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https://www.readbyqxmd.com/read/28803444/atypical-parkinsonian-syndromes-a-general-neurologist-s-perspective
#1
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/28802934/psychosis-in-parkinson-s-disease
#2
Dominic H Ffytche, Dag Aarsland
Although illusions, hallucinations and delusions did not play a prominent role in James Parkinson's original clinical descriptions, the longitudinal view of disease progression he advocated has important lessons for the study of such symptoms today. A focus on longitudinal progression rather than individual symptoms led to the concept of PD psychosis-a spectrum of positive symptoms in Parkinson's disease. The publication of criteria for PD psychosis in 2007 helped unify the disparate set of symptoms, raising their profile and resulting in a rapid expansion of literature focussing on clinical aspects, mechanisms, and treatment...
2017: International Review of Neurobiology
https://www.readbyqxmd.com/read/28768349/-bilateral-hippocampal-primary-cns-lymphoma-unusual-differential-diagnosis-of-rapid-progressive-dementia
#3
Stefan Weidauer, Sylvia Hartmann, Jan Fabricius
We report on a 63-year old man suffering from rapid progressive dementia due to bilateral hippocampal primary CNS lymphoma. Early diagnosis especially in secondary dementia is essential because of potentially treatable aetiologies with regression of cognitive and behaviour abnormalities. Characteristic imaging findings especially in inflammatory and neoplastic lesions affecting the hippocampal structures are discussed.
July 2017: Fortschritte der Neurologie-Psychiatrie
https://www.readbyqxmd.com/read/28756447/a-spatial-dashboard-for-alzheimer-s-disease-in-new-south-wales
#4
Hamish Robertson, Nick Nicholas, Amit Dhagat, Joanne Travaglia
This paper illustrates a proof of concept scenario for the application of comprehensive data visualisation methods in the rapidly changing aged care sector. The scenario we explored is population ageing and the dementias with an emphasis on the spatial effects of change over time at the Statistical Area 2 (SA2) level for the state of New South Wales. We did this using a combination of methods, culminating in the use of the Tableau software environment to explore the intersections of demography, epidemiology and their formal cost of care implications...
2017: Studies in Health Technology and Informatics
https://www.readbyqxmd.com/read/28755001/identification-of-clusters-of-rapid-and-slow-decliners-among-subjects-at-risk-for-alzheimer-s-disease
#5
Dragan Gamberger, Nada Lavrač, Shantanu Srivatsa, Rudolph E Tanzi, P Murali Doraiswamy
The heterogeneity of Alzheimer's disease contributes to the high failure rate of prior clinical trials. We analyzed 5-year longitudinal outcomes and biomarker data from 562 subjects with mild cognitive impairment (MCI) from two national studies (ADNI) using a novel multilayer clustering algorithm. The algorithm identified homogenous clusters of MCI subjects with markedly different prognostic cognitive trajectories. A cluster of 240 rapid decliners had 2-fold greater atrophy and progressed to dementia at almost 5 times the rate of a cluster of 184 slow decliners...
July 28, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28750578/dynamic-predictions-in-bayesian-functional-joint-models-for-longitudinal-and-time-to-event-data-an-application-to-alzheimer-s-disease
#6
Kan Li, Sheng Luo
In the study of Alzheimer's disease, researchers often collect repeated measurements of clinical variables, event history, and functional data. If the health measurements deteriorate rapidly, patients may reach a level of cognitive impairment and are diagnosed as having dementia. An accurate prediction of the time to dementia based on the information collected is helpful for physicians to monitor patients' disease progression and to make early informed medical decisions. In this article, we first propose a functional joint model to account for functional predictors in both longitudinal and survival submodels in the joint modeling framework...
