Written spelling agraphia

When a disorder of single word writing is seen in conjunction with preserved oral spelling and intact written grapheme formation ("written spelling agraphia"), the deficit may be presumed to lie somewhere between letter choice and written motor output. Two potential deficits are considered: (1) visual letter codes are not activated properly and (2) the information contained within properly activated visual letter codes fails to reach intact graphic motor patterns. Two patients with written spelling agraphia were each given a series of tests aimed at distinguishing between the two possibilities. Results suggest that the written spelling agraphia seen in these two patients arises from different underlying deficits. The results are discussed in terms of the patients' CT scan lesion sites, which were markedly different.     

Agraphia selective for written spelling : An experimental case study

A 56-year-old right-handed man suffered left posterior parieto-temporal ischemia leading to mild aphasia, Gerstmann syndrome, and a novel variant of agraphia. This variant compromised his spelling by writing and manual sorting of letters more than his oral spelling. The dissociation was experimentally documented. It principally involved the intrusion of extraneous letters, independent of input modality. It did not generalized to numbers or an arbitrary code. Postoperatively the disability disappeared. It was concluded that the programs which translate letter choice into visual terms for purposes of written (as distinct from oral) spelling either originate or are transmitted in a distinct cerebral location. This location, which may be the left posterior parasagittal parietal area, can be selectively impaired by a focal lesion.     

Developmental phonological and lexical agraphia in adults

Many cases of agraphia from acquired cerebral lesions may be divided into two groups, phonological and lexical, suggesting two dissociable spelling systems. Studies of developmental agraphia have described some children who have spelling patterns similar to acquired phonological or lexical agraphia. This study analyzed spelling results from 22 adolescent and adult subjects with developmental agraphia (DA) and compared them to those from control subjects and subjects with acquired agraphia (AA). On the basis of spelling ability, subjects with DA could be divided into two groups. Analysis of the profile of spelling abilities indicated that the two groups of subjects with DA were almost indistinguishable from the two groups of subjects with AA, phonological and lexical. This supports the contention that DA in adults may be divided into phonological and lexical groups and further supports the two-system hypothesis for linguistic agraphia.     

Lesion localization of phonological agraphia.

Phonological agraphia is a neurolinguistically specific profile of spelling impairment. It is characterized by an impairment in spelling pronounceable pseudowords ('sild') and by an impairment in spelling real words related to their familiarity, length, and often, part of speech (Shallice, 1981; Roeltgen, 1985; Bub & Chertkow, 1988). We report two cases of phonological agraphia, each with a different lesion profile. Analysis of the small number of published cases that include lesion data, plus our own cases, suggests that phonological agraphia can be produced by lesions in a wide range of perisylvian cortical regions that have in common some role in central phonological processes.     

Writing with the right hemisphere

We studied writing abilities in a strongly right-handed man following a massive stroke that resulted in virtually complete destruction of the language-dominant left hemisphere. Writing was characterized by sensitivity to lexical-semantic variables (i.e., word frequency, imageability, and part of speech), semantic errors in writing to dictation and written naming, total inability to use the nonlexical phonological spelling route, and agrammatism in spontaneous writing. The reliance on a lexical-semantic strategy in spelling, semantic errors, and impaired phonology and syntax were all highly consistent with the general characteristics of right hemisphere language, as revealed by studies of split-brain patients and adults with dominant hemispherectomy. In addition, this pattern of writing closely resembled the syndrome of deep agraphia. These observations provide strong support for the hypothesis that deep agraphia reflects right hemisphere writing.     

Alexia and agraphia with spared spelling and letter recognition abilities

A patient with alexia and agraphia had intact spelling and comprehension of spelled words and used a letter-naming strategy to read and write. We propose that there is a graphemic area important for distinguishing graphemic features and for programming movements used in writing. In this patient this area was not functioning or did not have access to the area of visual word imagés. Therefore, he used an ideographic letter-naming strategy to verbally circumvent his disability and gain access to the area of visual word images.     

Spatial neglect, Balint-Homes' and Gerstmann's syndrome, and other spatial disorders

