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.     

Dorsal type letter-by-letter reading accompanying alexia with agraphia due to a lesion of the lateral occipital gyri

ABSTRACT

We report a patient with alexia with agraphia accompanied by letter-by-letter reading after hemorrhage in the left middle and inferior occipital gyri that spared the angular gyrus and the fusiform gyrus. Kanji (Japanese morphograms) and kana (Japanese phonetic writing) reading and writing tests revealed that alexia with agraphia was characterized by kana-predominant alexia and kanji-predominant agraphia. This type of “dorsal” letter-by-letter reading is discernable from conventional ventral type letter-by-letter reading that is observed in pure alexia in that (1) kinesthetic reading is less effective, (2) kana or literal agraphia coexists, and (3) fundamental visual discrimination is nearly normal.     

Neural mechanism of reading]

We conducted positron emission tomography studies on reading and found that two distinct areas were activated, i.e. the left fusiform/inferior temporal gyri (posterior inferior temporal cortex, Area 37) by kanji words and the fusiform/inferior occipital gyri (posterior occipital gyri, Area 18/19) by kana words. Clinically, alexia and agraphia for kanji is caused by a posterior inferior temporal cortex lesion. Moreover, pure alexia more impaired for kanji results from a fusiform gyrus lesion, whereas pure alexia for kana occurs because of damage to the posterior occipital gyri. These experimental and clinical findings suggest that impaired letter identification in Area 18/ 19 causes pure alexia for kana, disrupted visual images of words in Area 37 results in alexia with agraphia for kanji, and impaired access to the visual image storage (Area 37) yields pure alexia dominantly disturbed for kanji.     

Naming difficulties in alexia with agraphia for kanji after a left posterior inferior temporal lesion.

The case is described of a patient with alexia and agraphia for kanji, and severe anomia after a subcortical haemorrhage in the left posterior inferior temporal area. Magnetic resonance imaging at four months after onset showed a lesion in the inferior temporal and fusiform gyri, extending from the temporo-occipital junction toward the anterior third of the temporal lobe. Comparison with other reported cases of alexia with agraphia and anomia made it clear that when accompanied by severe anomia, the lesions extended either forward to the anterior part of the middle temporal gyrus or medially to the parahippocampal gyrus. It is suggested that the disconnection of association fibres between the parahippocampal, fusiform, middle, and inferior temporal gyri, especially between the parahippocampal gyrus and the other temporal gyri, or the cortical damage to the posterior part of these gyri is essential for the production of anomia.     

Neural mechanism of reading and writing in the Japanese language

Three Japanese patients presenting with pure alexia showed agraphia for Kanji in addition. A left angular gyrus lesion caused agraphia for both Kanji and Kana, but Kanji reading was preserved. A left posterior inferior temporal (PIT) lesion resulted in alexia and agraphia for Kanji, while the Kana function was preserved. These results imply that the semantic processing of reading Kanji words depends upon the left PIT area, while the phonological reading of Kana is mediated by the left angular gyrus. The PIT area also plays an important role in writing Kanji words.     

Naming difficulties seen in a case of alexia with agraphia caused by a left postero-inferior temporal lesion]

A 71-year-old right-handed man presented writing and reading difficulties as well as naming difficulties. Neuropsychological examinations revealed mild fluent type aphasia accompanied by alexia and agraphia predominantly affecting kanji and also severe naming difficulties. Brain MRI showed cerebral subcortical hemorrhage extending from the anterior one-third of the left temporal lobe to the temporo-occipital junction involving the fusiform gyrus. The analysis of the several reported cases with severe alexia with agraphia for kanji including ours revealed a close correlation between the severity of kanji writing disturbances and that of naming difficulties. It was also shown that cases with severe naming difficulties had lesion extending anteriorly to the anterior middle temporal gyrus or medially to the parahippocampal gyrus, suggesting that the disconnection between the parahippocampal gyrus and other cortices including the temporal lobe was essential for production of naming difficulties.     

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.     

Fusiform type alexia: pure alexia for words in contrast to posterior occipital type pure alexia for letters

OBJECTIVE: To clarify the behavioral differences between patients with pure alexia from different lesions. METHODS: Two patients with pure alexia caused by damage to the fusiform or posterior occipital gyri were given reading and writing tests including kanji (Japanese morphograms) and kana (Japanese phonetic writing). RESULTS: Patient 1 (pure alexia from a fusiform gyrus lesion) had difficulty reading both kanji and kana, with kanji reading more impaired, and imageability and visual complexity effects (imageable or less complex words/characters were read better than nonimageable or more complex words/characters), whereas patient 2 (pure alexia from a posterior occipital gyri lesion) showed selective impairment of kana reading. CONCLUSION: Pure alexia for kanji (and kana; fusiform type) is characterized by impairments of both whole-word reading, as represented in kanji reading, and letter identification, and is different from pure alexia for kana (posterior occipital type) in which letter identification is primarily impaired. Thus, fusiform type pure alexia should be designated pure alexia for words, whereas posterior occipital type pure alexia should be designated pure alexia for letters.     

