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https://www.readbyqxmd.com/read/27932977/human-neural-stem-cell-transplantation-rescues-cognitive-defects-in-app-ps1-model-of-alzheimer-s-disease-by-enhancing-neuronal-connectivity-and-metabolic-activity
#1
Xueyuan Li, Hua Zhu, Xicai Sun, Fuxing Zuo, Jianfeng Lei, Zhanjing Wang, Xinjie Bao, Renzhi Wang
Alzheimer's disease (AD), the most frequent type of dementia, is featured by Aβ pathology, neural degeneration and cognitive decline. To date, there is no cure for this disease. Neural stem cell (NSC) transplantation provides new promise for treating AD. Many studies report that intra-hippocampal transplantation of murine NSCs improved cognition in rodents with AD by alleviating neurodegeneration via neuronal complement or replacement. However, few reports examined the potential of human NSC transplantation for AD...
2016: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/27929803/an-autopsy-verified-case-of-ftld-tdp-type-a-with-upper-motor-neuron-predominant-motor-neuron-disease-mimicking-mm2-thalamic-type-sporadic-creutzfeldt-jakob-disease
#2
Yuichi Hayashi, Yasushi Iwasaki, Akira Takekoshi, Nobuaki Yoshikura, Takahiko Asano, Maya Mimuro, Akio Kimura, Katsuya Satoh, Tetsuyuki Kitamoto, Mari Yoshida, Takashi Inuzuka
Here we report an autopsy-verified case of frontotemporal lobar degeneration (FTLD)-transactivation responsive region (TAR) DNA binding protein (TDP) type A with upper motor neuron-predominant motor neuron disease mimicking MM2-thalamic-type sporadic Creutzfeldt-Jakob disease (sCJD). A 69-year-old woman presented with an 11-month history of progressive dementia, irritability, insomnia, and gait disturbance without a family history of dementia or prion disease. Neurological examination revealed severe dementia, frontal signs, and exaggerated bilateral tendon reflexes...
November 2016: Prion
https://www.readbyqxmd.com/read/27915998/an-update-on-semantic-dementia-genetics-imaging-and-pathology
#3
REVIEW
Ramon Landin-Romero, Rachel Tan, John R Hodges, Fiona Kumfor
Progressive and relatively circumscribed loss of semantic knowledge, referred to as semantic dementia (SD) which falls under the broader umbrella of frontotemporal dementia, was officially identified as a clinical syndrome less than 50 years ago. Here, we review recent neuroimaging, pathological, and genetic research in SD. From a neuroimaging perspective, SD is characterised by hallmark asymmetrical atrophy of the anterior temporal pole and anterior fusiform gyrus, which is usually left lateralised. Functional magnetic resonance imaging (fMRI) studies have revealed widespread changes in connectivity, implicating the anterior temporal regions in semantic deficits in SD...
December 5, 2016: Alzheimer's Research & Therapy
https://www.readbyqxmd.com/read/27915581/ftld-tdp-and-progressive-supranuclear-palsy-in-comorbidity-a-report-of-two-cases-with-different-clinical-presentations
#4
Kateřina Storey, Silvie Johanidesová, Radoslav Matěj, Jiří Keller, Zdeněk Rohan, Robert Rusina
Frontotemporal lobar degeneration with transactive response DNA-binding protein 43 (FTLD-TDP) and progressive supranuclear palsy (PSP) are distinct neurodegenerations with different clinical presentations. We report two cases with FTLD-TDP and PSP in comorbidity: a patient with amnestic dementia developing frontal lobe dementia, Parkinsonism and supranuclear gaze palsy and a patient with cerebellar ataxia and nystagmus developing akinesia, rigidity, and subcortical dementia. Neuropathological examination revealed neuronal and glial tau pathology together with ubiquitin, and phospho-TDP-43-immunoreactivities in the hippocampus, striatum, mesencephalon, and frontal and temporal cortices...
