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https://www.readbyqxmd.com/read/27905341/heidenhain-variant-of-creutzfeldt-jakob-disease-in-a-patient-who-had-bovine-bioprosthetic-valve-implantation
#1
Jehard Hashoul, Waleed Saliba, Irina Bloch, Haneen Jabaly-Habib
Creutzfeldt-Jakob disease (CJD) is a rare neurodegenerative disorder characterized by rapidly progressing dementia, general neurologic deterioration, and death. When the leading symptoms are visual disturbances, it is termed as the Heidenhain variant of CJD (HvCJD). CJD was reported following prion-contaminated pericardium transplants but never after bovine bioprosthetic cardiac valve. In this case report, we describe HvCJD in a patient who had a bovine bioprosthetic cardiac valve implant. An 82-year-old-woman was referred to neuro-ophthalmology clinic for unexplained visual loss that started 1 month previously...
October 2016: Indian Journal of Ophthalmology
https://www.readbyqxmd.com/read/27884882/lymphomatosis-cerebri-diagnostic-challenges-and-review-of-the-literature
#2
Paul J Lee, Idanis Berrios, Carolina Ionete, Thomas Smith
Lymphomatosis cerebri (LC) is a rare variant of a primary central nervous system non-Hodgkin's lymphoma (PCNSL) characterised by diffuse infiltration of tumour cells throughout the brain parenchyma. We present a 68-year-old immunocompetent woman with headaches, dizziness, blurred vision, localised right leg weakness and rapidly progressive dementia. A brain MRI demonstrated diffuse T2 hyperintense white matter lesions that did not enhance with contrast. The clinical differential diagnosis of these lesions included metastatic disease, infectious or inflammatory process such as sarcoidosis, lymphoma, demyelinating disease and less likely vascular aetiology, such as vasculitis or ischaemic stroke...
November 24, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27876002/sporadic-creutzfeldt-jakob-disease-with-unusual-initial-presentation-as-posterior-reversible-encephalopathy-syndrome-a-case-report
#3
Edgaras Dirzius, Renata Balnyte, Vesta Steibliene, Rymante Gleizniene, Inga Gudinaviciene, Andrius Radziunas, Kestutis Petrikonis
BACKGROUND: Creutzfeldt - Jakob disease (CJD) is a rapidly progressive and fatal neurodegenerative prion disease. MRI findings are included in diagnostic criteria for probable CJD, giving a sensitivity and specificity more than 90%, but the atypical radiological presentations in the early stage of the disease could cause the diagnostic difficulties. CJD can be definitively diagnosed by histopathological confirmation, brain biopsy or at autopsy. CASE PRESENTATION: We present a case of 53-year-old woman with a history of a rapidly progressive dementia with symptoms of visual impairment, increased extrapyramidal type muscle tonus, stereotypical movements and ataxic gait resulting in the patient's death after13 months...
November 22, 2016: BMC Neurology
https://www.readbyqxmd.com/read/27870938/evaluation-of-%C3%AE-synuclein-as-a-novel-cerebrospinal-fluid-biomarker-in-different-forms-of-prion-diseases
#4
Franc Llorens, Niels Kruse, Matthias Schmitz, Nadine Gotzmann, Ewa Golanska, Katrin Thüne, Orgeta Zejneli, Eirini Kanata, Tobias Knipper, Maria Cramm, Peter Lange, Saima Zafar, Beata Sikorska, Pawel P Liberski, Eva Mitrova, Daniela Varges, Christian Schmidt, Theodoros Sklaviadis, Brit Mollenhauer, Inga Zerr
INTRODUCTION: Accurate diagnosis of prion diseases and discrimination from alternative dementias gain importance in the clinical routine, but partial overlap in cerebrospinal fluid (CSF) biomarkers impedes absolute discrimination in the differential diagnostic context. METHODS: We established the clinical parameters for prion disease diagnosis for the quantification of CSF α-synuclein in patients with sporadic (n = 234) and genetic (n = 56) prion diseases, in cases with cognitive impairment or dementia (n = 278), and in the neurologic control (n = 111) group...
