Search

OBJECTIVE: To determine the validity of parental ratings of their child's overall recovery and social reintegration after pediatric traumatic brain injury (TBI). SETTING: Primary care hospital/medical center. PARTICIPANTS: Ninety-nine children aged 6 to 16 years (80 with TBI and 19 orthopedic trauma controls) evaluated as outpatients. MAIN OUTCOME MEASURES: Parental ratings of overall recovery and social reintegration; neuropsychological test performance. RESULTS: Severity of injury correlated with postacute parental ratings of the child's overall recovery (r = -0.498, N = 84, P = .001) and social reintegration (r = -0.507, N = 84, P = .001). A similar correlation was observed between TBI severity and a known 'objective' marker of recovery (Wechsler Intelligence Scale for Children-III Coding subtest; r = -0.503, N = 84, P = .001). CONCLUSIONS: The present findings support the concurrent validity of parental perspectives of a child's overall recovery and social reintegration after pediatric TBI. Incorporating these views may assist in the rehabilitation of children following brain injury. Parental reasons for judging a child's recovery as 'incomplete' may differ as a function of severity of injury. [ABSTRACT FROM AUTHOR]

Children who suffer moderately severe-to-severe traumatic brain injury often fail to develop normal cognitive and affective functioning necessary for independent adult living. The cognitive and psychiatric consequences of pediatric traumatic brain injury are outlined and 6 barriers to providing neuropsychological rehabilitation for these children are identified. Suggestions are made to help reduce the impact of those barriers. [ABSTRACT FROM AUTHOR]

OBJECTIVE: To examine the relationship between relatives' distress level and their ratings of impaired awareness for persons with traumatic brain injury (TBI). DESIGN AND OUTCOME MEASURES: Participants were 25 patients with TBI, 16 with probably dementia, and 15 with memory complaints but no dementia. Participants completed the Barrow Neurological Institute Screen for Higher Cerebral Functions. Relatives of all patients completed the Patient Competency Rating Scale (Relative Form). Relatives also rated their distress level on a scale from 0 (no distress) to 10 (severe distress) and then rated the patient's level of awareness of their difficulties, also on a scale from 0 (not aware) to 10 (completely aware). SETTING: Clinical neuropsychology outpatient service of a neurological institute and medical center. RESULTS: For relatives of patients with TBI, a significant correlation of -0.52 (P = .006) was found. Correlations for the dementia and memory complaint groups were -0.62 (P = .03) and -0.39 (P = .20), respectively. CONCLUSIONS: The presence of brain dysfunction associated with neuropsychological disturbances appears to influence the magnitude of the relationship between the distress level of family members and their ratings of impaired awareness in persons with TBI. [ABSTRACT FROM AUTHOR]

Over the last 20 years, numerous papers that are relevant to understanding the problem of impaired self-awareness after moderately severe to severe traumatic brain injury have appeared. This article reviews many of these papers and summarizes salient findings relevant to rehabilitation and future research. [ABSTRACT FROM AUTHOR]

Twenty five (25) out of 29 patients (86%) with unequivocal memorydisorders who received Memory Compensation Training (MCT) completed both a brief survey regarding their use of memory compensations and the Memory Compensation Questionnaire. Twenty-two (22) of the 25 patients (88%) reported daily use of memory compensation several months to years after the onset of their memory problems. The benefits included, by their report, being more productive, less disorganized, and less confused. Patients with memory disorders, who received MCT, also reported more frequent use of compensations than older normal adults studied by Dixon et al. [6]. The present sample of patients report proportionally spending more time to remember and greater reliance on those around them to help them to remember. The findings suggest that patients who are taught memory compensations tend to use them after the training period and show a different pattern of compensatory activities compared to normal older adults. [ABSTRACT FROM AUTHOR]

A Swedish translation of the Barrow Neurological Institute Screen for Higher Cerebral Functions was administered to 52 normal control subjects and 36 patients with well-documented brain dysfunction. Findings replicated those reported in American samples. Level of performance was strikingly similar between Swedish controls and American controls, especially in individuals between 15 and 39 years. Swedish patients with brain dysfunction performed at levels significantly below the Swedish control subjects. The sensitivity of the test was 83% (correctly classifying 30 of 36 patients); patients with a higher level of education were misclassified. The present study replicates earlier findings and adds to the construct validity of the Barrow Neurological Institute Screen. This test may also prove useful for studying rehabilitation outcomes in Swedish patients. [ABSTRACT FROM AUTHOR]

The cause(s) of lack of awareness of cognitive decline in neurodegenerative diseases can be multifactorial. Yet neurologically oriented research on anosognosia of cognitive decline almost exclusively assumes that the underlying disturbance of neuro-networks that support various cognitive functions accounts for the reduced self-awareness. Cultural and psychosocial factors, including the person's emotional state, however, can contribute to the underreporting or avoidance of admitting to cognitive impairments in neurodegenerative diseases. Research on the causes of lack of awareness of cognitive decline in neurodegenerative disorders needs to include these variables. We briefly present two case examples of underreporting or "unawareness" of memory difficulties in persons with mild cognitive impairment (MCI) (or minor neurocognitive disorder). One presented with classic anosognosia for memory impairment, while the other initially reported no memory impairment but later admitted to "denying" her memory difficulties secondary to anxiety. Based on these patients' clinical presentations and available research, we suggest three potential screening items that may help identify probable denial of memory impairments when studying anosognosia in MCI., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Prigatano, Russell and Meites.)

