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Frontotemporal deterioration

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https://www.readbyqxmd.com/read/29370934/mutation-analysis-of-the-tia1-gene-in-chinese-patients-with-amyotrophic-lateral-sclerosis-and-frontotemporal-dementia
#1
Zhenhua Yuan, Bin Jiao, Lihua Hou, Tingting Xiao, Xixi Liu, Junling Wang, Jun Xu, Lin Zhou, Xinxiang Yan, Beisha Tang, Lu Shen
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by the progressive loss of motor neurons in the brain and spinal cord. Frontotemporal dementia (FTD) is a group of dementia syndromes characterized by the progressive deterioration of behaviors, executive dysfunction, and verbal impairment. Increasing evidence indicates that these 2 diseases share a common genetic etiology and pathophysiological mechanism. Recently, rare mutations in the low-complexity domain of the RNA-binding protein T-cell-restricted intracellular antigen-1 (TIA1) gene were identified in Caucasian ALS and ALS-FTD patients...
December 27, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/29352102/teenage-onset-progressive-myoclonic-epilepsy-due-to-a-familial-c9orf72-repeat-expansion
#2
Jelle van den Ameele, Ivana Jedlickova, Anna Pristoupilova, Anne Sieben, Sara Van Mossevelde, Chantal Ceuterick-de Groote, Helena Hůlková, Radoslav Matej, Alfred Meurs, Christine Van Broeckhoven, Samuel F Berkovic, Patrick Santens, Stanislav Kmoch, Bart Dermaut
BACKGROUND: The progressive myoclonic epilepsies (PME) are a heterogeneous group of disorders in which a specific diagnosis cannot be made in a subset of patients, despite exhaustive investigation. C9orf72 repeat expansions are emerging as an important causal factor in several adult-onset neurodegenerative disorders, in particular frontotemporal lobar degeneration and amyotrophic lateral sclerosis. An association with PME has not been reported previously. OBJECTIVE: To identify the causative mutation in a Belgian family where the proband had genetically unexplained PME...
January 19, 2018: Neurology
https://www.readbyqxmd.com/read/29286511/cerebral-impact-of-prenatal-irradiation-by-131i-an-experimental-model-of-clinical-neuroradioembryological-effects
#3
V V Talko, K M Loganovsky, I P Drozd, Ye V Tukalenko, T K Loganovska, S Yu Nechayev, S V Masiuk, Ye M Prokhorova
Human brain in prenatal period is a most vulnerable to ionizing radiation body structure. Unlike atomic bombings or radiological interventions in healthcare leading at most to external irradiation the intensive internal exposure may occur upon nuclear reactor accidents followed by substantial release and fallout of radioactive 131I. The latter can lead to specific neuroradioembryological effects. OBJECTIVE: To create an experimental model of prenatal cerebral radiation effects of 131I in human and to determine the experimental and clinical neuroradioembryological effects...
December 2017: Problemy Radiat︠s︡iĭnoï Medyt︠s︡yny Ta Radiobiolohiï
https://www.readbyqxmd.com/read/29226876/serum-c-peptide-visfatin-resistin-and-ghrelin-are-altered-in-sporadic-and-grn-associated-frontotemporal-lobar-degeneration
#4
Roberta Zanardini, Luisa Benussi, Silvia Fostinelli, Claudia Saraceno, Miriam Ciani, Barbara Borroni, Alessandro Padovani, Giuliano Binetti, Roberta Ghidoni
Frontotemporal lobar degeneration (FTLD) is a group of complex neurodegenerative disease characterized by progressive deterioration of the frontal and anterior temporal lobes of the brain resulting in different heterogeneous conditions, mainly characterized by personality changes, behavioral disturbances, such as binge eating, and deficits in language and executive functions. Null mutations in progranulin gene (GRN) are one of the most frequent genetic determinants in familial frontotemporal dementia. Recently, progranulin was recognized as an adipokine involved in diet-induced obesity and insulin resistance revealing its metabolic function...
