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Edinburgh Cognitive and Behavioural ALS Screen (ECAS) is a brief, standardized assessment of cognitiveimpairment inamyotrophic lateral sclerosis., Objective: We aimed to createa normative dataset for the ECAS Czech version (ECAS-CZ) in order to make the assessment applicable for clinical settings., Method: Included were 102 healthy participants (mean age: 54.92 ± 14.55; education: 14.52 ± 2.44; 54:48 females/males) that fulfilled rigorous exclusion criteria and controlled for depressive symptoms., Results: The internal consistency of ECAS-CZ was acceptable (Cronbach's α = .69). We found medium correlations ( rho ≈ .5) of age and education with ECAS-CZ Total score but not with gender. Cut-offs with -2 SD 's threshold are presented for the differentiation of cognitive impairment. We report percentile values for ECAS-CZ Total including all subscales., Conclusion: We provide normative values for ECAS-CZ that are well suited for the detection of cognitive impairment in clinical settings especially for patients with ALS.Supplemental data for this article is available online at https://doi.org/10.1080/13854046.2021.1978553 .

The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) was developed to assess cognitive and behavioural changes in an anterior frontotemporal syndrome (executive functions, language, fluency and behaviour), common in Amyotrophic Lateral Sclerosis (ALS) and also assesses posterior cerebral dysfunction (memory and visuospatial abilities)., Objectives: To validate the ECAS in behavioural variant Frontotemporal Dementia (bvFTD) without ALS, as compared with Alzheimer's disease (AD), against comprehensive neuropsychological assessment. Compare its sensitivity to that of the Addenbrooke's Cognitive Examination (ACE-III) and investigate behavioural changes in both types of dementia., Methods: Retrospective study of 16 people with bvFTD (without ALS), 32 with AD, and 48 healthy controls completed the ECAS, ACE-III and extensive neuropsychological assessment., Results: The ECAS showed higher sensitivity (94%) and marginally lower specificity (96%) than the ACE-III for both the bvFTD and AD groups. The anterior composite subscore was sensitive for bvFTD (94%), and slightly less so for AD (84%), while the posterior composite subscore was sensitive for AD (97%), and less so for bvFTD (75%). All people with bvFTD that were impaired on the ECAS total and anterior composite scores were also impaired on the anterior function's tests of the neuropsychological assessment. A cut-off of four or more behavioural domains affected differentiated well between the bvFTD and AD groups, while a qualitative analysis of the behavioural interview found different themes between groups., Conclusions: The ECAS is a valid and sensitive assessment for bvFTD without ALS and for AD. The carer behavioural interview makes it particularly suitable to detect behavioural abnormalities related to frontal lobe disorders., (© 2021 The Authors. International Journal of Geriatric Psychiatry published John Wiley & Sons Ltd.)

The Edinburgh Cognitive and Behavioral ALS Screen (ECAS) was developed to assess cognitive and behavioral changes common in Amyotrophic Lateral Sclerosis and other diseases affecting motor functions. It focuses on domains typically affected by the frontotemporal syndrome (executive and language functions, fluency and behavior), but assesses also memory and visuospatial functions., Objectives: (A) To investigate the relationship between the ECAS and the Addenbrooke's Cognitive Examination (ACE-III). (B) To investigate the effects of age, education, and IQ on the ECAS and create appropriate cutoff scores to determine abnormality., Methods: (A) 57 healthy participants (aged 35-80) were assessed with the ECAS, the Wechsler Abbreviated Scale of Intelligence (WASI-II), and the ACE-III. (B) 80 healthy participants (aged 51-80) were divided into four groups according to age and education; and were tested with the ECAS and the WASI-II., Results: The ECAS and the ACE-III have a good convergent validity with a significant correlation. Regression analysis revealed that IQ, followed by age, were the strongest predictors of the total ECAS score. IQ predicted 24% of the ECAS and 46% of the ACE-III variance. Education was not a significant predictor over and above IQ for both the ECAS and the ACE-III. Abnormality cutoff scores adjusted for age and education are presented., Conclusions: The ECAS shows good convergent validity with the ACE-III, but is less influenced by intelligence and presents less ceiling effects. The inclusion of an executive function assessment and behavioral interview in the ECAS makes it particularly useful for the assessment of frontal lobe disorders.