November 18, 2016: Alzheimer's & Dementia: the Journal of the Alzheimer's Association
https://www.readbyqxmd.com/read/27867500/neurological-complications-in-chronic-kidney-disease
#5
REVIEW
Ria Arnold, Tushar Issar, Arun V Krishnan, Bruce A Pussell
Patients with chronic kidney disease (CKD) are frequently afflicted with neurological complications. These complications can potentially affect both the central and peripheral nervous systems. Common neurological complications in CKD include stroke, cognitive dysfunction, encephalopathy, peripheral and autonomic neuropathies. These conditions have significant impact not only on patient morbidity but also on mortality risk through a variety of mechanisms. Understanding the pathophysiological mechanisms of these conditions can provide insights into effective management strategies for neurological complications...
January 2016: JRSM Cardiovascular Disease
https://www.readbyqxmd.com/read/27866858/clinical-variables-and-biomarkers-in-prediction-of-cognitive-impairment-in-patients-with-newly-diagnosed-parkinson-s-disease-a-cohort-study
#6
Anette Schrag, Uzma Faisal Siddiqui, Zacharias Anastasiou, Daniel Weintraub, Jonathan M Schott
BACKGROUND: Parkinson's disease is associated with an increased incidence of cognitive impairment and dementia. Predicting who is at risk of cognitive decline early in the disease course has implications for clinical prognosis and for stratification of participants in clinical trials. We assessed the use of clinical information and biomarkers as predictive factors for cognitive decline in patients with newly diagnosed Parkinson's disease. METHODS: The Parkinson's Progression Markers Initiative (PPMI) study is a cohort study in patients with newly diagnosed Parkinson's disease...
November 17, 2016: Lancet Neurology
https://www.readbyqxmd.com/read/27862810/angiotensin-converting-enzyme-insertion-deletion-polymorphism-and-the-longitudinal-progression-of-alzheimer-s-disease
#7
Ping-Song Chou, Meng-Ni Wu, Mei-Chuan Chou, I Chien, Yuan-Han Yang
AIM: The angiotensin-converting enzyme gene (ACE) insertion (I)/deletion (D) polymorphism is considered a biologically plausible gene for Alzheimer's disease (AD) in cross-sectional studies. The present study aimed to investigate the longitudinal effect of ACE I/D polymorphism on AD progression. METHODS: This 3-year observational study investigated the longitudinal effect of ACE I/D polymorphism on AD progression. Clinically diagnosed AD patients with a clinical dementia rating (CDR) of 0...
November 10, 2016: Geriatrics & Gerontology International
https://www.readbyqxmd.com/read/27862176/inflammasomes-hormesis-and-antioxidants-in-neuroinflammation-role-of-nrlp3-in-alzheimer-disease
#8
REVIEW
Manuela Pennisi, Rosalia Crupi, Rosanna Di Paola, Maria Laura Ontario, Rita Bella, Edward J Calabrese, Roberto Crea, Salvatore Cuzzocrea, Vittorio Calabrese
Alzheimer disease (AD) is a progressive neurodegenerative disorder leading to cognitive decline, neuropsychiatric symptoms, disability, caregiver burden, and premature death. It represents the most prevalent cause of dementia, and its incidence rates exponentially increase with increasing age. The number of Americans living with AD is rapidly increasing. An estimated 5.4 million Americans of all ages have AD in 2016. One in nine people aged 65 and older has AD, and by midcentury, someone in the United States will develop the disease every 33 sec...
