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Rapid progressive dementia

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https://www.readbyqxmd.com/read/29346380/rapidly-progressive-dementia-an-eight-year-2008-2016-retrospective-study
#1
Patil Anuja, Vishnu Venugopalan, Naheed Darakhshan, Pandit Awadh, Vinny Wilson, Goyal Manoj, Modi Manish, Lal Vivek
BACKGROUND AND PURPOSE: Rapidly progressive dementia (RPD) is an emergency in cognitive neurology, defined as cognitive impairment affecting the daily living activities developed over less than 1 year. This study investigated the profile of patients with rapidly progressive dementia at first presentation. METHODS: Retrospective case analysis was done in 187 patients with rapidly progressive dementia who presented to the Postgraduate Institute of Medical Education and Research, Chandigarh, India from January 2008 to August 2016...
2018: PloS One
https://www.readbyqxmd.com/read/29318507/mid-term-prognosis-of-non-functioning-pituitary-adenomas-with-high-proliferative-potential-really-an-aggressive-variant
#2
Yoshikazu Ogawa, Hidefumi Jokura, Kuniyasu Niizuma, Teiji Tominaga
Pituitary adenomas with high proliferation rate and rapid growth are well known, but the clinical characteristics, prognosis, and treatment algorithm remain unclear. The clinical characteristics and mid-term prognosis of patients with non-functioning pituitary adenomas with high proliferative potential were retrospectively investigated. This study identified 53 patients with Ki-67 labeling index of > 3% among 845 patients with non-functioning pituitary adenoma (6.3%) initially treated by surgery. Prophylactic treatment was not applied for patients with residual tumor, but salvage treatment was performed if tumor progression was identified within the follow-up period...
January 9, 2018: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/29310343/cerebrospinal-fluid-real-time-quaking-induced-conversion-test-for-sporadic-creutzfeldt-jakob-disease-in-an-18-year-old-woman-a-case-report
#3
Yuan Yao, Xiaoping Dong, Hongzhi Guan, Qiang Lu
RATIONALE: Sporadic Creutzfeldt-Jakob disease (sCJD) mainly occurs in the elderly, with the peak age of onset ranging from 55 to 75 years. The symptoms of sCJD are not unique, and laboratory tests such as magnetic resonance imaging (MRI), electroencephalogram (EEG) and cerebrospinal fluid (CSF)14-3-3 protein have low sensitivity or specificity. Therefore, excluding treatable diseases and establishing a diagnosis could be difficult in young patients with suspected sCJD. Recently, real-time quaking-induced conversion (RT-QuIC) has been used in the diagnosis of sCJD, with more than 95% sensitivity and 100% specificity...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29276596/applicability-of-long-term-electroencephalography-in-pre-mortem-diagnosis-of-creutzfeldt-jakob-disease-a-case-report
#4
Sanaz Attaripour Isfahani, Michelle Dougherty, Gediminas Peter Gliebus
Creutzfeldt-Jakob disease accounts for more than 90% of all sporadic prion disease cases. The molecular MM2 genotype has been divided into cortical and thalamic subtypes based on structures involved and is characterized clinically by progressive dementia without ataxia or typical electroencephalography changes. Proposed diagnostic criteria for MM2 cortical type sporadic Creutzfeldt-Jakob disease include progressive dementia, cortical hyper-intensity on diffusion-weighted magnetic resonance imaging, increased cerebrospinal fluid 14-3-3 protein level, and the exclusion of other types of dementia...
2017: SAGE Open Medical Case Reports
https://www.readbyqxmd.com/read/29259496/immunosenescence-in-aging-between-immune-cells-depletion-and-cytokines-up-regulation
#5
REVIEW
Maria Teresa Ventura, Marco Casciaro, Sebastiano Gangemi, Rosalba Buquicchio
Background: The immunosenescence is a relatively recent chapter, correlated with the linear extension of the average life began in the nineteenth century and still in progress. The most important feature of immunosenescence is the accumulation in the "immunological space" of memory and effector cells as a result of the stimulation caused by repeated clinical and subclinical infections and by continuous exposure to antigens (inhalant allergens, food, etc.). This state of chronic inflammation that characterizes senescence has a significant impact on survival and fragility...
