Dissociations of writing and praxis: Two cases in point

For normal writing it is essential that both motoric and linguistic competence be present; disruption of one or the other of these faculties may result in qualitatively different types of agraphia. Two right-handed patients became agraphic after left hemisphere lesions; pure apraxic agraphia in the absence of limb apraxia developed in one patient and pure linguistic agraphia in association with severe ideomotor limb apraxia in the other. The performance of these patients not only serves to illustrate the dissociation between the motoric and linguistic faculties that underlie writing but also confirms that ideomotor limb apraxia and apraxic agraphia are distinct and dissociable entities.     

Progressive agraphia can be a harbinger of degenerative dementia

By investigating three patients with progressive agraphia, we explored the possibility that this entity is an early sign of degenerative dementia. Initially, these patients complained primarily of difficulties writing Kanji (Japanese morphograms) while other language and cognitive impairments were relatively milder. Impairments in writing Kana (Japanese syllabograms), verbal language, executive function, visuo- and visuospatial cognition and memory were identified by neuropsychological testing. The agraphia was compatible with a peripheral type, based on deficits at the interface between the central letter selection and the graphemic motor execution (Patient 1) or at the stage of central letter selection as well (Patients 2 and 3). Agraphia was generally more prominent, although not exclusive, for Kanji probably because of later acquisition and larger total number of Kanji letters leading to lower frequency of use and familiarity per letter. Concurrent or subsequent emergence of non-fluent aphasia, ideomotor apraxia, executive dysfunction and asymmetric akinetic-rigid syndrome in two patients suggested degenerative processes involving the parietal-occipital-temporal regions, basal ganglia and striato-frontal projections. We propose that progressive agraphia may be one of the early symptoms of degenerative dementia such as corticobasal degeneration.     

Multiple component agraphia in a patient with atypical cerebral dominance: an error analysis

A 52-year-old man with atypical cerebral dominance (left-handed for writing but mixed handedness for other tasks) suffered an extensive right hemisphere stroke, resulting in a combination of deficits that has not been previously reported. There were profound visual constructive and visual perceptual disturbances and a spatial agraphia, which were consistent with a nondominant hemisphere lesion. There was also a severe apraxic agraphia, which is typically associated with a dominant hemisphere lesion, but no other signs of dominant hemisphere dysfunction such as linguistic disturbance or limb-motor apraxia were present. This case serves to highlight the functional and anatomical relationship between handwriting and other forms of praxis; the various sources of error in letter formation; the need to be specific in labeling and describing agraphias ; and the role of a detailed analysis of writing errors in delineating the neuropsychological processes involved in handwriting.     

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.     

Cerebellar-induced apraxic agraphia: a review and three new cases

Apraxic agraphia is a writing disorder due to a loss or lack of access to motor engrams that program the movements necessary to produce letters. Clinical and functional neuroimaging studies have demonstrated that the neural network responsible for writing includes the superior parietal region and the dorsolateral and medial premotor cortex. Recent studies of two cases with atypical lesion localisations in the left thalamus and the right cerebellum support the hypothesis that the written language network is larger than previously assumed. The aim of this study is twofold: (1) to provide a survey of cases of apraxic agraphia published between 1973 and June 2010, and (2) to provide further evidence for a role of the cerebellum in writing via three additional cases who presented with apraxic agraphia after ischemic damage in the cerebellum. Functional neuroimaging studies by means of brain perfusion SPECT showed perfusional deficits in the anatomoclinically suspected supratentorial areas, subserving language dynamics, syntax, naming, writing and executive functioning.     

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.     

The transcoding processes in patients with agraphia to dictation

The main purpose of the present study is to prove that the mechanism of transcoding, that is, of transposition of certain units of a sign system into the corresponding ones of another sign system, is an independent mechanism. We attempted to demonstrate this thesis with reference to certain transcoding processes within the verbal function of writing to dictation, in particular, by examining the agraphic disturbances in aphasics on this form of writing. We found that this type of agraphia in patients with an intact input stage (i.e., intact auditory and visual word perception) but with a blocked shift from the phonetic to the graphemic level (even in cases with intact auditory and visual word perception), or in those with a blocked shift to the grapho-motor one (even in cases with intact copying), is due to disturbed mechanisms of transcoding. The fact that in patients with pure writing-to-dictation disability copied writing is not disturbed proves that the transcoding process is selectively blocked. Transcoding processes in copying and total agraphia are discussed by comparison.     

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。     

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.     

