Funciones ejecutivas en pacientes con apnea obstructiva del sueño: explorando el modelo prefrontal / Executive functions in patients with obstructive sleep apnea: exploring the prefrontal model

Introducción El modelo prefrontal propone que los individuos con apnea obstructiva del sueño (AOS) manifiestan conductas similares a un síndrome disejecutivo como resultado de las alteraciones de gases en la sangre y la fragmentación del sueño. Objetivo Comparar las funciones ejecutivas en pacientes con AOS con valores normativos y explorar su relación con las alteraciones de gases en la sangre y la fragmentación del sueño. Pacientes y métodos Se reclutó a pacientes de la comunidad general y de un hospital de tercer nivel. La puntuación obtenida en la evaluación neuropsicológica se contrastó con la t de Student para una muestra. Posteriormente, se estimó un análisis de regresión lineal múltiple mediante parámetros polisomnográficos de hipercapnia, hipoxemia y fragmentación del sueño como variables predictoras, y la puntuación de funciones ejecutivas como variable que se debe predecir. Resultados Pese a que el desempeño en la evaluación neuropsicológica del 26% de esta muestra se clasificó como alteración ejecutiva, los indicadores de fragmentación del sueño y alteraciones de gases no predijeron el desempeño ejecutivo. Conclusión Una fracción de los pacientes con AOS mostró un desempeño similar a un síndrome disejecutivo; no obstante, permanecen indefinidos los factores que subyacen y favorecen este tipo de manifestaciones cognitivas. La atención temprana de este problema de salud pública podría ser la mejor herramienta disponible en aras de mejorar la calidad de vida y prevenir riesgos a la salud. (AU)

INTRODUCTION According to the prefrontal model, individuals with obstructive sleep apnea (OSA) manifest behaviours mimicking dysexecutive syndrome as a result of blood gas abnormalities and sleep fragmentation. OBJECTIVE. To compare executive functions in OSA patients with normative values and explore their relationship with blood gas abnormalities and sleep fragmentation. PATIENTS AND METHODS Patients were recruited from the wider community and from a tertiary care hospital. The score obtained in the neuropsychological assessment was compared with Student’s t-test for a sample. A multiple linear regression analysis was subsequently estimated, using polysomnographic parameters of hypercapnia, hypoxemia and sleep fragmentation as the predictor variables, and the executive function score as the variable to be predicted. RESULTS Although the neuropsychological assessment performance of 26% of this sample was classified as executive impairment, indicators of sleep fragmentation and gas abnormalities failed to predict the performance of executive functions. CONCLUSION. A proportion of the patients with OSA presented performance similar to a dysexecutive syndrome; however, the factors underlying and fostering this type of cognitive manifestation remain unclear. Early treatment for this public health problem could be the best tool available for improving quality of life and preventing health risks. (AU)

Partial Klüver-Bucy syndrome in a Paediatric patient: A post-neurosurgical and neuropsychological cases.

A variety of cognitive, behavioural, and emotional impairments have been reported in the literature that are associated with the resection of the temporal cortex. Klüver-Bucy syndrome is one infrequently reported disorder in the paediatric population. This paper describes the neuropsychological findings of a female paediatric patient at 7 and 10 years of age with a diagnosis of partial Klüver-Bucy syndrome (pKBS) following total resection of the amygdala and right hippocampus to resect a glioma. The patient presented emotional problems, aggressiveness, hypermetamorphosis, social indifference, and behavioural dysexecutive syndrome, which was found at both 7 and 10 years, but with a decrease in the severity of alterations in attention, impulsivity, hyperactivity, and aggressive behaviour in a second evaluation after she had a neuropsychological intervention. These findings describe the neuropsychological profile of paediatric case with resection of the amygdala and right temporal lobe.

Same, Same but Different? A Multi-Method Review of the Processes Underlying Executive Control.

