Pseudoword spelling ability predicts response to word spelling treatment in acquired dysgraphia.

Although rehabilitation of acquired dysgraphia can be quite effective, identifying predictors of responsiveness to treatment is useful for prognosis and individualization of treatment protocols. This study examined whether various features of treatment response were predicted by the integrity of one or more of the central cognitive components of spelling: orthographic long-term memory, orthographic working memory, and phoneme-grapheme conversion. Twenty dysgraphic individuals received 12 weeks of bi-weekly, individualized, lexically-based spelling rehabilitation using a spell-study-spell paradigm. Linear multiple regression modelling examined whether the type and severity of the dysgraphic deficit, assessed before rehabilitation, predicted the magnitude and rate of improvement, generalization to untrained items and maintenance of treatment gains. The results revealed that pseudoword spelling accuracy - indexing the integrity of the phoneme-grapheme conversion system - was the only factor examined that significantly predicted the rate of accuracy gains for trained words as well as the extent of generalization to untrained words. Pre-treatment pseudoword spelling accuracy also predicted retention of gains for trained and untrained words at 3-month follow-up. These findings reveal that the integrity of the phoneme-grapheme conversion system prior to dysgraphia rehabilitation may play a key role in rehabilitation-driven recovery, even when the treatment approach targets lexical rather than pseudoword spelling processes.

Handwriting in Alzheimer's Disease.

BACKGROUND: Agraphia is a typical feature in the clinical course of Alzheimer's disease (AD). OBJECTIVE: Assess the differences between AD and normal aging as regards kinematographic features of handwriting and elucidate writing deficits in AD. METHODS: The study included 23 patients with AD (78.09 years/SD = 7.12; MMSE 21.39/SD = 3.61) and 34 healthy controls (75.56 years/SD = 5.85; MMSE 29.06/SD = 0.78). Both groups performed alphabetical and non-alphabetical writing tasks. The kinematographic assessment included the average number of inversions per stroke (NIV; number of peaks in the velocity profile in a single up or down stroke), percentage of automated segments, frequency (average number of strokes per second), writing pressure, and writing velocity on paper. RESULTS: A total of 14 patients showed overt writing difficulties reflected by omissions or substitutions of letters. AD patients showed less automated movements (as measured by NIV), lower writing velocity, and lower frequency of up-and-down strokes in non-alphabetical as well as in alphabetical writing. In the patient group, Spearman correlation analysis between overt writing performance and NIV was significant. That means patients who had less errors in writing a sentence showed a higher automaticity in handwriting. The correctness of alphabetical writing and some kinematographic measures in writing non-alphabetical material reached excellent diagnostic values in ROC analyses. There was no difference in the application of pressure on the pen between patients and controls. CONCLUSION: Writing disorders are multi-componential in AD and not strictly limited to one processing level. The slow and poorly automated execution of motor programs is not bound to alphabetical material.

Serial position effects in graphemic buffer impairment: An insight into components of orthographic working memory.

This study investigated the nature of graphemic buffer functioning and impairment, through analysis of the spelling impairment shown by GEC, a man with acquired dysgraphia and clear characteristics of graphemic buffer impairment. We discuss GEC's error patterns in relation to different processes of orthographic working memory. This is the first study to show the contribution of these processes in one individual through performance on different spelling tasks. GEC's spelling errors in writing to dictation showed a linear serial position effect, including deletions of final letters. These "fragment errors" can be explained as the result of information rapidly decaying from the buffer (reduced temporal stability). However, in tasks that reduced working memory demands, GEC showed a different error distribution that may indicate impairment to a different buffer process (reduced representational distinctiveness). We argue that different error patterns can be a reflection of subcomponents of orthographic working memory that can be impaired separately.

Dystypia Associated with Diaschisis of the Middle Frontal Gyri after Left Angular Infarction.

