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The behavioural variant of frontotemporal dementia (bvFTD) is a frequent cause of early-onset dementia. The diagnosis of bvFTD remains challenging because of the limited accuracy of neuroimaging in the early disease stages and the absence of molecular biomarkers, and therefore relies predominantly on clinical assessment. BvFTD shows significant symptomatic overlap with non-degenerative primary psychiatric disorders including major depressive disorder, bipolar disorder, schizophrenia, obsessive-compulsive disorder, autism spectrum disorders and even personality disorders. To date, ∼50% of patients with bvFTD receive a prior psychiatric diagnosis, and average diagnostic delay is up to 5-6 years from symptom onset. It is also not uncommon for patients with primary psychiatric disorders to be wrongly diagnosed with bvFTD. The Neuropsychiatric International Consortium for Frontotemporal Dementia was recently established to determine the current best clinical practice and set up an international collaboration to share a common dataset for future research. The goal of the present paper was to review the existing literature on the diagnosis of bvFTD and its differential diagnosis with primary psychiatric disorders to provide consensus recommendations on the clinical assessment. A systematic literature search with a narrative review was performed to determine all bvFTD-related diagnostic evidence for the following topics: bvFTD history taking, psychiatric assessment, clinical scales, physical and neurological examination, bedside cognitive tests, neuropsychological assessment, social cognition, structural neuroimaging, functional neuroimaging, CSF and genetic testing. For each topic, responsible team members proposed a set of minimal requirements, optimal clinical recommendations, and tools requiring further research or those that should be developed. Recommendations were listed if they reached a ≥ 85% expert consensus based on an online survey among all consortium participants. New recommendations include performing at least one formal social cognition test in the standard neuropsychological battery for bvFTD. We emphasize the importance of 3D-T1 brain MRI with a standardized review protocol including validated visual atrophy rating scales, and to consider volumetric analyses if available. We clarify the role of 18F-fluorodeoxyglucose PET for the exclusion of bvFTD when normal, whereas non-specific regional metabolism abnormalities should not be over-interpreted in the case of a psychiatric differential diagnosis. We highlight the potential role of serum or CSF neurofilament light chain to differentiate bvFTD from primary psychiatric disorders. Finally, based on the increasing literature and clinical experience, the consortium determined that screening for C9orf72 mutation should be performed in all possible/probable bvFTD cases or suspected cases with strong psychiatric features. [ABSTRACT FROM AUTHOR]

Objective Research has shown that neurologic conditions, such as traumatic brain injury and multiple sclerosis, result in a number of cognitive and functional deficits. However, little is known about the relationship between various cognitive domains and ability to perform everyday activities. The Community Shopping Task (CST), a naturalistic assessment task conducted in a simulated environment, was used to examine functional abilities and cognitive correlates of everyday functioning in individuals with neurologic conditions. Method Thirty-four participants with neurologic conditions and 34 healthy controls completed the CST as well as traditional paper–pencil measures of cognition. In addition, all participants completed a questionnaire assessing instrumental activities of daily living (IADLs). Results The results indicated that participants with neurologic conditions required significantly more cues and time to complete the CST compared to control participants and that immediate memory and executive functioning were important predictors of CST performance. Furthermore, time to complete the CST accounted for a significant amount of variance in IADL performance, over and beyond the traditional measures of cognition. Conclusions These results provide evidence that a naturalistic task completed in an everyday environment can enhance our understanding of how daily functioning is impacted in individuals with neurologic conditions and subsequently inform rehabilitation strategies. [ABSTRACT FROM AUTHOR]

Abstract: This paper presents a historical overview and current perspective of the Automated Neuropsychological Assessment Metrics (ANAM®) test system. We discuss the history of its development along with a synopsis of the evolution of computerized testing that has occurred and led to ANAM® over the past 30 years within the Department of Defense (DoD). We include a description of our current system and test library. Finally, we present an overview of advanced development projects that are presently underway. We have intentionally avoided addressing issues of reliability, stability, clinical sensitivity, and construct validity in this paper. These issues are presented in other reports in this special issue. [Copyright &y& Elsevier]

Objective. Clinical practice guidelines recommend the radial nerve mechanosensitivity evaluation in patients with lateral epicondylalgia. Despite different positions and sequences having been described, no research analyzed how each variation triggers symptoms associated with lateral epicondylalgia. The aim of this study was to explore the effects of different positions and sequences in the upper limb neural tension test 2b (ULNT2b) in symptom responses in patients with lateral epicondylalgia. Methods. In this observational study, 66 patients underwent 4 test conditions: standard ULNT2b, ULNT2b proximal to distal, ULNT2B with resisted supination, and resisted supination isolated. Paresthesia sensations, symptom reproduction, pain intensity (measured using a visual analog scale), and distribution of painful symptoms data were collected. Results. Significant differences in paresthesia sensations were observed between groups, with significant differences between the standard ULNT2B and other ULNT variations or resisted supination maneuvers. Symptom reproduction also differed significantly across groups, with significant differences between the standard ULNT2B and other ULNT or resisted supination tests. The positive/negative test and percentage of distribution of painful symptoms scores varied significantly across 4 conditions in both lateral and frontal views. Although pain intensity scores during tests were comparable among the tests, distribution of painful symptoms differed significantly. Conclusion. Variations in the ULNT2b test can affect symptom responses in patients with lateral epicondylalgia. The standard ULNT2b test appears more effective at reproducing symptoms, intensity of paresthesia, and distribution of painful symptoms compared to other ULNT variations and the resisted supination test. Impact. ULNT2b sequences have been shown to elicit varying responses concerning paresthesia, replication of familiar symptoms, positive/negative test results, and distribution of painful symptoms. Clinicians should consider specific test variations during the patients' radial nerve mechanosensitivity assessment to identify aggravating factors reproducing recognizable symptoms. A control group of asymptomatic participants and the role of presence of other comorbidities, psychological factors, or the duration of symptoms were not considered in this study and might play an important role influencing the results of the tests. [ABSTRACT FROM AUTHOR]

Memory assessment is an important component of a neuropsychological evaluation, but far fewer visual than verbal memory instruments are available. We examined the preliminary psychometric properties and clinical utility of a novel, motor-free paper and pencil visuospatial memory test, the Indiana faces in places test (IFIPT). The IFIPT and general neuropsychological performance were assessed in 36 adults with amnestic mild cognitive impairment (aMCI) and 113 older adults with no cognitive impairment at baseline, 1 week, and 1 year. The IFIPT is a visual memory test with 10 faces paired with spatial locations (three learning trials and non-cued delayed recall). Results showed that MCI participants scored lower than controls on several variables, most notably total learning (p < .001 at all three time points), delayed recall (baseline p = .03, 1 week p < .001, 1 year p < .001), and false-positive errors (range p = .03 to <0.001). The IFIPT showed similar test–retest reliability at 1-week and 1-year follow-up to other neuropsychological tests (r = 0.71–0.84 for MCI and 0.53–0.72 for controls). Diagnostic accuracy was modest for this sample (areas under the receiver operating characteristic curve between 0.64 and 0.66). Preliminary psychometric analyses support further study of the IFIPT. The measure showed evidence of clinical utility by demonstrating group differences between this sample of healthy adults and those with MCI. [ABSTRACT FROM PUBLISHER]

