Search

Individuals with primary progressive aphasia (PPA) suffer a gradual decline in communication ability as a result of neurodegenerative disease. Language treatment shows promise as a means of addressing these difficulties but much remains to be learned with regard to the potential value of treatment across variants and stages of the disorder. We present two cases, one with semantic variant of PPA and the other with logopenic PPA, each of whom underwent treatment that was unique in its focus on training self-cueing strategies to engage residual language skills. Despite differing language profiles and levels of aphasia severity, each individual benefited from treatment and showed maintenance of gains as well as generalization to untrained lexical items. These cases highlight the potential for treatment to capitalize on spared cognitive and neural systems in individuals with PPA, improving current language function as well as potentially preserving targeted skills in the face of disease progression.

Background: Treatment for lexical retrieval impairment has been shown to yield positive outcomes in individuals with aphasia due to focal lesions, but there has been little research regarding the treatment of such impairments in individuals with progressive aphasia. Aims: The purpose of this study was to examine the therapeutic effects of a semantic treatment for anomia in progressive aphasia relative to the outcome in an individual with stroke-induced aphasia. Methods & Procedures: Two individuals with progressive aphasia and one with aphasia resulting from stroke participated in the study. Each participant presented with fluent, anomic aphasia; however, one of the patients with progressive aphasia demonstrated characteristics indicating a likely progression towards non-fluency. Each participant received a brief, intensive treatment intended to improve lexical retrieval in the context of generative naming for selected semantic categories. Treatment tasks included guided lexical retrieval prompted by the identification and elaboration of items within semantic subcategories, as well as other semantic tasks. Treatment outcomes were quantified using standard effects sizes as well as nonparametric tests comparing pre- versus post-treatment performance. Outcomes & Results: One of the individuals with progressive aphasia showed large treatment effects for lexical retrieval of items from targeted semantic categories. The other progressive aphasia patient showed very small effects overall for treated categories. The individual with the focal lesion due to stroke showed medium-sized effects for trained categories as well as significant improvement on a standardised measure of naming. Conclusions: Findings indicate that intensive, semantically based treatment for lexical retrieval can result in positive outcomes in individuals with progressive as well as stroke-induced aphasia. Examination of individual differences suggests that the status of semantic and episodic memory may provide predictive information regarding responsiveness to treatment. [ABSTRACT FROM AUTHOR]

Background: According to a dual-route model of written language processing, spelling of irregular words provides an index of the status of lexical spelling procedures, whereas nonword spelling provides information about non-lexical processing that relies on phoneme-grapheme conversion. Because regular words can be spelled using either route, accuracy for such words may reflect the combined function of the two routes, and may be mathematically predicted on the basis of spelling accuracy for irregular words and nonwords. Aims: The purpose of the present study was to examine the application of a dual-route prediction equation, and a related multiple regression model, to evaluate pre-post treatment spelling performance of individuals with acquired alexia/agraphia. Methods & Procedures: Eight individuals with language impairment due to left hemisphere damage received behavioral treatment to improve their written spelling. Their spelling performance was examined before and after treatment on untrained word lists with regular and irregular spellings, and pronounceable nonwords, and concurrence between predicted and observed spelling of regular words was evaluated. Outcomes & Results: The group demonstrated significantly improved spelling performance after treatment, and the prediction equation and multiple regression model both accurately predicted regular word performance on the basis of irregular word and nonword scores. In addition, the multiple regression model provided potentially useful information about the relative contribution of the lexical and non-lexical routes to spelling performace. Conclusions: The prediction equation and related multiple regression model used in this study can offer novel insight into the cognitive processes available to individuals with agraphia as well as provide a quantitative means to characterize response to treatment. [ABSTRACT FROM AUTHOR]

Background: It has been proposed that anomia following left inferior temporal lobe lesions may have two different underlying mechanisms with distinct neural substrates. Specifically, naming impairment following damage to more posterior regions (BA 37) has been considered to result from a disconnection between preserved semantic knowledge and phonological word forms (pure anomia), whereas anomia following damage to anterior temporal regions (BAs 38, 20/21) has been attributed to the degradation of semantic representations (semantic anomia). However, the integrity of semantic knowledge in patients with pure anomia has not been demonstrated convincingly, nor were lesions in these cases necessarily confined to BA 37. Furthermore, evidence of semantic anomia often comes from individuals with bilateral temporal lobe damage, so it is unclear whether unilateral temporal lobe lesions are sufficient to produce significant semantic impairment. Aims: The main goals of this study were to determine whether anomia following unilateral left inferior temporal lobe damage reflected a loss of semantic knowledge or a post-semantic deficit in lexical retrieval and to identify the neuroanatomical correlates of the naming impairment. Methods & Procedures: Eight individuals who underwent left anterior temporal lobectomy (L ATL) and eight individuals who sustained left posterior cerebral artery strokes (L PCA) completed a battery of language measures that assessed lexical retrieval and semantic processing, and 16 age- and education-matched controls also completed this battery. High-resolution structural brain scans were collected to conduct lesion analyses. Outcomes & Results: Performance of L ATL and L PCA patients was strikingly similar, with both groups demonstrating naming performance ranging from moderately impaired to unimpaired. Anomia in both groups occurred in the context of mild deficits to semantic knowledge, which manifested primarily as greater difficulty in naming living things than nonliving things and greater difficulty in processing visual/perceptual as opposed to functional/associative semantic attributes. Lesion analyses indicated that both patient groups sustained damage to anterior inferior temporal lobe regions implicated in semantic processing. Conclusions: These results contribute to a better understanding of the cognitive mechanism of naming impairment in patients with temporal lobe damage and support the notion that pure anomia and semantic anomia represent two endpoints along a continuum of semantic impairment. Unilateral left temporal lobe lesions in our patients resulted in relatively mild semantic deficits that were apparent primarily in lexical production tasks, whereas severe semantic impairment likely requires bilateral temporal lobe damage. [ABSTRACT FROM AUTHOR]