Dual neural circuit model of reading and writing

In the hypothetical neural circuit model of reading and writing that was initially proposed by Dejerine and subsequently confirmed by Geschwind, the left angular gyrus was considered as a unique center for processing letters. Japanese investigators, however, have repeatedly pointed out that this angular gyrus model cannot fully explain the disturbances observed in reading and writing Kanji letters in Japanese patients with various types of alexia with or without agraphia. In 1982, I proposed a dual neural circuit model of reading and writing Japanese on the basis of neuropsychological studies on the various types of alexia with or without agraphia without aphasia. This dual neural circuit model proposes that apart from the left angular gyrus which was thought to be a node for phonological processing of letters, the left posterior inferior temporal area, also acts as a node for semantic processing of letters. Further investigations using O15-PET activation on normal subjects revealed that the left middle occipital gyrus (area 19 of Brodmann) and the posterior portion of the left inferior temporal gyrus (area 37 of Brodmann) are the cortical areas responsible for reading Japanese letters; the former serving for phonological reading and the latter for semantic reading. This duality of the neural circuit in processing letters was later applied to explain disturbances in reading English, and was finally accepted as a valid model for other alphabetic letter systems too.     

Temporal lobe epilepsy manifesting as alexia with agraphia for kanji

Alexia with agraphia results from lesions of the left angular gyrus or the left posteroinferior temporal lobe. In Japanese or Korean persons, lesions of the latter cause alexia with agraphia for ideograms. We describe a case of alexia with agraphia for kanji (Japanese ideograms) caused by temporal lobe epilepsy. After generalized convulsions, a 32-year-old man noticed that he had difficulty in reading and writing kanji, although he could read and write kana (Japanese syllabograms). His EEG showed frequent sharp waves on the left occipitotemporal region. MRI of the brain revealed a hyperintense lesion on the left hippocampus. Single-photon-emission computed tomography revealed marked hyperperfusion at the left hippocampus and the left posteroinferior temporal cortex. Antiepileptic drugs improved his alexia with agraphia for kanji. This is the first report describing alexia with agraphia for kanji caused by temporal lobe epilepsy.     

Pure alexia in Japanese and agraphia without alexia in kanji. The ability dissociation between reading and writing in kanji vs kana

A 60-year-old right-handed Japanese man with infarction of the left occipital lobe and inferior temporal gyrus initially showed pure alexia in kana and kanji. Later, though pure alexia in kana persisted, his kanji reading improved markedly, but with little improvement of kanji writing. We speculate that different pathways are involved in kanji reading and writing. Wernicke's area and its surrounding left middle temporal lobe might play the most important role for kanji reading when visual information is transmitted by any pathway. The pathway from Wernicke's area to the left occipital lobe via the middle and inferior temporal pathway may be indispensable for kanji writing. We postulate "agraphia without alexia in kanji" due to left inferior temporal subcortical damage.     

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.     

Pure alexia for kana. Characterization of alexia with lesions of the inferior occipital cortex

OBJECTIVE: To characterize reading impairments caused by lesions in the posterior occipital cortices. METHODS: We gave six patients with these lesions reading and writing tests and located a critical site for alexia using MRI and SPECT. RESULTS: The patients read three-character kana (Japanese syllabograms) nonwords, and five-character kana nonwords significantly or at a near significant level more poorly and slowly than normal subjects, whereas they read kanji (Japanese morphograms) almost correctly but more slowly. Letter-by-letter reading with a single-kana character identification impairment (in five patients), a word-length effect, kinesthetic facilitation, a lexicality effect, and minor to mild agraphia for kanji (in three patients) were observed. These deficits were characteristic of pure alexia. Alexia disappeared within a few months except in one patient who had extensive hypoperfusion in the left occipital lobe. A shared lesion was located in the left posterior fusiform/inferior occipital gyri (Area 18/19) on MRI, and there was blood flow reduction around this area on SPECT. This area coincided with the activation site for kana word covert reading in our previous study. CONCLUSIONS: These results suggest that pure alexia particularly for kana, or more generally pure alexia for letters, is caused by a lesion in the posterior inferior occipital cortex, characterized primarily by impaired kana character or letter identification, with relatively preserved kanji or word recognition.     

