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Front Neurol 2019 3;10:172. Epub 2019 Apr 3.

German Center for Vertigo and Balance Disorders, Ludwig-Maximilians University, Munich, Germany.

To review current knowledge of the perception of verticality, its normal function and disorders. This is based on an integrative graviceptive input from the vertical semicircular canals and the otolith organs. The special focus is on human psychophysics, neurophysiological and imaging data on the adjustments of subjective visual vertical (SVV) and the subjective postural vertical. Read More

J Proteomics 2019 Apr 15. Epub 2019 Apr 15.

Clinical Neuroproteomics Unit, Navarrabiomed, Complejo Hospitalario de Navarra (CHN), Universidad Pública de Navarra (UPNA), Irunlarrea, 3, 31008 Pamplona, Spain; Proteored-ISCIII, Proteomics Unit, Navarrabiomed, Complejo Hospitalario de Navarra (CHN), Universidad Pública de Navarra (UPNA), Irunlarrea 3, 31008 Pamplona, Spain; IdiSNA, Navarra Institute for Health Research, Pamplona, Spain, Irunlarrea 3, 31008 Pamplona, Spain. Electronic address:

Mild olfactory dysfunction has been observed in frontotemporal dementias (FTD). However, the underlying molecular mechanisms associated to this deficit are poorly understood. We applied quantitative proteomics to analyze pathological effects on the olfactory bulb (OB) from progressive supranuclear palsy (PSP) and frontotemporal lobar degeneration (FTLD-TDP43) subjects respect to elderly non-FTD group. Read More

J Neurol 2019 Apr 15. Epub 2019 Apr 15.

Department of Medicine, Surgery and Dentistry, Neuroscience Section, Center for Neurodegenerative Diseases (CEMAND), University of Salerno, 84131, Baronissi (Salerno), Italy.

Introduction: Movement Disorder Society (MDS) new diagnostic criteria for Progressive Supranuclear palsy (PSP) identifying different disease phenotypes were recently released. The aim of the present study is to report on the cognitive and behavioral features of the different phenotypes diagnosed according to the MDS criteria.

Methods: Forty-nine PSP patients underwent an extensive battery of clinical assessments. Read More

Sci Rep 2019 Apr 15;9(1):6079. Epub 2019 Apr 15.

Clinical Memory Research Unit, Department of Clinical Sciences, Lund University, 212 24, Malmö, Sweden.

There is a need for methods that distinguish Parkinson's disease (PD) from progressive supranuclear palsy (PSP) and multiple system atrophy (MSA), which have similar characteristics in the early stages of the disease. In this prospective study, we evaluate mapping of apparent susceptibility based on susceptibility weighted imaging (SWI) for differential diagnosis. We included 134 patients with PD, 11 with PSP, 10 with MSA and 44 healthy controls. Read More

J Nucl Med 2019 Apr 12. Epub 2019 Apr 12.

University of Turku.

Multiple system atrophy (MSA), progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) have overlapping signs and symptoms with Parkinson's disease (PD), and these similarities complicate their clinical diagnostics. Although presynaptic dopaminergic brain imaging with PET and SPECT is clinically widely used for patients with suspected PD, the benefit of functional imaging in atypical parkinsonism syndromes remains unclear. We compared striatal presynaptic dopaminergic function in MSA parkinsonism variant (MSA-P), MSA cerebellar variant (MSA-C), PSP, CBS and PD using combined quantitative data from all published studies. Read More

Expert Rev Neurother 2019 Apr 11. Epub 2019 Apr 11.

g Department of Neurological Sciences , Santa Maria University Hospital , Terni , Italy.

Introduction: Tauopathies are heterogeneous clinicopathological entities characterized by abnormal neuronal and/or glial inclusions of the microtubule-binding protein tau. Primary tauopathies considered to be diseases correspond to a major class of frontotemporal lobar degeneration (FTLD) neuropathology (FTLD-Tau), including several forms of frontotemporal dementia (FTD) clinical syndromes. Little progress has been made in the past 20 years in developing effective disease-modifying drugs for primary tauopathies and available symptomatic treatments have limited efficacy. Read More

Acta Neurol Scand 2019 Mar 23. Epub 2019 Mar 23.

