeth and Cortelli, Pietro and Fanciulli, Alessandra and Ferreira, Joaquim J. and Freeman, Roy and Halliday, Glenda and Hoglinger, Gunter U. and Iodice, Valeria and Kaufmann, Horacio and Klockgether, Thomas and Kostic, Vladimir and Krismer, Florian and Lang, Anthony and Levin, Johannes and Low, Phillip and Mathias, Christopher and Meissner, Wassillios G. and Kaufmann, Lucy Norcliffe and Palma, Jose-Alberto and Panicker, Jalesh N. and Pellecchia, Maria Teresa and Sakakibara, Ryuji and Schmahmann, Jeremy and Scholz, Sonja W. and Singer, Wolfgang and Stamelou, Maria and Tolosa, Eduardo and Tsuji, Shoji and Seppi, Klaus and Poewe, Werner and Wenning, Gregor K.}, doi = {10.1002/mds.27701}, journal-iso = {MOVEMENT DISORD}, journal = {MOVEMENT DISORDERS}, volume = {34}, unique-id = {35515639}, issn = {0885-3185}, year = {2019}, eissn = {1531-8257}, pages = {975-984}, orcid-numbers = {Vignatelli, Luca/0000-0002-9051-7091; Antonini, Angelo/0000-0003-1040-2807; Berg, Daniela/0000-0001-5796-5442; Kaufmann, Horacio/0000-0002-1851-9981; Krismer, Florian/0000-0002-4493-5073; Levin, Johannes/0000-0001-5092-4306; Palma, Jose-Alberto/0000-0002-1345-6774; Sakakibara, Ryuji/0000-0002-5803-169X; Seppi, Klaus/0000-0001-6503-1455} } @article{MTMT:35515641, title = {Similarities and differences in cerebellar grey matter volume and disrupted functional connectivity in idiopathic Parkinson's disease and multiple system atrophy}, url = {https://m2.mtmt.hu/api/publication/35515641}, author = {Wang, Na and Zhang, Liang and Yang, HuaGuang and Liu, Hu and Luo, XiaoGuang and Fan, GuoGuang}, doi = {10.1016/j.neuropsychologia.2018.12.019}, journal-iso = {NEUROPSYCHOLOGIA}, journal = {NEUROPSYCHOLOGIA}, volume = {124}, unique-id = {35515641}, issn = {0028-3932}, keywords = {CEREBELLUM; dentate nucleus; functional connectivity; grey matter volume}, year = {2019}, eissn = {1873-3514}, pages = {125-132} } @article{MTMT:35515640, title = {Use of Magnetic Resonance Imaging and Artificial Intelligence in Studies of Diagnosis of Parkinson's Disease}, url = {https://m2.mtmt.hu/api/publication/35515640}, author = {Xu, Jingjing and Zhang, Minming}, doi = {10.1021/acschemneuro.9b00207}, journal-iso = {ACS CHEM NEUROSCI}, journal = {ACS CHEMICAL NEUROSCIENCE}, volume = {10}, unique-id = {35515640}, issn = {1948-7193}, keywords = {Artificial intelligence; Magnetic Resonance Imaging; machine learning; Parkinson's disease}, year = {2019}, eissn = {1948-7193}, pages = {2658-+} } @article{MTMT:35515648, title = {Progressive Supranuclear Palsy: an Update}, url = {https://m2.mtmt.hu/api/publication/35515648}, author = {Armstrong, Melissa J.}, doi = {10.1007/s11910-018-0819-5}, journal-iso = {CURR NEUROL NEUROSCI}, journal = {CURRENT NEUROLOGY AND NEUROSCIENCE REPORTS}, volume = {18}, unique-id = {35515648}, issn = {1528-4042}, keywords = {PROGRESSIVE SUPRANUCLEAR PALSY; clinical diagnostic criteria; Diagnostic imaging Progressive supranuclear palsy/therapy}, year = {2018}, eissn = {1534-6293} } @article{MTMT:35514027, title = {Specificity and sensitivity of magnetic resonance imaging findings in the diagnosis of progressive supranuclear palsy}, url = {https://m2.mtmt.hu/api/publication/35514027}, author = {Bacchi, Stephen and Chim, Ivana and Patel, Sandy}, doi = {10.1111/1754-9485.12613}, journal-iso = {J MED IMAG RADIAT ON}, journal = {JOURNAL OF MEDICAL IMAGING AND RADIATION ONCOLOGY}, volume = {62}, unique-id = {35514027}, issn = {1754-9477}, keywords = {Magnetic Resonance Imaging; Parkinsonian Disorders; PROGRESSIVE SUPRANUCLEAR PALSY; support vector machine; Multiple system atrophy}, year = {2018}, eissn = {1754-9485}, pages = {21-31}, orcid-numbers = {Bacchi, Stephen/0000-0001-5130-8628} } @article{MTMT:35511657, title = {Present and Future of Ultra-High Field MRI in Neurodegenerative Disorders}, url = {https://m2.mtmt.hu/api/publication/35511657}, author = {Donatelli, Graziella and Ceravolo, Roberto and Frosini, Daniela and Tosetti, Michela and Bonuccelli, Ubaldo and Cosottini, Mirco}, doi = {10.1007/s11910-018-0841-7}, journal-iso = {CURR NEUROL NEUROSCI}, journal = {CURRENT NEUROLOGY AND NEUROSCIENCE REPORTS}, volume = {18}, unique-id = {35511657}, issn = {1528-4042}, keywords = {Parkinson's disease; Alzheimer's disease; amyotrophic lateral sclerosis; 7 T MRI; Ultra-high field MR}, year = {2018}, eissn = {1534-6293}, orcid-numbers = {Donatelli, Graziella/0000-0002-5325-0746; Frosini, Daniela/0000-0002-4049-7424; Tosetti, Michela/0000-0002-2515-7560} } @article{MTMT:32483776, title = {Structural Imaging in Atypical Parkinsonism}, url = {https://m2.mtmt.hu/api/publication/32483776}, author = {Heim, Beatrice and Krismer, Florian and Seppi, Klaus}, doi = {10.1016/bs.irn.2018.08.010}, journal-iso = {INT REV NEUROBIOL}, journal = {INTERNATIONAL REVIEW OF NEUROBIOLOGY}, volume = {142}, unique-id = {32483776}, issn = {0074-7742}, abstract = {Qualitative and quantitative structural magnetic resonance imaging offer objective measures of the underlying neurodegeneration in atypical parkinsonism. Regional changes in tissue volume, signal changes and increased deposition of iron as assessed with different structural MRI techniques are surrogate markers of underlying neurodegeneration and may reflect cell loss, microglial proliferation and astroglial activation. Structural MRI has been explored as a tool to enhance diagnostic accuracy in differentiating atypical parkinsonian disorders (APDs). Moreover, the longitudinal assessment of serial structural MRI-derived parameters offers the opportunity for robust inferences regarding the progression of APDs. This review summarizes recent research findings as (1) a diagnostic tool for APDs as well as (2) as a tool to assess longitudinal changes of serial MRI-derived parameters in the different APDs.}, year = {2018}, eissn = {2162-5514}, pages = {67-148}, orcid-numbers = {Heim, Beatrice/0000-0002-6749-3044; Krismer, Florian/0000-0002-4493-5073; Seppi, Klaus/0000-0001-6503-1455} } @article{MTMT:27525276, title = {Possible multiple system atrophy with predominant parkinsonism in a patient with chronic schizophrenia: a case report}, url = {https://m2.mtmt.hu/api/publication/27525276}, author = {Komatsu, Hiroshi and Kato, Masaaki and Kinpara, Teiko and Ono, Takashi and Kakuto, Yoshihisa}, doi = {10.1186/s12888-018-1714-y}, journal-iso = {BMC PSYCHIATRY}, journal = {BMC PSYCHIATRY}, volume = {18}, unique-id = {27525276}, issn = {1471-244X}, year = {2018}, eissn = {1471-244X} } @article{MTMT:35515644, title = {Progressive supranuclear palsy and idiopathic Parkinson's disease are associated with local reduction of in vivo brain viscoelasticity}, url = {https://m2.mtmt.hu/api/publication/35515644}, author = {Lipp, Axel and Skowronek, Cornelia and Fehlner, Andreas and Streitberger, Kaspar-Josche and Braun, Juergen and Sack, Ingolf}, doi = {10.1007/s00330-017-5269-y}, journal-iso = {EUR RADIOL}, journal = {EUROPEAN RADIOLOGY}, volume = {28}, unique-id = {35515644}, issn = {0938-7994}, keywords = {Magnetic resonance elastography; MRE. Parkinson's disease. Progressive supranuclear palsy. Tau protein. Mesencephalon}, year = {2018}, eissn = {1432-1084}, pages = {3347-3354}, orcid-numbers = {Skowronek, Cornelia/0000-0002-7408-9322} } @article{MTMT:35515645, title = {The diagnostic accuracy of the hummingbird and morning glory sign in patients with neurodegenerative parkinsonism}, url = {https://m2.mtmt.hu/api/publication/35515645}, author = {Mueller, Christoph and Hussl, Anna and Krismer, Florian and Heim, Beatrice and Mahlknecht, Philipp and Nocker, Michael and Scherfler, Christoph and Mair, Katherina and Esterhammer, Regina and Schocke, Michael and Wenning, Gregor K. and Poewe, Werner and Seppi, Klaus}, doi = {10.1016/j.parkreldis.2018.04.005}, journal-iso = {PARKINSONISM RELAT D}, journal = {PARKINSONISM AND RELATED DISORDERS}, volume = {54}, unique-id = {35515645}, issn = {1353-8020}, keywords = {PARKINSONISM; Parkinson's disease; Multiple system atrophy; MR parkinsonism index; midbrain-to-pontine area ratio; hummingbird sign; Progressive supranudear palsy}, year = {2018}, eissn = {1873-5126}, pages = {90-94}, orcid-numbers = {Krismer, Florian/0000-0002-4493-5073; Heim, Beatrice/0000-0002-6749-3044; Mahlknecht, Philipp/0000-0003-0671-0516; Scherfler, Christoph/0000-0002-4885-5265; Seppi, Klaus/0000-0001-6503-1455} } @article{MTMT:35515647, title = {Role