Dystypia without aphasia, agraphia, or apraxia is a rare symptom and has been suggested to result from a lesion in the left middle frontal cortex. We herein describe a man with dystypia with agraphia due to infarction of the left angular gyrus. His dystypia seemed to result from the convergence failure of the kana into the alphabetical spellings. During dystypia, hypoperfusion of the bilateral middle frontal cortices was discovered. However, after his symptoms improved, blood flow in the middle frontal cortices returned to normal. This case suggests that the middle frontal cortex is downstream of the angular gyrus in the dictating pathway and a lesion in the left middle frontal cortex could cause pure dystypia.

Evolution of Writing Impairment in Spanish Patients with Alzheimer's Disease.

BACKGROUND: Although some studies suggest that writing difficulties may be one of the early symptoms of Alzheimer's disease (AD), they have been scarcely studied compared to oral language. Particularly noteworthy is the paucity of longitudinal studies that enable the observation of writing impairment as cognitive decline progresses. OBJECTIVE: The aim of this study was to examine the characteristics of writing in patients with AD and to monitor the deterioration of their performance over a follow-up period. METHODS: Sixty-four participants (half with AD and half healthy elderly) were compared in a word and pseudo-word dictation task. Patients were evaluated every 6 months over a 2.5 year follow-up period. RESULTS: The evolution of patient performance and error profile shows a typical pattern of deterioration, with early damage to the lexical pathway, which later extends to the phonological pathway and eventually affects peripheral processes. CONCLUSION: These results confirm the presence of writing difficulties from the early stages of AD, supporting the value of this task for early diagnosis. Furthermore, it allows us to explain the contradictory data obtained in previous investigations.

A Rare Clinical Antity; Pure Gerstmann Syndrome.

Gerstmann syndrome is defined as a tetrad including agraphia, acalculia, finger agnosia, and right-left disorientation. In the case studies presented in the literature, it has been reported that Gerstmann syndrome usually appears as an incomplete tetrad of symptoms or accompanied by cognitive deficits including aphasia, alexia, apraxia and some perceptual disorders. Here, we present of the patient with left angular and supramarginal gyrus infarction affecting the parietal lobe. In addition to the symptoms mentioned above, the patient had alexia and anomic aphasia as well. We discussed the clinic appearance and reviewed the current literature.

Acquisition of handwriting in children with and without dysgraphia: A computational approach.

Handwriting is a complex skill to acquire and it requires years of training to be mastered. Children presenting dysgraphia exhibit difficulties automatizing their handwriting. This can bring anxiety and can negatively impact education. 280 children were recruited in schools and specialized clinics to perform the Concise Evaluation Scale for Children's Handwriting (BHK) on digital tablets. Within this dataset, we identified children with dysgraphia. Twelve digital features describing handwriting through different aspects (static, kinematic, pressure and tilt) were extracted and used to create linear models to investigate handwriting acquisition throughout education. K-means clustering was performed to define a new classification of dysgraphia. Linear models show that three features only (two kinematic and one static) showed a significant association to predict change of handwriting quality in control children. Most kinematic and statics features interacted with age. Results suggest that children with dysgraphia do not simply differ from ones without dysgraphia by quantitative differences on the BHK scale but present a different development in terms of static, kinematic, pressure and tilt features. The K-means clustering yielded 3 clusters (Ci). Children in C1 presented mild dysgraphia usually not detected in schools whereas children in C2 and C3 exhibited severe dysgraphia. Notably, C2 contained individuals displaying abnormalities in term of kinematics and pressure whilst C3 regrouped children showing mainly tilt problems. The current results open new opportunities for automatic detection of children with dysgraphia in classroom. We also believe that the training of pressure and tilt may open new therapeutic opportunities through serious games.

Progressive aphasia, apraxia of speech and agraphia in corticobasal degeneration: A 12-case series clinical and neuropsychological descriptive study.

