scholarly journals Fatal insomnia: the elusive prion disease

2021 ◽  
Vol 14 (6) ◽  
pp. e241289
Author(s):  
Dharmini Patel ◽  
Hagar Ibrahim ◽  
Julia Rankin ◽  
David Hilton ◽  
Marcelo A Barria ◽  
...  
Keyword(s):  
Prion Protein ◽  
Prion Disease ◽  
Short Term Memory ◽  
Brain Biopsy ◽  
Best Supportive Care ◽  
Protein Markers ◽  
Short History ◽  
Short Term ◽  
History Of ◽  
Ocular Convergence

A previously well 54- year-old woman presented with a short history of diplopia, cognitive decline, hallucinations and hypersomnolence. The patient had progressive deterioration in short-term memory, ocular convergence spasm, tremor, myoclonus, gait apraxia, central fever, dream enactment and seizures. Results of investigations were normal including MRI brain, electroencephalogram, cerebrospinal fluid (CSF, including CSF prion protein markers) and brain biopsy. The patient died from pneumonia and pulmonary embolus. Brain postmortem analysis revealed neuropathological changes in keeping with Fatal familial insomnia (FFI); the diagnosis was confirmed on genetic testing. FFI is caused by an autosomal dominant and highly penetrant pathogenic Prion Protein gene PRNP. Although usually familial, fatal insomnia (FI) also occurs in a rare sporadic form. FI is a rare human prion disease with prominent sleep disturbance, autonomic, motor, cognitive and behavioural involvement. Patient management is with best supportive care and early suspected diagnosis allows for timely palliation.

Clinical diagnosis of LGI1 antibody encephalitis in an 83-year-old woman

2021 ◽  
Vol 14 (1) ◽  
pp. e237398
Author(s):  
Jonathan E Attwood ◽  
Saniya Naseer ◽  
Sophia Michael ◽  
Josie Riley
Keyword(s):  
Cognitive Impairment ◽  
Clinical Diagnosis ◽  
Memory Impairment ◽  
Short Term Memory ◽  
Autoimmune Encephalitis ◽  
Short Term ◽  
Term Memory ◽  
Blood Tests ◽  
Abnormal Movements ◽  
History Of

An 83-year-old woman was referred to hospital with a 2-week history of short-lived episodic unpleasant sensations in her head and running down her body. This was accompanied by new short-term memory impairment and arm spasms. Initial investigations including blood tests and brain imaging did not reveal the diagnosis. The patient developed an increasing frequency of abnormal movements of her face and arm. These were clinically recognised as faciobrachial dystonic seizures (FBDS). FBDS are pathognomonic of an autoimmune encephalitis caused by an antibody directed against leucine-rich glioma-inactivated 1 (LGI1). The clinical diagnosis resulted in treatment with immunotherapy, leading to cessation of seizures and rapid cognitive recovery. Later, the predicted serology was confirmed. This reversible and under-recognised cause of cognitive impairment, typically affecting elderly patients, can be diagnosed clinically to enable early and effective treatment.

scholarly journals Stacked Ensemble of Recurrent Neural Networks for Predicting Turbocharger Remaining Useful Life

2019 ◽  
Vol 10 (1) ◽  
pp. 69 ◽  
Author(s):  
Peyman Sheikholharam Mashhadi ◽  
Sławomir Nowaczyk ◽  
Sepideh Pashami
Keyword(s):  
Short Term Memory ◽  
Remaining Useful Life ◽  
Short Term ◽  
Diagnostic Models ◽  
History Of ◽  
Useful Life ◽  
Long Short Term Memory ◽  
Low Performance ◽  
Limited Sensing

Predictive Maintenance (PM) is a proactive maintenance strategy that tries to minimize a system’s downtime by predicting failures before they happen. It uses data from sensors to measure the component’s state of health and make forecasts about its future degradation. However, existing PM methods typically focus on individual measurements. While it is natural to assume that a history of measurements carries more information than a single one. This paper aims at incorporating such information into PM models. In practice, especially in the automotive domain, diagnostic models have low performance, due to a large amount of noise in the data and limited sensing capability. To address this issue, this paper proposes to use a specific type of ensemble learning known as Stacked Ensemble. The idea is to aggregate predictions of multiple models—consisting of Long Short-Term Memory (LSTM) and Convolutional-LSTM—via a meta model, in order to boost performance. Stacked Ensemble model performs well when its base models are as diverse as possible. To this end, each such model is trained using a specific combination of the following three aspects: feature subsets, past dependency horizon, and model architectures. Experimental results demonstrate benefits of the proposed approach on a case study of heavy-duty truck turbochargers.

