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Background: Stroke profoundly affects quality of life (QOL), including loss of employment, reduced social activity, shrinking social networks and low mood. Dysgraphia (impaired writing) is a common symptom of aphasia yet is rarely targeted in rehabilitation. Recent technological advances might challenge this, since much communication is now conducted digitally through writing. The rehabilitation of writing may therefore help to address the wider consequences of stroke and aphasia., Aims: Can assistive technology (AT) training for people with dysgraphia: (1) improve written output, and are gains achieved only with AT? (2) improve reading comprehension scores, and are gains achieved only with AT? and (3) affect social participation, mood or QOL METHODS AND PROCEDURES: DESIGN: A mixed-methods, repeated measures, small group study design was adopted (qualitative outcomes will be reported elsewhere)., Participants: Recruited from community settings, for example, Stroke Association communication support groups., Inclusion Criteria: over 18 years old, aphasia due to stroke, acquired dysgraphia, writing more impaired than speech, fluent English prior to stroke, access to computer and Internet., Exclusion Criteria: currently receiving speech and language therapy, significant cognitive impairment, neuromuscular/motor-speech impairments/structural abnormalities, developmental dyslexia, uncorrected visual/auditory impairments., Procedures: Screening and diagnostic assessments at time T1 (first baseline). Outcome measures at T1; repeated at T2 (second baseline), T3 (end of intervention), T4 (3-month follow up). Social participation assessment and cognitive monitoring at T2, T3, T4., Intervention: Seven-ten hours individual therapy weekly and additional email support. Participants were trained to operate Dragon NaturallySpeaking (speech to text package) and ClaroRead (read writing aloud). Outcome measures were administered on pen and paper (control) and on computer, with AT enabled only at T3, T4., Outcomes and Results: Computer narrative writing was significantly improved by AT training (Friedman's χ 2 (3) = 8.27, p = 0.041), indicating a compensatory effect of AT. Though reading comprehension significantly improved in the computer condition (Friedman's χ 2 (3) = 21.07, p = 0.001), gains could not be attributed to the AT. Gains were achieved only when measures were administered on the keyboard, with AT enabled. Thus, a compensatory rather than remediatory effect was suggested. Social network size significantly increased; there were no significant changes in mood/QOL. Individual success rates varied., Conclusion and Implications: The customisable AT training was acceptable to participants and resulted in significantly improved narrative writing. Compensatory AT interventions are a useful adjunct to remediatory writing interventions and may particularly support functional writing., What This Paper Adds: What is already known on this subject Writing is rarely spared in aphasia and may present as the most impaired communication modality. Yet, people with aphasia report that writing is seldom included in their rehabilitation. Many communication activities are now conducted digitally through writing, therefore rehabilitation of this is more important than ever before. This study sought to address whether an assistive technology (AT) software package can improve writing and whether any changes were compensatory or remediatory. What this study adds to existing knowledge This group study found that AT training led to gains in written discourse and social network in people with aphasia and dysgraphia. Gains were not replicated in handwritten tasks, suggesting this was a compensatory therapeutic approach. What are the clinical implications of this work? AT programs such as this may present speech and language therapists with a practical, pragmatic adjunct to writing or typing therapy, particularly for clients with chronic, intractable impairments for whom remediatory therapy may have a low chance of success., (© 2023 The Authors. International Journal of Language & Communication Disorders published by John Wiley & Sons Ltd on behalf of Royal College of Speech and Language Therapists.)

Background: Deep brain stimulation (DBS) has been proven to be a safe, reversible, cost-effective procedure for treatment of Parkinson's disease. Final electrode position remains a significant factor determining the outcomes of subthalamic nucleus DBS (STN-DBS). This study aims to analyze the final lead position in three-dimensional plane and its effect on gross and fine motor outcomes in cases of advanced Parkinson's disease operated for STN-DBS., Methods: Patients who underwent bilateral STN-DBS were prospectively followed for improvement in gross motor outcomes at 6 months. Improvement in dysgraphia was analyzed by Fahn-Tolosa-Marin Tremor Rating Scale Part B Score. Postoperative outcomes were correlated with final electrode position., Results: A total of 64 Patients (128 leads) were analyzed. Patients who were less than 65 years of age at time of surgery had more significant reduction in UPDRS III (P=0.02). Cases with deviation of left x less than 3 mm had significant reduction in UPDRS III (P=0.05) and speech sub-scores (P=0.05). Deviation less than 2 mm in left x was associated with reduction in gait sub-scores (P=0.04). Optimal placement of right y electrode was associated with reduction in UPDRS III (P=0.02). Significant reduction in Fahn-Tolosa-Marin Tremor Rating Scale Part B Score was noted after DBS (P=0.001)., Conclusions: Subthalamic nucleus DBS thus results in significantly improved functional outcome particularly in patients with age less than 65 years. Accurate final electrode position is associated with maximum clinical benefit and improvement in dysgraphia.

