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Rapid progressive dementia

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https://www.readbyqxmd.com/read/29125945/periodic-eeg-patterns-in-sporadic-creutzfeld-jakob-disease-can-be-benzodiazepine-responsive-and-be-difficult-to-distinguish-from-non-convulsive-status-epilepticus
#1
Justus Marquetand, Susanne Knake, Adam Strzelczyk, Bernhard J Steinhoff, Holger Lerche, Matthis Synofzik, Niels K Focke
PURPOSE: Periodic discharges in EEG and a history of rapidly progressive dementia are known to be associated with sporadic Creutzfeld-Jakob Disease (sCJD). Doubts regarding this rare but fatal diagnosis can arise, when episodic symptoms (seizures, psychiatric features, speech disturbances) are present and the EEG shows epileptiform discharges within this periodicity. This scenario may indicate non-convulsive status epilepticus (NCSE), which is - in contrast to sCJD - a treatable and frequent condition...
November 4, 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/29123489/plasmapheresis-responsive-rapid-onset-dementia-with-predominantly-frontal-dysfunction-in-the-context-of-hashimoto-s-encephalopathy
#2
Dominique Endres, Magnus S Vry, Petra Dykierek, Anne N Riering, Eva Lüngen, Oliver Stich, Rick Dersch, Nils Venhoff, Daniel Erny, Irina Mader, Philipp T Meyer, Ludger Tebartz van Elst
Background: Hashimoto's encephalopathy (HE) is a rare immunological neuropsychiatric disorder characterized by increased antithyroid antibodies and mixed neurological and psychiatric symptoms. HE has been previously discussed as a differential diagnosis for rapid progressive dementia. However, most of these patients suffered from additional neurological symptoms, like ataxia or seizures. Case presentation: Here, we present the case of a 59-year-old female patient suffering rapid onset dementia with salient frontal executive dysfunction...
2017: Frontiers in Psychiatry
https://www.readbyqxmd.com/read/29111000/natural-history-and-predictors-of-survival-in-progressive-supranuclear-palsy
#3
Maura Cosseddu, Alberto Benussi, Stefano Gazzina, Marta A Manes, Valentina Dell'Era, Viviana Cristillo, Rosanna Turrone, Antonella Alberici, Barbara Borroni
BACKGROUND: Progressive supranuclear palsy is a neurodegenerative disorder characterized by high functional disability and rapidly progressive dependency. The predictors of survival are still unclear. METHODS: The predictors of survival were evaluated in a group of clinically diagnosed PSP patients, focusing primarily on extensive cognitive assessment. RESULTS: The mean survival time from symptom onset was 8.25±3.0years. Sex, age at onset, education, occupation and severity of extrapyramidal symptoms did not correlate with survival...
November 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/29103041/association-of-the-new-variant-tyr424asp-at-tbk1-gene-with-amyotrophic-lateral-sclerosis-and-cognitive-decline
#4
Irene Piaceri, Valentina Bessi, Sabrina Matà, Cristina Polito, Andrea Tedde, Valentina Berti, Silvia Bagnoli, Arianna Braccia, Monica Del Mastio, Alberto Moggi Pignone, Alberto Pupi, Sandro Sorbi, Benedetta Nacmias
A new risk gene associated with amyotrophic lateral sclerosis (ALS) has recently been identified: the Tank-binding kinase 1 (TBK1) gene. Up to now, 90 TBK1 variants have been described in ALS patients with or without frontotemporal dementia (FTD), thus making TBK1 the third or fourth most frequent genetic cause of ALS and FTD. A point mutation analysis in a cohort of 69 Italian ALS patients was performed in order to analyze the frequency of TBK1 mutations and the correlation with clinical phenotypes. The analysis identified the novel variant p...