Attention, working memory, and executive control are commonly considered distinct cognitive functions with important reciprocal interactions. Yet, longstanding evidence from lesion studies has demonstrated both overlap and dissociation in their behavioural expression and anatomical underpinnings, suggesting that a lower dimensional framework could be employed to further identify processes supporting goal-directed behaviour. Here, we describe the anatomical and functional correspondence between attention, working memory, and executive control by providing an overview of cognitive models, as well as recent data from lesion studies, invasive and non-invasive multimodal neuroimaging and brain stimulation. We emphasize the benefits of considering converging evidence from multiple methodologies centred on the identification of brain mechanisms supporting goal-driven behaviour. We propose that expanding on this approach should enable the construction of a comprehensive anatomo-functional framework with testable new hypotheses, and aid clinical neuroscience to intervene on impairments of executive functions.

Cognitive Complications of COVID-19 Infection.

SARS-CoV-2 is associated with a post-infectious neurocognitive syndrome characterized by fatigue and deficits in attention, memory, and executive function. As screening cognitive testing generally remains normal, the pathophysiologic basis of these symptoms remains controversial and there is no standardized treatment paradigm. We present a clinical case demonstrative of typical neurocognitive sequelae of SARS-CoV-2 infection, highlighting medical and social factors that may have contributed to the severity of symptoms. We discuss the pathophysiologic evidence for cognitive "brain fog" following COVID-19 infection as well as lifestyle changes and rehabilitation strategies that may improve recovery. As the benefits of pharmacologic therapy remain unproven, we close with a brief discussion of medication options that might be appropriate targets for future clinical trials in the context of rehabilitative treatment.

Reduced penetrance of an eastern French mutation in ATL1 autosomal-dominant inheritance (SPG3A): extended phenotypic spectrum coupled with brain 18F-FDG PET.

ATL1-related spastic paraplegia SPG3A is a pure form of hereditary spastic paraplegia. Rare complex phenotypes have been described, but few data concerning cognitive evaluation or molecular imaging of these patients are available. We relate a retrospective collection of patients with SPG3A from the Neurology Department of Nancy University Hospital, France. For each patient were carried out a 18F-FDG PET (positron emission tomography), a electromyography (EMG), a sudoscan®, a cerebral and spinal cord MRI (magnetic resonance imaging) with measurement of cervical and thoracic surfaces, a neuropsychological assessment. The present report outlines standardised clinical and paraclinical data of five patients from two east-France families carrying the same missense pathogenic variation, NM_015915.4(ATL1): c.1483C > T p.(Arg495Trp) in ATL1. Mean age at onset was 14 ± 15.01 years. Semi-quantitatively and in comparison to healthy age-matched subjects, PET scans showed a significant cerebellar and upper or mild temporal hypometabolism in all four adult patients and hypometabolism of the prefrontal cortex or precuneus in three of them. Sudoscan® showed signs of small fibre neuropathy in three patients. Cervical and thoracic patients' spinal cords were significantly thinner than matched-control, respectively 71 ± 6.59mm2 (p = 0.01) and 35.64 ± 4.35mm2 (p = 0.015). Two patients presented with a dysexecutive syndrome. While adding new clinical and paraclinical signs associated with ATL1 pathogenic variations, we insist here on the variable penetrance and expressivity. We report small fibre neuropathy, cerebellar hypometabolism and dysexecutive syndromes associated with SPG3A. These cognitive impairments and PET findings may be related to a cortico-cerebellar bundle axonopathy described in the cerebellar cognitive affective syndrome (CCAS).

The Combined Use of Neuropsychiatric and Neuropsychological Assessment Tools to Make a Differential Dementia Diagnosis in the Presence of "Long-Haul" COVID-19.