Department of Neurology, Dokkyo Medical University, Mibu, Japan.

Background: We investigated serum insulin-like growth factor (IGF)-1 levels in patients with neurodegenerative diseases and correlated these levels with clinical parameters.

Methods: One hundred and fifty-six patients with neurodegenerative diseases were included in this study, and serum IGF-1 levels were determined.

Results: Serum IGF-1 levels (mean ± standard error) were not significantly different among the patients with different neurodegenerative diseases: Parkinson's disease (PD; n = 73), 112. Read More

BMC Neurol 2019 Mar 20;19(1):42. Epub 2019 Mar 20.

Department of Pathology and Molecular Medicine Third Faculty of Medicine, Charles University and Thomayer Hospital, Videnska 800, 14059, Prague 4 - Krc, Czech Republic.

Background: We aimed to produce a detailed neuropathological analysis of pyramidal motor system pathology and provide its clinical pathological correlation in cases with definite progressive supranuclear palsy (PSP).

Methods: Pyramidal motor system pathologies were analyzed in 18 cases with neuropathologically confirmed PSP. Based on a retrospective clinical analysis, cases were subtyped according to Movement Disorder Society criteria for clinical diagnosis of PSP as probable, possible or suggestive of PSP with Richardson's syndrome (n = 10), PSP with predominant corticobasal syndrome (n = 3), PSP with predominant parkinsonism (n = 3), PSP with predominant speech/language disorder (n = 1), and PSP with progressive gait freezing (n = 1). Read More

Mov Disord 2019 Mar 20. Epub 2019 Mar 20.

Department of Functional Brain Imaging Research (DOFI), Clinical Research Cluster, National Institute of Radiological Sciences (NIRS), National Institutes for Quantum and Radiological Science and Technology (QST), Chiba, Chiba, Japan.

Background: [ C]pyridinyl-butadienyl-benzothiazole 3 is a PET imaging agent designed for capturing pathological tau aggregates in diverse neurodegenerative disorders, and would be of clinical utility for neuropathological investigations of PSP.

Objectives: To explore the usefulness of [ C]pyridinyl-butadienyl-benzothiazole 3/PET in assessing characteristic distributions of tau pathologies and their association with clinical symptoms in the brains of living PSP patients.

Methods: We assessed 13 PSP patients and 13 age-matched healthy control subjects. Read More

Mov Disord 2019 Mar 18. Epub 2019 Mar 18.

Department of Neurology, Technische Universität München, Munich, Germany.

Background: The Movement Disorder Society criteria for progressive supranuclear palsy define diagnostic allocations, stratified by certainty levels and clinical predominance types. We aimed to study the frequency of ambiguous multiple allocations and to develop rules to eliminate them.

Methods: We retrospectively collected standardized clinical data by chart review in a multicenter cohort of autopsy-confirmed patients with progressive supranuclear palsy, to classify them by diagnostic certainty level and predominance type and to identify multiple allocations. Read More

Cortex 2019 Feb 22;115:294-308. Epub 2019 Feb 22.

NEtS Center, School of Advanced Studies IUSS Pavia, Pavia, Italy; IRCCS San Giovanni di Dio Fatebenefratelli, Brescia, Italy. Electronic address:

A progressive speech/language disorder, such as the non fluent/agrammatic variant of primary progressive aphasia and progressive apraxia of speech, can be due to neuropathologically verified Progressive Supranuclear Palsy (PSP). The prevalence of linguistic deficits and the linguistic profile in PSP patients who present primarily with a movement disorder is unknown. In the present study, we investigated speech and language performance in a sample of clinically diagnosed PSP patients using a comprehensive language battery, including, besides traditional language tests, a detailed analysis of connected speech (picture description task assessing 26 linguistic features). Read More

JAMA Neurol 2019 Mar 18. Epub 2019 Mar 18.

Department of Neuroscience, Mayo Clinic, Jacksonville, Florida.

Importance: The association between the microtubule-associated protein tau (MAPT) H1 haplotype and the risk of progressive supranuclear palsy (PSP) has been well documented. However, the specific H1 subhaplotypes that drive the association have not been evaluated in large studies, nor have they been studied in relation to neuropathologic severity of disease.