This paper describes the methods and sample characteristics of a series of Spanish normative studies (The NEURONORMA project). The primary objective of our research was to collect normative and psychometric information on a sample of people aged over 49 years. The normative information was based on a series of selected, but commonly used, neuropsychological tests covering attention, language, visuo-perceptual abilities, constructional tasks, memory, and executive functions. A sample of 356 community dwelling individuals was studied. Demographics, socio-cultural, and medical data were collected. Cognitive normality was validated via informants and a cognitive screening test. Norms were calculated for midpoint age groups. Effects of age, education, and sex were determined. The use of these norms should improve neuropsychological diagnostic accuracy in older Spanish subjects. These data may also be of considerable use for comparisons with other normative studies. Limitations of these normative data are also commented on. [ABSTRACT FROM PUBLISHER]

Abstract: Conducting assessment with individuals with physical disability, visual impairment or blindness, and hearing impairment or deafness poses significant challenges for the neuropsychologist. Although standards for psychological testing have been devised to address assessment of persons with disabilities, little research has been conducted to validate neuropsychological test accommodation and modification practices that deviate from standard test administration or to develop test parameters and interpretive guidelines specifically for persons with different physical or sensory disabilities. This paper reviews issues pertaining to neuropsychological test selection and administration, common accommodation and modification practices, test development and validation, and disability-related factors that influence interpretation of test results. Systematic research is needed to develop methodological parameters for testing and to ensure reliable and valid neuropsychological assessment practices for individuals with physical or sensory disabilities. [Copyright &y& Elsevier]

Abstract: Cognition has become increasingly important as an outcome measure in studies of medication. Traditional neuropsychological assessment is limited in its ability to detect subtle, medication-related changes and it is not suitable for the rapid serial assessment required in most clinical trials. Thus, investigators have turned to computerized neuropsychological assessment for its repeatability, sensitivity to subtle cognitive changes, and ease of administration. The automated neuropsychological assessment metrics (ANAM) is one such computerized battery that has been used to measure the effects of numerous CNS-active drugs. This paper is an exhaustive review of studies that have used ANAM to measure cognitive changes associated with pharmacological treatments. The benefits and limitations of using ANAM in clinical trials are discussed. [Copyright &y& Elsevier]

Abstract: This paper describes the development and ongoing validation of the ANAM-sports medicine battery (ASMB) for use in concussion surveillance and management. A review of previous research utilizing the ASMB highlights current issues in concussion surveillance including: tracking cognitive recovery, effect of previous concussion history on acute concussion presentation, and clinical decision making using computerized measures. ASMB interpretation using reliable change indices or impairment indices is highlighted. Future development of the ASMB is discussed as it relates to interpretation of ASMB, development of appropriate norms, and defining adequate baseline assessment. This includes the definition of practice effects, the effects of maturation on test performance and definition of adequate baseline assessment that clearly defines a subject''s normal cognitive performance level. ASMB is ready for cautious introduction into clinical practice for use by neuropsychologists with experience in both sports concussion and computerized testing. [Copyright &y& Elsevier]

Abstract: In this paper, we summarize studies that used ANAM tests to assess the effects of environmental stressors. The findings document performance changes resulting from conditions relevant to military operational medicine. These conditions included radiation exposure, toxins, high altitude, undersea conditions, Marine basic training, advanced military training, and fatigue. The results of these studies demonstrate that ANAM detects cognitive changes in extreme environments. [Copyright &y& Elsevier]

Abstract: ANAM measures were based on procedures used in both clinical and performance assessment. This paper reviews data from studies designed to delineate specific neurocognitive functions assessed by more commonly used ANAM measures. Data are brought together from both exploratory and confirmatory analyses. Current findings support the use of these measures for assessing aspects of attention, processing efficiency, and working memory. Recommendations for further research include the need for larger samples and the inclusion of specific marker variables. [Copyright &y& Elsevier]

Background Rocky Mountain spotted fever (RMSF) is a deadly tickborne disease disproportionately affecting Arizona tribal communities. While the acute clinical effects of RMSF are well-documented, more complete understanding of the long-term health consequences is needed to provide guidance for providers and patients in highly impacted areas. Methods We performed a retrospective review of hospitalized RMSF cases from 2 tribal communities in Arizona during 2002–2017. Medical records from acute illness were abstracted for information on clinical presentation, treatment, and status at discharge. Surviving patients were interviewed about disease recovery, and patients reporting incomplete recovery were eligible for a neurologic examination. Results Eighty hospitalized cases of RMSF met our inclusion criteria and were reviewed. Of these, 17 (21%) resulted in a fatal outcome. Among surviving cases who were interviewed, most (62%) reported full recovery, 15 (38%) reported ongoing symptoms or reduced function following RMSF illness, and 9 (23%) had evidence of neurologic sequelae at the time of examination. Sequelae included impaired cognition, weakness, decreased deep tendon reflexes, seizures, and cranial nerve dysfunction. Longer hospitalization (25.5 days vs 6.2 days, P <.001), a higher degree of disability at discharge (median modified Rankin score 1 vs 0, P =.03), and delayed doxycycline administration (6.2 days vs 4.1 days, P =.12) were associated with long-term sequelae by logistic regression. Conclusions Although the etiology of sequelae is not able to be determined using this study design, life-altering sequelae were common among patients surviving severe RMSF illness. Delayed administration of the antibiotic doxycycline after day 5 was the strongest predictor of morbidity. [ABSTRACT FROM AUTHOR]

Abstract: In this article we review studies in which Automated Neuropsychological Assessment Metrics (ANAM) measures were used to screen for impairment in various clinical populations. These clinical groups include patients with multiple sclerosis, systemic lupus erythematosus, Parkinson''s disease, Alzheimer''s dementia, acquired brain injury, and migraine headache. Data are also presented from a group of outpatient referrals unselected with respect to clinical condition. Findings support the use of ANAM as a screening procedure for identifying the impaired patient. [Copyright &y& Elsevier]

Abstract: Computerized neuropsychological assessment has integrated slowly into research and practice since the introduction of the personal computer. Though initial integration of technology to the laboratory and clinical setting utilized specialized hardware and software, newer generation assessment tools are integrated with “off-the-shelf” operating systems. Further, neuropsychological assessment is beginning to find Internet-based application for remote assessment. As these applications are more broadly applied, it is essential to understand potential errors that can be created both in test administration and in reaction time measurement due to hardware and software interactions. In this article, user considerations are specifically addressed for resident and Internet-enabled assessment software. Potential hardware and software conflicts are defined and potential remediation is suggested. Computerized assessment is a valuable tool for neuropsychologists as long as it is used responsibly with an understanding of the potential technical complications. [Copyright &y& Elsevier]

Stroke is a leading cause of disability, with deficits encompassing multiple functional domains. The heterogeneity underlying stroke poses significant challenges in the prediction of post-stroke recovery, prompting the development of neuroimaging-based biomarkers. Structural neuroimaging measurements, particularly those reflecting corticospinal tract injury, are well-documented in the literature as potential biomarker candidates of post-stroke motor recovery. Consistent with the view of stroke as a 'circuitopathy', functional neuroimaging measures probing functional connectivity may also prove informative in post-stroke recovery. An important step in the development of biomarkers based on functional neural network connectivity is the establishment of causality between connectivity and post-stroke recovery. Current evidence predominantly involves statistical correlations between connectivity measures and post-stroke behavioural status, either cross-sectionally or serially over time. However, the advancement of functional connectivity application in stroke depends on devising experiments that infer causality. In 1965, Sir Austin Bradford Hill introduced nine viewpoints to consider when determining the causality of an association: (i) strength; (ii) consistency; (iii) specificity; (iv) temporality; (v) biological gradient; (vi) plausibility; (vii) coherence; (viii) experiment; and (ix) analogy. Collectively referred to as the Bradford Hill Criteria, these points have been widely adopted in epidemiology. In this review, we assert the value of implementing Bradford Hill's framework to stroke rehabilitation and neuroimaging. We focus on the role of neural network connectivity measurements acquired from task-oriented and resting-state functional MRI, EEG, magnetoencephalography and functional near-infrared spectroscopy in describing and predicting post-stroke behavioural status and recovery. We also identify research opportunities within each Bradford Hill tenet to shift the experimental paradigm from correlation to causation. [ABSTRACT FROM AUTHOR]

