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https://www.readbyqxmd.com/read/28441414/regional-early-and-progressive-loss-of-brain-pericytes-but-not-vascular-smooth-muscle-cells-in-adult-mice-with-disrupted-platelet-derived-growth-factor-receptor-%C3%AE-signaling
#1
Angeliki Maria Nikolakopoulou, Zhen Zhao, Axel Montagne, Berislav V Zlokovic
Pericytes regulate key neurovascular functions of the brain. Studies in pericyte-deficient transgenic mice with aberrant signaling between endothelial-derived platelet-derived growth factor BB (PDGF-BB) and platelet-derived growth factor receptor β (PDGFRβ) in pericytes have contributed to better understanding of the role of pericytes in the brain. Here, we studied PdgfrβF7/F7 mice, which carry seven point mutations that disrupt PDGFRβ signaling causing loss of pericytes and vascular smooth muscle cells (VSMCs) in the developing brain...
2017: PloS One
https://www.readbyqxmd.com/read/28417072/atypical-juvenile-presentation-of-gm2-gangliosidosis-ab-in-a-patient-compound-heterozygote-for-c-259g%C3%A2-%C3%A2-t-and-c-164c%C3%A2-%C3%A2-t-mutations-in-the-gm2a-gene
#2
Carla Martins, Catherine Brunel-Guitton, Anne Lortie, France Gauvin, Carlos R Morales, Grant A Mitchell, Alexey V Pshezhetsky
GM2-gangliosidosis, AB variant is an extremely rare autosomal recessive inherited disorder caused by mutations in the GM2A gene that encodes GM2 ganglioside activator protein (GM2AP). GM2AP is necessary for solubilisation of GM2 ganglioside in endolysosomes and its presentation to β-hexosaminidase A. Conversely GM2AP deficiency impairs lysosomal catabolism of GM2 ganglioside, leading to its storage in cells and tissues. We describe a 9-year-old child with an unusual juvenile clinical onset of GM2-gangliosidosis AB...
June 2017: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/28405088/dural-arteriovenous-fistula-as-a-treatable-dementia
#3
Ikponmwosa Enofe, Ike Thacker, Sadat Shamim
Dementia is a chronic loss of neurocognitive function that is progressive and irreversible. Although rare, dural arteriovenous fistulas (DAVFs) could present with a rapid decline in neurocognitive function with or without Parkinson-like symptoms. DAVFs represent a potentially treatable and reversible cause of dementia. Here, we report the case of an elderly woman diagnosed with a DAVF after presenting with new-onset seizures, deteriorating neurocognitive function, and Parkinson-like symptoms.
April 2017: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/28405022/therapeutic-reduction-of-ataxin-2-extends-lifespan-and-reduces-pathology-in-tdp-43-mice
#4
Lindsay A Becker, Brenda Huang, Gregor Bieri, Rosanna Ma, David A Knowles, Paymaan Jafar-Nejad, James Messing, Hong Joo Kim, Armand Soriano, Georg Auburger, Stefan M Pulst, J Paul Taylor, Frank Rigo, Aaron D Gitler
Amyotrophic lateral sclerosis (ALS) is a rapidly progressing neurodegenerative disease that is characterized by motor neuron loss and that leads to paralysis and death 2-5 years after disease onset. Nearly all patients with ALS have aggregates of the RNA-binding protein TDP-43 in their brains and spinal cords, and rare mutations in the gene encoding TDP-43 can cause ALS. There are no effective TDP-43-directed therapies for ALS or related TDP-43 proteinopathies, such as frontotemporal dementia. Antisense oligonucleotides (ASOs) and RNA-interference approaches are emerging as attractive therapeutic strategies in neurological diseases...
