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1.
J Sci Food Agric ; 104(11): 7013-7017, 2024 Aug 30.
Article in English | MEDLINE | ID: mdl-38497443

ABSTRACT

BACKGROUND: The harvest of fruit can be significantly advanced with the thriving development of intelligent and automated robot technologies. Nevertheless, the picking success rate of tomato fruit still requires improvement as some fruits are unexpectedly damaged inside, which is imperceptible by machine vision. Herein, a modelling method based on modified Voronoi algorithm is proposed to reconstruct the cellular structure of tomato pericarp. RESULTS: Based on the reconstructed micro-model, the compression physical behaviour of the pericarp cells is simulated to observe internal local stress and potential damage. It is revealed that the simulation result for pericarps of tomatoes with different ripeness is highly consistent to the experimental tests, which has well validated the feasibility of this modelling and simulation method. CONCLUSION: A Voronoi-based modelling method is proposed for micro-reconstruction of tomato pericarp, and the corresponding compression simulation results agree well with the experimental tests. Such result can be utilized as reference to improve the grasping force control for harvesting robot to avoid invisible damage induced by accident overload issue. With the predicting result, superior success rate can be achieved to enhance robot performance. © 2024 Society of Chemical Industry.


Subject(s)
Fruit , Robotics , Solanum lycopersicum , Solanum lycopersicum/chemistry , Robotics/instrumentation , Fruit/chemistry , Algorithms , Food Handling/methods , Food Handling/instrumentation
2.
Mitochondrial DNA B Resour ; 6(10): 2865-2866, 2021.
Article in English | MEDLINE | ID: mdl-34532572

ABSTRACT

The present study for the first time describes the complete mitochondrial (mt) genome of Antheraea pernyi Guérin-Méneville 1855 strain Luhong, a genetic lethal mutant exhibiting especially red skin color. The mt genome is 15,563 bp in length that is the smallest among the sequenced A. pernyi inbred strains. This genome displays an identical genomic component and gene order to other six known A. pernyi mt genomes. The mt genome-based phylogenetic analysis clustered Luhong with four strains exhibiting yellow skin color, consistent with the traditional view that all of them belonged to the yellow blood lineage.

3.
Org Biomol Chem ; 18(37): 7425-7430, 2020 09 30.
Article in English | MEDLINE | ID: mdl-32936165

ABSTRACT

An efficient approach to synthesize heteroaromatic 2-amines from one-pot desulfurization/dehydrogenative cyclization of aryl isothiocyanates with ortho-substituted amines in water was developed. This approach tolerated a wide range of functional groups on the aromatic ring, providing a practical and environment-friendly process to synthesize heteroaromatic 2-amines in moderate to excellent yields. A plausible mechanism was proposed and the role of TBAB and Cu2O in the present strategy was suggested with the help of ESI mass spectrometry.

4.
Front Genet ; 9: 327, 2018.
Article in English | MEDLINE | ID: mdl-30279697

ABSTRACT

Objective: To examine cognitive deficits and associated brain activity in fragile X-associated tremor/ataxia syndrome (FXTAS) patients with parkinsonism (FXTp+), in relation to FXTAS patients without parkinsonism (FXTp-), and normal elderly controls (NC). Methods: Retrospective reviews were performed in 65 FXTAS patients who participated in the event-related brain potential (ERP) study and also had either a videotaped neurological examination or a neurological examination for extrapyramidal signs. Parkinsonism was defined as having bradykinesia with at least one of the following: rest tremor, postural instability, hypermyotonia, or rigidity. Eleven FXTp+ patients were identified and compared to 11 matched FXTp- and 11 NC. Main ERP measures included the N400 congruity effect, N400 repetition effect, and the late positive component (LPC) repetition effect. Results: When compared with FXTp- and NC, the FXTp+ group showed more severe deficits in executive function, cued-recall, recognition memory, along with a significantly reduced N400 repetition effect (thought to index semantic processing and verbal learning/memory) which was correlated with poorer verbal memory. Across all patients, FMR1 mRNA levels were inversely correlated with delayed recall on the California Verbal Learning Test (CVLT). Interpretation: The findings of more prominent executive dysfunction and verbal learning/memory deficits in FXTp+ than FXTp- are consistent with findings in Parkinson's disease (PD), and may indicate that concomitant and/or synergistic pathogenetic mechanisms associated with PD play a role in FXTAS. These results have implications not only for understanding the cognitive impairments associated with the parkinsonism subtype of FXTAS, but also for the development of new interventions for these patients.

