Language impairment in sporadic and familial (type 8) amyotrophic lateral sclerosis: A comparative study.

INTRODUCTION/AIMS: Language is frequently affected in patients with sporadic amyotrophic lateral sclerosis (sALS), with reduced performance in naming, syntactic comprehension, grammatical expression, and orthographic processing. However, the language profile of patients with familial type 8 ALS (ALS8), linked to p.P56S VAPB mutation, remains unclear. We investigated language in patients with ALS8 by examining their auditory comprehension and verbal production. METHODS: We included three groups of participants: (1) patients with sALS (n = 20), (2) patients with familial ALS8 (n = 22), and (3) healthy controls (n = 21). The groups were matched for age, sex, and education level. All participants underwent a comprehensive language battery, including the Boston Diagnostic Aphasia Examination, the reduced Token test, letter fluency, categorical fluency (animals), word definition from the Cambridge Semantic Memory Research Battery, and a narrative discourse analysis. Participants also were evaluated using Addenbrooke's Cognitive Exam-Revised Version, the Hospital Anxiety and Depression Scale, and the ALS Functional Rating Scale-Revised. RESULTS: Compared to controls, sALS and ALS8 patients had impaired performance on oral (syntactic and phonological processing) comprehension and inappropriate discourse cohesion. sALS and ALS8 did not differ in any language measure. There was no correlation between language scores and functional and psychiatric scales. DISCUSSION: ALS8 patients exhibit language deficits that are independent of motor features. These findings are consistent with the current evidence suggesting that ALS8 has prominent non-motor features.

Using in vivo functional and structural connectivity to predict chronic stroke aphasia deficits.

Focal brain damage caused by stroke can result in aphasia and advances in cognitive neuroscience suggest that impairment may be associated with network-level disorder rather than just circumscribed cortical damage. Several studies have shown meaningful relationships between brain-behaviour using lesions; however, only a handful of studies have incorporated in vivo structural and functional connectivity. Patients with chronic post-stroke aphasia were assessed with structural (n = 68) and functional (n = 39) MRI to assess whether predicting performance can be improved with multiple modalities and if additional variance can be explained compared to lesion models alone. These neural measurements were used to construct models to predict four key language-cognitive factors: (i) phonology; (ii) semantics; (iii) executive function; and (iv) fluency. Our results showed that each factor (except executive ability) could be significantly related to each neural measurement alone; however, structural and functional connectivity models did not explain additional variance above the lesion models. We did find evidence that the structural and functional predictors may be linked to the core lesion sites. First, the predictive functional connectivity features were found to be located within functional resting-state networks identified in healthy controls, suggesting that the result might reflect functionally specific reorganization (damage to a node within a network can result in disruption to the entire network). Second, predictive structural connectivity features were located within core lesion sites, suggesting that multimodal information may be redundant in prediction modelling. In addition, we observed that the optimum sparsity within the regularized regression models differed for each behavioural component and across different imaging features, suggesting that future studies should consider optimizing hyperparameters related to sparsity per target. Together, the results indicate that the observed network-level disruption was predicted by the lesion alone and does not significantly improve model performance in predicting the profile of language impairment.

Language impairments in seropositive and seronegative autoimmune encephalitis.

BACKGROUND AND OBJECTIVE: Autoimmune encephalitis (AE) is a rare neuroinflammatory disease affecting the central nervous system. To examine language functions in patients with different subsets of AE consisting of seropositive and seronegative groups. METHODS: Fifty-two patients were recruited from neurology departments in Melbourne, Australia, who met clinical criteria for possible AE. Language tests include the Naming Test from the Sydney Language Battery (SydBat), the semantic fluency trial from the Controlled Oral Word Association Test (COWAT), and the Vocabulary and Similarities subtests of the Weschler Abbreviated Scale of Intelligence-Second Edition. The results were standardised with normative data. RESULTS: The mean age of our cohort was 52.5 years old, with the average time from hospital admission to recruitment being 38.41 months. At an aggregate level, none of the mean language test z-scores were below normative data. At the patient level, impairment rates were 18.37% for COWAT (animals), 28.57% for SydBat (naming), 4.65% for Similarities, and 4.55% for Vocabulary. Chi-squared goodness of fit tests indicated that observed performances were significantly below expected performances for the SydBat (naming) test (p < 0.0001) and COWAT (animals) (p = 0.004). DISCUSSION: While, on average, language functions were within normal limits in patients with AE, but a subgroup exhibited lower performance in semantic fluency and visual confrontation naming, with impairment rates below expected norms. To advance understanding of language in chronic AE patients, exploring the impact of seizure burden, antiseizure medication use, and the relationship of language functions with other cognitive functions is crucial.

