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Fusiform type alexia: pure alexia for words in contrast to posterior occipital type pure alexia for letters.

OBJECTIVE: To clarify the behavioral differences between patients with pure alexia from different lesions. METHODS: Two patients with pure alexia caused by damage to the fusiform or posterior occipital gyri were given reading and writing tests including kanji (Japanese morphograms) and kana (Japanese phonetic writing). RESULTS: Patient 1 (pure alexia from a fusiform gyrus lesion) had difficulty reading both kanji and kana, with kanji reading more impaired, and imageability and visual complexity effects (imageable or less complex words/characters were read better than nonimageable or more complex words/characters), whereas patient 2 (pure alexia from a posterior occipital gyri lesion) showed selective impairment of kana reading. CONCLUSION: Pure alexia for kanji (and kana; fusiform type) is characterized by impairments of both whole-word reading, as represented in kanji reading, and letter identification, and is different from pure alexia for kana (posterior occipital type) in which letter identification is primarily impaired. Thus, fusiform type pure alexia should be designated pure alexia for words, whereas posterior occipital type pure alexia should be designated pure alexia for letters.

Aged↗

Visual complexity in letter-by-letter reading: "pure" alexia is not pure.

Standard accounts of pure alexia have favoured the view that this acquired disorder of reading arises from damage to a left posterior occipital cortex mechanism dedicated to the processing of alphanumeric symbols. We challenge these accounts in two experiments and demonstrate that patients with this reading deficit are also impaired at object identification. In the first experiment, we show that a single subject, EL, who shows all the hallmark features of pure alexia, is impaired at picture identification across a large set of stimuli. As the visual complexity of pictures increases, so EL's reaction time to identify the stimuli increases disproportionately relative to the control subjects. In the second experiment, we confirm these findings with a larger group of five pure alexic patients using a selected subset of high- and low-visual complexity pictures. These findings suggest that the deficit giving rise to pure alexia is not restricted to orthographic symbols per se but, rather, is a consequence of damage to a more general-purpose visual processing mechanism.

Adult↗

Left hemisphere pathways in reading: inferences from pure alexia without hemianopia.

In pure alexia, reading is impaired despite almost normal speech, spelling, and writing. We studied a right-handed man with pure alexia, but no hemianopia. He had more difficulty reading longer words (word-length effect), but had no selective reading impairment in phonologic or semantic analysis. Clinical-CT correlation suggests that (1) left hemisphere visual pathways crucial for reading arise from or pass close to the left occipitotemporal or inferior temporal gyrus, and (2) relevant transcallosal fibers from the right hemisphere course inferior to the posterior horn of the left lateral ventricle before ascending to left hemisphere language areas.

Aged↗

Visual word activation in pure alexia.

A patient with pure alexia (DM) is shown to perform rapid and accurate lexical decisions for common words without the ability to recover their complete identity. We provide evidence using a speeded decision task that DM is not forced to rely on a laborious analysis of individual letter forms when judging the lexical status of orthographic patterns varying in length, though he clearly must use this approach to fully identify a word for explicit report. By contrast, the ability to rapidly classify a word apparently does not extend to judgements of its superordinate category. DM makes semantic decisions for visual words by adopting the same inefficient procedure he uses for verbal report of their identity. The results provide further constraints on the functional deficit responsible for pure alexia. We argue that DM is able to monitor the overall activation of word units without achieving full identification and that such a process may be a characteristic of the normal reading mechanism.

Adult↗

Music alexia in a patient with mild pure alexia: disturbed visual perception of nonverbal meaningful figures.

A 26-year-old female pianist suffered from an intracerebral hematoma caused by an arteriovenous malformation of the left occipital parasplenial region, which was operated on seven months after the onset. Incomplete right hemianopsia, mild pure alexia, and partially disturbed naming of visual objects persisted several months after the removal of the malformation. Evaluation of musical ability one and three months after surgery showed that her auditory recognition of music was intact. She could sing and play melodies already learned and could dictate well the notes after hearing tones. However, she had difficulty in reading music, especially the pitch of notes, even for simple sequences of 4 notes. In contrast, her rhythm reading was fairly good. Her visual recognition of other symbolic figures like road signs was also markedly impaired. These results suggest that her visual recognition of written music as well as of other symbolic figures underwent a preliminary verbal decoding in the left hemisphere and that pitch reading was more dependent on verbal processing than rhythm reading.

Adult↗

[Pure alexia secondary to progressive multifocal leukoencephalopathy as first manifestation of HIV infection].