5.
Neuroreport ; 29(5): 356-362, 2018 03 21.
Article in English | MEDLINE | ID: mdl-29346174

ABSTRACT

In the present study, the time course of tonal and vowel information processing of the spoken words in Mandarin Chinese was investigated using a delayed-response paradigm. Idiomatic materials, providing semantically highly constraining contexts, were utilized. Besides being presented normally, the terminal monosyllabic words in idioms were manipulated with Tonal, Vowel, or Triple violations (i.e. with consonantal, tonal, and vowel mismatches). Event-related potential results showed that all three violations elicited larger widespread negativities in comparison with the Control condition, with the Triple violation effect starting first from 150 ms, then the Vowel violation, and the Tonal violation being the latest. The different starting times of the violation effects suggest that the access of tonal information is slower than that of vowel information, even though the lexical tones are very important in distinguishing the meaning of Chinese words.


Subject(s)
Brain/physiology , Evoked Potentials , Pattern Recognition, Physiological/physiology , Recognition, Psychology/physiology , Speech Perception/physiology , Adult , Electroencephalography , Female , Humans , Male , Phonetics , Pitch Perception/physiology , Young Adult
6.
Neuroimage Clin ; 17: 188-197, 2018.
Article in English | MEDLINE | ID: mdl-29159036

ABSTRACT

Only a subset of mild cognitive impairment (MCI) patients progress to develop a form of dementia. A prominent feature of Alzheimer's disease (AD) is a progressive decline in language. We investigated if subtle anomalies in EEG activity of MCI patients during a word comprehension task could provide insight into the likelihood of conversion to AD. We studied 25 amnestic MCI patients, a subset of whom developed AD within 3-years, and 11 elderly controls. In the task, auditory category descriptions (e.g., 'a type of wood') were followed by a single visual target word either semantically congruent (i.e., oak) or incongruent with the preceding category. We found that the MCI convertors group (i.e. patients that would go on to convert to AD in 3-years) had a diminished early posterior-parietal theta (3-5 Hz) activity induced by first presentation of the target word (i.e., access to lexico-syntactic properties of the word), compared to MCI non-convertors and controls. Moreover, MCI convertors exhibited oscillatory signatures for processing the semantically congruent words that were different from non-convertors and controls. MCI convertors thus showed basic anomalies for lexical and meaning processing. In addition, both MCI groups showed anomalous oscillatory signatures for the verbal learning/memory of repeated words: later alpha suppression (9-11 Hz), which followed first presentation of the target word, was attenuated for the second and third repetition in controls, but not in either MCI group. Our findings suggest that a subtle breakdown in the brain network subserving language comprehension can be foretelling of conversion to AD.


Subject(s)
Alzheimer Disease/physiopathology , Brain Waves , Brain/physiopathology , Cognitive Dysfunction/physiopathology , Disease Progression , Semantics , Acoustic Stimulation , Aged , Aged, 80 and over , Alzheimer Disease/diagnosis , Alzheimer Disease/psychology , Cognitive Dysfunction/psychology , Comprehension/physiology , Electroencephalography , Evoked Potentials , Humans , Middle Aged , Speech Perception/physiology
7.
Sci Rep ; 6: 25916, 2016 05 16.
Article in English | MEDLINE | ID: mdl-27180951

