Cognitive Manifestations Research Articles

Neuropsychological profile of CSF1R-related leukoencephalopathy.

The neuropsychological profile of CSF1R-related leukoencephalopathy (CRL) is undefined. This study defines the profile, contrasts it with that of other dementia syndromes, and highlights measures sensitive to cognitive impairment. We administered a standardized battery of neuropsychological tests to five consecutive CRL cases. The neuropsychological profile of CRL reflects impaired general cognitive function, processing speed, executive function, speeded visual problem solving, verbal fluency, and self-reported depression and anxiety. Confrontation naming and memory are preserved. Within cognitive domains, certain measures more frequently identified impairment than others. CRL impairs general cognitive function, processing speed, executive function. Language and visual problem solving may be impaired if processing speed is required. Confrontation naming and memory are uniquely preserved, contrasting CRL to other dementia syndromes. Cognitive screens excluding processing speed and executive function may not detect CRL cognitive manifestations. Findings sharply define cognitive impairment of CRL and inform cognitive test selection.

Differential Diagnosis of ICD-11 Personality Disorder and Autism Spectrum Disorder in Adolescents.

The International Classification of Diseases 11th Revision (ICD-11) introduces fundamentally new diagnostic descriptions for personality disorder and autism spectrum disorder. Instead of the traditional categorical taxonomies, both personality disorder and autism spectrum disorder are described as being on a continuum. Accumulating research has pointed out that, in some cases, adolescents with autism spectrum disorder are at risk of being confused with having a personality disorder, which particularly applies to female adolescents. Case reports describe how adult autistic women struggled with social and identity roles as children and adolescents, using compensatory strategies such as social imitation and other types of camouflaging. Furthermore, some adolescents with autism display emotion dysregulation and self-injury. The ICD-11 recognizes that features of autism spectrum disorder may resemble features of personality disorder, but the two diagnoses have not yet been formally compared to one another. The present article therefore sought to outline and discuss the overlap and boundaries between the ICD-11 definitions of personality disorder and autism spectrum disorder and propose guiding principles that may assist practitioners in differential diagnosis with female adolescents. We specifically highlight how aspects of the self and interpersonal functioning along with emotional, cognitive, and behavioral manifestations may overlap across the two diagnoses. Restricted, repetitive, and inflexible patterns of behavior, interests, and activities are core features of autism spectrum disorder, which may be masked or less pronounced in female adolescents. Collecting a developmental history of the early presence or absence of autistic features is vital for a conclusive diagnosis, including features that are typically camouflaged in females. A number of future directions for research and clinical practice are proposed.

Physical Function and Association with Cognitive Function in Patients in a Post-COVID-19 Clinic-A Cross-Sectional Study.

Patients with long-term health sequelae of COVID-19 (post-COVID-19 condition) experience both physical and cognitive manifestations. However, there is still uncertainty about the prevalence of physical impairment in these patients and whether there is a link between physical and cognitive function. The aim was to assess the prevalence of physical impairment and investigate the association with cognition in patients assessed in a post-COVID-19 clinic. In this cross-sectional study, patients referred to an outpatient clinic ≥ 3 months after acute infection underwent screening of their physical and cognitive function as part of a comprehensive multidisciplinary assessment. Physical function was assessed with the 6-Minute Walk Test, the 30 s Sit-to-Stand Test and by measuring handgrip strength. Cognitive function was assessed with the Screen for Cognitive Impairment in Psychiatry and the Trail Making Test-Part B. Physical impairment was tested by comparing the patients' performance to normative and expected values. Association with cognition was investigated using correlation analyses and the possible explanatory variables regarding physical function were assessed using regression analyses. In total, we included 292 patients, the mean age was 52 (±15) years, 56% were women and 50% had been hospitalised during an acute COVID-19 infection. The prevalence of physical impairment ranged from 23% in functional exercise capacity to 59% in lower extremity muscle strength and function. There was no greater risk of physical impairment in previously hospitalised compared with the non-hospitalised patients. There was a weak to moderate association between physical and cognitive function. The cognitive test scores had statistically significant prediction value for all three outcomes of physical function. In conclusion, physical impairments were prevalent amongst patients assessed for post-COVID-19 condition regardless of their hospitalisation status and these were associated with more cognitive dysfunction.

