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The role of level of representation in the use of paired associate learning for rehabilitation of alexia.

Patients with phonological alexia (difficulty reading pseudowords) frequently have concomitant difficulty reading functor words and verbs compared with concrete nouns. The current study compares two techniques for helping two patients with phonological alexia regain the ability to read functors and verbs. One technique follows the approach of reorganization of function, while the other relies on the stimulation approach. Study 1, employing a reorganization approach, resulted in both patients increasing their reading accuracy from approximately 10 to 90% or greater. Study 2, using a stimulation approach, resulted in significant improvement, however neither patient was able to achieve accuracy greater than 59%. Study 3 reverted back to the reorganization approach using the same words from Study 2. Both patients demonstrated significant success, achieving 90% or greater accuracy. Whereas the reorganization approach meets with far greater success than the stimulation approach, both approaches can be seen as instances of paired associate learning. An explanation of the advantage of the reorganization approach is developed which focuses on the nature of the pairings in the paired associate learning paradigm: it is proposed that pairings within the same level of representation are easier to learn than pairings that cut across levels of representation.

Aged↗

Musical alexia for rhythm notation: a discrepancy between pitch and rhythm.

In the process of reading music, the reading of rhythm and pitch might be differentiated, although there is no evidence of this to date. There have been cases of disorders restricted to the reading of pitch, but none in which the disorder has been restricted to the reading of rhythm. We present a case of musical alexia and agraphia with Wernicke's aphasia. An in-depth assessment of the subject's musical reading ability showed that her musical alexia was restricted to unfamiliar melodies. When a melody was divided into rhythm elements and pitch elements, pitch reading was preserved, but rhythm reading was severely disturbed. This is the first case reported of a disorder restricted to rhythm reading, and suggests the independence of rhythm reading and pitch reading.

Aphasia, Wernicke↗

Evidence for preserved reading in 'pure alexia'.

We describe 4 patients who developed pure alexia after infarctions of the left cerebral hemisphere. All subjects employed a letter-by-letter strategy (with varying degrees of success) to explicitly identify visually presented words. Although all 4 subjects explicitly denied that they could identify briefly presented words, they all performed significantly better than chance on lexical decision and forced-choice semantic categorization tasks with briefly presented words which they could not explicitly identify. Three subjects regained the ability to explicitly identify briefly presented words; these subjects were more accurate with nouns than functors and words of high as compared with low imageability. Additionally, these subjects were impaired in the processing of suffixes. These data are not accommodated by the 'disconnection' account of pure alexia but are more consistent with the hypothesis that reading in these patients is mediated by the right hemisphere.

Adult↗

Reading in pure alexia. The effect of strategy.

A number of investigators have demonstrated that patients with pure alexia comprehend briefly presented words which they are unable to explicitly identify. We suggested previously that these patients may read by means of two distinct procedures: a laborious letter-by-letter method and a 'whole-word' procedure which, at least initially, does not support explicit word identification. We report a test of this proposal in a patient with pure alexia. We reasoned that if the patient had access to two distinct and incompatible procedures, he might be induced to switch from one to the other by changing task demands. We found that when instructed to name words, the patient employed a letter-by-letter strategy; in contrast, when instructed to make lexical decision or semantic judgements about rapidly presented words, he appeared to use a 'whole-word' strategy. These data support the hypothesis that two distinct procedures are available to this patient. We argue, further, that is necessary to suppress use of the letter-by-letter strategy to demonstrate whole word reading capability in pure alexics, and that failure to do so may account for negative findings in other cases reported in the literature.

Cerebral Infarction↗

Knowledge, implicit knowledge and metaknowledge in visual agnosia and pure alexia.

Residual or implicit knowledge has been observed in patients with object agnosia, optic aphasia and pure alexia. Previous investigators have considered implicit knowledge in these patients to be dissociated from awareness on the basis of intact semantic capabilities that are consistent with right hemisphere processing. The absence of explicit verbal identification is presumably dependent upon damaged left hemisphere systems. We describe a 72-year-old woman with a left occipital infarction, object agnosia and pure alexia who was unable to explicitly identify visual stimuli (objects and words), but was able to make reliable judgements of her residual knowledge on forced-choice matching tasks. While the patient could not consistently demonstrate awareness of knowledge prior to stimulus matching ('Do you know what this is?'), she was able to reliably demonstrate awareness of knowledge for response accuracy ('Are you sure?') assessed after stimulus matching. Further, the extent of the patient's metaknowledge corresponded to her degree of preserved knowledge. We propose that this pattern of performance suggests limited or partial access to preserved semantic knowledge which, though degraded, is not 'non-conscious'.

Aged↗

Ideogram reading in alexia.

A case of alexia with agraphia in a Japanese patient is presented. Reading difficulty was severe in words composed of phonograms (Kana), while reading of words composed of Ideograms (Kanji) was better preserved. Writing was severely impaired in both types of characters. Occlusion of the angular branch of the left middle cerebral artery was demonstrated by carotid arteriography and was considered responsible for the symptoms. Two additional cases of alexia with agraphia from the Japanese literature are reviewed. Their linguistic features were similar to the present case. A hypothesis of a functional disconnexion between visual and auditory-oral systems is proposed to explain why Kana processing was more severly affected than Kanji processing.

