Is it cognitive effort you measure? Comparing three task paradigms to the Need for Cognition scale.

Measuring individual differences in cognitive effort can be elusive as effort is a function of motivation and ability. We report six studies (N = 663) investigating the relationship of Need for Cognition and working memory capacity with three cognitive effort measures: demand avoidance in the Demand Selection Task, effort discounting measured as the indifference point in the Cognitive Effort Discounting paradigm, and rational reasoning score with items from the heuristic and bias literature. We measured perceived mental effort with the NASA task load index. The three tasks were not correlated with each other (all r's < .1, all p's > .1). Need for Cognition was positively associated with effort discounting (r = .168, p < .001) and rational reasoning (r = .176, p < .001), but not demand avoidance (r = .085, p = .186). Working memory capacity was related to effort discounting (r = .185, p = .004). Higher perceived effort was related to poorer rational reasoning. Our data indicate that two of the tasks are related to Need for Cognition but are also influenced by a participant's working memory capacity. We discuss whether any of the tasks measure cognitive effort.

Central vein sign and other radiographic features distinguishing myelin oligodendrocyte glycoprotein antibody disease from multiple sclerosis and aquaporin-4 antibody-positive neuromyelitis optica.

BACKGROUND: Myelin oligodendrocyte glycoprotein antibody disease (MOGAD) can radiographically mimic multiple sclerosis (MS) and aquaporin-4 (AQP4) antibody-positive neuromyelitis optica spectrum disorder (NMOSD). Central vein sign (CVS) prevalence has not yet been well-established in MOGAD. OBJECTIVE: Characterize the magnetic resonance imaging (MRI) appearance and CVS prevalence of MOGAD patients in comparison to matched cohorts of MS and AQP4+ NMOSD. METHODS: Clinical MRIs from 26 MOGAD patients were compared to matched cohorts of MS and AQP4+ NMOSD. Brain MRIs were assessed for involvement within predefined regions of interest. CVS was assessed by overlaying fluid-attenuated inversion recovery (FLAIR) and susceptibility-weighted sequences. Topographic analyses were performed on spinal cord and orbital MRIs when available. RESULTS: MOGAD patients had fewer brain lesions and average CVS+ rate of 12.1%, compared to 44.4% in MS patients (p = 0.0008). MOGAD spinal cord and optic nerve involvement was lengthier than MS (5.8 vs 1.0 vertebral segments, p = 0.020; 3.0 vs 0.5 cm, p < 0.0001). MOGAD patients tended to have bilateral/anterior optic nerve pathology with perineural contrast enhancement, contrasting with posterior optic nerve involvement in NMOSD. CONCLUSION: CVS+ rate and longer segments of involvement in the spinal cord and optic nerve can differentiate MOGAD from MS, but do not discriminate as well between MOGAD and AQP4+ NMOSD.

Clinical and laboratory features distinguishing MOG antibody disease from multiple sclerosis and AQP4 antibody-positive neuromyelitis optica.