The longer term neurocognitive/neuropsychiatric consequences of moderate/severe COVID-19 infection have not been explored. The case herein illustrates a complex web of differential diagnosis. The onset, clinical trajectory, treatment course/response, serial neuroimaging findings, and neuropsychological test data were taken into account when assessing a patient presenting 8 months post-COVID-19 (with premorbid attention-deficit hyperactivity disorder, diabetes mellitus, mood difficulties, and a positive family history of vascular dementia). Her acute COVID-19 infection was complicated by altered mental status associated with encephalopathy and bacterial pneumonia. After recovery from COVID-19, the patient continues to experience persisting cognitive and emotive difficulties despite an ongoing psychopharmacotherapy regimen (16 + years), psychotherapy (15 + sessions), and speech-language pathology SLP; 2 × week/for 12 weeks). The purpose of her most recent and comprehensive neuropsychological evaluation was to determine the presence/absence of neurocognitive disorder. The patient is a 62-year-old Caucasian woman. Cognitive screening was completed 3 months post-acute COVID-19 as part of an SLP evaluation, and a full neuropsychological evaluation was conducted 8 months post-COVID-19 recovery on an outpatient basis (in person). The patient had serial neuroimaging. Initial neurological evaluation during acute COVID-19 included unremarkable brain computed tomography (CT)/magnetic resonance imaging. However, follow-up CT (without contrast) revealed, in part, "asymmetric perisylvian atrophy on the left." Full neuropsychological evaluation at 8 months post-COVID-19 recovery revealed a dysexecutive syndrome characterized by language dysfunction and affective theory-of-mind deficit, consistent with dementia. There is need for careful use of differential diagnosis in COVID-19 patients with multiple risk factors that make them more susceptible to long-term neurological complications post-COVID-19. Differential diagnosis should involve multidisciplinary assessment (e.g., neuropsychology, SLP, neurology, and psychiatry).

Exploring behavioural and cognitive dysexecutive syndrome in patients with focal prefrontal cortex damage.

This study's objectives were to characterize the frequency and profile of behavioral and cognitive dysexecutive syndromes in patients with focal prefrontal cortex damage and how these syndromes overlap. We also examined the contribution of the prefrontal brain regions to these syndromes. Therefore, thirty patients with prefrontal cortex damage and thirty control subjects were compared on their performances using the GREFEX battery assessing the dysexecutive syndromes. The results showed that combined behavioral and cognitive dysexecutive syndrome was observed in 53.33%, while pure cognitive dysexecutive syndrome was observed in 20% and behavioral in 26.67%. Also, almost all behavioral and cognitive dysexecutive disorders discriminated frontal patients from controls. Moreover, correlations and regression analyses between task scores in both domains of dysexecutive syndromes showed that the spectrum of behavioral disorders was differentially associated with cognitive impairment of initiation, inhibition, generation, deduction, coordination, flexibility and the planning process. Furthermore, the patterns of cognitive and behavioral dysexecutive syndrome were both predictors of impairment in daily living activities and loss of autonomy. Finally, frontal regions contributing to different dysexecutive syndromes assessed by MRI voxel lesion symptom analysis indicate several overlapping regions centered on the ventromedial and dorsomedial prefrontal cortex for both domains of dysexecutive syndrome. This study concludes that damage to the frontal structures may lead to a diverse set of changes in both cognitive and behavioral domains which both contribute to loss of autonomy. The association of the ventromedial and dorsomedial prefrontal regions to both domains of dysexecutive syndrome suggests a higher integrative role of these regions in processing cognition and behavior.

Three cases of Creutzfeldt-Jakob disease presenting with a predominant dysexecutive syndrome.

Creutzfeldt-Jakob disease (CJD) is a rare, uniformly fatal prion disease. Although CJD commonly presents with rapidly progressive dementia, ataxia, and myoclonus, substantial clinicopathological heterogeneity is observed in clinical practice. Unusual and predominantly cognitive clinical manifestations of CJD mimicking common dementia syndromes are known to pose as an obstacle to early diagnosis and prognosis. We report a series of three patients with probable or definite CJD (one male and two females, ages 52, 58 and 68) who presented to our tertiary behavioral neurology clinic at Mayo Clinic Rochester that met criteria for a newly defined progressive dysexecutive syndrome. Glucose hypometabolism patterns assessed by 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) strongly resembled those of dysexecutive variant of Alzheimer's disease (dAD). However, magnetic resonance imaging (MRI) demonstrated restricted diffusion in neocortical areas and deep nuclei, while cerebrospinal fluid biomarkers indicated abnormal levels of 14-3-3, total-tau, and prion seeding activity (RT-QuIC), establishing the diagnosis of CJD. Electroencephalogram (EEG) additionally revealed features previously documented in atypical cases of CJD. This series of clinical cases demonstrates that CJD can present with a predominantly dysexecutive syndrome and FDG-PET hypometabolism typically seen in dAD. This prompts for the need to integrate information on clinical course with multimodal imaging and fluid biomarkers to provide a precise etiology for dementia syndromes. This has important clinical implications for the diagnosis and prognosis of CJD in the context of emerging clinical characterization of progressive dysexecutive syndromes in neurodegenerative diseases like dAD.

