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PubMed1996

[Preserved implicit reading and the recovery of explicit reading in a pure alexic].

We described a 55-year-old, right-handed, university-educated Japanese man who showed pure alexia after an infarction in the territory of the left posterior cerebral artery. Damage to the corpus callosum was limited to the most caudal part of the splenium. In the early days of his illness, he demonstrated the inability to read aloud on either Kana (Japanese syllabograms) or Kanji (Japanese morphograms). In contrast, he could semantically categorize Kanji-words such as animals or non-animals. Subsequently, he gradually regained the ability of reading aloud Kanji as well as Kana. At that time, we further investigated the relation between his reading ability and the attributes of 881 Kanji characters including hieroglyphicity, concreteness, and familiarity. We found that he could more readily read Kanjis with higher hieroglyphicity, concreteness, and familiarity. The reading times for Kanji-words were significantly shorter than those of corresponding Kana-words. Moreover, he showed some difficulty to read Kana-words and Kana combinations without meaning when asked to perform repetitive opposing movements with his thumb and little finger inhibiting kinesthetic reading. The effect was not observed when he read Kanji-words. These results lead us to suggest the following: (1) the two components of reading, reading aloud and comprehension abilities, are dissociable in a pure alexic, especially in Kanji reading; and (2) recovery mechanisms underlying Kanji and Kana reading were different in our case. Namely, restitution of the direct connection through the residual splenium fibers between the visual word form processing area in the right hemisphere and the left angular gyrus was attributed to Kanji reading, and utilization of kinesthetic reading played an important role in Kana reading.

Dyslexia, Acquired↗

PubMed1994

Reading with the right hemisphere: evidence from transcranial magnetic stimulation.

Although many patients with dominant hemisphere damage retain some reading ability, the anatomic substrate of the preserved reading remains controversial. We tested the hypothesis that the right hemisphere mediates the reading of some patients with acquired dyslexia by using Transcranial Magnetic Stimulation (TMS) to assess the effect of transient cortical stimulation on reading. A patient with (partially recovered) pure alexia was asked to read aloud briefly presented words, half of which were shown in association with TMS of the right or left hemisphere. Consistent with the right hemisphere reading hypothesis, stimulation of the right but not the left hemisphere disrupted oral reading.

Cerebral Infarction↗

PubMed2001

Does dyslexia develop from learning the alphabet in the wrong hemisphere? A cognitive neuroscience analysis.

A new perspective is described which views developmental dyslexia as the outcome of learning to write the alphabet in the nondominant (right) hemisphere. The letter-level and whole-word subtypes of dyslexia are seen as differing responses adopted to cope with this predicament. Striking similarities between dyslexics and callosotomy patients in the allocation of covert attention to lateralized stimuli provide direction for integrating a diversity of dyslexic research within this framework. This synthesis, together with information from pure alexia, brain activation, and reading research, lends insight into the neural circuitry of the compensatory strategies adopted by the two dyslexic subtypes.

Brain↗

PubMed2001

Hyperacute cognitive stroke syndromes.

Cognitive syndromes are common clinical manifestations of hyperacute stroke and may be the single or dominant presenting features. They are related to acute dysfunction of complex integrated distributed functional networks serving different cognitive domains. The most common cortical syndromes include nonfluent or fluent aphasia, neglect, collor agnosia, pure alexia and Balint's syndrome. Disturbances of declarative memory are common following posterior cerebral artery and thalamic strokes. Abulia can follow thalamic, caudate and capsular lesions. Intraventricular and subarachnoid haemorrhages can cause preeminent neuropsychological changes. Disorientation is present in about 40% of acute stroke patients and delirium complicates the course of 25% of acute strokes. Some hyperacute cognitive stroke syndromes are useful indicators of later disability. Cognitive syndromes may pose special difficulties to neurology residents, unless formal teaching in neuropsychology and psychiatry is included in their training programs.

Acute Disease↗

PubMed1991

Two systems for colour-naming defects: verbal disconnection vs colour imagery disorder.

