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Progressive supranuclear palsy

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https://www.readbyqxmd.com/read/28814874/la-belle-indiff%C3%A3-rence-revisited-a-case-report-on-progressive-supranuclear-palsy-misdiagnosed-as-conversion-disorder
#1
Petra J van Meerkerk-Aanen, Lars de Vroege, David Khasho, Aziza Foruz, J Thies van Asseldonk, Christina M van der Feltz-Cornelis
BACKGROUND: Since the advent of computed tomography and magnetic resonance imaging scans, neurological disorders have less often been falsely labeled as conversion disorder (CD). However, misdiagnosis of a neurological disorder as CD still occurs, especially in cases with insidious onset. Misinterpretation of la belle indifférence may contribute to such misdiagnosis. Here, we describe a case of progressive supranuclear palsy/Richardson's syndrome (PSPS) misdiagnosed as a case of CD. CASE: A 62-year-old woman consulted two different neurologists in 2012 because of falling spells since 2009 and was diagnosed with CD...
2017: Neuropsychiatric Disease and Treatment
https://www.readbyqxmd.com/read/28805573/nonmotor-features-in-atypical-parkinsonism
#2
Kailash P Bhatia, Maria Stamelou
Atypical parkinsonism (AP) comprises mainly multiple system atrophy (MSA), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD), which are distinct pathological entities, presenting with a wide phenotypic spectrum. The classic syndromes are now called MSA-parkinsonism (MSA-P), MSA-cerebellar type (MSA-C), Richardson's syndrome, and corticobasal syndrome. Nonmotor features in AP have been recognized almost since the initial description of these disorders; however, research has been limited...
2017: International Review of Neurobiology
https://www.readbyqxmd.com/read/28803444/atypical-parkinsonian-syndromes-a-general-neurologist-s-perspective
#3
REVIEW
Angela B Deutschländer, Owen A Ross, Dennis W Dickson, Zbigniew K Wszolek
The differential diagnosis of atypical parkinsonian syndromes is challenging. These severe and often rapidly progressive neurodegenerative disorders are clinically heterogeneous and show significant phenotypic overlap. Here we review clinical, imaging, neuropathologic and genetic features of multiple system atrophy (MSA), progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), and frontotemporal lobar degeneration (FTLD). The terms CBD and FTLD refer to pathologically confirmed cases of corticobasal syndrome (CBS) and frontotemporal dementia (FTD)...
August 12, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28799303/clinical-profile-of-dysphagia-in-patients-with-parkinson-s-disease-progressive-supranuclear-palsy-and-multiple-system-atrophy
#4
Sulena, Dipti Gupta, Anjani Kumar Sharma, Baltej Singh
BACKGROUND: Swallowing changes are commonly observed in Parkinson's and Parkinsonism plus syndromes. Expeditious identification is necessary to provide early intervention in this population to avoid risk of aspiration and swallowing complications. OBJECTIVE: To investigate swallowing problems using detailed case history and swallowing speed on 3 ounce water test in three groups i.e. PD, MSA and PSP groups and further, to compare it with control group. SUBJECT AND METHODS: Cross sectional study design...
August 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/28782342/-hummingbird-sign-in-a-patient-with-guam-parkinsonism-dementia-complex
#5
Tianrong Yeo, Louis Cs Tan
We present a case of a 71-year-old male Chamorro patient from Guam who presented with progressive supranuclear palsy (PSP)-Richardson's syndrome. Considering his strong family history of parkinsonism and a PSP phenotype, he was clinically diagnosed with Guam parkinsonism-dementia complex (PDC). Magnetic resonance imaging (MRI) of the brain revealed prominent midbrain atrophy with preserved pontine volume, forming the 'hummingbird' sign, which has not been described before in Guam PDC. Molecular analysis of the chromosome 9 open reading frame 72 gene (C9orf72) showed only 6 GGGGCC repeats...
August 8, 2017: Journal of Movement Disorders
https://www.readbyqxmd.com/read/28780180/neuropathology-of-parkinson-disease
#6
Dennis W Dickson
INTRODUCTION: Parkinson's disease (PD) is characterized by bradykinesia, rigidity, postural instability and tremor. Several pathologic processes can produce this syndrome, but neurodegeneration accompanied by neuronal inclusions composed of α-synuclein (Lewy bodies) is considered the typical pathologic correlate of PD. METHODS: The neuropathologic features of PD are reviewed based upon personal experience and review of the literature. Molecular pathology of PD is summarized from cell biological and animal studies...