January 1, 2017: Statistical Methods in Medical Research
https://www.readbyqxmd.com/read/28741078/-analysis-of-2000-surgically-treated-proximal-femoral-fractures-multiple-variables-influence-mortality
#7
Franz Müller, Michael Galler, Tanja Kottmann, Michael Zellner, Christian Bäuml, Bernd Füchtmeier
BACKGROUND: Due to demographic change, more proximal femoral fractures can be expected in the future. However, accurate growth rates as well as follow-up research extending more than 1 year postsurgery are still lacking. MATERIALS AND METHODS: First, we defined inclusion and exclusion criteria for the retrospective cohort study. Based on these, we collected all surgical interventions conducted between 1 January 2006 and 31 December 2015. For a total of 2000 consecutive procedures, we retrieved and analysed a total of 12 variables influencing mortality...
July 24, 2017: Der Unfallchirurg
https://www.readbyqxmd.com/read/28726030/intravascular-large-b-cell-lymphoma-presenting-clinically-as-rapidly-progressive-dementia
#8
REVIEW
F M Brett, D Chen, T Loftus, Y Langan, S Looby, S Hutchinson
BACKGROUND: In patients presenting with rapidly progressive dementia, prion disease may enter the differential diagnosis. The commonest malignancies masquerading as prion disease are primary CNS lymphoma and intravascular large B-cell lymphoma, both rare and difficult to diagnose without brain biopsy. CASE PRESENTATION: This 82-year-old lady with a past history of hypertension, presented with rapidly progressive cognitive impairment and ataxia. The possibility of sCJD was raised...
July 19, 2017: Irish Journal of Medical Science
https://www.readbyqxmd.com/read/28704802/trajectories-of-depressive-symptoms-and-their-relationship-to-the-progression-of-dementia
#9
Maria Lage Barca, Karin Persson, Rannveig Eldholm, Jūratė Šaltytė Benth, Hege Kersten, Anne-Brita Knapskog, Ingvild Saltvedt, Geir Selbaek, Knut Engedal
BACKGROUND: The relationship between progression of Alzheimer's disease and depression and its underlying mechanisms has scarcely been studied. METHODS: A sample of 282 outpatients with Alzheimer's disease (AD; 105 with amnestic AD and 177 with Alzheimer's dementia) from Norway were followed up for an average of two years. Assessment included Cornell Scale for Depression in Dementia and Clinical Dementia Rating Scale (CDR) at baseline and follow-up to examine the relationship between AD and depression...
November 2017: Journal of Affective Disorders
https://www.readbyqxmd.com/read/28692821/dental-care-utilization-in-patients-with-different-types-of-dementia-a%C3%A2-longitudinal-nationwide-study-of-58-037-individuals
#10
Seyed-Mohammad Fereshtehnejad, Sara Garcia-Ptacek, Dorota Religa, Jacob Holmer, Kåre Buhlin, Maria Eriksdotter, Gunilla Sandborgh-Englund
INTRODUCTION: Dementia may be associated with discontinuation of regular dental checkups, which in turn results in poorer oral health. METHODS: We investigated the trend of change in dental care utilization and the number of teeth before and after being diagnosed with dementia. Longitudinal cognitive- and dental health-related information were merged using data on 58,037 newly diagnosed individuals from the Swedish Dementia Registry and Swedish Dental Health Register during 2007 to 2015...
July 8, 2017: Alzheimer's & Dementia: the Journal of the Alzheimer's Association
https://www.readbyqxmd.com/read/28680785/a-case-report-of-probable-sporadic-creutzfeldt-jakob-disease-how-to-approach-early-diagnosis
#11
Bowei Tan, Carlos Morales Mangual, Iftekhar Mahmud, Nosakhare D Tongo, Larisa Mararenko, Arthur Kay
Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare and fatal spongiform encephalopathy characterized by rapidly progressive dementia and myoclonus. The rarity of this disease and varied initial symptoms make the early diagnosis fairly challenging. Here, we present a case initially admitted for confusion and bizarre behaviors. She had acute deterioration of mental status, akinetic mutism, and myoclonus jerks four weeks later. Cerebrospinal fluid (CSF) analysis was positive for protein 14-3-3. Brain magnetic resonance imaging (MRI) showed hyperintensities in the bilateral cortex, basal ganglia, and thalami in diffusion-weighted imaging (DWI)...
