Developmental surface dyslexia and dysgraphia in a child with corpus callosum agenesis: an approach to diagnosis and treatment.

We present a case study detailing cognitive performance, functional neuroimaging, and effects of a hypothesis-driven treatment in a 10-year-old girl diagnosed with complete, isolated corpus callosum agenesis. Despite having average overall intellectual abilities, the girl exhibited profound surface dyslexia and dysgraphia. Spelling treatment significantly and persistently improved her spelling of trained irregular words, and this improvement generalized to reading accuracy and speed of trained words. Diffusion weighted imaging revealed strengthened intrahemispheric white matter connectivity of the left temporal cortex after treatment and identified interhemispheric connectivity between the occipital lobes, likely facilitated by a pathway crossing the midline via the posterior commissure. This case underlines the corpus callosum's critical role in lexical reading and writing. It demonstrates that spelling treatment may enhance interhemispheric connectivity in corpus callosum agenesis through alternative pathways, boosting the development of a more efficient functional organization of the visual word form area within the left temporo-occipital cortex.

Frontal allographic agraphia in a patient with behavioral variant frontotemporal dementia.

We report a patient with behavioral variant frontotemporal dementia who developed agraphia, irritability, perseverative and stereotyped behavior, and dietary changes. MRI revealed bilateral frontal convexity atrophy. Neuropsychological examination showed fluent aphasia with perseverative allographic agraphia, mild semantic impairment, and dysexecutive syndrome. Allographic agraphia featured unidirectional conversion from hiragana (cursive form of Japanese phonograms) and kanji (Japanese morphograms) to katakana (square form of Japanese phonograms), as opposed to mutual (bidirectional) conversion between hiragana and katakana in parieto-occipital gyri lesions. Furthermore, all letters of the word were converted and this whole-word conversion may be characteristic of perseverative behavior in frontotemporal dementia.

A case of aneurysmal subarchnoid haemorrhage and superficial siderosis complicated by prospagnosia, simultagnosia and alexia without agraphia.

A 42-year-old lady presented with acute aneurysmal subarachnoid haemorrhage and developed difficulty recognising faces (prosopagnosia), inability to process visual information in busy environments (simultagnosia) and difficulty to read (alexia). She was subsequently found to have superficial siderosis on MRI.

Tonal and orthographic analysis in a Cantonese-speaking individual with nonfluent/agrammatic variant primary progressive aphasia.

Clinical understanding of primary progressive aphasia (PPA) has been established based on English-speaking population. The lack of linguistic diversity in research hinders the diagnosis of PPA in non-English speaking patients. This case report describes the tonal and orthographic deficits of a multilingual native Cantonese-speaking woman with nonfluent/agrammatic variant PPA (nfvPPA) and progressive supranuclear palsy. Our findings suggest that Cantonese-speaking nfvPPA patients exhibit tone production impairments, tone perception deficits at the lexical selection processing, and linguistic dysgraphia errors unique to logographic script writer. These findings suggest that linguistic tailored approaches offer novel and effective tools in identifying non-English speaking PPA individuals.

Using a digital spelling aid to improve writing in persons with post-stroke aphasia: An intervention study.

BACKGROUND: Intervention studies aimed to improve the written production of single words by persons with aphasia have yielded promising results and there is growing interest in interventions targeting text writing. The development of technical writing aids offers opportunities for persons with aphasia, and studies have shown that using them can have a positive impact on written output. AIMS: The aim was to investigate what impact training to use a computerised spell checker had on text writing in persons with aphasia. METHODS & PROCEDURES: The study had a multiple-baseline single-case experimental design replicated across six male Swedish participants with mild-to-moderate post-stroke aphasia. The participants received training twice a week during 8 weeks, learning how to use the spell checker. At baseline and before every session, the participants wrote two texts which were logged in a keystroke-logging tool. Dependent variables were continuously measured in the texts, and the participants performed tests of language function and answered questionnaires on reading and writing habits and health-related quality of life before and after the intervention. The participants were also interviewed about how they had experienced the training. The results were evaluated on individual and group level. RESULTS: The study showed that systematic individual training involving a spell checker was experienced as positive by the participants and that they all described their writing ability in more positive terms after the intervention. Evaluation showed statistically significant improvements on group level for the dependent variables of spelling accuracy, rated syntax, writing speed and proportion of unedited text during text writing when using the spell checker. The intervention also had a generalising effect on writing speed and editing during text writing without the spell checker and on spelling accuracy in a dictation test. The participants who had the greatest spelling problems were the ones who showed the most progress, but participants with only minor writing difficulties at baseline also improved. CONCLUSIONS & IMPLICATIONS: The study shows that a digital spelling aid constitutes effective support for people with aphasia and may also affect levels other than spelling. The training had a generalising positive effect on text writing and spelling in a test. Although writing difficulties is a persisting symptom in aphasia, it can be supported and improved through use of digital spelling aids. Hence, treatment of writing ability should always be included in the rehabilitation of people with aphasia. WHAT THIS PAPER ADDS: What is already known on this subject Use of a technical writing aid can have a positive impact on the written output of persons with aphasia. Using a digital spell checker may improve spelling as well as other levels of writing, but it has not been investigated using a keystroke-logging tool in combination with language-test scores and results from questionnaires. What this paper adds to existing knowledge Through analyses on both individual and group level, this study shows that a digital spelling aid constitutes effective support for people with aphasia and also affects levels other than spelling. The training had a generalising positive effect on text writing and spelling in a test. What are the potential or actual clinical implications of this work? Digital spelling support, which is a relatively simple and inexpensive technology, can support and improve text writing in persons with post-stroke aphasia.

