Article
Neuroimaging
Rodolfo G. Gatto, Peter R. Martin, Farwa Ali, Heather M. Clark, Joseph R. Duffy, Rene L. Utianski, Hugo Botha, Mary M. Machulda, Dennis W. Dickson, Keith A. Josephs, Jennifer L. Whitwell
Summary: Degeneration of the SCP and DRTT are diagnostic features of both PSP-RS and PSP-SL, and the associations with clinical metrics validate the role of these tracts in PSP-related clinical features, particularly in PSP-SL.
NEUROIMAGE-CLINICAL
(2022)
Article
Medicine, General & Internal
In Jun Han, Hyeok Gyu Kwon, Woong-Woo Lee, Ra Gyoung Yoon, Hyoseon Choi, Hyun Jung Kim
Summary: This paper describes the changes over time in the corticobulbar tract (CBT) using diffusion tensor tractography (DTT) in a dysphagic patient with progressive supranuclear palsy (PSP). The patient initially presented with dysarthria, gait disturbance, and bradykinesia, and later developed dysphagia symptoms. DTI imaging showed progressive thinning of the CBTs. This study suggests that analyzing the CBT using DTT can help predict the degree of dysphagia and prognosis in PSP patients.
Article
Clinical Neurology
Mari Miyata, Shingo Kakeda, Tetsuya Yoneda, Satoru Ide, Kazumasa Okada, Hiroaki Adachi, Yukunori Korogi
Summary: PADRE imaging can enhance myelin density and delineate the SCP. This study aimed to determine if SCP atrophy can be distinguishable on PADRE imaging and evaluate its diagnostic performance compared with previous MRI findings. The results showed that assessing SCP with PADRE imaging can accurately differentiate PSP from PD.
Article
Clinical Neurology
Ryuichi Koizumi, Akio Akagi, Yuichi Riku, Jun Sone, Hiroaki Miyahara, Fumiaki Tanaka, Mari Yoshida, Yasushi Iwasaki
Summary: Progressive supranuclear palsy (PSP) can be diagnosed despite asymmetrical parkinsonism, and atrophy of the superior cerebellar peduncle (SCP) is more frequent in PSP than in Parkinson's disease. This study investigated the laterality of brain lesions, including SCP, in 48 PSP autopsy cases. Asymmetrical SCP atrophy was found in 14.6% of the cases, and the atrophic side of the SCP corresponded to the dominant side of tau pathology in the cerebellar dentate nucleus. This study is the first to report the presence of asymmetrical SCP atrophy and systematically asymmetrical degeneration of the Guillain-Mollaret triangle in PSP cases.
Article
Neurosciences
Jong Hyeon Ahn, Junmo Kwon, Ji Hye Won, Kyoungseob Byeon, Jinyoung Youn, Hyunjin Park, Jin Whan Cho
Summary: This study investigated waiting impulsivity in progressive supranuclear palsy-Richardson's syndrome (PSP-RS) and introduced a novel evaluation method. The results showed that the JTG sign is a surrogate marker of waiting impulsivity in PSP-RS patients, enriching the current understanding of waiting impulsivity in PSP patients.
FRONTIERS IN NEUROSCIENCE
(2023)
Article
Neuroimaging
Irene Sintini, Kenton Kaufman, Hugo Botha, Peter R. Martin, Stacy R. Loushin, Matthew L. Senjem, Robert I. Reid, Christopher G. Schwarz, Clifford R. Jack, Val J. Lowe, Keith A. Josephs, Jennifer L. Whitwell, Farwa Ali
Summary: Progressive supranuclear palsy is a neurodegenerative disorder characterized by tau inclusions and neurodegeneration, commonly presenting with gait impairments and postural instability. Analysis of multimodal imaging biomarkers and laboratory-based measurements can provide valuable insights into the relationship between neurodegeneration and gait/balance abnormalities in this condition.
NEUROIMAGE-CLINICAL
(2021)
Article
Neurosciences
Alexander Rau, Wolfgang H. Jost, Theo Demerath, Elias Kellner, Marco Reisert, Horst Urbach
Summary: This study analyzed diffusion microstructure imaging (DMI) in patients with suspected atypical Parkinson syndromes and healthy controls. The results showed widespread axonal loss in PSP patients, and the ROI-based approach had the highest accuracy in identifying PSP in the thalamus and frontal white matter.
Article
Clinical Neurology
Shweta Prasad, Archith Rajan, Shaik Afsar Pasha, Sandhya Mangalore, Jitender Saini, Madhura Ingalhalikar, Pramod Kumar Pal
Summary: Significant alterations in the structural connectivity of the whole brain connectome were observed in PSP-RS. The higher degree of abnormality observed in nodes belonging to the frontal lobe and basal ganglia substantiates the predominant frontal dysfunction and parkinsonism observed in PSP-RS. The findings of this study support the concept that PSP-RS may be a network-based disorder.