BACKGROUND: Despite initial underreporting of language dysfunctions in corticobasal syndrome (CBS), aphasia is now recognized as a frequent feature of this disease. Aphasia in CBS seems clinically overlying to a non-fluent/agrammatic primary progressive aphasia (nfaPPA), which is also a clinical phenotype associated with corticobasal degeneration (CBD) pathology. However, the clinical features of aphasia in CBS still remain poorly delineated, resulting in misjudgements in the differential diagnosis from a PPA presentation of the disease. AIMS: To investigate the language disorders of this syndrome, also through a systematic examination of recoding skills (reading, written spelling and repetition) and articulatory disturbances, which have been rarely examined in previous studies. METHODS & PROCEDURES: We present a clinical and neuropsychological descriptive study of the language impairments in a case series of 12 aphasic patients with a clinical diagnosis of CBS. Language assessment was conducted by means of the Esame NeuroPsicologico dell'Afasia, a comprehensive Italian battery for language functions, the Token Test, and the Apraxia of Speech Rating Scale. OUTCOMES & RESULTS: The language profile of the patients showed a severe expressive language disorder, characterized by non-fluent speech, apraxia of speech (AoS) with predominant stuttering-like dysfluencies, spatial/apraxic agraphia, lack of word-finding and defective sentence repetition. Severe limb apraxia, visual-spatial deficit and alien hand syndrome were also present. Neuroimaging showed bilateral left asymmetric atrophies and hypometabolism in the frontal premotor, parietal posterior and temporal areas. CONCLUSIONS & IMPLICATIONS: These findings suggest that aphasia in CBS might present as a 'mixed PPA', instead of an nfaPPA as previously stated, showing a combination of features of the nfa and logopenic variants of the PPA, associated with AoS, stuttering and agraphia, which might be additional important cognitive markers for the clinical diagnosis of CBS and discriminating features of an nfaPPA presentation of a CBD. These results might also suggest specific intervention areas in the rehabilitation of patients with CBS. What this paper adds What is already known on the subject Language disorders in CBS patients usually present clinically overlying to an nfaPPA, which is also a clinical phenotype associated with CBD pathology, according to recent diagnostic criteria. However, the clinical features of aphasia in CBS still remain poorly delineated, and this raises difficulties and misjudgements for clinicians in the differential diagnosis from a PPA presentation of the disease. What this paper adds to existing knowledge This study shows that the language profile of our CBS patients was characterized by severe expressive language disorders, with non-fluent speech, apraxia of speech (AoS) with predominant stuttering-like dysfluencies, spatial/apraxic agraphia, lack of word-finding, and defective sentence repetition. These findings suggest that aphasia in CBS might present as a 'mixed PPA', rather than an nfaPPA as previously stated, showing a combination of features of the nfa and logopenic variants of the PPA associated with AoS, stuttering and agraphia. What are the potential or actual clinical implications of this work? These results suggest that AoS, stuttering and agraphia might be important additional cognitive markers for the clinical diagnosis of CBS, and discriminating features of an nfaPPA presentation of a CBD. The language disorders exhibited in the present study might also support speech and language therapists in targeting specific intervention areas in the rehabilitation of patients with CBS.

Does surface dyslexia/dysgraphia relate to semantic deficits in the semantic variant of primary progressive aphasia?

