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Progressive supranuclear palsy

Andreas Hahn, Martin Schain, Maria Erlandsson, Petter Sjölin, Gregory M James, Olof T Strandberg, Douglas Hägerström, Rupert Lanzenberger, Jonas Jögi, Tomas G Olsson, Ruben Smith, Oskar Hansson
: Aggregation of hyperphosphorylated tau is a major hallmark of many neurodegenerative diseases, including Alzheimer's disease. In vivo imaging with positron emission tomography (PET) may offer important insights in pathophysiological mechanisms, diagnosis and disease progression. We describe different strategies for quantification of (18)F-AV1451 (T807) tau binding, including models with blood sampling and non-invasive alternatives. METHODS: 15 subjects (4 controls, 6 Alzheimer's disease (AD), 3 progressive supranuclear palsy (PSP), 2 cortico basal syndrome (CBS)) underwent 180 min PET with (18)F-AV1451 and arterial blood sampling...
October 20, 2016: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
Manuela Cristina Matesan, Saeed Elojeimy, Satoshi Minoshima
Molecular brain imaging I-FP-CIT SPECT is an important tool in evaluation of patients with parkinsonism. However, various neurodegenerative etiologies cannot be differentiated by I-FP-CIT SPECT alone. We present a case of progressive supranuclear palsy with abnormal I-FP-CIT SPECT and abnormal Tc-HMPAO SPECT depicted by quantitative analyses but unremarkable MRI 16 months after the onset of symptoms. Brain autopsy demonstrated presence of neuronal and glial tau pathology in both cortical and subcortical regions confirming the diagnosis of progressive supranuclear palsy...
October 18, 2016: Clinical Nuclear Medicine
Salvatore Nigro, Gennarina Arabia, Angelo Antonini, Luca Weis, Andrea Marcante, Alessandro Tessitore, Mario Cirillo, Gioacchino Tedeschi, Stefano Zanigni, Giovanna Calandra-Buonaura, Caterina Tonon, Gianni Pezzoli, Roberto Cilia, Mario Zappia, Alessandra Nicoletti, Calogero Edoardo Cicero, Michele Tinazzi, Pierluigi Tocco, Nicolò Cardobi, Aldo Quattrone
OBJECTIVES: To investigate the reliability of a new in-house automatic algorithm for calculating the Magnetic Resonance Parkinsonism Index (MRPI), in a large multicentre study population of patients affected by progressive supranuclear palsy (PSP) or Parkinson's disease (PD), and healthy controls (HC), and to compare the diagnostic accuracy of the automatic and manual MRPI values. METHODS: The study included 88 PSP patients, 234 PD patients and 117 controls. MRI was performed using both 3T and 1...
October 19, 2016: European Radiology
Graham Fairfoul, Lynne I McGuire, Suvankar Pal, James W Ironside, Juliane Neumann, Sharon Christie, Catherine Joachim, Margaret Esiri, Samuel G Evetts, Michal Rolinski, Fahd Baig, Claudio Ruffmann, Richard Wade-Martins, Michele T M Hu, Laura Parkkinen, Alison J E Green
We have developed a novel real-time quaking-induced conversion RT-QuIC-based assay to detect alpha-synuclein aggregation in brain and cerebrospinal fluid from dementia with Lewy bodies and Parkinson's disease patients. This assay can detect alpha-synuclein aggregation in Dementia with Lewy bodies and Parkinson's disease cerebrospinal fluid with sensitivities of 92% and 95%, respectively, and with an overall specificity of 100% when compared to Alzheimer and control cerebrospinal fluid. Patients with neuropathologically confirmed tauopathies (progressive supranuclear palsy; corticobasal degeneration) gave negative results...
October 2016: Annals of Clinical and Translational Neurology
Mansi R Khanna, Jane Kovalevich, Virginia M-Y Lee, John Q Trojanowski, Kurt R Brunden
A group of neurodegenerative diseases referred to as tauopathies are characterized by the presence of brain cells harboring inclusions of pathological species of the tau protein. These disorders include Alzheimer's disease and frontotemporal lobar degeneration due to tau pathology, including progressive supranuclear palsy, corticobasal degeneration, and Pick's disease. Tau is normally a microtubule (MT)-associated protein that appears to play an important role in ensuring proper axonal transport, but in tauopathies tau becomes hyperphosphorylated and disengages from MTs, with consequent misfolding and deposition into inclusions that mainly affect neurons but also glia...