Agraphia↗

Patients with hemianopic alexia adopt an inefficient eye movement strategy when reading text.

Patients with an acquired homonymous hemianopia often adapt over a period of a few months to compensate for some of the impairments caused by their visual field defect. Changes in their eye movement patterns have been demonstrated as performance on visual tasks improves with time; however, these patients often complain of persistent text reading problems. Using a video-based eye-movement tracking system, we investigated the text reading behaviour of patients with established hemianopic alexia (>6 months post deficit), a condition affecting left-to-right readers, with a homonymous field defect that encroaches into their right foveal/parafoveal visual field. Word-based analyses of text reading are standard in experiments involving normal readers, but this is the first time these methods have been extended to patients with hemianopic alexia. Using this method, we compared the patients' reading scanpaths to those generated by normal controls reading the same passages, and a random model generated by matching the patients' eye movement data to random permutations of the text they read. We demonstrate that patients adopt an inefficient reading strategy, fixating to the left of the preferred viewing location of words of four letters and longer. Fixating to the left of the normal preferred viewing location not only results in less of the fixated word being processed by the language system; ensuing fixations are also more likely to land within the same word (a refixation). It is this refixation rate that is the main factor in slowing reading times in these patients. Our data suggests that patients are able to extract some useful visual information from text to aid the planning of reading scanpaths as their behaviour differs critically from the random model. Potential reasons for this patient group failing to produce an effective reading strategy are discussed.

Adult↗

Visual word recognition in the left and right hemispheres: anatomical and functional correlates of peripheral alexias.

According to a simple anatomical and functional model of word reading, letters displayed in one hemifield are first analysed through a cascade of contralateral retinotopic areas, which compute increasingly abstract representations. Eventually, an invariant representation of letter identities is created in the visual word form area (VWFA), reproducibly located within the left occipito-temporal sulcus. The VWFA then projects to structures involved in phonological or lexico-semantic processing. This model yields detailed predictions on the reading impairments that may follow left occipitotemporal lesions. Those predictions were confronted to behavioural, anatomical and functional MRI data gathered in normals and in patients suffering from left posterior cerebral artery infarcts. In normal subjects, alphabetic stimuli activated both the VWFA and the right-hemispheric symmetrical region (R-VWFA) relative to fixation, but only the VWFA showed a preference for alphabetic strings over simple chequerboards. The comparison of normalized brain lesions with reading-induced activations showed that the critical lesion site for the classical syndrome of pure alexia can be tightly localized to the VWFA. Reading impairments resulting from deafferentation of an intact VWFA from right- or left-hemispheric input were dissected using the same methods, shedding light on the connectivity of the VWFA. Finally, the putative role of right-hemispheric processing in the letter-by-letter reading strategy was clarified. In a letter-by-letter reader, the R-VWFA assumed some of the functional properties normally specific to the VWFA. These data corroborate our initial model of normal word perception and underline that an alternative right-hemispheric pathway can underlie functional recovery from alexia.

Adult↗

The neural bases of prosopagnosia and pure alexia: recent insights from functional neuroimaging.

PURPOSE OF REVIEW: To discuss whether recent functional neuroimaging results can account for clinical phenomenology in visual associative agnosias. RECENT FINDINGS: Functional neuroimaging studies in healthy human subjects have identified only two regions of ventral occipitotemporal cortex that invariantly respond to individual faces and visual words, respectively. The signature of face identity coding in the fusiform neural response was shown to be missing in a patient with prosopagnosia. Another case study established that a surgical lesion close to the region sensitive to visual words can result in pure alexia. SUMMARY: Evidence is increasing that functional specialization for processing face identity and visual word forms is restricted to two specialized sensory modules in the occipitotemporal cortex. A structural or functional lesion to face-sensitive and word-sensitive regions in the ventral occipitotemporal cortex can provide the most parsimonious account for the clinical syndromes of prosopagnosia and agnosic alexia. This review suggests that functional specialization should be considered in terms of whether exclusively one brain region (instead of many) underpins a defined function and not as whether this brain region underpins exclusively one cognitive function. Such functional specialization seems to exist for at least two higher-order visual perceptual functions, face and word identification.

Dyslexia↗

Neuroimaging findings in a patient recovering from global alexia to spelling dyslexia.

The authors report findings in a 67-year-old right-handed man who had an ischemic infarct in the territory of the left posterior cerebral artery. The clinical manifestation consisted mainly of total alexia without agraphia. The patient gradually recovered, subsequently showing the syndrome of spelling dyslexia. Cerebral MR-images revealed a circumscript infarction of medial and basal parts of left temporal lobe. In the acute stage [99mTc]HM-PAO SPECT was characterized by a diminished uptake in the definitely infarcted area and hyperfixation in the region of the left forceps major. Because high retention of HM-PAO indicates potentially salvageable tissue after an ischemic event, the depicted area might be correlated with the recovery of function. Thus, the authors' neuroimaging data give further support to the assumption that the left forceps major is a critical area for global alexia, whereas spelling dyslexia is due to involvement of the left medio-basal temporal lobe.