Objective: To comprehensively evaluate the associations of MAPT haplotypes with the risk of PSP and the severity of tau pathology using a large series of neuropathologically confirmed PSP cases. Read More

J Neurol Neurosurg Psychiatry 2019 Mar 13. Epub 2019 Mar 13.

Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, University College London, London, UK.

Objective: The high degree of clinical overlap between atypical parkinsonian syndromes (APS) and Parkinson's disease (PD) makes diagnosis challenging. We aimed to identify novel diagnostic protein biomarkers of APS using multiplex proximity extension assay (PEA) testing.

Methods: Cerebrospinal fluid (CSF) samples from two independent cohorts, each consisting of APS and PD cases, and controls, were analysed for neurofilament light chain (NF-L) and Olink Neurology and Inflammation PEA biomarker panels. Read More

PLoS One 2019 13;14(3):e0213666. Epub 2019 Mar 13.

Elton Laboratory for Molecular Neuroendocrinology, Lily and Avraham Gildor Chair for the Investigation of Growth Factors, Department of Human Molecular Genetics and Biochemistry, Sackler Faculty of Medicine, Sagol School of Neuroscience and Adams Super Center for Brain Studies, Tel Aviv University, Tel Aviv, Israel.

The microtubule (MT) associated protein Tau is instrumental for the regulation of MT assembly and dynamic instability, orchestrating MT-dependent cellular processes. Aberration in Tau post-translational modifications ratio deviation of spliced Tau isoforms 3 or 4 MT binding repeats (3R/4R) have been implicated in neurodegenerative tauopathies. Activity-dependent neuroprotective protein (ADNP) is vital for brain formation and cognitive function. Read More

Pac Symp Biocomput 2019 ;24:260-271

Departments of Computer Science, Stanford University, Stanford, CA 94305, USA.

Autism spectrum disorder (ASD) is a heritable neurodevelopmental disorder affecting 1 in 59 children. While noncoding genetic variation has been shown to play a major role in many complex disorders, the contribution of these regions to ASD susceptibility remains unclear. Genetic analyses of ASD typically use unaffected family members as controls; however, we hypothesize that this method does not effectively elevate variant signal in the noncoding region due to family members having subclinical phenotypes arising from common genetic mechanisms. Read More

Acta Neuropathol Commun 2019 Mar 11;7(1):37. Epub 2019 Mar 11.

Department of Neurology, Massachusetts General Hospital, WACC, Suite 715, 15th Parkman St., Boston, MA, 02114, USA.

[F-18]-MK-6240, a novel tau positron emission tomography (PET) tracer recently discovered for the in vivo detection of neurofibrillary tangles, has the potential to improve diagnostic accuracy in the detection of Alzheimer disease. We have examined regional and substrate-specific binding patterns as well as possible off-target binding of this tracer on human brain tissue to advance towards its validation. We applied [F-18]-MK-6240 phosphor screen and high resolution autoradiography to postmortem samples from patients with a definite pathological diagnosis of Alzheimer disease, frontotemporal lobar degeneration-tau (Pick's disease, progressive supranuclear palsy and corticobasal degeneration), chronic traumatic encephalopathy, frontotemporal lobar degeneration-Tar DNA-binding protein 43 (TDP-43), dementia with Lewy bodies, cerebral amyloid angiopathy and elderly controls free of pathologic changes of neurodegenerative disease. Read More

Psychogeriatrics 2019 Mar 10. Epub 2019 Mar 10.

Neurobehavioral and Robotic Rehabilitation Unit, IRCCS, Scientific Institute of Research and Care 'Bonino Pulejo', Messina, Italy.

Acta Neuropathol Commun 2019 Mar 7;7(1):36. Epub 2019 Mar 7.

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

Tauopathies are neurodegenerative disorders characterized by aggregation of microtubule associated tau protein in neurons and glia. They are clinically and pathologically heterogeneous depending on the isoform of tau protein that accumulates (three or four 31-to-32-amino-acid repeats [3R or 4R] in the microtubule binding domain), as well as the cellular and neuroanatomical distribution of tau pathology. Growing evidence suggests that distinct tau conformers may contribute to the characteristic features of various tauopathies. Read More

Neurology 2019 Apr 6;92(14):e1547-e1557. Epub 2019 Mar 6.