December 3, 2016: Neurocase
https://www.readbyqxmd.com/read/27905359/-clinical-and-neuropsychological-features-of-alzheimer-s-disease-in-the-combination-with-cerebrovascular-disease
#5
N A Trusova, O S Levin, A V Arablinsky
AIM: To study clinical/neuropsychological and neuroimaging characteristics of Alzheimer's disease in the combination with cerebrovascular disease (CVD). MATERIAL AND METHODS: Ninety patients with dementia, including 35 patients with AD, 35 patients with mixed dementia (MD) and 20 patients with vascular dementia, were examined. The character of dementia was established according to NINCDS-ADRDA and NINDS-AIREN criteria. The neuropsychological battery included Addenbrooke's Cognitive Examination (ACE-R), Montreal Cognitive Assessment scale (MoCA), fluency test and the visual memory test (SCT)...
2016: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
https://www.readbyqxmd.com/read/27893874/association-of-perivascular-localization-of-aquaporin-4-with-cognition-and-alzheimer-disease-in-aging-brains
#6
Douglas M Zeppenfeld, Matthew Simon, J Douglas Haswell, Daryl D'Abreo, Charles Murchison, Joseph F Quinn, Marjorie R Grafe, Randall L Woltjer, Jeffrey Kaye, Jeffrey J Iliff
Importance: Cognitive impairment and dementia, including Alzheimer disease (AD), are common within the aging population, yet the factors that render the aging brain vulnerable to these processes are unknown. Perivascular localization of aquaporin-4 (AQP4) facilitates the clearance of interstitial solutes, including amyloid-β, through the brainwide network of perivascular pathways termed the glymphatic system, which may be compromised in the aging brain. Objectives: To determine whether alterations in AQP4 expression or loss of perivascular AQP4 localization are features of the aging human brain and to define their association with AD pathology...
November 28, 2016: JAMA Neurology
https://www.readbyqxmd.com/read/27885222/the-development-of-a-self-administered-dementia-checklist-the-examination-of-concurrent-validity-and-discriminant-validity
#7
Fumiko Miyamae, Chiaki Ura, Naoko Sakuma, Hirotoshi Niikawa, Hiroki Inagaki, Mutsuo Ijuin, Tsuyoshi Okamura, Mika Sugiyama, Shuichi Awata
PURPOSE: The present study aims to develop a self-administered dementia checklist to enable community-residing older adults to realize their declining functions and start using necessary services. A previous study confirmed the factorial validity and internal reliability of the checklist. The present study examined its concurrent validity and discriminant validity. METHODS: The authors conducted a 3-step study (a self-administered survey including the checklist, interviews by nurses, and interviews by doctors and psychologists) of 7,682 community-residing individuals who were over 65 years of age...
2016: Nihon Ronen Igakkai Zasshi. Japanese Journal of Geriatrics
https://www.readbyqxmd.com/read/27884882/lymphomatosis-cerebri-diagnostic-challenges-and-review-of-the-literature
#8
Paul J Lee, Idanis Berrios, Carolina Ionete, Thomas Smith
Lymphomatosis cerebri (LC) is a rare variant of a primary central nervous system non-Hodgkin's lymphoma (PCNSL) characterised by diffuse infiltration of tumour cells throughout the brain parenchyma. We present a 68-year-old immunocompetent woman with headaches, dizziness, blurred vision, localised right leg weakness and rapidly progressive dementia. A brain MRI demonstrated diffuse T2 hyperintense white matter lesions that did not enhance with contrast. The clinical differential diagnosis of these lesions included metastatic disease, infectious or inflammatory process such as sarcoidosis, lymphoma, demyelinating disease and less likely vascular aetiology, such as vasculitis or ischaemic stroke...