ABSTRACT

Using unimodal auditory tasks of word-matching and meaning-matching, this study investigated how the phonological and semantic processes in Chinese disyllabic spoken word recognition are modulated by top-down mechanism induced by experimental tasks. Both semantic similarity and word-initial phonological similarity between the primes and targets were manipulated. Results showed that at early stage of recognition (~150-250 ms), an enhanced P2 was elicited by the word-initial phonological mismatch in both tasks. In ~300-500 ms, a fronto-central negative component was elicited by word-initial phonological similarities in the word-matching task, while a parietal negativity was elicited by semantically unrelated primes in the meaning-matching task, indicating that both the semantic and phonological processes can be involved in this time window, depending on the task requirements. In the late stage (~500-700 ms), a centro-parietal Late N400 was elicited in both tasks, but with a larger effect in the meaning-matching task than in the word-matching task. This finding suggests that the semantic representation of the spoken words can be activated automatically in the late stage of recognition, even when semantic processing is not required. However, the magnitude of the semantic activation is modulated by task requirements.


Subject(s)
Recognition, Psychology/physiology , Speech Perception/physiology , Adult , Electroencephalography , Evoked Potentials, Auditory , Female , Humans , Male , Reaction Time , Semantics , Young Adult
8.
Sci Rep ; 6: 21719, 2016 Feb 22.
Article in English | MEDLINE | ID: mdl-26898832

ABSTRACT

Progressive cognitive deficits are common in patients with fragile X-associated tremor/ataxia syndrome (FXTAS), with no targeted treatment yet established. In this substudy of the first randomized controlled trial for FXTAS, we examined the effects of NMDA antagonist memantine on attention and working memory. Data were analyzed for patients (24 in each arm) who completed both the primary memantine trial and two EEG recordings (at baseline and follow-up) using an auditory "oddball" task. Results demonstrated significantly improved attention/working memory performance after one year only for the memantine group. The event-related potential P2 amplitude elicited by non-targets was significantly enhanced in the treated group, indicating memantine-associated improvement in attentional processes at the stimulus identification/discrimination level. P2 amplitude increase was positively correlated with improvement on the behavioral measure of attention/working memory during target detection. Analysis also revealed that memantine treatment normalized the P2 habituation effect at the follow-up visit. These findings indicate that memantine may benefit attentional processes that represent fundamental components of executive function/dysfunction, thought to comprise the core cognitive deficit in FXTAS. The results provide evidence of target engagement of memantine, as well as therapeutically relevant information that could further the development of specific cognitive or disease-modifying therapies for FXTAS.


Subject(s)
Ataxia/drug therapy , Attention/drug effects , Cognitive Dysfunction/drug therapy , Evoked Potentials/drug effects , Excitatory Amino Acid Antagonists/therapeutic use , Fragile X Syndrome/drug therapy , Memantine/therapeutic use , Tremor/drug therapy , Aged , Ataxia/complications , Ataxia/physiopathology , Cognitive Dysfunction/complications , Cognitive Dysfunction/physiopathology , Drug Administration Schedule , Electroencephalography , Executive Function/drug effects , Female , Fragile X Syndrome/complications , Fragile X Syndrome/physiopathology , Humans , Male , Memory, Short-Term/drug effects , Middle Aged , Neuropsychological Tests , Prospective Studies , Treatment Outcome , Tremor/complications , Tremor/physiopathology
9.
Neuroreport ; 27(2): 67-72, 2016 Jan 20.
Article in English | MEDLINE | ID: mdl-26619230

ABSTRACT

The present study investigated the effect of lexical competition on the time course of spoken word recognition in Mandarin Chinese using a unimodal auditory priming paradigm. Two kinds of competitive environments were designed. In one session (session 1), only the unrelated and the identical primes were presented before the target words. In the other session (session 2), besides the two conditions in session 1, the target words were also preceded by the cohort primes that have the same initial syllables as the targets. Behavioral results showed an inhibitory effect of the cohort competitors (primes) on target word recognition. The event-related potential results showed that the spoken word recognition processing in the middle and late latency windows is modulated by whether the phonologically related competitors are presented or not. Specifically, preceding activation of the competitors can induce direct competitions between multiple candidate words and lead to increased processing difficulties, primarily at the word disambiguation and selection stage during Mandarin Chinese spoken word recognition. The current study provided both behavioral and electrophysiological evidences for the lexical competition effect among the candidate words during spoken word recognition.