November 8, 2016: Journal of Neuroscience Research
https://www.readbyqxmd.com/read/27849640/rapidly-progressive-dementia-prevalence-and-causes-in-a-neurologic-unit-of-a-tertiary-hospital-in-brazil
#9
Adalberto Studart Neto, Herval R Soares Neto, Mateus M Simabukuro, Davi J F Solla, Márcia R R Gonçalves, Ida Fortini, Luiz H M Castro, Ricardo Nitrini
BACKGROUND: Rapidly progressive dementia (RPD) is usually associated with Creutzfeldt-Jakob disease, a fatal condition. Current advances in the understanding of immune-mediated diseases allow the diagnosis of previously unrecognized treatable RPDs. OBJECTIVE OF THE STUDY: The objective of the study was to describe the prevalence and causes of RPD in a neurology service, identifying potentially reversible causes. METHODS: We carried out a cross-sectional evaluation of all patients admitted to the neurology unit of a tertiary hospital in Brazil between March 2012 and February 2015...
November 15, 2016: Alzheimer Disease and Associated Disorders
https://www.readbyqxmd.com/read/27845782/a-targeted-proteomic-multiplex-csf-assay-identifies-increased-malate-dehydrogenase-and-other-neurodegenerative-biomarkers-in-individuals-with-alzheimer-s-disease-pathology
#10
R W Paterson, W E Heywood, A J Heslegrave, N K Magdalinou, U Andreasson, E Sirka, E Bliss, C F Slattery, J Toombs, J Svensson, P Johansson, N C Fox, H Zetterberg, K Mills, J M Schott
Alzheimer's disease (AD) is the most common cause of dementia. Biomarkers are required to identify individuals in the preclinical phase, explain phenotypic diversity, measure progression and estimate prognosis. The development of assays to validate candidate biomarkers is costly and time-consuming. Targeted proteomics is an attractive means of quantifying novel proteins in cerebrospinal and other fluids, and has potential to help overcome this bottleneck in biomarker development. We used a previously validated multiplexed 10-min, targeted proteomic assay to assess 54 candidate cerebrospinal fluid (CSF) biomarkers in two independent cohorts comprising individuals with neurodegenerative dementias and healthy controls...
November 15, 2016: Translational Psychiatry
https://www.readbyqxmd.com/read/27833227/creutzfeldt-jakob-disease-phenotype-and-course-our-experience-from-a-tertiary-center
#11
Sadanandavalli Retnaswami Chandra, Thomas Gregor Issac, Mariamma Philip, Veeranna Gadad
INTRODUCTION: Prion diseases are protein conformation disorders and neither caused by viroid or virus but is a transmissible particle labeled a prion by Pruisner. Normal prion protein becomes infectious by a different folding, but the triggers are not known. Based on the characteristic brain pathology, they are grouped under spongiform encephalopathy affecting both man and animals. Estimated prevalence is one per million. Creutzfeldt-Jakob disease (CJD) registry from National Institute and Neurosciences (NIMHANS), Bengaluru, reported 69 cases in 30 years...
September 2016: Indian Journal of Psychological Medicine
https://www.readbyqxmd.com/read/27809929/insight-of-brain-degenerative-protein-modifications-in-the-pathology-of-neurodegeneration-and-dementia-by-proteomic-profiling
#12
REVIEW
Sunil S Adav, Siu Kwan Sze
Dementia is a syndrome associated with a wide range of clinical features including progressive cognitive decline and patient inability to self-care. Due to rapidly increasing prevalence in aging society, dementia now confers a major economic, social, and healthcare burden throughout the world, and has therefore been identified as a public health priority by the World Health Organization. Previous studies have established dementia as a 'proteinopathy' caused by detrimental changes in brain protein structure and function that promote misfolding, aggregation, and deposition as insoluble amyloid plaques...
November 3, 2016: Molecular Brain
https://www.readbyqxmd.com/read/27803826/a-corticobasal-syndrome-variant-of-familial-creutzfeldt-jakob-disease-with-stroke-like-onset
#13
Ján Necpál, Martin Stelzer, Silvia Koščová, Michal Patarák
Creutzfeldt-Jakob disease (CJD) is an untreatable rare human prion disease characterized by rapidly progressive dementia along with various neurological features, including myoclonus and sometimes other movement disorders. The clinical course is typically insidious and rapid, leading to an early death. In general, the most common form is sporadic CJD; however, Slovakia is typical for a high percentage of genetic cases. We present an unusual case report of a 65-year-old man with a sudden, stroke-like onset of motor aphasia with right-sided levodopa unresponsive parkinsonism, alien hand, and other characteristic features of corticobasal syndrome (CBS), with rapid deterioration and death on the 32nd day of the disease...