Methodologies for assessing morphosyntactic ability in people with Alzheimer's disease.

BACKGROUND: The detection and description of language impairments in neurodegenerative diseases like Alzheimer's Disease (AD) play an important role in research, clinical diagnosis and intervention. Various methodological protocols have been implemented for the assessment of morphosyntactic abilities in AD; narrative discourse elicitation tasks and structured experimental tasks for production, offline and online structured experimental tasks for comprehension. Very few studies implement and compare different methodological protocols; thus, little is known about the advantages and disadvantages of each methodology. AIMS: To discuss and compare the main behavioral methodological approaches and tasks that have been used in psycholinguistic research to assess different aspects of morphosyntactic production and comprehension in individuals with AD at the word and sentence levels. METHODS: A narrative review was conducted through searches in the scientific databases Google Scholar, Scopus, Science Direct, MITCogNet, PubMed. Only studies written in English, that reported quantitative data and were published in peer-reviewed journals were considered with respect to their methodological protocol. Moreover, we considered studies that reported research on all stages of the disease and we included only studies that also reported results of a healthy control group. Studies that implemented standardized assessment tools were not considered in this review. OUTCOMES & RESULTS: The main narrative discourse elicitation tasks implemented for the assessment of morphosyntactic production include interviews, picture-description and story narration, whereas the main structured experimental tasks include sentence completion, constrained sentence production, sentence repetition and naming. Morphosyntactic comprehension in AD has been assessed with the use of structured experimental tasks, both offline (sentence-picture matching, grammaticality judgment) and online (cross-modal naming,speeded sentence acceptability judgment, auditory moving window, word detection, reading). For each task we considered studies that reported results from different morphosyntactic structures and phenomena in as many different languages as possible. CONCLUSIONS & IMPLICATIONS: Our review revealed strengths and weaknesses of these methods but also directions for future research. Narrative discourse elicitation tasks as well as structured experimental tasks have been used in a variety of languages, and have uncovered preserved morphosyntactic production but also deficits in people with AD. A combination of narrative discourse elicitation and structured production tasks for the assessment of the same morphosyntactic structure has been rarely used. Regarding comprehension, offline tasks have been implemented in various languages, whereas online tasks have been mainly used in English. Offline and online experimental paradigms have often produced contradictory results even within the same study. The discrepancy between the two paradigms has been attributed to the different working memory demands they impose to the comprehender or to the different parsing processes they tap. Strengths and shortcomings of each methodology are summarized in the paper, and comparisons between different tasks are attempted when this is possible. Thus, the paper may serve as a methodological guide for the study of morphosyntax in AD and possibly in other neurodegenerative diseases. WHAT THIS PAPER ADDS: What is already known on this subject For the assessment of morphosyntactic abilities in AD, various methodological paradigms have been implemented: narrative discourse elicitation tasks and structured experimental tasks for production, and offline and online structured experimental tasks for comprehension. Very few studies implement and compare different methodological protocols; thus, little is known about the advantages and disadvantages of each methodology. What this paper adds to existing knowledge The paper presents an overview of methodologies that have been used to assess morphosyntactic production and comprehension of people with AD at the word and sentence levels. The paper summarizes the strengths and shortcomings of each methodology, providing both the researcher and the clinician with some directions in their endeavour of investigating language in AD. Also, the paper highlights the need for further research that will implement carefully scrutinized tasks from various experimental paradigms and will explore distinct aspects of the AD patients' morphosyntactic abilities in typologically different languages. What are the potential or actual clinical implications of this work? The paper may serve as a reference point for (psycho-)linguists who wish to study morphosyntactic abilities in AD, and for speech and language therapists who might need to apply morphosyntactic protocols to their patients in order to assess them or design appropriate therapeutic interventions for production and comprehension deficits.