Brain-damaged patients with lesion or dysfunction involving the parietal cortex may show a variety of neuropsychological impairments involving spatial cognition. The more frequent and disabling deficit is the syndrome of unilateral spatial neglect that, in a nutshell, consists in a bias of spatial representation and attention ipsilateral to of extrapersonal, personal (ie, the body) space, or both, toward the side of the hemispheric lesion. The deficit is more frequent and severe after damage to the right hemisphere, involving particularly the posterior-inferior parietal cortex at the temporo-parietal junction. Damage to these posterior parietal regions may also impair visuospatial short-term memory, which may be associated with and worsen spatial neglect. The neural network supporting spatial representation, attention and short-term memory is, however, more extensive, including the right premotor cortex. Also disorders of drawing and building objects (traditionally termed constructional apraxia) are a frequent indicator of posterior parietal damage in the left and in the right hemispheres. Other less frequent deficits, which, however, have a relevant localizing value, include optic ataxia (namely, the defective reaching of visual objects, in the absence of elementary visuo-motor impairments), which is typically brought about by damage to the superior parietal lobule. Optic ataxia, together with deficits of visual attention, of estimating distances and depth, and with apraxia of gaze, constitutes the severely disabling Balint-Holmes' syndrome, which is typically associated with bilateral posterior parietal and occipital damage. Finally, lesions of the posterior parietal lobule (angular gyrus) in the left hemisphere may bring about a tetrad of symptoms (left-right disorientation, acalculia, finger agnosia, and agraphia) termed Gerstmann's syndrome, that also exists in a developmental form.     

Short-term memory impairment and unilateral dichotic listening extinction in a child with Landau-Kleffner syndrome: auditory or phonological disorder?

The neuropsychological profile of a child with a Landau-Kleffner syndrome is presented here. The observed cognitive difficulties included verbal short-term memory and seemed partially compensated for when the experimental assessments bypassed the auditory channel. This case study is especially challenging since the child, whose phonological skills were quite efficient and who exhibited a dichotic listening unilateral extinction, had developed average reading and spelling abilities. The fact that B.E.'s performance on memory tasks was quite poor when the stimuli were presented auditorily and more efficient when the stimuli were presented visually, strongly suggests that the observed memory impairment was due to a deficit at the level of cortical auditory processing. B.E.'s phonological skills were efficient, suggesting a neuropsychological dissociation between phonological ability and auditory processing. The fact that B.E. dramatically recovered language and easily acquired reading and spelling accounts for the hypothesis that compensatory strategies allowed him to develop phonological skills from predominantly visual input.     

Selective uppercase dysgraphia with loss of visual imagery of letter forms: a window on the organization of graphomotor patterns

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.     

Perceptual and academic patterns of learning-disabled/gifted students.

This research explored ways gifted children with learning disabilities perceive and recall auditory and visual input and apply this information to reading, mathematics, and spelling. 24 learning-disabled/gifted children and a matched control group of normally achieving gifted students were tested for oral reading, word recognition and analysis, listening comprehension, and spelling. In mathematics, they were tested for numeration, mental and written computation, word problems, and numerical reasoning. To explore perception and memory skills, students were administered formal tests of visual and auditory memory as well as auditory discrimination of sounds. Their responses to reading and to mathematical computations were further considered for evidence of problems in visual discrimination, visual sequencing, and visual spatial areas. Analyses indicated that these learning-disabled/gifted students were significantly weaker than controls in their decoding skills, in spelling, and in most areas of mathematics. They were also significantly weaker in auditory discrimination and memory, and in visual discrimination, sequencing, and spatial abilities. Conclusions are that these underlying perceptual and memory deficits may be related to students' academic problems.     

Influence of auditory-verbal, visual-verbal, visual, and visual-visual processing speed on reading and spelling at the end of Grade 1

This study examined cognitive processing speed through four modalities (auditory-verbal, visual-verbal, visual, and visual-visual) at the end of Grade 1 and how it influences reading and spelling. The subjects were 124 French-speaking children, selected for their contrasting performance on reading and spelling tasks. The children in the first group (N=69) were average readers; the second group of children (N=55) performed worse or much worse on all reading and spelling tasks. The experimental design consisted of a set of 10 tasks administered in two sessions. The major findings reveal that: (1) the children with reading difficulties displayed low and slow performance on most cognitive tasks, whatever the modality; (2) auditory-verbal and visual-verbal processing speed significantly predicted written language, which was not the case with the visual modalities; and (3) that visual problems did not appear to be a potential cause of reading problems in most delayed readers. The findings also confirm the independence of phonological and naming-speed skills in reading development and reading impairment.     

Modelling relations between sensory processing, speech perception, orthographic and phonological ability, and literacy achievement

The general magnocellular theory postulates that dyslexia is the consequence of a multimodal deficit in the processing of transient and dynamic stimuli. In the auditory modality, this deficit has been hypothesized to interfere with accurate speech perception, and subsequently disrupt the development of phonological and later reading and spelling skills. In the visual modality, an analogous problem might interfere with literacy development by affecting orthographic skills. In this prospective longitudinal study, we tested dynamic auditory and visual processing, speech-in-noise perception, phonological ability and orthographic ability in 62 five-year-old preschool children. Predictive relations towards first grade reading and spelling measures were explored and the validity of the global magnocellular model was evaluated using causal path analysis. In particular, we demonstrated that dynamic auditory processing was related to speech perception, which itself was related to phonological awareness. Similarly, dynamic visual processing was related to orthographic ability. Subsequently, phonological awareness, orthographic ability and verbal short-term memory were unique predictors of reading and spelling development.     

