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Clin Neurol Neurosurg 2019 Feb 11;179:1-3. Epub 2019 Feb 11.

Neurology, Neurophysiology and Neurobiology Unit, Department of Medicine, Università Campus Bio-Medico di Roma, Rome, Italy.

Acta Neuropathol Commun 2019 Feb 15;7(1):22. Epub 2019 Feb 15.

AXON Neuroscience R&D Services SE, Dvorakovo nabrezie 10, 811 02, Bratislava, Slovakia.

Tau neuronal and glial pathologies drive the clinical presentation of Alzheimer's disease and related human tauopathies. There is a growing body of evidence indicating that pathological tau species can travel from cell to cell and spread the pathology through the brain. Throughout the last decade, physiological and pathological tau have become attractive targets for AD therapies. Read More

J Neural Transm (Vienna) 2019 Feb 14. Epub 2019 Feb 14.

Neurodegeneration Imaging Group, Maurice Wohl Clinical Neuroscience Institute, 125 Coldharbour Lane, Camberwell, London, SE5 9NU, UK.

The dementia spectrum encompasses a range of disorders with complex diagnosis, pathophysiology and limited treatment options. Positron emission tomography (PET) imaging provides insights into specific neurodegenerative processes underlying dementia disorders in vivo. Here we focus on some of the most common dementias: Alzheimer's disease, Parkinsonism dementias including Parkinson's disease with dementia, dementia with Lewy bodies, progressive supranuclear palsy and corticobasal syndrome, and frontotemporal lobe degeneration. Read More

Mov Disord 2019 Feb 13. Epub 2019 Feb 13.

Neuroimaging Research Unit, Institute of Molecular Bioimaging and Physiology, National Research Council, Catanzaro, Italy.

Background: No prospective study of patients with Parkinson's disease (PD) has investigated the appearance of vertical gaze abnormalities, a feature suggestive of progressive supranuclear palsy (PSP).

Objective: To identify, within a cohort of patients with an initial diagnosis of PD, those who developed vertical gaze abnormalities during a 4-year follow-up, and to investigate the performance of new imaging biomarkers in predicting vertical gaze abnormalities.

Methods: A total of 110 patients initially classified as PD and 74 controls were enrolled. Read More

Neurobiol Aging 2019 Jan 16;76:194-200. Epub 2019 Jan 16.

Department of Neurology, Pennsylvania State University-Milton S. Hershey Medical Center, Hershey, PA, USA; Department of Pharmacology, Pennsylvania State University-Milton S. Hershey Medical Center, Hershey, PA, USA; Department of Radiology, Pennsylvania State University-Milton S. Hershey Medical Center, Hershey, PA, USA; Department of Neurosurgery, Pennsylvania State University-Milton S. Hershey Medical Center, Hershey, PA, USA; Department of Kinesiology, Penn State University-Milton S. Hershey Medical Center, Hershey, PA, USA. Electronic address:

Previous multimodal magnetic resonance imaging (MRI) studies of parkinsonian syndromes have focused primarily on motor-related basal ganglia structures. The present study investigated MRI changes in nonmotor-related limbic structures in 35 Parkinson's disease, 16 multiple system atrophy parkinsonian subtype, 17 progressive supranuclear palsy, and 37 control subjects. Mean diffusivity (MD), fractional anisotropy, transverse relaxation rate (R2*), quantitative susceptibility mapping, and volume measurements were obtained from the amygdala, hippocampus, and nucleus accumbens (NAc) to examine differences between groups and to test for associations with clinical scores. Read More

J Mov Disord 2019 Jan 30;12(1):1-13. Epub 2019 Jan 30.

Department of Neurology, Korea University College of Medicine, Korea University Ansan Hospital, Ansan.

Abnormal eye movements are commonly observed in movement disorders. Ocular motility examination should include bedside evaluation and laboratory recording of ocular misalignment, involuntary eye movements, including nystagmus and saccadic intrusions/oscillations, triggered nystagmus, saccades, smooth pursuit (SP), and the vestibulo-ocular reflex. Patients with Parkinson's disease (PD) mostly show hypometric saccades, especially for the selfpaced saccades, and impaired SP. Read More

Mov Disord 2019 Feb 6. Epub 2019 Feb 6.