Background: The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) is a short assessment by which neuropsychological symptoms can be detected and quantified in people with ALS. To avoid potential practice effects with repeated administration, here we present alternative versions of the ECAS suitable for measuring change over time., Objective: To develop two alternate versions of the ECAS: ECAS-B and ECAS-C., Method: One hundred and forty-nine healthy adult participants were recruited. Thirty participants completed a pilot study in developing the alternate versions. Two groups of 40 participants were administered the ECAS-B or ECAS-C and compared to published data of the original ECAS (ECAS-A) to determine equivalence. An additional 39 participants were administered the ECAS consecutively, either repeating the original version (ECAS-A-A-A) serially or the different versions (ECAS-A-B-C) to determine potential practice effects. Recordings of assessments were scored by a second researcher to determine inter-rater reliability., Results: No significant differences were found between versions (A, B, C) of the composite performance measures of ALS Specific, ALS Non-Specific, and ECAS Total scores. Repeated serial administration of ECAS-A (A-A-A) produced some practice effects for composite scores, whereas no such effects were found when alternate versions were administered serially (A-B-C). Exceptionally high intra-class correlations were found for all three versions of the ECAS suggesting a high degree of rater agreement., Conclusion: The newly developed alternate forms of the ECAS are both highly equitable to the original ECAS-A and enable avoidance of practice effects, thus supporting their use in measuring cognition and behaviour over time.

Our objective was to assess the validity of the Edinburgh Cognitive and Behaviour ALS Screen (ECAS), a multi-domain screen designed to detect cognitive deficits in patients with motor disorders. Forty ALS patients (without pre-diagnosed dementia) and 40, age-, gender- and education-matched healthy controls were recruited. All participants underwent extensive neuropsychological assessment and the ECAS. Performance at neuropsychological assessment across five domains (fluency, executive function, language, memory and visuospatial function) was compared to the ECAS ALS-Specific (fluency, executive functions and social cognition, language), ALS Non-specific (memory, visuospatial functions), and Total scores. Data from the healthy controls produced population-based abnormality cut-offs: composite score performance ≤ 2 SD in any domain classified impairment at neuropsychological assessment. Thirty-three percent of patients were impaired, most commonly in a single domain (executive or language dysfunction). Receiver Operator Curve (ROC) analyses using ECAS Total scores and ALS-Specific scores revealed 85% sensitivity and 85% specificity in the detection of cognitive impairment characteristic of ALS (fluency, executive function, language). A five-point borderline range produced optimal values (ALS-Specific Score 77-82, and ECAS-Total Score 105-110). In conclusion, validation against gold standard extensive neuropsychology demonstrated that the ECAS is a screening tool with high sensitivity and specificity to impairment characteristic of ALS.

Despite growing interest in cognitive impairment of ALS patients, there are only limited studies available that characterize cognitive deficits in the Chinese ALS population through highly sensitive and specific screening tools. The Chinese version of the Addenbrooke's Cognitive Examination-revised (ACE-R) and mini-mental state examination (MMSE) were applied to evaluate cognitive function in 145 sporadic ALS patients and 50 healthy controls. The mean onset age was 50.72 ± 12.38 years. Results showed that the prevalence of cognitive deficits was 14.48% and 30.34% based on the MMSE and ACE-R, respectively. Patients had a broad range of cognitive impairment domains, including language (26.21%), orientation/attention (24.13%), visuospatial ability (24.13%), memory (23.45%) and verbal fluency (22.76%). Logistic regression indicated that older age of onset, female gender and lower educational levels were potential determinants of cognitive deficits in ALS. Multiple regression analyses showed that the cognitive deficit during the baseline visit was not associated with the progression rate of ALS. In conclusion, cognitive impairment is common in Chinese ALS patients, but does not necessarily worsen the progression of ALS. ACE-R is superior to MMSE in detecting deficits in patients. Cognitive impairment in ALS patients may relate to age of onset, female gender and lower education level.