Neuropsychological, learning and psychosocial profile of primary school aged children with the velo-cardio-facial syndrome (22q11 deletion): evidence for a nonverbal learning disability?

In this exploratory study, the neuropsychological and learning profile of nine primary school age children with velo-cardio-facial syndrome (VCFS) was studied by systematic neuropsychological testing. In five out of nine children, the following profile was found: a VIQ-PIQ discrepancy (in favor of the VIQ), significantly better scores (.05 level) for reading (decoding) and spelling compared to arithmetic, deficient tactile-perceptual skills (difficulties mainly on the left side of the body), weak but not deficient visual-perceptual abilities, deficient visual-spatial skills, extremely poor psychomotor skills (gross motor skills more deficient than fine motor skills), problems with processing of new and complex material, poor visual attention, good auditory memory and relatively good language skills. These findings correspond to the pattern of neuropsychological assets and deficits that has been described for the syndrome of nonverbal learning disabilities (NLD) (Rourke, 1987, 1988, 1989, 1995). The psychosocial profile of all nine children with VCFS also correspond to that of children with NLD. Further studies on the relationship between cognitive function, behavior, psychiatric disorder and abnormalities in brain anatomy in young people with VCFS will be needed. In clinical practice, it is worthwhile exploring in greater depth the neuropsychological functions of children with VCFS to rule out NLD, since they may benefit from specific remediation following the learning principles of the NLD-treatment.     

Working Memory Interference Control Deficit in Children Referred by Teachers for ADHD Symptoms

In this exploratory study, the neuropsychological and learning profile of nine primary school age children with velo-cardio-facial syndrome (VCFS) was studied by systematic neuropsychological testing. In five out of nine children, the following profile was found: a VIQ-PIQ discrepancy (in favor of the VIQ), significantly better scores (.05 level) for reading (decoding) and spelling compared to arithmetic, deficient tactile-perceptual skills (difficulties mainly on the left side of the body), weak but not deficient visual-perceptual abilities, deficient visual-spatial skills, extremely poor psychomotor skills (gross motor skills more deficient than fine motor skills), problems with processing of new and complex material, poor visual attention, good auditory memory and relatively good language skills. These findings correspond to the pattern of neuropsychological assets and deficits that has been described for the syndrome of nonverbal learning disabilities (NLD) (Rourke, 1987, 1988, 1989, 1995). The psychosocial profile of all nine children with VCFS also correspond to that of children with NLD. Further studies on the relationship between cognitive function, behavior, psychiatric disorder and abnormalities in brain anatomy in young people with VCFS will be needed. In clinical practice, it is worthwhile exploring in greater depth the neuropsychological functions of children with VCFS to rule out NLD, since they may benefit from specific remediation following the learning principles of the NLD-treatment.     

失用性失写的脑机制

失用性失写是指人们知道该如何书写, 但实际上却不会书写或错误书写的一种现象。失用性失写涉及的神经网络包括皮层与皮层连接、皮层与皮质下连接。其中前者包括额-顶叶相关脑区的连接、顶-枕叶相关脑区的连接以及额-顶-枕相关脑区的连接, 这些神经网络分别负责操作空间表征图像、回忆以及检索字母的形状等; 后者包括小脑与额-顶叶的连接、左丘脑-大脑皮质的连接以及纹状体-内囊-左额顶叶的连接等, 它们分别与图像输出程序、书写运动过程、操作指令的执行过程以及字素形成过程等有关。今后的研究应该集中在完善失用性失写的研究范式、深入探讨失用性失写的心理机制以及推动其康复工作的开展等方面。     

Deep agraphia

A case of agraphia is documented which resembles in every respect the pattern of results obtained in deep dyslexia. Pronounceable pseudowords could not be written to dictation while concrete nouns were more accurately transcribed than abstract nouns. Verbs and functions words were written very poorly and oral spelling was impaired. In addition, semantic paragraphias were present. In contrast to the deficit observed in writing, no such impairment was found for reading. It is concluded that writing can occur without phoneme-grapheme conversion and that codes generated for reading are functionally distinct from codes generated for writing.     

Written spelling deficit of Broca''s aphasics

The purpose of this study was to investigate the nature of the written spelling deficit manifested by Broca's aphasics. Four spelling tests were given to eight Broca's aphasic patients. Analysis of misspelling errors led the investigators to conclude that Broca's aphasics do not spell phonically, but rather adhere exclusively to a visual/orthographic strategy. Possible cognitive deficits underlying the spelling problem are identified. The written spelling deficit is related to other features of the syndrome of Broca's aphasia, and finally, speculations are offered concerning the neurological substrate of written spelling in Broca's aphasic patients.     

Developmental Surface Dysgraphia: What Is the Underlying Cognitive Impairment?

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.     

Letter-by-letter reading: natural recovery and response to treatment

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