The scientific literature on neuropsychological correlates of Mild Cognitive Impairment of the Amnestic Type (MCI-A) often reports large group findings and employs multivariate statistics to describe domains of cognitive impairment associated with the transition of MCI-A to early dementia, typically of the Alzheimer's Type (AD). Individual patients may vary, however, in terms of specific changes in their neuropsychological test performance as they transition from MCI-A to probable AD. The subjective experiences of individuals during this time of transition can also vary but rarely are reported. Tracking both the patient's subjective experiences and their performance on neuropsychological measures provides a more complete picture of the patient's clinical situation. These combined sets of information help the clinical neuropsychologist provide a more individualized and personally relevant service. We present a phenomenological and neuropsychological case analysis of a 67-year-old woman who transitioned from MCI-A to probable early AD in an attempt to illustrate how such a combined analysis is helpful in their psychological care.

In the 1970s and 1980s, a multitude of cognitive rehabilitation programs proliferated to facilitate recovery after brain injury. However only a few programs provided a framework for ameliorating disturbances in the cognitive, psychological, and interpersonal spheres of the brain-injured patient. Greatly influenced by Leonard Diller and Yehuda Ben-Yishay's ideas and methods, George P. Prigatano began, in early 1980, a holistic neuropsychological rehabilitation program at the Presbyterian Hospital in Oklahoma City (Oklahoma). The objective of this paper is to summarize the contributions of George P. Prigatano to neuropsychological rehabilitation and clinical neuropsychology during his 50th year of practice. The main body of the paper is structured in three sections. The first section briefly explains the history of neuropsychological rehabilitation in the twentieth century and the emergence of holistic neuropsychological rehabilitation programs in the 1970s. The second section describes the contributions of George P. Prigatano to neuropsychological rehabilitation and clinical neuropsychology (written by AGM). In the third section, the second author (GPP) prepared an autobiographical statement, which attempts to summarize some of the personal and professional experiences which influenced his work. George P. Prigatano's contributions to neuropsychological rehabilitation and clinical neuropsychology are essential to understanding the therapeutic approaches currently used in the treatment of brain-injured patients., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 García-Molina and Prigatano.)

Hypomelanosis of Ito (HI) is a neurocutaneous disorder associated with central nervous system abnormalities, including speech delay and intellectual disability. The long term neuropsychological and social characteristics of these children are unknown. Neuropsychological observations and parental reports were obtained yearly on a child with HI from ages 7 to 18 years. Serial measures of intelligence revealed stable verbal and perceptual reasoning scores with later improvements in working memory and processing speed performance. Speech articulation improved at age 12, as did the speed of right-hand finger tapping. Improved social integration occurred, but anxiety persisted throughout this developmental period.

Decline in executive functioning, before frank dementia occurs, has been reported in patients with a history of stroke and malignant brain tumors. This may also be true in patients with advanced Parkinson's disease (PD). In this paper, we summarize the limited research on the motor and cognitive predictors of mortality in advanced PD. We then provide 2 case vignettes of patients with end of life advanced PD who demonstrated a substantial decline in working memory and speech festination. We contrast these patients' neuropsychological features to a third patient with advanced PD who shows no signs of impending death. Monitoring neuropsychological signs of executive dysfunction, explaining the neuropsychological dysfunctions to the patient and spouse while recognizing the past and retained cognitive competencies of the person is an important component of end of life psychological care. In the context of this type of consultation, the patient may experience an opportunity to communicate their emotional concerns prior to death which further reduces the anxiety associated with death.

