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The case history of a fifty seven year old Kannada-English bilingual who suddenly lost his ability to read is presented. The relative severity of the reading disorder at various levels, in Kannada and English, as well as its recovery is described. The possible reasons for the findings as well as their implications for the interpretation of alexia as a total inability to read, are discussed.
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In pure alexia, acquired inability to read contrasts with normal speech and handwriting. Rare cases of right hemisphere lesions causing this syndrome are usually attributed to right hemisphere dominance for language. After infarction of the right occipital lobe and thalamus, a fully right-handed man became alexia, but language and spontaneous and dictated writing were intact. Left hemispatial neglect and constructional disturbances were marked, and we suggest that pure alexia was mimicked by the impairment in visuospatial perception. Injury of the nondominant hemisphere and thalamus together may have been important in causing these deficits.
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.
A patient with alexia without agraphia (pure alexia) is reported. Bedside examination of the visual fields disclosed no abnormalities; but perimetric examination demonstrated an incomplete right homonymous hemianopsia with some sparing of the peripheral part of the visual field. The literature on pure alexia without hemianopsia is reviewed and it is concluded that this syndrome has hardly been described with certainity.
Even though the PET study revealed a total infarct in the territory of the left PCA in our 3 cases of pure alexia, it is still obscure which part of the left occipital lobe is most closely associated with the occurrence of the pure alexia. In order to elucidate the intralobar localization of the pure alexia, it is needed to have an ideal case who shows an pure alexia due to the localized lesion within the left occipital lobe. Furthermore, high-resolution PET scanner will circumvent the problem in detecting the metabolism and blood flow in the corpus callosum which plays an important role in the pathogenesis. We have shown that the occlusion of the right PCA also produced a left unilateral agnosia which is one of the common neurological signs in the right MCA infarction. To tell whether the responsible lesion for the unilateral spatial agnosia differs between the PCA occlusion and the MCA occlusion, the correlation study should be carried out in a greater number of the subjects. Two distinctive neuropsychological manifestations, cerebral color blindness and prosopagnosia, have been considered to be produced by the bilateral occipital lesion. The PET studies disclosed reduction of blood flow and oxygen metabolism in both occipital lobes in our particular patient who exhibited cerebral color blindness and prosopagnosia.
In 1892, the French neurologist Jules Déjerine suggested that pure alexia resulted from an occipital lesion that selectively disconnected visual input from a region of the brain that housed "optical images of words." In this issue of Neuron, Gaillard and colleagues offer evidence consistent with Déjerine's proposal and provide new insights to the functional role of the "visual word form area."
Two patients with pure alexia were studied with tachistoscopically presented stimuli to examine factors influencing their ability to distinguish words from nonwords and to derive semantic information at exposures too brief for explicit letter identification. Both patients had profound right hemianopia and computerized tomography (CT) evidence of splenial destruction. Both patients were successful in making word/nonword decisions for high-frequency, but not low-frequency, words. They could judge semantic class membership reliably for such common categories as animals and vegetables, but not for arbitrarily selected categories, such as office-related items. Judgments about the gender of people's names and place versus person name distinctions were made with high reliability. Results are interpreted as evidence for limited word recognition and semantic-processing capacity in the right hemisphere.
BACKGROUND: The two most common types of acquired reading disorder resulting from damage to the territory of the dominant posterior cerebral artery are hemianopic and pure alexia. Patients with pronounced hemianopic alexia have a right homonymous hemianopia that encroaches into central or parafoveal vision; they read individual words well, but generate inefficient reading saccades when reading along a line of text. Patients with pure alexia also often have a hemianopia but are more disabled, making frequent errors on individual words; they have sustained damage to a brain region that supports efficient word identification. OBJECTIVE: To investigate the differences in lesion site between hemianopic alexia and pure alexia groups, as rehabilitative techniques differ between the two conditions. METHODS: High-resolution magnetic resonance images were obtained from seven patients with hemianopic alexia and from six patients with pure alexia caused by a left occipital stroke. The boundary of each lesion was defined and lesion volumes were then transformed into a standard stereotactic space so that regional comparisons could be made. RESULTS: The two patient groups did not differ in terms of damage to the medial left occipital lobe, but those with pure alexia had additional lateral damage to the posterior fusiform gyrus and adjacent tissue. CONCLUSIONS: Clinicians will be able to predict the type of reading disorder patients with left occipital lesions have from simple tests of reading speed and the distribution of damage to the left occipital lobe on brain imaging. This information will aid management decisions, including recommendations for reading rehabilitation.