May 30, 2017: Curēus
https://www.readbyqxmd.com/read/28671123/prion-protein-interactome-identifying-novel-targets-in-slowly-and-rapidly-progressive-forms-of-alzheimer-s-disease
#12
Saima Zafar, Mohsin Shafiq, Neelam Younas, Matthias Schmitz, Isidre Ferrer, Inga Zerr
Rapidly progressive Alzheimer's disease (rpAD) is a variant of AD distinguished by a rapid decline in cognition and short disease duration from onset to death. While attempts to identify rpAD based on biomarker profile classifications have been initiated, the mechanisms which contribute to the rapid decline and prion mimicking heterogeneity in clinical signs are still largely unknown. In this study, we characterized prion protein (PrP) expression, localization, and interactome in rpAD, slow progressive AD, and in non-dementia controls...
2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28668775/towards-an-early-clinical-diagnosis-of-sporadic-cjd-vv2-ataxic-type
#13
Simone Baiardi, Anna Magherini, Sabina Capellari, Veronica Redaelli, Anna Ladogana, Marcello Rossi, Fabrizio Tagliavini, Maurizio Pocchiari, Giorgio Giaccone, Piero Parchi
INTRODUCTION: Sporadic Creutzfeldt-Jakob disease (sCJD) includes a broad spectrum of clinical-pathological subtypes, which complicates the clinical differential diagnosis with other rapidly progressive neurological syndromes. AIM: To provide a better characterisation of clinical features and results of diagnostic investigations, especially at an early disease stage, in patients with sCJDVV2, the second most common sCJD subtype. METHODS: We evaluated neurological symptoms/signs, and results of brain diffusion-weighted resonance imaging (DW-MRI), electroencephalographic recordings (EEG) and cerebrospinal fluid (CSF) biomarker studies in 120 patients with a definite (n=93) or probable (n=27) diagnosis of sCJDVV2...
July 1, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28660843/rapidly-progressive-fronto-temporal-dementia-ftd-associated-with-frontotemporal-lobar-degeneration-ftld-in-the-presence-of-fused-in-sarcoma-fus-protein-a-rare-sporadic-and-aggressive-form-of-ftd
#14
Nicholas I Bradfield, Catriona McLean, John Drago, David G Darby, David Ames
Fronto-temporal dementia (FTD) associated with Fused in Sarcoma (FUS) protein accumulation is an uncommon cause of FTD with a distinct syndrome of young age onset behavioral variant FTD, without a family history of FTD and caudate atrophy. We present a sporadic case of a 61-year-old patient with mixed features of both behavioral variant FTD with later semantic language dissolution associated with pathologically proven FUS. He was older than usual for FUS pathology, his course was rapidly progressive, and he had atypical language features...
June 29, 2017: International Psychogeriatrics
https://www.readbyqxmd.com/read/28658400/high-phenotypic-variability-in-gerstmann-str%C3%A3-ussler-scheinker-disease
#15
Jerusa Smid, Adalberto Studart, Michele Christine Landemberger, Cleiton Fagundes Machado, Paulo Ribeiro Nóbrega, Nathalie Henriques Silva Canedo, Rodrigo Rizek Schultz, Michel Satya Naslavsky, Sérgio Rosemberg, Fernando Kok, Leila Chimelli, Vilma Regina Martins, Ricardo Nitrini
Gerstmann-Sträussler-Scheinker is a genetic prion disease and the most common mutation is p.Pro102Leu. We report clinical, molecular and neuropathological data of seven individuals, belonging to two unrelated Brazilian kindreds, carrying the p.Pro102Leu. Marked differences among patients were observed regarding age at onset, disease duration and clinical presentation. In the first kindred, two patients had rapidly progressive dementia and three exhibited predominantly ataxic phenotypes with variable ages of onset and disease duration...