Objective: Impaired self-awareness (ISA) of altered functional capacities is a common sequelae of severe acquired brain injury that can severely hamper neuro-rehabilitation in this clinical population. ISA is frequently associated with anosodiaphoria and/or apathy. Although several scales are available to measure apathy, no tools have been published to specifically assess anosodiaphoria after acquired brain injury. In this paper, we reported an initial effort to develop an anosodiaphoria subscale in a commonly used measure of ISA, that is, the Patient Competency Rating scale-neurorehabilitation form (PCRS-NR)., Method: A sample of 46 participants with severe acquired brain injury completed a functional, ISA, apathy, and anosodiaphoria assessment. One informal caregiver of each patient participated in the study. Thus, we were able to obtain external data on his/her level of functional competencies, and self-awareness, which allowed separating patients with low self-awareness (LSA) from those with high self-awareness (HSA). Finally, the patients were compared with 44 healthy age-gender-years of formal education matched control participants (HCs)., Results: Compared to both patients with HSA and HCs, patients with LSA demonstrated greater anosodiapvhoria and lower levels of functioning than both HSA patients and HCs. A stronger relationship emerged between ISA and anosodiaphoria rather than with apathy., Conclusions: These initial findings provide support that PCRS scale can be adapted to measure anosodiaphoria as well as ISA. The findings reveal a stronger correlation between this measure of anosodiaphoria and ISA compared with the correlation of apathy to ISA. The present method for measuring anosodiaphoria takes into account the actual levels of patients' functioning., (© The Author(s) 2021. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Objective: This paper describes the BNI Screen for Higher Cerebral Functions (BNIS) and reviews studies that comment on its reliability, validity, and clinical and research utility. The ability of the BNIS to assess non-cognitive higher brain functions is also described., Methods: We reviewed the original administration manual, studies published in the BNI Quarterly of the Barrow Neurological Institute, and peer-reviewed studies on the BNI Screen identified by an academic database, PubMed and Google Scholar. Thirty-two studies were reviewed that describe normative data, psychometric properties, sensitivity and specificity estimates, the relationship of demographic factors to test performance, and its research utility., Results: The BNIS is a time efficient screening test often taking no longer than 12-18 minutes. In addition to cognitive functioning, it aids in assessing conation, awareness of memory impairment, and affects expression and perception. Sensitivity estimates ranged from 80% to 92.3%. Specificity estimates ranged from 38.9% to 90%. Its construct, concurrent, and predictive validity have been supported by a series of international studies using different language translations of the test., Conclusion: The BNIS is a useful screening test for identifying patients with underlying brain disorders that uniquely measures domains of functioning not sampled by other existing screening tests.

Objective: Selective motor inhibition is known to decline with age. The purpose of this study was to determine the frequency of failures at inhibitory control of adjacent finger movements while performing a repetitive finger tapping task in young, middle-aged and older adults. Potential education and sex effects were also evaluated., Methods: Kinematic recordings of adjacent finger movements were obtained on 107 healthy adults (ages 20-80) while they performed a modified version of the Halstead Finger Tapping Test (HTFF). Study participants were instructed to inhibit all finger movements while tapping with the index finger., Results: Inability to inhibit adjacent finger movements while performing the task was infrequent in young adults (2.9% of individuals between 20 and 39 years of age) but increased with age (23.3% between the ages of 40 and 59; 31.0% between ages 60 and 80). Females and males did not differ in their inability to inhibit adjacent finger movements, but individuals with a college education showed a lower frequency of failure to inhibit adjacent finger movements (10.3%) compared to those with a high school education (28.6%). These findings were statistically significant only for the dominant hand., Conclusion: Selective motor inhibition failures are most common in the dominant hand and occur primarily in older healthy adults while performing the modified version of the HFTT. Monitoring selective motor inhibition failures may have diagnostic significance.

Background Patient Competency Rating Scale (PCRS-R-BR) is a clinical tool to evaluate the degree of competence in cognitive skills perceived by patients with brain injuries. However, no studies have investigated the influence of sociodemographic variables on self-report and self-awareness of healthy individuals. Aim This study aimed to (1) present normative data from the PCRS-R-BR in a healthy adult Brazilian sample; and (2) investigate psychometric properties of the scale Method One hundred and fifty-four adults that were divided in three age groups and two education groups and their informants completed the PCRS-R-BR. Results Score on the PCRS-R-BR Patient's Form differed as a function of age with younger adults reporting less competency than older individuals. An education effect was found on Attention/Working memory Factor on the Informant's PCRS-R-BR with informants of higher education adults reporting better competency than lower education individuals. A gender effect was observed on the Informant's Form. The Informant's Form scores of informants of women were higher than the scores provided by the informants of men. PCRS-R-BR showed adequate consistency coefficients and six factors. Conclusion PCRS-R-BR scores showed acceptable validity evidence and provides information regarding how age and gender effects may influence ratings in a Brazilian sample.

Initial brain imaging studies on recovery of motor functioning after stroke suggested their potential prognostic value in neurorehabilitation. However, the value of brain imaging in documenting brain changes associated with cognitive and behavioral treatment effects seem less likely. Also, neuroimaging studies at that time seem to have little, if any, value for treatment planning. Advances in neuroimaging technology are beginning to challenge these initial impressions. In this clinical commentary, we propose that advances in the field of neuroimaging have relevance for the future development of neuropsychological rehabilitation. Neuropsychological rehabilitation is entering a new era that involves collaboration with neuroimaging and associated studies on neuroplasticity. We recognize that this may seem "aspirational" rather than practical in most rehabilitation settings. However, we provide examples of how this can be achieved as illustrated by collaborative efforts of clinicians and scientists in the SARAH Network of Rehabilitation Hospitals in Brazil. We also review selective papers on neuroplasticity, spontaneous recovery and diaschisis that have relevance for research which will expand and further develop the field of neuropsychological rehabilitation.

