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Parkinson's Plus Syndrome

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https://www.readbyqxmd.com/read/30508027/association-of-cerebrospinal-fluid-neurofilament-light-protein-levels-with-cognition-in-patients-with-dementia-motor-neuron-disease-and-movement-disorders
#1
Bob Olsson, Erik Portelius, Nicholas C Cullen, Åsa Sandelius, Henrik Zetterberg, Ulf Andreasson, Kina Höglund, David J Irwin, Murray Grossman, Daniel Weintraub, Alice Chen-Plotkin, David Wolk, Leo McCluskey, Lauren Elman, Leslie M Shaw, Jon B Toledo, Jennifer McBride, Pilar Hernandez-Con, Virginia M-Y Lee, John Q Trojanowski, Kaj Blennow
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...
December 3, 2018: JAMA Neurology
https://www.readbyqxmd.com/read/30502924/quantification-of-brain-derived-extracellular-vesicles-in-plasma-as-a-biomarker-to-diagnose-parkinson-s-and-related-diseases
#2
Takuma Ohmichi, Masato Mitsuhashi, Harutsugu Tatebe, Takashi Kasai, Omar M Ali El-Agnaf, Takahiko Tokuda
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)...
November 20, 2018: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/30314846/progress-in-the-treatment-of-parkinson-plus-syndromes
#3
Nahid Olfati, Ali Shoeibi, Irene Litvan
Progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), multiple system atrophy (MSA), and dementia with Lewy bodies (DLB) are the four major proteinopathic neurodegenerative disorders. Currently, there are no disease modifying therapies for these disorders. However, better understanding of the etiopathogenic mechanisms of these disorders has allowed the development of novel therapeutic approaches. These mainly include strategies directed to the pathologic conformational shift, seeding and aggregation, as well as transcellular spread of the proteins that aggregate in the brain which are α-synuclein and tau...
October 3, 2018: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/30308173/abrogating-native-%C3%AE-synuclein-tetramers-in-mice-causes-a-l-dopa-responsive-motor-syndrome-closely-resembling-parkinson-s-disease
#4
Silke Nuber, Molly Rajsombath, Georgia Minakaki, Jürgen Winkler, Christian P Müller, Maria Ericsson, Barbara Caldarone, Ulf Dettmer, Dennis J Selkoe
α-Synuclein (αS) regulates vesicle exocytosis but forms insoluble deposits in Parkinson's disease (PD). Developing disease-modifying therapies requires animal models that reproduce cardinal features of PD. We recently described a previously unrecognized physiological form of αS, α-helical tetramers, and showed that familial PD-causing missense mutations shift tetramers to aggregation-prone monomers. Here, we generated mice expressing the fPD E46K mutation plus 2 homologous E→K mutations in adjacent KTKEGV motifs...
October 10, 2018: Neuron
https://www.readbyqxmd.com/read/30181086/what-a-neurologist-should-know-about-pet-and-spect-functional-imaging-for-parkinsonism-a-practical-perspective
#5
Stéphane Thobois, Stéphane Prange, Christian Scheiber, Emmanuel Broussolle
The diagnosis of a parkinsonian syndrome based on clinical criteria remains sometimes difficult, especially at disease onset. Brain or heart molecular imaging techniques (SPECT or PET) can provide a major help to improve and speed up diagnosis, influencing treatment strategies. Presynaptic dopaminergic imaging using either [18 F]-Dopa PET or 123 I -2β-Carbomethoxy-3β-(4-Iodophenyl)- N-(3-Fluoropropyl) Nortropane ([123 I]-Ioflupane)SPECT demonstrates or rules out the presence of a dopaminergic degenerative process...