of Neuroimaging on Differentiation of Parkinson's Disease and Its Related Diseases}, url = {https://m2.mtmt.hu/api/publication/35515647}, author = {Ogawa, Toshihide and Fujii, Shinya and Kuya, Keita and Kitao, Shin-ichiro and Shinohara, Yuki and Ishibashi, Mana and Tanabe, Yoshio}, doi = {10.33160/yam.2018.09.001}, journal-iso = {YONAGO ACTA MED}, journal = {YONAGO ACTA MEDICA}, volume = {61}, unique-id = {35515647}, issn = {0513-5710}, keywords = {Parkinson's disease; Atypical parkinsonian syndrome; I-123-FP-CIT dopamine transporter imaging; I-123-metaiodobenzylguanidine myocardial scintigraphy; neuromelanin-sensitive MR imaging}, year = {2018}, eissn = {1346-8049}, pages = {145-155}, orcid-numbers = {Shinohara, Yuki/0000-0001-6586-4086} } @article{MTMT:35515643, title = {A new MR imaging index for differentiation of progressive supranuclear palsy-parkinsonism from Parkinson's disease}, url = {https://m2.mtmt.hu/api/publication/35515643}, author = {Quattrone, Aldo and Morelli, Maurizio and Nigro, Salvatore and Quattrone, Andrea and Vescio, Basilio and Arabia, Gennarina and Nicoletti, Giuseppe and Nistico, Rita and Salsone, Maria and Novellino, Fabiana and Barbagallo, Gaetano and Le Piane, Emilio and Pugliese, Pierfrancesco and Bosco, Domenico and Vaccaro, Maria Grazia and Chiriaco, Carmelina and Sabatini, Umberto and Vescio, Virginia and Stana, Carlo and Rocca, Federico and Gulla, Domenico and Caracciolo, Manuela}, doi = {10.1016/j.parkreldis.2018.07.016}, journal-iso = {PARKINSONISM RELAT D}, journal = {PARKINSONISM AND RELATED DISORDERS}, volume = {54}, unique-id = {35515643}, issn = {1353-8020}, keywords = {Third ventricle; Progressive supranuclear palsy-parkinsonism; magnetic resonance parkinsonism index; Magnetic resonance parkinsonism index 2.0; Pons area-midbrain area ratio}, year = {2018}, eissn = {1873-5126}, pages = {3-8}, orcid-numbers = {Nigro, Salvatore/0000-0001-7566-7983; Quattrone, Andrea/0000-0003-2071-2083; Vescio, Basilio/0000-0002-7172-8520; Barbagallo, Gaetano/0000-0002-2237-3006} } @article{MTMT:30540237, title = {Clinical and Imaging Features of Multiple System Atrophy: Challenges for an Early and Clinically Definitive Diagnosis}, url = {https://m2.mtmt.hu/api/publication/30540237}, author = {Watanabe, Hirohisa and Riku, Yuichi and Hara, Kazuhiro and Kawabata, Kazuya and Nakamura, Tomohiko and Ito, Mizuki and Hirayama, Masaaki and Yoshida, Mari and Katsuno, Masahisa and Sobue, Gen}, doi = {10.14802/jmd.18020}, journal-iso = {J MOV DISORD}, journal = {JOURNAL OF MOVEMENT DISORDERS}, volume = {11}, unique-id = {30540237}, issn = {2005-940X}, abstract = {Multiple system atrophy (MSA) is an adult-onset, progressive neurodegenerative disorder. Patients with MSA show various phenotypes during the course of their illness, including parkinsonism, cerebellar ataxia, autonomic failure, and pyramidal signs. Patients with MSA sometimes present with isolated autonomic failure or motor symptoms/signs. The median duration from onset to the concomitant appearance of motor and autonomic symptoms is approximately 2 years but can range up to 14 years. As the presence of both motor and autonomic symptoms is essential for the current diagnostic criteria, early diagnosis is difficult when patients present with isolated autonomic failure or motor symptoms/signs. In contrast, patients with MSA may show severe autonomic failure and die before the presentation of motor symptoms/signs, which are currently required for the diagnosis of MSA. Recent studies have also revealed that patients with MSA may show nonsupporting features of MSA such as dementia, hallucinations, and vertical gaze palsy. To establish early diagnostic criteria and clinically definitive categorization for the successful development of disease-modifying therapy or symptomatic interventions for MSA, research should focus on the isolated phase and atypical symptoms to develop specific clinical, imaging, and fluid biomarkers that satisfy the requirements for objectivity, for semi- or quantitative measurements, and for uncomplicated, worldwide availability. Several novel techniques, such as automated compartmentalization of the brain into multiple parcels for the quantification of gray and white matter volumes on an individual basis and the visualization of alpha-synuclein and other candidate serum and cerebrospinal fluid biomarkers, may be promising for the early and clinically definitive diagnosis of MSA.