June 2017: Arquivos de Neuro-psiquiatria
https://www.readbyqxmd.com/read/28655059/association-of-autonomic-dysfunction-with-disease-progression-and-survival-in-parkinson-disease
#16
Eduardo De Pablo-Fernandez, Carmen Tur, Tamas Revesz, Andrew J Lees, Janice L Holton, Thomas T Warner
Importance: Evidence suggests that development of autonomic dysfunction (AutD) may negatively affect disease course and survival in patients with synucleinopathies. However, the few available studies on Parkinson disease (PD) have conflicting results, comprise a small number of patients, have short follow-up periods, and lack pathologic confirmation of the diagnosis. Objective: To examine the association of time of onset of AutD with disease progression and survival in PD...
August 1, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28649609/insomnia-and-somnolence-in-idiopathic-rbd-a-prospective-cohort-study
#17
Ronald B Postuma, Jean-François Gagnon, Amelie Pelletier, Jacques Y Montplaisir
Although some sleep disorders are markers of prodromal Parkinson's disease and dementia with Lewy bodies, it is unclear whether insomnia and somnolence can predict disease. We assessed a large cohort of patients with idiopathic rapid eye movement sleep behavior disorder and age/sex matched controls, comparing the Epworth sleepiness scale, the Insomnia Severity Index, the Pittsburgh Sleep Quality Index, and polysomnographic variables. In those with repeated scales, we assessed change over time. Finally, we assessed whether sleep abnormalities predicted defined neurodegenerative disease...
2017: NPJ Parkinson's Disease
https://www.readbyqxmd.com/read/28649596/phenoconversion-from-probable-rapid-eye-movement-sleep-behavior-disorder-to-mild-cognitive-impairment-to-dementia-in-a-population-based-sample
#18
Youngsin Jung, Brendon P Boot, Michelle M Mielke, Tanis J Ferman, Yonas E Geda, Eric McDade, Teresa J H Christianson, David S Knopman, Erik K St Louis, Michael H Silber, Ronald C Petersen, Bradley F Boeve
INTRODUCTION: Rapid eye movement sleep behavior disorder (RBD) is strongly associated with synucleinopathies. In 2012, we reported an increased risk of mild cognitive impairment (MCI) and Parkinson disease (PD) in cognitively normal Olmsted County, Minnesota, residents, aged 70 to 89 years with probable RBD. Here, we examine their progression to dementia and other neurodegenerative phenotypes. METHODS: Fifteen participants with RBD who were diagnosed with either MCI or PD were longitudinally followed, and their subsequent clinical courses were reviewed...
2017: Alzheimer's & Dementia: Diagnosis, Assessment & Disease Monitoring
https://www.readbyqxmd.com/read/28641977/a-novel-fluorescent-probe-reveals-starvation-controls-the-commitment-of-amyloid-precursor-protein-to-the-lysosome
#19
Leanne K Hein, Pirjo M Apaja, Kathryn Hattersley, Randall H Grose, Jianling Xie, Christopher G Proud, Timothy J Sargeant
Alzheimer's disease is the most important cause of dementia but there is no therapy that has been demonstrated to stop or slow disease progression. Amyloid precursor protein (APP) is the source of amyloid-β (Aβ), which aggregates in Alzheimer's disease to form toxic oligomeric species. The endo-lysosomal system can clear APP and Aβ from the cell if these molecular species are trafficked through to the lysosome. Currently, there are no easy methods available for the analysis of lysosomal APP trafficking. We therefore generated a fusion protein (tandem-fluorescent, or tf-APP) that allows detection of changes in APP trafficking using accessible techniques such as flow cytometry...
June 19, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28631971/rapidly-progressive-dementia-an-unusual-cause
#20
Aastha Takkar, Veenu Singla, Manish Modi, Vivek Gupta, Manoj K Goyal, Vivek Lal
Brain metastases are known to be present with a history of increased intra-cranial pressure headache, seizures or altered sensorium. Corresponding to the clinical brain metastases are the most feared complication of a systemic cancer. Classically, brain metastases are associated with significant morbidity and are known to present with a history of increased intracranial pressure headache, seizures or altered sensorium. Neuroimaging is usually suggestive of massive peri-lesional edema and mass effect surrounding the space occupying lesions...
August 2017: Neuroradiology Journal
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