Systematic Review and Meta-analysis of Language Symptoms due to Cerebellar Injury.

To date, cerebellar contribution to language is well established via clinical and neuroimaging studies. However, the particular functional role of the cerebellum in language remains to be clarified. In this study, we present the first systematic review of the diverse language symptoms in spoken language after cerebellar lesion that were reported in case studies for the last 30 years (18 clinical cases from 13 papers), and meta-analysis using cluster analysis with bootstrap and symptom co-occurrence analysis. Seven clusters of patients with similar language symptoms after cerebellar lesions were found. Co-occurrence analysis revealed pairs of symptoms that tend to be comorbid. Our results imply that the "linguistic cerebellum" has a multiform contribution to language function. The most possible mechanism of such contribution is the cerebellar reciprocal connectivity with supratentorial brain regions, where the cerebellar level of the language network has a general modulation function and the supratentorial level is more functionally specified. Based on cerebellar connectivity with supratentorial components of the language network, the "linguistic cerebellum" might be further functionally segregated.

Language patterns in Japanese patients with Alzheimer disease: A machine learning approach.

AIM: The authors applied natural language processing and machine learning to explore the disease-related language patterns that warrant objective measures for assessing language ability in Japanese patients with Alzheimer disease (AD), while most previous studies have used large publicly available data sets in Euro-American languages. METHODS: The authors obtained 276 speech samples from 42 patients with AD and 52 healthy controls, aged 50 years or older. A natural language processing library for Python was used, spaCy, with an add-on library, GiNZA, which is a Japanese parser based on Universal Dependencies designed to facilitate multilingual parser development. The authors used eXtreme Gradient Boosting for our classification algorithm. Each unit of part-of-speech and dependency was tagged and counted to create features such as tag-frequency and tag-to-tag transition-frequency. Each feature's importance was computed during the 100-fold repeated random subsampling validation and averaged. RESULTS: The model resulted in an accuracy of 0.84 (SD = 0.06), and an area under the curve of 0.90 (SD = 0.03). Among the features that were important for such predictions, seven of the top 10 features were related to part-of-speech, while the remaining three were related to dependency. A box plot analysis demonstrated that the appearance rates of content words-related features were lower among the patients, whereas those with stagnation-related features were higher. CONCLUSION: The current study demonstrated a promising level of accuracy for predicting AD and found the language patterns corresponding to the type of lexical-semantic decline known as 'empty speech', which is regarded as a characteristic of AD.

Rescue of Neglect and Language Impairment After Stroke Thrombectomy.