From the Departments of Clinical Neurosciences (C.J.L., I.T.S.C.-G., P.V.R., A.W., E.W., J.B.R.) and Psychology (T.W.R.), and Behavioral and Clinical Neuroscience Institute (T.W.R., J.B.R.), University of Cambridge, UK; University Medical Centre Hamburg-Eppendorf (E.W.), University of Hamburg, Germany; and MRC Cognition and Brain Sciences Unit (J.B.R.), Cambridge, UK.

Objective: To determine the influence of apathy, impulsivity, and behavioral change on survival in patients with frontotemporal dementia, progressive supranuclear palsy, and corticobasal syndrome.

Methods: We assessed 124 patients from the epidemiologic PiPPIN (Pick's Disease and Progressive Supranuclear Palsy, Prevalence and Incidence) study. Patients underwent detailed baseline cognitive and behavioral assessment focusing on apathy, impulsivity, and behavioral change. Read More

Front Neurol 2019 19;10:116. Epub 2019 Feb 19.

Healthscope and La Trobe Centre for Sport and Exercise Medicine Research, School of Allied Health, La Trobe University, Melbourne, VIC, Australia.

To understand the benefits and feasibility of using supervised, home-based, music-cued training to improve gait speed and stability in community-dwelling people with Progressive Supranuclear Palsy. Feasibility trial incorporating a single group repeated-measures design. Human movement laboratory and participants' homes. Read More

Front Neurol 2019 14;10:101. Epub 2019 Feb 14.

Department of Neurology, Medical University of Warsaw, Warsaw, Poland.

Neuroimaging in the context of examining atypical parkinsonian tauopathies is an evolving matter. Positron Emission Tomography (PET) and Single Photon Emission Computed Tomography (SPECT) bring tools, which may be reasonable in supplementary examination, however cannot be interpreted as a gold standard for correct diagnosis. The review presents advantages and limitations of tau radiotracers in PET, metabolic PET and perfusion SPECT. Read More

Neuropsychologia 2019 Apr 2;127:148-157. Epub 2019 Mar 2.

Neuropsychology Research Unit, School of Psychology, The University of Queensland, St. Lucia, Brisbane, QLD, 4072, Australia; Queensland Brain Institute, The University of Queensland, St. Lucia, Brisbane, QLD, 4072, Australia; Neurology Department, Royal Brisbane and Women's Hospital, Herston, Brisbane, Australia. Electronic address:

Progressive supranuclear palsy (PSP) is an atypical parkinsonian disorder that can present with language production deficits in addition to the characteristic progressive parkinsonian motor symptoms. Although typical parkinsonism treatments such as pharmacotherapy are not effective in PSP, non-invasive brain stimulation techniques such as transcranial direct current stimulation (tDCS) have shown promise for treating cognitive deficits relating to this disorder. We report the case of KN, who presented with reduced verbal fluency and connected speech production in the context of PSP. Read More

J Neurol 2019 Mar 5. Epub 2019 Mar 5.

School of Psychology, The University of Sydney, Sydney, Australia.

The objective of the study is to determine the utility of a simple reaction time task as a marker of general cognitive decline across the frontotemporal lobar degeneration (FTLD) spectrum and in Alzheimer's disease (AD). One hundred and twelve patients presenting with AD or FTLD affecting behaviour (behavioural-variant frontotemporal dementia), language (progressive non fluent aphasia, logopenic progressive aphasia, semantic dementia) or motor function (corticobasal syndrome, progressive supranuclear palsy, frontotemporal dementia-motor neuron disease) and 25 age-matched healthy controls completed the Psychomotor Vigilance Task (PVT), a 3-min reaction time (RT) task. The proportion of lapses (RT > 500 ms) was significantly increased in dementia patients compared to healthy controls, except for semantic dementia, and correlated with all cognitive functions except language. Read More

Acta Neuropathol Commun 2019 Mar 4;7(1):34. Epub 2019 Mar 4.