Department of Neurology, Mayo Clinic Rochester, Rochester, Minnesota, USA.

Background: In 2017, the International Parkinson and Movement Disorder Society put forward new clinical criteria for the diagnosis of PSP, recognizing diverse PSP phenotypes. In this study, we compared the sensitivity and specificity of the new criteria with the National Institutes of Neurological Disease and Society for Progressive Supranuclear Palsy criteria at different times.

Methods: Patients with clinical parkinsonism, clinical and/or neuropathological diagnosis of PSP, were identified from the Society for Progressive Supranuclear Palsy brain bank. Read More

Alzheimers Dement (Amst) 2019 Dec 24;11:115-124. Epub 2019 Jan 24.

Faculty of Medicine and Health, Charles Perkins Centre and Discipline of Pathology, University of Sydney, Sydney, Australia.

Introduction: Exploring the degree of heritability in a large cohort of frontotemporal lobar degeneration with tau-immunopositive inclusions (FTLD-tau) and determining if different FTLD-tau subtypes are associated with stronger heritability will provide important insight into disease pathogenesis.

Methods: Using modified Goldman pedigree classifications, heritability was examined in pathologically proven FTLD-tau cases with dementia at any time (n = 124) from the Sydney-Cambridge collection.

Results: Thirteen percent of the FTLD-tau cohort have a suggested autosomal dominant pattern of inheritance, 25% have some family history, and 62% apparently sporadic. Read More

Front Neurol 2019 22;10. Epub 2019 Jan 22.

Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria.

Differentiating idiopathic Parkinson's disease (IPD) from atypical Parkinsonian disorders (APD) is challenging, especially in early disease stages. Postural instability and gait difficulty (PIGD) are substantial motor impairments of IPD and APD. Clinical evidence implies that patients with APD have larger PIGD impairment than IPD patients. Read More

J Clin Exp Neuropsychol 2019 Feb 4:1-7. Epub 2019 Feb 4.

b Memory and Aging Center, Department of Neurology , University of California , San Francisco , CA, USA.

Progressive supranuclear palsy (PSP) is associated with a variety of cognitive deficits, as well as motor and psychiatric disturbances. As clinical trials for PSP evolve, briefer screening instruments will be needed to determine cognitive effects of interventions. The Repeatable Battery for the Assessment of Neuropsychological Status (RBANS) may fill this gap. Read More

J Neuroimmune Pharmacol 2019 Jan 31. Epub 2019 Jan 31.

Department of Biomedical Sciences, Parkinson's Disorder Research Program, Iowa Center for Advanced Neurotoxicology, Iowa State University, Ames, IA, 50011, USA.

Adult-onset neurodegenerative disorders, like Parkinson's disease (PD) and dementia with Lewy bodies (DLB), that share the accumulation of aggregated α-synuclein (αSyn) as their hallmark molecular pathology are collectively known as α-synucleinopathies. Diagnosing α-synucleinopathies requires the post-mortem detection of αSyn in various brain regions. Recent efforts to measure αSyn in living patients include quantifying αSyn in different biofluids as a biomarker for PD. Read More

Mol Neurobiol 2019 Jan 31. Epub 2019 Jan 31.

Russian Academy of Medical Sciences, Moscow, 113152, Russia.

With continuing cooperation from 18 domestic and international brain banks over the last 36 years, we have analyzed the aluminum content of the temporal lobe neocortex of 511 high-quality human female brain samples from 16 diverse neurological and neurodegenerative disorders, including 2 groups of age-matched controls. Temporal lobes (Brodmann areas A20-A22) were selected for analysis because of their availability and their central role in massive information-processing operations including efferent-signal integration, cognition, and memory formation. We used the analytical technique of (i) Zeeman-type electrothermal atomic absorption spectrophotometry (ETAAS) combined with (ii) preliminary analysis from the advanced photon source (APS) hard X-ray beam (7 GeV) fluorescence raster-scanning (XRFR) spectroscopy device (undulator beam line 2-ID-E) at the Argonne National Laboratory, US Department of Energy, University of Chicago IL, USA. Read More

Neurology 2019 Jan 30. Epub 2019 Jan 30.

