bluuebunny/crossref_metadata_2025_split huggingface dataset:
A-266 Neuropsychological Profile of a 5-Year-Old Boy with Gould Syndrome
Authors: Cap Caitlyn J , Manning Madeline , Ball Kayleen 
Published: August 2022

Abstract
Objective: Gould syndrome (COL4A1/A2-related disorders) is an extremely rare, genetic disorder characterized by cerebral vasculature defects, ocular dysgenesis, myopathy, and cerebral cortical abnormalities. Many individuals with COL4A1 mutations exhibit seizures, stroke, visual field defects, and hydrocephalus. To date, research regarding neuropsychological sequelae of Gould syndrome has been sparse. Therefore, this presentation aims to present clinical considerations for pediatric patients with Gould syndrome.
Method: Patient is a 5-year-old Hispanic boy born at 35-weeks following perinatal stroke, pre-term birth, and suspected cyanosis. After birth, genetic testing revealed a COL4A1p.G1234R gene mutation (i.e., Gould syndrome). Patient’s medical history is also notable for cerebral palsy, epilepsy, encephalopathy, hypertonia, global developmental delays with language regression, and attention-deficit/hyperactivity disorder. Previous brain MRIs have found decreased white matter density in both cerebral hemispheres. Abbreviated neuropsychological testing was completed during an inpatient pre-surgical epilepsy evaluation. Patient was non-verbal.
Results: Non-verbal intellectual ability was Below Average (IQ=74). Receptive language was Average (SS=93). Further, bilateral fine-motor speed and dexterity and visual motor integration were Exceptionally Low. Standardized questionnaires revealed ongoing concerns related to attention, hyperactivity, and poor adaptive functioning. Diagnostic impressions included Global Developmental Delay.
Conclusion: Gould syndrome can include a variety of neurological sequelae depending on neurological defects and incidents. These findings are consistent with the “early vulnerability” position of brain development, which proposes that earlier age at brain insult may result in greater impairments in cognitive functioning (Jacomb et al., 2018). Therefore, early intervention and targeted long-term interventions appears imperative to improve function outcomes for pediatric patients with Gould syndrome.
A-268 Personal Adjustment of Young Adult Survivors of Leukemia/Lymphoma: Examining the Association with Cognition and Sex
Authors: Balderrama Melissa , Whitaker Ashley M , Mendoza Leanne 
Published: August 2022

Abstract
Objective: Survivors of pediatric acute lymphoblastic leukemia/non-Hodgkin’s Lymphoma (ALL/NHL) are at risk of cognitive sequelae of treatment, as well as psychosocial distress. The objectives of this study were to (1) describe self-perceptions of personal adjustment in young adult survivors of pediatric ALL/NHL and (2) examine associations between personal adjustment, cognition, and sex.
Method: Thirty-five young adult (x ̅=19.4 years), primarily Latino, survivors of ALL/NHL underwent neuropsychological evaluation, which included assessment of self-reported personal adjustment (Behavior Assessment System for Children – Third Edition [BASC-3]), working memory, processing speed, and sustained attention.
Results: Personal adjustment was approximately 0.5 standard deviations below normative expectations (mean T=45.5), with over 28% of the sample endorsing concerns in this area (compared to 18% of the normative population). Self-reliance was the lowest personal adjustment subscale (mean T=42.2), with nearly half (49%) endorsing concerns (compared to 15-21% of the normative population). There were no significant correlations between cognitive measures and personal adjustment subscales. Females endorsed a higher number of elevated personal adjustment subscales compared to males, t(33)=1.99, p=.027, which parallels their poorer working memory performance, t(36)=-2.14; p=.02.
Conclusion: Young adult survivors of ALL/NHL are at greater risk of concerns regarding personal adjustment than peers, particularly lower self-reliance. While there does not appear to be a direct relationship between cognition and personal adjustment in this group, females reported more areas of concern and demonstrated worse working memory, confirming their particular vulnerability to long-term cognitive and psychosocial sequelae.
A-269 Sex Differences in the Behavioral Profile of Adolescents with Autism Spectrum Disorder
Authors: Joyce Cali , Jacobowitz Jordan 
Published: August 2022