The semantic variant of primary progressive aphasia (sv-PPA) is a degenerative condition which causes surface dyslexia/dysgraphia, resulting in reading/writing errors of irregular words with non-transparent grapheme-to-phoneme correspondences (e.g., 'plaid') as opposed to regular words (e.g., 'cat'). According to connectionist models, most authors have attributed this deficit to semantic impairments, but this assumption is at odds with symbolic models, such as the DRC account, stating that the reading/writing of irregulars relies on the mental lexicon. Our study investigated whether sv-PPA affects the lexicon in addition to the semantic system, and whether semantic or lexical deficits cause surface dyslexia/dysgraphia, while challenging the two major models of written language. We explored a cohort of 12 sv-PPA patients and 25 matched healthy controls using a reading and writing task, a semantic task (category decision: living vs. non-living), and a lexical task (lexical decision: word vs. no-neighbor non-word). Correlation analyses were conducted to assess the relationship between reading/writing scores of irregulars and semantic vs. lexical performance. Furthermore, item-by-item analyses explored the consistency of reading/writing errors with item-specific semantic and lexical errors. Results showed that sv-PPA patients are impaired at reading and writing irregular words, and that they have impaired performance in both the semantic and the lexical task. Reading/writing scores with irregulars correlated significantly with performance in the lexical but not the semantic task. Item-by-item analyses revealed that failure in the lexical task on a given irregular word is a good predictor of reading/writing errors with that item (positive predictive value: 77.5%), which was not the case for the semantic task (positive predictive value: 42.5%). Our findings show that sv-PPA is not restricted to semantic damage but that it also comprises damage to the mental lexicon, which appears to be the major factor for surface dyslexia/dysgraphia. Our data support symbolic models whereas they challenge connectionist accounts.

Motor functions of higher education students with dysgraphia.

This study examines the motor skills and motor-related daily functions of higher education students with and without dysgraphia, and their contribution in predicting handwriting performance. The sample included 82 higher education students aged 20-35 years old. Thirty-four were students without any known developmental disorder (NDD) and 48 students had dysgraphia. We individually administered a test battery evaluating handwriting performance, fine-motor skills, and visual-motor spatial-organization skills. Students also filled out a questionnaire relating to their fine- and gross-motor-related daily functions. Overall, the NDD students had significantly better motor skills and motor-related daily functions. Additionally, the motor skills and daily functions explained 62.9% of the variance in handwriting performance, and they correctly classified 90% of the students into the handwriting performance groups. Yet only visual-motor spatial organization and fine-motor-related daily functions significantly contributed to the fit of the model. These findings suggest that students with dysgraphia continue to encounter handwriting difficulties in higher education. These difficulties are linked to poor motor skills and motor-related daily functions. Therefore, higher education students with dysgraphia may require assistance and accommodations throughout their studies, not only with regard to their academic performance, but also in their motor-related daily functions.

Re-learning to be different: Increased neural differentiation supports post-stroke language recovery.

Identifying the neural changes that support recovery of cognitive functions after a brain lesion is important to advance our understanding of human neuroplasticity, which, in turn, forms the basis for the development of effective treatments. To date, the preponderance of neuroimaging studies has focused on localizing changes in average brain activity associated with functional recovery. Here, we took a novel approach by evaluating whether cognitive recovery in chronic stroke is related to increases in the differentiation of local neural response patterns. This approach is supported by research indicating that, in the intact brain, local neural representations become more differentiated (dissimilar) with learning (Glezer et al., 2015). We acquired fMRI data before and after 21 individuals received approximately 12 weeks of behavioral treatment for written language impairment due to a left-hemisphere stroke. We used Local-Heterogeneity Regression Analysis (Purcell and Rapp, 2018) to measure local neural response differentiation associated with written language processing, assuming that greater heterogeneity in the pattern of activity across adjacent neural areas indicates more well-differentiated neural representations. First, we observed pre to post-treatment increases in local neural differentiation (Local-Hreg) in the ventral occipital-temporal cortex of the left hemisphere. Second, we found that, in this region, higher local neural response differentiation prior to treatment was associated with less severe written language impairment, and that it also predicted greater future responsiveness to treatment. Third, we observed that changes in neural differentiation were systematically related to performance changes for trained and untrained items. Fourth, we did not observe these brain-behavior relationships for mean BOLD responses, only for Local-Hreg. Thus, this is the first investigation to quantify changes in local neural differentiation in the recovery of a cognitive function and the first to demonstrate the clear behavioral relevance of these changes. We conclude that the findings provide strong support for the novel hypothesis that the local re-differentiation of neural representations can play a significant role in functional recovery after brain lesion.

Developmental dysgraphia is often associated with minor neurological dysfunction in children with developmental coordination disorder (DCD).

