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https://www.readbyqxmd.com/read/28211814/are-major-dementias-triggered-by-poor-blood-flow-to-the-brain-theoretical-considerations
#1
Jack C de la Torre
There is growing evidence that chronic brain hypoperfusion plays a central role in the development of Alzheimer's disease (AD) long before dyscognitive symptoms or amyloid-β accumulation in the brain appear. This commentary proposes that dementia with Lewy bodies (DLB), frontotemporal dementia (FTD), and Creutzfeldt-Jakob disease (CJD) may also develop from chronic brain hypoperfusion following a similar but not identical neurometabolic breakdown as AD. The argument to support this conclusion is that chronic brain hypoperfusion, which is found at the early stages of the three dementias reviewed here, will reduce oxygen delivery and lower oxidative phosphorylation promoting a steady decline in the synthesis of the cell energy fuel adenosine triphosphate (ATP)...
February 15, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28210978/future-directions-in-imaging-neurodegeneration
#2
REVIEW
Joseph C Masdeu
Neuroimaging comprises a powerful set of instruments to diagnose various neurodegenerative disorders, clarifies their neurobiology, and monitors their treatment. Magnetic resonance imaging depicts volume changes, as well as abnormalities in functional and structural connectivity. Positron emission tomography (PET) allows for the quantification of regional cerebral metabolism, characteristically altered in Alzheimer's disease, amyotrophic lateral sclerosis, diffuse Lewy-body disease, and the frontotemporal dementias...
January 2017: Current Neurology and Neuroscience Reports
https://www.readbyqxmd.com/read/28209520/deconstructing-empathy-neuroanatomical-dissociations-between-affect-sharing-and-prosocial-motivation-using-a-patient-lesion-model
#3
Suzanne M Shdo, Kamalini G Ranasinghe, Kelly A Gola, Clinton J Mielke, Paul V Sukhanov, Bruce L Miller, Katherine P Rankin
Affect sharing and prosocial motivation are integral parts of empathy that are conceptually and mechanistically distinct. We used a neurodegenerative disease (NDG) lesion model to more directly examine the neural correlates of these two aspects of real-world empathic responding. The study enrolled 275 participants, including 44 healthy older controls and 231 patients diagnosed with one of five neurodegenerative diseases (75 Alzheimer's disease, 58 behavioral variant frontotemporal dementia (bvFTD), 42 semantic variant primary progressive aphasia (svPPA), 28 progressive supranuclear palsy, and 28 non-fluent variant (nfvPPA)...
February 13, 2017: Neuropsychologia
https://www.readbyqxmd.com/read/28207460/anthropometric-outcome-measures-in-patients-with-metopic-craniosynostosis
#4
Scott J Farber, Dennis C Nguyen, Gary B Skolnick, Sybill D Naidoo, Matthew D Smyth, Kamlesh B Patel
PURPOSE: Treatment of metopic craniosynostosis is performed by either fronto-orbital advancement (FOA) or endoscopic-assisted techniques. Interfrontal angle (IFA) is a validated measure of trigonocephaly, but requires a computed tomography scan. The most common direct measure to assess surgical outcome in patients with trigonocephaly is frontal width (ft-ft). The aim of this study is to determine if frontal width correlates with IFA and successful surgical correction 1 year after treatment...
February 15, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28205215/multicenter-validation-of-an-mmse-moca-conversion-table
#5
David Bergeron, Kelsey Flynn, Louis Verret, Stéphane Poulin, Rémi W Bouchard, Christian Bocti, Tamàs Fülöp, Guy Lacombe, Serge Gauthier, Ziad Nasreddine, Robert Jr Laforce
BACKGROUND: Accumulating evidence points to the superiority of the MoCA over the MMSE as a cognitive screening tool. To facilitate the transition from the MMSE to the MoCA in clinical and research settings, authors have developed MMSE-MoCA conversion tables. However, it is unknown whether a conversion table generated from Alzheimer's disease (AD) patients would apply to patients with other dementia subtypes like vascular dementia or frontotemporal dementia. Furthermore, the reliability and accuracy of MMSE-MoCA conversion tables has not been properly evaluated...
