Kanji (Morphogram) and Kana (Phonogram) Problem in Japanese Alexia and Agraphia.

The kanji and kana (or kanji vs. kana) problem in the Japanese language denotes the dissociation between kanji (morphograms) and kana (phonograms) in reading/comprehension and writing. Since paragraphia of kana in a patient with amyotrophic lateral sclerosis was first reported in 1893, kanji-kana dissociation has been the central topic in Japanese aphasiology. Recent advancements in lesion-to-symptom analyses and functional imaging studies have identified some areas whose damage causes dissociative disturbances of reading or writing between kanji and kana. That is, (1) angular alexia with agraphia causes kanji agraphia; alexia of kana with an angular gyrus lesion is the result of a damage to the middle occipital gyrus; (2) alexia with agraphia for kanji is caused by a posterior inferior temporal cortex (mid-fusiform/inferior temporal gyri; visual word form area) lesion, whereas pure agraphia for kanji is caused by a posterior middle temporal gyrus lesion; and (3) pure alexia, particularly for kanji, results from a mid-fusiform gyrus lesion (Brodmann's Area [BA] 37), whereas pure alexia for kana results from a posterior fusiform/inferior occipital gyri lesion (BA 18/19).

A Patient With Word Blindness in the Seventeenth Century.

Johann Jakob Wepfer (1620-1695), city physician in Schaffhausen, Switzerland, published two books on "apoplexy." He proposed new ideas about the events in the brain during such attacks, based on Harvey's theory of the circulation of the blood. Wepfer postulated extravasation of whole blood or serum in the brain, in opposition to the Galenic notion of blocked ventricles. His case histories are remarkably precise and untainted by interpretation. This allows the recognition of a patient with word blindness, who was also unable to read words written by himself. Unlike patients with pure "alexia without agraphia," he could not write complete sentences because of additional language defects, especially speech comprehension. Jules Dejerine (1849-1917) would, in 1892, not only describe a patient with the pure form of this syndrome (cécité verbale avec intégrité de l'écriture spontanée et sous dictée) but also provide an explanation of its anatomical basis.

SSc and the 2013 diagnostic criteria: the case of Paul Klee's manual pathology and dysgraphia.

The German-Swiss modernist painter Paul Klee (1879-1940) suffered in the final years of his life from a severe illness, diagnosed in 1936 as scleroderma, later renamed SSc. New classification criteria for this disease issued in 2013 now allow for a diagnosis to be confirmed. Important for this process, however, is the question of whether or not Klee's hands were affected by his illness. The morphology of the artist's hands and evidence of dysgraphic changes in his handwriting are reviewed as indications of his manual pathology. Despite his illness, Klee triumphed over his infirmity, simplifying his painting and drawing styles and substantially increasing his artistic output from 1936 until his death in 1940.

Tomas Tranströmer's stroke of genius: language but no words.

In 1990, the widely acclaimed Swedish poet Tomas Tranströmer lost his speech and the ability to use his right hand as a result of a stroke. As if anticipating his own fate, in 1974, he referred in his longest poem Baltics the story of the Russian composer Vissarion Shebalin who suffered the same symptoms as Tranströmer following a brain bleed: "Then, cerebral hemorrhage: paralysis on the right side with aphasia." An amateur pianist himself, Tranströmer carried on playing left-handed piano pieces after the stroke. In spite of a severe nonfluent dysphasia with dysgraphia, Tranströmer kept producing a poetic language of the highest caliber in accordance with his 1979 no less prophetic verse "language but no words." And through music and poetry, overcame the great communication barriers imposed by a large dominant hemispheric stroke. A nonprolific writer before the stroke, after it Tranströmer became disproportionately brief compared to his prestroke production, confining most of his poetry to the agrammatical and telegraphic haiku style.

Written language production disorders: historical and recent perspectives.