The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) has recently been developed as a fast and easy cognitive screening tool specifically designed for patients with motor impairments in routine clinical use. The German/Swiss-German version of the ECAS was validated in a German-Swiss consortium. One hundred and thirty-six non-demented ALS patients and 160 healthy controls were included in the study. In addition, the Frontal Assessment Battery (FAB), Montreal Cognitive Assessment (MoCA) and Consortium to Establish a Registry for Alzheimer's Disease plus Scale (CERAD plus) were administered to subgroups of patients. Results showed that administration of ECAS was fast (mean 24 min). Similar to the population in the UK version, ALS patients performed significantly worse in the ALS-specific functions (p < 0.001), specifically in the domain of language (p < 0.001), verbal fluency (p = 0.005) and executive functions (p = 0.02), but not for the non-ALS-specific functions. Carers reported behavioural abnormalities in about 30% and psychotic symptoms in 6% of the patients. Compared to ECAS, FAB, MoCA and CERAD were more generic and performance was not adjusted to motor speed. We conclude that the German/Swiss-German version of the ECAS is a fast and easy to administer cognitive screening instrument sensitive for ALS-specific dysfunctions and behaviour change.

The cerebellar cognitive affective syndrome (CCAS) was first described by Schmahmann and Sherman as a constellation of symptoms including dysexecutive syndrome, spatial cognitive deficit, linguistic deficits and behavioural abnormalities in patients with a lesion in the cerebellum with otherwise normal brain. Neurosurgical patients with cerebellar tumours constitute one of the cohorts in which the CCAS has been described. In this paper, we present a critical review of the literature of this syndrome in neurosurgical patients. Thereafter, we present a prospective clinical study of 10 patients who underwent posterior fossa tumour resection and had a detailed post-operative neuropsychological, neuropsychiatric and neuroradiological assessment. Because our findings revealed a large number of perioperative neuroradiological confounding variables, we reviewed the neuroimaging of a further 20 patients to determine their prevalence. Our literature review revealed that study design, methodological quality and sometimes both diagnostic criteria and findings were inconsistent. The neuroimaging study (pre-operative, n = 10; post-operative, n = 10) showed very frequent neuroradiological confounding complications (e.g. hydrocephalus; brainstem compression; supratentorial lesions and post-operative subdural hygroma); the impact of such features had largely been ignored in the literature. Findings from our clinical study showed various degree of deficits in neuropsychological testing (n = 1, memory; n = 3, verbal fluency; n = 3, attention; n = 2, spatial cognition deficits; and n = 1, behavioural changes), but no patient had full-blown features of CCAS. Our study, although limited, finds no robust evidence of the CCAS following surgery. This and our literature review highlight a need for guidelines regarding study design and methodology when attempting to evaluate neurosurgical cases with regard to the potential CCAS.

This study presents the Edinburgh Cognitive and Behavioural ALS Screen (ECAS), developed for ALS patients with physical disability for use by health care professionals. The screen is designed to detect the specific profile of cognition and behaviour changes in ALS and to differentiate it from other disorders. Forty-eight ALS patients (none with evident dementia), 40 healthy controls and 20 carers were recruited. The ECAS, a 15-20-min screen, includes an ALS-Specific score (executive functions and social cognition; fluency; language); an ALS Non-specific score (memory; visuospatial functions); and a carer behaviour screen of five domains characteristic of frontotemporal dementia (FTD). Data from healthy controls produced abnormality cut-offs of 77/100 ALS-Specific score; 24/36 ALS Non-specific score; 105/136 ECAS Total. Twenty-nine percent of patients showed abnormal ALS-Specific scores, and 6% also showed abnormal ALS Non-specific scores. The most prevalent deficit occurred in language functions (35%) followed by executive functions and fluency (23% each). Forty percent of carers reported behaviour change in at least one domain, while 15% met criteria for possible FTD. In conclusion, the ECAS is an effective within-clinic assessment for ALS that determines the presence, severity and type of cognitive and/or behavioural changes, an essential first step to managing these symptoms.

