Acute Necrotizing Encephalopathy Associated with Coronavirus Disease 2019 in an Infant.

A 5-week-old infant born at term was diagnosed with acute necrotizing encephalopathy associated with severe acute respiratory syndrome coronavirus 2 as evidenced by clinical presentation, neuroimaging, and cerebrospinal fluid studies. Our patient was treated with high-dose intravenous methylprednisolone, tocilizumab, and intravenous immunoglobulin with significant short-term clinical improvement but long-term sequelae.

Age Effects on Women's and Men's Dyadic and Solitary Sexual Desire.

While most studies on sexuality in later life report that sexual desire declines with age, little is known about the exact nature of age effects on sexual desire. Using self-reported dyadic sexual desire relating to a partner, dyadic sexual desire relating to an attractive person, and solitary sexual desire from a large (N > 8000) and age diverse (14.6-80.2 years) online sample, the current study had three goals: First, we investigated relationships between men and women's sexual desire and age. Second, we examined whether individual differences such as gender/sex, sexual orientation, self-rated masculinity, relationship status, self-rated attractiveness, and self-rated health predict sexual desire. Third, we examined how these associations differed across sexual desire facets. On average, the associations between age and both men and women's sexual desire followed nonlinear trends and differed between genders/sexes and types of sexual desire. Average levels of all types of sexual desire were generally higher in men. Dyadic sexual desire related positively to self-rated masculinity and having a romantic partner and solitary desire was higher in people with same-sex attraction. We discuss the results in the context of the evolutionary hypothesis that predict an increase of sexual desire and female reproductive effort prior to declining fertility. Our findings both support and challenge beliefs about gender/sex specificity of age effects on sexual desire and highlight the importance of differentiating between desire types.

Cognitive phenotypes in childhood idiopathic epilepsies.

OBJECTIVE: The objective of this study was to identify cognitive phenotypes in children with new-onset focal and generalized idiopathic epilepsies and determine their relationship with epilepsy syndrome, brain structure, neurodevelopmental history, and family characteristics. METHODS: One hundred thirty-eight children with new-onset epilepsy and 95 controls (age: 8-18) underwent neuropsychological, clinical, and quantitative MR evaluations. Control participants' neuropsychological data were subjected to confirmatory factor analysis and then resultant factor scores were applied to participants with epilepsy and subjected to latent class analysis. Identified cognitive phenotypes were examined in relation to epilepsy syndrome, quantitative neuroimaging, and familial and neurodevelopmental variables. RESULTS: Confirmatory factor analysis identified five cognitive factors (verbal, perceptual, speed, attention, executive), and latent class analysis identified three clusters of participants with epilepsy: 1) average and similar to controls, 2) mild impairment across multiple cognitive domains, and 3) impairment across all domains with severe attentional impairment, representing 44%, 44%, and 12% of the epilepsy sample, respectively. Cognitive phenotype membership was not associated with epilepsy syndrome but was associated with increasing abnormalities in brain structure, parental IQ, and features of early developmental history. SIGNIFICANCE: Cognitive phenotypes are present in idiopathic childhood epilepsies that are unassociated with traditional epilepsy syndromes but are associated with measures of brain structure, family history, and neurodevelopmental features.

Is lower IQ in children with epilepsy due to lower parental IQ? A controlled comparison study.