February 15, 2017: Journal of the American Geriatrics Society
https://www.readbyqxmd.com/read/28202835/-a-very-rare-case-of-diffuse-subependymal-metastases-from-small-cell-lung-carcinoma
#6
Ichiro Kawahara, Takashi Fujimoto, Keisuke Ozono, Makoto Hirose, Keisuke Toyoda, Naoki Kitagawa, Tooru Ikeda, Kazuyoshi Uchihashi
While parenchymal metastases are common in solid systemic cancers, subependymal metastases are rare. Approximately half of the reported cases of intraventricular metastases originate from renal carcinoma. A 65-year-old man presented with general fatigue, appetite loss, nausea, and disorientation. Radiological examination revealed diffuse periventricular tumors. The patient underwent an open biopsy via right frontotemporal craniotomy. The patient was diagnosed with metastatic small cell lung carcinoma after histopathological examination...
February 2017: No Shinkei Geka. Neurological Surgery
https://www.readbyqxmd.com/read/28202484/glioblastoma-with-primitive-neuronal-pattern-in-a-girl-aged-3-months-a-rare-diagnosis-at-an-unusual-age
#7
Chi Hau Tan, Teik Beng Phung, Chris Xenos
A girl aged 3 months presented with multiple seizures within 12 hours and was noted to have a palpable swelling over the right temporal region. MRI of the brain revealed a large right frontotemporal tumour, suggestive of supratentorial primitive neuroectodermal tumour. She underwent a stealth-guided craniotomy and debulking of the tumour. Histopathology and immunochemistry of the specimen, however, indicated a high-grade tumour with glioblastoma and neuroblastic components. She underwent adjuvant chemotherapy following the surgery and is well at 7-month follow-up...
February 15, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28199994/association-between-montreal-cognitive-assessment-sub-item-scores-and-corresponding-cognitive-test-performance-in-patients-with-frontotemporal-dementia-and-related-disorders
#8
Kristy K L Coleman, Brenda L Coleman, Julia D MacKinley, Stephen H Pasternak, Elizabeth C Finger
The Montreal Cognitive Assessment (MoCA), a brief screening test developed to detect patients with mild cognitive impairment, is used in clinical settings across North America [Nasreddine et al.: J Am Geriatr Soc 2005;53:695-699]. The MoCA has been demonstrated to be sensitive to cognitive deficits in frontotemporal dementias (FTD) and related disorders [Coleman et al.: Alzheimer Dis Assoc Disord 2016;30:258-263]. Given attentional impairments in patients with FTD, whether and to what extent the abbreviated items on the MoCA may predict performance on corresponding assessments is not known...
February 16, 2017: Dementia and Geriatric Cognitive Disorders
https://www.readbyqxmd.com/read/28197542/the-proline-arginine-dipeptide-from-hexanucleotide-repeat-expanded-c9orf72-inhibits-the-proteasome
#9
Rahul Gupta, Matthews Lan, Jelena Mojsilovic-Petrovic, Won Hoon Choi, Nathaniel Safren, Sami Barmada, Min Jae Lee, Robert Kalb
An intronic hexanucleotide repeat expansion (HRE) mutation in the C9ORF72 gene is the most common cause of familial ALS and frontotemporal dementia (FTD) and is found in ∼7% of individuals with apparently sporadic disease. Several different diamino acid peptides can be generated from the HRE by noncanonical translation (repeat-associated non-ATG translation, or RAN translation), and some of these peptides can be toxic. Here, we studied the effects of two arginine containing RAN translation products [proline/arginine repeated 20 times (PR20) and glycine/arginine repeated 20 times (GR20)] in primary rat spinal cord neuron cultures grown on an astrocyte feeder layer...
January 2017: ENeuro
https://www.readbyqxmd.com/read/28196768/cerebrospinal-fluid-markers-detect-alzheimer-s-disease-in-nonamnestic-dementia
#10
Carly Oboudiyat, Tamar Gefen, Eleni Varelas, Sandra Weintraub, Emily Rogalski, Eileen H Bigio, M-Marsel Mesulam
INTRODUCTION: The accuracy of cerebrospinal fluid (CSF) biomarkers for detecting Alzheimer's disease (AD) pathology has not been fully validated in autopsied nonamnestic dementias. METHODS: We retrospectively evaluated CSF β-amyloid 1-42, phosphorylated-tau, and amyloid-tau index as predictors of Alzheimer pathology in patients with primary progressive aphasia, frontotemporal dementia, and progressive supranuclear palsy. RESULTS: Nineteen nonamnestic autopsied cases with relevant CSF values were included...