OBJECTIVES: Children with developmental coordination disorder (DCD) are particularly affected by handwriting disorders, which remain poorly understood and are not clearly defined. The aim of our study is to provide a better understanding of handwriting disorders, and specifically of dysgraphia in children with DCD. METHODS: Sixty-five children with DCD (5-15 years), enrolled according to DSM-5, were assessed with handwriting testing and standardized assessments of neuropsychological, neurovisual, MRI and neuropsychomotor functions, with special attention paid to muscular tone examination. RESULTS: While handwriting disorders were strongly represented in our sample of children with DCD (89%), dysgraphia appeared uncommon (17%) and was closely related to several specific dysfunctions of laterality establishment; mild pyramidal tract dysfunction with distal phasic stretch reflex (PSR) in lower limbs; digital praxis slowness (both P<0.05). DISCUSSION: In our sample, dysgraphia was closely related to minor neurological dysfunction (MND) suggesting a disturbance of motor control at the level of the corticospinal motor pathway. This highlights the uncommon character of dysgraphia in children with DCD for which diagnosis should be made through a particular attention to evaluation of MND with muscular tone examination. This consideration, both in the research setting and in clinical practice, appears necessary to avoid inaccurate clinical diagnosis and to optimize appropriate therapeutic management.

The nature and treatment of phonological text agraphia.

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.

Lexical processing and distributional knowledge in sound-spelling mapping in a consistent orthography: A longitudinal study of reading and spelling in dyslexic and typically developing children.

This study examined the ability to master lexical processing and use knowledge of the relative frequency of sound-spelling mappings in both reading and spelling. Twenty-four dyslexic and dysgraphic children and 86 typically developing readers were followed longitudinally in 3rd and 5th grades. Effects of word regularity, word frequency, and probability of sound-spelling mappings were examined in two experimental tasks: (a) spelling to dictation; and (b) orthographic judgment. Dyslexic children showed larger regularity and frequency effects than controls in both tasks. Sensitivity to distributional information of sound-spelling mappings was already detected by third grade, indicating early acquisition even in children with dyslexia. Although with notable differences, knowledge of the relative frequencies of sound-spelling mapping influenced both reading and spelling. Results are discussed in terms of their theoretical and empirical implications.

Do reading and spelling share orthographic representations? Evidence from developmental dysgraphia.

Both spelling and reading depend on knowledge of the spellings of words. Despite this commonality, observed dissociations between spelling and reading in cases of acquired and developmental deficits suggest some degree of independence between the cognitive mechanisms involved in these skills. In this paper, we examine the relationship between spelling and reading in two children with developmental dysgraphia. For both children, we identified significant deficits in spelling that affected the processing of orthographic long-term memory representations of words. We then examined their reading skills for similar difficulties. Even with extensive testing, we found no evidence of a reading deficit for one of the children. We propose that there may be an underlying difficulty that specifically affects the learning of orthographic word representations for spelling. These results lead us to conclude that at least some components of lexical orthographic representation and processing develop with considerable independence in spelling and reading.

Developmental graphemic buffer dysgraphia in English: A single case study.

A single case study is reported of a 10-year-old, English-speaking boy, L.S., who presented with spelling errors similar to those described in acquired graphemic buffer dysgraphia (GBD). We used this case to evaluate the appropriateness of applying adult cognitive models to the investigation of developmental cognitive disorders. The dual-route model of spelling guided this investigation. L.S. primarily made "letter errors" (deletions, additions, substitutions, transpositions, or a combination of these errors) on words and nonwords and in all input (aural and visual) and output modalities (writing, typing, oral spelling); there was also some evidence of a length effect and U-shaped serial position curve. An effect of lexical variables on spelling performance was also found. We conclude that the most parsimonious account is an impairment at the level of the graphemic buffer and without systematic cognitive neuropsychological investigation, the nature of L.S.'s spelling difficulty would likely have been missed.