Research on the relationship between sluggish cognitive tempo (SCT) and scores on neuropsychological tests (such as those measuring processing speed and reaction time) is inconclusive, and the association between SCT and motor incoordination and dysgraphia has not been objectively investigated. Mothers of 413 elementary school children (6-12 years of age) rated their children on the Pediatric Behavior Scale (PBS), which yields psychological problem scores, including SCT. Children were administered an extensive battery of neuropsychological tests assessing processing and performance speed, working memory, immediate and delayed recall, sustained attention, response inhibition, cognitive flexibility, fine motor manipulative skill, verbal fluency and retrieval, set shifting, and interference control, as well as intelligence and reading and math achievement. Only three of the 19 correlations between SCT and neuropsychological scores were significant, and all involved graphomotor tests (two timed and one untimed). In regression analysis, the strongest independent predictor of SCT was the maternal PBS incoordination factor score, followed by ratings of autism, inattention, and depression. Neuropsychological test scores did not contribute significantly more to predicting SCT. Among the incoordination PBS factor items, clumsy and draws or writes poorly were significant SCT predictors. Our novel and unexpected findings showed that motor incoordination was a stronger correlate of SCT than other variables assessed in our study, including those previously linked with SCT. Future SCT research needs to include measures of incoordination and dysgraphia in order to replicate and expand upon the current findings. Our results suggest that SCT traits are not reliably measured by currently available neuropsychological tests.

Acute carbon monoxide (CO) intoxication may result in delayed neurological sequelae, which can include amnesia, ataxia, aphasia, emotional lability, disorientation, dysphagia, and other manifestations. A 27-year-old man reported symptoms of aphasia with agraphia and alexia in a review after CO intoxication. The patient received outpatient speech therapy, as well as repeated sessions of hyperbaric oxygen for 15 days, interspersing speech therapy with hyperbaric oxygen therapy for two months. After this period of combined treatment the aphasic symptomatology remitted, and oral and written language was normal. The complete disappearance of aphasia with agraphia and alexia confirms the efficacy of the combined intervention. More data from large clinical studies are needed to assess the outcomes of hyperbaric oxygen treatment in patients with delayed neurological sequelae after CO intoxication, but this case suggests it may be a good therapeutic option in combination with specific speech therapy., Competing Interests: The authors of this paper declare no conflicts of interest exist with this submission., (Copyright© Undersea and Hyperbaric Medical Society.)

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., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

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., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

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.

A 62-year-old male presented with progressive isolated writing and spelling difficulties. Neurological, neuropsychological, speech, and language evaluations identified only minimal additional abnormalities. The presenting characteristics did not meet criteria for any particular variant of primary progressive aphasia; his clinical presentation is best described as primary progressive aphasia, with a predominant, almost pure agraphia. Brain MRI showed asymmetric, bilateral parenchymal volume loss, with left hippocampal atrophy. Fluorodeoxyglucose-F18 positron emission tomography showed hypometabolism in the lateral left frontal lobe, including Exner's area. Beta-amyloid and tau-positron emission tomography scans were negative, indicating the etiology was not Alzheimer's disease. The underlying neurodegenerative process is most likely related to TDP-43, although a 4-repeat tauopathy cannot be excluded. Following his clinical evolution, and ultimately identifying the underlying pathology from autopsy, will elucidate the etiology of this interesting clinical presentation.

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.

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].

Objective: The purpose of this study is to create an agraphia test battery specific to Turkish language, to obtain normative data for the performance and error types of this test and to demonstrate its success in detecting cognitive disorders in mild cognitive impairment (MCI) cases that can't be diagnosed by formal neuropsychological tests due to the fact that writing is a complex function., Method: For this purpose, 20 healthy control (HC) subjects, 20 MCI cases and 20 Alzheimer's disease (AD) patients with Clinical Dementia Rating (CDR) of 1 were evaluated with Ege Agraphia Test Battery., Results: Significant differences between the performance points and the error types of HC subjects, MCI cases and AD patients were obtained. As the cognitive impairment of the subjects in the study got worse, the writing skills also became worse revealing lower test points. Besides, some statistically significant differences between the error types of MCI cases and AD patients were found., Conclusion: Ege Agraphia Test Battery is not only a practical test, but also is the first defined agraphia test specific for Turkish language. Writing disorders in cases with MCI support the view that MCI is a transition period for AD. Further studies are required to increase the test data and proper rearrangements of the test battery.

Taking into account findings in the literature, the author aimed to test whether specific graphical characteristics of handwriting can distinguish patients diagnosed with schizophrenic disorders from healthy controls. Handwriting samples (one sample from each person) from 60 outpatients (29 women, 31 men; age M = 28.5, SD = 5.4) with paranoid schizophrenia were analyzed by three documents examiners and were compared to samples from 60 controls (30 men, 30 women, age M = 28.0, SD = 3.0) without psychiatric disorders. Document examiners assessed 32 graphical features potentially related to schizophrenia. The comparisons between groups revealed that only 7 out of 32 handwriting properties were significantly different in the handwriting of schizophrenic outpatients from controls: the calligraphic forms of letters, loops in ovals, lacking of dots, tremor, sinusoidal baseline, and irregularities size of lower zone. These findings are discussed in terms of motor disturbances in schizophrenia and in relation to the previous research on handwriting of other mental disorders. Similarities between the graphical patterns of handwriting of schizophrenic patients and those of other mental disorders and/or other mental states have been demonstrated., (© The Author(s) 2016.)

Background: Recent studies show that besides freezing of gait (FOG), many people with Parkinson's disease (PD) also suffer from freezing in the upper limbs (FOUL). Up to now, it is unclear which task constraints provoke and explain upper limb freezing., Objective: To investigate whether upper limb freezing and other kinematic abnormalities during writing are provoked by (i) gradual changes in amplitude or by (ii) sustained amplitude generation in patients with and without freezing of gait., Methods: Thirty-four patients with PD, including 17 with and 17 without FOG, performed a writing task on a touch-sensitive writing tablet requiring writing at constant small and large size as well as writing at gradually increasing and decreasing size. Patients of both groups were matched for disease severity, tested while 'on' medication and compared to healthy age-matched controls., Results: Fifty upper limb freezing episodes were detected in 10 patients, including 8 with and 2 without FOG. The majority of the episodes occurred when participants had to write at small or gradually decreasing size. The occurrence of FOUL and the number of FOUL episodes per patient significantly correlated with the occurrence and severity of FOG. Patients with FOUL also showed a significantly smaller amplitude in the writing parts outside the freezing episodes., Conclusions: Corroborating findings of gait research, the current study supports a core problem in amplitude control underlying FOUL, both in maintaining as well as in flexibly adapting the cycle size.