October 2016: Alzheimer's & Dementia: the Journal of the Alzheimer's Association
Y Lee, D K Lee, J M Lee, S J Chung, J J Lee, Y H Sohn, P H Lee
BACKGROUND AND PURPOSE: Although early cerebellar symptoms are one of the exclusive criteria in the diagnosis of progressive supranuclear palsy (PSP), cerebellar involvement in PSP is evident both clinically and pathologically. However, structural analysis focusing on the cerebellum has not been previously studied in patients with PSP. We aimed to evaluate cerebellar involvement in PSP using a magnetic resonance imaging-based segmental volumetric analysis. METHODS: We retrospectively enrolled 48 patients with PSP composed of 25 patients with PSP-Richardson's syndrome (RS) and 23 patients with pure akinesia with gait freezing, 39 patients with Parkinson's disease (PD) and 34 healthy controls...
October 16, 2016: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
Shubir Dutt, Richard J Binney, Hilary W Heuer, Phi Luong, Suneth Attygalle, Priyanka Bhatt, Gabe A Marx, Jonathan Elofson, Maria C Tartaglia, Irene Litvan, Scott M McGinnis, Bradford C Dickerson, John Kornak, Dana Waltzman, Lisa Voltarelli, Norbert Schuff, Gil D Rabinovici, Joel H Kramer, Clifford R Jack, Bruce L Miller, Howard J Rosen, Adam L Boxer
OBJECTIVE: To examine the utility and reliability of volumetric MRI in measuring disease progression in the 4 repeat tauopathies, progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS), to support clinical development of new tau-directed therapeutic agents. METHODS: Six- and 12-month changes in regional MRI volumes and PSP Rating Scale scores were examined in 55 patients with PSP and 33 patients with CBS (78% amyloid PET negative) compared to 30 normal controls from a multicenter natural history study...
October 14, 2016: Neurology
Sangwon Han, Minyoung Oh, Jungsu S Oh, Sang Ju Lee, Seung Jun Oh, Sun Ju Chung, Hee Kyung Park, Jae Seung Kim
Importance: Pure akinesia with gait freezing (PAGF) is a clinical syndrome characterized by freezing of gait, handwriting, and speech without abnormal eye movement or cognitive impairment. Several studies have suggested that PAGF may be a variant of progressive supranuclear palsy (PSP). However, the characteristics of striatal dopamine transporter loss in PAGF are unknown. Objective: To investigate the subregional pattern of striatal dopamine transporter loss in patients with PAGF in comparison with patients with PSP and those with Parkinson disease (PD)...
October 10, 2016: JAMA Neurology
Alexander Frizell Santillo, Karl Lundblad, Markus Nilsson, Maria Landqvist Waldö, Danielle van Westen, Jimmy Lätt, Erik Blennow Nordström, Susanna Vestberg, Olof Lindberg, Christer Nilsson
Disinhibition is an important symptom in neurodegenerative diseases. However, the clinico-anatomical underpinnings remain controversial. We explored the anatomical correlates of disinhibition in neurodegenerative disease using the perspective of grey and white matter imaging. Disinhibition was assessed with a neuropsychological test and a caregiver information-based clinical rating scale in 21 patients with prefrontal syndromes due to behavioural variant frontotemporal dementia (n = 12) or progressive supranuclear palsy (n = 9), and healthy controls (n = 25)...
2016: PloS One
Cristian A Lasagna-Reeves, Maria de Haro, Shuang Hao, Jeehye Park, Maxime W C Rousseaux, Ismael Al-Ramahi, Paymaan Jafar-Nejad, Luis Vilanova-Velez, Lauren See, Antonia De Maio, Larissa Nitschke, Zhenyu Wu, Juan C Troncoso, Thomas F Westbrook, Jianrong Tang, Juan Botas, Huda Y Zoghbi
Many neurodegenerative proteinopathies share a common pathogenic mechanism: the abnormal accumulation of disease-related proteins. As growing evidence indicates that reducing the steady-state levels of disease-causing proteins mitigates neurodegeneration in animal models, we developed a strategy to screen for genes that decrease the levels of tau, whose accumulation contributes to the pathology of both Alzheimer disease (AD) and progressive supranuclear palsy (PSP). Integrating parallel cell-based and Drosophila genetic screens, we discovered that tau levels are regulated by Nuak1, an AMPK-related kinase...
