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Alexia caused by a fusiform or posterior inferior temporal lesion.

We evaluated the alexia and agraphia of three patients with different lesions using Japanese kanji (morphograms) and kana (phonograms) and made a lesion-to-symptom analysis. Patient 1 (pure alexia for both kanji and kana and minor agraphia for kanji after a fusiform lesion) made more paragraphic errors for kanji, whereas patient 2 (alexia with agraphia for kanji after a posterior inferior temporal lesion) showed severe reading and writing disturbances and more agraphic errors for kanji. Brodmann Area 37 was affected in both patients, but in patient 2 the lesion was located lateral to that in patient 1. Patient 3 showed agraphia without alexia after restricted lesion to the angular gyrus. We believe that pure alexia (patient 1) results from a disconnection between the medial fusiform gyrus and posterior inferior temporal area (the lateral fusiform and inferior temporal gyri), whereas alexia with agraphia for kanji (patient 2), corresponding to lexical agraphia in Western countries, results from damage to the posterior inferior temporal area, in which whole-word images of words are thought to be stored. Furthermore, restricted lesion in the angular gyrus (patient 3) does not produce alexia; the alexic symptom of "angular" alexia with agraphia may be the result of damage to the adjacent lateral occipital gyri.

Aged↗

The role of the posterior fusiform gyrus in reading.

Studies of skilled reading [Price, C. J., & Mechelli, A. Reading and reading disturbance. Current Opinion in Neurobiology, 15, 231-238, 2005], its acquisition in children [Shaywitz, B. A., Shaywitz, S. E., Pugh, K. R., Mencl, W. E., Fulbright, R. K., Skudlarski, P., et al. Disruption of posterior brain systems for reading in children with developmental dyslexia. Biological Psychiatry, 52, 101-110, 2002; Turkeltaub, P. E., Gareau, L., Flowers, D. L., Zeffiro, T. A., & Eden, G. F. Development of neural mechanisms for reading. Nature Neuroscience, 6, 767-773, 2003], and its impairment in patients with pure alexia [Leff, A. P., Crewes, H., Plant, G. T., Scott, S. K., Kennard, C., & Wise, R. J. The functional anatomy of single word reading in patients with hemianopic and pure alexia. Brain, 124, 510-521, 2001] all highlight the importance of the left posterior fusiform cortex in visual word recognition. We used visual masked priming and functional magnetic resonance imaging to elucidate the specific functional contribution of this region to reading and found that (1) unlike words, repetition of pseudowords ("solst-solst") did not produce a neural priming effect in this region, (2) orthographically related words such as "corner-corn" did produce a neural priming effect, but (3) this orthographic priming effect was reduced when prime-target pairs were semantically related ("teacher-teach"). These findings conflict with the notion of stored visual word forms and instead suggest that this region acts as an interface between visual form information and higher order stimulus properties such as its associated sound and meaning. More importantly, this function is not specific to reading but is also engaged when processing any meaningful visual stimulus.

Adolescent↗

The eye movements of Japanese pure alexic patients during single word and nonword reading.

Two Japanese patients with pure alexia, SH and YH, who showed right homonymous hemianopia following a left occipital lobe lesion, demonstrated letter-by-letter (LBL) reading in pronouncing Japanese kana words and nonwords. In contrast to alphabetic letters, each Japanese kana character has an invariant and identical pronunciation whether it appears in isolation or as a component of any word and nonword string. It is important to investigate the eye movements as well as reading latency and duration in Japanese-speaking LBL readers. Relative to normal controls, these patients demonstrated a more robust string-length effect, which was characterized by larger increases in reading latency and duration as well as in the number of fixations as the string length increased. We propose that in pure alexia, parallel activation of orthographic representations is abnormally delayed but not completely abolished.

Aged↗

Pure somaesthetic alexia: somaesthetic-verbal disconnection for letters.

We studied a patient who manifested a bilateral reading disorder through the somaesthetic modality, without deficit of elementary tactile sensation or tactile object naming, due to a left parietal infarct. Detailed investigation established the following points. (i) The patient showed normal function on elementary somaesthetic examination, normal function on high level tactile perception, except for minimal impairment of the right hand on the two-point discrimination test, and normal latencies on the somatosensory evoked potential in both hands. (ii) The patient had difficulty in reading letters using any somaesthetic strategy (graphaesthesia, directional joint kinaesthesia and active touch) with either hand. (iii) On a same-different judgement task, the patient's performance with the right hand was slightly defective on graphaesthesia and active touch, but performance with the left hand was within the normal range for all of the strategies. The patient's disorder was highly category specific and modality specific, indicating that somaesthetic letter reading can be disrupted not only independent of other high level somaesthetic functions, like object recognition, but also independent of other modes of reading functions, such as visual reading. A lesion involving the intraparietal sulcus, the upper part of the inferior parietal lobule and the adjacent white matter in the left hemisphere may be capable of compromising the pathways for somaesthetic letter reading with both hands.

Agraphia↗

Visual object agnosia and pure word alexia: correlation of functional magnetic resonance imaging and lesion localization.

