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Progressive aphasia

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https://www.readbyqxmd.com/read/28441826/-a-childhood-onset-rapid-onset-dystonia-parkinsonism-family-with-atp1a3-gene-mutation-and-literatures-review
#1
C L Zhang, F Yin, F He, N Gai, Z Q Shi, J Peng
Objective: To explore clinical characteristics, treatment, and prognosis of a family with childhood-onset rapid-onset dystonia parkinsonism (RDP) caused by ATP1A3 gene mutation and review literatures. Method: The clinical data of a RDP child, his brother and mother had been analyzed retrospectively. This family was admitted to Xiangya Hospital in January 2016. DNA samples were analyzed by the next-generation sequencing and confirmed by Sanger sequencing. Related literature from PubMed, Online Mendelian Inheritance in Man (OMIM), CNKI and Wanfang databases to date (up to October 2016) with"Rapid-onset dystonia-parkinsonism"RDP"DYT12" as key words was reviewed...
April 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28434708/dual-phase-18-f-florbetapir-positron-emission-tomography-in-patients-with-primary-progressive-aphasia-alzheimer-s-disease-and-healthy-controls-a-preliminary-study
#2
Hung-Chou Kuo, Ing-Tsung Hsiao, Chia-Ju Hsieh, Chu-Yun Huang, Kuo-Lun Huang, Yau-Yau Wai, Wen-Li Chuang, Mei-Ping Kung, Yi-Chuan Chu, Tzu-Chen Yen, Kun-Ju Lin, Chin-Chang Huang
BACKGROUND: To determine whether dual-phase (18)F-florbetapir positron emission tomography imaging with perfusion-like and amyloid deposition information can distinguish among primary progressive aphasia (PPA), Alzheimer's disease (AD), and healthy controls (HCs). METHODS: Patients diagnosed with PPA, including four semantic dementia (SD) and two progressive nonfluent aphasia (PNFA), as well as one logopenic variant (LV) of PPA, were studied. All PPA patients, and age-/sex-matched patients with probable AD (n=8) and HCs (n=8) were subjected to dual-phase (18)F-florbetapir imaging...
April 20, 2017: Journal of the Formosan Medical Association, Taiwan Yi Zhi
https://www.readbyqxmd.com/read/28433979/lethal-high-acute-disseminated-encephalomyelitis-adem-triggered-by-toxic-effect-of-synthetic-cannabinoid-black-mamba
#3
Kiran Samra, Ian S Boon, Gregory Packer, Saiju Jacob
A previously well 25-year-old man presented with agitation, double incontinence and left-sided incoordination. His symptoms started after smoking a synthetic cannabinoid (black mamba) 5 days earlier. Over 48 hours, he developed aphasia, generalised hypertonia, hyper-reflexia and dense left hemiparesis. This progressed to profuse diaphoresis, fever, tachycardia, hypertension and a possible seizure necessitating admission to the intensive care unit. CT head and cerebrospinal fluid analysis were unremarkable...
April 22, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28432988/comprehension-of-concrete-and-abstract-words-in-semantic-variant-primary-progressive-aphasia-and-alzheimer-s-disease-a-behavioral-and-neuroimaging-study
#4
Sven Joubert, Guillaume T Vallet, Maxime Montembeault, Mariem Boukadi, Maximiliano A Wilson, Robert Jr Laforce, Isabelle Rouleau, Simona M Brambati
The aim of this study was to investigate the comprehension of concrete, abstract and abstract emotional words in semantic variant primary progressive aphasia (svPPA), Alzheimer's disease (AD), and healthy elderly adults (HE) Three groups of participants (9 svPPA, 12 AD, 11 HE) underwent a general neuropsychological assessment, a similarity judgment task, and structural brain MRI. The three types of words were processed similarly in the group of AD participants. In contrast, patients in the svPPA group were significantly more impaired at processing concrete words than abstract words, while comprehension of abstract emotional words was in between...
April 19, 2017: Brain and Language
https://www.readbyqxmd.com/read/28427300/object-decision-test-borb-normative-data-for-the-adult-quebec-population-and-performance-in-alzheimer-s-disease-and-the-semantic-variant-of-primary-progressive-aphasia
#5
Alexandre St-Hilaire, Marie-Claude Blackburn, Maximiliano A Wilson, Robert Jr Laforce, Carol Hudon, Joël Macoir
Object decision (OD) test is one subtest of the Birmingham Object Recognition Battery (BORB). It is useful for differential diagnosis among several neurodegenerative diseases. However, normative data provided with this battery count on very few subjects and do not control for the effect of age, which limits interpretability. The purpose of Study 1 was to provide normative data for the OD test of the BORB (version A-hard). The objectives of Study 2 were to establish the diagnostic validity of this task and predictive validity of the normative data in the case of the semantic variant of primary progressive aphasia (svPPA) and Alzheimer's disease (AD)...
