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https://www.readbyqxmd.com/read/28541543/validation-of-the%C3%A2-d%C3%A3-pistage-cognitif-de-qu%C3%A3-bec-a-new-cognitive-screening-tool-for-atypical-dementias
#1
Robert Laforce, Leila Sellami, David Bergeron, Audrey Paradis, Louis Verret, Marie-Pierre Fortin, Michèle Houde, Martin Roy, Stéphane Poulin, Joël Macoir, Carol Hudon, Rémi W Bouchard
Objective: This study aimed to validate and provide normative data for the Dépistage Cognitif de Québec (DCQ; www.dcqtest.org), a new cognitive screening tool for atypical dementias. Method: The DCQ was developed by expert behavioral neurologists and clinical neuropsychologists based on updated criteria for Alzheimer's disease, primary progressive aphasia, and behavioral variant frontotemporal dementia. It targets five relevant domains: Memory, Visuospatial, Executive, Language, and Behavior...
May 25, 2017: Archives of Clinical Neuropsychology: the Official Journal of the National Academy of Neuropsychologists
https://www.readbyqxmd.com/read/28541286/impaired-prosaposin-lysosomal-trafficking-in-frontotemporal-lobar-degeneration-due-to-progranulin-mutations
#2
Xiaolai Zhou, Lirong Sun, Oliver Bracko, Ji Whae Choi, Yan Jia, Alissa L Nana, Owen Adam Brady, Jean C Cruz Hernandez, Nozomi Nishimura, William W Seeley, Fenghua Hu
Haploinsufficiency of progranulin (PGRN) due to mutations in the granulin (GRN) gene causes frontotemporal lobar degeneration (FTLD), and complete loss of PGRN leads to a lysosomal storage disorder, neuronal ceroid lipofuscinosis (NCL). Accumulating evidence suggests that PGRN is essential for proper lysosomal function, but the precise mechanisms involved are not known. Here, we show that PGRN facilitates neuronal uptake and lysosomal delivery of prosaposin (PSAP), the precursor of saposin peptides that are essential for lysosomal glycosphingolipid degradation...
May 25, 2017: Nature Communications
https://www.readbyqxmd.com/read/28539871/protein-quality-control-and-the-amyotrophic-lateral-sclerosis-frontotemporal-dementia-continuum
#3
Hamideh Shahheydari, Audrey Ragagnin, Adam K Walker, Reka P Toth, Marta Vidal, Cyril J Jagaraj, Emma R Perri, Anna Konopka, Jessica M Sultana, Julie D Atkin
Protein homeostasis, or proteostasis, has an important regulatory role in cellular function. Protein quality control mechanisms, including protein folding and protein degradation processes, have a crucial function in post-mitotic neurons. Cellular protein quality control relies on multiple strategies, including molecular chaperones, autophagy, the ubiquitin proteasome system, endoplasmic reticulum (ER)-associated degradation (ERAD) and the formation of stress granules (SGs), to regulate proteostasis. Neurodegenerative diseases are characterized by the presence of misfolded protein aggregates, implying that protein quality control mechanisms are dysfunctional in these conditions...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28534652/a-review-of-the-evidence-of-zolpidem-efficacy-in-neurological-disability-after-brain-damage-due-to-stroke-trauma-and-hypoxia-a-justification-of-further-clinical-trials
#4
J A Sutton, R P Clauss
During 15 years, 23 clinical reports and 6 studies have demonstrated associations between sub-sedative doses of zolpidem and recoveries from brain damage due to strokes, trauma and hypoxia. Clinical findings include unexpected awakenings from vegetative states and regressions of stroke symptoms after dosing that disappear during elimination and reappear on repeat dosing. Initially single-photon emission computed tomography scans showed improved perfusion within, around and distant from infarctions. Then positron emission tomography scans and electroencephalography detected renewed metabolic and neuronal activity...
May 23, 2017: Brain Injury: [BI]
https://www.readbyqxmd.com/read/28531301/a-political-case-of-penetrating-cranial-trauma-the-injury-of-james-scott-brady
#5
Richard Menger, Piyush Kalakoti, Rimal Hanif, Osama Ahmed, Anil Nanda, Bharat Guthikonda
James Brady, the White House press secretary during President Ronald Reagan's first term in office, was 1 of 4 people (including the President) wounded during an attempted assassination attempt on President Reagan's life on March 30, 1981. John Hinckley, Jr. was found not guilty of this attempt by reason of insanity. The assassination attempt was a ploy by Hinckley, Jr. to impress the actress Jodie Foster. Brady was the most seriously injured of the 4 who were wounded. He suffered a gunshot wound to the left forehead that traveled through the left frontal lobe, corpus callosum, and then into the right frontal and temporal lobes...