February 11, 2017: Alzheimer's & Dementia: the Journal of the Alzheimer's Association
https://www.readbyqxmd.com/read/28195509/reduced-subarachnoid-fluid-diffusion-in-enlarged-subarachnoid-spaces-of-infancy
#11
Matthew T Whitehead, Bonmyong Lee, Audrey McCarron, Stanley T Fricke, Gilbert Vezina
Background and purpose Enlargement of the subarachnoid spaces in infancy (ESSI) is a common cause of macrocephaly without proven explanation. We have observed subarachnoid diffusion to be decreased in these patients. We aim to quantify the diffusivity of ventricular and subarachnoid cerebrospinal fluid in ESSI patients, to determine if diffusion characteristics deviate from normocephalic infants, and to propose a unique mechanism for ESSI. Materials and methods 227 consecutive brain magnetic resonance exams from different macrocephalic children were retrospectively reviewed after institutional review board waiver...
January 1, 2017: Neuroradiology Journal
https://www.readbyqxmd.com/read/28192553/clinical-evidence-of-disease-anticipation-in-families-segregating-a-c9orf72-repeat-expansion
#12
Sara Van Mossevelde, Julie van der Zee, Ilse Gijselinck, Kristel Sleegers, Jan De Bleecker, Anne Sieben, Rik Vandenberghe, Tim Van Langenhove, Jonathan Baets, Olivier Deryck, Patrick Santens, Adrian Ivanoiu, Christiana Willems, Veerle Bäumer, Marleen Van den Broeck, Karin Peeters, Maria Mattheijssens, Peter De Jonghe, Patrick Cras, Jean-Jacques Martin, Marc Cruts, Peter P De Deyn, Sebastiaan Engelborghs, Christine Van Broeckhoven
Importance: Patients carrying a C9orf72 repeat expansion leading to frontotemporal dementia and/or amyotrophic lateral sclerosis have highly variable ages at onset of disease, suggesting the presence of modifying factors. Objective: To provide clinical-based evidence for disease anticipation in families carrying a C9orf72 repeat expansion by analyzing age at onset, disease duration, and age at death in successive generations. Design, Setting, and Participants: This cohort study was performed from June 16, 2000, to June 1, 2016, in 36 extended Belgian families in which a C9orf72 repeat expansion was segregating...
February 13, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28184974/-causes-of-death-in-amyotrophic-lateral-sclerosis-results-from-the-rhineland-palatinate-als-registry
#13
J Wolf, A Safer, J C Wöhrle, F Palm, W A Nix, M Maschke, A J Grau
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is associated with an increased mortality. Knowledge of possible causes of death could lead to an individualization of the palliative treatment concept and result in a differentiated palliative treatment pathway. Currently, only few systematic data are available on the heterogeneity of causes of death associated with ALS. OBJECTIVE: Analysis of the various causes of death in a prospective population-based German cohort of ALS patients...
February 9, 2017: Der Nervenarzt
https://www.readbyqxmd.com/read/28181891/-chronic-traumatic-encephalopathy-an-old-acquaintance-in-athletes
#14
E G B Vijverberg, A C M Pijnenburg, P Scheltens, Y A L Pijnenburg
- Chronic traumatic encephalopathy (CTE) is a neurodegenerative disease caused by repetitive head injuries like those seen in sports such as boxing, American football and soccer.- The clinical features of CTE are a range of cognitive, psychiatric and motor symptoms, and histopathology involves deposits of hyperphosphorylated tau protein and the presence of TAR DNA-binding protein (TDP-43) with relatively little beta-amyloid.- CTE is difficult to differentiate clinically from Alzheimer's disease, frontotemporal dementia and psychiatric disorders because of the major symptom overlap between these conditions...
2017: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/28181693/frontotemporal-lobar-degeneration-tdp-with-multiple-system-atrophy-phenocopy-syndrome
#15
Ana Luísa Sousa, Ricardo Taipa, Niall Quinn, Tamas Revesz, Manuel Melo Pires, Marina Magalhães
Multiple system atrophy (MSA) is a neurodegenerative disorder presenting with parkinsonism, cerebellar involvement, autonomic dysfunction and pyramidal signs (1). Two main clinical subtypes of MSA are recognized: a parkinsonian-type (MSA-P) associated with predominant nigrostriatal degeneration and a cerebellar-type (MSA-C) with predominant olivopontocerebellar atrophy. A 'definite' diagnosis requires pathological confirmation with demonstration of glial cytoplasmic inclusions comprising alpha-synuclein protein aggregates (1)...
