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Progressive aphasia

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https://www.readbyqxmd.com/read/29309493/rates-of-amyloid-imaging-positivity-in-patients-with-primary-progressive-aphasia
#1
Miguel A Santos-Santos, Gil D Rabinovici, Leonardo Iaccarino, Nagehan Ayakta, Gautam Tammewar, Iryna Lobach, Maya L Henry, Isabel Hubbard, Maria Luisa Mandelli, Edoardo Spinelli, Zachary A Miller, Peter S Pressman, James P O'Neil, Pia Ghosh, Andreas Lazaris, Marita Meyer, Christa Watson, Soo Jin Yoon, Howard J Rosen, Lea Grinberg, William W Seeley, Bruce L Miller, William J Jagust, Maria Luisa Gorno-Tempini
Importance: The ability to predict the pathology underlying different neurodegenerative syndromes is of critical importance owing to the advent of molecule-specific therapies. Objective: To determine the rates of positron emission tomography (PET) amyloid positivity in the main clinical variants of primary progressive aphasia (PPA). Design, Setting, and Participants: This prospective clinical-pathologic case series was conducted at a tertiary research clinic specialized in cognitive disorders...
January 8, 2018: JAMA Neurology
https://www.readbyqxmd.com/read/29305438/asymmetric-tdp-pathology-in-primary-progressive-aphasia-with-right-hemisphere-language-dominance
#2
Garam Kim, Shahrooz Vahedi, Tamar Gefen, Sandra Weintraub, Eileen H Bigio, Marek-Marsel Mesulam, Changiz Geula
OBJECTIVE: To quantitatively examine the regional densities and hemispheric distribution of the 43-kDa transactive response DNA-binding protein (TDP-43) inclusions, neurons, and activated microglia in a left-handed patient with right hemisphere language dominance and logopenic-variant primary progressive aphasia (PPA). METHODS: Phosphorylated TDP-43 inclusions, neurons, and activated microglia were visualized with immunohistochemical and histologic methods. Markers were quantified bilaterally with unbiased stereology in language- and memory-related cortical regions...
January 5, 2018: Neurology
https://www.readbyqxmd.com/read/29304167/the-language-disorder-of-prion-disease-is-characteristic-of-a-dynamic-aphasia-and-is-rarely-an-isolated-clinical-feature
#3
Diana Caine, Akin Nihat, Philippa Crabb, Peter Rudge, Lisa Cipolotti, John Collinge, Simon Mead
BACKGROUND: Akinetic mutism is a key diagnostic feature of prion diseases, however, their rapidly progressive nature makes detailed investigation of the language disorder in a large cohort extremely challenging. This study aims to position prion diseases in the nosology of language disorders and improve early clinical recognition. METHODS: A systematic, prospective investigation of language disorders in a large cohort of patients diagnosed with prion diseases. 568 patients were included as a sub-study of the National Prion Monitoring Cohort...
2018: PloS One
https://www.readbyqxmd.com/read/29300972/a-recurrent-de-novo-missense-mutation-in-ubtf-causes-developmental-neuroregression
#4
Camilo Toro, Roderick T Hori, May Christine V Malicdan, Cynthia J Tifft, Amy Goldstein, William A Gahl, David R Adams, Fauni Harper, Lynne A Wolfe, Jianfeng Xiao, Mohammad M Khan, Jun Tian, Kevin A Hope, Lawrence T Reiter, Michel G Tremblay, Thomas Moss, Alexis L Franks, Chris Balak, Mark S LeDoux
UBTF (upstream binding transcription factor) exists as two isoforms; UBTF1 regulates rRNA transcription by RNA polymerase 1, whereas UBTF2 regulates mRNA transcription by RNA polymerase 2. Herein, we describe 4 patients with very similar patterns of neuroregression due to recurrent de novo mutations in UBTF (GRCh37/hg19, NC_000017.10:g.42290219C>T, NM_014233.3:c.628G>A) resulting in the same amino acid change in both UBTF1 and UBTF2 (p.Glu210Lys [p.E210K]). Disease onset in our cohort was at 2.5 to 3 yrs and characterized by slow progression of global motor, cognitive and behavioral dysfunction...