Abstract
Objective: Literature suggests discrepant prevalence rates between males and females with autism spectrum disorder (ASD) may be due to sex-specific ASD phenotypes. This study sought to identify sex-specific behavioral profiles for adolescents with ASD, as measured by the Autism Diagnostic Observation Schedule (ADOS-2). Sex differences in intellectual ability for individuals with ASD were also examined.
Method: A sample of adolescents with ASD across the United States was retrieved from the National Database for Autism Research (NDAR) (N=80, 65 males, 15 females; M age=12.17 years, SD=.97). Participants were included within the age parameters (11 years, zero months to 16 years, 11 months) with data on Wechsler Intelligence Scale for Children (WISC-V) and ADOS-2. The study was a cross-sectional design analyzing the relationship between biological sex and intellectual abilities, overall autism severity, and deficits within social affect and repetitive and restricted behaviors and interests (RRBIs).
Results: Significant sex differences were found in total severity of ASD after controlling for IQ, F(1,77)=4.59, p=.04. Adjusted marginal means demonstrated that males have higher ADOS total scores [M =13.37; 95% CI (12.47,14.34)] than females [M=11.13; 95% CI (9.33,12.98)]. No sex differences were found within the domain of intellectual ability, t(78)=.34, p= .738, or across the linear combination of the dependent variables of social affect and RRBIs, F(2,76)=2.95, p=.058.
Conclusions: This study provides further evidence that males are diagnosed with ASD at higher rates than females and suggests males present with more severe ASD pathology than females on the ADOS-2. Additional analyses are required to understand the full extent of clinical implications.
A-271 Assessment of Autistic Filipino-Americans using the ECLECTIC Model
Authors: Romero Regilda Anne , Ordonez Cherry , Dulay Mario , Agbayani Kristina 
Published: August 2022

Abstract
Objective: Autism Spectrum Disorder is a neurodevelopmental condition characterized by difficulties with social communication and restricted/repetitive behaviors. It is reported to occur in all racial and ethnic groups; however, literature on assessing autistic Filipino-Americans is scarce. Therefore, multicultural assessment guidelines are imperative in the diagnostic process as there are specific cultural factors that should be considered such as perception on disabilities, cultural values, acculturation level, etc. We discuss the use of the ECLECTIC model in assessing a US-born Filipino-American child of immigrant parents.
Methods: In addition to commonly used assessment battery, the following theoretical constructs were implemented to asses this case: Education, Culture and Acculturation, Language and Language Proficiency, Economics, Communication, Testing Situation, Intelligence Concept, and Context of Immigration. For instance, the family’s cultural and educational backgrounds were taken into consideration. Additionally, the patient’s “fluency in Japanese,” which caregivers’ thought was evidence of multilingual skills, was in fact stereotyped speech.
Results: The evaluation revealed significant weakness in reasoning, academic skills, and adaptive skills. All autism-related measures indicated autism. Further, results revealed that his expressive communication was limited. Of note, what the family thought was functional communication in another language was actually delayed echolalia from videos.
Conclusions: Cultural factors significantly affected this family’s perception of the patient’s behaviors and social interactions. Using a cultural lens is essential in autism assessments because of the strong relationship between social cognition/behaviors and culture. Additionally, assessment will enhance clinical formulation and help identify potential biases that may interfere with appropriate autism diagnosis and effective recommendations, particularly in the Filipino-American community.
A-273 MRI White Matter Hyperintensities and Neuropsychological Performance in a Young Adult Clinical Sample
Authors: Paredes Jory , Daniel Michael 
Published: August 2022