Aged↗

Alexia without agraphia associated with right occipital lesion.

A 78 year old, right handed man developed the syndrome of alexia without agraphia due to a right occipital thrombotic stroke. The cerebral dominance test strongly suggests that his right hemisphere is dominant. This is believed to be the first case of alexia without agraphia secondary to a right occipital lesion in a right handed person.

Aged↗

Phonological alexia: three dissociations.

Three dissociations were observed in a case of alexia: a disturbance of reading, without comparable disturbance of oral expression, oral comprehension, writing, or spelling aloud; a disturbance of the phonological reading process, without disturbance of the non-phonological reading process; a disturbance located at the level of the phonological stage, without disturbance of the perceptual and expressive stages. This pattern of results has been called phonological alexia.

Brain Neoplasms↗

Alexia with agraphia of kanji (Japanese morphograms).

The case of the right-handed young Japanese woman with alexia with agraphia of kanji (the Japanese morphograms) due to a small circumscribed haematoma in the left posterior inferior temporal gyrus is described. Her chief complaint was the inability to read and write kanji. Detailed examination showed that her alexia with agraphia was much more predominant for kanji than kana (the Japanese syllabograms). These facts suggest that the processing of kanji and kana involves different intrahemispheric mechanisms.

Adult↗

Naming difficulties in alexia with agraphia for kanji after a left posterior inferior temporal lesion.

The case is described of a patient with alexia and agraphia for kanji, and severe anomia after a subcortical haemorrhage in the left posterior inferior temporal area. Magnetic resonance imaging at four months after onset showed a lesion in the inferior temporal and fusiform gyri, extending from the temporo-occipital junction toward the anterior third of the temporal lobe. Comparison with other reported cases of alexia with agraphia and anomia made it clear that when accompanied by severe anomia, the lesions extended either forward to the anterior part of the middle temporal gyrus or medially to the parahippocampal gyrus. It is suggested that the disconnection of association fibres between the parahippocampal, fusiform, middle, and inferior temporal gyri, especially between the parahippocampal gyrus and the other temporal gyri, or the cortical damage to the posterior part of these gyri is essential for the production of anomia.

Aged↗

Acquired alexia with agraphia syndrome in childhood.

The acquired alexia with agraphia syndrome is a conspicuous disorder of reading and writing in the absence of significant other language impairments that has mainly been recorded in adults. Pure cases are rare, with most patients displaying mild aphasic deficits. In children, acquired reading and writing disorders are generally reported as part of more encompassing aphasic syndromes affecting oral and written language equally, for example, Broca or Wernicke aphasia. Documented instances of predominant acquired reading and writing disorders in childhood are exceptional. We report an 11-year-old, right-handed boy who sustained a left temporoparieto-occipital hematoma following rupture of an arteriovenous malformation and who consecutively presented with the acquired alexia with agraphia syndrome associated with word-finding difficulties. Neuropsychologic and neurolinguistic data showed that there was no concomitant Gerstmann and/or angular gyrus syndrome. Th e recoveryfrom the anomia was quite favorable, but recovery of written language was more protracted and acted on the patient's further scholastic achievement. This case is reminiscent of a historical childhood case reported in 1939 and is consonant with adult cases in terms of lesion location and semiologic picture.

Agraphia↗

Alexia without agraphia.

This typical case of alexia without agraphia, caused by an astrocytoma, is the first recorded case in the literature of alexia without agraphia with a right homonymous hemianopia caused by a tumor. This case differs from previously reported cases of the syndrome by its fluctuating clinical course and its slow progression. All but one of previously reported cases had been of vascular or surgical etiology. This course and the associated radiographic findings should differentiate the neoplastic from the vascular etiology of this most fascinating syndrome.

Astrocytoma↗

Reversible alexia, mitochondrial myopathy, and lactic acidemia.

A 11-year-old boy of short stature had recurrent right temporal pounding headaches of 7 months' duration, and progressive visual loss for 3 days. There was a left hemianopia, alexia without agraphia, and diffuse muscle weakness. Investigation established the presence of a mitochondrial myopathy with pyruvate and lactic acidemia and increased serum content of sarcoplasmic enzymes. On treatment with prednisone, the patient's strength and reading skill improved, symptoms resolved, and muscle enzymes returned to normal. Three attempts to reduce steroids resulted in accentuation of symptoms, seizures, weakness, regression of reading skills, and elevation of serum enzymes. The alexia was also reversible.

Child↗

Right unilateral dyscopia of letters in alexia without agraphia.

In two cases of pure alexia with computed tomography (CT) evidence of lesions in the left occipital lobe, the left hand copied letters normally, but the right hand copied defectively. The findings reinforce the disconnection theory of alexia without agraphia.

Aged↗