April 20, 2017: Nature
https://www.readbyqxmd.com/read/28375912/disorders-of-taste-and-smell
#5
Ronald DeVere
PURPOSE OF REVIEW: This article aims to educate clinical neurologists on the importance of taste and smell disorders in clinical neurology. These disorders commonly occur in head trauma, multiple sclerosis, seizure disorders, and neurodegenerative diseases such as idiopathic Parkinson disease and dementia, mild cognitive impairment, and Alzheimer disease, just to name a few. This article covers the basic anatomy of smell and taste, notes the important points of taking a proper history, and discusses smell and taste testing, which are inexpensive, minimally time-consuming procedures...
April 2017: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/28348546/dementia-with-lewy-bodies-molecular-pathology-in-the-frontal-cortex-in-typical-and-rapidly-progressive-forms
#6
Paula Garcia-Esparcia, Irene López-González, Oriol Grau-Rivera, María Francisca García-Garrido, Anusha Konetti, Franc Llorens, Saima Zafar, Margarita Carmona, José Antonio Del Rio, Inga Zerr, Ellen Gelpi, Isidro Ferrer
OBJECTIVES: The goal of this study was to assess mitochondrial function, energy, and purine metabolism, protein synthesis machinery from the nucleolus to the ribosome, inflammation, and expression of newly identified ectopic olfactory receptors (ORs) and taste receptors (TASRs) in the frontal cortex of typical cases of dementia with Lewy bodies (DLB) and cases with rapid clinical course (rpDLB: 2 years or less) compared with middle-aged non-affected individuals, in order to learn about the biochemical abnormalities underlying Lewy body pathology...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28320296/biological-basis-for-amyloidogenesis-in-alzheimer-s-disease
#7
REVIEW
T V Andreeva, W J Lukiw, E I Rogaev
Certain cellular proteins normally soluble in the living organism under certain conditions form aggregates with a specific cross-β sheet structure called amyloid. These intra- or extracellular insoluble aggregates (fibers or plaques) are hallmarks of many neurodegenerative pathologies including Alzheimer's disease (AD), Huntington's disease, Parkinson's disease, prion disease, and other progressive neurological diseases that develop in the aging human central nervous system. Amyloid diseases (amyloidoses) are widespread in the elderly human population, a rapidly expanding demographic in many global populations...
February 2017: Biochemistry. Biokhimii︠a︡
https://www.readbyqxmd.com/read/28316870/dural-arteriovenous-fistula-manifested-as-rapid-progressive-dementia-successfully-treated-by-endovascular-embolization-only
#8
Heewon Hwang, Yun Kyung La, Min Seok Baek, Kyoungwon Baik, Sang Hyun Suh, Won-Joo Kim
A 43-year-old male presented with daytime sleepiness at work and indifferent behavior like never before. Two weeks prior to hospital admission, he had episodic memory loss with well preserved remote memory. Brain MRI showed a dural arteriovenous fistula (DAVF) in the right lateral transverse sinus with a bilateral thalamic venous infarction. Cerebral angiography confirmed a right transverse sigmoid dural arteriovenous fistula with a feeding artery of the right occipital artery and left posterior meningeal artery...
March 2017: Neurointervention
https://www.readbyqxmd.com/read/28290426/rapidly-progressive-dementia-with-myoclonus-not-creutzfeldt-jakob-disease
#9
M Saini, N M Varghese, Khin Hnin Su Wai, N K Loh
No abstract text is available yet for this article.
March 2017: Neurology India
https://www.readbyqxmd.com/read/28286214/cognitive-status-in-the-oldest-old-and-centenarians-a-condition-crucial-for-quality-of-life-methodologically-difficult-to-assess
#10
REVIEW
Beatrice Arosio, Rita Ostan, Daniela Mari, Sarah Damanti, Francesco Ronchetti, Sara Arcudi, Maria Scurti, Claudio Franceschi, Daniela Monti
Human life expectancy and the number of the oldest old are rapidly increasing worldwide. Advanced age is the main risk factor for dementia, representing one of the major causes of disability/dependency among older people with a strong impact on their families/caregivers. Centenarians have reached the extreme limits of human life escaping or delaying the major age-related diseases. Thus, these extraordinary individuals embody the best model to answer the crucial question if cognitive decline and dementia are progressive and unavoidable occurrences of increasing age...