Purpose of Review: Within the last decade, the neurobiology of action processing has moved from an obscure topic of specialist interest to one of the most popular themes in modern neuroscience. However, the wealth of literature and the diversity of approaches and theoretical models can make the field complex and, at times, bewildering. This review presents the main currents of research, examining their theoretical underpinnings in an interdisciplinary context., Recent Findings: The presence of specific deficits in verb and action processing has been documented in a wide range of neurodegenerative diseases, including parkinsonian syndromes and motor neuron disease. Interestingly, most of these disorders affect the motor system, suggesting a systematic relationship between motor functions and their cognitive and linguistic representations. Action processing has been explored with a whole spectrum of methodologies, from neuroimaging to transcranial and intracranial stimulation. The findings have been integrated with other influential concepts and theories, including mirror neurons and embodied cognition., Summary: Converging evidence from patient and imaging studies links the concepts of actions and their processing with the execution of actions through the motor system. The theory of embodied cognition remains influential as well as controversial. However, the points of criticism have changed, reflecting recent paradigm shifts.

Cognitive impairment in amyotrophic lateral sclerosis is characterized by deficits on tests of executive function; however, the contribution of abnormal processing speed is unknown. Methods are confounded by tasks that depend on motor speed in patients with physical disability. Structural and functional magnetic resonance imaging studies have revealed multi-system cerebral involvement, with evidence of reduced white matter volume and integrity in predominant frontotemporal regions. The current study has two aims. First, to investigate whether cognitive impairments in amyotrophic lateral sclerosis are due to executive dysfunction or slowed processing speed using methodology that accommodates motor disability. This is achieved using a dual-task paradigm and tasks that manipulate stimulus presentation times and do not rely on response motor speed. Second, to identify relationships between specific cognitive impairments and the integrity of distinct white matter tracts. Thirty patients with amyotrophic lateral sclerosis and 30 age- and education-matched control subjects were administered an experimental dual-task procedure that combined a visual inspection time task and digit recall. In addition, measures of executive function (including letter fluency) and processing speed (visual inspection time and rapid serial letter identification) were administered. Integrity of white matter tracts was determined using region of interest analyses of diffusion tensor magnetic resonance imaging data. Patients with amyotrophic lateral sclerosis did not show impairments on tests of processing speed, but executive deficits were revealed once visual inspection time was combined with digit recall (dual-task) and in letter fluency. In addition to the corticospinal tracts, significant differences in fractional anisotropy and mean diffusivity were found between groups in a number of prefrontal and temporal white matter tracts including the anterior cingulate, anterior thalamic radiation, uncinate fasciculus and hippocampal portion of the cingulum bundles. Significant differences also emerged in the anterior corona radiata as well as in white matter underlying the superior, medial and inferior frontal gyri and the temporal gyri. Dual-task performance significantly correlated with fractional anisotropy measures in the middle frontal gyrus white matter and anterior corona radiata. Letter fluency indices significantly correlated with fractional anisotropy measures of the inferior frontal gyrus white matter and corpus callosum in addition to the corticospinal tracts and mean diffusivity measures in the white matter of the superior frontal gyrus. The current study demonstrates that cognitive impairment in amyotrophic lateral sclerosis is not due to generic slowing of processing speed. Moreover, different executive deficits are related to distinct prefrontal tract involvement in amyotrophic lateral sclerosis with dual-task impairment associating with dorsolateral prefrontal dysfunction and letter fluency showing greater dependence on inferolateral prefrontal dysfunction.

Background: Progressive MS is associated with a high frequency of cognitive impairment. However, it is not clear to what extent this reflects global dysfunction, or independent deficits in specific functions., Objective: To characterise patterns of cognitive impairment in progressive MS on a multi-dimensional cognitive assessment tool well established in neurodegenerative diseases., Methods: Patients with secondary (SPMS; n=60) and primary progressive MS (PPMS; n=28) were assessed using the Addenbrooke's Cognitive Examination-Revised (ACE-R) multi-dimensional cognitive assessment scale. Independent dimensions of impairment and their relative contribution to the overall burden of cognitive dysfunction were then determined by factor analysis., Results: Two independent dimensions of impairment were seen: frontal-executive (attention, verbal fluency, recall) on one hand, and language and visuospatial functions on the other. These accounted for 55% and 45% respectively of the variance not explained by a global influence (14.2% and 11.6% respectively of total variance). Isolated language and visuospatial dysfunction was seen in both groups, whereas isolated impairment in frontal-executive functions was underrepresented in SPMS (p=0.001) and not seen in PPMS patients (p=0.040)., Conclusions: In addition to a prominent global influence on cognitive performance, patients with progressive MS commonly exhibit language and visuospatial deficits. Evaluation of these abilities should therefore be included in clinical assessment of cognition in progressive MS.