From the Departments of Neuroscience, Rehabilitation, Ophthalmology, Genetics, and Maternal and Child Health (M.P., F.N.) and Health Sciences (S.M.), University of Genoa; IRCCS Ospedale Policlinico San Martino (M.P., S.M., F.N.), Genoa, Italy; Cognitive Neuroscience Division, Department of Neurology (E.D.H.), Gertrude H. Sergievsky Center, New York; Taub Institute for Research on Alzheimer's Disease and the Aging Brain (E.D.H., W.C.K.), Columbia University Medical Center, New York, NY; Department of Neurology (S.S.), UCSF Memory and Aging Center, UCSF, San Francisco, CA; Department of Pathology and Laboratory Medicine (S.S., B.G.), Indiana University School of Medicine, Indianapolis; Nuclear Medicine Unit (S.M.), IRCCS AOU San Martino, IST, Genoa, Italy; Behavioral Neurology Unit (E.M.W.), National Institute of Neurological Disorders and Stroke, NIH, Bethesda, MD; Psychiatry and Behavioral Sciences & Cognitive Neurology/Alzheimer's Disease Research Center (J.G.), Feinberg School of Medicine and Department of Psychology, Northwestern University; and Brain Injury Research, Cognitive Neuroscience Lab, Think and Speak Lab (J.G.), Shirley Ryan AbilityLab, Chicago, IL.

Objective: To evaluate brain Fluorodeoxyglucose PET (FDG-PET) differences among patients with a clinical diagnosis of corticobasal syndrome (CBS) and distinct underling primary pathologies.

Methods: We studied 29 patients with a diagnosis of CBS who underwent FDG-PET scan and postmortem neuropathologic examination. Patients were divided into subgroups on the basis of primary pathologic diagnosis: CBS-corticobasal degeneration (CBS-CBD) (14 patients), CBS-Alzheimer disease (CBS-AD) (10 patients), and CBS-progressive supranuclear palsy (CBS-PSP) (5 patients). Read More

Am J Nucl Med Mol Imaging 2018 20;8(6):360-372. Epub 2018 Dec 20.

Department of Nuclear Medicine, Kyungpook National University Hospital Daegu, Republic of Korea.

Combined use of F-N-(3-fluoropropyl)-2β-carboxymethoxy-3β-(4-iodophenyl)nortropane (FP-CIT) for dopamine transporter imaging and F-fludeoxyglucose (FDG) for glucose metabolism shows good diagnostic performance for differential diagnosis of Parkinson disease (PD) and Parkinson plus syndrome (multiple system atrophy, progressive supranuclear palsy, corticobasal degeneration, and dementia with Lewy bodies). A recent study showed that F-FP-CIT positron emission tomography (PET) with early perfusion imaging is useful for the differential diagnosis of PD and Parkinson plus syndrome with lower radiation exposure, time, and cost. In this review, we summarize the advantages of using F-FP-CIT PET for perfusion and dopamine transporter imaging, as well as clinical features useful for the differential diagnosis of PD and Parkinson plus syndrome. Read More

Curr Opin Neurol 2019 Jan 28. Epub 2019 Jan 28.

Parkinson's Disease and Movement Disorders Unit, Neurology Service, Hospital Clínic de Barcelona Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) Institut de Neurociències Centro de Investigación Biomedica en Red Enfermedades Neurodegenerativas (CIBERNED).

Purpose Of Review: Anti-IgLON5 disease is a novel entity characterized by a distinctive sleep disorder associated with a variety of neurological symptoms, antibodies against IgLON5, and pathological findings of neuronal tauopathy. The characteristic sleep disorder occurs in most patients, but other neurological symptoms are also important because they can be the presenting and most disabling problem and mimic other conditions. This review focuses on nonsleep neurological symptoms and presentations of anti-IgLON5 disease. Read More

Neurol Int 2018 Dec 20;10(4):7921. Epub 2018 Dec 20.

Department of Neurology, National Hospital Organization Hyogo-Chuo National Hospital, Sanda, Japan.