}, keywords = {Early Diagnosis; biomarker; Diagnostic criteria; Atypical symptom}, year = {2018}, eissn = {2093-4939}, pages = {107-120}, orcid-numbers = {Hirayama, Masaaki/0000-0003-0092-7837} } @article{MTMT:35515642, title = {INTRODUCTION}, url = {https://m2.mtmt.hu/api/publication/35515642}, journal-iso = {CURR OPIN NEUROL}, journal = {CURRENT OPINION IN NEUROLOGY}, volume = {31}, unique-id = {35515642}, issn = {1350-7540}, year = {2018}, eissn = {1473-6551}, pages = {2-24} } @article{MTMT:30641119, title = {Advances in progressive supranuclear palsy: new diagnostic criteria, biomarkers, and therapeutic approaches}, url = {https://m2.mtmt.hu/api/publication/30641119}, author = {Boxer, Adam L. and Yu, Jin-Tai and Golbe, Lawrence I. and Litvan, Irene and Lang, Anthony E. and Hoeglinger, Guenter U.}, doi = {10.1016/S1474-4422(17)30157-6}, journal-iso = {LANCET NEUROL}, journal = {LANCET NEUROLOGY}, volume = {16}, unique-id = {30641119}, issn = {1474-4422}, abstract = {Progressive supranuclear palsy (PSP), previously believed to be a common cause of atypical parkinsonism, is now recognised as a range of motor and behavioural syndromes that are associated with a characteristic 4-repeat tau neuropathology. New research criteria that recognise early presentations of PSP and operationalise diagnosis of the full spectrum of clinical phenotypes have been reported. The Movement Disorders Society PSP diagnostic criteria include syndromes with few or mild symptoms that are suggestive of underlying PSP pathology and could provide an opportunity for earlier therapeutic interventions in the future. These criteria also include definitions for variant PSP syndromes with different patterns of movement, language, or behavioural features than have been conclusively associated with PSP pathology. Data from new diagnostic biomarkers can be combined with the clinical features of disease to increase the specificity of the new criteria for underlying PSP pathology. Because PSP is associated with tau protein abnormalities, there is growing interest in clinical trials of new tau-directed therapies. These therapies are hypothesised to have disease-modifying effects by reducing the concentration of toxic forms of tau in the brain or by compensating for loss of tau function. Since tau pathology is also central to Alzheimer's disease and chronic traumatic encephalopathy, a successful tau therapeutic for PSP might inform treatment of other neurodegenerative diseases.}, keywords = {ALZHEIMERS-DISEASE; DOUBLE-BLIND; POSITRON-EMISSION-TOMOGRAPHY; Corticobasal degeneration; FRONTOTEMPORAL LOBAR DEGENERATION; NEUROFILAMENT LIGHT-CHAIN; BEHAVIORAL VARIANT; atypical parkinsonian syndromes; PSP-PARKINSONISM}, year = {2017}, eissn = {1474-4465}, pages = {552-563}, orcid-numbers = {Yu, Jin-Tai/0000-0002-7686-0547; Litvan, Irene/0000-0002-3485-3445; Lang, Anthony E./0000-0003-1229-3667} } @article{MTMT:35510831, title = {Positron emission tomography imaging of tau pathology in progressive supranuclear palsy}, url = {https://m2.mtmt.hu/api/publication/35510831}, author = {Coakeley, Sarah and Cho, Sang Soo and Koshimori, Yuko and Rusjan, Pablo and Harris, Madeleine and Ghadery, Christine and Kim, Jinhee and Lang, Anthony E. and Wilson, Alan and Houle, Sylvain and Strafella, Antonio P.}, doi = {10.1177/0271678X16683695}, journal-iso = {J CEREBR BLOOD F MET}, journal = {JOURNAL OF CEREBRAL BLOOD FLOW AND METABOLISM}, volume = {37}, unique-id = {35510831}, issn = {0271-678X}, keywords = {NEUROPATHOLOGY; Parkinson's disease; positron emission tomography; Brain imaging; Movement disorder}, year = {2017}, eissn = {1559-7016}, pages = {3150-3160}, orcid-numbers = {Rusjan, Pablo/0000-0003-0075-2918; Kim, Jinhee/0000-0002-1019-7455; Houle, Sylvain/0000-0002-4231-6316} } @article{MTMT:26922940, title = {Emerging Diagnostic and Therapeutic Strategies for Tauopathies}, url = {https://m2.mtmt.hu/api/publication/26922940}, author = {Coughlin, David and Irwin, David J}, doi = {10.1007/s11910-017-0779-1}, journal-iso = {CURR NEUROL NEUROSCI}, journal = {CURRENT NEUROLOGY AND NEUROSCIENCE REPORTS}, volume = {17}, unique-id = {26922940}, issn = {1528-4042}, year = {2017}, eissn = {1534-6293} } @article{MTMT:35514033, title = {Combined Diffusion Tensor Imaging and Apparent Transverse Relaxation Rate Differentiate Parkinson Disease and Atypical Parkinsonism}, url = {https://m2.mtmt.hu/api/publication/35514033}, author = {Du, G. and Lewis, M. M. and Kanekar, S. and Sterling, N. W. and He, L. and Kong, L. and Li, R. and Huang, X.