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.     

Unilateral agraphia after section of the posterior half of the truncus of the corpus callosum

A Japanese patient who had undergone section of the posterior half of the truncus of the corpus callosum in the process of removing an arteriovenous malformation was examined with a battery of special tests. He showed agraphia only with the left hand (unilateral agraphia). The agraphia was more prominent for the words written in Kana characters (phonograms) than for the words written in Kanji characters (ideograms). The patient showed neither unilateral apraxia, nor tactile alexia, nor tactile anomia.     

Pure agraphia in Romaji after left inferior frontal gyrus infarction: A case of selective deficit in syllable-to-grapheme conversion in Japanese

The Japanese writing system employs two distinct categories of characters: Kana and Kanji. The difference between Kana and Kanji writing corresponds roughly to that between phonological and lexical systems in Western languages. When typing, most Japanese use alphabetical characters based purely on phonological rules. In particular, the Romaji system is used, in which a syllable consists of a single vowel, a consonant–vowel compound (e.g., ka, ki), or a sequence of consonant–y(semivowel)–vowel (e.g., kya, kyu). We describe a right-handed Japanese patient who developed pure agraphia that affected Romaji writing but preserved Kana and Kanji writing and who had a lesion in the left pars opercularis of the inferior frontal gyrus that extended to the anterior precentral gyrus. The patient demonstrated literal paragraphia in spelling Romaji across modalities. Our findings suggested that the patient’s agraphia in Romaji after a confined left frontal infarction was manifested by a selective impairment in syllable-to-grapheme conversion.     

Ideogram writing in a disconnection syndrome

A case of disconnection-type agraphia coupled with alexia was reported. The patient showed several asymmetrical manual capacities between the two hands, i.e., dissociated difficulty of Kanji (ideogram) writing between the two hands, left unilateral difficulty of Kana (phonogram) writing, right unilateral dyscopia of letters as well as geometrical figures, and right unilateral difficulty in drawing without a model. Anatomically, lesions involved most of the corpus callosum in its posterior portion including the splenium and the left medial occipital lobe. From these data, a possible liguistic capacity of the right hemisphere was suggested.     

Anatomy of posterior pathways in reading: a reassessment

Contemporary accounts of the neurology of reading stem from Dejerine's original visual-verbal disconnection hypothesis of pure alexia. Reassessment of Dejerine's traditional formulations for posterior left hemisphere pathways in reading suggests that the occipital cortex-left angular gyrus-Wernicke's area scheme is undoubtedly oversimplified. The role of left angular gyrus cortex in reading is unsettled, and although clinically undefined, more inferior portions of the left temporal lobe may also contribute to the reading process. Nevertheless, to a surprising extent, the neuroanatomic edifice erected by Dejerine remains largely intact.     

Subangular alexia without agraphia or hemianopsia

A 40 year old right-handed woman underwent resection of an unruptured vascular malformation which was located just deep to the left posterior insula. Postoperatively she demonstrated a transient syndrome of alexia without agraphia or hemianopsia. Analysis of her postoperative findings, in conjunction all available anatomical data, let to the conclusion that the responsible lesion was in the white matter of the left occipitotemporal region, below the angular gyrus and lateral to the lateral ventricle. Hence the lesion in the present case was truly subangular, and is therefore distinctly differentiated from the classical lesion in alexia without agraphia, which may be described as splenio-occipital.     

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.     

Phonological alexia with optic and tactile anomia: a neuropsychological and anatomical study

We describe a patient with phonological alexia caused by a small hemorrhage in the posterior-inferior portion of the left temporal lobe. The lesion induced a highly selective impairment of phonological reading without concomitant oral language deficits other than anomia for objects presented in the visual and tactile modalities. We propose that an intact dorsal pathway from inferior visual association areas to Wernicke's area via the angular gyrus could mediate reading by the lexical route, while damage to a ventral pathway disrupted the patient's ability to read nonwords. We suggest further that although visually and tactually presented objects could be recognized and both verbally and nonverbally identified, they could not be named because of a disconnection from the area of word representations.     

A functional lesion in developmental dyslexia: left angular gyral blood flow predicts severity.

Functional imaging studies have shown reduced regional cerebral blood flow (rCBF) in temporal and inferior parietal regions in dyslexia. To relate such abnormalities to the severity of dyslexia, correlations between reading skill and rCBF during a series of reading tasks and visual fixation were mapped for 17 right-handed dyslexic men, ages 18-40, and 14 matched controls. These correlations uniquely identified the left angular gyrus as the most probable site of a functional lesion in dyslexia: Here, higher rCBF was associated with better reading skill in controls (p <.01), but with worse reading skill in dyslexia (p <.01). This suggests that greater reliance on this region normally facilitates reading, but impairs reading in dyslexia. Thus, developmental dyslexia may share a common localization with alexia.     

Iconic reading in a case of alexia without agraphia caused by a brain tumor: a tachistoscopic study

The lack of nonverbal reading comprehension in the clinical disconnection syndrome, alexia without agraphia, has been contradictory to the relative reading comprehension of the right hemisphere in split brains. We report a 39-year-old patient with verbal alexia without agraphia caused by brain tumor. On rapid tachistoscopic presentation of object names, he denied seeing anything but showed nonverbal reading comprehension by pointing to the corresponding objects. He lost this ability when he recovered ability to name individual letters of the object names. Our results suggest that even partial verbal reading such as the naming of single letters makes demonstration of iconic reading impossible and that total functional disconnection from verbalization, as initially noted in this case or in split brain studies, is necessary to show nonverbal reading comprehension.     