Abstract
Objective: Investigate the association of white-matter hyperintensities (WMH) on routine clinical MRI and neuropsychological test performance in a young adult clinical sample referred for neuropsychological assessment.
Method: From a sample of 607 patients that underwent comprehensive neuropsychological evaluation, two groups were selected based on MRI results: 1) normal (n = 50 (30 females), Mage = 46.20, Medu = 15.16) and 2) WMH without other MRI abnormality (n = 35 (20 females), Mage = 47.83, Medu = 14.24). Exclusion criteria included dementia, other brain neuropathology (e.g. stroke, neurodegeneration, traumatic brain injury, multiple sclerosis, etc.), below normal scores on performance validity tests, and age &gt; 59 years.
Results: Independent samples t-tests revealed statistically significant group differences for: Block Design (d=.55), Digit Span Backward (d=.49), Spatial Span Backward (d=.49), DKEFS Trails Number (d=.78), DKEFS Trails Letter (d=.59), and Matrix Reasoning (d=.52); although, means for both groups were within the average range for all neuropsychological tests. Using logistic regression analyses, odds ratios were calculated for a person with WMH having an impaired index score using two z-score cut-offs: -1.67 and -1.00. WMH were associated with an index z-score &lt; -1.00 on Matrix Reasoning (OR = 8.44, 95% CI: 1.69-42.22). A trend was near statistical significance (p= .06) at the -1.00 z-score cut-off on attention/processing speed tests (OR = 3.19, 95% CI: .93-16.39). Results for -1.67 cut-off were not significant.
Conclusions: These findings indicate that even in younger adults, WMH are associated with increased probability of mild inefficiency/decline in problem-solving/reasoning, and may be associated with mild decline in attention/processing speed.
A-275 Distinguishing Regional Cerebral Blood Flow between Posttraumatic Stress Disorder and Attention-Deficit/Hyperactivity Disorder in the Child Population
Authors: Ramrattan Aruna , Metsky Jake , Arias Brittny , Golden Charles J , Willheumier Kristen , Amen Daniel 
Published: August 2022

Abstract
Objective: This study aimed to examine the regional cerebral blood flow (rCBF) of several brain regions to evaluate differences between Post-Traumatic Stress Disorder (PTSD) and Attention-Deficit/Hyperactivity Disorder (ADHD).
Method: The sample consisted of PTSD patients (n=89) with a mean age of 12.10 years, SD=3.88, and ADHD patients (n=2568) with a mean age of 12.19 years, SD=3.39. To test the hypothesis that rCBF within these two disorders are distinguishable, one-way ANOVAs were run with all 17 brain regions in the SPECT Child Database.
Results: Tests revealed statistical significance (increased rCBF (p&lt;.001)) for PTSD bilaterally in the limbic system (baseline and concentration), bilaterally in the basal ganglia (baseline and concentration), and in the vermis (baseline and concentration). Statistical significance (p&lt;.001) was indicated for ADHD bilaterally in the cerebellum (concentration), right-hemisphere parietal (baseline and concentration), and right-hemisphere temporal (baseline and concentration).
Conclusions: While results indicated increased rCBF between various brain regions, this study also indicated two general regions that may significantly characterize each disorder to help us conceptualize neuropsychological functioning more meaningfully. Bilaterally, the limbic region generally produced greater rCBF in PTSD participants compared to ADHD; bilaterally, the cerebellum generally produced greater rCBF within ADHD participants. Literature correlates our limbic region with emotion, learning, memory, aggression and is particularly associated with anxiety disorders. The cerebellum is associated with refining fine movements initiated by the motor cortex. As impulsivity is a key criterion in ADHD, we may surmise that higher rCBF activity in the cerebellum results from increased efforts in controlling movements and coordination. Additional investigation is necessary.
A-278 Neuropsychological Profile of a Child with KCNQ2
Authors: Hershkovich Arielle , Weiss Erica F , McGinley John , Bronshteyn Diana , Masur David , Facchini Ronda 
Published: August 2022