Neural Substrates of Psychomotor Speed Deficits in Cerebral Small Vessel Disease: A Brain Disconnectome Mapping Study.

It remains unknown which factors influence how brain disconnectivity derived from White Matter Hyperintensity (WMH) lesions leads to psychomotor speed dysfunction, one of the earliest and most common cognitive manifestations in the cerebral Small Vessel Disease (cSVD) population. While the burden of WMH has been strongly linked to psychomotor speed performance, the effect that different locations and volumes of WMH may have on cSVD-related cognitive impairment remains unclear. Therefore, we aimed to explore (1) whether global WMH, deep WMH (DWMH), and periventricular (PVWMH) volumes display different psychomotor speed associations; (2) whether tract-specific WMH volume shows stronger cognitive associations compared with global measures of WMH volume; (3) whether specific patterns of WMH location lead to different degrees of disconnectivity. Using the BCBToolkit, we investigated which pattern of distribution and which locations of WMH lesion result in impaired psychomotor speed in a well-characterized sample (n = 195) of cSVD patients without dementia. Two key findings emerge from our study. First, global (and not tract-specific) measures of WMH volume were associated with psychomotor speed performance. Second, disconnection maps revealed the involvement of callosal tracts, association and projection fibers, and frontal and parietal cortical brain areas related to psychomotor speed, while the lesion location influenced such associations. In conclusion, psychomotor deficits are affected differently by WMH burden and topographic distribution through brain disconnection in non-demented cSVD patients.

Intrafamilial Heterogeneity in Spinocerebellar Ataxia Type 21 (P8-11.015)

<h3>Objective:</h3> To emphasize the variability of clinical presentations of genetic movement disorders as depicted by a family with SCA21. <h3>Background:</h3> Spinocerebellar ataxia type 21 (SCA21) is a rare autosomal dominant condition that can have a variety of motor, cognitive, or behavioral manifestations. This clinical variability is seen both between and within families with the same mutation. <h3>Design/Methods:</h3> N/A <h3>Results:</h3> A 51-year-old woman presented to clinic for evaluation of lifelong worsening balance impairment and hand clumsiness. Her physical exam noted marked ataxia and dysmetria in the extremities, ataxic gait, and spastic extremities, and neuropsychiatric testing was remarkable for mild impairments. Following further workup, a family history of neurologic complaints was taken, which revealed that one of her three children had tremors. When brought to clinic, the patient’s daughter revealed a lifelong history of bilateral hand tremors with worsening over the previous two years. She further noted a slowness in her thinking that required special education courses, as well as other mild motor difficulties. Given the potential for a genetic condition, an ataxia panel was ordered, which revealed a c.509C&gt;Tp.P170L mutation in the TMEM240 gene in the patient and her daughter. <h3>Conclusions:</h3> This case emphasizes the importance of recognizing intrafamilial clinical heterogeneity in the diagnosis of genetic movement disorders. It highlights the potential benefits of an investigation into a family history notable for neurological disorders, which may provide the opportunity for appropriate testing and diagnosis. The variable presentations of spinocerebellar ataxias within families have been explored in a limited number of case reports. The identification of a possible unknown factor driving these differences in presentation could have potential therapeutic implications. While all reported cases of SCA21 have some degree of gait and limb ataxia, only one case has reported tremor onset followed by ataxia and dystonia, as seen in this case. <b>Disclosure:</b> Emma *Use 368242 Wetmore has nothing to disclose. Dr. Decker has nothing to disclose. Maysen Mesaros has nothing to disclose. An immediate family member of Dr. Rodriguez-Porcel has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Anmeal. Dr. Rodriguez-Porcel has received publishing royalties from a publication relating to health care.