May 22, 2017: Neurosurgery
https://www.readbyqxmd.com/read/28529873/white-matter-hyperintensities-are-seen-only-in-grn-mutation-carriers-in-the-genfi-cohort
#6
Carole H Sudre, Martina Bocchetta, David Cash, David L Thomas, Ione Woollacott, Katrina M Dick, John van Swieten, Barbara Borroni, Daniela Galimberti, Mario Masellis, Maria Carmela Tartaglia, James B Rowe, Caroline Graff, Fabrizio Tagliavini, Giovanni Frisoni, Robert Laforce, Elizabeth Finger, Alexandre de Mendonça, Sandro Sorbi, Sébastien Ourselin, M Jorge Cardoso, Jonathan D Rohrer
Genetic frontotemporal dementia is most commonly caused by mutations in the progranulin (GRN), microtubule-associated protein tau (MAPT) and chromosome 9 open reading frame 72 (C9orf72) genes. Previous small studies have reported the presence of cerebral white matter hyperintensities (WMH) in genetic FTD but this has not been systematically studied across the different mutations. In this study WMH were assessed in 180 participants from the Genetic FTD Initiative (GENFI) with 3D T1- and T2-weighed magnetic resonance images: 43 symptomatic (7 GRN, 13 MAPT and 23 C9orf72), 61 presymptomatic mutation carriers (25 GRN, 8 MAPT and 28 C9orf72) and 76 mutation negative non-carrier family members...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28527630/diagnostic-accuracy-of-csf-neurofilament-light-chain-protein-in-the-biomarker-guided-classification-system-for-alzheimer-s-disease
#7
Simone Lista, Nicola Toschi, Filippo Baldacci, Henrik Zetterberg, Kaj Blennow, Ingo Kilimann, Stefan J Teipel, Enrica Cavedo, Antonio Melo Dos Santos, Stéphane Epelbaum, Foudil Lamari, Bruno Dubois, Roberto Floris, Francesco Garaci, Harald Hampel
We assessed the diagnostic accuracy of cerebrospinal fluid (CSF) neurofilament light chain (NFL) protein in the classification of patients with Alzheimer's disease (AD) and cognitively healthy control individuals (HCs) and patients with frontotemporal dementia (FTD) as comparisons. Particularly, we tested the performance of CSF NFL concentration in differentiating patient groups stratified by fluid biomarker profiles, independently of the severity of cognitive impairment (mild cognitive impairment (MCI) and AD dementia individuals), using a biomarker-guided descriptive classification system for AD...
May 17, 2017: Neurochemistry International
https://www.readbyqxmd.com/read/28527524/c9orf72-hexanucleotide-repeat-expansions-and-ataxin-2-intermediate-length-repeat-expansions-in-indian-patients-with-amyotrophic-lateral-sclerosis
#8
Priyam Narain, James Gomes, Rohit Bhatia, Inder Singh, Perumal Vivekanandan
Repeat expansions in the chromosome 9 open reading frame 72 (C9orf72) gene have been recognized as a major contributor to amyotrophic lateral sclerosis (ALS) and frontotemporal dementia in the Caucasian population. Intermediate length repeat expansions of CAG (polyQ) repeat in the ATXN2 gene have also been reported to increase the risk of developing ALS in North America and Europe. We screened 131 ALS patients and 127 healthy controls from India for C9orf72 and ATXN2 repeat expansions. We found pathogenic hexanucleotide expansions in 3 of the 127 sporadic ALS patients, in 1 of the 4 familial ALS patients, and in none of the healthy controls...
April 26, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/28523532/progressive-pathological-changes-in-neurochemical-profile-of-the-hippocampus-and-early-changes-in-the-olfactory-bulbs-of-tau-transgenic-mice-rtg4510
#9
Jieun Kim, In-Young Choi, Karen E Duff, Phil Lee
Tauopathies such as Alzheimer's disease and frontotemporal lobe degeneration (FTLD-tau) dementia, characterized by pathologic aggregation of the microtubule-associated tau protein and formation of neurofibrillary tangles, have been linked to neurodegeneration and cognitive decline. The early detection of cerebral abnormalities and the identification of biological contributors to the continuous pathologic processes of neurodegeneration in tauopathies critically hinge on sensitive and reliable measures of biomarkers in the living brain...