February 9, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28176002/dysfunction-of-the-blood-cerebrospinal-fluid-barrier-and-n-methyl-d-aspartate-glutamate-receptor-antibodies-in-dementias
#16
Mandy Busse, Ralf Kunschmann, Henrik Dobrowolny, Jessica Hoffmann, Bernhard Bogerts, Johann Steiner, Thomas Frodl, Stefan Busse
N-Methyl-D-aspartate glutamate receptor (NMDA-R) antibodies (Abs) could play a role in neurodegenerative disorders. Since, in these diseases, NMDA-R Abs were detected in serum, but only sporadic in cerebrospinal fluid (CSF), the origin and impact of the Abs are still unresolved. We examined the presence of NMDA-R Abs in serum and CSF using a cell-based immunofluorescence assay as well as the function of the blood-CSF-barrier (B-CSF-B) by determination of Q albumin (ratio of albumin in CSF and serum) in patients with mild cognitive impairment (MCI; N = 59) and different types of dementia, Alzheimer's disease (AD; N = 156), subcortical ischemic vascular dementia (SIVD; N = 61), and frontotemporal dementia (FTD; N = 34)...
February 8, 2017: European Archives of Psychiatry and Clinical Neuroscience
https://www.readbyqxmd.com/read/28174682/glucose-deprivation-regulates-the-progranulin-sortilin-axis-in-pc12-cells
#17
Ken-Ichi Kawashima, Yuri Ishiuchi, Miki Konnai, Saori Komatsu, Hitoshi Sato, Hideo Kawaguchi, Nobumitsu Miyanishi, Jérôme Lamartine, Masugi Nishihara, Taku Nedachi
Progranulin (PGRN) is a growth factor implicated in several neurodegenerative diseases, such as frontotemporal lobar degeneration. Despite its important role in the central nervous system (CNS), the mechanisms controlling PGRN expression in the CNS are largely unknown. Recent evidence, however, suggested that several stressors, such as hypoxia, acidosis, or oxidative stress, induce PGRN expression. The present study was mainly aimed at determining whether and, if so, how glucose deprivation affects PGRN expression in PC12 cells...
February 2017: FEBS Open Bio
https://www.readbyqxmd.com/read/28174516/anocva-in-r-a-software-to-compare-clusters-between-groups-and-its-application-to-the-study-of-autism-spectrum-disorder
#18
Maciel C Vidal, João R Sato, Joana B Balardin, Daniel Y Takahashi, André Fujita
Understanding how brain activities cluster can help in the diagnosis of neuropsychological disorders. Thus, it is important to be able to identify alterations in the clustering structure of functional brain networks. Here, we provide an R implementation of Analysis of Cluster Variability (ANOCVA), which statistically tests (1) whether a set of brain regions of interest (ROI) are equally clustered between two or more populations and (2) whether the contribution of each ROI to the differences in clustering is significant...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28173118/early-microgliosis-precedes-neuronal-loss-and-behavioural-impairment-in-mice-with-a-frontotemporal-dementia-causing-chmp2b-mutation
#19
Emma L Clayton, Renzo Mancuso, Troels Tolstrup Nielsen, Sarah Mizielinska, Holly Holmes, Nicholas Powell, Frances Norona, Jytte Overgaard Larsen, Carmelo Milioto, Katherine M Wilson, Mark F Lythgoe, Sebastian Ourselin, Jörgen E Nielsen, Peter Johannsen, Ida Holm, John Collinge, Peter L Oliver, Diego Gomez-Nicola, Adrian M Isaacs
No abstract text is available yet for this article.
January 16, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28167528/heat-shock-induced-phosphorylation-of-tar-dna-binding-protein-43-tdp-43-by-mapk-erk-kinase-regulates-tdp-43-function
#20
Wen Li, Ashley N Reeb, Binyan Lin, Praveen Subramanian, Erin E Fey, Catherine R Knoverek, Rachel L French, Eileen H Bigio, Yuna M Ayala
TAR DNA binding protein (TDP-43) is a highly conserved and essential DNA- and RNA-binding protein that controls gene expression through RNA processing, in particular, regulation of splicing. Intracellular aggregation of TDP-43 is a hallmark of amyotrophic lateral sclerosis and ubiquitin-positive frontotemporal lobar degeneration. This TDP-43 pathology is also present in other types of neurodegeneration including Alzheimer's disease. We report here that TDP-43 is a substrate of MEK, a central kinase in the MAPK/ERK signaling pathway...
February 6, 2017: Journal of Biological Chemistry
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