The task of recognition of oral spelling (stimulus: "C-A-T", response: "cat") is often administered to individuals with acquired written language disorders, yet there is no consensus about the underlying cognitive processes. We adjudicate between two existing hypotheses: Recognition of oral spelling uses central reading processes, or recognition of oral spelling uses central spelling processes in reverse. We tested the recognition of oral spelling and spelling to dictation abilities of a single individual with acquired dyslexia and dysgraphia. She was impaired relative to matched controls in spelling to dictation but unimpaired in recognition of oral spelling. Recognition of oral spelling for exception words (e.g., colonel) and pronounceable nonwords (e.g., larth) was intact. Our results were predicted by the hypothesis that recognition of oral spelling involves the central reading processes. We conclude that recognition of oral spelling is a useful tool for probing the integrity of the central reading processes.

Lexical orthographic information provides the basis for recovering the meanings of words in reading and for generating correct word spellings in writing. Research has provided evidence that an area of the left ventral temporal cortex, a subregion of what is often referred to as the visual word form area (VWFA), plays a significant role specifically in lexical orthographic processing. The current investigation goes beyond this previous work by examining the neurotopography of the interface of lexical orthography with semantics. We apply a novel lesion mapping approach with three individuals with acquired dysgraphia and dyslexia who suffered lesions to left ventral temporal cortex. To map cognitive processes to their neural substrates, this lesion mapping approach applies similar logical constraints to those used in cognitive neuropsychological research. Using this approach, this investigation: (a) identifies a region anterior to the VWFA that is important in the interface of orthographic information with semantics for reading and spelling; (b) determines that, within this orthography-semantics interface region (OSIR), access to orthography from semantics (spelling) is topographically distinct from access to semantics from orthography (reading); (c) provides evidence that, within this region, there is modality-specific access to and from lexical semantics for both spoken and written modalities, in both word production and comprehension. Overall, this study contributes to our understanding of the neural architecture at the lexical orthography-semantic-phonological interface within left ventral temporal cortex.

We report a case of a 47-year-old woman with 35-year history of multiple sclerosis, who showed alien hand signs, a rare behavioural disorder that involves unilateral goal-directed movements that are contrary to the individual's intention. Alien hand syndrome has been described in multiple sclerosis (MS) only occasionally and is generally suggestive of callosal disconnection. The patient presented also with bilateral limb apraxia and left hand agraphia, raising the possibility of cortical dysfunction or disconnection, in addition to corpus callosum and white matter involvement. Her specific pattern of symptoms supports the role of the corpus callosum in interhemispheric communication for complex as well as fine motor activities and may indicate that it can serve as both an inhibitory and excitatory function depending on task demands.

Written language production is often the least examined neuropsychological function, yet it provides a sensitive and subtle sign to a variety of different behavioral disorders. The dissociation between written and spoken language and reading and writing first came to clinical prominence in the nineteenth century, with respect to ideas about localization of function. Twentieth century aphasiology research focused primarily on patients with unifocal lesions from cerebrovascular accidents, which have provided insight into the various levels of processing involved in the cognitively complex task of producing written language. Recent investigations have provided a broader perspective on writing impairments in a variety of disorders, including progressive and diffuse brain disorders, and functional brain imaging techniques have been used to study the underlying processes in healthy individuals.

We report patterns of dysgraphia in participants with primary progressive aphasia that can be explained by assuming disruption of one or more cognitive processes or representations in the complex process of spelling. These patterns are compared to those described in participants with focal lesions (stroke). Using structural imaging techniques, we found that damage to the left extrasylvian regions, including the uncinate, inferior fronto-occipital fasciculus, and sagittal stratum (including geniculostriate pathway and inferior longitudinal fasciculus), as well as other deep white and grey matter structures, was significantly associated with impairments in access to orthographic word forms and semantics (with reliance on phonology-to-orthography to produce a plausible spelling in the spelling to dictation task). These results contribute not only to our understanding of the patterns of dysgraphia following acquired brain damage but also the neural substrates underlying spelling.

Primary progressive aphasia is clinically heterogeneous. We report a patient, alias Don, with a novel form of progressive aphasia, characterised by deep dyslexia and dysgraphia and dissociated access to phonological and orthographic word forms. The hallmarks of deep dyslexia and dysgraphia were present early in the course and persisted over time. Writing was initially poorer than reading, but this reversed over time. There was a lack of concordance between reading and writing errors. Don benefited from a semantic mediation strategy to learn letter sounds, involving associating letters with a country name (e.g., A=Afghanistan). Remarkably, he continued to be able to generate those phonologically complex country names when no longer able to name or sound letters. Don's performance is compatible with a traditional dual-route account of deep dyslexia and dysgraphia. The findings have potential practical implications for speech and language therapy in progressive aphasia. Moreover, they illustrate both the remarkable specificity yet clinical diversity in presentation of progressive aphasia., (Copyright © 2012 Elsevier Srl. All rights reserved.)