2017: Clinical and Molecular Allergy: CMA
https://www.readbyqxmd.com/read/29248770/development-and-validation-of-an-uhplc-esi-qtof-ms-method-for-quantification-of-the-highly-hydrophilic-amyloid-%C3%AE-oligomer-eliminating-all-d-enantiomeric-peptide-rd2-in-mouse-plasma
#6
Michelle Hupert, Anne Elfgen, Elena Schartmann, Sarah Schemmert, Brigitte Buscher, Janine Kutzsche, Dieter Willbold, Beatrix Santiago-Schübel
During preclinical drug development, a method for quantification of unlabeled compounds in blood plasma samples from treatment or pharmacokinetic studies in mice is required. In the current work, a rapid, specific, sensitive and validated liquid chromatography mass-spectrometric UHPLC-ESI-QTOF-MS method was developed for the quantification of the therapeutic compound RD2 in mouse plasma. RD2 is an all-D-enantiomeric peptide developed for the treatment of Alzheimer's disease, a progressive neurodegenerative disease finally leading to dementia...
December 8, 2017: Journal of Chromatography. B, Analytical Technologies in the Biomedical and Life Sciences
https://www.readbyqxmd.com/read/29244089/the-historical-progression-from-adl-scrutiny-to-iadl-to-advanced-adl-assessing-functional-status-in-the-earliest-stages-of-dementia
#7
Robert Fieo, Laura Zahodne, Ming X Tang, Jennifer J Manly, Ron Cohen, Yaakov Stern
Background: Decrements in instrumental activities (IADL) have been observed in the prodromal phase of dementia. Given the long pre-dementia stage in neuro-degenerative diseases, it has been proposed that subtle functional changes may precede clinical IADL impairment. Incorporating more challenging advanced ADLs (e.g., volunteer work) into the assessment process may increase the sensitivity of functional measures, thus expanding the window for monitoring or interventions. Methods: Longitudinal cohort study (follow-ups, 18-24 month), subjects aged >60 (n=3635)...
December 13, 2017: Journals of Gerontology. Series A, Biological Sciences and Medical Sciences
https://www.readbyqxmd.com/read/29226870/alzheimer-s-disease-progression-factors-influencing-cognitive-decline
#8
Camilla Ferrari, Gemma Lombardi, Cristina Polito, Giulia Lucidi, Silvia Bagnoli, Irene Piaceri, Benedetta Nacmias, Valentina Berti, Debora Rizzuto, Laura Fratiglioni, Sandro Sorbi
BACKGROUND: Alzheimer's disease (AD) patients present high variability in the rate of cognitive decline. Despite the wide knowledge on factors influencing dementia risk, little is known on what accounts for AD progression. Previous studies on this topic have mainly analyzed each factor separately without taking into account the interaction between genetic and non-genetic factors. OBJECTIVE: The aim of the present study is to evaluate the role of demographic, clinical, therapeutic, and genetic factors and their interaction on cognitive decline among newly diagnosed AD patients...
December 8, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29170501/idiopathic-rem-sleep-behaviour-disorder-and-neurodegeneration-an-update
#9
REVIEW
Birgit Högl, Ambra Stefani, Aleksandar Videnovic
So-called idiopathic rapid eye movement (REM) sleep behaviour disorder (RBD), formerly seen as a rare parasomnia, is now recognized as the prodromal stage of an α-synucleinopathy. Given the very high risk that patients with idiopathic RBD have of developing α-synucleinopathies, such as Parkinson disease (PD), PD dementia, dementia with Lewy bodies or multiple system atrophy, and the outstandingly high specificity and very long interval between the onset of idiopathic RBD and the clinical manifestations of α-synucleinopathies, the prodromal phase of this disorder represents a unique opportunity for potentially disease-modifying intervention...
November 24, 2017: Nature Reviews. Neurology
https://www.readbyqxmd.com/read/29125945/periodic-eeg-patterns-in-sporadic-creutzfeld-jakob-disease-can-be-benzodiazepine-responsive-and-be-difficult-to-distinguish-from-non-convulsive-status-epilepticus
#10
Justus Marquetand, Susanne Knake, Adam Strzelczyk, Bernhard J Steinhoff, Holger Lerche, Matthis Synofzik, Niels K Focke
PURPOSE: Periodic discharges in EEG and a history of rapidly progressive dementia are known to be associated with sporadic Creutzfeld-Jakob Disease (sCJD). Doubts regarding this rare but fatal diagnosis can arise, when episodic symptoms (seizures, psychiatric features, speech disturbances) are present and the EEG shows epileptiform discharges within this periodicity. This scenario may indicate non-convulsive status epilepticus (NCSE), which is - in contrast to sCJD - a treatable and frequent condition...