October 30, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29097599/brains-with-sporadic-creutzfeldt-jakob-disease-and-copathology-showed-a-prolonged-end-stage-of-disease
#5
Aitzol Miguelez-Rodriguez, Jorge Santos-Juanes, Ikerne Vicente-Etxenausia, Katty Perez de Heredia-Goñi, Beatriz Garcia, Luis M Quiros, Laura Lorente-Gea, Isabel Guerra-Merino, Jose J Aguirre, Ivan Fernandez-Vega
AIMS: To investigate the expression of major proteins related to primary neurodegenerative diseases and their prognostic significance in brains with Creutzfeldt-Jakob disease (CJD). MATERIALS AND METHODS: Thirty consecutive cases of confirmed CJD during the period 2010-2015 at Basque Brain bank were retrospectively reviewed. Moreover, major neurodegenerative-associated proteins (phosphorylated Tau, 4R tau, 3R tau, alpha-synuclein, TDP43, amyloid beta) were tested...
November 2, 2017: Journal of Clinical Pathology
https://www.readbyqxmd.com/read/29095328/c9orf72-intermediate-repeat-expansion-in-a-patient-with-psychiatric-disorders-and-progressive-cerebellar-ataxia
#6
Mario Meloni, Rita Farris, Paolo Solla, Marcello M Mascia, Francesco Marrosu, Antonino Cannas
INTRODUCTION: Large expansions of the noncoding GGGGCC repeat (more than 30) in the first intron of the C9ORF72 gene have been demonstrated to cause amyotrophic lateral sclerosis and frontotemporal dementia. Recent papers have investigated the possible pathogenic role and associated clinical phenotypes of hexanucleotide expansions with intermediate repeat lengths ranging between 20 and 29 repeats. CASE REPORT: We report a case of a 71-year-old Sardinian female patient with a long history of psychiatric disorders such as mixed anxiety-depressive disorder associated with somatization disorder and histrionic personality who developed a slowly progressive cerebellar syndrome, mild cognitive impairment, pyramidal signs, and rapid eye movement sleep behavior disorder with imaging abnormalities on the DaTSCAN single-photon emission computed tomography indicating an alteration in the presynaptic dopaminergic system...
November 2017: Neurologist
https://www.readbyqxmd.com/read/29095104/pathological-examination-is-required-for-the-case-of-rapidly-progressive-dementia-with-only-positive-result-of-rt-quic-assay
#7
Yuichi Hayashi
No abstract text is available yet for this article.
November 2, 2017: Prion
https://www.readbyqxmd.com/read/29082576/longitudinal-cortical-thinning-and-cognitive-decline-in-patients-with-early-versus-late-stage-subcortical-vascular-mci
#8
Juyoun Lee, Sang Won Seo, Jin-Ju Yang, Young Kyoung Jang, Jin San Lee, Yeo Jin Kim, Juhee Chin, Jong Min Lee, Sung Tae Kim, Kyung-Han Lee, Jae Hong Lee, Jae Seung Kim, Seonwoo Kim, Heejin Yoo, Ae Young Lee, Duk L Na, Hee Jin Kim
BACKGROUND AND PURPOSE: Biomarker changes in cognitively impaired patients with small vessel disease is largely unknown. We evaluated the rate of amyloid/lacune progression, cortical thinning, and cognitive decline in subcortical vascular mild cognitive impairment (svMCI) patients. METHODS: Seventy two svMCI patients were divided into early stage (ES-svMCI, n=39) and late stage (LS-svMCI, n=33) according to their clinical dementia rating sum of box score. Patients were annually followed-up with neuropsychological tests and brain MRI for three years, and underwent a second [(11) C] Pittsburgh compound B (PiB)-PET within a mean interval of 32...
October 30, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/29080930/dysfunction-of-gabaergic-neurons-in-the-parafacial-zone-mediates-sleep-disturbances-in-a-streptozotocin-induced-rat-model-of-sporadic-alzheimer-s-disease
#9
Jin-Zhi Song, Su-Ying Cui, Xiang-Yu Cui, Xiao Hu, Yu-Nu Ma, Hui Ding, Hui Ye, Yong-He Zhang
Sleep disturbances are prevalent among patients with Alzheimer's disease (AD) and often precede the onset and progression of dementia. However, there are no reliable animal models for investigating sleep disturbances in patients with sporadic AD (sAD), which accounts for more than 90% of all AD cases. In the present study, we characterize the sleep/wake cycles and explore a potential mechanism underlying sleep disturbance in a rat model of sAD induced via intracerebroventricular (icv) injection of streptozotocin (STZ)...