April 21, 2017: Neuropsychology, Development, and Cognition. Section B, Aging, Neuropsychology and Cognition
https://www.readbyqxmd.com/read/28424650/the-role-of-the-left-anterior-temporal-lobe-for-unpredictable-and-complex-mappings-in-word-reading
#6
Marilyne Joyal, Simona M Brambati, Robert J Laforce, Maxime Montembeault, Mariem Boukadi, Isabelle Rouleau, Joël Macoir, Sven Joubert, Shirley Fecteau, Maximiliano A Wilson
The anterior temporal lobes (ATLs) have been consistently associated with semantic processing which, in turn, has a key role in reading aloud single words. This study aimed to investigate (1) the reading abilities in patients with the semantic variant of primary progressive aphasia (svPPA), and (2) the relationship between gray matter (GM) volume of the left ATL and word reading performance using voxel-based morphometry (VBM). Three groups of participants (svPPA, Alzheimer's Disease, AD and healthy elderly adults) performed a reading task with exception words, regular words and pseudowords, along with a structural magnetic resonance imaging scan...
2017: Frontiers in Psychology
https://www.readbyqxmd.com/read/28413741/serial-magnetic-resonance-imaging-in-creutzfeldt-jakob-disease-a-case-report-and-literature-review
#7
Ahmed H Qavi, Tasnim F Imran, Zachariah Hasan, Fariha Ilyas, Usman Ghani, Salman Assad, Shabih Hasan
Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, invariably fatal brain disorder. CJD usually appears in later life and runs a rapid course. Typically, the onset of symptoms occurs about age 60 and about 90% of individuals die within one year. We report a case of 67-year-old male presented with progressive aphasia, confusion, dysphagia and inability to carry out activities of daily life (ADLs) over a period of three to four weeks. The patient had past medical history of chronic atrial fibrillation and hypertension...
March 14, 2017: Curēus
https://www.readbyqxmd.com/read/28413716/data-driven-regions-of-interest-for-longitudinal-change-in-three-variants-of-frontotemporal-lobar-degeneration
#8
COMPARATIVE STUDY
Richard J Binney, Aleksandr Pankov, Gabriel Marx, Xuanzie He, Faye McKenna, Adam M Staffaroni, John Kornak, Suneth Attygalle, Adam L Boxer, Norbert Schuff, Maria-Luisa Gorno-Tempini, Michael W Weiner, Joel H Kramer, Bruce L Miller, Howard J Rosen
INTRODUCTION: Longitudinal imaging of neurodegenerative disorders is a potentially powerful biomarker for use in clinical trials. In Alzheimer's disease, studies have demonstrated that empirically derived regions of interest (ROIs) can provide more reliable measurement of disease progression compared with anatomically defined ROIs. METHODS: We set out to derive ROIs with optimal effect size for quantifying longitudinal change in a hypothetical clinical trial by comparing atrophy rates in 44 patients with behavioral variant of frontotemporal dementia (bvFTD), 30 with the semantic variant primary progressive aphasia (svPPA), and 26 with the nonfluent variant PPA (nfvPPA) to atrophy in 97 cognitively healthy controls...
April 2017: Brain and Behavior
https://www.readbyqxmd.com/read/28412599/group-intervention-for-individuals-with-primary-progressive-aphasia-and-their-spouses-who-comes-first
#9
Regina Jokel, Jed Meltzer, J D R, L D M, J J C, E A N, C D T
Primary progressive aphasia (PPA) is a neurodegenerative dementia in which language impairment is the first and most dominant symptom. There is a considerable dearth of interventions for PPA although language rehabilitation has made headway in managing the disorder. Thus far, no comprehensive services have been proposed for PPA clients and/or their spouses. This paper describes the first structured group intervention program designated exclusively for people with PPA and their caregivers. This pilot project originates from a clinical service and presents supporting evidence for initiation of a larger study to establish an evidence-based intervention for PPA...
April 9, 2017: Journal of Communication Disorders
https://www.readbyqxmd.com/read/28410663/frontotemporal-dementia
#10
REVIEW
Nicholas T Olney, Salvatore Spina, Bruce L Miller
Frontotemporal dementia (FTD) is a heterogeneous disorder with distinct clinical phenotypes associated with multiple neuropathologic entities. Presently, the term FTD encompasses clinical disorders that include changes in behavior, language, executive control, and often motor symptoms. The core FTD spectrum disorders include behavioral variant FTD, nonfluent/agrammatic variant primary progressive aphasia, and semantic variant PPA. Related FTD disorders include frontotemporal dementia with motor neuron disease, progressive supranuclear palsy syndrome, and corticobasal syndrome...
