A case of aneurysmal subarchnoid haemorrhage and superficial siderosis complicated by prospagnosia, simultagnosia and alexia without agraphia.

A 42-year-old lady presented with acute aneurysmal subarachnoid haemorrhage and developed difficulty recognising faces (prosopagnosia), inability to process visual information in busy environments (simultagnosia) and difficulty to read (alexia). She was subsequently found to have superficial siderosis on MRI.

Transcranial direct current stimulation with multiple oral re-reading therapy for pure alexia without agraphia: a case report.

Pure alexia without agraphia is characterized by impaired reading due to damage to the occipitotemporal cortex with preserved writing skills. In this case report, we investigate the effect of multiple oral re-reading (MOR) therapy adjunct with transcranial direct current stimulation (tDCS) in improving reading recovery of a 64-year-old patient with pure alexia without agraphia following a stroke. His MRI revealed an area of infarct with microhemorrhages at the left occipitotemporal region. The patient was blinded to each therapy and underwent seven consecutive sessions of sham tDCS followed by seven consecutive sessions of real tDCS, coupled with 1-hour MOR therapy during each session. Western Aphasia Battery (WAB) was performed at baseline, before sham and real-tDCS, and 6 weeks after completing tDCS therapy. The patient showed improvement using both sham and real-tDCS with better reading comprehension, average reading time, and word per minute after real-tDCS. This study suggests that MOR, coupled with tDCS therapy may accelerate the reading recovery in patients with pure alexia.

When mild pure alexia may not be reducible to hemianopic alexia.

Individuals with pure alexia often have visual field defects such as right homonymous hemianopia. Relatively few attempts have been made to develop criteria to differentiate pure alexia from hemianopic alexia. In this Commentary we provide concrete suggestions to distinguish the two disorders. We also report on additional assessments with two previously reported cases for whom the diagnosis of pure alexia was called into question and an alternative proposal was offered that the reading deficits were instead due to hemianopia. We show that the results of clinical and neuropsychological tests do not support the account that the reading impairment was caused by the visual field defect. In particular, for both cases, the right homonymous hemianopia was not complete, and a split-field reading task demonstrated an inability also to read words presented in the intact left visual field. In conclusion, pure alexics may indeed show fairly modest word-length effects; however, the presence of right homonymous hemianopia and a non-extreme gradient of reading speed alone are not sufficient grounds to put in doubt the diagnosis. We propose that a fuller clinical and neuropsychological examination taking into account the possible confounding effects of the visual field defects will help to distinguish pure alexia from hemianopic alexia.

Visual crowding in pure alexia and acquired prosopagnosia.

Visual crowding is a phenomenon that impairs object recognition when the features of an object are positioned too closely together. Crowding limits recognition in normal peripheral vision and it has been suggested to be the core deficit in visual agnosia, leading to a domain-general deficit in object recognition. Using a recently developed tool, we test whether crowding is the underlying deficit in four patients with category specific agnosias: Two with pure alexia and two with acquired prosopagnosia. We expected all patients to show abnormal crowding. We find that the two patients with acquired prosopagnosia show abnormal crowding effects in foveal vision, while the pure alexic patients do not, and that this constitutes a significant dissociation. Thus, abnormal crowding cannot explain all cases of visual agnosia. Much recent work has focused on similarities between pure alexia and acquired prosopagnosia. Here we show a difference in a basic visual mechanism-visual crowding.

Alexia Without Agraphia in a Right-Handed Individual Following Right Occipital Stroke.

Alexia without agraphia is a disconnection syndrome that typically involves damage to the occipital lobe, with splenium involvement, in the dominant left hemisphere. We describe an exceptionally rare case of a right-handed individual displaying this deficit following a right-sided occipital stroke. A report of a single case of a 65-year-old man is presented with data from appointments with the neurology and neuropsychology departments that occurred approximately 10 and 12 months following the patient's stroke. During the evaluation, he exhibited a marked deficit in his ability to read, with vision grossly intact. His ability to write single words and short phrases from dictation was intact, but he was later unable to read them. This case demonstrates the complexity of the organization of language in the human brain. Although a large majority of individuals exhibit language dominance in their left hemispheres, it remains possible that some right-handed individuals may show atypical organization of language. This highlights the need for clinicians to consider atypical cortical organization when observed deficits may not necessarily match expected lesions within the cortex.