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.     

Letter-by-letter reading: natural recovery and response to treatment

The present investigation provides a longitudinal study of an individual (RB) with acquired alexia following left posterior cerebral artery stroke. At initial testing, RB exhibited acquired alexia characterized by letter-by-letter (LBL) reading, mild anomic aphasia, and acquired agraphia. Repeated measures of reading accuracy and rate were collected for single words and text over the course of one year, along with probes of naming and spelling abilities. Improvements associated with natural recovery (i.e., without treatment) were documented up to the fourth month post onset, when text reading appeared to be relatively stable. Multiple oral reading (MOR) treatment was initiated at 22 weeks post-stroke, and additional improvements in reading rate and accuracy for text were documented that were greater than those expected on the basis of spontaneous recovery alone. Over the course of one year, reading reaction times for single words improved, and the word-length effect that is the hallmark of LBL reading diminished. RB's response to treatment supports the therapeutic value of MOR treatment to in LBL readers. His residual impairment of reading and spelling one-year post stroke raised the question as to whether further progress was impeded by degraded orthographic knowledge.     

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.     

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.     

Neuroanatomical correlates of oral reading in acute left hemispheric stroke

Oral reading is a complex skill involving the interaction of orthographic, phonological, and semantic processes. Functional imaging studies with nonimpaired adult readers have identified a widely distributed network of frontal, inferior parietal, posterior temporal, and occipital brain regions involved in the task. However, while functional imaging can identify cortical regions engaged in the process under examination, it cannot identify those brain regions essential for the task. The current study aimed to identify those neuroanatomical regions critical for successful oral reading by examining the relationship between word and nonword oral reading deficits and areas of tissue dysfunction in acute stroke. We evaluated 91 patients with left hemisphere ischemic stroke with a test of oral word and nonword reading, and magnetic resonance diffusion-weighted and perfusion-weighted imaging, within 24-48 h of stroke onset. A voxel-wise statistical map showed that impairments in word and nonword reading were associated with a distributed network of brain regions, including the inferior and middle frontal gyri, the middle temporal gyrus, the supramarginal and angular gyri, and the middle occipital gyrus. In addition, lesions associated with word deficits were found to be distributed more frontally, while nonword deficits were associated with lesions distributed more posteriorly.     

A case of literal alexia: evidence for a disconnection syndrome

We describe a patient with literal alexia, agraphia, and only minimal aphasia, resulting from a left occipito-parietal ischemic infarction. Neither impaired visual processing nor impaired oral output could explain the patient's letter reading deficit, since he retained the ability to match letters of mixed case, and remained able to spell aloud and to pronounce and comprehend orally spelled words. His residual word reading was characterized by better performance for nouns and highly imageable words than for functors , poorly imageable words, and nonwords. We propose that this patient's letter reading deficit was due to a partial disconnection of his visual from his linguistic systems, with residual word reading accomplished by a semantically mediated compensatory mechanism. We suggest, further, that the dissociations between word and letter reading observed in some alexic individuals may be attributed, in part, to a difference in the relative contributions of dorsal and ventral occipital structures to the reading process.     

Parietal versus temporal lobe components in spatial cognition: Setting the mid‐point of a horizontal line

Recent anatomo‐clinical correlation studies have extended to the superior temporal gyrus, the right hemisphere lesion sites associated with the left unilateral spatial neglect, in addition to the traditional posterior‐inferior‐parietal localization of the responsible lesion (supramarginal gyrus, at the temporo‐parietal junction). The study aimed at teasing apart, by means of repetitive transcranial magnetic stimulation (rTMS), the contribution of the inferior parietal lobule (angular gyrus versus supramarginal gyrus) and of the superior temporal gyrus of the right hemisphere, in making judgments about the mid‐point of a horizontal line, a widely used task for detecting and investigating spatial neglect. rTMS trains at 25 Hz frequency were delivered over the inferior parietal lobule (angular gyrus and supramarginal gyrus), the superior temporal gyrus and the anterior parietal lobe of the right hemisphere, in 10 neurologically unimpaired participants, performing a line bisection judgment task. rTMS of the inferior parietal lobule at the level of the supramarginal gyrus brought about a rightward error in the bisection judgment, ipsilateral to the side of the rTMS, with stimulation over the other sites being ineffective. The neural correlates of computing the mid‐point of a horizontal segment include the right supramarginal gyrus in the inferior parietal lobule and do not extend to the angular gyrus and the superior temporal gyrus. These rTMS data in unimpaired subjects constrain the evidence from lesion studies in brain‐damaged patients, emphasizing the major role of a subset of relevant regions.     

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.     

Impaired activation of the phonological lexicon: effects upon oral reading

The role of the phonological lexicon in oral reading is examined in a patient with a small focal left hemisphere lesion. Impaired access to the patient's phonological lexicon is suggested by a number of findings, including the production of phonemic errors across a variety of tasks; increasing difficulty in word production with increasing word length; and difficulty on tests of homophone and rhyme judgments. Two competing models of reading are tested: the nonlexical ("rules") and the lexical ("no-rules") models. The rules model predicts that a disturbance in the phonological lexicon will result in surface alexia; the no-rules model predicts phonological alexia. Results indicate that the patient's reading is most similar to phonological alexia, providing support for the no-rules model. The applicability of the no-rules model to other forms of acquired alexia is explored.