ACTA NEUROLOGICA SCANDINAVICA
(2021)
Article
Geriatrics & Gerontology
Lavinia A. Barlescu, Hans-Peter Mueller, Ingo Uttner, Albert C. Ludolph, Elmar H. Pinkhardt, Hans-Juergen Huppertz, Jan Kassubek
Summary: The study investigated regional white matter alterations in PSP and PD patients compared to healthy controls using T1w MRI and DTI data. Significant changes in entropy and homogeneity were observed in CC areas I, II, and III in both PSP and PD patients, with the highest inhomogeneity in area II in the PSP cohort. Receiver operating characteristics curves showed potential for differentiating between PSP and PD with areas under the curve values of 0.86, 0.72, and 0.69, respectively.
FRONTIERS IN AGING NEUROSCIENCE
(2021)
Article
Clinical Neurology
Tomohiko Imai, Kenichi Sakamoto, Tatsuji Hasegawa, Yoko Shioda, Yoshiyuki Tsutsumi, Satoshi Sakaue, Toshihiko Imamura, Akira Morimoto, Tomoko Iehara
Summary: The study aimed to confirm the hypothesis that brain white matter damage is involved in the pathogenesis and disease progression of Langerhans cell histiocytosis (LCH)-associated neurodegenerative disease (ND) by analyzing pediatric patients with LCH using diffusion tensor imaging (DTI).
Article
Clinical Neurology
Yasuo Terao, Shin-ichi Tokushige, Satomi Inomata-Terada, Hideki Fukuda, Akihiro Yugeta, Yoshikazu Ugawa
Summary: The velocity profile of horizontal saccades can serve as an indicator of brainstem and cerebellar output dysfunction. Altered velocity profiles in progressive supranuclear palsy (PSP) patients suggest pathology in the brainstem and cerebellum. This study's findings are of significance for diagnosis and treatment of PSP patients.
CLINICAL NEUROPHYSIOLOGY
(2022)
Article
Clinical Neurology
Natalie E. Adams, Amirhossein Jafarian, Alistair Perry, Matthew A. Rouse, Alexander D. Shaw, Alexander G. Murley, Thomas E. Cope, W. Richard Bevan-Jones, Luca Passamonti, Duncan Street, Negin Holland, David Nesbitt, Laura E. Hughes, Karl J. Friston, James B. Rowe
Summary: Adams et al. use PET measures of synaptic density to enhance biophysical models of cortical dynamics and demonstrate the link between synaptic loss, neurophysiology, and cognitive deficits. Their inclusion of regional synaptic density in a mesoscale model of human cortical function significantly increases model evidence and predicts individual differences in behavior. This method can be applied to assess the mechanisms of other neurological disorders and test the effects of experimental pharmacology.
Review
Clinical Neurology
Francesca Baschieri, Maria Vitiello, Pietro Cortelli, Giovanna Calandra-Buonaura, Francesca Morgante
Summary: Autonomic dysfunction is frequently observed in patients with PSP, but objective testing does not always reflect actual autonomic impairment.
JOURNAL OF NEUROLOGY
(2023)
Review
Clinical Neurology
Katie A. Peterson, Karalyn Patterson, James B. Rowe
Summary: PSP and CBS can present changes in speech and language alongside or before motor symptoms, making their differential diagnosis challenging in the early stages. Language impairment is often an early and persistent issue in CBS and PSP, highlighting the need for improved language screening and detailed language assessments. Improved language assessment may help in differential diagnosis and inform clinical management decisions.
JOURNAL OF NEUROLOGY
(2021)
Article
Clinical Neurology
Mike Wattjes, Hans-Juergen Huppertz, Nima Mahmoudi, Sophia Stoecklein, Sophia Rogozinski, Florian Wegner, Martin Klietz, Ivayla Apostolova, Johannes Levin, Sabrina Katzdobler, Carsten Buhmann, Andrea Quattrone, Georg Berding, Matthias Brendel, Henryk Barthel, Osama Sabri, Guenter Hoeglinger, Ralph Buchert, Alzheimers Dis Neuroimaging Initiat
Summary: This study compared the value of different MRI reading strategies and automatic classification methods in the diagnosis of PSP. The results showed that the fully automatic classification method using support vector machine performed the best, especially in the diagnosis of vPSP. Therefore, it is recommended to use machine learning methods for fully automatic classification in settings with a broad phenotypic PSP spectrum.