March 9, 2017: Mechanisms of Ageing and Development
https://www.readbyqxmd.com/read/28258683/-patient-with-creutzfeld-jakob-disease-a-case-report
#11
Żanna Pastuszak, Kazimierz Tomczykiewicz, Adam Stępień, Renata Piusińska-Macoch, Joanna Klimczuk, Agnieszka Rolewska, Dariusz Galbarczyk
Creutzfeldt-Jakob disease (CJD) is a rare syndrome of central nervous system caused by infectious protein called prion. There are four types of CJD: sporadic (sCJD), familial (fCJD), jatrogenic (jCJD) and variant (vCJD). The most frequent symptoms are rapidly progressing dementia, mioclonias, akinetic mutism and signs of cerebellum dysfunction. In sCJD, MRI often shows high signal intensity in the putamen and caudate nucleus on T2-weighted images while in vCJD pulvinar sign is often observed. 70% patients with CJD often has characteristic generalized periodic sharp wave pattern in electroencephalography...
February 20, 2017: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/28229442/-rapidly-progressive-dementia-and-parkinsonism-associated-to-multiple-dural-arteriovenous-fistulas
#12
P Mejia, L M Piedra, X Merchan-Del Hierro
INTRODUCTION: Rapidly progressive dementias are an infrequent group of diseases characterised by cognitive deterioration and other neurological disorders that develop over a period ranging from weeks to months. Their causation is varied and includes a large number of neurodegenerative, toxic, metabolic, autoimmune, infectious and vascular conditions. CASE REPORT: We report the case of a 69-year-old male who was admitted to hospital due to a rapidly progressive dementia and parkinsonism caused by multiple dural arteriovenous fistulas, which were successfully treated by means of endovascular therapy...
March 1, 2017: Revista de Neurologia
https://www.readbyqxmd.com/read/28203571/evolutionary-history-of-multiple-dural-fistula
#13
Braulio Martinez-Burbano, Edgar Patricio Correa Diaz, Carolina Jácome Sánchez
Intracranial dural arteriovenous fistulas (DAVFs) are abnormal communications between arteries and veins or dural venous sinuses, which sit between the sheets of the dura. They represent 10% to 15% of intracranial vascular malformations. Clinical manifestations and prognosis depend on the pattern of venous drainage and location. The clinical presentation of DAVF may be mistaken for vascular or nonvascular brain pathologies. For that reason, within the differential diagnosis come a wide range of conditions, such as secondary headaches, encephalopathies, dementias including those with rapid progression, neurodegenerative diseases, inflammatory processes, or tumors typically at the orbital level or in the cavernous sinus...
October 2016: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28182102/a-case-report-of-nonvasculitic-autoimmune-inflammatory-meningoencephalitis-with-sensory-ganglionopathy-a-rare-presentation-of-sj%C3%A3-gren-syndrome
#14
João Peres, Simão Cruz, Rita Oliveira, Luís Santos, Ana Valverde
A 68-year-old Caucasian female was admitted to the emergency department with a progressive history of behavioural symptoms and anxiety followed by visual and auditory hallucinations, forgetfulness, and impaired gait in the previous 3 months. On examination she was psychotic and had a postural and rest tremor of the upper limbs, cogwheel rigidity of the four limbs, retropulsion on standing position, and inability to walk. During the following 2 weeks she developed xerostomia and unilateral parotiditis that improved with steroids...