Subject(s)
Cerebral Cortex/physiology , Evoked Potentials, Auditory , Phonetics , Recognition, Psychology/physiology , Speech Perception/physiology , Adult , Electroencephalography , Female , Humans , Male , Time Factors , Young Adult
10.
Neuropsychologia ; 63: 34-42, 2014 Oct.
Article in English | MEDLINE | ID: mdl-25111034

ABSTRACT

BACKGROUND: Fragile X-associated tremor/ataxia syndrome (FXTAS), a neurodegenerative disorder caused by FMR1 gene premutations, typically associated with frontal-subcortical type cognitive impairments. High prevalence (~50%) of superimposed Alzheimer׳s pathology has been reported in FMR1 premutation carriers, and standardized neuropsychological tests have not yielded any robust discriminators between FXTAS and Alzheimer׳s disease (AD) dementia. The similarities/differences in memory processes between FXTAS and early AD remain underexplored. METHODS: 32-channel event-related potentials (ERPs) were obtained from a semantic judgment task in which semantically congruous (50%) and incongruous pairs repeat pseudorandomly. The N400 and late positive component (LPC) of 25 FXTAS patients (M(age)=71.2, MMSE=26.6) were compared to a matched group of 25 patients with MCI or early AD (1 mild AD dementia, 24 amnestic MCI, of whom 18 later converted to AD; M(age)=73.4, MMSE=26.4), and 25 healthy elderly. RESULTS: Both patient groups showed similar reductions in the N400 repetition effect and N400 congruity effect amplitudes, compared to controls, reflecting abnormal semantic priming and repetition priming. The MCI/AD group, however, had significantly smaller LPC word repetition effects and poorer learning and memory on the CVLT than FXTAS. The LPC and N400 repetition effects both correlated with verbal memory across all subjects, but only N400 correlated with memory in FXTAS. CONCLUSION: FXTAS patients show relative sparing of the LPC repetition effect, and less disruption of explicit memory than prodromal/early AD. N400 abnormalities in FXTAS appear to account for much of their mild impairments in verbal learning and memory.


Subject(s)
Ataxia/physiopathology , Cerebral Cortex/physiopathology , Cognitive Dysfunction/physiopathology , Evoked Potentials , Fragile X Syndrome/physiopathology , Memory, Episodic , Repetition Priming , Tremor/physiopathology , Aged , Amnesia/complications , Amnesia/physiopathology , Cognitive Dysfunction/complications , Electroencephalography , Female , Humans , Male , Semantics
11.
J Neurodev Disord ; 6(1): 30, 2014.
Article in English | MEDLINE | ID: mdl-25097672

ABSTRACT

Changes in the fragile X mental retardation 1 gene (FMR1) have been associated with specific phenotypes, most specifically those of fragile X syndrome (FXS), fragile X tremor/ataxia syndrome (FXTAS), and fragile X primary ovarian insufficiency (FXPOI). Evidence of increased risk for additional medical, psychiatric, and cognitive features and conditions is now known to exist for individuals with a premutation, although some features have been more thoroughly studied than others. This review highlights the literature on medical, reproductive, cognitive, and psychiatric features, primarily in females, that have been suggested to be associated with changes in the FMR1 gene. Based on this review, each feature is evaluated with regard to the strength of evidence of association with the premutation. Areas of need for additional focused research and possible intervention strategies are suggested.