December 1, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29075567/prosocial-deficits-in-behavioral-variant-frontotemporal-dementia-relate-to-reward-network-atrophy
#5
Virginia E Sturm, David C Perry, Kristie Wood, Alice Y Hua, Oscar Alcantar, Samir Datta, Katherine P Rankin, Howard J Rosen, Bruce L Miller, Joel H Kramer
INTRODUCTION: Empathy and shared feelings of reward motivate individuals to share resources with others when material gain is not at stake. Behavioral variant frontotemporal dementia (bvFTD) is a neurodegenerative disease that affects emotion- and reward-relevant neural systems. Although there is diminished empathy and altered reward processing in bvFTD, how the disease impacts prosocial behavior is less well understood. METHODS: A total of 74 participants (20 bvFTD, 15 Alzheimer's disease [AD], and 39 healthy controls) participated in this study...
October 2017: Brain and Behavior
https://www.readbyqxmd.com/read/29031901/als-and-ftd-insights-into-the-disease-mechanisms-and-therapeutic-targets
#6
Rajka M Liscic
Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are neurodegenerative disorders, related by signs of deteriorating motor and cognitive functions, and short survival. The causes are still largely unknown and no effective treatment currently exists. It has been shown that FTLD may coexist with ALS. The overlap between ALS and frontotemporal dementia (FTD), the clinical syndrome associated with FTLD, occurs at clinical, genetic, and pathological levels. The hallmark proteins of the pathognomonic inclusions are SOD-1, TDP-43 or FUS, rarely the disease is caused by mutations in the respective genes...
October 12, 2017: European Journal of Pharmacology
https://www.readbyqxmd.com/read/28936074/syndromes-of-rapidly-progressive-cognitive-decline-our-experience
#7
Sadanandavalli Retnaswami Chandra, Lakshminarayanapuram Gopal Viswanathan, Anupama Ramakanth Pai, Rahul Wahatule, Suvarna Alladi
BACKGROUND: Dementias are fairly slowly progressive degenerative diseases of brain for which treatment options are very less and carry a lot of burden on family and society. A small percentage of them are rapidly progressive and mostly carry a different course outcome. However, there are no definite criteria other than the time line for these patients. AIMS: The aim of this was to identify and categorize the causes and course of rapidly progressive dementias seen in our center...
August 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/28793933/can-cognitive-assessment-really-discriminate-early-stages-of-alzheimer-s-and-behavioural-variant-frontotemporal-dementia-at-initial-clinical-presentation
#8
Sophia Reul, Hubertus Lohmann, Heinz Wiendl, Thomas Duning, Andreas Johnen
BACKGROUND: Neuropsychological testing is considered crucial for differential diagnosis of Alzheimer's disease (AD) and behavioural variant frontotemporal dementia (bvFTD). In-depth neuropsychological assessment revealed specific dysfunctions in the two dementia syndromes. However, a significant overlap of cognitive impairments exists in early disease stages. We questioned whether a standard neuropsychological assessment at initial clinical presentation can delineate patients with AD versus bvFTD...
August 9, 2017: Alzheimer's Research & Therapy
https://www.readbyqxmd.com/read/28042017/the-extended-lateral-supraorbital-approach-and-extradural-anterior-clinoidectomy-through-a-frontopterio-orbital-window-technical-note-and-pilot-surgical-series
#9
Hugo Andrade-Barazarte, Max Jägersberg, Sirajeddin Belkhair, Rachel Tymianski, Mazda K Turel, Karl Schaller, Juha A Hernesniemi, Michael Tymianski, Ivan Radovanovic
BACKGROUND: Lateral approaches to treat anterior cranial fossa lesions have evolved since the first frontotemporal approach described by Dandy in 1918. We describe a less invasive approach to perform extradural anterior clinoidectomy through a lateral supraorbital (LSO) approach for anterior circulation aneurysms and anterolateral skull base lesions. METHODS: The extended LSO approach involves performing a standard lateral supraorbital craniotomy followed by drilling of the sphenoid wing and lateral wall of the orbit through the frontal bony opening of the LSO approach, without any temporal extension of the craniotomy...
