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Rapid progressive dementia

E Candeias, A I Duarte, I Sebastião, M A Fernandes, A I Plácido, C Carvalho, S Correia, R X Santos, R Seiça, M S Santos, C R Oliveira, P I Moreira
Type 2 diabetes (T2D) is a highly concerning public health problem of the twenty-first century. Currently, it is estimated that T2D affects 422 million people worldwide with a rapidly increasing prevalence. During the past two decades, T2D has been widely shown to have a major impact in the brain. This, together with the cognitive decline and increased risk for dementia upon T2D, may arise from the complex interaction between normal brain aging and central insulin signaling dysfunction. Among the several features shared between T2D and some neurodegenerative disorders (e...
October 11, 2016: Molecular Neurobiology
Woojae Myung, Chunsoo Lee, Jin Hong Park, Sook-Young Woo, Seonwoo Kim, Sangha Kim, Jae Won Chung, Hyo Shin Kang, Shinn-Won Lim, Junbae Choi, Duk L Na, Seong Yoon Kim, Jae-Hong Lee, Seol-Heui Han, Seong Hye Choi, Sang Yun Kim, Bernard J Carroll, Doh Kwan Kim
High occupational attainment has been known as a marker of cognitive reserve. Previous studies in the general population have shown that high occupational attainment is associated with reduced risk of Alzheimer's disease (AD). However, few studies have assessed the effect of occupational attainment on the clinical course of mild cognitive impairment (MCI). In this study, we evaluated whether individuals with high occupational attainment show more rapid progression from MCI to AD. Participants (n = 961) with MCI were recruited from a nationwide, hospital-based multi-center cohort, and were followed for up to 60 months (median: 17...
September 20, 2016: Journal of Alzheimer's Disease: JAD
Maria Gabriella Vita, Dorina Tiple, Alessandra Bizzarro, Anna Ladogana, Elisa Colaizzo, Sabina Capellari, Marcello Rossi, Piero Parchi, Carlo Masullo, Maurizio Pocchiari
We report a case of rapidly evolving neurological disease in a patient with neuropathological lesions of Creutzfeldt-Jakob disease (CJD), Lewy body dementia (LBD), chronic subcortical vascular encephalopathy and meningothelial meningioma. The coexistence of severe multiple pathologies in a single patient strengthens the need to perform accurate clinical differential diagnoses in rapidly progressive dementias.
September 16, 2016: Neuropathology: Official Journal of the Japanese Society of Neuropathology
Marcos Ramírez, Andrés Gallardo, Aarón Vidal, Sebastián Cornejo, Darío Ramírez, Danilo Medinas, Gonzalo Bustamante, Renzo Pasquali, Claudio Hetz
Creutzfeldt-Jakob disease has a higher incidence in Chile than in other countries. The post mortem pathological characterization of brain tissue is necessary to reach a definitive diagnosis. We report a 73 years old man with a history compatible with of a rapidly progressive dementia, in which the first electroencephalographic study showed a pattern consistent with non-convulsive status epilepticus. Besides discarding this diagnosis, it was necessary to rule out other causes of rapidly progressive dementia such as Hashimoto encephalopathy...
June 2016: Revista Médica de Chile
André Karch, Franc Llorens, Matthias Schmitz, Amandeep Arora, Saima Zafar, Peter Lange, Christian Schmidt, Inga Zerr
BACKGROUND: Cerebrospinal fluid (CSF) biomarkers are routinely used for the differential diagnosis of rapidly progressive dementia, but are also affected by patients' characteristics. OBJECTIVE: To assess if stratification by age, sex, and genetic risk factors improves the accuracy of cerebrospinal fluid (CSF) biomarkers in patients with rapidly progressive dementia. METHODS: 1,538 individuals with sporadic Creutzfeldt-Jakob disease (CJD), 173 with classic Alzheimer's disease (cAD), 37 with rapidly progressive Alzheimer's disease (rpAD), and 589 without signs of dementia were included in this retrospective diagnostic study...