Lexical Capture: A Developmental Disorder of Reading and Spelling

1989 ◽  
Vol 41 (3) ◽  
pp. 471-487 ◽  
Author(s):  
Elaine Funnell ◽  
Margaret Davison
Keyword(s):  
Developmental Disorder ◽  
Short Term Memory ◽  
Learning Difficulties ◽  
Current Evidence ◽  
Short Term ◽  
Impaired Performance ◽  
International Phonetic Alphabet ◽  
History Of ◽  
Specific Difficulty ◽  
Reading And Spelling

We report a case of a 35-year-old teacher, Louise, with a history of learning difficulties and current evidence of developmental phonological dyslexia and dysgraphia. Her reading, spelling, and remembering of novel stimuli written in conventional alphabetic script was poor, but she performed significantly better when the same items were written in the International Phonetic Alphabet, a system that she learned when studying linguistics. Her impaired performance in tasks of phonemic segmentation and short-term memory, which are generally associated with impaired reading and spelling of unfamiliar material, could not account for her specific difficulty with alphabetic stimuli. Instead, her problems appear to result from a lexical strategy, which we have called “lexical capture”.

scholarly journals Interaction Histories and Short-Term Memory: Enactive Development of Turn-Taking Behaviours in a Childlike Humanoid Robot

Philosophies ◽  
2019 ◽  
Vol 4 (2) ◽  
pp. 26 ◽  
Author(s):  
Frank Broz ◽  
Chrystopher L. Nehaniv ◽  
Hatice Kose ◽  
Kerstin Dautenhahn
Keyword(s):  
Humanoid Robot ◽  
Short Term Memory ◽  
Social Feedback ◽  
Short Term ◽  
Term Memory ◽  
Human Partner ◽  
Turn Taking ◽  
History Of ◽  
Immediate Experience

In this article, an enactive architecture is described that allows a humanoid robot to learn to compose simple actions into turn-taking behaviours while playing interaction games with a human partner. The robot’s action choices are reinforced by social feedback from the human in the form of visual attention and measures of behavioural synchronisation. We demonstrate that the system can acquire and switch between behaviours learned through interaction based on social feedback from the human partner. The role of reinforcement based on a short-term memory of the interaction was experimentally investigated. Results indicate that feedback based only on the immediate experience was insufficient to learn longer, more complex turn-taking behaviours. Therefore, some history of the interaction must be considered in the acquisition of turn-taking, which can be efficiently handled through the use of short-term memory.

Usability Testing of a mHealth System for Swallowing Therapy in Patients Following Stroke

2021 ◽  
pp. 1-7
Author(s):  
Georgina Papadopoulos-Nydam ◽  
Jana Maureen Rieger ◽  
Gabriela Constantinescu
Keyword(s):  
User Satisfaction ◽  
Short Term Memory ◽  
Usability Testing ◽  
Testing Time ◽  
System Efficiency ◽  
Short Term ◽  
Swallowing Therapy ◽  
The Family ◽  
History Of ◽  
System Effectiveness

Purpose The objective of this study was to evaluate the usability of a mobile health (mHealth) system designed for dysphagia exercise in persons with a history of stroke. Method Five participants with a history of stroke were recruited from a tertiary health center and assessed for their ability to use and interact with the system. After being introduced to the technology, participants were asked to independently complete five tasks, one at a time. Assistance was available when required or requested. Usability was evaluated with respect to effectiveness, efficiency, and user satisfaction when completing the prespecified goals. Results Four men and one woman between the ages of 50 and 83 years ( M = 65.4) completed the usability testing. Time from stroke onset varied from 1 month to 2.5 years. Additional poststroke challenges related to the usability of the mHealth system included reduced range of motion or mobility, vision, and short-term memory difficulties. Independent success (system effectiveness) varied in this user subgroup, and the research clinician or the family member was required to adjust the level and type of support they provided (system efficiency). All participants reported satisfaction with the use of the system. Conclusion Usability of and satisfaction with this mHealth system and others like it can be achieved in individuals who have had a stroke, either as an independent user or as a patient–caregiver dyad.

