Musical alexia for rhythm notation: a discrepancy between pitch and rhythm

In the process of reading music, the reading of rhythm and pitch might be differentiated, although there is no evidence of this to date. There have been cases of disorders restricted to the reading of pitch, but none in which the disorder has been restricted to the reading of rhythm. We present a case of musical alexia and agraphia with Wernicke's aphasia. An in-depth assessment of the subject's musical reading ability showed that her musical alexia was restricted to unfamiliar melodies. When a melody was divided into rhythm elements and pitch elements, pitch reading was preserved, but rhythm reading was severely disturbed. This is the first case reported of a disorder restricted to rhythm reading, and suggests the independence of rhythm reading and pitch reading.     

Alexia due to ischemic stroke of the visual word form area

The visual word form area (VWFA) is a region in the posterior left occipitotemporal cortex adjacent to the fusiform gyrus hypothesized to mediate word recognition. Evidence supporting the role of this area in reading comes from neuroimaging studies of normal subjects, case-controlled lesion studies, and studies of patients with surgical resection of the VWFA for tumors or epilepsy. Based on these prior reports, a small discrete lesion to the VWFA would be expected to cause alexia in a literate person without prior brain process, but such a case has not previously been reported to our knowledge. Here, we report the case of a previously-healthy 63-year-old man with the acute onset of alexia without other significant impairments. Magnetic resonance imaging (MRI) of the brain revealed a small ischemic stroke localized to the inferior left occipitotemporal cortex, corresponding to the approximate location of the putative VWFA. Characteristic of pure alexia, testing in the weeks following the stroke revealed a letter-by-letter reading strategy and a word length effect on single word reading. Formal visual field testing was normal. There was no color anomia, or object or face recognition deficits, although a mild agraphia may have been present. This case of acute-onset alexia in a previously normal individual due to a small stroke restricted to the VWFA and sparing occipital cortex and white matter pathways supports the conclusion that the VWFA is crucial for reading.     

Reading of ideograms and phonograms in Japanese patients after partial commissurotomy

The reading of three Japanese patients who had undergone section of the splenium of the corpus callosum for pineal tumours was examined by the tachistoscopic technique. Oral reading of isolated words and their comprehension (matching of words to pictures) in the left visual half-field were found to be impaired 1 yr post-operatively both for ideograms and for phonograms. Two or three years after operation, retests indicated that the deficit (unilateral alexia) was comparatively restricted to the reading of phonograms. It is argued that unilateral alexia is not due to a disorder in the visual perception of words.     

Pure alexia after damage to the right fusiform gyrus in a right-handed male

Pure alexia refers to a rare acquired reading disorder commonly associated with damage to the posterior part of the left medial occipito-temporal (fusiform) gyrus, which is known as the visual word-form area (VWFA) and thought to be the neural basis for visual processing of letters and words. Right-sided lesions very rarely lead to pure alexia in right-handed individuals. We report a case of a 33-year-old right-handed man with isolated pure alexia resulting from a hemorrhagic lesion to the right fusiform gyrus. A limited recovery of reading skills was observed within six weeks post onset. During this period, the patient spontaneously developed a letter-by-letter reading strategy. Functional magnetic resonance imaging revealed right-hemisphere dominance for language as well as bilateral reading-related activity in the fusiform gyri. Our case indicates that pure alexia may arise as a consequence of damage to the right fusiform gyrus even in right-handed patients (who still may have right hemisphere dominance for language and reading skills), and may lead to a severe reading disorder, as in individuals with left-hemisphere dominance for language.     

Reading and writing Japanese: Kanji versus Kana]

In my talk, I reviewed studies on the neural substrates of Kanji vs. Kana, two types of Japanese characters, written since the 1980s. More Specifically, I reviewed the development of the studies on (1) Kanji and Kana in pure alexia/agraphia, (2) alexia with agraphia of Kanji and (3) 'musical letters' vs. 'literary letters', and reported new findings from those studies. In the 1980s, we frequently studied patients with partial callosal lesions and those with pure alexia, and many of the studies were on the neural substrates of Kanji vs. Kana. Later, we discovered cases of alexia with agraphia of Kanji caused by lesions in the posterior part of the left inferior temporal gyrus, leading us to understand the neural substrates of Kanji and Kana in more detail. In addition to the reading and writing of 'literary letters', we studied the neural mechanisms of the reading and writing of 'musical letters', i.e. musical scores. Our study showed that the neural mechanisms of reading and writing musical scores were similar to those of reading and writing 'literary letters' in professional musicians, although those neural mechanisms varied slightly.     