August 30, 2018: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/30133155/mitochondrial-dna-mutation-m-3243a-g-heterogeneous-clinical-picture-for-cardiologists-m-3243a-g-a-phenotypic-chameleon
#6
Katharina Niedermayr, Gerhard Pölzl, Sabine Scholl-Bürgi, Christine Fauth, Ulrich Schweigmann, Edda Haberlandt, Ursula Albrecht, Manuela Zlamy, Wolfgang Sperl, Johannes A Mayr, Daniela Karall
OBJECTIVE: In general, a mitochondrial disorder is diagnosed on the basis of symptom combinations and confirmed by genetic findings. However, patients carrying the m.3243A>G mutation in the mitochondrial tRNA leucine 1 (MT-TL1) do not always meet all the proposed criteria for the most frequently encountered mitochondrial syndrome "MELAS," an acronym for Mitochondrial Encephalomyopathy, Lactic Acidosis, and at least one Stroke-like episode. We here present various phenotypic characteristics of the mitochondrial mutation m...
August 21, 2018: Congenital Heart Disease
https://www.readbyqxmd.com/read/29995769/gastrointestinal-nervous-system-%C3%AE-synuclein-as-a-potential-biomarker-of-parkinson-disease
#7
Fudong Yan, Ying Chen, Min Li, Yingqing Wang, Wenmin Zhang, Xiaochun Chen, Qinyong Ye
Lewy bodies (LB) play an essential role in the development, survival, and function maintenance of midbrain dopaminergic (DA) neurons in Parkinson disease (PD). Alpha-synuclein (α-synuclein) is the major component of Lewy bodies and is a potential target for Parkinson's disease (PD) therapies. α-synuclein can be detected in the gastrointestinal (GI) nervous system, but whether there is any association between altered α-synuclein expression in the GI nervous system and the onset of PD is not known. The answer to this question presents the opportunity for a promising biomarker in the pre-clinical diagnosis of PD...
July 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29983692/expanding-the-spectrum-of-dopa-responsive-dystonia-drd-and-proposal-for-new-definition-drd-drd-plus-and-drd-look-alike
#8
REVIEW
Woong-Woo Lee, Beomseok Jeon, Ryul Kim
Previously, we defined DRD as a syndrome of selective nigrostriatal dopamine deficiency caused by genetic defects in the dopamine synthetic pathway without nigral cell loss. DRD-plus also has the same etiologic background with DRD, but DRD-plus patients have more severe features that are not seen in DRD because of the severity of the genetic defect. However, there have been many reports of dystonia responsive to dopaminergic drugs that do not fit into DRD or DRD-plus (genetic defects in the dopamine synthetic pathway without nigral cell loss)...
July 9, 2018: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/29905019/i-123-datscan-spect-brain-imaging-in-parkinsonian-syndromes-utility-of-the-putamen-to-caudate-ratio
#9
Manuela Matesan, Santhosh Gaddikeri, Katelan Longfellow, Robert Miyaoka, Saeed Elojeimy, Shana Elman, Shu-Ching Hu, Satoshi Minoshima, David Lewis
BACKGROUND AND PURPOSE: Computer-based analysis of Dopamine transporter imaging (DaTscan) can aid in image interpretation. In this study, we examined the distribution of putamen-to-caudate ratios (PCRs) obtained by using a clinically available semiquantification method. METHODS: Medical records of 32 patients (M:16) with a diagnosis of Parkinson's disease (PD) (n = 22) or Parkinson's plus syndromes (PPS) (n = 10) based on clinical follow-up, were retrospectively reviewed...
November 2018: Journal of Neuroimaging: Official Journal of the American Society of Neuroimaging
https://www.readbyqxmd.com/read/29510692/are-dementia-with-lewy-bodies-and-parkinson-s-disease-dementia-the-same-disease
#10
Kurt A Jellinger, Amos D Korczyn
BACKGROUND: Dementia with Lewy bodies (DLB) and Parkinson's disease dementia (PDD), which share many clinical, neurochemical, and morphological features, have been incorporated into DSM-5 as two separate entities of major neurocognitive disorders with Lewy bodies. Despite clinical overlap, their diagnosis is based on an arbitrary distinction concerning the time of onset of motor and cognitive symptoms, namely as early cognitive impairment in DLB and later onset following that of motor symptoms in PDD...