December 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/29123489/plasmapheresis-responsive-rapid-onset-dementia-with-predominantly-frontal-dysfunction-in-the-context-of-hashimoto-s-encephalopathy
#11
Dominique Endres, Magnus S Vry, Petra Dykierek, Anne N Riering, Eva Lüngen, Oliver Stich, Rick Dersch, Nils Venhoff, Daniel Erny, Irina Mader, Philipp T Meyer, Ludger Tebartz van Elst
Background: Hashimoto's encephalopathy (HE) is a rare immunological neuropsychiatric disorder characterized by increased antithyroid antibodies and mixed neurological and psychiatric symptoms. HE has been previously discussed as a differential diagnosis for rapid progressive dementia. However, most of these patients suffered from additional neurological symptoms, like ataxia or seizures. Case presentation: Here, we present the case of a 59-year-old female patient suffering rapid onset dementia with salient frontal executive dysfunction...
2017: Frontiers in Psychiatry
https://www.readbyqxmd.com/read/29111000/natural-history-and-predictors-of-survival-in-progressive-supranuclear-palsy
#12
Maura Cosseddu, Alberto Benussi, Stefano Gazzina, Marta A Manes, Valentina Dell'Era, Viviana Cristillo, Rosanna Turrone, Antonella Alberici, Barbara Borroni
BACKGROUND: Progressive supranuclear palsy is a neurodegenerative disorder characterized by high functional disability and rapidly progressive dependency. The predictors of survival are still unclear. METHODS: The predictors of survival were evaluated in a group of clinically diagnosed PSP patients, focusing primarily on extensive cognitive assessment. RESULTS: The mean survival time from symptom onset was 8.25±3.0years. Sex, age at onset, education, occupation and severity of extrapyramidal symptoms did not correlate with survival...
November 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/29103041/association-of-the-new-variant-tyr424asp-at-tbk1-gene-with-amyotrophic-lateral-sclerosis-and-cognitive-decline
#13
Irene Piaceri, Valentina Bessi, Sabrina Matà, Cristina Polito, Andrea Tedde, Valentina Berti, Silvia Bagnoli, Arianna Braccia, Monica Del Mastio, Alberto Moggi Pignone, Alberto Pupi, Sandro Sorbi, Benedetta Nacmias
A new risk gene associated with amyotrophic lateral sclerosis (ALS) has recently been identified: the Tank-binding kinase 1 (TBK1) gene. Up to now, 90 TBK1 variants have been described in ALS patients with or without frontotemporal dementia (FTD), thus making TBK1 the third or fourth most frequent genetic cause of ALS and FTD. A point mutation analysis in a cohort of 69 Italian ALS patients was performed in order to analyze the frequency of TBK1 mutations and the correlation with clinical phenotypes. The analysis identified the novel variant p...
October 30, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29097599/brains-with-sporadic-creutzfeldt-jakob-disease-and-copathology-showed-a-prolonged-end-stage-of-disease
#14
Aitzol Miguelez-Rodriguez, Jorge Santos-Juanes, Ikerne Vicente-Etxenausia, Katty Perez de Heredia-Goñi, Beatriz Garcia, Luis M Quiros, Laura Lorente-Gea, Isabel Guerra-Merino, Jose J Aguirre, Ivan Fernandez-Vega
AIMS: To investigate the expression of major proteins related to primary neurodegenerative diseases and their prognostic significance in brains with Creutzfeldt-Jakob disease (CJD). MATERIALS AND METHODS: Thirty consecutive cases of confirmed CJD during the period 2010-2015 at Basque Brain bank were retrospectively reviewed. Moreover, major neurodegenerative-associated proteins (phosphorylated Tau, 4R tau, 3R tau, alpha-synuclein, TDP43, amyloid beta) were tested...
November 2, 2017: Journal of Clinical Pathology
https://www.readbyqxmd.com/read/29095328/c9orf72-intermediate-repeat-expansion-in-a-patient-with-psychiatric-disorders-and-progressive-cerebellar-ataxia
#15
Mario Meloni, Rita Farris, Paolo Solla, Marcello M Mascia, Francesco Marrosu, Antonino Cannas
INTRODUCTION: Large expansions of the noncoding GGGGCC repeat (more than 30) in the first intron of the C9ORF72 gene have been demonstrated to cause amyotrophic lateral sclerosis and frontotemporal dementia. Recent papers have investigated the possible pathogenic role and associated clinical phenotypes of hexanucleotide expansions with intermediate repeat lengths ranging between 20 and 29 repeats. CASE REPORT: We report a case of a 71-year-old Sardinian female patient with a long history of psychiatric disorders such as mixed anxiety-depressive disorder associated with somatization disorder and histrionic personality who developed a slowly progressive cerebellar syndrome, mild cognitive impairment, pyramidal signs, and rapid eye movement sleep behavior disorder with imaging abnormalities on the DaTSCAN single-photon emission computed tomography indicating an alteration in the presynaptic dopaminergic system...