Background: In writing, linguistic (i.e., spelling) and nonlinguistic (i.e., arranging strokes or letters) functions are processed by the left and right hemispheres, respectively. The configuration of Korean alphabet, 'Hangul' invokes nonlinguistic, visuospatial functions that other writing systems use less extensively. Patients with Alzheimer's disease (AD) have bilateral involvement of temporoparietal-frontal areas that are responsible for processing language and visuospatial functions., Objectives: The aim of this study was to examine the nature of Hangul writing dysfunction, which may be associated with bilateral hemispheric impairments in AD., Methods: A sample of 75 patients with AD and 20 healthy controls (HC) performed a Hangul writing task. Neuroimaging positron emission tomography (PET) data of 22 patients were utilized to measure the regional brain glucose metabolism associated with Hangul writing., Results: The writing performance of the AD group was significantly reduced and different types of errors were observed as the disease got worse. Glucose hypometabolism correlated with Hangul writing impairment was located in the right occipitotemporal lobe and left temporoparietal lobe., Conclusions: The PET findings demonstrate that impairment in Hangul writing performance in Korean AD patients is closely related to a functional decline in both the right and left hemispheres. The study provides a unique contribution to the knowledge of dysgraphia in a non-alphabetical writing system as well as the underlying neuropathology of dysgraphic features in such languages., (Copyright © 2012 Elsevier B.V. All rights reserved.)

The case study of Martial, a French 9-year-old boy, who exhibits severe mixed dyslexia and surface dysgraphia is reported. Despite very poor pseudo-word reading, Martial has preserved phonological processing skills as his good oral language, good phoneme awareness and good verbal short-term memory show. He exhibited a strong length effect when reading briefly presented words but no sign of mini-neglect. His letter-string processing abilities were assessed through tasks of whole and partial report. In whole report, Martial could only name a few letters from briefly displayed 5-consonant strings. He showed an initial-position advantage and a sharper than expected left-to-right gradient of performance. He performed better when asked to report a single cued letter within the string but then showed an atypical right-side advantage. The same rightward attentional bias was observed in whole report when top-down control was prevented. Otherwise, Martial showed preserved single letter identification skills and good processing of 5-letter strings when letters were sequentially displayed one at a time. His poor letter-string processing thus reflects a parallel visual processing disorder that is compatible with either a visual attention (VA) span or a visual short-term memory disorder. Martial was further engaged in a complex reaching movement task involving VA and simultaneous processing. He performed motor sequences not as a whole but as a succession of independent motor units, suggesting that his attention was not allocated in parallel to the two to-be-reached targets prior to movement execution. Against a more basic motor disorder however, he showed good performance in a task of cyclical pointing movements. The overall findings suggest that Martial suffers from a visual simultaneous processing disorder that disturbs letter identification in strings. Instead of being restricted to letter-string processing, this VA disorder might extend to non-verbal task., (Copyright © 2011 Elsevier Srl. All rights reserved.)

The goal of this study was to examine past-tense spelling deficits in developmental dyslexia and their relationship to phonological abilities, spoken morphological awareness and word specific orthographic memory. Three groups of children (28 9-year-old dyslexic, 28 chronological age-matched and 28 reading/spelling age-matched children) completed a battery of tests including spelling regularly inflected words (e.g., kissed) and matched one-morpheme words (e.g., wrist). They were also assessed on a range of tests of reading and spelling abilities and associated linguistic measures. Dyslexic children were impaired in relation to chronological age-matched controls on all measures. Furthermore, they were significantly poorer than younger reading and spelling age-matched controls at spelling inflected verbs, supporting the existence of a specific deficit in past-tense spelling in dyslexia. In addition to under-using the -ed spelling on inflected verbs, the dyslexic children were less likely to erroneously apply this spelling to one-morpheme words than younger controls. Dyslexics were also poorer than younger controls at using a consistent spelling for stems presented in isolation versus as part of an inflected word, indicating that they make less use of the morphological relations between words to support their spelling. In line with this interpretation, regression analyses revealed another qualitative difference between the spelling and reading age-matched group and the dyslexic group: while both spoken morphological awareness and orthographic word specific memory were significant predictors of the accuracy of past-tense spelling in the former group, only orthographic memory (irregular word reading and spelling) was a significant factor in the dyslexic group. Finally, we identified a subgroup of seven dyslexic children who were severely deficient in past-tense spelling. This subgroup was also significantly worse than other dyslexics and than younger controls on scores of orthographic memory. The implications of our findings for teaching and remediation strategies are discussed., (Copyright © 2011 Elsevier Srl. All rights reserved.)

Introduction: The objective of this study is to determine which cognitive processes underlying spelling are most affected in the three variants of primary progressive aphasia (PPA): Logopenic variant primary progressive aphasia (lvPPA), Semantic variant primary progressive aphasia (svPPA), and Nonfluent variant primary progressive aphasia (nfvPPA)., Methods: 23 PPA patients were administered The Johns Hopkins Dysgraphia Battery to assess spelling. Subtests evaluate for effects of word frequency, concreteness, word length, grammatical word class, lexicality (words vs pseudowords), and "regularity" by controlling for the other variables. Significant effects of each variable were identified with chi square tests. Responses on all spelling to dictation tests were scored by error type. 16 of the 23 subjects also had a high resolution MRI brain scan to identify areas of atrophy., Results: We identified 4 patterns of spelling that could be explained by damage to one or more cognitive processes underlying spelling. Nine patients (3 unclassifiable, 4 with lvPPA, 2 with svPPA) had dysgraphia explicable by impaired access to lexical representations, with reliance on sublexical phonology-to-orthography conversion (POC). Two patients (with nfvPPA) showed dysgraphia explicable by impaired access to lexical representations and complete disruption of sublexical POC. Seven patients (4 with lvPPA, 1 with svPPA, 2 unclassifiable) showed dysgraphia explicable by impaired access to lexical-semantic representations and/or lexical representations with partially spared sublexical POC mechanisms. Five patients (1 with nfvPPA, 2 with svPPA, 1 with lvPPA, and 1 unclassifiable) showed dysgraphia explicable by impairment of the graphemic buffer., Conclusions: Any cognitive process underlying spelling can be affected in PPA. Predominance of phonologically plausible errors, more accurate spelling of regular words than irregular words, and more accurate spelling of pseudowords than words (indicating spared POC mechanisms) may indicate a low probability of progression to nfvPPA., (Copyright © 2009 Elsevier Srl. All rights reserved.)

