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Front Neurol 2021 25;12:644064. Epub 2021 May 25.

Department of Neurology, Hannover Medical School, Hanover, Germany.

The German research networks and prospectively collect comprehensive clinical data, imaging data and biomaterials of patients with a clinical diagnosis of progressive supranuclear palsy. Progressive supranuclear palsy is a rare, adult-onset, neurodegenerative disease with striking clinical heterogeneity. Since now, prospective natural history data are largely lacking. Read More

Front Neurosci 2021 25;15:643115. Epub 2021 May 25.

Institute of Human Genetics, Faculty of Medicine and University Hospital Cologne, University of Cologne, Cologne, Germany.

In the adult human brain, six isoforms of the microtubule-associated protein TAU are expressed, which result from alternative splicing of exons 2, 3, and 10 of the gene. These isoforms differ in the number of N-terminal inserts (0N, 1N, 2N) and C-terminal repeat domains (3R or 4R) and are differentially expressed depending on the brain region and developmental stage. Although all TAU isoforms can aggregate and form neurofibrillary tangles, some tauopathies, such as Pick's disease and progressive supranuclear palsy, are characterized by the accumulation of specific TAU isoforms. Read More

PLoS One 2021 10;16(6):e0253079. Epub 2021 Jun 10.

Department of Neurology, National Hospital Organization, Higashinagoya National Hospital, Nagoya, Japan.

Introduction: Skeletal muscle mass loss has been associated with decreased physical performance; however, the body composition characteristics in progressive supranuclear palsy (PSP) are not well understood. We investigated body composition parameters, focusing on skeletal muscle mass, in patients with PSP and compared them with those of healthy older adults.

Methods: This retrospective cross-sectional study included 39 patients with PSP and 30 healthy older adults (control group). Read More

Cell Mol Bioeng 2021 Jun 22;14(3):231-240. Epub 2021 Mar 22.

Department of Biomedical Engineering, Rensselaer Polytechnic Institute, Troy, NY 12180 USA.

Introduction: Cell chirality is an intrinsic cellular property that determines the directionality of cellular polarization along the left-right axis. We recently show that endothelial cell chirality can influence intercellular junction formation and alter trans-endothelial permeability, depending on the uniformity of the chirality of adjacent cells, which suggests a potential role for cell chirality in neurodegenerative diseases with blood-brain barrier (BBB) dysfunctions, such as Alzheimer's disease (AD). In this study, we determined the effects of AD-related proteins amyloid-β (Aβ), tau, and apolipoprotein E4 (ApoE4) on the chiral bias of the endothelial cell component in BBB. Read More

J Neural Transm (Vienna) 2021 Jun 8. Epub 2021 Jun 8.

Department of Neurology, Medical University of Innsbruck, Anichstrasse 35, 6020, Innsbruck, Austria.

Differential diagnosis of parkinsonian syndromes is considered one of the most challenging in neurology. Quantitative MR planimetric measurements were reported to discriminate between progressive supranuclear palsy (PSP) and non-PSP-parkinsonism. Several studies have used midbrain to pons ratio (M/P) and the Magnetic Resonance Parkinsonism Index (MRPI) in distinguishing PSP patients from those with Parkinson's disease. Read More

Nat Commun 2021 06 8;12(1):3452. Epub 2021 Jun 8.

Department of Radiology, Mayo Clinic, Rochester, MN, USA.

Progressive apraxia of speech is a neurodegenerative syndrome affecting spoken communication. Molecular pathology, biochemistry, genetics, and longitudinal imaging were investigated in 32 autopsy-confirmed patients with progressive apraxia of speech who were followed over 10 years. Corticobasal degeneration and progressive supranuclear palsy (4R-tauopathies) were the most common underlying pathologies. Read More

Drugs 2021 Jun 8. Epub 2021 Jun 8.

Department of Neuroscience and Physiology, Neuroscience Institute, New York University Grossman School of Medicine, Science Building, 11th floor, 435 East 30th Street, New York, NY, 10016, USA.

Tau immunotherapies have advanced from proof-of-concept studies to over a dozen clinical trials for Alzheimer's disease (AD) and other tauopathies. Mechanistic studies in animal and culture models have provided valuable insight into how these therapies may work but multiple pathways are likely involved. Different groups have emphasized the importance of intracellular vs extracellular antibody-mediated clearance of the tau protein and there is no consensus on which pool of tau should ideally be targeted. Read More

Acta Neuropathol 2021 Jun 6. Epub 2021 Jun 6.

Normandie Univ, UNIROUEN, Inserm U1245, CHU Rouen, Department of Genetics and CNR-MAJ, F-76000, Rouen, France.