September 2, 2016: Journal of Alzheimer's Disease: JAD
Jonathan D Rohrer, Ione O C Woollacott, Katrina M Dick, Emilie Brotherhood, Elizabeth Gordon, Alexander Fellows, Jamie Toombs, Ronald Druyeh, M Jorge Cardoso, Sebastien Ourselin, Jennifer M Nicholas, Niklas Norgren, Simon Mead, Ulf Andreasson, Kaj Blennow, Jonathan M Schott, Nick C Fox, Jason D Warren, Henrik Zetterberg
OBJECTIVE: To investigate serum neurofilament light chain (NfL) concentrations in frontotemporal dementia (FTD) and to see whether they are associated with the severity of disease. METHODS: Serum samples were collected from 74 participants (34 with behavioral variant FTD [bvFTD], 3 with FTD and motor neuron disease and 37 with primary progressive aphasia [PPA]) and 28 healthy controls. Twenty-four of the FTD participants carried a pathogenic mutation in C9orf72 (9), microtubule-associated protein tau (MAPT; 11), or progranulin (GRN; 4)...
September 27, 2016: Neurology
Marie Y Davis, Catherine O Johnson, James B Leverenz, Daniel Weintraub, John Q Trojanowski, Alice Chen-Plotkin, Vivianna M Van Deerlin, Joseph F Quinn, Kathryn A Chung, Amie L Peterson-Hiller, Liana S Rosenthal, Ted M Dawson, Marilyn S Albert, Jennifer G Goldman, Glenn T Stebbins, Bryan Bernard, Zbigniew K Wszolek, Owen A Ross, Dennis W Dickson, David Eidelberg, Paul J Mattis, Martin Niethammer, Dora Yearout, Shu-Ching Hu, Brenna A Cholerton, Megan Smith, Ignacio F Mata, Thomas J Montine, Karen L Edwards, Cyrus P Zabetian
Importance: Parkinson disease (PD) is heterogeneous in symptom manifestation and rate of progression. Identifying factors that influence disease progression could provide mechanistic insight, improve prognostic accuracy, and elucidate novel therapeutic targets. Objective: To determine whether GBA mutations and the E326K polymorphism modify PD symptom progression. Design, Setting, and Participants: The entire GBA coding region was screened for mutations and E326K in 740 patients with PD enrolled at 7 sites from the PD Cognitive Genetics Consortium...
October 1, 2016: JAMA Neurology
Julia Heller, Nikolina Brcina, Imis Dogan, Florian Holtbernd, Sandro Romanzetti, Jörg B Schulz, Johannes Schiefer, Kathrin Reetz
Idiopathic rapid eye movement (REM) sleep behavior disorder (RBD) is a parasomnia characterized by the loss of physiological atonia of skeletal muscles with abnormal behavior during dream sleep. RBD may be the initial manifestation of neurodegenerative diseases, particularly of α-synucleinopathies such as Parkinson's disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA). However, gauging the individual risk of subsequent phenoconversion and making assumptions on the type of disease that may subsequently follow RBD is challenging...
June 25, 2016: Sleep Medicine Reviews
Michael D Lovelace, Bianca Varney, Gayathri Sundaram, Nunzio F Franco, Mei Li Ng, Saparna Pai, Chai K Lim, Gilles J Guillemin, Bruce J Brew
The kynurenine pathway (KP) is the major metabolic pathway of the essential amino acid tryptophan (TRP). Stimulation by inflammatory molecules, such as interferon-γ (IFN-γ), is the trigger for induction of the KP, driving a complex cascade of production of both neuroprotective and neurotoxic metabolites, and in turn, regulation of the immune response and responses of brain cells to the KP metabolites. Consequently, substantial evidence has accumulated over the past couple of decades that dysregulation of the KP and the production of neurotoxic metabolites are associated with many neuroinflammatory and neurodegenerative diseases, including Parkinson's disease, AIDS-related dementia, motor neurone disease, schizophrenia, Huntington's disease, and brain cancers...