BACKGROUND: Antibodies to myelin oligodendrocyte glycoprotein (MOG) are associated with a CNS inflammatory disorder distinct from multiple sclerosis (MS) and aquaporin-4 antibody-positive neuromyelitis optica (NMO). Knowledge of the clinical spectrum of MOG antibody disease (MOGAD) remains incomplete, particularly in comparison to two related inflammatory demyelinating diseases, MS and NMO. OBJECTIVE: Compare demographics, clinical characteristics, estimated disability, laboratory results, and treatment responses of a U.S. MOGAD cohort with age- and sex-matched MS and NMO patients. DESIGN, SETTING, AND PARTICIPANTS: This observational, case-control, single-center study identified each group via ICD-10 diagnosis code searches through the electronic medical records of adult patients seen at the John L. Trotter MS Center between January 1, 2019 and January 1, 2020. MOGAD and NMO patients were confirmed to have at least one positive antibody test; those in the MS group had a confirmed diagnosis by a physician with MS subspecialty training. Data were collected after IRB approval. RESULTS: Twenty-six patients were included in each group. MOGAD patients were predominantly Caucasian (88.5%) with mean onset age of 43.9 years. MOGAD patients had no comorbid other autoimmune diseases and comparatively lower rates of family members with autoimmune disease (20.0%) than either MS (40.0%) or NMO (34.6%) matched cohorts. 91% of MOGAD attacks were monofocal, and over 70% presented with optic neuritis. Severity of MOGAD attacks was similar to that of seropositive NMO, but the robust degree of recovery was more similar to MS. Four MOGAD patients converted to negative antibody status, with no attacks occurring after conversion. Serum ANA and ENA were less frequently elevated in MOGAD (21.7%, 5.0%) than in seropositive NMO patients (66.7%, 42.9%). Elevated IgG synthesis rate and positive CSF-restricted oligoclonal bands were not seen in our MOGAD cohort, and only one MOGAD patient had an elevated IgG index. Despite anti-CD20 therapy, 28.6% of MOGAD patients continued to suffer relapses. CONCLUSIONS: MOGAD was characterized by a predominantly monofocal presentation (typically optic neuritis) and severe attacks with better recovery than seen with seropositive NMO attacks. Lack of CSF-restricted oligoclonal bands distinguished MOGAD from MS.

Adaptive functioning in children with neurofibromatosis type 1: relationship to cognition, behavior, and magnetic resonance imaging.

AIM: To characterize the adaptive behavior profile of children with neurofibromatosis type 1 (NF1) and determine its relationship to neuropsychological functioning and non-neoplastic T2-weighted hyperintense brain lesions on brain magnetic resonance imaging (MRI). METHOD: In this cross-sectional study, we retrospectively reviewed neuropsychological reports from 104 children with NF1 (56 males, 48 females; mean age 10y 4mo; standard deviation [SD] 3y 4mo; range 3y 5mo-17y 6mo), and extracted data from a range of cognitive and behavioral measures, including the Adaptive Behavior Assessment System (ABAS). Brain MRI was retrospectively reviewed in 42 individuals. RESULTS: Adaptive Behavior Assessment System scores were continuously distributed and pathologically shifted by 0.79 to 1.26SD across Conceptual, Social, and Practical domains, and 46.5% of individuals had a composite score in the borderline or impaired range. Impairment in adaptive functioning was correlated with deficits in executive function (r=-9.543, p<0.001), externalizing problems (r=-0.366, p<0.001), and attention (r=-9.467, p=0.001). Cluster analysis revealed three distinct phenotypic subgroups, one of which exhibited normal cognitive ability, but impaired adaptive functioning, with persistent deficits in executive function, behavioral problems, and attention-deficit/hyperactivity disorder symptomatology. There was no relationship between ABAS scores and the number or location of unidentified bright objects. INTERPRETATION: Adaptive functioning deficits are common among children with NF1 and are associated with impairment in other cognitive/behavioral domains, independent of general cognitive ability. WHAT THIS PAPER ADDS: Deficits in adaptive behavior are common in children with neurofibromatosis type 1 (NF1). Poor adaptive functioning is associated with impairments in executive function, externalizing behaviors, and attention, regardless of cognitive ability. The presence or location of unidentified bright objects do not predict adaptive behavior skills in children with NF1.