Differential reorganization of fusiform gyrus in two types of alexia after stroke

Lesions affecting the left fusiform gyrus (FG) commonly result in dyslexia and recovery largely depends on efficient reorganization of the reading network. We performed a follow-up fMRI study to elucidate the reorganization patterns of the FG according to the recovery of reading ability in two patients (MH with pure alexia and KM with alexia with agraphia) after stroke involving the left FG. Initially, MH was an effortful letter-by-letter (LBL) reader, and she improved to become a proficient LBL reader. The initial fMRI results showed scattered activation on occipital and ventral temporal cortex during reading, which was localized to right FG in the follow-up study. KM's severe alexia with agraphia did not improve, even after 6 months had passed since the onset of the alexia. The initial and follow-up fMRI results showed no significant activation in the bilateral FG or central higher language areas during word reading. Our results suggest that the reorganization of the FG is different according to the type of alexia and the amount of clinical recovery in each patient. Also, the successful reorganization of the visual component of reading in the right FG is responsible for the recovery of LBL reading in pure alexia.     

Differential Reorganization of Fusiform Gyrus in Two Types of Alexia after Stroke

Lesions affecting the left fusiform gyrus (FG) commonly result in dyslexia and recovery largely depends on efficient reorganization of the reading network. We performed a follow-up fMRI study to elucidate the reorganization patterns of the FG according to the recovery of reading ability in two patients (MH with pure alexia and KM with alexia with agraphia) after stroke involving the left FG. Initially, MH was an effortful letter-by-letter (LBL) reader, and she improved to become a proficient LBL reader. The initial fMRI results showed scattered activation on occipital and ventral temporal cortex during reading, which was localized to right FG in the follow-up study. KM's severe alexia with agraphia did not improve, even after 6 months had passed since the onset of the alexia. The initial and follow-up fMRI results showed no significant activation in the bilateral FG or central higher language areas during word reading. Our results suggest that the reorganization of the FG is different according to the type of alexia and the amount of clinical recovery in each patient. Also, the successful reorganization of the visual component of reading in the right FG is responsible for the recovery of LBL reading in pure alexia.     

Double dissociation of Hangul and Hanja reading in Korean patients with stroke

The orthographic system of the Korean language consists of both phonogram (Hangul) and ideogram (Hanja). We report 2 patients who revealed selective impairment in reading either of orthographies after the brain damages. YJ, a 67-year-old man, showed Broca's aphasia and severe apraxia of speech after a stroke in the left inferior parietal lobe. He demonstrated predominant difficulties in reading phonogram. KS, a 51-year-old woman, had an intracerebral hemorrhage in the left parietal lobe. She showed anomic aphasia and a selective impairment on reading ideogram. These findings support the notion that recognition of visual words is processed in different ways depending on the characteristics of orthographic systems.     

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.     

Alexia and agraphia with lesions of the angular and supramarginal gyri: Evidence for the disruption of sequential processing

ObjectiveTo determine the features of alexia or agraphia with a left angular or supramarginal gyrus lesion.MethodsWe assessed the reading and writing abilities of three patients using kanji (Japanese morphograms) and kana (Japanese syllabograms).ResultsPatient 1 showed kana alexia and kanji agraphia following a hemorrhage in the left angular gyrus and the adjacent lateral occipital gyri. Patient 2 presented with minimal pure agraphia for both kanji and kana after an infarction in the left angular gyrus involving part of the supramarginal gyrus. Patient 3 also showed moderate pure agraphia for both kanji and kana after an infarction in the left supramarginal and postcentral gyri. All three patients made transposition errors (changing of sequential order of kana characters) in reading. Patient 1 showed letter-by-letter reading and a word-length effect and made substitution errors (changing hiragana [one form of kana] characters in a word to katakana [another form of kana] characters and vice versa) in writing.ConclusionAlexia occurs as “angular” alexia only when the lesion involves the adjacent lateral occipital gyri. Transposition errors suggest disrupted sequential phonological processing from the angular and lateral occipital gyri to the supramarginal gyrus. Substitution errors suggest impaired allographic conversion between hiragana and katakana attributable to a dysfunction in the angular/lateral occipital gyri.     

Selective Kana agraphia: a case report

We present a Japanese man with selective Kana (phonogram) agraphia as a sequela of two cerebral infarctions in a part of the left angular gyrus and its adjoining posterior superior temporal gyrus and the left corona radiata. The agraphia of this patient showed the following features: (1) His writing difficulty was greater for Kana than for Kanji (ideogram) when a word was polysyllabic. (2) Kana errors consisted of perseveration and substitution with another letter, resulting in jargon agraphia. (3) This jargon agraphia often contained a correct number of letters for a target word. Based on these findings, we speculate that the basic defect of this agraphia lies in the process of converting an acoustic word image into a Kana motor grapheme sequence.     

Alexia without agraphia: an exemplary deficit, cherished by neuropsychologists

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.