October 28, 2017: Metabolic Brain Disease
https://www.readbyqxmd.com/read/29077801/introduction-to-the-special-issue-on-clinical-neuropsychology-of-movement-disorders
#10
Alexander I Tröster
The special issue on the clinical neuropsychology of movement disorders provides an overview for the non-subspecialist clinical neuropsychologist and other clinical neuroscientists of the neuropsychological features, assessment and treatment of Parkinson's disease and Lewy body dementias, atypical parkinsonian disorders (corticobasal syndrome, progressive supranuclear palsy, and multiple system atrophy), Huntington's disease, dystonia, and amyotrophic lateral sclerosis. Additionally, articles provide overviews of neuropsychological and ethical issues related to deep brain stimulation and a discussion of non-pharamcologic and non-invasive treatment of cognitive dysfunction in Parkinson's disease...
November 1, 2017: Archives of Clinical Neuropsychology: the Official Journal of the National Academy of Neuropsychologists
https://www.readbyqxmd.com/read/29067342/the-effects-of-noncoding-aquaporin-4-single-nucleotide-polymorphisms-on-cognition-and-functional-progression-of-alzheimer-s-disease
#11
Kevin G Burfeind, Charles F Murchison, Shawn K Westaway, Matthew J Simon, Deniz Erten-Lyons, Jeffrey A Kaye, Joseph F Quinn, Jeffrey J Iliff
INTRODUCTION: The glymphatic system is a brain-wide perivascular network that facilitates clearance of proteins, including amyloid β, from the brain interstitium through the perivascular exchange of cerebrospinal fluid and interstitial fluid. The astrocytic water channel aquaporin-4 (AQP4) is required for glymphatic system function, and impairment of glymphatic function in the aging brain is associated with altered AQP4 expression and localization. In human cortical tissue, alterations in AQP4 expression and localization are associated with Alzheimer's disease (AD) status and pathology...
September 2017: Alzheimer's & Dementia: Translational Research & Clinical Interventions
https://www.readbyqxmd.com/read/29053115/-syndrome-of-rapid-eye-movement-sleep-behavior-disorder-and-nocturia-in-parkinson-s-disease
#12
M R Nodel, Yu V Ukraintseva, N N Yakhno
AIM: Parasomnia, a syndrome of rapid eye movement sleep behavior disorder (RBD), is a common non-motor impairment in patients with Parkinson's disease (PD). The relationship between RBD with other symptoms of PD affecting night sleep, in particular, nocturia, is understudied. An aim of the study was to determine the symptoms related to night sleep disturbances in PD patients with RBD and assess the dynamics of these disturbances with the disease progression taking into account RBD onset...
2017: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
https://www.readbyqxmd.com/read/29051562/motifs-in-the-tau-protein-that-control-binding-to-microtubules-and-aggregation-determine-pathological-effects
#13
Aurélien Lathuilière, Pamela Valdés, Stéphanie Papin, Matthias Cacquevel, Catherine Maclachlan, Graham W Knott, Andreas Muhs, Paolo Paganetti, Bernard L Schneider
Tau pathology is associated with cognitive decline in Alzheimer's disease, and missense tau mutations cause frontotemporal dementia. Hyperphosphorylation and misfolding of tau are considered critical steps leading to tauopathies. Here, we determine how motifs controlling conformational changes in the microtubule-binding domain determine tau pathology in vivo. Human tau was overexpressed in the adult mouse forebrain to compare variants carrying residues that modulate tau propensity to acquire a β-sheet conformation...
October 19, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29038245/mitochondrial-calcium-dysregulation-contributes-to-dendrite-degeneration-mediated-by-pd-lbd-associated-lrrk2-mutants
#14
Manish Verma, Jason Callio, P Anthony Otero, Israel Sekler, Zachary P Wills, Charleen T Chu
Mutations in leucine-rich repeat kinase 2 (LRRK2) contribute to development of late-onset familial Parkinson's disease (PD), with clinical features of motor and cognitive dysfunction indistinguishable from sporadic PD. Calcium dysregulation plays an important role in PD pathogenesis, but the mechanisms of neurodegeneration remain unclear. Recent reports indicate enhanced excitatory neurotransmission in cortical neurons expressing mutant LRRK2, which occurs prior to the well-characterized phenotype of dendritic shortening...