The spoken language of individuals with mild aphasia and age-matched control subjects was studied under conditions of isolation, focused attention, and divided attention. A picture-description task was completed alone and in competition with a tone-discrimination task. Regardless of condition, individuals with aphasia performed more poorly on most morphosyntactic, lexical, and pragmatic measures of spoken language than control subjects. Increasing condition complexity resulted in little quantitative or qualitative change in the spoken language of the control group. In contrast, the individuals with aphasia showed dual-task interference; as they shifted from isolation to divided-attention conditions, they produced fewer syntactically complete and complex utterances, fewer words, and poorer word-finding accuracy. In pragmatic terms, their communication was considered less successful and less efficient. These results suggest that decrements of attentional capacity or its allocation may negatively affect the quantity and quality of the spoken language of individuals with mild aphasia. [ABSTRACT FROM AUTHOR]

Language performance requires support from central cognitive/linguistic abilities as well as the more peripheral sensorimotor skills to plan and implement spoken and written communication. Both output modalities are vulnerable to impairment following damage to the language-dominant hemisphere, but much of the research to date has focused exclusively on spoken language. In this study we aimed to examine an integrated model of language processing that includes the common cognitive processes that support spoken and written language, as well as modality-specific skills. To do so, we evaluated spoken and written language performance from 87 individuals with acquired language impairment resulting from damage to left perisylvian cortical regions that collectively constitute the dorsal language pathway. Comprehensive behavioral assessment served to characterize the status of central and peripheral components of language processing in relation to neurotypical controls ( n = 38). Performance data entered into principal components analyses (with or without control scores) consistently yielded a strong five-factor solution. In line with a primary systems framework, three central cognitive factors emerged: semantics, phonology, and orthography that were distinguished from peripheral processes supporting speech production and allographic skill for handwriting. The central phonology construct reflected performance on phonological awareness and manipulation tasks and showed the greatest deficit of all the derived factors. Importantly, this phonological construct was orthogonal to the speech production factor that reflected repetition of words/non-words. When entered into regression analyses, semantics and phonological skill were common predictors of language performance across spoken and written modalities. The speech production factor was also a strong, distinct predictor of spoken naming and oral reading, in contrast to allographic skills which only predicted written output. As expected, visual orthographic processing contributed more to written than spoken language tasks and reading/spelling performance was strongly reliant on phonological and semantic abilities. Despite the heterogeneity of this cohort regarding aphasia type and severity, the marked impairment of phonological skill was a unifying feature. These findings prompt greater attention to clinical assessment and potential treatment of underlying phonological skill in individuals with left perisylvian damage., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Beeson, Rising, Sachs and Rapcsak.)

Purpose The aim of this tutorial is to share lessons learned from a speech, language, and hearing sciences department at a land-grant, Hispanic-serving institution (HSI) after revising the graduate admissions review process and subsequent discussions related to equity in assessment. This tutorial describes the department as a case example and includes guiding questions that may be helpful for other organizations. Method An adaptive case study approach was used and centered on supporting students at Hispanic-serving institutions. The approach was characterized by structured collaborative reflection throughout the process and engagement of relevant stakeholders at multiple levels. The adaptive method allowed for interim synthesis of stakeholder discussions to inform subsequent phases of the reflection process. Results This tutorial shares critical motivations, barriers, facilitators, and phases that were identified in moving toward holistic evaluation for graduate admissions. Targeted areas for continued improvement related to diversity, equity, and inclusion are described. Conclusions This tutorial outlines lessons learned from changing graduate admissions practices toward holistic review. Self-reflection prompts are provided for institutions and organizations considering changes to their review process. In order to best support diverse communities, increased workforce diversity is needed in the speech, language, and hearing sciences professions, and holistic review practices are recommended as one way to support increased diversity, equity, and inclusion.

This study investigated the underlying nature of apraxia of speech (AOS) by testing two competing hypotheses. The Reduced Buffer Capacity Hypothesis argues that people with AOS can plan speech only one syllable at a time Rogers and Storkel [1999. Planning speech one syllable at a time: The reduced buffer capacity hypothesis in apraxia of speech. Aphasiology , 13 (9-11), 793-805. https://doi.org/10.1080/026870399401885]. The Program Retrieval Deficit Hypothesis states that selecting a motor programme is difficult in face of competition from other simultaneously activated programmes Mailend and Maas [2013. Speech motor programming in apraxia of speech: Evidence from a delayed picture-word interference task. American Journal of Speech-Language Pathology , 22 (2), S380-S396. https://doi.org/10.1044/1058-0360(2013/12-0101)]. Speakers with AOS and aphasia, aphasia without AOS, and unimpaired controls were asked to prepare and hold a two-word utterance until a go-signal prompted a spoken response. Phonetic similarity between target words was manipulated. Speakers with AOS had longer reaction times in conditions with two similar words compared to two identical words. The Control and the Aphasia group did not show this effect. These results suggest that speakers with AOS need additional processing time to retrieve target words when multiple motor programmes are simultaneously activated.

Purpose Lexical retrieval impairment is a universal characteristic of aphasia and a common treatment focus. Although naming improvement is well documented, there is limited information to shape expectations regarding long-term recovery. This was the motivation for a retrospective study of longitudinal data on the Boston Naming Test (BNT; Kaplan, Goodglass, & Weintraub, 1983, 2000). Method BNT scores were analyzed from a heterogeneous cohort of 42 individuals with anomia associated with a range of aphasia types. The data were collected over the course of 20 years from individuals who had participated in treatment and received at least 2 BNT administrations. A linear mixed model was implemented to evaluate effects of initial BNT score, time postonset, and demographic variables. For those over 55 years of age, BNT change was evaluated relative to data from the Mayo Clinic's Older Americans Normative Studies. Results There was a significant average improvement of +7.67 points on the BNT in individuals followed for an average of 2 years. Overall, the average rate of improvement was +5.84 points per year, in contrast to a decline of 0.23 points per year in a healthy adult cohort from the Mayo Clinic's Older Americans Normative Studies. Naming recovery was approximately linear, with significant main effects of initial BNT score (i.e., initial severity) and time postonset; the greatest changes were noted in those whose initial severity was moderate. Conclusions These findings indicate a positive prognosis for naming improvement over time regardless of demographic factors and provide estimates for clinical predictions for those who seek rehabilitation during the chronic phase.