Background and Purpose: Although National Institutes of Health Stroke Scale scores provide an objective measure of clinical deficits, data regarding the impact of neglect or language impairment on outcomes after mechanical thrombectomy (MT) is lacking. We assessed the frequency of neglect and language impairment, rate of their rescue by MT, and impact of rescue on clinical outcomes. Methods: This is a retrospective analysis of a prospectively collected database from a comprehensive stroke center. We assessed right (RHS) and left hemispheric strokes (LHS) patients with anterior circulation large vessel occlusion undergoing MT to assess the impact of neglect and language impairment on clinical outcomes, respectively. Safety and efficacy outcomes were compared between patients with and without rescue of neglect or language impairment. Results: Among 324 RHS and 210 LHS patients, 71% of patients presented with neglect whereas 93% of patients had language impairment, respectively. Mean age was 71±15, 56% were females, and median National Institutes of Health Stroke Scale score was 16 (12­20). At 24 hours, MT resulted in rescue of neglect in 31% of RHS and rescue of language impairment in 23% of LHS patients, respectively. RHS patients with rescue of neglect (56% versus 34%, P<0.001) and LHS patients with rescue of language impairment (64 % versus 25%, P<0.01) were observed to have a higher rate of functional independence compared to patients without rescue. After adjusting for confounders including 24-hour National Institutes of Health Stroke Scale, rescue of neglect among RHS patients was associated with functional independence (P=0.01) and lower mortality (P=0.01). Similarly, rescue of language impairment among LHS patients was associated with functional independence (P=0.02) and lower mortality (P=0.001). Conclusions: Majority of LHS-anterior circulation large vessel occlusion and of RHS-anterior circulation large vessel occlusion patients present with the impairment of language and neglect, respectively. In comparison to 24-hour National Institutes of Health Stroke Scale, rescue of these deficits by MT is an independent and a better predictor of functional independence and lower mortality.

The molecular and phenotypic spectrum of CLCN4-related epilepsy.

OBJECTIVE: This study was undertaken to expand the phenotypic and genetic spectrum of CLCN4-related epilepsy and to investigate genotype-phenotype correlations. METHODS: We systematically reviewed the phenotypic and genetic spectrum of newly diagnosed and previously reported patients with CLCN4-related epilepsy. Three novel variants identified in four patients reported in this study were evaluated through in silico prediction and functional analysis by Western blot, immunofluorescence, and electrophysiological measurements. RESULTS: Epilepsy was diagnosed in 54.55% (24/44) of individuals with CLCN4-related disorders and was drug-resistant in most cases. Of 24 patients, 15 had epileptic encephalopathy and four died at an early age; 69.57% of patients had seizure onset within the first year of life. Myoclonic seizures are the most common seizure type, and 56.25% of patients presented multiple seizure types. Notably, seizure outcome was favorable in individuals with only one seizure type. All patients showed intellectual disability, which was severe in 65.22% of patients. Additional common features included language delay, behavioral disorders, and dysmorphic features. Five patients benefitted from treatment with lamotrigine. Most variants, which were mainly missense (79.17%), were inherited (70.83%). Whereas frameshift, intragenic deletion, or inherited variants were associated with milder phenotypes, missense or de novo variants led to more severe phenotypes. All evaluated CLCN4 variants resulted in loss of function with reduced ClC-4 currents. Nonetheless, genotype-phenotype relationships for CLCN4-related epilepsy are not straightforward, as phenotypic variability was observed in recurrent variants and within single families. SIGNIFICANCE: Pathogenic CLCN4 variants contribute significantly to the genetic etiology of epilepsy. The phenotypic spectrum of CLCN4-related epilepsy includes drug-resistant seizures, cognitive and language impairment, behavioral disorders, and congenital anomalies. Notably, the mutation type and the number of seizure types correlate with the severity of the phenotype, suggesting its use for clinical prognosis. Lamotrigine can be considered a therapeutic option.

STN-DBS affects language processing differentially in Parkinson's disease: Multiple-case MEG study.

OBJECTIVES: In this study, we investigated the effects of bilateral and unilateral deep brain stimulation of the subthalamic nucleus (STN-DBS) in PD patients on neural responses associated with two aspects of spoken language processing: semantics of action-related verbs and morphosyntactic processing. MATERIALS AND METHODS: Using a passive unattended paradigm to present spoken linguistic stimuli, we recorded magnetoencephalographic (MEG) responses in three PD patients in four DBS conditions: left unilateral STN-DBS, right unilateral STN-DBS, bilateral STN-DBS, and no STN-DBS. To ensure that any observed effects of DBS on the neuromagnetic responses could be attributed to the linguistic context per se and were not merely induced by the electrical stimulation, we assessed the effects of STN-DBS on linguistic contrasts within each stimulation condition. Hence, we contrasted the processing of action vs. abstract verbs as well as the processing of correct vs. incorrect morphosyntactic inflections within each DBS condition. RESULTS: The results revealed that, compared to the DBS-off state, both bilateral and right unilateral stimulation of the STN yielded significant dissociations in the processing of action and abstract verbs, with greater neuromagnetic responses for action verbs compared to abstract verbs. For morphosyntax processing, only left unilateral stimulation yielded significant dissociations (relative to the DBS-off state), with greater neuromagnetic responses to the incorrect inflections compared to the correct inflections. CONCLUSION: The results reflect differential effects of unilateral and bilateral STN-DBS on neuromagnetic responses associated with the processing of spoken language. They suggest that different specific aspects of linguistic information processing in PD are affected differently by STN-DBS.

