But the Scores Don't Show How I Really Function: A Feedback Method to Reveal Cognitive Distortions Regarding Normal Neuropsychological Test Performance.

Neuropsychologists commonly provide feedback to patients with a high degree of cognitive complaints in the context of normal neuropsychological test performance. Patients are sometimes not reassured by this feedback because they blame external factors (e.g., the tests are wrong) and do not develop insight about internal factors (e.g., distorted self-perception) that could lead to better understanding about why high symptom reporting is present in the context of normal test results. This article presents the first formal feedback model for neuropsychologists to use with such patients that helps reveal the presence of cognitive distortions that result in high cognitive complaints. The critical elements of this model incorporate self-ratings of neuropsychological test performance, comparing those ratings to actual test performance, and presenting discrepancies to patients in an objective, professional, and respectful manner. Ways in which this feedback model may lead to improved patient outcomes and reductions in healthcare costs are discussed.

CADASIL and multiple sclerosis: A case report of prolonged misdiagnosis.

CADASIL is Cerebral Autosomal-Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy [corrected]. (CADASIL) is sometimes misdiagnosed as multiple sclerosis (MS). MS and CADASIL are not known to co-occur and brain magnetic resonance imagining (MRI) findings can help with differential diagnosis. Despite the availability of this information, a case report is presented of a 61-year-old woman who was misdiagnosed with MS at age 50, tested positive for CADASIL at age 56, described incorrectly as having both conditions simultaneously, and continued on MS disease-modifying medications, resulting in financial and physical hardship. Neuropsychological consultation helped initiate removal of the MS diagnosis and treatment. Better understanding is needed among clinicians that MS and CADASIL are not known to co-exist, that no association has been found between MS and the NOTCH3 mutations that cause CADASIL, and that neuroimaging and clinical features can help distinguish between the two conditions in addition to genetic testing. This case study highlights how neuropsychological consultation involves more than testing, can help improve diagnostic decision making, and can improve outcomes by reducing costs to the patient and the healthcare system.

Assessment of response bias in neurocognitive evaluations.

BACKGROUND: In clinical neuropsychological practice, assessment of response validity (e.g., effort, over-reporting, under-reporting) is an essential component of the assessment process. By contrast, other health care professionals, including those in neurorehabilitation settings, often omit assessment of this topic from their evaluations or only rely on subjective impressions. OBJECTIVE: To provide the first comprehensive review of response validity assessment in the neurorehabilitation literature, including why the topic is often avoided, what methods are commonly used, and how to decrease false positives. METHODS: A literature review and documentation of personal experience and perspectives was used to review this topic. RESULTS: There is a well-established literature on the necessity and utility of assessing response validity, particularly in patients who have external incentives to embellish their presentation or to under-report symptoms. There are many reasons why non-neuropsychologists typically avoid assessment of this topic. This poses a significant problem, particularly when patients exaggerate or malinger, because it can lead to misdiagnosis and it risks increasing the cost of healthcare by performing unnecessary tests and treatments, unfair distribution of disability/compensation resources, and a reduced access to these and other health resources by patients who genuinely need them. CONCLUSIONS: There is a significant need for non-neuropsychologists to develop and incorporate symptom and performance validity assessments in clinical evaluations, including those in neurorehabilitation settings.

Assessment of effort in children: a systematic review.

The assessment of response validity is now considered an important and necessary component of neuropsychological evaluations. One way for assessing response validity is with performance validity tests (PVTs), which measure the degree of effort applied to testing to achieve optimal performance. Numerous studies have shown that normal and neurologically impaired children are capable of passing certain free-standing PVTs using adult cutoffs. Despite this, PVT use appears to be more common in adults compared to children. The overall purpose of this systematic review is to provide the reader with a general overview of the existing literature on PVTs in children. As part of this review, goals are to inform the reader why PVT use is not as prevalent in children compared to adults, to discuss why PVTs and related methods are important in pediatric cognitive evaluations, and to discuss practical limitations and future directions.

Dementia does not preclude very reliable responding on the MMPI-2 RF: a case report.