Maximal recovery from acquired language impairment may require progression from one behavioural treatment protocol to the next in order to build upon residual and relearned cognitive-linguistic and sensory-motor processes. We present a five-stage treatment sequence that was initiated at one year post stroke in a woman with acquired impairments of spoken and written language. As is typical of individuals with left perisylvian damage, she demonstrated marked impairment of phonological retrieval and sublexical phonology, but she also faced additional challenges due to impaired letter shape knowledge and visual attention. The treatment sequence included (1) written spelling of targeted words, (2) retraining sublexical sound-to-letter correspondences and phonological manipulation skills, (3) training strategic approaches to maximise interactive use of lexical, phonological, and orthographic knowledge, (4) lexical retrieval of spoken words, and finally (5) sentence-level stimulation to improve grammatical form of written narratives. This Phase II clinical study documented positive direct treatment outcomes along with evidence of a significant reduction in the underlying deficits and generalisation to untrained items and language tasks. Improvements on a comprehensive assessment battery were realised as functional gains in everyday written and spoken communication, including improved lexical retrieval and grammatical complexity of written narratives. This case provides a valuable example of the cumulative therapeutic benefit of sequential application of theoretically motivated treatment protocols.

Purpose Recent studies confirm the utility of speech-language intervention in primary progressive aphasia (PPA); however, long-term outcomes, ideal dosage parameters, and relative benefits of intervention across clinical variants warrant additional investigation. The purpose of this study was to determine whether naming treatment affords significant, lasting, and generalized improvement for individuals with semantic and logopenic PPA and whether dosage manipulations significantly affect treatment outcomes. Method Eighteen individuals with PPA (9 semantic and 9 logopenic variant) underwent lexical retrieval treatment designed to leverage spared cognitive-linguistic domains and develop self-cueing strategies to promote naming. One group (n = 10) underwent once-weekly treatment sessions, and the other group (n = 8) received the same treatment with 2 sessions per week and an additional "booster" treatment phase at 3 months post-treatment. Performance on trained and untrained targets/tasks was measured immediately after treatment and at 3, 6, and 12 months post-treatment. Results Outcomes from the full cohort of individuals with PPA showed significantly improved naming of trained items immediately post-treatment and at all follow-up assessments through 1 year. Generalized improvement on untrained items was significant up to 6 months post-treatment. The positive response to treatment was comparable regardless of session frequency or inclusion of a booster phase. Outcomes were comparable across PPA subtypes, as was maintenance of gains over the post-treatment period. Conclusion This study documents positive naming treatment outcomes for a group of individuals with PPA, demonstrating strong direct treatment effects, maintenance of gains up to 1 year post-treatment, and generalization to untrained items. Lexical retrieval treatment, in conjunction with daily home practice, had a strong positive effect that did not require more than 1 clinician-directed treatment session per week. Findings confirm that strategic training designed to capitalize on spared cognitive-linguistic abilities results in significant and lasting improvement, despite ongoing disease progression, in PPA.

Purpose Auditory-perceptual assessment, in which trained listeners rate a large number of perceptual features of speech samples, is the gold standard for the differential diagnosis of motor speech disorders. The goal of this study was to investigate the feasibility of applying a similar, formalized auditory-perceptual approach to the assessment of language deficits in connected speech samples from individuals with aphasia. Method Twenty-seven common features of connected speech in aphasia were defined, each of which was rated on a 5-point scale. Three experienced researchers evaluated 24 connected speech samples from the AphasiaBank database, and 12 student clinicians evaluated subsets of 8 speech samples each. We calculated interrater reliability for each group of raters and investigated the validity of the auditory-perceptual approach by comparing feature ratings to related quantitative measures derived from transcripts and clinical measures, and by examining patterns of feature co-occurrence. Results Most features were rated with good-to-excellent interrater reliability by researchers and student clinicians. Most features demonstrated strong concurrent validity with respect to quantitative connected speech measures computed from AphasiaBank transcripts and/or clinical aphasia battery subscores. Factor analysis showed that 4 underlying factors, which we labeled Paraphasia, Logopenia, Agrammatism, and Motor Speech, accounted for 79% of the variance in connected speech profiles. Examination of individual patients' factor scores revealed striking diversity among individuals classified with a given aphasia type. Conclusion Auditory-perceptual rating of connected speech in aphasia shows potential to be a comprehensive, efficient, reliable, and valid approach for characterizing connected speech in aphasia.

The purpose of this study was to test two competing hypotheses about the nature of the impairment in apraxia of speech (AOS). The Reduced Buffer Capacity Hypothesis argues that people with AOS can hold only one syllable at a time in the speech motor planning buffer. The Program Retrieval Deficit Hypothesis, states that people with AOS have difficulty accessing the intended motor program in the context where several motor programs are activated simultaneously. The participants included eight speakers with AOS, most of whom also had aphasia, nine speakers with aphasia without AOS, and 25 age-matched control speakers. The experimental paradigm prompted single word production following three types of primes. In most trials, prime and target were the same (e.g., bill-bill). On some trials, the initial consonant differed in one phonetic feature (e.g., bill-dill; Similar) or in all phonetic features (fill-bill; Different). The dependent measures were accuracy and reaction time. The results revealed a switch cost - longer reaction times in trials where the prime and target differed compared to trials where they were the same words - in all groups; however, the switch cost was significantly larger in the AOS group compared to the other two groups. These findings are in line with the prediction of the Program Retrieval Deficit Hypothesis and suggest that speakers with AOS have difficulty with selecting one program over another when several programs compete for selection., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Purpose Recovery from aphasia after stroke has a decelerating trajectory, with the greatest gains taking place early and the slope of change decreasing over time. Despite its importance, little is known regarding evolution of language function in the early postonset period. The goal of this study was to characterize the dynamics and nature of recovery of language function in the acute and early subacute phases of stroke. Method Twenty-one patients with aphasia were evaluated every 2-3 days for the first 15 days after onset of acute ischemic or hemorrhagic stroke. Language function was assessed at each time point with the Quick Aphasia Battery (Wilson, Eriksson, Schneck, & Lucanie, 2018), which yields an overall summary score and a multidimensional profile of 7 different language domains. Results On a 10-point scale, overall language function improved by a mean of 1.07 points per week, confidence interval [0.46, 1.71], with 19 of 21 patients showing positive changes. The trajectory of recovery was approximately linear over this time period. There was significant variability across patients, and patients with more impaired language function at Day 2 poststroke experienced greater improvements over the subsequent 2 weeks. Patterns of recovery differed across language domains, with consistent improvements in word finding, grammatical construction, repetition, and reading, but less consistent improvements in word comprehension and sentence comprehension. Conclusion Overall language function typically improves substantially and steadily during the first 2 weeks after stroke, driven mostly by recovery of expressive language. Information on the trajectory of early recovery will increase the accuracy of prognoses and establish baseline expectations against which to evaluate the efficacy of interventions. Supplemental Material https://doi.org/10.23641/asha.7811876.