Developmental dysgraphia: An overview and framework for research.

Developmental deficits in the acquisition of writing skills (developmental dysgraphias) are common and have significant consequences, yet these deficits have received relatively little attention from researchers. We offer a framework for studying developmental dysgraphias (including both spelling and handwriting deficits), arguing that research should be grounded in theories describing normal cognitive writing mechanisms and the acquisition of these mechanisms. We survey the current state of knowledge concerning developmental dysgraphia, discussing potential proximal and distal causes. One conclusion emerging from this discussion is that developmental writing deficits are diverse in their manifestations and causes. We suggest an agenda for research on developmental dysgraphia, and suggest that pursuing this agenda may contribute not only to a better understanding of developmental writing impairment, but also to a better understanding of normal writing mechanisms and their acquisition. Finally, we provide a brief introduction to the subsequent articles in this special issue on developmental dysgraphia.

Lexical decision performance in developmental surface dysgraphia: Evidence for a unitary orthographic system that is used in both reading and spelling.

The relationship between spelling, written word recognition, and picture naming is investigated in a study of seven bilingual adults who have developmental surface dysgraphia in both Greek (their first language) and English (their second language). Four of the cases also performed poorly at orthographic lexical decision in both languages. This finding is consistent with similar results in Italian that have been taken as evidence of a developmental impairment to a single orthographic system that is used for both reading and spelling. The remaining three participants performed well at orthographic lexical decision. At first sight, preserved lexical decision in surface dysgraphia is less easy to explain in terms of a shared orthographic system. However, the results of subsequent experiments showed clear parallels between the nature of the reading and spelling difficulties that these three individuals experienced, consistent with the existence of a single orthographic system. The different patterns that were observed were consistent with the claims of Friedmann and Lukov (2008. Developmental surface dyslexias. Cortex, 44, 1146-1160) that several distinct sub-types of developmental surface dyslexia exist. We show that individual differences in spelling in surface dysgraphia are also consistent with these sub-types; there are different developmental deficits that can give rise, in an individual, to a combination of surface dyslexia and dysgraphia. Finally, we compare the theoretical framework used by Friedmann and her colleagues that is based upon the architecture of the DRC model with an account that relies instead upon the Triangle model of reading].

Neural substrates of sublexical processing for spelling.

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.

The oral spelling profile of posterior cortical atrophy and the nature of the graphemic representation.

Spelling is a complex cognitive task where central and peripheral components are involved in engaging resources from many different cognitive processes. The present paper aims to both characterize the oral spelling deficit in a population of patients affected by a neurodegenerative condition and to clarify the nature of the graphemic representation within the currently available spelling models. Indeed, the nature of graphemic representation as a linear or multi-componential structure is still debated. Different hypotheses have been raised about its nature in the orthographic lexicon, with one positing that graphemes are complex objects whereby quantity and identity are separately represented in orthographic representations and can thus be selectively impaired. Posterior cortical atrophy (PCA) is a neurodegenerative condition that mainly affects visuoperceptual and visuospatial functions. Spelling impairments are considered part of the disease. Nonetheless the spelling deficit has received little attention so far and often it has been interpreted in relation to peripheral impairments such as writing difficulties associated with visuoperceptual and visuospatial deficits. In the present study we provide a detailed characterization of the oral spelling profile in PCA. The data suggest that multiple deficits underpin oral spelling problems in PCA, with elements of surface and phonological dysgraphia but also suggesting the involvement of the graphemic buffer. A large phenotypic individual variability is reported. Moreover, the larger proportion and the specific nature of errors involving geminate (i.e., double) as compared to non-geminate (i.e., non-double) letters suggest that a further central impairment might be associated with the abstract graphemic representation of letter numerosity. The present study contributes to the clinical characterization of PCA and to the current debate in the cognitive literature on spelling models; findings, despite not definitive, support the hypothesis that graphemic representations are multidimensional mental objects that separately encode information about grapheme identity and quantity.