May 2017: Neurologic Clinics
https://www.readbyqxmd.com/read/28407792/treatment-of-post-anesthesia-dementia-with-perispinal-etanercept-injection-and-hyperbaric-oxygen-therapy-a-case-report
#11
Steve Best, Dan G Pavel
BACKGROUND: We report on the first case of successful treatment for post-anesthesia dementia with perispinal etanercept injection combined with hyperbaric oxygen therapy. CASE PRESENTATION: Our patient was a 77-year-old Caucasian man of Mexican ethnicity who presented to our clinic 4.5 years after a knee replacement surgery. Immediately post-surgery, the patient began to show dramatic cognitive, physical, and emotional impairment compared with his presurgical state; these symptoms were still present when he arrived at our clinic...
April 14, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28391035/selective-verbal-recognition-memory-impairments-are-associated-with-atrophy-of-the-language-network-in-non-semantic-variants-of-primary-progressive-aphasia
#12
Aneesha S Nilakantan, Joel L Voss, Sandra Weintraub, M-Marsel Mesulam, Emily J Rogalski
Primary progressive aphasia (PPA) is clinically defined by an initial loss of language function and preservation of other cognitive abilities, including episodic memory. While PPA primarily affects the left-lateralized perisylvian language network, some clinical neuropsychological tests suggest concurrent initial memory loss. The goal of this study was to test recognition memory of objects and words in the visual and auditory modality to separate language-processing impairments from retentive memory in PPA...
April 6, 2017: Neuropsychologia
https://www.readbyqxmd.com/read/28387812/gene-based-association-studies-report-genetic-links-for-clinical-subtypes-of-frontotemporal-dementia
#13
Aniket Mishra, Raffaele Ferrari, Peter Heutink, John Hardy, Yolande Pijnenburg, Danielle Posthuma
Genome-wide association studies in frontotemporal dementia showed limited success in identifying associated loci. This is possibly due to small sample size, allelic heterogeneity, small effect sizes of single genetic variants, and the necessity to statistically correct for testing millions of genetic variants. To overcome these issues, we performed gene-based association studies on 3348 clinically identified frontotemporal dementia cases and 9390 controls (discovery, replication and joint-cohort analyses). We report association of APOE and TOMM40 with behavioural variant frontotemporal dementia, and ARHGAP35 and SERPINA1 with progressive non-fluent aphasia...
April 5, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28387671/predicting-development-of-amyotrophic-lateral-sclerosis-in-frontotemporal-dementia
#14
Tim Van Langenhove, Olivier Piguet, James R Burrell, Cristian Leyton, David Foxe, Melissa Abela, Lauren Bartley, Woojin S Kim, Eve Jary, Yue Huang, Carol Dobson-Stone, John B Kwok, Glenda M Halliday, John R Hodges
BACKGROUND: A proportion of patients with frontotemporal dementia (FTD) also develop amyotrophic lateral sclerosis (ALS). OBJECTIVE: We aimed to establish the risk of developing ALS in patients presenting with FTD and to identify the relevant clinical variables associated with progression from FTD to FTD-ALS. METHODS: Of 218 consecutive patients with FTD, 10.1% had a dual FTD-ALS diagnosis at presentation. The remaining 152 FTD patients with follow-up of at least 12 months were included in the present study...
April 3, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28386688/beyond-the-midbrain-atrophy-wide-spectrum-of-structural-mri-finding-in-cases-of-pathologically-proven-progressive-supranuclear-palsy
#15
REVIEW
Keita Sakurai, Aya M Tokumaru, Keigo Shimoji, Shigeo Murayama, Kazutomi Kanemaru, Satoru Morimoto, Ikuko Aiba, Motoo Nakagawa, Yoshiyuki Ozawa, Masashi Shimohira, Noriyuki Matsukawa, Yoshio Hashizume, Yuta Shibamoto
PURPOSE: Recently, it has been recognized that pathologically proven progressive supranuclear palsy (PSP) cases are classified into various clinical subtypes with non-uniform symptoms and imaging findings. This article reviews essential imaging findings, general information, and advanced magnetic resonance imaging (MRI) techniques for PSP and presents these MRI findings of pathologically proven typical and atypical PSP cases for educational purposes. METHODS: With the review of literatures, notably including atypical pathologically proven PSP cases, MRI and clinical information of 15 pathologically proven typical and atypical PSP cases were retrospectively evaluated...