2016: Frontiers in Immunology
Helen O' Brien, Helen Mohan, Celia O' Hare, John Vincent Reynolds, Rose Anne Kenny
OBJECTIVE: The aim of this study was to highlight the vulnerability of the aging brain to surgery and anesthesia, examine postoperative cognitive outcomes, and recommend possible interventions. BACKGROUND: Surgeons are facing increasingly difficult ethical and clinical decisions given the rapidly expanding aging demographic. Cognitive function is not routinely assessed either preoperatively or postoperatively. Potential short and long-term cognitive implications are rarely discussed with the patient despite evidence that postoperative cognitive impairment occurs in up to 65% of older patients...
August 17, 2016: Annals of Surgery
A Vrdoljak, V Ivkovic, S Karanovic, Z Dika, V Domislovic, K Dapic, L Gallineo, E Ivandic, J Josipovic, I Vukovic, J Kos, M Laganovic, T Zeljkovic Vrkic, M Fistrek Prlic, I Pecin, M Fucek, J Sertic, N Leko, B Jelakovic
OBJECTIVE: Chronic kidney disease(CKD) is established CV risk factor, and already early renal impairment(RI) increases risk for hypertension(HT) and loss of renal function. It was reported that blood pressure (BP) and metabolic derangements are associated with glomerular hyperfiltration(GHF), and GHF increases risk of developing microalbuminuria (MA) in HT stage 1. Our aim was to analyze whether GHF predicts progression to HT and RI in apparently healthy subjects. DESIGN AND METHOD: Out of 954 subjects enrolled in ENAH follow-up study, 371 (137 m, 234 w; mean age = 46 years) were eligible for further analysis:100 with optimal, 72 with normal BP, 70 with PHT (high normal BP), and 129 with newly diagnosed untreated HT...
September 2016: Journal of Hypertension
Caroline Seer, Florian Lange, Dejan Georgiev, Marjan Jahanshahi, Bruno Kopp
Cognitive impairment is a common non-motor symptom of Parkinson's disease (PD), but the nature of cognitive changes varies considerably between individuals. According to the dual-syndrome hypothesis, one cluster of patients is characterized by deficits in executive function that may be related to fronto-striatal dysfunction. Other patients primarily show non-frontal cognitive impairments that progress rapidly to PD dementia (PDD). We provide a comprehensive review of event-related potential (ERP) studies to identify ERP measures substantiating the heterogeneity of cognitive impairment in PD...
August 3, 2016: Neuroscience and Biobehavioral Reviews
Alfried Kohlschütter, Angela Schulz
CLN2 disease is an inherited metabolic storage disorder caused by the deficiency of the lysosomal enzyme tripeptidyl peptidase 1 (TPP1). The disease affects mainly the brain and the retina and is characterized by progressive dysfunction of the central nervous system, leading to dementia, epilepsy, loss of motor function and blindness. The classical late infantile type begins at around three years of age with epilepsy and/or a standstill of psychomotor development, followed by a rapid loss of all abilities and death in childhood...
June 2016: Pediatric Endocrinology Reviews: PER
Stuart J McCarter, Erik K St Louis, Bradley F Boeve
Sleep disorders appear to be frequent comorbidities in patients with frontotemporal dementia (FTD). Insomnia and excessive daytime sleepiness commonly occur in patients with FTD and significantly contribute to caregiver burden and burnout. Sleep is severely fragmented in FTD patients, likely secondary to behavioral disturbances, other primary sleep disorders such as sleep disordered breathing and restless leg syndrome, and neurodegeneration of nuclei involved in sleep and wakefulness. Treatment of primary sleep disorders may improve excessive daytime sleepiness and sleep quality and may improve daytime cognitive functioning...