May 18, 2017: Neurochemical Research
https://www.readbyqxmd.com/read/28522837/age-related-penetrance-of-the-c9orf72-repeat-expansion
#10
Natalie A Murphy, Karissa C Arthur, Pentti J Tienari, Henry Houlden, Adriano Chiò, Bryan J Traynor
A pathogenic hexanucleotide repeat expansion within the C9orf72 gene has been identified as the major cause of two neurodegenerative syndromes, amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). This mutation is known to have incomplete penetrance, with some patients developing disease in their twenties and a small portion of carriers surviving to their ninth decade without developing symptoms. Describing penetrance by age among C9orf72 carriers and identifying parameters that alter onset age are essential to better understanding this locus and to enhance predictive counseling...
May 18, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28521344/the-interlenticulostriate-approach-to-very-high-riding-distal-basilar-trunk-aneurysms
#11
Ralph Rahme, Almaz Kurbanov, Jeffrey T Keller, Todd A Abruzzo, Lincoln Jimenez, Andrew J Ringer, Ondrej Choutka, Mario Zuccarello
BACKGROUND: Most high-riding distal basilar trunk aneurysms can be surgically approached via the transsylvian route and its orbitozygomatic variant. However, on rare occasions, the basilar bifurcation may be unusually high and an approach above the carotid terminus may be required. OBJECTIVE: In this cadaveric study, we sought to determine the feasibility and exposure limits of the interlenticulostriate approach (ILSA). METHODS: A standard transsylvian approach was performed in 10 cerebral hemispheres of 5 formalin-fixed, silicone-injected cadaver heads...
June 1, 2017: Operative Neurosurgery (Hagerstown, Md.)
https://www.readbyqxmd.com/read/28520677/the-use-of-an-electrophysiological-brain-function-index-in-the-evaluation-of-concussed-athletes
#12
M Alison Brooks, Jeffrey J Bazarian, Leslie S Prichep, Samanwoy Ghosh Dastidar, Thomas M Talavage, William Barr
OBJECTIVE: To evaluate the effectiveness of the electroencephalographic (EEG) Brain Function Index (BFI) for characterizing sports-related concussive injury and recovery. PARTICIPANTS: Three hundred fifty-four (354) male contact sport high school and college athletes were prospectively recruited from multiple locations over 6 academic years of play (244 control baseline athletes and 110 athletes with a concussion). METHODS: Using 5 to 10 minutes of eyes closed resting EEG collected from frontal and frontotemporal regions, a BFI was computed for all subjects and sessions...
May 17, 2017: Journal of Head Trauma Rehabilitation
https://www.readbyqxmd.com/read/28520598/imaging-plus-x-multimodal-models-of-neurodegenerative-disease
#13
Neil P Oxtoby, Daniel C Alexander
PURPOSE OF REVIEW: This article argues that the time is approaching for data-driven disease modelling to take centre stage in the study and management of neurodegenerative disease. The snowstorm of data now available to the clinician defies qualitative evaluation; the heterogeneity of data types complicates integration through traditional statistical methods; and the large datasets becoming available remain far from the big-data sizes necessary for fully data-driven machine-learning approaches...
May 16, 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/28515555/quantitative-balance-and-gait-measurement-in-patients-with-frontotemporal-dementia-and-alzheimer-diseases-a-pilot-study
#14
Selva Ganapathy Velayutham, Sadanandavalli Retnaswami Chandra, Srikala Bharath, Ravi Girikamatha Shankar
INTRODUCTION: Alzhiemers disease and Frontotemporal dementia are common neurodegenerative dementias with a wide prevalence. Falls are a common cause of morbidity in these patients. Identifying subclinical involvement of these parameters might serve as a tool in differential analysis of these distinct parameters involved in these conditions and also help in planning preventive strategies to prevent falls. PATIENTS AND METHODS: Eight patients in age and gender matched patients in each group were compared with normal controls...
March 2017: Indian Journal of Psychological Medicine
https://www.readbyqxmd.com/read/28515553/long-loop-reflex-2-in-patients-with-cortical-dementias-a-pilot-study
#15
Sadanandavalli Retnaswami Chandra, Thomas Gregor Isaac, Mahesh Mane, Srikala Bharath, B C Nagaraju
INTRODUCTION: Dementia is a major public health problem and it appears to be a global epidemic. The prevalence is doubling every 5 years and it is expected that 70% of persons above 60 years will live in developing countries by 2020 and 15% of them are likely to suffer from dementia. Disease modifying treatments work only if initiated very early; however, diagnostic tools are not always able to clearly differentiate the different types in very early stage. Therefore, inexpensive and easily available biomarkers are needed to know if collectively they will improve the sensitivity of specific diagnosis...