Phenotypic subtypes of progressive dysexecutive syndrome due to Alzheimer's disease: a series of clinical cases.

Diagnostic criteria for a progressive dysexecutive syndrome due to Alzheimer's disease (dAD) were proposed. Clinical observations suggest substantial variability in the clinico-radiological profiles within this syndrome. We report a case series of 6 patients with dAD highlighting this heterogeneity. Average age at diagnosis was 57.3 years, and patients were followed annually with clinical, cognitive, and multimodal imaging assessments for an average of 3.7 years. Cases were divided based into three subtypes based on their pattern of FDG-PET hypometabolism: predominantly left parieto-frontal (ldAD), predominantly right parieto-frontal (rdAD), or predominantly biparietal (bpdAD) (n = 2 for each). Prominent executive dysfunction was evidenced in all patients. ldAD cases showed greater impairment on measures of verbal working memory and verbal fluency compared to other subtypes. rdAD cases showed more severe alterations in measures of visual abilities compared to language-related domains and committed more perseverative errors on a measure of cognitive flexibility. bpdAD cases presented with predominant cognitive flexibility and inhibition impairment with relative sparing of working memory and a slower rate of clinical progression. rdAD and bpdAD patients developed neuropsychiatric symptoms, whereas none of the ldAD patients did. For each subtype, patterns of tau deposition relatively corresponded to the spatial pattern of FDG hypometabolism. dAD cases could be differentiated from two clinical cases of atypical AD variants (language and visual) in terms of clinical, cognitive and neuroimaging profiles, suggesting that dAD subtypes represent clinical entities separable from other variants of the disease. The recognition of distinct dAD phenotypes has clinical relevance for diagnosis, prognosis, and symptom management.

A wearable ring-shaped inertial system to identify action planning impairments during reach-to-grasp sequences: a pilot study.

BACKGROUND: The progressive ageing of the population is leading to an increasing number of people affected by cognitive decline, including disorders in executive functions (EFs), such as action planning. Current procedures to evaluate cognitive decline are based on neuropsychological tests, but novel methods and approaches start to be investigated. Reach-to-grasp (RG) protocols have shown that intentions can influence the EFs of action planning. In this work, we proposed a novel ring-shaped wearable inertial device, SensRing, to measure kinematic parameters during RG and after-grasp (AG) tasks with different end-goals. The aim is to evaluate whether SensRing can characterize the motor performances of people affected by Mild Neurocognitive Disorder (MND) with impairment in EFs. METHODS: Eight Individuals with dysexecutive MND, named d-MND, were compared to ten older healthy subjects (HC). They were asked to reach and grasp a can with three different intentions: to drink (DRINK), to place it on a target (PLACE), or to pass it to a partner (PASS). Twenty-one kinematic parameters were extracted from SensRing inertial data. RESULTS: Seven parameters resulted able to differentiate between HC and d-MND in the RG phase, and 8 features resulted significant in the AG phase. d-MND, indeed, had longer reaction times (in RG PLACE), slower peak velocities (in RG PLACE and PASS, in AG DRINK and PLACE), longer deceleration phases (in all RG and AG DRINK), and higher variability (in RG PLACE, in AG DRINK and PASS). Furthermore, d-MND showed no significant differences among conditions, suggesting that impairments in EFs influence their capabilities in modulating the action planning based on the end-goal. CONCLUSIONS: Based on this explorative study, the system might have the potential for objectifying the clinical assessment of people affected by d-MND by administering an easy motor test. Although these preliminary results have to be investigated in-depth in a larger sample, the portability, wearability, accuracy, and ease-of use of the system make the SensRing potentially appliable for remote applications at home, including analysis of protocols for neuromotor rehabilitation in patients affected by MND.

Cognitive and behavioral associated changes in manifest Huntington disease: A retrospective cross-sectional study.

