Read by QxMD icon Read

Rapid dementia

Celeste Sassi, Michael A Nalls, Perry G Ridge, Jesse R Gibbs, Michelle K Lupton, Claire Troakes, Katie Lunnon, Safa Al-Sarraj, Kristelle S Brown, Christopher Medway, Jenny Lord, James Turton, Jose Bras, Sonja Blumenau, Mareike Thielke, Christa Josties, Dorette Freyer, Annette Dietrich, Monia Hammer, Michael Baier, Ulrich Dirnagl, Kevin Morgan, John F Powell, John S Kauwe, Carlos Cruchaga, Alison M Goate, Andrew B Singleton, Rita Guerreiro, Angela Hodges, John Hardy
Mendelian adult-onset leukodystrophies are a spectrum of rare inherited progressive neurodegenerative disorders affecting the white matter of the central nervous system. Among these, cerebral autosomal dominant and recessive arteriopathy with subcortical infarcts and leukoencephalopathy, cerebroretinal vasculopathy, metachromatic leukodystrophy, hereditary diffuse leukoencephalopathy with spheroids, and vanishing white matter disease present with rapidly progressive dementia as dominant feature and are caused by mutations in NOTCH3, HTRA1, TREX1, ARSA, CSF1R, EIF2B1, EIF2B2, EIF2B3, EIF2B4, and EIF2B5, respectively...
February 2, 2018: Neurobiology of Aging
Yi-Qi Lin, Sheng-Di Chen
Increasing evidence indicates a strong association between rapid eye movement sleep behavior disorder (RBD) and Parkinson's disease - cognitive impairment (PD-CI). Numerous longitudinal and cross-sectional studies have shown that RBD may be an important risk factor and predictor of Parkinson's disease - mild cognitive impairment (PD-MCI) and Parkinson's disease dementia (PDD); which may be explained by the association of mechanisms between RBD and PD-CI, including neurotransmitter alterations, genetic mutation, neuroinflammation, alpha-synuclein inclusion, abnormal cerebral metabolism and cortical activity slowing...
April 2018: Sleep Medicine
Richard Salazar
INTRODUCTION: Sporadic Creutzfeldt-Jakob disease is a prion disease characterized by rapidly progressive dementia, ataxia and myoclonus. Atypical phenotype masquerading as stroke, movement disorders or autoimmune encephalitis have been described. Here, I report a probable case of sCJD with an atypical presentation associated with anti-Zic4 antibody and review the literature of neuronal antibodies in CJD. CASE REPORT: A 70 year-old gentleman is admitted with a 2-month history of recurrent stroke-like symptoms associated with behavioral disturbances, gait ataxia and rapidly progressive dementia...
March 5, 2018: Clinical Neurology and Neurosurgery
Annie Lannuzel, Régine Edragas, Angéla Lackmy, Benoit Tressières, Véronique Pelonde, Mireille Edimo Nana Kaptué, Sylvie Mécharles, Alexis Demas, Billy François, Eavan McGovern, Marie Vidailhet, Bertrand Gaymard, Emmanuel Roze
BACKGROUND: A high prevalence of an atypical levodopa-resistant parkinsonism has been reported in the Caribbean island of Guadeloupe. These seminal observations have not been replicated or extended to neighbouring populations who share genetic and environmental characteristics. METHODS: To further characterise this atypical parkinsonism we prospectively investigated 305 consecutive patients with neurodegenerative parkinsonism in a community-based population from Guadeloupe and Martinique, a neighbouring French Caribbean island where the population has similar environmental and genetic backgrounds...
February 6, 2018: Journal of the Neurological Sciences
Morten Gersel Stokholm, Alex Iranzo, Karen Østergaard, Mónica Serradell, Marit Otto, Kristina Bacher Svendsen, Alicia Garrido, Dolores Vilas, Peter Parbo, Per Borghammer, Joan Santamaria, Arne Møller, Carles Gaig, David J Brooks, Eduardo Tolosa, Nicola Pavese
BACKGROUND: The majority of patients diagnosed with idiopathic rapid eye movement sleep behaviour disorder (iRBD) progress over time to a Lewy-type α-synucleinopathy such as Parkinson's disease or dementia with Lewy bodies. This in vivo molecular imaging study aimed to investigate if extrastriatal monoaminergic systems are affected in iRBD patients and if this coincides with neuroinflammation. METHODS: We studied twenty-one polysomnography-confirmed iRBD patients with18 F-DOPA and11 C-PK11195 positron emission tomography (PET) to investigate extrastriatal monoaminergic function and microglial activation...
March 6, 2018: Neurobiology of Disease
Tomaž Rus, Bogdan Lorber, Maja Trošt, Srečko Dobrecovič, Nuška Čakš Jager, Mara Popović, Milica G Kramberger
Background: Creutzfeldt-Jakob disease (CJD) is a rare fatal neurodegenerative disorder presenting with rapid cognitive decline and additional signs. The clinical characteristics of an increasing number of sporadic CJD (sCJD) patients admitted to the Ljubljana University Medical Centre are presented as well as the incidence of sCJD in Slovenia in 2015 compared to previous years. Methods: We investigated patients presenting with rapidly progressive dementia and at least one additional sign...