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.

Dysgraphia and dysprosody in a patient with arteriovenous malformation: a case report.

Arteriovenous malformation (AVM) results from development of abnormal connections between veins and arteries. This study reported anAVM case suffering from dysgraphia and dysprosody. According to the results after the trauma, the patient's handwriting was identified as macrographic and illegible, and written letters and verbs were neglected in free writing or dictation. Moreover, prosody of the patient's utterances was changed. Finally, an intervention was conducted to improve the writing impairments whereby they eventually enhanced. AVM can adversely affect communication opportunities and working life due to these impairments. Thus referring the patient to speech and language pathologists seems sensible and necessary.

Transcranial direct current stimulation with multiple oral re-reading therapy for pure alexia without agraphia: a case report.

Pure alexia without agraphia is characterized by impaired reading due to damage to the occipitotemporal cortex with preserved writing skills. In this case report, we investigate the effect of multiple oral re-reading (MOR) therapy adjunct with transcranial direct current stimulation (tDCS) in improving reading recovery of a 64-year-old patient with pure alexia without agraphia following a stroke. His MRI revealed an area of infarct with microhemorrhages at the left occipitotemporal region. The patient was blinded to each therapy and underwent seven consecutive sessions of sham tDCS followed by seven consecutive sessions of real tDCS, coupled with 1-hour MOR therapy during each session. Western Aphasia Battery (WAB) was performed at baseline, before sham and real-tDCS, and 6 weeks after completing tDCS therapy. The patient showed improvement using both sham and real-tDCS with better reading comprehension, average reading time, and word per minute after real-tDCS. This study suggests that MOR, coupled with tDCS therapy may accelerate the reading recovery in patients with pure alexia.

Could pure agraphia be the only sign of stroke? Lessons from two case reports.

Agraphia is defined as the disruption of the previously intact writing skills due to an acquired brain damage. Stroke remains the most common cause of language impairment; however, writing disorders, including agraphia, are underestimated in patients with stroke. In this regard, we report two patients presenting with pure agraphia as an early symptom of stroke. Both patients complained of at least two difficulties in visualizing letter formation beforehand, the frequent need for verbal cues, misuse of lines and margins, poorly legible signature, and writing and thinking at the same time (e.g., creative thinking and taking notes). They underwent brain magnetic resonance imaging which revealed a small lacunar infarction of the left insula and external capsule (patient 1) and a small hemorrhagic lesion in the posterior limb of the left internal capsule (patient 2). To our knowledge, this is the first report on pure agraphia as the presenting symptom of stroke. We suggest that all patients with acute agraphia, even when presenting as an isolated symptom, should be evaluated for stroke, in order to better facilitate its diagnosis and treatment.

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.

Dorsal type letter-by-letter reading accompanying alexia with agraphia due to a lesion of the lateral occipital gyri.

We report a patient with alexia with agraphia accompanied by letter-by-letter reading after hemorrhage in the left middle and inferior occipital gyri that spared the angular gyrus and the fusiform gyrus. Kanji (Japanese morphograms) and kana (Japanese phonetic writing) reading and writing tests revealed that alexia with agraphia was characterized by kana-predominant alexia and kanji-predominant agraphia. This type of "dorsal" letter-by-letter reading is discernable from conventional ventral type letter-by-letter reading that is observed in pure alexia in that (1) kinesthetic reading is less effective, (2) kana or literal agraphia coexists, and (3) fundamental visual discrimination is nearly normal.

Proactive interference apraxic agraphia: a writing and drawing disorder associated with corticobasal syndrome.

Proactive interference is when a previously performed task impairs performance on a current task. It is often associated with memory tasks and has not been reported to interfere with writing or drawing. We evaluated a left-handed man diagnosed with corticobasal syndrome who had a two-year history of progressive agraphia. On the sentence writing and clock drawing tasks, he initially wrote letters and numbers correctly but revealed an increase of movement errors as the tasks progressed. We propose the term "proactive interference apraxic agraphia" for this novel disorder. Prefrontal dysfunction may cause an impairment in disengaging from previously activated motor programs.

Selective impairment of On-reading (Chinese-style pronunciation) in alexia with agraphia for kanji due to subcortical hemorrhage in the left posterior middle temporal gyrus.