Phonological alexia and agraphia are written language disorders characterised by disproportionate difficulty reading and spelling nonwords in comparison to real words. In phonological alexia, it has been shown that, despite relatively accurate reading of words in isolation, text-level reading deficits are often marked and persistent. Specifically, some individuals demonstrate difficulty reading functors and affixes in sentences, a profile referred to as phonological text alexia. In this paper, we demonstrate an analogous manifestation of the phonological impairment on text-level writing and suggest the term "phonological text agraphia". We examined four individuals with phonological alexia/agraphia who also showed disproportionate difficulty writing well-formed sentences in comparison to their grammatical competence in spoken utterances. Implementation of a phonological treatment protocol resulted in significantly improved sublexical phonology skills as well as improvements in grammatical accuracy of written narratives. These findings support the notion of a common phonological impairment underlying nonword reading/spelling deficits and sentence-level difficulties.

Phonological deficits are common in aphasia after left-hemisphere stroke, and can have significant functional consequences for spoken and written language. While many individuals improve through treatment, the neural substrates supporting improvements are poorly understood. We measured brain activation during pseudoword reading in an individual through two treatment phases. Improvements were associated with greater activation in residual left dorsal language regions and bilateral regions supporting attention and effort. Gains were maintained, while activation returned to pre-treatment levels. This case demonstrates the neural support for improved phonology after damage to critical regions and that improvements may be maintained without markedly increased effort.

We used fMRI to examine the neural substrates of sublexical phoneme-grapheme conversion during spelling in a group of healthy young adults. Participants performed a writing-to-dictation task involving irregular words (e.g., choir), plausible nonwords (e.g., kroid), and a control task of drawing familiar geometric shapes (e.g., squares). Written production of both irregular words and nonwords engaged a left-hemisphere perisylvian network associated with reading/spelling and phonological processing skills. Effects of lexicality, manifested by increased activation during nonword relative to irregular word spelling, were noted in anterior perisylvian regions (posterior inferior frontal gyrus/operculum/precentral gyrus/insula), and in left ventral occipito-temporal cortex. In addition to enhanced neural responses within domain-specific components of the language network, the increased cognitive demands associated with spelling nonwords engaged domain-general frontoparietal cortical networks involved in selective attention and executive control. These results elucidate the neural substrates of sublexical processing during written language production and complement lesion-deficit correlation studies of phonological agraphia., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Individuals with primary progressive aphasia (PPA) show selective breakdown in regions within the proposed dorsal (articulatory-phonological) and ventral (lexical-semantic) pathways involved in language processing. Phonological STM impairment, which has been attributed to selective damage to dorsal pathway structures, is considered to be a distinctive feature of the logopenic variant of PPA. By contrast, phonological abilities are considered to be relatively spared in the semantic variant and are largely unexplored in the nonfluent/agrammatic variant. Comprehensive assessment of phonological ability in the three variants of PPA has not been undertaken. We investigated phonological processing skills in a group of participants with PPA as well as healthy controls, with the goal of identifying whether patterns of performance support the dorsal versus ventral functional-anatomical framework and to discern whether phonological ability differs among PPA subtypes. We also explored the neural bases of phonological performance using voxel-based morphometry. Phonological performance was impaired in patients with damage to dorsal pathway structures (nonfluent/agrammatic and logopenic variants), with logopenic participants demonstrating particular difficulty on tasks involving nonwords. Binary logistic regression revealed that select phonological tasks predicted diagnostic group membership in the less fluent variants of PPA with a high degree of accuracy, particularly in conjunction with a motor speech measure. Brain-behavior correlations indicated a significant association between the integrity of gray matter in frontal and temporoparietal regions of the left hemisphere and phonological skill. Findings confirm the critical role of dorsal stream structures in phonological processing and demonstrate unique patterns of impaired phonological processing in logopenic and nonfluent/agrammatic variants of PPA.

Pure alexia (PA) arises from damage to the left posterior fusiform gyrus (pFG) and the striking reading disorder that defines this condition has meant that such patients are often cited as evidence for the specialisation of this region to processing of written words. There is, however, an alternative view that suggests this region is devoted to processing of high acuity foveal input, which is particularly salient for complex visual stimuli like letter strings. Previous reports have highlighted disrupted processing of non-linguistic visual stimuli after damage to the left pFG, both for familiar and unfamiliar objects and also for novel faces. This study explored the nature of face processing deficits in patients with left pFG damage. Identification of famous faces was found to be compromised in both expressive and receptive tasks. Discrimination of novel faces was also impaired, particularly for those that varied in terms of second-order spacing information, and this deficit was most apparent for the patients with the more severe reading deficits. Interestingly, discrimination of faces that varied in terms of feature identity was considerably better in these patients and it was performance in this condition that was related to the size of the length effects shown in reading. This finding complements functional imaging studies showing left pFG activation for faces varying only in spacing and frontal activation for faces varying only on features. These results suggest that the sequential part-based processing strategy that promotes the length effect in the reading of these patients also allows them to discriminate between faces on the basis of feature identity, but processing of second-order configural information is most compromised due to their left pFG lesion. This study supports a view in which the left pFG is specialised for processing of high acuity foveal visual information that supports processing of both words and faces., (Copyright © 2015 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Purpose: Damage to left ventral occipito-temporal cortex can give rise to written language impairment characterized by pure alexia/letter-by-letter (LBL) reading, as well as surface alexia and agraphia. The purpose of this study was to examine the therapeutic effects of a combined treatment approach to address concurrent LBL reading with surface alexia/agraphia., Method: Simultaneous treatment to address slow reading and errorful spelling was administered to 3 individuals with reading and spelling impairments after left ventral occipito-temporal damage due to posterior cerebral artery stroke. Single-word reading/spelling accuracy, reading latencies, and text reading were monitored as outcome measures for the combined effects of multiple oral re-reading treatment and interactive spelling treatment., Results: After treatment, participants demonstrated faster and more accurate single-word reading and improved text-reading rates. Spelling accuracy also improved, particularly for untrained irregular words, demonstrating generalization of the trained interactive spelling strategy., Conclusion: This case series characterizes concomitant LBL with surface alexia/agraphia and demonstrates a successful treatment approach to address both the reading and spelling impairment.