January 2, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29298550/maximising-recovery-from-aphasia-with-central-and-peripheral-agraphia-the-benefit-of-sequential-treatments
#5
Pélagie M Beeson, Chelsea Bayley, Christine Shultz, Kindle Rising
Maximal recovery from acquired language impairment may require progression from one behavioural treatment protocol to the next in order to build upon residual and relearned cognitive-linguistic and sensory-motor processes. We present a five-stage treatment sequence that was initiated at one year post stroke in a woman with acquired impairments of spoken and written language. As is typical of individuals with left perisylvian damage, she demonstrated marked impairment of phonological retrieval and sublexical phonology, but she also faced additional challenges due to impaired letter shape knowledge and visual attention...
January 3, 2018: Neuropsychological Rehabilitation
https://www.readbyqxmd.com/read/29277589/transdural-propagation-of-glioblastoma-through-the-foramen-rotundum
#6
Seiichiro Imataka, Yukinori Akiyama, Rintaro Yokoyama, Nobuhiro Mikuni
BACKGROUND: Glioblastoma, also known as glioblastoma multiforme (GBM), is the most common primary malignant cerebral tumor in adults. Although GBM is one of the most aggressive tumors in the brain, propagation through the dura mater is very rare. CASE DESCRIPTION: A 59-year-old man presented with progressive headache and aphasia. Magnetic resonance (MR) imaging identified an abnormal mass extending transcranially through the widened foramen rotundum into the infratemporal fossa and cavernous sinus...
December 22, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/29273692/efficacy-of-intensive-aphasia-therapy-in-patients-with-chronic-stroke-a-randomised-controlled-trial
#7
Benjamin Stahl, Bettina Mohr, Verena Büscher, Felix R Dreyer, Guglielmo Lucchese, Friedemann Pulvermüller
OBJECTIVE: Recent evidence has fuelled the debate on the role of massed practice in the rehabilitation of chronic post-stroke aphasia. Here, we further determined the optimal daily dosage and total duration of intensive speech-language therapy. METHODS: Individuals with chronic aphasia more than 1 year post-stroke received Intensive Language-Action Therapy in a randomised, parallel-group, blinded-assessment, controlled trial. Participants were randomly assigned to one of two outpatient groups who engaged in either highly-intensive practice (Group I: 4 hours daily) or moderately-intensive practice (Group II: 2 hours daily)...
December 22, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/29260037/cefepime-induced-neurotoxicity-a-case-series-and-review-of-the-literature
#8
Cigdem Isitan, Andrew Ferree, Anna DePold Hohler
Cefepime is a fourth generation cephalosporin which is bactericidal for broad spectrum of organisms. This is a case-series of three patients who presented to our hospital with confusion secondary to cefepime use to treat urinary tract infection (UTI) and health care associated pneumonia (HCAP), after excluding other common etiologies of altered mental status (AMS). Of these three patients, one had progressive expressive aphasia and the other two demonstrated asynchronous myoclonic activity of the limbs. The symptoms were seen within four to five days of initiating the treatment and resolved within three days of discontinuation of cefepime...
September 2017: ENeurologicalSci
https://www.readbyqxmd.com/read/29245281/spontaneous-spinal-epidural-hematoma-mimicking-transient-ischemic-attack-a-case-report
#9
Chuqiao Li, Rui He, Xiaoqiang Li, Yulan Zhong, Li Ling, Fangming Li
RATIONALE: Spontaneous spinal epidural hematoma (SSEH) is a rare but highly disabling neurological emergency. The initial presentations are variable. Most patients of SSEH present with paraplegia or tetraplegia clinically, but recurrent hemiparesis with complete spontaneous recovery, mimicking transient ischemic attack (TIA), is a very rare initial presentation of SSEH. PATIENT CONCERNS: A 71-year-old female presented to the emergency department with 2 episodes of transient right hemiparesis in 5 hours...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29241649/the-structure-of-the-mental-lexicon-what-primary-progressive-aphasias-reveal
#10
Clara Sanches, Alexandre Routier, Olivier Colliot, Marc Teichmann
Like recursive syntax, a structured mental lexicon is specific to the human species but its internal organization remains unclear. It is thought to contain information about the semantic, syntactic (e.g., gender) and formal (orthographic/phonological) features of a word. Previous studies suggested that these three components might be separated at the behavioral level and that they might be implemented by temporal cortices. However, the available investigations are based on case reports or small-cohort studies with patients demonstrating post-stroke aphasia, and they did not contrast the three lexical components in a directly comparable way...