Continuous glucose monitoring (CGM) is a method to examine glucose concentration in subcutaneous interstitial fluid sequentially. CGM can disclose glucose fluctuation (GF), which can be unrecognized in routine blood tests. A limited number of studies suggest advanced Parkinsonian syndromes (PS) is at risk of GF, however, the report of CGM in PS is scarce. Read More

Cerebellum Ataxias 2019 17;6. Epub 2019 Jan 17.

1Neurological Institute, University Hospitals, Cleveland, OH USA.

Eye movements are frequently considered diagnostic markers indicating involvement of the cerebellum. Impaired amplitude of saccades (saccade dysmetria), impaired gaze holding function (horizontal or downbeat nystagmus), and interrupted (choppy) pursuit are typically considered hallmarks of cerebellar disorders. While saccade dysmetria is a frequently considered abnormality, the velocity of saccades are rarely considered part of the constellation of cerebellar involvement. Read More

Acta Neuropathol Commun 2019 Jan 23;7(1):10. Epub 2019 Jan 23.

Department of Neuroscience, Mayo Clinic Jacksonville, 4500 San Pablo Road, Jacksonville, FL, 32224, USA.

Pathogenic mutations in the tau gene (microtubule associated protein tau, MAPT) are linked to the onset of tauopathy, but the A152T variant is unique in acting as a risk factor for a range of disorders including Alzheimer's disease (AD), progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), and dementia with Lewy bodies (DLB). In order to provide insight into the mechanism by which A152T modulates disease risk, we developed a novel mouse model utilizing somatic brain transgenesis with adeno-associated virus (AAV) to drive tau expression in vivo, and validated the model by confirming the distinct biochemical features of A152T tau in postmortem brain tissue from human carriers. Specifically, Tau-AAV mice exhibited increased tau phosphorylation that unlike animals expressing the pathogenic P301L mutation remained localized to the soluble fraction. Read More

Mov Disord Clin Pract 2018 Nov-Dec;5(6):603-606. Epub 2018 Nov 8.

Department of Neurology Rutgers Robert Wood Johnson Medical School New Brunswick New Jersey USA.

Background: Progressive supranuclear palsy (PSP) is a neuropathologically defined disease with a broad clinical spectrum. It can initially be mistaken for other neurodegenerative diseases. Diagnosis of PSP earlier in the course may reduce its psychological and financial burden, permit earlier access to neuroprotective interventions, and avoid unnecessary diagnostic and therapeutic measures. Read More

Mol Psychiatry 2019 Jan 11. Epub 2019 Jan 11.

Division of Clinical Geriatrics, Center for Alzheimer Research, Department of Neurobiology, Care Sciences and Society, Karolinska Institutet, Stockholm, Sweden.

The accumulation of pathological misfolded tau is a feature common to a collective of neurodegenerative disorders known as tauopathies, of which Alzheimer's disease (AD) is the most common. Related tauopathies include progressive supranuclear palsy (PSP), corticobasal syndrome (CBS), Down's syndrome (DS), Parkinson's disease (PD), and dementia with Lewy bodies (DLB). Investigation of the role of tau pathology in the onset and progression of these disorders is now possible due the recent advent of tau-specific ligands for use with positron emission tomography (PET), including first- (e. Read More

Neurol Neurochir Pol 2019 Jan 8. Epub 2019 Jan 8.

Department of Neurology, Medical University of Warsaw, 8 Kondratowicza str, 03-242 Warsaw, Poland.

Clinical Rationale For The Study: Autonomic nervous system (ANS) involvement in different parkinsonian syndromes has been frequently discussed. It is well established in multiple system atrophy (MSA), whereas it is less evident in progressive supranuclear palsy (PSP).

Aims Of The Study: The aims were to assess the presence and pattern of ANS involvement in MSA and PSP using noninvasive tests i. Read More

Front Cell Dev Biol 2018 20;6:163. Epub 2018 Dec 20.

PROTECT, INSERM U1141, Université Paris Diderot, Sorbonne Paris Cité, Paris, France.

Microtubule-associated protein tau (MAPT) hyperphosphorylation and aggregation, are two hallmarks of a family of neurodegenerative disorders collectively referred to as tauopathies. In many tauopathies, including Alzheimer's disease (AD), progressive supranuclear palsy (PSP) and Pick's disease, tau aggregates are found associated with highly sulfated polysaccharides known as heparan sulfates (HSs). In AD, amyloid beta (Aβ) peptide aggregates associated with HS are also characteristic of disease. Read More

Cerebellum 2019 Jan 8. Epub 2019 Jan 8.