}, doi = {10.3174/ajnr.A5136}, journal-iso = {AM J NEURORADIOL}, journal = {AMERICAN JOURNAL OF NEURORADIOLOGY}, volume = {38}, unique-id = {35514033}, issn = {0195-6108}, year = {2017}, eissn = {1936-959X}, pages = {966-972} } @article{MTMT:32483781, title = {Magnetic resonance imaging for the diagnosis of Parkinson's disease}, url = {https://m2.mtmt.hu/api/publication/32483781}, author = {Heim, Beatrice and Krismer, Florian and De Marzi, Roberto and Seppi, Klaus}, doi = {10.1007/s00702-017-1717-8}, journal-iso = {J NEURAL TRANSM}, journal = {JOURNAL OF NEURAL TRANSMISSION}, volume = {124}, unique-id = {32483781}, issn = {0300-9564}, abstract = {The differential diagnosis of parkinsonian syndromes is considered one of the most challenging in neurology and error rates in the clinical diagnosis can be high even at specialized centres. Despite several limitations, magnetic resonance imaging (MRI) has undoubtedly enhanced the diagnostic accuracy in the differential diagnosis of neurodegenerative parkinsonism over the last three decades. This review aims to summarize research findings regarding the value of the different MRI techniques, including advanced sequences at high- and ultra-high-field MRI and modern image analysis algorithms, in the diagnostic work-up of Parkinson's disease. This includes not only the exclusion of alternative diagnoses for Parkinson's disease such as symptomatic parkinsonism and atypical parkinsonism, but also the diagnosis of early, new onset, and even prodromal Parkinson's disease.}, keywords = {Parkinson's disease; PROGRESSIVE SUPRANUCLEAR PALSY; Multiple system atrophy; Atypical parkinsonism; MRT}, year = {2017}, eissn = {1435-1463}, pages = {915-964}, orcid-numbers = {Heim, Beatrice/0000-0002-6749-3044; Krismer, Florian/0000-0002-4493-5073; Seppi, Klaus/0000-0001-6503-1455} } @article{MTMT:35514030, title = {Differential diagnosis of parkinsonism by a combined use of diffusion kurtosis imaging and quantitative susceptibility mapping}, url = {https://m2.mtmt.hu/api/publication/35514030}, author = {Ito, Kenji and Ohtsuka, Chigumi and Yoshioka, Kunihiro and Kameda, Hiroyuki and Yokosawa, Suguru and Sato, Ryota and Terayama, Yasuo and Sasaki, Makoto}, doi = {10.1007/s00234-017-1870-7}, journal-iso = {NEURORADIOLOGY}, journal = {NEURORADIOLOGY}, volume = {59}, unique-id = {35514030}, issn = {0028-3940}, keywords = {Parkinson's disease; Multiple system atrophy; Diffusion kurtosis imaging; Quantitative susceptibility mapping; progressive supranuclear palsy syndrome}, year = {2017}, eissn = {1432-1920}, pages = {759-769}, orcid-numbers = {Sato, Ryota/0000-0002-3418-2987} } @article{MTMT:30412607, title = {Potential clinical utility of multiple system atrophy biomarkers}, url = {https://m2.mtmt.hu/api/publication/30412607}, author = {Jellinger, Kurt A.}, doi = {10.1080/14737175.2017.1392239}, journal-iso = {EXPERT REV NEUROTHER}, journal = {EXPERT REVIEW OF NEUROTHERAPEUTICS}, volume = {17}, unique-id = {30412607}, issn = {1473-7175}, abstract = {Introduction: Multiple system atrophy (MSA), an adult-onset, fatal disorder of uncertain etiology, characterized by parkinsonism, cerebellar, autonomic and motor dysfunctions, is an -synucleinopathy with glioneuronal degeneration involving multiple parts of the nervous system. The clinical variants correlate with the morphological phenotypes of striatonigral degeneration (MSA-P), olivoponto-cerebellar atrophy (MSA-C), and mixed type MSA. Neuropathological hallmark is the deposition of aberrant -synuclein in glia and neurons forming cytoplasmic