January 2018: Dementia and Geriatric Cognitive Disorders Extra
Malaz Boustani, Catherine A Alder, Craig A Solid
OBJECTIVES: To describe the essential components of an Agile Implementation (AI) process, which rapidly and effectively implements evidence-based healthcare solutions, and present a case study demonstrating its utility. DESIGN: Case demonstration study. SETTING: Integrated, safety net healthcare delivery system in Indianapolis. PARTICIPANTS: Interdisciplinary team of clinicians and administrators. MEASUREMENTS: Reduction in dementia symptoms and caregiver burden; inpatient and outpatient care expenditures...
March 7, 2018: Journal of the American Geriatrics Society
Ina Djonlagic, Daniel Aeschbach, Stephanie Litwack Harrison, Dennis Dean, Kristine Yaffe, Sonia Ancoli-Israel, Katie Stone, Susan Redline
The pathophysiological processes of Alzheimer's dementia predate its clinical manifestation. Sleep disturbances can accelerate the aging process and are common features of dementia. This study examined whether quantitative sleep electroencephalogram changes predate the clinical development of mild cognitive impairment and/or incident dementia. We collected data from a nested case-control sample of women (mean age 83 years) from the Sleep and Cognition Study, an ancillary study to the longitudinal Study of Osteoporotic Fractures, who were characterized as cognitively normal at the time of a baseline polysomnography study (Study of Osteoporotic Fractures visit 8) based on a Mini-Mental Status Exam (MMSE) score >24...
March 5, 2018: Journal of Sleep Research
Alissa Knight, John Fouyaxis, Geoff Jarrad, Kinga Beski, Gerald Cho, Niranjan Bidargaddi
The rapid ageing of the population is a worldwide inexorable demographic transformation. At a time of immense social, political and economic change, the growing elderly population is at the forefront of global burden, placing an increasing strain on the federal, state, and local budgets. Many public policy responses to the impending ageing epidemic have begun, particularly with regards to dementia prevention and quality of life for older adults. However, to date, the fruition of such efforts remains to be discovered...
2018: Studies in Health Technology and Informatics
John Grundy, Kon Mouzakis, Rajesh Vasa, Andrew Cain, Maheswaree Curumsing, Mohamed Abdelrazek, Niroshine Fernando
By the 2050, it is estimated that the proportion of people over the age of 80 will have risen from 3.9% to 9.1% of population of Organisation for Economic Cooperation and Development countries. A large proportion of these people will need significant help to manage various chronic illnesses, including dementia, heart disease, diabetes, limited physical movement and many others. Current approaches typically focus on acute episodes of illness and are not well designed to provide adequately for daily living care support...
2018: Studies in Health Technology and Informatics
John R Hodges, Olivier Piguet
The landscape of frontotemporal dementia (FTD) has evolved remarkably in recent years and is barely recognizable from two decades ago. Knowledge of the clinical phenomenology, cognition, neuroimaging, genetics, pathology of the different subtypes of FTD, and their relations to other neurodegenerative conditions, has increased rapidly, due in part, to the growing interests into these neurodegenerative brain conditions. This article reviews the major advances in the field of FTD over the past 20 years, focusing primarily on the work of Frontier, the frontotemporal dementia clinical research group, based in Sydney, Australia...
February 28, 2018: Journal of Alzheimer's Disease: JAD
Dong Gyu Park, Soojin Lee, Young Min Moon, Duk L Na, Ji Hyang Jeong, Kyung Won Park, Yoon Hwan Lee, Tae Sung Lim, Seong Hye Choi, So Young Moon
BACKGROUND AND PURPOSE: We investigated predictors of institutionalization in patients with Alzheimer's disease (AD) in South Korea. METHODS: In total, 2,470 patients with AD aged 74.5±7.8 years (mean±standard deviation, 68.1% females) were enrolled from November 2005 to December 2013. The dates of institutionalization were identified from the public Long-Term-Care Insurance program in January 2014. We used a Cox proportional-hazards model to identify predictors for future institutionalization among characteristics at the time of diagnosis in 2,470 AD patients...
February 28, 2018: Journal of Clinical Neurology
Esteban Luna, Samantha C Decker, Dawn M Riddle, Anna Caputo, Bin Zhang, Tracy Cole, Carrie Caswell, Sharon X Xie, Virginia M Y Lee, Kelvin C Luk
The accumulation of misfolded α-synuclein (aSyn) and neuron loss define several neurodegenerative disorders including Parkinson's disease (PD) and dementia with Lewy bodies (DLB). However, the precise relationship between pathology and neurotoxicity and why these processes disproportionately affect certain neuron subpopulations are poorly understood. We show here that Math2-expressing neurons in the hippocampal Cornu ammonis (CA), a region significantly affected by aSyn pathology in advanced PD and DLB, are highly susceptible to pathological seeding with pre-formed fibrils (PFFs), in contrast to dentate gyrus neurons, which are relatively spared...