The effects of homonymous hemianopia in experimental studies of alexia.

Pure alexia is characterized by an increased word-length effect in reading. However, this disorder is usually accompanied by right homonymous hemianopia, which itself can cause a mildly increased word-length effect. Some alexic studies have used hemianopic patients with modest word-length effects: it is not clear (a) whether they had pure alexia and (b) if not, whether their results could be explained by the field defect. Our goal was to determine if impairments in visual processing claimed to be related to alexia could be replicated in homonymous hemianopia alone. Twelve healthy subjects performed five experiments used in two prior studies of alexia, under both normal and simulated hemianopic conditions, using a gaze-contingent display generated by an eye-tracker. We replicated the increased word-length effect for reading time with right homonymous hemianopia, and showed a similar effect for a lexical decision task. Simulated hemianopia impaired scanning accuracy for letter or number strings, and slowed object part processing, though the effect of configuration was not greater under hemianopic viewing. Hemianopia impaired the identification of words whose letters appeared and disappeared sequentially on the screen, with better performance on a cumulative presentation in which the letters remained on the screen. The reporting of trigrams was less accurate with hemianopia, though syllabic structure did not influence the results. We conclude that some impairments that have been attributed to the processing defects underlying alexia may actually be due to right homonymous hemianopia. Our results underline the importance of considering the contribution of accompanying low-level visual impairments when studying high-level processes.

What lies beneath: a comparison of reading aloud in pure alexia and semantic dementia.

Exaggerated effects of word length upon reading-aloud performance define pure alexia, but have also been observed in semantic dementia. Some researchers have proposed a reading-specific account, whereby performance in these two disorders reflects the same cause: impaired orthographic processing. In contrast, according to the primary systems view of acquired reading disorders, pure alexia results from a basic visual processing deficit, whereas degraded semantic knowledge undermines reading performance in semantic dementia. To explore the source of reading deficits in these two disorders, we compared the reading performance of 10 pure alexic and 10 semantic dementia patients, matched in terms of overall severity of reading deficit. The results revealed comparable frequency effects on reading accuracy, but weaker effects of regularity in pure alexia than in semantic dementia. Analysis of error types revealed a higher rate of letter-based errors and a lower rate of regularization responses in pure alexia than in semantic dementia. Error responses were most often words in pure alexia but most often nonwords in semantic dementia. Although all patients made some letter substitution errors, these were characterized by visual similarity in pure alexia and phonological similarity in semantic dementia. Overall, the data indicate that the reading deficits in pure alexia and semantic dementia arise from impairments of visual processing and knowledge of word meaning, respectively. The locus and mechanisms of these impairments are placed within the context of current connectionist models of reading.

Bilateral hemispheric processing of words and faces: evidence from word impairments in prosopagnosia and face impairments in pure alexia.

Considerable research has supported the view that faces and words are subserved by independent neural mechanisms located in the ventral visual cortex in opposite hemispheres. On this view, right hemisphere ventral lesions that impair face recognition (prosopagnosia) should leave word recognition unaffected, and left hemisphere ventral lesions that impair word recognition (pure alexia) should leave face recognition unaffected. The current study shows that neither of these predictions was upheld. A series of experiments characterizing speed and accuracy of word and face recognition were conducted in 7 patients (4 pure alexic, 3 prosopagnosic) and matched controls. Prosopagnosic patients revealed mild but reliable word recognition deficits, and pure alexic patients demonstrated mild but reliable face recognition deficits. The apparent comingling of face and word mechanisms is unexpected from a domain-specific perspective, but follows naturally as a consequence of an interactive, learning-based account in which neural processes for both faces and words are the result of an optimization procedure embodying specific computational principles and constraints.