Occurrence of speech-language disorders in the acute phase following pediatric acquired brain injury: results from the Ghent University Hospital.

AIMS: This study investigated the occurrence of speech-language disorders during the acute phase of recovery in children with acquired brain injury (ABI) with an age between 0 and 16 years. METHODS: A retrospective chart analysis was performed including 228 children (n = 118 boys, n = 110 girls) who consecutively presented with ABI over a 10-year period (2006-2016) at the children's rehabilitation center at Ghent University Hospital. Descriptive statistical analyses were applied. RESULTS: 71.1% (162/228) of the children who were admitted to the rehabilitation center presented with a speech-language disorder. Within this sample (n = 162), results demonstrated the occurrence of acquired disorders in language (48.9%), speech (35.1%), learning (33.3%), swallowing (21.5%), and early communicative functions (17.4%). The proportion of children presenting with disturbances in early communicative functions differed by ABI cause. More than half (10/18, 58.8%) of the children who presented with ABI following inflammatory processes demonstrated disorders in early communicative functions. CONCLUSIONS: Especially in young children who present with inflammatory processes as the ABI cause, speech-language pathologists (SLPs) must be aware of disorders in early speech-language development. The present findings allow the SLP to appropriately plan research, education, and clinical management.

Relationships among language ability, the arcuate fasciculus and lesion volume in patients with putaminal hemorrhage: a diffusion tensor imaging study.

Relationships among language ability, arcuate fasciculus and lesion volume were investigated by use of diffusion tensor tractography in patients with putaminal hemorrhage. Thirty-three right-handed patients within six weeks of hemorrhage onset were recruited. Correlation of the aphasia quotient with subset (fluency, comprehension, repetition, naming) scores, diffusion tensor tractography parameters and lesion volume of patients, aphasia quotient (r = 0.446) with subset (naming: r = 0.489) score had moderate positive correlations with fractional anisotropy of the left arcuate fasciculus. The aphasia quotient subset (repetition) score had a strong positive correlation with fractional anisotropy of the left arcuate fasciculus (r = 0.520), whereas, aphasia quotient subset (fluency and comprehension) scores had no significant correlations with fractional anisotropy of the left arcuate fasciculus after Benjamini-Hochberg correction. Aphasia quotient (r = 0.668) with subset (fluency: r = 0.736, comprehension: r = 0.739, repetition: r = 0.649, naming: r = 0.766) scores had strong positive correlations with the tract volume of the left arcuate fasciculus and strong negative correlations with lesion volume (r = -0.521, fluency: r = -0.520, comprehension: r = -0.513, repetition: r = -0.518, naming: r = -0.562). Fractional anisotropy of the left arcuate fasciculus had a moderate negative correlation with lesion volume (r = -0.462), whereas the tract volume of the left arcuate fasciculus had a strong negative correlation with lesion volume (r = -0.700). According to the result of mediation analysis, tract volume of the left arcuate fasciculus fully mediated the effect of lesion volume on the aphasia quotient. Regarding the receiver operating characteristic curve, the lesion volume cut-off value was 29.17 cm3 and the area under the curve (0.74), sensitivity (0.77) and specificity (0.80) were higher than those of fractional anisotropy, tract volume and aphasia quotient cut-off values. It was found that level of language disability was related to lesion volume as well as to injury severity of arcuate fasciculus in the dominant hemisphere of patients with putaminal hemorrhage. In particular, the tract volume of the arcuate fasciculus in the dominant hemisphere fully mediated the effect of lesion volume on language ability. Additionally, a lesion volume of approximately 30 cm3 was helpful in discriminating arcuate fasciculus discontinuation in the dominant hemisphere.