Introduction: Self-awareness of cognitive, emotional, functional, and social performance is critical for compliance with treatment. However, few studies have investigated self-awareness and the associated effects on other cognitive variables in patients with temporal lobe epilepsy (TLE) after surgical treatment., Aim: This study was designed to investigate the prevalence of impaired self-awareness (ISA) in patients with TLE who have undergone surgical treatment. Associated correlations with clinical variables (frequency of seizures before surgery, time elapsed since the epilepsy diagnosis, depression, and anxiety) and verbal and visual episodic memory function and differences between patients with right and left TLE were also investigated., Method: Twenty-three adults with TLE after surgical treatment were assessed with the Patient Competency Rating Scale (PCRS-R-BR), the Rey Auditory Verbal Learning Task (RAVLT), and the Modified Ruche Visuospatial Learning Test (RUCHE-M). Patients were considered to have memory dysfunction if delayed recall as assessed with the RUCHE-M or RAVLT was at or below the 25th percentile. Patients were considered to have ISA if PCRS-R-BR discrepancy scores were at or above the 75th percentile. Underestimated cognitive ability (UCA) was defined as a PCRS-R-BR discrepancy percentile score ≤25. Results were analyzed using frequency, Spearman correlation, regression analyses, and the Mann-Whitney test., Results: Frequency analysis of the total sample indicated ISA in 39.13% of patients (n = 9), UCA in 39.13% of patients (n = 9), and impaired verbal and/or visual memory performance in 69.56% of patients (n = 16). Moderate positive correlations were found between the frequency of seizures before surgical treatment and relatives' reports, as well as between the duration of time that had elapsed since the epilepsy diagnosis and patient reports. Negative and moderate correlations were found between the frequency of seizures and the discrepancy score, as well as between depression and patient reports. No differences in PCRS-R-BR were found between patients with right vs. left TLE. No clinical variables significantly predicted self-report or self-awareness., Conclusion: Patients with TLE exhibit various patterns of ISA and negative effects on cognitive function after surgical treatment. Emotional factors and relatives' reports must be considered when assessing these patients., Competing Interests: Declaration of competing interest The authors declare no conflicts of interest., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Persistent denial of severe and acute pain following orthopedic injuries has not been previously reported. We present a case of a 24-year-old woman with a history of schizoaffective disorder who suffered severe pain secondary to acute orthopedic injuries who insisted, "I am fine! There is nothing wrong with me." Her maladaptive denial resulted in an initial refusal of necessary medical/surgical care, but she eventually accepted the necessary treatments despite her persistent belief she did not need such care. Her verbalizations and behaviors were characterized by active avoidance and angry reactions when a consulting psychiatrist spoke to her regarding her clinical condition. A modified version of the Conscious Avoidance subscale of the Denial of Illness Questionnaire was useful in measuring the severity level of her denial. This case report suggests that the behavioral features of psychological denial appear different from those associated impaired self-awareness secondary to an underlying brain disorder., (Copyright © 2020 Prigatano, McKnight, Andrews and Caplan.)

While a number of empirical studies have appeared on impaired self-awareness (ISA) after traumatic brain injury (TBI) over the last 20 years, the relative role of denial (as a psychological method of coping) has typically not been addressed in these studies. We propose that this failure has limited our understanding of how ISA and denial differentially affect efforts to rehabilitate persons with TBI. In this selective review paper, we summarize early findings in the field and integrate those findings with more recent observations (i.e., 1999-2019). We believe that this synthesis of information and expert clinical opinion will inform future research on ISA and denial as well as approaches to rehabilitation for persons with TBI., (Copyright © 2020 Prigatano and Sherer.)

Introduction : This study attempts to demonstrate that kinematic recordings of finger movements help explain the well-known effects of age, education, and sex on the Halstead Finger Tapping Test (HFTT). Method : High-speed kinematic recordings were obtained on 107 healthy adults (ages 21 to 80 years) while they performed a modified version of the Halstead Finger Tapping Test (HFTT). The number of "valid" taps and "invalid" taps (i.e., lever movements that did not produce an increase in the mechanical number count), tapping speed variability, "learning" and "fatigue" effects was obtained. Results : Previous age, education, and sex effects were replicated. Males had faster start and stop times when finger tapping and these measures correlated with the number of valid taps per 10 s. Educational level correlated with start times, not stop times. Age correlated only with the number of valid taps. Variability of tapping movements correlated with the number of invalid taps, but not valid taps. Females had more invalid taps than males. Fatigue and learning effects were independent of the person's age, education, and sex. Conclusion : Kinematic recordings of finger tapping help explain the well-known age, education, and sex effects on finger tapping speeds. A modified method of administrating the HFTT is also introduced to assess fatigue and learning effects while performing this task.

In a retrospective chart review, 39 patients referred for a clinical neuropsychological examination were identified as showing either mild cognitive impairment of the amnestic type (MCI-A; N  = 21) or subjective memory complaints but with normal memory function (SMC; N  = 18). During the clinical interview, patients and informants were routinely asked to make subjective ratings regarding the patient's cognitive and affective functioning in everyday life. The purpose of this study was to determine whether these two patient groups (and their informants) significantly differed in their subjective reports about level of cognitive and affective difficulties. It was predicted that SMC patients would report higher levels of cognitive and emotional dysfunction than MCI-A patients. It was further predicted that MCI-A patients would underreport cognitive difficulties (compared to informant reports); SMC patients would demonstrate the opposite pattern. Results supported these predictions and suggest that routine assessment of subjective experiences of patients in conjunction with informant ratings may aid clinical diagnosis, particularly when the primary complaint is a decline in memory.

