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Pure alexia: clinical-pathologic evidence for a lateralized visual language association cortex.

Traditional views of pure alexia have held that the disorder results from a disconnection between the secondary visual cortices of both hemispheres and the angular gyrus of the dominant hemisphere. Evidence has accumulated, however, suggesting the importance of the posterior inferior temporal area in visual language processing. We describe clinical-pathological support for the presence of a lateralized visual language association area residing in the dominant posterior inferior temporal lobe.

Dyslexia, Acquired↗
PubMed1992

Pure alexia and right hemiachromatopsia in posterior dementia.

A 66 year old, right handed woman presented with pure alexia and right hemiachromatopsia (PARH) in the context of a posterior dementia. PARH was accompanied by prosopagnosia, 2-D object agnosia, and environmental agnosia. Visual fields were normal to confrontation testing. The pathological anatomy of PARH involves circumscribed damage to the lingual and fusiform gyri and paraventricular white matter of the left occipital cortex, two contiguous cortical regions functionally specialised for processing colour and word form information, respectively.

Aged↗
PubMed1990

The evolution of pure alexia: a longitudinal study of recovery.

This case report documents the partial recovery, over a 12-month period, of pure alexia in an adult female following a left occipital infarction. Measures of speed and accuracy were obtained on an oral reading and a lexical decision task immediately postonset and then on 10 subsequent occasions. Explicit letter-by-letter reading was observed only during the first week poststroke but a significant effect of word length was seen in all testing sessions. Reading accuracy was relatively good at all stages and reading latency showed a remarkable decrease over time but did not reach normal reading rates. The inability to use higher-order orthographic knowledge, as manifest in the absence of a word superiority effect, was still noted at one year postonset. We therefore concluded that the change in behavior was attributable to increased proficiency in the use of the adaptive letter-by-letter procedure rather than to the resolution of the underlying deficit. It is suggested that longitudinal neurobehavioral studies add to our understanding of the alexic deficit and provide insight into the recovery process.

Adult↗
PubMed2004

Visual command hallucinations in a patient with pure alexia.

Around 25% of patients with visual hallucinations secondary to eye disease report hallucinations of text. The hallucinated text conveys little if any meaning, typically consisting of individual letters, words, or nonsense letter strings (orthographic hallucinations). A patient is described with textual visual hallucinations of a very different linguistic content following bilateral occipito-temporal infarcts. The hallucinations consisted of grammatically correct, meaningful written sentences or phrases, often in the second person and with a threatening and command-like nature (syntacto-semantic visual hallucinations). A detailed phenomenological interview and visual psychophysical testing were undertaken. The patient showed a classical ventral occipito-temporal syndrome with achromatopsia, prosopagnosia, and associative visual agnosia. Of particular significance was the presence of pure alexia. Illusions of colour induced by monochromatic gratings and a novel motion-direction illusion were also observed, both consistent with the residual capacities of the patient's spared visual cortex. The content of orthographic visual hallucinations matches the known specialisations of an area in the left posterior fusiform gyrus--the visual word form area (VWFA)--suggesting the two are related. The VWFA is unlikely to be responsible for the syntacto-semantic hallucinations described here as the patient had a pure alexic syndrome, a known consequence of VWFA lesions. Syntacto-semantic visual hallucinations may represent a separate category of textual hallucinations related to the cortical network implicated in the auditory hallucinations of schizophrenia.

Aged↗
PubMed1998

Rehabilitation of a case of pure alexia: exploiting residual abilities.

We present a case study of a 43-year-old woman with chronic and stable pure alexia. Using a multiple baseline design we report the results of two different interventions to improve reading. First, a restitutive treatment approach using an implicit semantic access strategy was attempted. This approach was designed to exploit privileged access to lexical-semantic representations and met with little success. Treatment was then switched to a substitutive treatment strategy, which involved using the patient's finger to pretend to copy the letters in words and sentences. Reading using this motor cross-cuing strategy was 100% accurate and doubled in speed after 4 weeks of intervention. We propose that this patient's inability to benefit from the implicit semantic access treatment approach may be in part related to her inability to suppress the segmental letter identification process of word recognition.

Adult↗
PubMed1991

[Central visual fields in pure alexia "without hemianopsia"--visual dysfunction in the right hemifield, and alexia for "kana" words in the left].

Following a left occipito-temporal subcortical hematoma, a 57-year-old, right-handed man developed pure alexia that was more prominent in kana words, especially in kana nonwords, than in kanji letters. Although a kinetic perimetry with a Goldmann perimeter showed his visual fields to be full, a static perimetry with a Humphrey visual field analyzer disclosed decreased visual sensitivity in the right visual field in its central 30 degrees. In addition, a tachnistoscopic examination with Landolt rings revealed his visual acuity (the ability of two points discrimination) to have decreased in the right half of his central visual filed in its 3 degrees. In the right central vision, he was unable to recognize the letters, pictures or colors presented by the tachistoscope. Concerning the reading, the more letters in kana words or the higher the number of strokes in kanji letters, the more difficulty he experienced in orally reading both kana and kanji. On the contrary, in the left central vision, kanji reading was not so affected by an increased number of strokes as the kana-word reading which became difficult when the number of the letters increased. CT scan showed subcortical hematoma in the left occipitotemporal region. Magnetic resonance imaging 3 months after onset revealed a localized injury in the region between the left postero-inferior temporal lobe and the infero-lateral occipital lobe, including the fusiform gyrus. None of the splenium, the lingual gyrus or the optic area were affected. The spared structures also included the angular gyrus, the Wernicke area and their subcortical white matter.(ABSTRACT TRUNCATED AT 250 WORDS)

Cerebral Hemorrhage↗
PubMed1994

Pure alexia: attempted rehabilitation and its implications for interpretation of the deficit.