Varieties of alexia from fusiform, posterior inferior temporal and posterior occipital gyrus lesions

Reading impairments of three alexia patients, two pure alexia and one alexia with agraphia, due to different lesions were examined quantitatively, using Kanji (Japanese morphogram) words, Kana (Japanese phonetic writing) words and Kana nonwords. Kana nonword reading was impaired in all three patients, suggesting that widespread areas in the affected occipital and occipitotemporal cortices were recruited in reading Kana characters (corresponding to European syllables). In addition, the findings in patient 1 (pure alexia for Kanji and Kana from a fusiform and lateral occipital gyri lesion) and patient 2 (pure alexia for Kana from a posterior occipital gyri lesion) suggested that pure alexia could be divided into two types, i.e. ventromedial type in which whole-word reading, together with letter identification, is primarily impaired because of a disconnection of word-form images from early visual analysis, and posterior type in which letter identification is cardinally impaired. Another type of alexia, alexia with agraphia for Kanji from a posterior inferior temporal cortex lesion (patient 3), results from deficient whole-word images of words per se, and thus should be designated "orthographic alexia with agraphia". To account for these impairments, a weighted dual-route hypothesis for reading is suggested.     

Pure agraphia and Gerstmann's syndrome as a visuospatial-language dissociation: an experimental case study

A right-handed man suffered a left parieto-occipital cerebral infarction, causing agraphia with Gerstmann's syndrome but without major aphasia, alexia, or apraxia. Oral spelling was superior to written spelling. Experiments were performed involving (1) analysis of errors in writing, (2) tasks of visual imagery, and (3) identifying letters drawn without leaving a visual trace. The results suggest that the agraphia and Gerstmann's syndrome are due to a dissociation of language skills and visuospatial skills caused by a dominant parieto-occipital lesion.     

Squaring the round: An unusual progressive graphomotor impairment with post‐mortem findings

Investigations of neurodegenerative disorders may reveal functional relationships in the cognitive system. C.S. was a 63‐year‐old right‐handed man with post‐mortem confirmed Pick's disease with a range of progressive impairments including non‐fluent aphasia, speech, limb, oculomotor, and buccofacial apraxia, but mostly intact intelligence, perception, orientation, memory, semantics, and phonology. During progression, agrammatism in writing with impairments in syntactic comprehension emerged in parallel with an unusual graphomotor deficit in drawing and writing, with an increasing deterioration of graphic short‐term memory. We investigated C.S.'s graphomotor deficit longitudinally using tests of writing and drawing on letters, words, and sentences and drawing to command and copying. We also tested C.S.'s short‐term graphemic buffer experimentally. Analysis showed deficits on selective aspects of graphomotor implementation of writing and drawing, mainly affecting the production of circles and curves, but not short straight lines in drawing and writing, and graphomotor short‐term memory, which paralleled impairments of written syntax and syntactic comprehension. We believe this to be the first detailed analysis of such an unusual progressive impairment in graphomotor production, which may be related to problems with agrammatic agraphia and impairments affecting shared components of cognition reflecting damage to shared neural networks. Alternatively, they may simply reflect the effects of coincidental damage to separate mechanisms responsible for aspects of writing, drawing, and syntactic processing. Longitudinal investigations of emerging deficits in progressive conditions like C.S.'s provides an opportunity to examine the progressive emergence of symptoms in an individual with multiple progressive impairments as they appear and examine putative relationships between them.     

The use of abstract graphemic information in lexical access

Three experiments investigated the nature of the information required for the lexical access of visual words. A four-field masking procedure was used, in which the presentation of consecutive prime and target letter strings was preceded and followed by presentations of a pattern mask. This procedure prevented subjects from identifying, and thus intentionally using, prime information. Experiment I extablished the existence of a semantic priming effect on target identification, demonstrating the lexical access of primes under these conditions. It also showed a word repetition effect independent of letter case. Experiment II tested whether this repetition effect was due to the activation of graphemic or phonemic information. The graphemic and phonemic similarity of primes and targets was varied. No evidence for phonemic priming was found, although a graphemic priming effect, independent of the physical similarity of the stimuli, was obtained. Finally Experiment III demonstrated that, irrespective of whether the prime was a word or a nonword, graphemic priming was equally effective. In both Experiments II and III, however, the word repetition effect was stronger than the graphemic priming effect. It is argued that facilitation from graphemic priming was due to the prime activating a target representation coded for abstract (non-visual) graphemic features, such as letter identities. The extra facilitation from same identity priming was attributed to semantic as well as graphemic activation. The implications of these results for models of word recognition are discussed.     