Although fMRI is increasingly used to assess language-related brain activation in patients with aphasia, few studies have examined the hemodynamic response function (HRF) in perilesional, and contralesional areas of the brain. In addition, the relationship between HRF abnormalities and other variables such as lesion size and severity of aphasia has not been explored. The objective of this study was to investigate changes in HRF signal during language-related neural activation in patients with stroke-induced aphasia (SA). We also examined the status of the HRF in patients with aphasia due to nonvascular etiology, namely, primary progressive aphasia (PPA). Five right handed SA patients, three PPA patients, and five healthy individuals participated in the study. Structural damage was quantified with T1-weighted MR images. Functional MR imaging was performed with long trial event-related design and an overt naming task to measure BOLD signal time to peak (TTP) and percent signal change (ΔS). In SA patients, the average HRF TTP was significantly delayed in the left hemisphere regions involved in naming compared to healthy participants and PPA patients. However, ΔS was not different in SA patients compared to the other two groups. Delay in HRF TTP in the left hemisphere naming network of SA patients was correlated with lesion size and showed a negative correlation with global language function. There were no significant differences in the HRF TTP and ΔS in the right hemisphere homologues of the naming network or in the left and the right occipital control regions across the three groups. In PPA patients, HRF had a normal pattern. Our results indicate that abnormal task-related HRF is primarily found in the left hemisphere language network of SA patients and raise the possibility that abnormal physiology superimposed on structural damage may contribute to the clinical deficit. Follow-up investigations in a larger sample of age-matched healthy individuals, SA, and PPA patients will be needed to further confirm and extend our findings.

This paper introduces a Bidirectional Iterative Parcellation (BIP) procedure designed to identify the location and size of connected cortical regions (parcellations) at both ends of a white matter tract in diffusion weighted images. The procedure applies the FSL option "probabilistic tracking with classification targets" in a bidirectional and iterative manner. To assess the utility of BIP, we applied the procedure to the problem of parcellating a limited set of well-established gray matter seed regions associated with the dorsal (arcuate fasciculus/superior longitudinal fasciculus) and ventral (extreme capsule fiber system) white matter tracts in the language networks of 97 participants. These left hemisphere seed regions and the two white matter tracts, along with their right hemisphere homologues, provided an excellent test case for BIP because the resulting parcellations overlap and their connectivity via the arcuate fasciculi and extreme capsule fiber systems are well studied. The procedure yielded both confirmatory and novel findings. Specifically, BIP confirmed that each tract connects within the seed regions in unique, but expected ways. Novel findings included increasingly left-lateralized parcellations associated with the arcuate fasciculus/superior longitudinal fasciculus as a function of age and education. These results demonstrate that BIP is an easily implemented technique that successfully confirmed cortical connectivity patterns predicted in the literature, and has the potential to provide new insights regarding the architecture of the brain., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Recent visual neuroscience investigations suggest that ventral occipito-temporal cortex is retinotopically organized, with high acuity foveal input projecting primarily to the posterior fusiform gyrus (pFG), making this region crucial for coding high spatial frequency information. Because high spatial frequencies are critical for fine-grained visual discrimination, we hypothesized that damage to the left pFG should have an adverse effect not only on efficient reading, as observed in pure alexia, but also on the processing of complex non-orthographic visual stimuli. Consistent with this hypothesis, we obtained evidence that a large case series (n = 20) of patients with lesions centered on left pFG: 1) Exhibited reduced sensitivity to high spatial frequencies; 2) demonstrated prolonged response latencies both in reading (pure alexia) and object naming; and 3) were especially sensitive to visual complexity and similarity when discriminating between novel visual patterns. These results suggest that the patients' dual reading and non-orthographic recognition impairments have a common underlying mechanism and reflect the loss of high spatial frequency visual information normally coded in the left pFG.

Purpose: Treatment studies have documented the therapeutic and functional value of lexical writing treatment for individuals with severe aphasia. The purpose of this study was to determine whether such retraining could be accomplished using the typing feature of a cellular telephone, with the ultimate goal of using text messaging for communication., Method: A 31-year-old man with persistent Broca's aphasia, severe apraxia of speech, global dysgraphia, and right hemiparesis participated in this study. Using a multiple baseline design, relearning and maintenance of single-word spellings (and oral naming) of targeted items were examined in response to traditional Copy and Recall Treatment (CART) for handwriting and a new paradigm using 1-handed typing on a cell phone keyboard (i.e., a texting version of CART referred to as T-CART)., Results: Marked improvements were documented in spelling and spoken naming trained in either modality, with stronger maintenance for handwriting than cell phone typing. Training resulted in functional use of texting that continued for 2 years after treatment., Conclusions: These results suggest that orthographic retraining using a cell phone keyboard has the potential to improve spelling knowledge and provide a means to improve functional communication skills. Combined training with both handwriting and cell phone typing should be considered in order to maximize the durability of treatment effects.