On the basis of data indicating the failure to encode letters as abstract orthographic identities in a pure alexic patient (D.M.) coupled with hypotheses about the effect of such a failure on word reading, an attempt at changing the nature of letter processing in D.M. was conducted. The training procedures failed to produce any fundamental change in the operations used by D.M. to encode isolated letters or words. However, rapid and massive benefits occurred in the overall speed of reading as a result of the training program. These appear to result from an increased rate of letter identification and the faster integration of individual letters into letter combinations. The observations gathered throughout this rehabilitation attempt provided evidence which constrains the range of possible explanations for the characteristic features of pure alexia. It is proposed that the letter-by-letter reading procedure which is the hallmark of the disorder may follow from an incapacity to directly encode visual letters as abstract orthographic types.

Adult↗
PubMed1999

Can treatment for pure alexia improve letter-by-letter reading speed without sacrificing accuracy?

An experimental treatment study designed to improve both the accuracy and the speed of reading was administered to a patient with pure alexia and impaired letter naming. The study focused on the use of letter-by-letter reading. A two-stage approach was employed. The first stage implemented a tactile-kinesthetic strategy to improve accuracy. The second stage concentrated on speed. At the end of the treatment, patient DL was reading both trained and untrained words more accurately and with considerably greater speed than prior to treatment. Accuracy and speed of reading at the sentence level improved as well.

Aged↗
PubMed2006

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↗
PubMed1998

Optic aphasia with pure alexia: a mild form of visual associative agnosia? A case study.

A single-case study is reported of a naming disorder selective to the visual modality. The patient showed intact access to structural knowledge of objects and letters, but impaired access to complete semantic knowledge of objects and alphabetical knowledge of letters from visual input. The impairment was most striking when the patient had to discriminate between semantically similar objects or within a given symbolic repertoire, i.e. letters. The co-occurrence of a partial deficit of visual recognition for objects and for letters indicated features of optic aphasia and pure alexia. This symmetric performance between object and letter processing may also constitute a mild form of visual associative agnosia.

Aged↗
PubMed2003

Mechanisms of pure alexia: spatially based impairment, letter identification deficit, or both?

We studied reading performance for words and for isolated letters in a pure alexic patient. She performed reasonably well when naming isolated letters but was slower in reading letters than a control subject when reaction times (RTs) were recorded. When the patient read isolated letters, RTs were slower for a subset of letters that cannot be recognized from their left part alone (e.g. "b", an ambiguous letter, could be read "b" "h" "l" or "k" whereas "a" has no predictable confounders). We observed a significant positive correlation between the RTs for reading a word and the mean RTs for reading each of its composing letters before its uniqueness point (i.e. the point, when reading from the left to the right, where a word cannot be a word other than the one it is). This result suggests that, in our patient, the letter identification deficit can account for the slow, letter-by-letter reading behaviour, insofar as each letter represents a perceptual problem. Our findings can be accounted for by a deficit in the parallel processing of the left and right parts of each letter, compounded with a bias to process first the left part of the letter, and may thus reconcile the hypotheses of spatially-based deficit (Rapp and Caramazza, 1991) and of a perceptual deficit occurring at the letter identification level (Behrmann and Shallice, 1995; Perri et al., 1996).

Aged↗
PubMed1993

Single-character processing in a case of pure alexia.

The processing of single characters in a pure alexic patient was studied in an attempt to identify the impairment responsible for his reading disorder. Observations from Experiments 1 to 4 suggested a deficit of identification of alphanumeric stimuli without any impairment affecting the elaboration of a structural description of visual stimulation. Experiment 5 indicated that the identification disorder results from a defect in the selective processes--activation and/or inhibition--that must come into play for achieving an appropriate match between a structural description of the stimulation and representations of the identities of known stimuli. The possible implications of this deficit in single-character identification for word reading are discussed.

Adult↗
PubMed1988

Internal representations and the conceptual operation of color in pure alexia with color naming defects.

This research examined the structure of internal representation and the conceptual operation of color in two pure alexic cases (Case I and Case II) with color naming defects. Experiment I investigated the structure of the internal representation of different kinds of colors using a similarity judgment task. Experiment II examined categorical judgments of perceived colors using a two-alternative-forced choice task. Experiment III tested the classification of perceived colors using a color sorting task. The performance of Case I essentially fell within the normal range while the results of Case II showed some impairment in the conceptual operation of color. Analysis of the responses obtained from these experiments indicated that the color naming defects in Case I can be explained in terms of visual-verbal disconnection. However, the naming defects in Case II reflect disfunction in some other higher cortical processes coupled with visual-verbal disconnection.

Color Perception↗
PubMed1996

Reading speed in pure alexia.

This study investigated possible causes of differences in reading speed between two alexic patients who read words letter by letter. As both patients appeared to rely on serial left-to-right processing of letters within words, the difference in reading speed did not seem to be related to any differences in the extent to which the patients could recognize letters in words in parallel or 'ends-in'. Differences in reading speed also seemed to be unrelated to the patients ability to identify individual letters since their letter recognition accuracy was very similar. Furthermore, although patient PD was significantly slower at reading words aloud than patient DC, PD was in fact significantly quicker than DC on a test that has previously been used to assess letter recognition skills in letter-by-letter readers. It is therefore concluded that PD reads words more slowly because of an additional impairment at the level of the word form system. The results therefore reinforce the distinction between Type 1 and Type 2 letter-by-letter readers that was first drawn by Patterson and Kay.

Adult↗