inclusions that cause cell dysfunction/demise.Areas covered: While our knowledge of the pathogenesis of this proteinopathy is still incomplete, updated consensus criteria and combined biomarkers have increased diagnostic accuracy. Multimodal imaging of structural and functional brain changes gives insight into the pathophysiology and may evaluate disease progression. Currently, the most useful CSF biomarkers are a combination of light chain neurofilament (elevated in MSA), catecholaminergic metabolites, and proteins (-synuclein, DJ-1, tau). Several blood substances (neurofilament light chain, microRNAs) are non-invasive biomarkers.Expert commentary: Recent studies suggest that the combination of neuroimaging and fluid biomarkers may be more successful than using single markers to increase the accuracy of the clinical (differential) diagnosis of MSA. Multidisciplinary research to develop more reliable markers for a more exact and early diagnosis and targets for effective treatment are urgently needed.}, year = {2017}, eissn = {1744-8360}, pages = {1189-1208} } @article{MTMT:30700626, title = {Current Understanding of Neurodegenerative Diseases Associated With the Protein Tau}, url = {https://m2.mtmt.hu/api/publication/30700626}, author = {Josephs, Keith A.}, doi = {10.1016/j.mayocp.2017.04.016}, journal-iso = {MAYO CLIN PROC}, journal = {MAYO CLINIC PROCEEDINGS}, volume = {92}, unique-id = {30700626}, issn = {0025-6196}, abstract = {Primary tauopathies are a group of neurodegenerative diseases in which tau is believed to be the major contributing factor of the neurodegenerative process. In primary tauopathies, there is a disassociation between tau (a microtubule-associated protein) and microtubules as a result of tau hyperphosphorylation. This disassociation between tau and microtubules results in tau fibrillization and inclusion formation as well as in microtubule dysfunction. There are different clinical syndromes associated with different primary tauopathies, and some clinical syndromes can be associated with multiple primary tauopathies. Hence, although some clinical syndromes are highly specific and almost diagnostic of a primary tauopathy, many are not, making it difficult to diagnose a primary tauopathy. Recently, radioligands that bind to tau and can be combined with positron emission tomography to detect fibrillary tau antemortem have been developed, although preliminary data suggest that these ligands may not be sensitive in detecting tau associated with many primary tauopathies. Another recent advancement in the field is evidence suggesting that tau may exhibit properties similar to those of prions, although infective transmission has not been shown. There have been a few clinical trials targeting tau and microtubule dysfunction, although none have had any disease-modifying effects. Understanding tau biology is critical to the development of pharmacological agents that could have disease-modifying effects on primary tauopathies. (C) 2017 Mayo Foundation for Medical Education and Research}, keywords = {ALZHEIMERS-DISEASE; DIAGNOSTIC-CRITERIA; Microtubule; POSITRON-EMISSION-TOMOGRAPHY; PROGRESSIVE SUPRANUCLEAR PALSY; Corticobasal degeneration; superior cerebellar peduncle; tauopathy; MIDBRAIN ATROPHY}, year = {2017}, eissn = {1942-5546}, pages = {1291-1303} } @article{MTMT:35515646, title = {Role of Magnetic Resonance Imaging in the Diagnosis of Multiple System Atrophy}, url = {https://m2.mtmt.hu/api/publication/35515646}, author = {Kim, Han-Joon and Jeon, Beomseok and Fung, Victor S. C.}, doi = {10.1002/mdc3.12404}, journal-iso = {MOV DISORD CLIN PRAC}, journal = {MOVEMENT DISORDERS CLINICAL PRACTICE}, volume = {4}, unique-id = {35515646}, issn = {2330-1619}, keywords = {diagnosis; Differential diagnosis; Magnetic resonance imaging (MRI); Progression; Multiple system atrophy}, year = {2017}, eissn = {2330-1619}, pages = {12-20}, orcid-numbers = {Kim, Han-Joon/0000-0001-8219-9663} } @article{MTMT:35461603, title = {9.4 T MR microscopy of the substantia nigra with pathological validation in controls and disease}, url = {https://m2.mtmt.hu/api/publication/35461603}, author = {Massey, L.A. and Miranda, M.A. and Al-Helli, O. and Parkes, H.G. and Thornton, J.S. and So, P.