We present a case of a 64-year-old, right-handed female with a metastatic breast cancer lesion involving the left posterior inferior temporal lobe causing complete loss of the ability to recognize visually common objects and words. After her symptoms resolved on corticosteroid therapy, functional magnetic resonance imaging (fMRI) mapping demonstrated strong left-hemispheric dominance for word recognition and right-hemispheric dominance for object recognition. The case illustrates the relationships among ventral occipito-temporal cortical activation, lesion localization, and lesion-induced deficits of higher visual function. The relationship between hemispheric dominance determined by fMRI and risk of postoperative deficit depends on the specific visual function of interest.

Agnosia↗

Clinical management of agnosia.

Agnosia is a neurological recognition deficit that affects a single modality. Visual agnosias include pure object agnosia, prosopagnosia, akinetopsia, and pure alexia. Auditory agnosias include pure word deafness, phonagnosia, and pure sound agnosia. New neuroimaging tools have permitted scientists to better understand the loci of lesions that cause various agnosias and from that knowledge to develop theories about the processing networks that contribute to perception and recognition in each modality. These research data, in turn, inform the rehabilitation process. By utilizing current knowledge about neuroprocessing networks, clinical professionals can differentially diagnose agnosias from aphasia and other cognitive deficits. Practical approaches to treatment of agnosia will follow once the diagnosis is established.

Agnosia↗

Residual reading capability in a patient with alexia without agraphia.

A case of pure alexia due to an ischemic lesion of the occipital temporal region is described. Written words could be matched but not read. Immediate memory span for graphemes was defective. The reading defect probably depends on the inability to modify the written word "globally"; the phonological process was intact, but the memory disturbance impeded reading. The dissociation is explained by the preservation of word forms, which are linked to the semantic stage. Non-written stimuli trigger a "meaning" which evokes the word form and so the written word is recognized even though it cannot be read.

Agnosia↗

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↗

Auditory repetition priming is impaired in pure alexic patients.

Alexia without agraphia, or "pure" alexia, is an acquired impairment in reading that leaves writing skills intact. Repetition priming for visually presented words is diminished in pure alexia. However, it is not possible to verify whether this priming deficit is modality-specific or modality independent because reading abilities are compromised. Hence, auditory repetition priming was assessed with lexical decision and word stem completion tasks in pure alexic patients with lesions in left inferior temporal-occipital cortex and the splenium. Perceptually based, modality-specific priming models predict intact auditory priming, since auditory association cortex is spared in the patients. Alternatively, modality-independent models, which suggest that priming reflects the temporary modification of an amodal system, might predict impairments. Baseline performance was matched in the patients and controls, although lexical decision priming measures showed an interaction between group and repetition lag. The patients showed intact immediate priming but significantly less priming than controls at longer delays. Furthermore, word stem completion priming was abolished in the patients. One explanation for the deficit is that left inferior temporal-occipital cortex supports amodal aspects of priming, as suggested by recent neuroimaging results. Another possibility is that long-term auditory priming relies on covert orthographic representations which were unavailable in the patients. The results provide support for interactive models of word identification.

Aged↗

Alexia without agraphia or hemianopia.

The case of a right-handed man with pure alexia without hemianopia due to intraparenchymal haematoma is reported. Ct scan and nuclear magnetic resonance showed a lesion in the lateral temporo-occipital area confirming the assumption that the anatomic pathways responsible for reading run close to the lateral temporo-occipital or inferior temporal gyrus.

Agraphia↗

[Alexia without agraphia. Anatomical basis and physiopathological mechanisms].

Clinical, neuropsychological and radiological signs were studied in a patient suffering from pure alexia associated with right superior quadrantanopia. The lesion responsible for the defects was located in the periventricular white substance at the level of the left inferior occipitotemporal convolutions. These structures seem to constitute the pathway of the visual information channelled from the two hemispheres towards the language centres. The lesion therefore disconnects the angular gyrus from its visual information and gives rise to alexia without agraphia.

Aged↗

[Interhemispheric disconnection syndrome in glioma of the posterior part of the corpus callosum].

The syndrome of interhemispheric disconnexion has been systematically researched in 10 cases of medial and posterior callosal tumours. The cases have been documented with CT Scann and two of them were anatomically verified. Two had a complete disconnexion syndrome; another, whose lesion was situated on the forceps major, had no sign of disconnexion. In the others, the semiology was proportional to the extent of the tumour, sometimes minimal and in this case, only corresponding to an extinction of the left ear in the dichotic test, which therefore appeared in our series as the most constant sign of a transfer-trouble. Among the other signs, left tactile anomia was found in two patients, left ideomotor apraxia in three, left visual anomia in five. Seven patients had a right constructional apraxia, which was bilateral in five of them. Left agraphia was noted in five patients, among which three had also a right hand dysgraphia, whose physiopathology is discussed. Tactile alexia was noted in three patients, two had a trouble of somesthesic information's transfer, one had a pure alexia (left occipital lesion plus splenium). Signs of hemispheric independence were uncommon: one patient presented a "foreign-hand sign".

Agraphia↗

Deep dyslexic phenomena in a letter-by-letter reader.