INTRODUCTION: Behavioral and cognitive changes can be observed across all Huntington disease (HD) stages. Our multicenter and retrospective study investigated the association between cognitive and behavioral scale scores in manifest HD, at three different yearly timepoints. METHODS: We analyzed cognitive and behavioral domains by the Unified Huntington's Disease Rating Scale (UHDRS) and by the Problem Behaviors Assessment Short Form (PBA-s), at three different yearly times of life (t0 or baseline, t1 after one year, t2 after two years), in 97 patients with manifest HD (mean age 48.62 ± 13.1), from three ENROLL-HD Centers. In order to test the disease progression, we also examined patients' motor and functional changes by the UHDRS, overtime. RESULTS: The severity of apathy and of perseveration/obsession was associated with the severity of the cognitive decline (p < .0001), regardless of the yearly timepoint. The score of irritability significantly and positively correlated with perseveration errors in the verbal fluency test at t0 (r = .34; p = .001), while the psychosis significantly and negatively correlated with the information processing speed at t0 (r = -.21; p = .038) and significantly and positively correlated with perseveration errors in the verbal fluency test at t1 (r = .35; p < .0001). The disease progression was confirmed by the significant worsening of the UHDRS-Total Motor Score (TMS) and of the UHDRS-Total Functional Capacity (TFC) scale score after two-year follow-up (p < .0001). CONCLUSION: Although the progression of abnormal behavioral manifestations cannot be predicted in HD, the severity of apathy and perseveration/obsessions are significantly associated with the severity of the cognitive function impairment, thus contributing, together, to the disease development and to patients' loss of independence, in addition to the neurological manifestations. This cognitive-behavior pattern determines a common underlying deficit depending on a dysexecutive syndrome.

Frontal Dysexecutive Syndrome in Brain Tumors: A Pragmatic Insight to an Old Problem.

Brain tumors have long been considered one of the most prevalent causes of potentially reversible cognitive impairment. An accurate underlying cause of cognitive impairment due to brain tumor needs to be evaluated pragmatically. Patterns of cognitive impairment associated with brain tumors depend mainly on their location, lateralization, pathological classification and secondary effects of the treatment, as well as the structural plasticity and diaschisis. Hence, it is not rare that lesions with different locations and histologies may manifest with a similar pattern of cognitive impairment due to the complex interplay of determinants. We herein report 3 patients with brain tumors affecting different locations and with differing histologies, who shared a similar presentation as "frontal dysexecutive syndrome" masqueraded as psychiatric conditions. Detailed examination of saccades and pursuit along with eye movements and conventional motor examinations were essential not only to diagnose brain tumor as the potential cause of cognitive impairment, but also to rule out other coexisting etiologies with completely different underlying pathological mechanisms (i.e., Huntington's disease in 1 of the cases). A detailed neurological examination, including eye movement assessment, in patients with psychiatric symptoms provides not only important clues to delineate the underlying anatomical substrate involved, but also helps clinicians to make an accurate diagnosis and to select appropriate therapeutic options.

Eye Movement Technique to Improve Executive Function in Patients With Stroke: A Randomized Controlled Trial.

Objective: To investigate the efficacy of eye movement technique for the treatment of executive dysfunction of patients with stroke. Methods: This was a prospective, single-blinded, randomized, controlled, single-center clinical trial conducted from June 2018 to December 2019 in patients with stroke. The patients were randomized 1:1 to the routine (conventional management) and eye-move group (routine management plus eye movement technique: 5-min goal management training, 5-min computer-aided working memory, and 10 min of inhibitory control training and set conversion training). The intervention lasted 6 weeks, followed by a 4-week follow-up. The primary endpoint was the Behavioral Assessment of the Dysexecutive Syndrome (BADS) score. The secondary endpoints mainly included the Montreal Cognitive Assessment (MoCA), Wisconsin Card Sorting Test (WCST), and modified Barthel Index (MBI) scores. Results: Sixty-four patients were enrolled (32/group). After the 6-week intervention, the BADS and WCST scores of the eye-move group were significantly improved than those of the routine group (all P < 0.05), but the effects were attenuated in certain subscores after follow-up (all P > 0.05). The MoCA and MBI scores of the eye-move group were significantly higher, and the reaction time was significantly lower than those of the routine group at 4 weeks after the intervention (all P < 0.05). After follow-up, the MBI scores of the eye-move group were still higher than that of the routine group (P < 0.001), but there were no differences for MoCA scores and reaction time (both P > 0.05). Conclusion: The eye movement technique could improve the executive function of patients with stroke. These results have to be confirmed. This was a prospective, single-blinded, randomized, controlled, single-center clinical trial (ChiCTR2000036393). Clinical Trial Registration: [www.chictr.org.cn], identifier [ChiCTR2000036393].