March 3, 2018: Acta Neuropathologica
Brenna Cholerton, Catherine O Johnson, Brian Fish, Joseph F Quinn, Kathryn A Chung, Amie L Peterson-Hiller, Liana S Rosenthal, Ted M Dawson, Marilyn S Albert, Shu-Ching Hu, Ignacio F Mata, James B Leverenz, Kathleen L Poston, Thomas J Montine, Cyrus P Zabetian, Karen L Edwards
INTRODUCTION: Identification of factors associated with progression of cognitive symptoms in Parkinson's disease (PD) is important for treatment planning, clinical care, and design of future clinical trials. The current study sought to identify whether prediction of cognitive progression is aided by examining baseline cognitive features, and whether this differs according to stage of cognitive disease. METHODS: Participants with PD in the Pacific Udall Center Clinical Consortium who had longitudinal data available and were nondemented at baseline were included in the study (n = 418)...
February 9, 2018: Parkinsonism & related Disorders
Leonel T Takada, Mee-Ohk Kim, Stacy Metcalf, Ignacio Illán Gala, Michael D Geschwind
Genetic prion diseases (gPrDs) are caused by autosomal-dominant mutations in the prion protein gene (PRNP). Although the first PRNP mutations identified, and most since, are PRNP missense, octapeptide repeat insertions, deletion and nonsense mutations have now also been shown to cause gPrD. Based on clinicopathologic features of familial disease, gPrDs historically have been classified into three forms: familial Jakob-Creutzfeldt disease, Gerstmann-Sträussler-Scheinker disease, and fatal familial insomnia...
2018: Handbook of Clinical Neurology
Takuya Kobayashi, Masafumi Kubota, Toshiyuki Takahashi, Ayaka Nakasato, Taro Nomura, Junichi Furuya, Hisatomo Kondo
PURPOSE: One of the most prominent issues in a super-aging society is the rapid increase in dementia patients. Cross-sectional studies in dentistry have indicated that patients with dementia have worse oral health compared to healthy people. The purpose of this study was to clarify the influence of tooth loss on brain structure by comparing the volumes of gray matter (GM) and white matter (WM) between edentulous and dentulous subjects. METHODS: Subjects were recruited from the Denture Clinic at Iwate Medical University Hospital Dental Center...
February 20, 2018: Journal of Prosthodontic Research
Bilal Khalil, Dmytro Morderer, Phillip L Price, Feilin Liu, Wilfried Rossoll
The development, maturation, and maintenance of the mammalian nervous system rely on complex spatiotemporal patterns of gene expression. In neurons, this is achieved by the expression of differentially localized isoforms and specific sets of mRNA-binding proteins (mRBPs) that regulate RNA processing, mRNA trafficking, and local protein synthesis at remote sites within dendrites and axons. There is growing evidence that axons contain a specialized transcriptome and are endowed with the machinery that allows them to rapidly alter their local proteome via local translation and protein degradation...
February 17, 2018: Brain Research
Rodolfo Savica, Boeve F Bradley, Michelle M Mielke
Importance: This article reviews the epidemiological evidence of features of α-synucleinopathies that precede clinical onset of disease, proposes a clinical timeline, and attempts to define the different premotor and clinical phenotypes associated with α-synucleinopathies. Observations: The pathological hallmarks of the α-synucleinopathies (Parkinson disease, Parkinson disease dementia, dementia with Lewy bodies, and multisystem atrophy) begin years before a clinical diagnosis...
February 19, 2018: JAMA Neurology
Iris Zahirovic, Gustav Torisson, Carina Wattmo, Elisabet Londos
BACKGROUND: Elderly persons with a dementia diagnosis often suffer from different neuropsychiatric symptoms (NPS) such as delusions, hallucinations, depression, anxiety, irritability and agitation. Currently, the medical treatment for NPS consists mostly of psychotropic medication such as hypnotics/sedatives, anxiolytics and antipsychotics. In elderly persons with dementia, usage of antipsychotics is less appropriate because of the risk of side effects such as parkinsonism, rapid cognitive decline, cerebrovascular events and finally mortality...
February 17, 2018: BMC Geriatrics
Susan D Shenkin, Christopher Fox, Mary Godfrey, Najma Siddiqi, Steve Goodacre, John Young, Atul Anand, Alasdair Gray, Joel Smith, Tracy Ryan, Janet Hanley, Allan MacRaild, Jill Steven, Polly L Black, Julia Boyd, Christopher J Weir, Alasdair Mj MacLullich
INTRODUCTION: Delirium is a severe neuropsychiatric syndrome of rapid onset, commonly precipitated by acute illness. It is common in older people in the emergency department (ED) and acute hospital, but greatly under-recognised in these and other settings. Delirium and other forms of cognitive impairment, particularly dementia, commonly coexist. There is a need for a rapid delirium screening tool that can be administered by a range of professional-level healthcare staff to patients with sensory or functional impairments in a busy clinical environment, which also incorporates general cognitive assessment...
February 10, 2018: BMJ Open
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"