September 2016: Current Neurology and Neuroscience Reports
Liis Sabre, Mark Braschinsky, Pille Taba
BACKGROUND: Neurosyphilis is defined as any involvement of the central nervous system by the bacterium Treponema pallidum. Movement disorders as manifestations of syphilis have been reported quite rarely. CASE PRESENTATION: We report a case of a 42-year-old Russian man living in Estonia with rapidly progressive dementia and movement disorders manifesting as myoclonus, cerebellar ataxia and parkinsonism. The mini mental state examination score was 12/30. After excluding different neurodegenerative causes, further diagnostic testing was consistent with neurosyphilis...
2016: BMC Research Notes
A Dulamea, E Solomon
OBJECTIVE: Sporadic Creutzfeldt-Jakob disease (CJD) is a human prion disease, rapidly progressive and fatal, characterized by spongiform encephalopathy. The characteristic triad of signs - rapidly progressive dementia, myoclonus and periodic sharp wave complexes (PSWC) on electroencephalography (EEG) - usually appear in the late stages of the disease. The clinical diagnosis of CJD ante-mortem involves the exclusion of the rapidly progressive non-prionic dementias, the definitive diagnosis requiring brain tissue confirmation...
April 2016: Journal of Medicine and Life
Sarah Morgan, Richard W Orrell
INTRODUCTION: Amyotrophic lateral sclerosis (ALS) or motor neuron disease is a rapidly progressive neurodegenerative disorder. The primary involvement is of motor neurons in the brain, spinal cord and peripherally. There is secondary weakness of muscles and primary involvement of other brain regions, especially involving cognition. SOURCES OF DATA: Peer-reviewed journal articles and reviews. AREAS OF AGREEMENT: The pathogenesis of ALS remains largely unknown...
September 2016: British Medical Bulletin
Gamze Balci Camsari, Melissa E Murray, Neill R Graff-Radford
Many dementia subtypes have more shared signs and symptoms than defining ones. We review 8 cases with 4 overlapping syndromes and demonstrate how to distinguish the cases. These include focal cortical presentations of Alzheimer's disease (AD; posterior cortical atrophy and corticobasal syndrome [CBS]), fluent aphasia (semantic dementia and logopenic aphasia), late-onset slowly progressive dementia (hippocampal sclerosis and limbic predominant AD) and rapidly progressive dementia (Creutzfeldt-Jakob disease and limbic encephalitis)...
August 2016: Neurologic Clinics
Francesco Carlo Morabito, Maurizio Campolo, Nadia Mammone, Mario Versaci, Silvana Franceschetti, Fabrizio Tagliavini, Vito Sofia, Daniela Fatuzzo, Antonio Gambardella, Angelo Labate, Laura Mumoli, Giovanbattista Gaspare Tripodi, Sara Gasparini, Vittoria Cianci, Chiara Sueri, Edoardo Ferlazzo, Umberto Aguglia
A novel technique of quantitative EEG for differentiating patients with early-stage Creutzfeldt-Jakob disease (CJD) from other forms of rapidly progressive dementia (RPD) is proposed. The discrimination is based on the extraction of suitable features from the time-frequency representation of the EEG signals through continuous wavelet transform (CWT). An average measure of complexity of the EEG signal obtained by permutation entropy (PE) is also included. The dimensionality of the feature space is reduced through a multilayer processing system based on the recently emerged deep learning (DL) concept...
May 3, 2016: International Journal of Neural Systems
Sergio L Schmidt, Juliana J Schmidt, Julio C Tolentino, Carlos G Ferreira, Sergio A de Almeida, Regina P Alvarenga, Eunice N Simoes, Guilherme J Schmidt, Nathalie H S Canedo, Leila Chimelli
BACKGROUND: Limbic encephalitis was originally described as a rare clinical neuropathological entity involving seizures and neuropsychological disturbances. In this report, we describe cerebral patterns visualized by positron emission tomography in a patient with limbic encephalitis and cholangiocarcinoma. To our knowledge, there is no other description in the literature of cerebral positron emission tomography findings in the setting of limbic encephalitis and subsequent diagnosis of cholangiocarcinoma...
2016: Journal of Medical Case Reports
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