December 11, 2017: Neuropsychologia
https://www.readbyqxmd.com/read/29228180/longitudinal-structural-and-molecular-neuroimaging-in-agrammatic-primary-progressive-aphasia
#11
Katerina A Tetzloff, Joseph R Duffy, Heather M Clark, Edythe A Strand, Mary M Machulda, Christopher G Schwarz, Matthew L Senjem, Robert I Reid, Anthony J Spychalla, Nirubol Tosakulwong, Val J Lowe, Clifford R Jack, Keith A Josephs, Jennifer L Whitwell
The agrammatic variant of primary progressive aphasia affects normal grammatical language production, often occurs with apraxia of speech, and is associated with left frontal abnormalities on cross-sectional neuroimaging studies. We aimed to perform a detailed assessment of longitudinal change on structural and molecular neuroimaging to provide a complete picture of neurodegeneration in these patients, and to determine how patterns of progression compare to patients with isolated apraxia of speech (primary progressive apraxia of speech)...
December 8, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29223682/neocortical-origin-and-progression-of-gray-matter-atrophy-in-nonamnestic-alzheimer-s-disease
#12
Jeffrey S Phillips, Fulvio Da Re, Laynie Dratch, Sharon X Xie, David J Irwin, Corey T McMillan, Sanjeev N Vaishnavi, Carlo Ferrarese, Edward B Lee, Leslie M Shaw, John Q Trojanowski, David A Wolk, Murray Grossman
Amnestic Alzheimer's disease (AD) is characterized by early atrophy of the hippocampus and medial temporal lobes before spreading to the neocortex. In contrast, nonamnestic Alzheimer's patients have relative sparing of the hippocampus, but the pattern in which the disease spreads is unclear. We examined spreading disease in nonamnestic AD using a novel magnetic resonance imaging-based analysis adapted from pathologic staging studies, applied here to cross-sectional imaging data. We selected 240 T1-weighted scans from 129 patients with pathology confirmed by autopsy or cerebrospinal fluid, and atrophy maps were computed relative to 238 scans from 115 elderly controls...
November 21, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/29223183/conversion-between-addenbrooke-s-cognitive-examination-iii-and-mini-mental-state-examination
#13
Jordi A Matías-Guiu, Vanesa Pytel, Ana Cortés-Martínez, María Valles-Salgado, Teresa Rognoni, Teresa Moreno-Ramos, Jorge Matías-Guiu
BACKGROUND: We aim to provide a conversion between Addenbrooke's Cognitive Examination III (ACE-III) and Mini-Mental State Examination (MMSE) scores, to predict the MMSE result based on ACE-III, thus avoiding the need for both tests, and improving their comparability. METHODS: Equipercentile equating method was used to elaborate a conversion table using a group of 400 participants comprising healthy controls and Alzheimer's disease (AD) patients. Then, reliability was assessed in a group of 100 healthy controls and patients with AD, 52 with primary progressive aphasia and 22 with behavioral variant frontotemporal dementia...
December 10, 2017: International Psychogeriatrics
https://www.readbyqxmd.com/read/29213499/an-interdisciplinary-approach-aiding-the-diagnosis-of-primary-progressive-aphasia-a-case-report
#14
Nadia Shigaeff, Mayra Zanetti, Sibelle de Almeida Tierno, Ana Beatriz Galhardi Di Tommaso, Thais Cristina Marques, Fábio Gazelato de Mello Franco
Frontotemporal dementia (FTD) is one of the most common causes of early-onset dementia with primary progressive aphasia (PPA) being the second-most-frequent form of this degenerative disease. Despite the similarity with progressive dementia (especially in early stages of Alzheimer´s disease), three types of PPA can be differentiated: semantic, agrammatic and logopenic (subtype discussed in this study). To date, no medications have been shown to improve or stabilize cognitive deficits in patients with PPA. We report the case of a 62-year-old woman with difficulty naming objects and planning...