November 24, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27876002/sporadic-creutzfeldt-jakob-disease-with-unusual-initial-presentation-as-posterior-reversible-encephalopathy-syndrome-a-case-report
#9
Edgaras Dirzius, Renata Balnyte, Vesta Steibliene, Rymante Gleizniene, Inga Gudinaviciene, Andrius Radziunas, Kestutis Petrikonis
BACKGROUND: Creutzfeldt - Jakob disease (CJD) is a rapidly progressive and fatal neurodegenerative prion disease. MRI findings are included in diagnostic criteria for probable CJD, giving a sensitivity and specificity more than 90%, but the atypical radiological presentations in the early stage of the disease could cause the diagnostic difficulties. CJD can be definitively diagnosed by histopathological confirmation, brain biopsy or at autopsy. CASE PRESENTATION: We present a case of 53-year-old woman with a history of a rapidly progressive dementia with symptoms of visual impairment, increased extrapyramidal type muscle tonus, stereotypical movements and ataxic gait resulting in the patient's death after13 months...
November 22, 2016: BMC Neurology
https://www.readbyqxmd.com/read/27869586/hyperfamiliarity-in-amnestic-and-vascular-mild-cognitive-impairment
#10
Pei Shi Chia, Shahul Hameed, Kok Pin Yong, Ling Ling Chan, Simon Kang Seng Ting
OBJECTIVE: Hyperfamiliarity is a phenomenon where new stimuli are perceived as familiar. Previous studies have demonstrated familiarity disorder in mild cognitive impairment (MCI), but mostly from the perspective of a neuropsychological approach, and the exact correlation of MCI aetiologies with the phenomenon remains uncertain. Based on current evidence suggesting a frontal-subcortical pathway contributing to familiarity processing, we hypothesize that individuals with a vascular aetiology of MCI will likely suffer more familiarity deficits...
November 21, 2016: Canadian Journal of Neurological Sciences. le Journal Canadien des Sciences Neurologiques
https://www.readbyqxmd.com/read/27869457/autosomal-dominant-cerebellar-ataxia-deafness-and-narcolepsy-adca-dn-associated-with-progressive-cognitive-and-behavioral-deterioration
#11
Lisa A S Walker, Pierre Bourque, Andra M Smith, Jodi Warman Chardon
Objective: Autosomal dominant cerebellar ataxia with deafness and narcolepsy (ADCA-DN) is an emerging syndrome caused by mutations in the C-terminus end of the TS domain of the DNMT1 gene. ADCA-DN is also associated with sensorimotor polyneuropathy, extrapyramidal, and dysautonomic signs, as well as dementia. Little has been reported about the progressive cognitive impairment associated with ADCA-DN. Our objective is to provide a detailed characterization of the cognitive profile of ADCA-DN. Method: Three members of a kindred with ADCA-DN underwent comprehensive neuropsychological testing and neuroimaging...
November 21, 2016: Neuropsychology
https://www.readbyqxmd.com/read/27866203/frontal-assessment-battery-as-a-useful-tool-to-differentiate-mild-cognitive-impairment-due-to-subcortical-ischemic-vascular-disease-from-alzheimer-disease
#12
Yen-Hsuan Hsu, Ching-Feng Huang, Chung-Ping Lo, Tzu-Lan Wang, Chi-Cheng Yang, Min-Chien Tu
BACKGROUND: Prominent executive dysfunction can differentiate vascular dementia from Alzheimer disease (AD). However, it is unclear whether the Frontal Assessment Battery (FAB) screening tool can differentiate subcortical ischemic vascular disease (SIVD) from AD at the pre-dementia stage. In addition, the neural correlates of FAB performance have yet to be clarified. METHODS: Patients with mild cognitive impairment (MCI) due to SIVD (MCI-V), MCI due to AD (MCI-A), and demographically matched controls completed the Mini-Mental State Examination, Taiwanese FAB (TFAB), Category Fluency, and Chinese Version of the Verbal Learning Test, and underwent magnetic resonance imaging...