Department of Pathology and Laboratory Medicine, Institute on Aging and Center for Neurodegenerative Disease Research, 3600 Spruce St. 3 Maloney, Philadelphia, PA, 19104, USA.

Pathological tau aggregates in Alzheimer's disease (AD) and frontotemporal lobar degeneration-tau (FTLD-tau) adopt distinct conformations differentiated by the AD-tau specific monoclonal antibody (mAb) GT-38 that are not readily visualized using phosphorylation-specific anti-tau mAbs. To determine the extent of co-morbid AD-tau pathology in FTLD-tau, we performed immunohistochemical (IHC) staining with GT-38 and assigned Braak stages of AD-tau in a cohort 180 FTLD-tau cases consisting of corticobasal degeneration (CBD; n = 49), progressive supranuclear palsy (PSP; n = 109), and Pick's disease (PiD; n = 22). Nearly two-thirds of patients (n = 115 of 180, 63. Read More

Neuroimage Clin 2019 Feb 19;22:101722. Epub 2019 Feb 19.

Max Planck Institute for Human Cognitive and Brain Sciences Leipzig, Germany; Clinic of Cognitive Neurology, University of Leipzig & FTLD Consortium Germany, Germany. Electronic address:

Objective: Progressive supranuclear palsy (PSP) is an atypical parkinsonian syndrome characterized by vertical gaze palsy and postural instability. Midbrain atrophy is suggested as a hallmark, but it has not been validated systematically in whole-brain imaging.

Methods: We conducted whole-brain meta-analyses identifying disease-related atrophy in structural MRI. Read More

Can J Neurol Sci 2019 Mar 1;46(2):174-183. Epub 2019 Mar 1.

Department of Medical Neuroscience,Dalhousie University,Halifax, Nova Scotia,Canada.

Background: Primary Progressive Aphasia (PPA) is a syndrome characterized by an isolated impairment of language function at disease onset. The cholinergic system is implicated in language function and cholinergic deficits are seen in the brains of individuals with PPA. One major source of cholinergic innervation of the cerebral cortex is the nucleus basalis of Meynert (NBM) within which lies the nucleus subputaminalis (NSP). Read More

Neurology 2019 Mar 27;92(13):e1479-e1486. Epub 2019 Feb 27.

From the Department of Neurology (T.M.M., A.v.R., H.B.K., R.A.J.E., B.R.B., M.M.V.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen; Department of Laboratory Medicine (T.M.M., H.B.K., M.M.V.), Radboud University Medical Center, Nijmegen; Parkinson Center Nijmegen (T.M.M., A.v.R., R.A.J.E., B.R.B., M.M.V.), the Netherlands; and Department of Neurology (P.O., M.O.), Ulm University Hospital, Germany.

Objective: To investigate the diagnostic value of serum neurofilament light chain (NFL) in patients with clear signs of parkinsonism but whose specific diagnosis was yet uncertain.

Methods: Serum samples were collected from patients with clear signs of parkinsonism but with uncertain diagnosis at the inclusion. Clinical diagnoses of Parkinson disease (PD) and atypical parkinsonism disorders (APDs) were established after 3 years of follow-up and updated again after a maximum of 12 years in case longer follow-up data were available. Read More

Mol Neurodegener 2019 02 27;14(1):11. Epub 2019 Feb 27.

Department of Neurology, Hope Center for Neurological Disorders, Knight Alzheimer's Disease Research Center, Washington University in St. Louis, St. Louis, MO, 63110, USA.

Background: Alzheimer's disease is characterized by two main neuropathological hallmarks: extracellular plaques of amyloid-β (Aβ) protein and intracellular aggregates of tau protein. Although tau is normally a soluble monomer that bind microtubules, in disease it forms insoluble, hyperphosphorylated aggregates in the cell body. Aside from its role in AD, tau is also involved in several other neurodegenerative disorders collectively called tauopathies, such as progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), some forms of frontotemporal dementia, and argyrophilic grain disease (AGD). Read More

Front Neurol 2019 12;10:74. Epub 2019 Feb 12.

Department of Neurology, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea.