The contribution of thalamic pathology to the clinical manifestations of frontotemporal dementia phenotypes (P5-12.004)

<h3>Objective:</h3> To systematically characterise thalamus pathology in frontotemporal dementia (FTD) phenotypes <h3>Background:</h3> FTD is classically associated with distinctive cortical atrophy patterns. However, the spectrum of cognitive and behavioural manifestations in FTD arise from multi-synaptic network dysfunction. The thalamus is a key hub of several corticobasal and corticocortical circuits. The main circuits relayed via the thalamic nuclei include the dorsolateral prefrontal circuit; the anterior cingulate circuit; and the orbitofrontal circuit. <h3>Design/Methods:</h3> Thalamus pathology was quantitatively evaluated at a nuclear, vertex and morphometric level in a prospective, multimodal imaging study using a standardised neuroimaging protocol. 10 patients with behavioural FTD (bvFTD), 15 patients with non-fluent variant primary progressive aphasia (nfvPPA) and 5 patients with semantic variant PPA (svPPA) were evaluated with reference to 100 healthy controls. All patients underwent detailed clinical and neuropsychological profiling and whole-genome sequencing. <h3>Results:</h3> Volumetric analyses revealed widespread bilateral thalamic nuclear volume loss in bvFTD; selective bilateral with left-sided predominant thalamic degeneration was captured in nfvPPA; and more focal, left-lateralised thalamic volume loss identified in svPPA. Vertex analyses showed diffuse thalamic surface changes in bvFTD and nfvPPA, but not svPPA. Morphometric analyses demonstrated extensive symmetric intra-thalamic changes in nfvPPA, and mostly right-sided changes in both bvFTD and svPPA. <h3>Conclusions:</h3> FTD is associated with considerable focal thalamic degeneration. Phenotype-specific thalamic traits mirror established cortical vulnerability patterns. Thalamic nuclei associated with behaviour and language functions are preferentially involved. FTD is not solely associated with cortical atrophy. The manifestations of FTD are likely to stem from cortico-subcortical circuitry dysfunction and not exclusively driven by focal cortical changes. <b>Disclosure:</b> Dr. McKenna has nothing to disclose. Dr. Hutchinson has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Hutchinson has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Teva. Dr. Hutchinson has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Teva. Dr. Bede has nothing to disclose.

Growth of Angiomyolipomas in Patients with Tuberous Sclerosis Complex Treated with mTOR Inhibitor Differs by Indication for Treatment (P8-13.003)

<h3>Objective:</h3> To determine whether growth rate differs for patients treated with mTOR inhibitor for SEGA or AML or no treatment. <h3>Background:</h3> Tuberous Sclerosis Complex (TSC) is characterized by epilepsy, renal angiomyolipoma (AML), subependymal giant cell astrocytoma (SEGA), and other cognitive, dermatologic, pulmonary, and cardiac manifestations. mTOR inhibitors are prescribed to reduce the size of AMLs or SEGA and reduce seizure frequency. Thus, the indication for initiating mTOR therapy differs for each patient. <h3>Design/Methods:</h3> Patients seen in the Atrium Health Wake Forest Baptist Adult Tuberous Sclerosis clinic were retrospectively evaluated. Patients were eligible if they were adults (age &gt;18 years), had at least 1 renal AML, and had at least two instances of imaging (CT or MRI) to evaluate renal AMLs. Data was collected on patient demographics, current/prior mTOR use, renal involvement, and AML size. AML size was defined as the largest diameter of the dominant lesion. Change in median diameter of the dominant lesions of each treatment was calculated. <h3>Results:</h3> 44 patients were reviewed (mean age = 35.6±13.8, 61% female); 22 met eligibility. AML growth differed for patients prescribed mTOR inhibitor to treat SEGA (n=3, mean treatment 25.5 months, 0.5 mm/year decrease on the right, 1.2 mm/year decrease on the left) compared to AML (n=5, mean treatment = 16.25 months, 0.96 mm/year decrease on the right, 3.5 mm/year decrease on the left). Patients who had never used an mTOR inhibitor displayed a 1.7 mm/year increase in AML size in the right kidney and 1.9 mm/year increase in AML size in the left kidney. <h3>Conclusions:</h3> In this cohort of adult TSC patients with AMLs, patients who did not receive mTOR therapy experienced annual growth in AML size. Those having ever taken an mTOR inhibitor experienced annual decrease in AML size. The median decrease in size differed between patients who initiated treatment for SEGA vs. AMLs. <b>Disclosure:</b> Miss Neal has nothing to disclose. Dr. Thakker has nothing to disclose. Mr. Mallett has nothing to disclose. Dr. Silvia has nothing to disclose. The institution of Dr. Boggs has received research support from Liva Nova. Dr. Boggs has a non-compensated relationship as a Chairman PAB with Epilepsy Alliance North Carolina that is relevant to AAN interests or activities. Theodore B Stem has nothing to disclose. Dr. Pathak has nothing to disclose. Dr. Strowd has received personal compensation for serving as an employee of Kaplan. Dr. Strowd has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Monteris Medical, Inc. Dr. Strowd has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novocure. Dr. Strowd has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for American Academy of Neurology. The institution of Dr. Strowd has received research support from Southeastern Brain Tumor Foundation. The institution of Dr. Strowd has received research support from Jazz Pharmaceuticals. The institution of Dr. Strowd has received research support from National Institutes of Health. The institution of Dr. Strowd has received research support from Alpha Omega Alpha. The institution of Dr. Strowd has received research support from American Board of Psychiatry and Neurology. Dr. Strowd has received publishing royalties from a publication relating to health care. Dr. Strowd has received publishing royalties from a publication relating to health care.