Abstract MP085: Cognitive Impairment Mediates the Impact of Short Sleep Duration on Mortality in Individuals with Cardiovascular or Cerebrovascular Disease

Circulation ◽  
2017 ◽  
Vol 135 (suppl_1) ◽  
Author(s):  
Julio Fernandez-Mendoza ◽  
Fan He ◽  
Alexandros N Vgontzas ◽  
Duanping Liao ◽  
Edward O Bixler
Keyword(s):  
Cognitive Impairment ◽  
Sleep Duration ◽  
Processing Speed ◽  
Short Term Memory ◽  
Executive Attention ◽  
Short Term ◽  
Short Sleep Duration ◽  
Short Sleep ◽  
History Of ◽  
The Impact

Background: Cardiovascular disease (CVD) and cerebrovascular disease (CBV) have been associated with short sleep duration and mortality. Furthermore, short sleep duration has been associated with impaired cognition. Most studies have been limited by using self-report measures and treating sleep duration as a sole, independent predictor, thus, its role in predicting mortality is still not well-established. Hypothesis: We hypothesized that 1) short sleep duration increases the impact of CVD and CBV on mortality and 2) cognitive impairment mediates the association of short sleep duration with mortality in those with CVD or CBV. Methods: We addressed this question in the Penn State Adult Cohort, a random, general population sample of 1,741 men and women (48.7 ± 13.5 years) who were studied in the sleep laboratory and followed-up for 16.7 ± 4.6 years. CVD was defined by a history of heart disease, including hypertension or diabetes, and CBV by a history of stroke. Polysomnographic (PSG) total sleep time was classified as normal (≥ 6 hours) and short (< 6 hours) sleep duration based on the median of the cohort. All individuals underwent a comprehensive neuropsychological evaluation, including Symbol Digit Modalities Test, Trail Making Test, Benton Visual Retention Test, Thurstone Word Fluency Test, and Mini-Mental State Examination. We tested the interaction between CVD, CBV and PSG sleep duration on mortality using Cox proportional hazard models controlling for multiple potential confounders. Results: The hazard ratios (95%CI) of mortality associated with CVD and CBV were 0.9 (0.6-1.3) and 1.3 (0.5-3.1) for individuals with normal sleep duration and 1.8 (1.3-2.5) and 2.4 (1.3-4.4) for individuals with short sleep duration (P-interaction < .05). In individuals with CVD or CBV, short sleep duration was associated with impaired processing speed, executive attention, and short-term memory (all Ps < .05). Cognitive impairment significantly mediated the impact of short sleep duration on mortality in those with CVD or CBV [proportion of mediation effects were 6.5% (1.4%-18.6%), 4.5% (0.4%-14.2%), and 6.2% (1.0%-18.4%) for processing speed, executive attention and short-term memory, respectively]. Conclusions: The risk of mortality associated with CVD and CBV is significantly increased in those with short sleep duration. Although cognitive impairment significantly mediated this association, its modest effect suggests that future studies should examine other underlying mechanisms linking short sleep duration with mortality in individuals with CVD or CBV.

Short-term memory in the in situ canine myocardium

1984 ◽  
Vol 247 (1) ◽  
pp. H8-H16
Author(s):  
O. Nwasokwa ◽  
K. Sagawa ◽  
H. Suga
Keyword(s):  
Short Term Memory ◽  
Isometric Force ◽  
Myocardial Performance ◽  
Short Term ◽  
Force Development ◽  
Length Relationship ◽  
History Of ◽  
Systolic Time ◽  
Length Changes ◽  
Isotonic Shortening