In order to determine the relationship between regional neuropathology and severity of clinical features in Richardson's syndrome (PSP-RS), the following hypotheses were tested: (1) executive dysfunction relates to prefrontal pathology; (2) language difficulties to pathology in Broca's area and/or the perirhinal cortex; and (3) visuospatial impairment to pathology in the supramarginal region. A prospectively studied case series of brain donors at a specialist clinic in Addenbrooke's Hospital Cambridge, UK, were examined. All those fulfilling postmortem criteria for PSP-RS and their last cognitive assessment within 24 months of death (N = 11/25) were included. The degree of regional neuronal loss and neuronal tau deposition across a number of cortical brain regions was performed and compared to 10 age- and sex-matched controls from the Sydney Brain Bank. Stepwise multiple linear regressions were used to determine the neuropathological correlates to cognitive scores and revealed the following. Executive dysfunction, as indexed by letter fluency, related to the degree of tau deposition in the superior frontal gyrus and supramarginal cortices (p < 0.020), language deficits related to neuron loss in the perirhinal gyrus (p < 0.001) and tau deposition in Broca's area (p = 0.020), while visuospatial dysfunction and global cognitive impairment related to tau deposition in the supramarginal gyrus (p < 0.007). The severity of cognitive deficits relate to regional cortical tau deposition in PSP-RS, although language impairment related to neuronal loss in the perirhinal region. Global cognitive dysfunction related most to the severity of tau deposition in the supramarginal gyrus warranting further research on the role of this brain region in PSP-RS.

Introduction: The motor features of corticobasal syndrome (CBS) are well recognized but the fact that many, if not all, affected patients develop cognitive impairment is still underrecognized. The dementia of CBS overlaps most with a language variant of frontotemporal dementia: progressive nonfluent aphasia (PNFA). The aim of this study was to determine the usefulness of Addenbrooke's Cognitive Examination-Revised (ACE-R) in the evaluation of CBS and to document similarities and differences between CBS and PNFA., Materials and Methods: Patients with well-defined CBS or PNFA from two tertiary referral centers were selected along with matched controls., Results: Twenty-one patients with CBS, 23 patients with PNFA and 47 age- and education- matched controls were included. Both CBS and PNFA groups showed substantial impairment on the ACE-R (f = 17.3-80.2, p < 0.001) and were significantly impaired in all domains (p < 0.001). The only significant difference between CBS and PNFA was in the visuospatial domain (p < 0.009), being worse in CBS. Using a cutoff of 88/89 out of 100, 90% of CBS and 82.6% of PNFA patients were impaired. At this cutoff of 88/89, ACE-R in CBS had sensitivity and specificity values of 91 and 98%, respectively., (Copyright © 2011 S. Karger AG, Basel.)

This article reports the severity and profile of neuropsychological impairment on a prevalent cohort of patients with a clinical diagnosis of either multiple system atrophy (n=372) or progressive supranuclear palsy (n=311) from the Neuroprotection and Natural History in Parkinson Plus Syndromes cohort. The Dementia Rating Scale and Frontal Assessment Battery were used to assess global cognition and executive dysfunction. For the Dementia Rating Scale impairment was observed in approximately 57% of the progressive supranuclear palsy group and 20% of the multiple system atrophy group. In the former, impairment in a single cognitive domain was observed in 40%, with the same number showing impairment in multiple domains, while in the latter the figures were 28.6 and 13.5%, respectively. On the Frontal Assessment Battery, impairment was observed in 62.0% of patients with progressive supranuclear palsy and 31.8% of those with multiple system atrophy. Although the progressive supranuclear palsy group performed worse overall, the cognitive profiles of the two groups on the Dementia Rating Scale subscales were identical, with the main impairment of the Initiation and Perseveration subscale. The impaired patients in the two groups were largely indistinguishable, qualitatively and quantitatively. Impairment was associated with greater age and clinical disability in both groups and was evident even in the early stages (22% in multiple system atrophy and 50% in progressive supranuclear palsy). Where a pathological diagnosis was available, the original clinical diagnosis was confirmed in the majority of cases, including those with significant cognitive impairment. The rate of impairment in those with a confirmed pathological diagnosis was comparable to that of the sample as a whole. These results demonstrate, in the largest prospectively recruited cohort of patients with progressive supranuclear palsy and multiple system atrophy studied to date, the existence of a cognitive profile similar to that previously reported in idiopathic Parkinson's disease. The results indicate a high level of cognitive impairment associated with progressive supranuclear palsy, but also point to comparable dysfunction in a substantial proportion of the patients with multiple system atrophy. Significant cognitive impairment appears consistent with a diagnosis of multiple system atrophy, even early in the disease, with important implications for diagnosis, research and management.