Recent data suggest mechanistic links among perturbed iron homeostasis, oxidative stress, and misfolded protein aggregation in neurodegenerative diseases. Iron overload and toxicity toward dopaminergic neurons have been established as playing a role in the pathogenesis of Parkinson's disease (PD). Brain iron accumulation has also been documented in atypical parkinsonian syndromes (APS), mainly comprising multiple system atrophy (MSA), and progressive supranuclear palsy (PSP). Read More

Parkinsonism Relat Disord 2019 Feb 19. Epub 2019 Feb 19.

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

Introduction: We investigated the imaging counterpart of two functional domains (ocular motor dysfunction and postural instability) in progressive supranuclear palsy (PSP) patients classified according to the new clinical diagnostic criteria.

Methods: Forty-eight patients with probable PSP-Richardson's syndrome (PSP-RS), 30 with probable PSP-parkinsonism (PSP-P), 37 with Parkinson's disease (PD), and 38 controls were enrolled. For each functional domain, PSP patients were stratified by two certainty levels: vertical supranuclear gaze palsy (O1) and slowness of vertical saccades (O2) for ocular motor dysfunction; early unprovoked falls and tendency to fall on the pull-test for postural instability. Read More

Mov Disord Clin Pract 2018 May-Jun;5(3):325-326. Epub 2018 Mar 23.

Department of Neurology Charité University Medicine Berlin Charitéplatz 1, 10117, Berlin Germany.

Neurobiol Dis 2019 Feb 21;127:142-146. Epub 2019 Feb 21.

Neurodegenerative Diseases Research Unit, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, USA; Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. Electronic address:

Atypical parkinsonism syndromes are a heterogeneous group of neurodegenerative disorders that include corticobasal degeneration (CBD), Lewy body dementia (LBD), multiple system atrophy (MSA), and progressive supranuclear palsy (PSP). The APOE ε4 allele is a well-established risk factor for Alzheimer's disease; however, the role of APOE in atypical parkinsonism syndromes remains controversial. To examine the associations of APOE ε4 and ε2 alleles with risk of developing these syndromes, a total of 991 pathologically-confirmed atypical parkinsonism cases were genotyped using the Illumina NeuroChip array. Read More

Parkinsonism Relat Disord 2019 Feb 14. Epub 2019 Feb 14.

Department of Human Neurosciences, "Sapienza" University of Rome, V.le delle Università 30, 00185, Rome, Italy; IRCCS NEUROMED, Via Atinense 18, 86077, Pozzilli, IS, Italy. Electronic address:

Introduction: Alpha-synuclein (α-syn) aggregation is the pathological hallmark of Parkinson's Disease (PD). In this study, we measured α-syn total (α-syn), oligomeric α-syn (α-syn) and α-syn/α-syn ratio in the saliva of patients affected by PD and in age and sex-matched healthy subjects. We also compared salivary α-syn measured in PD with those detected in Progressive Supranuclear Palsy (PSP), in order to assess whether salivary α-syn can be used as a biomarker for PD and for the differential diagnosis between PD and PSP. Read More

Neurol Res Int 2019 16;2019:7247325. Epub 2019 Jan 16.

Université de Lille 2, INSERM U1171, "Degenerative & Vascular Cognitive Disorders", CHU Lille, 59000, France.

Purpose: The Boston criteria for cerebral amyloid angiopathy (CAA) have to be confirmed by postmortem examination. The present study investigates the incidence and the cerebrovascular impact of the severity of CAA in various neurodegenerative dementia diseases.

Material And Methods: 208 patients underwent an autopsy. Read More

Alzheimers Dement (Amst) 2019 Dec 25;11:136-141. Epub 2019 Jan 25.

Dementia Research Centre, Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, University College London, London, United Kingdom.

Introduction: Frontotemporal dementia (FTD) is a heterogeneous neurodegenerative disorder with multiple genetic and pathological causes. It is characterized by both cortical and subcortical atrophies, with previous studies showing early involvement of the amygdala. However, no prior study has specifically investigated the atrophy of different subnuclei of the amygdala. Read More

Clin Neurol Neurosurg 2019 Apr 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 Mar 14;126(3):233-251. 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 Apr 13;34(4):487-495. 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 Apr 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 01 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 Feb 31;56(2):1531-1538. 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 Mar 30;92(10):e1121-e1135. 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