Numerous accounts of pure alexia have suggested that prelexical impairment precludes rapid access to orthographic information in patients with the disorder. We report a patient with features of both pure and partially recovered deep dyslexia in whom we demonstrate prelexical deficits in maintaining a reliable abstract representation of the right side of letter arrays, as well as in modulating a "spotlight" of visual attention. These deficits, we suggest, encourage the patients's use of a letter-by-letter reading strategy; despite them, however, he demonstrates rapid, accurate reading of some, but not all classes of words. Furthermore, the patient's reading is influenced by both prelexical and lexical-semantic factors such that speed and accuracy are optimal for high imagiability nouns of few letters. Finally, the patient accurately names orally spelled words of all classes. Taken together, these data are consistent with the hypothesis that rapid reading may be enabled by lexical-semantic support from a right hemisphere-mediated processing system which recognizes words as whole, thereby mitigating the effect of the prelexical deficits.

Brain Ischemia↗

Impaired reading in patients with right hemianopia.

A left occipital stroke may result in alexia for two reasons, which may coexist depending on the distribution of the lesion. A lesion of the left lateroventral prestriate cortex or its afferents impairs word recognition ("pure" alexia). If the left primary visual cortex or its afferents are destroyed, resulting in a complete right homonymous hemianopia, rightward saccades during text reading are disrupted ("hemianopic" alexia). By using functional imaging, we showed two separate but interdependent systems involved in reading. The first, subserving word recognition, involved the representation of foveal vision in the left and right primary visual cortex and the ventral prestriate cortex. The second system, responsible for the planning and execution of reading saccades, consisted of the representation of right parafoveal vision in the left visual cortex, the bilateral posterior parietal cortex (left > right), and the frontal eye fields (right > left). Disruption of this distributed neural system was demonstrated in patients with severe right homonymous hemianopia, commensurate with their inability to perform normal reading eye movements. Text reading, before processes involved in comprehension, requires the integration of perceptual and motor processes. We have demonstrated these distributed neural systems in normal readers and have shown how a right homonymous hemianopia disrupts the motor preparation of reading saccades during text reading.

Adult↗

[Alexia with agraphia produced by localized infarction in the inferior posterior region of the left temporal lobe].

There have been several reports on alexia with agraphia due to hemorrhage or trauma in the inferior posterior region of the left temporal lobe since Yamadori (1982) first reported a case of cerebral hemorrhage. We presented the first case of alexia with agraphia due to a circumscribed infarct in the inferior posterior region of the left temporal lobe. A 65-year-old right-handed man had an acute onset of inability to read Japanese "kana" letters and to write any letters. He had no difficulty in speaking or understanding. Neurological examination on admission was normal except for homonymous quadrantanopia in the right upper field. Neuropsychological findings: Spontaneous speech was fluent without dysprosody. There were no disturbances in auditory understanding or in repetition. He could read Japanese "kanji" characters correctly, however, he could not read any "kana" letters. Writing was also disturbed severely even as to his address, name or telephone number. Calculation was also difficult. There were no disturbances in naming colors, no ideomotor or constructional apraxia nor visual agnosia. X-ray computed tomography (CT) scan showed a faint low density area with ring enhancement suggesting a cerebral infarct in the inferior posterior region of the left temporal lobe. Patient's hospital course was characterized by a marked improvement of alexia, especially of "kana" letters as compared with that of "kanji" characters. Dissociative improvement of alexia compared with agraphia in this case could be explained by the fact that the lesion was in close contact with the occipital lobe and that he also had pure alexia in the early stage.

Agraphia↗

Brain activations during letter-by-letter reading: a follow-up study.

Lesions affecting the ventral cortex of the left temporal lobe commonly yield a selective reading impairment known as pure alexia. It is thought to result from the disruption or deafferentation of the Visual Word Form Area (VWFA), a region in the left lateral occipitotemporal sulcus activated whenever normal subjects are viewing alphabetic strings. Most pure alexic patients retain the ability to identify single letters, and develop a strategy of letter-by-letter (LBL) reading. We recently studied fMRI activations in LBL readers and clarified the underlying mechanisms. However, LBL reading is a dynamic process which may improve over months or years of practice, although the cerebral bases of this continuing improvement are currently unknown. We had the opportunity to run the same behavioural testing and fMRI experiment a second time in an alexic patient, 8 months after collecting the data reported by Cohen et al. [Cohen, L., Henry, C., Dehaene, S., Molko, N., Lehéricy, S., Martinaud, O., Lemer, C., and Ferrieux, S. (2004). The pathophysiology of letter-by-letter reading. Neuropsychologia, 42, 1768-1780]. We analyze the changes that occurred over this period in the pattern of reading-related activations, while the patient's LBL reading improved. The activation level decreased in most of the overall network between the two sessions. This general trend contrasted with a focal increase restricted to specific left frontal and parietal areas. When studying the contrast between words and consonant strings, which may be taken as a correlate of LBL reading, we also found a general decrease, except for similar left frontal and parietal regions, which showed a significant increase. We suggest that the pattern of evolution fits with the minimal hypothesis of normal strategic abilities and skill learning, associated with perceptual tuning in right-hemispheric structures able to substitute the disrupted VWFA.

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↗