The Minnesota Multiphasic Personality Inventory (MMPI) family of personality tests has long been used by psychologists, in part because it provides extensive information on the validity of patient responses. Although much of the research on MMPI validity indicators has focused on over-reporting or under-reporting symptoms, the consistency (i.e., reliability, a requirement for validity) of responding is also critical to examine. Clinicians tend to avoid using the MMPI-2 or the MMPI-2-RF (Restructured Form) in patients with dementia based on the belief that severe cognitive impairment would make reliable responding impossible given the large number of items (567 and 338, respectively). In contrast with this belief we present the case of a 65-year-old woman with severe memory impairments and executive dysfunction due to a non-specific dementia syndrome who was able to provide remarkably consistent responding on the MMPI-2-RF. Implications and future directions are discussed.

Word memory test profiles in two cases with surgical removal of the left anterior hippocampus and parahippocampal gyrus.

One principle underlying the use of the Word Memory Test (WMT) as an effort test is that with good effort, recognition scores above the cutoffs will be observed. However, to understand the limits of effort testing, it is necessary to study people known to have severe impairment and significant neuropathology involving memory structures. Goodrich-Hunsaker and Hopkins ( 2009 ) reported that three amnesic patients with bilateral hippocampal damage had severely impaired free recall of the WMT word list but passed the recognition subtests of the WMT, which are often called effort subtests. We tested two patients with surgical resections in the left anterior temporal region to treat chronic intractable epilepsy; both patients had suffered postoperative strokes. Patient A was a 15-year-old boy and Patient B was a 58-year-old woman. Despite destruction of the left anterior hippocampus and the parahippocampal gyrus and despite impairment of free recall, both cases passed the easy WMT effort subtests. These data reinforce previous findings that people with severe impairment of free recall will score much higher on the verbal recognition memory subtests than on the more difficult memory subtests. Even severe memory impairment and/or removal of hippocampal areas do not necessarily lead to failure on the easy WMT recognition subtests.

Young child with severe brain volume loss easily passes the word memory test and medical symptom validity test: implications for mild TBI.

The Word Memory Test (WMT) and Medical Symptom Validity Test (MSVT) are two commonly used free-standing measures of test-taking effort. The use of any test as a measure of effort is enhanced when evidence shows that it can be easily passed by patients with severe neurological conditions. The opportunity arose to administer the WMT and MSVT to a 9-year-old girl (referred to as CJ) with severe congenital bilateral brain tissue loss (shown via a compelling brain MRI image), chronic epilepsy, an extremely low Full Scale IQ, extremely low adaptive functioning, developmental delays, numerous severe cognitive impairments, and treatment with multiple high-dose benzodiazepines. She received extensive early intervention services and numerous academic accommodations. Despite this set of problems, CJ passed the WMT and MSVT at perfect to near perfect levels. Implications for failure on these tests among patients with known or alleged mild traumatic brain injury are discussed.

Regionally distinct white matter lesions do not contribute to regional gray matter atrophy in patients with multiple sclerosis.

PURPOSE: To determine to what extent T1- and T2-regional lesion volumes (RLVs) contribute to total and/or regional gray matter (GM) atrophy in multiple sclerosis (MS). METHODS: We studied 110 (67 relapsing-remitting and 43 secondary-progressive) MS patients. SABRE program was used to parcel the brain into 13 regions per hemisphere. Total and regional GM fractions (GMFs) were determined in each region to correct for intraregional size variability. Partial correlations were used to determine associations (holding the converse constant) between RLVs, GMF, and regional GMFs (P < .001 to avoid Type 1 error). RESULTS: Partial correlations between RLVs and regional GMFs (controlling for total GMF) for the total MS group were not significant for any of the 26 regions for T2, whereas they were significant for two of the 26 regions for T1. Partial correlations between RLVs and total GMF (controlling for regional GMF) for the total MS group were significant in 9 of 26 regions for T2 (largest r = right lateral inferior frontal, -.45) and 5 of 26 regions for T1 (largest r = right inferior parietal, -.45). CONCLUSIONS: Results suggest a model whereby a distinct generalized disease process accounts for GM atrophy better than regionally distinct Wallerian degeneration.

A model to approaching and providing feedback to patients regarding invalid test performance in clinical neuropsychological evaluations.