April 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28038340/spatio-temporal-and-kinematic-gait-analysis-in-patients-with-frontotemporal-dementia-and-alzheimer-s-disease-through-3d-motion-capture
#10
Rosaria Rucco, Valeria Agosti, Francesca Jacini, Pierpaolo Sorrentino, Pasquale Varriale, Manuela De Stefano, Graziella Milan, Patrizia Montella, Giuseppe Sorrentino
Alzheimer's disease (AD) and behavioral variant of Frontotemporal Dementia (bvFTD) are characterized respectively by atrophy in the medial temporal lobe with memory loss and prefrontal and anterior temporal degeneration with dysexecutive syndrome. In this study, we hypothesized that specific gait patterns are induced by either frontal or temporal degeneration. To test this hypothesis, we studied the gait pattern in bvFTD (23) and AD (22) patients in single and dual task ("motor" and "cognitive") conditions...
February 2017: Gait & Posture
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
#11
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/27831627/-pathological-gambling-and-epilepsy-in-patients-with-frontotemporal-dementia-two-case-reports
#12
Sheila Castro-Suarez, Peggy Martinez, María Meza
Frontotemporal dementia is a neurodegenerative disorder of which the behavioral variant is most common. This condition is currently considered the most common cause of dementia in people younger than 60 years. Here, we present two unrelated cases in which the typical symptoms were cognitive and behavioral progressive deterioration and psychiatric disorders such as disinhibition, impulsive acts, apathy, lack of empathy, stereotypies, and changes in eating habits. The first case exhibited pathological gambling as the initial symptom and resided in a psychiatric facility for a year...
July 2016: Revista Peruana de Medicina Experimental y Salud Pública
https://www.readbyqxmd.com/read/27741293/transient-global-amnesia-deteriorates-the-network-efficiency-of-the-theta-band
#13
Young Ho Park, Jeong-Youn Kim, SangHak Yi, Jae-Sung Lim, Jae-Won Jang, Chang-Hwan Im, SangYun Kim
Acute perturbation of the hippocampus, one of the connector hubs in the brain, is a key step in the pathophysiological cascade of transient global amnesia (TGA). We tested the hypothesis that network efficiency, meaning the efficiency of information exchange over a network, is impaired during the acute stage of TGA. Graph theoretical analysis was applied to resting-state EEG data collected from 21 patients with TGA. The EEG data were obtained twice, once during the acute stage (< 24 hours after symptom onset) and once during the resolved stage (> 2 months after symptom onset) of TGA...
2016: PloS One
https://www.readbyqxmd.com/read/27380626/identifying-specific-clinical-symptoms-of-behavioral-variant-frontotemporal-dementia-versus-differential-psychiatric-disorders-in-patients-presenting-with-a-late-onset-frontal-lobe-syndrome
#14
Annemiek Dols, Saskia van Liempt, Flora Gossink, Welmoed A Krudop, Sietske Sikkes, Yolande A L Pijnenburg, Max L Stek
OBJECTIVE: Early differentiation between psychiatric disorders and behavioral variant frontotemporal dementia (bvFTD) is of paramount importance in patients with the late-onset frontal lobe syndrome. As bvFTD in patients will deteriorate, psychiatric disorders are treatable. To date, misdiagnosis often occurs due to an overlap of symptoms and lack of specific biomarkers. The aim of our study was to investigate whether specific symptoms could separate bvFTD from psychiatric disorders. METHODS: In a naturalistic, prospective, multicenter study, 137 patients (aged 45-75 years, 72% male) with a late-onset frontal lobe syndrome were included based on their scores on the Frontal Behavioral Inventory (FBI) and the Stereotypy Rating Inventory (SRI) from April 2011 to June 2013...
October 2016: Journal of Clinical Psychiatry
https://www.readbyqxmd.com/read/27367173/preservation-of-cognitive-and-musical-abilities-of-a-musician-following-surgery-for-chronic-drug-resistant-temporal-lobe-epilepsy-a-case-report
#15
Shantala Hegde, Rose Dawn Bharath, Malla Bhaskara Rao, Karthik Shiva, Arivazhagan Arimappamagan, Sanjib Sinha, Jamuna Rajeswaran, Parthasarathy Satishchandra
Mesial temporal lobe epilepsy (TLE) affects a range of cognitive functions and musical abilities. We report a 16-year-old boy diagnosed with drug-resistant right-medial TLE. He is a professional musician, trained in Carnatic classical music. Clinical, electrophysiological, magnetic resonance imaging (MRI) and positron emission tomography evaluation localized the seizure focus to the right medial temporal lobe. Patient underwent detailed neuropsychological evaluation and functional MRI (fMRI) for musical abilities prior to surgery...