AIM: The aim of this study was to determine the relationship between parent and child Full-scale IQ (FSIQ) in children with epilepsy and in typically developing comparison children and to examine parent-child IQ differences by epilepsy characteristics. METHOD: The study participants were 97 children (50 males, 47 females; age range 8-18y; mean age 12y 3mo, SD 3y1mo) with recent-onset epilepsy including idiopathic generalized (n=43) and idiopathic localization-related epilepsies (n=54); 69 healthy comparison children (38 females, 31 males; age range 8-18y; mean age 12y 8mo, SD 3y 2mo), and one biological parent per child. All participants were administered the Wechsler Abbreviated Scale of Intelligence (WASI). FSIQ was compared in children with epilepsy and typically developing children; FSIQ was compared in the parents of typically developing children and the parents of participants with epilepsy; parent-child FSIQ differences were compared between the groups. RESULTS: FSIQ was lower in children with epilepsy than in comparison children (p<0.001). FSIQ of parents of children with epilepsy did not differ from the FSIQ of the parents of typically developing children. Children with epilepsy had significantly lower FSIQ than their parents (p<0.001), whereas comparison children did not. The parent-child IQ difference was significantly higher in the group with epilepsy than the comparison group (p=0.043). Epilepsy characteristics were not related to parent-child IQ difference. INTERPRETATION: Parent-child IQ difference appears to be a marker of epilepsy impact independent of familial IQ, epilepsy syndrome, and clinical seizure features. This marker is evident early in the course of idiopathic epilepsies and can be tracked over time.

Brain development in children with new onset epilepsy: a prospective controlled cohort investigation.

PURPOSE: To characterize prospective neurodevelopmental changes in brain structure in children with new and recent-onset epilepsy compared to healthy controls. METHODS: Thirty-four healthy controls (mean age 12.9 years) and 38 children with new/recent-onset idiopathic epilepsy (mean age 12.9 years) underwent 1.5 T magnetic resonance imaging (MRI) at baseline and 2 years later. Prospective changes in total cerebral and lobar gray and white matter volumes were compared within and between groups. RESULTS: Prospective changes in gray matter volume were comparable for the epilepsy and control groups, with significant (p < 0.0001) reduction in total cerebral gray matter, due primarily to significant (p < 0.001) reductions in frontal and parietal gray matter. Prospective white matter volume changes differed between groups. Controls exhibited a significant (p = 0.0012) increase in total cerebral white matter volume due to significant (p < 0.001) volume increases in the frontal, parietal, and temporal lobes. In contrast, the epilepsy group exhibited nonsignificant white matter volume change in the total cerebrum (p = 0.51) as well as across all lobes (all p's > 0.06). The group by white matter volume change interactions were significant for total cerebrum (p = 0.04) and frontal lobe (p = 0.04). DISCUSSION: Children with new and recent-onset epilepsy exhibit an altered pattern of brain development characterized by delayed age-appropriate increase in white matter volume. These findings may affect cognitive development through reduced brain connectivity and may also be related to the impairments in executive function commonly reported in this population.

Standing out and not fitting in: The Dark Triad traits and social values.

We replicated and extended (N = 207) work on the social values (i.e., obedience, tradition, security, benevolence, universalism, self-direction, stimulation, hedonism, achievement, and power) linked to the Dark Triad traits (i.e., narcissism, psychopathy, and Machiavellianism). Each of the traits was positively associated with values of achievement and power. Psychopathy and narcissism were both negatively correlated with benevolence, and psychopathy and Machiavellianism were negatively correlated with obedience. Psychopathy was also negatively correlated with tradition. Sex differences in the values of tradition, benevolence, and power were mediated by psychopathy. We suggest that high rates of the Dark Triad traits facilitate, for men, holding social values that emphasize standing out whereas low rates facilitate, for women, fitting in.

The Psychological Science Accelerator: Advancing Psychology through a Distributed Collaborative Network.

Concerns have been growing about the veracity of psychological research. Many findings in psychological science are based on studies with insufficient statistical power and nonrepresentative samples, or may otherwise be limited to specific, ungeneralizable settings or populations. Crowdsourced research, a type of large-scale collaboration in which one or more research projects are conducted across multiple lab sites, offers a pragmatic solution to these and other current methodological challenges. The Psychological Science Accelerator (PSA) is a distributed network of laboratories designed to enable and support crowdsourced research projects. These projects can focus on novel research questions, or attempt to replicate prior research, in large, diverse samples. The PSA's mission is to accelerate the accumulation of reliable and generalizable evidence in psychological science. Here, we describe the background, structure, principles, procedures, benefits, and challenges of the PSA. In contrast to other crowdsourced research networks, the PSA is ongoing (as opposed to time-limited), efficient (in terms of re-using structures and principles for different projects), decentralized, diverse (in terms of participants and researchers), and inclusive (of proposals, contributions, and other relevant input from anyone inside or outside of the network). The PSA and other approaches to crowdsourced psychological science will advance our understanding of mental processes and behaviors by enabling rigorous research and systematically examining its generalizability.