Frontotemporal dementias: main syndromes and underlying brain changes.

PURPOSE OF REVIEW: Frontotemporal dementia (FTD) is a rare dementia, that accounts for about 15% of all dementia cases. Despite consensus diagnostic criteria, FTD remains difficult to diagnose in life because of its complex and variable clinical phenomenology and heterogeneous disorders. This review provides an update on the current knowledge of the main FTD syndromes -- the behavioural variant, semantic variant, and nonfluent/agrammatic variant-- their brain abnormalities and genetic profiles. RECENT FINDINGS: The complexity of the clinical features in FTD has become increasingly apparent, particularly in the domain of behaviour. Such behaviour changes are now also being recognized in the language variants of FTD. Initial interest on emotion processing and social cognition is now complemented by studies on other behavioural disturbance, that spans gambling, antisocial behaviours, repetitive behaviours, and apathy. At a biological level, novel pathological subcategories continue to be identified. From a genetic viewpoint, abnormalities in three genes explain nearly three quarters of familial cases of FTD. SUMMARY: In the absence of effective drug treatments, novel approaches are needed to target some of the most disabling features of FTD, such as language loss or behaviour disturbance. Recent interventions appear promising but will require confirmation.

Development of the clinical assessment scale in autoimmune encephalitis.

OBJECTIVE: There is no scale for rating the severity of autoimmune encephalitis (AE). In this study, we aimed to develop a novel scale for rating severity in patients with diverse AE syndromes and to verify the reliability and validity of the developed scale. METHODS: The key items were generated by a panel of experts and selected according to content validity ratios. The developed scale was initially applied to 50 patients with AE (development cohort) to evaluate its acceptability, reproducibility, internal consistency, and construct validity. Then, the scale was applied to another independent cohort (validation cohort, n = 38). RESULTS: A new scale consisting of 9 items (seizure, memory dysfunction, psychiatric symptoms, consciousness, language problems, dyskinesia/dystonia, gait instability and ataxia, brainstem dysfunction, and weakness) was developed. Each item was assigned a value of up to 3 points. The total score could therefore range from 0 to 27. We named the scale the Clinical Assessment Scale in Autoimmune Encephalitis (CASE). The new scale showed excellent interobserver (intraclass correlation coefficient [ICC] = 0.97) and intraobserver (ICC = 0.96) reliability for total scores, was highly correlated with modified Rankin scale (r = 0.86, p < 0.001), and had acceptable internal consistency (Cronbach α = 0.88). Additionally, in the validation cohort, the scale showed high interobserver reliability (ICC = 0.99) and internal consistency (Cronbach α = 0.92). INTERPRETATION: CASE is a novel clinical scale for AE with a high level of clinimetric properties. It would be suitable for application in clinical practice and might help overcome the limitations of current outcome scales for AE. ANN NEUROL 2019;85:352-358.

Pure word deafness in a patient with bilateral ischemic stroke in the superior temporal gyrus (STG).

INTRODUCTION: Pure word deafness is a rare neurological disorder linked with an inability to comprehend speech. The precise localization of damage is still unclear. CASE PRESENTATION: A 72-year-old woman presented with acute verbal contact disturbances, disorientation, and anxiety. In the neurological examination a slight right hemiparesis was found, together with a pure word deafness (PWD) in neuropsychological tests. Neuroimages confirmed bilateral ischemic lesions in the superior temporal gyrus (STG) acute in the left hemisphere. CONCLUSIONS: Our case suggested that bilateral, symmetric lesions in the region of STG could cause pure world deafness. The diversity and severity of symptoms confirm the necessity of pure world deafness classification for subtypes.