The relationship of cognitive impairment and clinical manifestations of myasthenia gravis

BACKGROUND. There are many studies in the literature suggesting that cognitive impairment is more pronounced in patients with myasthenia gravis compared with healthy people, but there is no explanation of the reasons and mechanisms. However, the reverse character the influence of intellectual-mnestic disorders on the course of myasthenia gravis has not been studied enough.&#x0D; AIM. To compare clinical, anamnestiс, laboratory and instrumental data in patients with myasthenia gravis according to the presence or absence of cognitive impairment.&#x0D; MATERIAL AND METHODS. 61 patients with a confirmed diagnosis of myasthenia gravis were divided into 2 groups according to the Montreal cognitive test. Patients scoring 25 or less were assigned to the first group, those scoring 26 points or more to the second group.&#x0D; A comparative assessment was made according to the myasthenia gravis impairment index, age of onset, duration of the disease, duration of hospitalization, severity of myasthenia gravis according to clinical scales, body mass index, electroneuromyography data, levels of antibodies to the acetylcholine receptor and skeletal muscles, and daily doses of the drugs received.&#x0D; RESULTS. In the 1st group, there was a significantly higher score on the Generalised Disorders subscale of the Myasthenia Gravis Impairment Index; the severity of Quantitative myasthenia gravis score was significantly higher in the 1st group than in the 2nd both for the total score and for the Musculoskeletal Lesions subscale. On average, 49 mg more pyridostigmine bromide was required in the first group, while the average body mass index was significantly lower in the first group.&#x0D; CONCLUSION. The presence of a cognitive deficit in patients with myasthenia gravis may worsen the clinical picture of the disease, which should be taken into account by clinicians, especially in elderly patients, who are at increased risk of developing cognitive disorders.

The role of comorbidity in the development of Long-COVID

BACKGROUND. Post-COVID syndrome is a set of conditions that occur in patients after a coronavirus infection. To date, there remains great interest in identifying the relationships between the influence of concomitant diseases on the course of SARS-CoV-2 infection and the selection of a justified treatment regimen from the point of view of the pathogenesis of the formation of long-term consequences of the disease.&#x0D; OBJECTIVE. To investigate the role of comorbidity in the development of Long-COVID, to study and analyze the results of treatment with a combination of Xavron, Tivorel and Xylate in patients with asthenic manifestations of Long-COVID.&#x0D; MATERIALS AND METHODS. A clinical and neurological examination of 50 patients aged 47 to 76 years with a diagnosis of chronic cerebral ischemia and an asthenic syndrome that persisted for more than 3 months after a confirmed SARS-CoV-2 infection was carried out. All patients were treated according to a clinical protocol approved by the MOH of Ukraine. In addition to the main treatment, 20 patients received a 10-day course of infusions of a combination of the drugs Xavron in a dose of 30 mg No. 10, Tivorel 100.0 No. 5 and Xylate 200.0 No. 5 every other day. Efficacy was assessed before treatment, on the 5th and 10th day of treatment using the MMSE, DASS-21, FAS and MOND scales.&#x0D; RESULTS AND DISCUSSION. A stable positive relationship was found between the number of CIRS-G comorbidity scores and the MOND score. A positive correlation coefficient between comorbidity and asthenic syndrome, assessed on the FAS scale, was also expressed – 0.699 (p&lt;0.001). A somewhat weaker negative relationship was found between anxiety and the number of points on the CIRS-G scale – -0.474 (p=0.035). Assessment of depression and stress on the DASS-21 scale did not show a reliably significant correlation effect. After treatment, the severity of neurological symptoms, assessed by the MOND scale, decreased from 5.6±1.6 to 2.8±1 (p&lt;0.01). Depression and anxiety scores on the DASS-21 scale decreased moderately from 4.9±3.6 to 2.8±1.5 (p&lt;0.01) and from 6.3±2.3 to 4.3±1.5 (p&lt;0.01), respectively. A more significant regression was observed on the stress scale – from 10.3±3.1 to 7.5±1.9 (p&lt;0.01). The difference in the severity of fatigue from 28.8±4.1 to 20±1.2 was especially noticeable (p&lt;0.01). CONCLUSIONS. Asthenic syndrome, which is prevalent in the Long-COVID, directly depends on the background of chronic diseases. The combination of Xavron, Tivorel and Xylate is pathogenetically justified and safe in the treatment of patients with Long-COVID. The use of this combination leads to a reduction of asthenic syndrome, which, in turn, has a positive effect on the reduction of cognitive manifestations of Long-COVID in the form of “brain fog”.