Written language production is often the least examined neuropsychological function, yet it provides a sensitive and subtle sign to a variety of different behavioral disorders. The dissociation between written and spoken language and reading and writing first came to clinical prominence in the nineteenth century, with respect to ideas about localization of function. Twentieth century aphasiology research focused primarily on patients with unifocal lesions from cerebrovascular accidents, which have provided insight into the various levels of processing involved in the cognitively complex task of producing written language. Recent investigations have provided a broader perspective on writing impairments in a variety of disorders, including progressive and diffuse brain disorders, and functional brain imaging techniques have been used to study the underlying processes in healthy individuals.

Who actually read Exner? Returning to the source of the frontal "writing centre" hypothesis.

We have translated the most famous text of Sigmund Exner (1846-1926), which relates to the existence of a localised "writing centre" in the brain. We discuss its relevance to modern studies and understanding of writing and agraphia. In Exner's most famous text, he hypothesised about the eponymous "Exner's Area", a discrete area within the brain that was located in the left middle frontal gyrus, which was dedicated to the function of writing. This text in German, included in a book published in 1881 "Untersuchungen über die Lokalisation der Functionen in der Grosshirnrinde des Menschen" (Studies on the localisation of functions in the cerebral cortex of humans), lent itself to passionate debates during the following decades on the possibility of finding a specific writing centre in left middle frontal gyrus. Modern authors still refer back to the evidence cited in this seminal text. However, over the 281 pages of Exner's book, only a few chapters dealt with agraphia. Only four of the 167 case reports in the book explicitly mention agraphia. Although Exner describes the anatomical details of these lesions (from autopsies), no patient had pure agraphia, and only one case had an isolated lesion of the posterior part of the middle frontal gyrus. The small number of patients, the absence of pure agraphia symptoms, and the variation in the anatomy of these lesions are the main reasons why Exner's hypothesis of a writing centre in left middle frontal gyrus has been continually debated until now. More than the seminal publication of Sigmund Exner on agraphia, we think that the diffusion of his hypothesis was partly due to the influence that Exner and his family had within the scientific community at the turn of the 20th century.

Chapter 37: alexia and agraphia.

Studies of alexia and agraphia have played important roles in understanding how complex cognitive functions are related to brain structure and activity. Modern interests in brain-behavior relations began during the second half of the 19th century as an outgrowth of flawed correlative studies by neuroanatomist Franz Gall and subsequent clinical-pathological analyses by Jean-Baptiste Boulliaud on speech and the frontal lobes. In 1856, Louis Victor Marcé drew attention to writing disorders and postulated a cerebral faculty for writing. Following Paul Broca's epochal reports on aphemia, many European physicians investigated reading and writing impairments after brain injury. Albert Pitres published the first detailed description of isolated agraphia, and Adolf Kussmaul identified alexia as an isolated symptom of brain disease. Jules Dejerine in 1892 provided the first clinical-pathological descriptions of pure alexia, and he suggested a key role for the left parietal lobe in reading and writing. In the 20th century, varieties of agraphia or alexia were linked to apraxia (Hugo Liepmann), impaired body image (Josef Gerstmann), spatial misperception, and interhemispheric disconnection. Other analyses focused on error types that defined new clinical syndromes (e.g. deep dyslexia) and provided evidence for cognitive modularity.

[Alexia without agraphia: an exemplary deficit, cherished by neuropsychologists]. / L'alexie sans agraphie : un déficit exemplaire, choyé par les neuropsychologues.

Alexia without agraphia is the result of a left inferotemporal cortical lesion. This unilateral lesion provokes an almost irrecoverable word reading disability. This anatomical-clinical observation has identified as crucial an area on the left fusiform gyrus, which represents a sort of "letter cranial bump". This area, located near the left V4, has been labeled the visual word form area (VWFA). The VWFA ensures visual word processing whatever the visual field stimulated. In normal subjects, imaging methods show an activation of the VWFA during reading. In patients, the alexia that follows a VWFA lesion proves that this area is an essential relay for cerebral word reading processing. Therefore, pure alexia is no longer explained by a posterior interhemispheric disconnection.