Primary Objective : The primary aim of this study was to determine the frequency of severe impaired self-awareness (ISA) in patients with severe traumatic brain injury (TBI) and the correlates of selected clinical, neuropsychiatric and cognitive variables. The secondary aim of the study was to assess depression and apathy on the basis of their level of self-awareness. Methods : Thirty patients with severe TBI and 30 demographically matched healthy control subjects (HCs) were compared on measures of ISA, depression, anxiety, alexithymia, neuropsychiatric symptoms and cognitive flexibility. Results : Twenty percent of the patients demonstrated severe ISA. Severe post-acute ISA was associated with more severe cognitive inflexibility, despite the absence of differences in TBI severity, as evidenced by a Glasgow Coma Scale (GCS) score lower than 9 in all cases in the acute phase. Patients with severe ISA showed lower levels of depression and anxiety but tended to show more apathy and to have greater difficulty describing their emotional state than patients with severe TBI who showed minimal or no disturbance in self-awareness. Conclusion : These findings support the general hypothesis that severe ISA following severe TBI is typically not associated with depression and anxiety, but rather with apathy and cognitive inflexibility.

Introduction: The Barrow Neurological Institute (BNI) Screen for Higher Cerebral Functions (BNIS) has been translated into several languages and found useful in evaluating multiple domains of cognitive and affective dysfunction, particularly in neuro-rehabilitation settings. Normative data from countries with high literacy rates have reported strikingly similar mean level of performance scores on this test, with age typically correlating higher with total score performance than education. In the present study, we obtain convenience sample normative data from a native Brazilian population on a Portuguese translation of the BNIS (i.e., BNIS-PT)., Method: The BNIS was translated into Portuguese by two native speaking Portuguese neuropsychologists who were also fluent in English. It was then administered to 201 normally functioning native Brazilian individuals who varied considerably in age and formal educational training., Results: The mean BNIS total score was similar to what previous studies reported, but primarily in younger adults with at least 12 years of formal education. In this Brazilian sample, the correlation of educational level and BNIS total score was r = .68, p < .001. The correlation of age and BNIS total score was r = -.36, p < .001. This is the opposite pattern to that observed in previous standardization studies. The strong correlation of education with performance in various subtests was observed in all age groups (ages ranging from 15 to 85 years)., Conclusion: This standardization study provides guidelines for calculating expected average performance levels on the BNIS-PT for Brazilian individuals with varying degrees of age and education. Educational level positively correlated with test performance on the BNIS-PT and was repeatedly observed to overshadow the effects of age, suggesting its important role in the development of higher cerebral functions in multiple domains in a Brazilian sample of normally functioning individuals.

Traditionally, anosognosia for cognitive or motor impairments in patients with Parkinson's disease (PD) was viewed as unlikely unless the patient was demented. More recent research has suggested that a portion of non-demented PD patients (30%-50%) in fact have impaired subjective awareness (ISA) of their motor impairments (ISAm). This empirical finding has implications for the clinical neuropsychological examination of PD patients and raises theoretical questions relevant to the broader study of anosognosia seen in other patient groups. The purpose of this paper is to primarily review our own research in this area and to summarize research findings of other investigators who have examined ISA in PD patients. Our secondary goal is to demonstrate the relevance of assessing ISA when conducting a neuropsychological examination of PD patients. Our findings suggest ISAm in PD patients is related to motor signs of right hemispheric dysfunction, but the brain imaging correlates of ISA for hypokinesias appear different than those obtained for ISA for dyskinesia in this patient group., (© The Author 2017. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Objective: The purpose of this study was to investigate the contribution of executive functions (EF) components and episodic and working memory variables, as well as clinical and demographic factors, to awareness of cognitive ability in traumatic brain injury (TBI)., Methods: Sixty-five TBI patients (mild: n = 26; moderate/severe: n = 39) took part in the study. Independent stepwise regression models were calculated for EF and memory predictors, with awareness being measured by patient/informant discrepancy in the Patient Competency Rating Scale., Results: Models with EF variables indicated that semantic verbal fluency and age are the best predictors of awareness, whereas models including mnemonic functions suggested verbal delayed episodic recall and TBI severity as predictors., Conclusions: These results are discussed in relation to clinical implications, such as the need to focus efforts of rehabilitation in the cognitive abilities related to awareness, and theoretical models.

Many patients with epilepsy caused by hypothalamic hamartomas (HHs) have cognitive impairments during the course of the disease or following neurosurgical treatment. The purpose of this study was to assess cognitive function in these patients, as well as factors influencing preoperative cognitive performance and cognitive outcome after neurosurgical treatment. Using the two largest and most detailed neuropsychology datasets on HH and epilepsy from two centers, we retrospectively report on cognitive functions in 48 patients with structural epilepsy due to HH (mean age ± standard deviation [SD] 20 ± 12 years, range 5-53 years, median 16 years; disease duration mean 17 ± 11 years). Intelligence, verbal learning and recall, and speed and executive functions (processing speed and cognitive flexibility) were assessed before and on average 19 (±11) months after surgery (interstitial radiosurgery: N = 22; neurosurgical resection/disconnection: N = 26). Prior to neurosurgical treatment, 52% of patients showed impaired executive and 62% showed reduced verbal memory functions. A trend for a detrimental effect of higher drug load on cognitive functioning was found. After neurosurgical treatment, intellectual functions for the entire cohort tended to increase. This correlated with improved seizure frequency and decreased number of antiepileptic drugs (AEDs). However, postoperative outcomes for individual patients were highly variable, with significant deteriorations in 17% (processing speed) to 34% (cognitive flexibility and verbal learning), and performance increases in 17% (intellectual functioning) up to 39% (processing speed) of the patients. Higher levels of presurgical performance were significant predictors of cognitive decline after surgery. These results are highly relevant for patient consultation and may help with therapeutic decisions., (Wiley Periodicals, Inc. © 2017 International League Against Epilepsy.)