Since the first demonstrations of network hyperexcitability in scientific models of Alzheimer's disease, a growing body of clinical studies have identified subclinical epileptiform activity and associated cognitive decline in patients with Alzheimer's disease. An obvious problem presented in these studies is lack of sensitive measures to detect and quantify network hyperexcitability in human subjects. In this study we examined whether altered neuronal synchrony can be a surrogate marker to quantify network hyperexcitability in patients with Alzheimer's disease. Using magnetoencephalography (MEG) at rest, we studied 30 Alzheimer's disease patients without subclinical epileptiform activity, 20 Alzheimer's disease patients with subclinical epileptiform activity and 35 age-matched controls. Presence of subclinical epileptiform activity was assessed in patients with Alzheimer's disease by long-term video-EEG and a 1-h resting MEG with simultaneous EEG. Using the resting-state source-space reconstructed MEG signal, in patients and controls we computed the global imaginary coherence in alpha (8-12 Hz) and delta-theta (2-8 Hz) oscillatory frequencies. We found that Alzheimer's disease patients with subclinical epileptiform activity have greater reductions in alpha imaginary coherence and greater enhancements in delta-theta imaginary coherence than Alzheimer's disease patients without subclinical epileptiform activity, and that these changes can distinguish between Alzheimer's disease patients with subclinical epileptiform activity and Alzheimer's disease patients without subclinical epileptiform activity with high accuracy. Finally, a principal component regression analysis showed that the variance of frequency-specific neuronal synchrony predicts longitudinal changes in Mini-Mental State Examination in patients and controls. Our results demonstrate that quantitative neurophysiological measures are sensitive biomarkers of network hyperexcitability and can be used to improve diagnosis and to select appropriate patients for the right therapy in the next-generation clinical trials. The current results provide an integrative framework for investigating network hyperexcitability and network dysfunction together with cognitive and clinical correlates in patients with Alzheimer's disease. [ABSTRACT FROM AUTHOR]

The article describes the case of a 33-year-old man with right sided weakness and vision loss and diagnosed with sickle cell disease. Topics discussed include laboratory findings upon arrival, clinical characteristics of sickle cell disease, and inconsistency of the patient's neurologic examination. Also mentioned are multiple approaches for accurate diagnosis of hemoglobinopathies.

Background: Valproic acid (VPA) is a broad-spectrum anticonvulsant drug. Under normal conditions, this drug is highly protein bound. However, in patients with hypoalbuminemia, the free fraction can increase substantially while the total VPA levels remain in therapeutic range. The neurologic activity and toxicity of the drug are directly related to free drug levels. Methods: Our in-house free VPA assay was validated using 20 patient samples obtained from a reference laboratory (RL1). It was further evaluated by parallel testing with RL1 using samples collected from our patients. Subsequently, sample handling effects were investigated by comparing free VPA levels measured in our laboratory to 3 selected RLs with different sample transportation conditions. Results: No significant bias was observed between the in-house assay (y) and RL1 (x) assay in free VPA measurement (y = 1.12x + 0.072, r = 0.994). However, patient samples collected in our institution and sent to RL1 revealed significant negative bias (y = 0.776x − 3.861, r = 0.954). A large discrepancy in free VPA levels was further observed from identical aliquots of the same samples transported to 3 RLs in different conditions. Conclusions: Our study demonstrated that sample handling has significant impact on free VPA levels. The observed magnitude of variation exceeds a clinically acceptable limit and could alter clinical decisions. [ABSTRACT FROM AUTHOR]

Objectives Accurate, clinically meaningful outcome measures that are responsive to change are essential for selecting interventions and assessing their effects. Little guidance exists on the selection and administration of neurological impairment tests in children with a neurological condition. Clinicians therefore frequently modify adult assessments for use in children, yet the literature is inconsistent. This study aims to establish consensus on neurological conditions most likely to require neurological impairment test in pediatrics and the barriers, enablers, and modifications perceived to enhance test reliability. Methods Over a 2-round modified Delphi study, a panel of experts (n = 24) identified neurological conditions perceived to typically require pediatric neurological testing and the modifications to address barriers/enablers to testing. Experts comprised physical therapists with evidence of advanced training or research in pediatrics. Using a 6-point Likert scale (6 = strongly agree, 5 = agree, 4 = somewhat agree, 3 = somewhat disagree, 2 = disagree, 1 = strongly disagree), experts rated statements from existing literature. Thematic analyses were conducted on responses to open-ended questions. A priori consensus was pre-set at 65% agreement/disagreement. Median, mode, and interquartile ranges estimated perceived importance. Cessation was pre-determined by non-consensus items <10% and panel fatigue. Results Experts reached consensus on 107/112 (96%) items, including identifying 25/26 (96%) neurological conditions they perceived to require routine neurological testing. Experts strongly agreed with high importance that appropriately trained, experienced therapists are less variable when testing children. Communication modifications were perceived as most important. Conclusion High levels of consensus support the use of lower limb neurological testing in a range of pediatric neurological conditions. Trained clinicians should document modifications such as visual aid use. Using recommended modifications could encourage consistency among clinicians. Impact This is the first study to our knowledge to identify the barriers and enablers to pediatric neurological testing. Barriers and enablers were partially addressed through suggested modifications. Further rigorous examination of these modifications is required to support their use. Lay Summary This study supports that clinicians should adapt their communication for children and young people with neurological problems to include visual aids and equipment demonstration. [ABSTRACT FROM AUTHOR]

Background: posterior fossa tumors are more common in children that in adults. Between 54% and 70% of all childhood brain tumors originate in the posterior fossa. About 15-20% of brain tumors in adults occur in the posterior fossa. Certain types of poeterior fossa tumors, such as medulloblastoma, pineoblastoma, ependymomas, primitive neuroectodermal tumors, and astrocytomas of the cerebellum and brain stem, occur more frequently in children. Aim of the Work: the aim of this study was to review the preoperative diagnostic modalities, surgical procedures and outcome for different midline posterior fossa space occupying lesions. Patients and Methods: this is retrospective study has been conducted on 50 patients with posterior fossa midline space occupying lesions during the period March 2013 to October 2015, at Department of Neurosurgery, Ain Shams University. All Patients have been subjected to preoperative clinical assessment; thorough history taking, complete general and neurological examination according to Ain Shams University neurosurgical sheet. Results: this study was conducted on 50 patients with midline posterior fossa space occupying lesions. All of them underwent a midline suboccipital approach. The pre and post-operative data were collected and analyzed. This study included 50 patient, 26 female (52%) and 24 male (48%) patients. The mean age ranges from 1 year to 60 years. Conclusion: surgery is the treatment of choice for the cases of posterior fossa mid line lesions especially in children and adolescents to decreases the risk of disabilities and increases the survival. [ABSTRACT FROM AUTHOR]