Post-stroke language disorders.

Post-stroke language disorders are frequent and include aphasia, alexia, agraphia and acalculia. There are different definitions of aphasias, but the most widely accepted neurologic and/or neuropsychological definition is that aphasia is a loss or impairment of verbal communication, which occurs as a consequence of brain dysfunction. It manifests as impairment of almost all verbal abilities, e.g., abnormal verbal expression, difficulties in understanding spoken or written language, repetition, naming, reading and writing. During the history, many classifications of aphasia syndromes were established. For practical use, classification of aphasias according to fluency, comprehension and abilities of naming it seems to be most suitable (nonfluent aphasias: Broca's, transcortical motor, global and mixed transcortical aphasia; fluent aphasias: anomic, conduction, Wernicke's, transcortical sensory, subcortical aphasia). Aphasia is a common consequence of left hemispheric lesion and most common neuropsychological consequence of stroke, with a prevalence of one-third of all stroke patients in acute phase, although there are reports on even higher figures. Many speech impairments have a tendency of spontaneous recovery. Spontaneous recovery is most remarkable in the first three months after stroke onset. Recovery of aphasias caused by ischemic stroke occurs earlier and it is most intensive in the first two weeks. In aphasias caused by hemorrhagic stroke, spontaneous recovery is slower and occurs from the fourth to the eighth week after stroke. The course and outcome of aphasia depend greatly on the type of aphasia. Regardless of the fact that a significant number of aphasias spontaneously improve, it is necessary to start treatment as soon as possible. The writing and reading disorders in stroke patients (alexias and agraphias) are more frequent than verified on routine examination, not only in less developed but also in large neurologic departments. Alexia is an acquired type of sensory aphasia where damage to the brain causes the patient to lose the ability to read. It is also called word blindness, text blindness or visual aphasia. Alexia refers to an acquired inability to read due to brain damage and must be distinguished from dyslexia, a developmental abnormality in which the individual is unable to learn to read, and from illiteracy, which reflects a poor educational background. Most aphasics are also alexic, but alexia may occur in the absence of aphasia and may occasionally be the sole disability resulting from specific brain lesions. There are different classifications of alexias. Traditionally, alexias are divided into three categories: pure alexia with agraphia, pure alexia without agraphia, and alexia associated with aphasia ('aphasic alexia'). Agraphia is defined as disruption of previously intact writing skills by brain damage. Writing involves several elements: language processing, spelling, visual perception, visuospatial orientation for graphic symbols, motor planning, and motor control of writing. A disturbance of any of these processes can impair writing. Agraphia may occur by itself or in association with aphasias, alexia, agnosia and apraxia. Agraphia can also result from 'peripheral' involvement of the motor act of writing. Like alexia, agraphia must be distinguished from illiteracy, where writing skills were never developed. Acalculia is a clinical syndrome of acquired deficits in mathematical calculation, either mentally or with paper and pencil. These language disturbances can be classified differently, but there are three principal types of acalculia: acalculia associated with language disturbances, including number paraphasia, number agraphia, or number alexia; acalculia secondary to visuospatial dysfunction with malalignment of numbers and columns, and primary anarithmetria entailing disruption of the computation process.

Do patients with pure alexia suffer from a specific word form processing deficit? Evidence from 'wrods with trasnpsoed letetrs'.

It is widely accepted that letter-by-letter reading and a pronounced increase in reading time as a function of word length are the hallmark features of pure alexia. Why patients show these two phenomena with respect to underlying cognitive mechanisms is, however, much less clear. Two main hypotheses have been proposed, i.e. impaired discrimination of letters and deficient processing of word forms. While the former deficit can easily be investigated in isolation, previous findings favouring the latter seem confounded. Applying a word reading paradigm with systematically manipulated letter orders in two patients with pure alexia, we demonstrate a word form processing deficit that is not attributable to sublexical letter discrimination difficulties. Moreover, pure alexia-like fixation patterns could be induced in healthy adults by having them read sentences including words with transposed letters, so-called 'jumbled words'. This further corroborates a key role of deficient word form processing in pure alexia. With regard to basic reading research, the present study extends recent evidence for relative, rather than precise, encoding of letter position in the brain.