December 2016: Neurocase
https://www.readbyqxmd.com/read/27258418/uncovering-the-neural-bases-of-cognitive-and-affective-empathy-deficits-in%C3%A2-alzheimer-s-disease-and-the-behavioral-variant-of-frontotemporal-dementia
#16
Nadene Dermody, Stephanie Wong, Rebekah Ahmed, Olivier Piguet, John R Hodges, Muireann Irish
Loss of empathy is a core presenting feature of the behavioral-variant of frontotemporal dementia (bvFTD), resulting in socioemotional difficulties and behavioral transgressions. In contrast, interpersonal functioning remains relatively intact in Alzheimer's disease (AD), despite marked cognitive decline. The neural substrates mediating these patterns of loss and sparing in social functioning remain unclear, yet are relevant for our understanding of the social brain. We investigated cognitive versus affective aspects of empathy using the Interpersonal Reactivity Index (IRI) in 25 AD and 24 bvFTD patients and contrasted their performance with 22 age- and education-matched controls...
May 30, 2016: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27190928/behavioural-variant-frontotemporal-dementia-with-bilateral-insular-hypometabolism-a-case-report
#17
Ananya Mahapatra, Mamta Sood, Roshan Bhad, Manjari Tripathi
Fronto-Temporal Dementia (FTD) is a cluster of syndromes, characterized by progressive deterioration of cognition, language and/or behavioural changes associated with degeneration of the frontal and temporal lobes. A 53-year-old man was admitted with a history of gradually progressive behavioural disturbances, disinhibition, unprovoked anger outbursts, apathy, disorganised behaviour and impaired self-care. A clinical diagnosis of Fronto temporal Dementia (behavioural variant) was made. Extensive investigations found no abnormality except in FDG-PET scan of the brain which revealed hypo metabolism in bilateral anterior insular region...
April 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27134676/use-of-interleukin-2-for-management-of-natalizumab-associated-progressive-multifocal-leukoencephalopathy-case-report-and-review-of-literature
#18
REVIEW
Divyanshu Dubey, Yinan Zhang, Donna Graves, Allen D DeSena, Elliot Frohman, Benjamin Greenberg
A 51-year-old woman with relapsing-remitting multiple sclerosis (RRMS) and 3-year history of natalizumab use developed expressive aphasia. A brain magnetic resonance image (MRI) showed left frontotemporal and right parietal lesion with mild contrast enhancement and cerebrospinal fluid (CSF) was positive for John Cunningham virus (JCV) by polymerase chain reaction (PCR). The patient received five cycles of plasmapheresis followed by intravenous immunoglobulin. Despite this intervention, her speech deteriorated and she developed right hemiparesis...
May 2016: Therapeutic Advances in Neurological Disorders
https://www.readbyqxmd.com/read/27104894/dementia-and-rapid-mortality-who-is-at-risk
#19
Salka S Staekenborg, Yolande A L Pijnenburg, Afina W Lemstra, Philip Scheltens, Wiesje M Vd Flier
BACKGROUND: Dementia is typically known for its insidious onset and slowly progressive course, but a subgroup deteriorates fast and dies within years or even months. OBJECTIVE: The purpose of this study was to characterize dementia patients with a rapidly progressive course to death and evaluate their cause of death. METHODS: We retrospectively included all patients from the Amsterdam Dementia Cohort who died within two years after diagnosis...
April 19, 2016: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27042904/frontotemporal-dementias
#20
REVIEW
Elizabeth C Finger
PURPOSE OF REVIEW: This article reviews the common behavioral and cognitive features of frontotemporal dementia (FTD) and related disorders as well as the distinguishing clinical, genetic, and pathologic features of the most common subtypes. RECENT FINDINGS: Advances in clinical phenotyping, genetics, and biomarkers have enabled improved predictions of the specific underlying molecular pathology associated with different presentations of FTD. Evaluation of large international cohorts has led to recent refinements in diagnostic criteria for several of the FTD subtypes...
April 2016: Continuum: Lifelong Learning in Neurology
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