October 1, 2016: Neuron
Ruben Smith, Martin Schain, Christer Nilsson, Olof Strandberg, Tomas Olsson, Douglas Hägerström, Jonas Jögi, Edilio Borroni, Michael Schöll, Michael Honer, Oskar Hansson
BACKGROUND: Progressive supranuclear palsy (PSP) is difficult to diagnose accurately. The recently developed tau PET tracers may improve the diagnostic work-up of PSP. METHODS: Regional tau accumulation was studied using (18) F-AV-1451 PET in 11 patients with PSP and 11 age-matched healthy controls in the Swedish BioFinder study. RESULTS: (18) F-AV-1451 standard uptake volume ratios were significantly higher in the basal ganglia in PSP patients when compared with controls (globus pallidus 1...
October 6, 2016: Movement Disorders: Official Journal of the Movement Disorder Society
Monica Sanchez-Contreras, Michael G Heckman, Pawel Tacik, Nancy Diehl, Patricia H Brown, Alexandra I Soto-Ortolaza, Elizabeth A Christopher, Ronald L Walton, Owen A Ross, Lawrence I Golbe, Neill Graff-Radford, Zbigniew K Wszolek, Dennis W Dickson, Rosa Rademakers
BACKGROUND: Mutations in the leucine-rich repeat kinase 2 gene (LRRK2) are the most common genetic cause of Parkinson's disease (PD). Unexpectedly, tau pathology has been reported in a subset of LRRK2 mutation carriers. METHODS: To estimate the frequency of pathogenic LRRK2 mutations and to evaluate the association of common LRRK2 variants with risk of primary tauopathies, we studied 1039 progressive supranuclear palsy (PSP) and 145 corticobasal degeneration patients from the Mayo Clinic Florida brain bank and 1790 controls ascertained at Mayo Clinic...
October 6, 2016: Movement Disorders: Official Journal of the Movement Disorder Society
Vincent Huin, Vincent Deramecourt, Dominique Caparros-Lefebvre, Claude-Alain Maurage, Charles Duyckaerts, Eniko Kovari, Florence Pasquier, Valérie Buée-Scherrer, Julien Labreuche, Hélène Behal, Luc Buée, Claire-Marie Dhaenens, Bernard Sablonnière
BACKGROUND: Progressive supranuclear palsy (PSP) is a rare neurodegenerative disease causing parkinsonian symptoms. Altered DNA methylation of the microtubule-associated protein tau gene correlates with the expression changes in Alzheimer's disease and Parkinson's disease brains. However, few studies examine the sequences beyond the constitutive promoter. OBJECTIVES: Because activating different microtubule-associated protein tau gene control regions via methylation might regulate the differential tau expression constituting the specific signatures of individual tauopathies, we compared methylation of a candidate promoter, intron 0...
October 6, 2016: Movement Disorders: Official Journal of the Movement Disorder Society
Timothy Rittman, Mikail Rubinov, Petra E Vértes, Ameera X Patel, Cedric E Ginestet, Boyd C P Ghosh, Roger A Barker, Maria Grazia Spillantini, Edward T Bullmore, James B Rowe
Abnormalities of tau protein are central to the pathogenesis of progressive supranuclear palsy, whereas haplotype variation of the tau gene MAPT influences the risk of Parkinson disease and Parkinson's disease dementia. We assessed whether regional MAPT expression might be associated with selective vulnerability of global brain networks to neurodegenerative pathology. Using task-free functional magnetic resonance imaging in progressive supranuclear palsy, Parkinson disease, and healthy subjects (n = 128), we examined functional brain networks and measured the connection strength between 471 gray matter regions...