Abstract
Objective: The KCNQ2 gene mutations (KCNQ2), a known cause of neuronal potassium channel dysfunction in brain cells, causes susceptibility to seizures beginning within the first days of life. Seizure semiology, severity and cognitive sequela can vary depending on the type of KCNQ2 mutation. Cognitive impact ranges from moderate to severe; no known consistent presentation. KCNQ2 affects approximately 2.8 in 100,000 births: over 3,000 annual cases worldwide. We describe the neuropsychological findings of an 11-year-old female with a monoallelic mutation of the KCNQ2 gene, and history of seizures. EEG indicated generalized epilepsy syndrome with a right occipital focal area of dysfunction. We highlight the importance of neuropsychological evaluation in medical and psychological management of children with KCNQ2.
Methods: Medical history includes multifocal seizures beginning at 2 days old and significant anxiety, both managed with medication, language delay, and a diagnosis of KCNQ2 at age 9. A comprehensive neuropsychological evaluation was conducted to assess slower processing speed, behavioral concerns, and anxiety.
Results: Evaluation revealed low average overall cognition and average academic performances, with strengths in verbal comprehension, fluid reasoning, and written expression. Cognitive processing speed was atypical, with slow processing speed affecting her performance across multiple domains. Behavior assessment supports Generalized Anxiety Disorder.
Conclusion: This case of a girl with KCNQ2 demonstrated an atypical cognitive and behavioral profile, contributing to our limited knowledge of how KCNQ2 presents. The patterns of her performances add to the literature of the KCNQ2 gene mutation and raise important questions regarding the role of KCNQ2 in manifestations of cognitive and behavioral symptoms.
A-279 Progressive Neuropsychological Deficits Secondary to an Acute Disseminated Encephalomyelitis Attack 11 Years Later Complicated by Possible New Pathology
Authors: Khan Mohammadi , Phillips Matthew S , Yang Jessica , Fink Joseph 
Published: August 2022

Abstract
Objective: Acute Disseminated Encephalomyelitis (ADEM) often presents as an abrupt, brief, and intense attack causing widespread inflammation and demyelination to the central nervous system. Most patients typically make a complete neurological recovery; however, a few demonstrate long-term, persistent impairments.
Method: A 64-year-old white female presented for a neuropsychological evaluation (2021) following a progressive one-year decline in her memory and motor abilities, which per family, reportedly overlay persisting residual deficits from an ADEM attack she had in 2010. Nearly 11 years following the attack, she reported continued reliance on her family in completing most of her instrumental, and even some basic activities of daily living since the attack. Initial neuroimaging following the attack (2010) revealed extensive bilateral white matter (WM) lesions, whereas current neuroimaging (2021) (despite not explicitly commenting on the resolution status of her lesions), continued to reveal widespread hyperintense signals in WM regions, progressing cerebral atrophy, and the possibility of superimposed normal pressure hydrocephalus (i.e., ventriculomegaly).
Results: Her neuropsychological profile (with no prior baseline) was marked by substantial impairments in immediate and delayed verbal/visual memory, attention, language, motor abilities, visuospatial skills, executive functions, along with mild anxiety.
Conclusions: This neurocognitive profile displayed atypical findings in patient recovery following an ADEM attack. Given the test results and the continued presence of extensive hyperintense signals in WM regions, it is possible her initial brain lesions (due to ADEM) remained unresolved. This, along with the possible intervening effects of new pathology (e.g., NPH and possible vascular changes) are likely exacerbating her existing deficits related to ADEM.
A-282 Verbal Fluencies are Differentially Associated with Processing Speed in Temporal Lobe Epilepsy
Authors: Stanek Kelly M , Park Yong D , Murro Anthony M , Moore-Hill Debra , Vale Fernando 
Published: August 2022

Abstract
Objective: The current study sought to better understand the impact of processing speed on pre-surgical assessment of verbal fluencies in temporal lobe epilepsy (TLE) by examining whether processing speed is differentially related to category and letter fluencies across patients with left and right unilateral TLE.
Method: The retrospective data sample included 36 adults aged 17-60 (56% female) with cryptogenic TLE and both video EEG evidence for unilateral seizure focus (right TLE n =16; left TLE n=20) and confirmed left hemisphere language dominance, who had undergone pre-surgical neuropsychological evaluation including assessment of category fluency (Animal Naming), letter fluency (FAS), and processing speed (Coding). Primary partial correlation analyses controlled for age and years of education.
Results: After controlling for demographic variables in each of the following analyses, there was a statistically significant relationship between Coding and Animal Naming (r=.47, p&lt;.01) but not FAS in the full sample of TLE patients. Similarly in the left TLE group, there was a statistically significant relationship between Coding and Animal Naming (r=.47, p&lt;.05) but not FAS. In the right TLE group, neither Animal Naming nor FAS were statistically significantly related to Coding.
Conclusion: Results suggest that processing speed may have a greater influence on measurement of category as opposed to letter fluency in individuals with left TLE but not right TLE. While further research in larger samples is indicated, a better understanding of these relationships is important in assessing the lateralizing/localizing value of semantic and phonemic fluencies in pre-surgical neurocognitive profiles of patients with unilateral TLE with impaired processing speed.
A-285 Malingering of Executive Function in Traumatic Brain Injury
Authors: Revels-Strother Olivia , Suhr Julie 
Published: August 2022