The use of symptom validity assessment has become commonplace in clinical neuropsychological evaluations. However, clinicians often struggle with how to provide patients with feedback regarding invalid responding or effort, because of the sensitive nature of the information that must be conveyed. A conceptual framework for providing such feedback is outlined in clinical neuropsychological evaluations, and recommendations for how to handle complaints are offered. Our feedback model is not meant to apply to individuals referred by attorneys or other non-clinical third parties (e.g., independent medical examination companies).

Children with moderate/severe brain damage/dysfunction outperform adults with mild-to-no brain damage on the Medical Symptom Validity Test.

PRIMARY OBJECTIVE: This study sought independent confirmation that the English computerized Medical Symptom Validity Test can be easily passed by children with moderate-to-severe brain injury/dysfunction (e.g. traumatic brain injury, stroke) and/or developmental disabilities. In addition, it was hypothesized that a higher percentage of such children would pass the MSVT compared to adults with mild traumatic brain injury or head injury (MTBI/HI) and would rate the task as easier. METHODS: Thirty-eight children and 67 adults were administered the MSVT during an outpatient neuropsychological evaluation. RESULTS: Two children (5%) failed the MSVT, whereas 14 (21%) of adults failed. Children performed significantly better on the MSVT and rated it as significantly easier compared to adults who failed the MSVT. There were no such differences when children were compared to adults who passed the MSVT. CONCLUSIONS: Findings independently validate the use of the MSVT with children and demonstrate symptom exaggeration in a sub-set of adult MTBI/HI patients.

Regional specificity of magnetization transfer imaging in multiple sclerosis.

BACKGROUND: The goal of this study was to develop and validate a method for generation of regional magnetization transfer ratio (MTR). We also studied the topography of MTR changes in multiple sclerosis (MS) and in normal controls (NC), and preliminarily examined the clinical usefulness of this method. METHODS: We examined 45 patients with MS (relapsing remitting [RR] = 28 and secondary progressive[SP] = 17] and 19 NC. Mean disease duration was 14.3 years and median Expanded Disability Status Scale was 3.0. Regions of the brain were determined using semiautomated brain region extraction (SABRE). Twenty-six regional masks were automatically applied to MTR maps that were further split into gray matter (GM) and white matter (WM)compartments. RESULTS: Mean MTR from 12 SABRE regions differed significantly between MS patients and NC. For WM, all regional mean MTRs differed significantly between RR, SP, and NC participants(P < .001). In regression analysis, only 3 regions remained significantly different when corrected for total T2-LV. The regression model predicting disability selected GM mean MTR of the right medial inferior frontal region (P = .031). CONCLUSIONS: The study results showed that this regional MTR approach is reproducible, reliable and clinically relevant. MTI changes occur selectively in specific sub-regions.

Independent contributions of cortical gray matter atrophy and ventricle enlargement for predicting neuropsychological impairment in multiple sclerosis.

The primary goal of this study was to investigate associations between regional gray matter (GM) atrophy and neuropsychological function in multiple sclerosis (MS), while accounting for the influence of central brain atrophy (i.e. third ventricle enlargement). Using a cross-sectional design, we studied 59 MS patients with brain MRI and neuropsychological testing. Regional gray matter fractions (rGMFs) were calculated from MRI images for 11 homologous brain areas using the semiautomatic brain region extraction (SABRE) technique. Neuropsychological testing followed consensus panel guidelines and included tests emphasizing episodic memory, working memory and processing speed. The analytic approach was stepwise linear regression, with forward selection and p<0.05 threshold for significance. Consistent with previous research, there were significant correlations between third ventricle width and neuropsychological tests. Stepwise linear regression analyses controlling for third ventricle width retained rGMFs obtained from specific regions within the prefrontal cortex. Left frontal atrophy was associated with tests emphasizing auditory/verbal memory. Right frontal atrophy was associated with impairment in visual episodic and working memory. For the first time, we show an independent relationship between cortical atrophy and cognitive impairment after accounting for the effects of central atrophy.

Progressive cerebral disease in lymphomatoid granulomatosis causes anterograde amnesia and neuropsychiatric disorder.