September 9, 2016: Neurobiology of Aging
Carlos Guevara, Katherina Bulatova, Gareth J Barker, Guido Gonzalez, Nicolas A Crossley, Matthew J Kempton
BACKGROUND: The absence of markers for ante-mortem diagnosis of progressive supranuclear palsy (PSP), results in this disorder being commonly mistaken for other conditions, such as idiopathic Parkinson's disease (IPD). Such mistakes occur particularly in the initial stages, when "plus syndrome" has not yet clinically emerged. OBJECTIVE: To investigate the global brain volume and tissue loss in patients with PSP relative to patients with IPD and healthy controls and correlations between clinical parameters and magnetic resonance imaging (MRI)-derived brain volume estimates...
2016: Frontiers in Aging Neuroscience
Zdenek Rohan, Ivan Milenkovic, Mirjam I Lutz, Radoslav Matej, Gabor G Kovacs
Pathological protein deposits in oligodendroglia are common but variable features of various neurodegenerative conditions. To evaluate oligodendrocyte response in neurodegenerative diseases (NDDs) with different extents of oligodendroglial protein deposition we performed immunostaining for tubulin polymerization-promoting protein p25α (TPPP/p25α), α-synuclein (α-syn), phospho-tau, ubiquitin, myelin basic protein, and the microglial marker HLA-DR. We investigated cases of multiple system atrophy ([MSA] n = 10), Lewy body disease ([LBD] n = 10), globular glial tauopathy ([GGT] n = 7) and progressive supranuclear palsy ([PSP] n = 10)...
September 26, 2016: Journal of Neuropathology and Experimental Neurology
Chiara Cerami, Alessandra Dodich, Lucia Greco, Sandro Iannaccone, Giuseppe Magnani, Alessandra Marcone, Elisabetta Pelagallo, Roberto Santangelo, Stefano F Cappa, Daniela Perani
BACKGROUND AND OBJECTIVE: Primary progressive aphasia (PPA) is a clinical syndrome due to different neurodegenerative conditions in which an accurate early diagnosis needs to be supported by a reliable diagnostic tool at the individual level. In this study, we investigated in PPA the FDG-PET brain metabolic patterns at the single-subject level, in order to assess the case-to-case variability and its relationship with clinical-neuropsychological findings. MATERIAL AND METHODS: 55 patients (i...
September 20, 2016: Journal of Alzheimer's Disease: JAD
Adrienne M Keener, Yvette M Bordelon
Parkinsonism is a clinical syndrome, which is characterized by bradykinesia, rigidity, rest tremor, and postural instability. Idiopathic Parkinson disease (PD) is the most common cause of this syndrome, though there are several other important etiologies that must be considered. These include the atypical Parkinsonian disorders multiple system atrophy (MSA), dementia with Lewy Bodies (DLB), progressive supranuclear palsy (PSP), and corticobasal syndrome (CBS); as well as secondary causes of parkinsonism. These various disease entities may be distinguished based on key clinical features, which is critical for the purposes of diagnosis, treatment, and prognosis...
August 2016: Seminars in Neurology
Emily Owens, Keith A Josephs, Rodolfo Savica, Anhar Hassan, Bryan Klassen, James Bower, Demetrius Maraganore, Joseph Matsumoto, J E Ahlskog
Gait freezing as a presenting and relatively restricted condition is uncommon but a distinctive disorder. This entity was initially defined as "pure akinesia with gait freezing", and later a neuropathological substrate of progressive supranuclear palsy has been recognized. Limited studies have reported the clinical evolution after presentation, which is important for patient counseling. The objective of this study was to assess the demographic and clinical features, treatment-response, neuroimaging, and evolution of pure akinesia with gait freezing...
September 13, 2016: Journal of Neurology
Thomas Arendt, Jens T Stieler, Max Holzer
Most neurodegenerative diseases are characterized by intracellular aggregates of insoluble proteins. As for the majority of these disorders, aetiology and pathogenesis are only poorly understood; current nosological concepts are largely based on these molecular signatures of protein aggregates which also provide valuable tools for neuropathological differential diagnosis. The microtubule associated protein tau is one of these proteins that form intracellular fibrillary deposits in neurons and glial cells of a large variety of disorders today collectively referred to as tauopathies...
September 9, 2016: Brain Research Bulletin
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