Multiple levels of letter representation in written spelling: evidence from a single case of dysgraphia with multiple deficits

In this paper, we report a detailed analysis of the impaired performance of a dysgraphic individual, AD, who produced similar rates of letter-level errors in written spelling, oral spelling, and typing. We found that the distribution of various letter error types displayed a distinct pattern in written spelling on the one hand and in oral spelling and typing on the other. In particular, noncontextual letter substitution errors (i.e., errors in which the erroneous letter that replaces the target letter does not occur elsewhere within the word) were virtually absent in oral spelling and typing and mainly found in written spelling. In contrast, letter deletion errors and multiple-letter errors were typically found in oral spelling and very exceptional in written spelling. Only contextual letter substitution errors (i.e., errors in which the erroneous letter that replaces the target letter is identical to a letter occurring earlier or later in the word) were found in similar proportions in the three tasks. We argue that these contrasting patterns of letter error distribution result from damage to two distinct levels of letter representation and processing within the spelling system, namely, the amodal graphemic representation held in the graphemic buffer and the letter form representation computed by subsequent writing-specific processes. Then, we examined the relationship between error and target in the letter substitution errors produced in written and oral spelling and found evidence that distinct types of letter representation are processed at each of the hypothetized levels of damage: symbolic letter representation at the graphemic level and representation of the component graphic strokes at the letter form processing level.     

Is lesion of Exner's area linked to progressive agraphia in amyotrophic lateral sclerosis with dementia? An autopsy case report

Agraphia, as a neuropsychological symptom of ALS, especially ALS with dementia (ALS-D), has recently attracted more attention. However, the brain lesion responsible has not been identified. Here we present an autopsy case of ALS-D of a patient with obvious agraphia, without aphasia, that also presented cerebrospinal degeneration with TDP-43-pathology compatible with ALS-D. Of the pre-motor frontal lobe cortices, degeneration and immuno-histochemical pathology were most obvious in the caudal area of the left middle frontal gyrus, or Exner's area. Assuring this area plays a pivotal role in the kanji and kana formation used in writing the Japanese language, this case of ALS-D showed both agraphia and Exner's area stressed pathological lesions. It may thus be the first case to indicate an intimate relationship between the neuropsychological symptoms and an associated lesion for ALS-D.     

The effect of phonemic processing on the retention of graphemic representations for words and nonwords

The three experiments reported in this study were each conducted in two phases. The first phase of Experiment 1 involved a same-different comparison task requiring “same” responses for both mixed-case (e.g., MAIN main) and pure-case (e.g., near near) pairs. This was followed by Phase 2, a surprise recognition test in which a graphemic effect on word retention was indicated by the superior recognition accuracy obtained for pure-case compared with mixed-case pairs. The first phases of Experiments 2 and 3 involved pronounceability and imageability judgment tasks, respectively. Graphemic retention was assessed by contrasting recognition accuracy for letter strings presented, during Phase 2, in their original Phase 1 case, with letter strings presented, during Phase 2, in. a graphemically dissimilar new case. The experiments provided evidence that there was minimal retention of the graphemic representations from which the phonemic representations of words are generated and, further, that the locus of this effect is probably postlexical. Nonwords were recognized more accurately than words in all three experiments. The latter result was attributed to differences between nonwords and words in both graphemic retention and semantic distinctiveness.     

Cognitive resource limitations during sentence comprehension in Parkinson's disease

We report a single-case study of peripherally acquired dyslexia that meets the clinical criteria of "alexia without agraphia." The patient, AA, has a large infarct involving the left posterior cerebral artery. The most striking feature is a severe impairment in recognizing single visually presented letters that precludes explicit or implicit access to reading, even in a letter-by-letter fashion. AA can, however, differentiate letters from similar nonsense characters and digits, and he is also able to identify alphanumeric signs when the visual channel is bypassed (through somesthesic or kinesthesic presentation). Spelling tasks are also well performed. Since there is a breakdown in mapping a visually presented letter to its abstract graphemic representation, we propose the term "visuographemic alexia" for this kind of reading disorder. The pattern of deficits is interpreted following theoretical models previously developed in cognitive neuropsychology. An alexia for arabic numerals with preserved comprehension lends additional support for the crucial processing of different notational systems (e.g., phonographic vs logographic). More general perceptive disorders do not seem to account for these patterns; they are material-specific. Finally, we attempt to specify functional correlations with the implied neural networks.     

Intransigent vowel-consonant position in Korean dysgraphia: evidence of spatial-constructive representation

Dysgraphia due to a focal brain lesion can be characterized by substitution, transposition, deletion and/or addition errors of graphemes or strokes. However, those linguistic errors can be language-specific because the writing system of a given language may influence error patterns. We investigated a Korean stroke patient, a 57-year-old English teacher with dysgraphia both in Korean Han-geul [see text] and in English alphabet writings. The results of an experimental testing revealed transposition errors between a consonant and a vowel only in English but not in Korean writings. This austerity of vowel-consonant position may be attributed to a unique Korean writing system of a spatially well-formed syllabic configuration or block with consonant(s) and a vowel. In light of a neuropsychological model of writing, which depicts a multi-level spelling and writing process, we suggest a spatial-constructional component of internal orthographic representations in Korean writing. This Korean graphemic configuration feature may be resistant to a focal, left cerebral damage, and thus, we also discuss our results in terms of cerebral lateralization of the writing processes.