MOVEMENT DISORDERS
(2023)
Article
Clinical Neurology
Michela Leocadi, Elisa Canu, Camilla Cividini, Tommaso Russo, Giordano Cecchetti, Claudia Celico, Rosalinda Cardamone, Valeria Barcella, Giuseppe Magnani, Federica Agosta, Massimo Filippi
Summary: We describe the brain structural damage and cognitive profile evolution of an adult patient with 17q21.31 microduplication, a rare genetic condition associated with psychomotor delay and behavioral disturbances. The patient displayed obsessive and repetitive behaviors, memory loss, and poor social interaction. MRI scans revealed greater gray matter atrophy in the right hemisphere compared to controls. The patient's condition rapidly progressed and led to a diagnosis of early onset dementia. This study highlights the complex interaction between genetic substrate and clinical phenotypes.
JOURNAL OF NEUROLOGY
(2023)
Article
Clinical Neurology
Roberta Messina, Carole H. Sudre, Diana Y. Wei, Massimo Filippi, Sebastien Ourselin, Peter J. Goadsby
Summary: The objective of this study was to identify MRI biomarkers that distinguish between migraine and cluster headache patients, as well as investigate shared imaging features. Clinical, functional, and structural MRI data were collected from 20 migraineurs, 20 cluster headache patients, and 15 healthy controls. Support vector machine algorithms were used to classify headache patients from controls, and regional differences and associations with clinical characteristics were examined. The results showed that MRI could accurately classify headache patients from controls, with accuracies of 80% for all headache patients, 89% for migraine, and 98% for cluster headache. The bilateral hypothalamic and periaqueductal gray functional networks were found to be important in classifying both migraine and cluster headache patients. The presence of restlessness was the most important clinical feature in distinguishing between the two groups.
ANNALS OF NEUROLOGY
(2023)
Review
Clinical Neurology
Massimo Filippi, Edoardo Gioele Spinelli, Camilla Cividini, Alma Ghirelli, Silvia Basaia, Federica Agosta
Summary: By using mathematical models applied to functional resting-state MRI, the brain can be organized into nodes and edges to form the functional brain connectome. This has significant implications for the clinical diagnosis, follow-up, prediction of progression, patient stratification, and treatment response recording in neurodegenerative diseases.
EXPERT REVIEW OF NEUROTHERAPEUTICS
(2023)
Article
Clinical Neurology
Andrea Gardoni, Federica Agosta, Elisabetta Sarasso, Silvia Basaia, Elisa Canu, Michela Leocadi, Veronica Castelnovo, Andrea Tettamanti, Maria Antonietta Volonte, Massimo Filippi
Summary: This study aimed to assess cerebellar atrophy and activity alterations during functional MRI (fMRI) gait-simulating motor- and dual-tasks in Parkinson's disease patients with postural instability and gait disorders (PD-PIGD). The results showed that PD-PIGD patients had reduced volumes of cerebellar motor and non-motor areas and increased activity of cognitive areas during fMRI tasks. These findings suggest that the increased activity of non-motor cerebellar areas may be a compensatory mechanism in response to cerebellar motor dysfunction in PD-PIGD.
JOURNAL OF NEUROLOGY
(2023)
Review
Clinical Neurology
Michela Leocadi, Elisa Canu, Angela Paldino, Federica Agosta, Massimo Filippi
Summary: This review aims to define awareness impairment and related disturbances in neurodegenerative diseases and provides an update on the use of MRI in studying awareness in these disorders. It highlights the importance of awareness impairment in AD and FTLD and discusses the application of MRI in investigating different aspects of awareness in these populations.
JOURNAL OF NEUROLOGY
(2023)
Article
Clinical Neurology
Monica Margoni, Mor Gueye, Alessandro Meani, Elisabetta Pagani, Lucia Moiola, Paolo Preziosa, Massimo Filippi, Maria A. Rocca
Summary: This study found that there is enlargement of the choroid plexus (CP) in pediatric multiple sclerosis (MS) patients, suggesting its early involvement in the pathophysiology of the disease. The higher CP volume, especially in female patients, supports the hypothesis of sex-related differences in pediatric MS.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
(2023)
Article
Biochemistry & Molecular Biology
Alessia Fiore, Paolo Preziosa, Nicolo Tedone, Monica Margoni, Carmen Vizzino, Damiano Mistri, Mor Gueye, Maria A. Rocca, Massimo Filippi
Summary: Gray matter atrophy in multiple sclerosis (MS) progresses non-randomly and may be associated with specific neurotransmitter distributions in the central nervous system. This study investigated the correlations among regional GM atrophy, neurotransmitter maps, and clinical manifestations in a large group of MS patients.