Reading aloud requires mapping an orthographic form to a phonological one. The mapping process relies on sublexical statistical regularities (e.g. 'oo' to |uː|) or on learned lexical associations between a specific visual form and a series of sounds (e.g. yacht to/jɑt/). Computational, neuroimaging, and neuropsychological evidence suggest that sublexical, phonological and lexico-semantic processes rely on partially distinct neural substrates: a dorsal (occipito-parietal) and a ventral (occipito-temporal) route, respectively. Here, we investigated the spatiotemporal features of orthography-to-phonology mapping, capitalizing on the time resolution of magnetoencephalography and the unique clinical model offered by patients with semantic variant of primary progressive aphasia (svPPA). Behaviourally, patients with svPPA manifest marked lexico-semantic impairments including difficulties in reading words with exceptional orthographic to phonological correspondence (irregular words). Moreover, they present with focal neurodegeneration in the anterior temporal lobe, affecting primarily the ventral, occipito-temporal, lexical route. Therefore, this clinical population allows for testing of specific hypotheses on the neural implementation of the dual-route model for reading, such as whether damage to one route can be compensated by over-reliance on the other. To this end, we reconstructed and analysed time-resolved whole-brain activity in 12 svPPA patients and 12 healthy age-matched control subjects while reading irregular words (e.g. yacht) and pseudowords (e.g. pook). Consistent with previous findings that the dorsal route is involved in sublexical, phonological processes, in control participants we observed enhanced neural activity over dorsal occipito-parietal cortices for pseudowords, when compared to irregular words. This activation was manifested in the beta-band (12-30 Hz), ramping up slowly over 500 ms after stimulus onset and peaking at ∼800 ms, around response selection and production. Consistent with our prediction, svPPA patients did not exhibit this temporal pattern of neural activity observed in controls this contrast. Furthermore, a direct comparison of neural activity between patients and controls revealed a dorsal spatiotemporal cluster during irregular word reading. These findings suggest that the sublexical/phonological route is involved in processing both irregular and pseudowords in svPPA. Together these results provide further evidence supporting a dual-route model for reading aloud mediated by the interplay between lexico-semantic and sublexical/phonological neurocognitive systems. When the ventral route is damaged, as in the case of neurodegeneration affecting the anterior temporal lobe, partial compensation appears to be possible by over-recruitment of the slower, serial attention-dependent, dorsal one. [ABSTRACT FROM AUTHOR]

Ataxia, causing imbalance, dizziness and falls, is a leading cause of neurological disability. We have recently identified a biallelic intronic AAGGG repeat expansion in replication factor complex subunit 1 (RFC1) as the cause of cerebellar ataxia, neuropathy, vestibular areflexia syndrome (CANVAS) and a major cause of late onset ataxia. Here we describe the full spectrum of the disease phenotype in our first 100 genetically confirmed carriers of biallelic repeat expansions in RFC1 and identify the sensory neuropathy as a common feature in all cases to date. All patients were Caucasian and half were sporadic. Patients typically reported progressive unsteadiness starting in the sixth decade. A dry spasmodic cough was also frequently associated and often preceded by decades the onset of walking difficulty. Sensory symptoms, oscillopsia, dysautonomia and dysarthria were also variably associated. The disease seems to follow a pattern of spatial progression from the early involvement of sensory neurons, to the later appearance of vestibular and cerebellar dysfunction. Half of the patients needed walking aids after 10 years of disease duration and a quarter were wheelchair dependent after 15 years. Overall, two-thirds of cases had full CANVAS. Sensory neuropathy was the only manifestation in 15 patients. Sixteen patients additionally showed cerebellar involvement, and six showed vestibular involvement. The disease is very likely to be underdiagnosed. Repeat expansion in RFC1 should be considered in all cases of sensory ataxic neuropathy, particularly, but not only, if cerebellar dysfunction, vestibular involvement and cough coexist. [ABSTRACT FROM AUTHOR]

Agrammatic aphasia affects grammatical language production and can result from a neurodegenerative disease. Although it typically presents with concomitant apraxia of speech, this is not always the case. Little is known about the clinical course and imaging features of patients that present with agrammatism in the absence of apraxia of speech, which we will refer to as progressive agrammatic aphasia. We aimed to make a detailed description of the longitudinal clinical, linguistic, and neuroimaging features of a cohort of 11 patients with progressive agrammatic aphasia to provide a complete picture of this syndrome. All patients underwent detailed speech and language, neurological and neuropsychological assessments, 3 T structural and diffusion tensor imaging MRI, 18F-fluorodeoxyglucose and Pittsburgh compound B PET. The 11 patients were matched by age and gender to 22 patients who had mixed apraxia of speech and agrammatism. The progressive agrammatic aphasia patients performed abnormally on tests of language, general cognition, executive function, and functional ability at baseline and declined in these measures over time. Only two patients eventually developed apraxia of speech, while parkinsonism was absent-to-mild throughout all visits for all patients. When compared to the patients with mixed apraxia of speech and agrammatism, the patients with progressive agrammatic aphasia performed better on tests of motor speech and parkinsonism but more poorly, and declined faster over time, on tests of general aphasia severity, agrammatism, and naming. The patients with progressive agrammatic aphasia also showed different neuroimaging abnormalities, with greater atrophy, hypometabolism and white matter tract degeneration in the prefrontal and anterior temporal lobes compared to patients with mixed apraxia of speech and agrammatism. These differences were more pronounced as the disease progressed. These results demonstrate that progressive agrammatic aphasia has a different clinical disease course and different underlying neuroanatomical abnormalities than patients with the more common syndrome of mixed agrammatism and apraxia of speech. This supports the distinction of progressive agrammatic aphasia and has implications for the classification of patients with agrammatic aphasia. [ABSTRACT FROM AUTHOR]

Background: Brain tumours often present with varied, non-specific features with other diagnoses usually being more likely.Objective: To examine how different symptoms and patient demographics predict variations in time to brain tumour diagnosis.Methods: We conducted a secondary analysis of brain tumour cases from National Audit of Cancer Diagnosis in Primary Care. We grouped neurological symptoms into six domains (headache, behavioural/cognitive change, focal neurology, 'fits, faints or falls', non-specific neurological, and other/non-specific) and calculated times for patient presentation, GP referral, specialist consultation and total pathway interval. We calculated odds ratios (ORs) for symptom domains comparing the slowest to other quartiles.Results: Data were available for 226 cases. Median (interquartile range) time for the total pathway interval was 24 days (7-65 days). The most common presentation was focal neurology (33.2%) followed by 'fits, faints or falls' and headache (both 20.8%). Headache only (OR = 4.11, 95% CI = 1.10, 15.5) and memory complaints (OR = 4.82, 95% CI = 1.15, 20.1) were associated with slower total pathway compared to 'fits, faints or falls'. GPs were more likely to consider that there had been avoidable delays in referring patients with headache only (OR = 4.17, 95% CI = 1.14, 15.3).Conclusion: Patients presenting to primary care with headache only or with memory complaints remain problematic with potentially avoidable delays in referral leading to a longer patient pathway. This may or may not impact on the efficacy and morbidity of therapies. Additional aids are required to help doctors differentiate when to refer headaches and memory complaints urgently for a specialist opinion. [ABSTRACT FROM AUTHOR]

Durable impairments of consciousness are currently classified in three main neurological categories: comatose state, vegetative state (also recently coined unresponsive wakefulness syndrome) and minimally conscious state. While the introduction of minimally conscious state, in 2002, was a major progress to help clinicians recognize complex non-reflexive behaviours in the absence of functional communication, it raises several problems. The most important issue related to minimally conscious state lies in its criteria: while behavioural definition of minimally conscious state lacks any direct evidence of patient's conscious content or conscious state, it includes the adjective 'conscious'. I discuss this major problem in this review and propose a novel interpretation of minimally conscious state: its criteria do not inform us about the potential residual consciousness of patients, but they do inform us with certainty about the presence of a cortically mediated state. Based on this constructive criticism review, I suggest three proposals aiming at improving the way we describe the subjective and cognitive state of non-communicating patients. In particular, I present a tentative new classification of impairments of consciousness that combines behavioural evidence with functional brain imaging data, in order to probe directly and univocally residual conscious processes. [ABSTRACT FROM AUTHOR]