Microduplications of the 17q21.31 chromosomal region encompassing the MAPT gene, which encodes the Tau protein, were identified in patients with a progressive disorder initially characterized by severe memory impairment with or without behavioral changes that can clinically mimic Alzheimer disease. The unique neuropathological report showed a primary tauopathy, which could not be unanimously classified in a given known subtype, showing both 4R- and 3R-tau inclusions, mainly within temporal cortical subregions and basal ganglia, without amyloid deposits. Read More

Front Genet 2021 20;12:657636. Epub 2021 May 20.

Department of Biomedical Engineering, School of Fundamental Sciences, China Medical University, Shenyang, China.

Neurodegenerative Diseases (NDs) are age-dependent and include Alzheimer's disease (AD), Parkinson's disease (PD), progressive supranuclear palsy (PSP), frontotemporal dementia (FTD), and so on. There have been numerous studies showing that accelerated aging is closely related (even the driver of) ND, thus promoting imbalances in cellular homeostasis. However, the mechanisms of how different ND types are related/triggered by advanced aging are still unclear. Read More

Mov Disord 2021 Jun 5. Epub 2021 Jun 5.

PET Center, Huashan Hospital, Fudan University, Shanghai, China.

Background: F-APN-1607 is a novel tau PET tracer characterized by high binding affinity for 3- and 4-repeat tau deposits. Whether F-APN-1607 PET imaging is clinically useful in PSP remains unclear.

Objectives: The objective of this study was to investigate the clinical utility of F-APN-1607 PET in the diagnosis, differential diagnosis, and assessment of disease severity in patients with PSP. Read More

PLoS One 2021 4;16(6):e0252825. Epub 2021 Jun 4.

Department of Ophthalmology, University Medical Center Mainz, Mainz, Germany.

Introduction: Niemann-Pick type C (NPC) is a lysosomal storage disease that is progressive and life-limiting, with an estimated incidence of 1:120,000 live births. In addition to systemic manifestation with (hepato-)splenomegaly, there are a number of neurological manifestations (ataxia, dysarthria, dementia, cataplexy, epileptic seizures, and psychiatric disorders). Characteristic is vertical supranuclear gaze palsy, which is often overlooked. Read More

Environ Sci Pollut Res Int 2021 Jun 3. Epub 2021 Jun 3.

Department of Pharmacy, Faculty of Medicine and Pharmacy, University of Oradea, Oradea, Romania.

The mitochondria, apart from being known as the cell's "powerhouse," are crucial in the viability of nerve cells. Any damage to these cellular organelles can result in their cellular level dysfunction which includes rapidly multiplying reactive oxygen species (ROS) from the mitochondrial membrane, impaired calcium ion homeostasis, and disturbed mitochondrial dynamics by the formation of permeability transition pore in mitochondria. All these impaired biochemical changes lead to various neurological disorders such as progressive supranuclear palsy (PSP), Parkinson's disease (PD), and Alzheimer's disease (AD). Read More

Brain Sci 2021 May 25;11(6). Epub 2021 May 25.

Department of Clinical Neurosciences, School of Medicine, "Carol Davila" University of Medicine and Pharmacy, 020021 Bucharest, Romania.

Diagnosing atypical parkinsonism can be an error-exposed undertaking in the context of elaborate criteria coupled with time restraints on their comprehensive application. We conducted a retrospective, descriptive study of diagnostic accuracy among physicians at two tertiary neurology centers in Romania and developed an algorithmic tool for comparison purposes. As many as 90 patients qualified for inclusion in the study, with 77 patients actually complying with atypical parkinsonism criteria. Read More

J Mov Disord 2021 May 26;14(2):103-113. Epub 2021 May 26.

Parkinson and Other Movement Disorders Center, Department of Neurosciences, University of California San Diego, La Jolla, CA, USA.

Typically, progressive supranuclear palsy (PSP) is clinically characterized by slow vertical saccades or supranuclear gaze palsy, levodopa-resistant parkinsonism with predominant axial symptoms, and cognitive executive impairment. Over the past decades, various PSP phenotypes, including PSP with predominant parkinsonism, PSP with corticobasal syndrome, PSP with progressive gait freezing, and PSP with predominant frontal dysfunction, have been identified from pathologically confirmed cases. Expanding knowledge led to new diagnostic criteria for PSP that with increased disease awareness led to increased PSP prevalence estimates. Read More

Brain 2021 Jun 1. Epub 2021 Jun 1.

Instituto de Investigaciones en Ingeniería Genética y Biología Molecular (INGEBI-CONICET), Buenos Aires, Argentina.

