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.

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.

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.

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.

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.

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.

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].

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.

Some Thoughts About Thinking.

In this first-person case history, the writer Alberto Manguel chronicles the experience of losing his ability to write and speak during a stroke. He was reassured somewhat by his continued ability to read and to quote mentally from literature that he had memorized. Within hours after the stroke, he regained the ability to write. He remained unable to speak for a month. In this essay he ponders eloquently the relationship between thought and language, and describes how it felt not to be able to bridge the gap between thought and speech during his period of aphasia.

Generalisation after treatment of acquired spelling impairments: A review.

This paper provides a comprehensive review of treatment studies of acquired dysgraphia and the occurrence of generalisation after this treatment. The aim is to examine what determines the occurrence of generalisation by investigating the link between the level of impairment, the method of treatment, and the outcome of therapy. We present the outcomes of treatment with regard to generalisation in 40 treatment studies. We derive general principles of generalisation which provide us with a better understanding of the mechanism of generalisation: (1) Direct treatment effects on representations or processes; (2) interactive processing and summation of activation; and (3) strategies and compensatory skills. We discuss the implications of these findings for our understanding of the cognitive processes used for spelling. Finally, we provide suggestions for the direction of further research into this important area, as a better understanding of the mechanism of generalisation could maximise treatment effects for an individual with acquired dysgraphia.

Cascading activation from lexical processing to letter-level processing in written word production.

Descriptions of language production have identified processes involved in producing language and the presence and type of interaction among those processes. In the case of spoken language production, consensus has emerged that there is interaction among lexical selection processes and phoneme-level processing. This issue has received less attention in written language production. In this paper, we present a novel analysis of the writing-to-dictation performance of an individual with acquired dysgraphia revealing cascading activation from lexical processing to letter-level processing. The individual produced frequent lexical-semantic errors (e.g., chipmunk → SQUIRREL) as well as letter errors (e.g., inhibit → INBHITI) and had a profile consistent with impairment affecting both lexical processing and letter-level processing. The presence of cascading activation is suggested by lower letter accuracy on words that are more weakly activated during lexical selection than on those that are more strongly activated. We operationalize weakly activated lexemes as those lexemes that are produced as lexical-semantic errors (e.g., lethal in deadly → LETAHL) compared to strongly activated lexemes where the intended target word (e.g., lethal) is the lexeme selected for production.

The analysis of perseverations in acquired dysgraphia reveals the internal structure of orthographic representations.

At a minimum, our long-term memory representations of word spellings consist of ordered strings of single letter identities. While letter identity and position must certainly be represented, it is by no means obvious that this is the only information that is included in orthographic representations, nor that representations necessarily have a one-dimensional "flat" structure. Evidence favours the alternative hypothesis that orthographic representations, much like phonological ones, are internally rich, complex multidimensional structures, though many questions remain regarding the precise nature of the internal complexity of orthographic representations. In this investigation, we test competing accounts of the internal structure of orthographic representations by analysing the perseveration errors produced by an individual with acquired dysgraphia, L.S.S. The analysis of perseveration errors provides a novel and powerful method for investigating the question of the independence of different representational components. The results provide clear support for the hypothesis that letter quantity and syllabic role information are associated with, but separable from, letter identity information. Furthermore, the results indicate that digraphs-letter pairs associated with a single phoneme (e.g., the SH in FISH)-are units of orthographic representation. These results contribute substantially to the further development of the multidimensional hypothesis, providing both new and converging evidence regarding the nature of the internal complexity of orthographic representations.

Morphological-compound dysgraphia in an aphasic patient: "A wild write through the lexicon".

We describe the case of a dysgraphic aphasic individual--S.G.W.--who, in writing to dictation, produced high rates of formally related errors consisting of both lexical substitutions and what we call morphological-compound errors involving legal or illegal combinations of morphemes. These errors were produced in the context of a minimal number of semantic errors. We could exclude problems with phonological discrimination and phonological short-term memory. We also excluded rapid decay of lexical information and/or weak activation of word forms and letter representations since S.G.W.'s spelling showed no effect of delay and no consistent length effects, but, instead, paradoxical complexity effects with segmental, lexical, and morphological errors that were more complex than the target. The case of S.G.W. strongly resembles that of another dysgraphic individual reported in the literature--D.W.--suggesting that this pattern of errors can be replicated across patients. In particular, both patients show unusual errors resulting in the production of neologistic compounds (e.g., "bed button" in response to "bed"). These patterns can be explained if we accept two claims: (a) Brain damage can produce both a reduction and an increase in lexical activation; and (b) there are direct connections between phonological and orthographic lexical representations (a third spelling route). We suggest that both patients are suffering from a difficulty of lexical selection resulting from excessive activation of formally related lexical representations. This hypothesis is strongly supported by S.G.W.'s worse performance in spelling to dictation than in written naming, which shows that a phonological input, activating a cohort of formally related lexical representations, increases selection difficulties.

Intervention for a multi-character processing deficit in a Greek-speaking child with surface dyslexia.

A case study with a 12-year-old boy, R.F., who was a monolingual speaker of Greek is reported. R.F. showed slow word reading and a difficulty in spelling irregular words but not nonwords. Assessments revealed that R.F. did not appear to have a phonological deficit, but indicated impaired multicharacter processing ability for visually presented letter arrays. On the basis of previous research linking multicharacter processing and reading we developed an intervention aimed at improving R.F.'s ability to report letter arrays of increasing length. Following a 9-week programme, improvement was observed, and investigation of R.F.'s reading revealed gains in single word reading speed and accuracy. The findings support the significance of intervention studies for testing hypotheses regarding causal relationships among cognitive processes and the notion of specific profiles of developmental dyslexia/dysgraphia in both opaque and transparent orthographies.

Making sense of deviance: identifying dissociating cases within the case series approach.

The case series approach in cognitive neuropsychology provides a means to test theories that make quantitative predictions about associations between different components of the cognitive system [Schwartz, M. F., & Dell, G. S. (2010). Case series investigations in cognitive neuropsychology. Cognitive Neuropsychology, 27, 477-494]. However, even when the predicted association is borne out the study may include outliers-observations that deviate significantly from the rest of the data. These outliers may reveal individual cases whose cognitive impairments dissociate from other cases included in the study. These dissociating cases can pose a significant challenge to the theory being tested. Using a recent case series that investigated the underlying causes of letter perseveration in spelling [Fischer-Baum, S., & Rapp, B. (2012). Underlying cause(s) of letter perseveration errors. Neuropsychologia, 50, 305-318], I discuss statistical and theoretical issues that arise when using outlier detection techniques to identify dissociating cases in a case series study.

A retrospective analysis of the sentence writing component of the Mini Mental State Examination: cognitive and affective aspects.

BACKGROUND: One of the components of the Mini Mental State Examination (MMSE) is the request to write a sentence. We investigated the relationship between the characteristics of the written sentence of the MMSE and the cognitive and affective status of elderly patients. METHODS: The characteristics of the sentence were compared to the total MMSE score, sociodemographic characteristics, tests evaluating cognition and affective status, and diagnoses. RESULTS: The number of words was significantly associated with the degree of cognitive impairment, whereas the emotional polarity of sentences and concerns about health were associated with depression. CONCLUSIONS: Characteristics of the MMSE sentence may provide important additional information regarding both cognition and affect when assessing older people.