2017: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/28180075/independent-value-added-by-diffusion-mri-for-prediction-of-cognitive-function-in-older-adults
#15
Julia A Scott, Duygu Tosun, Meredith N Braskie, Pauline Maillard, Paul M Thompson, Michael Weiner, Charles DeCarli, Owen T Carmichael
The purpose of this study was to determine whether white matter microstructure measured by diffusion magnetic resonance imaging (dMRI) provides independent information about baseline level or change in executive function (EF) or memory (MEM) in older adults with and without cognitive impairment. Longitudinal data was acquired from the Alzheimer's Disease Neuroimaging Initiative (ADNI) study from phases GO and 2 (2009-2015). ADNI participants included were diagnosed as cognitively normal (n = 46), early mild cognitive impairment (MCI) (n = 48), late MCI (n = 29), and dementia (n = 39) at baseline...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28178858/technology-based-tools-and-services-for-people-with-dementia-and-carers-mapping-technology-onto-the-dementia-care-pathway
#16
Klara Lorenz, Paul P Freddolino, Adelina Comas-Herrera, Martin Knapp, Jacqueline Damant
The extent to which technology may be able to support people with dementia and their carers along the care pathway and in different care settings is of interest to policy makers and governments. In this paper we provide an overview of the role of technology in dementia care, treatment and support by mapping existing technologies - by function, target user and disease progression. Technologies identified are classified into seven functions: memory support, treatment, safety and security, training, care delivery, social interaction and other...
January 1, 2017: Dementia
https://www.readbyqxmd.com/read/28164774/tauopathies-focus-on-changes-at-the-neurovascular-unit
#17
Alena Michalicova, William A Banks, Jaroslav Legath, Andrej Kovac
In the past, the blood-brain barrier (BBB) had been characterized mainly as a layer of endothelial cells forming the vessel/capillary wall of the brain. More recently, the BBB is considered to be a part of a highly dynamic and interactive system called the neurovascular unit (NVU), consisting of vascular cells, glial cells, and neurons. The list of central nervous system (CNS) pathologies involving BBB dysfunction is rapidly growing. The opening of the BBB and subsequent infiltration of serum components to the brain can lead to host of processes resulting in progressive synaptic and neuronal dysfunction and loss...
February 3, 2017: Current Alzheimer Research
https://www.readbyqxmd.com/read/28153848/cjd-mimics-and-chameleons
#18
REVIEW
Simon Mead, Peter Rudge
Rapidly progressive dementia mimicking Creutzfeldt-Jakob disease (CJD) is a relatively rare presentation but a rewarding one to become familiar with, as the potential diagnoses range from the universally fatal to the completely reversible. Patients require urgent decisions about assessment and investigation and have quickly evolving needs for treatments and support, through symptom management and end-of-life care in most cases. We have based this pragmatic review on the experiences of a specialist prion referral centre in the UK, which, unsurprisingly, is strongly biased towards seeing patients with CJD...
April 2017: Practical Neurology
https://www.readbyqxmd.com/read/28127435/dementia-gait-disturbance-and-urinary-incontinence-in-a-patient-with-pulmonary-sarcoidosis
#19
Gonzalo Labarca, Romina Ramirez, Ximena Monsalve, Isabel Mira-Avendano
Hydrocephalus is an uncommon presentation of neurosarcoidosis. We discuss the case of a 67-year-old woman with a prior diagnosis of hypothyroidism, systemic hypertension, and lung sarcoidosis who presented with a 1-month history of progressive impairment of consciousness leading to prostration and loss of sphincter control. At admission, patient was febrile with altered speech and without focalization. Laboratory results showed leucocytosis and mild anaemia. Cerebrospinal fluid (CSF) analysis was characterized by mild elevated protein level, increased cell count, normal glucose and adenosine deaminase (ADA), negative cytology, and no bacterial isolations...
September 2016: Respirology Case Reports
https://www.readbyqxmd.com/read/28122429/suspected-perinatal-depression-revealed-to-be-hereditary-diffuse-leukoencephalopathy-with-spheroids
#20
Josefine Blume, Robert Weissert
Early motor symptoms of neurodegenerative diseases often appear in combination with psychiatric symptoms, such as depression or personality changes, and are in danger of being misdiagnosed as psychogenic in young patients. We present the case of a 32-year-old woman who presented with rapid-onset depression, followed by a hypokinetic movement disorder and cognitive decline during pregnancy. Genetic testing revealed a mutation in the colony-stimulating factor 1 receptor gene, which led to the diagnosis of hereditary diffuse leukoencephalopathy with spheroids...
January 2017: Journal of Movement Disorders
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