Department of Neurodegenerative Diseases, Hertie-Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany.

Falls in patients with neurodegenerative diseases (NDDs) have enormous detrimental consequences. A better understanding of the interplay between physical activity (PA) and fall risk might help to reduce fall frequency. We aimed to investigate the association between sensor-based PA and fall risk in NDDs, using "falls per individual PA exposure time" as a novel measure. Read More

Acta Neuropathol Commun 2019 Jan 3;7(1). Epub 2019 Jan 3.

King's College London, Institute of Psychiatry, Psychology & Neuroscience, Department of Basic and Clinical Neuroscience, Maurice Wohl Clinical Neuroscience Institute (K1.24), 5 Cutcombe Road, London, SE5 9RX, UK.

Human tauopathies including Alzheimer's disease, progressive supranuclear palsy and related disorders, are characterized by deposition of pathological forms of tau, synaptic dysfunction and neuronal loss. We have previously identified a pathogenic C-terminal tau fragment (Tau35) that is associated with human tauopathy. However, it is not known how tau fragmentation affects critical molecular processes in cells and contributes to impaired physiological function. Read More

J Neurol Neurosurg Psychiatry 2018 Dec 31. Epub 2018 Dec 31.

Reta Lila Weston Institute of Neurological Studies, UCL Queen Square Institute of Neurology, London, UK

Background: Development of autonomic failure is associated with more rapid disease course and shorter survival in patients with Parkinson's disease and multiple system atrophy. However, autonomic symptoms have not been specifically assessed as a prognostic factor in progressive supranuclear palsy (PSP). We evaluated whether development of autonomic symptoms is associated with disease progression and survival in PSP. Read More

Alzheimers Dement (N Y) 2018 17;4:746-755. Epub 2018 Dec 17.

Global R&D Partners, LLC, San Diego, CA, USA.

Introduction: Extracellular tau is hypothesized to mediate the onset and progression of tauopathies, including Alzheimer's disease, progressive supranuclear palsy, and a subset of frontotemporal lobar degenerations. A putative strategy for treating these disorders is to reduce extracellular tau levels using tau-directed immunotherapy. The results of the first-in-human study of BIIB092 (formerly BMS-986168/IPN007), a humanized monoclonal antibody that binds to N-terminal tau, are reported here. Read More

Neurosci Lett 2018 Dec 21;696:132-139. Epub 2018 Dec 21.

Banner Sun Health Research Institute, Sun City, AZ, United States.

Many studies have been directed at understanding mechanisms of tau aggregation and therapeutics, nearly all focusing on the brain. It is critical to understand the presence of tau in peripheral tissues since this may provide new insights into disease progression and selective vulnerability. The current study sought to determine the presence of select tau species in peripheral tissues in elderly individuals and across an array of tauopathies. Read More

Mov Disord 2018 Dec 21. Epub 2018 Dec 21.

Parkinson Institute, Azienda Socio Sanitaria Territoriale (ASST) Gaetano Pini-CTO, Milan, Italy.

Background: Although several studies have suggested that abnormalities in gut microbiota may play a critical role in the pathogenesis of PD, data are still extremely heterogeneous.

Methods: 16S gene ribosomal RNA sequencing was performed on fecal samples of 350 individuals, subdivided into idiopathic PD (n = 193, of whom 39 were drug naïve) stratified by disease duration, PSP (n = 22), MSA (n = 22), and healthy controls (HC; n = 113). Several confounders were taken into account, including dietary habits. Read More

Curr Top Behav Neurosci 2018 Dec 21. Epub 2018 Dec 21.

South Tees Hospitals NHS Foundation Trust, The James Cook University Hospital, Middlesbrough, UK.

Progressive supranuclear palsy is often considered a disease of the motor system and is characterised by a profound oculomotor impairment. The oculomotor system is also known to be fundamentally important in cognitive processes such as attention and working memory, but the way in which these functions are affected by PSP is not well understood. In this chapter we outline the pathology and typical presentation of PSP, with a focus on the oculomotor impairment, briefly outline the role of the oculomotor system in spatial cognition and discuss some key studies examining spatial attention and memory in PSP. Read More

BMJ Case Rep 2018 Nov 28;11(1). Epub 2018 Nov 28.