Neuroprosthetics research in amputee patients aims at developing new prostheses that move and feel like real limbs. Targeted muscle and sensory reinnervation (TMSR) is such an approach and consists of rerouting motor and sensory nerves from the residual limb towards intact muscles and skin regions. Movement of the myoelectric prosthesis is enabled via decoded electromyography activity from reinnervated muscles and touch sensation on the missing limb is enabled by stimulation of the reinnervated skin areas. Here we ask whether and how motor control and redirected somatosensory stimulation provided via TMSR affected the maps of the upper limb in primary motor (M1) and primary somatosensory (S1) cortex, as well as their functional connections. To this aim, we tested three TMSR patients and investigated the extent, strength, and topographical organization of the missing limb and several control body regions in M1 and S1 at ultra high-field (7 T) functional magnetic resonance imaging. Additionally, we analysed the functional connectivity between M1 and S1 and of both these regions with fronto-parietal regions, known to be important for multisensory upper limb processing. These data were compared with those of control amputee patients (n = 6) and healthy controls (n = 12). We found that M1 maps of the amputated limb in TMSR patients were similar in terms of extent, strength, and topography to healthy controls and different from non-TMSR patients. S1 maps of TMSR patients were also more similar to normal conditions in terms of topographical organization and extent, as compared to non-targeted muscle and sensory reinnervation patients, but weaker in activation strength compared to healthy controls. Functional connectivity in TMSR patients between upper limb maps in M1 and S1 was comparable with healthy controls, while being reduced in non-TMSR patients. However, connectivity was reduced between S1 and fronto-parietal regions, in both the TMSR and non-TMSR patients with respect to healthy controls. This was associated with the absence of a well-established multisensory effect (visual enhancement of touch) in TMSR patients. Collectively, these results show how M1 and S1 process signals related to movement and touch are enabled by targeted muscle and sensory reinnervation. Moreover, they suggest that TMSR may counteract maladaptive cortical plasticity typically found after limb loss, in M1, partially in S1, and in their mutual connectivity. The lack of multisensory interaction in the present data suggests that further engineering advances are necessary (e.g. the integration of somatosensory feedback into current prostheses) to enable prostheses that move and feel as real limbs. [ABSTRACT FROM AUTHOR]

Primary progressive aphasia is a syndrome characterized by progressive loss of language abilities with three main phenotypic clinical presentations, including logopenic, non-fluent/agrammatic, and semantic variants. Previous imaging studies have shown unique anatomic impacts within language networks in each variant. However, direct measures of spontaneous neuronal activity and functional integrity of these impacted neural networks in primary progressive aphasia are lacking. The aim of this study was to characterize the spatial and temporal patterns of resting state neuronal synchronizations in primary progressive aphasia syndromes. We hypothesized that resting state brain oscillations will show unique deficits within language network in each variant of primary progressive aphasia. We examined 39 patients with primary progressive aphasia including logopenic variant (n = 14, age = 61 ± 9 years), non-fluent/agrammatic variant (n = 12, age = 71 ± 8 years) and semantic variant (n = 13, age = 65 ± 7 years) using magnetoencephalographic imaging, compared to a control group that was matched in age and gender to each primary progressive aphasia subgroup (n = 20, age = 65 ± 5 years). Each patient underwent a complete clinical evaluation including a comprehensive battery of language tests. We examined the whole-brain resting state functional connectivity as measured by imaginary coherence in each patient group compared to the control cohort, in three frequency oscillation bands-delta-theta (2-8 Hz); alpha (8-12 Hz); beta (12-30 Hz). Each variant showed a distinct spatiotemporal pattern of altered functional connectivity compared to age-matched controls. Specifically, we found significant hyposynchrony of alpha and beta frequency within the left posterior temporal and occipital cortices in patients with the logopenic variant, within the left inferior frontal cortex in patients with the non-fluent/agrammatic variant, and within the left temporo-parietal junction in patients with the semantic variant. Patients with logopenic variant primary progressive aphasia also showed significant hypersynchrony of delta-theta frequency within bilateral medial frontal and posterior parietal cortices. Furthermore, region of interest-based analyses comparing the spatiotemporal patterns of variant-specific regions of interest identified in comparison to age-matched controls showed significant differences between primary progressive aphasia variants themselves. We also found distinct patterns of regional spectral power changes in each primary progressive aphasia variant, compared to age-matched controls. Our results demonstrate neurophysiological signatures of network-specific neuronal dysfunction in primary progressive aphasia variants. The unique spatiotemporal patterns of neuronal synchrony signify diverse neurophysiological disruptions and pathological underpinnings of the language network in each variant. [ABSTRACT FROM AUTHOR]

Although frequency-specific network analyses have shown that functional brain networks are altered in patients with Alzheimer's disease, the relationships between these frequency-specific network alterations remain largely unknown. Multiplex network analysis is a novel network approach to study complex systems consisting of subsystems with different types of connectivity patterns. In this study, we used magnetoencephalography to integrate five frequency-band specific brain networks in a multiplex framework. Previous structural and functional brain network studies have consistently shown that hub brain areas are selectively disrupted in Alzheimer's disease. Accordingly, we hypothesized that hub regions in the multiplex brain networks are selectively targeted in patients with Alzheimer's disease in comparison to healthy control subjects. Eyes-closed resting-state magnetoencephalography recordings from 27 patients with Alzheimer's disease (60.6 ± 5.4 years, 12 females) and 26 controls (61.8 ± 5.5 years, 14 females) were projected onto atlas-based regions of interest using beamforming. Subsequently, source-space time series for both 78 cortical and 12 subcortical regions were reconstructed in five frequency bands (delta, theta, alpha 1, alpha 2 and beta band). Multiplex brain networks were constructed by integrating frequency-specific magnetoencephalography networks. Functional connections between all pairs of regions of interests were quantified using a phase-based coupling metric, the phase lag index. Several multiplex hub and heterogeneity metrics were computed to capture both overall importance of each brain area and heterogeneity of the connectivity patterns across frequency-specific layers. Different nodal centrality metrics showed consistently that several hub regions, particularly left hippocampus, posterior parts of the default mode network and occipital regions, were vulnerable in patients with Alzheimer's disease compared to control subjects. Of note, these detected vulnerable hubs in Alzheimer's disease were absent in each individual frequency-specific network, thus showing the value of integrating the networks. The connectivity patterns of these vulnerable hub regions in the patients were heterogeneously distributed across layers. Perturbed cognitive function and abnormal cerebrospinal fluid amyloid-β42 levels correlated positively with the vulnerability of the hub regions in patients with Alzheimer's disease. Our analysis therefore demonstrates that the magnetoencephalography-based multiplex brain networks contain important information that cannot be revealed by frequency-specific brain networks. Furthermore, this indicates that functional networks obtained in different frequency bands do not act as independent entities. Overall, our multiplex network study provides an effective framework to integrate the frequency-specific networks with different frequency patterns and reveal neuropathological mechanism of hub disruption in Alzheimer's disease. [ABSTRACT FROM AUTHOR]

Introduction: 'Burning Feet Syndrome' affected up to one third of Far Eastern Prisoners of War in World War 2. Recently discovered medical records, produced by RAF Medical Officer Nowell Peach whilst in captivity, are the first to detail neurological examinations of patients with this condition. Methods: The 54 sets of case notes produced at the time were analysed using modern diagnostic criteria to determine if the syndrome can be retrospectively classed as neuropathic pain. Results: With a history of severe malnutrition raising the possibility of a peripheral polyneuropathy, and a neuroanatomically plausible pain distribution, this analysis showed that Burning Feet Syndrome can now be described as a 'possible' neuropathic pain syndrome. Conclusion: After 70 years, the data painstakingly gathered under the worst of circumstances have proved to be of interest and value in modern diagnostics of neuropathic pain. [ABSTRACT FROM AUTHOR]