March 6, 2018: BMC Medicine
https://www.readbyqxmd.com/read/29458746/stridor-combined-with-other-sleep-breathing-disorders-in-multiple-system-atrophy-a-tailored-treatment
#11
Saleheddine Rekik, Francis Martin, Pauline Dodet, Stefania Redolfi, Smaranda Leu-Semenescu, Jean-Christophe Corvol, David Grabli, Isabelle Arnulf
OBJECTIVES: To determine the frequency of sleep breathing disorders in multiple systemic atrophy (MSA, combining Parkinsonism, cerebellar syndrome, and dysautonomia) and evaluate the benefit/tolerance of various modes of ventilation. METHODS: We retrospectively analyzed 45 patients with MSA having undergone a videopolysomnography. Their sleep characteristics were compared to those of 45 patients with Parkinson's disease and 45 healthy controls, matched for age and sex...
February 2018: Sleep Medicine
https://www.readbyqxmd.com/read/29400144/palliative-care-triggers-in-progressive-neurodegenerative-conditions-an-evaluation-using-a-multi-centre-retrospective-case-record-review-and-principal-component-analysis
#12
Jamilla Hussain, Victoria Allgar, David Oliver
BACKGROUND: The use of specific triggers has been suggested to help identify patients with progressive neurological disease who would benefit from palliative care. AIM: This study aimed to improve the evidence base for the use of triggers for patients with progressive neurological disease. DESIGN: An evaluation of palliative care services was undertaken using a retrospective case note review of the timing and presence of triggers in the last 2 years of life...
April 2018: Palliative Medicine
https://www.readbyqxmd.com/read/29381583/99mtc-trodat-1-spect-ct-imaging-as-a-complementary-biomarker-in-the-diagnosis-of-parkinsonian-syndromes
#13
Bhagwant R Mittal, Apurva Sood, Jaya Shukla, Rakhee Vatsa, Priya Bhusari, Ritu Shree, Sahil Mehta, Manoj Goyal, Manish Modi
INTRODUCTION: Parkinson's disease (PD) and Parkinson plus syndromes (PPS) are neurodegenerative movement disorders caused by loss of dopamine in the basal ganglia. The diagnosis of both PD and PPS is complex as it is made solely on the basis of clinical features, with no established imaging modality to aid in the diagnosis. Technetium-99m-labeled tropane derivative (Tc-TRODAT-1) binds to the dopamine transporters present in the presynaptic membrane of the dopaminergic nerve terminal. The aim of this prospective study was to investigate the potential usefulness of Tc-TRODAT-1 imaging in the diagnosis of PD and PPS...
April 2018: Nuclear Medicine Communications
https://www.readbyqxmd.com/read/29326035/myoclonus-in-the-elderly-a-retrospective-analysis-of-clinical-and-electrophysiological-characteristics-of-patients-referred-to-an-electrophysiology-laboratory
#14
Meral E Kiziltan, Aysegul Gunduz, Melih Tutuncu, Sibel Ertan, Hulya Apaydin, Gunes Kiziltan
BACKGROUND AND OBJECTIVE: Late-onset myoclonus in the elderly is mainly related to dementia or systemic disease. In this report, we aimed to investigate the clinical and electrophysiological features of patients with late-onset myoclonus. PATIENTS AND METHOD: We retrospectively assessed the medical records of patients who were referred to our electromyography laboratory. From these records, we included all patients who had myoclonus which started after the age of 60 years and in whom it was confirmed by polymyography...
April 2018: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/29285760/subthalamic-deep-brain-stimulation-and-trunk-posture-in-parkinson-s-disease
#15
C A Artusi, M Zibetti, A Romagnolo, M G Rizzone, A Merola, L Lopiano
OBJECTIVES: We sought to assess the efficacy of subthalamic nucleus deep brain stimulation (STN-DBS) in Parkinson's disease (PD)-associated trunk posture abnormalities retrospectively analyzing data from 101 patients reporting mild-to-severe trunk posture abnormalities of a cohort of 216 PD patients treated with STN-DBS at our center. METHODS: Abnormal trunk posture was rated on a scale of 0 (normal) to 4 (marked flexion with an extreme abnormality of posture) as per the grading score reported in the Unified Parkinson's Disease Rating Scale...