Mutations in CASK cause X-linked intellectual disability, microcephaly with pontine and cerebellar hypoplasia, optic atrophy, nystagmus, feeding difficulties, GI hypomotility, and seizures. Here we present a patient with a de novo carboxyl-terminus splice site mutation in CASK (c.2521-2A>G) and clinical features of the rare FG syndrome-4 (FGS4). We provide further characterization of genotype-phenotype correlations in CASK mutations and the presentation of nystagmus and the FGS4 phenotype. There is considerable variability in clinical phenotype among patients with a CASK mutation, even among variants predicted to have similar functionality. Our patient presented with developmental delay, nystagmus, and severe gastrointestinal and gastroesophageal complications. From a cognitive and neuropsychological perspective, language skills and IQ are within normal range, although visual-motor, motor development, behavior, and working memory were impaired. The c.2521-2A>G splice mutation leads to skipping of exon 26 and a 9 base-pair deletion associated with a cryptic splice site, leading to a 28-AA and a 3-AA in-frame deletion, respectively (p.Ala841&#95;Lys843del and p.Ala841&#95;Glu868del). The predominant mutant transcripts contain an aberrant guanylate kinase domain and thus are predicted to degrade CASK's ability to interact with important neuronal and ocular development proteins, including FRMD7. Upregulation of CASK as well as dysregulation among a number of interactors is also evident by RNA-seq. This is the second CASK mutation known to us as cause of FGS4. © 2017 Wiley Periodicals, Inc., (© 2017 Wiley Periodicals, Inc.)

Introduction: Anosognosia or impaired self-awareness of motor symptoms (ISAm) has been rarely investigated in Parkinson's disease (PD). We here studied the relationship between ISAm during periods with and without dopaminergic medication (ON- and OFF-state), and clinical, neuropsychological, and neuroimaging data to further elucidate behavioural aspects and the neurobiological underpinnings of ISAm., Methods: Thirty-one right-handed, non-demented, non-depressed PD patients were included. ISAm was evaluated using a recently developed scale that assesses awareness of dyskinesia, resting tremor, and bradykinesia. The test was applied during both ON- and OFF-states. Multiple correlation analyses between ISAm and behavioural data were conducted. In addition, imaging of glucose metabolism using 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) was performed to investigate the neural basis of ISAm. A multiple regression approach was applied to investigate metabolism alterations related to ISAm., Results: In the OFF-state, higher ISAm was associated with left-sided disease onset, older age, and shorter disease duration. Concerning FDG-PET data, there was a significant negative correlation between higher OFF-state ISAm and decreased glucose metabolism in the right inferior frontal gyrus (IFG). In the ON-state, ISAm was not significantly correlated with clinical or behavioural data. However, there was a significant correlation between higher ISAm and an increased metabolism in the bilateral medial frontal gyrus, left IFG, right superior frontal gyrus and right precentral gyrus., Conclusion: The results support the role of the right hemisphere in awareness of motor symptoms in the OFF-state. In the ON-state, dopaminergic medication and dyskinesia influence ISAm and relate to metabolism changes in bilateral frontal regions., (Copyright © 2016 Elsevier Ltd. All rights reserved.)

Objective: Patients with Parkinson's disease (PD) can show impaired self-awareness of motor deficits (ISAm). We developed a new scale that measures ISAm severity of hyper- and hypokinetic movements in PD during medication on state and defined its psychometric criteria., Method: Included were 104 right-handed, non-depressed, non-demented patients. Concerning ISAm, 38 motor symptoms were assessed using seven tasks, which were performed and self-rated concerning presence of deficit (yes/no) by all patients. The whole procedure was videotaped. Motor symptoms were then evaluated by two independent experts, blinded for patient's ratings, concerning presence, awareness of deficit, and severity. Exploratory principal component analysis (promax rotation) was applied to reduce items. Principal axis factoring was conducted to extract factors. Reliability was examined regarding internal consistency, split-half reliability, and interrater reliability. Validity was verified by applying two additional measures of ISAm., Results: Of the initial 38 symptoms, 15 remained, assessed in five motor tasks and merged to a total severity score. Factor analysis resulted in a four factor solution (dyskinesia, resting tremor right hand, resting tremor left hand, bradykinesia). For all subscales and the total score, measures of reliability (values 0.64-0.89) and validity (effect sizes>0.3) were satisfactory. Descriptive results showed that 66% of patients had signs of ISAm (median 2, range 0-15), with ISAm being most distinct for dyskinesia., Conclusions: We provide the first validation of a test for ISAm in PD. Using this instrument, future studies can further analyze the pathophysiology of ISAm, the psychosocial sequelae, therapeutic strategies and compliance with therapy.