Posterior fusiform gyrus (BA 37) is responsible for Hanja (ideogram) alexia in stroke patients. Patients with semantic dementia (SD) have lesions in the basal temporal area. The close proximity in these two lesions and the fact that reading ideograms requires holistic processing as is necessary in recognition of objects, suggests a possibility that ideogram alexia/agraphia may occur in patients with SD. We established and carried out Hanja and Hangul (phonogram) reading/writing tasks on six SD patients and nine Alzheimer's disease (AD) patients as control to see if these two patient groups show dissociation in the two sets of tests. SPM analysis was performed on the SD patients' PET images to look for any dysfunctions in the posterior fusiform gyrus. The SD patients manifested Hanja alexia/agraphia whereas Hangul reading/writing ability was relatively preserved. There were group differences between SD and AD in the Hanja tasks but not in the Hangul tasks. The SPM analysis revealed no hypometabolism in the posterior fusiform gyrus, but only in the middle and the anterior part of the temporal gyrus. Dysfunction in the middle temporal gyrus (BA 21) may have disrupted the temporal lobe connections preventing the function of the posterior fusiform gyrus.

Research in the cognitive and neural sciences has long posited a distinction between the long-term memory (LTM) storage of information and the short-term buffering of information that is being actively manipulated in working memory (WM). This basic type of distinction has been posited in a variety of domains, including written language production-spelling. In the domain of spelling, the primary source of empirical evidence regarding this distinction has been cognitive neuropsychological studies reporting deficits selectively affecting what the cognitive neuropsychological literature has referred to as the orthographic lexicon (LTM) or the graphemic buffer (WM). Recent papers have reexamined several of the hallmark characteristics of impairment affecting the graphemic buffer, with implications for our understanding of the nature of the orthographic LTM and WM systems. In this paper, we present a detailed case series study of 4 individuals with acquired spelling deficits and report evidence from both error types and factors influencing error rates that support the traditional distinction between these cognitive systems involved in spelling. In addition, we report evidence indicating possible interaction between these systems, which is consistent with a variety of recent findings in research on spelling.

In this study we investigated 12 cases of "mixed dysgraphia", a spelling impairment where regular words are spelt better than either ambiguous words or regular non-words. Two explanations of mixed dysgraphia were formerly offered by Luzzatti et al. (1998): (i) a double functional lesion of the orthographic output lexicon (or damage to its access) and of the acoustic-to-phonological conversion; and (ii) some kind of interaction/summation between lexical and sublexical spelling routes when processing regular words. We first analysed whether a double functional lesion was sufficient to explain the mixed dysgraphia, checking acoustic-to-phonological conversion by means of the repetition of words and non-words: the answer was positive in five cases and uncertain in three. We tested the remaining four cases to see if there was an interaction between lexical and sublexical processing of regular words, quantifying for each patient, on a probabilistic basis, the separate contribution of the residual lexical and sublexical resources. We investigated whether the processing along these routes was simultaneous but independent ("independent cooperation") or if instead there was "interaction", i.e., the simultaneous activity led to an added increase of efficiency over and above the mere combination of separate success probabilities. For one case the processing along the two routes was independent, in the other three cases an interaction resulted. Following the same approach, we found that for the five cases with a double functional lesion, the observed success on regular word spelling was higher than that expected on a probabilistic basis, but the interpretation of this finding was different.

Although semantic dementia (SD) is defined as a selective disruption of conceptual knowledge, a number of group studies have now demonstrated that SD patients also show impaired performance on tasks not usually considered to have a high semantic load (e.g., reading words aloud and lexical or object decision). The aim of the current study was to document the relative deterioration, over time, of a number of semantic and so-called 'non-semantic' tasks in LF, a single case of SD for whom - by virtue of his work as a published cartoonist - we also have extensive data regarding his pre-morbid linguistic and drawing skills. In five testing rounds over a period of five years we administered semantic tests of object naming and object definition (on both of which LF was progressively impaired, as expected for a diagnosis of SD), plus verbal and non-verbal 'non-semantic' tasks of reading aloud, spelling, object and lexical decision, and delayed copy drawing. Initially, his only striking 'non-semantic' deficit was in the domain of spelling - a pronounced surface dysgraphia in an individual with demonstrably superior pre-morbid spelling skill. Over time, and in line with his declining semantic system, LF's performance gradually deteriorated on all of the 'non-semantic' tasks. The most vulnerable items on most tasks were those with low frequency and an atypical form. This report adds to the growing body of evidence that a number of cognitive processes not usually considered to be 'semantic' in their demands rely on the integrity of semantic knowledge for successful execution. Furthermore, it provides the first indication that these non-semantic deficits might emerge in an order predictable from the typicality structure of the relevant domain.