Tauopathies are neurodegenerative diseases caused by the abnormal metabolism of the microtubule associated protein Tau, which is highly expressed in neurons and critically involved in microtubule dynamics. In the adult human brain, the alternative splicing of exon 10 in tau pre-mRNA produces equal amounts of protein isoforms with either three (3 R) or four (4 R) microtubule binding domains. Imbalance in the 3 R : 4 R tau ratio is associated with primary tauopathies that develop atypical parkinsonism, such as Progressive Supranuclear Palsy and Corticobasal Degeneration. Read More

Dement Geriatr Cogn Dis Extra 2021 Jan-Apr;11(1):64-70. Epub 2021 Apr 15.

Department of Medicine, Chulalongkorn University, Bangkok, Thailand.

Introduction: Molecular imaging has been developed and validated in Thai patients, comprising a portion of patients in the dementia registry. This should provide a more accurate diagnosis of the etiology of dementia, which was the focus of this study.

Methods: This was a multicenter dementia study. Read More

Front Neurol 2021 12;12:573401. Epub 2021 May 12.

Department of Defense/Uniformed Services University (DoD/USU) Brain Tissue Repository & Neuropathology Program, Uniformed Services University of the Health Science (USU), Bethesda, MD, United States.

We aimed to detect the possible accelerating role of previous traumatic brain injury (TBI) exposures on the onset of later cognitive decline assessed across different brain diseases. We analyzed data from the National Alzheimer's Coordinating Center (NACC), which provide information on history of TBI and longitudinal data on cognitive and non-cognitive domains for each available subject. At the time of this investigation, a total of 609 NACC subjects resulted to have a documented history of TBI. Read More

Parkinsons Dis 2021 10;2021:9990679. Epub 2021 May 10.

Department of Neurology, Hannover Medical School, Carl-Neuberg-Straße 1, Hannover 30625, Germany.

Progressive supranuclear palsy (PSP) is an atypical Parkinson syndrome with axial akinetic-rigid symptoms, early postural instability, and ocular motor impairments. Patients experience a rapid loss of autonomy and care dependency; thus, caregivers must assist in the activities of daily living early in the course of the disease. Caregiver burden is an extremely important factor in disease management. Read More

J Cereb Blood Flow Metab 2021 May 27:271678X211018904. Epub 2021 May 27.

Center for Neuropathology and Prion Research, University Hospital of Munich, LMU Munich, Munich, Germany.

The novel tau-PET tracer [F]PI-2620 detects the 3/4-repeat-(R)-tauopathy Alzheimer's disease (AD) and the 4R-tauopathies corticobasal syndrome (CBS) and progressive supranuclear palsy (PSP). We determined whether [F]PI-2620 binding characteristics deriving from non-invasive reference tissue modelling differentiate 3/4R- and 4R-tauopathies. Ten patients with a 3/4R tauopathy (AD continuum) and 29 patients with a 4R tauopathy (CBS, PSP) were evaluated. Read More

Rofo 2021 May 25. Epub 2021 May 25.

Clinic for Radiology and Neuroradiology, University Hospital Schleswig-Holstein - Campus Kiel, Germany.

Background:  Diagnosis of Parkinson's disease and atypical parkinsonism is based on clinical evaluation of the patient's symptoms and on magnetic resonance imaging (MRI) of the brain, which can be supplemented by nuclear medicine techniques. MRI plays a leading role in the differentiation between Parkinson's disease and atypical parkinsonism. While atypical parkinsonism is characterized by relatively specific MRI signs, imaging of Parkinson's disease previously lacked such signs. Read More

Neurosci Lett 2021 May 21;757:135975. Epub 2021 May 21.

Division of Neurology and Gerontology, Department of Internal Medicine, School of Medicine, Iwate Medical University, 2-1-1 Idai-dori, Yahaba-cho, Shiwa-gun, Iwate 028-3694, Japan. Electronic address:

Parkinson's disease (PD) is a progressive neurodegenerative disorder characterized by motor and non-motor symptoms. Because no curative therapy is available for PD, elucidation of its pathophysiology is important to establish more effective treatments. Oxidative stress (OS) has gained attention and been investigated as one of the candidates involved in the pathogenesis of PD. Read More

Eur J Nucl Med Mol Imaging 2021 May 22. Epub 2021 May 22.

Department of Psychiatry, University Hospital Cologne, Cologne, Germany.

Purpose: Dynamic 60-min positron emission tomography (PET) imaging with the novel tau radiotracer [F]PI-2620 facilitated accurate discrimination between patients with progressive supranuclear palsy (PSP) and healthy controls (HCs). This study investigated if truncated acquisition and static time windows can be used for [F]PI-2620 tau-PET imaging of PSP.

Methods: Thirty-seven patients with PSP Richardson syndrome (PSP-RS) were evaluated together with ten HCs. Read More

Front Aging Neurosci 2021 4;13:635896. Epub 2021 May 4.

Cognitive Neurology and Aphasia Unit, Centro de Investigaciones Médico-Sanitarias, University of Malaga, Málaga, Spain.