12.
J Neurodev Disord ; 6(1): 28, 2014.
Article in English | MEDLINE | ID: mdl-25136377

ABSTRACT

The fragile X-associated tremor/ataxia syndrome (FXTAS) is a late-onset neurodegenerative disorder affecting a subset of carriers of the FMR1 (fragile X mental retardation 1) premutation. Penetrance and expression appear to be significantly higher in males than females. Although the most obvious aspect of the phenotype is the movement disorder that gives FXTAS its name, the disorder is also accompanied by progressive cognitive impairment. In this review, we address the cognitive neuropsychological and neurophysiological phenotype for males and females with FXTAS, and for male and female unaffected carriers. Despite differences in penetrance and expression, the cognitive features of the disorder appear similar for both genders, with impairment of executive functioning, working memory, and information processing the most prominent. Deficits in these functional systems may be largely responsible for impairment on other measures, including tests of general intelligence and declarative learning. FXTAS is to a large extent a white matter disease, and the cognitive phenotypes observed are consistent with what some have described as white matter dementia, in contrast to the impaired cortical functioning more characteristic of Alzheimer's disease and related disorders. Although some degree of impaired executive functioning appears to be ubiquitous among persons with FXTAS, the data suggest that only a subset of unaffected carriers of the premutation - both female and male - demonstrate such deficits, which typically are mild. The best-studied phenotype is that of males with FXTAS. The manifestations of cognitive impairment among asymptomatic male carriers, and among women with and without FXTAS, are less well understood, but have come under increased scrutiny.

13.
Neuropsychologia ; 63: 165-74, 2014 Oct.
Article in English | MEDLINE | ID: mdl-25172388

ABSTRACT

In the present study, two experiments were carried out to investigate the time course of spoken word recognition in Mandarin Chinese using both event-related potentials (ERPs) and behavioral measures. To address the hypothesis that there is an early phonological processing stage independent of semantics during spoken word recognition, a unimodal word-matching paradigm was employed, in which both prime and target words were presented auditorily. Experiment 1 manipulated the phonological relations between disyllabic primes and targets, and found an enhanced P2 (200-270 ms post-target onset) as well as a smaller early N400 to word-initial phonological mismatches over fronto-central scalp sites. Experiment 2 manipulated both phonological and semantic relations between monosyllabic primes and targets, and replicated the phonological mismatch-associated P2, which was not modulated by semantic relations. Overall, these results suggest that P2 is a sensitive electrophysiological index of early phonological processing independent of semantics in Mandarin Chinese spoken word recognition.


Subject(s)
Brain/physiology , Recognition, Psychology/physiology , Speech Perception/physiology , Acoustic Stimulation , Adolescent , Adult , Evoked Potentials, Auditory , Female , Humans , Male , Semantics , Young Adult
14.
Neuropsychopharmacology ; 39(12): 2760-8, 2014 Nov.
Article in English | MEDLINE | ID: mdl-24871547

ABSTRACT

Older FMR1 premutation carriers may develop fragile X-associated tremor/ataxia syndrome (FXTAS), a neurodegenerative disorder manifesting cognitive deficits that often subsequently progress to dementia. To date, there is no specific treatment available for FXTAS. Studies have demonstrated the premutation-associated overactivation of glutamatergic receptors in neurons. Memantine, a NMDA receptor antagonist approved for treatment of Alzheimer's disease, thus was tested in the first placebo-controlled, double-blind, randomized clinical trial in FXTAS. Prior event-related brain potential (ERP) studies in FXTAS found reduced N400 repetition effect, a glutamate-related electrophysiological marker of semantic priming, and verbal memory processes. This substudy of the randomized clinical trial of memantine in FXTAS sought to use the N400 repetition effect to evaluate effects of chronic memantine treatment on verbal memory. Subsequent recall and recognition memory tests for the experimental stimuli were administered to characterize verbal memory. Data from 41 patients who completed the 1-year memantine trial (21 on memantine) and also completed longitudinal ERP studies were analyzed. Results showed treatment-associated benefits on both cued-recall memory and N400 repetition effect amplitude. Importantly, improvement in cued recall was positively correlated with amplitude increase of the N400 repetition effect. The placebo group, in contrast, displayed a significant reduction of the N400 repetition effect after 1 year. These results suggest that memantine treatment may have beneficial effects on verbal memory in FXTAS. Additional studies of memantine, perhaps in combination with other therapeutic agents, appear warranted, as symptomatic treatments and neuroprotective treatments are both needed for this recently recognized neurodegenerative disorder.