Disturbances of self-awareness are observed in a wide variety of patients. While group studies can provide useful information concerning potential mechanisms underlying these complex disturbances, experienced clinicians, such as Babinski, recognized the potential value of repeated observations on individual patients to insure the reliability of findings and to aid in diagnosis. This paper describes patterns of impaired self-awareness (ISA) that are observed in clinical practice that suggest a model for clinical classification. Repeated observations are reported on four patients ranging from anosognosia for hemiplegia (AHP), ISA associated with bilateral cerebral dysfunction with frontal lobe involvement, and apparent denial of disability (DD). A patient who presents with denial of ability (DA) is also studied for comparison purposes. When coupled with brain imaging findings, the nature of the patients' subjective responses to feedback regarding their functional capacities, speed of finger tapping in the left, nondominant hand, and their capacity to express and perceive affect suggests different clinical correlates in these four conditions., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

Objective: Disturbances of affect expression and perception, as well as accuracy of predicting memory difficulties, have been reported in various brain dysfunctional groups. Screening tests of higher cerebral functions seldom sample these dimensions. The goal of this study was to determine if patients with mild cognitive impairment (MCI) of the amnestic type would demonstrate impairments in these domains, as well as show expected memory deficits., Methods: Thirty-nine French-speaking patients with a clinical diagnosis of MCI were compared with 39 age- and education-matched normal functioning individuals on the French translation of the Barrow Neurological Institute Screen for Higher Cerebral Functions (BNIS). Patients and controls also made subjective ratings regarding their cognitive and affective functioning in everyday life., Results: Patients with MCI performed significantly worse than controls on the BNIS subtests sampling memory, orientation, affect expression and perception, and accurate prediction of memory performance. They did not differ on other subtests of the BNIS, as predicted. Poor self-awareness (i.e., inaccurate prediction of the number of words one could recall after distraction) correlated with self-reported deficits in several areas of cognitive and affective functioning., Conclusion: This sample of MCI patients demonstrated disturbances not only in memory but also in self-awareness and affect expression and perception on the BNIS. These dimensions should be included in the neuropsychological assessment of patients suspected of MCI., (Copyright © 2014 John Wiley & Sons, Ltd.)

A nondemented, 55-year-old woman with a 20-year history of relapsing-remitting multiple sclerosis (MS) recently began having significant difficulties performing her job after several years of successful employment. While the patient acknowledged that others considered her job performance as being below standards, she did not subjectively experience any change in her cognitive functioning that would negatively impact job performance. She had no explanation as to why her job performance was now considered unsatisfactory. She also appeared to be in no distress over her situation. Was the patient's unawareness a form of anosognosia or psychological denial of her clinical condition? We provide neuropsychological, neuroimaging, and behavioral descriptions of the patient that suggest that the underlying disturbance appeared to be a neuropsychologically based, impaired self-awareness (ISA). Clinical suggestions are provided for distinguishing between ISA and denial of disability (DD) in MS patients.

Objective: To determine whether patients with hypothalamic hamartoma (HH) improve in their cognitive functioning after neurosurgical resection of their HH and explore what variables correlate with cognitive outcome., Methods: Thirty-two patients underwent preoperative and postoperative neuropsychological testing. The age range of patients was between 3.3 and 39.3 years (mean 12.2 years, SD 7.0). The average time interval between surgery and postoperative neuropsychological testing was 23.4 months (range 5.1-47.2 months). Tests administered varied on the basis of the patient's age and clinical condition., Results: As a group, measures of overall intelligence showed improvement postsurgery, with associated improvement in processing speed. Memory scores did not demonstrate consistent improvement or decline. Duration of epilepsy, age at surgery, and level of neurocognitive functioning prior to surgery were correlated with postsurgical cognitive status. Patients who had mental retardation but were testable generally showed the greatest gains., Conclusions: Despite the great variability in level of cognitive impairment in patients with HH and refractory epilepsy, level of intelligence may show mild to moderate improvements postsurgery if no surgical complications occur. The variables that predict cognitive outcome are not fully delineated, but testable individuals with the greatest presurgical cognitive impairment and those with the shortest duration of epilepsy appear to make the greatest gains in intellectual functioning., Classification of Evidence: This study provides Class IV evidence that single surgical resection for HH was associated with improvement in some subset measures of intellectual functioning, but not memory. Factors that predict better outcomes cannot be determined.

Background: Holistic approaches to neuropsychological rehabilitation have progressively been recognized as an important form of rehabilitative care for persons who have a history of moderate to severe traumatic brain injury., Objective: After providing historical and contemporary perspectives, identify challenges and opportunities facing the field of neuropsychological rehabilitation., Methods: Selective literature review from neuropsychological rehabilitation, neurosciences, learning theory, cognitive neuropsychology, and psychotherapy that highlight challenges to the development of holistic neuropsychological rehabilitation., Results: Ten challenges and associated opportunities that face the field of holistic neuropsychological rehabilitation are identified. Illustrations of these challenges and opportunities are provided., Conclusions: Effectively addressing the challenges that face holistic neuropsychological rehabilitation will result in greater advances for this field of care for both children and adults.