Asymmetric Bálint's syndrome with multimodal agnosia, bilateral agraphesthesia, and ineffective kinesthetic reading due to subcortical hemorrhage in the left parieto-occipito-temporal area.

We report a patient with asymmetric Bálint's syndrome (predominantly right-sided oculomotor apraxia and simultanagnosia and optic ataxia for the right hemispace), and multimodal agnosia (apperceptive visual agnosia and bilateral associative tactile agnosia) with accompanying right hemianopia, bilateral agraphesthesia, hemispatial neglect, global alexia with unavailable kinesthetic reading, and lexical agraphia for kanji (Japanese morphograms), after hemorrhage in the left parieto-occipito-temporal area. The coexistence of tactile agnosia, bilateral agraphesthesia, and ineffective kinesthetic reading suggests that tactile-kinesthetic information can be interrupted because of damage to the fiber connection from the parietal lobe to the occipito-temporal area, leading to these tactually related cognitive impairments.

An Efficient Bedside Measure Yields Prognostic Implications for Language Recovery in Acute Stroke Patients.

BACKGROUND: It is estimated that ∼30% of stroke survivors have aphasia, a language disorder resulting from damage to left-hemisphere language networks. In acute care settings, efficient identification of aphasia is critical, but there is a paucity of efficient bedside assessments. OBJECTIVE: To determine whether objective measures on a picture description task administered within 48 hours post stroke (a) predict language recovery, (b) estimate left-hemisphere lesion volume and location, and (c) correlate with other bedside language assessments. METHOD: Behavioral data were scored at acute and chronic time points. Neuroimaging data were used to determine associations between the picture description task, other language assessments, and lesion volume and location. RESULTS: Acute content units, age, and total lesion volume predicted communication recovery; F3,18 = 3.98, P = 0.024; r = 0.40. Significant correlations were found between the picture description task and lesion volume and location. Picture description outcomes were also associated with other clinical language assessments. DISCUSSION: This picture description task quickly predicted the language performance (communication recovery and outcome) for patients who suffered a left-hemisphere stroke. Picture description task measures correlated with damage in the left hemisphere and with other, more time-consuming and cumbersome language assessments that are typically administered acutely at bedside. CONCLUSION: The predictive value of this picture description task and correlations with existing language assessments substantiate the clinical importance of a reliable yet rapid bedside measure for acute stroke patients that can be administered by a variety of health care professionals.

Neuropsychological functions and psychiatric symptoms in late-onset manifestation of pantothenate kinase-associated neurodegeneration: a clinical case report.

Purpose of the study: Pantothenate Kinase-associated Neurodegeneration (PKAN) is a form of Neurodegeneration with brain iron accumulation (NBIA) due to gene mutations. Classical phenotype showed progressive neurological symptoms associated to a characteristic pattern of basal ganglia iron deposits. The atypical case, with adult-onset manifestation, could have neuropsychiatric symptoms with behavioral deficits. We described an adult-onset case of Pantothenate Kinase-associated Neurodegeneration.Materials and methods: The patient underwent neuropsychological and psychiatric evaluation and Magnetic Resonance Imaging, respectively for cognitive and behavioral assessment and to confirm the characteristic findings of this syndrome.Results: The patient showed atypical phenotype of Pantothenate Kinase-associated Neurodegeneration, characterized by language deficits, dixesecutive, and psychiatric manifestations, such as obsessive ideation, impulsivity, and disinhibition.Conclusions: This description could be helpful to a more correct diagnosis and clinical management.

Language ability and adult homelessness.