Subject(s)
Ataxia/drug therapy , Brain/drug effects , Fragile X Syndrome/drug therapy , Memantine/therapeutic use , Memory/drug effects , Nootropic Agents/therapeutic use , Speech Perception/drug effects , Tremor/drug therapy , Ataxia/physiopathology , Brain/physiopathology , Double-Blind Method , Electroencephalography , Evoked Potentials , Excitatory Amino Acid Antagonists/therapeutic use , Female , Fragile X Syndrome/physiopathology , Humans , Longitudinal Studies , Male , Memory/physiology , Middle Aged , Neuropsychological Tests , Receptors, N-Methyl-D-Aspartate/antagonists & inhibitors , Receptors, N-Methyl-D-Aspartate/metabolism , Speech Perception/physiology , Tremor/physiopathology
15.
Parkinsonism Relat Disord ; 20(4): 456-9, 2014 Apr.
Article in English | MEDLINE | ID: mdl-24491663

ABSTRACT

BACKGROUND: Parkinsonian features have been used as a minor diagnostic criterion for fragile X-associated tremor/ataxia syndrome (FXTAS). However, prior studies have examined parkinsonism (defined as having bradykinesia with at least rest tremor or postural instability) mostly in premutation carriers without a diagnosis of FXTAS. The current study was intended to elaborate this important aspect of the FXTAS spectrum, and to quantify the relationships between parkinsonism, FXTAS clinical staging and genetic/molecular measures. METHODS: Thirty eight (38) FXTAS patients and 10 age-matched normal controls underwent a detailed neurological examination that included all but one item (i.e. rigidity) of the motor section of the Unified Parkinson's Disease Rating Scale (UPDRS). RESULTS: The FXTAS patient group displayed substantially higher prevalence of parkinsonian features including body bradykinesia (57%) and rest tremor (26%), compared to the control group. Furthermore, parkinsonism was identified in 29% of FXTAS patients. Across all patients, body bradykinesia scores significantly correlated with FXTAS clinical stage, FMR1 mRNA level, and ataxic gait of cerebellar origin, while postural instability was associated with intention tremor. INTERPRETATION: Parkinsonian features in FXTAS appear to be characterized as bradykinesia concurrent with cerebellar gait ataxia, postural instability accompanied by intention tremor, and frequent rest tremor, representing distinctive patterns that highlight the need for further clinical studies including genetic testing for the FMR1 premutation. The association between FMR1 mRNA level and bradykinesia implicates pathophysiological mechanisms which may link FMR1 mRNA toxicity, dopamine deficiency and parkinsonism in FXTAS.


Subject(s)
Ataxia/complications , Fragile X Syndrome/complications , Parkinsonian Disorders/etiology , Tremor/complications , Aged , Ataxia/genetics , Female , Fragile X Mental Retardation Protein/genetics , Fragile X Syndrome/genetics , Humans , Male , Middle Aged , Parkinsonian Disorders/epidemiology , Prevalence , RNA, Messenger/analysis , Retrospective Studies , Tremor/genetics , Video Recording
16.
Cogn Neurosci ; 4(3-4): 143-51, 2013.
Article in English | MEDLINE | ID: mdl-24090465

ABSTRACT

We sought cognitive event-related potential (ERP) biomarkers of "Preclinical Alzheimer's disease" (Pre-AD) using an incidental verbal learning paradigm with high sensitivity to prodromal AD. Seven elderly persons, with normal cognition at the time of ERP recordings, but who showed subsequent cognitive decline or AD pathology at autopsy (n = 5, mean Braak stage = 2.8), were compared to 12 "robust" normal elderly (RNE) persons who remained cognitively normal (Mfollow-up = 9.0 years). EEG was recorded during a word repetition paradigm (semantically congruous (50%) and incongruous target words repeat ~10-140 seconds later). The RNE P600 congruous word repetition ERP effects (New minus Old congruous words) were significantly larger than in Pre-AD (mean amplitudes = 3.28 vs. 0.10 µV, p = .04). High group discrimination (84%) was achieved (by a P600 amplitude cutoff of ~1.5 µV). Abnormal P600 word repetition effects in cognitively normal elderly persons may be an important sign of synaptic dysfunction and Preclinical AD.