Alexia and agraphia: contrasting perspectives of J.-M. Charcot and J. Hughlings Jackson.

OBJECTIVE: To evaluate 19th-century concepts of cerebral localization for complex mental activities, focusing on alexia and agraphia in published writings of Jean-Martin Charcot (1825-1893) and John Hughlings Jackson (1835-1911). BACKGROUND: In the early 1860 s, Broca's reports on a special role for the left frontal lobe in articulate language ignited frenetic interest in cerebral localization. Disorders of written language (alexia and agraphia) were enmeshed in ensuing discussions of how the brain was organized for language and other complex behaviors. DESIGN/METHODS: Focused review and analysis of Charcot's and Hughlings Jackson's publications on aphasia, alexia, and agraphia. RESULTS: In the wake of Broca's observations, the extent to which language functions in general--or such specialized functions as reading and writing--might involve focal cerebral representation was controversial. Based on his clinical-pathologic approach to "regional diagnosis," Charcot came to value insights provided by "partial isolated aphasias." He described patients with isolated alexia and agraphia, and he proposed a functional-anatomic framework to accommodate these disorders. Adopting a hierarchical model of nervous system organization, Hughlings Jackson argued that reading and writing could not be dissociated from other aspects of "intellectual language." Charcot's reductionism was typical of his era, but Hughlings Jackson's more holistic approach was to gain ascendancy in early decades of the 20th century. CONCLUSIONS: Charcot's and Hughlings Jackson's positions on alexia and agraphia reflected contrasting philosophical approaches to the study of brain disorders. Their views informed the opinions of their contemporaries and neurologic heirs in important debates on cerebral organization.

Premature thoughts on writing disorders.

Three papers appeared in the 19th century describing the dissociation between speech and writing: Marce (1856), Ogle (1867), and Pitres (1884). An account of the convincing evidence of dissociations put forward in these papers is presented. Three explanations are proposed as to the reason why the observations reported by these authors were overlooked or rejected by their contemporaries, namely: (a) in the first half of the century it seems that very little knowledge of the processes underlying writing (as opposed to speech) was available, (b) the debates focussed on the independence of speech versus motor control and language versus the intellect, (c) parallelisms between phylogeny, ontogeny and aphasia impeded the application of the principle of double dissociations, including the dissociations between speech and writing. It is argued that this phenomenon in the history of aphasia is best captured by the concept of prematurity in scientific discovery proposed by Stent (1972, 2003).

The history of written language disorders: Reexamining Pitres' case (1884) of pure agraphia.

The first clinical description of pure agraphia was reported by the French neurologist Pitres in 1884. Pitres used the case study evidence to argue for modality-specific memory representations and the localization of writing. This article reviews Pitres's contribution to the study of acquired writing disorders, the components of writing models and the cerebral localization which subserve writing, in light of the views entertained by his contemporaries and current authors. Although numerous cases have been reported throughout this century, the view that writing can be impaired while other language functions and motor activities remain intact is still challenged.

18th century cases of noun-verb dissociation: the contribution of Carl Linnaeus.

The Neapolitan philosopher Vico reported the first known case of a verb production deficit in aphasia in 1744. The following year the Swedish naturalist Linnaeus described a case of impaired noun production. Hence a double dissociation of lexical category retrieval may have been documented within one year in the 18th century.

Jules Dejerine and the third alexia.

Modern concepts of pure alexia and alexia with agraphia are derived from Dejerine's eloquent clinicopathologic studies of the late 19th century. More recently, a third variety of alexia has been described in association with left frontal lesions causing Broca's aphasia. Dejerine also recognized this "third alexia." For Dejerine, alexia with Broca's aphasia was indispensible to his view of a left-hemisphere language zone in which cortical lesions disrupt all language modalities (speech, reading, and writing). Viewed in light of modern neurolinguistic advances, Dejerine's descriptions of the third alexia are surprisingly prescient.