August 1, 2017: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/28762086/regional-microstructural-damage-and-patterns-of-eye-movement-impairment-a-dti-and-video-oculography-study-in-neurodegenerative-parkinsonian-syndromes
#7
Martin Gorges, Melanie N Maier, Johannes Rosskopf, Olga Vintonyak, Elmar H Pinkhardt, Albert C Ludolph, Hans-Peter Müller, Jan Kassubek
Characteristic alterations of eye movement control are a common feature of neurodegenerative parkinsonism, including Parkinson's disease (PD), progressive supranuclear palsy (PSP), and multiple system atrophy (MSA). Regional microstructural alterations as assessed by diffusion tensor imaging (DTI) have been reported in PD, PSP, and MSA. Therefore, we investigated the specific association between eye movement disturbances and microstructural impairment in these diseases. Video-oculographic recordings of smooth pursuit and visually guided reactive saccades as well as fractional anisotropy (FA) maps computed from whole-brain DTI data were analyzed for 36 PD, 30 PSP, 18 MSA patients, and 23 matched healthy control subjects...
July 31, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28754841/in-vivo-retention-of-18-f-av-1451-in-corticobasal-syndrome
#8
Ruben Smith, Michael Schöll, Håkan Widner, Danielle van Westen, Per Svenningsson, Douglas Hägerström, Tomas Ohlsson, Jonas Jögi, Christer Nilsson, Oskar Hansson
OBJECTIVE: To study the usefulness of (18)F-AV-1451 PET in patients with corticobasal syndrome (CBS). METHODS: We recruited 8 patients with CBS, 17 controls, 31 patients with Alzheimer disease (AD), and 11 patients with progressive supranuclear palsy (PSP) from the Swedish BioFINDER study. All patients underwent clinical assessment, (18)F-AV-1451 PET, MRI, and quantification of β-amyloid pathology. A subset of participants also underwent (18)F-FDG-PET. RESULTS: In the 8 patients with CBS, 6 had imaging findings compatible with the corticobasal degeneration pathology and 2 with typical AD pathology...
July 28, 2017: Neurology
https://www.readbyqxmd.com/read/28747135/high-burden-and-depression-among-late-stage-idiopathic-parkinson-disease-and-progressive-supranuclear-palsy-caregivers
#9
Christian Schmotz, Carmen Richinger, Stefan Lorenzl
OBJECTIVES: Caregivers of patients with late-stage idiopathic Parkinson disease (IPD) and late-stage progressive supranuclear palsy (PSP) often suffer from severe psychological strain themselves. This study investigates the influence of the different kind of symptoms in IPD and PSP on the psychological burden of the caregivers. METHODS: Twenty patients with late-stage IPD and 20 patients with late-stage PSP and their caregivers were investigated. To measure the degree of motor, cognitive, and affective impairment of the patients, the instruments Subscale III of the Unified Rating Scale for Parkinsonism (UPDRS-III), a shortened 24-item version of the Mini-Mental State Examination, and the Geriatric Depression Scale (GDS-30) were used...
January 1, 2017: Journal of Geriatric Psychiatry and Neurology
https://www.readbyqxmd.com/read/28746923/parkinsonian-patients-with-striatal-cribriform-state-present-rapidly-progressive-axial-parkinsonism
#10
Seung Ha Lee, Chul Hyoung Lyoo, Hanna Cho, Juha O Rinne, Myung Sik Lee
OBJECTIVE: To define the significance of striatal cribriform state (SCS) observed in patients with primary progressive parkinsonism. METHODS: We reviewed medical records and brain magnetic resonance imaging studies of 1,260 patients with primary progressive parkinsonism. We identified 23 patients with SCS and analyzed their clinical features. RESULTS: All 23 patients had rapidly progressive parkinsonism predominated by postural instability and gait disturbance...