Preoperative clinical evaluation and noninvasive electroencephalogram in cortical dysplasia.

The preoperative evaluation of patients with suspected cortical dysplasia involves detailed neurologic and neuropsychologic evaluations, electroencephalography, and comprehensive neuroimaging and functional studies. The goal is to identify a focal region of seizure onset and to assess for motor impairments, speech and language difficulties, or developmental delay. Although the electroencephalogram abnormalities in cortical dysplasia are nonspecific, they typically include unusual high-amplitude (slowing and focal) activity. The association of cortical dysplasia with cardiovascular, dermatologic, and other systemic disorders is discussed.

Chiari I malformation in parturients.

STUDY OBJECTIVE: To assess complications of regional as well as general anesthesia in parturients with Chiari I malformation. DESIGN: Retrospective chart review. SETTING: Academic medical center. PATIENTS: All parturients in our institution who had the diagnosis of Chiari I malformation and delivered in our hospitals over a 50-year period. MAIN RESULTS: 12 parturients delivered 30 babies. Three deliveries were facilitated with general anesthesia. Nine deliveries were facilitated with central axis anesthesia, six with epidural anesthesia, two with a single injection of a spinal anesthetic, and one with a continuous spinal catheter. The patient who received a continuous spinal catheter developed a postdural puncture headache that resolved with an epidural blood patch. None of the patients who received general, spinal, or epidural anesthesia for their deliveries developed symptoms or had exacerbation of preexisting symptoms of Chiari I malformation. CONCLUSIONS: General anesthesia, as well as spinal and epidural anesthesia, appeared to be safe and effective in our series of vaginal or cesarean delivery patients. The small number of patients in our series does not negate the cautious recommendations of others, but suggests that general anesthesia, as well as spinal or epidural anesthesia, can be used safely and effectively in these patients.

New syndrome: focal dermal hypoplasia, morning glory anomaly, and polymicrogyria.

Regional skin hypoplasia has been described in several genetic syndromes, including focal dermal hypoplasia (FDH), microphthalmia with linear skin defects (MLS), oculocerebrocutaneous syndrome (OCCS), and terminal osseous dysplasia and pigmentary defects (TODP). All but OCCS have been reported to follow an X-linked inheritance pattern. We describe a 14-year-old girl with clinical features overlapping with these disorders. She had mild mental retardation, macrocephaly, microphthalmia, right-sided morning glory optic disc anomaly, palmar and lip pits, and polysyndactyly. A swirling pattern of skin hypopigmentation, papular hypopigmented and herniated skin lesions reminiscent of FDH most prominent over her face, head, hands, and feet was evident. Brain magnetic resonance imaging (MRI) showed polymicrogyria (most severely in the perisylvian and mesial frontal regions), enlarged left lateral ventricle, partial agenesis of the corpus callosum, and optic nerve tumor on the right. Dermatopathologic examination of the skin lesions was consistent with basaloid follicular hamartomas. The skin and digit anomalies observed overlap with FDH, but polymicrogyria, basaloid follicular hamartomas, optic nerve tumor, and morning glory anomaly have not previously been described in FDH. Skin defects in MLS are linear and the eyes typically have sclerocornea. Polymicrogyria has been described in OCCS, but not in any of the other three syndromes. The limb anomalies in TODP are reductions rather than polysyndactyly. Skin defects are localized to the face, and digital fibromas usually occur. While significant overlap exists between all four of the syndromes discussed, we believe that the constellation of anomalies observed in this girl most likely comprises a newly recognized syndrome.