March 2017: Indian Journal of Psychological Medicine
https://www.readbyqxmd.com/read/28515546/use-of-jonkman-et-al-score-for-visual-quantification-of-electroencephalography-as-a-tool-to-assess-disease-severity-in-cortical-dementias
#16
R Kiran Kumar, Sadanandavalli Retnaswami Chandra, Girish B Kulkarni, Rose Dawn Bharath
OBJECTIVES: To study electroencephalography (EEG) changes in patients with cortical dementias (Alzheimer's disease [AD] and frontotemporal dementia (FTD). (1) To correlate EEG changes with clinical severity of dementia as assessed by rating scales. (2) To correlate global gray matter volume (GGMV) with EEG scores and clinical severity rating scales. PATIENTS AND METHODS: This is a prospective cross-sectional study involving patients fulfilling the criteria for Probable AD and FTD...
March 2017: Indian Journal of Psychological Medicine
https://www.readbyqxmd.com/read/28515265/clinical-marker-for-alzheimer-disease-pathology-in-logopenic-primary-progressive-aphasia
#17
Lucia A A Giannini, David J Irwin, Corey T McMillan, Sharon Ash, Katya Rascovsky, David A Wolk, Vivianna M Van Deerlin, Edward B Lee, John Q Trojanowski, Murray Grossman
OBJECTIVE: To determine whether logopenic features of phonologic loop dysfunction reflect Alzheimer disease (AD) neuropathology in primary progressive aphasia (PPA). METHODS: We performed a retrospective case-control study of 34 patients with PPA with available autopsy tissue. We compared baseline and longitudinal clinical features in patients with primary AD neuropathology to those with primary non-AD pathologies. We analyzed regional neuroanatomic disease burden in pathology-defined groups using postmortem neuropathologic data...
May 17, 2017: Neurology
https://www.readbyqxmd.com/read/28514500/histology-of-the-distal-dural-ring
#18
Christopher S Graffeo, Avital Perry, William R Copeland, Aditya Raghunathan, Michael J Link
BACKGROUND: The distal dural ring (DDR) is a conserved intracranial anatomic structure marking the boundary point at which the internal carotid artery (ICA) exits the cavernous sinus (CS) and enters the subarachnoid space. Although the CS has been well described in a range of anatomic studies, to our knowledge no prior study has analyzed the histologic relationship between the ICA and DDR. Correspondingly, our objective was to assess the relationship of the DDR to the ICA and determine whether the DDR can be dissected from the ICA and thus divided, or can only be circumferentially trimmed around the artery...
May 17, 2017: Clinical Anatomy
https://www.readbyqxmd.com/read/28512218/ubiquitin-and-atp-dependent-unfoldase-activity-of-p97-vcp%C3%A2-nploc4%C3%A2-ufd1l-is-enhanced-by-a-mutation-that-causes-multisystem-proteinopathy
#19
Emily E Blythe, Kristine C Olson, Vincent Chau, Raymond J Deshaies
p97 is a "segregase" that plays a key role in numerous ubiquitin (Ub)-dependent pathways such as ER-associated degradation. It has been hypothesized that p97 extracts proteins from membranes or macromolecular complexes to enable their proteasomal degradation; however, the complex nature of p97 substrates has made it difficult to directly observe the fundamental basis for this activity. To address this issue, we developed a soluble p97 substrate-Ub-GFP modified with K48-linked ubiquitin chains-for in vitro p97 activity assays...
May 16, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28510586/truncation-of-the-tar-dna-binding-protein-43-is-not-a-prerequisite-for-cytoplasmic-relocalization-and-is-suppressed-by-caspase-inhibition-and-by-introduction-of-the-a90v-sequence-variant
#20
Heike J Wobst, Louise Delsing, Nicholas J Brandon, Stephen J Moss
The RNA-binding and -processing protein TAR DNA-binding protein 43 (TDP-43) is heavily linked to the underlying causes and pathology of neurodegenerative diseases such as amyotrophic lateral sclerosis and frontotemporal lobar degeneration. In these diseases, TDP-43 is mislocalized, hyperphosphorylated, ubiquitinated, aggregated and cleaved. The importance of TDP-43 cleavage in the disease pathogenesis is still poorly understood. Here we detail the use of D-sorbitol as an exogenous stressor that causes TDP-43 cleavage in HeLa cells, resulting in a 35 kDa truncated product that accumulates in the cytoplasm within one hour of treatment...
2017: PloS One
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