July 26, 2017: European Neurology
https://www.readbyqxmd.com/read/28744253/novel-eye-movement-disorders-in-whipple-s-disease-staircase-horizontal-saccades-gaze-evoked-nystagmus-and-esotropia
#11
Aasef G Shaikh, Fatema F Ghasia
Whipple's disease, a rare systemic infectious disorder, is complicated by the involvement of the central nervous system in about 5% of cases. Oscillations of the eyes and the jaw, called oculo-masticatory myorhythmia, are pathognomonic of the central nervous system involvement but are often absent. Typical manifestations of the central nervous system Whipple's disease are cognitive impairment, parkinsonism mimicking progressive supranuclear palsy with vertical saccade slowing, and up-gaze range limitation. We describe a unique patient with the central nervous system Whipple's disease who had typical features, including parkinsonism, cognitive impairment, and up-gaze limitation; but also had diplopia, esotropia with mild horizontal (abduction more than adduction) limitation, and vertigo...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28730936/an-industry-update-what-is-the-latest-news-in-therapeutic-delivery
#12
Iain Simpson
This Industry Update covers the period from 1 through 30 April 2017, and is based on information sourced from company press releases, scientific literature, patents and various news websites. Biogen expanded its portfolio of developmental drugs targeting neurological diseases by licensing a drug from Bristol-Myers Squibb which is currently under investigation for the treatment of progressive supranuclear palsy. Takeda announced a deal with NuBiyota focused on developing treatments for gastrointestinal disorders, based on the manipulation of microbes living in the gut...
July 2017: Therapeutic Delivery
https://www.readbyqxmd.com/read/28716886/clinical-neurology-and-epidemiology-of-the-major-neurodegenerative-diseases
#13
Michael G Erkkinen, Mee-Ohk Kim, Michael D Geschwind
Neurodegenerative diseases are a common cause of morbidity and cognitive impairment in older adults. Most clinicians who care for the elderly are not trained to diagnose these conditions, perhaps other than typical Alzheimer's disease (AD). Each of these disorders has varied epidemiology, clinical symptomatology, laboratory and neuroimaging features, neuropathology, and management. Thus, it is important that clinicians be able to differentiate and diagnose these conditions accurately. This review summarizes and highlights clinical aspects of several of the most commonly encountered neurodegenerative diseases, including AD, frontotemporal dementia (FTD) and its variants, progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), Parkinson's disease (PD), dementia with Lewy bodies (DLB), multiple system atrophy (MSA), and Huntington's disease (HD)...
July 17, 2017: Cold Spring Harbor Perspectives in Biology
https://www.readbyqxmd.com/read/28706282/retinal-degeneration-in-progressive-supranuclear-palsy-measured-by-optical-coherence-tomography-and-scanning-laser-polarimetry
#14
Birthe Stemplewitz, Robert Kromer, Eik Vettorazzi, Ute Hidding, Andreas Frings, Carsten Buhmann
This cross-sectional study compared the retinal morphology between patients with progressive supranuclear palsy (PSP) and healthy controls. (The retinal nerve fiber layer (RNFL) around the optic disc and the retina in the macular area of 22 PSP patients and 151 controls were investigated by spectral domain optical coherence tomography (SD-OCT). Additionally, the RNFL and the nerve fiber index (NFI) were measured by scanning laser polarimetry (SLP). Results of RNFL measurements with SD-OCT and SLP were compared to assess diagnostic discriminatory power...
July 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28705587/diagnosis-of-parkinson-s-disease-and-the-level-of-oxidized-dj-1-protein
#15
Yuko Yamagishi, Kazumasa Saigoh, Yoshiro Saito, Ikuko Ogawa, Yoshiyuki Mitsui, Yukihiro Hamada, Makoto Samukawa, Hidekazu Suzuki, Motoi Kuwahara, Makito Hirano, Noriko Noguchi, Susumu Kusunoki
Parkinson's disease (PD) is difficult to distinguish from progressive supranuclear palsy (PSP) and multiple system atrophy (MSA); in addition, biomarker studies in PD mostly focused on those found in the cerebrospinal fluid, and there are few reports of simple biomarkers identified by blood analysis. Previously, the DJ-1 gene was identified as a causative gene of familial PD. Oxidized DJ-1 protein (oxDJ-1) levels were reported to increase in the blood of patients with unmedicated PD. Therefore, we determined the levels of oxDJ-1 in the erythrocytes of patients with PD, PSP, and MSA using ELISA...