Functional magnetic resonance imaging is the most widely used imaging technique to study treatment-induced recovery in post-stroke aphasia. The longitudinal design of such studies adds to the challenges researchers face when studying patient populations with brain damage in cross-sectional settings. The present review focuses on issues specifically relevant to neuroimaging data analysis in aphasia treatment research identified in discussions among international researchers at the Neuroimaging in Aphasia Treatment Research Workshop held at Northwestern University (Evanston, Illinois, USA). In particular, we aim to provide the reader with a critical review of unique problems related to the pre-processing, statistical modeling and interpretation of such data sets. Despite the fact that data analysis procedures critically depend on specific design features of a given study, we aim to discuss and communicate a basic set of practical guidelines that should be applicable to a wide range of studies and useful as a reference for researchers pursuing this line of research., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Progressive alexia is an acquired reading deficit caused by degeneration of brain regions that are essential for written word processing. Functional imaging studies have shown that early processing of the visual word form depends on a hierarchical posterior-to-anterior processing stream in occipito-temporal cortex, whereby successive areas code increasingly larger and more complex perceptual attributes of the letter string. A region located in the left lateral occipito-temporal sulcus and adjacent fusiform gyrus shows maximal selectivity for words and has been dubbed the 'visual word form area'. We studied two patients with progressive alexia in order to determine whether their reading deficits were associated with structural and/or functional abnormalities in this visual word form system. Voxel-based morphometry showed left-lateralized occipito-temporal atrophy in both patients, very mild in one, but moderate to severe in the other. The two patients, along with 10 control subjects, were scanned with functional magnetic resonance imaging as they viewed rapidly presented words, false font strings, or a fixation crosshair. This paradigm was optimized to reliably map brain regions involved in orthographic processing in individual subjects. All 10 control subjects showed a posterior-to-anterior gradient of selectivity for words, and all 10 showed a functionally defined visual word form area in the left hemisphere that was activated for words relative to false font strings. In contrast, neither of the two patients with progressive alexia showed any evidence for a selectivity gradient or for word-specific activation of the visual word form area. The patient with mild atrophy showed normal responses to both words and false font strings in the posterior part of the visual word form system, but a failure to develop selectivity for words in the more anterior part of the system. In contrast, the patient with moderate to severe atrophy showed minimal activation of any part of the visual word form system for either words or false font strings. Our results suggest that progressive alexia is associated with a dysfunctional visual word form system, with or without substantial cortical atrophy. Furthermore, these findings demonstrate that functional MRI has the potential to reveal the neural bases of cognitive deficits in neurodegenerative patients at very early stages, in some cases before the development of extensive atrophy.

Connectionist theories of language propose that written language deficits arise as a result of damage to semantic and phonological systems that also support spoken language production and comprehension, a view referred to as the "primary systems" hypothesis. The objective of the current study was to evaluate the primary systems account in a mixed group of individuals with primary progressive aphasia (PPA) by investigating the relation between measures of nonorthographic semantic and phonological processing and written language performance and by examining whether common patterns of cortical atrophy underlie impairments in spoken versus written language domains. Individuals with PPA and healthy controls were administered a language battery, including assessments of semantics, phonology, reading, and spelling. Voxel-based morphometry was used to examine the relation between gray matter volumes and language measures within brain regions previously implicated in semantic and phonological processing. In accordance with the primary systems account, our findings indicate that spoken language performance is strongly predictive of reading/spelling profile in individuals with PPA and suggest that common networks of critical left hemisphere regions support central semantic and phonological processes recruited for spoken and written language.

Despite considerable recent progress in understanding the underlying neurobiology of primary progressive aphasia (PPA) syndromes, relatively little attention has been directed toward the examination of behavioral interventions that may lessen the pervasive communication problems associated with PPA. In this study, we report on an individual with a behavioral profile and cortical atrophy pattern consistent with the logopenic variant of PPA. At roughly two-and-a-half years post onset, his marked lexical retrieval impairment prompted administration of a semantically based intervention to improve word retrieval. The treatment was designed to improve self-directed efforts to engage the participant's relatively preserved semantic system in order to facilitate word retrieval. His positive response to an intensive (2-week) dose of behavioral treatment was associated with improved lexical retrieval of items within trained categories, and generalized improvement for naming of untrained items that lasted over a 6-month follow-up interval. These findings support the potential value of intensive training to achieve self-directed strategic compensation for lexical retrieval difficulties in logopenic PPA. Additional insight was gained regarding the neural regions that supported improved performance by the administration of a functional magnetic resonance imaging protocol before and after treatment. In the context of a picture-naming task, post-treatment fMRI showed increased activation of left dorsolateral prefrontal regions that have been implicated in functional imaging studies of generative naming in healthy individuals. The increased activation in these frontal regions that were not significantly atrophic in our patient (as determined by voxel-based morphometry) is consistent with the notion that neural plasticity can support compensation for specific language loss, even in the context of progressive neuronal degeneration.

Letter-by-letter (LBL) reading is the phenomenon whereby individuals with acquired alexia decode words by sequential identification of component letters. In cases where letter recognition or letter naming is impaired, however, a LBL reading approach is obviated, resulting in a nearly complete inability to read, or global alexia. In some such cases, a treatment strategy wherein letter tracing is used to provide tactile and/or kinesthetic input has resulted in improved letter identification. In this study, a kinesthetic treatment approach was implemented with an individual who presented with severe alexia in the context of relatively preserved recognition of orally spelled words, and mildly impaired oral/written spelling. Eight weeks of kinesthetic treatment resulted in improved letter identification accuracy and oral reading of trained words; however, the participant remained unable to successfully decode untrained words. Further testing revealed that, in addition to the visual-verbal disconnection that resulted in impaired word reading and letter naming, her limited ability to derive benefit from the kinesthetic strategy was attributable to a disconnection that prevented access to letter names from kinesthetic input. We propose that this kinesthetic-verbal disconnection resulted from damage to the left parietal lobe and underlying white matter, a neuroanatomical feature that is not typically observed in patients with global alexia or classic LBL reading. This unfortunate combination of visual-verbal and kinesthetic-verbal disconnections demonstrated in this individual resulted in a persistent multimodal alexia syndrome that was resistant to behavioral treatment. To our knowledge, this is the first case in which the nature of this form of multimodal alexia has been fully characterized, and our findings provide guidance regarding the requisite cognitive skills and lesion profiles that are likely to be associated with a positive response to tactile/kinesthetic treatment., (Copyright © 2011 Elsevier Ltd. All rights reserved.)

Although normalization of brain images is critical to the analysis of structural damage across individuals, loss of tissue due to focal lesions presents challenges to the available normalization algorithms. Until recently, cost function masking, as advocated by Brett and colleagues (2001), was the accepted method to overcome difficulties encountered when normalizing damaged brains; however, development of the unified segmentation approach for normalization in SPM5 (Ashburner & Friston, 2005) offered an alternative. Crinion et al. (2007) demonstrated this approach produced normalization results without cost function masking that appeared to be robust to lesion effects when tested using the same simulated lesions studied by Brett et al. (2001). The present study sought to confirm the validity of this approach in brains with focal damage due to vascular events. To do so, we examined outcomes of normalization using unified segmentation with and without cost function masking in 49 brain images with chronic stroke. Lesion masks were created using two approaches (precise and rough drawings of lesion boundaries), and normalization was implemented with both smoothed and unsmoothed versions of the masks. We found that failure to employ cost function masking produced less accurate results in real and simulated lesions, compared to masked normalization, both in terms of deformation field displacement and voxelwise intensity differences. Additionally, unmasked normalization led to significant underestimation of lesion volume relative to all four masking conditions, especially in patients with large lesions. Taken together, these findings suggest cost function masking is still necessary when normalizing brain images with chronic infarcts., (Copyright 2010 Elsevier Inc. All rights reserved.)