January 2017: Dementia & Neuropsychologia
https://www.readbyqxmd.com/read/29201014/impaired-interoceptive-accuracy-in-semantic-variant-primary-progressive-aphasia
#15
Charles R Marshall, Chris J D Hardy, Lucy L Russell, Camilla N Clark, Katrina M Dick, Emilie V Brotherhood, Rebecca L Bond, Catherine J Mummery, Jonathan M Schott, Jonathan D Rohrer, James M Kilner, Jason D Warren
Background: Interoception (the perception of internal bodily sensations) is strongly linked to emotional experience and sensitivity to the emotions of others in healthy subjects. Interoceptive impairment may contribute to the profound socioemotional symptoms that characterize frontotemporal dementia (FTD) syndromes, but remains poorly defined. Methods: Patients representing all major FTD syndromes and healthy age-matched controls performed a heartbeat counting task as a measure of interoceptive accuracy...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/29186630/music-models-aberrant-rule-decoding-and-reward-valuation-in-dementia
#16
Camilla N Clark, Hannah L Golden, Oliver McCallion, Jennifer M Nicholas, Miriam H Cohen, Catherine F Slattery, Ross W Paterson, Phillip D Fletcher, Catherine J Mummery, Jonathan D Rohrer, Sebastian J Crutch, Jason D Warren
Aberrant rule- and reward-based processes underpin abnormalities of socio-emotional behaviour in major dementias. However, these processes remain poorly characterised. Here we used music to probe rule decoding and reward valuation in patients with frontotemporal dementia syndromes and Alzheimer's disease relative to healthy age-matched individuals. We created short melodies that were either harmonically resolved ('finished') or unresolved ('unfinished'); the task was to classify each melody as finished or unfinished (rule processing) and rate its subjective pleasantness (reward valuation)...
November 24, 2017: Social Cognitive and Affective Neuroscience
https://www.readbyqxmd.com/read/29178408/spousal-recollections-of-early-signs-of-primary-progressive-aphasia
#17
Margaret Pozzebon, Jacinta Douglas, David Ames
BACKGROUND: Although primary progressive aphasia (PPA) is characterized by progressive loss of language and communication skills, knowledge about the earliest emerging signs announcing the onset of this condition is limited. AIMS: To explore spousal recollections regarding the earliest signs of PPA and to compare the nature of the earliest perceived symptoms across the three PPA variants. METHODS & PROCEDURES: In-depth interviews focusing on the earliest signs of illness onset were conducted with 13 spouses whose partners were diagnosed with PPA...
November 26, 2017: International Journal of Language & Communication Disorders
https://www.readbyqxmd.com/read/29172203/-factor-xa-inhibitor-for-the-treatment-of-cerebral-venous-thrombosis-with-intracerebral-hemorrhage-during-anticoagulation-therapy-a-case-report
#18
Takehiro Kitagawa, Tetsuhiro Kitahara
A 46-year-old man presented with headache, right hemiparesis, and motor aphasia. A diagnosis of cerebral venous thrombosis with subarachnoid hemorrhage was made. We started intravenous administration of unfractionated heparin. After 6 days in hospital, the right hemiparesis and motor aphasia worsened, and brain computed tomographic(CT)images demonstrated intracerebral hemorrhage in the left parietal lobe. Therefore, we switched treatment from heparin to apixaban. His condition improved and a brain magnetic resonance(MR)venogram after 7 days in hospital showed recanalization of the cerebral veins...
November 2017: No Shinkei Geka. Neurological Surgery
https://www.readbyqxmd.com/read/29149795/development-of-a-psycho-educational-support-program-for-individuals-with-primary-progressive-aphasia-and-their-care-partners
#19
Darby J Morhardt, Mary C O'Hara, Kristine Zachrich, Christina Wieneke, Emily J Rogalski
Primary progressive aphasia is a language-based dementia that initially spares other cognitive domains; however, aphasia interferes with many life roles such as work and interpersonal relationships. Psycho-educational programs, such as support groups have been shown to be effective for persons with Alzheimer's dementia; however, little is known regarding their effectiveness for persons with primary progressive aphasia. This paper describes the development of a program that offers support, education and activities for persons with primary progressive aphasia and their care-partners and its feasibility...
January 1, 2017: Dementia
https://www.readbyqxmd.com/read/29146215/-acute-methotrexate-related-neurotoxicity-and-pseudo-stroke-syndrome
#20
V Deneux, N Leboucq, L Saumet, S Haouy, T Akbaraly, N Sirvent
Treatment of acute lymphoblastic leukemia requires high-dose systemic and/or intrathecal methotrexate to prevent and/or treat central nervous system disorders. Acute neurotoxicity of methotrexate, of unknown etiopathogenesis, is characterized by the polymorphism of clinical manifestations, responsible for a potentially harmful diagnostic delay in these immunosuppressed patients. We describe five episodes of transient acute leukoencephalopathy mimicking a stroke, reported in the literature as "pseudo-stroke syndrome"...
November 13, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
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