About the role of visual field defects in pure alexia.

Pure alexia is an acquired reading disorder characterized by a disproportionate prolongation of reading time as a function of word length. Although the vast majority of cases reported in the literature show a right-sided visual defect, little is known about the contribution of this low-level visual impairment to their reading difficulties. The present study was aimed at investigating this issue by comparing eye movement patterns during text reading in six patients with pure alexia with those of six patients with hemianopic dyslexia showing similar right-sided visual field defects. We found that the role of the field defect in the reading difficulties of pure alexics was highly deficit-specific. While the amplitude of rightward saccades during text reading seems largely determined by the restricted visual field, other visuo-motor impairments-particularly the pronounced increases in fixation frequency and viewing time as a function of word length-may have little to do with their visual field defect. In addition, subtracting the lesions of the hemianopic dyslexics from those found in pure alexics revealed the largest group differences in posterior parts of the left fusiform gyrus, occipito-temporal sulcus and inferior temporal gyrus. These regions included the coordinate assigned to the centre of the visual word form area in healthy adults, which provides further evidence for a relation between pure alexia and a damaged visual word form area. Finally, we propose a list of three criteria that may improve the differential diagnosis of pure alexia and allow appropriate therapy recommendations.

Too little, too late: reduced visual span and speed characterize pure alexia.

Whether normal word reading includes a stage of visual processing selectively dedicated to word or letter recognition is highly debated. Characterizing pure alexia, a seemingly selective disorder of reading, has been central to this debate. Two main theories claim either that 1) Pure alexia is caused by damage to a reading specific brain region in the left fusiform gyrus or 2) Pure alexia results from a general visual impairment that may particularly affect simultaneous processing of multiple items. We tested these competing theories in 4 patients with pure alexia using sensitive psychophysical measures and mathematical modeling. Recognition of single letters and digits in the central visual field was impaired in all patients. Visual apprehension span was also reduced for both letters and digits in all patients. The only cortical region lesioned across all 4 patients was the left fusiform gyrus, indicating that this region subserves a function broader than letter or word identification. We suggest that a seemingly pure disorder of reading can arise due to a general reduction of visual speed and span, and explain why this has a disproportionate impact on word reading while recognition of other visual stimuli are less obviously affected.

Alexia without agraphia in Creutzfeldt-Jakob disease.

Progressive dementia uncommonly presents with focal deficits referable to posterior cortical dysfunction. We describe a 62 year-old man who presented with progressive visual disturbance in whom detailed cognitive testing documented alexia without agraphia. The only finding from diagnostic investigations was hypoperfusion of the posterior left temporoparietal region on brain SPECT scan. He rapidly progressed and Creutzfeldt-Jakob disease (CJD) was confirmed at autopsy. Although reading disorders may develop in association with posterior cortical atrophy due to CJD, this is the first reported autopsy-confirmed case presenting as alexia without agraphia.

Patterns of peripheral paralexia: pure alexia and the forgotten visual dyslexia?

The concept of visual dyslexia put forward by Marshall and Newcombe (1973) is assessed. After a long period of neglect it was resurrected in the late 1990s in a narrow form. In the current paper it is proposed that a wider form of the functional syndrome is useful to include amongst other conditions attentional dyslexia and neglect dyslexia. The variety of sub-forms would correspond to the behavioural effects of the different ways in which the orthographic processing systems can be impaired. What distinguishes the broader form from pure alexia is that the patient lacks the capacity to use a serial letter processing strategy, and so interpretation of visual dyslexia in terms of the impairment to the orthographic processing systems is not contaminated by the use of a compensatory strategy that results in processing operations which are qualitatively very different from the normal and highly opaque. The lack of a serial letter processing strategy makes visual dyslexia a much more transparent functional syndrome.