Objective: To compare the clinical features at presentation in patients with frontotemporal dementia (FTD) who develop amyotrophic lateral sclerosis (ALS) with those of patients with behavioral variant FTD (bvFTD) who do not develop ALS., Design: Archival data analysis on 61 deceased patients with FTD. We reviewed the clinical features at presentation (behavioral changes, psychotic symptoms, language, and executive and memory problems) and survival., Setting: Early Onset Dementia Clinic, Cambridge, England. Patients From a total of 156 patients with a clinical diagnosis of behavioral FTD, we selected 61 deceased patients with comprehensive medical records, including 43 with bvFTD and 18 with FTD/ALS., Main Outcome Measures: Clinical features and survival., Results: There was a significant association between the presence of delusions (50%; odds ratio, 4.4; 95% confidence interval, 1.3-14.5) and diagnosis of FTD/ALS (n = 18), whereas the behavioral features were identical in both groups. The interval between the onset of behavioral changes and diagnosis of ALS was less than 2 years in 12 (67%) of the patients with FTD/ALS. The median survival from symptom onset was significantly shorter for the FTD/ALS group (2.4 years; 95% confidence interval, 1.8-3.0 years) than for the bvFTD group (6.6 years; 5.6-7.6 years)., Conclusions: Delusions are particularly common in patients who develop FTD/ALS. The occurrence of delusions in the context of behavioral FTD should lead to an early search for ALS features.

Background: Stroke is one of the most common diseases to cause cognitive disorders in adults., Aims: To assess the frequency of cognitive deficits in stroke patients and to evaluate the prognostic value of cognitive syndromes for functional recovery., Methods: 200 consecutive patients were examined using a clinical screening battery for cognitive assessment in the second week after their first-ever stroke. 80 were re-examined after a 1-year follow-up., Results: In the post-acute stage, 78% patients were impaired in one or more cognitive domains. The most frequently affected cognitive abilities were attention (48.5%), language (27%), short-term memory (24.5%) and executive functions (18.5%). At the 1-year follow-up, attention deficits were still the most frequent symptom. In contrast, executive dysfunction, aphasia, and long-term memory disorder were significantly less frequent than in the post-acute period. Logistic regression analysis showed that older age, lower score on the Barthel Index, and the presence of executive dysfunction on initial examination were significant predictors of a poor functional outcome at the 1-year follow-up examination., Conclusions: Cognitive-behavioral syndromes are frequent and often chronic consequences of stroke. Executive deficits proved to be the most robust cognitive predictor of poor functional recovery after stroke., (Copyright 2008 S. Karger AG, Basel.)

The presence of cognitive impairment in corticobasal degeneration (CBD) is now widely recognised. Our review of the literature reveals that, although the pattern and severity of neuropsychological impairments can be highly variable across patients, several general trends can be identified. The most characteristic impairments are limb apraxia (usually ideomotor), constructional and visuospatial difficulties, acalculia, frontal dysfunction, and nonfluent aphasia. The limb apraxia is associated with deficits in drawing, copying, and handwriting, but there is emerging evidence that the problems with handwriting are not due exclusively to the apraxia. The findings with respect to episodic memory are more variable, but when there is impairment in this area, it tends to be milder than that seen in Alzheimer's disease. Semantic memory functioning appears relatively preserved but has been poorly studied. Problems with speech are common, and may be due to dysarthria or buccofacial apraxia. Aphasia, although initially considered rare, is in fact a common accompaniment of CBD, may be the presenting feature, and is typically nonfluent in type. More systematic investigation of the clinical and neuropathological overlap between progressive nonfluent aphasia (generally considered to be a form of frontotemporal dementia) and CBD is needed.