Purpose: Damage to left perisylvian cortex often results in impaired phonological processing abilities with written language profiles consistent with phonological alexia and phonological agraphia. The purpose of this article was to examine a behavioral treatment sequence for such individuals intended to strengthen phonological processing and links between phonology and orthography, as well as train a means to maximize use of residual orthographic and phonological knowledge for spelling., Method: Two women with persistent impairments of written language and phonological processing following damage to left perisylvian cortical regions participated in this study. Both exhibited characteristic features of phonological alexia and agraphia in that reading and spelling performance for real words was better preserved than nonwords (lexicality effect). A 2-stage treatment protocol was administered to strengthen sublexical skills (phonological treatment) and to train interactive use of lexical and sublexical information to maximize spelling performance (interactive treatment)., Results: Both participants improved phonological processing abilities and reading/spelling via the sublexical route. They also improved spelling of real words and were able to detect and correct most residual errors using an electronic spelling aid., Conclusions: Behavioral treatment served to strengthen phonological skills supporting reading and spelling, and provided a functional compensatory strategy to overcome residual weaknesses.

To examine the validity of different theoretical assumptions about the neuropsychological mechanisms and lesion correlates of phonological dyslexia and dysgraphia, we studied written and spoken language performance in a large cohort of patients with focal damage to perisylvian cortical regions implicated in phonological processing. Despite considerable variation in accuracy for both words and non-words, the majority of participants demonstrated the increased lexicality effects in reading and spelling that are considered the hallmark features of phonological dyslexia and dysgraphia. Increased lexicality effects were also documented in spoken language tasks such as oral repetition, and patients performed poorly on a battery of phonological tests that did not involve an orthographic component. Furthermore, a composite measure of general phonological ability was strongly predictive of both reading and spelling accuracy, and we obtained evidence that the continuum of severity that characterized the written language disorder of our patients was attributable to an underlying continuum of phonological impairment. Although patients demonstrated qualitatively similar deficits across measures of written and spoken language processing, there were quantitative differences in levels of performance reflecting task difficulty effects. Spelling was more severely affected than reading by the reduction in phonological capacity and this differential vulnerability accounted for occasional disparities between patterns of impairment on the two written language tasks. Our findings suggest that phonological dyslexia and dysgraphia in patients with perisylvian lesions are manifestations of a central or modality-independent phonological deficit rather than the result of damage to cognitive components dedicated to reading or spelling. Our results also provide empirical support for shared-components models of written language processing, according to which the same central cognitive systems support both reading and spelling. Lesion-deficit correlations indicated that phonological dyslexia and dysgraphia may be produced by damage to a variety of perisylvian cortical regions, consistent with distributed network models of phonological processing.

Anomia is a striking and consistent clinical feature of semantic dementia (SD), a progressive aphasia syndrome associated with focal cortical atrophy of the anterior temporal lobes. Word retrieval deficits in patients with SD have been attributed to the loss of conceptual knowledge, resulting in an impairment referred to as semantic anomia. Whereas an abundance of research has been dedicated to treatment for anomia in individuals with focal brain damage due to stroke, considerably less work has been done regarding treatment for patients with progressive language decline. The purpose of this article is to review the available literature concerning the nature and treatment of anomia in individuals with SD. Several studies have shown that new lexical learning remains possible in these patients. However, newly learned information is likely to be constrained by the learning context, and increased reliance on perceptual and autobiographical contextual information may be necessary to provide critical support for new vocabulary acquisition. There is also evidence suggesting that treatment may slow the progression of anomia over time, even affording some protective benefit to lexical items that are not yet lost. However, treatment efforts are likely to be most beneficial at early stages of the disease, when residual semantic knowledge as well as relatively spared episodic memory may support new learning.

Coltheart and co-workers [Castles, A., Bates, T. C., & Coltheart, M. (2006). John Marshall and the developmental dyslexias. Aphasiology, 20, 871-892; Coltheart, M., Rastle, K., Perry, C., Langdon, R., & Ziegler, J. (2001). DRC: A dual route cascaded model of visual word recognition and reading aloud. Psychological Review, 108, 204-256] have demonstrated that an equation derived from dual-route theory accurately predicts reading performance in young normal readers and in children with reading impairment due to developmental dyslexia or stroke. In this paper, we present evidence that the dual-route equation and a related multiple regression model also accurately predict both reading and spelling performance in adult neurological patients with acquired alexia and agraphia. These findings provide empirical support for dual-route theories of written language processing.

In order to examine the role of left perisylvian cortex in spelling, 13 individuals with lesions in this area were administered a comprehensive spelling battery. Their spelling of regular words, irregular words, and nonwords was compared with that of individuals with extrasylvian damage involving left inferior temporo-occipital cortex and normal controls. Perisylvian patients demonstrated a lexicality effect, with nonwords spelled worse than real words. This pattern contrasts with the deficit in irregular word spelling, or regularity effect, observed in extrasylvian patients. These findings confirm that damage to left perisylvian cortex results in impaired phonological processing required for sublexical spelling. Further, degraded phonological input to orthographic selection typically results in additional deficits in real word spelling.

The mandate for evidence-based practice has prompted careful consideration of the weight of the scientific evidence regarding the therapeutic value of various clinical treatments. In the field of aphasia, a large number of single-subject research studies have been conducted, providing clinical outcome data that are potentially useful for clinicians and researchers; however, it has been difficult to discern the relative potency of these treatments in a standardized manner. In this paper we describe an approach to quantify treatment outcomes for single-subject research studies using effect sizes. These values provide a means to compare treatment outcomes within and between individuals, as well as to compare the relative strength of various treatments. Effect sizes also can be aggregated in order to conduct meta-analyses of specific treatment approaches. Consideration is given to optimizing research designs and providing adequate data so that the value of treatment research is maximized.