2016: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/27802239/rapidly-progressive-frontotemporal-dementia-associated-with-mapt-mutation%C3%A2-g389r
#14
Lin Sun, Kathryn Chen, Xia Li, Shifu Xiao
Frontotemporal dementia includes a large spectrum of neurodegenerative disorders. Here, we report the case of a young patient with MAPT mutation G389R, who was 27 years old when he progressively developed severe behavioral disturbances. Initially, he presented with slowly progressive personality change. After 1 year, he exhibited moderate dementia with extrapyramidal and pyramidal symptoms. MRI showed frontotemporal atrophy. He rapidly progressed to severe dementia 3 years after onset. Genetic testing revealed a heterozygous guanine to cytosine mutation at the first base of codon 389 (c...
October 11, 2016: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27785004/psen1-l226f-mutation-in-a-patient-with-early-onset-alzheimer-s-disease-in-korea
#15
Eva Bagyinszky, Sun Ah Park, Hyung Jun Kim, Seong Hye Choi, Seong Soo A An, Sang Yun Kim
In this study, we report a first 226leucine (Leu) mutation to phenylalanine (Phe) in (PSEN1, CTC>TTC, L226F) in Asia from a Korean early-onset Alzheimer's disease (EOAD) patient. Polymerase chain reaction (PCR)-single strand conformation polymorphism, sequencing, and in silico predictions were performed. Previously, L226F was reported in EOAD patients by Zekanowski et al and Gómez-Tortosa et al. Disease phenotypes appeared in their thirties, and family history was positive in both cases. In our patient, age of onset was similar (37 years of age), but the mutation seemed to be de novo, since no affected family member was found...
2016: Clinical Interventions in Aging
https://www.readbyqxmd.com/read/27781099/related-or-not-development-of-spontaneous-creutzfeldt-jakob-disease-in-a-patient-with-chronic-well-controlled-hiv-a-case-report-and-review-of-the-literature
#16
M-Alain Babi, Bryan D Kraft, Sweta Sengupta, Haley Peterson, Ryan Orgel, Zachary Wegermann, Njira L Lugogo, Matthew W Luedke
BACKGROUND: We report a novel case of a rare disease: spontaneous Creutzfeldt-Jakob disease in a patient with well-controlled HIV. We explore the relationship between spontaneous Creutzfeldt-Jakob disease and HIV. CASE REPORT: A 66-year-old man with long-standing, well-controlled HIV infection presented with 3 months of progressive, subacute neurocognitive decline. His symptoms included conceptual apraxia, apathy, memory impairment, and gait disturbance, and were initially attributed to depressive "pseudo-dementia...
2016: SAGE Open Med Case Rep
https://www.readbyqxmd.com/read/27730513/middle-aged-diabetic-females-and-males-present-distinct-susceptibility-to-alzheimer-disease-like-pathology
#17
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
https://www.readbyqxmd.com/read/27662289/occupational-attainment-as-risk-factor-for%C3%A2-progression-from-mild-cognitive-impairment-to-alzheimer-s-disease-a%C3%A2-credos-study
#18
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
https://www.readbyqxmd.com/read/27634418/patient-with-rapidly-evolving-neurological-disease-with-neuropathological-lesions-of-creutzfeldt-jakob-disease-lewy-body-dementia-chronic-subcortical-vascular-encephalopathy-and-meningothelial-meningioma
#19
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
https://www.readbyqxmd.com/read/27598501/-creutzfeldt-jakob-disease-report-of-one-case
#20
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
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