Unilateral neglect, an attentional deficit in detecting and acting on information coming from one side of space, is a relatively common consequence of right hemisphere stroke. Although neglect has been observed following damage to a variety of brain structures, to date no reports exist of neglect phenomena arising from Huntington's Disease (HD). However, reports in the animal and human literature suggest that neglect is possible following damage to a primary site for Huntington's pathology, the basal ganglia. Here we present a patient (BG) with genetically proven HD who, in the context of the mild intellectual, executive and attentional impairments associated with the disorder, showed a remarkably severe and stable neglect for left space. MRI and electrophysiological results make it unlikely that the spatial bias could be accounted for by basic sensory loss. In addition, behavioural investigation indicated that, although BG's neglect operated in a very striking manner along body-centred co-ordinates (missing almost all information presented to her left), more general limitations in visual attention were apparent to the left-side of information presented entirely to the right of the body midline. Further evidence is presented showing that the neglect was manifest on tactile and auditory tasks as well as those presented in the visual domain. The presence of neglect in HD in this case is novel and somewhat puzzling, particularly as flourodeoyglucose positron emission tomography revealed bilateral caudate hypoperfusion. Reducing the statistical threshold on this analysis revealed a potential frontal hypometabolism that was more marked in the right than left hemisphere. However, as this was only apparent at a threshold below that normally considered acceptable (due to the resulting number of false positives), this possible account of the neglect must be viewed very cautiously.

We sought to determine the pattern of cognitive deficits in patients with Wilson's disease (WD) with different type and degree of neurological involvement, and to interpret the findings in relation to the underlying pathology. A total of 67 WD patients were examined with a neuropsychological test battery assessing different aspects of cognitive processing. The patients were subdivided into three groups: neurologically asymptomatic, neurological with pure basal ganglia lesions, and neurological with more extensive pathology. The results were compared with 50 matched healthy controls. Patients with a neurological form of WD showed a mild but definitive impairment in all cognitive functions. In contrast, the neurologically asymptomatic patients showed no deficits when compared with normal controls. Multifocal pathology was associated with more severe cognitive deficits than selective basal ganglia lesions but did not contribute significantly to memory impairment. A range of cognitive functions, including frontal-executive ability, aspects of memory and visuospatial processing, are affected in the neurologically symptomatic WD patients. In contrast, no subliminal deficits were observed in the asymptomatic patients. The lesions of the basal ganglia seem to be of central importance in explaining the symptomatology., (Copyright 2002 Movement Disorder Society)

To investigate the nature of the apraxia in corticobasal degeneration (CBD) five patients with CBD and five matched controls were compared on tests of: i) meaningless and symbolic gesture production, ii) a battery of semantic tasks based on 20 everyday items (involving naming and picture-picture matching according to semantic attributes, matching gestures-to-objects, object usage from name and with the real object) and iii) a novel tool test of mechanical problem solving. All five patients showed severe impairment in the production of meaningless and symbolic gestures from command, and by imitation, and were also impaired when using real objects. Deficits were not, however, restricted to action production: four were unable to match gestures to objects and all five showed impairment in the selection and usage of novel tools in the mechanical problem solving task. Surprising was the finding of an additional semantic knowledge breakdown in three cases, two of whom were markedly anomic. The apraxia in CBD is, therefore, multifactorial. There is profound breakdown in the organisation and co-ordination of motor programming. In addition, patients show central deficits in action knowledge and mechanical problem solving, which has been linked to parietal lobe pathology. General semantic memory may also be affected in CBD in some cases and this may then contribute to impaired object usage. This combination of more than one deficit relevant for object use may explain why CBD patients are far more disabled by their dyspraxia in everyday life than any other patient group.