The terms 'jargon aphasia' and 'jargon agraphia' describe the production of incomprehensible language containing frequent phonological, semantic or neologistic errors in speech and writing, respectively. Here we describe two patients with primary progressive aphasia (PPA) who produced neologistic jargon either in speech or writing. We suggest that involvement of the posterior superior temporal-inferior parietal region may lead to a disconnection between stored lexical representations and language output pathways leading to aberrant activation of phonemes in neologistic jargon. Parietal lobe involvement is relatively unusual in PPA, perhaps accounting for the comparative rarity of jargon early in the course of these diseases.

We report the case of patient MN, diagnosed with frontotemporal dementia, who exhibited a severe impairment in writing letters and words in upper-case print in the face of accurate production of the same stimuli in lower-case cursive. In contrast to her written production difficulties, MN was unimpaired in recognizing visually presented letters and words in upper-case print. We find a modest benefit of visual form cueing in the written production of upper-case letters, despite an inability to describe or report visual features of letters in any case or font. This case increases our understanding of the allographic level of letter-shape representation in written language production. It provides strong support for previous reports indicating the neural independence of different types of case and font-specific letter-shape information; it provides evidence that letter-shape production does not require explicit access to information about the visual attributes of letter shapes and, finally, it reveals the possibility of interaction between processes involved in letter-shape production and perception.

The present study aims to demonstrate that errors when writing are more common than expected in patients affected by primary lateral sclerosis (PLS) with severe dysarthria or complete mutism, independent of spasticity. Sixteen patients meeting Pringle's et al. [34] criteria for PLS underwent standard neuropsychological tasks and evaluation of writing. We assessed writing abilities in spelling through dictation in which a set of words, non-words and short phrases were presented orally and by composing words using a set of preformed letters. Finally, a written copying task was performed with the same words. Relative to controls, PLS patients made a greater number of spelling errors in all writing conditions, but not in copy task. The error types included: omissions, transpositions, insertions and letter substitutions. These were equally distributed on the writing task and the composition of words with a set of preformed letters. This pattern of performance is consistent with a spelling impairment. The results are consistent with the concept that written production is critically dependent on the subvocal articulatory mechanism of rehearsal, perhaps at the level of retaining the sequence of graphemes in a graphemic buffer. In PLS patients a disturbance in rehearsal opportunity may affect the correct sequencing/assembly of an orthographic representation in the written process.

Amyotrophic lateral sclerosis (ALS) with dementia (ALS-D) is known to exhibit characteristics of frontotemporal dementia. However, in clinical situations, it is often difficult to evaluate their cognitive functions because of impaired voluntary speech and physical disabilities. In order to identify characteristic and diagnostic cognitive symptoms of relatively advanced ALS-D patients, we retrospectively reviewed the clinical features of seven cases of clinically definitive ALS who had dementia, impaired voluntary speech, and physical disability. Their medical records showed that six out of seven patients made writing errors, and all of the patients demonstrated anosognosia. The writing errors consisted of paragraphia such as substitution, omission, or syntactic errors with individual differences in error types. Dissociation between kana and kanji were also observed. Anosognosia was evaluated by a self-rating scale with which the patients and the medical staff evaluated the patient's physical ability; the results indicated a large discrepancy between the evaluation by the patients and the medical staff. We emphasize that aphasic writing errors have been underestimated, particularly in ALS-D patients with impaired voluntary speech. We also reported that anosognosia was the most important and quantifiable symptom in ALS-D. The relationship between writing errors and anosognosia should be investigated further.

Since the observation of Auguste D. by Alöis Alzheimer, it is an acknowledged fact that writing is one of the cognitive functions that are weakened early in Alzheimer's disease (AD). This study aimed to examine the cognitive nature of this disorder and question the hypothesis of a standard progression (Platel et al., 1993) from lexical to other central and more peripheral processes. A large group of mild to moderate AD patients (n=59) and a group of healthy elderly controls were submitted to an extensive assessment of both the central and peripheral components of writing. A comparison of groups indicated that AD patients performed more poorly than controls on a wide range of writing measures. It revealed a predominantly lexical disorder, but also found evidence of associated disorders located at different stages in the spelling system (phonological route, graphemic buffer, allographic store, graphic motor patterns). A multiple single-case analysis, using a specific methodology, allowed us to delimit individual profiles of agraphia. It revealed a wide variety of agraphia syndromes, including a far from negligible number of patients with selective damage to one of the central or peripheral components, as well as patients with multiple writing impairments. A positive correlation was observed between the severity of the dementia and spelling/writing measures (lexical and allographic). This study does not support the hypothesis of a uniform progression. Rather, it points to heterogeneous profiles of agraphia and suggests that the first signs of writing impairment in AD stem from changes at different points in the broad anatomical network subserving spelling and writing abilities.

Multiple complaints in the domain of writing are common among children with Attention Deficit Hyperactivity Disorder (ADHD). In this work we sought to characterize the writing disorder by studying dysgraphia in twenty 6th grade boys with ADHD and normal reading skills matched to 20 healthy boys who served as a comparison group. Dysgraphia, defined as deficits in spelling and handwriting, was assessed according to neuropsychological explanatory processes within 3 primary domains: linguistic processing, motor programming and motor kinematics. Children with ADHD made significantly more spelling errors, but showed a unique pattern introducing letter insertions, substitutions, transpositions and omissions. This error type, also known as graphemic buffer errors, can be explained by impaired attention aspects needed for motor planning. Kinematic manifestations of writing deficits were fast, inaccurate and an inefficient written product accompanied by higher levels of axial pen pressure. These results suggest that the spelling errors and writing deficits seen in children with ADHD and normal reading skills stem primarily from non-linguistic deficits, while linguistic factors play a secondary role. Recommendations for remediation include educational interventions, use of word processing and judicious use of psychostimulants.