Departments of Neurology and Pathology, Oregon Health & Science University, Portland, Oregon, USA.

We report a case of a progressive supranuclear palsy-like phenotype with rapidly progressive dementia and prominent language and executive dysfunction. Pathological examination revealed no midbrain or white matter tauopathy, but rather chronic meningoencephalitis and other mixed pathology. The cerebrospinal fluid (CSF) in this case showed a novel antibody against central nervous system and renal tissue. Read More

Parkinsonism Relat Disord 2018 Dec 11. Epub 2018 Dec 11.

Neuroimaging Research Unit, Institute of Molecular Bioimaging and Physiology, National Research Council, Catanzaro, Italy; Neuroscience Research Centre, Magna Græcia University, Catanzaro, Italy. Electronic address:

Introduction: Several structural and functional neuroimaging studies have shown that the Supplementary Motor Area (SMA) is affected by tau pathology in patients with Progressive Supranuclear Palsy (PSP). The aim of the study was to investigate the biochemical profile of SMA in PSP patients, using proton magnetic resonance spectroscopy (H-MRS).

Methods: Sixteen PSP patients and 18 healthy controls participated in this study. Read More

Acta Neuropathol 2019 Feb 13;137(2):279-296. Epub 2018 Dec 13.

Institute of Neuroscience and Physiology, Department of Psychiatry and Neurochemistry, The Sahlgrenska Academy at University of Gothenburg, Göteborgsvägen 31, House V3/SU, 43180, Mölndal, Sweden.

Tau is an axonal microtubule-binding protein. Tau pathology in brain and increased tau concentration in the cerebrospinal fluid (CSF) are hallmarks of Alzheimer's disease (AD). Most of tau in CSF is present as fragments. Read More

Transl Psychiatry 2018 Dec 13;8(1):265. Epub 2018 Dec 13.

Department of Psychiatry, Washington University School of Medicine, 660S. Euclid Ave. Campus Box 8134, St. Louis, MO, 63110, USA.

Mutations in the microtubule-associated protein tau (MAPT) gene cause autosomal dominant frontotemporal lobar degeneration with tau inclusions (FTLD-tau). MAPT p.R406W carriers present clinically with progressive memory loss and neuropathologically with neuronal and glial tauopathy. Read More

J Neurol 2019 Feb;266(2):545-547

Department of Neurology, Institute of Psychological Medicine and Clinical Neurosciences, Cardiff University, Cardiff, CF14 4XW, UK.

Ann Indian Acad Neurol 2018 Oct-Dec;21(4):345-346

Department of Neurology, Seoul National University Hospital, Seoul, Republic of Korea.

JAMA Neurol 2018 Dec 3. Epub 2018 Dec 3.

Department of Psychiatry and Neurochemistry, The Sahlgrenska Academy, University of Gothenburg, Mölndal, Sweden.

Importance: Neuronal and axonal destruction are hallmarks of neurodegenerative diseases, but it is difficult to estimate the extent and progress of the damage in the disease process.

Objective: To investigate cerebrospinal fluid (CSF) levels of neurofilament light (NFL) protein, a marker of neuroaxonal degeneration, in control participants and patients with dementia, motor neuron disease, and parkinsonian disorders (determined by clinical criteria and autopsy), and determine its association with longitudinal cognitive decline.

Design, Setting, And Participants: In this case-control study, we investigated NFL levels in CSF obtained from controls and patients with several neurodegenerative diseases. Read More

J Neurol 2019 Feb 1;266(2):330-338. Epub 2018 Dec 1.

Neurologische Klinik und Poliklinik, Ludwig Maximilians Universität München, Munich, Germany.

The applause sign, i.e., the inability to execute the same amount of claps as performed by the examiner, was originally reported as a sign specific for progressive supranuclear palsy (PSP). Read More

Parkinsonism Relat Disord 2018 Nov 20. Epub 2018 Nov 20.