Current criteria for the clinical diagnosis of pathologically confirmed corticobasal degeneration (CBD) no longer reflect the expanding understanding of this disease and its clinicopathologic correlations. An international consortium of behavioral neurology, neuropsychology, and movement disorders specialists developed new criteria based on consensus and a systematic literature review. Clinical diagnoses (early or late) were identified for 267 nonoverlapping pathologically confirmed CBD cases from published reports and brain banks. Combined with consensus, 4 CBD phenotypes emerged: corticobasal syndrome (CBS), frontal behavioral-spatial syndrome (FBS), nonfluent/agrammatic variant of primary progressive aphasia (naPPA), and progressive supranuclear palsy syndrome (PSPS). Clinical features of CBD cases were extracted from descriptions of 209 brain bank and published patients, providing a comprehensive description of CBD and correcting common misconceptions. Clinical CBD phenotypes and features were combined to create 2 sets of criteria: more specific clinical research criteria for probable CBD and broader criteria for possible CBD that are more inclusive but have a higher chance to detect other tau-based pathologies. Probable CBD criteria require insidious onset and gradual progression for at least 1 year, age at onset ≥ 50 years, no similar family history or known tau mutations, and a clinical phenotype of probable CBS or either FBS or naPPA with at least 1 CBS feature. The possible CBD category uses similar criteria but has no restrictions on age or family history, allows tau mutations, permits less rigorous phenotype fulfillment, and includes a PSPS phenotype. Future validation and refinement of the proposed criteria are needed.

Addenbrooke's cognitive examination (ACE) is a cognitive screening tool that has developed through three stages: ACE, ACE-Revised (ACE-R), and ACE-Ⅲ. In addition, mini-Addenbrooke's Cognitive Examination (M-ACE) and ACE mobile are the additional versions that is derived from ACE-III. ACE and its related versions show better performance than Mini-Mental State Examination (MMSE) and Montreal Cognitive Assessment (MoCA) in detecting mild cognitive impairment in different neurological disorders. It has been translated into numerous languages, including Chinese. Through reviewing the history, validity, and comparison with other cognitive tests of Chinese versions of ACE, it aims to facilitate the clinical and scientific use, further development, improvement, and validation of Chinese versions of ACE in various neurological disorders and ultimately promote early identification and management of cognitive impairment in China. [ABSTRACT FROM AUTHOR]

Background: Bilingualism has been associated with cognitive benefits in healthy people as well as in patients with cognitive impairment due to stroke and dementia. However, the relationship between bilingualism and aphasia is more complex. While bilinguals are as likely as monolinguals to develop aphasia after stroke, studies of relationship between bilingualism and severity of poststroke language recovery are few and have produced conflicting results, with much evidence derived from immigrant populations or small case series. Aims: Against this background of limited number of studies, we set out to explore the relationship between bilingualism and severity of language impairment in stroke aphasia. We explored the hypothesis that enhanced cognitive abilities related to bilingualism may have a positive impact on recovery from aphasia. Methods & Procedures: We investigated 38 bilingual and 27 monolingual patients who participated in a longitudinal hospital-based stroke registry and were evaluated at least 3 months after stroke (mean 11.5 months). Patient performance on language and other cognitive functions was evaluated with Addenbrooke’s Cognitive Examination - Revised (ACE-R) validated for use in aphasia in local languages and for varying educational levels. The results of monolinguals and bilinguals were compared after accounting for confounding variables, including age, gender, education, occupation, medical, and stroke characteristics. Outcomes & Results: Aphasia severity as measured by the language domain sub-scores (total of language and fluency scores) of ACE-R was significantly higher in monolinguals compared with bilinguals (7.0 vs. 14.4, maximum score 40; p = 0.008, effect size = −0.691). Bilinguals performed significantly better than their monolingual counterparts in attention, memory, and visuospatial domains of ACE-R. A univariate general linear model analysis showed that bilingualism was significantly associated with higher language domain scores of ACE-R after adjusting for other confounding variables. Conclusions: The results suggest that although bilingual speakers are at equal risk of developing aphasia after stroke as monolingual ones, their aphasia is likely to be less severe. [ABSTRACT FROM AUTHOR]

The article presents brief information on progressive supranuclear palsy (PSP), a degenerative disease involving the gradual deterioration and death of specific volumes of the brain. It talks about physician John Clifford Richardson who states the disease extended beyond the motor system to include mental symptoms, personality changes and dementia. Topics discussed include natural history of disease, diogonostic criteria and rating scales, and neuropathological features of disease.