Cerebral localization of the center for reading and writing music

The mechanisms that underlie the ability to read and write music remain largely unclear compared to those involved in reading and writing language. We had the extremely rare opportunity to study the cerebral localization of the center for reading and writing music in the case of a professional trombonist. During rehearsal immediately before a concert, he suffered a hemorrhage that was localized to the left angular gyrus, the area that has long been known as the center for the ability to read and write. Detailed tests revealed that he showed symptoms of alexia with agraphia for both musical scores and language.     

Acquired alexia with agraphia syndrome in childhood

The acquired alexia with agraphia syndrome is a conspicuous disorder of reading and writing in the absence of significant other language impairments that has mainly been recorded in adults. Pure cases are rare, with most patients displaying mild aphasic deficits. In children, acquired reading and writing disorders are generally reported as part of more encompassing aphasic syndromes affecting oral and written language equally, for example, Broca or Wernicke aphasia. Documented instances of predominant acquired reading and writing disorders in childhood are exceptional. We report an 11-year-old, right-handed boy who sustained a left temporoparieto-occipital hematoma following rupture of an arteriovenous malformation and who consecutively presented with the acquired alexia with agraphia syndrome associated with word-finding difficulties. Neuropsychologic and neurolinguistic data showed that there was no concomitant Gerstmann and/or angular gyrus syndrome. Th e recoveryfrom the anomia was quite favorable, but recovery of written language was more protracted and acted on the patient's further scholastic achievement. This case is reminiscent of a historical childhood case reported in 1939 and is consonant with adult cases in terms of lesion location and semiologic picture.     

Impaired reading not due to visual field loss in a patient with a right-hemipsheric lesion

We describe a right-handed patient (M.B.), who developed left hemianopsia and a severe reading impairment after right occipital–parietal hemorrhage. The pattern of his reading deficit was very similar to that of pure alexia (alexia-without-agraphia): extremely slow reading times with frequent grapheme substitutions and omissions. A test of letter reading while controlling for saccadic eye movements and hemifield of presentation ruled out hemianoptic alexia. Although there have already been reports of reading impairments in right handers following right- hemispheric lesions, ours is, to the best of our knowledge, the first where visual field loss can be definitely excluded as the main cause. Based on a standard neuropsychological assessment and on additional behavioral tests, we argue that M.B.’s difficulties are unlikely to be due to right-hemisphere dominance for language. After considering several candidate explanations, we suggest that M.B.’s symptoms may be related to an impairment in attentional processes related to reading.     

The abilities of a musical savant and his family

The ability of a male autistic savant (TR) to play two unfamiliar piano pieces after listening to a taperecording was tested, closely following the procedures of Sloboda, Hermelin, and O'Connor (1985). Other components of TR's musical ability—pitch recognition, improvisation, and ability to provide harmonic accompaniment—were also examined. TR's musical precocity was examined in relation to his general level of intellectual functioning as indexed by a battery of standardized psychological tests of intelligence, memory, reading, visual organization, and creativity. His parents and two male siblings also completed tests of intelligence. Results from psychometric testing indicated that TR has idiosyncratic levels of cognitive functioning with difficulties in verbal reasoning but high levels of concentration and memory. His speed of information processing, as indicated by Inspection Time, was better than average. TR demonstrated perfect pitch recognition and other family members also demonstrated excellent relative pitch. TR's ability to recall and perform structured music within both the diatonic and wholetone systems was exceptional but dependent upon his familiarity with musical structure and was therefore organized and ruledriven. Furthermore, TR demonstrated competence in improvisation and composition, albeit restricted by his adherence to structural representations of familiar musical rules.     