2016: Dementia and Geriatric Cognitive Disorders
https://www.readbyqxmd.com/read/27861377/familial-atypical-parkinsonism-with-rare-variant-in-vps35-and-fbxo7-genes-a-case-report
#13
Tereza Bartonikova, Katerina Mensikova, Lenka Mikulicova, Radek Vodicka, Radek Vrtel, Marek Godava, Miroslav Vastik, Michaela Kaiserova, Pavel Otruba, Iva Dolinova, Martin Nevrly, Petr Kanovsky
BACKGROUND: A higher prevalence of parkinsonism was recently identified in southeastern Moravia (Czech Republic). Further research confirmed 3 large pedigrees with familial autosomal-dominant parkinsonism spanning 5 generations. METHODS: This case report concerns a patient belonging to one of these 3 pedigrees, in whom motor and oculomotor symptoms were accompanied by frontal-type dementia, who finally developed a clinical phenotype of progressive supranuclear palsy...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27859650/profile-of-cognitive-impairment-and-underlying-pathology-in-multiple-system-atrophy
#14
Shunsuke Koga, Adam Parks, Ryan J Uitti, Jay A van Gerpen, William P Cheshire, Zbigniew K Wszolek, Dennis W Dickson
BACKGROUND: The objectives of this study were to elucidate any potential association between α-synuclein pathology and cognitive impairment and to determine the profile of cognitive impairment in multiple system atrophy (MSA) patients. To do this, we analyzed the clinical and pathologic features in autopsy-confirmed MSA patients. METHODS: We retrospectively reviewed medical records, including neuropsychological test data, in 102 patients with autopsy-confirmed MSA in the Mayo Clinic brain bank...
November 15, 2016: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/27858712/self-consciousness-deficits-in-alzheimer-s-disease-and-frontotemporal-dementia
#15
Eva Ma Arroyo-Anlló, Adèle Turpin Boustonb, Marie-Noëlle Fargeau, Begoña Orgaz Baz, Roger Gil
Self-consciousness (SC) is multifaceted and considered to be the consciousness of one's own mental states. The main aim of this paper is to compare SC in patients with Alzheimer's disease (AD) and behavioral variant frontotemporal dementia (bvFTD). Three groups (control and patient groups) of 23 subjects each were assessed using an SC questionnaire. Both types of dementia clearly induce an alteration of SC. The bvFTD group showed a greater impairment in SC than the AD and control groups. The SC score was strongly associated with frontal functions...
November 14, 2016: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27858707/neural-basis-of-apathy-in-patients-with-amnestic-mild-cognitive-impairment
#16
Hiroaki Kazui, Ryuichi Takahashi, Yuki Yamamoto, Kenji Yoshiyama, Hideki Kanemoto, Yukiko Suzuki, Shunsuke Sato, Shingo Azuma, Takashi Suehiro, Eku Shimosegawa, Kazunari Ishii, Toshihisa Tanaka
BACKGROUND: Although apathy is associated with damage to the frontal and temporal lobes in Alzheimer's disease (AD), the crucial regions for apathy in patients with amnestic mild cognitive impairment (aMCI) are unknown. OBJECTIVE: To identify brain regions associated with apathy in aMCI patients. METHODS: The subjects of this study were 98 aMCI patients who were entered in our dementia registry between March 1, 2009 and April 30, 2015 and who satisfied our criteria for aMCI...