Subject(s)
Alzheimer Disease/diagnosis , Cognition Disorders/diagnosis , Verbal Learning/physiology , Aged , Alzheimer Disease/physiopathology , Analysis of Variance , Cognition Disorders/physiopathology , Discrimination, Psychological/physiology , Electroencephalography , Evoked Potentials/physiology , Female , Humans , Male , Repetition Priming/physiology , Retrospective Studies , Vocabulary
17.
Ann Neurol ; 74(2): 275-83, 2013 Aug.
Article in English | MEDLINE | ID: mdl-23686745

ABSTRACT

OBJECTIVE: To investigate the nature of cognitive impairments and underlying brain mechanisms in older female fragile X premutation carriers with and without fragile X-associated tremor/ataxia syndrome (FXTAS). METHODS: Extensive neuropsychological testing and cognitive event-related brain potentials (ERPs; particularly, the auditory P300) were examined in 84 female participants: 33 fragile X premutation carriers with FXTAS (mean age = 62.8 years), 25 premutation carriers without FXTAS (mean age = 55.4 years), and 26 normal healthy controls (mean age = 59.3 years). RESULTS: Both premutation groups exhibited executive dysfunction on the Behavioral Dyscontrol Scale, with subtle impairments in inhibition and performance monitoring in female carriers without FXTAS, and more substantial deficits in FXTAS women. However, the female carrier group without FXTAS showed more pronounced deficiencies in working memory. Abnormal ERPs were recorded over the frontal lobes, where FXTAS patients showed both P300 amplitude reduction and latency prolongation, whereas only decreased frontal P300 amplitudes were found in carriers without FXTAS. These frontal P300 measures correlated with executive function and information processing speed. INTERPRETATION: The neuropsychological testing and ERP results of the present study provide support for the hypothesis that executive dysfunction is the primary cognitive impairment among older female premutation carriers both with and without FXTAS, although these deficits are relatively mild compared to those in FXTAS males. These findings are consistent with a synergistic effect of the premutation and aging on cognitive impairment among older female fragile X premutation carriers, even in those without FXTAS symptoms.


Subject(s)
Ataxia/genetics , Fragile X Syndrome/genetics , Frontal Lobe/physiopathology , Heterozygote , Phenotype , Tremor/genetics , Aged , Ataxia/physiopathology , Event-Related Potentials, P300/physiology , Evoked Potentials/physiology , Evoked Potentials, Auditory/genetics , Executive Function/physiology , Female , Fragile X Syndrome/physiopathology , Humans , Middle Aged , Tremor/physiopathology
18.
Cereb Cortex ; 23(11): 2657-66, 2013 Nov.
Article in English | MEDLINE | ID: mdl-22918986

ABSTRACT

Executive dysfunction in fragile X-associated tremor/ataxia syndrome (FXTAS) has been suggested to mediate other cognitive impairments. In the present study, event-related potentials and neuropsychological testing were combined to investigate the brain mechanisms underlying the executive dysfunction in FXTAS. Thirty-two-channel electroencephalography was recorded during an auditory "oddball" task requiring dual responses. FXTAS patients (N= 41, mean age= 62) displayed prolonged latencies of N1 and P3 and reduced amplitudes of P2 and P3, whereas their N2 measures remained within the normal range, indicating relatively preserved early-stage auditory attention but markedly impaired late-stage attention and working memory updating processes (as indexed by P3). Topographical mapping revealed a typical parietal P3 peak preceded by a prominent fronto-central P3 in normal control subjects (N= 32), whereas FXTAS patients had decreased parietal P3 amplitude and diminished fronto-central positivities with a delayed onset (∼50 ms later than controls, P < 0.002). The P3 abnormalities were associated with lower executive function test (e.g., BDS-2) scores. Smaller P3 amplitudes also correlated with increased CGG repeat length of fragile X mental retardation 1 (FMR1) gene and higher FMR1 mRNA levels. These results indicate that abnormal fronto-parietal attentional network dynamics underlie executive dysfunction, the cardinal feature of cognitive impairment in FXTAS.


