Three patients with Gerstmann-Sträussler-Scheinker disease (GSS) caused by a serine-for-phenylalanine substitution at codon 198 of the prion protein gene (PRNP) were compared to 9 age- and education-matched non-mutation-carriers from the same large Indiana kindred (GSS–IK) on a comprehensive neuropsychological test battery. Clinically significant impairments in intelligence, secondary memory, attention and cognitive processing speed, executive ability, and manual motor skills were noted in 2 patients. The wide range and the severity of the cognitive deficits indicated generalized cerebral dysfunction consistent with global dementia. One patient, symptomatic for less than 1 year, had more selective deficits involving memory, motor skills, and verbal fluency, suggesting early subcortical involvement. (JINS, 1997, 3, 169–178.)

There have been several proposals for the mechanism by which we are able to recognize an object across a number of viewpoints. Viewpoint-dependent accounts suggest that recognition may be based on an incremental transformation (e.g., mental rotation) strategy, while a variety of viewpoint-independent mechanisms for object recognition have also been proposed. Recent research in neurobiology, based on the two cortical visual systems account, suggest that the processes of viewpoint-dependent and viewpoint-independent object recognition may rely on separate anatomical regions, and that brain lesions may leave patients with selective access to particular types of representation. Evidence from a variety of neuropsychological disorders are reviewed to support the position that viewpoint-independent object recognition depends upon the integrity of occipitotemporal structures. In addition, it is suggested that viewpoint-dependent processes (perhaps depending on occipitoparietal structures) may supplement this primary system under nonoptimal circumstances. (JINS, 1997, 3, 288–298.)

Sixteen right-handed male students were administered a unilateral lexical decision task in 4 conditions: a baseline condition and three sound conditions. In the sound conditions, the participants listened to noise, to music with a positive emotional valence, and to music with a negative emotional valence, while performing the visual half-field task. In the baseline condition, the noise condition, and the positive music condition, lexical decision latencies were shorter to right than to left visual field presentations. In the negative music condition, there was a selective enhancement of left visual field performance, which cancelled the visual field advantage completely. None of the concurrent sounds affected autonomic arousal as measured by heart rate. The results demonstrated that music with a negative emotional valence can alter the half-field asymmetry of a verbal task. The outcome was discussed in terms of right hemisphere priming due to negative emotional experience. (JINS, 1997, 3, 473–479.)

Qualitative features of the neuropsychological test performance of individuals with dementia of the Alzheimer type (DAT) were examined in a population-based study. Qualitative error scores were derived from measures of verbal and figural memory, verbal fluency and confrontation naming for 38 patients with clinically diagnosed DAT, 236 normal elderly (NE) individuals, and 72 others who were questionably demented and at risk (AR) for DAT. Persons with DAT made a greater proportion of intrusion and perseverative errors, and more lexical and semantic naming errors, than the NE participants. These measures provided fair specificity but poor sensitivity for the diagnosis of DAT, and a logistic model based on these measures correctly classified 98% of the NE participants, but only 29% of the DAT participants. The AR participants demonstrated a pattern of errors that was highly similar to that of the DAT patients, and when their scores were subjected to the logistic model, 90% were classified as NE and 10% as DAT. These results indicate that specific error types that have been associated with DAT in self-referred or clinic-based samples also occur in the general population to a greater degree in individuals with DAT or questionable dementia than in NE individuals. Furthermore, these qualitative features may have some diagnostic usefulness in that their presence provides reasonable specificity for DAT or questionable dementia. (JINS, 1997, 3, 387–393.)

Researchers disagree as to whether Lewy body disease (LBD) constitutes a variant of Alzheimer's (AD) or Parkinson's disease (PD), or alternatively, whether it is an independent disease process. The neuropathological, genetic, and clinical characteristics of LBD are reviewed and compared to those of AD and PD. Data for 150 cases of LBD reported in the literature were compiled and grouped according to neuropathological status. Patients with pure LBD (with limited or no concurrent AD pathology) tend to present at a younger age with extrapyramidal signs followed by dementia, whereas patients with mixed LBD–AD (concurrent LB and AD pathology) are somewhat older and tend to present with dementia. The cognitive profile of LBD patients, and the relationships among LBD, AD, and PD remain unclear due to methodological limitations and the paucity of studies comparing the groups directly. (JINS, 1997, 3, 179–194.)

Neuropsychological outcome was evaluated in a prospective, longitudinal follow-up study of children age 4 months to 7 years at injury with either mild-to-moderate (N = 35) or severe (N = 44) traumatic brain injury (TBI). Age-appropriate tests were administered at baseline, 6 months, 12 months, and 24 months after the injury. Performance was compared on (1) composite IQ and motor, (2) receptive and expressive language, and (3) Verbal and Perceptual–Performance IQ scores. In comparison to mild-to-moderate TBI, severe TBI in infants and preschoolers produced deficits in all areas. Interactions between task and severity of injury were obtained. Motor scores were lower than IQ scores, particularly after severe TBI. Both receptive and expressive scores were reduced following severe TBI. Expressive language scores were lower than receptive language scores for children sustaining mild-to-moderate TBI. While severe TBI lowered both Verbal and Perceptual– Performance IQ scores, Verbal IQ scores were significantly lower than Perceptual–Performance IQ scores after mild-to-moderate TBI. Mild injuries may produce subtle linguistic changes adversely impacting estimates of Verbal IQ and expressive language. Within the limited age range evaluated within this study, age at injury was unrelated to test scores: The impact of TBI was comparable in children ages 4 to 41 months versus 42 to 72 months at the time of injury. All neuropsychological scores improved significantly from baseline to the 6-month follow-up. However, no further change in scores was observed from 6 to 24 months after the injury. The persistent deficits and lack of catch-up over time suggest a reduction in the rate of acquisition of new skills after severe TBI. Methodological issues in longitudinal studies of young children were discussed. (JINS, 1997, 3, 581–591.)