July 10, 2017: Neuroscience Research
https://www.readbyqxmd.com/read/28704733/reversal-of-long-term-potentiation-like-plasticity-in-primary-motor-cortex-in-patients-with-progressive-supranuclear-palsy
#16
Matteo Bologna, Kelly Bertram, Giulia Paparella, Claudia Papi, Daniele Belvisi, Antonella Conte, Antonio Suppa, David R Williams, Alfredo Berardelli
OBJECTIVE: Abnormal primary motor cortex plasticity might be involved in the pathophysiology of progressive supranuclear palsy. In the present study we aimed to investigate possible abnormalities of depotentiation, a mechanism involved in plasticity regulation, in this condition. METHODS: Primary motor cortex excitability, investigated with single and paired-pulse transcranial magnetic stimulation, as well as long-term potentiation-like plasticity and its reversibility, were studied using theta burst stimulation in 15 patients with progressive supranuclear palsy and 11 healthy controls...
September 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/28702192/freezing-of-gait-is-an-early-clinical-feature-of-progressive-supranuclear-palsy
#17
Yasushi Osaki, Yukari Morita, Yuka Miyamoto, Kounosuke Furuta, Hirokazu Furuya
BACKGROUND AND AIM: Early clinical diagnosis of progressive supranuclear palsy (PSP) remains challenging. AIM: We attempted to identify any sign or symptom to diagnose PSP earlier. METHODS: A total of 401 patients, 40 with PSP and 361 with other neurodegenerative disorders, were included. We followed these patients for at least 1 year since 2009. We reviewed the signs and symptoms of patients with PSP in a standardized manner, and observed four manifestations: "vertical supranuclear gaze abnormality," "movement disorders," "pseudobulbar palsy" and "dementia of frontal type...
May 2017: Neurology and Clinical Neuroscience
https://www.readbyqxmd.com/read/28691164/pick-s-disease-with-neuronal-four-repeat-tau-accumulation-in-the-basal-ganglia-brain-stem-nuclei-and-cerebellum
#18
Chikako Ikeda, Osamu Yokota, Tomoko Miki, Shintaro Takenoshita, Hideki Ishizu, Yoko Mori, Kiyohiro Yamazaki, Yuki Ozaki, Shu-Ichi Ueno, Takeshi Ishihara, Masato Hasegawa, Seishi Terada, Norihito Yamada
It is very rare that cases of Pick's disease, a representative three-repeat (3R) tauopathy, also have significant four-repeat (4R) tau accumulation. Here, we report a Pick's disease case that clinically showed behavioral variant frontotemporal dementia without motor disturbance during the course, and pathologically had 3R tau-positive Pick bodies as well as numerous 4R tau-positive neuronal cytoplasmic inclusions (NCIs). Abundant 3R tau-positive 4R tau-negative spherical or horseshoe-shaped Pick bodies were found in the frontotemporal cortex, limbic region, striatum and pontine nucleus...
July 9, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28689259/differential-diagnosis-of-parkinsonism-by-a-combined-use-of-diffusion-kurtosis-imaging-and-quantitative-susceptibility-mapping
#19
Kenji Ito, Chigumi Ohtsuka, Kunihiro Yoshioka, Hiroyuki Kameda, Suguru Yokosawa, Ryota Sato, Yasuo Terayama, Makoto Sasaki
PURPOSE: We investigated whether diffusion kurtosis imaging (DKI) and quantitative susceptibility mapping (QSM) could detect pathological changes that occur in Parkinson's disease (PD), multiple system atrophy with predominant parkinsonism (MSA-P) or predominant cerebellar ataxia (MSA-C), and progressive supranuclear palsy syndrome (PSPS) and thus be used for differential diagnosis that is often difficult. METHODS: Seventy patients (41 with PD, 6 with MSA-P, 7 with MSA-C, 16 with PSPS) and 20 healthy controls were examined using a 3...
August 2017: Neuroradiology
https://www.readbyqxmd.com/read/28685158/imaging-tau-pathology-in-parkinsonisms
#20
REVIEW
Sarah Coakeley, Antonio P Strafella
The recent development of positron emission tomography radiotracers targeting pathological tau in vivo has led to numerous human trials. While investigations have primarily focused on the most common tauopathy, Alzheimer's disease, it is imperative that testing also be performed in parkinsonian tauopathies, such as progressive supranuclear palsy, corticobasal degeneration, and frontotemporal dementia and parkinsonism linked to chromosome 17. Tau aggregates differ in isoforms and conformations across disorders, and as a result one radiotracer may not be appropriate for all tauopathies...
2017: NPJ Parkinson's Disease
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