MOLECULAR PSYCHIATRY
(2023)
Article
Clinical Neurology
Giulia Berzero, Valentina Pieri, Pietro Mortini, Massimo Filippi, Gaetano Finocchiaro
Summary: The clinical use of liquid biopsy, specifically the analysis of cell-free DNA from plasma or cerebrospinal fluid, is increasingly important in oncology, particularly for brain tumours. It can provide valuable information for differential diagnosis, patient follow-up, and identifying targetable genetic alterations. However, there are challenges such as concomitant pathologies and invasive procedures to obtain cerebrospinal fluid, which could affect the accuracy and interpretation of the results. Further research and understanding of the mechanisms behind cfDNA shedding by tumours are needed to fully exploit its potential in clinical practice.
Article
Clinical Neurology
Susana Otero-Romero, Christine Lebrun-Frenay, Saul Reyes, Maria Pia Amato, Magda Campins, Mauricio Farez, Massimo Filippi, Yael Hacohen, Bernhard Hemmer, Rosa Juuti, Melinda Magyari, Celia Oreja-Guevara, Aksel Siva, Sandra Vukusic, Mar Tintore
Summary: This study developed a European consensus on vaccination strategies for people with multiple sclerosis (pwMS) who are candidates for disease-modifying therapies (DMTs). A multidisciplinary working group used formal consensus methodology to formulate recommendations based on the quality of evidence and risk-benefit balance. A total of 53 recommendations were agreed upon, aiming to homogenize immunization practices in pwMS.
EUROPEAN JOURNAL OF NEUROLOGY
(2023)
Article
Clinical Neurology
Damiano Mistri, Laura Cacciaguerra, Paola Valsasina, Elisabetta Pagani, Massimo Filippi, Maria A. Rocca
Summary: This study compared cognitive performance between primary progressive and secondary progressive multiple sclerosis (MS) patients, and investigated the structural and functional magnetic resonance imaging (MRI) correlates of their cognitive functions. The results showed that primary progressive and secondary progressive MS had similar cognitive scores in all domains, and cognitive dysfunction was associated with distinct patterns of brain structural abnormalities and involvement of different white matter tracts.
EUROPEAN JOURNAL OF NEUROLOGY
(2023)
Article
Clinical Neurology
Chiara Zanetta, Maria A. Rocca, Alessandro Meani, Vittorio Martinelli, Laura Ferre, Lucia Moiola, Massimo Filippi
Summary: This study investigated the effectiveness and safety of cladribine in RRMS patients. The results showed that cladribine reduced relapses and MRI activity, and stabilized disability. Patients who switched from first-line therapies or were naïve had better outcomes.
JOURNAL OF NEUROLOGY
(2023)
Letter
Clinical Neurology
Monica Margoni, Paolo Preziosa, Loredana Storelli, Mor Gueye, Lucia Moiola, Massimo Filippi, Maria A. Rocca
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
(2023)
Article
Clinical Neurology
Elisabetta Sarasso, Andrea Gardoni, Lucia Zenere, Elisa Canu, Silvia Basaia, Elisa Pelosin, Maria Antonietta Volonte, Massimo Filippi, Federica Agosta
Summary: This study aimed to assess the effects of action observation and MI training (AOT-MI) on MI abilities and brain function in PD-PIGD patients. The results showed that AOT-MI improved MI skills in PD-PIGD patients and promoted the functional plasticity of brain areas involved in MI processes and gait/balance control.
PARKINSONISM & RELATED DISORDERS
(2023)
Article
Neuroimaging
Anna Nigri, Manera Umberto, Mario Stanziano, Stefania Ferraro, Davide Fedeli, Jean Paul Medina Carrion, Sara Palermo, Laura Lequio, Federica Denegri, Federica Agosta, Massimo Filippi, Maria Consuelo Valentini, Antonio Canosa, Andrea Calvo, Adriano Chio, Maria Grazia Bruzzone, Cristina Moglia
Summary: C9orf72 mutation carriers with different neurological phenotypes show cortical and subcortical atrophy in multiple brain regions, even in pre-symptomatic phases. This study found disease-specific patterns of thalamo-cortico-striatal atrophy and functional alterations in ALS patients with C9orf72 mutation. Importance rating: 8/10.
NEUROIMAGE-CLINICAL
(2023)
Article
Clinical Neurology
Aleksandra Tomic, Elisabetta Sarasso, Silvia Basaia, Natasa Dragasevic-Miskovic, Marina Svetel, Vladimir S. Kostic, Massimo Filippi, Federica Agosta
Summary: The study found that DYT-S patients have thinning in the frontal and motor cortical regions, putaminal atrophy, and subcortical microstructural WM damage. DYT-A patients, on the other hand, show cortical thickening in middle frontal areas and WM damage in the corona radiata.
JOURNAL OF NEUROLOGY
(2023)