Neuropsychological and neurophysiological correlates of fatigue in post-acute patients with neurological manifestations of COVID-19: Insights into a challenging symptom.

More than half of patients who recover from COVID-19 experience fatigue. We studied fatigue using neuropsychological and neurophysiological investigations in post-COVID-19 patients and healthy subjects. Neuropsychological assessment included: Fatigue Severity Scale (FSS), Fatigue Rating Scale, Beck Depression Inventory, Apathy Evaluation Scale, cognitive tests, and computerized tasks. Neurophysiological examination was assessed before (PRE) and 2 min after (POST) a 1-min fatiguing isometric pinching task and included: maximum compound muscle action potential (CMAP) amplitude in first dorsal interosseous muscle (FDI) following ulnar nerve stimulation, resting motor threshold, motor evoked potential (MEP) amplitude and silent period (SP) duration in right FDI following transcranial magnetic stimulation of the left motor cortex. Maximum pinch strength was measured. Perceived exertion was assessed with the Borg-Category-Ratio scale. Patients manifested fatigue, apathy, executive deficits, impaired cognitive control, and reduction in global cognition. Perceived exertion was higher in patients. CMAP and MEP were smaller in patients both PRE and POST. CMAP did not change in either group from PRE to POST, while MEP amplitudes declined in controls POST. SP duration did not differ between groups PRE, increased in controls but decreased in patients POST. Patients' change of SP duration from PRE to POST was negatively correlated to FSS. Abnormal SP shortening and lack of MEP depression concur with a reduction in post-exhaustion corticomotor inhibition, suggesting a possible GABAB-ergic dysfunction. This impairment might be related to the neuropsychological alterations. COVID-19-associated inflammation might lead to GABAergic impairment, possibly representing the basis of fatigue and explaining apathy and executive deficits.

"Cool" and "Hot" Executive Functions in Patients With a Predominance of Negative Schizophrenic Symptoms.

BACKGROUND: Patients with psychosis often present significant neurocognitive deficits, with executive function deficits (EEFF) being one of the most relevant cognitive impairments with the greatest impact on the functioning of their daily lives. However, although various findings of executive involvement were reported, it is not entirely clear whether there is a differential pattern of involvement according to the clinical symptoms or the deficits occur in all or only in some subcomponents of EEFF. OBJECTIVE: The present study had a double objective: to study the specific deficits in the cool and hot EEFF in a group of psychotic patients with a predominance of negative symptoms; and determine the possible associations between the performance of the patients in the cool an hot EEFF tasks with the negative symptoms, and with the behavioral alterations associated with the dysexecutive syndrome. METHOD: 66 participants, 33 psychotic patients with a predominance of negative symptoms and 33 healthy control subjects matched in gender, age and educational level participated. Both groups were administered 4 cool EEFF tasks (coding/maintenance and updating of information in working memory, ability to change the mental set and planning), and 3 hot EEFF tasks (decision making in situations of uncertainty, recognition of emotions through facial expressions and theory of mind). In the group of patients, the Negative symptoms were evaluated through the Scale for the Evaluation of Negative Symptoms (SANS), and the behavioral alterations associated with dysexecutive syndrome through the subscale of "Executive Dysfunction" of the Frontal Systems Behavior Scale. RESULTS: Patients performed worse on three cool EEFF tasks and on two of the hot EEFF tasks. Additionally, we found a correlation between the SANS score and the "executive dysfunction" subscale, with the cold EEFF task that measures planning. CONCLUSION: Our findings showed that in psychotic patients with a predominance of negative symptoms, both, the cognitive (cool) and emotional (hot) components of executive functions are affected. The results reinforce the need for a cognitive rehabilitation treatment of the executive components of the working memory and of those more socio-emotional aspects.