This study directly compared four patients who, to varying degrees, showed the characteristics of deep dyslexia, dysphasia and/or dysgraphia--i.e., they made semantic errors in oral reading, repetition and/or spelling to dictation. The "primary systems" hypothesis proposes that these different conditions result from severe impairment to a common phonological system, rather than damage to task-specific mechanisms (i.e. grapheme-phoneme conversion). By this view, deep dyslexic/dysphasic patients should show overlapping deficits but previous studies have not directly compared them. All four patients in the current study showed poor phonological production across different tasks, including repetition, reading aloud and spoken picture naming, in line with the primary systems hypothesis. They also showed severe deficits in tasks that required the manipulation of phonology, such as phoneme addition and deletion. Some of the characteristics of the deep syndromes - namely lexicality and imageability effects - were typically observed in all of the tasks, regardless of whether semantic errors occurred or not, suggesting that the patients' phonological deficits impacted on repetition, reading aloud and spelling to dictation in similar ways. Differences between the syndromes were accounted for by variation in other primary systems--particularly auditory processing. Deep dysphasic symptoms occurred when the impact of phonological input on spoken output was disrupted or reduced, either as a result of auditory/phonological impairment, or for patients with good phonological input analysis, when repetition was delayed. 'Deep' disorders of reading aloud, repetition and spelling can therefore be explained in terms of damage to interacting primary systems such as phonology, semantics and vision, with phonology playing a critical role.

With increasing frequency the lexicon of every language incorporates words taken from other languages (loan words) that become of common use, but whose orthography does not necessarily conform to the rules of the native language. This is particularly true for languages like Italian, where most words can be correctly written by the application of a sublexical phonology-to-orthography conversion procedure. Here we report the case of a neurologically unimpaired, highly educated Italian person, with a specific deficit in writing loan words. The aim of the study is to clarify the nature of her dysgraphic impairment, if linked to a semantic deficit, or alternatively reflecting an acquisition deficit, present both in the phonological and orthographic output lexicons, or specific only for the orthographic lexicon. A qualitative analysis of her errors and a comparison with the performance of a group of age and education matched subjects showed that her error pattern was not an amplification of the task difficulty effect observed in the control subjects, but rather an expression of an impairment in acquiring specific writing procedures.

To account for the mechanism of number transcoding, many authors have proposed various models, for example, semantic-abstract model, lexical-semantic model, triple-code model, and so on. However, almost all of them are based on the symptoms of patients with left cerebral damage. Previously, I reported two Japanese patients with right posterior cerebral infarction showing pure misallocation of "0" (omission: "40,265"-->"4,265," addition: "107"-->"1,007," transposition: "4,072"-->"4,702") both in writing and oral reading of Arabic numerals. To examine whether the pure misallocation of "0" is commonly observed in patients with right cerebral damage, I investigated writing and oral reading of Arabic numerals in 18 patients with right cerebral damage and 16 healthy controls. All patients with right cerebral damage showed pure misallocation of "0" both in writing and reading. The pure misallocation of "0" due to right cerebral damage cannot be explained by current models. It may be more useful to explain the phenomenon by regarding an Arabic numeral as graph on a two-dimensional plane composed of two axes (place-holding values and digits).

We report the case of a left-handed man (MCR), who presented with a peripheral agraphia as an early sign of multiple sclerosis. His left-handed writing was neologistic, whilst oral spelling, typing and spelling with the right hand were intact. Structural MRI scanning revealed a lesion of the body of the corpus callosum. Dichotic listening tests indicated that MCR displayed left hemisphere dominance for language. It is proposed that MCR represents a case of a disconnection syndrome in which right hemisphere systems that provide the basis for movement templates during left-handed writing are isolated from left hemisphere language systems. Analysis of left-handed writing indicated that peripheral movement control was highly structured with both individual letter frequency and sequential dependencies between letters represented within these motor control units. This case represents an opportunity to explore the mechanisms of movement control for writing and to examine the characteristics of isolated letter templates.

This study evaluates the performance of children with diagnosed dysgraphia and a control group on the Rey-Osterrieth Complex Figure test. A group of 48 Greek elementary school children aged 6.6 to 12.5 years who were in regular school placement participated. All had been diagnosed with dysgraphia, using the writing scale of the Luria-Nebraska Neuropsychological Battery and were matched with a control group of 48 for sex, age, grade, and handedness. Both groups were administered the test, and the children were examined during copying and mnemonic reproduction of the complex figure. Analysis indicated that there were no significant differences between dysgraphic and nondysgraphic groups during the copying task but the dysgraphic group performed significantly lower during mnemonic reproduction of the Rey-Osterrieth Complex Figure. These results suggest that children with dysgraphia possibly suffer from cognitive difficulties that influence visual memory more than visuomotor skills.

Two main hypotheses have been proposed regarding the role of phonology in written word production. According to the phonological mediation hypothesis, the retrieval of the lexical phonological representation of a word is an obligatory prerequisite to the retrieval of its spelling. Therefore, deficits to the phonological lexicon should affect both spoken and written picture naming. In contrast, the orthographic autonomy hypothesis posits that the lexical orthographic representations of words can be accessed without any necessary phonological mediation. In support of this view, cases of preserved written naming despite impaired lexical phonology have been reported following brain damage. In this report, we replicate this basic pattern of performance in case YP, a 60-year-old woman with a pattern of frontotemporal dementia. As her disease progressed, YP's ability to write down the names of pictures remained very good despite a severe decline in oral naming. Further testing indicated that this deficit was not primarily due to an articulatory or post-lexical phonological deficit. YP's case provides strong additional support for the orthographic autonomy hypothesis. The significance of this case with respect to the characterization of dementia syndromes is discussed.