A central flaw in models of anosognosia is to consider metacognitive awareness and affective regulation and responsiveness as separate functional modular subsystems. This line of reasoning leads to an "either or" conceptualization of the probable causes of implicit awareness in anosognosic patients. Neuroscience research and clinical observations of patients suggest that anosognosia is often associated with a change in the affective status of the individual as well as a change in their explicit verbal descriptions of themselves. Studying anosognosic patients over time and including measures of psychological denial and anosodiaphoria are necessary when interpreting markers of implicit awareness.

Objective: To obtain normative data using a French translation of the BNI Screen for Higher Cerebral Functions (BNIS) administered to French-speaking individuals., Methods: Recruitment and administration of the BNIS to a convenience sample of 167 French-speaking individuals between the ages of 15-84 years., Results: Mean BNIS total score for a French sample was within 1 point of what was observed in the original standardization study using 200 English-speaking controls. Age and education correlated significantly with BNIS scores in a manner reported by other studies., Conclusion: The French translation of the BNIS appears to be a reliable method of assessing higher cerebral functioning in French-speaking individuals.

Background: This study investigated impaired self-awareness of motor deficits in nondemented, nondepressed Parkinson's disease (PD) patients during a defined clinical on state., Methods: Twenty-eight PD patients were examined. Patients' self-ratings and experts' ratings of patients' motor performance were compared. Patient-examiner discrepancies and level of impairment determined severity of impaired self-awareness. Motor exam assessed overall motor functioning, hemibody impairment, and 4 motor phenotypes. Neuropsychological tests were also conducted., Results: Signs of impaired self-awareness were present in 17 patients (60.7%). Higher severity of impaired self-awareness correlated significantly with higher postural-instability and gait-difficulty off scores (r = .575; P = .001), overall motor off scores (r = .569; P = .002), and higher left hemibody off scores (r = .490; P = .008). In multiple linear regression analyses, higher postural-instability and gait-difficulty off scores remained as the only significant predictor of impaired self-awareness severity., Conclusions: Postural instability and gait difficulties, disease severity, and right hemisphere dysfunction seem to contribute to impaired self-awareness., (Copyright © 2012 Movement Disorder Society.)

A 51-year-old woman presented with anosognosia for hemiplegia (AHP), neglect, and a complete loss of vision, for which she was almost immediately aware. Neuroimaging studies revealed intracranial hemorrhages in the medial temporal lobes bilaterally, extending back to the occipital cortex, but sparing the calcarine cortex. A large right frontal-parietal hemorrhage which extended to the posterior body of the corpus callosum was also observed. The patient's vision slowly improved, and by 11 months post onset, formal visual fields revealed improvement primarily in the left upper quadrants only. In contrast, resolution of her AHP occurred between the 26th and 31st day post onset. Awareness of motor impairment was correlated with her ability to initiate finger tapping in her left hemiplegic/paretic hand. During the time she was unaware of her motor deficits but aware of her visual impairments, her dreams did not reflect concerns over visual or motor limitations. The findings support a "modular" theory of anosognosia., (Copyright © 2010 Elsevier Srl. All rights reserved.)

Hypothalamic hamartomas (HHs) are associated with treatment-resistant epilepsy. Many patients also experience severe and sometimes disabling psychiatric problems. The most common behavioral symptoms consist of paroxysms of uncontrolled anger related to poor frustration tolerance. These can include violence, resulting in disrupted family or school relationships, and legal consequences including incarceration. In a large cohort of patients undergoing surgical resection of HHs for refractory epilepsy, 88% of families described an improvement in overall behavioral functioning [1]. Here, we describe four patients (three males, mean age=11.9 years) who underwent surgical resection of HHs largely for behavioral indications. Three patients had relatively well controlled seizures, and one had no history of epilepsy. All patients had striking improvement in their psychiatric comorbidity. HH resection can result in significant improvement in behavioral functioning, even in patients with relatively infrequent seizures. Further investigation under approved human research protocols is warranted., (Copyright © 2010 Elsevier Inc. All rights reserved.)

Kaufman's observation that the patients' reactions to their impairments and disabilities need to be addressed in stroke rehabilitation has been shown to be an accurate and perceptive statement. In this article, 3 levels of stroke rehabilitation are outlined, and the importance of focusing on the third level (the level of subjective experience) is emphasized. Identification of the patients' subjective experience allows one to understand what is most frustrating to them. After addressing those frustrations, patients are more eager to engage the rehabilitation process. Within the context of this rehabilitation process, helping patients clarify what their subjective or phenomenological state is as it relates to their stroke is crucial in having them not only engage the rehabilitation process, but ultimately find meaning in life in the face of their stroke. This can be a difficult task because patients often do not have the words to clarify what their inner psychological experiences are following a stroke. Helping to provide guidelines for this can result in a meaningful experience for both the patient and the therapists involved in their care.