Two subjects affected by pure alexia and showing no central dyschromatopsia or generalized aphasia, performed poorly on traditional tasks with visually-presented colour stimuli and on tasks with objects presented verbally. Three experiments were conducted to evaluate the possible role of mental colour imagery in recalling the colours of objects from memory. It was concluded that Case I, with left occipital lobe softening, had preserved imagery systems, but failed to recode the colours of mentally generated colour images, just as he failed to name visually presented colours, suggesting a language-imagery disconnection. In contrast, Case II, with a bilateral occipital lesion, had sustained damage to her long-term visual memories for colours as chromatic attributes of objects. This content-specific imagery deficit was concomitant with colour agnosia. The present findings are discussed in terms of current cognitive theories on imagery deficits.

Aged↗

PubMed1988

Cortical blindness caused by acute general cerebral swelling.

A 7-year-old girl who suffered from acute general cerebral swelling as a result of a traffic accident showed cortical blindness. Computed tomography (CT) scan on admission revealed marked slitlike ventricles and narrowing of the perimesencephalic cisterns, which indicated general cerebral swelling. While hospitalized, the patient developed transtentorial herniation twice on day 3, and CT scans at herniation episodes showed disappearance of the perimesencephalic cisterns. After recovery of consciousness, the patient showed cortical blindness, and during gradual recovery she showed pure alexia without agraphia. The visual evoked potentials at 8 weeks, 16 weeks, and 3 years 4 months after trauma showed normalization of the pattern, but revealed left occipital inactivity.

Acute Disease↗

PubMed1986

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.

Animals↗

PubMed2004

Specialization within the ventral stream: the case for the visual word form area.

Is there specialization for visual word recognition within the visual ventral stream of literate human adults? We review the evidence for a specialized "visual word form area" and critically examine some of the arguments recently placed against this hypothesis. Three distinct forms of specialization must be distinguished: functional specialization, reproducible localization, and regional selectivity. Examination of the literature with this theoretical division in mind indicates that reading activates a precise subpart of the left ventral occipitotemporal sulcus, and that patients with pure alexia consistently exhibit lesions of this region (reproducible localization). Second, this region implements processes adequate for reading in a specific script, such as invariance across upper- and lower-case letters, and its lesion results in the selective loss of reading-specific processes (functional specialization). Third, the issue of regional selectivity, namely, the existence of putative cortical patches dedicated to letter and word recognition, cannot be resolved by positron emission tomography or lesion data, but requires high-resolution neuroimaging techniques. The available evidence from single-subject fMRI and intracranial recordings suggests that some cortical sites respond preferentially to letter strings than to other categories of visual stimuli such as faces or objects, though the preference is often relative rather than absolute. We conclude that learning to read results in the progressive development of an inferotemporal region increasingly responsive to visual words, which is aptly named the visual word form area (VWFA).

Dyslexia↗

PubMed2004

The pathophysiology of letter-by-letter reading.

Pure alexia is a frequent and incapacitating consequence of left occipitotemporal lesions. It is thought to result from the disruption or the disconnection of the visual word form area (VWFA), a region reproducibly located within the left occipito-temporal sulcus, and encoding the abstract identity of strings of visual letters. Alexic patients often retain effective single letter recognition abilities, and develop an effortful letter-by-letter reading strategy which is the basis of most rehabilitation techniques. We study a patient who developed letter-by-letter reading following the surgical removal of left occipito-temporal regions. Using anatomical and functional MRI in the patient and in normal controls, we show that alexia resulted from the deafferentation of left fusiform cortex, and we analyze the network of brain regions subtending letter-by-letter reading. We propose that during letter-by-letter reading (1) letters are identified in the intact right-hemispheric visual system, with a central role for the region symetrical to the VWFA; (2) letters are serially transferred to the left hemisphere through the intact segment of the corpus callosum; (3) word identity is eventually recovered in the left hemisphere through verbal working memory processes involving inferior frontal and supramarginal cortex.

Adult↗

PubMed2001

Visual language and handwriting movement: functional magnetic resonance imaging at 3 tesla during generation of ideographic characters.