Department of Neurology, Kyoto Prefectural University of Medicine, Kyoto, 602-0841, Japan; Department of Molecular Pathobiology of Brain Diseases, Kyoto Prefectural University of Medicine, Kyoto, 602-0841, Japan. Electronic address:

Introduction: There is still a substantial unmet need for blood-based biomarkers to make an objective diagnosis of Parkinson's disease (PD) and the parkinsonism-plus syndromes. This study is aimed to determine whether enumeration of brain-derived exosomes (BDEs) in plasma is informative in the diagnosis of those diseases.

Methods: We have developed a specific method to enumerate the plasma levels of neuron-derived, astrocyte-derived, and oligodendrocyte-derived exosomes (NDEs, ADEs and ODEs, respectively), and quantified them individually in patients with PD (n = 15), multiple system atrophy (MSA, n = 15), progressive supranuclear palsy (PSP, n = 7) and disease controls (n = 15). Read More

Parkinsonism Relat Disord 2018 Nov 5. Epub 2018 Nov 5.

Center for Neurological Restoration, Cleveland Clinic, Cleveland, OH, USA.

The proper diagnosis of parkinsonian disorders usually involves three steps: identifying core features of parkinsonism; excluding other causes; and collating supportive evidence based on clinical signs or investigations. While the recognition of cardinal parkinsonian features is usually straightforward, the appreciation of clinical features suggestive of specific parkinsonian disorders can be challenging, and often requires greater experience and skills. In this review, we outline the clinical features that are relevant to the differential diagnosis of common neurodegenerative parkinsonian disorders, including Parkinson's disease, multiple system atrophy, progressive supranuclear palsy, and corticobasal degeneration. Read More

J Int Neuropsychol Soc 2019 Feb 3;25(2):195-203. Epub 2018 Dec 3.

4Clinical Memory Research Unit, Department of Clinical Sciences Malmö,Lund University,Lund,Sweden.

Objectives: The aim of this study was to assess the psychometric properties of a Swedish version of the Hayling test (HT-S) and its clinical utility in a group of patients with different frontotemporal dementia (FTD) syndromes. Early diagnosis of FTD is a challenge and requires a broad arsenal of assessment methods, neuropsychological tests not the least. The Hayling test assesses executive functions including initiation, efficiency and response inhibition. Read More

Parkinsonism Relat Disord 2018 Nov 26. Epub 2018 Nov 26.

Department of Neurology, University of Alabama at Birmingham, Birmingham, AL, USA; Alzheimer's Disease Center, University of Alabama at Birmingham, Birmingham, AL, USA; Evelyn F. McKnight Brain Institute, University of Alabama at Birmingham, Birmingham, AL, USA; Center for Neurodegeneration and Experimental Therapeutics, University of Alabama at Birmingham, Birmingham, AL, USA.

Introduction: Cognitive dysfunction is common in progressive supranuclear palsy (PSP) but its effect on medical decision-making has not been well studied. To address this gap in the research literature, we compared the medical decision-making capacity of patients with PSP to groups of patients with other neurodegenerative disorders. We also investigated the cognitive correlates of medical decision-making in our PSP sample. Read More

Mov Disord 2019 Feb 28;34(2):218-227. Epub 2018 Nov 28.

Sorbonne University, Univ. Pierre & Marie Curie Paris 06, Cnrs, Inserm, AP-HP Pitié-Salpêtrière hospital, Brain and Spinal Cord Institute, Paris, France.

Background: Deep brain stimulation of the pedunculopontine nucleus has been performed to treat dopamine-resistant gait and balance disorders in patients with degenerative diseases. The outcomes, however, are variable, which may be the result of the lack of a well-defined anatomical target.

Objectives: The objectives of this study were to identify the main neuronal populations of the pedunculopontine and the cuneiform nuclei that compose the human mesencephalic locomotor region and to compare their 3-dimensional distribution with those found in patients with Parkinson's disease and progressive supranuclear palsy. Read More

Handb Clin Neurol 2018 ;159:195-203

Brain and Mind Centre, University of Sydney Medical School, Sydney, NSW, Australia.