Association Between Behavioral Dysexecutive Syndrome and the Health-Related Quality of Life Among Stroke Survivors.

AIM: Behavioral dysexecutive syndrome (BDES) is one common neuropsychiatric comorbidity after stroke. Despite evidences suggesting the adverse effect of BDES on the survivors' outcome, little is known about the association between BDES and the health-related quality of life (HRQoL) among stroke survivors and how BDES impacts the HRQoL. This study aimed to address these questions. METHODS: This study included 219 patients with acute ischemic stroke consecutively admitted to a regional hospital in Hong Kong. BDES was defined as a Chinese version of the Dysexecutive Questionnaire (DEX) score of ≥20 assessed at three months after stroke. The HRQoL was assessed with the Chinese version of the Stroke-Specific Quality of Life (SSQoL) questionnaire encompassing 12 domains. Multivariate linear regression models were employed to examine the association between BDES symptoms and the SSQoL total and domain scores. Structural equation model (SEM) was further constructed to delineate the linking pathways linking BDES and the HRQoL. RESULTS: The study sample compromised mainly older patients with mild to moderate ischemic stroke. Compared with patients without BDES, those with BDES exhibited poorer performances regarding with the summarized SSQoL (226.2 ± 18.8 vs. 200.3 ± 29.8, p < 0.001) and almost all domains. The BDES symptoms were independently contributed to the whole HRQoL (SSQoL total score) (ß = -0.20, p = 0.002), specifically to the domains in personality (ß = -0.34, p < 0.001), language (ß = -0.22, p = 0.01), and work/productivity (ß = -0.32, p < 0.001), after adjusting demographic and clinical characteristics in linear models. The impacts of the BDES symptoms on the HRQoL were mainly explained by the indirect path mediated by depression and anxiety (path coefficient = -0.27, p < 0.05) rather than physical disability, while the resting was elucidated by the path directly linking BDES to the HRQoL (path coefficient = -0.17, p < 0.05). CONCLUSION: The present study preliminarily demonstrated a potential association between BDES and a lower level of the HRQoL, predominantly in domains of personality, language, and work/productivityafter acute ischemic stroke. This study also offered insights into the underlying mechanisms linking BDES and the HRQoL, implicating that integrative psychological therapies were urged to achieve better HRQoL after stroke.

Long-term normalization of cognitive and psychopathological alterations in a juvenile Niemann-Pick type C case.

Niemann-Pick type C (NP-C) disease is a neurovisceral atypical lysosomal lipid storage disorder with a poor prognosis. We present the 5-year neuropsychological follow-up of a patient with juvenile onset NP-C, spanning the pre-diagnostic stage to the period after treatment with miglustat (Actelion Pharmaceuticals Inc., CA, US). In the initial stages of the disease, the patient presented behavioral dysexecutive symptoms resembling those frequently observed in adult-onset forms and frontotemporal dementia, which frequently makes early diagnosis difficult in children. After 4 years of treatment, the impaired cognitive function and behavioral dysexecutive syndrome had been completely reversed. The variability of NP-C disease makes early diagnosis challenging. Evaluations of long-term neuropsychological development can help diagnose this neurodegenerative disease and document its progression.

Relation between cognitive and behavioral aspects of dysexecutive functioning in normal aging.

The aim of this study was to investigate the relation between cognitive and behavioral symptoms of dysexecutive functioning, understood as two aspects of dysexecutive syndrome, in an elderly nonclinical sample. Most previous studies have concentrated on clinical population. However, nonclinical population of elderly adults is, in this context, a group of special interest due to a large body of evidence indicating that executive functions decrease pronouncedly in the course of normal aging. The data were collected from 40 participants aged 67-86 years. None presented symptoms of cognitive disorder or depression or reported psychiatric or neurological problems. The Dysexecutive Questionnaire was used as a measure of behavioral aspect of dysexecutive syndrome. Participants also performed experimental tasks referring to three cognitive aspects of executive functioning (i.e., updating, inhibition, and attentional shifting). Analysis resulted in weak and nonsignificant correlations between cognitive and behavioral aspects of dysexecutive syndrome. Results are discussed in the context of previous research and diagnostic criteria of dysexecutive syndrome.