Pure alexia, following an infarction in the distribution of the left posterior cerebral artery, is attributed to damage of the left occipital lobe and the splenium of the corpus callosum. We describe a case of pure alexia in a 57-year-old woman with infarction of the left lateral geniculate body and the splenium of the corpus callosum, a variation on this classic disconnection syndrome.
Modern concepts of pure alexia and alexia with agraphia are derived from Dejerine's eloquent clinicopathologic studies of the late 19th century. More recently, a third variety of alexia has been described in association with left frontal lesions causing Broca's aphasia. Dejerine also recognized this "third alexia." For Dejerine, alexia with Broca's aphasia was indispensible to his view of a left-hemisphere language zone in which cortical lesions disrupt all language modalities (speech, reading, and writing). Viewed in light of modern neurolinguistic advances, Dejerine's descriptions of the third alexia are surprisingly prescient.
Patients with posterior cortical atrophy may have dorsal visual system (occipital-parietal) dysfunction (optic ataxia, visuospatial disorientation, and simultanagnosia), ventral visual system (occipital-temporal) dysfunction (pure alexia, prosopagnosia, visual anomia, and agnosia), or both. We report a professional musician with ventral system dysfunction whose first symptom was alexia for music. Subsequently, she developed pure alexia for words but had preserved sorting of words. These observations suggest that the ventral visual system is important in music and word reading. However, sorting of words may be mediated by the dorsal visual system.
Visual recognition disturbances are caused by lesions that affect visual cortex as well as white matter connections between visual cortex and temporal and parietal cortex. Homonymous visual field defects are often present but do not explain the recognition difficulty. In "alexia without agraphia" (pure alexia), the intact right visual cortex is disconnected from the left parietal language center by a lesion in the splenium. In "prosopagnosia," visual cortex is disconnected bilaterally from temporal cortex. In simultanagnosia, visual association cortex is damaged. The first condition is most commonly caused by left posterior cerebral artery occlusion, the second by bilateral posterior cerebral artery occlusion or head trauma, and the third by watershed infarction, tumors, abscesses, head trauma, leukoencephalopathies and Alzheimer's disease. When such disorders are suspected, the examiner should supplement the routine visual examination with suggested screening maneuvers.
Since Dejerine reported cases of alexia with agraphia in 1891 and of pure alexia in 1892, it is generally said that the former may occur due to the lesion of the left angular gyrus and the latter due to that of the medial inferior area of the left occipital lobe. In this article, we reported a case of alexia with agraphia who had the main lesion in the medial inferior area of the occipital lobe of the left hemisphere. A 62-year-old right-handed male showed alexia with agraphia. CT scan and single photon emission CT revealed the main lesion in the medial occipital area on the left side. Alexia with agraphia of the patient was characterized as follows: with regard to reading, though his recognition of forms as letter was nearly spared, he could neither read letters or words nor differentiate Kana- from Kanji-letters. Paralexic errors included confusion of Kana and Kanji. He manifested no kinesthetic facilitation in reading. Regarding writing, his disturbances were more severe in Kanji-writing, but there were paragraphia and difficulty of letter-form evocation even in Kana-writing. He could not write spontaneously or to dictation. His copying of letters was also disturbed. Since it is said that there is no difference between Kana- and Kanji-disturbance in Japanese pure alexics, an aspect of alexia of the patient may be common to pure alexia.(ABSTRACT TRUNCATED AT 250 WORDS)