A functional magnetic resonance imaging experiment at 3 tesla was performed to investigate the collaborative mechanism between visuospatial processing and motor execution in performing visual language generation tasks. Japanese Kanji, ideographic characters, were utilized to design tasks. The bilateral border portions between the inferior parietal lobule and the occipital lobe were involved during a Kanji puzzle task, which required subjects to combine several parts into a Kanji. The higher motor areas, such as the premotor areas and the pre-supplementary motor areas, were also activated bilaterally during the puzzle task. The parieto-occipital activation may be related to analysis of configuration or segmentation/integration of Kanji figures. Activation in the higher motor areas may be induced by cognitive components related to motor function to perform the visuospatial language task, such as intense reference for displayed characters and finding a proper character for puzzle solution. A collaborative mechanism in these areas may explain the effectiveness of tactile reading in letter recognition by patients with pure alexia or kinesthetic facilitation by Kanji users when recalling difficult Kanji.

Adult↗

PubMed2003

The myth of the visual word form area.

Recent functional imaging studies have referred to a posterior region of the left midfusiform gyrus as the "visual word form area" (VWFA). We review the evidence for this claim and argue that neither the neuropsychological nor neuroimaging data are consistent with a cortical region specialized for visual word form representations. Specifically, there are no reported cases of pure alexia who have deficits limited to visual word form processing and damage limited to the left midfusiform. In addition, we present functional imaging data to demonstrate that the so-called VWFA is activated by normal subjects during tasks that do not engage visual word form processing such as naming colors, naming pictures, reading Braille, repeating auditory words, and making manual action responses to pictures of meaningless objects. If the midfusiform region has a single function that underlies all these tasks, then it does not correspond to visual word form processing. On the other hand, if the region participates in several functions as defined by its interactions with other cortical areas, then identifying the neural system sustaining visual word form representations requires identification of the set of regions involved. We conclude that there is no evidence that visual word form representations are subtended by a single patch of neuronal cortex and it is misleading to label the left midfusiform region as the visual word form area.

Cognition↗

PubMed2000

Acquired dyslexia.

Disorders of reading are frequently encountered in patients with acquired cerebral lesions. Investigations in the past few decades have improved our understanding of these disorders. In this article we review the peripheral dyslexias, including neglect dyslexia, attentional dyslexia, and pure alexia (or alexia without agraphia), as well as the "central" dyslexias, including deep, surface, and phonological dyslexia. Current accounts of acquired dyslexia are also discussed. Finally, we briefly describe the reading tasks that serve to differentiate the different reading disorders.

Agraphia↗

PubMed1997

Consciousness of perception after brain damage.

Disturbances of visual perception after brain damage provide clues to understanding consciousness and the brain. In this article we review six visual disorders in which perception and consciousness are dissociated as a result of brain damage: blindsight, implicit shape perception in apperceptive visual agnosia, covert recognition of faces in prosopagnosia, unconscious perception in neglect and extinction, implicit reading in pure alexia, and implicit object recognition in associative visual agnosia. We consider these six disorders from the standpoint of three main schools of thought concerning consciousness and the brain, namely a localized system for consciousness, consciousness as a state of integration, and consciousness as a property of graded representation. The findings suggest that these syndromes do not share a single mechanism and that it is conceivable that more than one explanation will be necessary both within and across syndromes. We conclude on the basis of the current evidence that it is unlikely that any single brain system is necessary for conscious awareness of perception that does not play a role in perception as well.

Agnosia↗

PubMed2005

Using errorless learning to treat letter-by-letter reading: contrasting word versus letter-based therapy.

Some pure alexic readers have been shown to activate lexical and semantic knowledge under brief presentation conditions. This ability is not seen when letter-by-letter reading accuracy is high or the reading impairment is very severe. It is also unlikely to occur under normal untimed presentation because the pure alexic will make deliberate use of their letter-by-letter strategy. This paper presents data from a moderately severe letter-by-letter reader, FD, who had visual processing problems affecting reading. He also had other mild aphasic characteristics. FD showed implicit reading abilities under brief presentation conditions, being able to make lexical decisions and semantic categorisations well above chance. FD was given two therapy programmes, the first, whole word therapy to exploit this implicit ability and the second to improve letter-by-letter accuracy and speed. FD showed some improvement in reading ability after both therapy programmes, particularly for words of personal interest to him. His letter naming accuracy and reading of visually similar words were the most resistant to change. A striking effect of therapy was the cessation of FD's letter-by-letter reading and the emergence of some of the characteristics of deep dyslexia. Even when therapy concentrated on letter accuracy, FD did not revert back to his original letter-by-letter reading strategy. The results are discussed with reference to the two theories of pure alexia. Some conclusions are drawn about the need for therapists to examine and exploit all residual reading skills when devising therapeutic programmes.