The authors report neuropsychological (NP) and serial quantitative magnetic resonance imaging (MRI) findings of a 29-year-old woman with lymphomatoid granulomatosis (LG). Disease course was characterized by acute psychosis, tremor, fever, seizures, and progressive cognitive impairment. At the time of symptom onset, brain MRI revealed mild lesion volume and normal parenchymal volume. This was followed by dramatic progression of brain lesions and atrophy over 2 years, at which point the patient expired. Atrophy was most prominent in the mesial temporal lobes. NP testing revealed marked amnesia and mild impairments in other cognitive domains. To our knowledge, this is the first recorded case of LG in which bilateral temporal lobe atrophy is evident and accompanied by anterograde amnesia. We speculate that temporal lobe atrophy was influenced by the established susceptibility of this region in various neurological diseases.

Regional lobar atrophy predicts memory impairment in multiple sclerosis.

BACKGROUND AND PURPOSE: In recent studies, measures of whole brain atrophy were strongly correlated with neuropsychological testing, explaining more variance than measures of lesion burden in patients with multiple sclerosis. The relationship between regional lobar atrophy and cognitive impairment is yet to be examined. We endeavored to assess the clinical significance of regional lobar atrophy in multiple sclerosis. METHODS: In a cross-sectional study, we evaluated 31 patients with multiple sclerosis with brain MR imaging and neuropsychological testing. Impairment was determined by comparison with demographically matched healthy controls. MR imaging generated measures of lesion burden (fluid-attenuated inversion recovery hyperintense volume), general atrophy (brain parenchymal fraction), central atrophy (lateral ventricle volume), and lobar atrophy (regional brain parenchymal fraction of frontal, temporal, parietal, and occipital lobes in each hemisphere). Neuropsychological testing emphasized measures of processing speed and memory, because these are commonly affected in multiple sclerosis. RESULTS: Patients with multiple sclerosis showed significant atrophy and impairment on all neuropsychological tests. Regional atrophy accounted for the most variance in all regression models predicting memory performance. Left temporal atrophy was the primary predictor of auditory/verbal memory (partial r's = 0.55-0.61), and both left and right temporal atrophy predicted visual/spatial memory performance (partial r's = 0.51-0.67). Models predicting learning consistency retained frontal lobe atrophy measures (partial r's = 0.44-0.68). Central and general atrophy measures were the primary predictors in modeling processing speed (partial r's = 0.42-0.64). CONCLUSION: Regional atrophy accounts for more variance than lesion burden, whole brain atrophy, or lateral ventricle volume in predicting multiple sclerosis-associated memory dysfunction.

Correlating brain atrophy with cognitive dysfunction, mood disturbances, and personality disorder in multiple sclerosis.

Neuropsychological impairment is a common feature of multiple sclerosis. Affected patients often have deficits in information-processing speed and memory and exhibit psychopathological states such as depression. A minority of patients have rarer affect/mood disorders such as euphoria sclerotica and pathological laughter/crying. Neuropsychological impairment is a major predictor of low quality of life, unemployment, and caregiver distress. Studies evaluating correlations between neuropsychological impairment and findings on magnetic resonance imaging show that neuropsychological dysfunction is associated with lesion burden and diffuse disease in normal-appearing brain tissue. However, measures of tissue atrophy including whole-brain and central atrophy are especially well correlated with and predictive of cognitive impairment. Moreover, recent studies have shown that conventional measures of brain atrophy explain more variance in neuropsychological dysfunction than do measures of lesion burden. In particular, neuropsychological outcomes correspond highly with linear measures of subcortical atrophy such as ventricle enlargement. Within the domain of emotional dysfunction, both lesion burden and atrophy are related to major depressive disorder. Brain atrophy also predicts euphoria and disinhibition. The preliminary data suggest that although lesion burden primarily predicts depressive disorder, both lesion burden and atrophy predict the presence of euphoria. Euphoria and disinhibition likely represent personality change associated with worsening cognition as the disease progresses. Taken together, this growing body of data on magnetic resonance imaging to neuropsychological correlations supports the clinical relevance and validity of brain atrophy as a measure of disease progression in multiple sclerosis. Continuing research focuses on the possible relationship between measures of regional brain atrophy and cognitive and emotional impairment.