Background. Wernicke's encephalopathy is a serious medical condition associated with high morbidity and mortality caused by deficiency of thiamine. This disease is classically associated with alcoholism, but is underappreciated in the nonalcoholic population. There is growing acknowledgement of the development of Wernicke's encephalopathy in patients with malignancies. Methods. We conducted a literature review in PubMed for cases of Wernicke's encephalopathy occurring in patients with malignancy. We also present the case of a 47-year-old woman with recurrent laryngeal cancer and multiple hospital admissions for malnutrition. Neurological examination was notable for pendular nystagmus, severe gait ataxia, confusion, and poor memory consolidation. MRI of the brain was significant for T2-weighted fluid-attenuated inversion recovery hyperintensities in periaqueductal regions, medial thalami, and the tectal plate, typical for Wernicke's encephalopathy. She was treated with thiamine repletion, and had marked improvement in her mental status and some improvement in her vision problems and ataxia, although some nystagmus and significant short-term memory impairment persisted. Results. The literature review yielded dozens of case reports of Wernicke's encephalopathy in patients with malignancy, dominated by cases of patients with malignancies of the gastrointestinal system, followed by those with hematologic malignancies. Conclusions. Malignancy is an important risk factor for the development of Wernicke's encephalopathy. This diagnosis is underappreciated and difficult for the clinician to discern from multifactorial delirium. Clinicians should be aware to treat at-risk patients with thiamine immediately, especially if multiple risk factors are present. [ABSTRACT FROM AUTHOR]

SEE MATTAR ET AL DOI101093/AWW151 FOR A SCIENTIFIC COMMENTARY ON THIS ARTICLE: Functional brain networks demonstrate significant temporal variability and dynamic reconfiguration even in the resting state. Currently, most studies investigate temporal variability of brain networks at the scale of single (micro) or whole-brain (macro) connectivity. However, the mechanism underlying time-varying properties remains unclear, as the coupling between brain network variability and neural activity is not readily apparent when analysed at either micro or macroscales. We propose an intermediate (meso) scale analysis and characterize temporal variability of the functional architecture associated with a particular region. This yields a topography of variability that reflects the whole-brain and, most importantly, creates an analytical framework to establish the fundamental relationship between variability of regional functional architecture and its neural activity or structural connectivity. We find that temporal variability reflects the dynamical reconfiguration of a brain region into distinct functional modules at different times and may be indicative of brain flexibility and adaptability. Primary and unimodal sensory-motor cortices demonstrate low temporal variability, while transmodal areas, including heteromodal association areas and limbic system, demonstrate the high variability. In particular, regions with highest variability such as hippocampus/parahippocampus, inferior and middle temporal gyrus, olfactory gyrus and caudate are all related to learning, suggesting that the temporal variability may indicate the level of brain adaptability. With simultaneously recorded electroencephalography/functional magnetic resonance imaging and functional magnetic resonance imaging/diffusion tensor imaging data, we also find that variability of regional functional architecture is modulated by local blood oxygen level-dependent activity and α-band oscillation, and is governed by the ratio of intra- to inter-community structural connectivity. Application of the mesoscale variability measure to multicentre datasets of three mental disorders and matched controls involving 1180 subjects reveals that those regions demonstrating extreme, i.e. highest/lowest variability in controls are most liable to change in mental disorders. Specifically, we draw attention to the identification of diametrically opposing patterns of variability changes between schizophrenia and attention deficit hyperactivity disorder/autism. Regions of the default-mode network demonstrate lower variability in patients with schizophrenia, but high variability in patients with autism/attention deficit hyperactivity disorder, compared with respective controls. In contrast, subcortical regions, especially the thalamus, show higher variability in schizophrenia patients, but lower variability in patients with attention deficit hyperactivity disorder. The changes in variability of these regions are also closely related to symptom scores. Our work provides insights into the dynamic organization of the resting brain and how it changes in brain disorders. The nodal variability measure may also be potentially useful as a predictor for learning and neural rehabilitation. [ABSTRACT FROM AUTHOR]

Mental acts are conspicuously absent from philosophical debates over the nature of action. A typical protagonist of a typical scenario is far more likely to raise her arm or open the window than she is to perform a calculation in her head or talk to herself silently. One possible explanation for this omission is that the standard 'causalist' account of action, on which acts are analysed in terms of mental states causing bodily movements, faces difficulties in accommodating some paradigmatic cases of mental action--or so I shall argue. After drawing out these objections to causalism, I outline a more promising approach. Building on previous work, I show how the approach I favour, on which the attempt to analyse action is dispensed with, provides a unified account of both mental and physical action. [ABSTRACT FROM AUTHOR]

Dopaminergic signalling in the striatum contributes to reinforcement of actions and motivational enhancement of motor vigour. Parkinson's disease leads to progressive dopaminergic denervation of the striatum, impairing the function of cortico-basal ganglia networks. While levodopa therapy alleviates basal ganglia dysfunction in Parkinson's disease, it often elicits involuntary movements, referred to as levodopa-induced peak-of-dose dyskinesias. Here, we used a novel pharmacodynamic neuroimaging approach to identify the changes in cortico-basal ganglia connectivity that herald the emergence of levodopa-induced dyskinesias. Twenty-six patients with Parkinson's disease (age range: 51-84 years; 11 females) received a single dose of levodopa and then performed a task in which they had to produce or suppress a movement in response to visual cues. Task-related activity was continuously mapped with functional magnetic resonance imaging. Dynamic causal modelling was applied to assess levodopa-induced modulation of effective connectivity between the pre-supplementary motor area, primary motor cortex and putamen when patients suppressed a motor response. Bayesian model selection revealed that patients who later developed levodopa-induced dyskinesias, but not patients without dyskinesias, showed a linear increase in connectivity between the putamen and primary motor cortex after levodopa intake during movement suppression. Individual dyskinesia severity was predicted by levodopa-induced modulation of striato-cortical feedback connections from putamen to the pre-supplementary motor area (Pcorrected = 0.020) and primary motor cortex (Pcorrected = 0.044), but not feed-forward connections from the cortex to the putamen. Our results identify for the first time, aberrant dopaminergic modulation of striatal-cortical connectivity as a neural signature of levodopa-induced dyskinesias in humans. We argue that excessive striato-cortical connectivity in response to levodopa produces an aberrant reinforcement signal producing an abnormal motor drive that ultimately triggers involuntary movements. [ABSTRACT FROM AUTHOR]

Background: patients suspected of dementia frequently undergo additional diagnostic testing (e.g. brain imaging or neuropsychological assessment) after standard clinical assessment at a memory clinic. This study investigates the use of additional testing in an academic outpatient memory clinic and how it influences the initial diagnosis.Methods: the initial diagnosis after standard clinical assessment (history, laboratory tests, cognitive screening and physical and neurological examination) and the final diagnosis after additional testing of 752 memory clinic patients were collected. We specifically registered if, and what type of, additional testing was requested.Results: additional testing was performed in 518 patients (69%), 67% of whom underwent magnetic resonance imaging, 45% had neuropsychological assessment, 14% had cerebrospinal fluid analysis and 49% had (combinations of) other tests. This led to a modification of the initial diagnosis in 17% of the patients. The frequency of change was highest in patients with an initial non-Alzheimer's disease (AD) dementia diagnosis (54%, compared with 11 and 14% in patients with AD and ‘no dementia’; P < 0.01). Finally, after additional testing 44% was diagnosed with AD, 9% with non-AD dementia and 47% with ‘no dementia’.Conclusion: additional testing should especially be considered in non-AD patients. In the large group of patients with an initial AD or ‘no dementia’ diagnosis, additional tests have little diagnostic impact and may perhaps be used with more restraint. [ABSTRACT FROM PUBLISHER]