The diagnostic criteria for progressive supranuclear palsy (PSP) incorporate two speech-language disturbances (SLDs), non-fluent/agrammatic primary progressive aphasia and progressive apraxia of speech, but overlook the inclusion of other SLDs, including dynamic aphasia (DA). Thus, there is a need to reappraise the broad spectrum of SLDs in PSP to include other presenting phenotypes. Here we report findings from the study of two elderly patients with PSP presenting with DA and irrepressible echolalia. Read More

J Neurol Sci 2021 May 4;427:117477. Epub 2021 May 4.

UCL Queen Square Institute of Neurology Department of Clinical and Movement Neurosciences, Queen Square, London WC1N 3BG, United Kingdom. Electronic address:

Introduction: Dystonia occurring in the context of parkinsonism is well-known, e.g. as foot dystonia in young-onset Parkinson's disease (PD), anterocollis in multisystem atrophy (MSA) or blepharospasm (levator inhibition) in progressive supranuclear palsy. Read More

Curr Opin Neurol 2021 May 19. Epub 2021 May 19.

Institut du Cerveau - ICM, INSERM U 1127, CNRS UMR 7225, Sorbonne Université, Team 'Movement Investigations and Therapeutics' (MOV'IT) ICM, Centre de NeuroImagerie de Recherche - CENIR Department of Neuroradiology, Pitié-Salpêtrière Hospital, Assistance Publique - Hôpitaux de Paris (AP-HP), Paris, France.

Purpose Of Review: Differential diagnosis of Parkinsonism may be difficult. The objective of this review is to present the work of the last three years in the field of imaging for diagnostic categorization of parkinsonian syndromes focusing on progressive supranuclear palsy (PSP) and multiple system atrophy (MSA).

Recent Findings: Two main complementary approaches are being pursued. Read More

Mov Disord 2021 May 18. Epub 2021 May 18.

German Center for Neurodegenerative Diseases, DZNE, Bonn, Germany.

Background: Extracellular vesicles are small vesicles that are released from many cells, including neurons. α-Synuclein has recently been described in extracellular vesicles derived from the central nervous system and may contribute to the spreading of disease pathology in α-synuclein-related neurodegeneration.

Objectives: We aimed to examine the potential diagnostic value of α-synuclein in plasma extracellular vesicles from patients with Parkinson's disease (PD). Read More

Parkinsonism Relat Disord 2021 May 11;87:75-81. Epub 2021 May 11.

Department of Neurology, Graduate School of Medicine, Mie University, Mie, 514-8507, Japan.

Introduction: Several reports have shown that neuromelanin-sensitive magnetic resonance imaging (NMI) using 3T magnetic resonance imaging is useful for the differential diagnosis of Parkinson's disease (PD), progressive supranuclear palsy (PSP), and other neurological diseases. However, the number of cases in previous studies has been insufficient. We aimed to determine the relationship between NMI and severity of PD and related disorders, and thereby establish the diagnostic utility of NMI for diagnosing neurological diseases. Read More

Sci Rep 2021 May 13;11(1):10309. Epub 2021 May 13.

Neuroscience, Eli Lilly and Company, Erl Wood Manor, Windlesham, Surrey, GU20 6PH, UK.

Intracellular tau inclusions are a pathological hallmark of Alzheimer's disease, progressive supranuclear palsy, corticobasal degeneration and other sporadic neurodegenerative tauopathies. Recent in vitro and in vivo studies have shown that tau aggregates may spread to neighbouring cells and functionally connected brain regions, where they can seed further tau aggregation. This process is referred to as tau propagation. Read More

Mov Disord Clin Pract 2021 May 29;8(4):594-599. Epub 2021 Mar 29.

Edmond J. Safra Program in Parkinson's Disease, Rossy Program for PSP Research and the Morton and Gloria Shulman Movement Disorders Clinic Toronto Western Hospital Toronto Ontario Canada.

Background: Pallido-nigro-luysian atrophy (PNLA) is a rare neurodegenerative disorder with only a few cases reported to date. Although the clinical picture usually resembles progressive supranuclear palsy, pathological examination reveals more selective atrophy and loss of neurons in the globus pallidus, substantia nigra and subthalamic nucleus.

Objectives: To describe the clinical features and pathological findings of a patient with PNLA. Read More

Brief Funct Genomics 2021 May 8. Epub 2021 May 8.

Department of Biostatistics, Virginia Commonwealth University.

In the last decade, massive omics datasets have been generated for human brain research. It is evolving so fast that a timely update is urgently needed. In this review, we summarize the main multi-omics data resources for the human brains of both healthy controls and neuropsychiatric disorders, including schizophrenia, autism, bipolar disorder, Alzheimer's disease, Parkinson's disease, progressive supranuclear palsy, etc. Read More