-W. and White, M.J. and Mancini, L. and Strand, C. and Holton, J. and Lees, A.J. and Révész, Tamás and Yousry, T.A.}, doi = {10.1016/j.nicl.2016.11.015}, journal-iso = {NEUROIMAGE-CLIN}, journal = {NEUROIMAGE-CLINICAL}, volume = {13}, unique-id = {35461603}, issn = {2213-1582}, abstract = {Background The anatomy of the substantia nigra on conventional MRI is controversial. Even using histological techniques it is difficult to delineate with certainty from surrounding structures. We sought to define the anatomy of the SN using high field spin-echo MRI of pathological material in which we could study the anatomy in detail to corroborate our MRI findings in controls and Parkinson's disease and progressive supranuclear palsy. Methods 23 brains were selected from the Queen Square Brain Bank (10 controls, 8 progressive supranuclear palsy, 5 Parkinson's disease) and imaged using high field 9.4 Tesla spin-echo MRI. Subsequently brains were cut and stained with Luxol fast blue, Perls stain, and immunohistochemistry for substance P and calbindin. Once the anatomy was defined on histology the dimensions and volume of the substantia nigra were determined on high field magnetic resonance images. Results The anterior border of the substantia nigra was defined by the crus cerebri. In the medial half it was less distinct due to the deposition of iron and the interdigitation of white matter and the substantia nigra. The posterior border was flanked by white matter bridging the red nucleus and substantia nigra and seen as hypointense on spin-echo magnetic resonance images. Within the substantia nigra high signal structures corresponded to confirmed nigrosomes. These were still evident in Parkinson's disease but not in progressive supranuclear palsy. The volume and dimensions of the substantia nigra were similar in Parkinson's disease and controls, but reduced in progressive supranuclear palsy. Conclusions We present a histologically validated anatomical description of the substantia nigra on high field spin-echo high resolution magnetic resonance images and were able to delineate all five nigrosomes. In accordance with the pathological literature we did not observe changes in the nigrosome structure as manifest by volume or signal characteristics within the substantia nigra in Parkinson's disease whereas in progressive supranuclear palsy there was microarchitectural destruction. © 2016 The Authors}, keywords = {Aged; Aged; Adult; Adult; Female; Female; Middle Aged; Middle Aged; Male; Male; Humans; NEUROPATHOLOGY; immunohistochemistry; ARTICLE; human; Aged, 80 and over; priority journal; controlled study; pathology; nuclear magnetic resonance imaging; nuclear magnetic resonance imaging; Magnetic Resonance Imaging; clinical article; Aging; Aging; substantia nigra; substantia nigra; substantia nigra; Young Adult; Diagnostic Imaging; validation study; Tissue Banks; Parkinson disease; brain disease; calbindin; substance P; white matter; disease control; red nucleus; neuroanatomy; Brain Diseases; PROGRESSIVE SUPRANUCLEAR PALSY; very elderly; procedures; brain histology; tissue bank; Fast blue; cerebral crus}, year = {2017}, eissn = {2213-1582}, pages = {154-163} } @article{MTMT:32483784, title = {Manual MRI Morphometry in Parkinsonian Syndromes}, url = {https://m2.mtmt.hu/api/publication/32483784}, author = {Moeller, Leona and Kassubek, Jan and Suedmeyer, Martin and Hilker, Ruediger and Hattingen, Elke and Egger, Karl and Amtage, Florian and Pinkhardt, Elmar H. and Respondek, Gesine and Stamelou, Maria and Moeller, Franz and Schnitzler, Alfons and Oertel, Wolfgang H. and Knake, Susanne and Huppertz, Hans-Juergen and Hoeglinger, Guenter U.