A case of musical agraphia

Damage to the left upper parietal lobule causes pure agraphia. However, we experienced a patient who exhibited musical agraphia following such a lesion after the agraphia improved. The patient was a 53-year-old female piano teacher. After surgery, she exhibited agraphia and musical agraphia. There was no expressive amusia, receptive amusia, aphasia, agnosia or apraxia. Fifteen months post-surgery, when her agraphia had resolved, her abilities to read, write, and copy music were evaluated. She could read and write single notes and musical signs, but her ability to write a melody was seriously impaired. Furthermore, the salient impairment was in writing rhythm rather than pitch. She could copy music, but only slowly. We consider her a case of pure musical agraphia.     

A case of alexia with agraphia caused by the re-infarct in left lateral occipital gyrus]

The angular gyrus has been proposed as the key area of reading and writing function. In recent PET (positron emission topography) activation studies, role of angular gyrus in the reading and writing has been reestimated. Whether the angular gyrus is necessary for reading and writing or not is now under discussion and should be clinically revised. We experienced a case that presented classical alexia with agraphia of kana (Japanese syllabogram) caused by the re-infarct in left lateral occipital gyrus. This case showed the alexia with agraphia more apparent in Japanese kana than in kanji characters. Interestingly, no higher cortical dysfunction was revealed at the first cerebral infarction in left angular gyrus which was assumed as the key area for alexia with agraphia. This case supported the opinion which pointed out the importance of left occipital gyrus on Japanese kana reading.     

Structural anatomy of pure and hemianopic alexia

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.Although an acquired reading disorder (alexia) is usually part of a more generalised language disorder (aphasia), it can occur as an isolated deficit, usually as a consequence of damage to the brain within the distribution of the left posterior cerebral artery. The two common forms of isolated alexia are hemianopic alexia and pure alexia. The first is the result of a right homonymous hemianopia (RHH), which impairs text reading more than single‐word reading. This is because, in left‐to‐right readers, visual information to the right of fixation is needed to plan rightward reading saccades.^1,2,3 Scanning along a line of text is affected if the RHH encroaches to within 5° of fixation, in right foveal or parafoveal vision.⁴ However, the relationship between text‐reading speed and the number of degrees of sparing of central vision is not linear, and most symptomatic patients have defects encroaching to within 2–3° of fixation or less. By contrast, patients with pure alexia have a severe impairment of single‐word recognition. Although they often have an associated RHH, their syndrome is not a consequence of this deficit, and there have been a few patients with pure alexia without an accompanying field defect.¹ Rather, their impairment is the consequence of damage to a whole‐word recognition system that allows a skilled reader to recognise seven‐letter words as quickly as words of three letters. Patients with pure alexia have damage in the whole‐word recognition system, its connections to primary visual areas or, occasionally, its connections to “higher” language areas. Although these patients can still read, they rely on a more‐or‐less intact letter recognition system and a laborious reversed‐spelling procedure, covert or overt, to arrive at the word''s identity, so‐called “letter‐by‐letter reading”. Thus, the word “dog” is not recognised, but explicitly spelling out “d”, “o”, “g” permits essential reading.It is important to differentiate between the two conditions, as there is specific rehabilitation for hemianopic alexia.⁵ Although occasionally some success has been reported in retraining patients with pure alexia,⁶ most regard pure alexia as an irremediable condition. Pure alexia is more disabling, but many patients with hemianopic alexia find text reading so laborious that they give up recreational reading, and if reading speed is an important skill in their job, their continuing employment may be at risk.The aim of this study was to determine whether pure alexia and hemianopic alexia can be differentiated by the limits of their left occipital lesion on magnetic resonance images (MRIs). Although much has been written about the pathological anatomy of pure alexia,^7,8,9,10 this is the first study directly comparing lesion site and size between patients with pure alexia and hemianopic alexia. The outcome is practical, in terms of diagnosis and referral for appropriate rehabilitation. Further, it provides additional data that identify the region responsible for rapid whole‐word recognition, by excluding areas that may be damaged by posterior cerebral artery (PCA) territory stroke but do not contribute to the syndrome of pure alexia.     