FUNCIONAMIENTO ADAPTATIVO EN NIÑOS CON NEUROFIBROMATOSIS TIPO 1: RELACIÓN ENTRE COGNICIÓN, COMPORTAMIENTO E IMÁGENES DE RESONANCIA MAGNÉTICA: OBJETIVO: Caracterizar el perfil del comportamiento adaptativo de niños con neurofibromatosis tipo 1 (NF1) y determinar la relación entre el funcionamiento neuropsicológico y las lesiones hiperintensas cerebrales no neoplásicas en T2-pesado de la resonancia magnética cerebral (RM). MÉTODO: En este estudio transversal, revisamos de forma retrospectiva reportes neuropsicológicos de 104 niños con NF1 (56 varones, 48 mujeres, media de edad 10 años 4 meses; desviación estándar (DE) 3 años 4 meses; rango 3 años 5 meses a 17 años 6 meses), y se extrajeron datos de una serie de mediciones cognitivas y conductuales, incluyendo el test sistema de evaluación de la conducta adaptativa (Adaptative Behaivor Assesment System ABAS). Se revisaron 42 RM cerebrales de forma retrospectiva. RESULTADOS: Los resultados ABAS fueron continuamente distribuidos y se cambiaron patológicamente entre 0,79 a 1,26 DE en los dominios de lo conceptual, social y práctico, y 46,5 por ciento de los individuos tuvieron un puntaje limítrofe o sin afectación. La afectación en las funciones adaptativas fue correlacionada con los déficits en funciones ejecutivas (r = -9,543, p < 0,001), externalizar problemas (r = -0,366, p < 0,001), y atención (r = -9,467, p = 0,001). El análisis de grupo revelo tres subgrupos fenotípicos distintos, uno de ellos exhibía una habilidad cognitiva tipica, pero afectación en el funcionamiento adaptativo, con déficits persistentes en función ejecutiva, problemas conductuales, y sintomatología de déficit de atención/hiperactividad. No hubo relación entre el puntaje ABAS y el número o localización de imágenes brillantes no identificadas en la RM cerebral. INTERPRETACIÓN: Los déficits de funcionamiento adaptativo son comunes entre niños con NF1 y son asociados con afectación de otros dominios cognitivo/conductual, independiente de la habilidad cognitiva en general.

FUNCIONAMENTO ADAPTATIVO EM CRIANÇAS COM NEUROFIBROMATOSE TIPO 1: RELAÇÃO COM COGNIÇÃO, COMPORTAMENTO, E IMAGEM DE RESSONÂNCIA MAGNÉTICA: OBJETIVO: Caracterizar o comportamento adaptativo de crianças com neurofibromatose tipo 1 (NF1) e determinar sua relação com funcionamento neuropsicológico e lesões em T2 hiperintensas não neoplásticas ao exame de ressonância magnética (RM). MÉTODO: Neste estudo transversal, revisamos retrospectivamente os relatórios neuropsicológicos de 104 crianças com NF1 (56 do sexo masculino, 48 do sexo feminino; média de idade 10a 4m; desvio padrão [DP] 3a 4m; variação 3a 5m-17a6m), e extraímos dados de uma variedade de medidas cognitivas e comportamentais, incluindo o Sistema de Avaliação do Comportamento Adaptativo (SACA). Imagens de RM cerebral foram retrospectivamente revisadas em 42 indivíduos. RESULTADOS: Os escores SACA foram distribuídos continuamente, e patologicamente deslocados em 0,79 a 1,26 DP nos domínios Conceitual, Social e Prático, e 46,5 por cento dos indivíduos tiveram escore composto na faixa limítrofe ou deficiente. Deficiências no comportamento adaptativo se correlacionaram com déficits na função executiva (r = −9,543, p < 0,001), problemas externalizantes (r = −0,366, p < 0,001), e atenção (r = −9,467, p = 0,001). Análise agrupada revelou três subgrupos genotípicos distintos, um dos quais exibiu capacidade cognitiva normal, mas funcionamento adaptativo deficiente, e sintomatologia de transtorno de deficit de atenção e hiperatividade. Não houve relação entre escores SACA e o número ou localização de objetos luminosos não identificados. INTERPRETAÇÃO: Déficits no funcionamento adaptativo são comuns entre crianças com NF1 e são associados com deficiência em outros domínios cognitivos/comportamentais, independente da capacidade cognitiva geral.