Reimagining and Recalibrating Information Studies: iSchools, the Way Forward

Reviews the various definitions of information and proposes that it has a physical, linguistic and cognitive manifestations. The changing landscape of information studies and the factors that contributing to this are examined. The origins and development of library education in USA, UK and India are briefly mentioned. The changing nature of the information profession in terms of the various job titles that information professionals can fit into is reviewed. Briefly reviews the i-school movement, and suggests that i-schools are the way forward.

Age or age of onset: which is the best criterion to classify late-life depression?

In late-life depression (LLD), several differences between patients whose first episode is reported after age 65 (late-onset depression, LOD) and those with early-onset depression (EOD) might reflect the effects of brain ageing. To test this hypothesis, we analysed the impact of current age and age at illness onset on a number of clinical and cognitive manifestations in 438 outpatients with major depressive disorder aged >60 years, treated with venlafaxine for 12 weeks. When compared to the EOD group, patients with LOD were older ( P < 0.00001) and associated with lower depression severity ( P = 0.0029), lower global cognitive functioning [Mini-Mental State Examination (MMSE): P = 0.0001; Repeatable Battery for the Assessment of Neuropsychological Status: immediate memory, P = 0.0009, and delayed memory, P < 0.00001; Delis-Kaplan Executive Function System measuring executive functions: Trail-Making Test (TMT) - P = 0.0004 and Colour-Word Interference Test, Inhibition - P = 0.0063], and more dyskinesias (Abnormal Involuntary Movement Scale: P = 0.0006). After controlling for its interactions with age of onset, current age was inversely correlated with Montgomery Åsberg Depression Rating Scale scores at baseline ( P < 0.00001) and week 12 ( P = 0.0066), MMSE ( P < 0.00001), delayed memory ( P < 0.00001), and TMT ( P = 0.0021). Age of onset predicted impairment in immediate ( P = 0.023) and delayed memory ( P = 0.0181), and dyskinesias ( P = 0.0006). Although most features of LLD are related to ageing rather than to late-onset, LOD is a possible separate diagnostic entity characterised by memory dysfunction and increased liability to movement disorders.

Posterior Cingulate Epilepsy: A Systematic Review.

In this review, authors discuss epilepsy originating from posterior cingulate regions, a challenging entity to diagnose and most likely underrecognized. A systematic review of posterior middle and posterior cingulate epilepsy cases was conducted to present a summary of current knowledge about this localization-based type of epilepsy. The literature search identified 32 articles, for a total of 69 patients (34 with posterior middle cingulate epilepsy [pMCE] and 35 with posterior cingulate epilepsy [PCE]). Most patients were children and young adults with drug-resistant lesional epilepsy with high seizure burden. In both groups, most patients reported auras, mainly sensory, but various types were reported, including autonomic, behavioral, and cognitive manifestations. Most pMCE and PCE showed motor manifestations (mainly respectively asymmetric tonic posturing and automotor features). Impaired awareness during seizures was more frequently reported in PCE than in pMCE. As for ictal scalp EEG, epileptogenic abnormalities were poorly lateralized and did not localize the seizure onset zone. An ictal temporal involvement was only observed in PCE. Interictal EEG findings were nonspecific. As for other presurgical noninvasive investigations, data are limited, and no studies have adequately assessed their value. Partly explained by our inclusion criteria, most patients underwent a surgical procedure (either lesionectomy or resection of epileptogenic zone as defined by intracranial EEG study results), which overall yielded good outcomes.