Objective. Repetitive transcranial magnetic stimulation ( rTMS) has been shown to effectively treat depression, and its potential value in pain management is emphasized by recent studies. Transcranial magnetic stimulation ( TMS)-evoked activity in the prefrontal cortex may be associated with corticolimbic inhibitory circuits capable of decreasing pain perception. The present exploratory pilot study used functional magnetic resonance imaging ( fMRI) to examine the effects of left prefrontal rTMS on brain activity and pain perception. Design and Intervention. Twenty-three healthy adults with no history of depression or chronic pain underwent an 8-minute thermal pain protocol with fMRI before and after a single rTMS session. Participants received 15 minutes of either real ( N = 12) or sham ( N = 11) 10 Hz rTMS over the left prefrontal cortex (110% of resting motor threshold; 5 seconds on, 10 seconds off). Results. TMS was associated with a 13.30% decrease in pain ratings, while sham was associated with an 8.61% decrease ( P = 0.04). TMS was uniquely associated with increased activity in the posterior cingulate gyrus, precuneous, right superior frontal gyrus, right insula, and bilateral postcentral gyrus. Activity in the right superior prefrontal gyrus was negatively correlated with pain ratings ( r = −0.65, P = 0.02) in the real TMS group. Conclusions. Findings suggest that prefrontal rTMS may be capable of activating inhibitory circuits involved with pain reduction. [ABSTRACT FROM AUTHOR]

Background. Spinal metastases frequently arise in patients with cancer. Modern oncology provides numerous treatment options that include effective systemic, radiation, and surgical options. We delineate and provide the evidence for the neurologic, oncologic, mechanical, and systemic (NOMS) decision framework, which is used at Memorial Sloan-Kettering Cancer Center to determine the optimal therapy for patients with spine metastases. Methods. We provide a literature review of the integral publications that serve as the basis for the NOMS framework and report the results of systematic implementation of theNOMSguidedtreatment. Results. The NOMS decision framework consists of the neurologic,oncologic, mechanical, and systemic considerations and incorporates the use of conventional externalbeamradiation, spinal stereotactic radiosurgery, and minimally invasive and open surgical interventions. Review of radiation oncology and surgical literature that examine the outcomes of treatment of spinal metastatic tumors provides support for the NOMS decision framework. Application of the NOMS paradigm integrates multimodality therapy to optimize local tumor control,pain relief, and restoration or preservation of neurologic function and minimizes morbidity in this often systemically ill patient population. Conclusion. NOMS paradigm provides a decision framework that incorporates sentinel decision points in the treatment of spinal metastases. Consideration of the tumor sensitivity to radiation in conjunction with the extent of epidural extension allows determination of the optimal radiation treatment and the need for surgical decompression. Mechanical stability of the spine and the systemic disease considerations further help determine the need and the feasibility of surgical intervention. TheOncologist2013;18:744-751 [ABSTRACT FROM AUTHOR]

Symptom validity assessment is an important part of neuropsychological evaluation. There are currently several free-standing symptom validity tests (SVTs), as well as a number of empirically derived embedded validity indices, that have been developed to assess that an examinee is putting forth an optimal level of effort during testing. The use of embedded validity indices is attractive since they do not increase overall testing time and may also be less vulnerable to coaching. In addition, there are some instances where embedded validity indices are the only tool available to the neuropsychological practitioner for assessing an examinee's level of effort. As with free-standing measures, the sensitivity and specificity of embedded validity indices to suboptimal effort varies. The present study evaluated the diagnostic validity of 17 embedded validity indices by comparing performance on these indices to performance on combinations of free-standing SVTs. Results from the current medico-legal sample revealed that of the embedded validity indices, Reliable Digit Span had the best classification accuracy; however, the findings do not support the use of this embedded validity index in the absence of free-standing SVTs. [ABSTRACT FROM PUBLISHER]

The determination of examinee effort is an important component of a neuropsychological evaluation and relies heavily on the use of symptom validity tests (SVTs) such as the Test of Memory Malingering (TOMM) and the Word Memory Test (WMT). Diagnostic utility of SVTs varies. The sensitivity of traditional TOMM criteria to suboptimal effort is low. An index of response consistency across three trials of the TOMM was developed, denoted the Albany Consistency Index (ACI). This index identified a large proportion of examinees classified as optimal effort using traditional TOMM interpretive guidelines but suboptimal effort using the WMT profile analysis. In addition, previous research was extended, demonstrating a relationship between examinee performance on SVTs and neuropsychological tests. Effort classification using the ACI predicted the performance on the Global Memory Index from the Memory Assessment Scales. In conclusion, the ACI was a more sensitive indicator of suboptimal effort than traditional TOMM interpretive guidelines. [ABSTRACT FROM PUBLISHER]

Spatial neglect is a perplexing neuropsychological syndrome, in which patients fail to detect (and/or respond to) stimuli located contralaterally to their (most often right) hemispheric lesion. Neglect is characterized by a wide heterogeneity, and a role for multiple components has been suggested, but the exact nature of the critical components remains unclear. Moreover, many different lesion sites have been reported, leading to enduring controversies about the relative contribution of different cortical and/or subcortical brain regions. Here we report a systematic anatomo-functional study of 80 patients with a focal right hemisphere stroke, who were examined by a series of neuropsychological tests assessing different clinical manifestations of neglect. We first performed a statistical factorial analysis of their behavioural performance across all tests, in order to break down neglect symptoms into coherent profiles of co-varying deficits. We then examined the neural correlates of these distinct neglect profiles using a statistical voxel-based lesion-symptom mapping method that correlated the anatomical extent of brain damage with the relative severity of deficits along the different profiles in each patient. Our factorial analysis revealed three main factors explaining 82% of the total variance across all neglect tests, which suggested distinct components related to perceptive/visuo-spatial, exploratory/visuo-motor, and allocentric/object-centred aspects of spatial neglect. Our anatomical voxel-based lesion-symptom mapping analysis pointed to specific neural correlates for each of these components, including the right inferior parietal lobule for the perceptive/visuo-spatial component, the right dorsolateral prefrontal cortex for the exploratory/visuo-motor component, and deep temporal lobe regions for the allocentric/object-centred component. By contrast, standard anatomical overlap analysis indicated that subcortical damage to paraventricular white matter tracts was associated with severe neglect encompassing several tests. Taken together, our results provide new support to the view that the clinical manifestations of hemispatial neglect might reflect a combination of distinct components affecting different domains of spatial cognition, and that intra-hemispheric disconnection due to white matter lesions might produce severe neglect by impacting on more than one functional domain. [ABSTRACT FROM PUBLISHER]

Clinicians can use the prevalence of low scores to help interpret test performance. However, this information is limited for most test batteries. In 2007, Crawford, Garthwaite, and Gault presented Monte Carlo simulation software for estimating the base rates of low scores for any battery of tests. The purpose of this study is to examine the accuracy of a Monte Carlo simulation program for estimating the base rates of low scores. Base rates of low scores were: (a) calculated from large normative samples (actual base rates) for the Neuropsychological Assessment Battery and the Wechsler Adult Intelligence Scale-III/Wechsler Memory Scale-Third Edition and compared to (b) Monte Carlo estimations (estimated base rates). Monte Carlo estimations of the base rates of low scores had good accuracy when compared with the actual base rates of low scores for the two batteries. However, estimated base rates lose considerable accuracy in those with low or high intelligence. Monte Carlo simulation software is a potential option for clinicians to compute the base rates of low scores for any battery with published intercorrelations. However, the Monte Carlo program underestimates the base rates for those with low intelligence and overestimates the base rates for those with high intelligence. [ABSTRACT FROM PUBLISHER]

