Modification of the Patient Competency Rating Scale to Measure Anosodiaphoria after Severe Acquired Brain Injury: Preliminary Findings.

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.

Depression, apathy and impaired self-awareness following severe traumatic brain injury: a preliminary investigation.

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.

A de novo splice site mutation in CASK causes FG syndrome-4 and congenital nystagmus.

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_Lys843del and p.Ala841_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.

Anosognosia for hemiplegia after stroke is a multifaceted phenomenon: a systematic review of the literature.

Anosognosia is the lack of awareness or the underestimation of a specific deficit in sensory, perceptual, motor, affective or cognitive functioning due to a brain lesion. This self-awareness deficit has been studied mainly in stroke hemiplegic patients, who may report no deficit, overestimate their abilities or deny that they are unable to move a paretic limb. In this review, a detailed search of the literature was conducted to illustrate clinical manifestations, pathogenetic models, diagnostic procedures and unresolved issues in anosognosia for motor impairment after stroke. English and French language papers spanning the period January 1990-January 2007 were selected using PubMed Services and utilizing research words stroke, anosognosia, awareness, denial, unawareness, hemiplegia. Papers reporting sign-based definitions, neurological and neuropsychological data and the results of clinical trials or historical trends in diagnosis were chosen. As a result, a very complex and multifaceted phenomenon emerges, whose variable behavioural manifestations often produce uncertainties in conceptual definitions and diagnostic procedures. Although a number of questionnaires and diagnostic methods have been developed to assess anosognosia following stroke in the last 30 years, they are often limited by insufficient discriminative power or a narrow focus on specific deficits. As a consequence, epidemiological estimates are variable and incidence rates have ranged from 7 to 77% in stroke. In addition, the pathogenesis of anosognosia is widely debated. The most recent neuropsychological models have suggested a defect in the feedforward system, while neuro-anatomical studies have consistently reported on the involvement of the right cerebral hemisphere, particularly the prefrontal and parieto-temporal cortex, as well as insula and thalamus. We highlight the need for a multidimensional assessment procedure and suggest some potentially productive directions for future research about unawareness of illness.

Impaired self-awareness after moderately severe to severe traumatic brain injury.

Patients with moderately severe to severe traumatic brain injury (TBI) can demonstrate disturbances in self-awareness several months or years after injury. Patients may underreport cognitive and behavioral difficulties, which are the true residuals of their brain injury. Increasingly, research indicates that the residuals of these disturbances in consciousness greatly affect the process and outcome of rehabilitation. A recent model for conceptualizing disturbances of self-awareness after various forms of brain injury is reviewed.

Neuropsychology, the patient's experience, and the political forces within our field.

This article emphasizes the importance of both scientific and phenomenological approaches to the practice of clinical neuropsychology. Paying attention to patients experiences while they receive neuropsychological services is stressed because this aspect is often neglected in the training of clinical neuropsychologists. Finally, the reality of "hostile" forces within the politics of neuropsychology is considered briefly. Neuropsychologists need to consider three major issues that are important to our field and to the National Academy of Neuropsychology. First, neuropsychology is a scientific enterprise that deserves continued efforts at scholarship and clinically relevant research. The second issue concerns how scientific information is applied to patient care and the need for clinical neuropsychologists to understand both personal suffering and empirical data. The third issue is more delicate. It concerns the political forces in our field and my related personal observations.

Cognitive and affective improvement in brain dysfunctional patients who achieve inpatient rehabilitation goals.

OBJECTIVE: To evaluate whether improvements in specific cognitive and affective functions are associated with achieving inpatient rehabilitation goals after the acute onset of brain dysfunction. DESIGN: Retrospective analysis of data obtained in a prospective inception cohort study. STUDY SETTING: Medical center and neurological institute. SUBJECTS: Ninety-five heterogeneous brain dysfunctional patients who participated in an interdisciplinary inpatient neurorehabilitation program. Subjects were classified as having achieved their rehabilitation goals (Group 1) or not (Group 2) at time of discharge. MAIN OUTCOME MEASURE: Number of treatment goals attained. RESULTS: Although overall level of performance on the BNI Screen (BNIS) for Higher Cerebral Functions distinguished patients who later achieved their inpatient rehabilitation goals, on admission only performance on the visual spatial subtest was associated with later goal attainment. In contrast, at discharge, patients who showed greater improvement in awareness, affect, visual spatial skills, memory, and attention/concentration were likely to obtain their rehabilitation goals. This was true when the initial level of performance was taken into account. CONCLUSIONS: Measurement of overall neuropsychological status and specific visual spatial abilities at time of admission may help determine which brain dysfunctional patients will obtain inpatient rehabilitation goals. Improvement in a variety of cognitive and affective functions was associated with goal attainment. Early cognitive rehabilitation should focus on both cognitive and affective disturbances to facilitate recovery and rehabilitation outcome.