Test-retest reliability of resting-state EEG in young and older adults.

The quantification of resting-state electroencephalography (EEG) is associated with a variety of measures. These include power estimates at different frequencies, microstate analysis, and frequency-resolved source power and connectivity analyses. Resting-state EEG metrics have been widely used to delineate the manifestation of cognition and to identify psychophysiological indicators of age-related cognitive decline. The reliability of the utilized metrics is a prerequisite for establishing robust brain-behavior relationships and clinically relevant indicators of cognitive decline. To date, however, test-retest reliability examination of measures derived from resting human EEG, comparing different resting-state measures between young and older participants, within the same adequately powered dataset, is lacking. The present registered report examined test-retest reliability in a sample of 95 young (age range: 20-35 years) and 93 older (age range: 60-80 years) participants. A good-to-excellent test-retest reliability was confirmed in both age groups for power estimates on both scalp and source levels as well as for the individual alpha peak power and frequency. Partial confirmation was observed for hypotheses stating good-to-excellent reliability of microstates measures and connectivity. Equal levels of reliability between the age groups were confirmed for scalp-level power estimates and partially so for source-level power and connectivity. In total, five out of the nine postulated hypotheses were empirically supported and confirmed good-to-excellent reliability of the most commonly reported resting-state EEG metrics.

Impact of multiple sclerosis on mental health: A Cross-Sectional Study

IntroductionWhen we think of multiple sclerosis (MS), we usually talk about the sensory and motor symptoms of the disease and their impact on the functioning of the individual affected. However, this disability can lead to a wide range of symptoms, including psychological and cognitive manifestations that also have a significant impact on the quality of life of patientsObjectivesTo estimate the incidence of psychiatric disorders in patients with MS.MethodsA cross-sectional descriptive study that interested MS patients referred to the occupational pathology consultation of the Charles Nicolle Hospital, during the period from July 1, 2020, to September 30, 2022. The data collected concerned the characteristics of the disease. The detection of psychiatric disorders was studied through a validated self-questionnaire GHQ-12 (General Health Questionnaire).ResultsThe study population consisted of 26 cases. The average age was 38 ± 9 years. A predominance of females was noted in 77% of cases. Eight patients (31%) were smokers. Nine cases (47%) had a relapsing-remitting form and six cases (32%) had a primary progressive form. All patients were on disease-modifying therapy. The average duration of the disease was 6 ± 3 years. The average duration of work during the illness was 4 years [one year-12 years]. The average duration of work stoppage in the last 12 months of activity was 63 days [2-240 days], of which 54% was long-term sick leave. The mean GHQ-12 score was 4.38 [0-10]. Twenty patients (77%) had psychological disorders.ConclusionsThis study shows the high frequency of psychiatric disorders in our MS patients. The role of the neuropsychologist is therefore often crucial in the care of these patients.Disclosure of InterestNone Declared

Cognitive Impairment in Celiac Disease Patients: Scoping Review Exploring Psychological Triggers in a Chronic Condition

Celiac disease (CD), also known as gluten-sensitive enteropathy, is an inflammatory autoimmune reaction triggered by ingestion of gluten in genetically predisposed subjects. Celiac disease is often associated with a wide range of disorders, caused by immune responses and by malabsorption with subsequent nutritional deficiencies. Prevalent neurologic manifestations are ataxia, epilepsy, cerebral calcification, cerebral white matter lesions, peripheral neuropathy and myopathy, but also cognitive impairment. The study aimed to identify emerging and urgent research domains in order to establish a CD-specific patient-tailored protocol that includes both psychological and neuropsychological evaluations. We performed a systematic search of MEDLINE, PubMed, SCOPUS, Web of Science and Cochrane library in November 2022. We conducted a descriptive analysis of the characteristics of the included literature. Based on the exclusion/inclusion criteria, a total of seven articles were included in the scoping review process. This review demonstrated the lack of research on CD-related cognitive impairment key features and tries to focus on both cognitive and psychological manifestations as well as their two-way interaction. We tried to establish the specific areas involved, in order to have a comprehensive view of this condition and trying to determine a correct way of assessing CD cognitive impairment and its correlations with psychological distress and personal coping skills to a chronic condition. Nevertheless, research on this topic is progressively increasing and future studies should address specific key points.