November 14, 2016: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27828579/-effectiveness-of-a-cognitive-motorphysiotherapeutical-therapy-intervention-in-institutionalized-older-adults-with-mild-cognitive-impairment-and-mild-dementia
#17
Alessandra Vieira Menezes, Alessandra da Silva de Aguiar, Elysama Fernandes Alves, Layse Biz de Quadros, Poliana Penasso Bezerra
The aim of this study was to investigate the effectiveness of four months of cognitive-motor physiotherapy intervention, with a single weekly visit, on cognitive function, mobility and functional independence of institutionalized elderly individuals with mild cognitive impairment and mild dementia. In a controlled clinical trial, 15 subjects were allocated to experimental and control groups. Regarding the assessment of the subjects the following instruments were applied: Mini-Mental State Examination, Clinical Dementia Rating Scale, Verbal Fluency Test and Frontal Assessment Battery for analyzing cognitive functions; Berg Balance Scale and Timed Up and Go Test to verify mobility, and; Barthel Scale and Pfeffer Index for measurement of functional independence...
November 2016: Ciência & Saúde Coletiva
https://www.readbyqxmd.com/read/27802229/diverging-progression-of-network-disruption-and-atrophy-in-alzheimer-s-disease-and-semantic-dementia
#18
Jennifer Andreotti, Thomas Dierks, Lars-Olof Wahlund, Matthias Grieder
The progression of cognitive deficits in Alzheimer's disease and semantic dementia is accompanied by grey matter atrophy and white matter deterioration. The impact of neuronal loss on the structural network connectivity in these dementia subtypes is, however, not well understood. In order to gain a more refined knowledge of the topological organization of white matter alterations in dementia, we used a network-based approach to analyze the brain's structural connectivity network. Diffusion-weighted and anatomical MRI images of groups with eighteen Alzheimer's disease and six semantic dementia patients, as well as twenty-one healthy controls were recorded to reconstruct individual connectivity networks...
October 12, 2016: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27797808/clinicopathological-features-of-adult-onset-neuronal-intranuclear-inclusion-disease
#19
Jun Sone, Keiko Mori, Tomonori Inagaki, Ryu Katsumata, Shinnosuke Takagi, Satoshi Yokoi, Kunihiko Araki, Toshiyasu Kato, Tomohiko Nakamura, Haruki Koike, Hiroshi Takashima, Akihiro Hashiguchi, Yutaka Kohno, Takashi Kurashige, Masaru Kuriyama, Yoshihisa Takiyama, Mai Tsuchiya, Naoyuki Kitagawa, Michi Kawamoto, Hajime Yoshimura, Yutaka Suto, Hiroyuki Nakayasu, Naoko Uehara, Hiroshi Sugiyama, Makoto Takahashi, Norito Kokubun, Takuya Konno, Masahisa Katsuno, Fumiaki Tanaka, Yasushi Iwasaki, Mari Yoshida, Gen Sobue
Neuronal intranuclear inclusion disease (NIID) is a slowly progressive neurodegenerative disease characterized by eosinophilic hyaline intranuclear inclusions in the central and peripheral nervous system, and also in the visceral organs. NIID has been considered to be a heterogeneous disease because of the highly variable clinical manifestations, and ante-mortem diagnosis has been difficult. However, since we reported the usefulness of skin biopsy for the diagnosis of NIID, the number of NIID diagnoses has increased, in particular adult-onset NIID...
December 2016: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/27793189/microglial-neuroinflammation-contributes-to-tau-accumulation-in-chronic-traumatic-encephalopathy
#20
Jonathan D Cherry, Yorghos Tripodis, Victor E Alvarez, Bertrand Huber, Patrick T Kiernan, Daniel H Daneshvar, Jesse Mez, Philip H Montenigro, Todd M Solomon, Michael L Alosco, Robert A Stern, Ann C McKee, Thor D Stein
The chronic effects of repetitive head impacts (RHI) on the development of neuroinflammation and its relationship to chronic traumatic encephalopathy (CTE) are unknown. Here we set out to determine the relationship between RHI exposure, neuroinflammation, and the development of hyperphosphorylated tau (ptau) pathology and dementia risk in CTE. We studied a cohort of 66 deceased American football athletes from the Boston University-Veteran's Affairs-Concussion Legacy Foundation Brain Bank as well as 16 non-athlete controls...
October 28, 2016: Acta Neuropathologica Communications
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