Some persons with Alzheimer's disease (AD) lose the ability to recognize themselves, as when they cannot overtly recognize their reflection in a mirror. There is evidence, however, that covert or unconscious self-recognition might be displayed in such individuals. In this study, 3 persons with AD lacking the ability to overtly self-recognize demonstrated multiple instances of unconscious or covert self-recognition. A variety of interventions, inspired by research with prosopagnosics, was implemented to remediate this loss. Interventions enabled all participants to exhibit overt self-recognition, though each did so with the aid of a different intervention. In addition, successful overt self-recognition required a verbal probe and was entirely intervention-dependent: When the intervention was removed, overt self-recognition was lost. Results support a dissociation between explicit–declarative versus implicit–nondeclarative memory systems, and extends this dissociation into the realm of self-recognition in AD. (JINS, 1997, 3, 195–198.)

The preoperative cognitive test performance of 77 candidates for elective open-heart surgery that did not suffer from carotid artery stenosis, was compared with the performance of 37 normal controls. Multivariate analysis of variance, with state anxiety score and depression score as covariates, showed a significantly reduced cognitive performance in the surgical candidates. Post hoc analyses using univariate F tests revealed a significantly impaired word fluency, manual dexterity, verbal learning, and psychomotor speed. A stepwise multiple regression analysis revealed that, besides the usual demographic factors, preoperative variables indicating a more precarious heart function also contributed significantly to the estimation of the proportion of variance of the results on several impaired cognitive tests. Future research should focus on the etiology of the cognitive impairment in cardiovascular disease. (JINS, 1997, 3, 480–484.)

This article examines the evidence that the effect of head injury on young children may differ from that in adults, in that while in the latter the pattern is of deficits that recover with time since the accident, this is not necessarily the case with very young children. In this group, there may be no evidence of any deficit in the early days or weeks after injury, but the children may fail to develop some skills as quickly as children who have not had a head injury. Results from a series of studies of MHI in preschool children carried out over a more than 10-year period from Auckland Hospital and recently published studies of pediatric MHI from other groups are reviewed. It is concluded from a comparison of these data that there is a need for long-term prospective studies designed within a developmental framework to clarify the issue. (JINS, 1997, 3, 592–597.)

Introducing ice-water into the left ear of right-brain-damaged patients attenuates unilateral neglect symptoms. By examining EEG changes over each hemisphere during this procedure, we were able to test a hypothesis concerning the mechanism of cold-water calorics and the attention–arousal hypothesis of hemispatial neglect. We present a case study of an 83-year-old woman with a massive right-hemisphere CVA exhibiting severe hemispatial neglect. Caloric stimulation produced a leftward eye deviation to central position, and a temporary partial remission of neglect symptoms. Significant changes in EEG activation indicated a central mechanism associated with the regularization of eye gaze. Caloric stimulation also produced a significant interaction between EEG frequency band and hemisphere, indicating that while both hemispheres increased in cortical activation, the right hemisphere increase was significantly greater. This supports the activation–arousal hypothesis of neglect over the mutual inhibition model. (JINS, 1997, 3, 394–402.)

This issue of JINS sees the merging of two of its departments: “Critical Reviews/Updates” and “Dialogues” have been combined to form “Critical Reviews & Dialogues.”

In recent years, efforts have been made to computerize standard neuropsychological (NP) tests (Kane & Kay, 1992). This effort is in the service of increased productivity, lower costs, and an increase in the consistency of test administration. However, care must be taken to make sure the limitations of the computer interface do not compromise test validity.

To further investigate the usefulness of 3 purported measures of executive function (EF) in head injured children, we administered the Twenty Questions Test (TQT), Tower of London (TOL), and the Wisconsin Card Sorting Test (WCST) to 151 children who had sustained a closed head injury (CHI) of varying severity about 3 years earlier. In addition, we tested 89 normal controls. Fifty-seven of the patients were included in a longitudinal study that compared performance at 3 months and 36 months. All of the head injured children underwent magnetic resonance imaging for investigational purposes. Severity of CHI, as defined by the lowest Glasgow Coma Scale (GCS) score, affected performance on all 3 EF measures. Focal lesion volume incremented prediction of performance on TOL and WCST, but not TQT. Moderate intercorrelations of the test variables were obtained. Although all three EF measures depicted changes in performance over 3 years, a ceiling effect detracted from the sensitivity of the TOL to the impact of CHI on development. Implications of the findings for clinical applications are discussed. (JINS, 1997, 3, 598–607.)

Within the context of a longitudinal study investigating outcome for children following traumatic brain injury, this paper reports on the utility of neuropsychological testing in predicting academic outcome in children 2 years following traumatic brain injury (TBI). Twenty-nine school-age children who were admitted to hospital after TBI were assessed with a battery of neuropsychological and academic measures at 3 and 24 months postinjury. The neuropsychological battery included measures of memory, learning, and speed of information processing. Academic outcome was assessed in terms of post-TBI change in school placement. According to logistic regression analysis, change in placement from regular to special education at 2 years post-TBI was predicted by injury severity and by neuropsychological performance at 3 months post-TBI. Findings suggest that neuropsychological testing is useful in identifying children with special educational needs subsequent to TBI. (JINS, 1997, 3, 608–616.)

For too many members of our discipline, the practice of clinical neuropsychology is synonymous with the administration and interpretation of specialized tests of brain functioning. We have, in fact, become quite sophisticated in the assessment of brain-disordered patients. But the development of effective programs of neuropsychological treatment (which includes, but is not limited to, “cognitive rehabilitation”) remains one of the most pressing needs and awesome challenges for clinical and research neuropsychology.