Pharmacogenetics in the Treatment of Huntington's Disease: Review and Future Perspectives.

Huntington's disease (HD) is an autosomal dominant progressive brain disorder, caused by a pathological expansion of a CAG repeat that encodes the huntingtin gene. This genetic neurodegenerative rare disease is characterized by cognitive, motor, and neuropsychiatric manifestations. The aim of the treatment is symptomatic and addresses the hyperkinetic disorders (chorea, dystonia, myoclonus, tics, etc.) and the behavioural and cognitive disturbances (depression, anxiety, psychosis, etc.) associated with the disease. HD is still a complex condition in need of innovative and efficient treatment. The long-term goal of pharmacogenetic studies is to use genotype data to predict the effective treatment response to a specific drug and, in turn, prevent potential undesirable effects of its administration. Chorea, depression, and psychotic symptoms have a substantial impact on HD patients' quality of life and could be better controlled with the help of pharmacogenetic knowledge. We aimed to carry out a review of the available publications and evidence related to the pharmacogenetics of HD, with the objective of compiling all information that may be useful in optimizing drug administration. The impact of pharmacogenetic information on the response to antidepressants and antipsychotics is well documented in psychiatric patients, but this approach has not been investigated in HD patients. Future research should address several issues to ensure that pharmacogenetic clinical use is appropriately supported, feasible, and applicable.

Динамика школьной вовлеченности и ее взаимосвязь с развитием осознанной саморегуляции у подростков

&lt;p&gt;The phenomenon of school engagement, considered as a stable, directed and active participation of students in educational activities and in the school life in general, is of considerable interest to researchers in the field of educational psychology. According to modern scientific concepts, engagement can be assessed through behavioral, cognitive, emotional and social manifestations. The research had its purpose to study the dynamics of school engagement in adolescents, as well as to reveal the relationship of conscious self-regulation with behavioral and cognitive components of engagement based on the longitudinal data obtained on the sample of 6-8 grade students (N=80). A separate task was to find an answer to the question of whether the conscious self-regulation can be considered as a significant predictor of changes in the behavioral and cognitive engagement of students during their study in the secondary school. Methods: "Multidimensional Scale of School Engagement"(Wang et al., 2019; Fomina, Morosanova, 2020); "The Self-Regulation Profile of Learning Activity Questionnaire &amp;mdash; SRPLAQ" (Morosanova, Bondarenko, 2017). Statistical processing of longitudinal data (including the latent growth curve modeling) made it possible to reveal the negative dynamics of the students&amp;rsquo; behavioral and cognitive engagement during their study in the secondary school. The data analysis allowed to describe the effects of relationship between behavioral and cognitive engagement: a higher level of cognitive engagement contributes to a less pronounced decrease in behavioral engagement. The study established positive correlations of conscious self-regulation with both cognitive and behavioral engagement. The decrease in engagement is less pronounced in adolescents with a higher level of development of conscious self-regulation.&lt;/p&gt;

Thalamic pathology in frontotemporal dementia: Predilection for specific nuclei, phenotype-specific signatures, clinical correlates, and practical relevance.