Abstract
Objective: The current study explored whether the Delis-Kaplin Executive Functioning System (D-KEFS) Stroop test and Barkley Deficits in Executive Function System (BDEFS) measure are vulnerable to malingering and whether cutoffs on each could be identified to detect malingering.
Method: Using a simulator design, students recruited from the psychology participant pool at Ohio University (Mage=21.20, SD=5.85; 77.3% white, 52% freshmen, 68% assigned female, 12% prior ADHD diagnosis, 20% history of mild TBI, and 38.7% current psychological diagnoses) were randomly assigned to either malinger (simulator condition) Traumatic Brain Injury or perform normally (control condition). Participants were virtually administered the Test of Pre-Morbid Functioning, the D-KEFS, Test of Memory Malingering, and BDEFS. They also completed Qualtrics adherence items. Participants were excluded based on their Qualtrics response and for insufficient data.
Results: As hypothesized, malingerers scored worse than controls on both measures. The Inhibition subtest (both raw and scaled scores) of the D-KEFS Stroop discriminated simulators from controls and a cutoff for the raw score was identified that detected 48% of simulators, with 90% specificity. The BDEFS total score also discriminated malingerers from controls, and a cutoff was identified that detected 56% of simulators, with 90% specificity. Supplemental chi square analysis showed poor congruence between the two measures, p=.262.
Conclusions: The present study provides support for the potential utility of the D-KEFS Inhibition raw scores and BDEFS total scores as indicators of malingered executive functioning in TBI. Overall, results support the need to include both Performance Validity Tests and Symptom Validity Tests in assessment.
A-286 How Far We’ve Come: Promoting Resilience Following Significant Early Life Neurological Insult
Authors: Manning Madeline C , Cap Caitlyn J , Naidoo Reshma B 
Published: August 2022

Abstract
Objective: Despite empirical data indicating continued and increased negative neurocognitive effects of neurological insult due to early life exposure to brain resections, refractory seizures, and chemoradiotherapy, it is possible for patients to rewrite their narratives and demonstrate resilience and terrific response to rehabilitation. A longitudinal, single case study demonstrating the positive impact of therapeutic intervention is presented here.
Method: Patient is a 14-year-old Hispanic female diagnosed with atypical Teratoid Rhabdoid Tumor (ATRT) at age 2 was treated as per Dana Farber ATRT protocol, including brain resection and chemoradiation treatment. Neurological and cognitive complications due to treatment and late effects were observed. Patient also underwent a right temporal lobectomy (age 11) which controlled her refractory seizures and cognitive regression. Patient subsequently received intensive neurorehabilitation and serial neuropsychological assessments (age 4, 7, 10, 14).
Results: Early assessments (ages 4 and 7) indicated a decline in neurocognitive performance (exceptionally low range across domains). More recent assessments (ages 10 and 14), however, demonstrated a change in trajectory (ranging from low average to exceptionally low). The greatest growth was observed in verbal and quantitative reasoning, as well as executive functioning abilities.
Conclusions: Even for children who have had multiple, chronic, insidious early life neurocognitive insults, intensive and directive therapies can alter their trajectory and allow steady gains to be made. These findings highlight the importance of taking a biopsychosocial approach for informing therapy led by a strong treatment team. It is critical to integrate the strengths of the individual, family, treatment team, and environment in supporting the patient-family’s treatment goals.