We studied the effect of intracycle (short-term) mechanical history on canine myocardial performance. Intracycle muscle force and/or length history was varied, and the resultant changes in end-systolic force-length relationship were analyzed. Antecedent isotonic shortening impaired, whereas isometric force development enhanced end-systolic myocardial performance. A history of shortening concurrent with force development produced an intermediate effect. We conclude that decreasing force or length impairs whereas increasing length or force enhances performance in the same cycle. Different combinations of antecedent force and length changes affect end-systolic performance by algebraic summation (superposition) of their disparate effects. Time measurements established that 1) total systolic time varied little with altered history, 2) isotonic shortening took longer than isometric contraction in reaching a point P in the force-length plane, and 3) less time was therefore available for contraction after P with antecedent isotonic shortening than with antecedent isometric force development. This history-dependent time differential accounts for the corresponding differential in performance.

scholarly journals Clinical Laboratory Tests Used To Aid in Diagnosis of Human Prion Disease

2019 ◽  
Vol 57 (10) ◽  
Author(s):  
Allyson Connor ◽  
Han Wang ◽  
Brian S. Appleby ◽  
Daniel D. Rhoads
Keyword(s):  
Prion Protein ◽  
Prion Disease ◽  
Laboratory Testing ◽  
Clinical Laboratory ◽  
Prion Diseases ◽  
Neuron Specific Enolase ◽  
Brain Biopsy ◽  
Alpha Synuclein ◽  
Clinical Laboratory Testing ◽  
The Brain

ABSTRACT Prion diseases are a group of rapidly progressive and always fatal neurodegenerative disorders caused by misfolded prion protein in the brain. Although autopsy remains the gold-standard diagnostic tool, antemortem laboratory testing can be performed to aid in the diagnosis of prion disease. This review is meant to help laboratory directors and physicians in their interpretation of test results. Laboratory assays to detect both nonspecific biomarkers of prion disease and prion-specific biomarkers can be used. The levels of nonspecific biomarkers in cerebrospinal fluid (CSF) are elevated when rapid neurodegeneration is occurring in the patient, and these markers include 14-3-3, tau, neuron-specific enolase, S100B, and alpha-synuclein. These markers have various sensitivities and specificities but are overall limited, as the levels of any of these analytes can be elevated in nonprion disease that is causing rapid damage of brain tissue. Prion-specific assays used in clinical laboratory testing are currently limited to two options. The first option is second-generation real-time quaking-induced conversion (RT-QuIC) performed on CSF, and the second option is Western blotting of a brain biopsy specimen used to detect protease-resistant prion protein. Although both tests have exquisite specificity, RT-QuIC has a sensitivity of 92 to 97.2% in symptomatic individuals, compared to the brain biopsy Western blot sensitivity of 20 to 60%. RT-QuIC was added to the Centers for Disease Control and Prevention’s diagnostic criteria for prion disease in 2018. Other caveats of laboratory testing need to be considered, as sporadic, genetic, and acquired forms of prion disease have different clinical and laboratory presentations, and these caveats are discussed. Laboratory testing plays an important role in the diagnosis of prion disease, which is often challenging to diagnose.

scholarly journals Compulsory psychiatric admission

2000 ◽  
Vol 24 (2) ◽  
pp. 66-68 ◽  
Author(s):  
T. van Lysebetten ◽  
P. Igodt
Keyword(s):  
Hip Replacement ◽  
Short Term Memory ◽  
Psychiatric Treatment ◽  
Heavy Drinking ◽  
Personal Hygiene ◽  
Short Term ◽  
Psychiatric Admission ◽  
Accident And Emergency ◽  
History Of ◽  
Accident And Emergency Department

Y is a 55-year-old woman who was brought to the accident and emergency department, following an epileptic fit on the street. She had a known history of severe alcohol dependency and epilepsy. She had numerous past admissions, which were always terminated prematurely by her. She has not been adhering to the anti-epileptic treatment. Five days prior to admission her daughter found her in a grossly self-neglected state. There was evidence of recent heavy drinking and she had been incontinent of urine and faeces. Despite this the daughter had not requested help. At interview Y presented as a small unkempt lady, seemingly undernourished and frail. She had recently undergone a hip replacement and was found to have an infected suture wound on physical examination. There were no signs of acute confusion or intoxication. She was neither depressed, nor deluded. There were early signs of cognitive decline (short-term memory impairment). She refused voluntary psychiatric treatment and denied her self-neglect, in spite of her bad personal hygiene and infected wound.

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