Initial disturbances of consciousness and resultant impaired awareness in Spanish patients with traumatic brain injury.

The purpose of this prospective, between-subjects study was to look at impaired awareness cross-culturally in patients with traumatic brain injury (TBI) and to relate impaired awareness after injury to the initial estimates of disturbed consciousness at time of injury. The study was conducted in community and inpatient and outpatient rehabilitation centers in Barcelona and Madrid. Participants were 30 persons with primarily moderate to severe TBI who could complete a written questionnaire concerning their functioning and 28 age- and gender-matched controls. A Spanish translation of the Patient Competency Rating Scale (PCRS) was administered to each participant. Relatives or significant others also completed this scale on each participant using the relative's version (PCRS-R). Difference scores, obtained by subtracting PCRS-R from PCRS-P (PCRS-P minus PCRS-R), were used as a marker of impaired awareness. Individuals with TBI were rated (by self and significant others) as being less competent than controls. Forty percent of Spanish patients with TBI who suffered severe injuries tended to overestimate their behavioral competencies. The PCRS-P minus the PCRS-R difference scores tended to correlate with admitting Glasgow Coma Scale (GCS) scores and retrospective estimates of posttraumatic amnesia (PTA). Initial disturbances of consciousness, one measure of severity of brain injury, appeared to relate to later measures of impaired self-awareness in Spanish patients with TBI. Non-brain-injured controls did not tend to report levels of competency that differed from their relatives' reports.

Speed of finger tapping and goal attainment after unilateral cerebral vascular accident.

OBJECTIVE: To determine (1) if speed of finger tapping is bilaterally slow after an acute unilateral cerebral vascular accident (CVA) and (2) if speed of finger tapping and grip strength are related to achieving rehabilitation goals during the first few weeks after stroke. DESIGN: Prospective inception cohort study. STUDY SETTING: Medical center and neurological institute. PARTICIPANTS: Fifty-one patients with unilateral CVAs. MAIN OUTCOME MEASURES: Documentation of goal attainment at discharge and bilateral measures of speed of finger tapping and grip strength. RESULTS: Speed of finger tapping and grip strength were often bilaterally below normal limits after an acute unilateral CVA, with the contralateral hand most affected. Speed of finger tapping, but not grip strength, in the ipsilateral hand was associated with achieving rehabilitation goals. Speed of finger tapping in the contralateral hand as well as bilateral grip strength was not related to achievement of rehabilitation goals. CONCLUSIONS: Motor findings suggest that bilateral cerebral dysfunction may be common after an acute unilateral CVA. The speed of finger movement in the hand ipsilateral to the lesion may reflect the degree to which the so-called "unaffected" cerebral hemisphere has in fact maintained its functional integrity. As such, it may be a useful behavioral marker for predicting goal attainment during early stages of neurorehabilitation.

Prescribed versus actual length of stay and inpatient neurorehabilitation outcome for brain dysfunctional patients.

OBJECTIVE: To determine (1) whether brain dysfunctional patients have better rehabilitation outcomes if they receive prescribed length of stay (LOS) versus less than prescribed and (2) if LOS and cognitive status relate to goal attainment. DESIGN: Prospective inception cohort study. STUDY SETTING: Medical center and a neurological institute. PARTICIPANTS: One hundred six patients with acute static brain lesions. MAIN OUTCOME MEASURES: Documentation of goal attainment at discharge and maintenance of goals 6 months after discharge. All subjects also received neuropsychological tests at admission and discharge. RESULTS: Patients who received prescribed LOS achieved their rehabilitation goals at discharge more frequently than patients who received less than prescribed LOS. There was, however, no difference between groups as to maintenance of rehabilitation goals 6 months after discharge. There was no relation between number of days spent in neurorehabilitation and number of goals achieved at discharge. Cognitive status at discharge was strongly related to achieving rehabilitation goals. CONCLUSION: Although LOS may not specifically relate to goal attainment in a heterogeneous group of brain dysfunctional patients, patients who receive the prescribed LOS and who show notable improvement in cognitive status tend to achieve rehabilitation goals, compared with patients who do not.

Differentiating patients with higher cerebral dysfunction from patients with psychiatric or acute medical illness using the BNI Screen for Higher Cerebral Functions.