Neural Mechanisms of Rhythm Perception: Current Findings and Future Perspectives

Perception of temporal patterns is fundamental to normal hearing, speech, motor control, and music. Certain types of pattern understanding are unique to humans, such as musical rhythm. Although human responses to musical rhythm are universal, there is much we do not understand about how rhythm is processed in the brain. Here, I consider findings from research into basic timing mechanisms and models through to the neuroscience of rhythm and meter. A network of neural areas, including motor regions, is regularly implicated in basic timing as well as processing of musical rhythm. However, fractionating the specific roles of individual areas in this network has remained a challenge. Distinctions in activity patterns appear between “automatic” and “cognitively controlled” timing processes, but the perception of musical rhythm requires features of both automatic and controlled processes. In addition, many experimental manipulations rely on participants directing their attention toward or away from certain stimulus features, and measuring corresponding differences in neural activity. Many temporal features, however, are implicitly processed whether attended to or not, making it difficult to create controlled baseline conditions for experimental comparisons. The variety of stimuli, paradigms, and definitions can further complicate comparisons across domains or methodologies. Despite these challenges, the high level of interest and multitude of methodological approaches from different cognitive domains (including music, language, and motor learning) have yielded new insights and hold promise for future progress.     

Alexia caused by a fusiform or posterior inferior temporal lesion

We evaluated the alexia and agraphia of three patients with different lesions using Japanese kanji (morphograms) and kana (phonograms) and made a lesion-to-symptom analysis. Patient 1 (pure alexia for both kanji and kana and minor agraphia for kanji after a fusiform lesion) made more paragraphic errors for kanji, whereas patient 2 (alexia with agraphia for kanji after a posterior inferior temporal lesion) showed severe reading and writing disturbances and more agraphic errors for kanji. Brodmann Area 37 was affected in both patients, but in patient 2 the lesion was located lateral to that in patient 1. Patient 3 showed agraphia without alexia after restricted lesion to the angular gyrus. We believe that pure alexia (patient 1) results from a disconnection between the medial fusiform gyrus and posterior inferior temporal area (the lateral fusiform and inferior temporal gyri), whereas alexia with agraphia for kanji (patient 2), corresponding to lexical agraphia in Western countries, results from damage to the posterior inferior temporal area, in which whole-word images of words are thought to be stored. Furthermore, restricted lesion in the angular gyrus (patient 3) does not produce alexia; the alexic symptom of "angular" alexia with agraphia may be the result of damage to the adjacent lateral occipital gyri.     

"Apperceptive" alexia in posterior cortical atrophy

The most common presenting complaint in posterior cortical atrophy (PCA) is reading difficulty. Although often described as an alexia without agraphia, alexia in PCA may have multiple causes, including a primary visuoperceptual etiology, attentional alexia, and central reading difficulty. This study evaluated 14 patients with early PCA and disturbances in reading ability in comparison to 14 normal controls. All 14 patients had a progressive disorder of complex visual functions and neuroimaging evidence of occipitoparietal dysfunction. They underwent a task requiring identification of single letters with and without flanking distractors. They also read single words consisting of regular English spelling or irregular grapheme-phoneme correspondence (irregular words) and pronounceable nonsense words (pseudowords). The PCA patients made errors in letter identification when letters were flanked by visually similar letters or numbers. They could read most single regular and irregular words but made visual errors and had particular trouble with pseudowords. They could not use a letter-by-letter reading strategy effectively. The PCA patients had similar difficulties on other visuoperceptual tests. These findings are consistent with an alexia manifested by perceptual and attentional difficulty on attempting serial visual processing of letters in the context of other letters. This "apperceptive alexia" results when the configuration of letters into words is impaired during letter-by-letter reading. Disproportionate difficulty reading pseudowords suggests an additional impairment in phonological processing. PCA patients have variable neuropathology and individual patients may have other contributions to their reading impairment.     

Unilateral right cerebral representation of reading in a familial left-hander

Several recent hypotheses of cerebral functional organization in natural left-handers hold that language, including reading, is localized either unilaterally in the left hemisphere or bilaterally, but not unilaterally in the right hemisphere. A case of alexia without agraphia in a left-hander with an infarct in the right occipital lobe and splenium of the corpus callosum is reported. That a permanent and complete alexia resulted from a single, right hemisphere lesion indicated that reading was completely lateralized to that hemisphere. The case demonstrates that in some natural left-handers language functions can be unilaterally represented in the right hemisphere.     