In the context of forensic neuropsychological assessments, the professional interaction of law and psychology is viewed primarily as one where the retaining attorney or court dictates its needs to psychologists when resolving legal disputes. While this perspective is conceptually accurate, the positive and practical collaboration of law and psychology also relies on attorneys adhering to basic protections of sensitive psychological assessment procedures and tests. Objective testing is undermined when a practitioner of law engages in actions prior to, during, or following a neuropsychological examination in a manner that threatens the test security. An appreciation among practitioners of law and psychology regarding the necessity of test security is essential. This article reviews attorney actions that can affect test security, proposes a distinction by psychology between appropriate and problematic client preparation for a neuropsychological examination, integrates the available legal precedent regarding test security, and suggests productive measures to protect test security in medicolegal settings. [ABSTRACT FROM PUBLISHER]

The purpose of this study is to provide sophisticated psychometric information for advanced interpretation of the Neuropsychological Assessment Battery (NAB) with older adults. This information includes the base rates of low scores, intellectual-cognitive discrepancy scores, and a method for determining change. The NAB contains 24 co-normed neurocognitive tests across five domains (i.e., Attention, Language, Memory, Spatial, and Executive Functions); provides 36 primary T-scores, five domain indexes, and a total index score; and was co-normed with a measure of intellectual abilities (Reynolds Intellectual Assessment Scales; Reynolds Intellectual Screening Test [RIST]). Participants for this study were 742 older adults from the NAB standardization sample (mean age = 68.1, SD = 6.9). From the standardization sample, 42 older adults (mean age = 67.3 years, SD = 8.3) were administered the NAB two times (mean retest interval = 6.7 months, SD = 0.7). The base rates of low index scores and low primary scores are presented for the entire sample, as well as stratified by the level of intellectual abilities. RIST–NAB discrepancy scores are presented for the entire sample and for the different levels of intellectual abilities. Finally, information needed to interpret change in test performance on serial assessments is provided. [ABSTRACT FROM PUBLISHER]

The Rey–Osterrieth complex figure (ROCF) and the free and cued selective reminding test (FCSRT) are frequently used in clinical practice. The ROCF assesses visual perception, constructional praxis, and visuospatial memory, and the FCSRT assesses verbal learning and memory. As part of the Spanish Normative Studies (NEURONORMA), we provide age- and education-adjusted norms for the ROCF (copy and memory) and for the FCSRT. The sample consists of 332 and 340 participants, respectively, who are cognitively normal, community dwelling, and ranging in age from 50 to 94 years. Tables are provided to convert raw scores to age-adjusted scaled scores. These were further converted into education-adjusted scaled scores by applying regression-based adjustments. Although age and education affected the score of the ROCF and FCSRT, sex was found to be unrelated in this normal sample. The normative data presented here were obtained from the same study sample as all other NEURONORMA norms and the same statistical procedures were applied. These co-normed data will allow clinicians to compare scores from one test with all the tests included in the project. [ABSTRACT FROM PUBLISHER]

Abstract: The Rey–Osterrieth Complex Figure (ROCF) is probably one of the most popular measurement instruments of visuoconstructional abilities and nonverbal memory. It is frequently part of neuropsychological test protocols in epilepsy surgery centers. In this study we compared the traditional scoring system of the ROCF developed by Taylor (1998) with a qualitative system that assesses spatial-relational errors devised by Loring et al. [Loring, D. W., Lee, G. P., & Meador, K. J. (1988). Revising the Rey–Osterrieth: Rating right hemisphere recall. Archives of Clinical Neuropsychology, 3, 239–247] in a sample of left and right temporal lobe epilepsy patients undergoing pre-surgical evaluation. We investigated whether the relational-spatial scoring system would be more sensitive to right-sided memory deficits than the traditional Taylor version. There was no difference in the copy phase of the ROCF between the clinical and control groups. There was a significant difference between the control and the clinical groups when the 30-min delayed recall drawings were scored with the Taylor system. However, this system failed to find differences between left and right temporal lobe epileptic patients. On the other hand, comparisons with the qualitative scoring criteria used by Loring et al. [Loring, D. W., Lee, G. P., & Meador, K. J. (1988). Revising the Rey–Osterrieth: Rating right hemisphere recall. Archives of Clinical Neuropsychology, 3, 239–247] revealed that right temporal lobe patients made more spatial-relational errors than patients with left-sided foci. Frequency distribution of these scores for all the three groups and sensitivity and specificity to correctly classify right temporal lobe patients are presented. This investigation demonstrated that applying qualitative, material-specific scoring criteria improves temporal lobe epilepsy presurgical protocols. [Copyright &y& Elsevier]

Abstract: It is often assumed that neuropsychological measures are ecologically valid in ‘normal’ people, but this assumption has not yet been thoroughly evaluated. The aim of the present study was to evaluate the cross-sectional and longitudinal ecological validity of individual neuropsychological test scores and their composites in a large sample of neurologically intact people. Three neuropsychological composite measures were established, i.e. a “Memory Quotient”, an “Executive functioning and Speed Quotient”, and a “General Cognitive Quotient”. The ecological validity of the individual neuropsychological measures and their composites was low to moderate. Multivariate models that included both neuropsychological and non-cognitive variables (i.e. demographic variables, depressive symptoms and anxiety) accounted for 4.6–21.4% of the variance in daily life functioning. The General Cognitive Quotient was the neuropsychological measure that was the most consistently related to daily life functioning. [Copyright &y& Elsevier]

Abstract: Normative neuropsychological data for U.S. racial/ethnic minorities are limited. Extant norms are based on small, regional groups that may not be nationally representative. The objectives of this study were to (1) provide norms for a modified Symbol Digit Modalities Test (M-SDMT) based on a nationally representative sample of African Americans, Caribbean Blacks, and non-Latino Whites (NLW) living in areas with large populations of Blacks; and (2) determine significant correlates of M-SDMT performance. The M-SDMT was administered to a subset of respondents from the National Survey of American Life in standard face-to-face interviews. M-SDMT performance was influenced by race/ethnicity, age, education, and gender. African Americans and NLW groups had similar M-SDMT performances, which differed from Caribbean Blacks. The Black ethnic differences in M-SDMT were not explained by the sociodemographic factors considered in this study. Unlike previous work, this study supports the consideration of Black ethnicity when evaluating Black neuropsychological test performance. [Copyright &y& Elsevier]

Abstract: The ARES 1 [1] ARES was the Greek God of War. (ANAM 2 [2] Automated Neuropsychological Assessment Metrics. Readiness Evaluation System) is a cognitive testing system designed for operation on palm OS handheld computers i.e., Personal Digital Assistants (PDA). It provides an inexpensive and portable testing platform for field and clinical applications. ARES test batteries can be configured from a library of tests derived from the ANAM test system. ARES features include support of multiple users on a single PDA, a Microsoft Windows™ test battery authoring program, and a program for downloading, viewing, graphing, and archiving data. In validity tests, the same subjects were tested on identical ARES and conventional ANAM NeuroCog test batteries. Scores from the two platforms correlated highly, but absolute scores differed slightly. In reliability testing with the ARES Warrior battery, ARES scores were highly correlated in daily tests. [Copyright &y& Elsevier]