Robert Perna

Executive functioning and adaptive living skills after acquired brain injury.

Executive dysfunction is common following brain injury, with impairments involving attention, social pragmatics, higher-order thinking, judgment, and reasoning. Executive function impairments may have a direct impact on an individuals ability to return to instrumental activities of daily living (IADL), including employment, money management, driving, and maintaining a residence. Research has shown that neuropsychological executive function measures may be able to predict daily-living skills. There is limited research evaluating the relationship between executive functions and IADLs in adults with acquired brain injuries (ABI), with none investigating levels of proficiency as related to specific test scores. We hypothesize that neuropsychological executive function measures will have significant and moderate-to-strong correlations with participant-rated proficiency on functional tasks as measured by the Mayo-Portland Adaptability Inventory. Results support that IQ and some of the executive function measures (Processing Speed, Working Memory, and Trail-Making Test-Part B) correlated significantly and strongly and explained unique variance in all IADLs in this study. Data suggest that individuals with ABI who performed in the higher end of the low-average range or higher on measures of executive functioning tend to require little or no assistance to be independent with transportation, money management, living without support, and employment. Results also suggest that individuals with less executive dysfunction are likely to have greater overall community participation.

The value of the wechsler intelligence scale for children-fourth edition digit span as an embedded measure of effort: an investigation into children with dual diagnoses.

The Test of Memory Malingering (TOMM) is a measure of test-taking effort which has traditionally been utilized with adults, but which more recently has demonstrated utility with children. The purpose of this study was to investigate whether the Wechsler Intelligence Scale for Children-Fourth Edition (WISC-IV) Digit Span, commonly used in neuropsychological evaluations, can also be functional as an embedded measure by detecting effort in children with dual diagnoses; a population yet to be investigated. Participants (n = 51) who completed neuropsychological evaluations including the TOMM, WISC-IV, Wisconsin Card Sorting Test, Childrens Memory Scale, and Delis-Kaplan Executive Function System were divided into two groups: Optimal Effort and Suboptimal Effort, based on their TOMM Trial 2 scores. Digit Span findings suggest a useful scaled score of ≤4 resulted in optimal cutoff scores, yielding specificity of 91% and sensitivity of 43%. This study supports previous research that the WISC-IV Digit Span has good utility in determining optimal effort, even in children with dual diagnosis or comorbidities.

Long-Term Cognitive Sequelae: Abused Children Without PTSD

Many lines of research suggest that childhood abuse and neglect are associated with later developing psychiatric diagnoses, academic problems, cognitive difficulty, and possible brain changes as measured through brain imaging. Data were collected on children (N = 41) who completed a neuropsychological evaluation. Of those evaluated, 18 had a documented history of physical and/or emotional abuse or significant neglect and 23 had no history of abuse/neglect. When controlling for Full-Scale IQ (FSIQ), the abused children had significantly lower scores on measures of executive functioning (Wisconsin Card-Sorting Test-Categories, Maintenance of Set, and Perseveration and Wechsler Intelligence Scale for Children-Fourth Edition Working Memory), and effect sizes were large for these variables. Neither group had any test scores significantly lower than their FSIQ. Cross-tabulation analyses showed that the abused children were more likely to subsequently be diagnosed with a behavioral or emotional disorder. Consistent with psychobiological theories and imaging studies, our data are suggestive that childhood abuse and neglect are associated with later development of behavioral and emotional disorders and areas of cognitive weakness and possible impairment. Future research may be conducted to clarify these effects, the possibility of a dose–effect relationship, and timing of possible critical periods of brain vulnerability.

Test of memory malingering with children: The utility of Trial 1 and TOMMe10 as screeners of test validity

There is a growing body of research suggesting that the shorter versions of the Test of Memory Malingering (TOMM) may provide an accurate assessment of effort in children. During neuropsychological evaluations, some circumstances result in only one completed trial of the TOMM or partial completion of a trial. Research suggests that a cut-score of 40 or 41 on Trial1 is highly predictive of passing the TOMM overall. In the current study, 194 school-age children with academic and/or behavioral problems were used to compare the accuracy of TOMM1 and TOMMe10 (errors on the first 10 items of TOMM1) in predicting passing/failing of TOMM2. For the children in this sample, a score of < 40 items correct (≥ 10 errors) on TOMM1 was highly accurate in predicting a passing performance on the TOMM2 (sensitivity = .80, specificity = .91) with a Negative Predictive Value = .98 at the malingering base rate of 7% (TOMM2 failure in our sample). A score of 2 errors (8 items correct) on the TOMMe10 was slightly less sensitive than that of the TOMM1 (specificity = .96, sensitivity = .53) but with a similar Negative Predictive Value (.96). Consistent with the research from adult populations, TOMM1 and TOMMe10 appear to be quite accurate in predicting performance on the standard administration of the TOMM and may be useful screeners. However, compared to that found in adult samples, slight differences in suggested cutoffs for TOMM1 and TOMMe10 may be warranted for children.

Anti-N-Methyl-D-Aspartate Receptor Encephalitis: A Review and Neuropsychological Case Study

Objective: Anti-N-Methyl-d-Aspartate Receptor (NMDAR) Encephalitis is an autoimmune-mediated encephalitis, which may be associated with a tumor, which occurs when antibodies bind central NMDA receptors. Although typically diagnosed in women, approximately 20% of cases have been males. Due to the challenges with identification, imaging, and diverse symptom presentation, this syndrome is often misdiagnosed. Accurate diagnosis may provide an opportunity for introduction of disease-modifying therapies, which may alter disease trajectory. Moreover, neuropsychology has yet to fully clarify the pattern of impairments expected with this disorder. Methods: This manuscript reviews a single case study of a 42-year-old male diagnosed with NMDAR encephalitis. Neuropsychological evaluation was completed subsequent to diagnosis, treatment, and rehabilitation. Ongoing patient complaints, approximately six months post diagnosis, included reduced sustained attention, poor word retrieval, and daily forgetfulness. Adaptive skills were improved following rehabilitation. Results: Direct testing revealed mildly impaired sustained attention, processing speed, oral word fluency, and executive functioning. All other cognitive domains were within estimated premorbid range, low average to average. Conclusions: Neuropsychological deficits were consistent with mild frontal brain dysfunction and continued recovery. This case illustrates the need for medical and psychological practitioners to understand NMDAR encephalitis, its symptom presentation, and related neuropsychological impact; particularly with the potential for misdiagnosis.