April 6, 2017: Neuroradiology
https://www.readbyqxmd.com/read/28373956/a-neural-network-underlying-intentional-emotional-facial-expression-in-neurodegenerative-disease
#16
Kelly A Gola, Tal Shany-Ur, Peter Pressman, Isa Sulman, Eduardo Galeana, Hillary Paulsen, Lauren Nguyen, Teresa Wu, Babu Adhimoolam, Pardis Poorzand, Bruce L Miller, Katherine P Rankin
Intentional facial expression of emotion is critical to healthy social interactions. Patients with neurodegenerative disease, particularly those with right temporal or prefrontal atrophy, show dramatic socioemotional impairment. This was an exploratory study examining the neural and behavioral correlates of intentional facial expression of emotion in neurodegenerative disease patients and healthy controls. One hundred and thirty three participants (45 Alzheimer's disease, 16 behavioral variant frontotemporal dementia, 8 non-fluent primary progressive aphasia, 10 progressive supranuclear palsy, 11 right-temporal frontotemporal dementia, 9 semantic variant primary progressive aphasia patients and 34 healthy controls) were video recorded while imitating static images of emotional faces and producing emotional expressions based on verbal command; the accuracy of their expression was rated by blinded raters...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28357585/uneven-cerebral-hemodynamic-change-as-a-cause-of-neurological-deterioration-in-the-acute-stage-after-direct-revascularization-for-moyamoya-disease-cerebral-hyperperfusion-and-remote-ischemia-caused-by-the-watershed-shift
#17
Xian-Kun Tu, Miki Fujimura, Sherif Rashad, Shunji Mugikura, Hiroyuki Sakata, Kuniyasu Niizuma, Teiji Tominaga
Superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis is the standard surgical treatment for moyamoya disease (MMD). The main potential complications of this treatment are cerebral hyperperfusion (CHP) syndrome and ischemia, and their managements are contradictory to each other. We retrospectively investigated the incidence of the simultaneous manifestation of CHP and infarction after surgery for MMD. Of the 162 consecutive direct revascularization surgeries performed for MMD, we encountered two adult cases (1...
March 29, 2017: Neurosurgical Review
https://www.readbyqxmd.com/read/28355946/dysexecutive-symptoms-in-primary-progressive-aphasia
#18
Joël Macoir, Monica Lavoie, Robert Laforce, Simona M Brambati, Maximiliano A Wilson
Primary progressive aphasia (PPA) is a heterogeneous neurodegenerative condition in which the most prominent clinical feature is language difficulties. Other cognitive domains have been described to remain unaffected at the early stages of the disease and, therefore, excluded from diagnostic criteria. However, we show in this article that executive function (EF) disorders may be present in the 3 variants (nonfluent/agrammatic, logopenic, and semantic) of PPA. We also illustrate changes in language and EF by means of a 3-year behavioral and neuroimaging longitudinal study of a patient suffering from the semantic variant of PPA...
January 1, 2017: Journal of Geriatric Psychiatry and Neurology
https://www.readbyqxmd.com/read/28355115/complete-hematologic-and-molecular-response-in-adult-patients-with-relapsed-refractory-philadelphia-chromosome-positive-b-precursor-acute-lymphoblastic-leukemia-following-treatment-with-blinatumomab-results-from-a-phase-ii-single-arm-multicenter-study
#19
Giovanni Martinelli, Nicolas Boissel, Patrice Chevallier, Oliver Ottmann, Nicola Gökbuget, Max S Topp, Adele K Fielding, Alessandro Rambaldi, Ellen K Ritchie, Cristina Papayannidis, Lulu Ren Sterling, Jonathan Benjamin, Anthony Stein
Purpose Few therapeutic options are available for patients with Philadelphia chromosome-positive (Ph(+)) B-precursor acute lymphoblastic leukemia (ALL) who progress after failure of tyrosine kinase inhibitor (TKI) -based therapy. Here, we evaluated the efficacy and tolerability of blinatumomab in patients with relapsed or refractory Ph(+) ALL. Patients and Methods This open-label phase II study enrolled adults with Ph(+) ALL who had relapsed after or were refractory to at least one second-generation or later TKI or were intolerant to second-generation or later TKIs and intolerant or refractory to imatinib...
March 29, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28345017/proof-of-concept-demonstration-of-optimal-composite-mri-endpoints-for-clinical-trials
#20
Steven D Edland, M Colin Ard, Jaiashre Sridhar, Derin Cobia, Adam Martersteck, M Marsel Mesulam, Emily J Rogalski
BACKGROUND: Atrophy measures derived from structural MRI are promising outcome measures for early phase clinical trials, especially for rare diseases such as primary progressive aphasia (PPA), where the small available subject pool limits our ability to perform meaningfully powered trials with traditional cognitive and functional outcome measures. METHODS: We investigated a composite atrophy index in 26 PPA participants with longitudinal MRIs separated by two years...
September 2016: Alzheimer's & Dementia: Translational Research & Clinical Interventions
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