Acoustic Stimulation↗

PubMed2005

Left hemispatial visual neglect associated with a combined right occipital and splenial lesion: another disconnection syndrome.

Damage to the left occipital lobe and the splenium or forceps major is often associated with pure alexia, thought to be an occipital-temporoparietal disconnection syndrome. A patient with the parallel lesion, a combined right occipital and splenial lesion, showed severe left-sided visual spatial neglect, but no significant neglect in other sensory modalities. This visual neglect might be related to a disconnection between the visual information processed by the left occipital lobe and the right posterior temporal-inferior parietal areas that mediate attention in the left hemispace.

Aged↗

PubMed2002

Language-specific tuning of visual cortex? Functional properties of the Visual Word Form Area.

The first steps in the process of reading a printed word belong to the domain of visual object perception. They culminate in a representation of letter strings as an ordered set of abstract letter identities, a representation known as the Visual Word Form (VWF). Brain lesions in patients with pure alexia and functional imaging data suggest that the VWF is subtended by a restricted patch of left-hemispheric fusiform cortex, which is reproducibly activated during reading. In order to determine whether the operation of this Visual Word Form Area (VWFA) depends exclusively on the visual features of stimuli, or is influenced by language-dependent parameters, brain activations induced by words, consonant strings and chequerboards were compared in normal subjects using functional MRI (fMRI). Stimuli were presented in the left or right visual hemifield. The VWFA was identified in both a blocked-design experiment and an event-related experiment as a left-hemispheric inferotemporal area showing a stronger activation to alphabetic strings than to chequerboards, and invariant for the spatial location of stimuli. In both experiments, stronger activations of the VWFA to words than to strings of consonants were observed. Considering that the VWFA is equally activated by real words and by readable pseudowords, this result demonstrates that the VWFA is initially plastic and becomes attuned to the orthographic regularities that constrain letter combination during the acquisition of literacy. Additionally, the use of split-field stimulation shed some light on the cerebral bases of the classical right visual field (RVF) advantage in reading. A left occipital extrastriate area was found to be activated by RVF letter strings more than by chequerboards, while no symmetrical region was observed in the right hemisphere. Moreover, activations in the precuneus and the left thalamus were observed when subjects were reading RVF versus left visual field (LVF) words, and are likely to reflect the attentional component of the RVF advantage.

Adult↗

PubMed2005

Letter-by-letter acquired dyslexia is due to the serial encoding of letters.

Letter-by-letter acquired dyslexia (pure alexia) is assumed to be related to the serial encoding of letters, but the evidence for this assumption is somewhat indirect. Here, we demonstrate that the deficit is indeed due to serial encoding by comparing the performance of a letter-by-letter dyslexic reader with the performance of normal readers who were forced to read letter by letter; the data patterns are remarkably similar.

Dyslexia, Acquired↗

PubMed2003

"Bottom-up" and "top-down" effects on reading saccades: a case study.

OBJECTIVE: To investigate the role right foveal/parafoveal sparing plays in reading single words, word arrays, and eye movement patterns in a single case with an incongruous hemianopia. METHODS: The patient, a 48-year-old right handed male with a macular sparing hemianopia in his left eye and a macular splitting hemianopia in his right eye, performed various reading tasks. Single word reading speeds were monitored using a "voice-trigger" system. Eye movements were recorded while reading three passages of text, and PET data were gathered while the subject performed a variety of reading tasks in the camera. RESULTS: The patient was faster at reading single words and text with his left eye compared with his right. A small word length effect was present in his right eye but not his left. His eye movement patterns were more orderly when reading text with his left eye, making fewer saccades. The PET data provided evidence of "top-down" processes involved in reading. Binocular single word reading produced activity in the representation of foveal V1 bilaterally; however, text reading with the left eye only was associated with activation in left but not right parafoveal V1, despite there being visual stimuli in both visual fields. CONCLUSIONS: The presence of a word length effect (typically associated with pure alexia) can be caused by a macular splitting hemianopia. Right parafoveal vision is not critically involved in single word identification, but is when planning left to right reading saccades. The influence of top-down attentional processes during text reading can be visualised in parafoveal V1 using PET.

Hemianopsia↗