Individuals with left-hemisphere damage often have concomitant impairment of spoken and written language. Whereas some treatment studies have shown that reading paired with spoken naming can benefit both language modalities, little systematic research has been directed toward the treatment of spelling combined with spoken naming. The purpose of this study was to examine the therapeutic effect of pairing a lexical spelling treatment referred to as Copy and Recall Treatment (CART) with verbal repetition of target words. This approach (CART + Repetition) was compared with treatment using verbal repetition without the inclusion of orthographic training (Repetition Only). Two individuals with moderate aphasia and severe impairment of spelling participated in the study using a multiple baseline design across stimulus sets and treatment conditions. Both participants improved spelling of targeted words as well as spoken naming of those items, but improvement in spoken naming was marked for one individual in the CART + Repetition condition, while the other participant made smaller gains in spoken than written naming irrespective of treatment condition. Consideration of the participant profiles suggested that CART + Repetition provides greater benefit when there is some residual phonological ability and the treatment serves to stimulate links between orthography and phonology.

The present investigation provides a longitudinal study of an individual (RB) with acquired alexia following left posterior cerebral artery stroke. At initial testing, RB exhibited acquired alexia characterized by letter-by-letter (LBL) reading, mild anomic aphasia, and acquired agraphia. Repeated measures of reading accuracy and rate were collected for single words and text over the course of one year, along with probes of naming and spelling abilities. Improvements associated with natural recovery (i.e., without treatment) were documented up to the fourth month post onset, when text reading appeared to be relatively stable. Multiple oral reading (MOR) treatment was initiated at 22 weeks post-stroke, and additional improvements in reading rate and accuracy for text were documented that were greater than those expected on the basis of spontaneous recovery alone. Over the course of one year, reading reaction times for single words improved, and the word-length effect that is the hallmark of LBL reading diminished. RB's response to treatment supports the therapeutic value of MOR treatment to in LBL readers. His residual impairment of reading and spelling one-year post stroke raised the question as to whether further progress was impeded by degraded orthographic knowledge.

Written communication involves the linking of ideas and words to their appropriate spellings, and then moving the hand to actually write the desired words. Acquired impairments of writing are referred to as agraphia and can result from damage to various stages of the writing process. These impairments may reflect degraded knowledge about spelling and the correspondences between sounds and letters or difficulty with the selection and formation of letters. Characteristic features of agraphia syndromes are reviewed in this article, along with example approaches to remediation. The treatments variously serve to strengthen weakened abilities, take advantage of residual skills, and develop compensatory strategies.

Objective: To determine whether damage to left posterior inferior temporal cortex (PITC) is associated with agraphia and to characterize the nature of the spelling impairment., Background: Left angular gyrus may play a critical role in spelling. However, this traditional view is challenged by reports of agraphia after left temporo-occipital lesions and by functional imaging studies demonstrating activation of left PITC during writing in normal individuals., Methods: Patients with focal damage to the left temporo-occipital cortex and normal control subjects were administered a comprehensive spelling battery that included regular words, irregular words, and nonwords as stimuli., Results: Although patients performed worse than control subjects in all experimental conditions, the spelling deficit was particularly severe for irregular words, whereas regular word and nonword spelling were less impaired. Additional analyses indicated that orthographic regularity and word frequency had a much more pronounced effect on spelling accuracy in patients compared with control subjects. Most errors on irregular words were phonologically plausible, consistent with reliance on a sublexical phonologic spelling strategy (i.e., phoneme-grapheme conversion). Overall, the spelling impairment of the patients showed the characteristic profile of lexical agraphia. Lesion analyses indicated that the damage in the majority of patients encompassed an area within the left PITC (BA 37/20) where the authors previously obtained evidence of activation in a functional imaging study of writing in normal participants., Conclusions: The behavioral and neuroanatomic observations in the patients are consistent with functional imaging studies of writing in neurologically intact individuals and provide converging evidence for the role of left PITC in spelling. Together, these findings implicate left PITC as a possible neural substrate of the putative orthographic lexicon that contains stored memory representations for the written forms of familiar words.

Writing treatment that involved repeated copying and recall of target words was implemented with 8 individuals with severe aphasia in order to discern the best candidates for the treatment. Four of the 8 participants had strong positive responses to the copy and recall treatment (CART), relearning spellings for 15 targeted words during 10 to 12 weeks of treatment and up to 5 additional words during a month-long homework-based program. Of the 4 other participants, 3 learned the spellings of some target words but failed to reach criterion, and 1 had a poor treatment outcome. Insights regarding possible factors that limited success were gained by examination of individual responses to treatment as well as performance on the pretreatment assessments of semantic, phonological, and orthographic processes. Among the factors associated with success were (a) consistent, accurate completion of daily homework, (b) a relatively preserved semantic system, (c) the ability to discern words from nonwords, and (d) adequately preserved nonverbal visual problem-solving skills. Aphasia severity and minimal pretreatment spelling abilities did not necessarily limit the response to treatment. Participants with positive treatment outcomes demonstrated improved spelling of target words following repeated copying within a single treatment session, and accurately completed daily writing homework. Thus, pretreatment assessment and stimulability within initial treatment sessions provided indications of likely outcome.

Two individuals with anomic aphasia and acquired alexia were each provided treatment for their reading impairment. Although reading of single words in isolation was fairly accurate, their text reading was slow and effortful, including functor substitutions and semantic errors. Prior to treatment, reading reaction times for single words showed grammatical class and word-length effects. Both patients responded positively to a treatment protocol that included two phases: (1) multiple oral rereading of text, and (2) reading phrase-formatted text that had increased spacing between phrasal clauses. Their reading rates for text improved while maintaining good comprehension. Following treatment, reading reaction times for single words showed the elimination of grammatical class and word-length effects, suggesting improved access to word forms, particularly functors.

This study examined the effects of lesion location (frontal vs. posterior) and nature of distraction (nonverbal vs. verbal secondary, competing task) on mildly aphasic individuals' performances of listening tasks that required semantic judgments and lexical decisions under isolation, focused attention, and divided attention conditions. Despite comparable accuracy among all groups during isolation conditions, the aphasic groups responded less accurately and more slowly than the normal control group during focused and divided attention conditions. Generally, the two aphasic groups performed similarly, quantitatively and qualitatively. Demographic characteristics such as time post stroke did not correlate with performance decrements. Independent of group, all individuals showed greater disruption of auditory processing skills when the secondary task was verbal rather than nonverbal. Within a limited-capacity model of attention, the results suggest that aphasic individuals display impairments of attention and resource allocation and that these impairments negatively interact with their auditory processing abilities.