}, doi = {10.1002/mds.26921}, journal-iso = {MOVEMENT DISORD}, journal = {MOVEMENT DISORDERS}, volume = {32}, unique-id = {32483784}, issn = {0885-3185}, abstract = {Background: Several morphometric magnetic resonance imaging parameters may serve for differential diagnosis of parkinsonism. The objective of this study was to identify which performs best in clinical routine.Methods: We acquired multicentric magnetization-prepared rapid gradient echo sequences in patients with Parkinson's disease (n=204), progressive supranuclear palsy (n=106), multiple system atrophy-cerebellar, (n=21); multiple system atrophyparkinsonian (n=60), and healthy controls (n=73), performed manual planimetric measurements, and calculated receiver operator characteristics with leaveone- out cross-validation to propose cutoff values.Results: The midsagittal midbrain area was reduced in PSP versus all other groups (P<0.001). The midsagittal pons area was reduced in MSA-cerebellar, MSA-parkinsonian, and PSP versus PD patients and healthy controls (P<0.001). The midbrain/ pons area ratio was lower in PSP (P<0.001) and higher in MSA-cerebellar and MSA-parkinsonian versus PD and PSP (P<0.001).Conclusions: The midsagittal midbrain area most reliably identified PSP, the midsagittal pons area MSA-cerebellar. The midbrain/ pons area ratio differentiated MSA-cerebellar and PSP better than the magnetic resonance-Parkinson index. (C) 2017 International Parkinson and Movement Disorder Society}, keywords = {morphometry; Magnetic Resonance Imaging; Parkinson's disease; PROGRESSIVE SUPRANUCLEAR PALSY; Multiple system atrophy}, year = {2017}, eissn = {1531-8257}, pages = {778-782}, orcid-numbers = {Kassubek, Jan/0000-0002-7106-9270; Hattingen, Elke/0000-0002-8392-9004; Schnitzler, Alfons/0000-0002-6414-7939; Knake, Susanne/0000-0002-6298-0513} } @article{MTMT:32483779, title = {Role of magnetic resonance planimetry and magnetic resonance parkinsonism index in discriminating Parkinson's disease and progressive supranuclear palsy: a retrospective study based on 1.5 and 3 T MRI}, url = {https://m2.mtmt.hu/api/publication/32483779}, author = {Nizamani, Waseem Mehmood and Mubarak, Fatima and Barakzai, Muhammad Danish and Ahmed, Muhammad Saad}, doi = {10.2147/IJGM.S134297}, journal-iso = {INT J GEN MED}, journal = {INTERNATIONAL JOURNAL OF GENERAL MEDICINE}, volume = {10}, unique-id = {32483779}, abstract = {Objective: The objective of the study was to assess magnetic resonance (MR) planimetric measurements and MR parkinsonism index (MRPI) in differentiating progressive supranuclear palsy (PSP) from Parkinson's disease (PD) using 1.5 and 3 T MRI scanner.Subjects and methods: After ethical approval was obtained, analysis of 34 consecutive patients with PSP, 34 patients with PD and 34 healthy controls (HCs) was performed. HCs were age-matched adults without any history of neurodegenerative disease or movement disorders. Retrospective data from the past 10 years (from January 2006 to December 2015) were obtained from the Hospital Information Management System, and informed consent was obtained from all participants. The measurements of pons area-midbrain area ratio (P/M) and MCP width-superior cerebellar peduncle (SCP) width ratio (MCP/SCP) were used, and MRPI was calculated by the formula ([ P/M] x[ MCP/SCP]).Results: Midbrain area and SCP width in patients with PSP (19 males, 15 females; mean age =66.7 years) were significantly (P<0.001) smaller than in patients with PD (20 males, 14 females; mean age =66.7 years) and control participants (17 males, 17 females; mean age =66.1 years). P/M and MCP/SCP were significantly higher in patients with PSP than in patients with PD and control participants. All measurements showed some overlap of values between patients with PSP and patients from PD group and control participants. MRPI value was significantly higher in patients with PSP (mean 21.00) than in patients with PD (mean 9.50; P<0.001) and control participants (mean 9.6; P<0.001), without any overlap of values among groups. No correlation was found between the duration of disease, PSP rating scale, PSP staging system and MRPI in this study. No patient with PSP received a misdiagnosis when the index was used (sensitivity and specificity, 100%).Conclusion: MRPI should be made an essential part of all MRI brain reporting whenever differentiation between PD and PSP is sought for.}, keywords = {COGNITIVE DYSFUNCTION; Movement Disorders; PROGRESSIVE SUPRANUCLEAR PALSY; Parkinsonism Index}, year = {2017}, eissn = {1178-7074}, pages = {375-384}, orcid-numbers = {Nizamani, Waseem Mehmood/0000-0002-8978-1143} }