November 2017: Neurologist
https://www.readbyqxmd.com/read/29095104/pathological-examination-is-required-for-the-case-of-rapidly-progressive-dementia-with-only-positive-result-of-rt-quic-assay
#16
Yuichi Hayashi
No abstract text is available yet for this article.
November 2, 2017: Prion
https://www.readbyqxmd.com/read/29082576/longitudinal-cortical-thinning-and-cognitive-decline-in-patients-with-early-versus-late-stage-subcortical-vascular-mci
#17
Juyoun Lee, Sang Won Seo, Jin-Ju Yang, Young Kyoung Jang, Jin San Lee, Yeo Jin Kim, Juhee Chin, Jong Min Lee, Sung Tae Kim, Kyung-Han Lee, Jae Hong Lee, Jae Seung Kim, Seonwoo Kim, Heejin Yoo, Ae Young Lee, Duk L Na, Hee Jin Kim
BACKGROUND AND PURPOSE: Biomarker changes in cognitively impaired patients with small vessel disease is largely unknown. We evaluated the rate of amyloid/lacune progression, cortical thinning, and cognitive decline in subcortical vascular mild cognitive impairment (svMCI) patients. METHODS: Seventy two svMCI patients were divided into early stage (ES-svMCI, n=39) and late stage (LS-svMCI, n=33) according to their clinical dementia rating sum of box score. Patients were annually followed-up with neuropsychological tests and brain MRI for three years, and underwent a second [(11) C] Pittsburgh compound B (PiB)-PET within a mean interval of 32...
October 30, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/29080930/dysfunction-of-gabaergic-neurons-in-the-parafacial-zone-mediates-sleep-disturbances-in-a-streptozotocin-induced-rat-model-of-sporadic-alzheimer-s-disease
#18
Jin-Zhi Song, Su-Ying Cui, Xiang-Yu Cui, Xiao Hu, Yu-Nu Ma, Hui Ding, Hui Ye, Yong-He Zhang
Sleep disturbances are prevalent among patients with Alzheimer's disease (AD) and often precede the onset and progression of dementia. However, there are no reliable animal models for investigating sleep disturbances in patients with sporadic AD (sAD), which accounts for more than 90% of all AD cases. In the present study, we characterize the sleep/wake cycles and explore a potential mechanism underlying sleep disturbance in a rat model of sAD induced via intracerebroventricular (icv) injection of streptozotocin (STZ)...
October 28, 2017: Metabolic Brain Disease
https://www.readbyqxmd.com/read/29077801/introduction-to-the-special-issue-on-clinical-neuropsychology-of-movement-disorders
#19
Alexander I Tröster
The special issue on the clinical neuropsychology of movement disorders provides an overview for the non-subspecialist clinical neuropsychologist and other clinical neuroscientists of the neuropsychological features, assessment and treatment of Parkinson's disease and Lewy body dementias, atypical parkinsonian disorders (corticobasal syndrome, progressive supranuclear palsy, and multiple system atrophy), Huntington's disease, dystonia, and amyotrophic lateral sclerosis. Additionally, articles provide overviews of neuropsychological and ethical issues related to deep brain stimulation and a discussion of non-pharamcologic and non-invasive treatment of cognitive dysfunction in Parkinson's disease...
November 1, 2017: Archives of Clinical Neuropsychology: the Official Journal of the National Academy of Neuropsychologists
https://www.readbyqxmd.com/read/29067342/the-effects-of-noncoding-aquaporin-4-single-nucleotide-polymorphisms-on-cognition-and-functional-progression-of-alzheimer-s-disease
#20
Kevin G Burfeind, Charles F Murchison, Shawn K Westaway, Matthew J Simon, Deniz Erten-Lyons, Jeffrey A Kaye, Joseph F Quinn, Jeffrey J Iliff
INTRODUCTION: The glymphatic system is a brain-wide perivascular network that facilitates clearance of proteins, including amyloid β, from the brain interstitium through the perivascular exchange of cerebrospinal fluid and interstitial fluid. The astrocytic water channel aquaporin-4 (AQP4) is required for glymphatic system function, and impairment of glymphatic function in the aging brain is associated with altered AQP4 expression and localization. In human cortical tissue, alterations in AQP4 expression and localization are associated with Alzheimer's disease (AD) status and pathology...
September 2017: Alzheimer's & Dementia: Translational Research & Clinical Interventions
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