BACKGROUND: People experiencing homelessness are at increased risk of neurological disorder due to multiple factors such as substance abuse, infection, and higher rates of serious mental illness and traumatic brain injury. This could affect cognitive and language skills. Indeed, past research has suggested that certain language-related skills tend to be lower in people experiencing homelessness. However, that research has compared homeless samples with age-matched normative samples and not with samples of people from similar socio-economic backgrounds. Therefore, it is unclear whether homelessness is even a relevant factor, or if adults who are homeless tend to have appropriate linguistic skills relative to their social and educational background. AIMS: To compare the language skills of a group of adults with histories of homelessness with an education-matched control group. It was hypothesized that participants with histories of homelessness would have worse language performance than their matched controls. METHODS & PROCEDURES: A quasi-experimental design was employed involving 17 adults with histories of homelessness, mainly rough sleeping, in the city of Quito in Ecuador, and a sample of 16 adults who had never been homeless. All were assessed with measures of head injury, substance dependence, affective disorder and language skills. A paired-sample analysis was performed on homeless and control participants matched for educational background, used as an index of socio-economic background. OUTCOMES & RESULTS: The mean years of formal education was low in both the homeless sample (mean = 5.82 years) and the control sample (mean = 6.75 years). There were no differences between the groups for any demographic or clinical factors, nor for a measure of expected or 'premorbid' ability based on single-word reading, nor for current non-verbal cognitive functioning. In contrast, the homeless group scored significantly worse than the control group on measures of auditory comprehension and oral expression. CONCLUSIONS & IMPLICATIONS: Adults with histories of homelessness may have worse language skills than would be expected based on their educational backgrounds and non-verbal cognitive abilities. It is possible that some of this lower language ability is pathological, in the form of either a developmental language disorder or an acquired impairment. As such, some adults who are homeless may benefit from therapy directed at clinical language disorders.

Improving Discourse following Traumatic Brain Injury: A Tale of Two Treatments.

Persons with traumatic brain injury (TBI) often present with discourse-level deficits that affect functional communication. These deficits are not thought to be primarily linguistic in nature but instead are thought to arise from the interaction of linguistic and cognitive processes. Discourse processing treatment (DPT) is a discourse-based treatment protocol which targets discourse deficits frequently seen in TBI. Attention Process Training-2 (APT-2) is a published treatment protocol which targets four levels of attention. The purpose of this article is to investigate the effectiveness of DPT and APT-2 in improving discourse production and cognition in adults with TBI. Our results suggest that DPT results in greater improvement in discourse informativeness and coherence, but the combination of DPT and APT-2 resulted in greater generalization to untrained stimuli. Both DPT and APT-2 appear to have some potential to improve cognition, but there was intersubject variability with regard to which treatment is more effective.

[The Comprehensive Aphasia Test in Hungarian]. / CAT-H ­ új eljárás az afázia magyar nyelvû diagnosztikájában.

BACKGROUND AND PURPOSE: In this paper we present the Comprehensive Aphasia Test-Hungarian (CAT-H; Zakariás and Lukács, in preparation), an assessment tool newly adapted to Hungarian, currently under standardisation. The test is suitable for the assessment of an acquired language disorder, post-stroke aphasia. The aims of this paper are to present 1) the main characteristics of the test, its areas of application, and the process of the Hungarian adaptation and standardisation, 2) the first results from a sample of Hungarian people with aphasia and healthy controls. METHODS: Ninety-nine people with aphasia, mostly with unilateral, left hemisphere stroke, and 19 neurologically intact control participants were administered the CAT-H. In addition, we developed a questionnaire assessing demographic and clinical information. The CAT-H consists of two parts, a Cognitive Screening Test and a Language Test. RESULTS: People with aphasia performed significantly worse than the control group in all language and almost all cognitive subtests of the CAT-H. Consistent with our expectations, the control group performed close to ceiling in all subtests, whereas people with aphasia exhibited great individual variability both in the language and the cognitive subtests. In addition, we found that age, time post-onset, and type of stroke were associated with cognitive and linguistic abilities measured by the CAT-H. CONCLUSION: Our results and our experiences clearly show that the CAT-H provides a comprehensive profile of a person's impaired and intact language abilities and can be used to monitor language recovery as well as to screen for basic cognitive deficits in aphasia. We hope that the CAT-H will be a unique resource for rehabilitation professionals and aphasia researchers in aphasia assessment and diagnostics in Hungary.