Phonological alexia: three dissociations.

Three dissociations were observed in a case of alexia: a disturbance of reading, without comparable disturbance of oral expression, oral comprehension, writing, or spelling aloud; a disturbance of the phonological reading process, without disturbance of the non-phonological reading process; a disturbance located at the level of the phonological stage, without disturbance of the perceptual and expressive stages. This pattern of results has been called phonological alexia.     

Multiple Oral Re-reading treatment for alexia: The parts may be greater than the whole

This study examines the reasons for the success of Multiple Oral Re-reading (MOR; Moyer, 1979), a non-invasive, easily administered alexia treatment that has been reported in the literature and is currently in clinical use. The treatment consists of reading text passages aloud multiple times a day. Findings that MOR improves reading speed on practised as well as novel text have been inconsistent, making MOR's role in the rehabilitation of alexia unclear. We hypothesised that MOR's treatment mechanism works through repetition of high frequency words (i.e., bottom-up processing). We designed and controlled our text passages to test the hypothesis that participants would not improve on all novel text but would improve on text that includes a critical mass of the words contained in the passages they were re-reading. We further hypothesised that the improvement would be at the level of their specific alexic deficit. We tested four participants with phonological alexia and two with pure alexia during 8 weeks of MOR treatment. Contrary to the conclusions of previous studies, our results indicate that improvements in top-down processing cannot explain generalisation in MOR and that much of the improvement in reading is through repetition of the practised words. However, most patients also showed improvement when specific phrases were re-used in novel passages, indicating that practice of difficult words in context may be crucial to reading improvement.     

Congenital amusia in childhood: a case study

Here we describe the first documented case of congenital amusia in childhood. AS is a 10-year-old girl who was referred to us by her choir director for persisting difficulties in singing. We tested her with the child version of the Montreal Battery for the Evaluation of Amusia (MBEA) which confirmed AS's severe problems with melodic and rhythmic discrimination and memory for melodies. The disorder appears to be limited to music since her audiometry as well as her intellectual and language skills are normal. Furthermore, the musical disorder is associated to a severe deficit in detecting small pitch changes. The electrical brain responses point to an anomaly in the early stages of auditory processing, such as reflected by an abnormal mismatch negativity (MMN) response to small pitch changes. In singing, AS makes more pitch than time errors. Thus, despite frequent and regular musical practice, AS's profile is similar to the adult form of congenital amusia.     

Multimodal alexia: neuropsychological mechanisms and implications for treatment

Letter-by-letter (LBL) reading is the phenomenon whereby individuals with acquired alexia decode words by sequential identification of component letters. In cases where letter recognition or letter naming is impaired, however, a LBL reading approach is obviated, resulting in a nearly complete inability to read, or global alexia. In some such cases, a treatment strategy wherein letter tracing is used to provide tactile and/or kinesthetic input has resulted in improved letter identification. In this study, a kinesthetic treatment approach was implemented with an individual who presented with severe alexia in the context of relatively preserved recognition of orally spelled words, and mildly impaired oral/written spelling. Eight weeks of kinesthetic treatment resulted in improved letter identification accuracy and oral reading of trained words; however, the participant remained unable to successfully decode untrained words. Further testing revealed that, in addition to the visual-verbal disconnection that resulted in impaired word reading and letter naming, her limited ability to derive benefit from the kinesthetic strategy was attributable to a disconnection that prevented access to letter names from kinesthetic input. We propose that this kinesthetic-verbal disconnection resulted from damage to the left parietal lobe and underlying white matter, a neuroanatomical feature that is not typically observed in patients with global alexia or classic LBL reading. This unfortunate combination of visual-verbal and kinesthetic-verbal disconnections demonstrated in this individual resulted in a persistent multimodal alexia syndrome that was resistant to behavioral treatment. To our knowledge, this is the first case in which the nature of this form of multimodal alexia has been fully characterized, and our findings provide guidance regarding the requisite cognitive skills and lesion profiles that are likely to be associated with a positive response to tactile/kinesthetic treatment.