INTRODUCTION: Pure alexia, or alexia without agraphia, is usually caused by disconnection between the gyrus angularis and visual areas of both hemispheres. Cerebrovascular disease is the most frequent cause of this syndrome. CLINICAL CASE: We report the first case of a patient with pure alexia secondary to progressive multifocal leukoencephalopathy (PML) as the first manifestation of Human Immunodeficiency Virus (HIV) infection. Polymerase chain reaction in cerebrospinal fluid was positive for JC virus. Despite favorable immunological and virological response to highly active antiretroviral therapy, neurological symptoms and demyelinating lesions progressed during the first months, and right homonyous hemianopsia, acalculia, and color anomia were noted. In magnetic resonance imaging (MRI) demyelinating lesions involved white matter of both occipital lobes, with left predominance, forceps major and splenium of the corpus callosum, together with white matter of the left temporal lobe and both hippocampal regions. During the following months, coinciding cidofovir treatment, the patient had partial clinical improvement, but neurological deficits persisted. The size of MRI lesions significantly improved. CONCLUSIONS: PML is frequently the first manifestation of AIDS (acquired immunodeficiency syndrome). It should be considered as a possible diagnosis of pure alexia.

Alexia, Pure↗

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↗

Rapid word identification in pure alexia is lexical but not semantic.

Following the notion that patients with pure alexia have access to two distinct reading strategies-letter-by-letter reading and semantic reading-a training program was devised to facilitate reading via semantics in a patient with pure alexia. Training utilized brief stimulus presentations and required category judgments rather than explicit word identification. The training was successful for trained words, but generalized poorly to untrained words. Additional studies involving oral reading of nouns and of functors also resulted in improved reading of trained words. Pseudowords could not be trained to criterion. The results suggest that improved reading can be achieved in pure alexia by pairing rapidly presented words with feedback. Focusing on semantic processing is not essential to this process. It is proposed that the training strengthens connections between the output of visual processing and preexisting orthographic representations.

Dyslexia↗

The efficacy of kinesthetic reading treatment for pure alexia.

This paper presents an effective treatment for pure alexia by a type of single-case design, which we termed a "material-control single-case design" [Sugishita et al., Neuropsychologia, Vol. 31, 559-569, 1993]. Two patients with pure alexia were treated using kinesthetic reading (reading by tracing or copying the outline of each letter with the patient's finger). The results clearly demonstrated that both patients significantly improved their reading and copying performances. Their recovery of reading performance arose from improvement in copying. The results of tachistoscopic reading tests suggested that the patient obtained the ability to read without kinesthetic movements.

Adult↗

Jules Dejerine and his interpretation of pure alexia.

Dejerine's interpretation of pure alexia is routinely mentioned in all neuropsychological textbooks, yet the details of his account and the evidence on which it is based have never been subjected to a critical analysis. We provide such an evaluation in this paper, summarizing the behavioral data that Dejerine presented in his now famous case report and the theoretical framework he adopted to explain the phenomenon of alexia without agraphia. We also provide a link between Dejerine's work and current hypotheses on the nature of the syndrome.

Brain↗

The functional anatomy of single-word reading in patients with hemianopic and pure alexia.

We investigated single-word reading in normal subjects and patients with alexia following a left occipital infarct, using PET. The most posterior brain region to show a lateralized response was at the left occipitotemporal junction, in the inferior temporal gyrus. This region was activated when normal subjects, patients with hemianopic alexia and patients with an incomplete right homonymous hemianopia, but no reading deficit, viewed single words presented at increasing rates. This same area was damaged in a patient with pure alexia ("alexia without agraphia") and no hemianopia, who read words slowly using a letter-by-letter strategy. Although the exact level of the functional deficit is controversial, pure alexia is the result of an inability to map a percept of all the letters in a familiar letter string on to the mental representation of the whole word form. However, the commonest deficit associated with "pure" alexia is a right homonymous field defect; an impairment that may, by itself, interfere with single-word reading because of inability to see the letters towards the end of a word. The relative contributions of pure and hemianopic alexia in individual patients needs to be assessed, as the latter has been shown to respond well to specific rehabilitation programmes.

Adult↗

Large acute cerebral hemorrhage presenting with pure alexia.

Alexia (the acquired inability to read) is an uncommon presenting complaint in the emergency department (ED). It is usually associated with a lesion in the brain located within the dominant hemisphere near the parieto-occipital junction, with some involvement of the splenium of the corpus callosum. Our review of the literature revealed multiple distinct causes for the finding of alexia, and a majority of the cases uncovered also involved agraphia (the acquired inability to write) and frequently visual field defects. We present the case of an otherwise healthy 35-year-old white man who arrived at our ED with the chief complaint of having difficulty reading. He was, however, able to write, and he exhibited no defects in his visual fields on gross testing in our ED. The patient was found to have a large, acute, intraparenchymal hemorrhage in the right posterior/inferior parietal cortex, very near the occipital lobe. We present this case, followed by a brief discussion, to heighten awareness of the complaint of alexia with or without agraphia as a possible presenting symptom of intracranial hemorrhage, or ischemic cerebrovascular accident.

Adult↗