Frontotemporal dementia (FTD) and related diseases are important causes of younger-onset dementia. Falls may be a source of morbidity and mortality in FTD, but remain underreported, and very few high-quality studies have been performed. In this chapter, we briefly review the clinical features of FTD and related syndromes such as motor neuron disease (MND) and atypical parkinsonian syndromes, such as progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). Read More

Hum Brain Mapp 2018 Nov 26. Epub 2018 Nov 26.

Department of Medical and Surgical Sciences, Institute of Neurology, Magna Graecia University, Catanzaro, Italy.

Progressive supranuclear palsy (PSP) is a neurodegenerative disorder characterized by white matter (WM) changes in different supra- and infratentorial brain structures. We used track density imaging (TDI) to characterize WM microstructural alterations in patients with PSP-Richardson's Syndrome (PSP-RS). Moreover, we investigated the diagnostic utility of TDI in distinguishing patients with PSP-RS from those with Parkinson's disease and healthy controls (HC). Read More

Mov Disord 2019 Jan 23;34(1):105-113. Epub 2018 Nov 23.

Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.

Background: Elevated uptake of the [ F]AV-1451 tau-PET ligand has been observed cross-sectionally in subjects with progressive supranuclear palsy (PSP). However, it is unknown how the ligand performs longitudinally in PSP. We aimed to determine how regional measures of change on [ F]AV-1451 PET perform as longitudinal biomarkers of PSP compared with the more established biomarker of rate of midbrain atrophy. Read More

Can J Neurol Sci 2019 Jan 20;46(1):132-136. Epub 2018 Nov 20.

Division of Neurology, Department of Medicine,University of Alberta,Edmonton, Alberta,Canada.

Front Neurosci 2018 2;12:798. Epub 2018 Nov 2.

Institute of Neuroimmunology, Slovak Academy of Sciences, Bratislava, Slovakia.

Neurofibrillary pathology comprised of pathological tau protein is closely tied to a range of neurodegenerative disorders, the most common of which is Alzheimer's disease. While they are individually rarer, a range of other disorders, the tauopathies (including Pick's disease, progressive supranuclear palsy, corticobasal degeneration, primary progressive aphasia, and ∼50% of behavioral variant frontotemporal dementia cases) display pronounced underlying tau pathology. In all cases, the distribution and amount of tau pathology closely correlates with the severity and phenotype of cognitive impairment, and with the pattern and degree of brain atrophy. Read More

Dement Geriatr Cogn Disord 2018 13;46(5-6):285-297. Epub 2018 Nov 13.

Centre for Neuroscience of Speech, The University of Melbourne, Melbourne, Victoria,

Background: Frontotemporal lobar degeneration (FTLD) can result in a decline in behavior, language, and motor function. Mealtime disturbances are a common and significant outcome of FTLD. Disturbances during mealtimes can arise from dysphagia or may occur secondary to behavioral changes such as rapid eating, mealtime rigidity, and altered diet preferences. Read More

Neuropathol Appl Neurobiol 2019 Feb 3;45(1):81-87. Epub 2018 Dec 3.

Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, Queen Square, London, UK.

Frontotemporal dementias (FTDs) are clinically, genetically and pathologically heterogeneous neurodegenerative disorders that affect the frontal and anterior temporal lobes of the brain. They are relatively common causes of young-onset dementia and usually present with behavioural disturbance (behavioural variant FTD) or language impairment (primary progressive aphasia), but there is also overlap with motor neurone disease and the atypical parkinsonian disorders, corticobasal syndrome and progressive supranuclear palsy. At post mortem, neuronal inclusions containing tau, TDP-43 or infrequently FUS protein are seen in most cases. Read More

Parkinsonism Relat Disord 2018 Oct 6. Epub 2018 Oct 6.

Department of Neurology, Santa Maria University Hospital, Terni, Italy.

To establish a clinical diagnosis of a parkinsonian disorder, physicians rely on their ability to identify relevant red flags, in addition to cardinal features, to support or refute their working diagnosis in an individual patient. The term 'red flag', was originally coined in 1989 to define the presence of non-cardinal features that may raise a suspicion of multiple system atrophy (MSA), or at least suggest alternative diagnosis to Parkinson's disease (PD). Since then, the term 'red flag', has been consistently used in the literature to denote the clinical history or signs that may signal to physicians the possibility of an atypical parkinsonian disorder (APD). Read More