Subject(s)
Cerebral Cortex/physiopathology , Evoked Potentials, Auditory , Executive Function/physiology , Acoustic Stimulation , Attention/physiology , Cerebellar Diseases/physiopathology , Electroencephalography , Female , Fragile X Mental Retardation Protein/genetics , Fragile X Mental Retardation Protein/metabolism , Humans , Male , Memory, Short-Term/physiology , Middle Aged , Neuropsychological Tests
19.
J Alzheimers Dis ; 26 Suppl 3: 215-28, 2011.
Article in English | MEDLINE | ID: mdl-21971462

ABSTRACT

Cognitive event-related brain potential (ERP) studies of decision-making and attention, language, and memory impairments in Alzheimer's disease (AD) and mild cognitive impairment (MCI) are reviewed. Circumscribed lesions of the medial temporal lobe (MTL), as may be the case in individuals with amnestic MCI, generally produce altered plasticity of the late positive P600 component, with relative sparing of earlier sensory ERP components. However, as the neuropathology of AD extends to neocortical association areas, abnormalities of the P300 and N400 (and perhaps even P50) become more common. Critically, ERP studies of individuals at risk for AD may reveal neurophysiological changes prior to clinical deficits, which could advance the early detection and diagnosis of "presymptomatic AD".


Subject(s)
Alzheimer Disease/complications , Alzheimer Disease/pathology , Cognition Disorders/etiology , Evoked Potentials/physiology , Synapses/pathology , Animals , Brain Mapping , Electroencephalography , Humans , Neuropsychological Tests
20.
Neuropsychologia ; 48(9): 2476-87, 2010 Jul.
Article in English | MEDLINE | ID: mdl-20433856

ABSTRACT

BACKGROUND: We adapted an event-related brain potential word repetition paradigm, sensitive to early Alzheimer's disease (AD), for functional MRI (fMRI). We hypothesized that AD would be associated with reduced differential response to New/Old congruous words. METHODS: Fifteen mild AD patients (mean age=72.9) and 15 normal elderly underwent 1.5T fMRI during a semantic category decision task. RESULTS: We found robust between-groups differences in BOLD response to congruous words. In controls, the New>Old contrast demonstrated larger responses in much of the left-hemisphere (including putative P600 generators: parahippocampal, cingulate, fusiform, perirhinal, middle temporal (MTG) and inferior frontal gyri (IFG)); the Old>New contrast showed modest activation, mainly in right parietal and prefrontal cortex. By contrast, there were relatively few regions of significant New>Old responses in AD patients, mainly in the right-hemisphere, and their Old>New contrast did not demonstrate a right-hemisphere predominance. Across subjects, the spatial extent of New>Old responses in left medial temporal lobe (MTL) correlated with subsequent recall and recognition (r's>or=0.60). In controls, the magnitude of New-Old response in left MTL, fusiform, IFG, MTG, superior temporal and cingulate gyrus correlated with subsequent cued recall and/or recognition (0.51Old responses to congruous words in normal elderly). This network appears dysfunctional in mild AD patients, as reflected in decreased word repetition effects particularly in left association cortex, paralimbic and MTL structures.


Subject(s)
Alzheimer Disease/complications , Brain/blood supply , Memory Disorders/etiology , Memory Disorders/pathology , Semantics , Vocabulary , Acoustic Stimulation/methods , Aged , Aged, 80 and over , Brain/physiopathology , Brain Mapping , Discrimination, Psychological/physiology , Female , Functional Laterality/physiology , Humans , Image Processing, Computer-Assisted/methods , Logistic Models , Magnetic Resonance Imaging/methods , Male , Oxygen/blood , Photic Stimulation/methods , Reaction Time/physiology
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