May 2018: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/29222591/dementia-with-lewy-bodies-and-parkinson-s-disease-dementia-current-concepts-and-controversies
#16
REVIEW
Kurt A Jellinger
Dementia with Lewy bodies (DLB) and Parkinson's disease-dementia (PDD), although sharing many clinical, neurochemical and morphological features, according to DSM-5, are two entities of major neurocognitive disorders with Lewy bodies of unknown etiology. Despite considerable clinical overlap, their diagnosis is based on an arbitrary distinction between the time of onset of motor and cognitive symptoms: dementia often preceding parkinsonism in DLB and onset of cognitive impairment after onset of motor symptoms in PDD...
April 2018: Journal of Neural Transmission
https://www.readbyqxmd.com/read/29111028/csf-biomarkers-%C3%AE-amyloid-tau-proteins-and-a-synuclein-in-the-differential-diagnosis-of-parkinson-plus-syndromes
#17
Vasilios C Constantinides, George P Paraskevas, Evangelia Emmanouilidou, Olga Petropoulou, Anastasia Bougea, Kostas Vekrellis, Ioannis Evdokimidis, Eleftherios Stamboulis, Elisabeth Kapaki
INTRODUCTION: Differential diagnosis of Parkinson-plus patients (PSP, CBD, MSA) and Parkinson's disease (PD) patients is often not straightforward, particularly in atypical cases or at the initial stages of the diseases. Classic CSF biomarkers (amyloid-beta - Aβ42 , tau protein - τT and phosphorylated tau protein - τP-181 ) are established biomarkers in the diagnosis of Alzheimer's disease (AD). CSF a-synuclein (α-syn) has emerged as a promising biomarker in patients with Parkinsonism...
November 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28975044/recent-updates-on-acquired-hepatocerebral-degeneration
#18
REVIEW
Hae-Won Shin, Hee Kyung Park
BACKGROUND: Acquired hepatocerebral degeneration (AHD) refers to a chronic neurological syndrome in patients with advanced hepatobiliary diseases. This comprehensive review focuses on the pathomechanism and neuroimaging findings in AHD. METHODS: A PubMed search was performed using the terms "acquired hepatocerebral degeneration," "chronic hepatocerebral degeneration," "Non-Wilsonian hepatocerebral degeneration," "cirrhosis-related parkinsonism," and "manganese and liver disease...
2017: Tremor and Other Hyperkinetic Movements
https://www.readbyqxmd.com/read/28840137/uptake-index-of-123-i-metaiodobenzylguanidine-myocardial-scintigraphy-for-diagnosing-lewy-body-disease
#19
Yoshito Kamiya, Satoru Ota, Shintaro Okumiya, Kosuke Yamashita, Akihiro Takaki, Shigeki Ito
OBJECTIVES: Iodine-123 metaiodobenzylguanidine ((123)I-MIBG) myocardial scintigraphy has been used to evaluate cardiac sympathetic denervation in Lewy body disease (LBD), including Parkinson's disease (PD) and dementia with Lewy bodies (DLB). The heart-to-mediastinum ratio (H/M) in PD and DLB is significantly lower than that in Parkinson's plus syndromes and Alzheimer's disease. Although this ratio is useful for distinguishing LBD from non-LBD, it fluctuates depending on the system performance of the gamma cameras...
2017: Asia Oceania Journal of Nuclear Medicine & Biology
https://www.readbyqxmd.com/read/28803444/atypical-parkinsonian-syndromes-a-general-neurologist-s-perspective
#20
REVIEW
A B Deutschländer, O A Ross, D W Dickson, Z 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, clinical, imaging, neuropathological and genetic features of multiple system atrophy, progressive supranuclear palsy, corticobasal degeneration and frontotemporal lobar degeneration (FTLD) are reviewed. The terms corticobasal degeneration and FTLD refer to pathologically confirmed cases of corticobasal syndrome and frontotemporal dementia (FTD)...
January 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
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