Perinatal Cyanosis: Long-Term Cognitive Sequelae and Behavioral Consequences

Research suggests that serious perinatal asphyxia leading to long-term neurological consequences occurs in 1 to 6 out of every 1,000 newborns (Barkovich et al., 1998; Mcguire, 2007). In serious cases, encephalopathy follows the asphyxia and resultant hypoxia, leading to additional insult to the brain. The effects of brief or transient hypoxia and cyanosis have not been well researched. This study involved comparing children who had a brief perinatal episode to those who have not. The research hypothesis is that those children who have experienced a brief perinatal cyanotic episode will subsequently have cognitive and behavioral issues during childhood that will be measurable on neuropsychological testing or result in increased clinical diagnoses. A sample (N = 52) of school-aged children (M age = 10.5 years) was divided into those who had had a brief perinatal cyanotic episode (n = 14) and those who had not (n = 38). On neuropsychological testing, data from the tests administered did not suggest any negative effects of a brief cyanotic episode. The cyanotic group was significantly more likely to have a developmental disorder (speech or motor delay) and subsequently be diagnosed with attention-deficit hyperactivity disorder (ADHD). Given the high incidence of ADHD in the cyanotic group, it may be reasonable to construe cyanosis as a risk factor.

Early Developmental Delays: Neuropsychological Sequelae and Subsequent Diagnoses

Developmental delay is a frequent diagnosis given to young children when developmental milestones are not met in an age-expected time frame. Research on early delays in speech and motor milestones is unclear regarding possible long-term cognitive functioning. The purpose of this study was to investigate the neuropsychological profile of children who suffered early developmental delays in speech or motor function. Participants (N = 60) completed the Wechsler Intelligence Scale for Children-Fourth Edition, Wechsler Individual Achievement Test-Second Edition, Wisconsin Card-Sorting Test, Childrens Memory Test (CMT), the Delis-Kaplan Executive Function System, and the Child Behavior Checklist/Youth Self-Report. The Delay group had a significantly lower Full-Scale IQ (FSIQ), and when controlling for IQ (analysis of covariance), the Delay group had significantly lower scores on measures of immediate and delayed visual memory skills (CMT). Group scores were not significantly different for any other tests. Neither group had any test scores significantly below FSIQ, a finding suggesting developmental delays may subsequently lead to weaknesses but not impairments. Results appear to support the resiliency of the young brain. Chi-square analysis showed the Delay group was more likely to subsequently be diagnosed with attention-deficit hyperactivity disorder (ADHD) but not learning disorders. Data appear to suggest that early developmental delays may place children as risk for ADHD and perhaps visual memory weaknesses, though not clear impairments.

Megalencephaly-capillary malformation polymicrogyria: A review and complex pediatric case report

ABSTRACT Megalencephaly-Capillary Malformation Polymicrogyria (M-CAP) is a rare genetic disorder characterized by a spectrum of anomalies including macrocephaly and neurovascular malformations. Although developmental delays have been identified, research is devoid of neuropsychological data. This case report presents the neuropsychological profile of a 7-year-old, identified with M-CAP. Neuropsychological evaluation was completed subsequent to medical diagnosis. Reports from both parents and teachers included cognitive regression; specifically in the recall of learned material, reading, and information sequencing. Direct testing revealed a WISC-IV GAI at <1st percentile, a diverse range of scores across the battery, and a splinter skill strength of average to above on visual memory tests. Performance included below grade level reading and writing, reduced adaptive functioning, and reported executive dysfunction. Her strong visual memory skills were recommended as a medium to enhance learning. Neurocognitive deficits revealed diverse, multisystem and multifocal impairments. The neuropsychological evaluation also showed significant decline from the previous evaluation and prompted a neurologic consult and corrective surgical procedure.

The Neuropsychological Profile of Postural Orthostatic Tachycardia Syndrome: Sibling Case Study

Postural orthostatic tachycardia syndrome (POTS), estimated to affect 500,000 people in the United States alone, is a growing source of impairment and disability. Symptoms often consist of physical, cognitive, and psychological impairment. The symptoms are driven by fluctuations in cerebral blood flow. Cerebral perfusion is tightly controlled and linked to brain function as changes in posture can result in quick hemovascular regulation by the autonomic nervous system. Any delay in hemoregulation and cerebral blood flow can result in neurological symptoms. POTS is associated with chronic autonomic dysregulation, and people with POTS suffer differing degrees of cerebral hypoperfusion causing variability in symptoms. This article reviews the cases of two siblings (22 years old and 19 years old) who both suffer from POTS. They suffered physical symptoms along with difficulty organizing thoughts, sustaining attention, thinking quickly, multitasking, and recalling recent information, as well as depression and anxiety. These sisters have Full-Scale IQs (FSIQ) above average, yet they received academic accommodations and have ongoing cognitive dysfunction that prevents them from furthering their true potential. These cases illustrate the need for neuropsychologists and other treating providers to understand POTS, the related pathophysiology, and how cognitively debilitating POTS can be, even in the context of intact and high FSIQ scores.