The BNI Screen for Higher Cerebral Functions (BNIS) was administered to 41 patients with known cerebral dysfunction. 22 psychiatric patients (some of whom were psychotic) without documented brain lesions, and 22 medical inpatients without neurological or psychiatric diagnoses. Patients with cerebral dysfunction scored significantly lower than the medical and psychiatric patients (p < 0.05). Utilizing the recommended cutoff score of 47, 40 of the 41 brain-dysfunctional patients were correctly classified as impaired, but only seven of the medical and five of the psychiatric patients were correctly classified. Using age-based T-scores, 36 of the 41 brain-dysfunctional patients (87.8%) were correctly classified. Specificity improved slightly, but these numbers were still low (55%), primarily because psychotic patients performed like neurological patients (100%). This study provides further empirical validation of this screening instrument in identifying patients with brain disorders.

A cross-cultural study on impaired self-awareness in Japanese patients with brain dysfunction.

Japanese patients with brain dysfunction (21 with severe traumatic brain injury [TBI], 21 with right, and 21 with left cerebral vascular accidents [CVA]) were asked to make behavioral ratings regarding their competencies in several areas. Relatives of patients and physical therapists who treated them also rated each patient's behavioral competency. Japanese patients with TBI overestimated their behavioral competencies compared with therapists ratings, but not relatives' ratings. Japanese patients with TBI overstimated self-care skills but not their ability to interact in socioemotional situations. Patients who had right and left CVA did not differ in their mean ratings of behavioral competency. Among all patient groups, there was no correlation between self-reported competencies and performance on a neuropsychological test. Family ratings of patients' behavioral competencies correlated with the patients' neuropsychological test performance. Post hoc analyses of patients with TBI suggest that speed of finger tapping related to an impaired self-awareness. Whereas cultural factors may influence self-reports of behavioral competency, patients across cultures with brain dysfunction seem to have reduced insight into their actual level of neuropsychological functioning.

Suspected malingering and the Digit Memory Test: a replication and extension.

This study replicated a previous finding that suspected malingerers often perform at a level worse than patients with unequivocal brain dysfunction on the Digit Memory Test (DMT). In addition, it was demonstrated that patients suffering from aphasia as well as temporal and "frontal lobe" damage perform at a level higher than suspected malingerers. DMT performance of patients with true dementia, however, may be difficult to distinguish from suspected malingerers. If an individual is not demented (as judged by such tests as the BNI Screen for Higher Cerebral Functions) and performs at a level less than 95% across three trials of the DMT, the question of noncooperation with testing procedures or "malingering" should be considered.

Neuropsychological rehabilitation after brain injury: Scientific and professional issues.

Neuropsychological rehabilitation after acquired brain injury is an important contribution to our health-care system. Despite a growing database which supports its clinical utility, the insurance coverage for this form of care is now being threatened. This paper addresses some of the professional and scientific issues that psychologists need to face in order to continue to provide these services.

Differential response characteristics in nonepileptic and epileptic seizure patients on a test of verbal learning and memory.

Investigators have found it difficult to separate patients with nonepileptic seizures (NES) from those with true epileptic seizures (ES) using quantitative measures of neuropsychological test performance. We examined qualitative response characteristics on the California Verbal Learning Test of 41 patients undergoing continuous video/audio-EEG monitoring in an effort to distinguish these patient groups (12 patients with left temporal [LT] foci, 11 with right temporal [RT] foci, and 18 with NES). NES patients explicitly recognized fewer target words compared with ES patients. In addition, NES patients rarely made false-positive errors, which resulted in failure to endorse a significant number of items on the recognition list. This response tendency is called a negative response bias. In contrast, LT patients endorsed a high number of items on the recognition test, which resulted in a positive response bias. RT patients demonstrated no consistent response tendency. In our sample, a negative response bias index (ie, a cutoff score < 0) showed a sensitivity of 61% and a specificity of 91%. We propose that failure to explicitly recognize words following repeated exposure may reflect aspects of psychological denial in NES patients. Response bias indices may thus help identify patients with NES and may begin to explain the psychological mechanisms underlying this complex disorder.

Digit Memory Test: unequivocal cerebral dysfunction and suspected malingering.

The Digit Memory Test (DMT) (Hiscock & Hiscock, 1989), a forced-choice test for detecting malingering, was administered to 27 patients with unequivocal cerebral dysfunction, 5 patients with postconcussional syndrome, 6 suspected malingerers and 10 normal controls. Results indicate that, even in patients with severe, but static cerebral dysfunction and unequivocal memory disorder, DMT performance is between 95% to 100% correct. By contrast, the 6 patients in whom malingering was seriously considered performed at a level much below the other three groups (74% correct) but not significantly below chance. The DMT may be helpful in evaluating patients suspected of malingering even when they do not score significantly below chance.

Uses and abuses of neuropsychological testing in behavioral neurology.

This article provides a brief historical review of how psychological tests have been used to examine patients with brain dysfunction and summarizes the strengths and weaknesses of present-day neuropsychological testing procedures. Common uses and abuses of neuropsychological testing are listed with a few clinical examples.