Frontotemporal dementia (FTD) phenotypes are classically associated with distinctive cortical atrophy patterns and regional hypometabolism. However, the spectrum of cognitive and behavioral manifestations in FTD arises from multisynaptic network dysfunction. The thalamus is a key hub of several corticobasal and corticocortical circuits. The main circuits relayed via the thalamic nuclei include the dorsolateral prefrontal circuit, the anterior cingulate circuit, and the orbitofrontal circuit. In this paper, we have reviewed evidence for thalamic pathology in FTD based on radiological and postmortem studies. Original research papers were systematically reviewed for preferential involvement of specific thalamic regions, for phenotype-associated thalamic disease burden patterns, characteristic longitudinal changes, and genotype-associated thalamic signatures. Moreover, evidence for presymptomatic thalamic pathology was also reviewed. Identified papers were systematically scrutinized for imaging methods, cohort sizes, clinical profiles, clinicoradiological associations, and main anatomical findings. The findings of individual research papers were amalgamated for consensus observations and their study designs further evaluated for stereotyped shortcomings. Based on the limitations of existing studies and conflicting reports in low-incidence FTD variants, we sought to outline future research directions and pressing research priorities. FTD is associated with focal thalamic degeneration. Phenotype-specific thalamic traits mirror established cortical vulnerability patterns. Thalamic nuclei mediating behavioral and language functions are preferentially involved. Given the compelling evidence for considerable thalamic disease burden early in the course of most FTD subtypes, we also reflect on the practical relevance, diagnostic role, prognostic significance, and monitoring potential of thalamic metrics in FTD. Cardinal manifestations of FTD phenotypes are likely to stem from thalamocortical circuitry dysfunction and are not exclusively driven by focal cortical changes.

Does TMT internationalization promote corporate digital transformation? A study based on the cognitive process mechanism

PurposeCorporate digital transformation (DT) and top management team (TMT) internationalization seem to be concomitant phenomena in recent years, the former is a major change and a thorough transformation requiring continuously new technologies and ideas. Excitingly, the latter shows a relatively broad vision, a large risk appetite and interest in new things. Does TMT internationalization promote DT? This question is essential for DT. Given that, this article will aim to discuss and examine whether and how TMT internationalization affects corporate DT.Design/methodology/approachThis article takes China's A-share listed manufacturing companies from 2011 to 2019 as a sample. The quantitative text analysis method is used to measure attention related to digitalization. This paper discusses: (1) The decision-making logic and cognitive process mechanism of “TMT internationalization–attention related to digitalization–corporate DT”. (2) The moderating effect of shared corporate mission of the TMT on the cognitive process of decision-making, that is, the social process of transforming individual cognition into team cognition, and the social process of transforming team cognition into corporate decision-making.FindingsTMT internationalization promotes DT. As an external manifestation of team cognition, attention plays a positive role as an intermediary mechanism. Specifically, executives with overseas experience have higher urgency assessment and manageable assessment, thus affecting their attention to digitalization positively, thereby promoting DT. This article does not demonstrate the moderating effect of shared corporate mission on the cognitive process, but it promotes DT directly, and only plays a role in the precognitive stage.Originality/valueThis article is the first one to study the relationship between TMT internationalization and corporate DT, which has practical guiding significance for DT and the “going out” strategies of the TMT. Also, the combination of upper echelons theory and cognitive theory opens up the black box of the strategic process. Lastly, this research explores the formation process of team cognition, which is always neglected by previous studies of the TMT demographic characteristics.

Study of etiological patterns and various clinical presentations of anemia in children aged 6 months to 5 years admitted in a tertiary care hospital

Background: Nutritional anemia (NA) is the commonest cause of anemia in children. Iron deficiency is the most important contributing factor to nutritional anemia. Severe iron deficiency is associated with impaired brain development along with cognitive, behavioural, and psychomotor manifestations, particularly during the first two years. This study aimed to evaluate the clinical and etiological profile of anemia in children aged 6 months to 5 years. Material and methods: Hospital-based observational study conducted on children between 6 months to 5 years of age, admitted to Government General Hospital, Srikakulam and having anemia according to WHO classification. Results: Of the 157 children diagnosed with anemia over 18 months period, iron deficiency anemia is the commonest cause of anemia, seen in 107 children followed by sickle cell anemia seen in 21 children. Out of the sampled children, 154 children recovered, and 3 children succumbed to death. Conclusion: Nutritional anemia, particularly iron deficiency anemia is the most common type of anemia in 6 months to 5 years-old children. Co-morbidities like malnutrition, parasitic infestations, diarrheal diseases, and recurrent respiratory tract infections form a vicious cycle and result in nutritional anemia. Identifying the factors that are leading to iron deficiency anemia and implementing the control measures like early iron supplementation results in reducing morbidity and mortality.