October 16, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/29023987/prevalence-of-delusions-in-drug-na%C3%A3-ve-alzheimer-disease-patients-a-meta-analysis
#15
Leo Lai, Philip E Lee, Peter Chan, Mark C Fok, Ging-Yuek R Hsiung, Amir A Sepehry
INTRODUCTION: Neuropsychiatric symptoms (NPS) are common at all stages of Alzheimer disease (AD). Delusions in AD are associated with negative clinical consequences and may signal rapid disease progression. Hence, we sought to determine the prevalence of delusions in drug-naïve (no cholinesterase inhibitor or neuroleptic medications) AD patients. METHODS: In this meta-analysis, a search of the EMBASE, MEDLINE, and PsycINFO databases was performed. We selected studies reporting delusion prevalence measured by the Neuropsychiatric Inventory (NPI) in drug-naïve AD patients...
October 11, 2017: International Journal of Geriatric Psychiatry
https://www.readbyqxmd.com/read/28993533/clinical-reasoning-a-55-year-old-man-with-rapidly-progressive-dementia-and-parkinsonism
#16
Miguel Tábuas-Pereira, Isabel Santana, Gustavo Cordeiro Santo
No abstract text is available yet for this article.
October 10, 2017: Neurology
https://www.readbyqxmd.com/read/28993004/cerebral-amyloid-angiopathy-related-cognitive-impairment-the-search-for-a-specific-neuropsychological-pattern
#17
REVIEW
M Planton, N Raposo, J-F Albucher, J Pariente
Cerebral amyloid angiopathy is diagnosed in stroke units after lobar intracerebral hemorrhage. CAA can also be diagnosed in memory clinics when patients are referred for cognitive impairment assessment, and may be a reason for admission to emergency or neurology departments because of rapidly progressive cognitive or neurological decline, or a transient focal neurological episode. CAA may even be observed in older community-dwelling individuals. Neuropsychological impairment in CAA has been described over the past 20 years...
November 2017: Revue Neurologique
https://www.readbyqxmd.com/read/28992835/a-patient-with-rapidly-progressing-early-onset-dementia-and-insomnia
#18
Klark Turpen, Amy Thornbury, Mary Wagner, Richard B Berry, Johan Barretto, Ruby Williams, Scott Ryals
No abstract text is available yet for this article.
November 15, 2017: Journal of Clinical Sleep Medicine: JCSM: Official Publication of the American Academy of Sleep Medicine
https://www.readbyqxmd.com/read/28982489/palliative-care-in-neurology
#19
REVIEW
Maisha T Robinson, Robert G Holloway
Palliative medicine is a specialty that focuses on improving the quality of life for patients with serious or advanced medical conditions, and it is appropriate at any stage of disease, including at the time of diagnosis. Neurologic conditions tend to have high symptom burdens, variable disease courses, and poor prognoses that affect not only patients but also their families and caregivers. Patients with a variety of neurologic conditions such as Parkinson disease, dementia, amyotrophic lateral sclerosis, brain tumors, stroke, and acute neurologic illnesses have substantial unmet needs that can be addressed through a combination of primary and specialty palliative care...
October 2017: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/28965295/comprehensive-and-methodical-diagnostic-and-management-approaches-to-rapidly-progressive-dementia
#20
REVIEW
Supriya Mahajan, Brian S Appleby
Purpose of review The sudden emergence of a change in cognitive abilities or behavior is an important symptom that warrants medical evaluation and may represent the early stages of a rapidly progressive dementia (RPD). To correctly ascertain the cause of RPD in a given patient, the clinician must be methodical and knowledgeable about the range of potential causes and must move forward with supportive treatment, and in some cases empiric treatment, based on clinical features alone. Recent findings Significant advances in prion disease biomarkers, the molecular features of rapidly progressive Alzheimer's disease, and new detection of autoimmune limbic encephalitis disease entities have caused a shift in the diagnostic and treatment framework of RPD...
September 30, 2017: Current Treatment Options in Neurology
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