We report a patient who, after a left parieto-occipital lesion, showed alexia and selective dysgraphia for uppercase letters. He showed preserved oral spelling, associated with handwriting impairment in all written production; spontaneous writing, writing to dictation, real words, pseudowords, and single letters were affected. The great majority of errors were well-formed letter substitutions: most of them were located on the first position of each word, which the patient always wrote in uppercase (as he used to do before his illness). The patient also showed a complete inability to access the visual representation of letters. As demonstrated by a stroke segmentation analysis, letter substitutions followed a rule of graphomotor similarity. We propose that the patient's impairment was at the stage where selection of the specific graphomotor pattern for each letter is made and that the apparent selective disruption of capital case was due to a greater stroke similarity among letters belonging to the same case. We conclude that a visual format is necessary neither for spelling nor for handwriting., (Copyright 2000 Academic Press.)

Writing disorders are an early manifestation of Alzheimer's disease (AD), often more severe than language difficulties. AD patients produce shorter and less informative written descriptions of a complex picture than controls. These abbreviated texts also include many intrusions, semantic substitutions, and misspellings. Syntactic difficulties are characterized by a reduction of subordinate clauses rather than by the occurrence of grammatical errors. Lexical spelling is systematically more impaired and affected earlier than phonological spelling. With disease progression, the deficits of central writing processes extend to graphic difficulties and alteration of handwriting spatial organization. In the context of a semiotic hierarchy, an inverse relationship is suggested between writing acquisition during childhood and subsequent writing degradation in AD. The writing disturbance in AD is evidently related to a disruption in the anatomicofunctional cerebral network designed for writing processes, mainly in the parietal regions.

The purpose of this study was to investigate the cognitive causes underlying spelling difficulties in a case of developmental surface dysgraphia, AW. Our results do not support a number of possibilities that could be the cause of AW's poor orthographic lexicon, including difficulties in phonological processing, phonological short-term memory, configurational visual memory, and lexical semantic memory. We have found instead that AW performs poorly in tasks that involve detection of the order of adjacent letters in a word or the order of adjacent units in strings of consonants or symbols. Finally, he performs poorly in tasks that involve reconstructing the order of a series of complex visual characters (Japanese and Hindi characters) especially when these are presented sequentially. We advance the hypothesis that AW's poor spelling and good reading skills stem from an underlying pattern of cognitive abilities where a very good visual configurational memory is coupled with a poor ability to encode serial order. This may have resulted in a holistic word-based reading strategy, which, together with the original problem of encoding order, may have had detrimental effects for the acquisition of spelling.

Phonological alexia and agraphia are acquired disorders characterized by an impaired ability to convert graphemes to phonemes (alexia) or phonemes to graphemes (agraphia). These disorders result in phonological errors typified by adding, omitting, shifting, or repeating phonemes in words during reading or graphemes when spelling. In developmental dyslexia, similar phonological errors are believed to result from deficient phonological awareness, an oral language skill that manifests itself in the ability to notice, think about, or manipulate the individual sounds in words. The Auditory Discrimination in Depth (ADD) program has been reported to train phonological awareness in developmental dyslexia and dysgraphia. We used a multiple-probe design to evaluate the ADD program's effectiveness with a patient with a mild phonological alexia and mixed agraphia following a left hemisphere infarction. Large gains in phonological awareness, reading and spelling nonwords, and reading and spelling real words were demonstrated. A follow-up reassessment, 2 months posttreatment, found the patient had maintained treatment gains in phonological awareness and reading, and attained additional improvement in real word reading.

A 65-year-old woman with progressive visuospatial dysfunction for 2 years complained of later-onset associated memory impairment. MRI revealed diffuse cerebrocortical atrophy, which was especially severe in both parieto-occipital regions but spared the calcarine and pericalcarine cortices. Examination 5 years after onset revealed left visual hemi-neglect, oculomotor apraxia, optic ataxia, simultanagnosia, verbal alexia, lexical and spatial agraphia, and anterograde amnesia. This patient's disorder is considered in the context of previous reports on the array of cognitive disturbances associated with posterior cortical atrophy (pCA). Special emphasis is made on her reading and writing disturbances, because their prevalence and range of individual variability have not been established in pCA. This array of neuropsychological manifestations may help to distinguish among different clinical and etiological types of pCA, and to elucidate the pathophysiology of a syndrome that has been associated with conditions as diverse as Alzheimer's disease, subcortical gliosis, and prion diseases. The parameters described in our case may thus help to address these issues in clinico-pathological studies with large numbers of patients with pCA.

This study was conducted to delineate the pattern of the writing impairments in 12 patients with Alzheimer type dementia. The patients performed writing tasks involving regular and irregular words and nonwords given by dictation as well as a decision test composed of printed words and pictures requiring phonologic, lexical, and semantic processing. Writing from dictation demonstrated a predominant, but nonisolated, lexical deficit. In order to better evaluate this lexical disorder, the correlation between the dictation writing scores and the decision task scores was analyzed. No significant correlation was found among scores for irregular words, phonologically plausible errors, and scores of the lexical or semantic decision tasks, but there was a significant correlation among scores for the nonwords, nonphonological spelling errors, and scores of the phonologic decision task using printed words. These results would suggest that the "lexical" deficit in agraphia, i.e., difficulty in retrieval or loss of the spelling representations of words, is independent of the lexical or semantic capacities involved in other modalities.