We report a patient with alexia with agraphia for kanji after hemorrhage in the left posterior middle temporal gyrus. The results of single-character kanji reading and two-character on- (Chinese-style pronunciation), kun- (native Japanese pronunciation), and Jukujikun (irregular kun-) reading word tests revealed that the patient could not read kanji characters with on-reading but read the characters with kun-reading. We consider that this on-reading alexia was caused by disconnection between the posterior inferior temporal cortex (orthographic lexicon) and the posterior superior temporal gyrus (phonological lexicon), and preserved kun- and Jukujikun-reading was realized by bypassing the orthography-to-phonology route by the semantic route.

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

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

COVID-19 presenting with agraphia and conduction aphasia in a patient with left-hemisphere ischemic stroke.

COVID-19 following infection by SARS-CoV-2 can affect the brain causing confusion, depression, and dementia-like signs. Nonetheless, the presence of more specific neuropsychological signs because of COVID-19 remains unexplored. We report on LA, a patient who was affected by a left-hemisphere ischemic stroke, probably because of SARS-CoV-2. The patient showed a highly specific neuropsychological profile characterized by severe agraphia and some signs of conduction aphasia. All other cognitive and sensorimotor functions remained intact. We sustain that specific neuropsychological signs can be observed in patients with COVID-19. Therefore, in-depth and comprehensive neuropsychological assessment should be included to better explore and qualify the neuropsychological consequences of COVID-19. This is a new challenge for diagnosis and rehabilitation, with important consequences for the involved neuropsychological services.

Too harts, won sole: Using dysgraphia treatment to address homophone representation.

Previous spoken homophone treatment in aphasia found generalization to untreated homophones and interpreted this as evidence for shared phonological word form representations. Previous written treatment of non-homophones has attributed generalization to orthographic neighbours of treated items to feedback from graphemes to similarly spelled orthographic word forms. This feedback mechanism offers an alternative explanation for generalization found in treatment of spoken homophones. The aim of this study was to investigate the mechanism underpinning generalization (if any) from treatment of written homophones. To investigate this question a participant with acquired dysgraphia and impaired access to orthographic output representations undertook written spelling treatment. Generalization to untreated items with varying degrees of orthographic overlap was investigated. Three experimental sets included homographs (e.g., bank-bank), heterographs (e.g., sail-sale), and direct orthographic neighbours (e.g., bath-path). Treatment improved written picture naming of treated items. Generalization was limited to direct neighbours. Further investigation of generalization found that items with a greater number of close neighbours in the treated set showed greater generalization. This suggests that feedback from graphemes to orthographic word forms is the driving force of generalization. The lack of homograph generalization suggests homographs do not share a representation in the orthographic lexicon.

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.

Permanent isolated micrographia from traumatic basal ganglia injury.

Micrographia is a rare neurological finding in isolation. Most cases of isolated micrographia have been found in association with focal ischemia of the left basal ganglia. Here, we present a case of post-traumatic micrographia stemming from contusion to the left basal ganglia.

Technology-enhanced writing therapy for people with aphasia: results of a quasi-randomized waitlist controlled study.

BACKGROUND: Acquired writing impairment, or dysgraphia, is common in aphasia. It affects both handwriting and typing, and may recover less well than other aphasic symptoms. Dysgraphia is an increasing priority for intervention, particularly for those wishing to participate in online written communication. Effective dysgraphia treatment studies have been reported, but many did not target, or did not achieve, improvements in functional writing. Functional outcomes might be promoted by therapies that exploit digital technologies, such as voice recognition and word prediction software. AIMS: This study evaluated the benefits of technology-enhanced writing therapy for people with acquired dysgraphia. It aimed to explore the impact of therapy on a functional writing activity, and to examine whether treatment remediated or compensated for the writing impairment. The primary question was: Does therapy improve performance on a functional assessment of writing; and, if so, do gains occur only when writing is assisted by technology? Secondary measures examined whether therapy improved unassisted written naming, functional communication, mood and quality of life. METHODS & PROCEDURES: The study employed a quasi-randomized waitlist controlled design. A total of 21 people with dysgraphia received 12 h of writing therapy either immediately or after a 6-week delay. The primary outcome measure was a functional assessment of writing, which was administered in handwriting and on a computer with assistive technology enabled. Secondary measures were: The Boston Naming Test (written version), Communication Activities of Daily Living-2, Visual Analogue Mood Scales (Sad question), and the Assessment of Living with Aphasia. Analyses of variance (ANOVA) were used to examine change on the outcome measures over two time points, between which the immediate group had received therapy but the delayed group had not. Pre-therapy, post-therapy and follow-up scores on the measures were also examined for all participants. OUTCOMES & RESULTS: Time × group interactions in the ANOVA analyses showed that therapy improved performance on the functional writing assessment. Further interactions with condition showed that gains occurred only when writing was assisted by technology. There were no significant interactions in the analyses of the secondary outcome measures. A treatment effect on these measures was therefore unconfirmed. CONCLUSIONS & IMPLICATIONS: This study showed that 21 people with dysgraphia improved on a functional writing measure following therapy using assistive technology. The results suggest that treatment compensated for, rather than remediated, the impairment, given that unassisted writing did not change. Further studies of technology-enhanced writing therapy are warranted.